Description
Intent
Policy
BlackBoxWarning
Guidelines
References
  
  
  
  
  
CrossReferences
ApplicableDrugs
  
  
110
  
7/1/2023Rx.01.5CommercialOyenusi, Oluwadamilola

Obstructive sleep apnea (OSA) is characterized by recurrent, functional collapse during sleep of the velopharyngeal and/or oropharyngeal airway, causing substantially reduced or complete cessation of airflow despite ongoing breathing efforts. This leads to intermittent disturbances in gas exchange and fragmented sleep. Snoring and wake-time sleepiness are common complaints of OSA. 

Narcolepsy is a disorder of sleep-wake control in which elements of sleep intrude into wakefulness and elements of wakefulness intrude into sleep. The result is the classic tetrad of chronic daytime sleepiness with varying amounts of cataplexy, hypnagogic hallucinations, and sleep paralysis. Patients with narcolepsy type 1 (narcolepsy with cataplexy) typically present with moderate to severe daytime sleepiness, transient facial weakness or falls triggered by joking or laugher (partial or complete cataplexy), or the inability to move for one or two minutes immediately after awakening or just before falling asleep. Patients with narcolepsy type 2 have excessive daytime sleepiness without cataplexy.   

Shift work disorder is a form of circadian sleep-wake rhythm disorders characterized by difficulty with sleep or wakefulness at times that are imposed by shifts running counter to the light-dark cycle. As a result, patients accumulate sleep debt and have increased risk of accidents, errors and other adverse health outcomes. 

Multiple sclerosis (MS) is an immune-mediated, inflammatory, neurodegenerative disease of the central nervous system. Fatigue is a characteristic finding in patients with MS. It is usually described as physical exhaustion that is unrelated to the amount of activity performed. 

Pitolisant (Wakix®) indicated for the treatment of excessive daytime sleepiness (EDS) in adult patients with narcolepsy. The mechanism of action of pitolisant in excessive daytime sleepiness in adult patients with narcolepsy is unclear. However, its efficacy could be mediated through its activity as an antagonist/inverse agonist at histamine-3 (H3) receptors.

Solriamfetol (Sunosi™) is indicated to improve wakefulness in adult patients with excessive daytime sleepiness associated with narcolepsy and obstructive sleep apnea (OSA). The mechanism of action of solriamfetol is unclear but it could be attributed to its activity as a dopamine and norepinephrine reuptake inhibitor (DNRI)

The intent of this policy is to communicate the medical necessity criteria for pitolisant (Wakix®), and solriamfetol (Sunosi™) as provided under the member's prescription drug benefit.

Excessive daytime sleepiness in narcolepsy (Type 2)

INITIAL CRITERIA: Solriamfetol (Sunosi™) is approved when ALL of the following are met:

  1. Diagnosis of excessive daytime sleepiness in narcolepsy (Type 2); and
  2. Prescribed by or in consultation with a neurologist or sleep specialist; and
  3. Diagnosis was confirmed by one of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Member is 18 years of age or older; and
  5. Inadequate response or inability to tolerate BOTH of the following:
    1. Modafinil or armodafinil; and
    2. One stimulant product (e.g., amphetamine, methylphenidate)

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Solriamfetol (Sunosi™) is re-approved when there is documentation of positive clinical response to therapy. 

Reauthorization duration: 2 years

INITIAL CRITERIA: Pitolisant (Wakix®) is approved when ALL of the following are met:

  1. Diagnosis of excessive daytime sleepiness in narcolepsy (Type 2); and
  2. Member is 18 years of age or older; and
  3. Diagnosis was confirmed by ONE of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Prescribed by or in consultation with a neurologist or sleep specialist; and
  5. Inadequate response or inability to tolerate ALL of the following:
    1. Modafinil or armodafinil; and
    2. One stimulant product (e.g., amphetamine, methylphenidate); and
    3. Sunosi®

 

Initial authorization duration: 12 months

 

REAUTHORIZATION CRITERIA: Pitolisant (Wakix®) is re-approved when ALL of the following are met:

  1. Documentation to support the efficacy associated with the current regimen; and
  2. Yearly evaluation by a neurologist or sleep specialist

 

Reauthorization duration: 12 months

_______________________________________________________________________________________________

Cataplexy with narcolepsy (Type 1)

 

INITIAL CRITERIA: Pitolisant (Wakix®) is approved when ALL of the following are met:

  1. Diagnosis of cataplexy with narcolepsy (Type 1); and
  2. Member is 18 years of age or older; and
  3. Diagnosis was confirmed by ONE of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Prescribed by or in consultation with a neurologist or sleep specialist

 

Initial authorization duration: 12 months

 

REAUTHORIZATION CRITERIA:  Pitolisant (Wakix®) is re-approved when ALL of the following are met:

  1. Documentation to support the efficacy associated with the current regimen (including but not limited to reduction in the frequency of cataplexy attacks or an improvement in the Epworth sleepiness scale); and
  2. Yearly evaluation by a neurologist or sleep specialist

 

Reauthorization duration: 12 months

_______________________________________________________________________________

Obstructive sleep apnea/hypopnea syndrome (OSAHS)

INITIAL CRITERIA: Solriamfetol (Sunosi™) is approved when ALL of the following are met:

  1. Diagnosis of obstructive sleep apnea/hypopnea syndrome (OSAHS); and
  2. Member is 18 years of age or older; and
  3. Prescribed by or in consultation with a sleep specialist; and
  4. Used as adjunct to standard therapy; and
  5. Inadequate response or inability to tolerate ONE of the following:
    1. Modafinil or armodafinil; or
    2. One stimulant product (e.g., amphetamine, methylphenidate)


​​Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Solriamfetol (Sunosi™) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years  

 



N/A

Lange R, Volkmer M, Heesen C, et al: Modafinil effects in multiple sclerosis patients with fatigue. J Neurol 2009 Apr;256(4):645-650. Accessed April 20, 2023.

Olek M, Narayan R, Frohman E, Frohman T. Symptom management of multiple sclerosis in adults. UpToDate website. Last updated February 2022. Available at: www.uptodate.com. Accessed April 20, 2023.

Scammell T. Clinical features and diagnosis of narcolepsy in adults. UpToDate website. Last updated July 2022. Available at: www.uptodate.com. Accessed April 20, 2023.

Schweitzer PK, Rosenberg R, Zammit GK, et al: Solriamfetol for excessive sleepiness in obstructive sleep apnea (TONES 3): a randomized controlled trial. Am J Respir Crit Care Med 2019; 199(11):1421-1431.Strohl K.

Kryger MH. Overview of obstructive sleep apnea in adults. UpToDate website. Last updated March 2023. Available at: www.uptodate.com. Accessed April 20, 2023.

Sunosi™ (solriamfetol) [prescribing information]. Palo Alto, CA: Jazz Pharmaceuticals, Inc. November 2022. Available at: https://pp.jazzpharma.com/pi/sunosi.en.USPI.pdf. Accessed April 20, 2023.

Thorpy MJ, Shapiro C, Mayer G, et al: A randomized study of solriamfetol for excessive sleepiness in narcolepsy. Ann Neurol 2019; 85(3):359-370.Wyatt J.

Goldstein CA. Overview of circadian sleep-wake rhythm disorders. UpToDate website. Last updated May 2022. Available at www.uptodate.com. Accessed April 20, 2023.

Wakix® (pitolisant hydrochloride) [prescribing information]. Plymouth Meeting, PA: Harmony Biosciences, LLC. December 2022. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=8daa5562-824e-476c-9652-26ceef3d4b0e. Accessed April 20, 2023.​



223/16/20233/16/20246/29/2023 5:48 AMNo presence informationsrv_ppsgw_NP
Rx.01.33 Off-Label Use
Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit


Generic NameBrand Name
PitolisantWakix®
SolriamfetolSunosi™
118
  
7/1/2023Rx.01.122CommercialOyenusi, Oluwadamilola

Multiple sclerosis (MS) is the most common autoimmune, inflammatory, demyelinating disease affecting the central nervous system (CNS).  An unknown stimulus causes the immune system to attack the myelin sheath that protects nerves, leading to symptoms such as weakness, numbness, vision loss, and gait disturbances. More than 2.3 million people are affected by MS worldwide.

Dalfampridine (Ampyra®) is indicated to improve walking in patients with MS.

Dalfampridine is a broad spectrum potassium channel blocker.  The mechanism by which dalfampridine exerts its therapeutic effect has not been fully elucidated.  In animal studies, dalfampridine has been shown to increase conduction of action potentials in demyelinated axons through inhibition of potassium channels.

The intent of this policy is to communicate the medical necessity criteria dalfampridine (Ampyra®) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Dalfampridine (Ampyra®) is approved when ALL the following are met:

  1. Diagnosis of multiple sclerosis; and
  2. Member is 18 years of age or older; and
  3. Member has difficulty walking; and
  4. Prescribed by or in consultation with a neurologist.

Initial authorization duration: 6 months

CONTINUATION CRITERIA: Dalfampridine (Ampyra®) is re-approved when BOTH of the following are met:

  1. Documentation of a 20% improvement in walking speed; and
  2. Prescribed by or in consultation with a neurologist

Continuation authorization duration: 2 years  

N/A

Ampyra® (dalfampridine) [package insert]. Ardsley, NY. Acorda Therapeutics, Inc. June 2022. Available from: https://ampyra.com/prescribing-information.pdf. Accessed April 18, 2023.

Olek, MJ. Mowry, E. Pathogenesis and epidemiology of multiple sclerosis. UpToDate. March 2023. Available at: https://www.uptodate.com/contents/pathogenesis-and-epidemiology-of-multiple-sclerosis. Accessed April 18, 2023.​​


143/16/20233/16/20246/29/2023 5:49 AMNo presence informationsrv_ppsgw_NP
Off-Label Use Rx.01.33
Brand Name Generic Name
Ampyra Dalfampridine
119
  
7/1/2023Rx.01.112CommercialOyenusi, Oluwadamilola

Restless legs syndrome (RLS), also known as Willis-Ekbom disease, is a condition that is characterized by a constant urge to move the legs due to uncomfortable sensations. The symptoms are usually worse during the night and are relieved temporarily by movement of the legs. The risk of RLS has been linked to other conditions such as anemia, kidney disease, multiple sclerosis, and diabetes. Treatment drug classes for RLS included dopaminergic agents, alpha-2-delta calcium channel ligands, among others.

Post herpetic neuralgia (PHN) is a complication of herpes zoster that is classified by severe pain in the areas where the rash was located, even after it has cleared. The pain usually will resolve within a few weeks or months but it can last for years in some cases, which lead to an interference with daily life.

Gabapentin is structurally related to the neurotransmitter gamma aminobutyric acid (GABA).  It has no effect on GABA binding, uptake, or degradation.  The exact mechanism by which gabapentin exerts its effect in restless leg syndrome (RLS) and postherpetic neuralgia (PHN) is unknown.  In vitro studies show that gabapentin binds with high affinity to the alpha-2-delta subunit of voltage activated calcium channels.  The relationship of this binding and gabapentin's therapeutic effect is not known.  Gabapentin enacarbil is a prodrug of gabapentin.

Gabapentin (Gralise®) is indicated for the management of postherpetic neuralgia (PHN).

Gabapentin enacarbil (Horizant®) is indicated for the treatment of moderate-to-severe primary RLS and PHN in adults.

Pregabalin is structurally related to the neurotransmitter gamma aminobutyric acid (GABA).  It has no effect on GABA binding, uptake, or degradation.  The exact mechanism by which pregabalin exerts its effects is unknown. Pregabalin binds with high affinity to the alpha-2-delta subunit of voltage activated calcium channels in central nervous system tissues. This binding may be involved in pregabalin's antinociceptive and antiseizure effects.

Pregabalin (Lyrica CR®) is indicated for the management of neuropathic pain associated with diabetic peripheral neuropathy and postherpetic neuralgia (PHN).

The intent of this policy is to communicate the medical necessity criteria for gabapentin (Gralise®/Horizant®) and pregabalin (Lyrica CR®) as provided under the member's prescription drug benefit.

Post-herpetic neuralgia

 

INITIAL CRITERIA: Gabapentin (Gralise®), gabapentin (Horizant®) or pregabalin (Lyrica CR®) is approved when all of the following are met:

  1. Diagnosis of post-herpetic neuralgia, and
  2. Member is 18 years of age or older; and
  3. Inadequate response or inability to tolerate ONE of the following
    1. Gabapentin; or
    2. Pregabalin

Initial Authorization duration: 2 years

 

REAUTHORIZATION CRITERIA: Gabapentin (Gralise®), or gabapentin enacarbil (Horizant®), or Pregabalin (Lyrica CR®) is re-approved when there is positive clinical response to therapy.

Reauthorization duration: 2 years 


 

Restless legs syndrome

 

INITIAL CRITERIA: Gabapentin enacarbil (Horizant®) is approved ALL of the following are met:​

  1. Diagnosis of moderate-to-severe primary restless legs syndrome; and
  2. Inadequate response or inability to tolerate BOTH of the following:
    1. Pramipexole; and
    2. Ropinirole; and
  3. ​​Member is 18 years of age or olderInitial Authorization duration: 2 years

 

REAUTHORIZATION CRITERIA Gabapentin enacarbil (Horizant®) is re-approved when there is positive clinical response to therapy.

Reauthorization duration: 2 years 


Diabetic peripheral neuropathy

 

INITIAL CRITERIA: Pregabalin (Lyrica CR®) is approved when ALL of the following are met:

  1. Diagnosis of neuropathic pain associated with diabetic peripheral neuropathy; and
  2. Inadequate response or inability to tolerate ONE of the following:
    1. Gabapentin; or
    2. Pregabalin; and
  3. Member is 18 years of age or older

 

Initial Authorization duration: 2 years

 

REAUTHORIZATION CRITERIA Pregabalin (Lyrica CR®) is re-approved when there is positive clinical response to therapy.

 

Reauthorization duration: 2 years



 

None

Gabapentin. Available at: http://www.micromedexsolutions.com. Accessed April 19, 2023.

Gralise® (gabapentin) [package insert]. Newark, CA. Depomed, Inc. April 2020. Available at: https://www.gralise.com/static/Gralise_full_Prescribing_Information_-_Printed-b9a705a9011191f3f63f0a16e352cf4d.pdf . Accessed April 19, 2023.  

Dopp JM, Phillips BG. Sleep–Wake Disorders. In: DiPiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey L. eds. Pharmacotherapy: A Pathophysiologic Approach, 10e New York, NY: McGra`w-Hill; .http://accesspharmacy.mhmedical.com.libproxy.temple.edu/content.aspx?bookid=1861&sectionid=134128126. Accessed April 19, 2023.

Horizant® (gabapentin enacarbil) [package insert].Santa Clara, CA. Xenoport, Inc. August 2022. Available at: https://www.horizant.com/pdf/Horizant_PrescribingInformation.pdf . Accessed April 19, 2023.

Pregabalin. Available at: http://www.micromedexsolutions.com. Accessed April 19, 2023.

Lyrica CR® (pregabalin hydrochloride) [package insert]. New York, NY. Pfizer. June 2020. Available from https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/209501s000lbl.pdf. Accessed April 19, 2023.

Shingles (Herpes Zoster). Centers for Disease Control and Prevention. https://www.cdc.gov/shingles/about/complications.html. Published January 19, 2019. Accessed April 18, 2023. ​




 

153/16/20233/16/20246/29/2023 5:49 AMNo presence informationsrv_ppsgw_NP
Off Label Use Rx.01.33
 

Brand NameGeneric Name
Gralise®gabapentin
Horizant®Gabapentin enacarbil
Lyrica CR®pregabalin controlled-release
130
  
7/1/2023Rx.01.84CommercialOyenusi, Oluwadamilola

Insomnia is one of the most common medical complaints, generating more than 5 million office visits per year in the United States.  Typical complaints include difficulty falling asleep, staying asleep, and variable sleep.  Insomnia is present when all of the following criteria are met:

  1. A complaint of difficulty initiating sleep, difficulty maintaining sleep, or waking up too early. In children or individuals with dementia, the sleep disturbance may manifest as resistance to going to bed at the appropriate time or difficulty in sleeping without caregiver assistance.
  2. The above sleep difficulty occurs despite adequate opportunity and circumstances for sleep.
  3. The impaired sleep produces deficits in daytime function.

Insomnia is classified as short-term, long-term, or other depending upon the duration and causes.
 
Non-24-hour sleep-wake rhythm disorder is characterized by failure of the circadian system to maintain stable alignment (called "entrainment") to the 24-hour day. As a result, the circadian system "free runs" and typically shifts to progressively later phase positions. The most common cause is blindness, as the daily light-dark cycle is the most powerful environmental cue for synchronizing the hypothalamic pacemaker to the 24-hour day.

Sleep problems are often significant in individuals diagnosed with Smith-Magenis Syndrome and include difficulty falling asleep, shortened sleep cycles, frequent and prolonged nocturnal awakening (altered rapid eye movement [REM] sleep), excessive daytime sleepiness, daytime napping, snoring, and bedwetting. Sleep problem appear to be due to an inversion of melatonin secretion. 
 Zolpidem tartrate (Ambien®) is a gamma-aminobutyric acid (GABA) A receptor positive modulator, is indicated for the short-term treatment of insomnia characterized by difficulties with sleep initiation.

Zolpidem tartrate (Ambien CR®) is a gamma-aminobutyric acid (GABA) A receptor positive modulator, is indicated for the treatment of insomnia characterized by difficulties with sleep onset and/or sleep maintenance.
  
Zolpidem tartrate (Edluar®) and (Zolpimist®) are gamma-aminobutyric acid (GABA) A receptor positive modulators indicated for the treatment of insomnia characterized by difficulties with sleep initiation.  


Zolpidem tartrate sublingual tablet (Intermezzo®) is a GABAA agonist indicated for use as needed for the treatment of insomnia when a middle-of-the-night awakening is followed by difficulty returning to sleep. 
 

Zolpidem tartrate sublingual tablet (Intermezzo®) is a GABAA agonist indicated for use as needed for the treatment of insomnia when a middle-of-the-night awakening is followed by difficulty returning to sleep.  

 

Tasimelteon (Hetlioz®/ Hetlioz LQ®oz®) is indicated for:

  1. Capsules: Treatment of non-24-Hour Sleep-Wake Disorder (Non-24) in adults and nighttime sleep disturbances in Smith-Magenis Syndrome (SMS) in patients 16 years of age and older
  2. Oral suspension: Treatment of nighttime sleep disturbances in SMS in pediatric patients 3 years to 15 years of age

Tasimelteon (Hetlioz®/ Hetlioz LQ®) is an agonist of melatonin receptors MT1 and MT2 (greater affinity for the MT2 receptor than the MT1 receptor). Activation of MT1 is thought to preferentially induce sleepiness, while MT2 receptor activation preferentially influences regulation of circadian rhythms.


 

The intent of this policy is to communicate the medical necessity criteria for zolpidem tartrate (Ambien® and Ambien CR®), zolpidem (Intermezzo®, Zolpimist®), zolpidem tartrate sublingual tablets (Edluar®), eszopiclone 3mg (Lunesta®) and tasimelteon (Hetlioz®/Hetlioz LQ®) as provided under the member's prescription drug benefit.​


 


Insomnia
 
INITIAL CRITERIA: Edluar® 5mg, Zolpimist®, Intermezzo® 1.75mg are approved when ALL of the following are met:

  1. Diagnosis of insomnia; and
  2. Member is 18 years of age or older; and
  3. One of the following:
    1. Inadequate response or inability to tolerate TWO of the following:
      1. Eszopiclone
      2. Zaleplon
      3. Zolpidem; OR
    2. Inability to swallow capsules/tablets (i.e., dysphagia, gastrointestinal [GI] tubes)

 
INITIAL CRITERIA: Any of the following high dose products (brand and generic): zolpidem tartrate (Ambien®) 10mg, zolpidem tartrate ER (Ambien® CR) 12.5mg, zolpidem tartrate SL (Intermezzo®) 3.5mg, eszopiclone (Lunesta®) 3mg or Edluar® 10mg is approved when ALL of the following are met:

  1. Diagnosis of insomnia; and
  2. Member is 18 years of age or older; and
  3. Patient has been counseled on practices associated with good sleep hygiene (e.g. avoiding stimulants such as caffeine, nicotine, alcohol close to bed time, etc.); and
  4. Inadequate response to a two-week trial of the lower dose; and
  5. For brand products with generic equivalents only, inadequate response or inability to tolerate a generic equivalent

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Edluar® 5mg, Zolpimist®, Intermezzo® 1.75mg, zolpidem tartrate (Ambien®) 10mg, zolpidem tartrate ER (Ambien® CR) 12.5mg, zolpidem tartrate SL (Intermezzo®) 3.5mg, eszopiclone (Lunesta®) 3mg, or Edluar® 10mg is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2years​

 Non-24-hour sleep Sleep-Wake Disorder


INITIAL CRITERIA Tasimelteon (Hetlioz®/Hetlioz LQ®) is approved when ALL of the following are met:

  1. Prescribed by or in consultation with a sleep specialist; and
  2. Diagnosis of Non-24-hour sleep Sleep-Wake Disorder; and
  3. Member is 18 years of age or older; and
  4. Member is totally blind; and
  5. Member has circadian period greater than 24 hours; and
  6. Inadequate response or inability to tolerate ramelteon (Rozerem®)

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Hetlioz®/Hetlioz LQ® is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years 


Smith-Magenis Syndrome

 

INITIAL CRITERIA Tasimelteon (Hetlioz®/Hetlioz LQ®) is approved when ALL of the following are met:

  1. Prescribed by or in consultation with a sleep specialist; and
  2. Diagnosis of Smith-Magenis Syndrome; and
  3. Member experiences nighttime sleep disturbances; and
  4. One of the following:
    1. For Hetlioz only, member is 16 years of age or older; or
    2. For Hetlioz LQ only, member is 3 to 15 years of age

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Hetlioz®/Hetlioz LQ® is re-approved when there is documentation of positive clinical response to therapy.

 
Reauthorization duration: 2 years

 



Complex Sleep Behaviors: zolpidem tartrate (Ambien® and Ambien CR®), zolpidem (Intermezzo®, Zolpimist®), zolpidem tartrate sublingual tablets (Edluar®), eszopiclone 3mg (Lunesta®)

 

Complex sleep behaviors including sleepwalking, sleep driving, and engaging in other activities while not fully awake have been reported following use of zolpidem tartrate and eszopiclone. Some of these events have resulted in serious injuries, including death. Discontinue immediately if a patient experiences a complex sleep behavior. 


 

Allain H, Bentue-Ferrer D, Breton SL, Polard E, Gandon JM. Preference of insomniac patients between a single dose of zolpidem 10 mg versus zaleplon 10 mg. Hum Psychopharmacol. 2003;18(5):369-374.

 

Ambien® (zolpidem tartrate) [prescribing information]. Bridgewater, NJ: sanofi-aventis; February 2020. Available at https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=c36cadf4-65a4-4466-b409-c82020b42452&type=display. Accessed April 20, 2023.

 

Ambien CR® (zolpidem tartrate) [package insert]. Bridgewater, NJ: sanofi-aventis; February 2020. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=404c858c-89ac-4c9d-8a96-8702a28e6e76&type=display. Accessed April 20, 2023.

 

Ancoli-Israel S, Walsh JK, Mangano RM, Fujimori M. Zaleplon, a novel nonbenzodiazepine hypnotic, effectively treats insomnia in elderly patients without causing rebound effects. Prim Care Companion J Clin Psychiatry. 1999;1(4):114-120.

 

Bonnet MH, Arand DL. Risk factors, comorbidities, and consequences of insomnia in adults. UpToDate. April 2022. Available at: https://www.uptodate.com/contents/overview-of-insomnia?source=search_result&search=insomnia&selectedTitle=2~150. Accessed April 20, 2023.

 

Elie R, Ruther E, Farr I, Emilien G, Salinas E. Sleep latency is shortened during 4 weeks of treatment with zaleplon, a novel nonbenzodiazepine hypnotic. Zaleplon Clinical Study Group. Clin Psychiatry. 1999;60(8):536-544.

 

Edluar® [package insert]. Somerset, NJ: Meda Pharmaceuticals, Inc.; August 2022. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=a32884d0-85b5-11de-8a39-0800200c9a66&type=display. Accessed April 20, 2023.

 

Fry J, Scharf M, Mangano R, Fujimori M. Zaleplon improves sleep without producing rebound effects in outpatients with insomnia. Zaleplon Clinical Study Group. Int Clin Psychopharm. 2000;15(3):141-152.

 

Hetlioz® (tasimelteon) [prescribing information]. Vanda Pharmaceuticals, Inc.: Washington, DC.; January 2023. Available at:https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ca4a9b63-708e-49e9-8f9b-010625443b90&type=display. Accessed April 20, 2023.

 

Intermezzo® (zolpidem tartrate) [prescribing information]. Stamford, CT: Purdue Pharma LP; August 2022. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=913b6cfe-1fb0-44a8-817a-26374bbce995&type=display. Accessed April 20, 2023.

 

Lunesta® (eszopiclone) [prescribing information]. Marlborough, MA: Sunovion Pharmaceuticals Inc. August 2019. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=fd047b2b-05a6-4d99-95cb-955f14bf329f. Accessed April 20, 2023.

 

Krystal A, Walsh JK, Laska E, et al. Sustained efficacy of eszopiclone over 6 months of nightly treatment: Results of a randomized, double-blind, placebo-controlled study in adults with chronic insomnia. Sleep. 2003;26(7):793-799.

 

Rosenberg RP. Eszopiclone, a novel non-benzodiazepine sedative-hypnotic: Efficacy and safety in a model of transient insomnia (Abstract). Sleep. 2002;25(suppl):A68.

 

Roth T, Krystal A, Walsh J, Roehrs T, Wessel T, Caron J. Twelve months of nightly eszopiclone treatment in patients with chronic insomnia: Assessment of long-term efficacy and safety (Abstract). Sleep. 2004;27(suppl):A260.

 

Goldstein C. Overview of circadian sleep-wake rhythm disorders. UpToDate. May 2022. Available at: https://www.uptodate.com/contents/overview-of-circadian-sleep-wake-rhythm-disorders?source=search_result&search=non%2024&selectedTitle=1~94. Accessed April 20, 2023.

 

Zammit GK, McNabb LJ, Caron J, Amato DA, Roth T. Efficacy and safety of eszopiclone across 6 weeks of treatment for primary insomnia. Curr Med Res Opin. 2004:20(12):1979-1991.  

 

Zolpimist® (zolpidem tartrate spray) [prescribing information]. Louisville, KY: Magna Pharmaceuticals; August 2019. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=b2e26965-30bd-4dca-869d-8a70b23b6d62&type=display. Accessed April 20, 2023.​


 



 



 

183/16/20233/16/20246/29/2023 5:50 AMNo presence informationsrv_ppsgw_NP

 

Off-Label Use Rx.01.33

Quantity Lvel Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit Rx.01.76


 

Brand NameGeneric Name
Ambien® [CR]zolpidem tartrate immediate and extended release (PA applies to generic 10mg IR and 12.5mg CR)
Edluar®zolpidem sublingual tablets
Zolpimist®zolpidem
Intermezzo® zolpidem
Lunesta®eszopiclone
Hetlioz®/Hetlioz LQ®
tasimelton


 
 
132
  
7/1/2023Rx.01.155CommercialOyenusi, Oluwadamilola

Diclofenac epolamine 1.3% (Flector®/Licart® Patch) is indicated for the topical treatment of acu​te pain due to minor strains, sprains, and contusions in adults and pediatric patients 6 years or older.

Generic diclofenac epolamine patch is  indicated for the topical treat​ment of acute pain due to minor strains, sprains, and contusions in adults

Diclofenac sodium 1.5% and 2% solution (Pennsaid) is indicated for the treatment of the pain of osteoarthritis of the knee(s).

Diclofenac reversibly inhibit cyclooxygenase 1 and 2 (COX-1 and COX-2) enzymes, which results in decreased formation of prostaglandin precursors. Diclofenac also has antipyretic, analgesic, and anti-inflammatory properties.

The intent of this policy is to communicate the medical necessity criteria for diclofenac epolamine 1.3% (Flector®/Licart® Patch), diclofenac sodium 2% solution, and diclofenac sodium (Pennsaid®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: diclofenac epolamine (Flector®/Licart®) patch, diclofenac sodium 2% solution or Pennsaid®  is approved when BOTH of the following are met:

  1. Diagnosis of pain; and
  2. ONE of the following:
    1. BOTH of the following:
      1. Inadequate response or inability to tolerate TWO of the following:
        1. Meloxicam
        2. Celecoxib
        3. Other oral NSAID; and
      2. Inadequate response or inability to tolerate ONE of the following:
        1. Generic topical diclofenac gel 1%
        2. Generic topical diclofenac solution 1.5%; or
    2. BOTH of the following:
      1. Member is 65 years of age or older; and
      2. Inadequate response or inability to tolerate ONE of the following
        1. Generic topical diclofenac gel 1%
        2. Generic topical diclofenac solution 1.5%

Initial authorization duration: 6 months

REAUTHORIZATION CRITERIA: diclofenac epolamine (Flector®/Licart®) patch, diclofenac sodium 2% solution or Pennsaid®  is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years ​

Diclofenac

Cardiovascular risk: Nonsteroidal anti-inflammatory drugs (NSAIDs) may cause an increased risk of serious cardiovascular thrombotic events, myocardial infarction (MI), and stroke, which can be fatal. This risk may increase with duration of use. Patients with cardiovascular disease or risk factors for cardiovascular disease may be at greater risk.

Diclofenac is contraindicated for the treatment of perioperative pain in the setting of coronary artery bypass graft (CABG) surgery.

GI risk: NSAIDs cause an increased risk of serious GI adverse events, including bleeding, ulceration, and perforation of the stomach or intestines, which can be fatal. These reactions can occur at any time during use and without warning symptoms. Elderly patients are at greater risk for serious GI events.

Galer BS, Rowbotham M, Perander J, et al. Topical diclofenac patch relieves minor sports injury pain: results of a multicenter controlled clinical trial. J Pain Symptom Manage. 2000 Apr;19(4):287-94.

Flector patch (diclofenac epolamine patch) [product information].  New York, NY: Pfizer, Inc. April 2021. Available at: http://labeling.pfizer.com/ShowLabeling.aspx?id=829. Accessed April 19, 2023.

Licart™ (diclofenac epolamine) topical system [prescribing information]. Parsippany, NJ: IBSA Pharma Inc.: April 2021. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=851c8b39-6056-4bbc-812e-b9b13977c1ac. Accessed April 19, 2023.

Pennsaid (diclofenac sodium topical solution) Lake Forest, IL. Horizon Pharma USA Inc. [product information]. January 2022. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=802cd382-443d-48e3-9c9d-fd946c73c79f&type=display. Accessed April 19, 2023.

Diclofenac epolamine patch prescribing information. North Wales, PA. Teva Pharmaceuticals USA, Inc. December 2019. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=bda1c346-56ff-4197-9a19-7f0cc7f9ebad. Accessed April 19, 2023. ​



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Off-Label Use Rx.01.33

Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit Rx.01.76
Brand NameGeneric Name
Flector®/Licart® PatchDiclofenac epolamine 1.3% patch
Pennsaid® SolutionDiclofenac sodium 1.5%, 2% solution
139
  
7/1/2023Rx.01.158CommercialOyenusi, Oluwadamilola

Allergic rhinitis is a persistent condition that typically requires ongoing therapy.  Allergen avoidance along with pharmacologic therapy with nasal corticosteroids and oral antihistamines are standard management.  Allergen immunotherapy is reserved for severe or refractory cases.  Sublingual immunotherapy involves the application of the allergen to the sublingual tissue.  In the case of Odactra™, Oralair®, Grastek®, and Ragwitek®, the allergen is in a sublingual tablet which is self-administered, after the first dose.

The exact mechanism of sublingual allergen immunotherapy has not been fully elucidated.  Allergen extracts given sublingually are primarily taken up by dendritic cells in the mucosa and presented to T cells in the draining lymph nodes. Likely mechanisms of action include activation of T regulatory cells and downregulation of mucosal mast cells. Within the oral and sublingual mucosa, effector cells, such as mast cells, are less numerous, which may account for the lower rates of adverse systemic allergic reactions seen with sublingual immunotherapy.

Timothy grass pollen allergen extract (Grastek®) is indicated as immunotherapy for the treatment of grass pollen-induced allergic rhinitis with or without conjunctivitis confirmed by positive skin test or in vitro testing for pollen-specific IgE antibodies for Timothy grass or cross-reactive grass pollens in persons 5 through 65 years of age.

Short ragweed pollen allergen extract (Ragwitek®) is indicated as immunotherapy for the treatment of short ragweed pollen-induced allergic rhinitis, with or without conjunctivitis, confirmed by positive skin test or in vitro testing for pollen-specific IgE antibodies for short ragweed pollen in individuals 5 through 65 years of age.

Sweet Vernal, Orchard, Perennial Rye, Timothy, and Kentucky Blue Grass mixed pollens allergen extract (Oralair®) is indicated as immunotherapy for the treatment of grass pollen-induced allergic rhinitis with or without conjunctivitis confirmed by positive skin test or in vitro testing for pollen-specific IgE antibodies for any of the five grass species contained in this product in persons 10 through 65 years of age. 

Dermatophagoides farinae or Dermatophagoides pteronyssinus house dust mite allergen extract (Odactra™) is indicated as immunotherapy for house dust mite (HDM)-induced allergic rhinitis, with or without conjunctivitis, confirmed by positive in vitro testing for IgE antibodies to Dermatophagoides farinae or Dermatophagoides pteronyssinus house dust mites, or by positive skin testing to licensed house dust mite allergen extracts. Odactra™ is approved for use in individuals12 through 65 years of age.

The intent of this policy is to communicate the medical necessity criteria for house dust mite allergen extract (Odactra™), grass pollen allergen extract-5 grass (Oralair®), grass pollen allergen extract-timothy grass (Grastek®), and short ragweed pollen allergen extract (Ragwitek®) as provided under the member’s prescription drug benefit.

INITIAL CRITERIA:

Odactra™, Oralair®, Grastek® or Ragwitek® is approved when ALL of the following are met:

  1. FDA approved indication; and
  2. Patient has a positive skin test or in vitro test for ONE of the listed pollen-specific IgE antibodies:
    1. Timothy Grass or cross-reactive grass pollens (GRASTEK® only); or
    2. Any of the five grass species including sweet vernal, orchard perennial rye, timothy or Kentucky blue grass mixed pollens (ORALAIR® only); or
    3. Short ragweed pollen (RAGWITEK® only); or
    4. Dermatophagoides farina or Dermatophagoides pteronyssinus house dust mites (ORDACTRA™ only); and
  3. Prescribed by or in consultation with allergist or immunologist and
  4. Patient does not have any of the following:
    1. Severe, unstable or uncontrolled asthma; or
    2. History of eosinophilic esophagitis; and
  5. Patient has had an inadequate response or inability to tolerate BOTH of the following:
    1. Intranasal corticosteroid; and
    2. Antihistamine
Initial Authorization duration: 1 year 

REAUTHORIZATION CRITERIA

Odactra™, Oralair®, Grastek® or Ragwitek® is re-approved when ALL of the following are met:

  1. Use in the age group supported by FDA labeling; and
  2. Prescribed by or in consultation with allergist or immunologist; and
  3. Patient has experienced improvement in the symptoms of their allergic rhinitis OR a decrease in the number of medications needed to control allergy symptoms

Reauthorization duration: 1 year 

Severe allergic reactions:

Odactra™, Grastek®, Oralair® and Ragwitek® can cause life-threatening allergic reactions such as anaphylaxis and severe laryngopharyngeal restriction. 

Do not administer Odactra™, Grastek®, Oralair® and Ragwitek® to patients with severe, unstable or uncontrolled asthma. Observe patients in the office for at least 30 minutes following the initial dose.

Prescribe auto-injectable epinephrine, instruct and train patients on its appropriate use, and instruct patients to seek immediate medical care upon its use.

Odactra™, Grastek®, Oralair® and Ragwitek® may not be suitable for patients with certain underlying medical conditions that may reduce their ability to survive a serious allergic reaction.

Odactra™, Grastek®, Oralair® and Ragwitek® may not be suitable for patients who may be unresponsive to epinephrine or inhaled bronchodilators, such as those taking beta-blockers. 

 

Creticos PS. Sublingual immunotherapy (SCIT) for allergic rhinoconjunctivitis and asthma. UpToDate. Available at: http://www.uptodate.com/contents/sublingual-immunotherapy-for-allergic-rhinoconjunctivitis-and-asthma. Accessed April 17, 2023.

De Shazo RD, Kemp SF. Pharmacotherapy of allergic rhinitis. UpToDate. Available at: https://www.uptodate.com/contents/pharmacotherapy-of-allergic-rhinitis?search=pharmacotherapy-of-allergic-rhinitis.&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1. Accessed April 17, 2023.

Grastek® (Timothy grass pollen allergen extract) [package insert]. Whitehouse Station NJ. Merck and Co, Inc. December 2019. Accessed April 17, 2023.

Odactra™ (and house dust mite allergen extract) [package insert]. Whitehouse Station NJ. Merck and Co, Inc. January 2023. Available from: https://www.odactra.com/assets/pdf/odactra-full-pi.pdf. Accessed May 16, 2022.

Oralair® (Sweet Vernal, Orchard, Perennial Rye, Timothy, and Kentucky Blue Grass mixed pollens allergen extract) [package insert]. Lenoir NC. Greer Laboratories, Inc. November 2018. Accessed April 17, 2023.

Ragwitek® (short ragweed pollen allergen extract) [package insert]. Whitehouse Station NJ. Merck and Co, Inc. April 2021. Accessed April 17, 2023. ​



 

133/16/20233/16/20246/29/2023 5:51 AMNo presence informationsrv_ppsgw_NP
 Off- Label Use Rx.01.33


Brand Name

Generic Name

Grastek®

grass pollen allergen extract-timothy grass 

Oralair®

grass pollen allergen extract-5 grass

Ragwitek®

short ragweed pollen allergen extract

​Odactra™ ​house dust mite allergen extract
145
  
7/1/2023Rx.01.164CommercialOyenusi, Oluwadamilola

Interstitial lung diseases (ILDs) are a heterogenous group of disorders that are characterized by the inflammation and scarring of the lungs. Some ILDs have known causes and some are idiopathic. The treatment choices and prognosis vary among the different causes and types of ILDs.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, incurable fibrotic disorder of the lower respiratory tract that typically affects adults over the age of 40.  IPF is characterized by varying degrees of fibrosis, collagen deposits, and distortion of the pulmonary architecture. Although the specific initiating factor(s) leading to IPF are unknown, lung injury progresses due to the interaction of growth factors, cytokines, and other mediators, leading to fibroblast proliferation and excessive extracellular matrix deposition in the lungs.  Pharmacologic treatments are limited. Prior to the FDA approval of pirfenidone (Esbriet®) and nintedanib (Ofev®) in October 2014, no medications were approved for the treatment of IPF. Traditional approaches have included various anti-inflammatory and immunosuppressive agents; however, these approaches do not seem to be effective and are no longer considered part of routine maintenance care. Early trials of agents with antifibrotic properties were disappointing. Thus, treatment has predominantly been limited to supportive care, including oxygen therapy and pulmonary rehabilitation. Lung transplantation is also an option for selected patients. Five-year survival is approximately 20-30%

Systemic Sclerosis is a rare and heterogenous autoimmune disease characterized by immune dysregulation, microvascular damage, and organ fibrosis. Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis that tends to occur early in the course of disease.

Nintedanib (Ofev®) inhibits multiple receptor tyrosine kinases and nonreceptor tyrosine kinases, including platelet-derived growth factor (PDGFR alpha and PDGFR beta), fibroblast growth factor receptor (FGFR1, FGFR2, FGFR3), vascular endothelial growth factor (VEGFR1, VEGFR2, and VEGFR3), and Fms-like tyrosine kinase-3 (FLT3). Nintedanib binds competitively to the adenosine triphosphate (ATP) binding pocket of these receptors and blocks the intracellular signaling which is crucial for the proliferation, migration, and transformation of fibroblasts. Nintedanib (Ofev®) is indicated for the treatment of idiopathic pulmonary fibrosis and systemic sclerosis associated with Interstitial Lung Disease (SSc-ILD).

The precise mechanisms of action for pirfenidone (Esbriet®) have not been fully elucidated; however, pirfenidone may exert antifibrotic properties by decreasing fibroblast proliferation and the production of fibrosis-associated proteins and cytokines; may decrease the formation and accumulation of extracellular matrix (i.e., collagen) in response to transforming growth factor beta and platelet-derived growth factor. Pirfenidone is also believed to exert anti-inflammatory properties by decreasing the accumulation of inflammatory cells resulting from a variety of stimuli.  Pirfenidone (Esbriet®) is indicated for the treatment of idiopathic pulmonary fibrosis.

Nintedanib and pirfenidone appear to slow disease progression.  Neither medication is a cure for IPF.

The intent of this policy is to communicate the medical necessity criteria for pirfenidone (Esbriet®) and nintedanib (Ofev®) as provided under the member's prescription drug benefit.
Idiopathic Pulmonary Fibrosis (IPF)

INITIAL CRITERIA: Pirfenidone (Esbriet®) or Nintedanib (Ofev®) is approved when ALL of the following are met:

  1. Diagnosis of Idiopathic Pulmonary Fibrosis (IPF); and
  2. Member is 18 years of age or older; and
  3. Diagnosis was confirmed by BOTH of the following:
    1. High resolution CT scan or biopsy; and
    2. Member does not have evidence or suspicion of an alternative interstitial lung disease diagnosis; AND
  4. Prescribed by or in consultation with a pulmonologist or lung transplant specialist
Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA:
Pirfenidone (Esbriet®) or Nintedanib (Ofev®) is re-approved when ALL of the following are met:
  1. Documentation of positive clinical response to therapy (e.g., member has experienced stabilization from baseline or a less than 10% decline in forced vital capacity (FVC)); and
  2. Member has not experienced AST or ALT elevations greater than 5 times the upper limit of normal (ULN) or greater than 3 times ULN with signs or symptoms of severe liver damage; and
  3. The member is not actively smoking

Reauthorization duration: 12 months​

 

Systemic Sclerosis Associated Interstitial Lung Disease (SSc-ILD)

INITIAL CRITERIA: Nintedanib (Ofev®) is approved when ALL of the following are met:

  1. Diagnosis of Systemic Sclerosis Associated Interstitial Lung Disease (SSc-ILD); and
  2. Member is 18 years of age or older; and
  3. Diagnosis was confirmed by BOTH of the following:
    1. High resolution CT scan or biopsy; and
    2. Member does not have evidence or suspicion of an alternative interstitial lung disease diagnosis; and
  4. Prescribed by or in consultation with a pulmonologist or lung transplant specialist

Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Nintedanib (Ofev®) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy (e.g., member has experienced stabilization from baseline or a less than 10% decline in forced vital capacity (FVC)); and
  2. Member has not experienced AST or ALT elevations greater than 5 times the upper limit of normal (ULN) or greater than 3 times ULN with signs or symptoms of severe liver damage; and
  3. The member is not actively smoking

Reauthorization duration: 12 months

 

Chronic Fibrosing Interstitial Lung Disease (ILDs) with a progressive phenotype

INITIAL CRITERIA: Nintedanib (Ofev®) is approved when ALL of the following are met:

  1. Diagnosis of Chronic Fibrosing Interstitial Lung Disease (ILDs) with a progressive phenotype; and
  2. Member is 18 years of age or older; and
  3. Disease has a progressive phenotype as observed by one of the following:
    1. Decline of forced vital capacity (FVC); or
    2. Worsening of respiratory symptoms; or
    3. Increased extent of fibrosis seen on imaging; and
  4. Diagnosis was confirmed by BOTH of the following:
    1. High resolution CT scan or biopsy; and
    2. Member does not have evidence or suspicion of an alternative interstitial lung disease diagnosis; and
  5. Prescribed by or in consultation with a pulmonologist or lung transplant specialist

Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Nintedanib (Ofev®) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy (e.g. member has experienced stabilization from baseline or a less than 10% decline in forced vital capacity (FVC)); and
  2. Member has not experienced AST or ALT elevations greater than 5 times the upper limit of normal (ULN) or greater than 3 times ULN with signs or symptoms of severe liver damage; and
  3. The member is not actively smoking

Reauthorization duration: 12 months

None

Esbriet® (pirfenidone) [prescribing information]. South San Francisco, CA. Genentech, Inc. February 2023. Available at: https://www.gene.com/download/pdf/esbriet_prescribing.pdf . Accessed April 19, 2023.

King TE. Treatment of idiopathic pulmonary fibrosis. UpToDate. March 2023. Available at: https://www.uptodate.com/contents/treatment-of-idiopathic-pulmonary-fibrosis?source=see_link&sectionName=MEDICAL%20THERAPIES&anchor=H13191574#H13191574. Accessed April 19, 2023.

Ofev® (nintedanib) [prescribing information]. Ridgefield, CT. Boehringer Ingelheim. October 2022. Available at: https://docs.boehringer-ingelheim.com/Prescribing%20Information/PIs/Ofev/ofev.pdf . Accessed April 19, 2023.

Raghu G, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis: An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015 Jul;192:e3-e19. DOI: 10.1164/rccm.201506-1063ST


123/16/20233/16/20246/29/2023 5:51 AMNo presence informationsrv_ppsgw_NP

Off-Label Use Rx.01.33

Brand nameGeneric name
Ofev®nintedanib
Esbriet®pirfenidone
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7/1/2023Rx.01.200CommercialOyenusi, Oluwadamilola

Muscular dystrophies are an inherited, X-linked group of progressive myopathic disorders, resulting from defects in the dystrophin genes, the genes responsible for normal muscle function.  Duchenne muscular dystrophy (DMD) is the dystrophinopathy associated with the most severe clinical symptoms.  Becker muscular dystrophy (BMD) has a milder clinical course than DMD.  An intermediate group may be classified as mild DMD or severe BMD.

Duchenne muscular dystrophy is a type of muscular dystrophy of an inherited group of progressive myopathic disorders resulting from defects in a number of genes required for normal muscle function. Muscle weakness is the primary symptom. Duchenne muscular dystrophy is characterized by weakness, elevated creatine kinase and transaminases, growth delay, cardiomyopathy, orthopedic complications as well as cognitive and behavioral disorders. 

Deflazacort (Emflaza®) is a corticosteroid prodrug, whose active metabolite, 21-desDFZ acts through the glucocorticoid receptor to exert anti-inflammatory and immunosuppressive effects. The precise mechanism by which deflazacort exerts its therapeutic effects in patients with DMD is unknown.

Deflazacort (Emflaza®) is indicated for the treatment of DMD in patients 2 years of age and older.


 

The intent of this policy is to communicate the medical necessity criteria for deflazacort (Emflaza®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Deflazacort (Emflaza®) is approved when ALL of the following are met:

  1. Diagnosis of Duchenne muscular dystrophy (DMD) confirmed by ONE of the following:
    1. Mutation of the dystrophin gene; or
    2. Absence of the dystrophin protein confirmed by muscle biopsy; and
  2. Member is 2 years of age or older; and
  3. Prescribed by or in consultation with a neurologist who has experience treating children; and
  4. Member has had an inadequate response or inability to tolerate prednisone or prednisolone given at a dose of 0.75 mg/kg/day or 10 mg/kg/weekend; and
  5. Dose will not exceed 0.9 milligrams per kilogram of body weight once daily 

Initial authorization duration: 12 months


 

REAUTHORIZATION CRITERIA: Deflazacort (Emflaza®) is re-approved when BOTH of the following are met:

A. Member has experienced a benefit from therapy (e.g., improvement or preservation of muscle strength); and

B. Dose will not exceed 0.9 milligrams per kilogram of body weight once daily

 

Reauthorization duration: 12 months


 

​N/A

Darras BT. Duchenne and Becker muscular dystrophy: Glucocorticoid and disease-modifying treatment. UpToDate September 2022.  Available at: https://www.uptodate.com/contents/duchenne-and-becker-muscular-dystrophy-management-and-prognosis. Accessed April 18, 2023.

Emflaza® [package insert]. Northbrook, IL. Marathon Pharmaceuticals, LLC. June 2021. Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/208684s000,208685s000lbl.pdf. Accessed April 18, 2023.


Mesa LE, Dubrovsky AL, Corderi J, et al. Steroids in Duchenne muscular dystrophy--deflazacort trial. Neuromuscul Disord 1991; 1:261-6.

Darras BT. Duchenne and becker muscular dystrophy: clinical features and diagnosis. UpToDate website. Last updated June 2022. Available at: http://www.uptodate.com/. Accessed April 18, 2023.  ​




83/16/20233/16/20246/29/2023 5:52 AMNo presence informationsrv_ppsgw_NP

​Off-Label Use Rx.01.33

Brand Name

Generic Name

Emflaza®

Deflazacort


 

163
  
7/1/2023Rx.01.209CommercialOyenusi, Oluwadamilola

Lennox-Gastaut syndrome: A form of severe epilepsy that begins in childhood. It is characterized by multiple types of seizures and intellectual disability. People with Lennox-Gastaut syndrome begin having frequent seizures in early childhood, usually between ages 3 and 5 years.

Dravet syndrome (DS): A severe form of epilepsy which appears during the first year of life characterized by frequent, prolonged seizures often triggered by high body temperature (hyperthermia), developmental delay, speech impairment, loss of full control of bodily movements, low muscle tone, sleep disturbances, and other health problems.

Epidiolex ® (cannabidiol (CBD)); the exact mechanism by which CBD produces its anticonvulsant effects is unknown. CBD may exert a cumulative anticonvulsant effect, modulating a number of endogenous systems including, but not limited to, neuronal inhibition (synaptic and extrasynaptic GABA channels), modulation of intracellular calcium (TRPV, VDAC, GPR55), and possible anti-inflammatory effects (adenosine).

Diacomit® (stripentol); the mechanism of action by which stripentol exerts its anticonvulsant effect in humans is unknown. Possible mechanisms of action include direct effects mediated through the gamma-aminobutyric acid (GABA)A receptor and indirect effects involving inhibition of cytochrome P450 activity with resulting increase in blood levels of clobazam and its active metabolite. 

Onfi® (clobazam); The exact mechanism of action for clobazam, a 1,5-benzodiazepine, is not fully understood but is thought to involve potentiation of GABAergic neurotransmission resulting from binding at the benzodiazepine site of the GABAA receptor.​​​​


Banzel® (rufinamide); The precise mechanism(s) by which rufinamide exerts its antiepileptic effect is unknown. The results of in vitro studies suggest that the principal mechanism of action of rufinamide is modulation of the activity of sodium channels and, in particular, prolongation of the inactive state of the channel. Rufinamide (≥ 1 μM) significantly slowed sodium channel recovery from inactivation after a prolonged prepulse in cultured cortical neurons, and limited sustained repetitive firing of sodium-dependent action potentials (EC50 of 3.8 μM).

Fintepla® (fenfluramine): The mechanisms by which fenfluramine exerts its therapeutic effects in the treatment of seizures associated with Dravet syndrome are unknown. Fenfluramine and the metabolite, norfenfluramine, increase extracellular levels of serotonin through interaction with serotonin transporter proteins, and exhibit agonist activity at serotonin 5HT-2 receptors.

Sabril® (vigabatrin): The precise mechanism of vigabatrin's anti-seizure effect is unknown, but it is believed to be the result of its action as an irreversible inhibitor of gamma-aminobutyric acid transaminase (GABA-T), the enzyme responsible for the metabolism of the inhibitory neurotransmitter GABA. This action results in increased levels of GABA in the central nervous system.

Epidiolex ® is indicated for the treatment of seizures associated with Lennox-Gastaut syndrome, Dravet syndrome, or tuberous sclerosis complex in patients 1 year of age and older.

Diacomit® is indicated for the treatment of seizures associated with Dravet syndrome in patients 6 months of age and older and weighing 7 kg or more taking clobazam. There are no clinical data to support the use of Diacomit® as monotherapy in Dravet syndrome

Onfi® and Sympazan® are indicated for adjunctive treatment of seizures associated with Lennox-Gastaut syndrome (LGS) in patients2 years of age or older.

Banzel® indicated for adjunctive treatment of seizures associated with Lennox-Gastaut Syndrome (LGS) in pediatric patients 1 year of age and older, and in adults.

Fintepla® (fenfluramine) is indicated for the treatment of seizures associated with Dravet syndrome  and Lennox-Gastaut syndrome in patients 2 years of age and older.

Sabril® is indicated as monotherapy for pediatric patients with infantile spasms 1 month to 2 years of age for whom the potential benefits outweigh the potential risk of vision loss and as adjunctive therapy for adults and pediatric patients 2 years of age and older with refractory complex partial seizures who have inadequately responded to several alternative treatments and for whom the potential benefits outweigh the risk of vision loss

 The intent of this policy is to communicate the medical necessity criteria for Epidiolex ® (cannabidiol),Diacomit® (stiripentol), Onfi®, Sympazan® (clobazam), Banzel® (rufinamide), Fintepla® (fenfluramine), and Vigabatrin (Sabril®) as provided under the member's prescription drug benefit. 

Dravet Syndrome

INITIAL CRITERIA: Cannabidiol (Epidiolex) is approved when ALL of the following are met:

  1. Member is 1 years of age or older; and
  2. Baseline CBC, serum transaminases and total bilirubin obtained prior to initiating therapy; and
  3. Diagnosis of Dravet syndrome; and
  4. Inadequate response or inability to tolerate ONE of the following:
    1. Clobazam*; or
    2. Valproic acid; or
    3. Levetiracetam; o
    4. Topiramate; and
  5. Documentation of concurrent use with additional anti-epileptic(s); and
  6. Prescribed by or in consultation with a neurologist  

INITIAL CRITERIA: Stiripentol (Diacomit®) is approved when ALL of the following are met:

  1. Diagnosis of seizures associated with Dravet syndrome; and
  2. Member is 6 months of age or older and weighing 7kg or more; and
  3. Used in combination with clobazam*; and
  4. Prescribed by or in consultation with a neurologist

INITIAL CRITERIA: Clobazam (Onfi®, Sympazan®) tablets, films, or oral suspension is approved when ALL of the following are met:

  1. Member is 2 years of age and older; and
  2. Diagnosis of seizures associated with Dravet Syndrome (DS); and
  3. Used as adjunctive therapy; and
  4. Prescribed by or in consultation with a neurologist; and
  5. For brand Onfi® and brand Sympazan® only, inadequate response or inability to tolerate generic clobazam tablets or oral suspension 

INITIAL CRITERIA: Fenfluramine (Fintepla®) is approved when ALL of the following are met:

  1. Diagnosis of Dravet Syndrome; and
  2. Member is 2 years of age or older; and
  3. Inadequate response or inability to tolerate ONE of the following:
    1. Clobazam*; or
    2. Valproic acid; or
    3. Divalproex sodium; or
    4. Topiramate; or
    5. Levetiracetam; and
  4. Prescribed by or in consultation with a neurologist

Initial authorization duration: 2 years 

CONTINUATION CRITERIA: Cannabidiol (Epidiolex®), Stiripentol (Diacomit®), clobazam (Onfi®, Sympazan®), or fenfluramine (Fintepla®) is re-approved when ALL of the following are met:

  1. Documentation of reduction in seizure activity or intensity; and
  2. Stiripentol (Diacomit®) only, used in combination with clobazam*; and
  3. Prescribed by or in consultation with a neurologist

Continuation authorization duration: 2 years

Lennox-Gastaut Syndrome (LGS)

INITIAL CRITERIA: Cannabidiol (Epidiolex®) is approved when ALL of the following are met:

  1. Member is 1 years of age or older; and
  2. Baseline CBC, serum transaminases and total bilirubin obtained prior to initiating therapy; and
  3. Diagnosis of Lennox-Gastaut syndrome (LGS); and
  4. Inadequate response or inability to tolerate ONE of the following:
      1. Valproic acid; or 
      2. Lamotrigine; or
      3. Topiramate; or
      4. Felbamate; or
      5. Rufinamide*; or
      6. Clobazam*; and
  5. Documentation of concurrent use with additional anti-epileptic(s); and
  6. Prescribed by or in consultation with a neurologist

INITIAL CRITERIA: Rufinamide (Banzel®) tablet or suspension is approved when ALL of the following are met:

  1. Diagnosis of seizures associated with Lennox-Gastaut Syndrome (LGS); and
  2. Used as adjunctive therapy; and
  3. Member is 1 year of age or older; and
  4. Inadequate response or inability to tolerate ONE of the following generics:
      1. Valproic acid; or
      2. Lamotrigine; or
      3. Topiramate; or
      4. Felbamate; or
      5. Clobazam*; and
  5. Prescribed by or in consultation with a neurologist; and
  6. For rufinamide (Banzel®) suspension only; ONE of the following:
    1. Drug will be administered via nasogastric or gastronomy tube; or
    2. Member is unable to swallow an intact capsule or tablet
INITIAL CRITERIA: Fenfluramine (Fintepla®) is approved when ALL of the following are met: 

  1. Diagnosis of seizures associated with Lennox-Gastaut Syndrome (LGS); and 
  2. Member is 2 years of age or older; and 
  3. ​Inadequate response or inability to tolerate ONE of the following generics: 
    1. Valproic acid; or 
    2. Lamotrigine; or 
    3. Topiramate; or 
    4. Felbamate; or 
    5. Clobazam; and
  4. Prescribed by or in consultation with a neurologist
Initial authorization duration: 2 years

INITIAL CRITERIA: Clobazam (Onfi®, Sympazan®) tablets, films or oral suspension is approved when ALL of the following are met: 
  1. Member is 2 years of age and older; and 
  2. Diagnosis of seizure associated with Lennox-Gastaut Syndrome; and 
  3. Prescribed by or in consultation with a neurologist; and 
  4. For Brand Onfi® and brand Sympazan® only, inadequate response or inability to tolerate generic clobazam tablets or oral suspension 

Initial authorization duration: 2 years


CONTINUATION CRITERIA: Cannabidiol (Epidiolex®) , clobazam (Onfi®, Sympazan®), rufinamide (Banzel®), or fenfluramine (Fintepla®) is re-approved when there is documentation of reduction in seizure activity or intensity.

