ModifiedAmeriHealth Caritas VIP CarePolicy MA08.036g

Alglucosidase alfa (e.g., Lumizyme®), Avalglucosidase alfa-ngpt (Nexviazyme®), Cipaglucosidase alfa-atga (Pombiliti™)

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Medical-benefit coverage and medical-necessity requirements for Lumizyme, Nexviazyme, and Pombiliti in eligible individuals with Pompe disease, subject to benefit terms, documented medical necessity, and specified dosing and frequency requirements.

Policy Summary

PayerAmeriHealth Caritas VIP Care

PolicyCoverage of enzyme replacement therapies for Pompe disease

Policy CodePolicy MA08.036g

Change TypeModified

Effective DateMarch 28, 2025

Last ReviewDec 15, 2025

Next Review DateN/A

Key ActionDocument medical necessity in the individual's medical record and provide records upon request.

SourceLink

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ModifiedAmeriHealth Caritas VIP CarePolicy MA08.036g

Alglucosidase alfa (e.g., Lumizyme®), Avalglucosidase alfa-ngpt (Nexviazyme®), Cipaglucosidase alfa-atga (Pombiliti™)

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Policy Summary

PayerAmeriHealth Caritas VIP Care

PolicyCoverage of enzyme replacement therapies for Pompe disease

Policy CodePolicy MA08.036g

Change TypeModified

Effective DateMarch 28, 2025

Last ReviewDec 15, 2025

Next Review DateN/A

Key ActionDocument medical necessity in the individual's medical record and provide records upon request.

SourceLink

POLICY UPDATE CHANGES

Cipaglucosidase alfa-atga (Pombiliti) was added to the policy scope.

Avalglucosidase alfa-ngpt (Nexviazyme) was added to the policy scope.

Pombiliti-related HCPCS coding was added.

HCPCS coding for avalglucosidase alfa-ngpt was updated to J0219.

HCPCS code J3590 was added for Nexviazyme.

Alglucosidase alfa coverage requirements were updated.

Alglucosidase alfa criteria were expanded for younger individuals.

Alglucosidase alfa (Myozyme) was removed from the policy.

3Therapies addressed

4Covered HCPCS codes

1Code not eligible for reimbursement

40 kgPombiliti minimum weight

1 month+Minimum Lumizyme age

Covered and Noncovered Uses

Initial Lumizyme therapy

Lumizyme is covered when all of the following are met.

Lumizyme criteria

Confirmed Pompe disease: Deficiency of GAA in leukocytes, skin fibroblasts, or muscle, or confirmation of biallelic pathogenic variant(s) in the GAA gene.
Symptomatic disease, age, and dosing: Individual has symptomatic infantile-onset or juvenile/adult-onset Pompe disease, is 1 month of age or older, and receives Lumizyme 20 mg/kg intravenously every 2 weeks.Age >=1 month; 20 mg/kg IV every 2 weeks

Initial Nexviazyme therapy

Nexviazyme is covered when all of the following are met.

Nexviazyme criteria

Late-onset Pompe disease: Individual has late-onset Pompe disease and is at least 1 year of age.Age >=1 year
Confirmed GAA abnormality: Confirmed GAA enzyme deficiency from any tissue source, such as skin, fibroblasts, muscle, or blood, or two confirmed GAA gene mutations.
Weight-based dosing
- For actual body weight of at least 30 kg, the dose is 20 mg/kg every 2 weeks.Actual body weight >=30 kg; 20 mg/kg every 2 weeks
- For actual body weight below 30 kg, the dose is 40 mg/kg every 2 weeks.Actual body weight <30 kg; 40 mg/kg every 2 weeks

Initial Pombiliti therapy

Pombiliti is covered when all of the following are met.

Pombiliti criteria

Adult late-onset Pompe disease
- Individual is an adult with late-onset Pompe disease, including clinical signs and symptoms such as respiratory distress or skeletal-muscle weakness.
- Confirmed diagnosis: Deficiency of GAA enzyme from leukocytes, skin fibroblasts, muscle, or another tissue source, or confirmation of biallelic pathogenic variant(s) in the GAA gene.
Weight and prior enzyme-replacement therapy: Individual weighs at least 40 kg and has had an inadequate response or is unable to tolerate an enzyme-replacement therapy for Pompe disease, such as alglucosidase alfa or avalglucosidase alfa-ngpt.Weight >=40 kg

Experimental or investigational uses

Uses outside the covered indications are not covered unless supported as an accepted off-label use under the Company's off-label coverage policy.

Experimental or investigational uses: All other uses of Lumizyme and Nexviazyme are considered experimental/investigational and are not covered unless the indication is supported as an accepted off-label use under the Company medical policy on off-label coverage for prescription drugs and biologics.

