Alpha 1-Proteinase Inhibitors (Human) [Prolastin-C®, Aralast NP®, Zemaira®, Glassia®]
The purpose of this policy is to provide general information applicable to the administration of health benefits that Horizon Blue Cross Blue Shield of New Jersey and Horizon Healthcare of New Jersey, Inc. (collectively “Horizon BCBSNJ”) insures or administers. If the member’s contract benefits differ from the medical policy, the contract prevails. Although a service, supply or procedure may be medically necessary, it may be subject to limitations and/or exclusions under a member’s benefit plan. If a service, supply or procedure is not covered and the member proceeds to obtain the service, supply or procedure, the member may be responsible for the cost. Decisions regarding treatment and treatment plans are the responsibility of the physician. This policy is not intended to direct the course of clinical care a physician provides to a member, and it does not replace a physician’s independent professional clinical judgment or duty to exercise special knowledge and skill in the treatment of Horizon BCBSNJ members. Horizon BCBSNJ is not responsible for, does not provide, and does not hold itself out as a provider of medical care. The physician remains responsible for the quality and type of health care services provided to a Horizon BCBSNJ member.
Horizon BCBSNJ medical policies do not constitute medical advice, authorization, certification, approval, explanation of benefits, offer of coverage, contract or guarantee of payment.
Alpha1-Proteinase Inhibitor (Human) (Prolastin-C®, Aralast NP®, Zemaira®, Glassia®) is a preparation of purified human plasma-derived alpha1-proteinase inhibitor, alpha1-antitrypsin, which inhibits lung serine proteases and protects protein in alveolar walls from degradation; it is approved by the FDA for use in chronic augmentation and maintenance therapy in adults with emphysema due to deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency).
Alpha1-Proteinase Inhibitor (Human) (Prolastin-C®) was first approved in December 1987 to be manufactured by Talecris Biotherapeutics. Its purification process was modified to include solvent treatment and nanofiltration was approved in October 2009, and its name changed from Prolastin® to Prolastin-C®. Since the process modification, manufacturing of Prolastin® has been discontinued.
Alpha1-Proteinase Inhibitor (Human) (Aralast NP®) was first approved in December 2002 to be manufactured by Baxter. Its manufacture and purification process was modified and approved in May 2007, and its name changed from Aralast® to Aralast NP®. Manufacture of Aralast® has since been discontinued.
Alpha1-Proteinase Inhibitor (Human) (Zemaira®) was first approved in July 2003 to be manufactured by Aventis Behring.
Alpha1-Proteinase Inhibitor (Human) (Glassia®) was first approved in July 2010 to be manufactured by Kamada, Ltd.
(NOTE: For Medicare Advantage, please refer to the Medicare Coverage Section below for coverage guidance.)
1. Alpha1-Proteinase Inhibitor (Human) (Prolastin-C®, Aralast NP®, Zemaira®, Glassia®) is considered medically necessary for the following FDA-approved indication:
- Chronic augmentation and maintenance therapy in adults with deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency) with emphysema
2. The following criteria is required to be documented in order for chronic Alpha1-Proteinase Inhibitor replacement therapy to be considered medically necessary for 1 year in selected patients with alpha1-antitrypsin deficiency :
[INFORMATIONAL NOTE: The FDA approved recommended dosage is 60 mg/kg administered once weekly. This dose is intended to increase and maintain a level of functional alpha 1-antitrypsin in the epithelial lining of the lower respiratory tract providing adequate anti-elastase activity in the lung of individuals with alpha 1-antitrypsin deficiency. Administer intravenously at a rate no greater than 0.08 mL/kg/min. The recommended dosage of 60 mg/kg takes approximately 15 minutes to infuse.]