Continuation authorization duration: 2 years

Seizures associated with tuberous sclerosis complex

INITIAL CRITERIA: Cannabidiol (Epidiolex®) is approved when ALL of the following are met:

  1. Member is 1 years of age or older; and
  2. Baseline CBC, serum transaminases and total bilirubin obtained prior to initiating therapy; and
  3. Diagnosis of seizures associated with tuberous sclerosis complex; and
  4. Inadequate response or inability to tolerate ONE of the following:
    1. Vigabatrin*; or
    2. Carbamazepine; or
    3. Oxcarbazepine; and
  5. Documentation of concurrent use with additional anti-epileptic(s); and
  6. Prescribed by or in consultation with a neurologist

Initial authorization duration: 2 years

CONTINUATION CRITERIA: Cannabidiol (Epidiolex®) is re-approved when there is documentation of reduction in seizure activity or intensity.

Continuation authorization duration: 2 years

Refractory complex partial seizure or infantile spasms

INITIAL CRITERIA: Vigabatrin (Sabril®) is approved when ALL of the following are met:

  1. One of the following:
    1. BOTH of the following:
      1. Diagnosis of refractory complex partial seizures as adjunctive therapy; and
      2. Member is 2 years of age or older; or
    2. BOTH of the following:
      1. Diagnosis of infantile spasms; and
      2. Member is 1 month to 2 years of age; and
  2. For brand Sabril® only, inadequate response or inability to tolerate generic vigabatrin or vigadrone; and
  3. Prescribed by or in consultation with a neurologist

Initial authorization duration: 2 years 

CONTINUATION CRITERIA: Vigabatrin (Sabril®) is re-approved when there is documentation of reduction in seizure activity of intensity

Continuation authorization duration: 2 years

*Please note: prior authorization required

Onfi®, Sympazan® (clobazam): Concomitant use of benzodiazepines and opioids may result in profound sedation, respiratory depression, coma, and death. Reserve concomitant prescribing of these drugs for patients for whom alternative treatment options are inadequate. Limit dosages and durations to the minimum required. Follow patients for signs and symptoms of respiratory depression and sedation. The use of benzodiazepines, including ONFI and SYMPAZAN, , exposes users to risks of abuse, misuse, and addiction, which can lead to overdose or death. Before prescribing ONFI and SYMPAZAN,  and throughout treatment, assess each patient's risk for abuse, misuse, and addiction. Abrupt discontinuation or rapid dosage reduction of ONFI and SYMPAZAN,  after continued use may precipitate acute withdrawal reactions, which can be life-threatening. To reduce the risk of withdrawal reactions, use a gradual taper to discontinue ONFI and SYMPAZAN,  or reduce the dosage.

Fintepla® (fenfluramine): There is an association between serotonergic drugs with 5-HT2B receptor agonist activity, including fenfluramine (the active ingredient in Fintepla®), and valvular heart disease and pulmonary arterial hypertension. Echocardiogram assessments are required before, during, and after treatment with Fintepla®. Fintepla® is available only through a restricted program called the Fintepla® REMS.

Sabril® (vigabatrin): SABRIL causes progressive and permanent bilateral concentric visual field constriction in a high percentage of patients. In some cases, SABRIL may also reduce visual acuity. Risk increases with total dose and duration of use, but no exposure to SABRIL is known that is free of risk of vision loss. Risk of new and worsening vision loss continues as long as SABRIL is used, and possibly after discontinuing SABRIL. Unless a patient is formally exempted, periodic vision assessment is required for patients on SABRIL. However, this assessment cannot always prevent vision damage. SABRIL can cause permanent vision loss. SABRIL is available only through a restricted program called the SHARE Program.​

Banzel® (rufinamide) [prescribing information]. Woodcliff Lake, NJ: Eisai Inc.; December 2021. Available from: https://www.banzel.com/~/media/Files/BanzelPatient/BanzelPI.pdf. Accessed April 20, 2023.

 

Diacomit® (stripentol) [package insert]. Beauvais, France. Biocodex.  July 2022. Available from: https://www.diacomit.com/pdf/PI-Diacomit-2018.pdf. Accessed April 20, 2023.

 

“Dravet Syndrome." Genetic and Rare Diseases Information Center, U.S. Department of Health and Human Services, December 2016, rarediseases.info.nih.gov/diseases/10430/dravet-syndrome.

 

“Epidiolex (Cannabidiol) Dosing, Indications, Interactions, Adverse Effects, and More." Medscape Drugs & Diseases - Comprehensive Peer-Reviewed Medical Condition, Surgery, and Clinical Procedure Articles with Symptoms, Diagnosis, Staging, Treatment, Drugs and Medications, Prognosis, Follow-up, and Pictures, 26 June 2018, reference.medscape.com/drug/epidiolex-cannabidiol-1000225#10.

 

Epidiolex® (cannabidiol) [package insert]. Cambridge, United Kingdom. GW Pharmaceuticals Co. Ltd. January 2023. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=8bf27097-4870-43fb-94f0-f3d0871d1eec&type=display. Accessed April 20, 2023.

 

Fintepla® (fenfluramine) oral solution [prescribing information]. Emeryville, CA: Zogenix, Inc.; March 2023. Available from: https://www.fintepla.com/pdf/Fintepla-prescribing-information.pdf. Accessed ​April 20, 2023.

 

“Lennox-Gastaut Syndrome - Genetics Home Reference - NIH." U.S. National Library of Medicine, National Institutes of Health, July 2019, ghr.nlm.nih.gov/condition/lennox-gastaut-syndrome.

 

Onfi® (clobazam) [prescribing information]. Deerfield, IL: Lundbeck; January 2023. Available from: https://www.lundbeck.com/upload/us/files/pdf/Products/ONFI_PI_US_EN.pdf. Accessed April 20, 2023.

 

Sabril® (vigabatrin) [prescribing information]. Deerfield, IL: Lundbeck; October 2021. Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2013/020427s010s011s012,022006s011s012s013lbl.pdf. Accessed April 20, 2023.

 

Sympazan® (clobazam) [prescribing information]. Warren, NJ: Aquestive Therapeutics; March 2021. Available from: https://www.sympazan.com/pdfs/pi.pdf. Accessed April 20, 2023.​



113/16/20233/16/20246/29/2023 5:53 AMNo presence informationsrv_ppsgw_NP

​Off-Label Use Rx.01.33
​Applicable Age Edits Rx.01.2

Brand NameGeneric Name
Epidiolex®Cannabidiol
Diacomit®Stiripentol
Onfi®, Sympazan®
Clobazam
Banzel®Rufinamide
Fintepla®Fenfluramine

​Sabril®
​Vigabatrin
164
  
7/1/2023Rx.01.194CommercialOyenusi, Oluwadamilola

​Atopic dermatitis (eczema) is a chronic, relapsing inflammatory skin disease characterized by redness, lesions, and intense pruritus. The cause of atopic dermatitis is a combination of genetics and environmental factors, such as allergies and irritants. Atopic dermatitis is prevalent worldwide and occurs most frequently in children, but also affects many adults.  The onset of atopic dermatitis is most common between 3 to 6 months of age. Approximately 60% of patients develop atopic dermatitis within their first year of life and 90% by 5 years of age. More than 80% of children with atopic dermatitis have persistent symptoms into their adult years. More severe pruritus correlates to greater disease severity. Common features in infants include pruritic red, scaly and crusted lesions on the extensor surfaces and cheeks or scalp. In older children or adolescents, atopic dermatitis presents as lichenified plaques. In adults, it is more localized and lichenified. Atopic dermatitis associated with persistent pruritus could lead to sleep deprivation, symptoms of depression, anxiety, poor quality of life, and financial burden. Additionally, patients with atopic dermatitis are at increased risk for cutaneous bacterial, viral, and fungal infections due to persistent itching and scratching. 

American Academy of Dermatology Association current treatment guidelines:

  • Non-pharmacologic treatments
    • Topical moisturizers
    • Wet-wrap therapy
    • Bathing practices
  • Pharmacologic treatments
    • 1ST line: Topical corticosteroids
      • Introduced after failure of lesions to respond to good skin care and regular use of emollients alone
      • Treatment is individualized due to multiple formulations, dosage forms, and potencies
      • Choice of potency is based on patient's age, body area involved, and degree of inflammation
        • Acute flares: short course of medium-high potency topical corticosteroid, followed by a quick taper in potency
        • Long-term management: lowest-potency initially, followed by an upward titration if failed
    • 2nd line: Topical calcineurin inhibitors
      • Pimecrolimus cream: mild-moderate atopic dermatitis
      • Tacrolimus ointment: moderate-severe atopic dermatitis
      • Clinical situations in which a topical calcineurin is preferred:
        • Sensitive areas (face, neck, skin folds, anogenital)
        • Recalcitrance to steroids
        • Steroid-induced atrophy
        • Long-term uninterrupted topical steroid use

Asthma is a chronic airway disorder that is characterized by reversible airflow obstruction due to air hyper responsiveness and airway inflammation. Generally, asthma starts in childhood in relation to sensitization to common inhaled allergens, which stimulates T helper type 2 cell proliferation, subsequently releasing cytokines (IL-4, IL-5, and IL-13) that induce airway inflammation. Chronic airway inflammation leads to airway remodeling by thickening the airway walls, which creates a narrower pathway for air to travel.

Chronic rhinosinusitis with nasal polyps (CRSwNP) is a subtype of chronic rhinosinusitis (CRS), an inflammatory condition involving the paranasal sinuses and linings of the nasal passage, which persists for 12 weeks or longer. There is no cure for CRS, and therapy is intended to reduce symptoms and improve qualify of life. There is significant variability in the management of CRS which include saline washes and sprays, intranasal and systemic corticosteroids, antibiotics, and antileukotriene agents. Biologic agents are considered in patients with refractory disease when intranasal and oral corticosteroids failed to reduce polyp or to improve symptoms. 

Eosinophilic esophagitis (EoE) is a chronic allergic/immune condition of the esophagus. In EoE, large number of eosinophils are found in the inner lining of the esophagus resulting in inflammation which causes symptoms such as chest pain ,heartburn, difficulty swallowing, or impaction. 

Prurigo nodularis (PN) is an uncommon, chronic skin disorder affecting primarily older adults and is characterized by symmetrically distributed, multiple, firm, pruritic nodules. PN occurs in patients with chronic pruritus and is frequently associated with a history of atopic dermatitis.

Dupilumab (Dupixent®) is a fully human monoclonal IgG4 antibody that inhibits  interleukin-4 (IL-4) and interleukin-13 (IL-13) signaling by binding to the IL-4 receptor alpha subunit shared by IL-4 and IL-13 receptor complexes.  Dupilumab inhibits IL-4 signaling via the Type 1 receptor and both IL-4 and IL-13 signaling through the Type II receptor. IL-4 and IL-13 play a role in the pathogenesis of atopic dermatitis and asthma. Dupilumab reduces the release of pro-inflammatory cytokines. The mechanism of dupilumab (Dupixent®) action in asthma has not been definitively established.

Dupilumab (Dupixent®) is indicated in adults and children 6 months of age or older for the treatment of moderate to severe atopic dermatitis not adequately controlled with topical prescription therapy or when those therapies are inadvisable, and as an add-on maintenance treatment in patients with moderate-to-severe asthma aged 6 years and older with an eosinophilic phenotype or with oral corticosteroid dependent asthma. It is also indicated as an add-on maintenance treatment in adult patients with inadequately controlled chronic rhinosinusitis with nasal polyposis. Dupixent ® can be used with or without topical corticosteroids. It should not be used for the relief of acute bronchospasm or status asthmaticus. It is also used for the treatment of adult and pediatric patients aged 12 and older, weighing at least 40 kg, with eosinophilic esophagitis (EoE). It is also used for the treatment of adult patients with prurigo nodularis (PN).

The intent of this policy is to communicate the medical necessity criteria for dupilumab (Dupixent®) as provided under the member's prescription drug benefit. 

Atopic Dermatitis

INITIAL CRITERIA Dupilumab (Dupixent®) is approved ALL of the following are met:

  1. Member is 6 months of age or older; and
  2. Diagnosis of moderate-severe atopic dermatitis; and
  3. Prescribed by or in consultation with a dermatologist, allergist, or immunologist; and
  4. Paid claims or submission of medical records (e.g., chart notes) confirming an inadequate response or inability to tolerate ONE of the following:
    1. Topical steroids, medium potency or higher; or
    2. Topical tacrolimus; or
    3. Topical pimecrolimus; or
    4. Eucrisa; and
  5. No concurrent therapy with any other biologic agents; and
  6. Dosing and frequency does not exceed maximum FDA recommendation per indication requested

    Initial authorization duration:2 years

 

REAUTHORIZATION CRITERIA Dupilumab (Dupixent®) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy (e.g., reduction in body surface area involvement, reduction in pruritus severity); and
  2. Dosing and frequency does not exceed maximum FDA recommendation per indication requested ​

Reauthorization duration: 2 years

 DOSE
Adults

Initial dose: 600 mg (two 300mg injections)

Maintenance: 300 mg given every other week

Pediatric patients 6 months to 5 years of age

5 to less than 15 kg: 200 mg (one 200 mg injection) every 4 weeks

15 to less than 30 kg: 300 mg (one 300 mg injection) every 4 weeks

Pediatric patients 6 years to 17 years of age

15 to less than 30 kg: 600 mg (two 300 mg injections) initially, followed by 300 mg every 4 weeks

30 to less than 60 kg: 400 mg (two 200 mg injections) initially, followed by 200 mg every 2 weeks

60 kg or more: 600 mg (two 300 mg injections) initially, followed by 300 mg every 2 weeks

 

Moderate to severe asthma

INITIAL CRITERIA Dupilumab (Dupixent®) is approved when ALL of the following are met:

  1. Member is 6 years of age or older; and
  2. One of the following:
    1. Submission of documentation (e.g., chart notes) confirming a diagnosis of Moderate to severe asthma with an eosinophilic phenotype defined as blood eosinophil levels of at least 150 cells/mcL at baseline or at least 300 cells/mcL within the past 12 months; or
    2. Submission of documentation (e.g., chart notes) confirming a diagnosis of Moderate to severe oral corticosteroid-dependent asthma; and
  3. Paid claims or submission of documentation (e.g., chart notes) confirming member is currently being treated with ONE of the following or is unable to tolerate these medications:
    1. Medium to high dose inhaled corticosteroid (e.g., greater than or equal to 500mcg fluticasone propionate equivalent/day); and ONE additional controller medication; or
    2. One maximally dosed combination ICS/LABA product (e.g., Advair (fluticasone propionate/salmeterol), Dulera (mometasone/formoterol), Symbicort (budesonide/formoterol); and
  4. ​​One of the following:
    1. Patient has had at least two or more asthma exacerbations requiring systemic corticosteroids (e.g., prednisone) within the past 12 months; or
    2. Prior asthma-related hospitalization within the past 12 months; and​
  5. Prescribed by or in consultation with a pulmonologist or allergy/ immunology specialist; and
  6. No concurrent therapy with any other biologic agents; and
  7. Dosing and frequency does not exceed maximum FDA recommendation per indication requested

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA Dupilumab (Dupixent®) is re-approved when ALL of the following are met:

1.     Documentation of a positive clinical response to therapy (e.g., reduction in exacerbations, improvement in FEV1, decreased use of rescue medications); and

2.     Paid claims or submission of documentation (e.g., chart notes) confirming member is currently being treated with ONE of the following or is unable to tolerate these medications:

  1. Medium to high dose inhaled corticosteroid (e.g., greater than or equal to 500mcg fluticasone propionate equivalent/day); and ONE additional controller medication; or
  2. One maximally dosed combination ICS/LABA product (e.g., Advair (fluticasone propionate/salmeterol), Dulera (mometasone/formoterol), Symbicort (budesonide/formoterol); and

3.     Prescribed by or in consultation with a pulmonologist or allergy/immunology specialist; and

4.     No concurrent therapy with any other biologic agents; and

5.     Dosing and frequency does not exceed maximum FDA recommendation per indication requested

Reauthorization duration: 2 years

 DOSE
Adults and pediatric patients 12 years and older

Initial dose: 400 mg (two 200 mg injections)

Maintenance: 200 mg every 2 weeks

OR

Initial dose: 600 mg (two 300 mg injections)

Maintenance dose: 300 mg every 2 weeks

Dosage for patients with oral corticosteroid-dependent asthma or with co-morbid moderate-to-severe atopic dermatitis or adults with co-morbid

chronic rhinosinusitis with nasal polyposis

Initial dose: 600 mg (two 300 mg injections)

Maintenance dose: 300 mg every 2 weeks

Pediatric patients 6 years to 11 years of age

15 to less than 30 kg: 100 mg every other week OR 300 mg every 4 weeks

≥ 30 kg: 200 mg every other week

 

Chronic rhinosinusitis with nasal polyposis (CRSwNP)

INITIAL CRITERIA Dupilumab (Dupixent®) is approved when ALL of the following are met:

  1. Submission of documentation (e.g., chart notes) confirming diagnosis of chronic rhinosinusitis with nasal polyposis (CRSwNP); and
  2. Member is 18 years of age or older; and
  3. Prescribed by or in consultation with an allergist, immunologist, or Ear Nose Throat specialist; and
  4. Paid claims or submission of documentation (e.g., chart notes) confirming an inadequate response or inability to tolerate at least a 2-month treatment with intranasal corticosteroid (e.g., fluticasone); and
  5. Used in combination with another agent for CRSwNP; and
  6. No concurrent therapywith any other biologic agents; and
  7.  Dosing and frequency does not exceed maximum FDA recommendation per indication requested

Initial Authorization duration: 2 years

REAUTHORIZATION CRITERIA Dupilumab (Dupixent®) is re-approved when ALL of the following are met:

  1. Documentation of a positive clinical response to therapy (e.g., reduction in nasal polyps score [NPS; 0-8 scale], improvement in nasal congestion/obstruction score [NC; 0-3 scale]); and
  2. Prescribed by allergist, immunologist or Ear Nose Throat specialist; and
  3. Used in combination with another agent for CRSwNP; and
  4. No concurrent therapy with any other biologic agents and
  5.  Dosing and frequency does not exceed maximum FDA recommendation per indication requested

Reauthorization duration: 2 years

 

 DOSE
Adults300 mg every other week

 

_____________________________________________________________________________________________

Eosinophilic Esophagitis (EoE)

INITIAL CRITERIA Dupilumab (Dupixent®) is approved when ALL of the following are met:

  1. Submission of documentation (e.g., chart notes) confirming diagnosis of eosinophilic esophagitis (EoE); and
  2. Member has symptoms of esophageal dysfunction (e.g., dysphagia, food impaction, gastroesophageal reflux disease [GERD]/heartburn symptoms, chest pain, abdominal pain); and
  3. Member has at least 15 intraepithelial eosinophils per high power field (HPF); and
  4. Other causes of esophageal eosinophilia have been ruled out; and
  5. Both of the following:
    1. Member is 12 years of age or older; and
    2. Member weighs at least 40 kg; and
  6. Paid claims or submission of documentation (e.g., chart notes) confirming an inadequate response or inability to tolerate at least an 8-week trial of one of the following:
    1. Proton Pump Inhibitors (e.g., pantoprazole, omeprazole); or
    2. Topical (esophageal) corticosteroids (e.g., budesonide, fluticasone); and
  7. Prescribed by or in consultation with gastroenterologist or allergist/immunologist; and
  8. No concurrent therapy with any other biologic agents; and
  9. Dose and frequency does not exceed maximum FDA recommendation per indication requested

    Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA Dupilumab (Dupixent®) is re-approved when ALL of the following are met:

  1. Documentation of a positive clinical response to therapy as evidenced by improvement of at least one of the following from baseline:
    1. Symptoms (e.g., dysphagia, food impaction, heartburn, chest pain); or
    2. Histologic measures (e.g., esophageal intraepithelial eosinophil count); or
    3. Endoscopic measures (e.g., edema, furrows, exudates, rings, strictures); and
  2. No concurrent therapy with any other biologic agents; and
  3. Dose and frequency does not exceed maximum FDA recommendation per indication requested

Reauthorization duration: 2 years

 

 DOSE
Adults and pediatric patients 12 years of age and older, weighing at least 40 kg300 mg every week


Prurigo Nodularis (PN)

 

INITIAL CRITERIA: Dupilumab (Dupixent®) is approved when ALL of the following are met:

  1. Submission of documentation (e.g., chart notes) confirming a diagnosis of Prurigo Nodularis (PN); and
  2. Member is 18 years of age or older; and
  3. Paid claims or submission of documentation (e.g., chart notes) confirming inadequate response or inability to tolerate one previous PN treatment (e.g., topical corticosteroids, topical calcineurin inhibitors, topical capsaicin); and
  4. Prescribed by or in consultation with allergist/immunologist or dermatologist; and
  5. No concurrent therapy with any other biologic agents; and
  6. Dosing and frequency does not exceed maximum FDA recommendation per indication requested

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Dupilumab (Dupixent®) is re-approved when ALL of the following are met:

  1. Documentation of a positive clinical response to therapy as evidenced by at least ONE of the following:
    1. Reduction in the number of nodular lesions from baseline; or
    2. Improvement in symptoms (e.g., pruritus, inflammation) from baseline; and
  2. Dosing and frequency does not exceed maximum FDA recommendation per indication requested
Reauthorization duration: 2 years​


 DOSE
Adults

Initial dose: 600 mg (two 300 mg injections)

Maintenance dose: 300 mg given every other week


N/A

Beck LA, Thaci D, Hamilton JD, et al. Dupilumab treatment in adults with moderate-to-severe atopic dermatitis. N Engl J Med 2014; 371: 130-9. Available from: http://www.jaad.org/article/S0190-9622(16)30330-9/pdf. Accessed April 18, 2023.

Chin, Matthews MD, Gonsalves, Nirmala, MD. Eosinophilic Esophagitis Disease overview. American Collage of Gastroenterology. March 2021. Available at: https://gi.org/topics/eosinophilic-esophagitis/. Accessed April 18, 2023

Dupixent® (dupilumab) [prescribing information]. Tarrytown, NY: Regeneron Pharmaceuticals Inc; June 2022. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=595f437d-2729-40bb-9c62-c8ece1f82780&type=display. Accessed April 18, 2023.

Eichenfield LF, Wynnis LT, Chamlin SL, et al. Guidelines of care for the management of atopic dermatitis: section 1. Diagnosis and assessment of atopic dermatitis. J Am Acad Dermatol. 2014;70(2):338-351. Available from: http://www.jaad.org/article/S0190-9622(13)01095-5/pdf. Accessed April 18, 2023.

Eichenfield LF, Tom WL, Berger TG, et al. Guidelines of care for the management of atopic dermatitis: section 2. Management and treatment of atopic dermatitis with topical therapies. JamAcad Dermatol . 2014;71:116-132. Available from: http://www.jaad.org/article/S0190-9622(16)30330-9/pdf. Accessed April 18, 2023.

Hamilos, D, Holbrook, E. Chronic rhinosinusitis: Management. UpToDate. September 2022. Available at: https://www.uptodate.com/contents/chronic-rhinosinusitis-management?search=chronic%20rhinosinusitis%20with%20nasal%20polyps&source=search_result&selectedTitle=1~50&usage_type=default&display_rank=1. Accessed April 18, 2023.

Paller AS, Tom WL, Lebwohl MG, et al. Efficacy and safety of crisaborole ointment, a novel, nonsteroidal phosphodiesterase 4 (PDE4) inhibitor for the topical treatment of atopic dermatitis (AD) in children and adults. J Am Acad Dermatol. 2016;75(3):494-503. Available from: http://www.jaad.org/article/S0190-9622(16)30330-9/pdf. Accessed April 18, 2023.

Kudo M, Ishigatsubo Y, Aoki I. Pathology of asthma. Front Microbiol. 2013;4:263. Accessed April 18, 2023.

Weston WL, Howe W. Atopic dermatitis (eczema): pathogenesis, clinical manifestations, and diagnosis. UpToDate website. Last updated September 2022. Available at: www.uptodate.com. Accessed April 18, 2023.

Watsky, K. Prurigo nodularis. UpToDate website. Last updated November 2022. Available at: www.uptodate.com. Accessed April 18, 2023.


193/16/20233/16/20246/29/2023 5:53 AMNo presence informationsrv_ppsgw_NP

Off-Label Use Rx.01.33

Brand NameGeneric Name
Dupixent®dupilumab
165
  
7/1/2023Rx.01.216CommercialOyenusi, Oluwadamilola

​Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. LEMS is associated with reduced acetylcholine (ACh) release from the presynaptic nerve terminals. Antibodies directed against the voltage-gated calcium channel (VGCC) interfere with the normal calcium flux required for the release of ACh from the presynaptic nerve terminal. The most common symptoms of LEMS include proximal muscle weakness, fatigue, autonomic symptoms such as dry mouth, sluggish pupillary light response, erectile dysfunction in men, and reduced tendon reflexes. LEMS patients can be divided into two groups: patients with LEMS associated with underlying malignancy (paraneoplastic LEMS) and those without malignancy (non-paraneoplastic LEMS). For patients with paraneoplastic LEMS, treatment of malignancy may be the only intervention necessary to produce improvement in neurologic symptoms of LEMS.

Amifampridine (Firdapse®) is broad spectrum potassium channel blocker indicated for the treatment of LEMS in adults and pediatric patients 6 years of age and older. It blocks presynaptic voltage-gated potassium channels, prolonging the duration of the presynaptic action potential, lengthening the opening time of the VGCC, and increasing the presynaptic calcium levels. The increased calcium levels lead to an increase in the amount of ACh released. ACh then binds to muscle receptors and results in improved muscle function.    

​The intent of this policy is to communicate the medical necessity criteria for amifampridine (Firdapse®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Amifampridine (Firdapse®) is approved when ALL of the following are met:

  1. Member has a diagnosis of Lambert-Eaton myasthenic syndrome; and
  2. Member is 6 years of age or older and
  3. Neurological symptoms persist after treatment of malignancy, when malignancy is present; and
  4. Member has moderate to severe weakness that interferes with function; and
  5. Prescribed by or in consultation with a neurologist; and
  6. Member does not have history of seizures

Initial authorization duration: 3 months 

CONTINUATION CRITERIA: Amifampridine (Firdapse®) is re-approved when there is documentation of positive clinical response to therapy (e.g., improvement in dynamometry, Timed 25-Foot Walk Test, Timed Up and Go Test)..

Reauthorization duration: 2 years

​None

Firdapse® (amifampridine) [prescribing information]. Coral Gables, FL: Catalyst Pharmaceuticals, Inc.  September 2022. Available at: https://www.firdapse.com/pdfs/firdapse-pi.pdf. Accessed April 17, 2023.

Titulaer MJ, Lang B, Verschuuren JJGM. Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol. 2011[a]; 10:1098-1107.


Weinberg DH. Lambert-Eaton myasthenic syndrome: Clinical features and diagnosis. UpToDate Web site. Updated March 2023. www.uptodate.com. Accessed April 17, 2023.



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​Rx.01.33 Off-Label Use

Brand NameGeneric Name
Firdapse® amifampridine phosphate

168
  
7/1/2023Rx.01.214CommercialOyenusi, Oluwadamilola

Hyperhidrosis, defined as the secretion of sweat in amounts greater than physiologically necessary to main thermoregulation, can adversely affect an individual's social and emotional well-being and overall daily function. It commonly involves the axillae, palms, and soles, but may also affect the face, groin, or any area of the body. Prescription antiperspirants such as aluminum chloride hexahydrate 20% (Drysol®) and topical glycopyrronium (Qbrexza™) are first line options for the treatment of hyperhidrosis. Second line treatment options include onabotulinumtoxin A injections and microwave thermolysis.

Glycopyrronium (Qbrexza™) is a competitive inhibitor of acetylcholine receptors that are located on certain peripheral tissues, including sweat glands. In hyperhidrosis, glycopyrronium inhibits the action of acetylcholine on sweat glands, reducing sweating.

Glycopyrronium (Qbrexza™) is indicated for the topical treatment of primary axillary hyperhidrosis in adults and pediatric patients 9 years of age and older.

​The intent of this policy is to communicate the medical necessity criteria for glycopyrronium (QbrexzaTM) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: ​Qbrexza™ (glycopyrronium) is approved when ALL of the following are met:

  1. Diagnosis of primary axillary hyperhidrosis for at least 6 months; and
  2. Member is 9 years of age or greater; and
  3. Prescribed by or in consultation with a dermatologist; and
  4. Hyperhidrosis Disease Severity Scale grade 3 or 4; and
  5. Documentation of an inadequate response or inability to tolerate aluminum chloride (e.g. Drysol)

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Qbrexza™ (glycopyrronium) is re-approved when there is positive clinical response to therapy.

Reauthorization duration: 2 years

​N/A

Qbrexza™ (glycopyrronium) [prescribing information]. Menlo Park, CA. Dermira., Inc. April 2021. Available at: https://getqbrexza.com/wp-content/themes/qbrexzadtp/assets/pdf/qbrexza-pi.pdf. Accessed April 01, 202219, 2023.

Smith CC, Pariser D. Primary focal hyperhidrosis. UpToDate Web site. Updated December 2022. www.uptodate.com. Accessed April 19, 2023.

Wade R, Rice S, Llewellyn A, et al. Interventions for hyperhidrosis in secondary care: a systematic review and value-of-information analysis. Health Technol Assess. 2017;21(80):1-280.  ​


53/16/20233/16/20246/29/2023 5:53 AMNo presence informationsrv_ppsgw_NP

Off-Label Use Rx.01.33

Brand NameGeneric Name
Qbrexza™Glycopyrronium
174
  
7/1/2023Rx.01.219CommercialOyenusi, Oluwadamilola

Parkinson's disease (PD) is a neurodegenerative disorder caused by progressive dopamine depletion in the nigrostriatal pathway of the brain.  PD is characterized by manifestations of tremor, bradykinesia, and rigidity. PD is a motor condition that includes neuropsychiatric and other nonmotor manifestations.

The dopamine precursor levodopa is the most effective drug for the symptomatic treatment of PD, however; levodopa-induced complications (eg, motor fluctuations [“wearing off" phenomenon], dyskinesia, dystonia) develop in at least 50% of patients after 5 to 10 years of levodopa treatment. The risk of motor complications increases with higher levodopa doses and younger age of PD onset.

The cause of motor fluctuations is not clear, but it is hypothesized that they evolve as PD progresses because progressive degeneration of the nigrostriatal dopaminergic pathway reduces the ability of nerve terminals to store and release dopamine.  The response to exogenous levodopa becomes more pulse-like due to the inability of the nerve terminals to store and release dopamine.  Levodopa has a short half-life (90 minutes), rapid cycling pharmacokinetics (PK), and erratic intestinal absorption related to slowed intestinal motility.

The four main drugs or classes of drugs that have anti-parkinson activity are monoamine oxidase type B (MAO B) inhibitors, amantadine, dopamine agonists and levodopa. Initial therapy is individualized and requires a flexible trial-and-error approach. Individuals who exhibit mild symptoms with minimal impact on daily life are good candidates for MAO B inhibitor as initial therapy. For individuals with mild to moderate symptoms that impact daily living, either dopamine agonist or levodopa is recommended in individuals younger than 65; levodopa is preferred in those older than 65 years of age. Levodopa is the drug of choice in individuals with moderate to severe symptoms regardless of age.

Levodopa, the metabolic precursor of dopamine, crosses the blood-brain barrier and is presumably converted to dopamine in the brain. This is thought to be the mechanism whereby levodopa relieves symptoms of PD

Levodopa (Inbrija™) inhalation powder is indicated for the intermittent treatment of OFF episodes in patients with PD treated with carbidopa/levodopa. 

Istradefylline (Nourianz™) is an adenosine receptor antagonist indicated as adjunctive treatment to levodopa/carbidopa in adult patients with Parkinson's disease (PD) experiencing “off" episodes. The precise mechanism by which istradefylline exerts its therapeutic effect in PD is unknown.

The mechanism by which apomorphine hydrochloride treats Parkinson Disease is unknown. Apomorphine is a non-ergoline dopamine agonist that has high in-vitro affinity for the dopamine D4 receptor, and moderate affinity for the dopamine D2, D3, D5, and adrenergic a1D, a2B, and a2C receptors. Activity is suspected to be due to stimulation of post-synaptic dopamine D2-type receptors within the caudate-putamen in the brain.

Apomorphine hydrochloride (Kynmobi™) sublingual film is a non-ergoline dopamine agonist indicated for the acute, intermittent treatment of “off" episodes in patients with Parkinson's disease (PD).

Apomorphine hydrochloride (Apokyn®) injection for subcutaneous use is a non-ergoline dopamine agonist indicated for the acute, intermittent treatment of hypomobility, “off" episodes (“end-of-dose wearing off" and unpredictable “on/off" episodes) associated with advanced Parkinson's disease.

Adding a catechol-O-methyltransferase (COMT) inhibitor can prolong and potentiate the levodopa effect and thereby reduce "off" time when used as adjunctive therapy with levodopa.

The intent of this policy is to communicate the medical necessity criteria for levodopa inhalation (Inbrija™), istradefylline (Nourianz™), and apomorphine (Apokyn®, Kynmobi™) as provided under the member's prescription drug benefit.

Parkinson's Disease

INITIAL CRITERIA: Levodopa inhalation (Inbrija™), or istradefylline (Nourianz™) is approved when ALL of the following are met:

  1. Diagnosis of Parkinson's disease and member is experiencing intermittent off episodes; and
  2. Member is 18 years of age or older; and
  3. Concurrent use of carbidopa/levodopa containing product; and
  4. Prescribed by or in consultation with a neurologist; and
  5. Member had inadequate response or inability to tolerate TWO of the following:
    1. MAO-B Inhibitor (e.g., rasagiline, selegiline); or
    2. Dopamine Agonist (e.g., pramipexole, ropinirole); or
    3. COMT inhibitor (e.g., entacapone)


​Initial authorization duration: 2 years

REAUTHORIZATION CRITRIA: Levodopa inhalation (Inbrija®), or istradefylline (Nourianz™) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy; and
  2. Concurrent use of carbidopa/levodopa containing product


__________________________________________________________________________________________

Advanced Parkinson's Disease

INITIAL CRITERIA: Apomorphine (Apokyn®, Kynmobi™) is approved when ALL of the following are met: 

  1. Diagnosis of advanced Parkinson's disease and member is experiencing intermittent “off" episodes; and
  2. Member is 18 years of age or older; and
  3. One of the following:
    1. Member is receiving medication in combination with other medications for the treatment of Parkinson's disease (e.g., carbidopa/levodopa, pramipexole, ropinirole, etc…); or
    2. Member has a contraindication or intolerance to other medications for the treatment of Parkinson's disease; and
  4. Member is not using the medication with any 5-HT3 antagonist (e.g., ondansetron, granisetron, dolasetron, palonosetron, alosetron); and
  5. For Apomorphne (Apokyn® ) inadequate response or inability to tolerate apomorphine (Kynmobi™); and
  6. Prescribed by or in consultation with a neurologist

Initial authorization duration: 2 years

REAUTHORIZATION CRITRIA: Apomorphine (Apokyn®, Kynmobi™) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years​

​None

 

Apokyn® (apomorphine hydrochloride injection) [prescribing information]. Louisville, KY: US WorldMeds, LLC.; June 2022. Available from: https://www.apokyn.com/sites/all/themes/apokyn/content/resources/Apokyn_PI.pdf. Accessed April 17, 2023.

Inbrija™ [package insert]. Ardsley, NY. Acorda Therapeutics, Inc. December 2022. Available at: https://www.inbrija.com/prescribing-information.pdf. Accessed April 17, 2023.

Kynmobi™ (apomorphine hydrochloride sublingual film) [prescribing information]. Marlborough, Massachusetts: Sunovion Pharmaceuticals Inc.; September 2022. Available from: https://www.kynmobi.com/Kynmobi-Prescribing-Information.pdf. Accessed April 17, 2023.

Nourianz™ [package insert]. Bedminster, NJ. Kyowa Kirin, Inc., May 2020. Available at: https://www.nourianz.com/assets/pdf/nourianz-full-prescribing-information.pdf. Accessed April 17, 2023.

Chou KL. Clinical manifestations of Parkinson disease. UpToDate Web site. Updated March 2023. www.uptodate.com. Accessed April 17, 2023.

Fox SH, Katzenschlager R, Lim SY, et al; Movement Disorder Society Evidence-Based Medicine Committee. International Parkinson and Movement Disorder Society evidence-based medicine review: update on treatments for the motor symptoms of Parkinson's disease. Mov Disord. 2018;33(8):1248-1266.

Grosset DG, Dhall R, Gurevich T, et al. Long-term pulmonary safety of inhaled levodopa in Parkinson's disease subjects with motor fluctuations: a phase 3 open-label randomized study. Poster presented at: 2nd Pan American Parkinson's Disease and Movement Disorders Congress; June 22-24, 2018; Miami, FL.

Jankovic J. Epidemiology, pathogenesis, and genetics of Parkinson disease. UpToDate Web site. Updated March 223. www.uptodate.com. Accessed April 17, 2023.

LeWitt PA, Hauser RA, Grosset DG, et al. A randomized trial of inhaled levodopa (CVT-301) for motor fluctuations in Parkinson's disease. Mov Disord. 2016;31(9):1356-65.

LeWitt PA, Hauser RA, Pahwa R, et al; on behalf of the SPAN-PD Study Investigators. Safety and efficacy of CVT-301 (levodopa inhalation powder) on motor function during off periods in patients with Parkinson's disease: a randomised, double-blind, placebo-controlled phase 3 trial. Lancet Neurol. 2019;18:145-54.

Oertel WH, Berardelli A, Bloem BR, et al. Late (complicated) Parkinson's disease. In: Gilhus NE, Barnes MP, Brainin M, eds. European Handbook of Neurological Management. West Sussex, United Kingdom: Wiley-Blackwell; 2011:237-267.

Spindler MA, Tarsy D. Initial pharmacologic treatment of Parkinson disease. UpToDate Web site. Updated March 2023. www.uptodate.com. Accessed April 17, 2023.

Tarsy D. Medical management of motor fluctuations and dyskinesia in Parkinson disease. UpToDate Web site. Updated March 2023. www.uptodate.com. Accessed April 17, 2023.



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​Rx.01.33 Off-Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit

Brand NameGeneric Name
Inbrija™Levodopa inhalation
Nourianz™Istradefylline
Apokyn®Apomorphine
Kynmobi™Apomorphine
183
  
7/1/2023Rx.01.237CommercialOyenusi, Oluwadamilola

Neuromyelitis optica spectrum disorders (NMOSD), are inflammatory disorders of the central nervous system characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. NMOSD can be distinguished from multiple sclerosis and other central nervous system inflammatory disorders by the unique presence of the disease-specific aquaporin-4 (AQP4) antibody, which plays a direct role in the pathogenesis of NMOSD.

Common features of NMOSD include acute attacks characterized by bilateral or rapidly sequential optic neuritis (leading to visual loss), acute transverse myelitis (often causing limb weakness and bladder dysfunction), and the area postrema syndrome (with intractable hiccups or nausea and vomiting).

The precise mechanism by which Satralizumab-mwge (Enspryng™) exerts therapeutic effects in NMOSD is unknown but is presumed to involve inhibition of IL-6-mediated signaling through binding to soluble and membrane-bound IL-6 receptors. Satralizumab-mwge (Enspryng™) is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive.

The intent of this policy is to communicate the medical necessity criteria for Satralizumab-mwge (Enspryng™) as provided under the member's prescription drug benefit. 

INITAL CRITERIA: Satralizumab-mwge (Enspryng™) is approved when ALL of the following are met:

  1. Diagnosis of neuromyelitis optica spectrum disorder (NMOSD); and
  2. Member is anti-aquaporin-4 (AQP4) antibody positive; and
  3. Prescribed by or in consultation with a neurologist or ophthalmologist; and
  4. Member is 18 years of age or older

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Satralizumab-mwge (Enspryng™) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

​N/A

Enspryng™ (satralizumab-mwge) [prescribing information]. San Francisco, CA: Genentech, Inc; March 2022. Available from: https://www.gene.com/download/pdf/enspryng_prescribing.pdf. Accessed April 20, 2023.

Glisson C. Neuromyelitis optica spectrum disorders. UpToDate website. Last updated August 2022. Available at: www.uptodate.com. Accessed April 20, 2023.​


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Rx.01.33 Off Label Use​
Brand NameGeneric Name
Enspryng™Satralizumab-mwge
190
  
7/1/2023Rx.01.243CommercialOyenusi, Oluwadamilola


Spinal muscular atrophy (SMA) is characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem, which results in progressive muscle weakness and atrophy. The diagnosis of SMA should be suspected for any infant with unexplained weakness or hypotonia. Additional clues suggesting the diagnosis in infants, children, or adults include a history of motor difficulties, loss of motor skills, proximal muscle weakness, hyporeflexia or areflexia, tongue fasciculations, and signs of lower motor neuron disease on examination. These diseases classified as types 0 through 4, depending upon the age of onset and clinical course.

 

Risdiplam is a survival of motor neuron 2 (SMN2) splicing modifier designed to treat patients with spinal muscular atrophy (SMA) caused by mutations in chromosome 5q that lead to SMN protein deficiency. Using in vitro assays and studies in transgenic animal models of SMA, risdiplam was shown to increase exon 7 inclusion in SMN2 messenger ribonucleic acid (mRNA) transcripts and production of full-length SMN protein in the brain. In vitro and in vivo data indicate that risdiplam may cause alternative splicing of additional genes, including FOXM1 and MADD. FOXM1 and MADD are thought to be involved in cell cycle regulation and apoptosis, respectively, and have been identified as possible contributors to adverse effects seen in animals.

Evrysdi is indicated for the treatment of spinal muscular atrophy (SMA) in pediatric and adult patients.


The intent of this policy is to communicate the medical necessity criteria for Risdiplam (Evrysdi®) as provided under the member's prescription drug benefit. 



INITAL CRITERIA: Risdiplam (Evrysdi™) is approved when ALL of the following are met:

  1. Diagnosis of spinal muscular atrophy (SMA) type 1, 2 or 3; and
  2. BOTH of the following:
    • The mutation or deletion of genes in chromosome 5q resulting in one of the following:
      1. Homozygous gene deletion or mutation (e.g., homozygous deletion of exon 7 at locus 5q13); or
      2. Compound heterozygous mutation (e.g., deletion of SMN1 exon 7 [allele 1] and mutation of SMN1 [allele 2]); and
    • Member has at least 2 copies of SMN2; and 
  3. Prescribed by or in consultation with a neurologist or a psychiatrist with subspecialty certification in neuromuscular medicine; and
  4. Member is not to receive concomitant chronic survival motor neuron (SMN) modifying therapy for the treatment of SMA (e.g., Spinraza)

Initial authorization duration: 12 months


REAUTHORIZATION CRITERIA: Risdiplam (Evrysdi™) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy from pretreatment baseline status; and
  2. Prescribed by or in consultation with a neurologist or a psychiatrist with subspecialty certification in neuromuscular medicine; and
  3. Member is not to receive concomitant chronic survivor motor neuron (SMN) modifying therapy for the treatment of SMA (e.g., Spinraza)

Reauthorization duration: 2 years


​N/A

Evrysdi™ (risdiplam) [prescribing information]. San Francisco, CA: Genentech, Inc; March 2023. Available from: https://www.gene.com/download/pdf/evrysdi_prescribing.pdf. Accessed April 19, 2023.

Bodamer, OA. Spinal muscular atrophy. In: UpToDate. April 2023. Available from: www.uptodate.com. Accessed April 19, 2023.

Day JW, Annoussamy M, Baranello G, et al. SUNFISH Part 2: 24-month efficacy outcomes of risdiplam (RG7916) treatment in patients with Type 2 or 3 spinal muscular atrophy (SMA). Presented at the 2020 Virtual SMA Research & Clinical Care Meeting.

Servais L, Baranello G, Masson R, et al. FIREFISH Part 2: Efficacy and safety of risdiplam (RG7916) in infants with Type 1 spinal muscular atrophy (SMA). Presented at the 2020 Virtual SMA Research & Clinical Care Meeting.

Markowitz JA, Sing P, Darras BT. Spinal muscular atrophy: a clinical and research update. Pediatr Neurol. 2012;46(1):1-12.

Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027-1049.

Bertini E DJ, Muhaizea A, et al. RAINBOWFISH: A Study of Risdiplam (RG7916) in Newborns with Presymptomatic Spinal Muscular Atrophy. Presented at: World Muscle Society; October 1–5, 2019; Copenhagen, Denmark.

Mercuri E, Finkel RS, Muntoni F, et al. Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. J Neuromuscul Dis. 2018;28(2):103-115. Page 723

Stolte B, Bois JM, Kizina K, et al. Minimal clinically important differences in functional motor scores in adults with spinal muscular atrophy. Eur. J. Neurol. 2020; 0:1-9.

Pera, M., Coratti, G., Mazzone, E., et al. (2019). Revised upper limb module for spinal muscular atrophy: 12 month changes. Muscle Nerve. Apr;59(4):426-430.

Kirschner J, Butoianu N, Goemans N, et al. European ad-hoc consensus statement on gene replacement therapy for spinal muscular atrophy. Eur J Paediatr Neurol. 2020. https://doi.org/10.1016/j.ejpn.2020.07.001




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Rx.01.33 Off Label Use​


Brand NameGeneric Name
Evrysdi™
Risdiplam


192
  
7/1/2023Rx.01.248CommercialOyenusi, Oluwadamilola

​Cystic fibrosis is a progressive genetic condition that affects many areas of the body, including the lungs, digestive system, sweat glands and the reproductive system. Cystic fibrosis is caused by mutations in the CTFR gene, resulting in the abnormal transport of chloride and sodium ions across cell epithelia. In the respiratory system, this means there is less transport of water into the airway secretions, resulting in thick and viscous mucus accumulating in airways. These viscous secretions can obstruct the airways and promote respiratory infections, which can cause tissue damage. Common symptoms associated with cystic fibrosis include difficulty breathing, persistent productive cough and recurrent respiratory infections. Over time, these symptoms can worsen, resulting in worsening respiratory function and eventually even respiratory failure.

The precise mechanism of action of Bronchitol® in improving pulmonary function in cystic fibrosis patients is unknown.

Bronchitol® is indicated as add-on maintenance therapy to improve pulmonary function in adult patients 18 years and older with Cystic Fibrosis. Use Bronchitol® only for adults who have passed the Bronchitol® Tolerance Test.

The intent of this policy is to communicate the medical necessity criteria for Mannitol (Bronchitol®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Mannitol (Bronchitol®) is approved when all of the following are met:

  1. Diagnosis of cystic fibrosis; and
  2. Member is 18 years of age or older; and
  3. Member has passed the Bronchitol Tolerance Test (BTT); and
  4. Member has inadequate response or inability to tolerate ONE of the following:
    1. inhaled hypertonic saline; or
    2. Pulmozyme (dornase alfa); and
  5. Prescribed by or in consultation with one of the following:
    1. Pulmonologist; or
    2. Specialist associated with a cystic fibrosis care center
​Initial authorization duration: 2 years ​

REAUTHORIZATION CRITERIA: Mannitol (Bronchitol®) is re-approved when there is documentation of positive clinical response to therapy (e.g. improvement in lung function [forced expiratory volume in one second {FEV1}])

Reauthorization duration: 2 years 

​N/A

Bronchitol® (mannitol) [Package insert]. Cary, NC: Chiesi USA, Inc. October 2020. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=454f092e-dcd0-47bd-a521-b07400403dad. Accessed April 19, 2023.

Katikin JP. Cystic fibrosis: genetics and pathogenesis. UpToDate. February 2022. Available at: https://www.uptodate.com/contents/cystic-fibrosis-genetics-and-pathogenesis. Accessed April 19, 2023.

Katikin JP. Cystic fibrosis: clinical manifestations of pulmonary disease. UpToDate. February 2023. Available at: https://www.uptodate.com/contents/cystic-fibrosis-clinical-manifestations-of-pulmonary-disease. Accessed April 19, 2023. ​


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Rx.01.33 Off Label Use​

Brand Name

Bronchitol®

Generic Name

Mannitol

195
  
7/1/2023Rx.01.142CommercialOyenusi, Oluwadamilola

Epinephrine pens are indicated for the emergency treatment of allergic reactions (type I), including anaphylaxis, which may result from allergic reactions to insect stings, biting insects, foods, drugs, sera, diagnostic testing substances and other allergens, as well as idiopathic anaphylaxis or exercise-induced anaphylaxis.

Epinephrine is a sympathomimetic catecholamine that is an agonist at alpha and beta adrenergic receptors.  Through its effects at the alpha adrenergic receptors, epinephrine cause vasoconstriction to counter vasodilation and increased vascular permeability that may occur during an anaphylactic reaction.

Currently available products include Epi-Pen® , Symjepi™, Auvi-Q®, and epinephrine auto-injectors. 


 

The intent of this policy is to communicate the medical necessity criteria for Auvi-Q® , EpiPen®, and EpiPen Jr.® as provided under the member's prescription drug benefit.

Auvi Q®  , EpiPen®, EpiPen Jr.® is approved when ALL of the following are met:

  1. ONE of the following:
    1. Documentation of a demonstrated inability to use  one chemically equivalent generic agent; or 
    2. The member has a genuine allergic reaction to an inactive ingredient in generic agent(s) (allergic reaction(s) must be clearly documented in the patient's medical record); and
  2. For Auvi Q® only, documentation that the member or the member's caregivers would be unable to utilize the alternative epinephrine auto-injector devices (e.g., epinephrine) due to significant visual, physical, or functional impairment

Approval duration: 2 years 



 
 
 
None

Auvi-Q® (epinephrine injection) [package insert]. Richmond, VA. : Kaleo, Inc. August 2019.  Available at:  https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=6180fb40-7fca-4602-b3da-ce62b8cd2470&type=display. Accessed April 19, 2023.

 

EpiPen®, EpiPen Jr® (epinephrine injection) [package insert]. Morgantown, WE: Mylan Specialty L.P.; February 2023. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?type=display&setid=7560c201-9246-487c-a13b-6295db04274a. Accessed April 19, 2023.​




 

143/16/20233/16/20246/29/2023 5:56 AMNo presence informationsrv_ppsgw_NP
Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Pharmacy Benefit
Rx.01.33 Off-Label Use

 

Brand NameGeneric Name
Auvi-Q®, EpiPen®, EpiPen Jr®Epinephrine

196
  
7/1/2023Rx.01.203CommercialOyenusi, Oluwadamilola

Systemic lupus erythematosus (SLE) is an autoimmune disorder that is very heterogeneous with respect to its severity and the organs affected. Approximately 1.5 million Americans, primarily women of childbearing age, have a form of lupus. SLE represents approximately 70% of all lupus cases. Common clinical manifestations of SLE include pain, extreme fatigue, hair loss, cognitive issues, rashes (often the classic “butterfly rash”), arthritis and arthralgias. More severe clinical manifestations include renal, hematologic, or central nervous system involvement. SLE is often associated with relapses (which can be acute or chronic) and remissions.

Lupus nephritis (LN) is a form of glomerulonephritis that constitutes one of the most severe organ manifestation of systemic lupus erythematosus (SLE). Most patients with SLE who develop LN do so within 5 years of an SLE diagnosis and in many cases, LN is the presenting manifestation resulting in the diagnosis of SLE. Treatment of LN usually involves immunosuppressive therapy, typically with mycophenolate mofetil or cyclophosphamide and with glucocorticoids, although these treatments are not uniformly effective. Within 10 years of an initial SLE diagnosis, 5 to 20% of patients with LN develop end-stage kidney disease.   

BLyS, a B-cell survival factor, is overexpressed in patients with systemic lupus erythematosus (SLE) and other autoimmune diseases.  Belimumab is an inhibitor that targets B-lymphocyte stimulator (BLyS) protein, which may reduce the number of abnormal B cells by blocking the binding of BLyS to its receptors on B-cells. An intravenous (IV) formulation of belimumab was approved by the FDA in 2011.

A subcutaneous formulation of the medication was approved by the FDA in July 2017. 

Benlysta® (belimumab) is indicated for the treatment of:

  • Patients aged 5 years and older with active, autoantibody-positive systemic lupus erythematosus (SLE) who are receiving standard therapy
  • Patients aged 5 years and older with active lupus nephritis who are receiving standard therapy.
  • Limitations of Use: The efficacy of Benlysta has not been evaluated in patients with severe active central nervous system lupus. Benlysta has not been studied in combination with other biologics. Use of Benlysta is not recommended in these situations.

Voclosporin is a calcineurin-inhibitor immunosuppressant. Activation of lymphocytes involve an increase in intracellular calcium concentrations that bind to the calcineurin regulatory site and activate calmodulin binding catalytic subunit and through dephosphorylation, activates the transcription factor Nuclear Factor of Activated T-Cell Cytoplasmic (NFATc). The immunosuppressant activity results in inhibition of lymphocyte proliferation, T-cell cytokine production, and expression of T-cell activation surface antigens.

Lupkynis™ (voclosporin) is indicated in combination with a background immunosuppressive therapy regimen for the treatment of adult patients with active lupus nephritis
The intent of this policy is to communicate the medical necessity criteria for belimumab (Benlysta®) and voclosporin (Lupkynis™) as provided under the member's prescription drug benefit.

Systemic Lupus Erythematosus 

INITIAL CRITERIA Belimumab (Benlysta®) is approved when ALL of the following are met:

  1. Diagnosis of active systemic lupus erythematosus; and
  2. Autoantibody positive (ie, anti-nuclear antibody [ANA] titer greater than or equal to 1:80 or anti-dsDNA level greater than or equal to 30 IU/mL), antibodies to DNA [Anti-dsDNA], Anti-Smith [Anti-Sm]); and
  3. Currently receiving at least one standard of care treatment for active systemic lupus erythematosus (eg, antimalarials [eg, hydroxychloroquine], corticosteroids, NSAIDs, or immunosuppressants); and
  4. Prescribed by or in consultation with a rheumatologist; and
  5. Member is 5 years of age or older

Initial Authorization duration: 6 months

REAUTHORIZATION CRITERIA: Belimumab (Benlysta®) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

Lupus Nephritis

INITIAL CRITERIA Belimumab (Benlysta®) is approved when ALL of the following are met:

  1. Member has active lupus nephritis confirmed by kidney biopsy; and
  2. Member is receiving standard therapy for lupus nephritis (e.g. corticosteroids, immunosuppressants, azathioprine); and
  3. Prescribed by or in consultation with a rheumatologist or nephrologist; and
  4. Member is 5 years of age or older
​​​

INITIAL CRITERIA Voclosporin (Lupkynis™) is approved when ALL of the following are met:

  1. Diagnosis of active lupus nephritis; and
  2. Member is 18 years of age or older; and
  3. Used in combination with mycophenolate mofetil and corticosteroids; and
  4. Prescribed by or in consultation with nephrologist or rheumatologist 
Initial Authorization duration: 6 months

REAUTHORIZATION CRITERIA: Belimumab (Benlysta®) or Voclosporin (Lupkynis™) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years 

Lupkynis™ (Voclosporin)
Malignancies and serious infections: Increased risk for developing malignancies and serious infections with LUPKYNIS or other immunosuppressants that may lead to hospitalization or death.

Anders HJ, Saxena R, Zhao MH, Parodis I, Salmon JE, Mohan C. Lupus nephritis. Nat Rev Dis Primers. 2020 Jan 23;6(1):7. doi: 10.1038/s41572-019-0141-9. PMID: 31974366. Accessed February 01, 2023.

Benlysta® [Package Insert]. Rockville, MD: Human Genome Sciences, Inc.; February 2023. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=2fa3c528-1777-4628-8a55-a69dae2381a3&type=display. Accessed April 17, 2023.

Gladman DD, Pisetski DS, Curtis MR. Clinical manifestations of systemic lupus erythematosus in adults. UpToDate. Waltham, MA: UpToDate Inc. https://www-uptodate-com.proxy1.lib.tju.edu/contents/overview-of-the-clinical-manifestations-of-systemic-lupus-erythematosus-in-adults?source=search_result&search=lupus&selectedTitle=1~150. Accessed on April 17, 2023.

Lupus facts and statistics. Lupus Foundation of America Web Site. https://resources.lupus.org/entry/facts-and-statistics. Published 2017. Accessed April 17, 2023.

Lupkynis™ (voclosporin) [prescribing information]. Rockville, MD: Aurinia Pharma U.S., Inc.; January 2021. Available from: https://d1io3yog0oux5.cloudfront.net/auriniapharma/files/pages/lupkynis-prescribing-information/FPI-0011+Approved+USPI++MG.pdf. Accessed April 17, 2023.​



93/16/20233/16/20246/29/2023 5:56 AMNo presence informationsrv_ppsgw_NP

Rx.01.33 Off-Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit

Brand NameGeneric Name
Benlysta®belimumab
Lupkynis™voclosporin
207
  
7/1/2023Rx.01.226CommercialOyenusi, Oluwadamilola

Seizures can result from a shift in the normal balance of excitation and inhibition within the CNS as well as abnormal brain function. Epilepsy is a chronic medical disorder when two or more unprovoked seizures occur that can't be explained by a medical condition. Abnormal, excessive, and hypersynchronous electrical discharge of neurons in the brain can manifest epileptic seizures. Seizure clusters, also known as acute repetitive seizures are frequent seizure activities that are distinct from a patient's usual seizure pattern. Benzodiazepines are used as a rescue medication for seizure clusters in an outpatient setting.