Pombiliti must not be used in combination with other miglustat products, including Zavesca or Yargesa. It must be administered with oral miglustat, Opfolda.

Enzyme-replacement therapy is not recommended for individuals who have no symptoms or objective signs of Pompe disease, including proximal muscle weakness or reduced forced vital capacity (FVC) in either the upright or supine position.

HCPCS code J0220—injection of alglucosidase alfa, 10 mg, not otherwise specified—is listed as not eligible for reimbursement.

Applicable Procedure and Diagnosis Codes

CPT Procedure Code Number(s)CPT

N/A

ICD-10 Procedure Code Number(s)ICD-10

N/A

ICD-10 Diagnosis Code Number(s)ICD-10Covered

E74.02

Pompe disease

HCPCS Level II Code Number(s)HCPCSCovered

G0138	Intravenous infusion of cipaglucosidase alfa-atga, including provider/supplier acquisition and clinical supervision of oral administration of miglustat in preparation of receipt of cipaglucosidase alfa-atga
J0219	Injection, avalglucosidase alfa-ngpt, 4 mg
J0221	Injection, avalglucosidase alfa-ngpt, 4 mg
J1203	Injection, cipaglucosidase alfa-atga, 5 mg

Not Eligible for ReimbursementHCPCSNot Covered

J0220

Injection, alglucosidase alfa, 10 mg, not otherwise specified

Revenue Code Number(s)Revenue

N/A

Code added to represent avalglucosidase alfa-ngpt (Nexviazyme)HCPCS

J3590

Unclassified biologics

Pombiliti Minimum Weight

Minimum weightGreater than or equal to 40 kg

Applicable therapyPombiliti for adult individuals with late-onset Pompe disease

Related dosing20 mg/kg of actual body weight by intravenous infusion every other week

Documentation, Dosing, and Billing Responsibilities

Documentation Required

Medical-Record Documentation Required

The individual's medical record must reflect the medical necessity for the care provided. Maintain relevant records, which may include provider office, hospital, nursing home, home health, therapy, and test reports.

Prior Authorization

Support Nonstandard Dosing or Regimen Changes

The provider must submit supporting documentation, including published peer-reviewed literature, when requesting alglucosidase alfa (Lumizyme®) or avalglucosidase alfa-ngpt (Nexviazyme®) at an amount, dose, frequency, or regimen outside this policy's requirements. Any dose, frequency, or regimen change must also be submitted for new approval.

Changes may be based on member weight, incomplete therapeutic response, or other relevant factors.
The Company may modify dosing and frequency requirements based on FDA information, recognized pharmacology compendia, or published peer-reviewed research.

Pompe Disease Overview

Pompe disease is a rare inherited glycogen-storage disorder caused by absence or marked deficiency of lysosomal acid alpha-glucosidase (GAA), resulting in glycogen accumulation in cardiac, skeletal-muscle, and other tissues. Infantile-onset disease may cause cardiomyopathy, respiratory weakness, hypotonia, and early death, while juvenile- or adult-onset disease is characterized primarily by progressive skeletal-muscle and respiratory weakness. Enzyme-replacement therapy may improve cardiac and muscle function and reduce glycogen accumulation.

Key Clinical Terms

Pompe Disease

DefinitionA rare genetic disorder of glycogen metabolism caused by the absence or marked deficiency of lysosomal acid alpha-glucosidase (GAA)

Also known asGlycogen storage disease type II, GSD II, glycogenosis type II, or acid maltase deficiency

Disease effectGlycogen accumulates in cardiac, skeletal-muscle, and hepatic tissues, weakening affected organs

Late-Onset Pompe Disease (LOPD)

DefinitionLate-onset Pompe disease (LOPD) is Pompe disease presenting later in life with clinical signs and symptoms of the disease

Associated presentationRespiratory distress or skeletal-muscle weakness

Therapy-Specific Medical Necessity

Lumizyme

Lumizyme is covered for symptomatic Pompe disease when the diagnosis and dosing requirements are met.

Lumizyme initial therapy

Confirmed Pompe disease: Deficiency of GAA in leukocytes, skin fibroblasts, or muscle, or confirmation of biallelic pathogenic variant(s) in the GAA gene.
Symptomatic disease, age, and dosing: Individual has symptomatic infantile-onset or juvenile/adult-onset Pompe disease, is 1 month of age or older, and receives Lumizyme 20 mg/kg intravenously every 2 weeks.Age >=1 month; 20 mg/kg IV every 2 weeks

Nexviazyme

Nexviazyme is covered for late-onset Pompe disease when diagnosis, age, and weight-based dosing requirements are met.

Prior Therapy Requirement

Step	Requirement	Failure criterion
1	Prior enzyme-replacement therapy for Pompe disease, such as alglucosidase alfa or avalglucosidase alfa-ngpt, is required before Pombiliti coverage.	Inadequate response or inability to tolerate the prior enzyme-replacement therapy.

