CurrentCignaPolicy IP0120

Sickle Cell Disease - Adakveo (crizanlizumab-tmca) Coverage Criteria

Cigna's coverage policy for Adakveo (crizanlizumab-tmca) to reduce vasoocclusive/sickle cell-related crises in patients age 16 and older, including initial and continuation therapy criteria and dosing. Applies to benefits administered by Cigna Companies.

Policy Summary

PayerCigna

PolicySickle Cell Disease - Adakveo

Policy CodePolicy IP0120

Change TypeTerminology and criteria clarifications

Effective Date

Next Review DateApr 15, 2025

Key ActionSubmit prior authorization with documentation of age ≥16, at least one sickle cell-related crisis in prior 12 months (for initial therapy), hydroxyurea status or reason not a candidate, and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

Replaced the requirement 'vaso-occlusive crisis (VOC) in the previous 12-month period' with 'sickle cell-related crisis'.

Added a note detailing examples of patients ineligible for hydroxyurea therapy (e.g., pregnant or planning pregnancy, immunosuppressive condition).

Added note with examples of clinical benefit for patients currently receiving Adakveo (e.g., reduction in number of crises, delay in time to crises, reduced hospital days).

Removed 'Concomitant Oxbryta Therapy' from Conditions Not Covered.

≥16min patient age

1 yrinitial auth duration

5 mg/kgdose per infusion

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Adakveo (crizanlizumab-tmca)

FDA-Approved Indication

Approve for 1 year if the patient meets ONE of the following (A or B):

Overall approval path: Patient meets ONE of A or B

A) Initial Therapy: Approve if the patient meets ALL of the following (i, ii, iii, and iv)

A.i: Patient is ≥ 16 years of age

A.ii: Patient has had at least one sickle cell-related crisis in the previous 12-month period

A.iii

A.iii.a: Patient is currently receiving a hydroxyurea product
A.iii.b: According to the prescriber, patient has tried a hydroxyurea product and has experienced inadequate efficacy or significant intolerance
A.iii.c: According to the prescriber, patient is not a candidate for hydroxyurea therapy (examples: pregnant or planning pregnancy; immunosuppressive condition)

A.iv: Medication is prescribed by or in consultation with a physician who specializes in sickle cell disease (eg, hematologist)

B) Patient Currently Receiving Adakveo: Approve if the patient meets ALL of the following (i, ii, and iii)

B.i: Patient is ≥ 16 years of age

B.ii: According to the prescriber, patient is receiving clinical benefit from Adakveo therapy (examples: reduction in number of vasoocclusive/sickle cell-related crises; delay in time to crises; reduction in hospital days)

B.iii: Medication is prescribed by or in consultation with a physician who specializes in sickle cell disease (eg, hematologist)

Dosing

Dosing. Approve the following dosing regimens (A and B):

Dosing A: Up to 5 mg/kg given by intravenous infusion at Weeks 0 and 2 (infusion over approximately 30 minutes)5 mg/kg

Dosing B: Up to 5 mg/kg given by intravenous infusion for up to once every 4 weeks (maintenance dosing every 4 weeks)5 mg/kg every 4 weeks

Any use of Adakveo (crizanlizumab-tmca) outside the FDA‑approved indication is considered experimental, investigational, or unproven and may be denied. This includes any purpose, diagnosis, or patient population not explicitly covered under the policy’s FDA‑approved indication for reducing vasoocclusive/sickle cell‑related crises in patients meeting the stated criteria.

Coding and Billing Notes

Coding notemixed

No codes listed

Provider Actions, Prior Authorization and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization required. Initial approvals are for 1 year when criteria are met (patient ≥16 years, ≥1 sickle cell–related crisis in prior 12 months for initial therapy, or current clinical benefit when already receiving therapy).

Prior authorization required
Initial approvals: 1 year when criteria met
Examples of clinical benefit: reduction in vasoocclusive crises, increased time between crises, fewer hospital days

Step Therapy

Hydroxyurea Prerequisite

Hydroxyurea use is required as a prerequisite for initial approval unless contraindicated or clinically inappropriate. Approve initial therapy if the patient is currently receiving a hydroxyurea product, or the prescriber documents a trial with inadequate efficacy or significant intolerance, or documents that the patient is not a candidate for hydroxyurea (e.g., pregnancy, planning pregnancy, immunosuppressive condition).

Background

Adakveo (crizanlizumab‑tmca) is a monoclonal antibody indicated to reduce the frequency of vasoocclusive or sickle cell‑related crises in patients with sickle cell disease age ≥ 16 years. The policy authorizes treatment for an initial period of 1 year when patients meet the documented clinical criteria, and specifies the approved intravenous dosing regimen of 5 mg/kg administered at Weeks 0 and 2, then every 4 weeks thereafter.

Definitions

sickle cell-related crisis — definition/clarification

Termsickle cell-related crisis

Definition as used in this policyAn acute event of clinical worsening related to sickle cell disease used to qualify patients for initial or continuing Adakveo (crizanlizumab-tmca) therapy; policy replaced the prior phrasing 'vaso-occlusive crisis (VOC)' with 'sickle cell-related crisis' to broaden/clarify qualifying events.

Timeframe for initial approvalAt least one sickle cell-related crisis in the previous 12-month period is required for Initial Therapy.

Examples of clinical benefit for continuationReduction in number of vasoocclusive/sickle cell-related crises; delay in time to sickle cell-related crises; reduction in number of days in the hospital.

Documentation expectationPrescriber must attest to history of sickle cell-related crises (for initial therapy) or to clinical benefit from Adakveo (for continuation) in the medical record.

Documentation Required

Required Clinical Documentation

Document and submit clinical records that support the patient meets age and crisis-history requirements and the hydroxyurea prerequisite. For initial therapy, documentation must show patient is ≥16 years and has had at least one sickle cell–related crisis in the prior 12 months. Include prescriber specialty (or consultation) with a sickle cell disease specialist (e.g., hematologist) and prior therapy history (hydroxyurea use, trial results, intolerance, or contraindication).

Age documented: ≥16 years for initial or continuation approvals
History: ≥1 sickle cell–related crisis in previous 12 months for initial approval
Prescriber: prescribed by or in consultation with a sickle cell disease specialist (e.g., hematologist)
Prior therapy: documentation of current hydroxyurea, trial with inadequate efficacy, intolerance, or reason hydroxyurea is inappropriate