Midazolam (Nayzilam®) nasal spray is a benzodiazepine indicated for the acute treatment of intermittent, stereotypic  episodes of  frequent seizure activity (i.e., seizure clusters, acute repetitive seizures) that are distinct from a patient's usual seizure pattern in patients with epilepsy 12 years of age and older.

Diazepam (Valtoco®) nasal spray is a  benzodiazepine indicated for the acute treatment of intermittent, stereotypic episodes of frequent seizure activity (i.e., seizure clusters, acute repetitive seizures) that are distinct from a patient's usual seizure pattern in patients with epilepsy 6 years of age and older.

The exact mechanism of action for Nayzilam® and Valtoco® is not fully understood, but it is thought to involve potentiation of GABAergic neurotransmission resulting from binding at the benzodiazepine site of the GABAA receptor.


 

The intent of this policy is to communicate the medical necessity criteria for midazolam (Nayzilam®) nasal spray and diazepam (Valtoco®) nasal spray as provided under the member's prescription drug benefit.


 

INITIAL CRITERIA: Midazolam (Nayzilam®) or Diazepam (Valtoco®) nasal spray is approved when ALL of the following are met:

  1. Diagnosis of intermittent, stereotypic episodes of frequent seizure activity (i.e., seizure clusters, acute repetitive seizures) that are distinct from a patient's usual seizure pattern; and
  2. ONE of the following:
    1. For midazol​​am (Nayzilam®) only, member is 12 years of age or older; or
    2. For diazepam (Valtoco®) only, member is 6 years of age or older; and 
  3. ​Prescribed by or in consultation with a neurologist/epilepsy specialist


Initial Authorization duration: 2 years

REAUTHORIZATION CRITERIA: Midazolam (Nayzilam®) nasal spray or diazepam (Valtoco®) nasal spray is reapproved when there is documentation of positive clinical response to therapy.


Reauthorization duration: 2 years



 

Benzodiazepines (Nayzilam®, Valtoco®):

Concomitant use of benzodiazepines and opioids may result in profound sedation, respiratory depression, coma, and death. Reserve concomitant prescribing of these drugs for patients for whom alternative treatment options are inadequate. Limit dosages and durations to the minimum required. Follow patients for signs and symptoms of respiratory depression and sedation. The use of benzodiazepines exposes users to risks of abuse, misuse, and addiction, which can lead to overdose or death. Abuse and misuse of benzodiazepines commonly involve concomitant use of other medications, alcohol, and/or illicit substances, which is associated with an increased frequency of serious adverse outcomes. Before prescribing Nayzilam or Valtoco and throughout treatment, assess each patient's risk for abuse, misuse, and addiction. The continued use of benzodiazepines may lead to clinically significant physical dependence. The risks of dependence and withdrawal increase with longer treatment duration and higher daily dose. Although Nayzilam and Valtoco is indicated only for intermittent use if used more frequently than recommended abrupt discontinuation or rapid dosage reduction may precipitate acute withdrawal reactions, which can be life-threatening. For patients using Nayzilam or Valtoco more frequently than recommended, to reduce the risk of withdrawal reactions, use a gradual taper to discontinue. 


 


Nayzilam® (midazolam nasal spray) [prescribing information]. Smyrna, GA: UCB Inc.; January 2023. Available from: https://www.ucb-usa.com/_up/ucb_usa_com_kopie/documents/Nayzilam_PI.pdf. Accessed April 17, 2023.

Valtoco® (diazepam nasal spray) [prescribing information]. San Diego, CA: Neurelis, Inc.; January 2023. Available from: https://www.valtoco.com/sites/default/files/Prescribing_Information.pdf. Accessed April 17, 2023.

Jafarpour, Saba & Hirsch, Lawrence & Gaínza-Lein, Marina & Kellinghaus, Christoph & Detyniecki, Kamil. (2018). Seizure cluster: Definition, prevalence, consequences, and management. Seizure. 68. 10.1016/j.seizure.2018.05.013.​




 

43/16/20233/16/20246/29/2023 5:57 AMNo presence informationsrv_ppsgw_NP

Off-Label Use Rx.01.33

Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit Rx.01.76​


 

Inclusion of a drug in this table does not imply coverage. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.


 

Brand nameGeneric name
Nayzilam®Midazolam
Valtoco®Diazepam


 

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7/1/2023Rx.01.249CommercialOyenusi, Oluwadamilola

​Asthma is a chronic respiratory condition characterized by reversible airway obstruction associated with airway hyperresponsiveness and airway inflammation. Generally, most asthma starts in childhood in relation to sensitization to common inhaled allergens, such as dust mites, animal dander and pollens. These allergens stimulate T helper type 2 cell proliferation and subsequently stimulate the release of inflammatory mediators IL-4, IL-5 and IL-13. These inflammatory mediators then cause inflammation in the airways, which left untreated can result in airway remodeling or thickening of the airway walls, ultimately leading to airway narrowing and airflow obstruction.

Nasal polyps are benign outgrowths of the nasal mucosa and are a type of inflammatory mucosal nasal obstructions. While benign, they can cause some symptoms, such as stuffiness or a blocked feeling in the nose, feelings of pressure in the face and trouble smelling. Polyp tissues have been found to have high levels of T helper type 2 cytokines IL-5 and IL-13, as well as high levels of histamine, which are all thought to have a function in pathogenesis of nasal polyps. Development of nasal polyps is often associated with asthma and also with adverse reactions to aspirin and other NSAIDS.
Chronic idiopathic urticaria is defined as the occurrence of wheals and/or angioedema for a total duration of six weeks or more. It is characterized by the appearance of itchy, red-colored papules, also known as urticaria, wheals or hives, which can appear on any part of the body. Angioedema also occurs in some patients, characterized by swelling around the lips, cheeks, periorbital areas of the face, extremities, and genitals. Pathogenesis of chronic idiopathic urticaria is not fully understood but is thought to involve the activation of mast cells and basophils. 
 
Omalizumab inhibits the binding of IgE to the high-affinity IgE receptor FcRI on the surface of mast cells, basophils, and dendritic cells, resulting in FcRI down-regulation on these cells. In allergic asthmatics and those with nasal polyps, treatment with omalizumab inhibits IgE-mediated inflammation, as evidenced by reduced blood and tissue eosinophils and reduced inflammatory mediators, including IL-4, IL-5 and IL-13. The mechanism by which omalizumab improves symptoms of chronic idiopathic urticaria, however, is currently unknown.
Xolair® (omalizumab) is indicated for the treatment of asthma, nasal polyps and chronic idiopathic urticaria.
 

​The intent of this policy is to communicate the medical necessity criteria for Omalizumab (Xolair®)  as provided under the member's prescription drug benefit.

Allergic asthma

INITIAL CRITERIA: Omalizumab (Xolair®) is approved when ALL of the following are met:

  1. Submission of medical records (e.g., chart notes, lab values) confirming a diagnosis  of moderate to severe persistent allergic asthma; and
  2. Submission of documentation (e.g., chart notes, lab values) confirming a  positive skin test or in vitro reactivity to a perennial aeroallergen; and
  3. One of the following:
    1. Both of the following:
      1. Member is 12 years of age or older; and
      2. Submission of documentation (e.g., chart notes, lab values) confirming pre- treatment serum immunoglobulin IgE level between 30 to 700 IU/mL; or
    2. Both of the following:
      1. Member is 6 years to less than 12 years of age; and
      2. Submission of documentation (e.g., chart notes, lab values) confirming pre-treatment serum immunoglobulin IgE level between 30 to 1300 IU/mL; and
  4. Paid claims or submission of documentation (e.g., chart notes) confirming that the member ​is currently being treated with one of the following unless there is a contraindication or intolerance to one of these medications:
    1. Both of the following:
      1. High dose inhaled corticosteroid (ICS) (e.g. greater than 500 mcg fluticasone propionate equivalent/day); and
      2. Additional asthma controller medication (e.g. leukotriene receptor antagonist, long-acting beta-2 agonist [LABA], tiotropium); or
    2. One maximally -dosed combination ICS/ LABA product (e.g. Advair [fluticasone propionate/salmeterol], Breo Ellipta [fluticasone/vilanterol], etc.); and
  5. Prescribed by or in consultation with one of the following:
    1. Pulmonologist; or
    2. Allergist/ immunologist; and
  6. Treatment will be initiated in a healthcare setting with at least 3 doses administered under the supervision of a healthcare provider; and
  7. No concurrent therapy with any other biologic agents; and
  8. Dosing and frequency does not exceed maximum FDA recommendation for indication requested 

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Omalizumab (Xolair®) is re-approved when ALL of the following are met:

  1. Submission of documentation (e.g., chart notes) confirming a positive clinical response to therapy (e.g., reduction in exacerbations, improvement in forced expiratory volume in 1 second [FEV1], decreased use of rescue medications); and
  2. Prescribed by or in consultation with one of the following:
    1. Pulmonologist; or
    2. Allergist/Immunologist; and
  3. Paid claims or submission of documentation (e.g., chart notes) confirming that the member is currently being treated with one of the following unless there is a contraindication or intolerance to one of these medications:
    1. Both of the following:
      1. High dose inhaled corticosteroid (ICS) (e.g., greater than 500 mcg fluticasone propionate equivalent/day); and 
      2. Additional asthma controller medication (e.g., leukotriene receptor antagonist, long-acting beta-2 agonist [LABA], tiotropium); or 
    2. One maximally dosed combination ICS/ LABA product (e.g., Advair [fluticasone propionate/salmeterol], Breo Ellipta [fluticasone/vilanterol], etc.); and 
  4. No concurrent therapy with any other biologic agents; and
  5. Dosing and frequency does not exceed maximum FDA recommendation per indication requested
Reauthorization duration: 2 years
 DOSE
Adults and pediatric patients 6 years of age and older75 to 375 mg every 2 or 4 weeks. Determine dose (mg) and dosing frequency by serum total IgE level, measured before the start of treatment, and body weight (kg). Refer to omalizumab (Xolair®) Prescribing Information for additional information.


Chronic Idiopathic Urticaria

INITIAL CRITERIA Omalizumab (Xolair®) is approved when ALL of the following are met:

  1. Submission of medical records (e.g., chart notes, lab values) confirming a diagnosis of chronic idiopathic urticaria; and
  2. Member is 12 years of age or older; and
  3. Paid claims or submission of documentation (e.g., chart notes) confirming an inadequate response or inability to tolerate one second- generation antihistamine (e.g. desloratadine) at maximally tolerated dose in addition to ONE of the following:
    1. Substituting to a different second- generation antihistamine; or
    2. H2 antihistamine(e.g. famotidine); or
    3. Leukotriene receptor antagonist (e.g. montelukast); or
    4. Systemic corticosteroids; or
    5. cyclosporine; and
  4. Paid claims or submission of documentation (e.g., chart notes) confirming that Xolair will be used concurrently with an H1 antihistamine, unless there is a contraindication or intolerance to H1 antihistamine; and
  5. Prescribed by or in consultation with one of the following:
    1. Allergist/ immunologist; or
    2. Dermatologist; and
  6. Treatment will be initiated in a healthcare setting with at least 3 doses administered under the supervision of a healthcare provider; and
  7. No concurrent therapy with any other biologic agents; and
  8. Dosing and frequency does not exceed maximum FDA recommendation for indication requested ​

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Omalizumab (Xolair®) is re-approved when ALL of the following are met:

  1. Member’s disease status has been re-evaluated since the last authorization to confirm the member’s condition warrants continued treatment; and
  2. Submission of documentation (e.g., chart notes) confirming that the membermember has experienced at least one of the following:
    1. Reduction in itching severity from baseline; or
    2. Reduction in the number of hives from baseline; and
  3. Prescribed by or in consultation with one of the following:
    1. Dermatologist; or
    2. Allergist/Immunologist; and
  4. No concurrent therapy with any other biologic agents; and
  5. Dosing and frequency does not exceed maximum FDA recommendation for indication requested ​

Reauthorization duration: 2 years​

 DOSE
Adults and pediatric patients 12 years of age and older150 mg or 300 mg every 4 weeks.


Nasal Polyps

INITIAL CRITERIA: Omalizumab (Xolair®) is approved when ALL of the following are met:

  1. Submission of medical records (e.g., chart notes, lab values) confirming a diagnosis of nasal polyps; and
  2. Member has baseline serum IgE level of between 30 IU/mL and 1500 IU/mL; and
  3. Paid claims or submission of documentation confirming an inadequate response or inability to tolerate at least a 2-month treatment with  intranasal corticosteroid; and
  4. Used in combination with another agent for nasal polyps (e.g. intranasal corticosteroid); and
  5. Prescribed by or in consultation with one of the following:
    1. Allergist/Immunologist; or
    2. ENT specialist; or
    3. Pulmonologist; and
  6. Treatment will be initiated in a healthcare setting with at least 3 doses administered under the supervision of a healthcare provider; and
  7. No concurrent therapy with any other biologic agents; and
  8. Dosing and frequency does not exceed maximum FDA recommendation for indication requested ​

Initial authorization duration: 2 years 

REAUTHORIZATION CRITERIA: Omalizumab (Xolair®) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy (e.g., reduction in nasal polyps score [NPS: 0-8 scale], improvement in nasal congestion/obstruction score [NCS: 0-3 scale]); and
  2. Used in combination with another agent for nasal polyps (e.g. intranasal corticosteroid); and
  3. Prescribed by or in consultation with one of the following:
    1. Allergist/immunologist; or
    2. ENT specialist; or
    3. Pulmonologist; and
  4. No concurrent therapy with any other biologic agents; and
  5. Dosing and frequency does not exceed maximum FDA recommendation for indication requested ​

Reauthorization duration: 2 years

 DOSE
Adults 75 to 600 mg every 2 or 4 weeks. Determine dose (mg) and dosing frequency by serum total IgE level, measured before the start of treatment, and body weight (kg). Refer to omalizumab (Xolair®) Prescribing Information for additional information.


Anaphylaxis:

  • Anaphylaxis presenting as bronchospasm, hypotension, syncope, urticaria, and/or angioedema of the throat or tongue, has been reported to occur after administration of XOLAIR. Anaphylaxis has occurred as early as after the first dose of XOLAIR, but also has occurred beyond 1 year after beginning regularly administered treatment.
  • Because of the risk of anaphylaxis, initiate XOLAIR therapy in a healthcare setting and closely observe patients for an appropriate period of time after XOLAIR administration. Health care providers administering XOLAIR should be prepared to manage anaphylaxis which can be life-threatening.
  • Inform patients of the signs and symptoms of anaphylaxis and instruct them to seek immediate medical care should symptoms occur. Selection of patients for self-administration of XOLAIR should be based on criteria to mitigate risk from anaphylaxis


Xolair® (omalizumab) [prescribing information]. South San Francisco, CA: Genentech, inc.; March 2023. Available at: https://www.gene.com/download/pdf/xolair_prescribing.pdf. Accessed April 19, 2023.

Kudo M. Ishigatsubo Y, Aoki I. Pathology of asthma. Front Microbiol. 2013 Sep 10;4:263. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3768124. Accessed ​April 19, 2023.

Saini S. Chronic spontaneous urticaria: clinical manifestations, diagnosis, pathogenesis, and natural history. UpToDate. November 2021. Available at: https://www.uptodate.com/contents/chronic-spontaneous-urticaria-clinical-manifestations-diagnosis-pathogenesis-and-natural-history. Accessed April 19, 2023.

Hamilos DL, Holbrook EH. Chronic rhinosinusitis: clinical manifestations, pathophysiology, and diagnosis. UpToDate. September 2022. Available at: https://www.uptodate.com/contents/chronic-rhinosinusitis-clinical-manifestations-pathophysiology-and-diagnosis. Accessed April 19, 2023.


43/16/20233/16/20246/29/2023 5:57 AMNo presence informationsrv_ppsgw_NP
Rx.01.33 Off Label Use​
Brand NameGeneric Name
Xolair®Omalizumab

220
  
7/1/2023Rx.01.262CommercialOyenusi, Oluwadamilola

​Immunoglobulin A nephropathy (IgAN) or Berger’s disease is a condition that damages the glomeruli inside the kidneys and can cause kidney disease. The kidney gets inflamed and can cause the kidneys to leak blood and protein which leads to loss of kidney function and kidney failure. 

Budesonide is a corticosteroid with potent glucocorticoid activity and weak mineralocorticoid activity that undergoes substantial first pass metabolism. Mucosal B-cells present in the ileum, including the Peyer's patches, express glucocorticoid receptors and are responsible for the production of galactose-deficient IgA1 antibodies (Gd-Ag1) causing IgA nephropathy. Through their anti-inflammatory and immunosuppressive effects at the glucocorticoid receptor, corticosteroids can modulate B-cell numbers and activity. It has not been established to what extent TARPEYO's efficacy is mediated via local effects in the ileum vs systemic effects.

TARPEYO is indicated to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk of rapid disease progression, generally a urine protein-to-creatinine ratio (UPCR) ≥1.5 g/g.



​The intent of this policy is to communicate the medical necessity criteria for Budesonide (Tarpeyo™) as provided under the member's prescription drug benefit. 

​Budesonide (Tarpeyo™) is approved when ALL of the following are met:

  1. Diagnosis of primary immunoglobulin A nephropathy (IgAN) as confirmed by a kidney biopsy; and
  2. Member is 18 years of age or older; and
  3. Member is at risk of rapid disease progression (e.g., generally a urine protein-to-creatinine ratio (UPCR) greater than or equal to 1.5 g/g, or by other criteria such as clinical risk scoring using the International IgAN Prediction Tool); and
  4. Used to reduce proteinuria; and
  5. Estimated glomerular filtration rate (eGFR) greater than or equal to 35 ml/min/1.73 m2; and
  6. One of the following:
    1. Member has been on a minimum 90-day trial of maximally tolerated dose and will continue to receive therapy with one of the following:
      1. An angiotensin-converting enzyme (ACE) inhibitor (e.g., benazepril, lisinopril); or
      2. An angiotensin II receptor blocker (ARB) (e.g., losartan, valsartan); or
    2. Member is unable to tolerate BOTH ACE inhibitors and ARBs; and
  7. Inadequate response or inability to tolerate another glucocorticoid (e.g., prednisone, methylprednisolone); and
  8. Prescribed by or in consultation with a nephrologist

Authorization duration: 9 months



​N/A

Tarpeyo (budesonide) [package insert]. Stockhelm, Sweden: Calliditas Therapeutics AB. December 2021. Available at: https://dailymed.nlm.nih.gov/dailymed/lookup.cfm?setid=938cada4-d6bf-4252-836f-dd40f9eadb4d. Accessed April 18, 2023.

Cattran DC. IgA nephropathy: Treatment and prognosis. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed April 18, 2023.​​


23/16/20233/16/20246/29/2023 5:58 AMNo presence informationsrv_ppsgw_NP

​Rx.01.33 Off Label Use​

Brand NameGeneric Name
Tarpeyo™Budesonide



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7/1/2023Rx.01.268CommercialOyenusi, Oluwadamilola

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that causes muscle weakness, disability, and eventually death, with a median survival of three to five years. The hallmark of ALS is the combination of upper motor neuron (UMN) and lower motor neuron (LMN) involvement. The LMN findings of weakness, atrophy, and fasciculations are a direct consequence of muscle denervation. The UMN findings of hyperreflexia and spasticity result from degeneration of the lateral corticospinal tracts in the spinal cord.

Edaravone is an oral suspension indicated for the treatment of amyotrophic lateral sclerosis (ALS).

Edaravone is neuroprotective agent working in the central nervous system. Edaravone is a free radical and peroxynitrite scavenger that prevents oxidative damage to the cell membranes and may contribute to inhibiting the progression of ALD. However, the mechanism of edaravone slowing the decline of physical function in patients with ALS is unknown. 



​The intent of this policy is to communicate the medical necessity criteria for Edaravone (Radicava ORS®) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Edaravone (Radicava ORS®) is approved when ALL of the following are met:

  1. Diagnosis of “definite” or “probable” amyotrophic lateral sclerosis (ALS) per the revised EL Escorial and Airlie House diagnostic criteria; and
  2. Prescribed by or in consultation with a neurologist with expertise in the diagnosis of ALS; and
  3. Member has scores greater than or equal to 2 in all items of the ALS Functional Rating Scale-Revised (ALSFRS-R) criteria at the start of treatment; and 
  4. Member has a percent (%) forced vital capacity (%FVC) greater than or equal to 80% at the start of treatment 

Initial authorization duration: 12 months


REAUTHORIZATION CRITERIA: Edaravone (Radicava ORS®) is re-approved when BOTH of the following are met:

  1. Documentation of positive clinical response to therapy (e.g., slowing in the decline of functional abilities); and
  2. Member is not dependent on invasive ventilation of tracheostomy 

Reauthorization duration: 12 months 



​N/A

Radicava ORS® (Edaravone) [package insert]. Jersey City, NJ: Mitsubishi Tanabe Pharma America, Inc. May 2022. Available from: https://www.radicava.com/pdfs/radicava-prescribing-information.pdf. Accessed April 19, 2023.

 

Goyal NA. Disease-modifying treatment of amyotrophic lateral sclerosis. UpToDate website. Last updated January 2023. Available at: www.uptodate.com. Accessed April 19, 2023.


23/16/20233/16/20246/29/2023 5:58 AMNo presence informationsrv_ppsgw_NP

​​Rx.01.33 Off Label Use


Brand NameGeneric Name
Radicava ORS®Edaravone





225
  
7/1/2023Rx.01.222CommercialOyenusi, Oluwadamilola

Attention Deficit Hyperactivity Disorder (ADHD) is one of the most common neuropsychiatric disorders of childhood and adolescences where symptoms often persist into adulthood. It is associated with significant impairment in occupational, academic, and social functioning. ADHD in adults is characterized by symptoms of inattention, impulsiveness, restlessness, executive dysfunction, and emotional dysregulation. Compared to adults with ADHD, symptoms of inattention, hyperactivity and impulsive behaviors are more prominent in children and adolescents.

Prescription stimulants are an integral part of treatment for attention deficit hyperactivity disorder (ADHD) but it is also one of the most frequently abused prescription drugs in the United States. In 2016, an estimated 5,647,000, or 2.1% of persons aged 12 and older, reported misuse of prescription stimulants in the past year. In 2017, a total of 10,333 deaths involving psychostimulants occurred, representing 14.7% of drug overdose deaths and a 37.0% increase from 2016 per the CDC.

The cumulative dose limit is based on the cumulative daily dose of drugs with the same active ingredient.  Prior authorization is required when the request exceeds the recommended dose for medications with that active ingredient outlined below:

High cumulative daily dose limit - This limit is in place to ensure alternative medications have been reviewed and potential adverse effects have been accessed.

Active ingredient

Medications impacted

(brands and generics)

High cumulative daily dose
Amphetamine

Adzenys® [XR ODT/ER]

Dyanavel® [XR]

Evekeo® [ODT]

60mg/day

Amphetamine/

Dextroamphetamine

Adderall® [IR/XR]

Mydayis®

60mg/day
Dextroamphetamine

Dexedrine®

Zenzedi®

ProCentra®

Xelstrym™

60mg/day
LisdexamfetamineVyvanse®70mg/day
MethamphetamineDesoxyn®60 mg/day
DexmethylphenidateFocalin® [IR/XR]40mg/day
Methylphenidate

Ritalin® [IR/LA]

Daytrana®

Cotempla®

Metadate® [ER/CD] Methylin®

Quillivant® XR

Concerta®

Aptensio® XR

QuilliChew® ER

Adhansia® XR

Jornay PM™

Methylphenidate (Relexxii®)

72mg/day
SerdexmethylphenidateAzstarys™52.3 mg/day

 

​The intent of this policy is to communicate the medical necessity criteria for prescription stimulants under the member's prescription benefit when the cumulative dose of an active ingredient exceeds the limit set by the plan.

Doses above the high cumulative dose are approved when ALL of the following are met:

  1. Inadequate response or inability to tolerate an alternative active ingredient within the CNS stimulant drug class prior to increasing the dose beyond the cumulative high dose limit; and
  2. Member has been assessed for, and counseled by prescriber on ALL of the following:
    1. The risk for substance abuse; and
    2. The risk for cardiac related adverse events (i.e., hypertension); and
    3. The risk for new or worsening psychosis (i.e., maniac behavior); and
  3. Requests that exceed the cumulative daily dose for the total daily dose of the drug, dose frequency, or duration of therapy approved or recommended by the FDA or as stated in accepted compendia are considered off-label and are reviewed per Off-Label Use policy

Authorization duration: 12 months

INITIAL CRITERIA: Amphetamine (Evekeo®) is 
approved when ONE of the following is met:

  1. Diagnosis of narcolepsy and ALL of the following:
    1. Recommended by a neurologist or sleep specialist; and
    2. Inadequate response or inability to tolerate generic modafinil or armodafinil; and
    3. Diagnosis confirmed by ONE of the following tests:
      1. Polysomnography (PSG); or
      2. Multiple sleep latency test (MSLT); or
  2. Attention Deficit Disorder with Hyperactivity (ADHD) with inadequate response or inability to tolerate TWO of the following generic stimulant agents for ADHD:
    1. Methylphenidate
    2. Mixed amphetamine salts
    3. Dextroamphetamine
    4. Methamphetamine hydrochloride
    5. Dexmethylphenidate; or
  3. Exogenous Obesity:
    1. If member has weight loss rider please refer to Weight Loss Agents policy for approval criteria and duration; or
    2. If member does not have a weight loss agents rider; deny as benefit exclusion*

*Drugs that are used for weight loss are covered only with weight loss rider. 

Initial authorization duration: 2 years
 
REAUTHORIZATION CRITERIA: Amphetamine (Evekeo®) is 
re-approved when there is documentation of positive clinical response.
 
Reauthorization duration: 2 years

Quantity limit requests are approved when ONE of the following is met: (drug specific criteria below).

  1. Requests that exceed the cumulative daily dose, dose frequency, or duration of therapy approved or recommended by the FDA or as stated in accepted compendia1 are considered off-label and are reviewed per Off-Label Use policy, or
  2. Requests that do not exceed the cumulative daily dose, dose frequency or duration of therapy approved or recommended by the FDA or as stated in accepted compendia1 : A quantity limit exceeding those listed in the following table is approved when ONE of the following is met:
    1. Documentation of the inability to reach the requested dose with higher strengths of commercially available dosage forms due to member specific characteristics (i.e., inability to swallow larger pills, malabsorption, presence of a feeding tube, etc.); or
    2. The requested dose is not commercially available; or
    3. The requested dose is used for titration or loading-dose purposes (one-time authorization)

1 Please refer to the Off-Label Use policy for definition of accepted compendia

Authorization duration: 2 years

MedicationMaximum Quantity per day Quantity limit per rolling 30 days, unless otherwise specified (tablets, capsules, mL)
Amphetamine (Adzenys XR®) 130
Amphetamine (Adzenys ER®) 1.25mg/ml susp15 ml450
Dextroamphetamine/Amphetamine (Adderall®) 5mg, 7.5mg, 10mg, 12.5mg, 15mg and 20mg390
Dextroamphetamine/Amphetamine (Adderall®) 30mg260
Dextroamphetamine/Amphetamine (Adderall XR®) 5mg, 10mg, 15mg, 20mg, 25mg, 30mg130
Amphetamine (Dyanavel XR®) 2.5mg/ml Susp 8mL240
Amphetamine (Dyanavel XR®) 5mg, 10mg, 15mg, 20mg tablets130
Dextroamphetamine sulfate (Dexedrine®) 5mg cap SA390
Dextroamphetamine sulfate (Dexedrine®) 10mg cap SA6180
Dextroamphetamine sulfate (Dexedrine®) 15mg cap SA4120
Dextroamphetamine sulfate 5mg tablet390
Dextroamphetamine sulfate 10mg tablet390
Dexmethylphenidate HCL (Focalin®) 2.5mg, 5mg, 10mg260
Dexmethylphenidate HCL (Focalin XR®) 5mg, 10mg, 15mg, 20mg, 25mg, 30mg, 35mg, 40mg130
Dextroamphetamine sulfate (Procentra®) 5mg/5ml solution601800
Dextroamphetamine (Xelstrym™) 13.5 MG/9HR, 18 MG/9HR, 4.5 MG/9HR, 9 MG/9HR130
Dextroamphetamine (Zenzedi®) 2.5mg, 5mg, 7.5mg, 10mg, 15mg, 20mg tablet390
Dextroamphetamine (Zenzedi®) 30mg tablet260
Amphetamine (Evekeo®) 5mg390
Amphetamine (Evekeo®) 10mg4120
Amphetamine (Evekeo® ODT)390
Lisdexamfetamine dimesylate (Vyvanse®) 20mg, 30mg, 40mg, 50mg, 60mg, 70mg130
Methylphenidate HCL ER 24HR (Adhansia™ XR) 25mg, 35mg, 45mg, 55mg, 70mg, 85mg130
Methylphenidate (Aptensio XR®)130
Methylphenidate HCL (Concerta®) 18mg, 27mg, 54mg130
Methylphenidate HCL (Concerta®) 36mg tablet ER260
Methylphenidate HCL ER 72mg
osmotic release tab
130
Methylphenidate (Relexxii®) tab ER osmotic release 45mg, 63mg130
Methylphenidate (Daytrana patch®) 10mg/9hr, 15mg/9hr, 20mg/9hr, 30mg/9hr130
Methamphetamine HCL (Desoxyn®) 5mg tablet5150
Methylphenidate HCL ER capsule (Jornay™ PM) 20mg, 40mg, 60mg, 80mg, 100mg130
Methylpenidate HCL (Metadate CD®) 10mg, 20mg, 30mg, 40mg, 50mg, 60mg130
Methylphenidate HCL (Metadate ER®) 10mg, 20mg tablet SA390
Methylphenidate HCL (Methylin®) 2.5mg, 10mg chewable tablet6180
Methylphenidate HCL (Methylin®) 5mg chewable tablet390
Methylphenidate HCL (Methylin®) 10mg/5ml30900
Methylphenidate HCL (Methylin®) 5mg/5ml601800
Methylphenidate HCL (Ritalin®) 5mg, 10mg, 20mg390
Methylphenidate HCL (Ritalin LA®) 10mg, 40mg capsule130
Methylphenidate HCL (Ritalin LA®) 20mg capsule390
Methylphenidate HCL (Ritalin LA®) 30mg capsule260
Methylphenidate (Quillichew ER®) 20mg, 30mg260
Methylphenidate (Quillichew ER®) 40mg 130
Methylphenidate (Quillivant XR®) 5mg/mL 12mL360
Methylphenidate ER disintegrating tabs (Cotempla®) 8.6mg, 17.3mg, 25.9mg130
Serdexmethylphenidate-dexmethylphenidate (Azstarys™) 130

Adzenys XR-ODT™, Adzenys ER®, Adderall®, Adderall® XR, Adhansia XR™, Aptensio XR™, Azstarys™, Concerta®, Dyanavel® XR, Dexedrine®, Dextroamphetamine sulfate, Evekeo®, Focalin®, Focalin® XR, Jornay PM™, Metadate® CD/ER, methylphenidate ER, Mydayis®, QuilliChew ER™, Quillivant XR®, Ritalin®, Ritalin [LA]®, Zenzedi™, Vyvanse®, Cotempla®, Dexedrine® (dextroamphetamine), ProCentra®, Xelstrym™ 

  1. Abuse and dependence: CNS stimulants, other amphetamine-containing products, and methylphenidate, have a high potential for abuse and dependence. Assess the risk the risk of abuse prior to prescribing and monitor for signs of abuse and dependence while on therapy.

Methylin®, Ritalin®, Concerta®, Daytrana®, Relexxii®

  1. Drug dependence: should be given cautiously to patients with a history of drug dependence or alcoholism. Chronic, abusive use can lead to marked tolerance and psychological dependence with varying degrees of abnormal behavior. Frank psychotic episodes can occur, especially with parenteral abuse. Careful supervision is required during drug withdrawal from abusive use since severe depression may occur. Withdrawal following chronic therapeutic use may unmask symptoms of the underlying disorder that may require follow-up.

Desoxyn®

  1. Methamphetamine has a high potential for abuse. It should thus be tried only in weight reduction programs for patients in whom alternative therapy has been ineffective. Administration of methamphetamine for prolonged periods of time in obesity may lead to drug dependence and must be avoided. Particular attention should be paid to the possibility of subjects obtaining methamphetamine for non-therapeutic use or distribution to others, and the drugs should be prescribed or dispensed sparingly. Misuse of methamphetamine may cause sudden death and serious cardiovascular adverse events.     

Adzenys ER® (amphetamine) [prescribing information]. Grand Prairie, TX: Neos Therapeutics; December 2017. Accessed April 20, 2023. 

Adzenys XR-ODT™ (amphetamine) [prescribing information]. Grand Prairie, TX: Neos Therapeutics; February 2018. Accessed April 20, 2023.

Adderall® (dextroamphetamine/amphetamine) [prescribing information]. North Wales, PA: Teva Pharmaceuticals; July 2018. Accessed April 20, 2023.

Adderall XR® (dextroamphetamine/amphetamine) [prescribing information]. Social Circle, GA: Shire US Manufacturing Inc.; July 2019. Accessed April 20, 2023.

Adhansia XR™ (methylphenidate) [prescribing information]. Wilson, NC: Adlon Therapeutics L.P. July 2019. Accessed April 20, 2023.

American Psychiatry Association. Attention-Deficit/Hyperactivity Disorder. In: Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, American Psychiatric Association, Arlington, VA 2013. P.59

Aptensio XR™ (methylphenidate) [prescribing information]. Coventry, RI: Rhodes Pharmaeuticals; June 2019. Accessed April 20, 2023.

Azstarys™ (serdexmethylphenidate and dexmethylphenidate) [prescribing information]. Grand Rapids, MI: Corium, Inc. June 2021. Accessed April 20, 2023.

Concerta® (methylphenidate) [prescribing information]. Horsham, PA: Janssen Pharmaceuticals, Inc; January 2017. Accessed April 20, 2023.

Cotempla XR-ODT® (methylphenidate extended-release orally disintegrating tablet) [prescribing information. Grand Prairie, TX: Neos Therapeutics Brands, LLC. June 2017. Accessed April 20, 2023.

Daytrana® patch (methylphenidate) [prescribing information]. New York, NY: Noven Therapeutics, LLC; October 2019. Accessed April 20, 2023.

Desoxyn® (methamphetamine HCL) [prescribing information]. Lebanon, NJ: Recordati Rare Diseases, Inc; March 2019. Accessed April 20, 2023.

Dexedrine® (dextroamphetamine) [prescribing information]. Horsham, PA: Amedra Pharmaceuticals LLC; March 2019. Accessed April 20, 2023.

Dextroamphetamine sulfate [prescribing information]. Morgantown, WV: Mylan Pharmaceuticals Inc.; 2015. Accessed April 20, 2023.

Dosing Psychotropics: How High Can We Go? TCPR, September 2007, Vol. 5, Issue 9, Complex Psychopharmacology

Dyanavel® XR (amphetamine) [prescribing information]. Monmouth Junction, NJ: Tris Pharma Inc; February 2019. Accessed April 20, 2023.

Evekeo® (amphetamine) [prescribing information]. Atlanta, GA: Arbor Pharmaceuticals, LLC. September 2016. Accessed April 20, 2023.

Evekeo® ODT (amphetamine) [prescribing information]. Atlanta, GA. Arbor Pharmaceuticals, LLC. March 2019.  Accessed April 20, 2023.

Fayyad J, De Graaf R, Kessler R, et al. Cross-national prevalence and correlates of adult attention-deficit hyperactivity disorders. Br J Psychiatry 2007; 190:420

Focalin® (dexmethylphenidate) [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; November 2019. Accessed April 20, 2023.

Focalin XR® (dexmethylphenidate) [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; November 2019. Accessed April 20, 2023.

Jornay PM™ (methylphenidate) [prescribing information]. Cherry Hill, NJ: Ironshore Pharmaceuticals, Inc. April 2019. Accessed April 20, 2023.

Kessler RC, Adler L, Barkley R, et al. The prevalence and correlates of adult ADHD in the United States: results from the National Comorbidity Survey Replication. Am J Psychiatry 2006; 163:716

Kariisa M, Scholl L, Wilson N, et. Al. Drug Overdose Deaths Involving Cocaine and Psychostimulants with Abuse Potential — United States, 2003–2017. MMWR 2019; 68(17);388–395

Metadate CD® (methyphenidate HCL) [prescribing information]. Rochester, NY: Unither Manufacturing, LLC; 2016. Accessed April 20, 2023.

Metadate® ER (methylphenidate HCL) [prescribing information]. Rochester, NY: Unither Manufacturing, LLC; April 2018. Accessed April 20, 2023.

Methylin® (methylphenidate HCL) [prescribing information]. Florham Park, NJ: Shionogi Inc.; August 2017. Accessed April 20, 2023.

Mydayis® (amphetamine mix salt) [prescribing information]. Lexington, MA: Shire US Inc. September 2019. Accessed April 20, 2023.

ProCentra® (dextroamphetamine sulfate) [prescribing information]. Charlotte, NC: FSC Laboratories, Inc; 2010. Accessed April 20, 2023.

Quillichew ER™ (methylphenidate) [prescribing information]. Cupertino, CA: NextWave Pharmaceuticals, Inc.; August 2018. Accessed April 20, 2023.

Quillivant XR® (methylphenidate) [prescribing information]. Cupertino, CA: NextWave Pharmaceuticals, Inc.; August 2018. Accessed April 20, 2023.

Relexxii® (methylphenidate hydrochloride extended-release tablets) [prescribing information]. Alpharetta, GA: Vertical Pharmaceuticals, LLC; November 2021. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=b7677cf9-95e0-4091-ac51-8f237c3f2635. Accessed April 20, 2023.

Ritalin® (methylphenidate HCL) [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; January 2019. Accessed April 20, 2023.

Ritalin LA® (methylphenidate HCL) [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; November 2019. Accessed April 20, 2023.

Spencer et al. A Large, double-blind, randomized clinical trial of methylphenidate in the treatment of adults with attention-deficit/hyperactivity disorder. Biological Psychiatry Volume 57, Issue 5, March 1, 2005, pages 456-463

Spencer et al. Efficacy of a mixed amphetamine salts compound in adults with ADHD. Archives general Psychiatry (2001).

Swanson, J. Long-acting stimulants: development and dosing. Can Child Adolesc Psychiatr Rev. 2005 Aug; 14(Suppl 1): 4-9.

Vyvanse® (lisdexamphetamine dimesylate) [prescribing information]. Exton, PA: Shire LLC; January 2018. Accessed April 20, 2023.

Xelstrym™ (dextroamphetamine) [prescribing information]. Miami, FL: Noven Pharmaceuticals; November 2022. Available from: https://www.xelstrym.com/. Accessed April 20, 2023.

Yanofski, J. The Dopamine-Dilemma – Part II: Could Stimulants Cause Tolerance, Dependence, and Paradoxical Decompensation? Innovations in Clinical Neuroscience, 2011.

Zenzedi™ (dextroamphetamine) [prescribing information]. Atlanta, GA: Arbor Pharmaceuticals, Inc.; 2017 Accessed April 20, 2023.


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​Off-Label Use Rx.01.33

Prior Authorization Requirements for Select Drugs Rx.01.202

Weight Loss Agents Rx.01.94

Brand NameGeneric Name
Adderall® [IR/XR]Amphetamine/Dextroamphetamine
Mydayis®Amphetamine/Dextroamphetamine
Adzenys® [XR ODT/ER]Amphetamine
Dyanavel® XRAmphetamine
Evekeo® [ODT]Amphetamine
Dexedrine® [IR/SA]Dextroamphetamine sulfate
ProCentra®Dextroamphetamine sulfate
Zenzedi®Dextroamphetamine
Xelstrym®Dextroamphetamine
Focalin® [IR/XR]Dexmethylphenidate HCL
Vvyanse®Lisdexamfetamine dimesylate
Adhansia XR™Methylphenidate HCL
Aptensio® XRMethylphenidate HCL
Concerta®Methylphenidate HCL
Daytrana®Methylphenidate HCL
Jornay PM™Methylphenidate HCL
Metadate® [ER/CD]Methylphenidate HCL
Methylin®Methylphenidate HCL
Ritalin® [IR/LA]Methylphenidate HCL
Quillichew® ERMethylphenidate HCL
Quillivant® XRMethylphenidate HCL
Cotempla®Methylphenidate ER
Relexxii®Methylphenidate ER
Desoxyn®Methamphetamine HCL
Azstarys™Serdexmethylphenidate/Dexmethylphenidate

227
  
7/1/2023Rx.01.225CommercialOyenusi, Oluwadamilola

​Diabetic foot ulcers are a prevalent complication of diabetes mellitus and represent major causes of morbidity and mortality. 15% of all diabetic individuals are affected by foot ulcers during their lifetime and 15-20% of those patients go on to need an amputation. Risk factors for development of diabetic foot ulcers include neuropathy, peripheral vascular disease, and poor glycemic control. Peripheral neuropathy results in patient loss of sensation and can exacerbate the development of ulcerations. Peripheral vascular disease can lead foot tissues to become ischemic. Many wounds go unnoticed and worsen through repetitive pressure because patients are unable to detect trauma to their lower extremities.  Multidisciplinary treatment today includes: surgical debridement, dressings promoting a moist wound environment, wound off-loading, vascular assessment, treatment of active infection, and glycemic control.

Regranex® gel is a recombinant human platelet-derived growth factor that promotes cellular proliferation and angiogenesis and thereby improve ulcer healing. Regranex® gel is indicated for the treatment of lower extremity diabetic neuropathic ulcers that extend into the subcutaneous tissue or beyond and have an adequate blood supply. Regranex® gel is indicated as an adjunct to, and not a substitute for, good ulcer care practices.

The intent of this policy is to communicate the medical necessity criteria for becaplermin (Regranex®) gel as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Becaplermin (Regranex®) gel is approved when BOTH of the following are met:

  1. Member has a lower extremity diabetic neuropathic ulcer; and
  2. Treatment will be given in combination with ulcer wound care (e.g., debridement, infection control, and/or pressure relief)

Initial authorization duration: 6 months

REAUTHORIZATION CRITERIA: Becaplermin (Regranex®) gel is re-approved when ONE of the following is met:

  1. Documentation of lower extremity diabetic neuropathic ulcer at a different treatment site; or
  2. Documentation of continued need for treatment beyond 6 months

Reauthorization duration: 6 months 

​None

Pendsey, S. Understanding diabetic foot. Int J Diabetes Dev Ctries 2010; 30:75-9. Accessed April 1, 2022

Armstrong, D., J de Asla, R. Management of diabetic foot ulcers. UpToDate. March 2023. Available from: https://www.uptodate.com/contents/management-of-diabetic-foot-ulcers?search=diabetic%20foot%20ulcer&source=search_result&selectedTitle=1~61&usage_type=default&display_rank=1. Accessed April 17, 2023.

Regranex® (becaplermin gel) [prescribing information]. Fort Worth, TX: Smith & Nephew, Inc.; December 2019.

Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2018/103691s5134lbl.pdf. Accessed 17, 2023.


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​Off Label Use Rx.01.33

Brand nameGeneric name
Regranex®Becaplermin
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4/1/2023Rx.01.274CommercialOyenusi, Oluwadamilola

Tuberous sclerosis complex (TSC) is an inherited neurocutaneous disorder that involve many organ systems, including developmental delay and multiple benign hamartomas of the brain, eyes, heart, lung, liver, kidney, and skin. The expression of the disease varies substantially among individuals and within families. Some individuals with TSC may demonstrate only dermatologic features of the disease while others may develop more serious neurologic or systemic manifestations. Nearly all patients with TSC have one or more of the skin lesions characteristic of the disorder.

The mechanism of action of sirolimus in the treatment of angiofibroma associated with tuberous sclerosis is unknown. Tuberous sclerosis is associated with genetic defects in TSC1 and TSC2 which leads to the constitutive activation of mammalian target of rapamycin (mTOR). Sirolimus inhibits mTOR activation.

Hyftor™ is indicated for the treatment of facial angiofibroma associated with tuberous sclerosis in adults and pediatric patients 6 years of age and older.


​The intent of this policy is to communicate the medical necessity criteria for Sirolimus topical gel (Hyftor™) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Sirolimus (Hyftor™) is approved when ALL of the following are met:

  1. Diagnosis of facial angiofibroma associated with tuberous sclerosis complex; and
  2. Member is 6 years of age or older; and
  3. Member is not a candidate for laser therapy or surgical treatments; and
  4. Prescribed by or in consultation with a dermatologist

​Initial authorization duration: 6 months

REAUTHORIZATION CRITERIA: Sirolimus (Hyftor™) is re-approved with documentation of positive clinical response to therapy (e.g., improvement in size or redness of facial angiofibroma)

Reauthorization duration: 2 years​


​N/A

Randle S. Tuberous sclerosis complex: Management and prognosis. UpToDate. Available from: uptodate.com. Accessed February 24, 2023.

HyftorTM (sirolimus) [package insert]. Bethesda, MD: Nobelpharma America, LLC; March 2022. Available at https://www.hyftor.com/wp-content/uploads/2022/04/Approved-PI.pdf. Accessed February 24, 2023.


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​Rx.01.33 Off Label Use​

Brand NameGeneric Name
Hyftor™sirolimus


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7/1/2023Rx.01.271CommercialOyenusi, Oluwadamilola

Human papillomavirus (HPV) is a common cause of cutaneous and mucosal infection. Condylomata acuminata (CA; singular: condyloma acuminatum), also known as anogenital warts, are manifestations of HPV infection that occur in a subset of individuals with anogenital HPV infection. External CA typically manifest as soft papules or plaques on the external genitalia, perianal skin, perineum, or groin. For most patients, the presence of genital warts is concerning because of their cosmetic appearance, association with a sexually transmitted disease, bothersome symptoms, absence of a cure, and social stigma. Although treatment can eradicate the warts, disease recurrence is common and occurs in 20 to 30 percent of patients overall.

The mode of action of Veregen involved in the clearance of genital and perianal warts is unknown. In vitro, sinecatechins had anti-oxidative activity; the clinical significance of this finding is unknown.
 
Veregen is indicated for the topical treatment of external genital and perianal warts (Condylomata acuminata) in immunocompetent patients 18 years and older.



​The intent of this policy is to communicate the medical necessity criteria for Sinecatechins (Veregen®) as provided under the member's prescription drug benefit.


Sinecatechins (Veregen®) is approved when all of the following are met:

  1. Diagnosis of Condylomata acuminata (external genital and perianal warts); and
  2. Member is immunocompetent; and
  3. Member is 18 years of age or older; and
  4. Inadequate response or inability to tolerate imiquimod (generic Aldara®)

Authorization duration: 4 months

Total duration of treatment is limited to 16 weeks per lifetime



​N/A

Rosen T. Condylomata acuminata (anogenital warts) in adults: Epidemiology, pathogenesis, clinical features, and diagnosis. In: Post T, ed. UpToDate. December 2022. www.uptate.com. Accessed April 20, 2023.

 

Veregen® (Sinecatechins) [prescribing information]. Melville, New York: PharmaDerm®; February 2022. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=2c1cd745-79ab-487d-b759-995794cedb92. Accessed April 20, 2023.​



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​Rx.01.33 Off Label Use​

Brand NameGeneric Name
Veregen®Sinecatechins


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7/1/2023Rx.01.273CommercialOyenusi, Oluwadamilola

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that causes muscle weakness, disability, and eventually death, with a median survival of three to five years. Incidence rates for ALS in Europe and North America range between 1.5 and 4.7 per 100,000 person-years, while prevalence rates range between 2.7 and 7.4 per 100,000 person-years. The loss of motor neurons results in the primary clinical symptoms and signs of ALS.

Sodium phenylbutyrate is a histone deacetylase inhibitor that reduces an adaptive stress response in the endoplasmic reticulum. Taurursodiol (also known as ursodoxicoltaurine) appears to increase the threshold of cellular apoptosis by maintaining mitochondrial integrity through reduced membrane permeability. A coformulation of both agents, sodium phenylbutyrate-taurursodiol (PB-TURSO), is used to reduce neuronal cell death.

Relyvrio™ is indicated for the treatment of amyotrophic lateral sclerosis (ALS) in adults.


The intent of this policy is to communicate the medical necessity criteria for Sodium phenylbutyrate and Taurursodiol (Relyvrio™) as provided under the member's prescription drug benefit.


INITIAL CRITERIA Sodium phenylbutyrate and taurursodiol (Relyvrio™) is approved when ALL of the following are met:

  1. Diagnosis of amyotrophic lateral sclerosis (ALS); and
  2. Diagnosis of ALS is further supported by neurogenic changes in electromyography (EMG); and
  3. Baseline functional ability has been conducted prior to initiating treatment (e.g., speech, walking, climbing stairs, etc.); and
  4. Other differential diagnoses (e.g., multifocal motor neuropathy, cervical radiculomyelopathy, inflammatory myopathies, spinobulbar muscular atrophy, myasthenia gravis, etc.) have been ruled out); and
  5. Member has a percent (%) forced vital capacity (%FVC) or slow vital capacity (%SVC) greater than or equal to 60% at the start of treatment; and
  6. Member does not require permanent noninvasive ventilation or invasive ventilation; and
  7. Member has had ALS symptoms for less than or equal to 18 months; and
  8. Member is 18 years of age or older; and
  9. Prescribed by or in consultation with a neurologist with expertise in the diagnosis of ALS

Initial authorization duration: 6 months


REAUTHORIZATION CRITERIA Sodium phenylbutyrate and taurursodiol (Relyvrio™) is re-approved when BOTH of the following are met:
  1. Documentation of slowed disease progression from baseline; and
  2. Prescribed by or in consultation with a neurologist with expertise in the diagnosis of ALS

Reauthorization duration: 6 months​


​N/A

Maragakis NJ. Epidemiology and pathogenesis of amyotrophic lateral sclerosis. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed April 17, 2023.

Goyal NA. Disease-modifying treatment of amyotrophic lateral sclerosis. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com. Accessed April 17, 2023.

Relyvrio™ (sodium phenylbutyrate and taurursodiol) [package insert]. Cambridge, MA: Amylyx Pharmaceuticals, Inc; September 2022. Available at https://www.relyvrio.com/RELYVRIO-US-Prescribing-Information.pdf. Accessed April 17, 2023.



23/16/202312/8/20236/29/2023 5:59 AMNo presence informationsrv_ppsgw_NP

Rx.01.33 Off Label Use​

Brand NameGeneric Name
Relyvrio™Sodium phenylbutyrate and Taurursodiol


234
  
7/1/2023Rx.01.269CommercialOyenusi, Oluwadamilola

Cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD) is a rare, genetic, developmental epileptic encephalopathy characterized by early onset, treatment refractory seizures, motor impairments, and severe neurodevelopmental delays. CDD is estimated to affect 1 in 40,000 to 1 in 60,000 live births, with reported cases increasing as genetic testing becomes more common. The clinical severity of CDD is variable, and patients may experience one or more of several different seizure types, including infantile spasms (IS) and tonic-clonic, atonic, clonic, myoclonic, absence, and focal seizures. Patients with CDD generally respond poorly to currently approved drugs for varying seizure types, and there are currently no other approved treatments specifically for CDD.

The mechanism by which Ztalmy exerts its therapeutic effects in the treatment of seizures associated with CDD is unknown, but its anticonvulsant effects are thought to result from positive allosteric modulation in both the synaptic and extra synaptic gamma-aminobutyric acid-A (GABAA) receptors in the central nervous system (CNS), decreasing neuron excitability.

Ztalmy is indicated for the treatment of seizures associated with CDD in patients ≥ 2 years of age. 



​The intent of this policy is to communicate the medical necessity criteria for Ganaxolone (Ztalmy®) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA Ganaxolone (Ztalmy®) is approved when ALL of the following are met: 

  1. Diagnosis of cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD); and 
  2. Documentation of mutation in the CDKL5 gene; and 
  3. Member is experiencing motor seizures (e.g., bilateral tonic, generalized tonic-clonic, bilateral clonic, atonic, focal, or bilateral tonic-clonic); and 
  4. One of the following: 
    1. Inadequate response or inability to tolerate two formulary anticonvulsants (e.g., valproic acid, levetiracetam, lamotrigine); or 
    2. Continuation of therapy with requested medication; and 
  5. Member is 2 years of age or older; and 
  6. Prescribed by or in consultation with a neurologist 

Initial authorization duration: 6 months 

REAUTHORIZATION CRITERIA Ganaxolone (Ztalmy®) is re-approved when BOTH of the following are met: 
  1. Documentation of positive clinical response to therapy (e.g., reduction in frequency of major motor seizures compared to baseline); and 
  2. Prescribed by or in consultation with a neurologist 

Reauthorization duration: 2 years



​N/A

Amin S, Monaghan M, Aledo-Serrano A, et al. International Consensus recommendations for the assessment and management of individuals with CDKL5 deficiency disorder. Front Neurol. 2022;13:874695.

Food and Drug Administration (FDA). FDA approves drug for treatment of seizures associated with rare disease in patients two years of age and older [news release]. March 18, 2022. https://www.fda.gov/drugs/news-events-human-drugs/fda-approves-drug-treatment-seizures-associated-rare-disease-patients-two-years-age-and-older. Accessed April 19, 2023.

Food and Drug Administration. Ztalmy (ganaxolone) summary review. March 18, 2022. https://www.accessdata.fda.gov/drugsatfda_docs/nda/2022/215904Orig1s000SumR.pdf. Accessed April 19, 2023.

Jakimiec M, Paprocka J, Śmigiel R. CDKL5 deficiency disorder-a complex epileptic encephalopathy. Brain Sci. 2020;10(2):107.

Knight EMP, Amin S, Bahi-Buisson N, et al; Marigold Trial Group. Safety and efficacy of ganaxolone in patients with CDKL5 deficiency disorder: results from the double-blind phase of a randomised, placebo-controlled, phase 3 trial. Lancet Neurol. 2022;21(5):417-427.

Leonard H, Downs J, Benke TA, et al. CDKL5 deficiency disorder: clinical features, diagnosis, and management. Lancet Neurol. 2022;21(6):563-576.

Ztalmy (ganaxolone) [package insert]. Radnor, PA: Marinus Pharmaceuticals, Inc.; November 2022. Available from: https://marinuspharma.com/wp-content/uploads/2022/03/prescribing-information.pdf. Accessed April 19, 2023​.


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​Rx.01.33 Off Label Use​

Brand NameGeneric Name
Ztalmy® Ganaxolone


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Vulvovaginal candidiasis (VVC) is one of the most common causes of vulvovaginal itching and discharge. The disorder is characterized by inflammation in the setting of Candida species. Treatment is indicated for the relief of symptoms. Recurrent vulvovaginal candidiasis (RVVC) is defined as three or more episodes of symptomatic infection within one year.

Oteseconazole is an antifungal drug. It is an azole metalloenzyme inhibitor that inhibits the enzyme CYP51 (also known as 14α demethylase). It demethylates the 14-α position of lanosterol to yield ergosterol, a compound that plays a key role in maintaining the integrity of cell membranes in yeast and fungi, thereby inhibiting fungal growth.

Vivjoa™ is indicated to reduce the incidence of recurrent vulvovaginal candidiasis (RVVC) in females with a history of RVVC who are NOT of reproductive potential.



​The intent of this policy is to communicate the medical necessity criteria for oteseconazole (Vivjoa™) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Oteseconazole (Vivjoa™) is approved when ALL of the following are met:

  1. Diagnosis of recurrent vulvovaginal candidiasis (RVVC); and
  2. Member is not of reproductive potential; and
  3. Diagnosis of RVVC is confirmed by ONE of the following:
    1. Positive potassium hydroxide (KOH) preparation; or
    2. Vaginal fungal culture; and
  4. Member has experienced 3 or more symptomatic episodes of vulvovaginal candidiasis (VVC) within the past 12 months; and
  5. Inadequate response or inability to tolerate BOTH of the following:
    1. One intravaginal product (e.g., clotrimazole, miconazole, terconazole); and
    2. Oral fluconazole

Authorization duration: 4 months 


n/a

Sobel J. Candida vulvovaginitis: Treatment. UpToDate. Revised December 2022. Available from: uptodate.com. Accessed February 24, 2023.

VivjoaTM (oteseconazole) [package insert]. Durham, NC: Mycovia Pharmaceuticals, Inc; April 2022. Available at https://vivjoa.com/pi/VIVJOA-Full-Prescribing-Information.pdf. Accessed February 24, 2023.


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Rx.01.33 Off Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit​


Brand NameGeneric Name
Vivjoa™oteseconazole


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The US Food and Drug Administration (FDA) approves labeling that details uses for which a pharmaceutical agent can be marketed.  The approved uses identify the specific disease states that the agent has been shown to be safe, efficacious and meet all clinical requirements set forth by the FDA. An off-label or unlabeled use of a prescription drug or biologic is a use that has not been approved by the US Food and Drug Administration (FDA) and which is not identified in package labeling. Use of a drug for any indication, dose or dose frequency, treatment duration, patient population, or route of administration other than those approved by the FDA and listed on the label or packaging insert is considered an off-label or unlabeled use.Off-label use of prescription drugs and biologics not meeting the medical necessity criteria is considered experimental/investigational and may not be a covered by the prescription drug benefit.
 
