Adakveo (crizanlizumab-tmca) – Medical Benefit Coverage Criteria
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Clinical policy governing medical-benefit coverage of Adakveo (crizanlizumab-tmca) for members with sickle cell disease under UnitedHealthcare Commercial and Individual Exchange plans. It defines initial and continuation therapy criteria, exclusions, and applicable codes for providers and prior authorization processes.
No material clinical or coverage changes in this revision.
Coverage Criteria for Adakveo (crizanlizumab-tmca)
Initial Therapy Criteria
Covered when ALL of the following are met for Initial Therapy:
Initial therapy core criteria
- Age: Patient is 16 years of age or older>=16 years
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- Diagnosis of a sickle cell disease (includes HbSS, HbSC, sickle beta0 thalassemia, sickle beta+ thalassemia)
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VOC/history criteria
- Transition from Oxbryta: Patient is transitioning from treatment with Oxbryta (voxelotor) to Adakveo
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- VOC frequency: Patient has previously experienced 2 or more sickle cell-related vaso-occlusive crises within the previous 12 months>=2 in 12 months
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Hydroxyurea status
- On hydroxyurea: Patient is currently receiving hydroxyurea therapy
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- Hydroxyurea failure/intolerance: History of treatment failure, intolerance, or contraindication to hydroxyurea therapy
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- Provider attestation: Provider attests that the patient is not an appropriate candidate for hydroxyurea based on genotype (patient does not have HbSS or sickle beta0 thalassemia)
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- No chronic transfusion: Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy
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- No concomitant voxelotor: Patient is not receiving concomitant Oxbryta (voxelotor) therapy
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- Prescriber specialty: Adakveo is prescribed by, or in consultation with, a hematologist or other specialist with expertise in the diagnosis and management of sickle cell disease
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Continuation Therapy Criteria
Covered when ALL of the following are met for Continuation Therapy:
Continuation therapy core criteria
- Diagnosis of a sickle cell disease (includes HbSS, HbSC, sickle beta0 thalassemia, sickle beta+ thalassemia)
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- Clinical benefit: Patient has experienced a reduction in sickle cell-related vaso-occlusive crises and/or a decrease in severity of vaso-occlusive crises from pretreatment baseline while on Adakveo
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- No chronic transfusion: Patient is not receiving concomitant chronic, prophylactic blood transfusion therapy
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- No concomitant voxelotor: Patient is not receiving concomitant Oxbryta (voxelotor) therapy
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- Prescriber specialty: Adakveo is prescribed by, or in consultation with, a hematologist or other specialist with expertise in the diagnosis and management of sickle cell disease
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- Dosing: Adakveo maintenance dosing is in accordance with the United States Food and Drug Administration approved labeling
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- Authorization period: Reauthorization will be for no more than 12 months
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Not Proven / Not Medically Necessary
Not proven/Not medically necessary indications
- Pediatric <16: Pediatric patients less than 16 years of age with sickle cell disease
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- Myelofibrosis: Use of Adakveo for myelofibrosis
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Clinical evidence and guideline considerations
Clinical evidence and guideline considerations summarized:
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Adakveo (crizanlizumab-tmca) is not proven or medically necessary for use in pediatric patients who are less than 16 years of age. The policy explicitly lists pediatric patients <16 with sickle cell disease as an exclusion for coverage, and use in this population does not meet the criteria for medical necessity under this policy.
The safety and efficacy of Adakveo in pediatric patients younger than 16 years have not been established. The FDA-labeled indication for Adakveo applies to adults and pediatric patients aged 16 years and older, and the policy relies on this limitation when determining that use below age 16 is not supported by available evidence.
Reiterating coverage limits: Adakveo is not proven or medically necessary for pediatric patients under 16 years of age, and the policy also identifies use for myelofibrosis as not medically necessary. The document notes that the FDA indication is for patients aged ≥16 years, and safety/efficacy have not been established for those younger than 16.
Applicable Codes
| J0791 | Injection, crizanlizumab-tmca, 5 mg |
| 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 |
Provider Action Items, Prior Authorization, and Documentation
Prior Authorization Required
Prior authorization is required for Adakveo (crizanlizumab-tmca). Initial authorization will be for no more than 12 months and dosing must follow FDA-approved labeling. Adakveo maintenance/continuation authorizations are also limited to no more than 12 months with maintenance dosing per FDA labeling.
