Last Review Date: 04/06/2021

Date of Origin:  01/01/2012

Dates Reviewed:  12/2011, 02/2013, 08/2013, 06/2014, 06/2015, 01/2016, 10/2016, 03/2017, 09/2017, 12/2017, 03/2018, 06/2018, 04/2019, 04/2020, 04/2021

1. Length of Authorization

Coverage will be provided for 12 months and may be renewed.

2. Dosing Limits

A.  Quantity Limit (max daily dose) [NDC Unit]:

• Aralast NP 1 g/50 mL: 7 vials per week
• Aralast NP 0.5 g/25 mL: 1 vial per week
• Glassia 1 g/50 mL: 7 vials per week
• Prolastin-C 1 g/20 mL: 7 vials per week
• Prolastin-C Liquid 1g/20 mL: 7 vials per week
• Zemaira 1 g/20 mL: 3 vials per week
• Zemaira 4 g/76 mL: 1 vial per week
• Zemaira 5 g/95 mL: 1 vial per week

B.  Max Units (per dose and over time) [HCPCS Unit]:

• 700 billable units every 7 days

3. Initial Approval Criteria^1-6,8,9,12

• Patient is at least 18 years of age; AND

Universal Criteria

• Patient is not a tobacco smoker; AND
• Patient is receiving optimal medical therapy (e.g., comprehensive case management, pulmonary rehabilitation, vaccinations, smoking cessation, self-management skills, etc.); AND
• Patient does not have immunoglobulin-A (IgA) deficiency with antibodies against IgA; AND

Emphysema due to alpha-1-antitrypsin (AAT) deficiency †, Ф (applies only to Prolastin-C)

• Patient has an FEV₁ in the range of 30-65% of predicted; AND
• Patient has alpha-1-antitrypsin (AAT) deficiency with PiZZ, PiZ (null), or Pi (null, null) phenotypes; AND
• Patient has AAT-deficiency and clinical evidence of panacinar/panlobular emphysema; AND
• Patient has low serum concentration of AAT ≤ 57 mg/dL or ≤ 11 µM/L as measured by nephelometry

† FDA Approved Indication(s); Ф Orphan Drug

4. Renewal Criteria^1-6,8,9

Authorizations can be renewed based on the following criteria:

• Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
• Disease response with treatment as defined by elevation of AAT levels above baseline, substantial reduction in rate of deterioration of lung function as measured by percent predicted FEV₁, or improvement in CT scan lung density; AND
• Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include severe hypersensitivity reactions, etc.

5. Dosage/Administration^1-5

6. Billing Code/Availability Information

HCPCS Code & NDC:

7. References

1. Glassia [package insert]. Lexington, MA; Baxalta US Inc.; June 2017. Accessed March 2021.
2. Zemaira [package insert]. Kankakee, IL; CSL Behring LLC; April 2019. Accessed March 2021.
3. Aralast NP [package insert]. Lexington, MA; Baxalta US Inc.; December 2018. Accessed March 2021.
4. Prolastin-C Liquid [package insert]. Research Triangle Park, NC; Grifols Therapeutics, Inc.; August 2018. Accessed March 2021.
5. Prolastin-C [package insert]. Research Triangle Park, NC; Grifols Therapeutics, Inc.; June 2018. Accessed March 2021.
6. American Thoracic Society/European Respiratory Society Statement: Standards for the Diagnosis and Management of Individuals with Alpha-1 Antitrypsin Deficiency. American Thoracic Society; European Respiratory Society. Am J Respir Crit Care Med. 2003 Oct 1;168(7):818-900.
7. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. Global Initiative for Chronic Obstructive Lung Disease (GOLD); 2019.
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 (Miami). 2016; 3(3):668-682.
9. 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(2):109-16.
10. Stocks JM, Brantly M, Pollock D, et al. Multi-center study: the biochemical efficacy, safety and tolerability of a new α1-proteinase inhibitor, Zemaira. COPD. 2006;3:17–23.
11. Global Initiative for Chronic Obstructive Lung Disease (GOLD). Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. Global Initiative for Chronic Obstructive Lung Disease (GOLD); 2020.
12. Miravitlles M, Dirksen A, Ferrarotti I, et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency. Eur Respir J 2017; 50.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD), Local Coverage Determinations (LCDs), and Local Coverage Articles (LCAs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A