In determining whether there is clinical evidence to support a medical necessity determination, the pharmacy benefits manager ​ will consider the quality of the published evidence as well as an assessment of the following information as submitted by the requesting physician. Off-label uses are medically accepted if they are supported in either of the following:

(1) one or more authoritative compendia, and none list it as not indicated, unsupported, not recommended, or equivalent terms; or

(2) in peer-reviewed medical literature.

Reliable evidence must demonstrate that the proposed off-label use for the specified medical condition is safe and effective and that the treatment's beneficial effects outweigh its risks.

Peer-reviewed medical literature includes scientific, medical, and pharmaceutical publications in which original manuscripts are published only after having been critically reviewed for scientific accuracy, validity, and reliability by unbiased, independent experts prior to publication. In order for a use to be supported by clinical research, it must have been studied in at least two clinical trials conducted at different centers, and the results must have been published in national or international peer-reviewed journals with an editorial committee composed of physicians. Peer-reviewed medical literature does not include in-house publications of pharmaceutical manufacturing companies or abstracts (including meeting abstracts).

According to the National Cancer Institute, clinical trials are usually conducted in a series of steps called phases. These are outlined as follows:

Phase 0 trials are the first step in testing a new agent in people. Phase 0 trials will evaluate how the new agent is processed in the body and how it exerts its clinical effects in the body. Phase 0 trials enroll a small number of individuals (10-15 individuals) who are administered a very small amount of the new agent.

Phase I trials evaluate what dose is safe, how a new agent should be given (by mouth, injected into a vein, or injected into the muscle), and how often. Researchers watch closely for any harmful side effects. Phase I trials usually enroll a small number of individuals (20 or more individuals) and take place at only a few locations. The dose of the new therapy or technique is increased a little at a time. The highest dose with an acceptable level of side effects is determined to be appropriate for further testing.

Phase II trials study the safety and effectiveness of an agent or intervention and evaluate how it affects the human body. Phase II studies usually focus on a particular aspect of a disease and include fewer than 100 patients.

Phase III trials compare a new agent or intervention (or new use of a standard one) with the current standard therapy. Participants are randomly assigned to the standard group or the new group, usually by computer. This method, called randomization, helps to avoid bias and ensures that human choices or other factors do not affect the study's results. In most cases, studies move into Phase III testing only after they have shown promise in Phases I and II. Phase III trials often include large numbers of individuals across the country.

Phase IV trials are conducted to further evaluate the long-term safety and effectiveness of a treatment. They usually take place after the treatment has been approved for standard use. Several hundred to several thousand people may take part in a Phase IV study. These studies are less common than Phase I, II, or III trials.

Prescription pharmaceutical agents available in the United States have FDA approved labeling. The label specifies which disease states a drug can be used to treat. However, use of a pharmaceutical agent may expand past the approved labeling and into what is known as off-label use. Coverage for off-label or experimental use will require Prior Authorization.  

Off-label uses are medically accepted and thus approved when ONE of the following is met:

  1. The narrative text in American Hospital Formulary Service--Drug Information (AHFS-DI®) is supportive of the use; OR
  2. The use is classified as Category 1 or 2A by National Comprehensive Cancer Network (NCCN) Drugs and Biologics Compendium™; OR
  3. The use is classified as Class I or Class IIa in Micromedex®; OR
  4. Adequate published clinical research (supplied by the provider) as defined below:

Authorization duration: 2 years

PUBLISHED CLINICAL RESEARCH

In order for an off-label use to be supported by published clinical research, all of the following criteria must be met:

A. The prescription drug or biologic must have been studied in at least two clinical trials conducted at different centers and the results must have been published in a national or international peer-reviewed journals with an editorial committee composed of physicians.  Peer-reviewed medical literature includes scientific, medical, and pharmaceutical publications.  It does not include in-house publications of pharmaceutical manufacturing companies or abstracts (including meeting abstracts).

B. A use is considered supported by clinical research when it appears in at least two Phase III clinical trials that have definitively demonstrated its safety and effectiveness as an appropriate medical treatment for the condition.  If no Phase III trial evidence is available, at least two Phase II clinical trials with reasonably large patient samples showing consistent results of safety and efficacy may be considered in certain instances (e.g. rare diseases in which a Phase III study might be difficult to complete in a reasonable period of time after completion of the Phase II studies. Or when overwhelmingly good evidence of safety and effectiveness is noted in Phase II studies

Reliable evidence must demonstrate that the proposed off label use for the specified medical condition is safe and effective and that the beneficial effects of the treatment outweigh its risks.
In determining whether there is supportive clinical evidence for a particular use of a prescription drug or biologic, the Company considers the quality of the evidence in published, peer-reviewed medical literature.  Among other things, such consideration involves the assessment of the following:

1.     The prevalence and life history of the disease when evaluating the adequacy of the number of subjects and the response rate

2.     The effect on the individual's well-being and other responses to therapy that indicate effectiveness (e.g. reduction in mortality, morbidity, and signs and symptoms)

3.     Whether the clinical characteristics of the beneficiary and the indication are adequately represented in the published evidence

4.     Whether the study is appropriate to address the clinical question, such as:

  1. If the study design is appropriate to address investigative questions (e.g. in some clinical studies, it may be unnecessary or not feasible to use randomized, double-blind trial, placebos, or crossover)
  2. If non-randomized clinical trials with a significant number of subjects may be a basis for supportive clinical evidence for determining accepted uses of drugs
  3. Generally, case reports are considered uncontrolled, are based on anecdotal information, and do not provide adequate supportive clinical evidence for determining accepted uses of drugs.

5.     The off-label use is supported by published clinical research and the results have been published in major, peer-reviewed medical journals such as, but not limited to:

 

American Journal of MedicineGynecologic Oncology
American Journal of PsychiatryInternational Journal of Radiation, Oncology, Biology and Physics
Annals of Internal MedicineJournal of Clinical Oncology
Annals of OncologyJournal of Obstetrics and Gynecology
Annals of Surgical OncologyJournal of Pediatrics
Archives of Pediatric and Adolescent MedicineJournal of the National Cancer Institute
Biology of Blood and Marrow TransplantationJournal of the National Comprehensive Cancer Network (NCCN)
British Journal of CancerJournal of Urology
British Journal of HematologyLancet
British Journal of MedicineLancet Oncology
CancerLeukemia
Clinical Cancer ResearchPediatrics
DrugsRadiation Oncology
European Journal of CancerThe Journal of the American Medical Association

 

EXPERIMENTAL/INVESTIGATIONAL

Prescription drugs that are considered experimental/investigational are not covered because the safety and/or efficacy of the drug for those purposes cannot be established by a review of the available published peer reviewed literature.  Prescription drugs and biologics are considered experimental/investigational for any of the following:

  1. The prescription drug or biologic has not received FDA approval for any indication
  2. The off-label use of the prescription drug or biologic does not meet the medical necessity criteria listed in this policy (i.e. the off-label use is not recognized by the appropriate compendia or published clinical research)
  3. The FDA determined the prescription drug or biologic to be contraindicated for specific condition(s) or specific off-labels use(s)
  4. The off-label use is not medically accepted or not indicated by a compendium for specific conditions (i.e. the indication is Category # in NCCN, Class III in Micromedex®) or when the narrative text in AHFS-DI® or Clinical Pharmacology® is not supportive

 

a. The absence of narrative text for an off-label use is considered neither supportive nor non-supportive

REQUIRED DOCUMENTATION

The individual's medical record must reflect the medical necessity for the care provided.  These medical records may include, but are not limited to records from the professional provider's office, hospital, nursing home, home health agency, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider.  All documentation is to be available to the Company upon request.  Failure to produce the requested information may result in a denial for the service.

N/A
N/A
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N/A
 

This policy applies to all drugs that have clinical management policies addressing them.

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Aztreonam (Cayston®) is a monobactam antibiotic, which is part of the beta-lactam class, that binds to penicillin binding proteins of susceptible bacteria and leads to inhibition of bacterial cell wall synthesis and death of the cell.

Aztreonam (Cayston®) is indicated to improve respiratory symptoms in cystic fibrosis patients with pulmonary Pseudomonas aeruginosa infections. Safety and effectiveness has not been established in pediatric patients below the age of 7 years, patients with FEV1 <25% or >75% predicted, or patients colonized with Burkholderia cepacia.


 

The intent of this policy is to communicate the medical necessity criteria for aztreonam (Cayston®) as provided under the member’s prescription drug benefit.


 

INITIAL CRITERIA: Aztreonam (Cayston®) is approved when ALL of the following are met:

  1. Member is 7 years of age or older; AND
  2. Diagnosis of cystic fibrosis; AND
  3. Evidence of Pseudomonas aeruginosa in the lungs confirmed by culture; AND
  4. Susceptibility results indicating that the Pseudomonas aeruginosa is sensitive to aztreonam; AND
  5. FEV1 that is 25% to 75% of predicted

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Aztreonam (Cayston®) is re-approved when ALL of the following are met:

  1. Diagnosis of cystic fibrosis; AND
  2. Evidence of Pseudomonas aeruginosa in the lungs confirmed by culture; AND
  3. Documentation of positive clinical response to therapy (e.g. improvement in lung function demonstrated by improved FEV1)

Reauthorization duration: 2 years


N/A

Cayston® [package insert]. Foster City CA. Gilead Sciences. November 2019. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=67300ca3-8c53-4ce4-8e86-2c03be1f9b8a&type=display.  Accessed June 22, 2023.  

McCoy K, Quittner A, Oermann C, et al. Inhaled Aztreonam Lysine for chronic airway pseudomonas aeruginosa in cystic fibrosis. Am J Respir Crit Care 2008; 178(9): 921-928. Accessed June 22, 2023. 

Oermann C, Retsch-Bogart G, Quittner A, et al. An 18 month study of the safety and efficacy of repeated courses of inhaled Aztreonam Lysine in Cystic Fibosis. Pediatric Pulmonology2010; 45(11): 1121-1134. Accessed June 22, 2023.

Retsch-Bogart G, Quittner A, Gibson R, et al. Efficacy and Safety of inhaled Aztreonam lysine for airway pseudomonas in cystic fibrosis. Chest 2009; 135(5): 1223-1232.  Accessed June 22, 2023.​



 


 


 

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Rx.01.33 Off-Label Use
 


Brand Name Generic Name
Cayston® Aztreonam

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In the United States, it is estimated that 2.7 to 3.9 million people are chronically infected with HCV. Chronic HCV infection occurs after acute infection with the virus. It is estimated that approximately 75%-85% of acute infections become chronic. The remaining 15%-25% clear the virus without treatment and do not develop chronic HCV. Genotype (GT) 1 is the most prevalent, with the breakdown as follows: GT 1a: 46.2%, GT1b: 26.3%, GT2: 10.7%, GT3: 8.9%, GT4: 6.3%, GT6: 1.1%, mixed GT/ other: 0.5%. 

The goal of treating chronic HCV is to "reduce all-cause mortality and liver-related health adverse consequences, including end-stage liver disease and hepatocellular carcinoma, by the achievement of virologic cure as evidenced by a sustained virologic response (SVR)." SVR is defined as the "continued absence of detectable HCV RNA at least 12 weeks after completion of therapy" and is considered a marker for cure of HCV. Several benefits are associated with HCV cure, including decreases in liver inflammation, progression to liver fibrosis, hepatocellular carcinoma, liver-related mortality and liver transplantation. 

This policy follows the AASLD/IDSA guidelines.  Current treatment regimens consist of at least 2 agents.  Monotherapy is not recommended. 

Elbasvir/ grazeprevir (Zepatier®) is a product containing an HCV NS5A inhibitor (elbasvir) and an HCV NS3/4A protease inhibitor in a fixed dose combination.

Glecaprevir/pibrentasvir (Mavyret™) is a fixed-dose combination of glecaprevir, an HCV NS2/4A inhibitor, and pibrentasvir, an HCV NS5A inhibitor.  

Ledipasvir/ sofosbuvir (Harvoni®) is a combination product that contains an HCV NS5A replication inhibitor (ledipasvir) and an HCV NS5B RNA-dependent polymerase inhibitor (sofosbuvir) in a fixed dose combination. 

Ombitasvir/ paritaprevir/ ritonavir and dasabuvir (Viekira Pak®) is a combination product that contains an HCV NS5A replication inhibitor (ombitasivir), an HCV NS3/4A protease inhibitor (parataprevir) and a booster (ritonavir) as a fixed dose tablet along with an HCV NS5B RNA-dependent RNA polymerase inhibitor (dasabuvir) as a separate tablet.   

Sofosbuvir (Sovaldi®) inhibits HCV NS5B RNA-dependent RNA polymerase, which is essential for viral replication. 

Sofosbuvir/velpatasvir/voxilaprevir (Vosevi®) is a fixed-dose combination of sofosbuvir, an HCV nucleotide analog NS5B polymerase inhibitor; velpatasvir, an HCV NS5A inhibitor; and voxilaprevir, an HCV NS3/4A protease inhibitor. 

Velpatisvir/ sofosbuvir (Epclusa®) is a combination product that contains an HCV NS5A replication inhibitor (velpatisvir) and an HCV NS5B RNA-dependent polymerase inhibitor (sofosbuvir) in a fixed dose combination.



The intent of this policy is to communicate the medical necessity criteria for elbasvir/ grazeprevir (Zepatier®), ledipasvir/ sofosbuvir (Harvoni®), ombitasvir/ paritaprevir/ ritonavir and dasabuvir (Viekira Pak®), sofosbuvir (Sovaldi®), velpatasvir/ sofosbuvir (Epclusa®), glecaprevir/pibrentasvir (Mavyret™), and sofosbuvir/velpatasvir/voxilaprevir (Vosevi®) as provided under the member’s prescription drug benefit.


TREATMENT NAIVE

A. Genotype 1

MavyretTM is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: 56 days (8 weeks)


Velpatasvir/ sofosbuvir (Epclusa®) is approved when ALL of the following are met:

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 1; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: 84 days (12 weeks)*; and
  5. Inadequate response or inability to tolerate brand Epclusa® (applies to valpatasvir/sofosbuvir only), details of intolerability must be provided

 Ledipasvir/ sofosbuvir (Harvoni®) is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: 84 days (12 weeks)*; and
  5. Inadequate response or inability to tolerate brand Harvoni® (applies to lepipasvir/sofosbuvir only), details of intolerability must be provided

 Zepatier® is approved when ALL of the following are met:

  1. Age 12 years or older or weighing at least 30kg; and
  2. Diagnosis of HCV genotype 1; and
  3. Results of testing for baseline high fold-change NS5A resistance-associated variant are provided (for genotype 1a); and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  5. Documentation of a dispensed prescription for and inability to tolerate glecaprevir/ pibrentasvir (MavyretTM), brand Harvoni® or brand Epclusa®, details of intolerability must be provided; and
  6. Used concurrently with ribavirin for genotype 1a when baseline high fold-change NS5A resistance-associated variant are detected; and
  7. Approved length of therapy:
    1. Genotype 1a without baseline high fold-change NS5A resistance-associated variant or genotype 1b: 84 days (12 weeks)*
    2. Genotype 1a with baseline high fold-change NS5A resistance-associated variant: 112 days (16 weeks)*


Viekira Pak® is approved when ALL of the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 1; and
  3. ONE of the following:
      1. Genotype 1a without cirrhosis; or
      2. Genotype 1b with absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Documentation of a dispensed prescription for and inability to tolerate glecaprevir/ pibrentasvir (MavyretTM), brand Harvoni® or brand Epclusa®, details of intolerability must be provided; and​
  5. Used concurrently with ribavirin for genotype 1a; and
  6. Approved length of therapy: 84 days (12 weeks) *


B. Genotype 2

MavyretTM is approved when all of the following are met:

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 2; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: 56 days (8 weeks)*


Velpatasvir/ sofosbuvir (Epclusa®) is approved when ALL of the following are met: 

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 2; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only), details of intolerability must be provided.; and
  5. Approved length of therapy: 84 days (12 weeks)*


C. Genotype 3


MayvretTM is approved when ALL of the following are met:

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 3; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: : 56 days (8 weeks)*

 Velpatasvir/ sofosbuvir (Epclusa®) is approved when ALL of the following are met:

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 3; and
  3. Absence of decompensated cirrhosis (i.e. compensated cirrhosis or no cirrhosis); and
  4. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only), details of intolerability must be provided.; and
  5. Concurrent use of ribavirin if compensated cirrhosis and Y93H substitution present; and
  6. Approved length of therapy: 84 days (12 weeks)

Vosevi® is approved when ALL of the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 3; and
  3. Compensated cirrhosis; and
  4. Presence of Y93H; and​
  5. Approved length of therapy: 84 days (12 weeks)*


D. Genotype 4 

MavyretTM is approved when ALL of the following are met:

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 4; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: 56 days (8 weeks)
     

Velpatasvir/ sofosbuvir (Epclusa®) is approved when ALL of the following are met:

  1. Age 3 years or older; and 
  2. Diagnosis of chronic HCV genotype 4; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only), details of intolerability must be provided; and
  5. Approved length of therapy: 84 days (12 weeks)*


Ledipasvir/ sofosbuvir (Harvoni®) is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 4; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details of intolerability must be provided; and
  5. Approved length of therapy: 84 days (12 weeks)*

 
Zepatier® is approved when ALL of the following are met:

  1. Age 12 years or older or weighing at least 30kg; and
  2. Diagnosis of chronic HCV genotype 4; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Documentation of a dispensed prescription for and inability to tolerate glecaprevir/ pibrentasvir (MavyretTM), brand Harvoni® or  brand Epclusa®, details of intolerability must be provided; and​
  5. Approved length of therapy: 84 days (12 weeks)*


E. Genotype 5 or 6


MavyretTM is approved when ALL of the following are met:

  1. Age 3 years or older ; and
  2. Diagnosis of chronic HCV genotype 5 or 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Approved length of therapy: 56 days (8 weeks)


Velpatasvir/ sofosbuvir (Epclusa®) is approved when ALL of the following are met:

  1. Age 3 years or older ; and
  2. Diagnosis of chronic HCV genotype 5 or 6; and​
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only), details of intolerability must be provided; and
  5. Approved length of therapy: 84 days (12 weeks)*


Ledipasvir/ sofosbuvir (Harvoni®) is approved when ALL the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 5 or 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  4. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details of intolerability must be provided; and
  5. Approved length of therapy: 84 days (12 weeks)*


RETREATMENT (note: Retreatment for interferon or interferon plus first generation protease inhibitor failures should be treated as treatment naïve)

Sofosbuvir-Based and Elbasvir/Grazoprevir Treatment Failures

A. Genotype 1, 2, 4, 5 and 6

Mavyret™ is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 4, 5, 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Sofosbuvir-Based therapy or Zepatier (Elbasvir/Grazoprevir); and
  5. Approved length of therapy: 112 days (16 weeks)

Vosevi® is approved when ALL the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 4, 5, 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Sofosbuvir-Based therapy or Zepatier (Elbasvir/Grazoprevir); and
  5. Approved length of therapy: 84 days (12 weeks)*

B. Genotype 3

Vosevi® is approved when ALL the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 3 and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Sofosbuvir-Based therapy or Zepatier (Elbasvir/Grazoprevir); and
  5. Approved length of therapy: 84 days (12 weeks)*


Glecaprevir/Pibrentasvir Treatment Failures

A. Genotype 1, 2, 3, 4, 5, 6

Mavyret™ and Sovaldi® is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 3, 4, 5, 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Mavyret (Glecaprevir/Pibrentasvir); and
  5. Concurrent use of ribavirin; and
  6. Approved length of therapy: 112 days (16 weeks)

Vosevi® is approved when ALL the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 3, 4, 5, 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Mavyret (Glecaprevir/Pibrentasvir); and

Approved length of therapy: 84 days (12 weeks)


Multiple DAA Treatment Failures (All Genotypes), Including Vosevi® (Sofosbuvir/Velpatasvir/Voxilaprevir) or Sofosbuvir Plus Mavyret™ (Glecaprevir/Pibrentasvir)

A. Genotypes 1, 2, 4, 5, 6

Mavyret™ and Sovaldi is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 4, 5, 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Mavyret (Glecaprevir/Pibrentasvir) and Sovaldi (sofosbuvir) or Vosevi® (Sofosbuvir/Velpatasvir/Voxilaprevir); and
  5. Concurrent use of ribavirin; and
  6. Approved length of therapy: 112 days (16 weeks)

Vosevi® is approved when ALL the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 4, 5, 6; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Mavyret (Glecaprevir/Pibrentasvir) and Sovaldi (sofosbuvir) or Vosevi® (Sofosbuvir/Velpatasvir/Voxilaprevir; and
  5. Concurrent use of ribavirin (for members with compensated cirrhosis); and
  6. Approved length of therapy: 168 days (24 weeks)​

B. Genotype 3

Mavyret™ and Sovaldi is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 3; and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Mavyret (Glecaprevir/Pibrentasvir) and Sovaldi (sofosbuvir) or Vosevi® (Sofosbuvir/Velpatasvir/Voxilaprevir); and
  5. Concurrent use of ribavirin; and
  6. Approved length of therapy:
    1. NO cirrhosis - 112 days (16 weeks)
    2. Compensated cirrhosis up to 168 days (24 weeks)

Vosevi® is approved when ALL the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of chronic HCV genotype 3 and
  3. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  4. Member failed Mavyret (Glecaprevir/Pibrentasvir) and Sovaldi (sofosbuvir) or Vosevi® (Sofosbuvir/Velpatasvir/Voxilaprevir); and
  5. Concurrent use of ribavirin (for members with compensated cirrhosis); and
  6. Approved length of therapy: 168 days (24 weeks)



DECOMPENSATED CIRRHOSIS (moderate to severe hepatic impairment; Child Turcotte Pugh B or C)

A. Treatment Naive

Ledipasvir/ sofosbuvir (Harvoni®) is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 4, 5, or 6; and
  3. Documentation of decompensated cirrhosis (defined as Child Turcotte Pugh class B or C) or hepatocellular carcinoma; and
  4. One of the following:
    1. Concurrent use of ribavirin; or
    2. Member is unable to tolerate ribavirin; and
  5. Prescribed by a hepatitis C expert (i.e., hepatologist, liver transplant surgeon) ; and
  6. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details or intolerability must be provided; and
  7. Approved length of therapy:
    1. Without ribavirin: 168 days (24 weeks)*; or
    2. With ribavirin: 84 days (12 weeks)*


Velpatasvir/sofosbuvir (Epclusa®) is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 3, 4, 5, or 6; and
  3. Documentation of decompensated cirrhosis (defined as Child Turcotte Pugh class B or C) or hepatocellular carcinoma; and
  4. One of the following:
    1. Concurrent use of ribavirin; or
    2. Member is unable to tolerate ribavirin; and
  5. Prescribed by a hepatitis C expert (i.e., hepatologist, liver transplant surgeon) ; and
  6. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only), details of intolerability must be provided; and
  7. Approved length of therapy:
    1. With ribavirin: 168 days (24 weeks)*; or
    2. Without ribavirin: 168 days (24 weeks)*


B. Retreatment with Prior Sofosbuvir or NS5A inhibitor-base treatment

Ledipasvir/ sofosbuvir (Harvoni®) is approved when ALL of the following are met:

  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 4, 5, or 6; and
  3. Documentation of decompensated cirrhosis (defined as Child Turcotte Pugh class B or C) or hepatocellular carcinoma; and
  4. Prescribed by a hepatitis C expert (i.e., hepatologist, liver transplant surgeon) ; and
  5. Concurrent use of ribavirin; and
  6. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details or intolerability must be provided; and
  7. Approved length of therapy: 168 days (24 weeks)*
Velpatasvir/sofosbuvir (Epclusa®) is approved when ALL of the following are met:
  1. Age 3 years or older; and
  2. Diagnosis of chronic HCV genotype 1, 2, 3, 4, 5, or 6; and
  3. Documentation of decompensated cirrhosis (defined as Child Turcotte Pugh class B or C) or hepatocellular carcinoma; and
  4. Prescribed by a hepatitis C expert (i.e., hepatologist, liver transplant surgeon) ; and
  5. Concurrent use of ribavirin; and
  6. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only) details of intolerability must be provided; and
  7. Approved length of therapy: 168 days (24 weeks)*



RECURRENT HCV POST LIVER TRANSPLANT

Genotype 1

MavyretTM is approved when there is documentation of ALL of the following:

  1. Age 3 years or older; and 
  2. Diagnosis of HCV genotype 1; and
  3. Documentation of liver transplant; and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  5. Approved length of therapy: 84 days (12 weeks)*


Ledipasvir/ sofosbuvir (Harvoni®) is approved when there is documentation of ALL of the following:

  1. Age 3 years or older; and
  2. Diagnosis of HCV genotype 1; and
  3. Documentation of liver transplant; and
  4. Used with ribavirin; and
  5. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details of intolerability must be provided; and
  6. Approved length of therapy: 84 days (12 weeks)*​

Viekira Pak® is approved when ALL of the following are met:

  1. Age 18 years or older; and
  2. Diagnosis of HCV genotype 1; and
  3. Documentation of liver transplant; and
  4. Documentation of normal hepatic function; and
  5. Documentation of mild fibrosis; and
  6. Used with ribavirin; and
  7. Documentation of a dispensed prescription for and inability to tolerate glecaprevir/ pibrentasvir (Mavyret™) or brand Harvoni®, details of intolerability must be provided; and
  8. Approval length: 24 weeks


B. Genotype 2 or 3
MavyretTM is approved when ALL of the following are met:

  1. Age 3 years or older ; and
  2. Diagnosis of HCV genotype 2 or 3; and
  3. Documentation of liver transplant; and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  5. Approved length of therapy: 84 days (12 weeks)*


Velpatasvir/ sofosbuvir (Epclusa®) is approved when ALL the following are met:

  1. Age 3 years or older ; and
  2. Diagnosis of HCV genotype 2 or 3; and
  3. Documentation of liver transplant; and
  4. Used with ribavirin; and
  5. Presence of cirrhosis; and
  6. Inadequate response or inability to tolerate brand Epclusa® (applies to velpatasvir/sofosbuvir only), details of intolerability must be provided; and
  7. Approved length of therapy: 84 days (12 weeks)*

C. Genotype 4, 5 or 6

Mavyret™ is approved when there is documentation of ALL of the following:

  1. Age 3 years or older; and
  2. Diagnosis of HCV genotype 4, 5, or 6; and
  3. Documentation of liver transplant; and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis) ; and
  5. Approved length of therapy: 84 days (12 weeks)*

Ledipasvir/ sofosbuvir (Harvoni®) is approved when there is documentation of ALL of the following:
  1. Age 3 years or older; and
  2. Diagnosis of HCV genotype 4, 5, or 6; and
  3. Documentation of liver transplant; and
  4. Used with ribavirin; and
  5. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details of intolerability must be provided; and
  6. Approved length of therapy: 84 days (12 weeks)*



KIDNEY TRANSPLANT RECIPIENT

A.    Genotype 1 or 4

MavyretTM  is approved when there is documentation of ALL of the following:

  1. Age 3 years or older ; and
  2. Diagnosis of HCV genotype 1 or 4; and
  3. Documentation of kidney transplant; and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  5. Approved length of therapy: 84 days (12 weeks)*  

Ledipasvir/ sofosbuvir (Harvoni®) is approved when there is documentation of ALL of the following:

  1. Age 3 years or older; and
  2. Diagnosis of HCV genotype 1 or 4; and
  3. Documentation of kidney transplant; and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  5. Inadequate response or inability to tolerate brand Harvoni® (applies to ledipasvir/sofosbuvir only), details of intolerability must be provided; and
  6. Approved length of therapy: 84 days (12 weeks)*


B. Genotype 2,3,5, or 6


MavyretTM is approved when there is documentation of ALL of the following:

  1. Age 3 years or older; and
  2. Diagnosis of HCV genotype 2,3,5, or 6; and
  3. Documentation of kidney transplant; and
  4. Absence of decompensated cirrhosis (i.e., compensated cirrhosis or no cirrhosis); and
  5. Approved length of therapy: 84 days (12 weeks)*



*Approved length of therapy: if therapy was initiated prior to coverage with the plan, authorization will only be granted to allow completion of the specified therapy.  Authorizations will end 180 days after receipt of request.

Maximum doses per day apply

 

DrugMaximum dose per day (tablets)
velpatasvir/ sofosbuvir (Epclusa®)1
velpatasvir/ sofosbuvir (Epclusa®) pack 200-50mg2
velpatasvir/ sofosbuvir (Epclusa®) pack 150-37.5mg1
MavyretTM3
Mavyret™ pak 50-20mg5
Ledipasvir/sofosbuvir (Harvoni®)1

Sovaldi® 1
Viekira Pak®6
Zepatier®1
Vosevi®1


HCV Treatment Regimens that are not recommended and considered not medically necessary

  1. Sofosbuvir with ribavirin x 24 weeks (except in genotype 2 post liver transplant)
  2. Peg-interferon and ribavirin with or without sofosbuvir, simeprevir, telaprevir, or boceprevir)
  3. Monotherapy with peg-interferon, ribavirin, or a direct-acting antiviral
  4. Simeprevir, paritaprevir, or elbasvir/grazopevir based regimens in those decompensated cirrhosis

Epclusa® (velpatasvir/sofosbuvir), Sovaldi® (sofosbuvir), Viekira Pak® (ombitasvir/paritaprevir/ritonavir/dasabuvir), Harvoni® (ledipasvir/sofosbuvir),  Zepatier® (elbasvir/grazoprevir), Mavyret™ (glecaprevir and pivrentasvir) & Vosevi® (sofosbuvir/velpatasvir/voxilaprevir):
RISK OF HEPATITIS B REACTIVATION IN PATIENTS COINFECTED WITH HCV AND HBV:
Hepatitis B virus (HBV) reactivation has been reported, in some cases resulting in fulminant hepatitis, hepatic failure, and death. 

 

 

American Association for the Study of Liver Disease, Infectious Diseases Society or America, International Antiviral Society-USA. Recommendations for testing, managing and treating Hepatitis C. Available from https://www.hcvguidelines.org/.  Accessed August 03, 2023

Epclusa® (velpatasvir/sofosbuvir) [package insert]. Forest City, CA: Gilead Sciences, Inc; May, 2022. Available from: https://www.gilead.com/~/media/Files/pdfs/medicines/liver-disease/epclusa/epclusa_pi.pdf. Accessed August 03, 2023

Harvoni® (ledipasvir/sofosbuvir) [package insert]. Forest City, CA: Gilead Sciences, Inc; March 2020. Available from: https://www.gilead.com/~/media/Files/pdfs/medicines/liver-disease/harvoni/harvoni_pi.pdf. Accessed August 03, 2023

Hepatitis C Information for Health Professionals. Centers for Disease Control and Prevention. July 2019. Available from: http://www.cdc.gov/hepatitis/HCV/HCVfaq.htm. Accessed August 03, 2023

Mavyret™ (glecaprevir/pibrentasvir) [package insert]. North Chicago, IL: AbbVie, Inc.; June 2021. Available from https://www.rxabbvie.com/pdf/mavyret_pi.pdf. Accessed August 03, 2023

Sovaldi® (sofosbuvir) [package insert]. Forest City, CA: Gilead Sciences, Inc; April 2021. Available from: https://www.gilead.com/~/media/files/pdfs/medicines/liver-disease/sovaldi/sovaldi_pi.pdf?evo_source=SOVALDI. Accessed August 03, 2023

Viekira Pak® (ombitasvir/ paritaprevir/ ritonavir and dasabuvir) [package insert]. North Chicago, IL: AbbVie, Inc; Nov 2020. Available from: https://www.rxabbvie.com/pdf/viekirapak_pi.pdf. Accessed August 03, 2023

Vosevi™ (sofosbuvir/velpatasvir/voxilaprevir) [package insert]. Foster City, CA: Gilead Sciences, Inc.; November 2019. Available from: http://www.gilead.com/~/media/Files/pdfs/medicines/liver-disease/vosevi/vosevi_pi.pdf. Accessed August 03, 2023

Zepatier® (elbasvir/ grazeprovir) [package insert]. Whitehouse Station, NJ: Merck & Co, Inc; May 2022. Available from: https://www.merck.com/product/usa/pi_circulars/z/zepatier/zepatier_pi.pdf. Accessed August 03, 2023






286/8/20236/8/202410/1/2023 1:24 AMNo presence informationsrv_ppsgw_P
Off-label Drug Use Rx.01.33​
 


Brand NameGeneric Name
Epclusa®velpatasvir/ sofosbuvir
Harvoni®ledipasvir/ sofosbuvir
Viekira Pak®
ombitasvir/ paritaprevir/ ritonavir and dasabuvir
Sovaldi®
sofosbuvir
Zepatier®
elbasvir/grazoprevir
​MavyretTM

​glecaprevir/pibrentasvir 
​Vosevi®
​sofosbuvir/velpatasvir/voxilaprevir 
   
248
  
10/1/2023Rx.01.131CommercialOyenusi, Oluwadamilola

Lomitapide a synthetic lipid-lowering agent, directly binds and inhibits microsomal triglyceride transfer protein, which resides in the lumen of the endoplasmic reticulum, thereby preventing the assembly of apo B-containing lipoproteins in enterocytes and hepatocytes. This inhibits the synthesis of chylomicrons and very low-density lipoprotein (VLDL). The inhibition of the synthesis of VLDL leads to reduced levels of plasma LDL-C.

Lomitapide (Juxtapid®) is indicated as an adjunct to a low-fat diet and other lipid-lowering treatments, including LDL apheresis where available, to reduce low-density lipoprotein cholesterol (LDL-C), total cholesterol (TC), apolipoprotein B (apo B), and non-high-density lipoprotein cholesterol (non-HDL-C) in patients with homozygous familial hypercholesterolemia (HoFH).

Proprotein convertase subtilisin/ kexin type 9 (PCSK9) is a serine protease synthesized primarily by the liver and intestines.  PCSK9 promotes the degradation of low density lipoprotein (LDL) receptors, thus preventing them from being recycled back to the plasma membrane where they can bind more LDL. Inhibitors of PCSK9 increase recycling of LDL receptors which in turn increases the capacity to remove LDL cholesterol (LDL-C) from the blood. These agents are monoclonal antibodies administered subcutaneously.

Alirocumab (Praluent®) and evolocumab (Repatha®) are indicated:

  • in adults with established cardiovascular disease (CVD) to reduce the risk of myocardial infarction, stroke, and coronary revascularization ​
  • as an adjunct to diet, alone or in combination with other low-density lipoprotein cholesterol (LDL-C)-lowering therapies, in adults with primary hyperlipidemia, including heterozygous familial hypercholesterolemia (HeFH), to reduce LDL-C 
  • as an adjunct to other LDL-C-lowering therapies in patients with homozygous familial hypercholesterolemia (HoFH), to reduce LDL-C 

According to current guidelines, HMG-CoA reductase inhibitors (statins) are the mainstay of pharmacologic therapy for treating elevated LDL-C for both primary and secondary prevention of atherosclerotic cardiovascular disease.  Lifestyle modifications are a critical component of treating elevated LDL-C and should be used in conjunction with pharmacologic therapy.  

Clinical trials of PCSK9 inhibitors demonstrated reductions in LDL-C approximately 50-60%.  Reauthorization criteria will include a reduction from baseline of 25% or greater, which will assess adherence with the medication.

Bempedoic acid (Nexletol™) is an adenosine triphosphate-citrate lyase (ACL) inhibitor that lowers low-density lipoprotein cholesterol (LDL-C) by inhibition of cholesterol synthesis in the liver.  ACL is an enzyme upstream of 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase in the cholesterol biosynthesis pathway.  Bempedoic ​acid and its active metabolite, ESP15228, require coenzyme A (CoA) activation by very long-chain acyl-CoA synthetase 1 (ACSVL1) to ETC-1002-CoA and ESP15228-CoA, respectively.  ACSVL1 is expressed primarily in the liver.  Inhibition of ACL by ETC-1002-CoA results in decreased cholesterol synthesis in the liver and lowers LDL-C in blood via upregulation of low-density lipoprotein receptors.

Ezetimibe reduces blood cholesterol by inhibiting the absorption of cholesterol by the small intestine.  The molecular target of ezetimibe has been shown to be the sterol transporter, Niemann-Pick C1-Like 1 (NPC1L1), which is involved in the intestinal uptake of cholesterol and phytosterols.  Ezetimibe localizes at the brush border of the small intestine and inhibits the absorption of cholesterol, leading to a decrease in the delivery of intestinal cholesterol to the liver.  This causes a reduction of hepatic cholesterol stores and an increase in LDL receptors, resulting in clearance of cholesterol from the blood.

Bempedoic acid (Nexletol™) and bempedoic acid/ezetimibe (Nexlizet™) is indicated as an adjunct to diet and maximally tolerated statin therapy for the treatment of adults with heterozygous familial hypercholesterolemia or established atherosclerotic cardiovascular disease who require additional lowering of LDL-C.



 


The intent of this policy is to communicate the medical necessity criteria for lomitapide (Juxtapid®), alirocumab (Praluent®), evolocumab (Repatha®), bempedoic acid (Nexletol™), and bempedoic acid/ezetimibe (Nexlizet™) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Lomitapide (Juxtapid®) is approved when  ALL of the following are met:

  1. Diagnosis of Homozygous Familial Hypercholesterolemia; and
  2. Used as an adjunct to lipid lowering treatments and a low-fat diet with ONE of the following:
    1. Genetic confirmation of 2 mutant alleles at the LDL receptor, Apo B, PCSK9, or LDL receptor adaptor protein 1 (i.e. LDLRAP1 or ARH); or
    2. Untreated LDL-C > 500mg/dL or treated LDL cholesterol ≥ 300mg/dL with either of the following:
      1. Cutaneous or tendinous xanthoma prior to 10 years of age, or
      2. Elevated LDL cholesterol prior to lipid-lowering therapy consistent with HeFH in both parents; AND
  3. ONE of the following:
    1. Inadequate response to one of the following medications in combination with ezetimibe:
      1. Simvastatin (daily doses ≥ 40mg); or
      2. Atorvastatin (daily doses ≥ 20mg); or
      3. Rosuvastatin (daily doses ≥ 10mg); or
    2. Member has experience ONE of the following:
      1. Rhabdomyolysis or muscle symptoms with creatine kinase (CK) elevations > 10 times upper limit of normal (ULN) on any statin; or
      2. Myalgia (muscle symptoms without CK elevations) or myositis (muscle symptoms with CK elevations < 10 times ULN) with TWO statins; AND
  4. Inadequate response or inability to tolerate evolocumab (Repatha®) or alirocumab (Praluent®); and
  5. Prescribed by or in consultation with one of the following:
    1. Cardiologist; or
    2. Endocrinologist; or
    3. Lipid specialist
​Initial authorization duration: 6 months

Reauthorization criteria: Lomitapide (Juxtapid®) is re-approved when there is a reduction in LDL level of at least 25% since initiation of therapy.

Reauthorization duration: 12 months

 

INITIAL CRITERIA: Alirocumab (Praluent®) is approved when ALL of the following are met:

  1. Diagnosis of ONE of the following:
    1. Hyperlipidemia; or
    2. Homozygous familial hypercholesterolemia and one of the following:
      1. Diagnosis confirmed by genetic test; or
      2. Untreated LDL-C >500mg/dL with either of the following:
        1. Cutaneous or tendinous xanthoma prior to 10 years of age; or 
        2. Elevated LDL cholesterol prior to lipid-lowering therapy consistent with HeFH in both parents; or
    3. Atherosclerotic cardiovascular disease as diagnosed by either stress test, angiography, atherosclerotic event (e.g., MI, angina, stroke, claudication, carotid stenosis) or arterial intervention for atherosclerotic diseases (e.g., coronary, peripheral, carotid); and
  2. ONE of the following:
    1. LDL-C 70 mg/dL or greater after a minimum 8-week trial of at least moderate-intensity statin therapy; or
    2. Inability to tolerate statin therapy as documented by ONE of the following:
      1. Member has rhabdomyolysis or symptoms with creatine kinase (CK) exceeding 10 times the upper limit of normal (ULN) on any statin; or
      2. ONE of the following with TWO statins:
        1. Myalgia (no CK elevation); or
        2. Myositis (CK less than 10 times ULN); or
        3. Hepatotoxicity from statin use (increase AST/ALT exceeding 3 times ULN); OR
      3. Liver disease documented by Child Pugh A or worse or AST/ALT exceeding 3 times ULN for at least 6 weeks; and
  3. Inadequate response or inability to tolerate evolocumab (Repatha®)
Initial authorization duration: 6 months.


REAUTHORIZATION CRITERIA Alirocumab (Praluent®) is re-approved when there is a sustained reduction in LDL-C of at least 25% since initiation of therapy.

Reauthorization duration: 12 months

 

INITIAL CRITERIA: Evolocumab (Repatha®) is approved when ALL of the following are met:

  1. Diagnosis of ONE of the following:
    1. Hyperlipidemia; or
    2. Homozygous familial hypercholesterolemia and one of the following:
      1. Diagnosis confirmed by genetic test; or
      2. Untreated LDL-C >500mg/dL with either of the following:
        1. Cutaneous or tendinous xanthoma prior to 10 years of age; or
        2. Elevated LDL cholesterol prior to lipid-lowering therapy consistent with HeFH in both parents; or
    3. Atherosclerotic cardiovascular disease as diagnosed by either stress test, angiography, atherosclerotic event (e.g., MI, angina, stroke, claudication, carotid stenosis) or arterial intervention for atherosclerotic disease (e.g., coronary, peripheral, carotid); AND
  2. ONE of the following:
    1. LDL-C 70 mg/dL or greater after a minimum 8-week trial of at least moderate-intensity statin therapy; or
    2. Inability to tolerate statin therapy as documented by ONE of the following:
      1. Member had rhabdomyolysis or symptoms with creatine kinase (CK) exceeding 10 times the upper limit of normal (ULN) on any statin; or
      2. ONE of the following with TWO statins:
        1. Myalgia (no CK elevation); or
        2. Myositis (CK less than 10 times ULN; or
        3. Hepatotoxicity from statin use (increased AST/ALT exceeding 3 times ULN); OR
      3. Liver disease documented by Child Pugh A or worse OR AST/ALT exceeding 3 times ULN for at least 6 weeks​


Initial Authorization duration: 6 months

Reauthorization criteria:  Evolocumab (Repatha®) is re-approved when there is a sustained reduction in LDL-C of at least 25% since initiation of therapy

Reauthorization duration: 12 months

 

INITIAL CRITERIA: Bempedoic acid (Nexletol™), bempedoic acid/ezetimibe (Nexlizet™) is approved when ALL of the following are met:

  1. One of the following diagnoses:
    1. Heterozygous familial hypercholesterolemia (HeFH); or
    2. Atherosclerotic cardiovascular disease (ASCVD) as diagnosed by either stress test, angiography, atherosclerotic event (e.g., MI, angina, stroke, claudication, carotid stenosis) or arterial intervention for atherosclerotic diseases (e.g., coronary, peripheral, carotid); and
  2. One of the following:
    1. LDL-C 70 mg/dL or greater after at least 8 consecutive weeks of statin therapy and member will continue to receive statin therapy at maximally tolerated dose; or
    2. Inability to tolerate statin therapy as documented by ONE of the following:
      1. Member has rhabdomyolysis or symptoms with creatine kinase (CK) exceeding 10 times the upper limit of normal (ULN) on any statin; or
      2. ONE of the following with TWO statins:
        1. Myalgia (no CK elevation); or
        2. Myositis (CK less than 10 times ULN); or
        3. Hepatotoxicity from statin use (increase AST/ALT exceeding 3 times ULN); or
      3. Liver disease documented by Child Pugh A or worse or AST/ALT exceeding 3 times ULN for at least 6 weeks; and
  3. ONE of the following:
    1. Member has been receiving at least 8 consecutive weeks of ezetimibe (Zetia®) therapy as adjunct to maximally tolerated statin therapy; or
    2. Member has contraindication or intolerance to ezetimibe (Zetia®)

      Initial Authorization duration: 6 months

REAUTHORIZATION CRITERIA: Bempedoic acid (Nexletol™), Bempedoic acid/ezetimibe (Nexlizet™) is approved when ALL of the following are met:

  1. Documentation of sustained reduction of LDL-C by at least 17% from the time therapy began or sustained below 70mg/dL; and
  2. ONE of the following:
    1. Member continues to receive other lipid-lowering therapy (e.g., statins, ezetimibe) at the maximally tolerated dose; or
    2. Member has inability to tolerate other lipid-lowering therapy (e.g., statins, ezetimibe)

Reauthorization duration: 12 months

 

Risk of hepatotoxicity:

Lomitapide (Juxtapid®) can cause elevations in transaminases. In clinical trials, of patients treated with lomitapide had at least 1 elevation in ALT or AST at least 3 times the upper limit of normal (ULN) or higher. There were no concomitant clinically meaningful elevations of total bilirubin, international normalized ratio (INR), alkaline phosphatase or partial thromboplastin time (PTT).

Lomitapide also increase hepatic fat (hepatic steatosis), with or without concomitant increase in transaminases. In the trials of patients with heterozygous familial hypercholesterolemia and hyperlipidemia, the median absolute increase in hepatic fat was 6% (lomitapide) after 26 weeks of treatment from 0% at baseline, measured by magnetic resonance imaging (MRI) and 1% at baseline, measured by magnetic resonance spectroscopy (MRS) respectively. Hepatic steatosis is a risk factor for advanced liver disease, including steatohepatitis and cirrhosis.

 

Measure ALT, AST, alkaline phosphatase, and total bilirubin before initiating treatment and then ALT and AST regularly as recommended. During treatment, adjust the dose of lomitapide if the ALT or AST are at least 3 times the ULN. Discontinue lomitapide for clinically significant liver toxicity.

 

Because of the risk of hepatotoxicity, lomitapide are available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS).​

Farnier M, Bruckert E. Severe familial hypercholesterolemia: Current and future management. Arch Cardiovasc Dis. 2012 Dec; 105(12):656-65. Accessed June 23, 2023.


Goldberg AC, Hopkins PN, Toth PP, et al. Familial hypercholesterolemia: screening, diagnosis, and management of pediatric and adult patients: clinical guidance from the National Lipid Association Expert Panel on Familial Hypercholesterolemia. J Clin Lipidology. 2011;5:S1-S8. Accessed June 23, 2023.


Grundy SM, Cleeman JI, Merz NB, et al. Implications of recent clinical trials for the National Cholesterol Education Program Adult Treatment Panel III Guidelines. Circulation. 2004; 110:227-39. Accessed June 23, 2023.


Jacobson TA, Ito MK, Maki KC, et al. National Lipid Association recommendations for the patient-centered management of dyslipidemia: Part 1- full report. J Clin Lipidology. 2015;9:129-69. Accessed June 23, 2023.


Juxtapid® (lomitapide) [prescribing information.] Cambridge, MA. Aegerion Pharmaceuticals. December 2019. Available at: http://juxtapidpro.com/prescribing-information. Accessed June 23, 2023.


Raal FJ, Santos RD. Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment. Atherosclerosis. 2012 Aug; 223(2):262-8. Accessed June 23, 2023.


Stone NJ, Robinson J, Lichtenstein AH, et al. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce cardiovascular risk in adults: a report of the American College of Cardiology/ American Heart Association Task Force on practice guidelines. Circulation. 2013. Available from: http://circ.ahajournals.org/cgi/content/short/129/25_suppl_2/S1?rss=1&ssource=mfr. Accessed June 23, 2023.


Visser ME, Witztum JL, Stroes ES, et al. Antisense oligonucleotides for the treatment of dyslipidaemia. Eur Heart J. 2012 Jun; 33(12):1451-8. doi: 10.1093/eurheartj/ehs084. Epub 2012 May 24.


Lambert G, Sjouke B, Choque B, Kastelein JJP, Hovingh GK. The PCSK9 decade. J Lipid Res. 2012; 53(12): 2515-24. DOI: 10.1194/jlr.R026658. Accessed June 23, 2023.


Farnier M. PCSK9: From discovery to therapeutic applications. Arch Cardiovascular Dis. 2014; 107: 58-66. DOI: 10.1016/j.acvd.2013.10.007. Accessed June 23, 2023.


Goldberg AC, Hopkins PN, Toth PP, et al. Familial hypercholesterolemia: screening, diagnosis, and management of pediatric and adult patients: clinical guidance from the National Lipid Association Expert Panel on Familial Hypercholesterolemia. J Clin Lipidology. 2011;5:S1-S8. Accessed June 23, 2023


Jacobson TA, Ito MK, Maki KC, et al. National Lipid Association recommendations for the patient-centered management of dyslipidemia: Part 1- full report. J Clin Lipidology. 2015;9:129-69. Accessed June 23, 2023.


Lloyd-Jones DM, Morris PB, Ballantyne CM, Birtcher KK, Daly Jr DD, DePalma SM, Minissian, MB, Orringer CE, Smith Jr SC, 2017 Focused Update of the 2016 ACC Expert Consensus Decision Pathway on the Role of Non-Statin Therapies for LDL-Cholesterol Lowering in the Management of Atherosclerotic Cardiovascular Disease Risk: A Report of the American College of Cardiology Task Force on Exper Decision Consensus Pathways. Journal of the American College of Cardiology (2017), doi: 10.1016/j.jacc.2017.07.745. Accessed June 23, 2023.


Nexletol™ (bempedoic acid) [prescribing information]. Ann Arbor, MI: Esperion Therapeutics, Inc.; February 2020. Available from: https://pi.esperion.com/nexletol/nexletol-pi.pdf. Accessed June 23, 2023.


Nexlizet™ (bempedoic acid and ezetimibe) [prescribing information]. Ann Arbor, MI: Esperion Therapeutics, Inc.; February 2020. Available from: https://pi.esperion.com/nexlizet/nexlizet-pi.pdf. Accessed June 23, 2023.


Praluent® (alirocumab) [package insert]. Bridgewater, NJ. Sanofi-Aventis US LLC. April 2021.  Available from: http://products.sanofi.us/praluent/praluent.pdf. Accessed June 23, 2023.


Repatha® (evolocumab) [package insert]. Thousand Oaks, CA. Amgen Inc. February 2021. Available from: https://www.pi.amgen.com/~/media/amgen/repositorysites/pi-amgen-com/repatha/repatha_pi_hcp_english.pdf. Accessed June 23, 2023.


Stone NJ, Robinson J, Lichtenstein AH, et al. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce cardiovascular risk in adults: a report of the American College of Cardiology/ American Heart Association Task Force on practice guidelines. Circulation. 2013. https://doi.org/10.1161/01.cir.0000437738.63853.7a. Accessed June 23, 2023.


Varghese MJ. Familial hypercholesterolemia: a review. Ann Pediatr Cardiol. 2014;7:107-17. Accessed June 23, 2023.


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Off-Label Use Rx.01.33


Brand nameGeneric name
Juxtapid®Lomitapide
Praluent®alirocumab
Repatha®evolocumab
Nexletol™Bempedoic acid
Nexlizet™Bempedoic acid/ezetimibe

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10/1/2023Rx.01.134CommercialOyenusi, Oluwadamilola

The Food and Drug Administration (FDA) defines pharmacy compounding as the practice in which pharmacists combine, mix, or alter ingredients to create unique medications that meet the specific needs of an individual patient. Generally, drugs are compounded for patients that have allergic reactions to inactive ingredients in FDA approved products or for those patients who require a different formulation of a medication that is not commercially available.  

Compounding pharmacies are regulated by State Boards of Pharmacy and the FDA (if they are outsourcing facilities). For non-outsourcing facilities, drugs can be compounded only if certain conditions are met, such as, valid prescription requirement for an identified individual patient; or in limited quantities before obtaining the actual prescription by the pharmacy. Moreover, FDA restricts the production of essential copies of approved and unapproved non-prescription drugs.

A compounded product is not considered medically necessary when it replicates a commercially available product (unless the commercially available product is temporarily unavailable), contains a drug product or component that has been removed from the market because it is unsafe or not effective or contains a drug product or component that is excluded from the member's benefit. 


 

The intent of this policy is to communicate the medical necessity criteria for compounded products, consistent with Pharmacy Compounding of Human Drug Products Under Sections 503A and 503B of the Federal Food, Drug, and Cosmetic Act, where at least one ingredient is a prescription drug, as provided under the member's prescription drug benefit.

This policy will also be used to review requests for ingredients which are not considered standard coverage under the prescription drug benefit that are used in compounded products.  This includes requests for injectable medications that are used as part of a compound for a route of administration other than injectable. 


 

A compounded product, including a commercially available compounding kit, is considered medically necessary when ALL of the following are met:

  1. The active prescription ingredient(s) of the compound is FDA approved or supported by accepted compendium as stated in the Off-Label Use policy for the indication and route of administration; AND

  2. The product as compounded is not commercially available. This may include a current short supply* of the commercially available product or the member has a medical need for a dosage form, strength or formulation other than what can be accomplished with a commercially available product; AND

  3. Member had an inadequate response or inability to tolerate all commercially available therapeutic alternatives to treat the condition for which the compound has been requested; AND

  4. The compound does not contain any product(s) that were withdrawn or removed from the market due to safety reasons; AND

  5. The compound is not used for, nor does it contain, a product that is indicated for an excluded benefit (e.g., cosmetic)

Additionally, authorization may be placed to allow access to the prescription benefit for products that are not considered standard coverage (e.g. drugs administered intravenously) when all the following are met:

  1. All of the above criteria are for medically necessary are met for the compounded product; AND

  2. The product is being used in a compound that will be administered through a route that is considered standard coverage for the prescription benefit (e.g., oral, topical, inhalation, etc.). Bladder installation may be considered if the above criteria are met.

Authorization length for short supply of the commercially available product will be six months.All other authorizations: 2 years

http://www.accessdata.fda.gov/scripts/drugshortages/default.cfm

* http://www.ashp.org/Drug-Shortages/Current-Shortages


 


See labeling for specific ingredients used in a compound.


American Pharmacists Association. Frequently Asked Questions About Pharmaceutical Compounding. Available from: http://www.pharmacist.com/frequently-asked-questions-about-pharmaceutical-compounding. Accessed June 22, 2023.

ASHP Guidelines on Outsourcing Sterile Compounding Services. January 2014. Available from:  http://www.ajhp.org/content/71/2/145?sso-checked=true.  June 22, 2023.

International Academy of Compounding Pharmacist. Available from: http://www.iacprx.org/. Accessed June 22, 2023.

Pharmacy Compounding of Human Drug Products Under Section 503A of the Federal Food, Drug and Cosmetic Act. December 2016. Available from:  https://www.fda.gov/regulatory-information/search-fda-guidance-documents/pharmacy-compounding-human-drug-products-under-section-503a-federal-food-drug-and-cosmetic-act . Accessed  June 22, 2023.

Pharmacy Compounding of Human Drug Products Under Section 503B of the Federal Food, Drug and Cosmetic Act. January 2018. Available from http://www.fda.gov/downloads/Drugs/GuidanceComplianceRegulatoryInformation/Guidances/UCM510153.pdf. Accessed  June 22, 2023.

Report: Limited FDA Survey of Compounded Drug Products. June 2018. Available from: http://www.fda.gov/Drugs/GuidanceComplianceRegulatoryInformation/PharmacyCompounding/ucm155725.htm, Accessed  June 22, 2023.

USP Compounding Standards and Resources.  Available from: http://www.usp.org/usp-healthcare-professionals/compounding?gclid=CJfWt97qmsECFedzMgodCzgA_w. Accessed June 22, 2023.


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Off Label Use Policy (Rx.01.33)

Note: All routes of administration listed may not be covered under the pharmacy benefit, e.g. intravenous, intramuscular.

 

P​roduct

Description

Compound claims greater than $75

compound where the submitted claim cost is greater than or equal to $75

Various

Compounding kit

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10/1/2023Rx.01.149CommercialOyenusi, Oluwadamilola
Hemophilia is a bleeding disorder caused by clotting factor deficiencies.  Hemophilia is a rare X-linked, congenitally acquired disease that leads to a deficiency in coagulation factor, VIII (hemophilia A) or IX (hemophilia B).  Hemophilia A or B occurs in approximately 1 out of  10,000 births.  Hemophilia A is more common than hemophilia B, representing approximately 80-85% of hemophilia cases. The disorder is further characterized by the percentage of serum clotting factor activity as compared to the normal range:


  • Severe hemophilia: clotting factor levels are less than 1% of normal
  • Moderate hemophilia: clotting factor levels are 1-5% of normal
  • Mild hemophilia: clotting factors are 5-40% of normal

Treatment of hemophilia generally involves an infusion of the deficient factors to allow for more normalized clotting response and decreased incidence of uncontrolled bleeding events.

Treatment regimens are individualized based on disease severity and may include prophylactic infusions of clotting factors, on demand infusions of clotting factors, or a combination of both.  Perioperative infusions are also part of the treatment regimen.

While Hemophilia A and hemophilia B are the most common types of hemophilia, 1 in every 500,000 to 2 million people have coagulation disorders caused by deficiencies in clotting factors other than factor VIII or IX. The products listed below include those with indications to treat these rare forms of coagulation disorders.

