MHN Nexviazyme Coverage Policy Update

Coverage Criteria

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met:

Diagnosis of late-onset Pompe disease confirmed by one of the following: (a) enzyme assay confirming low GAA activity; (b) DNA testing; or (c) increased lysosomal glycogen.

Age: Patient is ≥ 1 year of age.>= 1 year

Concurrent therapy: Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda.

Dose limits: Dose does not exceed weight‑based limits: (a) members ≥ 30 kg: 20 mg/kg every 2 weeks; (b) members < 30 kg: 40 mg/kg every 2 weeks.20 mg/kg q2w or 40 mg/kg q2w

Maximum dose listed as 40 mg/kg/2 weeks.

Continuation Therapy — Continued therapy covered when ALL of the following are met

Continued therapy covered when ALL of the following are met:

Continuity/Previous approval: Member currently receiving medication via a Centene benefit, has previously met initial approval criteria, or is covered under applicable continuity of care regulations.

Clinical response: Member is responding positively to therapy as evidenced by improvement or maintenance of Pompe disease–specific measures (examples include improved/maintained forced vital capacity or 6‑minute walk test distance).

See Appendix D for examples.

Concurrent therapy: Nexviazyme is not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda.

Dose limits for continuation: If request is for a dose increase, the new dose must not exceed: (a) members ≥ 30 kg: 20 mg/kg every 2 weeks; (b) members < 30 kg: 40 mg/kg every 2 weeks.

High-level coverage conditions — Covered when ALL of the following are met

Covered when ALL of the following are met (high-level items documented in this portion of the policy):

Diagnostic confirmation: Biochemical or genetic testing confirming Pompe disease (late‑onset) is required.

Referenced in policy updates.

Age requirement: Patient is at least 1 year of age.>= 1 year

Minimum age updated per policy history.

No concomitant excluded therapies: Therapy is not used concomitantly with Pombiliti + Opfolda (and not with Lumizyme).

Explicit exclusion added to align with Pombiliti criteria.

Non‑FDA approved indications that are not specifically addressed in this policy are excluded from coverage unless the provider submits sufficient documentation of efficacy and safety consistent with the applicable off‑label use policies (see CP.CPA.09 for Commercial, HIM.PA.154 for Health Insurance Marketplace, and CP.PMN.53 for Medicaid) or evidence of coverage documents supporting the indication.

Concomitant administration of Nexviazyme with other enzyme replacement therapies is not permitted for coverage. Specifically, Nexviazyme is not authorized concurrently with Lumizyme or with the combination of Pombiliti and Opfolda. This exclusion was added to align with the Pombiliti criteria and appears in the policy history.

Policy history explicitly documents an exclusion for concomitant use with Pombiliti plus Opfolda; requests for combined therapy including Pombiliti+Opfolda will not be approved under this policy.

Use of Nexviazyme for non‑FDA approved indications without adequate off‑label evidence or without the documentation required by the applicable off‑label use policies is considered not authorized and will not be covered.

Coding

HCPCS CodesHCPCS

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Covered HCPCS Codes (informational)HCPCS

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Weight-based dosing thresholds — Patients ≥30 kg vs <30 kg dosing

Patients ≥ 30 kg20 mg/kg every 2 weeks

Patients < 30 kg40 mg/kg every 2 weeks

Maximum dosing limitMaximum dose listed as 40 mg/kg per 2 weeks

Minimum patient age

Minimum patient age>= 1 year

FDA indication age statementNexviazyme indicated for patients 1 year of age and older with late-onset Pompe disease

Policy history

Provider Actions & Billing

Prior Authorization

Prior Authorization Required

Prior authorization is required. Provider must submit documentation (such as office chart notes, lab results, or other clinical information) supporting that the member has met all approval criteria, including confirmation of late-onset Pompe disease, age, and dosing within policy limits.

Prior authorization required before initiation of therapy
Providers must document diagnostic confirmation, age, and dosing within policy limits

Billing Rule

Prior Authorization and Billing Code

Use HCPCS code J0219 (Injection, avalglucosidase alfa-ngpt, 4 mg) for billing requests. Coding listed is informational only; inclusion of a code does not guarantee coverage. Providers should reference the most up-to-date professional coding guidance prior to claim submission.

