CurrentEmpire BluecrossPolicy CC-0153

Adakveo (crizanlizumab) — coverage criteria for sickle cell disease

Clinical criteria for medical-benefit coverage and prior authorization of Adakveo (crizanlizumab) for members with sickle cell disease, including initial and continuation criteria, quantity limits, coding, and documentation expectations.

Policy Summary

PayerEmpire Bluecross

PolicyAdakveo (crizanlizumab) — coverage criteria for sickle cell disease

Policy CodePolicy CC-0153

Change TypeClarified codingrevised continuation criteria

Effective Date07/01/2025

Next Review Date

Key ActionSubmit prior authorization with documentation of diagnosis of sickle cell disease and records showing at least two sickle cell–related crises in the prior 12 months; for continuation, document reduction in acute complications since initiation.

SourceLink

POLICY UPDATE CHANGES

Added continuation criteria requiring documentation of reduction in acute complications since initiation.

Updated description for ICD-10 diagnosis codes D57.00-D57.819.

16+minimum age for initiation

>=2VOC requirement (12 mo)

5 mg/kgtypical dosing / qty

J0791HCPCS

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Coverage Criteria for Adakveo (crizanlizumab)

Initial Therapy

Covered when ALL of the following are met:

Initial Approval Criteria: Individual is 16 years of age or older; AND Documentation that individual had at least two episodes of sickle cell–related crises in the past 12 months; AND Individual has a diagnosis of sickle cell disease.

May be used with or without hydroxyurea.

Continuation Therapy

Covered when the following is met:

Continuation Approval Criteria: Documentation that individual experienced a reduction in acute complications of sickle cell disease (e.g., reduction in number of vaso-occlusive episodes, acute chest syndrome episodes) since initiation of Adakveo.

Not Medically Necessary / May Not Be Approved

May Not Be Approved: Use in combination with Oxbryta (voxelotor) is listed as a condition where requests may not be approved due to limited or no data supporting safety and efficacy of concomitant use (Absiola 2022).

Requests that do not meet the initial or continuation criteria may also be denied.

Concomitant use of crizanlizumab (Adakveo) with voxelotor (Oxbryta) is listed as an exclusion/contraindication for approval due to limited or no data supporting safety and efficacy of the combination. Clinical review should therefore consider combination therapy with voxelotor as a reason to withhold approval unless robust supporting evidence is provided.

Requests that do not include required documentation may be considered not medically necessary. Required information includes proof that the individual is 16 years of age or older, documentation of a diagnosis of sickle cell disease, and evidence of at least two sickle cell–related crises in the prior 12 months. Additionally, requests that propose concomitant use of voxelotor (Oxbryta) with crizanlizumab should be considered for denial or deemed not medically necessary because there is currently limited or no data to support the safety and efficacy of this combination.

Coding and Diagnosis Codes

Covered HCPCSHCPCSCovered

J0791

Injection, crizanlizumab-tmca, 5 mg [Adakveo]

Relevant ICD-10 Diagnosis CodesICD-10

D57.00-D57.819

Sickle-cell disorders

Prior VOCs

Required count>= 2 sickle cell–related crises in the prior 12 months

Time windowPast 12 months

Documentation requiredMedical records showing ER or facility admissions for SCD-related pain treated with parenteral narcotic or parenteral ketorolac

Prior Authorization and Provider Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Adakveo (crizanlizumab-tmca). Initial approvals: member must be ≥16 years old, have a documented diagnosis of sickle cell disease, and documentation showing at least two sickle cell–related crises in the prior 12 months. Continuation approvals: documentation must show a reduction in acute complications of sickle cell disease (for example, fewer vaso-occlusive episodes or acute chest syndrome) since starting therapy. Requests that do not meet these criteria may be denied.

HCPCS: J0791 (Injection, crizanlizumab-tmca, 5 mg [Adakveo])
ICD-10: D57.00-D57.819 (Sickle-cell disorders)

Denial Risk

Concomitant Therapy Caution

There is limited to no data supporting the safety and efficacy of concomitant use of voxelotor (Oxbryta) with crizanlizumab (Adakveo). Concomitant use with voxelotor may be a reason to deny approval; provide justification and supporting clinical documentation if treating concomitantly.

Background on Crizanlizumab

Crizanlizumab (Adakveo) is a monoclonal antibody that targets and inhibits P‑selectin, reducing adhesion of sickled red blood cells to the endothelium and platelets and thereby decreasing vaso‑occlusive crises (VOCs). It is administered by infusion on a monthly schedule (with initiation dosing as specified in the dosing guidance) and may be used with or without hydroxyurea in appropriate patients.

Definitions

Sickle Cell Crises — Definition

DefinitionER or facility admission for SCD-related pain treated with parenteral narcotic or parenteral ketorolac

Setting examplesEmergency department or other medical facility admissions

PurposeDefines events counted as vaso-occlusive crises for authorization and documentation

Crizanlizumab (Adakveo) — Drug definition

Drug classMonoclonal antibody that binds to and inhibits P-selectin

Brand nameAdakveo (crizanlizumab-tmca)

Mechanism