Skip to main content
HealthPartners

Coverage criteria policies

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 your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

Administrative Process

Drug therapies to replace alpha-1 proteinase inhibitor in individuals with alpha-1 antitrypsin deficiency require prior authorization from Pharmacy Administration. The setting of drug administration will be reviewed as part of the prior authorization.

Note:

For all inquiries, transfer to the Medical Injectable Line (ext 26135).

Coverage

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; and
  4. For commercial products only (does not apply to Medicare or Minnesota Health Care Programs products), medication administration must occur at a clinic office or home-infusion setting unless medical necessity is met based on the criteria below, supported by medical documentation:
    1. The patient has experienced a severe or life-threatening reaction with previous infusions of the same or similar products; or,
    2. The patient has a medical condition that renders him or her unstable, exceptionally complex, immunocompromised or otherwise high-risk such that continued oversight in the current facility is required; or,
    3. There are no alternative settings available to the patient as a result of both of the following:
      1. The patient is unable to use home-infusion services as documented by the physician, social worker, or infusion provider; AND,
      2. The patient is unable to access alternative settings due to unreasonable distance [>30 miles] or other extenuating circumstances.

Initial approvals will be for twelve months.

Annual reauthorizations will require medical chart documentation that the patient has been seen within the past 12 months and that markers of disease are improved by therapy. These include but may not be limited to normalization or improvements in alpha-1 antitrypsin blood levels.

Definitions

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®.

Setting: The type of physical site where the drug is provided. Settings include inpatient hospital, outpatient hospital, clinic office, or home-infusion.

  • Outpatient Hospital sites have physicians and practitioners on-site and are the appropriate site to manage unstable patients and patients experiencing certain moderate to severe adverse events. Hospital settings are typically the highest-cost, most-intensive, and are the highest level settings.
  • Clinic offices are lower level settings which are not outpatient hospital settings that can manage some unstable patients and patients experiencing adverse events. Physicians may or may not be readily available.
  • Home-infusion is a lower level setting, and is performed by a licensed nurse supported by a licensed pharmacy who have expertise in administering complex medications in a patient’s home. Home infusion providers regularly manage mild to moderate adverse events, and are prepared to manage severe adverse events if needed.

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

Codes

Description

J0257

Injection, alpha-1 proteinase inhibitor (human), (Glassia), 10 mg

J0256

Injection, alpha-1 proteinase inhibitor, human, 10 mg, not otherwise specified. (Aralast NP, Prolastin-C, Zemaira)

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

Codes

Description

00053720102

Alpha-1 Proteinase Inhibitor Human, Zemaira

00944288401

Alpha-1 Proteinase Inhibitor Human, Glassia

00944281401

Alpha-1 Proteinase Inhibitor Human, Aralast NP

00944281501

Alpha-1 Proteinase Inhibitor Human, Aralast NP

13533070001

Alpha-1 Proteinase Inhibitor Human, Prolastin-C

13533070002

Alpha-1 Proteinase Inhibitor Human, Prolastin-C

13533070101

Alpha-1 Proteinase Inhibitor Human, Prolastin-C

13533070310

Alpha-1 Proteinase Inhibitor Human, Prolastin-C

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

Products

This information is for most, but not all, HealthPartners 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. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.

References

  1. Prolastin/C Prescribing Information. Grifols Therapeutics, Inc., Research Triangle Park, NC. August 2016.
  2. Aralast/NP Prescribing Information. Baxter Healthcare Corporation, Westlake Village, CA. March 2014
  3. Glassia Prescribing Information. Baxalta US Inc. Westlake Village, CA June 2016
  4. Zemaira Prescribing Information. CSL Behring LLC, Kankakee, IL. September 2015.
  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.
  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.

Go to

Policy activity

  • 07/01/2011 - Date of origin
  • 04/27/2017 - Effective date
Review date
  • 11/2016
Revision date
  • 10/01/2016

Related content