 
DrugFactorHemophilia AHemophilia BAcquired hemophiliaVon Willebrand diseaseCongenital fibrinogen deficiencyHereditary factor X deficiencyCongenital factor XIII deficiencyControl of bleeding episodes, on demandPerioperative managementRoutine prophylaxis
Advate®Recombinant factor VIIIXXXX
Adynovate®Recombinant factor VIII, PEGylatedXXXX
Afstyla®Recombinant Factor VIIIXXXX
Alphanate®Factor VIII/ von Willebrand factor complex, humanXX (in certain circumstances)XXX
Alphanine SD®Factor IX, humanXXXX
Alprolix®Recombinant factor IX, Fc fusion proteinXXXX
Altuviiio™

Recombinant Fc-VWF-XTEN fusion

protein-ehtl

X      XXX
Benefix®Factor IX, recombinantXXXX
Coagadex®Factor X, humanXXX
Corifact®Factor XIII concentrate, humanXXX
Eloctate®Recombinant factor VIII, Fc fusion proteinXXXX
Esperoct®Recombinant, glycopegylated-exeiX      XXX
Feiba [NF] ®Anti-inhibitor coagulant complexX (with inhibitors)X (with inhibitors)XXX
Hemlibra®Factor IXa/ factor X, directed antibodyX        X
Hemofil M®Factor VIII, humanX XXX
Humate P®Factor VIII/ von Willebrand factor complex, humanXX (in certain circumstances)XXX
Idelvion®Factor IX, recombinant albumin fusion proteinXXXX
Ixinity®Factor IX, recombinantXXXX
Jivi®Recombinant, PEGylated, Factor VIIIX      XXX
Koate DVI®Factor VIII, humanXXXX
Kogenate FS®Recombinant factor VIIIXXXX
Kovaltry®Recombinant factor VIIIXXXX
Mononine®Factor IX, humanXXXX
Novoeight®Recombinant factor VIIIXXXX
Novoseven RT®Recombinant factor VIIaXXXXX
Nuwiq®Recombinant factor VIIIXXXX
Obizur®Recombinant factor VIII, porcine sequenceXX
Profilnine®Factor IX, humanXXX
Rebinyn®Recombinant Factor IX X     XX 
Recombinate®Recombinant factor VIIIXXXX
Riastap®Fibrinogen concentrate, humanXX
Rixubis®Factor IX, recombinantXXXX
Sevenfact®Factor VIIa, recombinantXX        
Tretten®Factor XIII A subunit, recombinantX (A subunit)X
Vonvendi®Von Willebrand Factor RecombinantXX
Wilate® von Willebrand/ factor VIII complex, humanXX
Xyntha® [Solufuse]Recombinant factor VIIIXXXX

The intent of this policy is to communicate the medical necessity criteria for Advate®, Adynovate®, Afstyla®, Alphanate®, Alphanine SD®, Alprolix®, Altuviiio™, Benefix®, Coagadex®, Corifact®, Eloctate®, Esperoct®, Feiba NF®, Hemlibra®, Hemofil M®, Humate P®, Idelvion®, Ixinity®, Jivi®, Koate DVI®, Kogenate FS®, Kovaltry®, Mononine®, Novoeight®, Novoseven RT®, Nuwiq®, Obizur®, Profilnine®, Rebinyn®, Recombinate®, Riastap®, Rixubis®, Sevenfact®, Tretten®, Vonvendi®, Wilate®, and Xyntha®/Xyntha® SolofuseTM as provided under the member's prescription drug benefit.

Advate®, Adynovate®, Afstyla®, Alphanate®, Alphanine SD®, Alprolix®, Altuviiio™, Bebulin®, Benefix®, Coagadex®, Corifact®, Eloctate®, Esperoct®, Feiba NF®, Helixate FS®, Hemlibra®, Hemofil M®, Humate P®, Idelvion®, Ixinity®, Jivi®, Koate DVI®, Kogenate FS®, Kovaltry®, Monoclate P®, Mononine®, Novoeight®, Novoseven RT®,Nuwiq®, Obizur®, Profilnine®, Rebinyn®, Recombinate®, Riastap®, Rixubis®, Sevenfact®, Tretten®, Vonvendi®, Wilate®, Xyntha®/Xyntha® Solofuse are approved when there is a diagnosis of an FDA approved indication​

Approval duration: 2 years 




Feiba® NF
Thrombotic/Thromboembolic events: Thrombotic and thromboembolic events have been reported during postmarketing surveillance following infusion of anti-inhibitor coagulant complex, particularly following the administration of high doses and/or in patients with thrombotic risk factors. Monitor patients receiving anti-inhibitor coagulant complex for signs and symptoms of thromboembolic events.
 

Hemlibra®
Cases of thrombotic microangiopathy and thrombotic events were reported when on average a cumulative amount of >100 U/kg/24 hours of activated prothrombin complex concentrate was administered for 24 hours or more to patients receiving HEMLIBRA prophylaxis. Monitor for the development of thrombotic microangiopathy and thrombotic events if aPCC is administered. Discontinue aPCC and suspend dosing of HEMLIBRA if symptoms occur.
 
Novoseven®, Sevenfact®
Serious arterial and venous thrombotic events following administration of Novoseven and Sevenfact RT have been reported.  Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive Novoseven and Sevenfact RT.  Monitor patients for signs and symptoms of activation of the coagulation system and for thrombosis.

Advate® (antihemophilic factor, human recombinant) [package insert]. Westlake Village, CA. Baxter Healthcare Corporation; March 2023.  Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=80fa03d2-cd4c-4155-9b57-1396c4fa42da&type=display#section-9. Accessed June 23, 2023.

Adynovate® (antihemophilic factor, recombinant, PEGylated) [package insert]. Westlake Village, CA. Baxalta US Inc. March 2023.  Available from: https://www.shirecontent.com/PI/PDFs/ADYNOVATE_USA_ENG.pdf. Accessed June 23, 2023.

Afstyla® (antihemophilic factor, recombinant, single chain) [package insert]. Kankakee, IL. CSL Berhing, LLC. April 2021. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=51f19873-a63f-4229-9477-5da4ecf31cde&type=display. Accessed June 23, 2023.

Alphanate® (antihemophilic factor/ von willebrand factor complex, human) [package insert]. Los Angeles, CA. Grifols Biologicals Inc. March 2021.  Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=5a7aea94-654c-4113-9014-c3137de0a931&type=display. Accessed June 23, 2023.

Alphanine SD® (coagulation factor IX, human) [package insert]. Los Angeles, CA. Grifols Biologicals Inc. March 2021.  Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=3e99052d-4442-4283-8915-c9a796c77008&type=display. Accessed June 23, 2023.

Alprolix® (coagulation factor IX, recombinant, Fc fusion protein) [package insert]. Cambridge, MA. Biogen Idec Inc. May 2023. Available from: http://products.sanofi.us/Alprolix/alprolix.html. Accessed June 23, 2023.

Altuviiio™ (antihemophilic factor (recombinant), Fc-VWF-XTEN fusion protein-ehtl) [package insert]. Waltham, MA. Bioverativ Therapeutics Inc. March 2023. Available from: https://products.sanofi.us/altuviiio/altuviiio.pdf. Accessed June 23, 2023.

BeneFIX® (coagulation factor IX, recombinant) [package insert]. Philadelphia, PA. Wyeth Pharmaceuticals Inc. June 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=85faa5bc-cee5-4ef1-8d80-bdbcb7eba1e4&type=display. Accessed June 23, 2023.

Bolton-Maggs PHB. Rare coagulation disorders. Available from: http://www1.wfh.org/publication/files/pdf-1188.pdf. Accessed June 23, 2023.

Coagadex® (coagulation factor X, human) [package insert]. Durham, NC. Bio Products Laboratory USA, Inc. September 2018. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ad20e6ff-98bc-47e6-aa00-7fb441edd2b0&type=display. Accessed June 23, 2023.

Corifact® (coagulation factor XIII, human) [package insert]. Kankakee, IL. CSL Behring LLC. December 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=96f1b658-01b0-43a7-9aba-693271389a78&type=display. Accessed June 23, 2023.

Eloctate® (antihemophilic factor, recombinant, Fc fusion protein) [package insert]. Cambridge, MA. December 2020. Biogen, Inc. Available from: http://products.sanofi.us/Eloctate/Eloctate.pdf. Accessed June 23, 2023.

Esperoct® (antihemophilic factor, recombinant, glycopegylated-exei) [prescribing information]. Bagsvaerd, Denmark: Novo Nordisk Inc.; October 2019. Available from: https://www.novo-pi.com/esperoct.pdf. Accessed June 23, 2023.

Feiba® (antiinhibitor coagulant complex) [package insert].  Westlake Village, CA. Baxter Healthcare Corporation. February 2020. Available from: http://www.baxalta.com/assets/documents/feiba_us_pi.pdf. Accessed June 23, 2023.

Feiba NF® (antiinhibitor coagulant complex) [package insert]. Westlake Village, CA. Baxter Healthcare Corporation. February 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=752604e5-4ea2-44f4-83ed-1569373f6412&type=display. Accessed June 23, 2023.

Hemlibra® (antihemophilic factor, humanized) [package insert]. South San Francisco, CA. Genentech, Inc. June 2020. Available from:  https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=2483adba-fab6-4d1b-96c5-c195577ed071. Accessed June 23, 2023.

Hemofil M® (antihemophilic factor, human) [package insert]. Westlake Village, CA. Baxter Healthcare Corporation. June 2018. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=b8953ff7-3bba-4a0b-a486-f26fb81f05d9&type=display. Accessed June 23, 2023.

Humate-P® (antihemophilic factor/ von Willebrand factor complex, human) Kankakee, IL. CSL Behring LLC. June 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=c5f4b63b-8d8b-45b3-b530-2f1c32a1b852&type=display. Accessed June 23, 2023.

Idelvion® (coagulation factor IX, recombinant, albumin fusion protein) [package insert]. Kankakee, IL. CSL Behring. July 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=f74301bf-95e2-44ab-a8b6-98aa07b04683&type=display. Accessed June 23, 2023..

Ixinity® (coagulation factor IX, recombinant) [package insert]. Baltimore, MD. Cangene Corporation. September 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=1cae46d1-f463-488a-9e8b-3431ef1de1e4&type=display. Accessed June 23, 2023.

Jivi® [antihemophilic factor (recombinant), PEGylated-aucl] [prescribing information]. Whippany, NJ. Bayer Healthcare LLC. August 2018. Available from: https://labeling.bayerhealthcare.com/html/products/pi/Jivi_PI.pdf. Accessed June 23, 2023.

Koate-DVI® (antihemophilic factor, human) [package insert]. Reasearch Triangle Park. NC. Grifols Therapeutics, Inc. June 2018. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=2f77d685-e382-b2e7-2414-0d3a3448688a&type=display. Accessed June 23, 2023.

Kogenate FS® (antihemophilic factor, recombinant) [package insert] Whippany, NJ. Bayer HealthCare LLC. December 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=01c6292d-b670-415b-9d8e-7df92c0adc0f&type=display, Accessed June 23, 2023.

Kovaltry® (antihemophilic factor recombinant) [package insert]. Whippany, NJ. Bayer HealthCare LLC. January 2021. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=89ba5048-469c-4f08-a173-81b0c6f3004d&type=display. Accessed June 23, 2023.

Mononine® (coagulation factor IX, human) [package insert].   Kankakee, IL. CSL Behring LLC. February 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=16f35cdb-7a3b-481b-a25b-0a065aea2c9b&type=display. Accessed June 23, 2023.

Novoeight® (antihemophilic factor, recombinant) [package insert]. Plainsboro, NJ. Novo Nordisk, Inc, July 2020. Available from: https://www.novo-pi.com/novoeight.pdf. Accessed June 23, 2023.

Novoseven RT® (coagulation factor VIIa, recombinant) [package insert]. Plainsboro, NJ. Novo Nordisk, Inc, July 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ea65c32f-8518-4383-b621-17056c0eebc0&type=display. Accessed June 23, 2023.

Nuwiq® (antihemophilic factor, recombinant) [package insert]. Hoboken, NJ. Octapharma USA, Inc. September 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=d6cb51f8-3acd-412c-93ec-41a8017dff3e&type=display. Accessed June 23, 2023.

Obizur® (antihemophilic factor, recombinant, porcine sequence) [package insert]. Westlake Village, CA. Baxter Healthcare Corporation. July 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=a69ccbb3-7648-4978-8c6d-7ca6b95a9b01&type=display. Accessed June 23, 2023.

Profilnine SD® (factor IX complex) [package insert]. Los Angeles, CA. Grisfols Biologicals Inc. June 2018. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=79821042-86a5-4a64-b621-76de0a0bbab2&type=display. Accessed June 23, 2023.

Rebinyn® (factor IX, recombinant) [package insert[. Plainsboro, NJ. Novo Nordisk Inc. June 2020.  https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=0ea37235-35fd-410d-b8c4-40ba15fe1294  Available from: Accessed June 23, 2023.

Recombinate® (antihemophilic factor, recombinant) [package insert]. Westlake Village, CA. Baxter Healthcare Corporation. June 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=2a569ec5-08d7-44ab-b649-384a496e173c&type=display. Accessed June 23, 2023.

Riastap® (fibrinogen injection) [package insert]. Kankakee, IL. CSL Behring LLC. July 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=903dc8d0-39da-462c-9dac-004e0c7a26cc&type=display. Accessed June 23, 2023.

Rixubis® (coagulation factor IX, recombinant) [package insert]. Westlake Village, CA. Baxter Healthcare Corporation. June 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=f5070a92-96b8-476a-a2dc-18b22d95e5e0&type=display. Accessed June 23, 2023.

Sevenfact® (coagulation factor VIIa (recombinant)-jncw]) [package insert]. Louisville, KY. HEMA Biologics. April 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=b23ad666-8ce0-4e0b-9eff-c6983ac5f7e6. Accessed June 23, 2023.

Srivastava A, et al. Guidelines for the management of hemophilia. Available form: https://www1.wfh.org/publication/files/pdf-1472.pdf. Accessed June 23, 2023.

Tretten® (coagulation factor XIII a-subunit, recombinant) [package insert]. Plainsboro, NJ. Novo Nordisk, Inc, June 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=8664978e-1814-4930-aca5-97a24455f6df&type=display. Accessed June 23, 2023.

Vonvendi® (von Willebrand factor, recombinant) [package insert]. Westlake Village, CA. Baxalta US, Inc. February 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=53b6d198-6175-4a26-8606-dc0d2a0f12d6&type=display. Accessed June 23, 2023.

Wilate® (Factor VII/VWF) [package insert]. Hoboken, NJ. Octapharma USA, Inc. September 2019. Available from: https://www.wilateusa.com/wp-content/uploads/2019/10/20190923_pil_18x_USA_26.pdf. Accessed June 23, 2023.

Xyntha® (antihemophilic factor, recombinant) [package insert]. Philadelphia, PA. Wyeth Pharmaceuticals Inc. August 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=52e97c7f-8240-4cd6-afd2-c452ab2f6e32&type=display. Accessed June 23, 2023.​




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Off-Label Use Rx.01.33
 

Drug Names

Advate®

Adynovate®

Afstyla®

Alphanate®

Alphanine SD®

Alprolix®

Altuviiio™

BeneFIX®

Coagadex®

Corifact®

Eloctate®

Esperoct®

Feiba NF®

Hemlibra®

Hemofil M®

Humate-P®

Idelvion®

Ixinity®

Jivi®

Koate-DVI®

Kogenate FS®

Kovaltry®

Mononine®

Novoeight®

Novoseven RT®

Nuwiq®

Obizur®

Profilnine SD®

Rebinyn®

Recombinate®

Riastap®

Rixubis®

Sevenfact®

Tretten®

Vonvendi®

Wilate®

Xyntha®


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10/1/2023Rx.01.166CommercialOyenusi, Oluwadamilola

Droxidopa (Northera®) is indicated for the treatment of orthostatic dizziness, lightheadedness, or the “feeling that you are about to black out” in adult patients with symptomatic neurogenic orthostatic hypotension caused by primary autonomic failure (Parkinson’s disease, multiple system atrophy, and pure autonomic failure), dopamine beta-hydroxylase deficiency, and non-diabetic autonomic neuropathy.  Effectiveness beyond two weeks of treatment has not been demonstrated. The continued effectiveness of droxidopa should be assessed periodically.

The exact mechanism of action of droxidopa in the treatment of neurogenic orthostatic hypotension is unknown.  Droxidopa is a synthetic amino acid analog that is directly metabolized to norepinephrine by dopa-decarboxylase, which is extensively distributed throughout the body. Droxidopa is believed to exert its pharmacological effects through norepinephrine and not through the parent molecule or other metabolites.  Norepinephrine increases blood pressure by inducing peripheral arterial and venous vasoconstriction. Droxidopa in humans induces small and transient rises in plasma norepinephrine. Droxidopa is also known as L-dihydroxyphenylserine, or L-DOPS.​


 

The intent of this policy is to communicate the medical necessity criteria for droxidopa (Northera®) as provided under the member’s prescription drug benefit.

INITIAL CRITERIA: Droxidopa (Northera®) is approved when ALL of the following are met:

  1. Diagnosis of symptomatic neurogenic orthostatic hypotension; and
  2. Prescribed by, or in consultation with, ONE of the following:
    1. Cardiologist; or
    2. Neurologist; or
    3. Nephrologist; and
  3. Member is 18 years of age or older; and
  4. Attempt has been made to manage neurogenic orthostatic hypotension through at least one non-pharmacologic intervention (e.g. use of compression stockings/abdominal binder, increasing salt/fluid intake, participation in regular exercise, discontinue or reduce hypotensive or antihypertensive medications); and 
  5. Inadequate response or inability to tolerate midodrine or fludrocortisone; and
  6. For the Brand Northera only, inadequate response or inability to tolerate generic droxidopa

Initial authorization duration: 1 month
 

REAUTHORIZATION CRITERIA: Droxidopa (Northera®) is re-approved when ALL of the following are met:

  1. Diagnosis of symptomatic neurogenic orthostatic hypotension; and
  2. For the Brand Northera only, inadequate response or inability to tolerate generic droxidopa
  3. Prescribed by, or in consultation with, ONE of the following:
    1. Cardiologist; or
    2. Neurologist; or
    3. Nephrologist; and
  4. Documentation of positive clinical response to therapy (e.g. improvement in symptoms such as orthostatic dizziness, lightheadedness, etc.)

Reauthorization duration: 12 months


Supine hypertension: Monitor supine blood pressure prior to and during treatment and more frequently when increasing doses. Elevating the head of the bed lessens the risk of supine hypertension, and blood pressure should be measured in this position. If supine hypertension cannot be managed by elevation of the head of the bed, reduce or discontinue Northera.​

Northera (droxidopa) [package insert]. Deerfield IL. Lundbeck Pharmaceutical LLC. July 2019. Available at:https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=2179f02c-48d7-48eb-8007-5ae43d8d16bc&type=display . Accessed June 22, 2023.

Droxidopa. Micromedex 2.0. Truven Health Analytics, Inc. Greenwood Village, CO. Available at: http://www.micromedexsolutions.com. Accessed June 22, 2023. 

Freeman R.  Current pharmacologic treatment for orthostatic hypotension. Clinical Autonomic Research 2008; 18(1):14-18. Accessed June 22, 2023.

Freeman R. Neurogenic orthostatic hypotension. New England Journal of Medicine 2008; 358:615-624. Accessed June 22, 2023.



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Off-Label Use Rx.01.33


Generic

Brand

Droxidopa

Northera®

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Heart failure affects approximately 5.1 million Americans.  While survival after a diagnosis of heart failure has improved, it is still about 50% at 5 years after diagnosis.  In patients with symptomatic heart failure and left ventricular ejection fraction (LVEF) < 40%, current guidelines recommend angiotensin converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARB), beta blockers, diuretics and aldosterone antagonists as standard of care.  With the exception of diuretics, these classes of medications decrease mortality from heart failure with reduced EF.  Diuretics provide symptomatic relief, however the effects of diuretics on morbidity and mortality are not known.

 
Ivabradine reduces spontaneous pacemaker activity at the cardiac sinus node by blocking the hyperpolarization-activated cyclic nucleotide-gated (HCN) channel to selectively inhibit If-current, thus reducing the heart rate. Ventricular repolarization and myocardial contractility are not affected.  Increased heart rate is an ineffective compensatory mechanism and is recognized as an independent risk factor in heart failure. 
 
Corlanor® (ivabridine) is indicated to reduce the risk of hospitalization for worsening heart failure in adult patients with stable, symptomatic chronic heart failure  with reduced left ventricular ejection fraction and for the treatment of stable symptomatic heart failure due to dilated cardiomyopathy in pediatric patients ages 6 months and older.
 
Ivabradine is also indicated for the treatment of stable symptomatic heart failure due to dilated cardiomyopathy in pediatric patients who are in sinus rhythm with an elevated heart rate.

 
Vericiguat is a stimulator of soluble guanylate cyclase (sGC), an important enzyme in the nitric oxide (NO) signaling pathway. When NO binds to sGC, the enzyme catalyzes the synthesis of intracellular cyclic guanosine monophosphate (cGMP), a second messenger that plays a role in the regulation of vascular tone, cardiac contractility, and cardiac remodeling. Heart failure is associated with impaired synthesis of NO and decreased activity of sGC, which may contribute to myocardial and vascular dysfunction. By directly stimulating sGC, independently of and synergistically with NO, vericiguat augments levels of intracellular cGMP, leading to smooth muscle relaxation and vasodilation.

 

Verquvo® (vericiguat) is indicated to reduce the risk of cardiovascular death and heart failure (HF) hospitalization following a hospitalization for heart failure or need for outpatient IV diuretics, in adults with symptomatic chronic HF and ejection fraction less than 45%.

The intent of this policy is to communicate the medical necessity criteria for Corlanor® (ivabradine) and Verquvo™ (vericiguat) as provided under the member's prescription drug benefit.

Chronic heart failure

INITAL CRITERIA: Corlanor® (ivabradine) is approved when ALL of the following are met:

  1. Diagnosis of stable, symptomatic chronic heart failure; and
  2. Member is 18 years of age or older; and
  3. Left ventricular ejection fraction (LVEF) ≤ 35%; and
  4. Sinus rhythm with resting heart rate ≥ 70 beats per minute; and
  5. Member is clinically stable for at least 4 weeks on an optimized and stable clinical regimen which includes:
    1. Maximally tolerated doses of beta blockers (i.e., bisoprolol, carvedilol, metoprolol succinate ER) or inability to tolerate these beta blockers; and
    2. ACE inhibitors or ARBs or inability to tolerate ACE inhibitor or ARB; and
  6. Prescribed by or in consultation with a cardiologist

INITIAL CRITERIA: Vericiguat (Verquvo™) is approved when ALL of the following are met:

  1. Diagnosis of chronic heart failure; and
  2. Member is age 18 or older; and
  3. Member has an ejection fraction of less than 45 percent; and
  4. Member has New York Heart Association (NYHA) Class II, III or IV symptoms; and
  5. One of the following:
    1. Member was hospitalized for heart failure within the last 6 months; or
    2. Member used outpatient intravenous diuretics (e.g., bumetanide, furosemide) for heart failure within the last 3 months; and
  6. Inadequate response or inability to tolerate both of the following at a maximally tolerated dose:
    1. One of the following:
      1. Angiotensin converting enzyme (ACE) inhibitor (e.g., captopril, enalapril); or
      2. Angiotensin II receptor blocker (ARB) (e.g., candesartan, valsartan); or
      3. Angiotensin receptor-neprilysin inhibitor (ARNI) [e.g., Entresto (sacubitril and valsartan)]; and
    2. Beta blocker (i.e.., bisoprolol, carvedilol, metoprolol succinate ER); and
  7. Prescribed by or in consultation with a cardiologist 

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Vericiguat (Verquvo™) or ivabradine (Corlanor®) is re-approved when there is documentation of positive clinical response to therapy

Reauthorization duration: 2 years


Inappropriate sinus tachycardia 

INITIAL CRITERIA: Ivabradine (Corlanor®) is approved when ALL of the following are met: 
  1. Diagnosis of inappropriate sinus tachycardia; and 
  2. Member is 18 years of age or older; and 
  3. Inadequate response or inability to tolerate one beta-blocker; and 
  4. Prescribed by or in consultation with a cardiologist 

Initial authorization duration: 2 years 

REAUTHORIZATION CRITERIA: Ivabradine (Corlanor®) is re-approved when there is documentation of positive clinical response to therapy. 

Reauthorization duration: 2 years 

Heart failure due to dilated cardiomyopathy 

INITIAL CRITERIA: Ivabradine (Corlanor®) is approved when ALL of the following are met: 
  1. Diagnosis of stable, symptomatic heart failure due to dilated cardiomyopathy; and 
  2. Member is 6 months of age to 18 years of age; and 
  3. Member is in sinus rhythm; and 
  4. Member has elevated heart rate; and 
  5. Prescribed by or in consultation with a cardiologist 

Initial authorization duration: 2 years 

REAUTHORIZATION CRITERIA: Ivabradine (Corlanor®) is re-approved when there is documentation of positive clinical response to therapy. 

Reauthorization duration: 2 years 



Vericiguat (Verquvo™)

  • Do not administer VERQUVO to a pregnant female because it may cause fetal harm. Females of reproductive potential: Exclude pregnancy before the start of treatment. To prevent pregnancy, females of reproductive potential must use effective forms of contraception during treatment and for one month after stopping treatment.

Borer JS, Bohm M, Ford I, et al. Efficacy and safety of ivabradine in patients with severe chronic systolic heart failure (from the SHIFT study). Am J Cardiol. 2014;113:497-503. Accessed June 26, 2023.

Corlanor (ivabradine) [package insert]. Thousand Oaks, CA: Amgen, Inc. August 2021. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=92018a65-38f6-45f7-91d4-a34921b81d0d&type=display. Accessed July 22, 2021.

Go AS, Mozaffarian D, Roger VL, et al. Heart disease and stroke statistics-2013 update: a report from the American Heart Association. Circulation. 2013. Doi: 10.1161/CIR.0b013e31828124ad. Available from: http://circ.ahajournals.org/content/127/1/e6.full.pdf+html. Accessed June 26, 2023.

Ivabradine. Micromedex. Available from: http://www.micromedexsolutions.com/. Accessed June 26, 2023.

Swedberg K, Komajda M, Bohm M, et al. Ivabradine and outcomes in chronic heart failure (SHIFT): a randomised placebo-controlled study. Lancet. 2010;376 (9744):875-85. Accessed June 26, 2023.

Yancy CW, Jessup M, Bozkurk B, et al. 2013 ACCF/AHA Guideline for the Management of Heart Failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2013. Doi: 10.1016/j.jacc.2013.05.020. Available from: http://circ.ahajournals.org/content/128/16/e240.full.pdf+html. Accessed June 26, 2023.

Yancy CW, Jessup M, Bozkurk B, et al.ACC/AHA/HFSA Focused Update on New Pharmacological Therapy for Heart Failure: An Update to the 2013 ACC/AHA Guidelines for the Management of Heart Failure. Circulation. 2016. DOI: 10.1161/CIR.0000000000000435. Available from: http://circ.ahajournals.org/content/circulationaha/early/2016/05/18/CIR.0000000000000435.full.pdf. Accessed June 26, 2023.

Cappato R, Castelvecchio S, Ricci C, et. Al. Clinical Efficacy of Ivabradine in Patients with Inappropriate Sinus Tachycardia. Journal of the American College of Cardiology. Vol. 60, No 15, 2012. October 9, 2012: 1323-9. Accessed June 26, 2023.

Olshansky B, Sullivan R. Inappropriate Sinus Tachycardia. Journal of the American College of Cardiology. Vol. 61, No 8, 2013. February 26, 2013:793-801. Accessed June 26, 2023.

Sheldon RS, Grubb BP, Olshansky B, et. Al. 2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope. Available at: https://www.hrsonline.org/Policy-Payment/Clinical-Guidelines-Documents/2015-HRS-Document-on-POTS-IST-VVS. Accessed June 26, 2023.

Verquvo™ (vericiguat) [prescribing information]. Whitehouse Station (NJ); Merck & Co. Inc.; February 2023. Available at: https://www.merck.com/product/usa/pi_circulars/v/verquvo/verquvo_pi.pdf. Accessed June 26, 2023.
 


 



 

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Rx.01.33 Off-Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit

Brand NameGeneric Name
Corlanor®ivabradine
Verquvo™vericiguat
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10/1/2023Rx.04.2Claim Payment PolicyOyenusi, Oluwadamilola

Presently, the FDA requires prescription drugs to demonstrate safety and efficacy prior to marketing, however, this was not always mandatory.  In 1906, the Pure Food and Drug Act prohibited adulterated, misbranded, poisonous or deleterious drugs from being manufactured, sold or transported.  The Pure Food and Drug Act was repealed and replaced with the Federal Food, Drug, and Cosmetic Act of 1938, which required evidence of safety for new drugs.  The law was amended further in 1962 to strengthen the requirements for safety and add an additional requirement for a manufacturer to demonstrate efficacy of the drug.

The Orange Book identifies FDA approved drugs that have undergone the required safety and efficacy requirements of the Federal Food, Drug, and Cosmetic Act.  If a medication is not included in the orange book, it has not demonstrated safety and efficacy in accordance with the Federal Food, Drug, and Cosmetic Act requirements.  Drugs that entered the market based solely on safety and drugs that were on the market prior to 1938 are not included in the Orange Book. 

Some drugs, mostly older products, are available on the market despite lacking FDA approval for a variety of historical reasons.  Examples include, but are not limited to, a manufacturer combining two approved products into a combination product without obtaining approval and a manufacturer marketing a currently approved product without obtaining FDA approval. 

 

Prescription Drugs are defined by the plan as:

A Legend Drug or Controlled Substance, which:

  • Has been approved by the Food and Drug Administration(FDA) for a specific use; and
  • Can, under federal or state law, be dispensed only pursuant to a Prescription Order

 

A non-FDA approved drug will be considered for formulary inclusion if all the following criteria are met:

  • The drug entered the market prior to 1938; and
  • The drug is not commercially available in an FDA approved form of the same route of administration; and
  • The drug meets the criteria outlined in the Experimental/Investigational Use Policy

The prescription drug benefit covers certain prescription drugs approved by the FDA pursuant to a prescription order.  Details of covered drugs may be found in the member’s benefit booklet.

 

The intent of this policy is to communicate the prescription drug benefit coverage based on medical necessity of drugs that are not approved by the Food and Drug Administration (FDA).

Coverage is subject to the terms, conditions, and limitations of the member's contract.

Prescription drugs that are commercially available but not approved by the FDA are not considered a covered benefit.

Some prescription drugs that are not FDA approved, and not otherwise excluded, will be covered under the pharmacy benefit for certain plans as required by the Patient Protection and Affordable Care Act (PPACA) or as defined in the member's benefit booklet . These items include but may not be limited to the following products as listed:

  • Prenatal vitamins, oral
  • Preventative vitamins and minerals as required by the PPACA
  • Cholecalciferol (vitamin D3) 50,000 units*
  • Sulfuric acid/ phenosulfonic acid solution*

Some prescription drugs that came to market prior to 1938, prior to the current FDA approval process, and not otherwise excluded, will be covered under the prescription drug benefit if they are deemed medically accepted as defined in the Off-Label Use policy.  These items include but may not be limited to the following products as listed: 

  • Aluminum chloride topical 
  • Hydrocortisone suppositories
  • Hyoscyamine 
  • Phenobarbital 
  • Opium tincture 
  • Morphine suppositories 
  • Sodium chloride for inhalation 
  • Homatropine ophthalmic
  • Thyroid products (e.g., Armour Thyroid, Nature-Thyroid, NP Thyroid, WP Thyroid) 
  • Phenazopyridine HCL (Pyridium) 100mg and 200mg tablets 
  • Nitro-time CPCR 
  • Salsalate tablet 

*Exceptions will be made for these products to meet essential health benefit requirements



 

 

Academy of Managed Care Pharmacy. Practice Advisory on Unapproved Medications. Available from: https://www.amcp.org/sites/default/files/2019-03/Practice%20Advisory%20on%20Unapproved%20Meds%20_memohead_.pdf. Accessed June 22, 2023.

US Food and Drug Administration. Federal Food and Drugs Act of 1906. Available from: https://www.fda.gov/about-fda/changes-science-law-and-regulatory-authorities/part-i-1906-food-and-drugs-act-and-its-enforcement. Accessed June 22, 2023.

US Food and Drug Administration. Laws Enforced by FDA. Available from https://www.fda.gov/regulatoryinformation/lawsenforcedbyfda/default.htm . Accessed June 22, 2023.

US Food and Drug Administration. Orange Book: Approved Drug Products with Therapeutic Equivalence Evaluations. Available from: http://www.accessdata.fda.gov/scripts/cder/ob/default.cfm. Accessed June 22, 2023.

US Food and Drug Administration. Orange Book Preface. Available from: http://www.fda.gov/Drugs/DevelopmentApprovalProcess/ucm079068.htm. Accessed June 22, 2023.

US Food and Drug Administration. Unapproved Prescription Drugs: Drugs Marketed in the United States That Do Not Have Required FDA Approval. Available from: https://www.fda.gov/drugs/guidancecomplianceregulatoryinformation/enforcementactivitiesbyfda/selectedenforcementactionsonunapproveddrugs/default.htm . Accessed June 22, 2023.

Preventive Health Services. Available from: https://www.healthcare.gov/coverage/preventive-care-benefits/. Accessed June 22, 2023.

Barowsky H, Schwartz SA. Method for Evaluating Diphenoxylate Hydrochloride: Comparison of Its Antidiarrheal Effect with that of Camphorated Tincture of Opium. JAMA. 1962;180(12):1058-1060.

Beckwith MC. Diarrhea in Palliative Care Patients. Journal of Pharmaceutical Care in Pain & Symptom Control. 2010;7(4):91-108.​


 



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​Cost Share Exception Policy for Preventive Medications and Women's Preventive Services under the PPACA Rx.01.178

Prescription Vitamins, Dietary Supplements, and Medical Foods Rx.04.5

Off-Label Use  Rx.01.33                   

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10/1/2023Rx.04.3Claim Payment PolicyOyenusi, Oluwadamilola

​Convenience packs combine two or more individual drug products into a single package.  Products included in a convenience pack may include prescription products, over the counter products, and/or products not approved by the Food and Drug Administration (FDA).   

​The intent of this policy is to communicate the coverage position of convenience packs.

 

Coverage is subject to the terms, conditions, and limitations of the member's contract.

Convenience packs as described above are not covered under the pharmacy benefit because each product is available independently.

A prescriber may issue a prescription or prescriptions for the individual components of the convenience pack.  The individual components will be covered pursuant to the terms of member's benefit.

Examples of convenience packs include, but are not limited to:

  • DermacinRx Clorhexacin, which contains the following:
    • Mupirocin 2% ointment – covered as a pharmacy benefit
    • Chlorhexidine gluconate 4% solution, dimethicone 5% cream not covered (not an FDA approved product)
  • Diclovix DM PAK 1.5-8% which contains the following:
    • Diclofenac sodium solution 1.5% - covered as a pharmacy benefit
    • Menthol gel 8% therapy pack – not covered (not FDA approved product)​​



​N/A

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​Non-FDA approved Products Rx.04.2

Off-Label Use Rx.01.33

 
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10/1/2023Rx.01.176CommercialOyenusi, Oluwadamilola

Periodic paralyses are a group of rare, inherited neuromuscular disorders, resulting from mutations in the genes that regulate muscle ion channels.  These disorders are characterized by episodes of painless muscle weakness where the affected muscles become weak and unable to contract.

The two most common types of periodic paralyses are hypo- and hyper-kalemic.  Hypokalemic periodic paralysis is characterized by a fall in potassium levels in the blood. In individuals with this mutation attacks often begin in adolescence and are triggered by strenuous exercise, high carbohydrate meals, or by injection of insulin, glucose, or epinephrine. Weakness may be mild and limited to certain muscle groups, or more severe and affect the arms and legs. Attacks may last for a few hours or persist for several days. Some patients may develop chronic muscle weakness later in life. Hyperkalemic periodic paralysis is characterized by a rise in potassium levels in the blood. Attacks often begin in infancy or early childhood and are precipitated by rest after exercise, fasting, cold exposure, or ingestion of small amounts of potassium. Attacks are usually shorter, more frequent, and less severe than the hypokalemic form. Muscle spasms are common. Weakness in an attack can be focal, affecting only one limb, but generalized weakness with hypotonia is more common.

Dichlorphenamide is an oral carbonic anhydrase inhibitor indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants. The precise mechanism by which dichlorphenamide exerts its therapeutic effects in patients with periodic paralysis is unknown. 



The intent of this policy is to communicate the medical necessity criteria for dichlorphenamide (Keveyis®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Dichlorphenamide (Keveyis®) is approved when of ALL of the following are met:

  1. Treatment of primary hyperkalemic periodic paralysis, primary hypokalemic paralysis, and related variants; and

  2. Prescribed by or in consultation with a neurologist; and

  3. No documentation of ANY of the following:

    1. Concomitant use of high dose aspirin; or

    2. Severe pulmonary disease; or

    3. Hepatic insufficiency; and

  4. Member is 18 years of age or older

 Initial authorization duration: 3 months

 

CONTINUAITON CRITERIA: Dichlorphenamide (Keveyis®) is reapproved when there is documentation of positive clinical response to therapy as evidenced by a decrease in weekly attack frequency from baseline​.

Continuation authorization duration: 2 years

​N/A

Dichlorphenamide. Micromedex 2.0. Truven Health Analytics, Inc. Greenwood Village, CO. Available at: http://www.micromedexsolutions.com/. Accessed June 23, 2023.

Gutmann L, Conwit R. Hyperkalemic periodic paralysis. UpToDate. November 2021.  Available at: https://www.uptodate.com/contents/hyperkalemic-periodic-paralysis. Accessed June 23, 2023.

Keveyis® (dichlorphenamide) [package insert]. Hawthorne NY. Taro Pharmaceuticals USA, Inc. November 2019. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2015/011366s030lbl.pdf. Accessed June 23, 2023.

National Institute of Neurological Disorders and Stroke (NINDS). NINDS Familial Periodic Paralyses Information Page. Available at: https://www.ninds.nih.gov/Disorders/All-Disorders/Familial-Periodic-Paralyses-Information-page. Last updated January 2023. Accessed June 23, 2023.

 



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Off-Label Use Rx.01.33


Brand Name

Generic Name

Keveyis®

Dichlorphenamide

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10/1/2023Rx.04.5Claim Payment PolicyOyenusi, Oluwadamilola

 

A dietary supplement is defined by the Food and Drug Administration as "a product intended for ingestion that contains a 'dietary ingredient' intended to add further nutritional value to (supplement) the diet. A 'dietary ingredient' may be one, or any combination, of the following substances:

  • a vitamin
  • a mineral
  • an herb or other botanical
  • an amino acid
  • a dietary substance for use by people to supplement the diet by increasing the total dietary intake
  • a concentrate, metabolite, constituent, or extract"

The US Food and Drug Administration (FDA) defines a medical food as a "food which is formulated to be consumed or administered enterally under the supervision of a physician and which is intended for the specific dietary management of a disease or condition for which distinctive nutritional requirements, based on recognized scientific principles, are established by medical evaluation." FDA section 5(b) of the Orphan Drug Act (21 U.S.C. 360ee (b) (3)). The FDA further defines medical foods as "foods that are specially formulated and processed (as opposed to a naturally occurring foodstuff used in a natural state) for a patient who is seriously ill or who requires use of the product as a major component of a disease or condition's specific dietary management." Medical foods are "intended for the dietary management of a patient who, because of therapeutic or chronic medical needs, has limited or impaired capacity to ingest, digest, absorb, or metabolize ordinary foodstuffs or certain nutrients, or who has other special medically determined nutrient requirements, the dietary management of which cannot be achieved by the modification of the normal diet alone." They also "provide nutritional support specifically modified for the management of the unique nutrient needs that result from the specific disease or condition, as determined by medical evaluation."

Current benefit language states the plan will not cover:

  • Dietary supplements
  • Amino acid supplements
  • Medical foods (exceptions may apply; refer to benefit language for details)
  • Prescription vitamins except for pre-natal and pediatric vitamins

The intent of this policy is to communicate the coverage of prescription vitamins, dietary supplements, and medical foods under the member’s prescription drug benefit.

Coverage is subject to the terms, conditions, and limitations of the member's contract.

Prescription vitamins, dietary supplements, and medical foods are not covered under the pharmacy benefit.

An exception to allow coverage under the member's plan will be in place for prescription medication that are either mandated when used as a preventive medication as described in the Patient Protection and Affordable Care Act (PPACA) or medically necessary for the treatment of a specific illness as determined by the plan.

The following prescription products are covered under the member's prescription benefit:

ProductsClinical Use
Calcitriol oralManagement of secondary hyperparathyroidism and resultant metabolic bone disease in patients with moderate to severe chronic renal failure (creatinine clearance 15 to 55 mL/min) not yet on dialysis.
Cholecalciferol (vitamin D3) 50,000 unitsTreatment of hypoparathyroidism, refractory rickets, also known as vitamin D resistant rickets, and familial hypophosphatemia. This is an over the counter preparation included to meet essential health benefit requirements.
Cyanocobalamin inhaledMaintenance of normal hematologic status in pernicious anemia patients who are in remission following intramuscular (IM) vitamin B12 therapy and who have no nervous system involvement
Dialysis vitamins oral as identified by indication in product labelWasting syndrome in chronic renal failure, uremia, and impaired metabolic function of the kidney
Doxercalciferol oralSecondary hyperparathyroidism (dialysis patients): Treatment of secondary hyperparathyroidism in patients with chronic kidney disease on dialysis.
Secondary hyperparathyroidism (predialysis patients): Treatment of secondary hyperparathyroidism in patients with stage 3 or 4 chronic kidney disease.
Ergocalciferol oralTreatment of hypoparathyroidism, refractory rickets, also known as vitamin D resistant rickets, and familial hypophosphatemia.
Paracalcitol oralHyperparathyroidism: For the prevention and treatment of secondary hyperparathyroidism associated with chronic kidney disease stage 3 and 4, and chronic kidney disease stage 5 in patients on hemodialysis or peritoneal dialysis.
Pediatric vitaminsNutritional supplement for children
Phytonadione oralAnticoagulant-induced prothrombin deficiency caused by coumarin or indandione derivatives.
Coagulation disorders: Phytonadione is indicated in the following coagulation disorders which are due to faulty formation of factors II, VII, IX and X when caused by vitamin K deficiency or interference with vitamin K activity.
Potassium aminobenzoate oral The treatment of scleroderma, dermatomyositis, morphea, linear scleroderma, pemphigus, and Peyronie's disease.
Prenatal vitaminsNutritional supplement used prior to conception, during pregnancy, and in the postnatal period
Prescription electrolytesElectrolyte repleters (i.e., potassium) and electrolyte depleters (ie calcium acetate)

Medical food guidance documents & regulatory information. Available from: http://www.fda.gov/Food/GuidanceRegulation/GuidanceDocumentsRegulatoryInformation/MedicalFoods/. Accessed June 22, 2023.

Preventive Health Services. Available from: https://www.healthcare.gov/coverage/preventive-care-benefits/. Accessed June 22, 2023.

Dietary supplements. Available from: https://www.fda.gov/food/dietarysupplements/. Accessed June 22, 2023.​




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Formulary Exception policy Rx.06.61
 

Medical Foods (ie, Enteral Nutrition and Nutritional Formulas) and Low-Protein Modified Food Products 08.00.18j

Non-FDA Approved Products Rx.04.2 

Off-Label Use Rx. 01.33

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10/1/2023Rx.01.182CommercialOyenusi, Oluwadamilola

Irritable bowel syndrome (IBS), a common, functional gastrointestinal disorder, is defined as abdominal discomfort associated with altered bowel habits.  Three subtypes of IBS exist: IBS with constipation (IBS-C), IBS with diarrhea (IBS-D), and mixed type (IBS-M).  Diagnosis of IBS is based solely on clinical criteria as there are no radiologic or endoscopic abnormalities in IBS and no biomarker has proven reliable for diagnosis.  Approximately 10-15% of the general adult population is affected by IBS.

Hepatic encephalopathy (HE) is defined as brain dysfunction caused by liver insufficiency and/or portosystemic shunting.  It manifests as a wide spectrum of neurological or psychiatric abnormalities ranging from subclinical alterations to coma.

Small Bowel Bacterial Overgrowth (SBBO)/Small Intestinal Bacterial Overgrowth (SIBO) is a condition in which the small bowel is colonized by excessive aerobic and anaerobic microbes. Therapy is typically begun after confirming SBBO/SIBO by breath test. Patients with SBBO/SIBO could present with bloating, flatulence, abdominal discomfort, or chronic watery diarrhea.

Rifaximin (Xifaxan®), a semisynthetic antibiotic, is derived from rifampin.  It inhibits bacterial protein synthesis and growth by binding to the beta-subunit of bacterial DNA-dependent RNA polymerase, blocking one of the steps in transcription.  Rifaximin is indicated for reduction in risk of overt HE recurrence in adults, treatment of IBS-D in adults, and Small Bowel Bacterial Overgrowth (SBBO)/Small Intestinal Bacterial Overgrowth (SIBO).  When treating IBS-D, the course of therapy is 14 days.  The regimen may be repeated up to 2 times.   



The intent of this policy is to communicate the medical necessity criteria for rifaximin (Xifaxan®) as provided under the member’s prescription drug benefit.


Hepatic disease with risk of hepatic encephalopathy

INITIAL CRITERIA: Rifaximin (Xifaxan®) 550 mg is approved when BOTH of the following are met:

  1. Diagnosis of hepatic disease with risk for hepatic encephalopathy (i.e., previous episode of hepatic encephalopathy, advanced liver disease, hepatocellular carcinoma); and
  2. Inadequate response or inability to tolerate lactulose

Initial authorization duration: 2 years (Maximum daily dose 2/day)

REAUTHORIZATION CRITERIA Rifaximin (Xifaxan®) 550mg is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years (Maximum daily dose 2/day)


Irritable Bowel Syndrome with Diarrhea

INITIAL CRITERIA: Rifaximin (Xifaxan®) 550mg is approved when All of the following are met:

  1. Diagnosis of irritable bowel syndrome- diarrhea; and
  2. Inadequate response or inability to tolerate an antispasmodic; and
  3. Member does not exceed 3 courses (42 days) of therapy

Initial authorization duration: 3 months (max of 14 days/84 days; Maximum daily dose 3/day)

REAUTHORIZATION CRITERIA: Rifaximin (Xifaxan®) 550mg is re-approved when ALL of the following are met:
  1. Diagnosis of irritable bowel syndrome – diarrhea; and
  2. BOTH of the following:
    1. Member does not exceed 3 courses (42 days) of therapy; and
    2. No documentation of rifaximin (Xifaxan®) treatment within the last 10 weeks.

Reauthorization duration: 3 months (max of 14 days/84 days; Maximum daily dose 3/day)


Small Bowel Bacterial Overgrowth (SBBO)/Small Intestinal Bacterial Overgrowth (SIBO)

INITIAL CRITERIA: Rifaximin (Xifaxan®) 550mg is approved when ALL of the following are met:

  1. Diagnosis of Small Bowel Overgrowth (SBBO)/Small Intestinal Bacterial Overgrowth (SIBO); and
  2. Inadequate response or inability to tolerate two of the following antibiotics:
    1. Neomycin; or
    2. Amoxicillin/clavulanic acid (Augmentin); or
    3. Ciprofloxacin (Cipro); or
    4. Trimethoprim-sulfamethoxazole (Bactrim); or
    5. Doxycycline (Vibramycin) or minocycline (Minocin) or tetracycline; or
    6. Metronidazole (Flagyl); or
    7. Cephalexin (Keflex)

Initial authorization duration: 3 months (Maximum daily dose 3/day)

​​REAUTHORIZATION CRITERIA: Rifaximin (Xifaxan®) 550mg is re-approved when there is documentation of Small Bowel Bacterial Overgrowth (SBBO)/Small Intestinal Bacterial Overgrowth (SIBO) recurrence following successful initial treatment


Reauthorization duration: 3 months (Maximum daily dose 3/day)




​None

American Association for the Study of Liver Diseases and European Association for the Study of the Liver. Hepatic Encephalopathy in Chronic Liver Disease: 2014 Practice Guideline by the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases. Journal of Hepatology. Available from: http://www.easl.eu/medias/cpg/Hepatic-Encephalopathy-Chronic-Liver-Disease/English-report.pdf. Accessed June 23, 2023.

Ford AC, Moayyedi P, Lacy, BE, et al. American College of Gastroenterology Monograph on the Management of Irritable Bowel Syndrome and Chronic Idiopathic Constipation. Am J Gastroenterol. 2014: 109:S2-S26; doi: 1001038/ajg.2014.187

Weinberg DS, Smalley W, Heidelbaugh JJ, et al. American Gastroenterological Association Institute Guideline on the Pharmacological Management of Irritable Bowel Syndrome. Gastroenterology. 2014; 147(5):1146-48.

Xifaxan® (rifaximin) [prescribing information]. Bridgewater, NJ: Salix Pharmaceuticals; September 2022. Available from: https://shared.salix.com/shared/pi/xifaxan550-pi.pdf. Accessed June 23, 2023.

Pimentel M. Small intestinal bacterial overgrowth: Clinical manifestations and diagnosis. In: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, February 2022. Accessed June 23, 2023.



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Off-Label Use Rx.01.33

 

Brand Name
Generic Name
Xifaxan®
Rifaximin


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10/1/2023Rx.04.6Claim Payment PolicyOyenusi, Oluwadamilola

​Injectable drugs indicated for administration by a healthcare professional are covered under the medical benefit.  There are times when an exception is requested to cover such drugs under the member's prescription drug benefit where they are otherwise excluded.

The intent of this policy is to describe circumstances when injectable medications typically covered under the medical benefit may be covered under the member’s prescription drug benefit.

Coverage is subject to the terms, conditions, and limitations of the member's contract.

The following medications are covered under the medical benefit:

  1. Cyanocobalamin (vitamin B12) injection

  2. Heparin injection

  3. Hydrocortisone injection

  4. Methotrexate injection (vial)

  5. Testosterone injection

  6. Estrogen vials

  7. Non-insulin syringes/needles

A benefit exception will be made to cover cyanocobalamin injection, heparin injection, hydrocortisone injection, methotrexate vials under the prescription drug benefit when ALL of the following are met:

  1. The requested drug is being used for a Food and Drug Administration approved indication or compendium supported indication as outlined in Off-label Use policy; and

  2. The prescriber assumes responsibility for teaching the member proper preparation, administration and disposal; and

  3. The prescriber provides a statement indicating the reason prescription drug benefit products are not an option, and

  4. ONE of the following:

    1. Provider does not stock the requested medication; or

    2. Access at the provider's office is not feasible due to frequency of administration

A benefit exception will be made to cover testosterone vials and estrogen vials under the prescription drug benefit when ALL of the following are met:

  1. One of the following is met:

    1. The requested drug is being used for a Food and Drug Administration approved indication or compendium supported indication as outlined in the Off-Label Use policy; or

    2. The requested drug is being used as hormone therapy in children, adolescents, and adults with persistent, well-documented gender dysphoria diagnosed in accordance with the criteria established in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition [DSM-5] or is gender diverse as defined in The World Professional Association for Transgender Health (WPATH): Standards of Care, 8th Version (SOC-8) as outlined in medical policy Gender Affirming Interventions (11.09.02); and 

  2. The prescriber assumes responsibility for teaching the member proper preparation, administration and disposal; and

  3. The prescriber provides a statement indicating the reason prescription drug benefit products are not an option, and

  4. ONE of the following:

    1. Provider does not stock the requested medication; or

    2. Access at the provider's office is not feasible due to frequency of administration

A benefit exception will be made to cover non-insulin syringes/needles under the prescription drug benefit when required to administer an injectable medication and not supplied with the product.

Coverage is subject to the terms of the member's prescription drug benefit, including but not limited to cost-share.

Authorization duration: 2 years

​N/A

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Off-Label Use Rx.01.33

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10/1/2023Rx.01.184CommercialOyenusi, Oluwadamilola

An interim clinical policy is the written description of the plan’s utilization management position concerning the use or application of a new or recently introduced product that is covered under the pharmacy benefit. These policies are implemented prior to review by the Pharmacy and Therapeutics Committee. Criteria are based on an initial clinical review performed by Clinical Pharmacy Services. The intent of the interim policy is to provide a means to manage newly approved products while allowing sufficient time for a thorough clinical review and evaluation by the Pharmacy and Therapeutics Committee. The product to which an interim clinical policy applies is considered medically necessary when requested for an indication approved by the Food and Drug Administration and, when available, there is inadequate response or inability to tolerate one generic alternative and one preferred brand with the same indication. The policy is effective as of the date of market entry.  A clinical policy, if issued, will be available when approved by the Pharmacy and Therapeutics Committee within 180 days of the drug market entry, designated by the Medispan release date. If a clinical policy is not approved within 180 days, the interim clinical policy will be retired. Interim clinical policy will remain in effect until the clinical policy effective date.

​The intent of this policy is to communicate the medical necessity criteria for new or recently introduced products as provided under the member’s prescription drug benefit.

A new or recently introduced product will be considered medically necessary when both of the following are met:

  1. Diagnosis of an FDA approved indications; and
  2. Inadequate response or inability to tolerate BOTH of the following (when available):
    1. One generic alternative with the same indication; and
    2. One preferred brand with the same indication

Authorization duration: 2 years

​N/A

​None

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​Off-Label Use Rx. 01.33

Applicable drugs will vary as new products are released to market.

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Idiopathic thrombocytopenia purpura (ITP): ITP is an immune disorder in which the blood doesn't clot normally. ITP can cause excessive bruising and bleeding and can be characterized as an unusually low level of platelets, or thrombocytes, in the blood results in ITP.

Thrombocytopenia in patients with hepatitis C: Thrombocytopenia can occur in patients with chronic hepatitis C virus (HCV) infection. The pathophysiology is multifactorial and includes direct bone marrow suppression, an overactive spleen, decreased production of thrombopoietin and therapeutic adverse effect all contributing to thrombocytopenia.

Aplastic Anemia: A blood disorder caused by failure of the bone marrow to make enough new blood cells. Bone marrow is a sponge-like tissue inside the bones that makes stem cells that differentiate into red blood cells, white blood cells, and platelets.

Thrombocytopenia in patients with chronic liver disease: Individuals with chronic liver disease have varying degree of thrombocytopenia which may be caused by impaired platelet production from decreased hepatic synthesis of thrombopoietin. In addition, individuals with advanced liver disease may have reduced platelet function due to coexisting uremia, infection, and/or endothelial abnormalities. These factors combined put the individuals with chronic liver disease at an increased risk for bleeding especially during procedures.

Mechanism of Action:

Eltrombopag olamine (Promacta®) tablets contain a thrombopoietin (TPO) receptor agonist for oral administration. Eltrombopag interacts with the transmembrane domain of the TPO receptor which initiates signaling cascades that induce proliferation and differentiation from bone marrow progenitor cells ultimately increasing platelet production. 

Eltrombopag olamine (Promacta®) is a thrombopoietin receptor agonist indicated for the treatment of:

  1. Thrombocytopenia in adult and pediatric patients 1 year and older with chronic idiopathic thrombocytopenia purpura (ITP) who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy. PROMACTA should be used only in patients with ITP whose degree of thrombocytopenia and clinical condition increase the risk for bleeding. 
  2. Thrombocytopenia in patients with chronic hepatitis C to allow the initiation and maintenance of interferon-based therapy. PROMACTA should only be used in patients with chronic hepatitis C whose degree of thrombocytopenia prevents the initiation of interferon-based therapy or limits the ability to maintain interferon-based therapy. 
  3. In combination with standard immunosuppressive therapy for the first-line treatment of adult and pediatric patients 2 years and older with severe aplastic anemia.
  4. Patients with severe aplastic anemia who have had an insufficient response to immunosuppressive therapy.

Fostamatinib disodium (Tavalisse) is a tyrosine kinase inhibitor with demonstrated activity against spleen tyrosine kinase (SYK). The major metabolite of fostamatinib, R406, inhibits signal transduction of Fc-activating receptors and B-cell receptor. The fostamatinib metabolite R406 reduces antibody-mediated destruction of platelets.

Fostamatinib disodium (Tavalisse) is indicated for the treatment of thrombocytopenia in adult patients with chronic immune thrombocytopenia (ITP) who have had an insufficient response to a previous treatment.

Avatrombopag (Doptelet®) and lusutrombopag (Mupleta®) tablets contain a thrombopoietin (TPO) receptor agonist for oral administration. They stimulate proliferation and differentiation of megakaryocytes from bone marrow progenitor cells resulting in an increased production of platelets. Avatrombopag does not compete with TPO for binding to the TPO receptor and has an additive effect with TPO on platelet production. Lusutrombopag induces megakaryocyte maturation by interacting with the transmembrane domain of human TPO receptor expressed on megakaryocytes.

Avatrombopag (Doptelet®) and Lusutrombopag (Mupleta®)are indicated for the treatment of thrombocytopenia in adult patients  with chronic liver disease who are scheduled to undergo a procedure. ​

Doptelet® (avatrombopag) is also indicated for the treatment of thrombocytopenia in adult patients with chronic immune thrombocytopenia who have had an insufficient response to a previous treatment.

​The intent of this policy is to communicate the medical necessity criteria for eltrombopag olamine (Promacta®), fostamatinib disodium (Tavalisse™), avatrombopag (Doptelet®), lusutrombopag (Mulpleta®) as provided under the member's prescription drug benefit.