- Prior authorization required
- Initial and reauthorization period: ≤ 12 months
- Dosing must follow FDA-approved labeling
Required Clinical Documentation
Providers must document clinical criteria to support medical necessity. Required documentation includes patient age, documented diagnosis/genotype of sickle cell disease, number of vasoocclusive crises (VOCs) in the prior 12 months or documentation of transition from voxelotor, hydroxyurea treatment status (on hydroxyurea, history of failure/intolerance/contraindication, or provider attestation that patient is not an appropriate candidate), and whether the patient is receiving chronic prophylactic transfusions or concomitant voxelotor therapy. Adakveo must be prescribed by or in consultation with a hematologist or other specialist with expertise in sickle cell disease.
- Document age (must be ≥ 16 years)
- Document sickle cell diagnosis and genotype
- Document VOC count in prior 12 months or transition from Oxbryta (voxelotor)
- Document hydroxyurea use or history of failure/intolerance/contraindication or provider attestation
- Document absence of chronic prophylactic transfusion and absence of concomitant voxelotor
- Document that prescribing clinician is a hematologist or specialist experienced in sickle cell disease
Policy and Benefit Plan Precedence
Coverage decisions should reference the member-specific benefit plan document first. This UnitedHealthcare Medical Benefit Drug Policy provides standard plan guidance but the member-specific benefit plan governs in the event of a conflict. Also check applicable federal or state mandates prior to authorization.
- Reference the member-specific benefit plan document when deciding coverage
- UnitedHealthcare Medical Benefit Drug Policy is interpretive for UnitedHealthcare standard plans and may be superseded by member-specific terms
- Check applicable federal/state mandates before applying policy
Hydroxyurea Requirement / Consideration
Hydroxyurea use is expected unless there is documentation of failure, intolerance, or contraindication, or the provider attests the patient is not an appropriate candidate (e.g., non-HbSS or non–sickle beta 0 thalassemia genotypes). Concurrent hydroxyurea is acceptable and may be required as part of criteria.
- Patient is currently receiving hydroxyurea therapy; or
- Patient has history of hydroxyurea treatment failure, intolerance, or contraindication; or
- Provider attests patient is not an appropriate candidate for hydroxyurea (document rationale)
Professional Society Guidance
Professional society guidance (NHLBI) supports use of hydroxyurea in adults with SCA who have ≥3 VOCs/year and recommends established prescribing and monitoring protocols. For patients not demonstrating response to hydroxyurea, consultation with a sickle cell expert is advised. These recommendations should inform treatment decisions and documentation.
- NHLBI recommends hydroxyurea for adults with ≥3 VOCs/year (strong recommendation)
- Use established prescribing and monitoring protocols for hydroxyurea
- Consult a sickle cell expert when clinical response to hydroxyurea is inadequate
Clinical Exclusions / Contraindication Triggers
Clinical exclusion and contraindication triggers that may lead to denial include: patient age < 16 years; concomitant chronic prophylactic blood transfusion therapy; concomitant Oxbryta (voxelotor) therapy; diagnoses for which Adakveo is not proven (e.g., myelofibrosis); and lack of required documentation (age, diagnosis/genotype, VOC history, hydroxyurea status, specialist involvement).
- Age < 16 years — not eligible
- Concomitant chronic prophylactic blood transfusion therapy — exclusion
- Concomitant Oxbryta (voxelotor) therapy — exclusion
- Use for diagnoses without demonstrated benefit (e.g., myelofibrosis) — exclusion
- Insufficient clinical documentation to meet criteria — potential denial
UnitedHealthcare Medical Benefit Drug Policy Notice
This policy is a UnitedHealthcare Medical Benefit Drug Policy intended to assist in interpreting UnitedHealthcare standard benefit plans (including Commercial and Individual Exchange where applicable). It is informational and does not constitute medical advice. Before applying this policy, review the member-specific benefit plan document and applicable mandates.
- UnitedHealthcare Medical Benefit Drug Policy — interpretive guidance for standard plans
- Applies to UnitedHealthcare Commercial and Individual Exchange benefit plans as noted in policy history
- Member-specific benefit plan governs in event of conflict
Background on Condition and Therapy
Sickle cell disease (SCD) is an inherited hemoglobin disorder in which abnormal hemoglobin polymerization causes red blood cells to sickle, resulting in hemolysis, inflammation, and microvascular occlusion. These pathophysiologic processes produce recurring vaso-occlusive crises (VOCs), acute pain episodes, and progressive end-organ injury. Therapies that interrupt the cellular interactions and adhesion events that contribute to vaso‑occlusion can reduce VOC frequency; Adakveo (crizanlizumab‑tmca) is a monoclonal antibody directed against P‑selectin that aims to block those interactions. Clinical trials (for example, SUSTAIN) demonstrated that crizanlizumab reduced the rate of VOCs and prolonged time to first and second crises compared with placebo over a 52‑week treatment period.
Definitions
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