CurrentHealth NetPolicy CP PHAR.160

Alglucosidase alfa (Lumizyme) Coverage Criteria

Defines clinical coverage and prior authorization requirements for alglucosidase alfa (Lumizyme) for treatment of Pompe disease across Health Net lines of business (Commercial, HIM, Medicaid). Applies to providers requesting authorization for Lumizyme.

Policy Summary

PayerHealth Net

PolicyAlglucosidase alfa (Lumizyme) Coverage Criteria

Policy CodePolicy CP PHAR.160

Change TypeMaterial clinical changes

Effective DateFeb 1, 2024

Next Review DateFeb 25, 2025

Key ActionProvider must submit supporting documentation (office notes, lab results, genetic testing, enzyme assay, or other clinical information) confirming diagnosis and meeting approval criteria.

SourceLink

POLICY UPDATE CHANGES

Added exclusion for concomitant use of Lumizyme with the combination of Pombiliti and Opfolda.

Added increased lysosomal glycogen as an additional diagnostic option to confirm Pompe disease.

20 mg/kgMax dose (q2w)

J0221HCPCS

6 moCommercial approval

12 moMedicaid/HIM approval

Present

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Coverage Criteria for Lumizyme (alglucosidase alfa)

inv-01: Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met:

Initial Approval for Lumizyme: 1) Diagnosis of Pompe disease (acid alpha‑glucosidase [GAA] deficiency) confirmed by one of the following: a) enzyme assay confirming low GAA activity; b) DNA testing showing pathogenic variants consistent with Pompe disease; c) increased lysosomal glycogen; 2) Requested dose does not exceed 20 mg/kg IV every 2 weeks; 3) Lumizyme is not prescribed concurrently with Nexviazyme or with the combination of Pombiliti and Opfolda; 4) Provider submits supporting documentation (office chart notes, laboratory results, genetic testing, enzyme assay, or other clinical information) confirming the diagnosis and that the above criteria are met.20 mg/kg every 2 weeks

Provider must supply documentation as listed; see coding/history for HCPCS codes.

inv-02: Continuation Therapy — Covered for continued therapy when ALL of the following are met

Covered for continued therapy when ALL of the following are met:

Continuation Therapy: 1) Member is currently receiving Lumizyme and previously met initial approval criteria OR member is currently receiving medication and is enrolled in an applicable continuity-of-care program; 2) Member is responding positively to therapy as evidenced by improvement or maintenance in Pompe disease measures (see Appendix D examples such as improved/maintained 6‑minute walk test, forced vital capacity for late‑onset disease, or motor function/survival for infantile‑onset); 3) If request includes a dose increase, the new dose does not exceed 20 mg/kg IV every 2 weeks; 4) Lumizyme is not prescribed concurrently with Nexviazyme or with the combination of Pombiliti and Opfolda.20 mg/kg every 2 weeks

See Appendix D for examples of acceptable measures of therapeutic response.

Use of alglucosidase alfa (Lumizyme) for non‑FDA approved indications that are not specifically addressed in this policy is not authorized unless the request includes sufficient documentation of efficacy and safety per the applicable off‑label use policies. See the referenced off‑label policies for the member’s line of business for details on required evidence and submission standards.

Requests for non‑FDA approved indications without adequate clinical documentation (e.g., supporting literature, clinical rationale, patient‑specific efficacy/safety data) will be reviewed in accordance with the applicable off‑label use policy and may be considered not medically necessary if the documentation does not meet off‑label policy requirements.

Initial Authorization Requirements

inv-14: Initial Therapy — Initial authorization requirements and dosing

Initial authorization requirements and dosing:

Initial Authorization: 1) Confirmed diagnosis of Pompe disease by enzyme assay (low GAA activity), DNA testing, or increased lysosomal glycogen; 2) Prescribed dosing consistent with labeled dosing and does not exceed 20 mg/kg IV every 2 weeks; 3) Lumizyme will not be used concurrently with Nexviazyme or with the combination of Pombiliti and Opfolda; 4) Provider submits required supporting documentation (chart notes, labs, genetic testing, enzyme assay) with the prior authorization request.20 mg/kg every 2 weeks

Approval durations vary by line of business; prior authorization required.