Chronic Immune (Idiopathic) Thrombocytopenic Purpura (ITP)

INITIAL CRITERIA: Eltrombopag olamine (Promacta®) is approved when ALL of the following are met:

  1. Diagnosis of relapsed/refractory chronic immune (idiopathic) thrombocytopenic purpura (ITP) for greater than 6 months; and
  2. Member is 1 year of age or older; and
  3. Baseline platelet count is less than 30,000/mcL; and
  4. Insufficient response to corticosteroids, immunoglobulins, or splenectomy; and
  5. Prescribed by or in consultation with a hematologist/oncologist​

Initial authorization duration: 2 years

INITIAL CRITERIA: Fostamatinib (Tavalisse™) is approved when all of the following are met:

  1. Diagnosis of chronic immune (idiopathic) thrombocytopenic purpura; and
  2. Baseline platelet count is less than 30,000/mcL; and
  3. Member's degree of thrombocytopenia and clinical condition increase the risk of bleeding; and
  4. Documentation of an inadequate response or inability to tolerate ONE of the following:
    1. Corticosteroids; or 
    2. Immunoglobulins; or
    3. Splenectomy; or
    4. Thrombopoietin receptor agonists (e.g., Nplate®, Promacta®); or
    5. Rituximab; and
  5. Prescribed by or in consultation with a hematologist/oncologist; and
  6. Member is 18 years of age or older

Initial authorization duration: 2 years

INITIAL CRITERIA: Avatrombopag (Doptelet®) is approved when ALL of the following are met:

  1. Diagnosis of Chronic Immune Thrombocytopenia (ITP) or relapsed/refractory ITP; and
  2. Baseline platelet count is less than 30,000mcL; and
  3. Inadequate response or inability to tolerate ONE of the following: 
    1. corticosteroids; or
    2. imunoglobulins; or
    3. splenectomy; or
    4. Rituxan (rituximab); and
  4. Member's degree of thrombocytopenia and clinical condition increase the risk of bleeding; and
  5.  Prescribed by or in consultation with a hematologist/oncologist; and
  6. Member is 18 years of age or older
Initial authorization duration: 2 years

CONTINUATION CRITERIA: Eltrombopag olamine (Promacta®), Fostamitinib (Tavalisse™), Avatrombopag (Doptelet®) is re-approved when ALL of the following are met:

  1. Diagnosis of chronic immune (idiopathic) thrombocytopenic purpura; and
  2. Documentation of a positive clinical response to Promacta®, Tavalisse™, Doptelet® therapy as evidence by an increase in platelet count to a level sufficient to avoid clinically important bleeding; and
  3. Prescribed by or in consultation with a hematologist/oncologist

Continuation authorization duration: 2 years.

Aplastic anemia

INITIAL CRITERIA: Eltrombopag olamine (Promacta®) is approved when ALL of the following are met:

  1. Diagnosis of severe aplastic anemia; and
  2. Member is 2 years of age or older; and
  3. One of the following:
    1. Inadequate response or inability to tolerate immunosuppressive therapy with antithymocyte globulin (ATG) and cyclosporine; or
    2. Documentation that the requested drug will be used in combination with antithymocyte globulin (ATG) and cyclosporin and
  4. Member has thrombocytopenia defined as platelet count less than 30,000/mcL; and
  5. Prescribed by or in consultation with a hematologist/oncologist

Initial authorization duration: 2 years

CONTINUATION CRITERIA: Eltrombopag olamine (Promacta®) is re-approved when ALL of the following are met:

  1. Diagnosis of relapsed/refractory chronic immune (idiopathic) thrombocytopenic purpura (ITP) or severe aplastic anemia; and
  2. Documentation of a positive clinical response to Promacta therapy; and
  3. Prescribed by or in consultation with a hematologist/oncologist

Continuation authorization duration: 2 years

Thrombocytopenia associated with hepatitis C

INITIAL CRITERIA: Eltrombopag olamine (Promacta®) is approved when ALL of the following are met:

  1. Diagnosis of thrombocytopenia associated with hepatitis C; and
  2. Member is 18 years of age or older; and
  3. ONE of the following:
    1. Member has thrombocytopenia defined as platelets less than 90,000/mcL for initiation (pre-treatment) of interferon-based therapy; or
    2. Member has thrombocytopenia defined as platelets less than 75,000/mcL for maintenance of optimal interferon-based therapy; and
  4. Prescribed by or in consultation with one of the following:
    1. Hematologist/ oncologist
    2. Gastroenterologist
    3. Hepatologist
    4. Infectious disease specialist
    5. HIV specialist

Initial authorization duration: 48 weeks

CONTINUATION CRITERIA: Eltrombopag olamine (Promacta®) is re-approved when ALL of the following are met:

  1. Diagnosis of thrombocytopenia associated with hepatitis C; and
  2. ONE of the following:
    1. For members that started treatment with Promacta prior to initiation of treatment with interferon, BOTH of the following:
      1. Member is currently on antiviral interferon therapy for treatment of chronic hepatitis C, and
      2. Member reached a threshold platelet count that allows initiation of antiviral interferon therapy with Promacta treatment by week 9; or
    2. For members that started treatment with Promacta while on concomitant treatment with interferon, member is currently on antiviral interferon therapy for treatment of chronic hepatitis C; and
  3. Prescribed by or in consultation with one of the following:
    1. Hematologist/ oncologist
    2. Gastroenterologist
    3. Hepatologist
    4. Infectious disease specialist
    5. HIV specialist

Continuation authorization duration: 48 weeks

Thrombocytopenia in Chronic Liver Disease Prior to Planned Procedure

Lusutrombopag (Mulpleta®) or Avatrombopag (Doptelet®) is approved when ALL of the following are met:

  1. Diagnosis of thrombocytopenia; and
  2. Member has chronic liver disease; and
  3. Member is scheduled to undergo a procedure; and
  4. Baseline platelet count is less than 50,000/mcL; and
  5. Member is 18 years of age or older

Approval duration: 1 month

Eltrombopag olamine (Promacta®):

1.     Risk For Hepatic Decompensation In Patients With Chronic Hepatitis C

  • In patients with chronic hepatitis C, eltrombopag in combination with interferon and ribavirin may increase the risk of hepatic decompensation. 

2.     Eltrombopag may increase the risk of severe and potentially life-threatening hepatotoxicity. Monitor hepatic function and discontinue dosing as recommended.

“Aplastic Anemia." Genetic and Rare Diseases Information Center, National Institute of Health, 5 July 2017, rarediseases.info.nih.gov/diseases/5836/aplastic-anemia. Accessed June 22, 2023.

Dahal, Sumit, et al. “Thrombocytopenia in Patients with Chronic Hepatitis C Virus Infection." Advances in Pediatrics., U.S. National Library of Medicine, 1 Mar. 2017, www.ncbi.nlm.nih.gov/pmc/articles/PMC5333732/. Accessed June 22, 2023.

Doptelet® (avatrombopag) [package insert]. Durham, NC. AkaRx, Inc. July 2021. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=e2d5960d-6c18-46cc-86bd-089222b09852&type=display. Accessed June 22, 2023.

Eltrombopag olamine (Promacta®) [package insert]. Basel, Switzerland. Novartis Pharmaceuticals Co. Ltd. March 2023. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=7714a0ed-34bb-46e6-a0a5-b363908b22c2&type=display. Accessed June 22, 2023.

Mulpleta® (lusutrombopag) [package insert]. Florham Park, NJ. Shionogi Inc., April 2020. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=f9fd0cfd-717d-4a87-99bc-de7b38807e55&type=display. Accessed June 22, 2023.

Shah, N. MD, Intagliata, N, MD. December 2021. Hemostatic abnormalities in patients with liver disease. UpToDate. Access June 22, 2023.

“Idiopathic Thrombocytopenic Purpura (ITP)." Mayo Clinic, Mayo Foundation for Medical Education and Research, 9 Aug. 2017, www.mayoclinic.org/diseases-conditions/idiopathic-thrombocytopenic-purpura/diagnosis-treatment/drc-20352330. Accessed June 22, 2023.

Tavalisse™ (fostamatinib) [package insert]. San Francisco, CA. Rigel Pharmaceutical, Inc. November 2020. Available from: https://tavalisse.com/downloads/pdf/Tavalisse-Full-Prescribing-Information.pdf. Accessed June 22, 2023.


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Rx.01.33 Off Label Use

Brand NameGeneric Name
Promacta®Eltrombopag olamine
Tavalisse™fostamatinib
Doptelet®avatrombopag
Mulpleta®lusutrombopag
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10/1/2023Rx.01.213CommercialOyenusi, Oluwadamilola

Mycobacterium avium complex (MAC) is the most common pulmonary nontuberculous mycobacterial (NTM) infections of the lung in almost all regions of the world. Antimycobacterial treatment is prolonged and potentially difficult to tolerate and should only be considered in individuals who meet the clinical, radiographic, and microbiologic criteria for the diagnosis of nontuberculous mycobacterial infection. Three-drug combination regimen is recommended for those treated for MAC pulmonary disease and treatment is continued until sputum cultures are consecutively negative for at least 12 months.

Amikacin liposome inhalation suspension (Arikayce®) is an aminoglycoside antibacterial indicated in adults who have limited or no alternative treatment options, for the treatment of Mycobacterium avium complex (MAC) lung disease as part of a combination antibacterial drug regimen in patients who do not achieve negative sputum cultures after a minimum of 6 consecutive months of a multidrug background regimen therapy. As only limited clinical safety and effectiveness data for ARIKAYCE are currently available, reserve ARIKAYCE for use in adults who have limited or no alternative treatment options. This drug is indicated for use in a limited and specific population of patients.  

​The intent of this policy is to communicate the medical necessity criteria for amikacin liposome inhalation suspension (Arikayce®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: ​Arikayce® (amikacin liposome inhalation suspension) is approved when ALL of the following are met:

1. Diagnosis of refractory Mycobacterium avium complex (MAC) lung disease; and

2. Member has not achieved negative sputum cultures after a minimum of 6 consecutive months of multidrug background regimen therapy; and

3. Documentation that the medication will be used as part of a combination antibacterial regimen; and

4. Member is 18 years of age or older; and

5. Prescribed by or in consultation with a pulmonologist or infectious diseases specialist

Initial authorization duration: 6 months

REAUTHORIZATION CRITERIA: Arikayce® (amikacin liposome inhalation suspension) is re-approved when both of the following are met:

  1. Documentation of positive clinical response to therapy; and
  2. Documentation that the medication will be used as part of a combination antibacterial regimen

Reauthorization duration: 12 months


​WARNING: RISK OF INCREASED RESPIRATORY ADVERSE REACTIONS

ARIKAYCE has been associated with a risk of increased respiratory adverse reactions, including, hypersensitivity pneumonitis, hemoptysis, bronchospasm, and exacerbation of underlying pulmonary disease that have led to hospitalizations in some cases.

Arikayce® (amikacin liposome inhalation suspension) [prescribing information]. Bridgewater, NJ. Insmed®. February 2023. Available at: https://www.arikayce.com/pdf/full-prescribing-information.pdf. Accessed June 22, 2023.

Kasperbauer, S. Treatment of Mycobacterium avium complex pulmonary infections in adults. UpToDate Web site. September 2021. www.uptodate.com. Accessed June 22, 2023. ​



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Rx.01.33 Off-Label Use

Brand NameGeneric Name
Arikayce®Amikacin liposome inhalation suspension
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10/1/2023Rx.01.215CommercialOyenusi, Oluwadamilola

​Hereditary transthyretin amyloidosis (hATTR) is a rare autosomal dominant, progressively debilitating, and often fatal genetic disease characterized by the accumulation of abnormal amyloid protein in tissues. Transthyretin (TTR) is produced primarily by the liver and is responsible for the transport of thyroxine and retinol binding protein-vitamin A complex. The hATTR genetic mutations lead to mutated TTR protein which results in destabilization from the TTR tetramer into monomers and oligomers, protein misfolding, and aggregation resulting in formation of TTR amyloid fibrils.   hATTR can present with peripheral and/or autonomic neuropathy, infiltrative cardiomyopathy, vitreous amyloid, or leptomenigeal disease.

Inotersen (Tegsedi™) is an antisense oligonucleotide that causes degradation of mutant and wild-type TTR mRNA through binding to the TTR mRNA, which results in a reduction of serum TTR protein and TTR protein deposits in tissues.

Inotersen (Tegsedi™) is indicated for the treatment of the polyneuropathy of hereditary transthyretin-mediated amyloidosis in adults.  

Transthyretin-mediated amyloidosis with cardiomyopathy (ATTR-CM) occurs when amyloidosis occurs in the cardiac tissue.  Wild type ATTR-CM typically presents in men aged 65 years and older, but it can occur in women and in younger patients. Amyloid accumulates in the cardiac tissue leading to atrial and ventricular wall thickening and diastolic dysfunction, arrhythmias, and preserved ejection fraction heart failure.  For wild type ATTR-CM, the median survival is reported to be 3.6 years.

 

Patients with wild type ATTR may also present with bilateral carpal tunnel syndrome as an initial symptom years before the onset of cardiac symptoms (Sekijima 2015).

Due to the lack of effective therapy, testing for ATTR-CM has been underutilized. Testing may include echocardiogram, cardiac magnetic resonance imaging (MRI), and nuclear scintigraphy. Tissue biopsy, either endomyocardial tissue or other locations such as abdominal fat pad, is the gold standard for diagnosis of amyloidosis. A non-invasive test to identify those with ATTR-CM is radiotracer 99mtechnetium pyrophosphate scan (99mTc-PYP). The FDA-has not yet approved 99mTc-PYP test for the diagnosis of ATTR-CM, but it is being used in clinical practice (Vyndaqel Dossier 2019).

Tafamidis meglumine (Vyndaqel®, Vyndamax ®) is a selective transthyretin (TTR) stabilizer, limiting the dissociation of the native TTR tetramer into monomers, which reduces TTR amyloid fibril formation.

Tafamidis meglumine (Vyndaqel®, Vyndamax ®) is indicated for the treatment of the cardiomyopathy of wild type or hereditary transthyretin-mediated amyloidosis (ATTR-CM) in adults to reduce cardiovascular mortality and cardiovascular-related hospitalization. 


 

​The intent of this policy is to communicate the medical necessity criteria for inotersen (TegsediTM) ), tafamidis meglumine (Vyndamax®, Vyndaqel®) as provided under the member's prescription drug benefit.

​INITIAL CRITERIA Tegsedi™ (inotersen) is approved when ALL of the following are met:

  1. Diagnosis of polyneuropathy of hereditary transthyretin-mediated (hATTR) amyloidosis with transthyretin mutation (e.g., V30M) confirmed by molecular genetic testing; and
  2. Documentation of ONE of the following baseline ambulation parameters in either the Familial Amyloid Polyneuropathy (FAP) Stage or Polyneuropathy Disability (PND) Score
    1. Stage 1 (unimpaired ambulation) or 2 (assisted ambulation) on the Familial Amyloid Polyneuropathy (FAP) staging tool; or
    2. Score I, II, IIIa, or IIIb on the Polyneuropathy Disability (PND) scoring tool; AND
  3. Documented presence of cardiac or renal manifestations, or motor, sensory, or autonomic neuropathy related to the hATTR amyloidosis with polyneuropathy (e.g., neuropathic pain, muscle weakness that affects daily living, orthostatic hypotension, diarrhea, nausea, vomiting, heart failure, arrhythmias, proteinuria, renal failure; vision disorders, such as vitreous opacity, dry eyes, glaucoma, or pupils with an irregular or scalloped appearance; and
  4. Documentation confirming the member has not had a liver transplant; and
  5. Prescribed by or in consultation with a neurologist, geneticist, or professional provider specializing in the treatment of amyloidosis; and
  6. Member is 18 years of age or older; and
  7. No concurrent use with patisiran (Onpattro), vutrisiran (Amvuttra), or any other RNA interference agents
​Initial authorization duration: 16 months


CONTINUATION CRITERIA Tegsedi™ (inotersen) is re-approved when  BOTH of the following are met:

  1. Documentation of one of the following
    1. Stage 1 (unimpaired ambulation) or 2 (assisted ambulation) on the Familial Amyloid Polyneuropathy (FAP) staging tool; or
    2. Score I, II, IIIa, or IIIb on the Polyneuropathy Disability (PND) scoring tool; and
  2. Documented improvement or stability in the signs and symptoms hATTR amyloidosis with polyneuropathy (e.g., neuropathic pain, muscle weakness that affects daily living, orthostatic hypotension, diarrhea, nausea, vomiting, heart failure, arrhythmias, proteinuria, renal failure; vision disorders, such as vitreous opacity, dry eyes, glaucoma, or pupils with an irregular or scalloped appearance), based on objective or standard evaluation scales.
Continuation authorization duration: 2 years​

 

INITIAL CRITERIA Tafamidis meglumine (Vyndamax®, Vyndaqel®) is approved when ALL of the following are met:

  1. Member is 18 years of age or older; AND
  2. Diagnosis of transthyretin-mediated amyloidosis with cardiomyopathy (ATTR-CM) confirmed by ONE of the following:
    1. Member has a transthyretin (TTR) mutation (e.g., V122I), or
    2. Cardiac or noncardiac tissue biopsy demonstrating histologic confirmation of TTR amyloid deposits, or
    3. All of the following: echocardiogram or cardiac magnetic resonance image suggestive of amyloidosis, scintigraphy scan suggestive of cardiac TTR amyloidosis, and absence of light-chain amyloidosis; AND
  3.  Prescribed by or in consultation with a cardiologist; and
  4. One of the following:
    1. History of heart failure (HF), with at least one prior hospitalization for HF, or
    2. Presence of clinical signs and symptoms of HF (e.g., dyspnea, edema); or
  5. Member has New York Heart Association (NYHA) Functional Class I, II, or III heart failure.

Initial authorization duration: 12 months



REAUTHORIZATION CRITERIA: Tafamidis meglumine (Vyndamax®, Vyndaqel®) is re-approved when ALL of the following are met:
  1. Positive clinical response to therapy; and
  2. Member continues to have NYHA Functional Class I, II, or III heart failure; and
  3. Prescribed by or in consultation with a cardiologist.

 Reauthorization duration: 12 months

 

​WARNING: THROMBOCYTOPENIA AND GLOMERULONEPHRITIS

Thrombocytopenia

  • TEGSEDI causes reductions in platelet count that may result in sudden and unpredictable thrombocytopenia, which can be life-threatening.
  • Testing prior to treatment and monitoring during treatment is required

Glomerulonephritis
  • TEGSEDI can cause glomerulonephritis that may require immunosuppressive treatment and may result in dialysis-dependent renal failure.
  • Testing prior to treatment and monitoring during treatment is required

 

Gorevic PD. Genetic factors in the amyloid diseases. UpToDate Web site. Updated December 18, 2018. Available from: http://www.uptodate.com/. Accessed June 29, 2023.

Grogan M, Scott CG, Kyle RA, et al. Natural history of wild type transthyretin cardiac amyloidosis and risk stratification using a novel staging system. J Am Coll Cardiol. 2016;68:1014-1020.

Hawkins PN, Ando Y, Dispenzeri A, et al. Evolving landscape in the management of transthyretin amyloidosis. Ann Med. 2015;47:625-638.

Klaassen SHC, Tromp J, Nienhuis HLA, et al. Involvement at presentation in hereditary transthyretin-derived amyloidosis and the value of N-terminal pro-B-type natriuretic peptide. Am J Cardiol. 2018;121:107-112.

Maurer MS, Grogan DR, Judge DP, et al. Tafamidis in transthyretin amyloid cardiomyopathy. Effects on transthyretin stabilization and clinical outcomes. Circ Heart Fail. 2015;8:519-526.

Maurer MS, Hanna M, Grogan M, et al. Genotype and phenotype of transthyretin cardiac amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey). J Am Coll Cardiol. 2016;68(2):161-172.

Maurer MS, Schwartz JH, Gundapaneni B, et al. Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy. N Engl J Med. 2018;379:1007-1016.

Fontana M. Cardiac amyloidosis: Epidemiology, clinical manifestations, and diagnosis. UpToDate Web site. Updated August 2022. Available from: https://www.uptodate.com/contents/cardiac-amyloidosis-clinical-manifestations-and-diagnosis?search=clinical-manifestations-and-diagnosis-of-amyloid-cardiomyopathy&source=search_result&selectedTitle=1~80&usage_type=default&display_rank=1. Accessed June 29, 2023.

Merlini G, Planté-Bordeneuve V, Judge DP, et al. Effects of tafamidis on transthyretin stabilization and clinical outcomes in patients with non-Val30Met transthyretin amyloidosis. J Cardiovasc Trans Res. 2013;6:1011-1020.

Mundayat R, Steward M, Alvir J, et al. Positive effectiveness of tafamidis in delaying disease progression in transthyretin familial amyloid polyneuropathy up to 2 years: an analysis from the Transthyretin Amyloidosis Outcomes Survey (THAOS) Neurol Ther. 2018;7:87-101.

Nativi-Nicolau J, Maurer MS. Amyloidosis cardiomyopathy: update in diagnosis and treatment of the most common types. Curr Opin Cardiol. 2018;33(5):571-579.

Pfizer Press Release. FDA issues complete response letter for Pfizer's tafamidis meglumine new drug application. https://investors.pfizer.com/investor-news/press-release-details/2012/FDA-Issues-Complete-Response-Letter-For-Pfizers-Tafamidis-Meglumine-New-Drug-Application/default.aspx. June 18, 2012. Accessed June 29, 2023.

Plante-Bordeneuve V. Update in the diagnosis and management of transthyretin familial amyloid polyneuropathy. J Neurol. 2014;261:1227-1233.

Ponikowski P, Voors AA, Anker SD, et al. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: The Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC). Developed with the special contribution of the Heart Failure Association (HFA) of the ESC. Eur Heart J. 2016;37(27):2129-2200.

Seferović PM, Polovina M, Bauersachs J, et al. Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology. Eur J Heart Fail. 2019;21:553-576.

Sekijima Y. Transthyretin (ATTR) amyloidosis: clinical spectrum, molecular pathogenesis and disease-modifying treatments. J Neurol Neurosurg Psychiatry. 2015;86:1036-1043.

Siddiqi OK, Ruberg FL. Cardiac amyloidosis: an update on pathophysiology, diagnosis, and treatment. Trends Cardiovasc Med. 2018;28:10-21.

Tegsedi™ (inotersen) [prescribing information]. Boston MA. Akcea Therapeutics, Inc. June 2022. Available at: https://tegsedi.com/wp-content/uploads/2018/10/prescribing-information.pdf. Accessed June 29, 2023.

Vyndaqel and Vyndamax (tafamidis, tafamidis meglumine )[prescribing information]. New York, NY: Pfizer Pharmaceuticals, Inc. April 2023. Available from: labeling.pfizer.com/ShowLabeling.aspx?id=11685. Accessed June 29, 2023.



 



 


 

 

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Off-Label Use Rx.01.33

Brand NameGeneric Name
Tegsedi™Inotersen
Vyndamax®, Vyndaqel®Tafamidis meglumine



265
  
10/1/2023Rx.01.217CommercialOyenusi, Oluwadamilola

 

Neurotrophic keratitis (NK) is a rare degenerative corneal disease caused by impairment in the first branch of the trigeminal nerve which leads to a decrease in or absence of corneal sensitivity. Loss of sensitivity impairs wound healing, leading to corneal epithelial breakdown, development of ulcerations, melting of the stroma, and corneal perforation. Diagnosis, prognosis, and treatment are based on disease severity, which is classified into 3 stages. Stage 1 (mild) NK is characterized by ocular surface irregularity and reduced vision; stage 2 (moderate) is characterized by a non-healing persistent epithelial defect (PED); and stage 3 (severe) exhibits corneal ulceration involving subepithelial (stromal) tissue, which may progress to corneal melting and perforation.  

Therapy for stage 1 disease aims to prevent epithelial breakdown, generally by administering preservative-free artificial tears and discontinuing all topical and systemic medications associated with ocular surface toxicity. The use of punctal plugs may also help increase tear volume. The goal of treatment for stage 2 NK is to promote healing of the epithelial defect and to avoid the development of a corneal ulcer. In addition to the therapies in the previous stage, topical antibiotics are recommended to prevent infections. Therapeutic corneal or scleral contact lenses may be used to promote healing; however, there may be an increased risk of secondary infections. Autologous serum eye drops, which contain components of natural tears, have increasingly been used to treat ocular surface disorders including NK. The main goal of treatment at stage 3 is to prevent corneal thinning and perforation. Various surgeries and procedures are available to treat ulcers not responding to medical treatment. Tarsorrhaphy is the most commonly used procedure to promote corneal healing. Alternative treatments include botulinum-induced ptosis, amniotic membrane transplantation, eyelid closure with tape, patching, and use of the conjunctival flap to cover the corneal surface.

Cenegermin-bkbj (Oxervate™) is a novel recombinant human nerve growth factor (rhNGF) produced in Escherichia coli that is structurally identical to human NGF. NGF is an endogenous protein involved in the differentiation and maintenance of neurons, which acts through specific high-affinity and low affinity NGF receptors in the anterior segment of the eye to support corneal innervation and integrity. Cengermin-bkbj (Oxervate™) is the first FDA-approved pharmacologic therapy indicated for the treatment of neurotropic keratitis (NK). Oxervate™ is an 8-week treatment cycle per affected eye(s).




 

​The intent of this policy is to communicate the medical necessity criteria for cenegermin-bkbj (Oxervate™) as provided under the member’s prescription drug benefit.

INITIAL CRITERIA Cenegermin-bkbj (Oxervate™) is approved when ALL of the following are met:

  1. Diagnosis of Stage 2 or 3 neurotrophic keratitis; and
  2. Documentation of an inadequate response or inability to tolerate at least one over-the-counter ocular lubricant used at an optimal dose and frequency for at least two weeks (e.g., artificial tears, lubricating gels/ointments, etc.); and
  3. Prescribed by or in consultation with an ophthalmologist; and
  4. Submission of chart documentation indicating treatment of left eye, right eye, or both; and
  5. Member will not exceed 8 weeks of Oxervate therapy per affected eye(s)

Initial authorization duration: 3 months

REAUTHORIZATION CRITERIA Cenegermin-bkbj (Oxervate™) is re-approved when ALL of the following are met:
  1. Submission of chart documentation indicating treatment of left eye, right eye, or both; and
  2. One of the following:
    1. Member has received less than or equal to 8 weeks of therapy (one course of therapy) per affected eye(s); and
    2. Documentation of clinical rationale for treatment greater than 8 weeks (e.g., member has a recurrence of neurotropic keratitis in the same eye, or treatment of a different eye); and
  3. Documentation of clinical response to prior Oxervate™ therapy; and
  4. Member will not exceed a total of 16 weeks of Oxervate™ therapy per affected eye(s); and
  5. Prescribed by or in consultation with an ophthalmologist

Reauthorization duration: 3 months

Lifetime limit: 16 weeks of therapy per affected eye



 

None

 

Bonini S, Lambiase A, Rama P, et al.; REPARO Study Group. Phase II randomized, double-masked, vehicle-controlled trial of recombinant human nerve growth factor for neurotrophic keratitis. Ophthalmology. 2018;125(9):1332-1343. Accessed June 22, 2023.

Dua HS, Said DG, Messmer EM, et al. Neurotrophic keratopathy. Prog Retin Eye Res. 2019; 66:107-131. Accessed June 22, 2023.

Oxervate® (cenegermin-bkbj) [prescribing information]. Boston, MA: Dompe U.S. Inc. October 2019. Available at:  https://oxervate.com/pdf/PrescribingInformation.pdf. Accessed June 22, 2023.

Pflugfelder SC, Massaro-Giordano M, Perez VL, Hamrah P, Deng SX, Espandar L, Foster CS, Affeldt J, Seedor JA, Afshari NA, Chao W, Allegretti M, Mantelli F, Dana R. Topical Recombinant Human Nerve Growth Factor (Cenegermin) for Neurotrophic Keratopathy: A Multicenter Randomized Vehicle-Controlled Pivotal Trial. Ophthalmology. 2020 Jan;127(1):14-26. Accessed June 22, 2023.

Pocobelli A, Komaiha C, De Carlo L, Pocobelli G, Boni N, Colabelli Gisoldi RAM. Role of Topical Cenegermin in Management of a Cornea Transplant in a Functionally Monocular Patient with Neurotrophic Keratitis and Facial Nerve Palsy: A Case Report. Int Med Case Rep J. 2020 Nov 11;13:617-621. Acccessed June 22, 2023.

Sacchetti M, Lambiase A. Diagnosis and management of neurotrophic keratisis. Clin Ophthalmol. 2014; 8:571-579. Accessed June 22, 2023.

Semeraro F, Forbice E, Romano V, et al. Neurotrophic keratitis. Opthalmologica. 2014;231(4):191-197. Accessed June 22, 2023.

Versura P, Giannaccare G, Pellegrini M, Sebastiani S, Campos EC. Neurotrophic keratitis: current challenges and future prospects. Eye Brain. 2018; 10:37-45. Accessed June 22, 2023.


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​Rx.01.33 Off-Label Use
Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit

Brand Name
Generic Name
Oxervate™cenegermin-bkbj
266
  
10/1/2023Rx.01.221CommercialOyenusi, Oluwadamilola

Commercial payers, including managed care organizations and self-funded groups, must ensure appropriate use of drugs in healthcare setting. One way to accomplish this goal is to review claims that exceed a defined threshold.

Prescription claims with a total cost exceeding $10,000 per claim reject for preliminary review at the point-of-sale with message “claim dollar amount exceeded", requiring the dispensing pharmacist to contact the pharmacy benefit manager. This will apply to any claim that exceeds $10,000, allowing them to be reviewed for clinical appropriateness prior to dispensing. For drugs not meeting approval criteria per Off-Label Use policy, the use for which the drug was prescribed would be deemed experimental/investigational.  


 

​The intent of this policy is to communicate the medical necessity criteria for claims exceeding $10,000 as provided under the member's prescription benefit.

Claims that exceed $10,000 are approved when the use, including indication, dose, quantity, and duration, for the requested drug is approved by the FDA or considered medically accepted per Off-Label Use policy.


Authorization duration: 2 years, or for the duration of applicable medical necessity approval (for submitted cost plus $3000) 


 

​N/A

Academy of Managed Care Pharmacy® (AMCP). Where We Stand - Fraud, Waste and Abuse in Prescription Drug Benefits. Revised April 2015. Available at: https://www.amcp.org/policy-advocacy/policy-advocacy-focus-areas/where-we-stand-position-statements/fraud-waste-and-abuse-prescription-drug-benefits. Accessed July 21, 2021.

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​Off Label Use Policy Rx.01.33
Compounded Products Rx.01.134
Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit Rx.01.76


 

​Drug claims where total drug cost exceeds $10,000



 

267
  
10/1/2023Rx.01.228CommercialOyenusi, Oluwadamilola

Elevated triglyceride levels are associated with and appear to be implicated in the pathogenesis of atherosclerosis and cardiovascular disease (CVD). Atherosclerosis is the most common underlying pathology in patients with CVD. Fasting plasma triglyceride concentrations may be categorized according to the National Cholesterol Education Program (NCEP) as normal (<150 mg/dL), borderline (150–199 mg/dL), high triglyceride (HTG; 200–499 mg/dL), and severe HTG (HTG; ≥500 mg/dL). Patients with triglycerides above 500 mg/dL are also at risk of pancreatitis. Elevated plasma triglyceride concentrations contribute to increased risk of cardiovascular disease, both directly and because such elevations are associated with risk factors such as obesity, metabolic syndrome, and type 2 diabetes mellitus. Diet and lifestyle changes along with treatment or elimination of secondary causes are recommended before direct pharmacotherapy. If these changes are not possible or not effective, initiating triglyceride-lowering pharmacotherapy may be required.

Vascepa® (icosapent ethyl) is indicated:

  1. As an adjunct to maximally tolerated statin therapy to reduce the risk of myocardial infarction, stroke, coronary revascularization, and unstable angina requiring hospitalization in adult patients with elevated triglyceride (TG) levels (≥ 150 mg/dL) and
    1. Established cardiovascular disease or
    2. Diabetes mellitus and 2 or more additional risk factors for cardiovascular disease.
  2. As an adjunct to diet to reduce TG levels in adult patients with severe (≥ 500 mg/dL) hypertriglyceridemia.

 

The mechanisms of action contributing to reduction of cardiovascular events with Vascepa® (icosapent ethyl) are not completely understood but are likely multi-factorial. Increased omega-3 fatty acid eicosapentaenoic acid (EPA) lipid composition from carotid plaque specimens and increased circulating EPA/arachidonic acid ratio have been observed following EPA treatment. EPA inhibits platelet aggregation under some ex vivo conditions. However, the direct clinical meaning of individual findings is not clear.

​The intent of this policy is to communicate the medical necessity criteria for icosapent ethyl (Vascepa®) as provided under the member's prescription drug benefit.


 

Severe Hypertriglyceridemia

INITIAL CRITERIA: Icosapent ethyl (Vascepa®) is approved when ALL of the following are met:

  1. Diagnosis of severe hypertriglyceridemia defined as pre-treatment triglyceride level greater than or equal to 500 mg/dl; and
  2. Member is 18 years of age or older; and  
  3. Medication will be used adjunct to an appropriate lipid-lowering diet; and
  4. Member has an inadequate response or inability to tolerate omega-3-acid ethyl esters (generic Lovaza®); and
  5. For Brand Vascepa® only, inadequate response or inability to tolerate generic Icosapent ethyl capsules

Initial authorization duration: 6 Months

REAUTHORIZATION CRITERIA: Icosapent ethyl (Vascepa®) is approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy; and
  2. Member continues to use the medication adjunct to an appropriate lipid-lowering diet

Reauthorization duration: 2 years

Hypertriglyceridemia—Reduction of risk of cardiovascular events

INITIAL CRITERIA Icosapent ethyl (Vascepa®) is approved when ALL of the following are met:

  1. Diagnosis of hypertriglyceridemia; and
  2. Member is 18 years of age or older; and  
  3. Member has pre-treatment triglyceride level between 150 mg/dL to 499 mg/dL; and
  4. ONE of the following: 
    1. Member has established cardiovascular disease; OR
    2. Both of the following:
      1. Diagnosis of diabetes mellitus; and
      2. Member has 2 or more additional risk factors for cardiovascular disease (e.g., cigarette smoking, hypertension, creatinine clearance less than 60 ml/min, etc.); and
  5. One of the following:
    1. Member has been receiving 12 consecutive weeks of statin therapy at maximally tolerated dose and will continue to receive statin therapy at maximally tolerated dose; or
    2. ​​Inability to tolerate statin therapy; and
  6. For Brand Vascepa® only, inadequate response or inability to tolerate generic Icosapent ethyl capsules

​Initial authorization duration: 6 months

 

REAUTHORIZATION CRITERIA Icosapent ethyl (Vascepa®) is re-approved when BOTH of the following are met:

  1. Documentation of positive clinical response to therapy; and
  2. One of the following:
    1. Member continues to receive statin therapy at maximally tolerated dose; or
    2. Inability to tolerate statin therapy

 

 

Reauthorization duration: 2 years


 


​N/A

Skulas-Ray AC, Wilson PWF, Harris WS et al on behalf of the American Heart Association. Omega-3 fatty acids for the management of hypertriglyceridemia. Circulation. 2019;140. Accessed June 23, 2023.

Vascepa® (icosapent ethyl) [prescribing information]. Bridgewater, NJ: Amarin Pharma, Inc.; September 2021. Available from: https://amarincorp.com/docs/Vascepa-PI.pdf. Accessed June 23, 2023.

Yuan, G, Al-Shali, KZ, Hegele, RA. Hypertriglyceridemia: its etiology, effects and treatment. CMAJ. 2007;176:1113–1120. doi: 10.1503/cmaj.060963. Accessed June 23, 2023.


 

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​Off-Label Use Rx.01.33​


 

Brand nameGeneric name
Vascepa®Icosapent ethyl


 

268
  
10/1/2023Rx.01.229CommercialOyenusi, Oluwadamilola

The life cycle of HIV can be broken down into 6 steps: (1) entry (binding and fusion), (2) reverse transcription, (3) integration, (4) replication (transcription and translation), (5) assembly, and (6) budding and maturation. The identification and understanding of these processes have provided the basis for antiretroviral agents used to treat HIV.

Antiretroviral agents are the standard of care for treating HIV infections. Antiretroviral therapies with different mechanism of action are usually taken in combination to suppress viral load, improve CD4 cell count, prolong survival, and reduce risk of transmitting HIV to others. Viral failure is defined as patients who had viral loads greater than 400 copies/mL and no viable antiretroviral combination therapy available due to failing at least four of six antiretroviral classes.

Fostemsavir is a prodrug without significant biochemical or antiviral activity that is hydrolyzed to the active moiety, temsavir, which is an HIV-1 attachment inhibitor. Temsavir binds directly to the gp120 subunit within the HIV-1 envelope glycoprotein gp160 and selectively inhibits the interaction between the virus and cellular CD4 receptors, thereby preventing attachment. Additionally, temsavir can inhibit gp120-dependent post-attachment steps required for viral entry into host cells

Rukobia (fostemsavir), in combination with other antiretroviral(s), is indicated for the treatment of HIV-1 infection failing their current antiretroviral regimen due to resistance, intolerance, or safety considerations.

Enfuvirtide is an antiretroviral drug that interferes with the entry of HIV-1 into cells by inhibiting fusion of viral and cellular membranes. Enfuvirtide binds to the first heptad-repeat (HR1) in the gp41 subunit of the viral envelope glycoprotein and prevents the conformational changes required for the fusion of viral and cellular membranes.

Fuzeon (enfuvirtide) in combination with other antiretroviral agents is indicated for the treatment of HIV-1 infection in treatment-experienced patients with evidence of HIV-1 replication despite ongoing antiretroviral therapy



​The intent of this policy is to communicate the medical necessity criteria for Rukobia® (fostemsavir) and Fuzeon® (enfuvirtide) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Fostemsavir (Rukobia®) is approved when ALL of the following are met:
  1. Diagnosis of HIV-1; and 
  2. Member is 18 years of age or older; and 
  3. Will be used in combination with other antiretroviral(s); and 
  4. Member is treatment-experienced with multi-drug resistant HIV-1 infection; and
  5. Member is failing their current antiretroviral regimen due to resistance, intolerance, or safety concerns.

Initial Authorization duration: 12 months

CONTINUATION CRITERIA: Fostemsavir (Rukobia®) is re-approved when there is documentation of positive clinical response to therapy (e.g., decrease in viral load from baseline; HIV-1 RNA <200 copies/mL was achieved; increase in CD4+ cell count over time)

Continuation authorization duration: 2 years

INITIAL CRITERIA: Enfuvirtide (Fuzeon®) is approved when ALL of the following are met: 
  1. Diagnosis of HIV-1; and
  2. Member weighs at least 11kg; and
  3. Will be used in combination with other antiretroviral(s); and
  4. Member is treatment experienced; and
  5. Member is experiencing HIV-1 replication despite ongoing antiretroviral therapy

Initial authorization duration: 12 months

CONTINUATION CRITERIA: Enfuvirtide (Fuzeon®) is re-approved when there is documentation of positive clinical response to therapy (e.g., decrease in viral load from baseline; HIV-1 RNA <200 copies/mL was achieved; increase in CD4+ cell count over time)

Continuation authorization duration: 2 years



​N/A

Rubkobia® (fostemsavir) [package insert]. Research Triangle Park, NC: ViiV Healthcare; January 2022. Available from: https://www.gsksource.com/pharma/content/dam/GlaxoSmithKline/US/en/Prescribing_Information/RUKOBIA/pdf/RUKOBIA-PI-PIL.PDF. Accessed June 22, 2023.

Henrich TJ, Kuritzkes DR. HIV-1 entry inhibitors: recent development and clinical use. Curr Opin Virol. 2013;3(1):51-57. doi:10.1016/j.coviro.2012.12.002. Accessed June 22, 2023.

Centers for Disease Control and Prevention. Estimated HIV incidence and prevalence in the United States, 2010–2015. HIV Surveillance Supplemental Report 2018;23(No. 1). http://www.cdc.gov/ hiv/library/reports/hiv-surveillance.html. Published March 2018. Accessed June 22, 2023.

Kozal M, Aberg J, Pialoux G, et al. Rostemsavir in Adults with Multidrug-Resistant HIV-1 infection, N Eng J Med. 2020 26 March; 382:1232-1243. Accessed June 22, 2023.

Rukobia (fostemsavir), Micromedex. Accessed June 22, 2023.

Fuzeon (enfuvirtide) prescribing information. South San Francisco (CA): Genentech, Inc.; December 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=6935e846-d5a1-49e5-89a2-f8ebe4d5590d#S12.4. Accessed June 22, 2023.


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​Rx.01.33 Off Label Use


Brand NameGeneric Name
Rukobia®Fostemsavir
Fuzeon®Enfuvirtide
269
  
10/1/2023Rx.01.240CommercialOyenusi, Oluwadamilola

Blepharoptosis, or ptosis of the eyelid, refers to drooping of the upper eyelid that usually results from a congenital or acquired abnormality of the muscles that elevate the eyelid. Ptosis may be the presenting sign or symptom of serious neurologic disease. Depending on the degree of ptosis, presenting symptoms may range from an asymptomatic subtle cosmetic defect to significant visual deficits. Standard of care is surgery. While effective in improving visual function and quality-of-life measures, there are risks associated with surgical intervention. The Müller muscle, an accessory smooth muscle, maintains upper eyelid elevation and is innervated by the sympathetic nervous system. Therefore, the Müller muscle is a common surgical and pharmacological target. 

Oxymetazoline is an alpha adrenoceptor agonist targeting a subset of adrenoreceptors in Mueller's muscle of the eyelid. Oxymetazoline (Upneeq™) is indicated for the treatment of acquired blepharoptosis in adults.

Oxymetazoline (Upneeq™), when used solely to change the appearance of any portion of the face, without improving the physiologic functioning of that portion of the body, is considered cosmetic use. 


​The intent of this policy is to communicate the medical necessity criteria for Oxymetazoline (Upneeq™) as provided under the member's prescription drug benefit. 


INITAL CRITERIA: Oxymetazoline (Upneeq™) is approved when ALL of the following are met:

  1. Diagnosis of acquired blepharoptosis; and
  2. Request is not for an excluded benefit (i.e. cosmetic - solely for lifting the eyelid to improve appearance); and
  3. Member has obstructed visual field in primary gaze or down gaze due to blepharoptosis; and
  4. One of the following:
    1. Marginal reflex distance-1 (MRD-1) is less than or equal to 2mm in primary gaze; or
    2. Marginal reflex distance-1 (MRD-1) is less than or equal to 2mm in down gaze; or
    3. Superior visual field loss of at least 12 degrees or 24 percent; and
  5. Other treatable causes of blepharoptosis have been ruled out (e.g., recent botulinum toxin injection, myasthenia gravis); and
  6. Prescribed by or in consultation with an ophthalmologist or optometrist
Initial authorization duration: 3 months​


REAUTHORIZATION CRITERIA: Oxymetazoline (Upneeq™) is re-approved when BOTH of the following are met:

  1. Documentation of positive clinical response to therapy (e.g., improvement in superior visual field, increase in Marginal reflex distance-1 [MRD-1]); and
  2. One of the following:
    1. Marginal reflex distance-1 (MRD-1) is less than or equal to 2mm in primary gaze; or
    2. Marginal reflex distance-1 (MRD-1) is less than or equal to 2mm in down gaze; or
    3. Superior visual field loss of at least 12 degrees or 24 percent

Reauthorization duration: 12 months



​N/A

Upneeq™ (oxymetazoline hydrochloride ophthalmic solution) [prescribing information]. Bridgewater, NJ: RVL Pharmaceuticals, Inc; May 2023. Available from: https://www.upneeq.com/Upneeq-PI.pdf. Accessed June 23, 2023.

Slonim CB, Foster S, Jaros M, et al. Association of Oxymetazoline Hydrochloride, 0.1%, Solution Administration With Visual Field in Acquired Ptosis: A Pooled Analysis of 2 Randomized Clinical Trials [published online ahead of print, 2020 Oct 1]. JAMA Ophthalmol. 2020;138(11):1168-1175. doi:10.1001/jamaophthalmol.2020.3812

Lee, MS. Overview of ptosis. UpToDate Web site. March 2023. www.uptodate.com. Accessed June 23, 2023.






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​Rx.01.33 Off Label Use

Brand NameGeneric Name
Upneeq™Oxymetazoline hydrochloride


270
  
10/1/2023Rx.01.255CommercialOyenusi, Oluwadamilola

Chronic kidney disease exacerbates the cardiovascular risk associated with type 2 diabetes.

Mineralocorticoid receptor overactivation is associated with kidney and cardiovascular diseases, through inflammation and fibrosis that lead to progressive kidney and cardiovascular dysfunction.

Finerenone is a nonsteroidal, selective antagonist of the mineralocorticoid receptor (MR), which is activated by aldosterone and cortisol and regulates gene transcription. Finerenone blocks MR mediated sodium reabsorption and MR overactivation in both epithelial (e.g., kidney) and nonepithelial (e.g., heart, and blood vessels) tissues. MR overactivation is thought to contribute to fibrosis and inflammation. Finerenone has a high potency and selectivity for the MR and has no relevant affinity for androgen, progesterone, estrogen, and glucocorticoid receptors.

Kerendia® (finerenone) is indicated to reduce the risk of sustained eGFR decline, end-stage kidney disease, cardiovascular death, nonfatal myocardial infarction, and hospitalization for heart failure in adult patients with chronic kidney disease associated with type 2 diabetes.



​The intent of this policy is to communicate the medical necessity criteria for finerenone (Kerendia®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA Finerenone (Kerendia®) is approved when ALL of the following are met:

  1. Diagnosis of chronic kidney disease (CKD) associated with type 2 diabetes (T2D); and
  2. Member is 18 years of age or older; and
  3. One of the following:
    1. Minimum 30-day supply trial of a maximally tolerated dose and will continue therapy with ONE of the following:
      1. Generic angiotensin-converting enzyme (ACE) inhibitor (e.g., benazepril, lisinopril); or
      2. Generic angiotensin II receptor blocker (ARB) (e.g., losartan, valsartan); or
    2. Member has contraindication or intolerance to ACE inhibitors or ARBs

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA Finerenone (Kerendia®) is re-approved with documentation of positive clinical response to therapy (e.g., reduced incidence of a sustained declined in eGFR, kidney failure, or renal death)

Reauthorization duration: 2 years

​N/A

Pitt B, Filippatos G, Agarwal R, et al. Cardiovascular events with finerenone in kidney disease and type 2 diabetes. N Engl J Med. 2021;385(24):2252-2263. doi: 10.1056/NEJMoa2110956. Accessed June 22, 2023.

Kerendia® (finerenone) [package insert]. Whippany, NJ: Bayer HealthCare Pharma, Inc.; September 2022. Available at https://www.accessdata.fda.gov/drugsatfda_docs/label/2021/215341s000lbl.pdf. Accessed June 22, 2023.

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​Rx.01.33 Off Label Use

Brand Name

Generic Name

​Kerendia®

​finerenone

272
  
10/1/2023Rx.01.261CommercialOyenusi, Oluwadamilola

​The normal eye creates a clear image by bending (refracting) light in order to focus it onto the retina. Refractive errors occur when a component of the eye's optical system fails to focus the optical image. Presbyopia ("aging sight") is a non-refractive error that also affects visual acuity. Presbyopia occurs when the lens loses its normal accommodating power and can no longer focus on objects viewed at arm's length or closer. Approximately 128 million adults in the United States are living with presbyopia. Presbyopia has traditionally been treated with corrective lenses or surgery.

Pilocarpine (Vuity™) is indicated for the treatment of presbyopia in adults.

Pilocarpine hydrochloride is a cholinergic muscarinic agonist which activates muscarinic receptors located at smooth muscles such as the iris sphincter muscle and ciliary muscle. Vuity contracts the iris sphincter muscle, constricting the pupil to improve near and intermediate visual acuity while maintaining some pupillary response to light. Vuity also contracts the ciliary muscle and may shift the eye to a more myopic state.

​The intent of this policy is to communicate the medical necessity criteria for Pilocarpine (Vuity™) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Pilocarpine (Vuity™) is approved when ALL of the following are met:

  1. Diagnosis of presbyopia; and
  2. Prescribed by or in consultation with ONE of the following:
    1. Ophthalmologist; or
    2. Optometrist; and
  3. Member is unable to use corrective lenses (e.g., eyeglasses or contact lenses)

Initial authorization duration: 1 month 

REAUTHORIZATION CRITERIA: Pilocarpine (Vuity™) is re-approved when BOTH of the following are met:
  1. Documentation of positive clinical response to therapy (e.g., improvement in near vision in low light conditions without loss of distance vision); and
  2. Prescribed by or in consultation with one of the following:
    1. Ophthalmologist; or
    2. Optometrist

Reauthorization duration: 6 months 

​N/A

Mian, SI. Visual impairment in adults: Refractive disorders and presbyopia. UpToDate. September 2022. Available at: https://www.uptodate.com/contents/visual-impairment-in-adults-refractive-disorders-and-presbyopia?search=presbyopia&source=search_result&selectedTitle=1~17&usage_type=default&display_rank=1. Accessed June 22, 2023.

Vuity (pilocarpine hydrochloride) [prescribing information]. North Chicago, IL: AbbVie Inc; March 2023. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=8d806897-8a2a-4518-8c68-0ec3b778de50 . Accessed June 22, 2023.​


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​Rx.01.33 Off Label Use

Brand NameGeneric Name
​Vuity™​Pilocarpine
273
  
10/1/2023Rx.01.263CommercialOyenusi, Oluwadamilola

Vernal keratoconjunctivitis (VKC) is an allergic inflammation of conjunctiva that is bilateral and usually seasonally recurrent. There are three types of vernal conjunctivitis: palpebral (papillae primarily involving upper tarsal conjunctiva), limbal (papillae located at limbus), and mixed (components of both palpebral and limbal types). Histopathologic exam of affected conjunctiva shows small lymphoid follicles composed of increased mast cells, eosinophils, and lymphocytes, mononuclear cells and macrophages, CD4 T lymphocytes and B lymphocytes, fibroblasts, and newly secreted collagen (extracellular matrix components). As disease progresses, cellular infiltration and new collagen deposition form giant papillae (squamous epithelial hyperplasia and dense fibrous tissue containing inflammatory cells). Inflammation of limbal palisades and tarsal conjunctiva produces nodules, due to firm attachments of conjunctiva.

Cyclosporine is a calcineurin inhibitor immunosuppressant agent when administered systemically. Following ocular administration, cyclosporine is thought to act by blocking the release of pro-inflammatory cytokines such as IL-2. The exact mechanism of action in the treatment of VKC is not known.

Verkazia® ophthalmic emulsion is a calcineurin inhibitor immunosuppressant indicated for the treatment of vernal keratoconjunctivitis in children and adults.


​The intent of this policy is to communicate the medical necessity criteria for Cyclosporine (Verkazia®) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA Cyclosporine (Verkazia®) is approved when ALL of the following are met:

  1. Diagnosis of moderate to severe vernal keratoconjunctivitis confirmed by the presence of clinical signs and symptoms (e.g., itching, photophobia, giant papillae at the upper tarsal conjunctiva or at the limbus, thick mucus discharge, conjunctival hyperaemia); and
  2. Member is 4 years of age or older; and
  3. Inadequate response or inability to tolerate one of the following:
    1. Topical ophthalmic “dual-acting” mast cell stabilizer and antihistamine (e.g., olopatadine, azelastine); or
    2. Topical ophthalmic mast cell stabilizers (e.g., cromolyn); and
  4. Inadequate response or inability to tolerate short term use (up to 2 to 3 weeks), of topical ophthalmic corticosteroids (e.g., dexamethasone, prednisolone, fluoromethalone); and
  5. Prescribed by or in consultation with one of the following:
    1. Ophthalmologist or
    2. Optometrist

Initial authorization duration: 6 months

REAUTHORIZATION CRITERIA Cyclosporine (Verkazia®) is re-approved when there is documentation of positive clinical response to therapy as evidenced by an improvement in clinical signs and symptoms (e.g., itching, photophobia, papillary hypertrophy, mucus discharge, conjunctival hyperaemia).

Reauthorization duration: 2 years



​N/A

DynaMed. Vernal Keratoconjunctivitis. EBSCO Information Services. https://www.dynamed.com/condition/vernal-keratoconjunctivitis. Accessed June 23, 2023.

 

Verkazia® (cyclosporine) [package insert]. Emeryville, CA: Santen Incorporated; June 2022. Available from: https://www.accessdata.fda.gov/drugsatfda_docs/label/2021/214965s000lbl.pdf. Accessed June 23, 2023.​




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Rx.01.33 Off Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit



Brand NameGeneric Name
Verkazia®Cyclosporine


274
  
10/1/2023Rx.01.265CommercialOyenusi, Oluwadamilola

​Hypertrophic cardiomyopathy (HCM) is a common inherited cardiovascular disease characterized by hypertrophy of a nondilated left ventricle in the absence of any other cardiac or systemic disease (such as hypertension) that could account for observed hypertrophy, microvascular dysfunction and myocardial fibrosis. Histopathological features include myofiber disarray and myocardial fibrosis resulting from microvascular ischemia and cell death. HCM is caused largely by mutations in genes encoding thick and thin contractile myofilament proteins of the cardiac sarcomere. Phenotypically, HCM can be obstructive (70% of patients), with presence of left ventricular outflow tract obstruction, or nonobstructive (30% of patients). Complications include syncope, heart failure, and sudden death.


Mavacamten is an allosteric and reversible inhibitor selective for cardiac myosin. Mavacamten modulates the number of myosin heads that can enter “on actin” (power-generating) states, thus reducing the probability of force-producing (systolic) and residual (diastolic) cross-bridge formation. Excess myosin actin cross-bridge formation and dysregulation of the super-relaxed state are mechanistic hallmarks of HCM. Mavacamten shifts the overall myosin population towards an energy-sparing, recruitable, super-relaxed state. In HCM patients, myosin inhibition with mavacamten reduces dynamic left ventricular outflow tract (LVOT) obstruction and improves cardiac filling pressures.

Mavacamten is indicated for treatment of adults with symptomatic New York Heart Association (NYHA) class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve functional capacity and symptoms.



​The intent of this policy is to communicate the medical necessity criteria for Mavacamten (Camzyos™) as provided under the member's prescription drug benefit.

​INITIAL CRITERIA: Mavacamten (Camzyos™) is approved when ALL of the following are met:


  1. Submission of medical records (e.g., chart notes, lab values) confirming a diagnosis of symptomatic New York Heart Association (NYHA) class II-III obstructive hypertrophic cardiomyopathy (HCM); and 
  2. Members is 18 years of age or older; and 
  3. Member’s baseline left ventricular ejection fraction (LVEF) is greater than or equal to 55%; and 
  4. Member has Valsalva left ventricular outflow tract (LVOT) peak gradient greater than or equal to 50 mmHg at rest or with provocation; and
  5. Inadequate response or inability to tolerate BOTH of the following at a maximally tolerated dose:
    1. Non-vasodilatingOne beta blocker (e.g., bisoprolol, propranolol); orand
    2. Calcium channel blocker (e.g., verapamil, diltiazem); and
  6. Prescribed by or in consultation with a cardiologist

Initial authorization duration: 6 months


REAUTHORIZATION CRITERIA: Mavacamten (Camzyos™) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response to therapy (e.g., improved symptom relief); and
  2. Member's left ventricular ejection fraction (LVEF) is greater than or equal to 50%; and
  3. Prescribed by or in consultation with a cardiologist.
Reauthorization duration: 12 months​


​Risk of Heart Failure

  • CAMZYOS can cause heart failure due to systolic dysfunction.
  • Echocardiogram assessments of left ventricular ejection fraction (LVEF) required before and during CAMZYOS use.
  • Initiation in patients with LVEF <55% not recommended. Interrupt if LVEF <50% or if worsening clinical status.
  • Certain CYP450 inhibitors and inducers are contraindicated in patients taking CAMZYOS because of an increased risk of heart failure.

CAMZYOS is available only through a restricted program called the CAMZYOS REMS Program.


DynaMed. Hypertrophic Cardiomyopathy. EBSCO Information Services. https://www.dynamed.com/condition/hypertrophic-cardiomyopathy. Accessed June 26, 2023.

 

Camzyos™ (mavacamten) [prescribing information]. Brisbane, CA: MyoKardia, Inc.; June 2023. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2022/214998s000lbl.pdf. Accessed June 26, 2023.


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​Rx.01.33 Off Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit




Brand NameGeneric Name
CamzyosTMMavacamten


275
  
10/1/2023Rx.01.266CommercialOyenusi, Oluwadamilola

Hemolytic anemia is a disease that results in the premature death (hemolysis) of red blood cells (RBC) at a faster rate than the body can produce. Hemolytic anemia can be categorized as intrinsic and extrinsic. Intrinsic hemolytic anemia is genetically inherited and results in abnormalities in the cell membrane and overall blood cell production. Extrinsic hemolytic anemia is acquired as a secondary effect resulting from certain immunologic diseases, infections and medications. The glycolytic pathway is a metabolic pathway that breaks down glucose into pyruvate via the Pyruvate kinase (PK) enzyme, which leads to the production of ATP and NADH. A deficiency in the pyruvate kinase enzyme in homozygous patients causes a defect in the glycolytic pathway that is known to cause hemolytic anemia. The exact mechanism of hemolysis is unknown.

Mitapivat is a Pyruvate Kinase activator that targets defective PK enzymes in RBC’s. 

Mitapivat (Pyrukynd®) is indicated for the treatment hemolytic anemia in adults with a PK enzyme deficiency.



