CurrentBlue Shield of CaliforniaPolicy N/A

Agalsidase beta (Fabrazyme)

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This policy governs medical-necessity review, prior authorization, dosing, coding, and site-of-care requirements for agalsidase beta (Fabrazyme) administered under the commercial medical benefit for Fabry disease.

Policy Summary

PayerBlue Shield of California

PolicyAgalsidase beta (Fabrazyme)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date07/01/2026

Next Review DateN/A

Key ActionObtain prior authorization or preservice review and submit documentation confirming a GLA gene mutation, age 2 years or older, and no concurrent migalastat use.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

Age 2+Minimum patient age

1 mg/kg q2wCovered intravenous dose

IndefiniteCoverage period

E75.21Fabry disease diagnosis code

First 2 doses

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CurrentBlue Shield of CaliforniaPolicy N/A

Agalsidase beta (Fabrazyme)

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Policy Summary

PayerBlue Shield of California

PolicyAgalsidase beta (Fabrazyme)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date07/01/2026

Next Review DateN/A

Key ActionObtain prior authorization or preservice review and submit documentation confirming a GLA gene mutation, age 2 years or older, and no concurrent migalastat use.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

Age 2+Minimum patient age

1 mg/kg q2wCovered intravenous dose

IndefiniteCoverage period

E75.21Fabry disease diagnosis code

First 2 doses

Hospital outpatient allowance

Coverage Criteria

Fabry disease initial coverage

Covered when ALL of the following are met:

Required criteria

GLA gene mutation: Patient has a galactosidase-alpha (GLA) gene mutationDocumented GLA gene mutation
Age requirement: Patient is 2 years of age and olderAge ≥ 2 years
No concomitant migalastat: Fabrazyme is not being used with migalastat (Galafold)No concurrent migalastat use

Concurrent use of Fabrazyme (agalsidase beta) with migalastat (Galafold) is excluded from the medical-necessity criteria. Coverage is not supported when both therapies are being used together.

Coding and Administration Parameters

Covered HCPCS CodesHCPCSCovered

J0180

Injection, agalsidase beta, 1 mg

ICD-10 Diagnosis CodesICD-10

E75.21

Fabry disease

Minimum age

Minimum age2 years and older

Coverage criterionPatient is 2 years of age and older

Medical-necessity thresholdAge ≥ 2 years

Covered dose and frequency

Maximum doseUp to 1 mg/kg

RouteIntravenously

Dosing intervalEvery 2 weeks

Provider Requirements and Review Outcomes

Prior Authorization

Prior Authorization Required for Fabry Disease

Prior authorization or preservice review is required for Fabrazyme (agalsidase beta) for Fabry disease. Coverage requires that all medical-necessity criteria are met.

The patient has a galactosidase-alpha (GLA) gene mutation.
The patient is 2 years of age or older.
Fabrazyme is not being used concurrently with migalastat (Galafold).

Documentation Required

Medical Necessity Documentation

Submit documentation supporting all medical-necessity requirements before treatment is reviewed. Documentation may include office chart notes, laboratory results, and other relevant clinical information.

Confirm the diagnosis of Fabry disease and document the GLA gene mutation.

Background

Fabrazyme (agalsidase beta) is a biosynthetic recombinant-DNA form of human alpha-galactosidase used as enzyme-replacement therapy. This policy addresses Fabrazyme treatment for Fabry disease, including medical-necessity review, prior authorization, dosing, coding, and administration requirements under the commercial medical benefit.

Definitions

Agalsidase beta

DrugAgalsidase beta

Biologic formBiosynthetic recombinant-DNA form of human alpha-galactosidase

USP categoryGenetic or enzyme or protein disorder: replacement, modifiers, treatment

Fabry disease

ConditionFabry disease

Policy statusFabry disease is listed as a condition covered under this policy, subject to the coverage criteria

Coverage-related requirementMedical necessity requires a documented galactosidase-alpha (GLA) gene mutation

Initial Therapy

Initial therapy for Fabry disease

Prior authorization or preservice review is required. Coverage is medically necessary when ALL of the following are met:

Initial Fabrazyme therapy

Required criteria
- GLA gene mutation: Patient has a galactosidase-alpha (GLA) gene mutationDocumented GLA gene mutation
- Minimum age: Patient is 2 years of age and olderAge ≥ 2 years
- Concomitant therapy restriction:

Quantity and Dosing Limits

Agalsidase beta (Fabrazyme)

DrugAgalsidase beta (Fabrazyme)

Quantity limitUp to 1 mg/kg

FrequencyEvery 2 weeks

Site-of-Care Requirements

Step Therapy

Use a preferred site of care when required

Members in specified plans may be required to receive Fabrazyme at a preferred site of service: the home, a physician's office, or an independent infusion center not associated with a hospital. Hospital outpatient administration is permitted only when the member cannot receive infusions in a preferred home or ambulatory setting and meets an approved criterion.

