ModifiedmhnPolicy CP.PHAR.521

Avalglucosidase Alfa-ngpt (Nexviazyme) coverage

Defines medical necessity and authorization criteria for Nexviazyme for treatment of late-onset Pompe disease for members aged ≥1 year across commercial, HIM, and Medicaid lines of business.

Policy Summary

Payermhn

PolicyAvalglucosidase Alfa-ngpt (Nexviazyme) coverage

Policy CodePolicy CP.PHAR.521

Change TypeMaterial operational updates

Effective DateAug 6, 2021

Next Review Date

Key ActionSubmit prior authorization with documentation demonstrating diagnostic confirmation and that Nexviazyme will not be used concurrently with Lumizyme or Pombiliti+Opfolda.

SourceLink

POLICY UPDATE CHANGES

Updated initial authorization duration from 6 months to 12 months for Medicaid/HIM.

Added exclusion for concomitant use with Pombiliti+Opfolda.

Added increased lysosomal glycogen as an additional option for confirming a Pompe disease diagnosis.

≥1Minimum covered age for indication

20 mg/kgDose ≥30 kg

40 mg/kgDose <30 kg

6 moInitial auth (Commercial)

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria

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

Covered when ALL of the following are met:

Initial Approval Criteria - Pompe Disease: 1. Diagnosis of late-onset Pompe disease confirmed by one of the following: a) Enzyme assay confirming low GAA activity; b) DNA testing; c) Increased lysosomal glycogen. 2. Age ≥ 1 year. 3. Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda. 4. Dose does not exceed any of the following: a) Members weighing ≥ 30 kg: 20 mg/kg every 2 weeks; b) Members weighing < 30 kg: 40 mg/kg every 2 weeks.

Approval duration: Medicaid/HIM 12 months; Commercial 6 months or to renewal date.

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

Covered when ALL of the following are met:

Continuation Criteria - Pompe Disease: 1. Member meets one of the following: a) Currently receiving medication via Centene benefit or previously met initial approval criteria; b) Member is currently receiving medication and is enrolled in a state/product with continuity of care regulations. 2. Member is responding positively to therapy as evidenced by improvement in the individual's Pompe disease manifestation profile (see Appendix D examples). 3. Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda. 4. If request is for a dose increase, new dose does not exceed: a) Members weighing ≥ 30 kg: 20 mg/kg every 2 weeks; b) Members weighing < 30 kg: 40 mg/kg every 2 weeks.

Approval duration: Medicaid/HIM 12 months; Commercial 6 months or to renewal date.

Non‑FDA approved indications are not authorized under this policy unless the provider submits sufficient documentation demonstrating efficacy and safety in accordance with the applicable off‑label use policy or evidence of coverage document. Follow the referenced off‑label policies: CP.CPA.09 (Commercial), HIM.PA.154 (Health Insurance Marketplace), or CP.PMN.53 (Medicaid) when requesting coverage for indications outside the FDA‑approved labeling.

Requests for Nexviazyme that propose concomitant use with Pombiliti+Opfolda are excluded and will not be authorized. This exclusion was added during the 2Q 2024 annual review to align with Pombiliti criteria; avoid requests that combine these products.

Nexviazyme is not authorized for concurrent use with Lumizyme or for use in combination with Pombiliti and Opfolda. Additionally, uses that are not FDA‑approved and are not supported by documentation consistent with the applicable off‑label use policies or evidence of coverage documents will not be covered.

Initial Therapy Criteria

inv-20: Initial therapy — Initial therapy dosing must follow weight-based regimen and diagnosis confirmation.

Initial therapy dosing must follow weight-based regimen and diagnosis confirmation.

Initial therapy - dosing and diagnosis: Diagnosis of late-onset Pompe disease confirmed by enzyme assay (low GAA activity), DNA testing, or increased lysosomal glycogen; member age ≥ 1 year; dosing per weight: ≥ 30 kg = 20 mg/kg every 2 weeks; < 30 kg = 40 mg/kg every 2 weeks; maximum dose 40 mg/kg every 2 weeks; Nexviazyme must not be prescribed concurrently with Lumizyme or Pombiliti + Opfolda.

Approval duration: Medicaid/HIM 12 months; Commercial 6 months or to renewal date.

inv-21: Initial therapy authorization — Initial authorization parameters updated in annual review notes.

Initial authorization parameters updated in annual review notes.

initial auth duration: Initial authorization duration is 12 months for Medicaid/HIM (updated from 6 months).

