CurrentHap Caresource MI Coordinated HealthPolicy N/A

Pompe Disease—Enzyme Replacement Therapy—Nexviazyme Utilization Management Medical Policy

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This policy governs prior authorization and medical-benefit coverage of intravenous Nexviazyme (avalglucosidase alfa-ngpt) for patients with late-onset Pompe disease. Coverage requires specified diagnostic, age, specialist-prescribing, and weight-based dosing criteria.

Policy Summary

PayerHap Caresource MI Coordinated Health

PolicyPompe Disease—Enzyme Replacement Therapy—Nexviazyme

Policy CodePolicy N/A

Change TypeClarification

Effective DateN/A

Last ReviewMay 6, 2026

Next Review DateN/A

Key ActionSubmit diagnostic confirmation and document the patient's age, diagnosis, weight category, requested dose, infusion frequency, and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

The genetic diagnostic criterion was revised to specify biallelic pathogenic or likely pathogenic acid alpha-glucosidase gene variants.

≥1 yearMinimum patient age for coverage

1 yearInitial approval duration

Every 2 weeksRequired infusion frequency

20 mg/kg

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CurrentHap Caresource MI Coordinated HealthPolicy N/A

Pompe Disease—Enzyme Replacement Therapy—Nexviazyme Utilization Management Medical Policy

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Sign up for all hap_caresource_mi_coordinated_health policy alerts

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Policy Summary

PayerHap Caresource MI Coordinated Health

PolicyPompe Disease—Enzyme Replacement Therapy—Nexviazyme

Policy CodePolicy N/A

Change TypeClarification

Effective DateN/A

Last ReviewMay 6, 2026

Next Review DateN/A

Key ActionSubmit diagnostic confirmation and document the patient's age, diagnosis, weight category, requested dose, infusion frequency, and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

The genetic diagnostic criterion was revised to specify biallelic pathogenic or likely pathogenic acid alpha-glucosidase gene variants.

≥1 yearMinimum patient age for coverage

1 yearInitial approval duration

Every 2 weeksRequired infusion frequency

20 mg/kg

Dose for patients ≥30 kg

40 mg/kgDose for patients <30 kg

RequiredPrescriber or consulting specialist requirement

Coverage Criteria

Initial Therapy Eligibility

Covered for 1 year when ALL of the following criteria are met:

ALL of the following

Patient is ≥ 1 year of age.
Patient has late-onset acid alpha-glucosidase deficiency (late-onset Pompe disease).
ONE of the following
- Patient has a laboratory test demonstrating deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue.
- Patient has a molecular genetic test demonstrating biallelic pathogenic or likely pathogenic acid alpha-glucosidase (GAA) gene variants.
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.

Approved Dosing

Approve ONE of the following dosing regimens:

ONE of the following

Patient ≥ 30 kg: Dose is 20 mg/kg administered by intravenous infusion once every 2 weeks.
Patient < 30 kg: Dose is 40 mg/kg administered by intravenous infusion once every 2 weeks.

Coverage of Nexviazyme is not recommended for circumstances not listed in the Recommended Authorization Criteria. Criteria will be updated as new published data are available.

Initial Therapy

Approve for 1 year when all indication criteria and one approved dosing regimen are met.

Initial Nexviazyme authorization

Clinical eligibility: Patient is at least 1 year old, has late-onset Pompe disease, has diagnostic confirmation by accepted enzyme activity or genetic testing, and has specialist prescribing or consultation.All clinical eligibility requirements
Dosing regimen
- Weight ≥30 kg: 20 mg/kg by intravenous infusion once every 2 weeks.≥30 kg

Continuation Therapy

Extended Approval

Extended approval is allowed when the patient continues to meet the clinical criteria and dosing requirements.

Continuation requirements: For extended approval, the patient continues to meet the policy's clinical eligibility criteria and established dosing requirements.

