CurrentCentenePolicy CP.PHAR.521

Avalglucosidase Alfa-ngpt (Nexviazyme) (PDF)

Clinical policy defining medical necessity criteria, dosing limits, approvals, continuation criteria, exclusions, and coding implications for avalglucosidase alfa-ngpt (Nexviazyme) for treatment of late-onset Pompe disease across Commercial, HIM, and Medicaid lines of business.

Policy Summary

PayerCentene

PolicyAvalglucosidase Alfa-ngpt (Nexviazyme) (PDF)

Policy CodePolicy CP.PHAR.521

Change TypeMultiple annual review updates (additions/revisions/exclusions)

Effective DateAug 6, 2021

Next Review Date

Key ActionProvider must submit documentation (office chart notes, lab results, or other clinical information) supporting that member meets all approval criteria.

SourceLink

POLICY UPDATE CHANGES

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

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

Added exclusion for concomitant use with Pombiliti + Opfolda to align with the Pombiliti criteria.

No significant clinical policy statement changes in multiple annual reviews; references reviewed and updated.

1FDA-approved indication (late-onset Pompe disease)

Age ≥1Minimum age for use

2Weight-based dosing tiers

J0219

Coverage Summary

Coverage stance: covered_with_criteria for avalglucosidase alfa-ngpt (Nexviazyme) for late-onset Pompe disease. Scope: clinical policy defining medical necessity criteria, dosing limits, approvals, continuation criteria, exclusions, and coding implications across Commercial, HIM, and Medicaid lines of business. FDA indication: Nexviazyme is indicated for treatment of patients 1 year of age and older with late-onset Pompe disease (GAA deficiency). Dosing tiers: for members weighing ≥ 30 kg: 20 mg/kg every 2 weeks; for members weighing < 30 kg: 40 mg/kg every 2 weeks, with maximum dose noted as 40 mg/kg per 2 weeks. High-level exclusions: concomitant use with Lumizyme or the combination of Pombiliti plus Opfolda is not permitted. Primary HCPCS code: J0219 (injection, avalglucosidase alfa-ngpt, 4 mg).

Key Thresholds / Limits

Age≥ 1 year

Dose for ≥ 30 kg≤ 20 mg/kg every 2 weeks

Dose for < 30 kg≤ 40 mg/kg every 2 weeks

Initial Therapy Criteria - Pompe Disease

Initial Approval Criteria - Pompe Disease

Covered when ALL of the following are met:

ALL of the following

ONE of
- Enzyme assay confirming low GAA activity
- DNA testing
- Increased lysosomal glycogen
Age ≥ 1 year≥ 1 year
Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda

Other Initial Indications / Off-label Pathways

Other diagnoses/indications (initial)

If the request does not meet the above or there has been a recent label change (within 6 months):

ANY of the following

If recent label change and drug is on formulary/PDL: follow no coverage criteria policy for the relevant line of business (CP.CPA.190 for commercial, HIM.PA.33 for marketplace, CP.PMN.255 for Medicaid)
If recent label change and drug NOT on formulary/PDL: follow non-formulary policy for the relevant line of business (CP.CPA.190 commercial, HIM.PA.103 marketplace, CP.PMN.16 Medicaid)
If requested use not listed under section III and criterion 1 above does not apply: refer to off-label use policy (CP.CPA.09 commercial, HIM.PA.154 marketplace, CP.PMN.53 Medicaid)

Continuation Therapy Criteria - Pompe Disease

Continued Therapy - Pompe Disease

Covered when ALL of the following are met:

ALL of the following

ONE of
- Currently receiving medication via Centene benefit or member has previously met initial approval criteria
- Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums CC.PHARM.03A and CC.PHARM.03B)
Member is responding positively to therapy as evidenced by improvement in the individual member's Pompe disease manifestation profile
examples include improved/maintained FVC and 6MWT

Exclusions / Not Authorized

Diagnoses/Indications Not Authorized

Non-FDA approved indications not addressed in this policy are not authorized unless sufficient documentation of efficacy and safety per off-label use policies or evidence of coverage documents is provided

Concomitant therapy exclusions: Nexviazyme must not be prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda. This concomitant use exclusion was added via a template change in the 2Q 2024 annual review to align with the Pombiliti criteria. Provider actions note that claims may be denied if Nexviazyme is prescribed concurrently with Lumizyme or with the combination of Pombiliti and Opfolda, and providers must submit documentation supporting that the member meets all approval criteria.

Applicable Codes

Drug HCPCS codeHCPCSCovered

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Provider Actions

Prior Authorization

Prior authorization required

Requests must meet the policy's initial or continued therapy criteria for authorization. Approval durations differ by line of business: Medicaid/HIM approvals are for 12 months; Commercial approvals are for 6 months or to the member's renewal date, whichever is longer.

J0219

Documentation Required

Submit documentation for approval

Provider must submit documentation such as office chart notes, lab results, or other clinical information supporting that the member meets all approval criteria.

Clinical Evidence

Evidence and references: Nexviazyme Prescribing Information (Genzyme; Sep 2023) and the Phase 1 open-label study reported in Neuromuscular Disorders 2019;29:167-86 are cited as key evidence sources. Appendix D examples (e.g., improved/maintained forced vital capacity or 6-minute walk test) are used to inform assessment of clinical response in continued therapy criteria.

Background

Background: Avalglucosidase alfa-ngpt (Nexviazyme) is a recombinant lysosomal glycogen-specific enzyme indicated for patients 1 year and older with late-onset Pompe disease (acid alpha-glucosidase [GAA] deficiency). The policy defines acceptable methods to confirm diagnosis (enzyme assay confirming low GAA activity, DNA testing, or increased lysosomal glycogen), specifies dosing limits by weight tiers, and requires monitoring of therapeutic response for continuation decisions.

Definitions

6MWT6 minute walk test

GAAacid alpha-glucosidase

Revision History

11.06.251Q 2026 annual reviewLatest

Updated initial authorization duration from 6 months to 12 months for Medicaid/HIM (material, revised).

12.02.241Q 2025 annual review

Added increased lysosomal glycogen as an additional option for confirming a Pompe disease diagnosis (material, added).

01.11.242Q 2024 template change

Added exclusion for concomitant use with Pombiliti + Opfolda to align with the Pombiliti criteria (material, added).