CurrentCommunity-CarePolicy N/A

Xenpozyme (olipudase alfa) Medical Benefit Medication Utilization Policy

Defines medical-benefit coverage criteria, quantity limits, authorization periods, continuation criteria, and applicable coding for olipudase alfa (Xenpozyme) for treatment of acid sphingomyelinase deficiency (ASMD).

Policy Summary

PayerCommunity-Care

PolicyXenpozyme (olipudase alfa) Medical Benefit Medication Utilization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionSubmit prior authorization with specialist involvement, confirmatory enzyme assay and documentation of non-CNS ASMD manifestations, and include patient weight for dosing approval.

SourceLink

POLICY UPDATE CHANGES

No material changes to clinical coverage or policy criteria.

1Covered Indication (ASMD)

6 mg/kgAuthorized Dose Limit

6 mo / 1 yrAuth Period (initial/continuation)

Coverage Summary

Coverage stance: covered_with_criteria. This policy defines medical-benefit coverage criteria for olipudase alfa (Xenpozyme) for treatment of acid sphingomyelinase deficiency (ASMD), including quantity limits, authorization periods, continuation criteria, and applicable coding. Initial approval requires all of the following: prescribed by or in consultation with a hepatologist, pulmonologist, medical geneticist, or a provider specializing in lysosomal storage disorders; a diagnosis of ASMD with specification of phenotype (Type A, Type A/B, or Type B); documentation of an enzyme assay demonstrating deficiency in acid sphingomyelinase activity; and documentation of non-central nervous system manifestations (e.g., hepatomegaly, splenomegaly, interstitial lung disease). Continuation of therapy requires ongoing diagnosis of ASMD and documentation of improvement in clinical signs and symptoms (e.g., splenomegaly, hepatomegaly, pulmonary function). Quantity limits authorize dosing up to 6 mg/kg every 28 days. Initial authorization is allowed for 6 months and continuation authorization for 1 year. Applicable coding includes HCPCS J3590 (Unclassified biologics).

Initial Therapy Criteria

Initial Approval

Covered when ALL of the following are met:

ALL of the following

Prescribed by, or in consultation with, a hepatologist, pulmonologist, medical geneticist, or provider who specializes in the treatment of lysosomal storage disorders
Diagnosis of acid sphingomyelinase deficiency (ASMD)
ASMD phenotype
- Type A
- Type A/B
- Type B
Documentation of an enzyme assay demonstrating deficiency in acid sphingomyelinase activity
Documentation of non-central nervous system ASMD manifestations (e.g. hepatomegaly, splenomegaly, interstitial lung disease, etc.)

Prior Authorization & Provider Requirements

Prior Authorization

Prior authorization and documentation requirements

Prior authorization is required. Documentation must include: prescribing by or consultation with a specialist (hepatologist, pulmonologist, medical geneticist, or provider who specializes in lysosomal storage disorders), confirmed diagnosis of acid sphingomyelinase deficiency (ASMD), enzyme assay results demonstrating deficient acid sphingomyelinase activity, specification of the ASMD phenotype (Type A, Type A/B, or Type B), documentation of non‑central nervous system ASMD manifestations (e.g., hepatomegaly, splenomegaly, interstitial lung disease), and the patient’s weight for approval.

J3590

Documentation Required

Continuation documentation

For continuation of therapy, submit documentation confirming the diagnosis of ASMD and objective improvement in clinical signs and symptoms (for example improvement in splenomegaly, hepatomegaly, pulmonary function, etc.).

Billing Rule

Quantity limit

If approved, dosing will be authorized up to 6 mg/kg every 28 days. A patient weight is required for approval.

J3590

Prior Authorization

Authorization period

Initial authorization is allowed for 6 months. Continuation authorization is allowed for 1 year. All authorizations are subject to benefit changes.

Background

Xenpozyme (olipudase alfa) is a biologic therapy indicated for acid sphingomyelinase deficiency (ASMD). For coverage, the policy requires specialist involvement (prescribed by or in consultation with a hepatologist, pulmonologist, medical geneticist, or provider who treats lysosomal storage disorders), a confirmatory enzyme assay demonstrating deficient acid sphingomyelinase activity, and documentation of non-CNS ASMD manifestations (examples: hepatomegaly, splenomegaly, interstitial lung disease). Definition: ASMD = Acid sphingomyelinase deficiency.