CurrentCignaPolicy IP0279

Pompe Disease - Enzyme Replacement Therapy - Nexviazyme

Defines Cigna's coverage and prior authorization requirements for Nexviazyme enzyme replacement therapy for late-onset Pompe disease in patients aged ≥1 year across Cigna-administered health plans.

Policy Summary

PayerCigna

PolicyPompe Disease - Enzyme Replacement Therapy - Nexviazyme

Policy CodePolicy IP0279

Change TypeTitle revised

Effective Date

Next Review DateJul 15, 2025

Key ActionObtain prior authorization and document age, diagnostic confirmation (enzyme assay or genetic testing), prescribing specialist or consultation, and intended dosing regimen.

SourceLink

POLICY UPDATE CHANGES

Policy Name updated from 'Avalglucosidase' to 'Pompe Disease - Enzyme Replacement Therapy - Nexviazyme.'

1 yearInitial approval duration

≥1 yrMinimum patient age

20 mg/kgDose ≥30 kg

40 mg/kgDose <30 kg

J0219HCPCS code

Coverage and Medical Necessity Criteria

FDA-Approved Indication — Acid Alpha-Glucosidase Deficiency (Pompe Disease)

Approve for 1 year if the patient meets ALL of the following (A, B, C, and D):

Main approval criteria: A) Patient is ≥ 1 year of age; AND B) Patient has late-onset acid alpha-glucosidase deficiency (late-onset Pompe disease); AND C) The diagnosis is established by ONE of the following (i or ii):

Diagnosis confirmation (one required): i. Laboratory test demonstrating deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue; OR ii. Molecular genetic test demonstrating biallelic pathogenic or likely pathogenic acid alpha-glucosidase (GAA) gene variants.

Prescriber requirement: D) The medication is prescribed by or in consultation with a geneticist, neurologist, a metabolic disorder sub-specialist, or a physician who specializes in the treatment of lysosomal storage disorders.

Approval duration: 1 year.

Policy Summary

PayerCigna

PolicyPompe Disease - Enzyme Replacement Therapy - Nexviazyme

Policy CodePolicy IP0279

Change TypeTitle revised

Effective Date

Next Review DateJul 15, 2025

Key ActionObtain prior authorization and document age, diagnostic confirmation (enzyme assay or genetic testing), prescribing specialist or consultation, and intended dosing regimen.

SourceLink

On This Page

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.

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior Authorization is required for benefit coverage of Nexviazyme. Approvals are recommended for patients who meet the FDA‑approved indication criteria (see policy). Approvals may be issued for up to 1 year and may be extended if the patient continues to meet the criteria and dosing. Nexviazyme must be prescribed by or in consultation with a physician who specializes in the condition being treated (geneticist, neurologist, metabolic disorder subspecialist, or physician who treats lysosomal storage disorders). Requests for doses outside the dosing in the policy will be reviewed case‑by‑case by a clinician (Medical Director or Pharmacist).

Approve for up to 1 year if patient meets ALL FDA‑approved indication criteria
Medication must be prescribed by or in consultation with an appropriate specialist
Requests for non‑standard dosing considered case‑by‑case

Step Therapy

No step therapy requirements are specified for Nexviazyme.

Documentation Required

Required Documentation

Requests must document the following: patient age; diagnosis confirmation by either an enzyme assay demonstrating deficient acid alpha‑glucosidase activity (blood, fibroblasts, or muscle) OR molecular genetic testing demonstrating biallelic pathogenic or likely pathogenic GAA variants; the specific Pompe disease subtype (late‑onset); the prescribing or consulting specialist type (geneticist, neurologist, metabolic disorder subspecialist, or physician who treats lysosomal storage disorders); and the requested dosing (including weight‑based calculation and intent to follow dosing regimen per policy).

Document patient age (must be ≥ 1 year for FDA‑approved indication)
Provide diagnostic confirmation: enzyme assay OR molecular genetic test showing biallelic pathogenic/likely pathogenic GAA variants
Specify disease subtype (late‑onset Pompe disease)
Include prescribing/consulting specialist specialty
Include requested dose and patient weight to support dosing regimen

Denial Risk

Coding / Claims Denial Risk

Claims submitted for services that are not accompanied by covered code(s) under the applicable Coverage Policy will be denied as not covered. When billing, providers must use the most appropriate codes as of the date of service.

Definitions

Late-onset Pompe disease — definition and clinical features

DefinitionLate-onset Pompe disease: acid alpha-glucosidase (acid α-glucosidase) deficiency manifesting any time after 12 months of age.

Key clinical featuresProgressive skeletal muscle weakness which can progress to respiratory insufficiency.

Contrast with infantile-onsetInfantile-onset typically presents in the first few months of life with hypotonia, feeding difficulties, and cardiopulmonary failure; late-onset manifests after 12 months with a more variable course.

Diagnostic confirmationEstablished by decreased acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue, or by molecular genetic testing demonstrating biallelic pathogenic GAA variants.

Relevance to policyNexviazyme is indicated for late-onset Pompe disease in patients ≥ 1 year of age per this coverage policy.

OpenPayer

Pompe Disease - Enzyme Replacement Therapy - Nexviazyme

Coverage and Medical Necessity Criteria

Coding and Billing

Provider Requirements and Authorization

Background

Definitions