Continuation Authorization Requirements

inv-15: Continuation Therapy — Continuation criteria

Continuation authorization requirements:

Continuation: 1) Member currently receiving Lumizyme and previously met initial approval criteria OR is in a continuity‑of‑care program; 2) Evidence of clinical response or maintenance documented (see Appendix D for acceptable measures); 3) Dose remains within the limit of 20 mg/kg IV every 2 weeks if a dose change is requested; 4) Lumizyme is not prescribed concurrently with Nexviazyme or with the combination of Pombiliti and Opfolda.20 mg/kg every 2 weeks

Approval durations: Commercial typically 6 months (or to member renewal date); Medicaid/HIM up to 12 months per policy.

Coding and Dosage

HCPCS CodesHCPCS

J0220	Injection, alglucosidase alfa, 10 mg, not otherwise specified
J0221	Injection, alglucosidase alfa (Lumizyme), 10 mg

Maximum dose

Maximum dose20 mg/kg every 2 weeks

RouteIntravenous (IV)

Per administration frequencyEvery 2 weeks (q2w)

Lumizyme (alglucosidase alfa) — Quantity limits

ProductLumizyme (alglucosidase alfa)

Dose limit per administration20 mg/kg per administration

PackagingSingle-use vial: 50 mg

Provider Requirements and Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required. Approval durations differ by line of business: Medicaid/HIM — 6 months; Commercial — 6 months or to the member's renewal date, whichever is longer. Dose does not exceed 20 mg/kg every 2 weeks.

Approval duration: Medicaid/HIM — 6 months; Commercial — 6 months or to member renewal date, whichever is longer
Dose limit: does not exceed 20 mg/kg every 2 weeks

Note

Formulary / Off‑label Routing

Follow formulary/non-formulary or off-label use routing for the member's line of business. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), refer to the applicable no coverage criteria policy: CP.CPA.190 (commercial), HIM.PA.33 (health insurance marketplace), CP.PMN.255 (Medicaid). For drugs NOT on the formulary/PDL, refer to the non-formulary policy: CP.CPA.190 (commercial), HIM.PA.103 (health insurance marketplace), CP.PMN.16 (Medicaid). If the requested use is not specifically listed and does not meet other criteria, refer to the off‑label use policies: CP.CPA.09 (commercial), HIM.PA.154 (health insurance marketplace), CP.PMN.53 (Medicaid).

Step Therapy / Routing Requirements

Requirement	Action / Routing
Drug is on the formulary (Commercial or Health Insurance Marketplace) or on the PDL (Medicaid)	Follow the relevant formulary coverage criteria; if no specific coverage criteria exist, follow the 'Non‑formulary / No coverage criteria' policy for the applicable line of business (CP.CPA.190 for Commercial, HIM.PA.33/HIM.PA.103 as applicable for Marketplace, CP.PMN.255/CP.PMN.16 for Medicaid).
Drug is NOT on the formulary (Commercial or Health Insurance Marketplace) or NOT on the PDL (Medicaid)	Refer to the non‑formulary policy for the applicable line of business before approval (CP.CPA.190 for Commercial, HIM.PA.103 for Marketplace, CP.PMN.16 for Medicaid).
Requested use (diagnosis, age, dosing regimen) is not specifically listed in this policy and criterion 1 (formulary/non‑formulary routing) does not apply	Route request to the off‑label use policy for the applicable line of business (CP.CPA.09 for Commercial, HIM.PA.154 for Marketplace, CP.PMN.53 for Medicaid) for review and documentation requirements prior to approval.

Site of Care

Note

No explicit site‑of‑care restriction stated

Policy does not impose a site‑of‑care restriction; there is no explicit limitation to infusion center or other settings in this policy.

Background on Pompe Disease and Therapy

Pompe disease is caused by deficiency of the enzyme acid alpha‑glucosidase (GAA), leading to progressive lysosomal accumulation of glycogen. Clinical onset is variable: infantile‑onset commonly presents with cardiomyopathy, hypotonia, respiratory compromise and is often fatal within the first year without treatment, while late‑onset forms show progressive skeletal muscle weakness and respiratory impairment. Diagnosis may be confirmed by enzyme assay demonstrating low GAA activity, DNA testing, or documentation of increased lysosomal glycogen. Alglucosidase alfa (Lumizyme) is an enzyme replacement therapy indicated for Pompe disease.

Key Definitions

Pompe disease (GAA deficiency) — Definition and diagnosis

DefinitionPompe disease is acid alpha-glucosidase (GAA) deficiency causing lysosomal glycogen accumulation

Diagnostic confirmation methodsEnzyme assay demonstrating low GAA activity; DNA testing (genetic testing); increased lysosomal glycogen

Clinical manifestationsRange from infantile-onset (cardiomyopathy, hypotonia, respiratory compromise, early mortality) to late-onset (progressive muscle weakness, respiratory involvement)