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    v1.0.31
    OpenPayerPolicy UpdatesPharmacy & Specialty DrugsAmeriHealth Caritas Gaucher Prior Auth | OpenPayer
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    ModifiedAmerihealth Caritas Vip CarePolicy MA08.023d

    Enzyme Replacement for the Treatment of Gaucher's Disease

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    This policy governs medical-benefit coverage and prior authorization for imiglucerase, taliglucerase alfa, and velaglucerase alfa as long-term enzyme replacement therapy for pediatric and adult members with specified forms of Gaucher disease. Coverage requires a confirmed diagnosis, applicable clinical manifestations, and compliance with drug-specific dosing and frequency requirements.

    Policy Summary
    PayerAmerihealth Caritas Vip Care
    PolicyEnzyme Replacement for the Treatment of Gaucher's Disease
    Policy CodePolicy MA08.023d
    Change TypeModified
    Effective DateMarch 20, 2026
    Next Review DateN/A
    Key ActionPrior approval is required for the requested drug, dose, and frequency, and a new approval must be requested when the member's dose, frequency, or regimen changes.
    SourceLink
    POLICY UPDATE CHANGES

    HCPCS code J0205 for alglucerase was deleted from the policy.

    Long-term enzyme replacement therapy with taliglucerase alfa was added for pediatric individuals.

    Dosing and frequency requirements for covered enzyme replacement agents and laboratory and/or genetic testing requirements were added.

    Alglucerase was changed from medically necessary to not eligible for reimbursement.

    OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.

    3Covered enzyme replacement drugs
    Type 1 or Type 3Eligible Gaucher disease forms
    2.5-60 U/kgImiglucerase initial dosing range
    Every 2 weeksCommon administration interval
    1Drug no longer reimbursable

    Medical Necessity and Coverage Scope

    Initial medical necessity

    Covered when all applicable criteria are met.

    • Covered diagnosis

      • Type 1 Gaucher disease: Confirmed Type 1 Gaucher disease.
      • Type 3 Gaucher disease: Clinical nonneuropathic manifestations of Type 3 Gaucher disease.

    • Diagnostic confirmation

      • Enzyme deficiency: Deficiency of glucosylceramidase in peripheral blood leukocytes or other nucleated cells.

      • GBA gene confirmation

        • Qualifying manifestation

          • Anemia.
          • Bone disease.
          • Hepatomegaly or splenomegaly.
          • Thrombocytopenia.
    • Drug-specific dosing and frequency: The requested imiglucerase, taliglucerase alfa, or velaglucerase alfa regimen conforms to the applicable dosing and frequency requirements.

    Coverage under this policy is limited to long-term enzyme replacement therapy for pediatric and adult members with a confirmed diagnosis of Type 1 Gaucher disease or clinical nonneuropathic manifestations of Type 3 Gaucher disease. All applicable diagnostic confirmation and drug-specific dosing and frequency requirements must be met. Other uses of imiglucerase, taliglucerase alfa, or velaglucerase alfa are considered experimental/investigational and are not covered unless supported as an accepted off-label use under the Company's applicable off-label coverage policy.

    This policy addresses enzyme replacement therapy covered under the member's medical benefit (Part B), subject to the applicable Evidence of Coverage and the policy's medical-necessity, dosing, and frequency requirements. It does not address coverage under the pharmacy benefit (Part D); coverage may depend on how the drug is prescribed, dispensed, or administered.

    Diagnosis and Drug Codes

    Covered ICD-10 Diagnosis CodesICD-10Covered
    E75.22Gaucher disease
    Covered HCPCS CodesHCPCSCovered
    J1786Injection, imiglucerase, 10 units
    J3060Injection, taliglucerace alfa, 10 units
    J3385Injection, velaglucerase alfa, 100 units

    Authorization, Documentation, and Coverage Risks

    Prior Authorization

    Prior Authorization for Dose and Frequency

    The Company requires prior approval for the requested drug, dose, and frequency of imiglucerase (Cerezyme®), velaglucerase alfa (VPRIV®), or taliglucerase alfa (Elelyso®) under the member’s medical benefit.

    • Submit the requested dose and frequency for utilization-management review before treatment.
    Billing Rule

    Medical-Benefit Coverage Criteria

    Medical-benefit coverage for enzyme replacement therapy for Gaucher’s disease applies when medical necessity criteria, including the policy’s dosing and frequency requirements, are met. This section addresses coverage under the medical benefit (Part B), not the pharmacy benefit (Part D).

    • Coverage remains subject to the applicable Evidence of Coverage.