Operational update applied during 1Q 2026 annual review.

Continuation Therapy Criteria

inv-22: Continuation therapy requirements — Continuation of therapy requires evidence of positive response.

Continuation of therapy requires evidence of positive response.

Continuation - evidence of response: Member is currently receiving medication via Centene benefit or previously met initial criteria (or is under continuity of care); documented positive response to therapy as evidenced by clinical parameters (examples include improved or maintained forced vital capacity or improved/maintained 6-minute walk test distance as noted in Appendix D).

See Appendix D for examples of therapeutic response.

inv-23: Continuation therapy/authorization duration — Continuation/renewal considerations reflected in annual reviews and template updates.

Continuation/renewal considerations reflected in annual reviews and template updates.

authorization duration update: Initial authorization duration updated from 6 months to 12 months for Medicaid/HIM.

Change recorded in 1Q 2026 annual review.

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Providers must obtain prior authorization and provide documentation that all approval criteria are met before Nexviazyme (avalglucosidase alfa-ngpt) will be covered. Approval durations vary by line of business (see Approval duration): Medicaid/HIM — 12 months; Commercial — 6 months or to the member's renewal date, whichever is longer.

Prior authorization applies to HCPCS J0219 (Injection, avalglucosidase alfa-ngpt, 4 mg) — prior authorization is implied by this policy's coding references; verify the appropriate code(s) when submitting requests and claims.
Inclusion or exclusion of codes in this policy does not guarantee coverage; providers should reference the most up-to-date professional coding guidance prior to claim submission.

Documentation Required

Providers must submit documentation (e.g., office chart notes, laboratory results, genetic testing, enzyme assays, or other clinical information) supporting that the member meets all applicable approval criteria. Documentation should include confirmation of diagnosis and any tests used to confirm late-onset Pompe disease (enzyme assay showing low GAA activity, DNA testing, or evidence of increased lysosomal glycogen), patient age, current and prior therapies, and dosing calculations consistent with policy limits.

Coding and Dosing

HCPCS CodesHCPCS

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

HCPCS Codes referencedHCPCS

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Weight-based dosing threshold

Threshold30 kg

Dose for patients ≥ 30 kg20 mg/kg every 2 weeks

Dose for patients < 30 kg40 mg/kg every 2 weeks

Quantity and Vial Information

Product availability / vial size

Vial size100 mg single-dose (lyophilized powder)

FormulationLyophilized powder in a single-dose vial

GAA descriptionContains acid alpha-glucosidase (GAA)

Site of Care and Infusion Considerations

Note

Infusion risks — monitor for hypersensitivity and infusion reactions

Monitor infusion-related risks associated with Nexviazyme; severe hypersensitivity reactions, infusion-associated reactions, and risk of acute cardiorespiratory failure are noted in the boxed warnings and should be considered during infusion.

No explicit site‑of‑care restriction provided, but monitor for hypersensitivity and infusion reactions per boxed warning.

Definitions

Late-onset Pompe disease — definition and diagnostic confirmation

DefinitionLate-onset Pompe disease: lysosomal acid alpha-glucosidase (GAA) deficiency presenting beyond infancy

Diagnostic confirmation optionsConfirmed by one of: enzyme assay showing low GAA activity; DNA testing; or increased lysosomal glycogen

Minimum covered agePatients 1 year of age and older

Nexviazyme (avalglucosidase alfa) — product definition

Product name (generic)|brandAvalglucosidase alfa-ngpt (Nexviazyme)

Class / descriptionHydrolytic lysosomal glycogen-specific enzyme; enzyme replacement therapy for Pompe disease

Background

Pompe disease is a genetic lysosomal disorder caused by deficiency of the acid alpha‑glucosidase (GAA) enzyme, leading to pathological glycogen accumulation in lysosomes and progressive muscle dysfunction. Late‑onset Pompe disease presents beyond infancy and can cause respiratory compromise and skeletal muscle weakness. Avalglucosidase alfa‑ngpt (Nexviazyme) is an enzyme replacement therapy approved for patients aged ≥ 1 year with late‑onset Pompe disease; diagnosis for policy purposes may be confirmed by low GAA activity (enzyme assay), DNA testing, or increased lysosomal glycogen. Policy updates note boxed warnings for severe hypersensitivity and infusion‑associated reactions and codify weight‑based dosing and authorization parameters.

Step Therapy

Step	Requirement
1	No step therapy requirements specified in these policy chunks.