Find policies, billing codes, payers, states, and providers
Lumizyme (alglucosidase alfa) — Pompe Disease (acid alpha‑glucosidase deficiency)
Customize your policy alerts
Sign up for Cigna Policy IP0440 alerts
Get alerted when Policy IP0440 changes without checking for updates manually.
Monitor payer policy activity
Defines Cigna's prior authorization, documentation, and clinical criteria for coverage of Lumizyme for patients with Pompe disease (acid alpha-glucosidase deficiency) for health benefit plans administered by Cigna Companies.
No material clinical or coverage changes in this revision.
Coverage Criteria for Lumizyme (alglucosidase alfa)
FDA-Approved Indication: Acid Alpha-Glucosidase Deficiency (Pompe Disease)
Covered when ALL of the following are met:
A) Diagnostic confirmation
- (i): Laboratory test demonstrating deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue
Documentation required
- (ii): Molecular genetic test demonstrating biallelic pathogenic or likely pathogenic acid alpha-glucosidase (GAA) gene variants
Documentation required
Requests for doses outside established dosing considered case-by-case
Lumizyme for any other use is considered not medically necessary.
Any use of Lumizyme outside the listed FDA-approved indication/criteria is considered not medically necessary.
Diagnostic Confirmation and Covered Indications
Molecular genetic testing documenting biallelic pathogenic or likely pathogenic GAA gene variants to establish diagnosis of Pompe disease.
Genetic test results are accepted as diagnostic confirmation when biallelic pathogenic/likely pathogenic variants are identified; documentation required.
Coding and Dosing Information
| J0221 | Injection, alglucosidase alfa, (Lumizyme), 10 mg |
Provider Requirements, Authorization, and Documentation
Prior authorization required
Prior authorization is required for benefit coverage of Lumizyme. Approval is recommended for patients who meet the policy Criteria and Dosing. Initial approvals are for 1 year; extended approvals are allowed if the patient continues to meet the Criteria and Dosing. Requests for doses outside the dosing documented in this policy will be considered case-by-case by a clinician.
- Prior authorization required for benefit coverage.
- Approvals recommended when Criteria and Dosing are met.
- Initial approval duration: 1 year; extensions allowed if criteria continue to be met.
Prescriber consultation requirement
Lumizyme must be prescribed by or in consultation with a geneticist, neurologist, a metabolic disorder sub‑specialist, or a physician who specializes in the treatment of lysosomal storage disorders.
- Prescriber requirement is part of the approval criteria and must be documented.
Required documentation
Documentation is required where noted in the criteria and must be patient‑specific; accepted documentation may include chart notes, laboratory tests, claims records, prescription receipts, and genetic test results. Proof of deficient acid alpha‑glucosidase activity or biallelic pathogenic/likely pathogenic GAA variants must be provided when applicable.
- Enzyme assay results (blood, fibroblasts, or muscle) when used for diagnosis [documentation required].
- Molecular genetic test demonstrating biallelic pathogenic/likely pathogenic GAA variants [documentation required].
Denial triggers for missing covered codes or authorization
Claims submitted for services that are not accompanied by covered diagnosis or procedure codes listed in this policy will be denied as not covered. Prior authorization is required for benefit coverage of Lumizyme; lack of authorization will result in denial of coverage.
- Use the most appropriate covered codes when billing; services billed for non‑covered conditions will be denied.
Specialist prescriber requirement
Lumizyme must be prescribed by or in consultation with a geneticist, neurologist, metabolic disorder sub‑specialist, or a physician who specializes in lysosomal storage disorders as required in the coverage criteria.
- Ensure documentation shows the prescribing or consulting specialist to meet the Prescriber requirement in the criteria.
Eligibility Requirements
No additional top-level eligibility requirements are listed for this policy.
Not Covered Uses
Testing or treatment uses of Lumizyme outside the specified indication (Pompe disease with documented enzyme deficiency or biallelic GAA gene variants) are not covered under this policy and are considered not medically necessary. Documentation of enzyme deficiency or molecular confirmation is required for coverage; requests not meeting the FDA‑approved indication will be denied.
Definitions and Diagnostic Confirmation
Background
Pompe disease is a lysosomal storage disorder caused by deficiency of the enzyme acid alpha‑glucosidase, leading to pathologic accumulation of glycogen, particularly in muscle. Clinical presentation ranges from infantile‑onset (onset before 12 months with hypotonia and cardiomyopathy) to late‑onset forms with progressive skeletal muscle weakness and respiratory insufficiency. Diagnosis is established by either biochemical demonstration of deficient acid alpha‑glucosidase activity in blood, fibroblasts, or muscle tissue, or by identification of biallelic pathogenic or likely pathogenic variants in the GAA gene. Enzyme replacement therapy with alglucosidase alfa (Lumizyme) is the indicated treatment for patients meeting these diagnostic criteria.
OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.