Lumizyme (alglucosidase alfa) (Intravenous)
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Defines Moda Health prior authorization, dosing limits, medical necessity criteria, renewal conditions, and billing codes for intravenous Lumizyme (alglucosidase alfa) for treatment of Pompe disease.
No material clinical or coverage changes in this revision.
Coverage Criteria for Lumizyme (alglucosidase alfa)
Initial Therapy
Prior authorization validity is provided when ALL of the following are met:
Supports FDA-approved and compendia indications for Pompe disease.
Continuation/Renewal
Prior authorization may be renewed when ALL of the following are met:
Dosage/Administration
Use in combination with other enzyme replacement therapies is not allowed. This therapy must not be administered concurrently with any other enzyme replacement treatment for Pompe disease.
Prior authorization will not be continued if there is unacceptable toxicity or a lack of clinical benefit. Examples of unacceptable toxicity include hypersensitivity/anaphylaxis, infusion-associated reactions (e.g., fever, chills, flu-like illness, rash/urticaria, pruritus), immune-mediated reactions, acute cardiorespiratory failure, or cardiac arrhythmia during general anesthesia. Lack of clinical benefit is defined as failure to stabilize or improve compared to pretreatment age-appropriate baseline measures (e.g., motor function, respiratory function, FVC, 6-minute walk test).
Confirmation of Pompe disease
Billing, HCPCS, NDC, and Diagnosis Codes
| J0221 | Injection, alglucosidase alfa, (lumizyme), 10 mg; 1 billable unit = 10 mg |
| 58468-0160-xx | Lumizyme 50 mg single-dose vial for injection |
| E74.02 | Pompe disease |
Provider Requirements, Documentation, and Denial Risks
Obtain prior authorization for 12 months and submit baseline clinical measures
Prior authorization is required for Lumizyme and will be issued initially for 12 months (365 days). Requests for authorization must include the baseline clinical measures specified in the initial approval criteria (age‑appropriate baseline values such as FVC, 6‑minute walk test, muscle/respiratory/cardiac measures) and any Site of Care specialty infusion program requirements when applicable.
- Initial authorization duration: 12 months (365 days).
- Include documented baseline age-appropriate clinical measures as specified under Initial Approval Criteria.
- For groups in scope of the Site of Care (SOC) specialty infusion program, include documentation that program requirements are met.
Do not combine with other enzyme replacement therapies
Lumizyme must not be used in combination with other enzyme replacement therapies; concurrent use with another ERT is a contraindicated combination under the universal criteria.
- Requests must confirm Lumizyme will not be given alongside any other enzyme replacement therapy.
Submit baseline age-appropriate clinical measures at initiation
At initiation, provide documented baseline age-appropriate values such as infantile-onset measures (muscle weakness, motor function, respiratory function, cardiac involvement, percent predicted FVC, and/or 6‑minute walk test) or late-onset measures (FVC and/or 6‑minute walk test) as part of the authorization request.
- Include specific baseline measures appropriate to the member's age and disease onset (infantile vs late-onset).
- If FVC or 6‑MWT are unsuitable (very young members), note that requests will be reviewed case-by-case.
Diagnosis confirmation and renewal failures are potential denial triggers
Authorization may be denied if the diagnosis of Pompe disease is not confirmed by either deficient GAA enzyme activity or detection of biallelic pathogenic/likely pathogenic GAA variants, or if renewal criteria such as absence of unacceptable toxicity, antibody monitoring, and clinical benefit are not met.
- Lack of confirmatory diagnostic evidence (GAA enzyme deficiency or biallelic pathogenic GAA variants) can trigger denial.
- Failure to meet renewal requirements — e.g., unacceptable toxicity, no monitoring for antibodies, or lack of stabilization/improvement compared to pretreatment baseline — may result in denial at renewal.
Indications Covered by Policy
Confirmation of Pompe disease
Background on Pompe Disease and Therapy
Pompe disease (acid alpha-glucosidase [GAA] deficiency) is an inherited metabolic disorder treated with enzyme replacement therapy, specifically intravenous alglucosidase alfa (Lumizyme). Diagnosis is confirmed by either a deficiency of GAA enzyme activity or detection of biallelic pathogenic or likely pathogenic variants in the GAA gene; infantile-onset disease may be rapidly suspected after positive newborn screening but should be confirmed by molecular testing or measurement of GAA activity in another tissue.
Definitions and Diagnostic Criteria
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