CurrentCommunity-CarePolicy N/A

Nexviazyme (avalglucosidase alfa) Medical Benefit Medication Utilization Policy

Defines medical benefit coverage, initial and continuation criteria, quantity limits, and authorization period for avalglucosidase alfa (Nexviazyme) for treatment of late-onset Pompe disease for Community-Care members.

Policy Summary

PayerCommunity-Care

PolicyNexviazyme (avalglucosidase alfa) Medical Benefit Medication Utilization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionSubmit prior authorization with diagnostic confirmation, specialist prescriber details, evidence of symptoms or objective signs, and current patient weight.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 yrauth period

q2wfrequency

20 or 40 mg/kgdose by weight

Apr 2024last review

Coverage Criteria for Avalglucosidase Alfa (Nexviazyme)

Initial Therapy

Covered when ALL of the following are met for initial approval:

Initial Approval Criteria: Clinical diagnosis of late-onset Pompe disease confirmed by genetic sequencing OR acid alpha-glucosidase enzyme assay; prescribed by a medical geneticist or neuromuscular specialist; patient is experiencing symptoms or objective signs of Pompe disease.

Continuation Therapy

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

Continuation Criteria: Diagnosis of late-onset Pompe disease; member is benefitting from therapy, is being monitored, and is appropriate to continue therapy.

This policy requires documentation that the member has a confirmed diagnosis of late-onset Pompe disease before initial approval. Confirmation must be by either genetic sequencing or an acid alpha-glucosidase enzyme assay. The medication must be prescribed by a medical geneticist or neuromuscular specialist, and the patient must be experiencing symptoms or objective signs of Pompe disease to meet initial coverage criteria.

Policy Summary

PayerCommunity-Care

PolicyNexviazyme (avalglucosidase alfa) Medical Benefit Medication Utilization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionSubmit prior authorization with diagnostic confirmation, specialist prescriber details, evidence of symptoms or objective signs, and current patient weight.

SourceLink

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For continuation of therapy, the member must have an established diagnosis of late-onset Pompe disease and demonstrate clinical benefit from therapy. Ongoing approval requires that the member is benefitting from therapy, is being monitored, and remains clinically appropriate to continue treatment.

Initial Therapy

Initial: Clinical diagnosis of late-onset Pompe disease confirmed by genetic sequencing OR acid alpha-glucosidase enzyme assay; prescribed by a medical geneticist or neuromuscular specialist; patient is experiencing symptoms or objective signs of Pompe disease.

Continuation of Therapy

Continuation: Diagnosis of late-onset Pompe disease; member is benefitting from therapy, is being monitored, and is appropriate to continue therapy.

Coding and Dosing

HCPCS CodesHCPCS

J0219

Injection, avalglucosidase alfa-ngpt, 4 mg

Weight-based dosing threshold

Weight threshold>= 30 kg uses 20 mg/kg every 2 weeks; < 30 kg uses 40 mg/kg every 2 weeks

Dosing frequencyEvery 2 weeks

Weight required for approvalA current patient weight is required for authorization

Authorized Dose and Quantity Limits

Quantity limits — avalglucosidase alfa (Nexviazyme)

Drug and brandavalglucosidase alfa (Nexviazyme)

Authorized dose (>= 30 kg)20 mg/kg every 2 weeks

Authorized dose (< 30 kg)40 mg/kg every 2 weeks

Authorization noteQuantities authorized up to the specified mg/kg dosing when approved

Prior Authorization and Provider Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Requests must include documentation confirming the diagnosis of late-onset Pompe disease and that the medication is being prescribed by an appropriate specialist. Approvals are issued for up to one year when criteria are met.

Required for avalglucosidase alfa (Nexviazyme) prior to authorization
Authorization period: up to 1 year
Quantity limits applied (see policy): up to 20 mg/kg (if ≥ 30 kg) or 40 mg/kg (if < 30 kg) every 2 weeks; weight required

Note

Provider Action Required

Action items for the ordering provider: ensure the prescriber is a medical geneticist or neuromuscular specialist, include evidence of clinical signs or symptoms of Pompe disease, and supply objective diagnostic confirmation as specified.

Prescriber specialty must be medical geneticist or neuromuscular specialist
Document clinical symptoms or objective signs consistent with late-onset Pompe disease
Include patient weight for dosing/quantity determination

Documentation Required

Required Documentation

Documentation submitted with the prior authorization must include definitive diagnostic confirmation and prescriber specialty, plus evidence of symptoms or clinical monitoring.

Diagnostic confirmation by genetic sequencing or acid alpha-glucosidase enzyme assay
Prescriber specialty clearly indicated (medical geneticist or neuromuscular specialist)
Record of symptoms/objective signs and monitoring demonstrating benefit for continuation requests

Denial Risk

Potential Triggers for Denial

Requests may be denied if required documentation or prescriber qualifications are not met, or if dosing/weight information is missing.

Denial triggers: lack of diagnostic confirmation by genetic sequencing or enzyme assay
Denial triggers: prescribed by a clinician who is not a medical geneticist or neuromuscular specialist
Denial triggers: absence of documented symptoms/objective signs or failure to demonstrate clinical benefit for continuation
Denial triggers: missing patient weight or dosing information required to assess quantity limits

Background

Late-onset Pompe disease is the indication addressed by this policy. Enzyme replacement with avalglucosidase alfa (Nexviazyme) is used to replace deficient acid alpha-glucosidase activity and slow progressive muscle weakness and respiratory compromise associated with the disorder. When approved, treatment is authorized for up to one year and dosing is weight-based: 20 mg/kg every 2 weeks for members ≥ 30 kg or 40 mg/kg every 2 weeks for members < 30 kg. A current weight is required for approval.

Definitions

Definition — Confirmed late-onset Pompe disease

Confirmed diagnosisDiagnosis confirmed by genetic sequencing or by acid alpha-glucosidase enzyme assay

Confirmatory methods — geneticGenetic sequencing

Confirmatory methods — enzyme assayAcid alpha-glucosidase enzyme assay

Site of Care Considerations

Note

Site of care note — weight required and dosing frequency

Site of care is not restricted by a specific setting in this policy; however, a patient weight is required for approval and dosing is authorized every 2 weeks per the weight-based limits.

Weight required for approval and to determine authorized quantity
Authorized dosing frequency: every 2 weeks
Authorized quantities: up to 20 mg/kg (if ≥ 30 kg) or 40 mg/kg (if < 30 kg)