Agalsidase beta (Fabrazyme)

Policy ID: I-55-022

Section: Injections

Effective Date: April 01, 2019

Revised Date: February 25, 2021

Revision Effective Date: May 01, 2021

Last Reviewed: March 17, 2021

Description

Agalsidase beta (Fabrazyme^®) is an exogenous source of the lysosomal enzyme α-glucosidase A (a-GalA), catalyzing the hydrosis of glycosphinogolipids, including globotriaosylceramide (GL-3), hence reducing its deposition in capillary endothelium of the kidney, heart, brain and other tissue types. α-glucosidase A deficiency, otherwise known as Fabry Disease, is an X- linked genetic disorder of glycosphingolipid metabolism. Deficiency of a-Gal leads to progressive accumulation of glycoshingolipids, predominantly GL-3, in many body tissues, occurring over a period of years. Clinical manifestations of the disease include renal failure, cardiomyopathy and cerebrovascular accidents.

Criteria

Coverage is subject to the specific terms of the member's benefit plan.

Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.

Agalsidase beta (Fabrazyme) may be considered medically necessary when the following criteria are met:

Individuals eight (8) years of age or older with a confirmed diagnosis of Fabry disease made by ONE of the following methods:
- For male individuals: documentation of complete deficiency or less than 1% of mean normal alpha-galactosidase A (α-Gal A) enzyme activity in leukocytes, dried blood spots, or serum (plasma) analysis; or
- For female individuals: documented galactosidase alpha gene; (GLA) mutation by gene sequencing; and
At least one of the following signs and symptoms:
- Angiokeratomas; (clusters of small, dark red spots on the skin); or
- Acroparesthesias; (episodes of pain, particularly in the hands and feet); or
- Corneal verticillata (whorls); or
- Corneal opacity; or
- Personal or family history of exercise, heat, or cold intolerance; or
- Decreased sweating (anhidrosis or hypohidrosis); or
- Personal or family history of renal failure; or
- Hearing manifestations (tinnitus); and
The medication is prescribed by or in consultation with a physician who specializes in the treatment of inherited metabolic disorders; and
Individual will not concomitantly be treated with migalastat.

Reauthorization Criteria

Reauthorization of agalsidase beta (Fabrazyme) may be considered medically necessary when the following criteria are met:

Individual diagnosed with Fabry disease; and
Provider attestation that individual has demonstrated disease stability or beneficial response to therapy; and
Individual will not concomitantly be treated with migalastat.

Agalsidase beta (Fabrazyme) for any other indication is considered experimental/investigational, and therefore, non-covered. Scientific evidence does not support the use of Agalsidase beta (Fabrazyme) for other indications.

Procedure Codes

J0180

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines.

Diagnosis Codes

E75.21

Professional Statements and Societal Positions Guidelines

Not Applicable

ND Committee Review

Internal Medical Policy Committee 11-19-2020 Updated diagnosis codes

Internal Medical Policy Committee 1-19-2021 Updated physical findings criteria to read signs and symptoms, removed "Anhydrosis or hypohidrosis (impaired sweating)" from this section

Internal Medical Policy Committee 3-17-2021 Added criteria "Individual will not concomitantly be treated with migalastat" and reauthorization criteria

Links

References (PDF)

Disclaimer

Current medical policy is to be used in determining a Member's contract benefits on the date that services are rendered. Contract language, including definitions and specific inclusions/exclusions, as well as state and federal law, must be considered in determining eligibility for coverage. Members must consult their applicable benefit plans or contact a Member Services representative for specific coverage information. Likewise, medical policy, which addresses the issue(s) in any specific case, should be considered before utilizing medical opinion in adjudication. Medical technology is constantly evolving and the Company reserves the right to review and update medical policy periodically.

Policy ID: I-55-021