- Member is at least 18 years of age; AND
- Non-smoking patients with alpha1-antitrypsin deficiency and evidence of progressive panacinar emphysema; AND
- Member has a forced expiratory volume in one second (FEV1) in the range of 30%- 65% predicted; AND
- Member has PiZZ, PiZ(null), or Pi(null,null) phenotype or other phenotypes associated with serum concentrations of alpha1-antitrypsin at time of diagnosis less than 11 micromoles/liter (µM/L) or 50 mg/dL measured by nephelometry or 80 mg/dL measured by radial immunodiffusion; AND
- Member’s weight and anticipated weekly dose of 60 mg/kg actual body weight
3. Continued therapy will be considered annually if the following criteria are met:
- Clinical evidence of efficacy as defined as reduction in rate of deterioration of lung function as measured by a decreased forced expiratory volume in 1 second (FEV1) rate of decline; AND
- Member continues to be a non-smoker
4. The use of aerosolized inhaled recombinant Alpha1-Proteinase Inhibitor (Human) is considered investigational in other conditions including, but not limited to, the following:
- Cystic Fibrosis
- Lung disease in patients in whom severe Alpha1-PI deficiency has not been established
- Diabetes mellitus
- ST-Segment Elevation Myocardial Infarction
There is no National Coverage Determination (NCD) or Local Coverage Determination (LCD) for jurisdiction JL for Alpha 1-Proteinase Inhibitors (Human) [Prolastin-C®, Aralast NP®, Zemaira®, Glassia®. Therefore, Medicare Advantage Products will follow the Horizon BCBSNJ medical policy.
Horizon BCBSNJ Medical Policy Development Process:
This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.
Alpha 1-Proteinase Inhibitors (Human) [Prolastin-C®, Aralast NP®, Zemaira®, Glassia®]
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12. Prolastin-C® (Alpha1-Proteinase Inhibitor (Human)). [Prescribing Information]. Research Triangle Park, NC. Talecris Biotherapeutics, Inc., 1/2013.
13. Aralast-NP® (Alpha1-Proteinase Inhibitor (Human)). [Prescribing Information]. Westlake Village, CA. Baxter Healthcare Corporation, March 2014.
14. Zemaira® (Alpha1-Proteinase Inhibitor (Human)). [Prescribing Information]. Kankakee, IL. CSL Behring LLC, September 2015.
15. 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.
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17. Phase II Study of the Safety and Efficacy of Inhaled Alpha-1 Antitrypsin (AAT ) in Cystic Fibrosis Patients. December 2009. [cited 2010 March 4]. Available from: URL: http://clinicaltrials.gov/ct2/show/NCT00499837?term=zemaira&rank=23
18. Griese M, Latzin P, Kappler M, et al. Alpha1-Antitrypsin Inhalation Reduces Airway Inflammation in Cystic Fibrosis Patients. Eur Respir J. 2007 Feb;29(2):240-50. Epub 2006 Oct 18.
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21. Glassia® Prescribing Information. Israel. Kamada Ltd; March 2014.
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23. Stoller JK, et al. Augmentation therapy with α1-antitrypsin: patterns of use and adverse events. Chest 2003;123:1425-34.
24. Gøtzsche PC, Johansen HK. Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease. Cochrane Database of Systematic Reviews 2010, Issue 7. Art. No.: CD007851. DOI: 10.1002/14651858.CD007851.pub2.
25. 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 Vol 168. pp 818–900, 2003.
26. MICROMEDEX® 2.0 (Healthcare Series). DRUGDEX® Evaluations. Alpha-1 Proteinase Inhibitor Human. Available at: http://www.thomsonhc.com. Accessed February 26, 2014.
27. Abbate A, Van Tassell BW, Christopher S, et al. Effects of Prolastin C (Plasma-Derived Alpha-1 Antitrypsin) on the Acute Inflammatory Response in Patients With ST-Segment Elevation Myocardial Infarction (from the VCU-Alpha 1-RT Pilot Study). Am J Cardiol 2015;115(1):8-12.
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29. 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):668-682.
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)
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Medical policies can be highly technical and are designed for use by the Horizon BCBSNJ professional staff in making coverage determinations. Members referring to this policy should discuss it with their treating physician, and should refer to their specific benefit plan for the terms, conditions, limitations and exclusions of their coverage.
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