Centene Imiglucerase (Cerezyme) Coverage Update

Coverage Criteria

inv-01: Initial Therapy - Gaucher Disease

Covered when ALL of the following are met:

Initial Approval - Gaucher Disease: 1) Diagnosis of type 1 (GD1) or type 3 (GD3) Gaucher disease confirmed by enzyme assay demonstrating deficiency of beta-glucocerebrosidase activity OR by DNA testing; 2) Member is symptomatic (eg, anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly); 3) Cerezyme is not prescribed concurrently with VPRIV (velaglucerase alfa) or Elelyso (taliglucerase alfa); 4) Documentation of member's current weight (in kg); 5) Dose does not exceed 60 units/kg every two weeks.dose ≤60 U/kg q2w

All five elements required for initial approval.

inv-02: Continuation Therapy - Gaucher Disease

Covered for continuation when ALL of the following are met:

Continued Therapy - Gaucher Disease: 1) Member meets one of the following: a) currently receiving medication via Centene benefit or previously met initial approval criteria; or b) member is currently receiving medication and is enrolled in a state/product with continuity of care regulations; 2) Member is responding positively to therapy as evidenced by increased or stabilized platelet count or hemoglobin, reduced or stabilized spleen or liver volume, or decreased bone pain; 3) Cerezyme is not prescribed concurrently with VPRIV (velaglucerase alfa) or Elelyso (taliglucerase alfa); 4) Documentation of member's current weight (in kg); 5) If request is for a dose increase, new dose does not exceed 60 units/kg every two weeks.dose ≤60 U/kg q2w

All elements required for continued approval.

inv-03: Other/Off-Label Requests

Other indications or recent label changes:

Other Indications/Off-Label Handling: If the drug has undergone a label change within the last 6 months (eg, new indication, age expansion, or dosing regimen) or the requested use is not listed under section III, refer to the appropriate formulary/no-coverage/non-formulary or off-label use policy for the member's line of business (policy references provided in the policy).

Refer to CP.CPA.190, HIM.PA.33, CP.PMN.255 or other listed policies depending on formulary status and line of business.

inv-04: Initial and general coverage criteria

Covered when ALL of the following are met

FDA-approved indication and documentation: Imiglucerase is covered for FDA-approved indications including Gaucher disease (GD1 and GD3); prior authorization must include documentation consistent with the Prescribing Information and registry evidence.

FDA indication updated to include GD3; see prescribing information.

Dose documentation: Member's current weight must be documented for dose calculation; dosing must not exceed maximum recommendations in the Prescribing Information.

Max dosing recommendations added; vial size 400 units available.

Approval duration: Initial approvals are for 12 months as updated in the annual review.12 months

Initial approval duration updated to 12 months; see continuation criteria for renewal.

Non‑FDA approved indications that are not explicitly addressed in this policy are not authorized unless the provider submits sufficient documentation demonstrating efficacy and safety in accordance with the applicable off‑label use policy. Refer to policy CP.CPA.09 for Commercial, HIM.PA.154 for Health Insurance Marketplace/ICHRA, or CP.PMN.53 for Medicaid when submitting evidence for non‑labeled indications.

Inclusion or exclusion of any procedure or diagnosis codes in this policy is provided for informational purposes only and does not guarantee coverage. Coding alone is not sufficient to determine medical necessity; providers must supply clinical documentation that supports the request and follow current professional coding guidance prior to claim submission.

Combination therapy with other enzyme replacement modalities is not supported. There is currently insufficient clinical evidence to justify the combination use of enzyme replacement therapy with miglustat (Zavesca) or eliglustat (Cerdelga), or the concurrent administration of two or more enzyme replacement therapies.

Coding

Product coding (not specified)NDC

NDC not listed

Product prescribing information referenced but no NDCs listed in this part

HCPCS CodesHCPCS

J1786

Injection, imiglucerase, 10 units

inv-11: Maximum dose — 60 units/kg every 2 weeks

Maximum dose60 units/kg every 2 weeks

Dosing range (per PI)Recommended dosage ranges from 2.5 U/kg IV 3 times/week to 60 U/kg once every 2 weeks

Dose titrationTitrate dosage based on clinical manifestations and therapeutic goals

inv-12: Initial approval duration — 12 months

Initial approval duration (updated)12 months

Previous commercial durationCommercial: 6 months or to member renewal date (prior to update)

Provider Actions & Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Provider must submit documentation (such as office chart notes, lab results, or other clinical information) supporting that the member has met all approval criteria, including diagnostic confirmation (enzyme assay or DNA testing) and evidence of symptomatic disease.

Prior authorization required for imiglucerase (J1786).
Initial and continued therapy requests must include supporting clinical documentation.

Documentation Required

Member Weight Documentation

Provider must include documentation of the member's current weight (in kg) with prior authorization requests. Weight documentation is required for accurate dose calculation and to verify the requested dose does not exceed policy maximums.