​The intent of this policy is to communicate the medical necessity criteria for Mitapivat (Pyrukynd®) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Mitapivat (Pyrukynd®) is approved when ALL of the following are met: 

  1. Diagnosis of hemolytic anemia confirmed by the presence of chronic hemolysis (e.g., increased indirect bilirubin, elevated lactated dehydrogenase [LDH], decreased haptoglobin, increased reticulocyte count); and
  2. Diagnosis of pyruvate kinase deficiency confirmed by molecular testing of ALL of the following mutations on the PLKR gene:
    1. Presence of at least 2 variant alleles in the pyruvate kinase liver and red blood cell (PKLR) gene, of which at least 1 was a missense variant; and
    2. Member is not homozygous for the c.1436G>A (p.R479H) variant; and 
    3. Member does not have 2 non-missense variants (without the presence of another missense variant) in the PKLR gene; and 
  3. Hemoglobin is less than or equal to 10g/dL; and 
  4. Member has symptomatic anemia or is transfusion dependent; and 
  5. Other causes of hemolytic anemias (e.g., infections, toxins, drugs) were ruled out; and 
  6. Member is 18 years of age and older; and 
  7. Prescribed by or in consultation with a hematologist 

Initial authorization duration: 6 months 

REAUTHORIZATION CRITERIA: Mitapivat (Pyrukynd®) re-approved when ALL of the following are met: 
  1. Documentation of positive clinical response to therapy (e.g., hemoglobin greater than or equal to 1.5g/dL from baseline sustained on 2 or more follow ups (weeks 16, 20 and 24) during the fixed dose period without transfusions; reduction in transfusions of greater than or equal to 33% in the number of red blood cell units transfused during the fixed dose period compared with the member’s historical transfusion burden; improvement in markers of hemolysis from baseline (e.g., bilirubin, lactated dehydrogenase [LDH], haptoglobin, reticulocyte count)); and 
  2. Prescribed by or in consultation with a hematologist 

Reauthorization duration: 12 months 




​N/A

Pyrukynd® (mitapivat) [prescribing information]. Agios Pharmaceuticals, Inc.; February 2022. https://www.pyrukynd.com/hcp/. Accessed June 22, 2023.

 

Braunstein EM. Glycolytic Pathway Defects. Merck & Co., Inc., Rahway, NJ.; June 2022. https://www.merckmanuals.com/professional/hematology-and-oncology/anemias-caused-by-hemolysis/glycolytic-pathway-defects. Accessed June 22, 2023.




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​Rx.01.33 Off Label Use​

Brand NameGeneric Name
Pyrukynd®Mitapivat


278
  
10/1/2023Rx.01.277CommercialOyenusi, Oluwadamilola

Proteinuria, also called albuminuria, is elevated protein in the urine. It is not a disease in and of itself but a symptom of certain conditions affecting the kidneys. Typically, too much protein in the urine means that the kidneys’ filters — the glomeruli — are not working properly and are allowing too much protein to escape in the urine. IgA nephropathy is an autoimmune disease resulting from dysregulation of mucosal-type IgA immune responses.

Sparsentan is a single molecule with antagonism of the endothelin type A receptor (ETAR) and the angiotensin II type 1 receptor (AT1R). Sparsentan has high affinity for both the ETAR (Ki= 12.8 nM) and the AT1R (Ki=0.36 nM), and greater than 500-fold selectivity for these receptors over the endothelin type B and angiotensin II subtype 2 receptors. Endothelin-1 and angiotensin II are thought to contribute to the pathogenesis of IgAN via the ETA R and AT1R, respectively.

Filspari™ is indicated to reduce proteinuria in adults with primary immunoglobulin A nephropathy (IgAN) at risk of rapid disease progression, generally a urine protein-to-creatinine ratio (UPCR) ≥1.5 g/g.



​The intent of this policy is to communicate the medical necessity criteria for Sparsentan (Filspari™) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Sparsentan (Filspari) is approved when all of the following are met:

  1. Diagnosis of primary immunoglobulin A nephropathy (IgAN) as confirmed by a kidney biopsy; and
  2. Documentation is provided that member is at risk of rapid disease progression [e.g., generally a urine protein-to-creatinine ratio (UPCR) greater than or equal to 1.5 g/g, or by other criteria such as clinical risk scoring using the International IgAN Prediction tool]; and
  3. Medication will be used to reduce proteinuria; and
  4. Member has an estimated glomerular filtration rate (eGFR) of greater than or equal to 30 mL/min/1.73m2; and
  5. Member had an inadequate response or inability to tolerate a minimum 90-day trial of a maximally tolerated dose of one of the following:
    1. Angiotensin-receptor blockers (ARB) (e.g., losartan, valsartan); or
    2. Angiotensin-converting enzyme (ACE) inhibitor (e.g., benazepril, lisinopril); and
  6. Medication will not be used in combination with any of the following:
    1. Angiotensin receptor blockers
    2. Endothelin receptor antagonists (ERAs) (e.g., ambrisentan, bosentan, Opsumit)
    3. Aliskiren; and
  7. Member is 18 years or age or older; and
  8. Prescribed by or in consultation with a nephrologist

 

Initial authorization duration: 1 year

 

CONTINUATION CRITERIA: Sparsentan (Filspari) is approved when BOTH of the following are met:

  1. Documentation of positive clinical response to therapy from baseline as demonstrated by a decrease in urine protein-to-creatinine ratio (UPCR); and
  2. Prescribed by or in consultation with a nephrologist

 

Reauthorization duration: 1 year


WARNING: HEPATOTOXICITY and EMBRYO-FETAL TOXICITY

Because of the risks of hepatotoxicity and birth defects, FILSPARI is available only through a restricted program called the FILSPARI REMS. Under the FILSPARI REMS, prescribers, patients, and pharmacies must enroll in the program [see Warnings and Precautions.

Hepatotoxicity

Some Endothelin Receptor Antagonists (ERAs) have caused elevations of aminotransferases, hepatotoxicity, and liver failure. In clinical studies, elevations in aminotransferases (ALT or AST) of at least 3-times the Upper Limit of Normal (ULN) have been observed in up to 2.5% of FILSPARI-treated patients, including cases confirmed with rechallenge. Measure transaminases and bilirubin before initiating treatment and monthly for the first 12 months, and then every 3 months during treatment. Interrupt treatment and closely monitor patients who develop aminotransferase elevations more than 3x. FILSPARI should generally be avoided in patients with elevated aminotransferases (>3x ULN) at baseline because monitoring for hepatotoxicity may be more difficult and these patients may be at increased risk for serious hepatotoxicity.

Embryo-Fetal Toxicity

FILSPARI can cause major birth defects if used by pregnant patients based on animal data, Use in Specific Populations. Therefore, pregnancy testing is required before the initiation of treatment, during treatment, and one month after discontinuation of treatment with FILSPARI. Patients who can become pregnant must use effective contraception before the initiation of treatment, during treatment, and for one month after discontinuation of treatment with FILSPARI.


Filspari™ (sparsentan) [package insert]. San Diego, CA: Travere Therapeutics, Inc. February 2023. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2023/216403s000lbl.pdf. Accessed July 31, 2023

Cattran DC. IgA nephropathy: Treatment and prognosis. UpToDate. July 2023. Available at: https://www.uptodate.com. Accessed July 31, 2023.

“Proteinuria." JHM, 19 Nov. 2019, www.hopkinsmedicine.org/health/conditions-and-diseases/proteinuria.


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​Rx.01.33 Off Label Use

Brand NameGeneric Name
Filspari™Sparsentan


279
  
10/1/2023Rx.01.278CommercialOyenusi, Oluwadamilola

Friedreich’s ataxia is a progressive neurodegenerative disorder that causes progressive damage to the central nervous system. This is a rare disease that is caused by a mutation in the Frataxin (FXN) gene. A mutation in this gene causes mitochondrial dysfunction which causes iron accumulation and generation of a reactive oxygen species. In doing so, oxidative stress builds up in the body and causes cell damage. Symptoms of Friedreich’s ataxia include: trouble walking, tiredness, loss of reflexes, slow/slurred speech, hearing loss, loss of sensation, and shortness of breath.

Omaveloxolone belongs to a therapeutic drug class known as Nrf2 Pathway Activators which are known to reduce oxidative stress. In patients that have ataxia, Nrf2 levels are lower than normal. By taking omaveloxolone the Nrf2 translocates to the nucleus which in turn lowers oxidative stress in patients. Oxidative stress plays a role in several conditions in the body including neurodegenerative diseases. Thus, by lowering oxidative stress, symptoms associated with Freidrich’s ataxia can be reduced.

Skyclarys® is indicated for Freidrich’s ataxia in adults and adolescents aged 16 and older.  



​The intent of this policy is to communicate the medical necessity criteria for Omaveloxolone (Skyclarys®) as provided under the member's prescription drug benefit. 

INITIAL CRITERIA: Omaveloxolone (Skyclarys) is approved when all of the following are met:

 

  1. Diagnosis of Friedreich ataxia as confirmed via genetic testing demonstrating mutation in FXN gene; and
  2. Member has a Modified Friedreich's Ataxia Rating Scale (mFARS) score of greater than or equal to 20 and less than or equal to 80; and
  3. Member has B-type natriuretic peptide value less than or equal to 200 pg/ml; and
  4. Member is 16 years of age or older; and
  5. Prescribed by or in consultation with one of the following:
    1. Neurologist; or
    2. Neurogeneticist; or
    3. Physiatrist (Physical Medicine and Rehabilitation Specialist)

Initial authorization duration: 1 year

CONTINUATION CRITERIA: Omaveloxolone (Skyclarys) is re-approved when BOTH of the following are met:

 

  1. Documentation of positive clinical response to therapy as evidenced by one of the following:
    1. A decrease in the rate of progression of Modified FA rating scale (mFARS) score; or
    2. An increase in peak work (in Watts/kg) during exercise testing from baseline; and
  2. Prescribed by or in consultation with one of the following:
    1. Neurologist; or
    2. Neurogeneticist; or
    3. Physiatrist (Physical Medicine and Rehabilitation Specialist)
​Reauthorization duration: 1 year




​N/A

Skyclarys (omaveloxolone) [package insert]. Plano, TX; Reata Pharmaceuticals. February 2023. Available from: https://www.skyclarys.com/docs/skyclarys_us_prescribing_information/. Accessed August 03, 2023.

Opal P. Friedreich ataxia. UpToDate. March 2023. Available at: https://www.uptodate.com. Accessed August 03, 2023.

Pizzino G, Irrera N, Cucinotta M, et al. Oxidative stress: Harms and benefits for human health. Oxidative medicine and cellular longevity. 2017. Accessed August 3, 2023. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5551541/.


16/8/20236/8/202410/1/2023 1:29 AMNo presence informationsrv_ppsgw_P

​Rx.01.33 Off Label Use

Brand NameGeneric Name
Skyclarys®Omaveloxolone


281
  
11/1/2023Rx.01.218CommercialOyenusi, Oluwadamilola
The Continuous Glucose Monitors (CGMs) are indicated for use in individuals with type 1 diabetes who require insulin and need to be monitored for unexplained glycemic fluctuations and hypoglycemic unawareness. Hypoglycemic unawareness is the inability of an individual to notice and recognize symptoms of hypoglycemia while they are experiencing them.
CGMs devices are considered an adjunct to be used with a traditional blood glucose monitor. These adjunctive devices allow individuals to track glucose levels and detect episodes of high and low blood sugar in real-time on an ongoing basis. The device consists of a disposable subcutaneous sensor, an external transmitter, and an external receiver (monitor), which can be a stand-alone device or built into an insulin pump. Sensors are worn as indicated by the device manufacturer in accordance with US Food and Drug Administration (FDA) labeling and are replaced on an ongoing basis.
Depending on the device sensor longevity capability, a CGMs sensor measures interstitial glucose levels for 6 to 14 days. Use of this device requires the glucose sensor to be implanted subcutaneously, usually in the abdomen or in an area above the buttocks. The transmitter is connected to the sensor by an adhesive patch, and glucose signals are sent from the sensor to the receiver every five minutes. Interstitial glucose values appear on the receiver or mobile device, where they can be read and reviewed by the individual. This data may be stored and downloaded for analysis. CGMs devices also allow for customization of threshold settings, such as alarms, to detect high and low glucose levels.
The FDA has approved several CGMs devices to assist in analyzing glycemic trends in the ongoing evaluation and management of individuals with diabetes. The FDA requires that alterations to an individual's insulin dosage or therapy are made only after confirmation of blood glucose levels with a traditional blood glucose monitor.

The intent of this policy is to communicate the medical necessity criteria for Continuous Glucose Monitors (Dexcom®, Medtronic®) as provided under the member’s prescription drug benefit.

Continuous glucose monitor (CGM) products (receivers, transmitters and sensors) are approved when ALL of the following are met:

    1. Diagnosis of diabetes; and
    2. Member is adherent to current diabetes treatment plan and participates in ongoing diabetes education and support; and
    3. One of the following:
      1. Member is treated with insulin; or
      2. Member is non-insulin treated and experiences significant hypoglycemia (e.g., recurrent, unexplained, severe [generally blood glucose levels <50 mg/dL] hypoglycemia or hypoglycemic unawareness).; and
    4. For Freestyle Libre only, documentation of the member’s inability to use Dexcom®

      Initial authorization duration: 2 years.

      REAUTHORIZATION CRITERIA: Continuous glucose monitor (CGM) products (receivers, transmitters and sensors) are approved when ALL of the following are met:
  1. Documentation that of a positive clinical response as evidenced by ONE of the following:
      1. Improvement in glycemic control (e.g., lower and/or maintain A1C levels); or
      2. Reduction or improvement in hypoglycemic events
  2. For Freestyle Libre only, documentation of the member's inability to use Dexcom®

    Reauthorization duration: 2 years

None

Dexcom® CGM. Available at: https://www.dexcom.com/continuous-glucose-monitoring. Accessed October 02, 2023.

Freestyle Libre® CGM. Available at: https://www.freestyle.abbott/us-en/home.html. Accessed October 02, 2023.

Medtronic® CGM. Available at: https://www.medtronicdiabetes.com/treatments/continuous-glucose-monitoring. Accessed October 02, 2023.

Weinstock, R. Management of blood glucose in adults with type 1 diabetes mellitus. UpToDate website. Updated February 2021 www.uptodate.com. Accessed October 02, 2023.
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Rx.01.33 Off-Label Use 

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit

Dexcom® line of products

Medtronic® line of products

Freestyle® line of products

Dexcom® G5 Receivers

Dexcom® G6 Receivers

Dexcom® G7 Receiver

Dexcom® G5 Sensors

Dexcom® G6 Sensors

Dexcom® G7 Sensor

Dexcom® G5 Transmitter Kit

Dexcom® G6 Transmitter Kit

Guardian™ Connect Transmitter

Guardian™ Sensor

Enlite® Sensor

Eversense® Sensor/Holder

 

 

 

Freestyle Libre® 14 Day Reader

Freestyle Libre® 14 Day Sensor

Freestyle Libre® 2 Reader

Freestyle Libre® 2 Sensor

Freestyle Libre® 3 Sensor

 


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BEDAQUILINE (SIRTURO®)

Bedaquiline (Sirturo®) is indicated as part of combination therapy in adults and pediatric patients (5 years and older and weighing at least 15 kg) with pulmonary multi-drug resistant tuberculosis (MDR-TB). Reserve Sirturo® for use when an effective treatment regimen cannot otherwise be provided

Bedaquiline (Sirturo®) is a diarylquinoline antimycobacterial drug that inhibits mycobacterial ATP synthase, an enzyme that is essential for the generation of energy in Mycobacterium tuberculosis.

ISAVUCONAZONIUM (CRESEMBA®)

Isavuconazonium (Cresemba®) is indicated for the treatment of invasive aspergillosis and invasive mucormycosis.

Isavuconazonium (Cresemba®) is the prodrug of isavuconazole, an azole antifungal.  Isavuconazole inhibits the synthesis of ergosterol, a key component of the fungal cell membrane, through the inhibition of cytochrome P-450 dependent enzyme lanosterol 14-alpha-demethylase. This enzyme is responsible for the conversion of lanosterol to ergosterol. An accumulation of methylated sterol precursors and a depletion of ergosterol within the fungal cell membrane weakens the membrane structure and function.

TEDIZOLID (SIVEXTRO®)

Tedizolid (Sivextro®) is indicated for the treatment of adult and pediatric patients 12 years of age and older for the treatment of acute bacterial skin and skin structure infections (ABSSSI) caused by designated susceptible bacteria.

Tedizolid (Sivextro®) binds to the 50S bacterial ribosomal subunit. This prevents the formation of a functional 70S initiation complex that is essential for the bacterial translation process and subsequently inhibits protein synthesis. Tedizolid is bacteriostatic against enterococci, staphylococci, and streptococci.


 

PRETOMANID

Pretomanid is indicated for the treatment of adults with pulmonary extensively drug resistant (XDR) or treatment-intolerant or nonresponsive multidrug-resistant (MDR) tuberculosis (TB) as part of a combination regimen with bedaquiline and linezolid. Pretomanid is indicated for use in a limited and specific population of patients

 

Pretomanid is a nitroimidazooxazine antimycobacterial drug. Pretomanid kills actively replicating M. tuberculosis by inhibiting mycolic acid biosynthesis, thereby blocking cell wall production. Under anaerobic conditions, against non-replicating bacteria, Pretomanid acts as a respiratory poison following nitric oxide release.

OMADACYCLINE (NUZYRA®)

NUZYRA is a tetracycline class antibacterial indicated for the treatment of adult patients with the following infections caused by susceptible microorganisms:

  • Community-acquired bacterial pneumonia (CABP)
  • Acute bacterial skin and skin structure infections (ABSSSI)



The intent of this policy is to communicate the medical necessity criteria for bedaquiline (Sirturo®), isavuconazonium (Cresemba®), tedizolid (Sivextro®), omadacycline (Nuzyra®) and pretomanid as provided under the member's prescription drug benefit. 

BEDAQUILINE (SIRTURO®)

Bedaquiline (Sirturo®) is approved when ALL of the following are met:

  1. Diagnosis of pulmonary multi-drug resistant tuberculosis (MDR-TB), for which an effective treatment regimen cannot otherwise be established; and
  2. Member is 5 years of age or older and weighing at least 15kg; and
  3. Prescribed by or in consultation with  an infection disease specialist or pulmonologists; and
  4. Bedaquiline is used as combination therapy as defined by ONE of the following:
    1. With at least 3 other drugs to which the member's MDR-TB isolate has been shown to be susceptible in vitro; or
    2. With at least 4 other drugs to which the patient's MDR-TB isolate is likely to be susceptible

Authorization duration: 24 weeks


ISAVUCONAZONIUM (CRESEMBA®)

Isavuconazonium (Cresemba®) is approved when ALL of the following are met:

  1. Member is 18 years of age or older; and
  2. Prescribed by or in consultation with ​an infectious diseases specialist; and 
  3. Diagnosis of ONE of the following:
    1. Treatment of invasive aspergillosis and inadequate response or inability to tolerate voriconazole; or
    2. Treatment of invasive mucormycosis

Authorization duration: 12 months


TEDIZOLID (SIVEXTRO®)

Tedizolid (Sivextro®) is approved when ALL of the following are met:

  1. Member is 12 years of age or older; and
  2. Prescribed by or in consultation with ​an infectious diseases specialist; and ​
  3. Documentation of use for the treatment of acute bacterial skin and skin structure infections (ABSSSI) caused by susceptible isolates of Gram-positive microorganisms and ONE of the following:
    1. Inadequate response or inability to tolerate ALL antibiotics to which the organism is susceptible; or
    2. Tedizolid is the only antibiotic to which the organism is susceptible; or
    3. There is a contraindication to using an alternative antibiotic to which the organism is susceptible to (e.g., drug-drug interaction concern with the alternative antibiotic and member’s existing drug regimen)

Authorization duration: 4 weeks​

PRETOMANID

Pretomanid is approved when ALL of the following are met:

  1. Diagnosis of pulmonary extensively drug resistant (XDR), treatment-intolerant or nonresponsive multidrug-resistant (MDR) tuberculosis (TB); and
  2. Medication will be used as part of combination regiment with bedaquiline (Sirturo®) and linezolid; and
  3. Member is 18 years of age or older; and
  4. Prescribed by or in consultation with an infectious disease specialist

​Authorization duration: 26 weeks

OMADACYCLINE (NUZYRA®)

Omadacycline (Nuzyra®) is approved when ALL of the following are met:

  1. Member is 18 years of age or older; and
  2. Prescribed by or in consultation with an infectious disease specialist; and
  3. One of the following:
    1. Documentation of use for the treatment of community-acquired bacterial pneumonia; or
    2. Documentation of use for the treatment of acute bacterial skin and skin structure infections (ABSSSI); and
  4.  ONE of the following:
    1. Inadequate response or inability to tolerate ALL antibiotics to which the organism is susceptible; or
    2. Omadacycline is the only antibiotic to which the organism is susceptible; or
    3. There is a contraindication to using an alternative antibiotic to which the organism is susceptible to (e.g., drug-drug interaction concern with the alternative antibiotic and member's existing drug regimen)

 

Authorization duration: 4 weeks


BEDAQUILINE (SIRTURO®)

An increased risk of death was seen in the bedaquiline group (11.4%) compared with the placebo group (2.5%) in 1 placebo-controlled trial. Only use SIRTURO in patients 5 years of age and older when an effective treatment regimen cannot otherwise be provided..

QT prolongation can occur with bedaquiline. Use with drugs that prolong the QT interval may cause addictive QT prolongation. Monitor ECGs. Discontinue SIRTURO if significant ventricular arrhythmia or QTcF interval >500 ms develops.


 


 

Pretomanid [prescribing information]. India: Mylan, Laboratories Limited; April 2019. Available from: https://www.tballiance.org/sites/default/files/assets/Pretomanid_Full-Prescribing-Information.pdf. Accessed December 5, 2023.

Cresemba® (isavuconazonium) [package insert]. Northbrook, IL. Astellas Pharma US. November 2022.  Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=8f7f73b8-586a-4df0-935f-fecd4696c16c&type=display. Accessed December 5, 2023.

Sirturo® (bedaquiline) [package insert]. Janssen Therapeutics; Titusville, NJ. September 2021. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=1534c9ae-4948-4cf4-9f66-222a99db6d0e&type=display.  Accessed December 5, 2023.

Sivextro® (tidizolid) [package insert]. White House Station, NJ: Merc and Co, Inc. March 2023. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=75672079-589f-451a-bdbf-eaebcfcc80a9&type=display. Accessed December 5, 2023.

Nuzyra® (omadacycline) [package insert]. Boston, MA: Paratek Pharmaceutical, Inc. May 2021. Available at: https://www.nuzyra.com/nuzyra-pi.pdf. Accessed December 5, 2023



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Rx.01.33 Off Label Use

Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit​


Brand NameGeneric Name
Cresemba®isavuconazonium
Sirturo®bedaquiline
Sivextro®tedizolid
 Pretomanid
Nuzyra®omadacycline


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1/1/2024Rx.01.89CommercialOyenusi, Oluwadamilola

Hyponatremia is defined as a relative excess of water in relation to sodium.  Treatment approaches depend on the duration, severity, and cause of hyponatremia.

Tolvaptan (Samsca®) is a selective vasopressin V2 receptor antagonist. Tolvaptan antagonizes the effect of vasopressin and causes an increase in urine water excretion that results in an increase in free water clearance, a decrease in urine osmolality and a resulting increase in serum sodium concentrations.

Tolvaptan (Samsca®) is indicated for the treatment of clinically significant hypervolemic and euvolemic hyponatremia (serum sodium < 125meq/L or less marked hyponatremia that is symptomatic and has resisted correction with fluid restriction) including patients with heart failure, cirrhosis, and syndrome of inappropriate antidiuretic hormone.

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by the growth of numerous fluid filled cysts in the kidneys which progressively decreases kidney function and lead to permanent kidney damage. It is the most common inherited disorder of the kidneys. Symptoms usually develop between the ages of 30 and 40, but they can begin earlier, even in childhood.

Tolvaptan (Jynarque®) is a selective vasopressin (V2) receptor antagonist.  Patients with autosomal dominant polycystic kidney disease have elevated levels of vasopressin. Tolvaptan works by preventing vasopressin from binding to its receptor which then decreases the rate of cell proliferation and fluid secretion into the cystic lumen, ultimately inhibiting the growth of the fluid filled cysts in the kidneys and slows the worsening of kidney function.

Tolvaptan (Jynarque®) is indicated to slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease (ADPKD).

The intent of this policy is to communicate the medical necessity criteria for tolvaptan (Samsca®, Jynarque®) as provided under the member's prescription drug benefit.


Hyponatremia

INITIAL CRITERIA Tolvaptan (Samsca®) is approved when ALL of the following are met:

  1. Member is 18 years of age or older; and
  2. Clinically significant hypervolemic or euvolemic hyponatremia  and ONE of the following:
    1. Serum sodium less than 125meq/L; or
    2. Serum sodium 125-134meq/L with symptoms (ie nausea, vomiting, headache, lethargy, confusion, etc);

      and
  3. Inadequate response or inability to tolerate therapies to control hyponatremia (ie fluid restriction, diuretics, demeclocycline,  etc); and
  4. Prescribed by or in consultation with a cardiologist, nephrologist, or endocrinologist; and
  5. Brand Samsca only, inadequate response or inability to tolerate generic tolvaptan​

Initial authorization duration: 30 days

REAUTHORIZATION CRITERIA Tolvaptan (Samsca®) is re-approved when ALL of the following are met:

  1. Documentation of positive clinical response; and
  2. Liver function is monitored and there are no signs or symptoms of liver injury; and
  3. Prescriber is aware of the risk of liver injury with use beyond 30 days
Reauthorization duration: 12 months


Autosomal dominant polycystic kidney disease

INITIAL CRITERIA Tolvaptan (Jynarque®) is approved when ALL of the following are met:

  1. Member is 18 years of age or older; and
  2. Diagnosis of autosomal dominant polycystic kidney disease with risk of rapidly progressing kidney disease; and
  3. Baseline serum transaminases and bilirubin obtained prior to initiation of therapy; and
  4. Prescribed by or in consultation with nephrologist or kidney transplant specialist

Initial authorization duration: 3 months
 
REAUTHORIZATION CRITERIA Tolvaptan (Jynarque®) is re-approved when ALL of the following are met:

  1. ONE of the following
    1. Decline in kidney function has slowed; or
    2. Kidney pain has improved; and
  2. Serum transaminase less than 3 times the upper limit of normal; AND
  3. Bilirubin less than 2 times upper limit of normal

Reauthorization duration: 12 months


Tolvaptan (Samsca®)
INITIATE AND RE-INITIATE IN A HOSPITAL AND MONITOR SERUM SODIUM
Tolvaptan (Samsca®) should be initiated and re-initiated in patients only in a hospital where serum sodium can be monitored closely.
Too rapid correction of hyponatremia (e.g., >12 mEq/L/24 hours) can cause osmotic demyelination resulting in dysarthria, mutism, dysphagia, lethargy, affective changes, spastic quadriparesis, seizures, coma and death. In susceptible patients, including those with severe malnutrition, alcoholism or advanced liver disease, slower rates of correction may be advisable.


Tolvaptan (Jynarque®) can cause serious and potentially fatal liver injury. Acute liver failure requiring liver transplantation has been reported.
Measure ALT, AST and bilirubin before initiating treatment, at 2 weeks and 4 weeks after initiation, then monthly for the first 18 months and every 3 months or symptoms indicative of hepatic injury can mitigate, but not eliminate, the risk of serious hepatotoxicity.
Because of the risks of serious liver injury, Jynarque® is available only through a restricted distribution program under a Risk Evaluation and Mitigation Strategy (REMS) called the JYNARQUE REMS Program

Jynarque® (tolvaptan) [package insert] Tokyo, Japan. Otsuka Pharmaceutical Co, Ltd. -October 2020. Available at: https://www.otsuka-us.com/media/static/JYNARQUE-PI.pdf. Accessed - November 17, 2023..

 

Samsca® (tolvaptan) [package insert]. Tokyo, Japan. Otsuka Pharmaceutical Co, Ltd. -April 2021. Available at:https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=5526617c-c7b9-4556-886d-729bbabbc566&type=display.  Accessed November 17, 2023.

 

Sterns RH. Overview of the treatment of hyponatremia in adults. UpToDate. June 2021. Available at: https://www.uptodate.com/contents/overview-of-the-treatment-of-hyponatremia-in-adults?source=search_result&search=hyponatremia&selectedTitle=2~150#H25. Accessed November 17, 2023.

 

Tolvaptan (Kidney Disease): MedlinePlus Drug Information." MedlinePlus, U.S. National Library of Medicine, 15 July 2018, medlineplus.gov/druginfo/meds/a609033.html.​



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Off-Label Use Policy Rx.01.33
 

Brand NameGeneric Name
Samsca®Tolvaptan
Jynarque®Tolvaptan
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1/1/2024Rx.01.45CommercialOyenusi, Oluwadamilola

Intranasal steroids are used for a variety of disorders including nasal polyps, non-allergic rhinitis, perennial allergic rhinitis and seasonal allergic rhinitis.  

Intranasal steroids provide anti-inflammatory effects on the nasal mucosa. Their exact mechanism is unknown but corticosteroids have been shown to have a wide range of actions on multiple cell types (e.g., mast cells, eosinophils, neutrophils, macrophages, lymphocytes) and mediators (e.g., histamine, eicosanoids, leukotrienes, cytokines) involved in inflammation.  

The intent of this policy is to communicate the medical necessity criteria for beclomethasone (Beconase AQ®), fluticasone propionate (Xhance™), beclomethasone (Qnasl®/ Qnasl®Children's), azelastine hydrochloride and fluticasone propionate (Dymista®), and olopatadine hydrochloride and mometasone furoate (Ryaltris®) as provided under the member's prescription drug benefit. 

 

Nasal polyps

INITIAL CRITERIA: Beconase AQ®, or fluticasone propionate (Xhance™)  is approved when BOTH of the following are met:

  1. Diagnosis of nasal polyps; and
  2. Inadequate response or inability to tolerate generic fluticasone propionate (trial for at least 90 days within the previous 365 days) 

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Beconase AQ®, or fluticasone propionate (Xhance®) is reapproved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

Allergic Rhinitis

INITIAL CRITERIA: Beconase AQ®, Children's Qnasl®*, azelastine hydrochloride and fluticasone propionate (Dymista), Qnasl®, or  Ryaltris® is approved when BOTH of the following are met:

  1. Diagnosis of allergic rhinitis; and
  2. Inadequate response or inability to tolerate BOTH of the following generic nasal sprays:
    1. Flunisolide; and
    2. Fluticasone propionate

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Beconase AQ®, Children's Qnasl®*, azelastine hydrochloride and fluticasone propionate (Dymista®), Qnasl®, or Ryaltris® is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

*Fluticasone propionate is approved for children under 6 years of age

None


Beconase AQ (beclomethasone dipropionate) [package insert]. Research Triangle Park, NC. GlaxoSmithKline. April 2019. https://www.gsksource.com/pharma/content/dam/GlaxoSmithKline/US/en/Prescribing_Information/Beconase_AQ/pdf/BECONASE-PI-PIL.PDF Accessed on December 5, 2023. 

Dymista (azelastine hydrochloride and fluticasone propionate) [package insert]. Somerset, NJ. Meda Pharmaceutical. September 2018. Available at: https://www.accessdata.fda.gov/drugsatfda_docs/label/2015/202236s008lbl.pdf. Accessed on December 5, 2023.  

Qnasl® (beclomethasone) [package insert]. Northridge, CA. Teva Respiratory, LLC. March 2018.  Available at: http://qnasl.com/Content/pdf/pi.pdf Accessed on December 5, 2023. 

Ryaltris® (olopatadine hydrochloride and mometasone furoate monohydrate nasal spray) [package insert]. Columbus, OH: Hikma Specialty USA Inc. January 2022. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=382eac74-a7ce-4f42-ac0a-b253308f335f. Accessed December 5, 2023.

Seidman MD et al. Clinical practice guideline: allergic rhinitis executive summary. Otolaryngol Head Neck Surg. 2015 Feb; 152(2): 197-206.

Wallace DV et al. Pharmacologic treatment of seasonal allergic rhinitis: synopsis of guidance from the 2017 Joint Task Force on practice parameters. Ann Intern med. 2017 Dec 19; 167(12):867-881.

Peters AT et al. Diagnosis and management of rhinosinusitis: a practice parameter update. Ann Allergy Asthma Immunol. 2014 Oct; 113(4): 347-85.


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Off-Label Use Rx.01.33
Brand NameGeneric Name
Beconase AQ®beclomethasone
Dymista®azelactine hydrochloride and fluticasone propionate
Qnasl®beclomethasone dipropionate
Qnasl®Childrensbeclomethasone dipropionate
Xhance™Fluticasone propionate
Ryaltris®olopatadine hydrochloride and mometasone furoate monohydrate





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The Company utilizes a tiered cost-sharing structure for medications covered under the pharmacy benefit.  Members should refer to their benefit booklet for more information.
 

Formulary Tier Exceptions

The following tier exceptions requests will be considered:

 

Select Drug Formulary

  1. Non-preferred drug to be covered at the:
    1. Preferred brand tier if the product is a brand medication; or
    2. Generic tier if the product is generic medication
  2. All other tiers are restricted to the benefit design and thus are not eligible for a tier exception

Value Formulary

  1. Non-formulary medication to be covered at the highest level of cost share.  These exceptions are not eligible for tier reduction.
  2. Non-preferred drug to be covered at the:
    1. Preferred brand tier if the product is a brand medication; or
    2. Generic tier if the product is generic medication
  3. All other tiers are restricted to the benefit design and thus are not eligible for a tier exception.

CHIP

  1. Non-preferred drug medication to be covered at the:
    1. Preferred brand tier if the product is a brand; or
    2. Generic tier if the product is generic
  2. Brand medication to be covered at the generic benefit level

The following tiers are defined by the benefit and are not eligible for a tier exception:

  1. Specialty tier
  2. Preferred brand tier
  3. Generic tier

Cost Share Exceptions for Preventive Care Services and Women's Preventive Services

The services listed in this policy are considered preventive care services when the criteria in this policy are met, when they are identified as preventive services in the Company's benefit contracts and when they are mandated by state or federal law.  This policy supports the preventative care services listed in the US Preventive Services Task Force (USPSTF) as A or B Recommendations and the Women's Preventive Services (WPS) provision of Patient Protection and Affordable Care Act (PPACA). These products are available without cost-sharing with a prescription when provided by a participating retail or mail-order pharmacy.

Based on the USPSTF recommendation the following products are available at zero-dollar cost-share.  All medications refer to generic, single ingredient products unless otherwise noted.

 
 
CategoryRecommendationMedication
​ASPIRIN

Pregnant Women Who Are At High Risk for Preeclampsia :

The USPSTF recommends the use of low-dose aspirin (81 mg/d) as preventive medication after 12 weeks of gestation in pregnant females who are at high risk for preeclampsia.

aspirin 81mg or less
TOBACCO CESSATION MEDICATIONTobacco cessation medication is covered as a preventive service for all adults who use tobacco products.Chantix ®, bupropion, Nicotrol®, generic nicotine gums and patches
FOLIC ACIDThe USPSTF recommends that all females planning or capable of pregnancy take a daily supplement containing 0.4 to 0.8 mg (400 to 800 µg) of folic acid.folic acid 400 mcg to 800 mcg (including generic prenatal vitamins with the above listed folic acid dose)
FLUORIDEIn accordance with the preventive exam age schedule set forth by American Academy of Pediatrics (AAP)/Bright Futures, oral fluoride, up to 0.5mg, is covered as a preventive service for children ages 6 months to 16 years whose water supply is deficient in fluoride.fluoride up to 0.5mg for children 6 months to 16 years of age
BREAST CANCER CHEMO-PREVENTIONThe USPSTF recommends that clinicians engage in shared, informed decision making with individuals who are at increased risk for breast cancer about medications to reduce their risk. For asymptomatic females 35 years or older without a prior diagnosis of breast cancer, ductal carcinoma in situ, or lobular carcinoma in situ, who are at increased risk for breast cancer and at low risk for adverse medication effects from breast cancer chemoprevention, clinicians should offer to prescribe risk-reducing medications, such as tamoxifen.tamoxifen 20mg
CONTRACEPTIVESThe contraceptive methods for women currently identified by the FDA include: (1) sterilization surgery for women; (2) surgical sterilization implant for women; (3) implantable rod; (4) IUD copper; (5) IUD with progestin; (6) shot/injection; (7) oral contraceptives (combined pill); (8) oral contraceptives (progestin only); (9) oral contraceptives extended/continuous use; (10) patch; (11) vaginal contraceptive ring; (12) diaphragm; (13) sponge; (14) cervical cap; (15) condom; (16) spermicide; (17) emergency contraception; and (18) emergency contraception (Ella)injection/shot, oral contraceptives, etonogestrel-ethinyl estradiol vaginal ring, diaphragms, sponge, cervical cap, condom, spermicide, emergency contraceptive, Ella®, Phexxi®.  [Note: IUDs and implantable products are covered under the medical benefit. See referenced policy]
BOWEL PREP FOR COLONOSCOPYThe USPSTF recommends screening for colorectal cancer starting at age 45 years and continuing until age 75 years.PEG 3350- electrolyte, Gavilyte-C, Gavilyte-G, Gavilyte-N, Trilyte with flavor packets, Gavilyte-H with bisacodyl, PEG-prep,PEG 3350 powder for solution
STATIN PREVENTIVE MEDICATION

The USPSTF recommends that clinicians prescribe a statin for the primary prevention of CVD for adults aged 40 to 75 years who have 1 or more CVD risk factors (i.e. dyslipidemia, diabetes, hypertension, or smoking) and an estimated 10-year risk of a cardiovascular event of 10% or greater.

lovastatin 10, 20, 40 mg
HIV PrEPPreexposure prophylaxis (PrEP) with effective antiretroviral therapy for persons who are at high risk of HIV acquisition

emtricitabine/tenofovir disoproxil

fumarate 200mg-300mg,

tenofovir 300mg



Certain medications have additional indications that are not addressed by the preventative care measure, such as raloxifene, or have generic alternatives, such as branded contraceptives.  Thus, the plan employs medical management to administer the requirements. The policy below outlines the process by which an exception can be obtained for medications that may apply to the USPSTF recommendation or the WPS provision of the PPACA but are not coded as $0 at the point-of-sale.

 

*This policy does not apply to the Premium Formulary
The intent of this policy is to communicate the medical necessity criteria for formulary exception requests as provided under the member's prescription drug benefit.
Tier Exceptions:

A non-preferred drug will be covered at the preferred tier for brand medications as listed below when there is documentation of inadequate response or inability to tolerate at least three preferred or generic tier alternatives in the same pharmacological class*. Reasonably equivalent therapeutic class alternatives will be considered when there are limited pharmacological class alternatives available.

  1. Brand medication to preferred brand tier or
  2. Generic medication to generic tier

Non-formulary Exceptions:

A medication that is non-formulary, will be covered at the appropriate level of cost share when the following is met:

There is documentation of inadequate response or inability to tolerate at least three formulary alternatives in the same pharmacological class*. Reasonably equivalent therapeutic class alternatives will be considered when there are limited pharmacological class alternatives available. Safety edits (age and quantity limits) will apply to non-formulary requests



CHIP (IN Focus ID 10093453)

A brand medication may be covered at the generic benefit level when there is documentation of an inadequate response or inability to tolerate at least three generic alternatives in the same pharmacological class*. Reasonably equivalent therapeutic class alternatives will be considered when there are limited pharmacological class alternatives available.


Compounded Products:

A non-preferred compounded product may be covered at the preferred (formulary) tier when there is an inadequate response or inability to tolerate/use all other formulary alternatives.

Note: Compounded products are specially made products to meet the needs of an individual member and are not considered generics and thus not eligible for an exception to the generic tier.

$0 Cost-Share Override

An exception to allow no-cost share is approved when:

  1. The drug is described as either a preventative medication identified by the US Preventive Services Task Force (USPSTF) or Women's Preventive Services provision of the Patient Protection and Affordable Care Act (PPACA); and if applicable
  2. For branded products, ALL of the following:
    1. Inadequate response or inability to tolerate the generic equivalent, if available
    2. Inadequate response or inability to tolerate a generic alternative
    3. The prescriber has provided documentation indicating the requested product is medically necessary

*If there are fewer than three alternatives, all alternatives in the pharmacological class must be considered.

Premium Formulary: This policy does not apply to the premium formulary.

Authorization duration: 2 years

Truvada® (emtricitabine and tenofovir disoproxil fumarate), tenofovir

  1. Severe acute exacerbations of hepatitis B virus (HBV) have been reported in HBV-infected patients who have discontinued Truvada® and tenofovir. Hepatic function should be monitored closely in HBV-infected patients who discontinue Truvada® and tenofovir. If appropriate, initiation of anti-hepatitis B therapy may be warranted.

Truvada® (emtricitabine and tenofovir disoproxil fumarate)

  1. Truvada® used for HIV-1 PrEP must only be prescribed to individuals confirmed to be HIV-negative immediately prior to initiating and at least every 3 months during use. Drug resistant HIV-1 variants have been identified with the use of Truvada® for HIV-1 PrEP following undetected acute HIV-1 infection. Do not initiate Truvada® for HIV-1 PrEP if signs or symptoms of acute HIV infection are present unless negative infection status is confirmed.
 
 
 

 

Bright Futures Period Schedule. Available at:  https://downloads.aap.org/AAP/PDF/periodicity_schedule.pdf. Accessed November 21, 2023.

USPSTF A and B Recommendations by Date. US Preventive Services Task Force Web Site. https://www.uspreventiveservicestaskforce.org/uspstf/recommendation-topics/uspstf-a-and-b-recommendations?SORT=D&DESC=1.  Updated September 2022. Accessed November 21, 2023.

Truvada® (emtricitabine and tenofovir disoproxil fumarate) [prescribing information]. Foster City, CA: Gilead Sciences, Inc.; June 2020. Available from: https://www.gilead.com/~/media/Files/pdfs/medicines/hiv/truvada/truvada_pi.pdf. Accessed November 21, 2023.

Viread® (tenofovir disoproxil fumarate) [prescribing information]. Foster City, CA: Gilead Sciences, Inc.; April 2019. Available from: https://www.gilead.com/~/media/Files/pdfs/medicines/liver-disease/viread/viread_pi.pdf. Accessed November 21, 2023.



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Rx.01.33 Off Label Use

Rx.01.2 Applicable Age Edits

Rx.01.134 Compounded Product

Rx.01.76 Quantity Level Limits for Pharmaceutical Covered under the Pharmacy Benefit

Rx.01.197 Opioid Policy

00.06.02 Preventive Care Services Medical Policy ​


 


N/A

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Huntington's disease (HD) is an inherited, progressive, neurodegenerative disease with no cure or disease modifying therapies currently available.  The disease is characterized by choreiform movements, psychiatric problems, and dementia. Therapy focuses on management of symptoms and supportive care. There are approximately 30,000 Americans with symptomatic HD.  Symptoms usually appear between the ages of 30 and 50 and progressively worsen.​

Chorea is a hyperkinetic movement disorder that may manifest in association with Huntington's Disease.  Chorea is characterized by involuntary brief, random, and irregular contractions.  Anti-chorea medications such as tetrabenazine, deutetrabenazine and valbenazine may be useful for controlling chorea in the setting of Huntington's disease, especially milder forms of chorea.   Other treatment options include atypical and typical neuroleptics, amantadine, and riluzole.

Tardive dyskinesia is a movement disorder that is characterized by random movement of various facial muscles, including the tongue and jaw. In more severe cases, it may also involve movements of the arms, legs, fingers, toes, trunk or hips. A common risk factor for developing tardive dyskinesia is long-term treatment with antipsychotic medications.  It has particularly been associated with first-generation antipsychotic treatment, but there are reports of patients receiving second-generation antipsychotics developing tardive dyskinesia.

Tourette syndrome (TS) is a neurological disorder manifested by motor and phonic tics with onset during childhood. Tics are the clinical hallmark of TS. Tics are sudden, brief, intermittent movements (motor tics) or utterances (phonic tics). Tics have been considered involuntary, but tics can temporarily be voluntarily suppressed. 


Mechanism of Action

Tetrabenazine,deutetrabenazine, and valbenazine reversibly inhibit the human vesicular monoamine transporter type 2 (VMAT2) resulting in decreased uptake of monoamines into synaptic vesicles and depletion of monamine stores.  

Tetrabenazine (Xenazine®) and deutetrabenazine (Austedo® [XR]) are indicated for the treatment of chorea associated with Huntington's disease.

Deutetrabenazine (Austedo® [XR]) and valbenazine (Ingrezza®) are indicated for the treatment of adults with tardive dyskinesia. ​



 

The intent of this policy is to communicate the medical necessity criteria for tetrabenazine (Xenazine®), deutetrabenazine (Austedo® [XR]) and valbenazine (Ingrezza®) as provided under the member's prescription drug benefit. 


Chorea associated with Huntington's disease

INITIAL CRITERIA: Tetrabenazine (Xenazine®) or deutetrabenazine (Austedo® [XR]) is approved when ALL of the following are met:

  1. Used for the treatment of chorea associated with Huntington's disease; and
  2. Member is 18 years of age or older; and
  3. Prescribed by or in consultation with a neurologist; and
  4. For brand Xenazine® only, inadequate response or inability to tolerate generic tetrabenazine

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Tetrabenazine (Xenazine®) or deutetrabenazine (Austedo® [XR]) is re-approved when there is documentation of positive clinical response to therapy.

 
Reauthorization duration: 2 years


Tardive Dyskinesia

INITIAL CRITERIA: Valbenazine (Ingrezza®) or deutetrabenazine (Austedo® [XR]) is approved when ALL of the following are met:

  1. Diagnosis of moderate to severe tardive dyskinesia; and
  2. Member is 18 years of age or older; and
  3. One of the following: 
    1. Persistent symptoms of tardive dyskinesia despite a trial of dose reduction, tapering, or discontinuation of the offending medication; or
    2. Member is not a candidate for a trial of dose reduction, tapering, or discontinuation of the offending medication; and
  4. Prescribed by or in consultation with one of the following:
    1. Neurologist; or
    2. Psychiatrist

Initial Authorization duration: 3 months

 

REAUTHORIZATION CRITERIA: Valbenazine (Ingrezza®) or deutetrabenazine (Austedo® [XR]) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years



Tourette's syndrome

INITIAL CRITERIA: Tetrabenazine (Xenazine®) is approved when ALL of the following are met:

  1. Diagnosis of Tourette's Syndrome; and
  2. Member has tics associated with Tourette's syndrome; and
  3. Inadequate response or inability to tolerate haloperidol or risperidone; and
  4. Prescribed by or on consultation with a neurologist or a psychiatrist

 

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Tetrabenazine (Xenazine®) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years


 

TETRABENAZINE (Xenazine®) and DEUTETRABENAZINE (Austedo® [XR]):
DEPRESSION AND SUICIDALITY 
Tetrabenazine and deutetrabenazine can increase the risk of depression and suicidal thoughts and behavior (suicidality) in patients with Huntington's disease.  Balance risks of depression and suicidality with the clinical need for control of chorea when considering the use of tetrabenazine and deutetrabenazine.  Close observation of patients for the emergence or worsening of depression, suicidality, or unusual changes in behavior should accompany therapy.  Inform patients, caregivers, and families of the risk of depression and suicidality, and instruct them to report behaviors of concern promptly to the treating physician.

Exercise caution in treating patients with a history of depression or prior suicide attempts or ideation, which are increased in frequency in Huntington disease.  Tetrabenazine and deutetrabenazine are contraindicated in patients who are actively suicidal, and in patients with untreated or inadequately treated depression​



 


Austedo® [XR] (deutetrabenazine) [ package insert]. North Wales, PA. Teva Pharmaceuticals. February 2023. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=7ea3c60a-45c7-44cc-afc2-d87fa53993c0&type=display.  Accessed November 20, 2023.

 

Ingrezza® (valbenazine) [package insert]. San Diego, CA: Neurocrine Biosciences. August 2022. Available at: https://ingrezza.com/HCP/PI.  Accessed November 20, 2023.

 

Jankovic, J. Tourette Syndrome: Pathogenesis, clinical features, and diagnosis. UpToDate. December 2022. Available at: https://www.uptodate.com/contents/tourette-syndrome-pathogenesis-clinical-features-and-diagnosis?search=tourette%20syndrome&source=search_result&selectedTitle=1~23&usage_type=default&display_rank=1. Assessed November 20, 2023.

 

Suchowersky O. Overview of chorea. UpToDate. September 2022. Available at: https://www.uptodate.com/contents/overview-of-chorea?source=machineLearning&search=chorea&selectedTitle=1~102&sectionRank=2&anchor=H29672870#H29672870.  Accessed November 20, 2023.

 

Suchowersky O. Huntington's disease: management. UpToDate. February 2023. Available at: https://www.uptodate.com/contents/huntington-disease-management?source=search_result&search=huntingtons&selectedTitle=2~50.  Accessed November 20, 2023.

 

Xenazine® (tetrabenazine) [package insert]. Deerfield, IL. Lundbeck.  November 2019. Available at:  https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ac768bab-8afa-4446-bc7f-caeeffec0cda&type=display.  Accessed November 20, 2023.

 

Tarditive Dyskinesia. National Alliance on Mental Illness Web Site. https://www.nami.org/About-Mental-Illness/Treatments/Mental-Health-Medications/Tardive-Dyskinesia. Accessed November 20, 2023.

 

What is Huntington's Disease? Huntington's Disease Society of America Web Site. https://hdsa.org/what-is-hd/overview-of-huntingtons-disease/. Accessed November 20, 2023.

 

Wimalasena K. Vesicular monoamine transporters: Structure-function, pharmacology, and medicinal chemistry. Med Res Rev. 2010;31(4):483-519. doi:10.1002/med.20187 ​




 

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Off-Label Use Rx.01.33

 

Brand Name Generic Name
Xenazine® Tetrabenazine
​Austedo® [XR]
​Deutetrabenazine
​Ingrezza®
Valbenazine 

 

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Hyperammonemia is a urea cycle disorder due to a deficiency of an enzyme in the pathway that can cause life-threatening metabolic decompensations in infancy. Survivors frequently have severe neurologic injury. Frequent vomiting and poor appetite with food refusal and protein aversion are common in patients with UCD. In newborns, central hyperventilation leading to respiratory alkalosis is an early sign of hyperammonemia. Infants become symptomatic after feeding in which initial signs include somnolence, inability to maintain normal body temperature, poor feeding followed by vomiting lethargy and coma.


 

N-acetylglutamate synthetase (NAGS) deficiency is a rare, autosomal, recessive genetic disorder in which lack of NAGS enzyme leads to hyperammonemia (excess ammonia).  NAGS deficiency is one of several urea cycle disorders.


Carglumic acid (Carbaglu®) is a synthetic structural analogue of N-acetylglutamate (NAG), which is produced from glutamate and acetyl-CoA in a reaction catalyzed by N-acetylglutamate synthase (NAGS), a mitochondrial liver enzyme. NAG acts as an essential allosteric activator of carbamoyl phosphate synthetase 1 (CPS 1) in liver mitochondria. CPS 1  catalyzes the first reaction  of the urea cycle, . NAG is the product of NAGS, a mitochondrial liver enzyme. Carglumic acid acts as a CPS 1 activator   in NAGS deficiency patients , thereby facilitating ammonia detoxification and urea production by removing the block in the urea cycle

Carglumic Acid (Carbaglu®) is indicated for adjunctive therapy in the treatment of acute hyperammonemia due to NAGS deficiency, propionic acidemia (PA) or methylmalonic acidemia (MMA), and maintenance therapy of chronic hyperammonemia due to the deficiency of the hepatic enzyme NAGS.

The intent of this policy is to communicate the medical necessity criteria for carglumic acid (Carbaglu®) as provided under the member's prescription drug benefit.

Acute Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency

APPROVAL CRITERIA: Carglumic Acid (Carbaglu®) is approved when all of the following are met:

  1. Diagnosis of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency; and 
  2. Medication will be used as adjunctive therapy to other ammonia lowering therapies (e.g., protein restriction, ammonia scavengers, dialysis) 
  3. Prescribed by or in consultation with a provider who specializes in the treatment of metabolic disorders

Authorization duration: 3 months

Acute Hyperammonemia due to Propionic Acidemia (PA) or Methylmalonic Acidemia (MMA)

APPROVAL CRITERIA: Carglumic Acid (Carbaglu®) is approved when all of the following:

  1. Diagnosis of acute hyperammonemia due to propionic acidemia (PA) or methylmalonic acidemia (MMA); and
  2. Medication will be used as adjunctive therapy to other ammonia lowering therapies (e.g., intravenous glucose, insulin, protein restriction, dialysis); and
  3. Patient's plasma ammonia level is greater than or equal to 50 micromol/L; and
  4. Medication will be used for a maximum duration of 7 days; and
  5. Prescribed by or in consultation with a provider who specializes in the treatment of metabolic disorders


​Authorization duration: 3 months

Chronic Hyperammonemia due to N-acetylglutamate Synthase (NAGS) Deficiency

INITIAL CRITERIA: Carglumic Acid (Carbaglu®) is approved when all of the following:

  1. Diagnosis of chronic hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency; and
  2. NAGS deficiency has been confirmed by genetic/mutational analysis; and
  3. Medication will be used as maintenance therapy; and
  4. Prescribed by or in consultation with a provider who specializes in the treatment of metabolic disorders

Initial authorization duration: 2 years

​REAUTHORIZATION CRITERIA: Carglumic acid (Carbaglu®) is re-approved when there is documentation of positive clinical response to therapy (e.g., plasma ammonia level within the normal range).


Reauthorization duration: 2 years​




N/A

Carbaglu® [package insert]. Lebanon NJ. Recordati Rare Diseases, Inc.  September 2021. Available at: https://www.carbaglu.com/wp-content/uploads/2020/01/carbaglu-prescribing-information.pdf. Accessed NOVEMBER 21, 2023.

N-acetylglutamate synthetase deficiency. National organization for rare disorders. Available at: http://rarediseases.org/rare-diseases/n-acetylglutamate-synthetase-deficiency/. Accessed NOVEMBER 21, 2023.

Lee B. Urea cycle disorders: clinical features and diagnosis. UpToDate website. June 2021. Available at http://www.uptodate.com/. Accessed Accessed NOVEMBER 21, 2023.


 


 

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Off-Labe Use Rx01.33
 

Brand Name Generic Name
Carbaglu® Carglumic Acid
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Male hypogonadism is characterized by low testosterone levels.  Primary hypogonadism is characterized by low testosterone levels in the setting of elevated luteinizing hormone (LH) and follicle-stimulating hormone (FSH) concentrations.  Examples of primary hypogonadism include, but are not limited to, Klinefelter syndrome, castration (physical or chemical), and trauma.  Secondary hypogonadism, also referred to as hypogonadotropic hypogonadism, is characterized by low testosterone levels in the setting of normal or low LH and FSH.  In this type of hypogonadism, dysfunction of the hypothalamus or pituitary is the underlying etiology.  Examples of hypogonadotopic hypogonadism include, but are not limited to, idiopathic hypogonadotropic hypogonadism, Kallman syndrome, and pituitary tumors, surgery, or destruction.


Gender dysphoria, according to the World Professional Association for Transgender Health (WPATH), is defined as the discomfort arising from incongruence between an individual's gender identity and their external sexual anatomy. The standard of care for individuals affected by gender dysphoria include extensive counseling, hormonal therapy and surgery. Androgen hormone therapy is used to induce physical changes to match gender identify in transgender men (female-to-male, FTM). The goal of therapy is to maintain hormone levels in the normal physiological range for the targeted gender, to stop menses and induce virilization, including a male pattern of sexual and facial hair, change in voice, and male physical contours. Both topical and injectable testosterone products are effective for the management of gender dysphoria.

The active ingredient in all products listed is testosterone. Exogenous testosterone serves to replace testosterone in individuals who are deficient.  Testosterone therapy is indicated for replacement therapy in patients with low testosterone levels due to primary hypogonadism (congenital or acquired) or hypogonadotropic hypogonadism (congenital or acquired). Testosterone enanthate intramuscular injection and methyltestosterone can also be used to stimulate puberty in carefully selected males with clearly delayed puberty. Methyltestosterone is also indicated for the treatment of metastasis from malignant tumor of breast in women 1 to 5 years postmenopausal with inoperable metastatic skeletal disease.

The intent of this policy is to communicate the medical necessity criteria for Androgel®, Androderm®, Fortesta®, Jatenzo®, Tlando®, Natesto®,  Testim®, Vogelxo®, Xyosted™, Kyzatrex®, methyltestosterone (Methitest®), and generic testosterone products as provided under the member’s prescription drug benefit.

Primary or secondary hypogonadism

INITIAL CRITERIA: Androgel®, generic transdermal testosterone products, Androderm®, Fortesta®, Natesto®, Testim®, Vogelxo®, Xyosted™, testosterone undecanoate (Jatenzo®, Tlando®, Kyzatrex®), or methyltestosterone (Methitest®) is approved when ALL of the following are met:

    1. Diagnosis of primary or secondary hypogonadism; and
    2. Member is 18 years of age or older; and
    3. ONE of the following:
      1. Negative history of prostate and breast cancer; OR
      2. History of prostate cancer status post prostatectomy and documentation that the risk versus benefit has been assessed; and
    1. For Androgel®, Androderm®,  Fortesta®, Natesto®, Testim®, Vogelxo®, Xyosted™, testosterone undecanoate (Jatenzo®, Tlando®, Kyzatrex®), methyltestosterone(Methitest®) only, inadequate response or inability to tolerate generic transdermal testosterone ; and
    2. New users only, low (morning) testosterone level

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA Androgel®, generic transdermal testosterone products, Androderm®, Fortesta®, Natesto®, Testim®, Vogelxo®, Xyosted™, testosterone undecanoate (Jatenzo®, Tlando®, Kyzatrex®), methyltestosterone (Methitest®) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years​

Gender dysphoria

INITIAL CRITERIA Androgel®, generic transdermal testosterone products, Androderm®, Fortesta®, Natesto®, Testim®, Vogelxo®, Xyosted™, testosterone undecanoate (Jatenzo®, Tlando®, Kyzatrex®), or methyltestosterone (Methitest®) is approved for use as hormone therapy in children, adolescents, and adults with gender dysphoria when there is documentation of persistent, well-documented gender dysphoria diagnosed in accordance with criteria established in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5)

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA Androgel®, generic transdermal testosterone products, Androderm®, Fortesta®, Natesto®, Testim®, Vogelxo®, Xyosted™, testosterone undecanoate (Jatenzo®, Tlando®, Kyzatrex®), methyltestosterone (Methitest®) is re-approved when there is documentation of positive clinical response to therapy.

