ModifiedmhnPolicy CP.PHAR.163

Velaglucerase Alfa (VPRIV) Coverage Criteria

Defines medical necessity, prior authorization and continuation criteria for VPRIV (velaglucerase alfa) for treatment of Gaucher disease for commercial, HIM, and Medicaid lines of business.

Policy Summary

Payermhn

PolicyVelaglucerase Alfa (VPRIV) Coverage Criteria

Policy CodePolicy CP.PHAR.163

Change TypeMaterial updates: boxed warning addedIL HIM step therapy bypassdosing/weight documentation clarified

Effective DateFeb 1, 2016

Next Review Date

Key ActionObtain prior authorization and document diagnosis, confirmatory enzyme assay or DNA test, current weight in kg, and ensure dose does not exceed 60 units/kg every 2 weeks.

SourceLink

POLICY UPDATE CHANGES

Added boxed warning from the prescribing information.

Added step therapy bypass for Illinois HIM per IL HB 5395.

Added explicit weight documentation requirement and maximum dose limit when switching from imiglucerase.

Added redirections to Cerdelga and Cerezyme and added Appendix B; added HIM line of business and retired HIM.PA.163.

Added step therapy bypass for IL HIM per IL HB 5395.

60 U/kgmaximum dose every two weeks

≥4 yrsminimum age

6 monthsapproval duration

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Coverage Criteria

inv-01: Initial Therapy

Covered when ALL of the following are met:

Initial Approval - Gaucher Disease: 1) Diagnosis of type 1 (GD1) or type 3 (GD3) confirmed by enzyme assay demonstrating deficiency of beta-glucocerebrosidase activity OR DNA testing; 2) Age ≥ 4 years; 3) Member is symptomatic (e.g., anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly); 4) For GD1: failure of Cerezyme and Cerdelga unless clinically significant adverse effects or both contraindicated; OR for GD3: failure of Cerezyme unless contraindicated or clinically significant adverse effects; 5) VPRIV is not prescribed concurrently with Elelyso (taliglucerase alfa) or Cerezyme (imiglucerase); 6) Documentation of member's current weight (kg); 7) Dose does not exceed 60 units/kg every two weeks.

inv-02: Continuation Therapy

Covered when ALL of the following are met:

Continuation Therapy - Gaucher Disease: 1) Member currently receiving medication via Centene benefit or has previously met initial approval criteria OR member is 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) VPRIV is not prescribed concurrently with Elelyso (taliglucerase alfa) or Cerezyme (imiglucerase); 4) Documentation of member's current weight (kg); 5) Dose does not exceed 60 units/kg every two weeks.

Requests for indications that are not FDA‑approved and are not specifically addressed in this policy are not routinely authorized. Such off‑label requests must be supported by sufficient clinical documentation demonstrating efficacy and safety in accordance with the applicable off‑label use policies (see CP.CPA.09 for Commercial, HIM.PA.154 for Marketplace, and CP.PMN.53 for Medicaid) or other evidence of coverage. Providers should submit the clinical rationale and supporting literature or evidence with the prior authorization request to allow appropriate review.

When there is a conflict between this clinical policy and state Medicaid coverage provisions, the state Medicaid requirements take precedence. Providers managing Medicaid members should follow the applicable state Medicaid manual and any state‑specific coverage directives rather than this policy where discrepancies exist.

Combination therapy involving enzyme replacement therapy (ERT) with miglustat (Zavesca) or eliglustat (Cerdelga), or the concurrent use of two or more different ERT products, is not supported by sufficient clinical evidence and therefore is not covered under this policy. Prior authorization requests that propose concurrent use of ERT with these oral agents or simultaneous use of multiple ERTs should include compelling clinical justification and supporting evidence; absent that, requests will be denied.

Coding and Billing

HCPCS CodesHCPCS

J3385

Injection, velaglucerase alfa, 100 units

Coding implications and redirectionsmixed

No codes listed

inv-08: Maximum dose

Maximum dose limitDose does not exceed 60 units/kg every two weeks

Dosing frequencyEvery 2 weeks (q2w)

ApplicabilityApplies to initial and continuation therapy criteria

inv-09: N/A

StatusReserved — no applicable data

NotesPlaceholder field retained for inventory completeness

ActionNo content required at this time

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required for VPRIV

Prior authorization is required for VPRIV. Providers must submit a PA request and supporting documentation demonstrating the diagnosis and eligibility criteria (e.g., diagnosis, enzyme assay or DNA testing confirming Gaucher disease, age, and symptomatic status). Failure to supply required documentation may result in denial.

Prior authorization required for VPRIV (velaglucerase alfa).
Include documentation of diagnosis: enzyme assay demonstrating beta‑glucocerebrosidase deficiency or DNA testing.
Include member age (must be ≥ 4 years for initial approval) and evidence of symptoms (anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly).

