CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Medicaid_Cmp_Nexviazyme_20250226_Medical 1

Defines prior-authorization coverage criteria for Nexviazyme (avalglucosidase alfa-ngpt) under Pharmacy benefit for Medicaid members and under Medical benefit for Commercial and Medicare-Medicaid Plan (MMP) members, including initial and continuation criteria, dosing, coverage durations, and applicable billing code(s).

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyMedicaid_Cmp_Nexviazyme_20250226_Medical 1

Policy CodePolicy N/A

Change TypeNo material change

Effective Date04/01/2022

Next Review Date

Key ActionSubmit prior authorization requests with documentation of diagnosis, baseline FVC and 6MWT, functional status, and prior therapy status for coverage approval.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes noted.

1Minimum age for coverage (years)

6 monthsInitial coverage duration

6 monthsContinuation coverage duration

575Maximum billed units per 14 days

Coverage Summary

Scope: This policy defines prior-authorization coverage criteria for Nexviazyme (avalglucosidase alfa-ngpt) under the Pharmacy benefit for Medicaid members and under the Medical benefit for Commercial and Medicare-Medicaid Plan (MMP) members. Coverage is aligned to FDA labeling and clinical evidence and includes initial and continuation criteria, dosing, coverage durations, and applicable billing codes.

Coverage stance: Nexviazyme (avalglucosidase alfa-ngpt) is covered with criteria for the treatment of late-onset Pompe disease (LOPD) per FDA labeling. Use outside the specified criteria or for investigational/other indications is restricted and may not be covered.

Key stats

Minimum age for coverage1 year

Initial coverage duration6 months

Continuation coverage duration6 months

Maximum billed units per 14 days575 units (2300 mg)

Dosing (LOPD)20 mg/kg every 2 weeks (members <30 kg may require 40 mg/kg)

HCPCS code / billable unitJ0219 — Injection, avalglucosidase alfa-ngpt, 4 mg (1 billable unit = 4 mg)

Ambulation thresholdAble to ambulate 40 meters without stopping and without assistive device

Forced Vital Capacity (FVC) threshold>30% and <=85% percent-predicted

Initial Therapy Criteria

Initial Therapy

Covered when ALL of the following are met:

ALL of the following

Patient is 1 year of age or older>= 1 year
Patient has documented diagnosis of late-onset Pompe disease (LOPD)
Diagnosis evidence
- Enzyme assay showing a deficiency of acid alpha-glucosidase (GAA) activity in the blood, skin, or muscle
- Genetic testing showing a mutation in the GAA gene
Medication is not being used concurrently with Lumizyme or Pombiliti
Weight <30 kg specific: Members weighing <30kg that require a dose of 40 mg/kg must have a documented failure, contraindication or intolerance to Lumizymeweight < 30 kg
Step therapy requirement: members <30 kg requiring 40 mg/kg must have documented failure, contraindication, or intolerance to Lumizyme
Patient has measurable signs of Pompe disease (motor weakness, impaired pulmonary function)
Patient has documented baseline percent-predicted forced vital capacity (FVC) and 6-minute walk test (6MWT)
Baseline measurements required at initiation
Functional and prior therapy conditions
- Patient does not require invasive ventilation
- Patient is able to ambulate 40 meters without stopping and without assistive deviceable to ambulate 40 meters without stopping and without assistive device
- Patient has a FVC of >30% but ≤85% percent-predicted>30% and <=85%
- Patient has not previously tried and failed Lumizyme

Dosing and step therapy exceptions

Nexviazyme is dosed according to the US FDA labeled dosing for LOPD. For members <30 kg a 40 mg/kg dose may be required; members <30 kg requiring 40 mg/kg must have documented failure, contraindication, or intolerance to Lumizyme.

Continuation of Therapy Criteria

Continuation of Therapy

Covered when ALL of the following are met:

ALL of the following

Patient continues to meet all initial criteria and is tolerating therapy with Nexviazyme
Continuation requires ongoing fulfillment of initial criteria and tolerability
Clinical response: Documentation of a positive clinical response to therapy as evidenced by an improvement or stabilization in percent-predicted FVC and/or 6MWTimprovement or stabilization in percent-predicted FVC and/or 6MWT

Provider Actions & Billing

Prior Authorization

Prior authorization required

Coverage reviewed prospectively via prior authorization; requests must document all criteria (diagnosis evidence, baseline FVC and 6MWT, functional status, prior therapy status) to be approved.

J0219

Documentation Required

Baseline and ongoing clinical measurements required

Submit documentation of baseline percent-predicted FVC and 6-minute walk test at initiation and demonstrate improvement or stabilization in these measures for continuation.

Baseline percent-predicted FVC at initiation
Baseline 6-minute walk test (6MWT) at initiation
Demonstrate improvement or stabilization in percent-predicted FVC and/or 6MWT for continuation

Billing Rule

Dosing and maximum billable units

LOPD dosing per FDA labeling: 20 mg/kg every 2 weeks; members weighing <30 kg may require 40 mg/kg. One billable unit = 4 mg. Maximum = 575 billable units (2300 mg) every 14 days.

J0219

Step Therapy

Step therapy for members <30 kg

For members weighing <30 kg who would require a 40 mg/kg dose, there must be documented failure, contraindication, or intolerance to Lumizyme before Nexviazyme is approved.

Clinical Evidence & References

Evidence summary: Prescribing information cited: Genzyme Corporation Nexviazyme prescribing information, September 2023 (accessed January 2025).

The policy aligns coverage to FDA labeling and available clinical evidence demonstrating improvements in respiratory and motor function for patients with Pompe disease; no additional detailed trial metrics are provided in this brief.

Background

Background: Nexviazyme is an FDA-approved recombinant human acid alpha-glucosidase enzyme replacement therapy for late-onset Pompe disease (LOPD). The policy aligns coverage to FDA labeling and clinical evidence and restricts use outside the specified criteria or for investigational indications.

Definitions (short glossary): LOPD = Late-onset Pompe disease; FVC = Percent-predicted forced vital capacity; 6MWT = 6-minute walk test.