Applicable plans include PPO, Direct Contract HMO, and, when applicable, ASO, Shared Advantage, and HMO (non-direct).
Hospital outpatient administration may be used for the first 2 doses of new or re-initiated therapy.
Additional clinical monitoring criteria require supporting documentation.

Step Therapy

Use home administration when applicable

When applicable under the member's plan, the member's home is a preferred administration setting for Fabrazyme. Confirm that home administration is available before using a hospital outpatient facility.

Preferred settings may include the member's home.
Hospital outpatient administration requires that the member cannot receive the infusion in the preferred home or ambulatory setting and meets an approved criterion.

Policy Summary

PayerBlue Shield of California

PolicyAgalsidase beta (Fabrazyme)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date07/01/2026

Next Review DateN/A

Key ActionObtain prior authorization or preservice review and submit documentation confirming a GLA gene mutation, age 2 years or older, and no concurrent migalastat use.

SourceLink

On This Page

Document the patient's age and relevant treatment history.

Document that Fabrazyme will not be used with migalastat (Galafold).

Documentation Required

Hospital Outpatient Site-of-Care Documentation

Hospital outpatient administration requires supporting documentation and is available only when the member cannot receive infusions in the preferred home or ambulatory setting and at least one hospital outpatient site-of-care criterion is met.

Preferred sites may include the home, a physician's office, or an independent infusion center not associated with a hospital, depending on the member's plan.
Document the applicable hospital outpatient criterion and why administration in the preferred home or ambulatory setting is not appropriate.
Applicable plans include PPO, Direct Contract HMO, and, when applicable, ASO, Shared Advantage, and non-direct HMO plans.

Billing Rule

Submit HCPCS Code and Corresponding NDC

For billing, submit Fabrazyme using the drug's assigned HCPCS code and the corresponding NDC. Do not use an unlisted, unspecified, or miscellaneous code when a specific drug code is assigned.

Include both the assigned HCPCS code listed in the drug policy and the corresponding national drug code (NDC).
Prohibited substitutes include unlisted, unspecified, or miscellaneous codes when a specific code is available.

Denial Risk

Failure to Meet Fabry Disease Criteria

Coverage may be denied when Fabrazyme is used without a documented GLA gene mutation, for a patient younger than 2 years, or concurrently with migalastat (Galafold).

GLA gene mutation is required.
Minimum age is 2 years.
Concurrent use with migalastat (Galafold) is not covered under these criteria.

Denial Risk

Unsupported Hospital Outpatient Administration

Administration in a hospital outpatient facility is not supported unless the member cannot receive infusions in the preferred home or ambulatory setting and one of the specified hospital outpatient criteria is documented. New therapy or re-initiation after at least 6 months off therapy supports only the first 2 doses; subsequent doses require medical necessity for continued hospital outpatient administration.

Starting new Fabrazyme therapy: hospital outpatient administration is allowed for the first 2 doses; continued use requires subsequent medical-necessity documentation.
Re-initiating Fabrazyme after being off therapy for at least 6 months: hospital outpatient administration is allowed for the first 2 doses; continued use requires subsequent medical-necessity documentation.
Additional clinical monitoring may support hospital outpatient administration when documentation shows: a previous severe adverse event; ongoing moderate to severe adverse events despite premedication such as acetaminophen, steroids, diphenhydramine, or fluids; clinical instability; or physical or cognitive instability.
Supporting documentation must be submitted for the applicable criterion. Without the required site-of-care conditions and documentation, hospital outpatient administration is not supported.

Unsupported block type: documentation

Fabrazyme is not being used with migalastat (Galafold)

No concurrent migalastat use

Step Therapy

Use physician office administration when applicable

When applicable under the member's plan, a physician's office is a preferred administration setting for Fabrazyme. Confirm that office administration is appropriate before using a hospital outpatient facility.

Preferred settings may include a physician's office.
Hospital outpatient administration requires that the member cannot receive the infusion in the preferred home or ambulatory setting and meets an approved criterion.

Step Therapy

Use a non-hospital-affiliated infusion center

When applicable under the member's plan, use an independent infusion center that is not associated with a hospital as a preferred administration setting. A hospital-affiliated infusion center does not meet this preferred-site description.

The independent infusion center must not be associated with a hospital.
Hospital outpatient administration requires separate qualifying criteria and supporting documentation.