Background

Late‑onset Pompe disease is an acid alpha‑glucosidase (GAA) deficiency treated with enzyme replacement therapy. Avalglucosidase alfa‑ngpt (Nexviazyme) is an FDA‑approved hydrolyzed lysosomal glycogen‑specific enzyme indicated for patients aged ≥ 1 year with late‑onset Pompe disease. Clinical monitoring of response commonly includes measures such as forced vital capacity and the 6‑minute walk test. Providers should administer therapy in settings capable of monitoring and managing infusion‑associated reactions and severe hypersensitivity, and be prepared for acute cardiorespiratory events during infusion.

Definitions

Nexviazyme — Avalglucosidase alfa-ngpt (definition/key drug identifier)

Trade nameNexviazyme

Generic name / descriptorAvalglucosidase alfa-ngpt (hydrolytic lysosomal glycogen-specific enzyme)

Formulation noteLyophilized powder, single-dose vial (100 mg)

Diagnostic confirmation — Biochemical or genetic testing demonstrating GAA deficiency or pathogenic GAA variants

Required diagnostic confirmationBiochemical testing (enzyme assay confirming low GAA activity) or genetic testing (pathogenic GAA variants)

Alternative confirmationIncreased lysosomal glycogen as supportive laboratory evidence

Initial Therapy Criteria

Initial Therapy — Initial authorization criteria

Covered when ALL of the following are met:

Diagnostic confirmation: Diagnosis confirmed by one of: enzyme assay confirming low GAA activity; DNA testing; or increased lysosomal glycogen.

Age: Patient is ≥ 1 year of age.>= 1 year

No concurrent excluded therapies: Not prescribed concurrently with Lumizyme or the combination of Pombiliti with Opfolda.

Dose within labeled limits: Dose does not exceed: members ≥ 30 kg: 20 mg/kg every 2 weeks; members < 30 kg: 40 mg/kg every 2 weeks.

Continuation Criteria

Continuation Therapy — Criteria for continued coverage

Criteria for continued coverage:

Ongoing therapy status: Member is currently receiving medication via a Centene benefit, previously met initial approval criteria, or is covered under applicable continuity of care regulations.

Clinical benefit: Evidence of positive response to therapy (examples include improved or maintained forced vital capacity or 6‑minute walk test distance).

See Appendix D for examples.

Continuation / review notes — routine annual reviews with no significant changes

Document-level continuation and review notes:

Annual review: Policy is reviewed on an annual cycle (moving to 1Q annual review); recent reviews noted no significant changes.

Step Therapy

Step	Routing instruction
1	If the drug has undergone a label change within the last 6 months or the requested use is not reflected in this policy, follow the referenced formulary/no‑coverage or non‑formulary routing policies (see CP.CPA.190, HIM.PA.33, CP.PMN.255 for formulary; CP.CPA.190, HIM.PA.103, CP.PMN.16 for non‑formulary). For uses not listed under section III and not covered by the above, refer to the off‑label use policies (CP.CPA.09, HIM.PA.154, CP.PMN.53).

Step

Routing instruction

If the drug has undergone a label change within the last 6 months or the requested use is not reflected in this policy, follow the referenced formulary/no‑coverage or non‑formulary routing policies (see CP.CPA.190, HIM.PA.33, CP.PMN.255 for formulary; CP.CPA.190, HIM.PA.103, CP.PMN.16 for non‑formulary). For uses not listed under section III and not covered by the above, refer to the off‑label use policies (CP.CPA.09, HIM.PA.154, CP.PMN.53).

Step	Note
1	Policy history documents that an exclusion for concomitant use with Pombiliti+Opfolda was added; no specific step‑therapy sequencing or failure criteria are provided in the policy text.

Quantity Limits

Avalglucosidase alfa-ngpt (Nexviazyme) — product vial and dosing per weight-based mg/kg schedule

Vial size100 mg per single-dose vial

Dosing for ≥30 kg20 mg/kg every 2 weeks (calculate number of 100 mg vials needed based on patient weight)

Dosing for <30 kg40 mg/kg every 2 weeks (calculate number of 100 mg vials needed based on patient weight)

Maximum dose noteMaximum dose cited as 40 mg/kg per 2 weeks

Site of Care

Note

Monitor and be prepared for infusion‑associated and severe hypersensitivity reactions

For infusion center administration, monitor for infusion‑associated reactions and severe hypersensitivity; be prepared to manage acute cardiorespiratory events per the product safety guidance.

Prepare for management of infusion reactions and acute cardiorespiratory failure in susceptible patients.

OpenPayer

Avalglucosidase Alfa-ngpt (Nexviazyme) coverage