ModifiedUnitedHealthcarePolicy CS2025D0085M

Adakveo (crizanlizumab-tmca) for sickle cell disease

Medical benefit drug policy governing coverage and prior authorization criteria for Adakveo (crizanlizumab-tmca) to reduce vaso-occlusive crises in patients with sickle cell disease; applies to UnitedHealthcare commercial populations except where state-specific exceptions noted.

Policy Summary

PayerUnitedHealthcare

PolicyAdakveo (crizanlizumab-tmca) for sickle cell disease

Policy CodePolicy CS2025D0085M

Change TypeCriteria revised to allow genotype‑based hydroxyurea ineligibilityapplication updated for state exclusions

Effective DateJun 1, 2025

Next Review Date

Key ActionSubmit prior authorization with provider attestation of hydroxyurea status or genotype‑based ineligibility and documentation that prescribing clinician is or consulted with a hematologist/sickle cell specialist.

SourceLink

POLICY UPDATE CHANGES

Revised coverage criteria; added criterion to allow coverage when the provider attests that the patient is not an appropriate candidate for hydroxyurea based on the patient's genotype (i.e., the patient does not have homozygous hemoglobin SS (HbSS) or sickle beta 0 thalassemia).

Added language to indicate this Medical Benefit Drug Policy does not apply to the state of Arizona; refer to the state's Medicaid clinical policy.

Updated References section to reflect the most current information.

≥16minimum age for coverage

≥2prior VOCs criterion

J0791

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Coverage Criteria for Adakveo (crizanlizumab-tmca)

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met:

Age: Patient is 16 years of age or older>=16 years

Diagnosis of a sickle cell disease (e.g., homozygous hemoglobin S [HbSS], sickle hemoglobin C disease [HbSC], sickle beta0 thalassemia, or sickle beta+ thalassemia)

VOC history or transition: Either patient is transitioning from treatment with Oxbryta (voxelotor) to Adakveo OR patient has experienced 2 or more sickle cell–related vasoocclusive crises within the previous 12 months

Hydroxyurea status: Either patient is currently receiving hydroxyurea OR has a history of treatment failure, intolerance, or contraindication to hydroxyurea OR provider attests the patient is not an appropriate candidate for hydroxyurea based on genotype (patient does not have HbSS or sickle beta0 thalassemia)

Concomitant therapy exclusions: Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy AND not receiving concomitant Oxbryta (voxelotor) therapy

Specialist and dosing: Adakveo is prescribed by, or in consultation with, a hematologist or other specialist with expertise in sickle cell disease AND initial dosing is in accordance with FDA-approved labeling

Authorization duration: Initial authorization will be for no more than 12 months

Continuation Therapy — Covered for continuation when ALL of the following are met

Covered for continuation when ALL of the following are met:

Diagnosis of a sickle cell disease (e.g., HbSS, HbSC, sickle beta0 thalassemia, or sickle beta+ thalassemia)

Clinical benefit: Patient has experienced a reduction in sickle cell–related vasoocclusive crises and/or a decrease in severity of vasoocclusive crises from pretreatment baseline while on Adakveo

Concomitant therapy exclusions: Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy AND not receiving concomitant Oxbryta (voxelotor) therapy

Specialist and dosing: Adakveo is prescribed by, or in consultation with, a hematologist or other specialist with expertise in sickle cell disease AND maintenance dosing is in accordance with FDA-approved labeling

Coverage when age and hydroxyurea considerations met — revised coverage stance

Revised coverage stance (summary from revision notes and FDA indication):

Age and indication: Patient is aged 16 years or older AND has sickle cell disease with vaso-occlusive crises (per FDA indication)age >= 16 years

FDA indication limits use to patients aged 16 years and older; safety and efficacy not established below 16.

Hydroxyurea requirement: Either the patient is an appropriate candidate for and has been treated with hydroxyurea OR the provider attests the patient is not an appropriate candidate for hydroxyurea due to genotype (patient does not have HbSS or sickle beta0 thalassemia)

Provider attestation is required when relying on genotype‑based ineligibility for hydroxyurea.

Adakveo (crizanlizumab‑tmca) is not proven or medically necessary for the treatment of pediatric patients younger than 16 years of age with sickle cell disease, and use in this population should be considered non‑covered under this policy.

This Medical Benefit Drug Policy does not apply to the state of Arizona. For members covered by Arizona Medicaid or where state policy governs, refer to Arizona’s Medicaid clinical policy for coverage guidance.

