Alpha-1 antitrypsin (AAT) deficiency enzyme replacement therapy: alpha-1 proteinase inhibitor (Aralast/NP, Glassia®, Prolastin/C and Zemaira®)

These services may or may not be covered by all CoOportunity Health plans. Please see your plan documents for your own coverage information. If there is a difference between this general information and your plan documents, your plan will be used to determine your coverage.

Administrative process

Drug therapies to replace alpha 1-proteinase inhibitor in individuals with alpha-1 antitrypsin deficiency are not covered without prior authorization from Pharmacy Administration.

Coverage for drug therapy in alpha-1 antitrypsin deficiency is generally provided when medically necessary.

Alpha-1 antitrypsin (AAT) inhibitor therapy is medically necessary for members with emphysema due to AAT deficiency when all the following criteria are met:

1. Low serum concentration of AAT < 11 micromoles/L (or less than 80mg/dL or less than 0.8 g/L), which is considered the threshold thought to protect against emphysema; and
2. Progressive panacinar emphysema is present with a documented rate of decline in forced expiratory volume in 1 second (FEV1); and
3. Ex-smoker or non-smoker.

Alpha-1 antitrypsin deficiency: Alpha-1 antitrypsin is an antiprotease found in human plasma that inhibits the neutrophil elastase enzyme from degrading elastin tissues in the lung. Deficiency is an inherited disorder associated with the early onset of pulmonary emphysema in adults. Although replacement therapies have not been shown to prevent or reverse emphysema in AAT deficiency, population studies suggest a minimum plasma threshold of 11 micromoles/L, below which there is insufficient AAT to protect the lung leading to emphysema.

Alpha-1 proteinase inhibitor therapy is indicated for chronic augmentation and maintenance therapy in adults with emphysema due to congenital deficiency of alpha1-proteinase inhibitor. The effect of augmentation therapy on pulmonary exacerbations and on the progression of emphysema in alpha1-deficiency has not been demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation and maintenance therapy of individuals is not available. It is not indicated as therapy for lung disease in patients in whom severe alpha1-proteinase deficiency has not been established.

Currently available alpha-1 proteinase inhibitor products include Aralast NP®, Glassia®, Prolastin®- C and Zemaira®.

If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

HCPCS Codes

J0257 – Injection, alpha-1 proteinase inhibitor (human), (Glassia), 10 mg
J0256 – Injection, alpha-1 proteinase inhibitor, human, 10 mg, not otherwise specified.
S9346 - Home infusion therapy, alpha-1-proteinase inhibitor (e.g., Prolastin); administrative services,
professional pharmacy services, care coordination and all necessary supplies and equipment
(drugs and nursing visits coded separately), per diem

NDC Codes

0944280201 - Alpha-1 Proteinase Inhibitor Human, Aralast NP
0944280202 - Alpha-1 Proteinase Inhibitor Human, Aralast NP
0944281201 - Alpha-1 Proteinase Inhibitor Human, Aralast NP
0944281401 - Alpha-1 Proteinase Inhibitor Human, Aralast NP
0944281501 - Alpha-1 Proteinase Inhibitor Human, Aralast NP
0944280202 - Alpha-1 Proteinase Inhibitor Human, Aralast NP
13533070001 - Alpha-1 Proteinase Inhibitor Human, Prolastin-C
13533070002 - Alpha-1 Proteinase Inhibitor Human, Prolastin-C
13533070011 - Alpha-1 Proteinase Inhibitor Human, Prolastin-C
00053720102 - Alpha-1 Proteinase Inhibitor Human, Zemaira
00944288401 - Alpha-1 Proteinase Inhibitor Human, Glassia

CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

References

1. Prolastin/C Prescribing Information. Grifols Therapeutics, Inc., Research Triangle Park, NC. November 2013.
2. Aralast/NP and Glassia Prescribing Information. Baxter Healthcare Corporation, Westlake Village, CA. March 2014.
3. Zemaira Prescribing Information. CSL Behring LLC, Kankakee, IL. May 2013.
4. Glassia FDA Review. www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/
   ApprovedProducts/LicensedProductsBLAs/ FractionatedPlasmaProducts/ucm217877.htm (Accessed 10/11/10)
5. Gotzsche PC, Johansen HK. Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease (Review). The Cochrane Collaboration.2010:7. www.thecochranelibrary.com (Accessed 11/11/10).
6. American Thoracic Society/European Respiratory Soceity Standards document for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003;168:818.
7. Abboud RT, Ford GT, and Chapman KR. Alpha1-antitrypsin deficiency: a position statement of the Canadian Thoracic Society. Can Respir J 2001 Mar-Apr;8(2):81-8.

This information is for most, but not all, CoOportunity Health plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

CoOportunity Health has contracted with HealthPartners Administrators, Inc. to provide claims processing, medical management and certain other administrative services.