CurrentCignaPolicy IP0440

Lumizyme (alglucosidase alfa) coverage

Defines Cigna's prior authorization and coverage criteria for Lumizyme (alglucosidase alfa) enzyme replacement therapy for Pompe disease for applicable benefit plans.

Policy Summary

PayerCigna

PolicyLumizyme (alglucosidase alfa) coverage

Policy CodePolicy IP0440

Change TypeName and dosing update

Effective DateJul 15, 2025

Next Review Date

Key ActionObtain prior authorization with documentation of diagnosis (enzyme activity or biallelic GAA variants) and specialist prescription; approvals typically granted for 1 year.

SourceLink

POLICY UPDATE CHANGES

Policy Name updated from 'Alglucosidase Alfa' to 'Pombe Disease - Enzyme Replacement Therapy - Lumizyme' and dosing information for Acid Alpha-Glucosidase Deficiency (Pompe Disease) was added; no criteria changes.

1 yeartypical approval duration

20 mg/kgmax per-dose

q2wmin dosing interval

J0221HCPCS code

Coverage Criteria for Lumizyme (alglucosidase alfa)

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FDA-Approved Indication

Approve for 1 year if the patient meets BOTH of the following (A and B):

Initial approval for Acid Alpha-Glucosidase Deficiency (Pompe Disease): A) Diagnosis established by ONE of: (i) laboratory test demonstrating deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue; OR (ii) molecular genetic test demonstrating biallelic pathogenic or likely pathogenic GAA gene variants; AND B) Lumizyme is prescribed by or in consultation with a geneticist, neurologist, metabolic disorder sub-specialist, or physician specializing in lysosomal storage disorders.duration=1 year

Dosing: each dose must not exceed 20 mg/kg IV no more frequently than once every 2 weeks.

Lumizyme (alglucosidase alfa) for any indication other than acid alpha-glucosidase deficiency (Pompe disease) is not medically necessary. Criteria will be updated as new published data become available.

Use of Lumizyme for indications other than acid alpha-glucosidase deficiency (Pompe disease) is considered not medically necessary.

Provider Requirements and Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for Lumizyme. Approvals are recommended when the patient meets the policy Criteria and Dosing and are provided for the duration indicated (typically 1 year for FDA-approved indication). Lumizyme must be prescribed by or in consultation with a specialist (geneticist, neurologist, metabolic disorder sub-specialist, or physician who treats lysosomal storage disorders).

Prior authorization required for Lumizyme
Approvals recommended when criteria met; provided for duration noted (eg, 1 year)
Specialist prescribing or consultation required

Prior Authorization

Dose Exception Review

Requests for doses outside the dosing limits specified in this policy will be reviewed on a case-by-case basis by a clinician (e.g., Medical Director or Pharmacist). Each dose in the policy must not exceed 20 mg/kg administered IV no more frequently than once every 2 weeks unless an exception is approved.

Dose limit: <= 20 mg/kg per dose
Dosing frequency: no more frequently than once every 2 weeks
Dose-exception requests reviewed case-by-case

Documentation Required

Required Documentation

Approval requires documentation of the diagnosis and confirmatory testing demonstrating acid α‑glucosidase deficiency, such as: laboratory demonstration of deficient acid α‑glucosidase activity in blood, fibroblasts, or muscle tissue, or molecular genetic testing showing biallelic pathogenic or likely pathogenic GAA variants. Also document specialist involvement and indication.

Laboratory demonstration of deficient acid α‑glucosidase activity OR molecular genetic test with biallelic pathogenic/likely pathogenic GAA variants
Documentation that Lumizyme is prescribed by or in consultation with an appropriate specialist
Receipt of sample product does not satisfy criteria

Denial Risk

Coding-based Denial Risk

Claims submitted without covered diagnosis or procedure codes listed under this Coverage Policy (including J0221 for alglucosidase alfa, 10 mg) or without required supporting documentation will be denied as not covered.

Covered HCPCS: J0221 (Injection, alglucosidase alfa (Lumizyme), 10 mg)
Claims lacking covered diagnosis/procedure codes or required documentation will be denied

Initial Therapy

Initial approval criteria and dosing

Initial Therapy: Diagnosis confirmed by enzyme activity testing or molecular genetic testing demonstrating biallelic pathogenic/likely pathogenic GAA variants; Lumizyme is prescribed by or in consultation with a geneticist, neurologist, metabolic disorder specialist, or physician specializing in lysosomal storage disorders.dose <=20 mg/kg every >=2 weeks

Approve for 1 year; site of care and dosing should be clinically appropriate and supported by evidence.

Continuation Therapy

Extended approvals allowed if patient continues to meet Criteria and Dosing.

Continuation: Continued approval requires ongoing documentation that the patient continues to meet the policy criteria and dosing limits (diagnosis, specialist involvement, and dosing not to exceed 20 mg/kg IV no more frequently than once every 2 weeks).renewal interval=1 year

Requests for doses outside established dosing are reviewed case-by-case by a clinician.

Step	Requirement	Prior Authorization	Prescriber Specialty
1	No step therapy requirements specified	Prior authorization is required for benefit coverage of Lumizyme; approvals are recommended when the patient meets the policy Criteria and Dosing	Lumizyme must be prescribed by or in consultation with a physician who specializes in the condition being treated (e.g., geneticist, neurologist, metabolic disorder specialist, or physician specializing in lysosomal storage disorders)

Site of Care Considerations

Note

Infusion site — ensure clinically appropriate site of care

Site of care should be reasonable and clinically appropriate; dosing and site should be supported by evidence and adjusted based on severity and response.

Infusion site and dosing must be clinically appropriate and supported by evidence‑based literature.
Adjust site of care based on severity, alternative treatments, and prior response to therapy.

Background

Pompe disease (glycogen storage disease type II) is a rare lysosomal storage disorder caused by deficiency of acid alpha-glucosidase, leading to pathologic glycogen accumulation in multiple tissues, particularly skeletal and cardiac muscle. Clinical presentation ranges from infantile-onset disease with hypotonia and cardiopulmonary failure to later-onset forms characterized by progressive skeletal muscle weakness and respiratory insufficiency. Diagnosis is established by demonstration of decreased enzyme activity or by molecular genetic testing for pathogenic GAA variants. Enzyme replacement therapy with Lumizyme (alglucosidase alfa) is the disease-specific treatment.

Definitions

Pompe disease — Definition and diagnostic criteria

DefinitionPompe disease (glycogen storage disease type II) is a rare lysosomal storage disorder caused by deficiency of acid α-glucosidase leading to accumulation of glycogen, particularly in muscle.

Diagnostic criteriaDiagnosis established by either (1) laboratory demonstration of decreased acid α-glucosidase activity in blood, fibroblasts, or muscle tissue, OR (2) molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic GAA gene variants.

Clinical manifestationsInfantile-onset: hypotonia, feeding difficulties, cardiopulmonary failure; Late-onset: progressive muscle weakness and possible respiratory insufficiency.

Definitive treatmentEnzyme replacement therapy with Lumizyme (alglucosidase alfa)