Pompe Disease Enzyme Replacement Therapy: Alglucosidase alfa (Lumizyme®) - Minnesota Health Care Programs
These services may or may not be covered by your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.
Treatment with alglucosidase alfa requires prior authorization from Pharmacy Administration.
Enzyme replacement for Pompe disease is generally covered when:
1. Prescribed by a specialist; and
2. Prescribed for patients with Pompe disease (GAA deficiency). The approved regimen is 20 mg/kg of body weight administered every 2 weeks as an intravenous infusion. A current weight is required; and
3. The patient and/or guardian has attested that they will adhere to the treatment plan; and
Initial approvals will be for twelve months.
Annual reauthorizations will require medical chart documentation that the patient has been seen within the past 12 months and that markers of disease are improved by therapy.
Pompe disease is a progressive, multisystemic, debilitating, and often fatal neuromuscular disorder. It results from an inherited deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA) (also called acid maltase), which is responsible for the breakdown of glycogen to glucose. The result is intralysosomal accumulation of glycogen, primary in muscle cells, that leads to a progressive loss of muscle function.
Pompe disease is also referred to as:
- Acid maltase deficiency (AMD)
- Glycogen storage disease (GSD) type II
- Glycogenosis type II
- Acid alpha-glucosidase (GAA) deficiency
- Lysosomal alpha-glucosidase deficiency
Infantile form: Symptoms of cardiomyopathy and a generalized lack of muscle tone generally appear during the first few months of life. Without treatment, most patients with classic infantile form deteriorate with death during the first one to two years of life from cardiac insufficiency due to cardiac muscle dysfunction.
Late-onset: May be present at any age. The primary symptom is skeletal muscle myopathy. Over time this results in progressive muscle weakness and death usually related to respiratory failure.
Lumizyme is a hydrolytic lysosomal glycogen-specific enzyme indicated for patients with Pompe disease (GAA deficiency).
If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.
Injection, alglucosidase alfa, (Lumizyme), 10 mg
Lumizyme single use 50 mg solution
Lumizyme 10 vial package of 50mg
CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.
This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.
- Lumizyme Prescribing Information Genzyme Corporation, Inc., Cambridge, MA. August 2014.
- van der Ploeg AT, Clemens PR, Corzo D, et al., A randomized study of alglusidase alfa in late-onset Pompe’s disease. N Eng J Med 2010;362:1396-406.