CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Nexviazyme (avalglucosidase alfa-ngpt) coverage

Defines prior authorization, coverage criteria, dosing, and billing for Nexviazyme for members of Neighborhood Health Plan of Rhode Island across Medicaid (pharmacy benefit) and Commercial/Medicare (medical benefit).

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyNexviazyme (avalglucosidase alfa-ngpt) coverage

Policy CodePolicy N/A

Change TypeNo material change

Effective DateApr 1, 2022

Next Review DateN/A

Key ActionSubmit prior authorization with documentation of diagnosis confirmation, baseline FVC and 6-minute walk test, and dosing per FDA label.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥1 yrMinimum patient age for coverage

6 monthsInitial authorization duration

J0219HCPCS code

20 mg/kg q14dStandard dosing

BoxedWarnings

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 for Nexviazyme (avalglucosidase alfa-ngpt)

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

Covered when ALL of the following are met:

Initial Therapy Criteria

Age: Member is 1 year of age or older
Diagnosis: Documented diagnosis of late-onset Pompe disease (LOPD) evidenced by either: (1) enzyme assay showing deficiency of acid alpha-glucosidase (GAA) activity in blood, skin, or muscle OR (2) genetic testing showing a mutation in the GAA gene
Concurrent therapy: Medication is not being used concurrently with Lumizyme or Pombiliti
Weight / Step therapy: Members weighing <30 kg who require a 40 mg/kg dose must have documented failure, contraindication, or intolerance to Lumizyme
MMP members who received this medication within the past 365 days are not subject to step therapy
Clinical signs: Member has measurable signs of Pompe disease (motor weakness, impaired pulmonary function)
Baseline measures: Documented baseline percent-predicted forced vital capacity (FVC) and 6-minute walk test (6MWT)
Functional status: Member does not require invasive ventilation; is able to ambulate 40 meters without stopping and without assistive device; has FVC >30% but ≤85%; has not previously tried and failed Lumizyme
Dosing: Nexviazyme is dosed according to the FDA-labeled dosing for LOPD (standard 20 mg/kg every 14 days; members <30 kg may require 40 mg/kg)

Authorization duration: Initial coverage duration: 6 months

inv-02: Continuation Therapy — ALL required for continuation

Continuation therapy covered when ALL of the following are met:

Continuation Therapy Criteria

Ongoing criteria: Member continues to meet all initial therapy criteria and is tolerating Nexviazyme
Clinical response: Documentation of a positive clinical response to therapy evidenced by improvement or stabilization in percent-predicted FVC and/or 6-minute walk test (6MWT)
Continuation duration: Continuation coverage duration: 6 months

Prior authorization: Therapy is subject to prior authorization review per policy

Neighborhood Health Plan of Rhode Island does not provide coverage for therapies used for investigational purposes. Investigational use is defined as use at a dose or for a condition other than those recognized as medically accepted indications in standard reference compendia or peer‑reviewed literature. Requests for coverage that fall outside the labeled or accepted indications or dosing will be considered investigational and are not covered.

inv-13: Initial Therapy (detailed entry overlaps with coverage criteria) — diagnosis confirmation, baseline measures, functional status, and dosing per label

Initial authorization requires confirmation of diagnosis, baseline measurements, assessment of functional status, and adherence to labeled dosing.

Diagnosis confirmation: Documented diagnosis of late-onset Pompe disease (LOPD) by either: (a) enzyme assay demonstrating GAA deficiency in blood, skin, or muscle; OR (b) genetic testing showing a pathogenic GAA mutation

Ensure supporting laboratory or genetic test reports are provided

Baseline measures: Documented baseline percent-predicted forced vital capacity (FVC) and baseline 6-minute walk test (6MWT)

Required for initial authorization

Functional status: Member does not require invasive ventilation, is able to ambulate 40 meters without stopping and without assistive device, and has FVC >30% but ≤85%

Assess and document motor and respiratory status prior to initiation

inv-14: Continuation of Therapy — ongoing benefit, tolerance, and objective evidence of improvement or stabilization required

Continuation of therapy is covered only when ALL of the following are met:

Ongoing eligibility: Member continues to meet all initial therapy criteria

Tolerance: Member is tolerating Nexviazyme without unacceptable adverse effects (e.g., severe hypersensitivity or significant infusion-associated reactions)

Monitor for infusion-associated reactions and hypersensitivity per product warnings

Objective evidence of benefit: Documentation of improvement or stabilization in percent-predicted FVC and/or 6MWT compared with baseline

Objective measures must be submitted to support continued coverage

Continuation duration: Continuation authorization duration: 6 months

Billing and Coding

Applicable HCPCS/CPT CodesHCPCSCovered

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Ambulation and pulmonary function requirement

6-minute walk test (6MWT)Baseline 6-minute walk test required; member must be able to ambulate 40 meters without stopping and without an assistive device

Forced vital capacity (FVC)Baseline percent‑predicted FVC required; FVC must be greater than 30% but less than or equal to 85%

Functional statusMember must not require invasive ventilation

Provider Actions and Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required — coverage will be reviewed prospectively via the prior authorization process and must meet initial criteria (diagnosis, baseline measures, dosing, etc.). Use outside of these guidelines may result in non-payment unless approved under an exception process.