Billing Rule

Prior Authorization and Coding Updates

Initial Therapy Criteria

inv-22: Initial Therapy

Covered when ALL of the following are met:

Initial Therapy - Gaucher Disease (Detailed): 1) Diagnosis of type 1 (GD1) or type 3 (GD3) confirmed by one of: a) enzyme assay demonstrating deficiency of beta-glucocerebrosidase activity; or b) DNA testing; 2) Age ≥ 4 years; 3) Member is symptomatic (e.g., anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly); 4) Step therapy: a) For GD1: documented failure of Cerezyme and Cerdelga unless clinically significant adverse effects or both contraindicated; b) For GD3: documented failure of Cerezyme unless contraindicated or clinically significant adverse effects; (Note: For Illinois HIM requests, step therapy requirements do not apply as of 1/1/2026 per IL HB 5395); 5) VPRIV is not prescribed concurrently with Elelyso (taliglucerase alfa) or Cerezyme (imiglucerase); 6) Documentation of member's current weight (kg); 7) Dose does not exceed 60 units/kg every two weeks; 8) If switching from imiglucerase, initiate VPRIV at the prior imiglucerase dose two weeks after the last imiglucerase dose.

Continuation Therapy Criteria

inv-23: Continuation Therapy

Covered when ALL of the following are met:

Continuation Therapy - Requirements: 1) Member currently receiving medication via Centene benefit or has previously met initial approval criteria OR member is currently receiving medication and enrolled in a state/product with continuity of care regulations; 2) Evidence of clinical response: increased or stabilized platelet count or hemoglobin, reduced or stabilized spleen or liver volume, or decreased bone pain; 3) VPRIV is not prescribed concurrently with Elelyso (taliglucerase alfa) or Cerezyme (imiglucerase); 4) Documentation of member's current weight (kg); 5) Dose does not exceed 60 units/kg every two weeks; 6) Continued adherence to dosing and safety monitoring per prescribing guidance (including consideration of hypersensitivity risk).

Step Therapy Requirements

Step	Prior therapy required before VPRIV	Exceptions / Notes
1
For GD1: failure of Cerezyme (imiglucerase) and Cerdelga (eliglustat) required prior to VPRIV
Unless clinically significant adverse effects are experienced or both agents are contraindicated; VPRIV not to be prescribed concurrently with Elelyso or Cerezyme; documentation of current weight (kg) and dose limit ≤ 60 units/kg every 2 weeks required

State/Line of Business	Step therapy bypass	Effective / Reference
Illinois HIM
Step therapy requirements do not apply (bypass) for Illinois HIM requests per IL HB 5395
Bypass effective as of 1/1/2026 per policy note; documented in policy updates (see coding/changes)

Quantity Limits and Dosing

inv-26: Velaglucerase alfa (VPRIV)

Vial sizeSingle-use vial: 400 units

DosingIndividualized dosing up to 60 units/kg every 2 weeks

AdministrationIntravenous infusion per dosing regimen; switch from imiglucerase 2 weeks after last imiglucerase dose

Weight documentationDocumentation of member's current weight (in kg) is required

Site of Care

Billing Rule

Infusion center administration and timing when switching from imiglucerase

VPRIV is administered intravenously in an infusion center per the dosing regimen. When switching from imiglucerase (Cerezyme), initiate VPRIV at the prior imiglucerase dose two weeks after the last imiglucerase infusion; document infusion site and timing.

Route: IV infusion in an infusion center
Switching from imiglucerase: start VPRIV at prior imiglucerase dose 2 weeks after last imiglucerase dose
Document infusion site, dose, and timing in clinical records

Background

Velaglucerase alfa (VPRIV) is a recombinant lysosomal glucocerebrosidase used as an enzyme replacement therapy (ERT) for the long‑term treatment of type 1 Gaucher disease and, when criteria are met, some patients with type 3. Gaucher disease commonly presents with anemia, thrombocytopenia, hepatosplenomegaly, and bone involvement; therapeutic response to ERT is monitored using hematologic parameters (hemoglobin and platelet count), organ volumes (liver and spleen), and assessment of bone pain. Dosing is individualized and guided by weight, with a documented maximum of 60 units/kg every 2 weeks. Clinicians should be aware of hypersensitivity risks (including anaphylaxis) in the product labeling and follow recommended monitoring during infusion.

Definitions

inv-19: Enzyme replacement therapy abbreviation (ERT)

AbbreviationERT = enzyme replacement therapy

ContextUsed throughout policy to refer to enzyme replacement therapies for Gaucher disease

Appendix referenceDefined in Appendix A: Abbreviation/Acronym Key

inv-20: Gaucher disease subtypes

GD1Type 1 Gaucher disease

GD3Type 3 Gaucher disease

Diagnostic confirmationDiagnosis of GD1 or GD3 must be confirmed by enzyme assay or DNA testing per initial criteria