CurrentUnitedHealthcarePolicy CS2026D0085O

Adakveo (Crizanlizumab-Tmca) — Medical Benefit Drug Policy (Coverage Criteria)

Medical benefit drug policy governing coverage criteria for Adakveo (crizanlizumab-tmca) for members with sickle cell disease under UnitedHealthcare Community Plan; defines initial and continuation authorization requirements, age limits, concurrent therapy restrictions, and coding references.

Policy Summary

PayerUnitedHealthcare

PolicyAdakveo (Crizanlizumab-Tmca) — Medical Benefit Drug Policy

Policy CodePolicy CS2026D0085O

Change TypeRoutine review — no material changes

Effective DateMay 1, 2026

Next Review Date

Key ActionObtain prior authorization for patients aged 16+ meeting criteria, including documentation of diagnosis, VOC history or transition from voxelotor, hydroxyurea status, and dosing per FDA labeling.

SourceLink

POLICY UPDATE CHANGES

Routine review; no content changes.

16+minimum age (yrs)

>=2VOC events prior 12 mo

12max initial auth (mo)

J0791HCPCS code

Not covered: <16

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Coverage Criteria for Adakveo (crizanlizumab-tmca)

Initial Therapy

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 (includes HbSS, HbSC, sickle beta0 thalassemia, sickle beta+ thalassemia)

Vaso-occlusive history or transition: Patient is transitioning from Oxbryta (voxelotor) to Adakveo OR patient has previously experienced 2 or more sickle cell-related vasoocclusive crises within the previous 12 months>=2 VOCs in prior 12 months or transitioning from voxelotor

Hydroxyurea status: Patient is currently receiving hydroxyurea therapy OR has history of treatment failure, intolerance, or contraindication to hydroxyurea therapy

Provider attestation required if not candidate based on genotype

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

Prescriber and dosing: Adakveo is prescribed by, or in consultation with, a hematologist or other specialist with expertise in SCD AND initial dosing is in accordance with FDA labeling

Initial authorization for no more than 12 months

Continuation Therapy

Covered for reauthorization when ALL of the following are met:

Diagnosis of a sickle cell disease (includes HbSS, HbSC, sickle beta0 thalassemia, sickle beta+ thalassemia)

Clinical response: Patient has experienced a reduction in sickle cell-related vasoocclusive crises and/or a decrease in severity of VOCs from pretreatment baseline while on Adakveodocumented clinical improvement

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

Prescriber and dosing: Adakveo is prescribed by, or in consultation with, a hematologist or other specialist with expertise in SCD AND maintenance dosing is in accordance with FDA labeling

Not proven / Not medically necessary

Excluded indications: Pediatric patients less than 16 years of age with sickle cell disease OR treatment of myelofibrosis

These uses are considered not proven or not medically necessary

The policy excludes certain uses from coverage. Specifically, pediatric patients less than 16 years of age with sickle cell disease and treatment of myelofibrosis are listed as indications for which Adakveo is not proven or medically necessary and therefore not covered under this policy.

Adakveo (crizanlizumab-tmca) is FDA‑indicated to reduce the frequency of vaso‑occlusive crises in patients aged 16 years and older. The policy notes that the safety and efficacy of Adakveo have not been established in pediatric patients below age 16, and thus use in patients younger than 16 is outside the established evidence base described in the FDA information.

Consistent with the limitations above, the policy considers use of Adakveo in patients younger than 16 years and for the indication of myelofibrosis to be not medically necessary. This determination reflects the absence of established safety and efficacy data in those populations and is explicitly listed as a noncovered/unsupported indication in the policy.

Coding and Diagnosis References

Covered HCPCS CodesHCPCSCovered

J0791

Injection, crizanlizumab-tmca, 5 mg

Relevant 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

1/6

Vaso-occlusive crises in prior 12 months — threshold

Threshold value>= 2 vaso-occlusive crises in the prior 12 months

Alternate path for eligibilityTransitioning from Oxbryta (voxelotor) may substitute for the VOC threshold

Measurement periodPrior 12 months (look-back period for VOC events)

Provider Requirements, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Adakveo (crizanlizumab-tmca). Providers must obtain prior authorization before initiation; initial approvals will be for no more than 12 months.

Initial authorization duration: up to 12 months
Adakveo must be prescribed by or in consultation with a hematologist or other specialist with expertise in sickle cell disease

Prior Authorization

Age and Indication Requirements (FDA and Policy)

Adakveo is indicated to reduce the frequency of vasoocclusive crises (VOCs) in patients with sickle cell disease aged 16 years and older. Coverage consideration requires the patient to be ≥16 years and have a diagnosis of sickle cell disease (e.g., HbSS, HbSC, HbSβ0, HbSβ+). Prior authorization consideration includes either transition from voxelotor (Oxbryta) to Adakveo or a history of ≥2 VOCs in the prior 12 months.

Age requirement: 16 years or older
Diagnosis: sickle cell disease (examples: HbSS, HbSC, HbSβ0, HbSβ+)

Background and Clinical Context

Sickle cell disease (SCD) is an inherited hemoglobin disorder characterized by vaso‑occlusion, hemolysis, recurrent pain crises (vaso‑occlusive crises or VOCs), and end‑organ complications. Adakveo (crizanlizumab‑tmca) is a monoclonal antibody that targets P‑selectin to reduce interactions that contribute to vaso‑occlusion, and it is indicated to reduce the frequency of VOCs in patients aged 16 years and older. However, the safety and efficacy of Adakveo have not been established in pediatric patients below age 16, and certain non‑SCD indications such as myelofibrosis are not supported by the evidence and are listed as not medically necessary in this policy.

