In the absence of coverage criteria from applicable Medicare statutes, regulations, NCDs, LCDs, CMS manuals, or other Medicare coverage documents, this policy uses internal coverage criteria developed by the Company in consideration of peer-reviewed medical literature, clinical practice guidelines, and/or regulatory status.
MEDICALLY NECESSARY
EMPHYSEMA WITH ALPHA-1 ANTITRYPSIN DEFICIENCY
Alpha-1 antitrypsin (AAT) therapy (e.g., Prolastin-C, Aralast NP, Glassia, Zemaira) is considered medically necessary and, therefore, covered for individuals 18 years of age and older who have AAT deficiency and clinical evidence of chronic emphysema without evidence of AAT-associated liver disease, when all of the following criteria are met:
- The individual has a low serum concentration of AAT less than 80 mg/dL (radial immunodiffusion) or 50 mg/dL (nephelometry) or less than 11 μmol/L (nephelometry) or less than 0.8 g/L (35% of normal), which is considered the threshold thought to protect against emphysema.
- The individual has a documented diagnosis of congenital AAT deficiency confirmed by one of the following:
- Pi*ZZ, Pi*Z(null) or Pi(null)(null) protein phenotypes (homozygous)
- Other rare AAT disease-causing alleles associated with serum AAT level <11 μmol/L
- The individual has progressive emphysema with a documented rate of decline in forced expiratory volume in 1 second (FEV1).
- The individual is a nonsmoker.
ACUTE GRAFT-VERSUS-HOST DISEASE (GVHD) AFTER HEMATOPOIETIC CELL TRANSPLANTATION
AAT therapy (e.g., Prolastin-C, Aralast NP, Glassia, Zemaira) administered intravenously is considered medically necessary and, therefore, covered for individuals 18 years of age and older who have acute graft-versus-host disease (GVHD) following hematopoietic cell transplantation in conjunction with systemic corticosteroids following no response (steroid-refractory disease) to first-line therapy options.
EXPERIMENTAL/INVESTIGATIONAL
All other uses of AAT therapy (e.g., Prolastin-C, Aralast NP, Glassia, Zemaira) are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics.
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 agencies, 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 drug.
