Alpha-1 Proteinase Inhibitor (Aralast NP®, Glassia®, Prolastin-C®, Zemaira®)
IMPORTANT REMINDER
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the medical policy and a health plan or government program (e.g., TennCare), the express terms of the health plan or government program will govern.
POLICY
I. INDICATIONS
The indications below including FDA-approved indications and compendial uses are considered a covered benefit provided that all the approval criteria are met and the member has no exclusions to the prescribed therapy.
FDA-Approved Indications
1. Aralast NP
Chronic augmentation therapy in adults with clinically evident emphysema due to severe congenital deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency)
2. Glassia
Chronic augmentation and maintenance therapy in adults with clinically evident emphysema due to severe hereditary deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency)
3. Prolastin-C
Chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency)
4. Zemaira
Chronic augmentation and maintenance therapy in adults with alpha1-proteinase inhibitor deficiency and clinical evidence of emphysema
All other indications are considered experimental/investigational and not medically necessary.
II. DOCUMENTATION
Submission of the following information is necessary to initiate the prior authorization review:
1. Pretreatment serum alpha1-antitrypsin (AAT) level
2. Pretreatment post-bronchodilation forced expiratory volume in 1 second (FEV1)
3. AAT protein phenotype or genotype
III. CRITERIA FOR INITIAL APPROVAL
Authorization of 12 months may be granted for treatment of emphysema due to alpha1-antitrypsin (AAT) deficiency when all of the following criteria are met:
1. The member’s pretreatment serum AAT level is less than 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry).
2. The member’s pretreatment post-bronchodilation forced expiratory volume in 1 second (FEV1) is greater than or equal to 25% and less than or equal to 80% of the predicted value.
3. The member has a documented PiZZ, PiZ (null), or Pi (null, null) (homozygous) AAT deficiency or other phenotype or genotype associated with serum AAT concentrations of less than 11 micromol/L (80 mg/dL by radial immunodiffusion or 50 mg/dL by nephelometry).
4. The member does not have the PiMZ or PiMS AAT deficiency.
IV. CONTINUATION OF THERAPY
Authorization of 12 months may be granted for continued treatment of emphysema due to alpha1-antitrypsin (AAT) deficiency when the member is experiencing beneficial clinical response from therapy.
V. OTHER
Note: If the member is a current smoker, they should be counseled on the harmful effects of smoking on pulmonary conditions and available smoking cessation options.
APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS
BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.
ADDITIONAL INFORMATION
For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).
REFERENCES
1. Aralast NP [package insert]. Lexington, MA: Baxalta US Inc.; December 2018.
2. Glassia [package insert]. Lexington, MA: Takeda Pharmaceuticals US Inc.; March 2022.
3. Prolastin-C Liquid [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; May 2020.
4. Prolastin-C [package insert]. Research Triangle Park, NC: Grifols Therapeutics Inc.; January 2022.
5. Zemaira [package insert]. Kankakee, IL: CSL Behring LLC; September 2022.
6. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818-900.
7. Marciniuk DD, Hernandez P, Balter M, et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2012;19:109-116.
8. Sandhaus RA, Turino G, Brantly ML, et al. The diagnosis and management of alpha-1 antitrypsin deficiency in the adult. Chronic Obstr Pulm Dis. 2016;3(3):668-82.
ORIGINAL EFFECTIVE DATE: 12/1/2016
MOST RECENT REVIEW DATE: 1/1/2024
ID_CHS
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
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