Document weight in the prior authorization submission.

Background

Gaucher disease (types 1 and 3) is a lysosomal storage disorder that primarily affects visceral organs, bone marrow and bone, and commonly produces anemia, thrombocytopenia, hepatosplenomegaly and bone disease. Imiglucerase (Cerezyme) is an analogue of human beta‑glucocerebrosidase used as enzyme replacement therapy for the non‑central nervous system manifestations of Type 1 and Type 3 Gaucher disease. While enzyme replacement can ameliorate systemic manifestations such as hematologic and visceral involvement and improve bone disease, it does not alter neurological progression in neuronopathic forms of the disease. Measures used to assess therapeutic response include hemoglobin level, platelet count, liver and spleen volume, and bone pain.

Definitions

inv-22: Cerezyme / Imiglucerase — Imiglucerase (Cerezyme): an analogue of human beta-glucocerebrosidase used as enzyme replacement therapy for non-CNS manifestations of Ty...

Drug descriptionImiglucerase (Cerezyme) is an analogue of human beta-glucocerebrosidase used as enzyme replacement therapy for non‑CNS manifestations of Type 1 and Type 3 Gaucher disease

Indication summaryIndicated for treatment of non‑central nervous system manifestations of Gaucher disease (GD1 and GD3) in adults and pediatric patients

Therapeutic classEnzyme replacement therapy (beta‑glucocerebrosidase analogue)

inv-23: Therapeutic response measures — Measures of therapeutic response include hemoglobin level, platelet count, liver and spleen volume, and bone pain; stability thresholds i...

Primary response measuresHemoglobin level, platelet count, liver volume, spleen volume, and bone pain

Initial Therapy Criteria

inv-25: Initial Therapy — Initial authorization criteria and approval duration

Initial authorization criteria and approval duration

Initial Therapy Nodes: Diagnosis confirmed by enzyme assay or DNA testing; member is symptomatic; Cerezyme not used concurrently with velaglucerase alfa or taliglucerase alfa; documentation of current weight in kg; dose ≤60 U/kg every two weeks.dose ≤60 U/kg q2w

Approval durations: Medicaid/HIM/ICHRA 12 months; Commercial 6 months or to the member's renewal date, whichever is longer.

inv-26: Initial therapy — Initial therapy rules updated

Initial therapy rules updated

Initial authorization duration updated to 12 months; prior authorization requests must include member weight and dosing consistent with the Prescribing Information.12 months

Continuation Therapy Criteria

inv-27: Continuation Therapy — Continuation approval requirements and duration by line of business

Continuation approval requirements and duration by line of business

Continuation Therapy Nodes: See Continued Therapy - Gaucher Disease criteria: member meets continuity/previous approval criteria; evidence of positive response; Cerezyme not used concurrently with other ERTs; documentation of current weight; dose limits upheld. Approvals: Medicaid/HIM/ICHRA 12 months; Commercial 6 months or to renewal date.dose ≤60 U/kg q2w

Documentation of positive response required for continuation.

inv-28: Continuation therapy — Continuation therapy provisions were updated in template changes; details available in other sections of the full policy.

Continuation therapy provisions were updated in template changes; details available in other sections of the full policy.

Continuation approvals and monitoring follow prior authorization policies and Prescribing Information; template updates applied to continuation therapy section.

Step Therapy

Step	Requirement / Notes
1	Concurrent use with other enzyme replacement therapies (e.g., VPRIV/velaglucerase alfa or Elelyso/taliglucerase alfa) is not supported; combination use with miglustat (Zavesca) or eliglustat (Cerdelga) is not supported. No formal step therapy sequence is specified in this policy.

Step	Description
Step 1	No formal initial step required; requests must comply with coverage criteria (diagnostic confirmation, symptomatic disease, weight documentation, and dosing within recommended limits). Concurrent or combination use with other ERTs, miglustat, or eliglustat is not supported.

Quantity Limits

inv-31: Imiglucerase (Cerezyme) — Maximum 60 U/kg every 2 weeks

Maximum dosing limitMaximum 60 U/kg every 2 weeks

Dosing guidanceRecommended dosage ranges from 2.5 U/kg IV 3 times/week to 60 U/kg q2w; titrate to clinical goals

Administration routeIntravenous infusion

inv-32: Imiglucerase (Cerezyme) vial — 400 units per vial (product availability)

Vial size400 units per vial

Product availability noteVial packaging: single‑use 400‑unit vials per product availability section

Dose calculation implication

Site of Care

Note

Administration by IV infusion (infusion center)

IV infusion is the administration route specified for imiglucerase (Cerezyme); specific site-of-care requirements are not detailed in this section of the policy.

Administration route: IV infusion (per dosing and administration section)
No additional site-of-care constraints are specified in the policy