Reauthorization duration: 2 years
 


 

Transdermal testosterone (Androgel®, Fortesta®, Testim®, Vogelxo®)

Secondary exposure: Virilization has been reported in children who were secondarily exposed to transdermal testosterone. Ensure that children avoid contact with unwashed or unclothed application sites in men using transdermal testosterone.  Advise patients to strictly adhere to recommended instructions for use.

Testosterone enanthate (Xyosted™) and testosterone undecanoate capsule (Jatenzo®, Tlando®, Kyzatrex®)

Blood pressure increase:

  • Xyosted™, Kyzatrex® and Jatenzo® can cause blood pressure (BP) increases that can increase the risk of major adverse cardiovascular events (MACE), including non-fatal myocardial infarction, non-fatal stroke and cardiovascular death.
  • Before initiating Xyosted™, Kyzatrex® and Jatenzo®, consider the patient's baseline cardiovascular risk and ensure blood pressure is adequately controlled.
  • Periodically monitor for and treat new-onset hypertension or exacerbations of pre-existing hypertension and re-evaluate whether the benefits of Xyosted™, Kyzatrex® and Jatenzo®  outweigh its risks in patients who develop cardiovascular risk factors or cardiovascular disease on treatment.
  • Due to this risk, use Xyosted™, Kyzatrex® and Jatenzo®  only for the treatment of men with hypogonadal conditions associated with structural or genetic etiologies.

Androderm® (testosterone) [package insert]. Irvine, CA. Allergan USA, Inc. May 2020. Available from: https://www.allergan.com/assets/pdf/androderm_pi. Accessed NOVEMBER 20, 2023.

AndroGel® (testosterone) [package insert]. North Chicago, IL. AbbVie. May 2019. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=8677ba5b-8374-46cb-854c-403972e9ddf3&type=displayAccessed NOVEMBER 20, 2023.

Bhasin S, Cunningham GR, Hayes FJ, Matsumoto AM, Snyder PJ, Swerdloff RS, Montori VM; Task Force, Endocrine Society. Testosterone therapy in men with androgen deficiency syndromes: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2010 Jun;95(6):2536-59.

Fortesta® (testosterone) [package insert]. Malvern, PA. Endo Pharmaceuticals, Inc. June 2020. Available from: http://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=053a7300-0bce-11e0-9d16-0002a5d5c51b&type=display. Accessed NOVEMBER 20, 2023.

Gooren L . Hormone treatment of the adult transsexual patient. Horm Res. 2005;64(Suppl 2):31–36

Gooren LJG , Giltay EJ  . Review of studies of androgen treatment of female-to-male transsexuals: effects and risks of administration of androgens to females. J Sex Med . 2008;5(4):765–776.

Jatenzo® (testosterone undecanoate) capsules [prescribing information]. Northbrook, IL. Clarus Therapeutics, Inc. March 2019. Available from: https://www.jatenzo.com/assets/pdfs/jatenzo-pi.pdf. Accessed NOVEMBER 20, 2023.

Kaplan AL, Trinh QD, Sun M, Carter SC, Nguyen PL, Shih YC, Marks LS, Hu JC. Testosterone replacement therapy following the diagnosis of prostate cancer: outcomes and utilization trends. J Sex Med. 2014 Apr;11(4):1063-70.

Kaufman J, Graydon RJ. Androgen replacement after curative radical prostatectomy for prostate cancer in hypogonadal men. J Urol. 2004;172(3):920-922.

Kyzatrex® (testosterone undecanoate) [prescribing information]. Raleigh, NC: Marius Pharmaceuticals LLC. September 2022. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=7f7167a7-2a25-47e2-acf5-33f499fce971. Accessed NOVEMBER 20, 2023.

Matsumoto AM. Diagnosis and evaluation of male hypogonadism. Medscape CME. 2008. Available from: http://www.medscape.org/viewarticle/575491. Accessed NOVEMBER 20, 2023.

Meriggiola MC , Gava G  . Endocrine care of transpeople part I. A review of cross-sex hormonal treatments, outcomes and adverse effects in transmen. Clin Endocrinol (Oxf) . 2015;83(5):597–606.

Methitest® (methyltestosterone) [prescribing information]. Bridgewater, NJ: Amneal Pharmaceuticals LLC.; October 2018. Available from: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=77bb4ef4-c10e-4acc-8225-651d003f4561. Accessed NOVEMBER 20, 2023.

Meza J, Weaver K, Martin S. FPIN's clinical inquiries. Testosterone therapy and risk recurrence after treatment of prostate cancer. Am Fam Physician. 2013 Oct 15;88(8):Online. Available from: http://www.aafp.org/afp/2013/1015/od5.pdf

Moore E , Wisniewski A , Dobs A  . Endocrine treatment of transsexual people: a review of treatment regimens, outcomes, and adverse effects. J Clin Endocrinol Metab . 2003;88(8):3467–3473.

Natesto® (testosterone) [package insert]. Malvern, PA. Endo Pharmaceuticals, Inc. December 2017. Available from: http://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=b0343bcc-7320-4bf2-bcb3-d95b6f4ba5fe&type=display. Accessed NOVEMBER 20, 2023.

Pastuszak AW, Pearlman AM, Lai WS, Godoy G, Sathyamoorthy K, Liu JS, Miles BJ, Lipshults LI, Khera M. Testosterone replacement therapy in patients with prostate cancer after radical prostatectomy. J Urol. 2013 Aug;190(2):639-44. Accessed NOVEMBER 20, 2023.

Seftel AD, Mack RJ, Secrest AR, et, al. Restorative increases in serum testosterone levels are significantly correlated to improvements in sexual functioning. J Androl. 2004; 25(6):963-972.

Steidle C, Schwartz S, Jacoby K, et, al. AA2500 testosterone gel normalizes androgen levels in aging males with improvements in body composition and sexual function. J Clin Endocrinol Metab. 2003; 88(6):2673-2681.

Testim® (testosterone) [package insert]. Malvern, PA. Auxilium Pharmaceuticals. April 2018. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=9f2aae1f-898d-4955-be31-678e0cf85395&type=display. Accessed NOVEMBER 20, 2023.

Testosterone. Micromedex. Available from: http://www.micromedexsolutions.com. Accessed NOVEMBER 20, 2023.

Tlando® (testosterone undecanoate) [package insert]. Ewing, NJ. Antares Pharma Inc. March 2022. Available from: https://www.tlando.com/application/files/9416/5366/3764/TLANDO_PI__Medication_Guide__FINAL__032822.pdf#hcpisi. Accessed NOVEMBER 20, 2023.

Vogelxo® (testosterone) [package insert]. Maple Grove, MN.  Upsher-Smith Laboraories, Inc. April 2020. Available from: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=2dd150f6-cdfd-4d51-8888-12b288f26262&type=display. Accessed NOVEMBER 20, 2023.

Wang C, Nieschlag E, Swerdloff R, Behre HM, Hellstrom WJ, Gooren LJ, Kaufman JM, Legros JJ, Lunenfeld B, Morales A, Morley JE, Schulman C, Thompson IM, Weidner W, Wu FCW. Investigation, treatment and monitoring of late-onset hypogonadism in males. ISA, ISSAM, EAU, EAA and ASA recommendations. Eur J Endocrinol. 2008 Nov;159(5):507-514.

Xyosted™ (testosterone enanthate) injection [package insert]. Ewing, NJ. Antares Pharma, Inc. November 2019. Available at: https://www.xyosted.com/PI.pdf. Accessed NOVEMBER 20, 2023.

The World Professional Association for Transgender Health. Standards of Care for the Heath of Transsexual, Transgender, and Gender Nonconforming People. 7th version. 2019. Available at: https://www.wpath.org/publications/soc. Accessed NOVEMBER 20, 2023.




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Rx.01.33 Off-Label Use
 


Brand NameGeneric Name
Androgel®Testosterone
Androderm®Testosterone
Fortesta®Testosterone
Natesto®Testosterone
Striant®Testosterone
Testim®Testosterone
Vogelxo®Testosterone
Jatenzo®, Tlando®, Kyzatrex®Testosterone undecanoate
Testred®, Android®, Methitest®Methyltestosterone
Xyosted™Testosterone enanthate

295
  
1/1/2024Rx.01.87CommercialOyenusi, Oluwadamilola

Parathyroid hormone (PTH) and calcitriol are the two major hormones that regulate calcium and phosphate homeostasis.  PTH maintains serum ionized calcium concentrations in a narrow range by stimulating renal tubular calcium reabsorption and bone resorption.  Chronic exposure to high PTH results in bone resorption, however intermittent administration of recombinant human PTH stimulates bone formation to a greater extent than resorption, at least over the first 12 months of therapy.  While PTH is an effective treatment for osteoporosis, it is generally not a first line drug due to route of administration (subcutaneous), long-term safety concerns, and availability of other agents.

Teriparatide is recombinant human PTH. Abaloparatide is a human parathyroid hormone related peptide (PTHrP(1-34)).

 Teriparatide (Forteo®) is indicated:

  • For the treatment of postmenopausal women with osteoporosis who are at high risk for fracture. These include women with a history of osteoporotic fracture, or who have multiple risk factors for fracture, or who have failed or are intolerant of previous osteoporosis therapy, based upon physician assessment.
  • To increase bone mass in men with primary or hypogonadal osteoporosis who are at high risk for fracture. These include men with a history of osteoporotic fracture, or who have multiple risk factors for fracture, or who have failed or are intolerant to previous osteoporosis therapy, based upon physician assessment.
  • For treatment of osteoporosis associated with sustained systemic glucocorticoid therapy at high risk fracture.

 

Abaloparatide (Tymlos™) is indicated: 
  • For the treatment of postmenopausal women with osteoporosis at high risk of fracture or patients who have failed or are intolerant to other available osteoporosis therapy.
  • For the treatment to increase bone density in men with osteoporosis at high risk for fracture or patients who have failed or are intolerant to other available osteoporosis therapy.


The intent of this policy is to communicate the medical necessity criteria for abaloparatide (Tymlos™) and teriparatide (Forteo®) as provided under the member's prescription drug benefit. 


 

 

Primary or hypogonadal osteoporosis in men and Glucocorticoid-induced osteoporosis in men or women

INITIAL CRITERIA: Teriparatide (Forteo®) is approved when ALL of the following are met:

  1. The member is 18 years of age or older; and
  2. ONE of the following:
    1. Primary or hypogonadal osteoporosis in men; or
    2. Glucocorticoid-induced osteoporosis in men or women (daily dose greater than or equal to 5mg prednisone or equivalent for at least 3 months); and
  3. ONE of the following:
    1. Member is high risk for fracture defined by ONE of the following:
      1. History of osteoporotic fractures; or
      2. At least two risk factors for a fracture (e.g., endocrine disorders, gastrointestinal disorders, use of medications associated with low bone mass or bone loss such as corticosteroids); or
      3. Member has a T score of at least -2.5 standard deviations below the young adult mean (T-score ≤ -2.5); or
    2. Inadequate response or inability to tolerate ONE of the following osteoporosis therapies:
      1. Bisphosphonates; or
      2. Hormone replacement therapy; or
      3. Selective estrogen receptor modulators (SERMs); or
      4. Calcitonin salmon (Miacalcin); or
      5. Denosumab (Prolia); and
  4. For Forteo® only, inadequate response or inability to tolerate Teriparatide® manufactured by Alvogen

Initial Authorization duration: 12 months


REAUTHORIZATION CRITERIA: Teriparatide (Forteo®) is re-approved when BOTH of the following are met:

  1. Documentation of positive clinical response; and
  2. ONE of the following:
    1. Cumulative lifetime therapy does not exceed 2 years; or
    2. For Forteo® only, member remains at or has returned to having a high risk for fracture despite a total of 24 months of use for parathyroid hormones

Reauthorization duration: 12 months

​​

Postmenopausal osteoporosis

INITIAL CRITERIA: Abaloparatide (Tymlos™) or teriparatide (Forteo®) is approved when ALL of the following are met:

  1. The member is 18 years of age or older; and
  2. Diagnosis of postmenopausal osteoporosis; and
  3. ONE of the following:
    1. Member is high risk for fracture defined by ONE of the following:
      1. Member has a T score of at least -2.5 standard deviation below the young adult mean (T-score ≤ -2.5); or
      2. History of osteoporotic fractures; or
      3. At least two risk factors for a fracture (e.g., endocrine disorders, gastrointestinal disorders, use of medications associated with low bone mass or bone loss such as corticosteroids); or
    2. Inadequate response or inability to tolerate ONE of the following osteoporosis therapies:
      1. Bisphosphonates; or
      2. Hormone replacement therapy; or
      3. Selective-estrogen receptor modulators (SERMs); or
      4. Calcitonin-salmon (Miacalcin®); or
      5. Denosumab (Prolia®); and
  4. For Forteo® only, inadequate response or inability to tolerate Teriparatide® manufactured by Alvogen​

Initial Authorization duration: 12 months  

REAUTHORIZATION CRITERIA: Abaloparatide (Tymlos™) or teriparatide (Forteo®) is re-approved when BOTH of the following are met:

  1. Documentation of positive clinical response; and
  2. ONE of the following:
    1. Cumulative lifetime therapy does not exceed 2 years; or
    2. For Forteo® only, member remains at or has returned to having a high risk for fracture despite a total of 24 months of use for parathyroid hormones 

Reauthorization duration: 12 months

Increase bone density in men with osteoporosis at high risk for fracture

INITIAL CRITERIA: Abaloparatide (Tymlos®) is approved when ALL of the following are met:

  1. Diagnosis of primary or hypogonadal osteoporosis; and
  2. The member is 18 years of age or older; and
  3. Both of the following:
    1. Bone mineral density (BMD) T-score of -2.5 or lower in the lumbar spine, femoral neck, total hip, or radius (one-third radius site); and
    2. One of the following:
      1. History of low-trauma fracture of the hip, spine, proximal humerus, pelvis, or distal forearm; or
      2. Inadequate response or inability to tolerate at least one osteoporosis treatment (e.g., alendronate, zoledronic acid, Prolia [denosumab])

​​​Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Abaloparatide (Tymlos®) is re-approved when BOTH of the following are met:
  1. Documentation of positive clinical response; and
  2. Cumulative lifetime therapy does not exceed 2 years

​Reauthorization duration: 12 months​

* Coverage duration of Teriparatide and Tymlos™ is limited to 730-day supply max per lifetime. All other treatment durations are considered Experimental/Investigational.

**Osteoporosis defined as T score of the individual's bone mineral density (BMD) is at least -2.5 standard deviations below the young adult mean OR history of osteoporotic fracture (i.e. hip, spine, etc.)


 


N/A

Forteo® (teriparatide) [package insert]. Indianapolis, IN. Lilly USA, LLC. April 2021. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=aae667c5-381f-4f92-93df-2ed6158d07b0&type=display. Accessed October 02, 2023.

Prolia® (denosumab) [package insert]. Thousand Oaks, CA. Amgen Inc. January 2023. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=49e5afe9-a0c7-40c4-af9f-f287a80c5c88&type=display. Accessed October 02, 2023.

Rosen CJ. Parathyroid hormone/parathyroid hormone-related protein analog for osteoporosis. UpToDate. January 2023. Available at: https://www.uptodate.com/contents/parathyroid-hormone-therapy-for-osteoporosis?source=search_result&search=teriparatide&selectedTitle=4~150. Accessed October 02, 2023.

Tymlos™ (abaloparatide) [package insert]. Waltham, MA: Radius Health, Inc. December 2022. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=712143d9-e21e-4013-bb3b-3426a21060a8&type=display. Accessed October 02, 2023.


 


 

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Rx.01.33 Off-Label Use
 

Brand Name

Generic Name

Tymlos™

abaloparatide

Forteo®

teriparatide


296
  
1/1/2024Rx.01.22CommercialOyenusi, Oluwadamilola

Individuals, who are transfusion-dependent, receive excess iron with each transfusion.  In non-transfusion-dependent thalassemia (NTDT), elevated iron levels are related to suppression of hepcidin levels, increased intestinal iron absorption, and increased release of recycled iron from the reticuloendothelial system.  The excess iron accumulates in various tissues, including cardiac, liver, pulmonary, and endocrine glands, due to lack of an active mechanism to excrete iron.  The goal of iron chelation therapy in iron overload is to reduce iron levels, prevent complications, and reduce morbidity.

Deferasirox (Exjade®/ Jadenu®) is indicated for the treatment of transfusional hemosiderosis (chronic iron overload due to blood transfusions) in individuals who are 2 years of age or older and for the treatment of chronic iron overload in patients 10 years of age and older with NTDT syndromes and with a liver iron concentration (LIC) of at least 5 mg Fe per gram of dry weight (Fe/ g dw) and a serum ferritin greater than 300 mcg/L.

Deferiprone (Ferriprox®) is an iron chelator indicated for the treatment of patients with transfusional iron overload due to thalassemia syndromes when current chelation therapy is inadequate.

Deferasirox (Exjade®/Jadenu®) is an orally active chelator that is selective for iron (as Fe3+). It is a tridentate ligand that binds iron with high affinity in a 2:1 ratio. Although deferasirox has very low affinity for zinc and copper, there are variable decreases in the serum concentration of these trace metals after the administration of deferasirox. The clinical significance of these decreases is uncertain.


Deferiprone (Ferriprox®) is a chelating agent with an affinity for ferric ion (iron III). Deferiprone binds with ferric ions to form neutral 3:1 (deferiprone:iron) complexes that are stable over a wide range of pH values.

The intent of this policy is to communicate the medical necessity criteria for deferasirox (Exjade®/ Jadenu®) and deferiprone (Ferriprox®) as provided under the member's prescription drug benefit.​

Chronic iron overload in blood transfusions dependent anemia

INITIAL CRITERIA: Deferasirox (Exjade®/Jadenu®) is approved when ALL of the following are met:

  1. Diagnosis of chronic iron overload due to blood transfusions; and
  2. Member is 2 years of age or older; and
  3. Serum ferritin levels are consistently greater than 1000 mcg/L (as demonstrated with at least two lab values within two months prior to treatment)
  4. For Brand Exjade and Brand Jadenu only, inadequate response or inability to tolerate generic deferasirox

Initial authorization duration: 12 months

 
​​CONTINUATION CRITERIA: Deferasirox (Exjade®/Jadenu®) is re-approved there is documentation of a decreased serum ferritin level compared with baseline level for transfusion dependent anemia.

Continuation duration: 2 years​


INITIAL CRITERIA: Deferiprone (Ferriprox®) is approved when all of the following are met:

  1. Diagnosis of transfusional iron overload due to Sickle Cell disease or other transfusion-dependent anemia; and
  2. Member is 3 years of age or older; and
  3. Inadequate response or inability to tolerate one of the following chelation therapy:
    1. Generic deferoxamine; or
    2. generic deferasirox; and
  4. For Brand Ferriprox tablets only, Inadequate response or inability to tolerate generic deferiprone tablets; and
  5. Current chelation therapy is inadequate

Initial authorization duration: 12 months


​​CONTINUATION CRITERIA: Deferiprone (Ferriprox®) is re-approved when there is documentation of positive clinical response to therapy (e.g., decline in serum ferritin levels from baseline).

Continuation duration: 2 years​


Chronic iron overload in non-transfusion-dependent Thalassemia Syndrome

INITIAL CRITERIA: Deferasirox (Exjade®/Jadenu®) is approved when ALL of the following are met:

  1. Diagnosis of chronic iron overload in Non-Transfusion-Dependent Thalassemia Syndromes; and
  2. Member is 10 years of age or older; and
  3. Serum ferritin levels are consistently greater than 300 mcg/L and liver iron concentration (LIC) of at least 5 milligrams of iron per gram of liver dry weight (mg Fe/g dw) (as demonstrated with at least two lab values within 2 months prior to treatment)

Initial authorization duration: 12 months

 
CONTINUATION CRITERIA: Deferasirox (Exjade®/Jadenu®) is re-approved when there is documentation of a decreased serum ferritin level compared with the baseline level or reduction in LIC (liver iron concentration) for non-transfusion dependent Thalassemia Syndrome

Continuation duration: 2 years


INITIAL CRITERIA: Deferiprone (Ferriprox®) is approved when ALL of the following are met:

  1. Diagnosis of transfusional iron overload due to Thalassemia Syndrome; and
  2. Member is 3 years of age or older; and
  3. Inadequate response or inability to tolerate one of the following chelation therapy:
    1. Generic deferoxamine; or
    2. Generic deferasirox; and
  4. For Brand Ferriprox tablets only, Inadequate response or inability to tolerate generic deferiprone tablets; and
  5. Current chelation therapy is inadequate

Initial authorization: 12 months
 
​​CONTINUATION CRITERIA: Deferiprone (Ferriprox®) is re-approved when there is documentation of positive clinical response to therapy (e.g., greater than or equal to 20% decline in serum ferritin levels from baseline).

Continuation duration: 2 years​


 

 

Deferasirox (Exjade/ Jadenu)

Renal failure: Deferasirox can cause acute renal failure and death, particularly in patients with comorbidities and those who are in the advanced stages of their hematologic disorders. Deferasirox is contraindicated in adults and pediatric patients with eGFR less than 40 ml/min/1.73m2. Use caution in pediatric patients with eGFR between 40 and 60 ml/min/1.3m2. For patients with renal impairment (eGFR 40-60 ml/min/1.73m2) reduce starting dose by 50%. Measure serum creatinine and determine creatinine clearance (CrCl)prior to initiation of therapy and monitor renal function at least monthly thereafter. For patients with baseline renal impairment or increased risk of acute renal failure, monitor creatinine weekly for the first month, then at least monthly thereafter. Monitor serum ferritin monthly to evaluate for overchelation. Use the minimum dose to establish and maintain a low iron burden. Consider dose reduction, interruption, or discontinuation based on increases in serum creatinine. Interrupt deferasirox therapy when acute kidney injury is suspected and during volume depletion.

Hepatic failure: Deferasirox can cause hepatic injury including hepatic failure and death. Measure serum transaminases and bilirubin in all patients prior to initiating treatment, every 2 weeks during the first month, and at least monthly thereafter. Avoid use of deferasirox in patients with severe (Child-Pugh class C) hepatic impairment and reduce the dose in patients with moderate (Child-Pugh class B) hepatic impairment. Interrupt deferasirox therapy when acute liver injury is suspected and during volume depletion.

GI hemorrhage: Deferasirox can cause GI hemorrhages, which may be fatal, especially in elderly patients who have advanced hematologic malignancies and/or low platelet counts. Monitor patients and discontinue deferasirox for suspected GI ulceration or hemorrhage.

Deferiprone (Ferriprox)

Agranulocytosis/Neutropenia: Deferiprone can cause agranulocytosis that can lead to serious infections and death. Neutropenia may precede the development of agranulocytosis. Measure the absolute neutrophil count (ANC) before starting deferiprone therapy and monitor the ANC weekly during therapy. Interrupt deferiprone therapy if neutropenia develops. If infection develops, interrupt deferiprone and monitor the ANC more frequently. Advise patients taking deferiprone to report immediately any symptoms indicative of infection. For neutropenia, instruct the patient to immediately discontinue deferiprone and all other medications with potential to cause neutropenia. Obtain a complete blood count (CBC), white blood count (WBC corrected for the presence of nucleated red blood cells, ANC and a platelet count daily until recovery. For agranulocytosis, consider hospitalization and other clinically appropriate management.


 



Biyani CS. Cystinuria. Available at http://emedicine.medscape.com/article/435678-overview. Accessed November 15, 2023.

 Exjade (deferasirox) [package insert]. East Hanover NJ. Novartis Pharmaceuticals Corporation.  Revised July 2020. Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=3495a70c-870c-4968-940e-8baea152cf85&type=display. Accessed November 15, 2023.

Ferriprox (deferiprone) [package insert]. Rockville MD. ApoPharma USA, Inc.  Revised  May 2020.  Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=97f7bfcb-8666-464c-87b6-9621ceca5ee2&type=display. Accessed November 15, 2023.

 Gilroy RK. Wilson Disease. Available at: http://emedicine.medscape.com/article/183456-overview#a1. Accessed November 15, 2023.

Jadenu (deferasirox) [package insert]. East Hanover NJ. Novartis Pharmaceuticals Corporation. Revised July 2020. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=fee89140-fff1-4443-9f42-24ac004fcda1. Accessed November 15, 2023.

 Mir M. Transfusion induced iron overload. Available at: http://emedicine.medscape.com/article/1389732-overview. Accessed November 15, 2023.

Musallam KM, Rivella S, Vichinsky E, Rachmilewitz. Non-transfusion-dependent thalassemias. Haematologica. June 2013;98:833-844. DOI: 10.3324/haematol.2012.066845


 

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Off-Label Use policy Rx.01.33


Brand NameGeneric Name
ExjadeDeferasirox
JadenuDeferasirox
Ferriprox
Deferiprone


 

298
  
1/1/2024Rx.01.54CommercialOyenusi, Oluwadamilola

Mecasermin (Increlex®) is indicated for the long-term treatment of growth failure in children with severe primary IGF-1 deficiency (Primary IGFD) or with growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH.

Mecasermin is a recombinant form of human insulin-like growth factor I (rhIGF-I) that mediates anabolic and growth-promoting effects of growth hormone. Endogenous Insulin Growth Factor1 (IGF-1) is required for normal growth and brain development. IGF-1 is in part directed towards stimulating the uptake of glucose, fatty acids, and amino acids so that metabolism supports growing tissues. Mecasermin increases in insulin sensitivity by mimicking some actions of insulin.

The intent of this policy is to communicate the medical necessity criteria for mescasermin (Increlex®) as provided under the member's prescription drug benefit.

INITIAL CRITERIA: Mecasermin (Increlex®) is approved when all of the following are met: 

  1. Diagnosis of growth failure in children with ONE of the following:
    1. All of the following:
      1. Severe primary insulin-like growth factor-1 deficiency (IGF-D); and
      2. Height standard deviation (SD) score of less than or equal to 3.0 SD scores below normal (growing at below the third percentile for age and sex); and
      3. Baseline IGF-1 (SD) score of less than or equal to 3.0 SD scores below normal (based on age- and sex-related reference ranges); and
      4. Normal or elevated GH level (based on GH stimulation testing); or
    2. Both of the following:
      1. Growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH;  and
      2. Measured titers of GH-neutralizing antibodies; and
  2. Member is 2 years of age or older; and
  3. Open epiphyses (bone growth plates) (bone age less than 14 years for girls and less than 16 years for boys); and
  4. Prescribed by or in consultation with an endocrinologist

 

Initial Authorization duration: 12 months

CONTINUATION CRITERIA: Continuation of mecasermin (Increlex®) is reapproved when all of the following are met:

  1. Growth velocity greater than or equal to 2 cm/year; and
  2. Yearly evaluation by an endocrinologist; and
  3. BOTH of the following
    1. Expected adult height is not obtained; and
    2. Documentation of expected adult height goal

 Continuation duration: 12 months

None

Increlex® (mecasermin) [prescribing information]. Basking Ridge, NJ. Ipsen Biopharmaceuticals, Inc. December 2019. Available online at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=a8b27a1b-a611-4f91-ad22-76d4b390c3ae&type=display#section-10.1.  Accessed on November 30, 2023

 

Mecasermin. Micromedex Solutions [Internet database]. Available at: www.micromedexsolutions.com. Accessed November 30, 2023

139/14/20239/14/20241/1/2024 1:24 AMNo presence informationsrv_ppsgw_P
Off-Label Use Rx.01.33

Brand NameGeneric Name
Increlex®mecasermin

299
  
1/1/2024Rx.01.109CommercialOyenusi, Oluwadamilola

Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of angioedema. The most frequently implicated areas during an attack of HAE include areas of the skin, gastrointestinal tract, and upper respiratory tract, including the larynx. Involvement of the larynx may lead to fatality by asphyxiation. During episodes of HAE, individuals experience severe edema of the affected areas, characterized by gradual worsening over 24 hours and resolution within 2-5 days without treatment. Importantly, symptoms of HAE exclude urticaria and pruritis.

Several forms of hereditary angioedema exist, with type I and II being most common. In most cases, individuals with HAE demonstrate a deficiency in C1 inhibitor caused by mutation in the C1 inhibitor gene. .

Neither anabolic steroids nor antifibrinolytic drugs, used for prophylaxis of HAE attacks, are reliably effective in treating acute HAE attacks.  Epinephrine, corticosteroids, and antihistamines are also not effective for treating HAE attacks and are not recommended by current guidelines.  Guidelines recommend that patients with HAE have access to an "effective, on-demand, HAE-specific agent" to manage acute attacks. 

Mechanism of Action

Vasodilation results from excessive bradykinin production, a downstream effect from a deficiency in C1 (a subset of Complement protein) inhibitor protein. C1-inhibitor protein inhibits kallikrein, which is a protease that activates the potent vasodilator, bradykinin.  Modulation of the C1 cascade is a target for the prophylaxis and treatment of acute attacks of HAE.   Patients with HAE have low levels of endogenous or functional C1 esterase inhibitor (C1INH). Although the events that induce attacks of angioedema in HAE patients are not well understood, it is thought that contact system activation occurs. Contact system activation results in increased levels of bradykinin which causes increases in vascular permeability which results in the clinical manifestations of HAE.

Sajazir™/Firazyr® (icatibant) inhibits bradykinin from binding the B2 receptor and thereby treats the clinical symptoms of an acute, episodic attack of hereditary angioedema.  Icatibant is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of hereditary angioedema (HAE) in adults 18 years of age and older.   

Haegarda® (C1 esterase inhibitor subcutaneous [human]) is a plasma-derived concentrate of C1 esterase inhibitor (human) that is indicated for routine prophylaxis to prevent HAE attacks in patients 6 years of age and older. 

Cinryze® (C1 esterase inhibitor [human]) is C1 esterase inhibitor indicated for routine prophylaxis against angioedema attacks in pediatric (6 years old and above), adolescent and adult patients with HAE.

Berinert® (C1 esterase inhibitor [human] is a plasma-derived C1 esterase inhibitor (human) indicated for the treatment of acute abdominal, facial, or laryngeal HAE attacks in adult and pediatric patients.

Orladeyo™ (berotralstat) is a plasma kallikrein inhibitor indicated for prophylaxis to prevent attacks of hereditary angioedema (HAE) in adults and pediatric patients 12 years and older.

Ruconest® is a C1 esterase inhibitor [recombinant] indicated for the treatment of acute attacks in adults and adolescent patients with HAE.

Takhzyro® (lanadelumab-flyo) is a human monoclonal antibody that acts to inhibit plasma kallikrein, and is indicated for prevention of HAE in patients 2 years of age and older. Kallikrein is a protease that activates bradykinin, a potent vasodilator implicated in the pathogenesis of angioedema attacks in patients with HAE. Inhibition of kallikrein results in downstream inhibition of bradykinin production.

The intent of this policy is to communicate the medical necessity criteria for Takhzyro® (lanadelumab-flyo), Haegarda® (C1 esterase inhibitor subcutaneous [human]), Cinryze® (C1 esterase inhibitor [human]), Berinert® (C1 esterase inhibitor [human]), Ruconest® (C1 inhibitor recombinant), Sajazir™/Firazyr® (icatibant), and Orladeyo™ (berotralstat) as provided under the member's pharmacy benefit. 

Prophylaxis against angioedema attacks

INITIAL CRITERIA: C1 esterase inhibitor (human) (Cinryze® or Haegarda®) is approved when ALL of the following are met:

  1. Diagnosis of hereditary angioedema (HAE) is confirmed by decreased serum levels of C4 and absence or marked decrease (less than 50 percent of normal) of the level or function of C1-INH; and
  2. Prescribed by or in consultation with an immunologist, allergist, or pulmonologist; and
  3. Member is 6 years of age or greater; and
  4. For Cinryze only, inadequate response or inability to tolerate ONE of the following:
    1. Haegarda; or
    2. Takhzyro; or
    3. Orladeyo
​Initial authorization duration: 2 years
 
REAUTHORIZATION CRITERIA CI esterase inhibitors (human) (Cinryze® or Haegarda®) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

INITIAL CRITERIA: Lanadelumab-flyo (Takhzyro®) injection or berotralstat (Orladeyo®is approved when all of the following are met:
  1. Diagnosis of hereditary angioedema (HAE); and
  2. One of the following:
    1. For Takhzyro, member is 2 years of age or older; or
    2. For Orladeyo, member is 12 years of age or older; and
  3. Prescribed by or in consultation with an immunologist, allergist, or pulmonologist

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: Lanadelumab-flyo (Takhzyro®), or berotralstat (Orladeyo®) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

_______________________________________________________________________________________________

Treatment of angioedema attacks

INITIAL CRITERIA: C1 esterase inhibitor (human) (Berinert®) is approved when ALL of the following are met:
  1. Diagnosis of treatment of acute abdominal, facial, or laryngeal attacks of HAE in adults and pediatric patients; and
  2. Prescribed by or in consultation with an immunologist, allergist, or pulmonologist; and
  3. One of the following:
    1. Inadequate response or inability to tolerate C1 esterase inhibitor recombinant (Ruconest®); or
    2. One of the following:
      1. Member is 12 years of age or younger; or
      2. Documentation that member has history of laryngeal attacks

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: C1 esterase inhibitors (human) (Berinert®) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

INITIAL CRITERIA: C1 esterase inhibitor recombinant (Ruconest®) is approved when ALL of the following are met:

  1. Diagnosis of treatment of acute attacks of HAE in adults and adolescents over the age of 12 years; and
  2. Prescribed by or in consultation with an immunologist, allergist, or pulmonologist.

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA: C1 esterase inhibitor recombinant (Ruconest®) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

INITIAL CRITERIA: Icatibant (Firazyr® /Sajazir™) is approved when ALL of the following criteria are met:

  1. Member is 18 years of age or older; and
  2. Diagnosis of hereditary angioedema; and
  3. Prescribed by or in consultation with an immunologist, allergist, or pulmonologist

Initial authorization duration: 2 years

REAUTHORIZATION CRITERIA Icatibant (Firazyr®/Sajazir™) is re-approved with documentation of positive clinical response to therapy.

Reauthorization duration: 2 years

None


Firazyr® (icatibant) [package insert]. Lexinton MA. Shire Orphan Therapeutics. Oct, 2021  Available at: https://dailymed.nlm.nih.gov/dailymed/fda/fdaDrugXsl.cfm?setid=ed6657ca-ab68-477a-9968-e12dc928b540&type=display. Accessed December 5, 2023

 

Zuraw BL, Bernstein JA, Lang DM, et al. A focused parameter update: hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema.  J Allergy Clin Immunol. 2013; Volume 131, issue 6, pages 1491-1493.e25. Accessed December 5, 2023.

 

Haegarda® (C1 esterase inhibitor subcutaneous [human]). [package insert]. Kanakee, IL: CSL Behring. January 2022. Available at: http://labeling.cslbehring.com/PI/US/HAEGARDA/EN/HAEGARDA-Prescribing-Information.pdf. Accessed December 5, 2023.

 

Cinryze® (C1 esterase inhibitor [human]). [package insert]. Lexington, MA: Shire ViroPharma Inc. January 2021. Available at: http://pi.shirecontent.com/PI/PDFs/Cinryze_USA_ENG.pdf. Accessed December 5, 2023.

 

Berinert® (C1 esterase inhibitor [human]). [package insert]. Kanakee, IL: CSL Behring. September 2021. Available at: http://labeling.cslbehring.com/PI/US/Berinert/EN/Berinert-Prescribing-Information.pdf. Accessed December 5, 2023

 

Orladeyo™ (berotralstat). [prescribing information]. Durham, NC: BioCryst Pharmaceuticals, Inc. March 2022. Available at: https://orladeyohcp.com/wp-content/uploads/ORLADEYO_PI_V1_2020.pdf. Accessed December 5, 2023.  

 

Ruconest® (C1 esterase inhibitor [recombinant]). [package insert]. Raleigh, NC: Santarus, Inc. April 2020. Available at: https://www.ruconest.com/PDF/ruconest-pi.pdf. Accessed December 5, 2023

 

Sajazir™ (icatibant) [package insert]. Cambridge, United Kingdom. Cycle Pharmaceuticals Ltd. June 2021. Available at: https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=1756aca0-21a1-4898-8d6c-9666738db45c. Accessed December 5, 2023

 

Takhzyro®. [package insert]. Lexington, MA: Dyax Corp., wholly-owned subsidiary of Shire US Inc. February 2023. Available at: https://www.shirecontent.com/PI/PDFs/TAKHZYRO_USA_ENG.pdf. Accessed December 5, 2023.

 

Zuraw B MD, Farkas H MD. Hereditary angioedema (due to C1 inhibitor deficiency): Pathogenesis and diagnosi. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. Accessed on December 5, 2023


209/14/20236/8/20241/1/2024 1:24 AMNo presence informationsrv_ppsgw_P

Rx.01.33 Off Label Use

​Rx.01.76 Quantity Level Limits for Pharmaceuticals Covered Under the Prescription Drug Benefit

Brand NameGeneric Name
Firazyr®/Sajazir™icatibant
Haegarda®C1 esterase inhibitor subcutaneous [human]
Cinryze®
C1 esterase inhibitor [human]
Berinert®C1 esterase inhibitor [human]
Orladeyo™

berotralstat

 

Ruconest®C1 esterase inhibitor [recombinant]
Takhzyro®lanadelumab-flyo

300
  
1/1/2024Rx.01.117CommercialOyenusi, Oluwadamilola

Cystic fibrosis (CF) is an inherited disease that affects mucus and sweat produced by secretory glands. The cystic fibrosis conductance regulator (CFTR) is a chloride channel present at the surface of epithelial cells in multiple organs. Mutations in this gene alter its ability to regulate the transport of chloride, sodium, and bicarbonate, leading to thick secretions in the lungs, pancreas, and other organs. Thickening of mucus can provide an environment for bacteria to grow, leading to repeated infections. Mucus blockage prevents pancreatic digestive enzymes from reaching the small intestine, reducing fat and protein absorption, which can lead to malnourishment. CF also causes sweat to become salty, which can lead to dehydration and fatigue when sweat leaves the body.

Approximately 30,000 people in the United States, and 70,000 worldwide, are living with cystic fibrosis (CF).  Over 2000 mutations in the CFTR gene have been identified.  The most common of which is the F508del mutation, affecting approximately 90% of those with CF.  Approximately 50% of those with CF are homozygous for F508Del mutation.  Other mutations in the CTFR gene include, but are not limited to: G551D, G1244E, G1349D, G178R, G551S, R117H, S1251N, S1255P, S549N and S549R,etc.

Ivacaftor (Kalydeco®)

Ivacaftor (Kalydeco®) is indicated for the treatment of CF in patients age 1 month and older who have one mutation in the CFTR gene that is responsive to ivacaftor based on clinical and/or in vitro assay data* as noted in the prescribing information.  

Ivacaftor is a potentiator of the CFTR protein, which promotes increased chloride transport by potentiating the gating (channel-open probability) of the CFTR protein, and improves the regulation of salt and water absorption and secretion in various tissues.

Lumacaftor/ivacaftor (Orkambi®)

Lumacaftor/ivacaftor (Orkambi®) is indicated for the treatment of CF in patients age 1 years and older who are homozygous for the F508del mutation in the CFTR gene*.

The F508del mutation causes protein misfolding resulting in impaired processing and gating of the CFTR protein. Lumacaftor improves the conformational stability of F508del-CFTR, resulting in increased processing and gating activity. Ivacaftor adds the benefit of potentiating gating of the CFTR protein.

*If genotype unknown, an FDA-cleared CF mutation test should be performed to detect the presence of the F508del mutation on both alleles of the CFTR gene.

Elexacaftor/tezacaftor/ivacaftor and ivacaftor (Trikafta™)

Elexacaftor/tezacaftor/ivacaftor and ivacaftor (Trikafta™) is indicated for the treatment of CF in patients aged 2 years and older who have at least one F508del mutation in the CFTR gene or a mutation in the CFTR gene that is responsive based on in vitro data.

Elexacaftor and tezacaftor bind to different sites on the CFTR protein and have an additive effect in facilitating the cellular processing and trafficking of F508del-CFTR to increase the amount of CFTR protein delivered to the cell surface compared to either molecule alone. Ivacaftor potentiates the channel open probability (or gating) of the CFTR protein at the cell surface.

*If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to confirm the presence of at least one F508del mutation.

Tezacaftor/ivacaftor (Symdeko®)

Tezacaftor/ivacaftor (Symdeko®) is indicated for the treatment of CF in patients age 6 years and older who are homozygous for the F508del mutation or who have at least one mutation in the CFTR gene that is responsive to tezacaftor/ivacaftor based on in vitro data and/or clinical evidence*.

Tezacaftor facilitates the cellular processing and trafficking of normal and select mutant forms of CFTR (including F508del-CFTR) to increase the amount of mature CFTR protein delivered to the cell surface. Ivacaftor is a potentiator of the CFTR protein, which facilitates increased chloride transport by potentiating the channel-open probability (or gating) of the CFTR protein at the cell surface

*If genotype unknown, an FDA-cleared CF mutation test should be performed to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing.

The intent of this policy is to communicate the medical necessity criteria for ivacaftor (Kalydeco®), lumacaftor/ivacaftor (Orkambi®), elexacaftor/tezacaftor/ivacaftor and ivacaftor (Trikafta™), and tezacaftor/ivacaftor (Symdeko®) as provided under the member's prescription drug benefit.
 

INITIAL CRITERIA:  Ivacaftor (Kalydeco® ) is approved when ALL of the following are met:

  1. Diagnosis of cystic fibrosis (CF); and
  2. Member is 1 month of age or older; and
  3. Prescribed by or in consultation with a pulmonologist; and
  4. Presence of one mutation in the CFTR gene that is responsive to ivacaftor as noted in the Prescribing Information (if the patient's genotype is unknown, an FDA-cleared test must be used to detect the presence of CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test)


INITIAL CRITERIA: Lumacaftor/ivacaftor (Orkambi® ) is approved when ALL of the following are met:

  1. Diagnosis of cystic fibrosis; and
  2. Member is 1 year of age or older; and
  3. Prescribed by or in consultation with a pulmonologist; and
  4. Homozygous for the F508del mutation in the CFTR gene (if the patient's genotype is unknown, an FDA-cleared CR mutation test must be performed to determine the presence of the F508del mutation on both alleles of the CFTR gene) 

INITIAL CRITERIA: Elexacaftor/tezacaftor/ivacaftor and ivacaftor (Trikafta™) is approved when ALL of the following are met:

  1. Diagnosis of cystic fibrosis (CF); and
  2. Member is 2 years of age or older; and
  3. One of the following:
    1. Member has at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene as detected by an FDA-cleared cystic fibrosis mutation test; OR
    2. Member has at least one mutation in the CFTR gene that is responsive based on in vitro data to elexacaftor/tezacaftor/ivacaftor (Trikafta™) as noted in the Prescribing Information; and
  4. Prescribed by or in consultation with a pulmonologist

INITIAL CRITERIA: Tezacaftor/ivacaftor (Symdeko®) is approved when ALL of the following are met: 

  1. Diagnosis of cystic fibrosis; and
  2. Member is 6 years of age or older; and
  3. Prescribed by or in consultation with a pulmonologist; and
  4. Documentation of one of the following:
    1. Member is homozygous for the F508del mutation; or
    2. Member has at least one tezacaftor/ivacaftor responsive mutation in the CFTR gene as noted in the Prescribing Information (If the patient's genotype is unknown, an FDA-cleared test must be used to detect the presence of CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test).

Initial Authorization duration: 2 years

REAUTHORIZATION CRITERIA: Ivacaftor (Kalydeco®), Lumacaftor/ivacaftor (Orkambi®), Elexacaftor/tezacaftor/ivacaftor and ivacaftor (Trikafta™), or Tezacaftor/ivacaftor (Symdeko®) is re-approved when there is positive clinical response to therapy (e.g., improvement in lung function or decreased number of pulmonary exacerbations).

Reauthorization duration: 2 years 

N/A


Accurso FJ, Rowe SM, Clancy JP, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010;363(21):1991-2003.

Aherns R, Rodriguez S, Yen K, Davies JC. VX-770 in subjects 6 to 11 years with cystic fibrosis and the G551D-CFTR mutation [abstract]. Pediatr Pulmonol. 2011;46(suppl 34):283.

Boyle MP, Bell S, Konstan MW, McColley SA, Wisseh S, Spencer-Green G. VX-809, an investigational CFTR corrector, in combination with VX-770, an investigational CFTR potentiator, in subjects with CF and homozygous for the F508del-CFTR mutation [abstract]. Pediatr Pulmonol. 2011;46(suppl 34):287.

Castellani C, Cuppens H, Macek M Jr, Cassiman JJ, Kerem E, Durie P, Tullis E,  Assael BM, Bombieri C, Brown A, Casals T, Claustres M, Cutting GR, Dequeker E, Dodge J, Doull I, Farrell P, Ferec C, Girodon E, Johannesson M, Kerem B, Knowles  M, Munck A, Pignatti PF, Radojkovic D, Rizzotti P, Schwarz M, Stuhrmann M, Tzetis M, Zielenski J, Elborn JS. Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice. J Cyst Fibros. 2008 May;7(3):179-96. doi: 10.1016/j.jcf.2008.03.009.

Chen Y, Luo X, Dubey N, et al. Drug-drug interaction between VX-770 and CYP3A modulators [abstract]. J Clin Pharmacol. 2011;51:1348.

Drumm ML, Ziady AG, Davis PB. Genetic variation and clinical heterogeneity in cystic fibrosis. Annu Rev Pathol. 2012;7:267-282.

Flume PA, Borowitz D, Liou T, et al. VX-770 in subjects with CF and homozygous for the F508del-CFTR mutation [abstract]. Pediatr Pulmonol. 2011;46(suppl 34):284-285.

Kalydeco® (Ivacaftor) [package insert]. Boston, MA. Vertex Pharmaceuticals, Inc; December 2020. Available at: https://pi.vrtx.com/files/uspi_ivacaftor.pdf. Accessed on December 5, 2023.

Orkambi® (lumacaftor/ivacaftor) [package insert]. Boston, MA: Vertex Pharmaceuticals Incorporated; February 2023. Available at: https://pi.vrtx.com/files/uspi_lumacaftor_ivacaftor.pdf. Accessed on December 5, 2023.​

McKone EF, Borowitz D, Drevinek P, et al. Long-term safety and efficacy of investigational CFTR potentiator, VX-770, in subjects with CF [abstract]. Pediatr Pulmonol. 2011;46(suppl 34):284.​

Mogayzel PJ Jr, Naureckas ET, Robinson KA, Mueller G, Hadjiliadis D, Hoag JB, Lubsch L, Hazle L, Sabadosa K, Marshall B; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9.

National Heart, Lung, and Blood Institute. Cystic Fibrosis. Bethesda, MD. Accessed February 01, 2023.

Ramsey BW, Davies J, McElvaney NG, et al; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011; 365(18):1663-1672.

Robinson KA, Saldanha IJ, McKoy NA. Management of infants with cystic fibrosis: a summary of the evidence for the Cystic Fibrosis Foundation working group on care of infants with cystic fibrosis. J Pediatr. 2009;155(suppl 6): S94-S105.

Simon, RH. Cystic fibrosis: Overview of the treatment of lung disease. In: UpToDate. Waltham, MA. Accessed on December 05, 2023.

Symdeko® (tezacaftor/ivacaftor) [package insert]. Boston, MA. Vertex Pharmaceutical Inc.; June 2022. Available at: https://pi.vrtx.com/files/uspi_tezacaftor_ivacaftor.pdf. Accessed December 5, 2023.

Trikafta™ (elexacaftor/tezacaftor/ivacaftor and ivacaftor) [package insert]. Boston, MA: Vertex Pharmaceuticals Incorporated; October 2021. Available at: https://pi.vrtx.com/files/uspi_elexacaftor_tezacaftor_ivacaftor.pdf. Accessed on December 5, 2023.

Van Goor F, Hadida S, Grootenhuis PD, et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A. 2009;106(44):18825-18830.​

Van Goor F, Yu H, Burton B, Huang T, Hoffman B, Negulescu P. VX-770 potentiation of CFTR forms with channel gating defects in vitro [abstract]. Pediatr Pulmonol. 2011;46(suppl 34):215.

Woodworth BA, Zhang S, Skinner D, Sorscher EJ, Rowe SM. Comparison of CFTR and ciliary beat frequency activation by the CFTR modulators VX-770, VRT532, and UCCF-152 in primary sinonasal epithelial cultures [abstract]. Pediatr Pulmonol. 2011;46(suppl 34):251-252.

Yu H, Burton B, van Goor F. VX-770, an investigational CFTR potentiator, acts on multiple CFTR forms in vitro [abstract]. Pediatr Pulmonol. 2010;45(suppl 33):318-319


259/14/20233/16/20241/1/2024 1:24 AMNo presence informationsrv_ppsgw_P

Off-Label Use Rx.01.33

Drug Name Generic Name 
Kalydeco®Ivacaftor
Orkambi®Lumacaftor/ivacaftor
Trikafta™Elexacaftor/tezacaftor/ivacaftor and ivacaftor
Symdeko®Tezacaftor/ivacaftor
301
  
1/1/2024Rx.01.124CommercialOyenusi, Oluwadamilola

Narcolepsy results from the loss of the neuropeptides, orexin-A and orexin-B (also known as hypocretin-1 and hypocretin-2), which have excitatory effects on the ox1 and ox2 receptors on postsynaptic neurons.  Narcolepsy is a clinical syndrome of daytime sleepiness with cataplexy, hypnagogic hallucinations, and sleep paralysis. It is the second most common cause of disabling daytime sleepiness after obstructive sleep apnea.  Typical age of onset of narcolepsy is teens to twenties. Patients with type 1 narcolepsy (narcolepsy with cataplexy) typically present with moderate to severe daytime sleepiness, transient facial weakness or falls triggered by joking or laughter (partial or complete cataplexy), or the inability to move for one or two minutes immediately after awakening or just before falling asleep. Patients with type 2 narcolepsy do not have cataplexy.

Sodium oxybate, a CNS depressant, is the sodium salt of gamma hydroxybutyrate, a metabolite of GABA.  It is hypothesized that the therapeutic effects of Xyrem® on cataplexy and excessive daytime sleepiness are mediated through GABA-B actions at noradrenergic and dopaminergic neurons, as well as at thalamocortical neurons.  The exact mechanism of sodium oxybate in narcolepsy is unknown.

Xywav™ is a CNS depressant. The exact mechanism of action in the treatment of narcolepsy is unknown. Xywav™ is a mixture of calcium oxybate, magnesium oxybate, potassium oxybate, and sodium oxybate (gamma-hydroxybutyrate). Gamma-hydroxybutyrate (GHB) is an endogenous compound and metabolite of the neurotransmitter GABA. It is hypothesized that the therapeutic effects of Xywav™ on cataplexy and excessive daytime sleepiness are mediated through GABA-B actions during sleep at noradrenergic and dopaminergic neurons, as well as at thalamocortical neurons. The active moiety of oxybate salts is the same as that of sodium oxybate (Xyrem®), and dosing is the same; the only difference is that oxybate salts (Xywav™) have 90 percent less sodium.

Sodium oxybate (Xyrem®/Lumryz) is indicated for the treatment of cataplexy in narcolepsy and excessive daytime sleepiness (EDS) in patients 7 years of age and older with narcolepsy.

Calcium, magnesium, potassium and sodium oxybates (Xywav™) is indicated for the treatment of cataplexy or excessive daytime sleepiness (EDS) in patients 7 years of age and older with narcolepsy and idiopathic hypersomnia (IH) in adults.


 

The intent of this policy is to communicate the medical necessity criteria for sodium oxybate (Xyrem®/Lumryz) and calcium, magnesium, potassium and sodium oxybates (Xywav™) as provided under the member's prescription drug benefit.

Cataplexy with narcolepsy (Type 1)

INITIAL CRITERIA: Sodium oxybate (Xyrem®) solution or calcium, magnesium, potassium and sodium oxybates (Xywav™) is approved when ALL of the following are met:

  1. Diagnosis of Cataplexy with narcolepsy (Type 1); and
  2. Member is 7 years of age or older; and
  3. Diagnosis was confirmed by ONE of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Prescribed by or in consultation with a neurologist or sleep specialist; and
  5. No concurrent use of sedative hypnotics and alcohol; and
  6. For Sodium oxybate solution and Xyrem® solution only, inadequate response or inability to tolerate TWO of the following:
    1. Xywav; and
    2. Wakix

Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Sodium oxybate (Xyrem®) solution or calcium, magnesium, potassium and sodium oxybates (Xywav™) is re-approved when ALL of the following are met:

  1. Documentation to support the efficacy associated with the current regimen (including but not limited to reduction in the frequency of cataplexy attacks or an improvement in the Epworth sleepiness scale); and
  2. There is no claim history of a sedative hypnotic agents within one year of the request; and
  3. For Sodium oxybate solution and Xyrem® solution only, inadequate response or inability to tolerate TWO of the following:
    1. Xywav; and
    2. Wakix
  4. Yearly evaluation by a neurologist or sleep specialist

​​Reauthorization duration: 12 months 

INITIAL CRITERIA: Sodium oxybate suspension extended-release (Lumryz™) is approved when ALL of the following are met:
  1. Diagnosis of Cataplexy with narcolepsy (Type 1); and
  2. Member is 18 years of age or older; and
  3. Diagnosis was confirmed by ONE of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Inadequate response or inability to tolerate TWO of the following:
    1. Xywav
    2. Wakix; and
  5. Prescribed by or in consultation with a neurologist or sleep specialist; and
  6. No concurrent use of sedative hypnotics and alcohol

Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Sodium oxybate suspension extended-release (Lumryz™) is re-approved when ALL of the following are met:

  1. Documentation to support the efficacy associated with the current regimen (including but not limited to reduction in the frequency of cataplexy attacks or an improvement in the Epworth sleepiness scale); and
  2. Inadequate response or inability to tolerate TWO of the following:
    1. Xywav
    2. Wakix; and
  3. There is no claim history of a sedative hypnotic agents within one year of the request; and
  4. Yearly evaluation by a neurologist or sleep specialist

 

Reauthorization duration: 12 month

______________________________________________________________________________________________

 

Excessive daytime sleepiness in narcolepsy (Type 2)

INITIAL CRITERIA: Sodium oxybate (Xyrem®) solution or calcium, magnesium, potassium and sodium oxybates (Xywav™) is approved when ALL of the following are met:

  1. Diagnosis of Excessive daytime sleepiness in narcolepsy (Type 2); and
  2. Member is 7 years of age or older; and
  3. Diagnosis was confirmed by ONE of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Inadequate response or inability to tolerate ALL of the following:
    1. Modafinil or armodafinil; and
    2. One stimulant product (e.g., amphetamine, methylphenidate); and
    3. Sunosi®; and
  5. Prescribed by or in consultation with a neurologist or sleep specialist; and
  6. No concurrent use of sedative hypnotics and alcohol; and
  7. For Sodium oxybate  and Xyrem® solution only, inadequate response or inability to tolerate TWO of the following:
    1. Xywav; and
    2. Wakix

​​Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Sodium oxybate (Xyrem®) solution or calcium, magnesium, potassium and sodium oxybates (Xywav™) is re-approved when ALL of the following are met:
  1. Documentation to support the efficacy associated with the current regimen (including but not limited to reduction in the frequency of cataplexy attacks or an improvement in the Epworth sleepiness scale); and
  2. There is no claim history of a sedative hypnotic agents within one year of the request; and
  3. For Sodium oxybate  and Xyrem® solution only, inadequate response or inability to tolerate TWO of the following:
    1. Xywav; and
    2. Wakix
  4. Yearly evaluation by a neurologist or sleep specialist

​Reauthorization duration: 12 months 


INITIAL CRITERIA: Sodium oxybate suspension extended-release (Lumryz™) is approved when ALL of the following are met:
  1. Diagnosis of Excessive daytime sleepiness in narcolepsy (Type 2); and
  2. Member is 18 years of age or older; and
  3. Diagnosis was confirmed by ONE of the following tests:
    1. Polysomnography (PSG); or
    2. Multiple sleep latency test (MSLT); and
  4. Inadequate response or inability to tolerate ALL of the following:
    1. Modafinil or armodafinil;
    2. One stimulant product (e.g., amphetamine, methylphenidate);
    3. Sunosi®; and
  5. Inadequate response or inability to tolerate TWO of the following:
    1. Xywav; and
    2. Wakix
  6. Prescribed by or in consultation with a neurologist or sleep specialist; and
  7. No concurrent use of sedative hypnotics and alcohol

Initial authorization duration: 12 months

REAUTHORIZATION CRITERIA: Sodium oxybate suspension extended-release (Lumryz™) is re-approved when ALL of the following are met:

  1. Documentation to support the efficacy associated with the current regimen (including but not limited to reduction in the frequency of cataplexy attacks or an improvement in the Epworth sleepiness scale); and
  2. Inadequate response or in