Agalsidase beta (Fabrazyme)

Policy ID: I-55-020

Section: Injections

Effective Date: April 01, 2019

Revised Date: November 17, 2020

Revision Effective Date: January 01, 2021

Last Reviewed: November 19, 2020

Description

Agalsidase beta (Fabrazyme^®) serves as an exogenous source of the lysosomal enzyme α-glucosidase A (a-GalA), catalyzing the hydrosis of glycosphinogolipids, includingglobotriaosylceramide (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 for the treatment of Fabry disease when ALL of 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: clinical 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
ONE or more of the following symptoms or physical findings attributable to Fabry disease:
- 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
- Anhydrosis or hypohidrosis (impaired sweating); 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.

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

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

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.

Section: Injections

Effective Date: October 01, 2019

Revised Date: September 30, 2019

Archived Date: December 31, 2020

Description

Agalsidase beta (Fabrazyme) serves as an exogenous source of the lysosomal enzyme 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. 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.

Agalsidase beta (Fabrazyme) may be considered medically necessary for the treatment of Fabry disease when ALL of 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: clinical documentation of complete deficiency or negligible (less than 5%) of mean normal alpha-galactosidase A (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
ONE or more of the following symptoms or physical findings attributable to Fabry disease:
- 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.
- Anhydrosis or hypohidrosis (impaired sweating); 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.

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. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.

Diagnosis Codes

E75.21

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