Provider Authorization Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for medical-benefit coverage of Nexviazyme. Initial approval is for 1 year when the patient meets the applicable criteria and dosing requirements; extended approvals may be allowed when criteria continue to be met.

Note

Specialist Prescriber or Consultation

Nexviazyme must be prescribed by or in consultation with a physician who specializes in the condition being treated.

Eligible specialists include a geneticist, neurologist, metabolic disorder sub-specialist, or physician specializing in lysosomal storage disorders.

Documentation Required

Clinical Definitions

Pompe disease

Clinical classificationRare lysosomal storage disorder; also called glycogen storage disease type II or acid maltase deficiency.

Underlying deficiencyDeficiency in acid alpha-glucosidase activity.

Pathologic effectAccumulation of glycogen, particularly in muscle.

Typical clinical courseProgressive muscle weakness that can progress to respiratory insufficiency.

Late-onset Pompe disease

DefinitionPompe disease with onset after 12 months of age.

Age of onset

Clinical Background

Pompe disease, also known as glycogen storage disease type II or acid maltase deficiency, is a lysosomal storage disorder caused by deficient acid alpha-glucosidase activity. This deficiency results in glycogen accumulation, particularly in muscle. Late-onset disease may begin after 12 months of age and commonly involves progressive muscle weakness with possible respiratory insufficiency; enzyme replacement therapy has an important role in management.

Policy Summary

PayerHap Caresource MI Coordinated Health

PolicyPompe Disease—Enzyme Replacement Therapy—Nexviazyme

Policy CodePolicy N/A

Change TypeClarification

Effective DateN/A

Last ReviewMay 6, 2026

Next Review DateN/A

Key ActionSubmit diagnostic confirmation and document the patient's age, diagnosis, weight category, requested dose, infusion frequency, and specialist involvement.

SourceLink

On This Page

Weight <30 kg: 40 mg/kg by intravenous infusion once every 2 weeks.<30 kg

Pompe Disease Diagnostic Confirmation

Document diagnostic confirmation of late-onset acid alpha-glucosidase deficiency (Pompe disease) with laboratory or molecular genetic testing.

Laboratory testing must demonstrate deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue.
Molecular genetic testing must demonstrate biallelic pathogenic or likely pathogenic acid alpha-glucosidase (GAA) gene variants.

Documentation Required

Clinical and Dosing Information

Submit the clinical and dosing information needed to evaluate the request for Nexviazyme.

Document that the patient is at least 1 year of age and has late-onset Pompe disease.
Document patient weight category: at least 30 kg or less than 30 kg.
For patients weighing at least 30 kg, document 20 mg/kg by intravenous infusion once every 2 weeks.
For patients weighing less than 30 kg, document 40 mg/kg by intravenous infusion once every 2 weeks.
Include the prescribing physician's name and specialty or the eligible specialist consulted.

Denial Risk

Requests Outside Authorization Criteria

Coverage of Nexviazyme is not recommended when the request does not meet the listed Recommended Authorization Criteria.

This includes circumstances not otherwise listed in the Recommended Authorization Criteria.

Note

Nonstandard Dosing

Doses outside the established regimens in this policy require case-by-case review before authorization.

Review is performed by a clinician, such as the Medical Director or a pharmacist.

May manifest any time after 12 months of age.

Policy-applicable populationPatients at least 1 year of age with late-onset Pompe disease.

Clinical presentationTypically includes progressive muscle weakness, which may progress to respiratory insufficiency.

Acid alpha-glucosidase deficiency confirmation

Enzyme activity pathwayLaboratory testing demonstrates deficient acid alpha-glucosidase activity in blood, fibroblasts, or muscle tissue.

Molecular genetic pathwayMolecular genetic testing demonstrates biallelic pathogenic or likely pathogenic acid alpha-glucosidase (GAA) gene variants.

Accepted pathway logicDiagnosis is established by one of the two accepted pathways: deficient enzyme activity testing or qualifying molecular genetic testing.