Notification



Notification Issue Date:



Medical Policy Bulletin


Title:Alpha 1-Antitrypsin Therapy (e.g., Prolastin-C®, Aralast NP™, Glassia™, Zemaira™)

Policy #:08.00.91c

This policy is applicable to the Company’s commercial products only. Policies that are applicable to the Company’s Medicare Advantage products are accessible via a separate Medicare Advantage policy database.


The Company makes decisions on coverage based on Policy Bulletins, benefit plan documents, and the member’s medical history and condition. Benefits may vary based on contract, and individual member benefits must be verified. The Company determines medical necessity only if the benefit exists and no contract exclusions are applicable.

When services can be administered in various settings, the Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member’s medical needs and condition. This decision is based on the member’s current medical condition and any required monitoring or additional services that may coincide with the delivery of this service.

This Medical Policy Bulletin document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy Bulletin will be reviewed regularly and be updated as scientific and medical literature becomes available. For more information on how Medical Policy Bulletins are developed, go to the About This Site section of this Medical Policy Web site.



Policy

Coverage is subject to the terms, conditions, and limitations of the member's contract.

MEDICALLY NECESSARY

Alpha 1-antitrypsin therapy (e.g., Prolastin-C®, Aralast NP, Glassia, Zemaira) is considered medically necessary and, therefore, covered for individuals who have alpha 1-antitrypsin deficiency and clinical evidence of chronic emphysema without evidence of alpha 1-antitrypsin--associated liver disease, when all of the following criteria are met:
  • The individual has a low serum concentration of alpha 1-antitrypsin (AAT) less than 80 mg/dL or less than 11 uM/L or less than 0.8 g/L (35 percent of normal), which is considered the threshold thought to protect against emphysema.
  • The individual has progressive panacinar emphysema with a documented rate of decline in forced expiratory volume in 1 second (FEV1).
  • The individual is a non-smoker.


EXPERIMENTAL/INVESTIGATIONAL

All other uses of alpha 1-antitrypsin therapy (e.g., Prolastin-C®, Aralast NP , Glassia, Zemaira) are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics.

REQUIRED DOCUMENTATION

The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the drug.
Guidelines

BENEFIT APPLICATION

Subject to the terms and conditions of the applicable benefit contract, alpha 1-antitrypsin therapy (e.g., Prolastin-C®, Aralast NP, Glassia, Zemaira) is covered under the medical benefits of the Company’s products when the medical necessity criteria listed in this medical policy is met.

For Medicare Advantage members, certain drugs are available through either the member's medical benefit (Part B benefit) or pharmacy benefit (Part D benefit), depending on how the drug is prescribed, dispensed, or administered. This medical policy only addresses instances when alpha 1-antitrypsin therapy (e.g., Prolastin-C®, Aralast NP, Glassia, Zemaira) is covered under a member's medical benefit (Part B benefit). It does not address instances when alpha 1-antitrypsin therapy (e.g., Prolastin-C®, Aralast NP, Glassia, Zemaira) is covered under a member’s pharmacy benefit (Part D benefit).

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

Alpha 1-proteinase inhibitor (Prolastin-C®) was approved by the FDA on October 16, 2009, for the treatment of individuals with alpha 1-antitrypsin deficiency and evidence of emphysema.

Alpha 1-proteinase inhibitor (Aralast NP) was approved by the FDA on May 4, 2007, for chronic augmentation therapy in patients having alpha 1-antitrypsin deficiency with clinically evident emphysema.

Alpha 1-proteinase inhibitor (Glassia) was approved by the FDA on July 1, 2010, and is indicated for chronic augmentation and maintenance therapy in adults with emphysema.

Alpha 1-proteinase inhibitor (Zemaira) was approved by the FDA on July 8, 2003, for treatment of individuals with deficiency and evidence of emphysema.

PEDIATRIC USE

The safety and effectiveness in the pediatric population have not been established.

Description

Alpha 1-antitrypsin (also known as congenital alpha 1-proteinase inhibitor) deficiency is an autosomal, codominant genetic disorder, differentiated by a deficient serum and lung concentrations of alpha 1-antitrypsin. A deficiency in alpha 1-antitrypsin leaves the neutrophil elastase uninhibited. Uncontrolled neutrophil elastase leads to progressive destruction of the pulmonary connective tissue and loss of the alveoli.

Some individuals with certain phenotypic variants have an increased risk of developing progressive emphysema. Individuals with the PiZZ variant typically have a serum alpha 1-antitrypsin levels less than 35% of the average normal. Ninety-five percent of clinically symptomatic alpha 1-antitrypsin deficient individuals are PiZZ phenotype. Individuals with Pi(null)(null) are associated with undetectable serum alpha 1-antitrypsin levels or levels less than 1 percent of the normal amount. Individuals with these low serum alpha 1-antitrypsin levels have a markedly increased risk of developing emphysema over their lifetime. In addition, PiSZ individuals, whose serum alpha 1-antitrypsin levels range from approximately 9 to 23 uM, are considered to have moderately increased risk of developing emphysema.

Alpha-1 Antitrypsin (alpha proteinase inhibitor) is used as a replacement therapy for individuals with severe alpha 1-antitrypsin deficiency and clinical evidence of emphysema. Several biological drugs have been approved by the US Food and Drug Administration (FDA) for alpha 1-antitrypsin therapy, with orphan drug status. Prolastin®, Aralast NP, Glassia, and Zemaira were approved by the FDA for the treatment of individuals with alpha 1-antitrypsin deficiency and evidence of emphysema. Prolastin® was replaced by Prolastin-C® in spring 2010.