Prospective prior authorization required
Exceptions process available but not guaranteed

Step Therapy

Step Therapy Requirement for <30 kg

Members <30 kg who require a dose of 40 mg/kg must have documented failure, contraindication, or intolerance to Lumizyme (step requirement) prior to approval.

Applies to members weighing <30 kg requiring 40 mg/kg dose
Documented step therapy failure/contraindication/intolerance to Lumizyme required

Documentation Required

Required Documentation

Documentation must include objective evidence of diagnosis and baseline functional measures. At a minimum, submit: enzyme assay or genetic testing confirming GAA deficiency/mutation; baseline percent‑predicted FVC; baseline 6‑minute walk test (6MWT); documentation of measurable signs of Pompe disease (for example motor weakness or impaired pulmonary function); current weight and dosing calculation; and notes on ventilatory status and ambulatory ability.

Diagnosis evidence: enzyme assay showing GAA deficiency OR genetic testing showing a mutation in the GAA gene
Baseline percent‑predicted FVC and baseline 6‑minute walk test (6MWT)
Documentation of measurable signs of Pompe disease (motor weakness, impaired pulmonary function)
Current weight and dosing (confirm FDA‑labeled dosing for LOPD)
Ventilatory status (invasive ventilation use) and ambulation ability (able to ambulate ≥40 meters without stopping and without assistive device)

Denial Risk

Use Outside Guidelines May Result in Non‑Payment

Use outside the guidelines may result in non‑payment — coverage is reviewed prospectively via prior authorization. If a request falls outside these criteria, consider submitting an exception request with supporting clinical rationale and documentation.

Requests not meeting criteria may be denied for payment
Exception requests can be submitted with clinical justification

Step Therapy / Therapeutic Alternatives

Step	Requirement / Condition	Action
1
Members weighing <30 kg who require a 40 mg/kg dose
Documented failure, contraindication, or intolerance to Lumizyme is required before Nexviazyme will be authorized

Quantity Limits and Dosing

avalglucosidase alfa-ngpt dosing and quantity limit

Billable units per administration575 billable units (1 billable unit = 4 mg) — equivalent to 2300 mg every 14 days

Dosing intervalEvery 14 days (q14 days)

Maximum dose statement in policyLOPD dosing listed as 20 mg/kg every 2 weeks; 575 billable units (2300 mg) specified as dose for LOPD

Site-of-Care and Benefit Assignment

Billing Rule

Infusion center / benefit distinction — pharmacy vs medical benefit by member type

Benefit and billing depend on member type and setting: for Medicaid members Nexviazyme is covered under the Pharmacy Benefit; for Commercial and Medicare members it is covered under the Medical Benefit (infusion center/site‑of‑care specifics not otherwise detailed).

Coordinate billing appropriately: Medicaid = pharmacy benefit; Commercial/Medicare = medical benefit (infusion billed via medical benefit).

Background — Disease and Therapy

Late‑onset Pompe disease (LOPD) is caused by deficiency of the lysosomal enzyme acid alpha‑glucosidase (GAA)

For LOPD the labeled dosing is 20 mg/kg every 2 weeks; members weighing <30 kg may require a 40 mg/kg dose. Clinical benefit is assessed by measures such as percent‑predicted forced vital capacity (FVC) and 6‑minute walk test (6MWT), and treatment carries warnings for infusion‑associated reactions, severe hypersensitivity (including anaphylaxis), and potential acute cardiorespiratory failure in susceptible individuals.

Definitions and Safety Warnings

LOPD (Late‑onset Pompe disease) — definition

DefinitionLate‑onset Pompe disease (LOPD) is a lysosomal acid alpha‑glucosidase (GAA) deficiency leading to progressive accumulation of glycogen in skeletal and respiratory muscles, causing progressive muscle and respiratory dysfunction

Onset and presentationAffects skeletal and respiratory muscles with progressive motor and pulmonary impairment

Diagnostic confirmationDiagnosis is confirmed by enzyme assay showing GAA deficiency or genetic testing showing a mutation in the GAA gene

Key safety warnings

Boxed warningsSevere hypersensitivity reactions including anaphylaxis — boxed warnings present

Infusion‑associated reactions (IARs)Infusion‑associated reactions may occur and require monitoring during and after infusion

Acute cardiorespiratory riskPotential risk of acute cardiorespiratory failure in susceptible individuals, particularly those with underlying respiratory compromise or fluid‑overload risk

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyNexviazyme (avalglucosidase alfa-ngpt) coverage

Policy CodePolicy N/A

Change TypeNo material change

Effective DateApr 1, 2022

Next Review DateN/A

Key ActionSubmit prior authorization with documentation of diagnosis confirmation, baseline FVC and 6-minute walk test, and dosing per FDA label.

SourceLink

On This Page

Measurable disease signs: Presence of measurable signs of Pompe disease such as motor weakness or impaired pulmonary function

Dosing per label: Dosing must follow FDA-labeled dosing for LOPD (standard: 20 mg/kg every 2 weeks; for members <30 kg a 40 mg/kg dose may be required)

Quantity limits: 575 billable units (2300 mg) every 14 days corresponds to 20 mg/kg dosing at specified weights

No concurrent enzyme therapy: Medication must not be used concurrently with Lumizyme or Pombiliti

Step therapy exception note: For members <30 kg requiring 40 mg/kg, documented failure/contraindication/intolerance to Lumizyme is required unless member received this medication within the past 365 days (MMP exception)