Dose and Frequency Limits

Lumizyme Quantity Limit

Quantity limit20 mg/kg

AdministrationIntravenous dose

FrequencyEvery 2 weeks

Nexviazyme Quantity Limits

Actual body weight at least 30 kg20 mg/kg every 2 weeks

Actual body weight below 30 kg40 mg/kg every 2 weeks

AdministrationIntravenous dosing based on actual body weight

Policy Summary

PayerAmeriHealth Caritas VIP Care

PolicyCoverage of enzyme replacement therapies for Pompe disease

Policy CodePolicy MA08.036g

Change TypeModified

Effective DateMarch 28, 2025

Last ReviewDec 15, 2025

Next Review DateN/A

Key ActionDocument medical necessity in the individual's medical record and provide records upon request.

SourceLink

On This Page

Combination and dosing requirement: Pombiliti is not used in combination with other miglustat products, is used with oral miglustat (Opfolda), and is administered at 20 mg/kg of actual body weight every other week by intravenous infusion.20 mg/kg actual body weight IV every other week

Other miglustat products include Zavesca and Yargesa.

Billing Rule

Use Accurate Billing Codes and Modifiers

Report all healthcare services, devices, and pharmaceuticals using the billing codes and modifiers that most accurately represent the services rendered, unless otherwise directed by the Company. Inclusion of a code does not guarantee reimbursement, and applicable eligibility, benefits, limitations, exclusions, precertification, referral requirements, contracts, and policies apply.

Coding requirements apply to applicable future coding changes, revisions, and updates.

Denial Risk

Noncovered Uses

All other uses of alglucosidase alfa (Lumizyme®) and avalglucosidase alfa-ngpt (Nexviazyme®) are considered experimental or investigational and are not covered unless the indication is supported as an accepted off-label use under the Company's policy for off-label coverage of prescription drugs and biologics.

Denial Risk

Missing Documentation May Result in Denial

All documentation must be available to the Company upon request. Failure to produce requested information may result in denial of coverage for the drug.

The Company may review and audit services regardless of the provider's participation status.

Step Therapy

Follow Pombiliti Miglustat Restrictions

Pombiliti must be used with oral miglustat (Opfolda) and must not be used in combination with other miglustat products, including Zavesca or Yargesa.

Required combination: oral miglustat (Opfolda).
Do not combine with Zavesca or Yargesa.

Confirmed by GAA enzyme deficiency from tissue or by biallelic pathogenic variants in the GAA gene

GAA

Full nameLysosomal acid alpha-glucosidase

Role in diagnosisDeficiency of GAA or confirmation of pathogenic GAA gene variants is used to confirm Pompe disease

Testing sourcesGAA deficiency may be identified in leukocytes, skin fibroblasts, muscle, blood, or another tissue source

Nexviazyme initial therapy

Late-onset Pompe disease: Individual has late-onset Pompe disease and is at least 1 year of age.Age >=1 year
Confirmed GAA abnormality: Confirmed GAA enzyme deficiency from any tissue source, such as skin, fibroblasts, muscle, or blood, or two confirmed GAA gene mutations.
Weight-based dosing
- For actual body weight of at least 30 kg, the dose is 20 mg/kg every 2 weeks.Actual body weight >=30 kg; 20 mg/kg every 2 weeks
- For actual body weight below 30 kg, the dose is 40 mg/kg every 2 weeks.Actual body weight <30 kg; 40 mg/kg every 2 weeks

Pombiliti

Pombiliti is covered for adult late-onset Pompe disease after inadequate response or intolerance to prior enzyme-replacement therapy and only with Opfolda.

Pombiliti initial therapy

Adult late-onset Pompe disease
- Individual is an adult with late-onset Pompe disease, including clinical signs and symptoms such as respiratory distress or skeletal-muscle weakness.
- Confirmed diagnosis: Deficiency of GAA enzyme from leukocytes, skin fibroblasts, muscle, or another tissue source, or confirmation of biallelic pathogenic variant(s) in the GAA gene.
Weight and prior enzyme-replacement therapy: Individual weighs at least 40 kg and has had an inadequate response or is unable to tolerate an enzyme-replacement therapy for Pompe disease, such as alglucosidase alfa or avalglucosidase alfa-ngpt.Weight >=40 kg
Combination and dosing requirement: Pombiliti is not used in combination with other miglustat products, is used with oral miglustat (Opfolda), and is administered at 20 mg/kg of actual body weight every other week by intravenous infusion.20 mg/kg actual body weight IV every other week
Other miglustat products include Zavesca and Yargesa.

Pombiliti Quantity Limit

Quantity limit20 mg/kg of actual body weight

AdministrationIntravenous infusion

FrequencyEvery other week