Adakveo is considered not proven and not medically necessary for the following indications: pediatric patients under 16 years of age and treatment of myelofibrosis. The FDA indication limits Adakveo to patients aged 16 years and older, and safety/efficacy in younger children have not been established.

The FDA approval for Adakveo specifies use to reduce vaso‑occlusive crises in adults and pediatric patients aged 16 years and older; therefore, use in children below age 16 is not supported by established safety and efficacy and is not covered. This rationale supports the policy exclusion for pediatric use under 16 and reinforces that requests for those patients may be denied.

Billing and Coding

Applicable HCPCS CodesHCPCS

J0791

Injection, crizanlizumab-tmca, 5 mg

Applicable ICD-10 Diagnosis CodesICD-10

D57.0	Hb-SS disease with crisis.
D57.00	Hb-SS disease with crisis, unspecified.
D57.01	Hb-SS disease with acute chest syndrome.
D57.02	Hb-SS disease with splenic sequestration.
D57.03	Hb-SS disease with cerebral vascular involvement.
D57.04	Hb-SS disease with dactylitis.
D57.09	Hb-SS disease with crisis with other specified complication.
D57.1	Sickle-cell disease without crisis.
D57.2	Sickle-cell/Hb-C disease.
D57.20	Sickle-cell/Hb-C disease without crisis.

1–10 of 52

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Prior Authorization, Documentation, and Denial Risks

Prior Authorization

Prior Authorization Required

Prior authorization is required. Requests must demonstrate the patient meets the policy's initial or continuation criteria for Adakveo (crizanlizumab-tmca) before authorization is granted.

Initial and continuation therapy requests require prior authorization.
Initial authorization will be for no more than 12 months; reauthorization/continuation authorizations will be for no more than 12 months.

Prior Authorization

Prior Authorization — Age and Genotype Constraints

Authorization is governed by age and genotype constraints in the coverage criteria. Adakveo is indicated for patients aged 16 years and older. Coverage may be granted when the provider documents that hydroxyurea is inappropriate by attestation (including genotype-based ineligibility).

Patient must be ≥ 16 years of age (FDA indication and policy exclusion for <16 years).
Provider may attest the patient is not an appropriate candidate for hydroxyurea based on genotype (e.g., patient does not have HbSS or sickle beta0 thalassemia) to meet the hydroxyurea-related criterion.

Background on Sickle Cell Disease and Therapy

Sickle cell disease (SCD) is an inherited hemoglobin disorder characterized by red blood cell sickling, hemolysis, chronic anemia, inflammation and recurrent vaso‑occlusion leading to acute pain episodes and end‑organ injury. Vaso‑occlusive crises (VOCs) are acute painful events often requiring medical facility visits and treatment with opioids or parenteral agents. Crizanlizumab (Adakveo) is a humanized monoclonal antibody that targets P‑selectin, blocking interactions among platelets, leukocytes and endothelium that contribute to vaso‑occlusion. Randomized trial data demonstrated that crizanlizumab reduces the annual rate of VOCs compared with placebo, providing the therapeutic rationale for use to prevent recurrent VOCs in eligible patients.

Definitions

Vaso-occlusive crisis (VOC) — definition

DefinitionAn acute episode of pain due to vaso-occlusion requiring a medical facility visit and treatment with oral or parenteral opioids, or parenteral NSAIDs; includes acute chest syndrome, hepatic or splenic sequestration, and priapism when requiring a medical facility visit.

Context from clinical trialSUSTAIN defined VOCs as acute episodes of pain leading to a healthcare visit with no cause other than a vaso-occlusive event requiring medical facility visit and opioids or parenteral NSAIDs.

Includes specific complicationsAcute chest syndrome, hepatic sequestration, splenic sequestration, and priapism are considered VOCs when they require a medical facility visit.

Vaso-occlusive crisis (VOC) — brief definition

Brief definitionAcute painful episodes in sickle cell disease targeted by crizanlizumab to reduce frequency (Adakveo indicated to reduce VOCs in patients aged ≥16 years).

Policy Revision History

2025-06-01revisionLatest

Added operational exclusion: policy does not apply to the state of Arizona; directs users to the state's Medicaid clinical policy.

2025-06-01material_change

Revised coverage criteria to permit authorization when provider attests the patient is not an appropriate candidate for hydroxyurea based on genotype (patient does not have HbSS or sickle beta0 thalassemia).

2025-06-01references_update