    Disease and Treatment Context

    Gaucher disease is a rare inherited metabolic disorder caused by deficient glucocerebrosidase, leading to accumulation of glucosylceramide in organs and tissues. Type 1 disease is nonneuronopathic and may involve anemia, thrombocytopenia, hepatosplenomegaly, and bone disease. Enzyme replacement therapy may reduce systemic manifestations of Type 3 disease but does not improve its neurologic manifestations; it does not alter the neurodegenerative course of Type 2 disease.

    Key Terms

    Gaucher disease
    DefinitionA rare, genetic, metabolic disorder.
    Underlying deficiencyDeficiency of the enzyme glucocerebrosidase.
    Resulting accumulationAccumulation of glucosylceramide in storage cells of the liver, spleen, bone marrow, and lymph nodes.
    Enzyme replacement therapy
    DefinitionLong-term replacement of the missing glucocerebrosidase enzyme.
    Therapeutic purposeMay reduce systemic manifestations of Gaucher disease, including those associated with Type 3 disease.
    Neurologic manifestations

    Initial Enzyme Replacement Therapy Criteria

    Drug-Specific Dosing and Frequency

    Imiglucerase (Cerezyme)
    Initial dosage range2.5 U/kg of body weight 3 times a week to 60 U/kg once every 2 weeks.
    Maintenance dosage30–60 U/kg every 2 weeks.
    Administration frequencyInitial therapy may be administered 3 times weekly or once every 2 weeks, depending on the dose; maintenance therapy is every 2 weeks.
    Taliglucerase alfa (Elelyso)
    Initial dose60 units/kg every 2 weeks.
    Subsequent dosingIndividualized based on achievement and maintenance of therapeutic goals.
    Switching from imiglucerase

    Administration Setting

    Billing Rule

    Home administration remains eligible

    Enzyme replacement intravenous therapy remains eligible for administration in a home setting, although home infusion code S9357 was removed from the policy document.

    • Home administration remains eligible.
    • Code S9357 was removed from the policy document.
    Policy Summary
    PayerAmerihealth Caritas Vip Care
    PolicyEnzyme Replacement for the Treatment of Gaucher's Disease
    Policy CodePolicy MA08.023d
    Change TypeModified
    Effective DateMarch 20, 2026
    Next Review DateN/A
    Key ActionPrior approval is required for the requested drug, dose, and frequency, and a new approval must be requested when the member's dose, frequency, or regimen changes.
    SourceLink
    On This Page
    Medical Necessity and Coverage ScopeDiagnosis and Drug CodesAuthorization, Documentation, and Coverage RisksDisease and Treatment ContextKey TermsInitial Enzyme Replacement Therapy CriteriaDrug-Specific Dosing and FrequencyAdministration Setting
  1. The drug’s benefit classification may depend on how it is prescribed, dispensed, or administered.

    2. Unsupported block type: documentation

    Unsupported block type: documentation

    Prior Authorization

    Update Treatment Information

    Submit accurate member and treatment information whenever the approved dose, frequency, or regimen changes so the Company can issue a new approval based on the updated request.

    • Report changes related to member weight, incomplete therapeutic response, or other treatment factors.
    • The Company may conduct postpayment reviews and audits of submitted claims.
    Denial Risk

    Alglucerase Not Eligible for Reimbursement

    Alglucerase (Ceredase®) is no longer manufactured and has been withdrawn from the market; it is not eligible for reimbursement.

    Denial Risk

    Uncovered or Investigational Use

    All other uses of imiglucerase (Cerezyme®), velaglucerase alfa (VPRIV™), or taliglucerase alfa (Elelyso™) are considered experimental or investigational and are not covered unless the indication is supported as an accepted off-label use under the Company’s medical policy for off-label coverage of prescription drugs and biologics.

    • Verify that the requested indication is covered before treatment is provided.
    Denial Risk

    Missing Records May Result in Denial

    The Company may review or audit services regardless of the provider’s participation status. Failure to produce requested medical records or other documentation may result in denial of the drug.

    • Maintain documentation that establishes medical necessity and supports the billed service.
    • Provide requested information promptly during Company reviews or audits.
    Denial Risk

    Unsupported Dosing Exception

    Exceptions to the listed dosing and frequency requirements require supporting published peer-reviewed literature demonstrating the basis for the requested amount or schedule.

    • Submit the literature with the exception request for imiglucerase (Cerezyme®), velaglucerase alfa (VPRIV®), or taliglucerase alfa (Elelyso®).
    • Requests without supporting evidence may not be approved.
    ERT does not affect the neurologic manifestations of Gaucher disease.
    For stable individuals switching from imiglucerase therapy, use the same unit/kg dose.
    Velaglucerase alfa (VPRIV)
    Treatment-naive adults60 Units/kg administered every other week.
    Treatment-naive pediatric individuals60 Units/kg administered every other week for pediatric individuals 4 years of age and older.
    Subsequent dosingDosages are further individualized.