State and School Employees' Health Insurance Plan - Alpha-1 Proteinase Inhibitors

POLICY NUMBER

S.5.01.528

Aralast NP [alpha-1-proteinase inhibitor (human)]

Glassia [alpha-1-proteinase inhibitor (human)]

Prolastin-C [alpha-1-proteinase inhibitor (human)]

Zemaira [alpha-1-Proteinase Inhibitor (human)]

 Please perform a search of the State Health Plan Medical Drug Formulary for drugs administered and billed through the medical setting.

DESCRIPTION

Alpha-1 antitrypsin deficiency is an inherited disorder that may cause lung and liver disease. The deficiency occurs worldwide but its prevalence varies by population. It is estimated that Alpha-1 antitrypsin deficiency ranges from about 1 in every 1,600 to about 1 in every 5,000 people. Alpha-1 antitrypsin, also called AAT, is a protein made in the liver. Normally, the protein travels through the bloodstream to protect organs from the harmful effects of other proteins. AAT deficiency occurs if the AAT proteins made in the liver are not the right shape and cannot get into the bloodstream. Because not enough AAT proteins travel to the lungs to protect them, this increases the risk of lung disease. People with alpha-1 antitrypsin usually develop the first signs and symptoms of lung disease between ages 20 and 50. Many people are likely undiagnosed, particularly people with a lung condition called chronic obstructive pulmonary disease (COPD). Some people with alpha-1 antitrypsin deficiency are misdiagnosed with asthma. Augmentation therapy is a treatment used only for people who have AAT-related lung disease. This therapy involves getting infusions of the AAT protein which raises the level of the protein in your blood and lungs.

Aralast NP [alpha-1-proteinase inhibitor (human)] is indicated for chronic augmentation therapy in adults with clinically evident emphysema due to severe congenital deficiency of Alpha₁-PI (alpha₁-antitrypsin deficiency).

Glassia [alpha-1-proteinase inhibitor (human)] is indicated for chronic augmentation and maintenance therapy in individuals with clinically evident emphysema due to severe hereditary deficiency of Alpha₁-PI (alpha₁-antitrypsin deficiency).

Prolastin-C [alpha-1-proteinase inhibitor (human)] is indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of Alpha1-PI (alpha1-antitrypsin deficiency).

Zemaira [alpha-1-proteinase inhibitor (human)] is indicated for chronic augmentation and maintenance therapy in adults with A₁-PI deficiency and clinical evidence of emphysema.

POLICY

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements.

Initial Review

The requested medication may be considered medically necessary when ALL of the following criteria are met:

1. The individual is 18 years of age or older;

2. The individual has a diagnosis of alpha-1 antitrypsin deficiency (AATD) with clinically evident emphysema;

3. The individual has a pre-treatment serum alpha-1 antitrypsin level less than 11µM/L (80 mg/dL by immunodiffusion or 57 mg/dL using nephelometry);

4. The individual does NOT have PI*MZ genotype (documentation required);

5. The individual does NOT have IgA deficiency (<7 mg/dL) with serum antibodies against IgA;

6. ONE of the following

   1. The individual has a baseline FEV1 of 65% or less of predicted; OR

   2. The individual has a baseline FEV1 greater than 65% but has experienced a rapid decline in lung function (e.g. change in FEV1 greater than 120 mL/year);

7. The individual is a non-smoker or participating in a smoking cessation program; 

8. The individual is currently on and will continue conventional COPD treatment (e.g., bronchodilators, supplemental oxygen, preventative vaccinations);

9. The prescriber is a specialist or has consulted with a specialist in the area of the individual’s diagnosis (e.g. pulmonologist);

10. The individual does not have any contraindication(s) to therapy with the requested agent; AND

11. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

Renewal Criteria

The requested medication may be approved for continuation when ALL of the following criteria are met:

1. The individual has previously been approved for the requested medication through the Blue Cross & Blue Shield of Mississippi review process. 

2. The individual has documented clinical improvement (i.e., slowing of disease progression or decrease in symptom severity and/or frequency);

3. The individual continues to be a non-smoker or is participating in a smoking cessation program;

4. The prescriber is a specialist or has consulted with a specialist in the area of the individual’s diagnosis (e.g. pulmonologist);

5. The individual does not have any contraindication(s) to therapy with the requested agent; AND

6. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary.

POLICY EXCEPTIONS

None

POLICY GUIDELINES

Medical Policy Manual coverage guidelines should not be used in lieu of the Participant's specific benefit plan language outlined in the Mississippi's State and School Employees’ Life and Health Insurance Plan.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Participant's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A. consistent with the symptoms or diagnosis and treatment of the Participant's condition, illness, or injury; and

B. appropriate with regard to standards of good medical practice; and

C. not solely for the convenience of the Participant, his or her Provider; and

D. the most appropriate supply or level of care which can safely be provided to the Participant. When applied to the care of an Inpatient, it further means that services for the Participant's medical symptoms or conditions require that the services cannot be safely provided to the Participant as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

POLICY HISTORY

07/01/2023: New policy added.

03/20/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding Glassia [alpha-1-proteinase inhibitor (human)]. Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Medically necessary criteria updated regarding age requirement, PI*MZ genotype, baseline FEV1, COPD treatment, and contraindications. Renewal criteria updated regarding clinical improvement and contraindications. Sources updated.

SOURCE(S)

1. American Thoracic Society; European Respiratory Society. 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 Oct 1;168(7):818-900. doi: 10.1164/rccm.168.7.818. PMID: 14522813.

2. Aralast NP prescribing information. Takeda Pharmaceuticals America, Inc. November 2024. Last accessed January 2025.

3. Glassia prescribing information. Takeda Pharmaceuticals America, Inc. November 2024. Last accessed January 2025.

4. Global Strategy for the Diagnosis, Management and Prevention of COPD, Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2017. Available from:

   http://www.goldcopd.org

   .

5. https://ghr.nlm.nih.gov/condition/alpha-1-antitrypsin-deficiency

6. https://www.nhlbi.nih.gov/health-topics/alpha-1-antitrypsin-deficiency

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(2):109-116.

8. Prolastin-C Liquid prescribing information. Grifols USA LLC. July 2022. Last accessed January 2025.

9. Prolastin-C prescribing information. Grifols USA, LLC. February 2022. Last accessed January 2025.

10. 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. doi: http://doi.org/10.15326/jcopdf.3.3.2015.0182

11. Stoller JK. Treatment of emphysema from alpha-1 antitrypsin deficiency. UpToDate, Connor RF (Ed), Wolters Kluwer. https://www.uptodate.com/contents/treatment-of-emphysema-from-alpha-1-antitrypsin-deficiency . Accessed on January 9, 2025.

12. Zemaira prescribing information. CSL Behring LLC. September 2024. Last accessed January 2025.

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Medically Necessary Codes

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