Alpha 1-antitrypsin therapy (e.g., Prolastin-C®, Aralast NP, Glassia, Zemaira) uses highly purified human alpha 1-antitrypsin derived from human plasma. Studies comparing alpha 1-antitrypsin preparations involved a limited number of participants with alpha 1-antitrypsin deficiency and emphysema. All preparations produced similar increases in the serum alpha 1-antitrypsin concentration and antigenic alpha 1-antitrypsin activity in lung epithelial lining fluid. Data from cohort studies, although limited, indicate that such replacement therapy is associated with a lower rate of decline of forced expiratory volume, thus protecting the lung tissue from further destruction. Safety and effectiveness have not been established in pediatric individuals.

There may be additional indications contained in the Policy section of this document due to evaluation of criteria highlighted in the Company’s off-label policy, and/or review of clinical guidelines issued by leading professional organizations and government entities.
References


Alpha 1-Proteinase Inhibitor (Human) (Aralast NP). [Prescribing Information]. Westlake Village, CA: Baxter Healthcare Corporation. 2014. Updated 3/2014. Available at: http://www.baxter.com/downloads/healthcare_professionals/products/Aralast_NP_PI.pdf. Accessed September 22, 2016.

Alpha 1-Proteinase Inhibitor (Human) (Glassia). Prescribing Information]. Westlake Village, CA: Baxter Healthcare Corporation. 2014. Updated 06/2016. Available at: http://www.baxter.com/downloads/healthcare_professionals/products/Glassia_PI.pdf. Accessed September 22, 2016.

Alpha-1 Proteinase Inhibitor Human. Micromedex® Healthcare Series. DrugDex®. [Micromedex® Web site]. 05/30/14. Available at: http://www.thomsonhc.com/micromedex2/librarian [via subscription only]. Accessed September 22, 2016.

Alpha 1-Proteinase Inhibitor (Human) (Prolastin-C). [Prescribing Information]. Research Triangle Park, NC: Grifolis Therapeutics Inc. Updated 6/2016. Available at: http://www.grifols-pi.info/inserts/Prolastin-C.pdf. Accessed September 22, 2016.

Alpha 1-Proteinase Inhibitor (Human) (Zemaira). [Prescribing Information]. Kanakakee, IL: CSL Behring LLC. 2013. Updated 09/2015. Available at: http://labeling.cslbehring.com/PI/US/Zemaira/EN/Zemaira-Prescribing-Information.pdf. Accessed September 22, 2016.

American Hospital Formulary Service (AHFS). Drug Information 2014. Alpha 1-Proteinase Inhibitor (Human). [STAT!Ref Web site]. 07/08/2014. Available at: http://online.statref.com. [via subscription only]. Accessed September 22, 2016.

American Thoracic Society, et. al. 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.

Elsevier Gold Standard’s Clinical Pharmacology Compendium. Alpha-1-proteinase Inhibitor. [MD Consult Web site]. 07/19/2011. Available at: http://www.mdconsult.com/das/pharm/body/457431238-3/1537184989/full/1183. [via subscription only]. Accessed September 22, 2016.

Marciniuk, DD, et al. Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: A Canadian Thoracic Society clinical practice guideline. Can Resp J. 2012;19(2):109-116.

Stockley, R. Alpha 1-antitrypsin review. Clin Chest Med. 2014;35:39-50.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Approval letter. Aralast NP. [FDA Web site]. 5/4/2007. Available at: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm052477.htm. Accessed on September 22, 2016.

US Food and Drug Administration (FDA). Center for Biologics Evaluation and Research. Approval letter. Zemaira. [FDA Web site]. 07/08/08. Available at: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm055333.htm. Accessed on September 22, 2016.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Premarket approval letter. Glassia. [FDA Web site]. 7/1/10. Available at: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/LicensedProductsBLAs/FractionatedPlasmaProducts/ucm217888.htm Accessed on September 22, 2016.





Coding

Inclusion of a code in this table does not imply reimbursement. Eligibility, benefits, limitations, exclusions, precertification/referral requirements, provider contracts, and Company policies apply.

The codes listed below are updated on a regular basis, in accordance with nationally accepted coding guidelines. Therefore, this policy applies to any and all future applicable coding changes, revisions, or updates.

In order to ensure optimal reimbursement, all health care services, devices, and pharmaceuticals should be reported using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company.

The Coding Table lists any CPT, ICD-9, ICD-10, and HCPCS billing codes related only to the specific policy in which they appear.

CPT Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD - 10 Procedure Code Number(s)

N/A


Professional and outpatient claims with a date of service on or before September 30, 2015, must be billed using ICD-9 codes. Professional and outpatient claims with a date of service on or after October 1, 2015, must be billed using ICD-10 codes.

Facility/Institutional inpatient claims with a date of discharge on or before September 30, 2015, must be billed with ICD-9 codes. Facility/Institutional inpatient claims with a date of discharge on or after October 1, 2015, must be billed with ICD-10 codes.


ICD -10 Diagnosis Code Number(s)

E88.01 Alpha-1-antitrypsin deficiency

J43.1 Panlobular emphysema

J43.8 Other emphysema




HCPCS Level II Code Number(s)



J0256 Injection, alpha 1-proteinase inhibitor (human), not otherwise specified, 10MG

J0257 Injection, alpha 1-proteinase inhibitor (human), (Glassia), 10MG

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



Revenue Code Number(s)

N/A

Coding and Billing Requirements


Cross References


Policy History

Revisions from 08.00.91c
09/26/2018This policy has been reissued in accordance with the Company's annual review process.


Effective 10/05/2017 this policy has been updated to the new policy template format.


Version Effective Date: 10/08/2014
Version Issued Date: 10/08/2014
Version Reissued Date: 09/26/2018

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