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Alglucosidase alfa (Lumizyme)

Section: Injections
Effective Date: April 01, 2020

Description

Alglucosidase alfa (Lumizyme®) is an enzyme replacement used for specific indications as a treatment of Pompe disease.

Pompe disease (glycogen storage disease type II, GSDII, glycogenosis type II, acid maltase deficiency) is an inherited disorder of glycogen metabolism caused by the absence or marked deficiency of the lysosomal enzyme GAA.

In the late onset forms infantile-onset form, Pompe disease results in intralysosomal accumulation of glycogen in various tissues, particularly cardiac and skeletal muscles, and hepatic tissues, leading to the development of cardiomyopathy, progressive muscle weakness, and impairment of respiratory function.

In the juvenile and adult-onset forms, intralysosomal accumulation of glycogen is limited primarily to skeletal muscle, resulting in progressive muscle weakness.

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.

Alglucosidase alfa (Lumizyme) may be considered medically necessary in individuals with infant-onset Pompe disease (GAA deficiency) when ONE of the following are met:

  • Confirmed diagnosis of infantile-onset Pompe disease with acid alpha-glucosidase deficiency (GAA) activity in skin fibroblasts of less than 1% of the normal mean (complete deficiency) associated with classic infantile onset Pompe disease; or a partial deficiency (2% to 40% of normal controls) of GAA enzyme activity associated with the non-classic infantile onset and late onset forms; or
  • GAA gene testing.

The use of alglucosidase alfa (Lumizyme) for any other indication is considered experimental/investigational because the safety and/or effectiveness of this service cannot be established by the available published peer-reviewed literature.

Procedure Codes

J0221

Alglucosidase alfa (Lumizyme) may also be considered medically necessary for individuals with juvenile and late-onset Pompe disease (GAA deficiency) when ALL of the following are met:

  • Diagnosis of Pompe disease based on:
    • GAA enzyme assay which shows reduced enzyme activity 2% to 40% partial deficiency of GAA non-classic infantile forms or late onset forms) of the lab specific normal mean value; and
    • Confirmed by ANY ONE of the following:
      • A second GAA enzyme activity assay in a separate sample (from purified lymphocytes or fibroblast [minimally invasive test]; or
      • A muscle biopsy [invasive testing]; or
      • GAA gene testing; and
    • One or more clinical symptoms of Pompe disease are present such as: progressive muscle weakness, respiratory insufficiency is present. (not an all-inclusive list).

The use of alglucosidase alfa (Lumizyme) for any other indication is considered experimental/investigational because the safety and/or effectiveness of this service cannot be established by the available published peer-reviewed literature.

Procedure Codes

J0221

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

E74.02

Links