CurrentUnitedHealthcarePolicy 2025D0085K

Adakveo (crizanlizumab-tmca) coverage for sickle cell disease

Policy Summary

PayerUnitedHealthcare

PolicyAdakveo (crizanlizumab-tmca) coverage for sickle cell disease

Policy CodePolicy 2025D0085K

Change TypeRevised coverage criteria; template and references updated

Effective DateJun 1, 2025

Next Review Date

Key ActionWhen requesting authorization, document initial and maintenance dosing per FDA labeling, limit authorizations to 12 months, and include provider attestation if asserting hydroxyurea inappropriateness based on genotype.

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).

Updated References section to reflect the most current information.

Template Update - Updated Benefit Considerations section.

1HCPCS code listed

~40ICD-10 codes listed

12 monthsInitial authorization max duration

Coverage Summary

Defines UnitedHealthcare medical benefit drug coverage criteria, applicable coding, dosing conformity, and authorization periods for Adakveo (crizanlizumab-tmca) to reduce vaso-occlusive crises in patients with sickle cell disease age 16 and older. Adakveo is indicated to reduce the frequency of vaso-occlusive crises in patients with sickle cell disease; the FDA indication specifies use in patients aged 16 years and older.

Quick facts and thresholds

Minimum covered age16 years

Initial authorization max duration12 months

Threshold for vaso-occlusive crises (VOCs)>= 2 VOCs in previous 12 months

HCPCS code count1 (J0791: Injection, crizanlizumab-tmca, 5 mg)

ICD-10 code count~40 (multiple D57.x codes listed)

Initial Therapy Criteria

Initial Therapy

Covered when ALL of the following are met:

ALL of the following

Patient is 16 years of age or older
Diagnosis of a sickle cell disease, including, but not limited to, homozygous hemoglobin S (HbSS), sickle hemoglobin C disease (HbSC), sickle beta 0 thalassemia, and sickle beta + thalassemia
ONE of
- Patient is transitioning from treatment with Oxbryta (voxelotor) to Adakveo
- Patient has previously experienced 2 or more sickle cell-related vasoocclusive crises within the previous 12 months

Continuation Therapy Criteria

Continuation Therapy

Covered when ALL of the following are met:

ALL of the following

Diagnosis of a sickle cell disease, including, but not limited to, homozygous hemoglobin S (HbSS), sickle hemoglobin C disease (HbSC), sickle beta 0 thalassemia, and sickle beta + thalassemia
Patient has experienced a reduction in sickle cell-related vasoocclusive crises and/or a decrease in severity of sickle cell-related vasoocclusive crises from pretreatment baseline while on Adakveo
Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy
Patient is not receiving concomitant Oxbryta (voxelotor) therapy

Limitations / Concomitant Therapy Exclusions

Exclusion-style criteria present in the policy/trees that may lead to denial include: the patient is receiving concomitant chronic, prophylactic blood transfusion therapy; the patient is receiving concomitant Oxbryta (voxelotor) therapy; and failure to meet prescriber requirements (Adakveo must be prescribed by, or in consultation with, a hematologist or other specialist with expertise in sickle cell disease). Additionally, authorization may be contingent on documentation and duration rules (initial and reauthorization limited to no more than 12 months), and claims may be denied if these conditions are not satisfied.

Applicable Codes

Drug HCPCSHCPCSCovered

J0791

Injection, crizanlizumab-tmca, 5 mg

Applicable Diagnosis Codes (Reference list)ICD-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

1/6

Provider Actions & Authorization Requirements

Prior Authorization

Authorization requirement and duration

Initial authorization limited to no more than 12 months; reauthorization limited to no more than 12 months. Reauthorization requires documentation of a reduction in vaso-occlusive crisis (VOC) frequency and/or a decrease in VOC severity from pretreatment baseline.

J0791

Documentation Required

Specialist prescribing requirement

Adakveo must be prescribed by, or in consultation with, a hematologist or other specialist with expertise in the diagnosis and management of sickle cell disease. When asserting that hydroxyurea is inappropriate based on the patient’s genotype, a provider attestation is required.

Clinical Evidence

1.63 vs 2.98 (45.3% lower; P=0.01)SUSTAIN median annual VOC rate (5 mg/kg vs placebo)

36% vs 17%Proportion with no VOC (crizanlizumab 5 mg/kg vs placebo)

In the SUSTAIN trial (52-week, randomized, placebo-controlled), high-dose crizanlizumab (5 mg/kg) reduced the median annual rate of VOCs versus placebo (1.63 vs 2.98, a 45.3% lower rate, P=0.01) and prolonged time to first and second crises (median time to first VOC ~ 4.07 vs 1.38 months; median time to second crisis ~ 10.32 vs 5.09 months). The FDA indication for Adakveo is for patients 16 years and older with sickle cell disease.

Definitions

Vaso-occlusive crisis (VOC): An acute episode of pain with no cause other than a vaso-occlusive event requiring a medical facility visit and oral or parenteral opioids, or parenteral NSAIDs; includes acute chest syndrome, hepatic sequestration, splenic sequestration, and priapism when requiring a facility visit.

Background

Adakveo (crizanlizumab-tmca) is a humanized monoclonal antibody that binds to P-selectin, blocking P-selectin interactions (including with PSGL-1) and dissociating preformed P-selectin/PSGL-1 complexes; by inhibiting P-selectin–mediated interactions among endothelium, platelets, red blood cells, and leukocytes, it is intended to reduce vaso-occlusive crises in sickle cell disease. The FDA indication is for patients aged 16 years and older.

Revision History

06/01/2025revised

07/01/2025revisedLatest

Template Update - Updated Benefit Considerations section.

06/01/2025clarified

Updated References section to reflect the most current information.

Policy Summary

PayerUnitedHealthcare

PolicyAdakveo (crizanlizumab-tmca) coverage for sickle cell disease

Policy CodePolicy 2025D0085K

Change TypeRevised coverage criteria; template and references updated

Effective DateJun 1, 2025

Next Review Date

On This Page

>= 2 VOCs in previous 12 months

ONE of

Patient is currently receiving hydroxyurea therapy
Patient has a history of treatment failure, intolerance, or contraindication to hydroxyurea therapy
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)

Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy

Patient is not receiving concomitant Oxbryta (voxelotor) therapy

Adakveo is prescribed by, or in consultation with, a hematologist, or other specialist with expertise in the diagnosis and management of sickle cell disease

Adakveo initial dosing is in accordance with the United States Food and Drug Administration approved labeling

Initial dosing per FDA labeling (informational; specific dosing schedule not restated in policy)

Initial authorization will be for no more than 12 months12 months

Adakveo is prescribed by, or in consultation with, a hematologist, or other specialist with expertise in the diagnosis and management of sickle cell disease

Adakveo maintenance dosing is in accordance with the United States Food and Drug Administration approved labeling

Maintenance dosing per FDA labeling (informational)

Reauthorization will be for no more than 12 months12 months

Billing Rule

Dosing conformity

Initial and maintenance dosing must follow United States Food and Drug Administration approved labeling. Claims should use HCPCS J0791, reported per 5 mg unit.

J0791

Denial Risk

Concomitant therapy exclusions

Claims may be denied if the member is receiving concomitant chronic, prophylactic blood transfusion therapy or concomitant Oxbryta (voxelotor) therapy.