Definitions Relevant to This Policy

Vaso-occlusive crisis (VOC) — facility visit / treatment definition

Core definitionAn acute episode of pain attributable to vaso-occlusion that may require medical facility visit and treatment with oral or parenteral opioids or parenteral NSAIDs

Included severe eventsAcute chest syndrome, hepatic or splenic sequestration, and priapism requiring a facility visit are also considered VOCs (as defined in trial)

Clinical consequenceVOC episodes are a primary cause of health care encounters in patients with SCD

Vaso-occlusive crisis (VOC) — trial outcomes / alternative references

Trial-outcome framingClinical trials measure reductions in VOC frequency and time to first/second crisis as primary outcomes (e.g., median VOCs/year and time to first crisis)

Example trial findings

Policy Revision History

2026-05-01routine reviewLatest

Routine review of the policy; no clinical content changes; archived previous policy version CS2026D0085N.

Policy Summary

PayerUnitedHealthcare

PolicyAdakveo (Crizanlizumab-Tmca) — Medical Benefit Drug Policy

Policy CodePolicy CS2026D0085O

Change TypeRoutine review — no material changes

Effective DateMay 1, 2026

Next Review Date

SourceLink

On This Page

Reauthorization for no more than 12 months

Clinical trigger: ≥2 VOCs in the previous 12 months OR transitioning from Oxbryta

Note

Informational Note on FDA Approval

FDA approval for Adakveo is provided for informational context only. FDA approval alone does not guarantee coverage; coverage decisions also consider plan-specific criteria and required documentation.

Step Therapy

Hydroxyurea Requirement / Step-Therapy Consideration

Members must be receiving hydroxyurea therapy unless there is documented intolerance, contraindication, treatment failure, or a genotype-based reason hydroxyurea is not appropriate (for example, patient does not have HbSS or HbSβ0). Providers must attest to hydroxyurea status and document justification if not on hydroxyurea.

Acceptable hydroxyurea exceptions: documented intolerance, contraindication, treatment failure, or genotype-based rationale (provider attestation)
If patient is on hydroxyurea: document current therapy
If not on hydroxyurea: document reason and specialist consultation

Documentation Required

Required Documentation to Support Prior Authorization

Required documentation to support prior authorization includes: patient age, diagnosis and genotype, number and dates of vasoocclusive crises in the prior 12 months (or documentation of transition from voxelotor), current hydroxyurea status or documented intolerance/contraindication/treatment failure, provider attestation when hydroxyurea is deemed inappropriate, and confirmation that the patient is not receiving concomitant chronic prophylactic transfusion or concomitant voxelotor.

Patient age (date of birth)
Diagnosis and genotype (e.g., HbSS, HbSC, HbSβ0, HbSβ+)
VOCs: dates and count in prior 12 months OR documentation of transition from Oxbryta
Hydroxyurea: current use OR documented intolerance/contraindication/treatment failure OR provider attestation of genotype-based inappropriateness
Concurrent therapy exclusions: not on chronic prophylactic transfusion; not on concomitant voxelotor

Documentation Required

Required Supporting References

Key supporting references that may be requested or used in review include the Adakveo package insert and pivotal trial publications. Providers should have these available when submitting prior authorization.

Adakveo (crizanlizumab-tmca) package insert (Novartis, June 2024)
Ataga KI et al., N Engl J Med. 2017;376:429-39 (crizanlizumab pivotal trial)
Relevant NHLBI and professional guidance on hydroxyurea use (e.g., 2014 NHLBI report, ASH guidance)

Denial Risk

Denial Triggers and Conditions to Avoid

Denial triggers include patients <16 years of age, diagnosis of myelofibrosis, lack of required documentation (age, diagnosis/genotype, VOC history, hydroxyurea status/attestation), concurrent prohibited therapies, or failure to show justification for not using hydroxyurea when required.

Pediatric patients <16 years — not established as safe/efficacious
Myelofibrosis — not proven or medically necessary
Missing or incomplete documentation for age, diagnosis/genotype, VOC history, hydroxyurea status/attestation
Concurrent chronic prophylactic transfusion or concomitant voxelotor therapy documented

MSH study: reduced median annual VOCs (2.5 vs 4.5/year) and longer time to first crisis (3.0 vs 1.5 months)

Regulatory noteFDA indicates Adakveo reduces VOC frequency in patients aged 16 years and older; FDA language is informational for coverage decisions

Hydroxyurea therapy — NHLBI definition and role

NHLBI roleHydroxyurea is a disease-modifying oral therapy recommended by NHLBI to reduce pain crises, acute chest syndrome, transfusion needs, and improve hemoglobin and HbF

Indications per NHLBITreat adults with SCA who have ≥3 moderate-to-severe pain crises in 12 months, or pain interfering with daily activities; also recommended for infants ≥9 months and children/adolescents regardless of severity

Use guidanceUse an established prescribing and monitoring protocol to ensure proper use and maximize benefits and safety