Adakveo (crizanlizumab-tmca) — coverage criteria for sickle cell disease
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Policy governs prior authorization and coverage criteria for Adakveo (crizanlizumab-tmca) to reduce vaso-occlusive crises in patients with sickle cell disease aged 16 and older for members of Maryland Physicians Care.
No material clinical or coverage changes in this revision.
Coverage Criteria for Adakveo (crizanlizumab-tmca)
inv-01: COVERAGE CRITERIA
Covered when ALL of the following are met:
Initial Approval - Sickle Cell Disease
- SCD genotype: Diagnosis of SCD with one of the following genotypes: HbSS, HbSß0-thalassemia, HbSß+-thalassemia, HbSC
genotype must be one listed
- Age >= 16 years>=16 years
- Prescriber: Prescribed by or in consultation with a hematologist or specialist with SCD training
- Hemoglobin: Hb >= 4 g/dL>=4 g/dL
- VOC history: Either: (a) >=2 VOCs in past 6 months while on hydroxyurea at up to maximally indicated doses OR (b) intolerance/contraindication to hydroxyurea and >=2 VOCs in past 12 months>=2 VOCs
Requires documentation of hydroxyurea use or intolerance
- L-glutamine: Failure of L-glutamine at up to maximally tolerated doses unless contraindicated or adverse effects
- Baseline VOC documentation: Documentation of baseline incidence of VOC over the last 12 months12 months
- Concurrent hydroxyurea: Adakveo prescribed concurrently with hydroxyurea unless contraindicated or clinically significant adverse effects
- No concurrent Oxbryta: Adakveo is not prescribed concurrently with Oxbryta
- No chronic transfusion: Member is not concurrently treated with chronic prophylactic blood transfusion therapy
- Dose does not exceed 5 mg/kg on Day 1 and Day 15, followed by 5 mg/kg every 4 weeks5 mg/kg
Follow manufacturer's dosing
inv-02: COVERAGE CRITERIA
Reauthorization may be granted annually when ALL of the following are met:
Renewal Criteria
- Clinical response: Chart documentation confirming positive response evidenced by documented improvement in incidence of VOC from baselineimprovement from baseline
- Concurrent hydroxyurea at renewal: Adakveo continues to be prescribed concurrently with hydroxyurea unless contraindicated or adverse effects
Prescriber must document reason if not on hydroxyurea
- No Oxbryta concurrent use: Prescriber attests Adakveo is not prescribed concurrently with Oxbryta
- Prescriber specialty at renewal: Prescribed by or in consultation with a hematologist or other specialist with SCD training
inv-03: COVERAGE CRITERIA
Use of Adakveo for any indication other than reducing vaso-occlusive crisis (VOC) frequency in sickle cell disease (SCD) is considered investigational and is not covered. This policy limits coverage to the specific indication and criteria described elsewhere in this document; any other clinical use of crizanlizumab-tmca falls outside the scope of coverage.
Any use of Adakveo that is outside the FDA‑approved indication and the specific coverage criteria outlined in this policy is considered investigational and not medically necessary. Providers should follow the policy’s required criteria and dosing guidance when requesting coverage.
Billing and Diagnosis Codes
| J0791 | Injection, crizanlizumab-tmca, 5mg |
| 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 |
| D57.09 | Hb-SS disease with crisis with other specified complication |
| D57.1 | Sickle-cell disease without crisis |
| D57.20 | Sickle-Cell/Hb-C disease without crisis |
| D57.21 | Sickle-cell/Hb-C with crisis |
| D57.211 | Sickle-cell/Hb-C with acute chest syndrome |
Provider Requirements, Authorization, and Documentation
Prior authorization required; initial ≤6 months, annual reauthorization
Prior authorization is required for Adakveo (crizanlizumab-tmca). Initial authorizations may be approved for up to 6 months; reauthorization is reviewed annually and may be granted for up to 1 year contingent on documented clinical benefit and continued concurrent hydroxyurea use unless contraindicated. Providers should obtain prior authorization before initiating therapy and submit renewal requests with required documentation each year.
- Initial authorization: up to 6 months.
- Reauthorization: reviewed annually; may be extended at 1-year intervals.
- Reauthorization contingent on documented improvement in VOC incidence from baseline and continued concurrent hydroxyurea use unless contraindicated.
Required prior therapies: hydroxyurea and L‑glutamine trials
Prior to approval, the provider must document trial and failure of hydroxyurea at maximally indicated doses (or document intolerance/contraindication). The provider must also document failure of L-glutamine at up to maximally tolerated doses unless contraindicated.
- Trial and failure of hydroxyurea at up to maximally indicated doses, or documented intolerance/contraindication.
- Failure of L-glutamine at up to maximally tolerated doses unless contraindicated or clinically significant adverse effects are experienced.
Required documentation to support PA and renewals
Submit office chart notes, lab results, or other clinical information confirming that all approval criteria are met, including diagnosis/genotype, baseline VOC frequency over the prior 12 months, prior therapies and responses, hemoglobin level, and prescriber specialty or consultation.
- Documentation of SCD genotype (HbSS, HbSβ0, HbSβ+, or HbSC).
- Baseline incidence of VOC over the last 12 months.
- Evidence of prior hydroxyurea use or documented intolerance/contraindication and response to L‑glutamine.
- Hemoglobin (Hb) ≥ 4 g/dL laboratory results.
- Prescribed by or in consultation with a hematologist or specialist with SCD training.
Triggers for denial: missing criteria or excessive dosing
Requests may be denied if the required clinical information is not provided or criteria are not met, including missing documentation of SCD genotype, age ≥16 years, baseline VOC frequency, prior hydroxyurea trial or documented intolerance, failure of L‑glutamine unless contraindicated, or if dosing exceeds the labeled regimen.
- Missing or undocumented SCD genotype (must be one of HbSS, HbSβ0, HbSβ+, HbSC).
- Member younger than 16 years (Age must be ≥16).
- No documentation of baseline VOC incidence or required VOC frequency criteria.
- No evidence of hydroxyurea trial at maximally indicated doses (or no documented intolerance/contraindication).
- No evidence of L‑glutamine failure unless contraindicated.
- Dose exceeding 5 mg/kg dosing schedule (Day 1, Day 15, then every 4 weeks).
Clinical Definitions
Drug Background
Adakveo (crizanlizumab‑tmca) is a selectin blocker that is FDA‑approved to reduce the frequency of vaso‑occlusive crises (VOCs) in patients with sickle cell disease aged 16 years and older. Treatment is intended as an adjunct to standard care and must be prescribed and dosed per the manufacturer’s labeling; uses beyond this FDA‑approved indication are considered investigational and excluded from coverage under this policy.
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