Agalsidase beta (Fabrazyme)

Section: Injections
Effective Date: October 01, 2019
Revised Date: September 30, 2019


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. 


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 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 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. 

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


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




  1. Title XVIII of the Social Security Act, Section 1862(a)(7).
  2. Title XVIII of the Social Security Act, Section 1862(a)(1)(A).
  3. Title XVIII of the Social Security Act, Section 1833(e).
  4. Fabrazyme® [package insert]. Cambridge, MA: Genzyme Corp. 2017, Revised 12/2018.
  6. Agalsidase. Clinical Pharmacology Compendia. [database online]. Tampa FL: Gold Standard, Inc. 2019.