CurrentNeighborhood Health Plan of Rhode IslandPolicy Evolent Clinical Guideline 3001 for Adakveo (crizanlizumab-tmca)

Adakveo (crizanlizumab-tmca) coverage for sickle cell disease

Defines authorized uses, clinical criteria, exclusions, coding, and prior authorization expectations for Adakveo for members of the payer lines of business; applies to providers requesting medication authorization from Evolent on behalf of the plan.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyAdakveo (crizanlizumab-tmca) coverage for sickle cell disease

Policy CodePolicy Evolent Clinical Guideline 3001 for Adakveo (crizanlizumab-tmca)

Change TypeTemplate conversionminor wording updates

Effective Date

Next Review Date

Key ActionSubmit prior authorization to Evolent demonstrating member meets clinical criteria, including age ≥16, ≥2 pain crises in 12 months, and hydroxyurea history if applicable.

SourceLink

POLICY UPDATE CHANGES

Converted to new Evolent guideline template and replaced prior UM ONC_1375 Adakveo (crizanlizumab) policy.

Updated 'continuation request' verbiage.

>=16minimum member age for coverage

>=2/12morequired pain crises

J0791HCPCS

5 mg/kgsingle dose max

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Coverage Criteria for Adakveo (crizanlizumab-tmca)

Initial/maintenance therapy criteria

Covered when ALL of the following are met

Age and diagnosis: Member is aged 16 years or older AND has sickle cell disease (HbSS, HbSC, HbS/beta0-thalassemia, HbS/beta+thalassemia, or other less common genotypes)

Clinical event frequency: Two or more sickle cell-related pain crises in the past 12 months>=2 in 12 months

Hydroxyurea co-therapy condition: If receiving Hydroxyurea, treatment must have been prescribed for at least 6 months AND on a stable dose of Hydroxyurea for at least 3 months6 months prescribed; 3 months stable dose

Applies only if member is receiving hydroxyurea

Additional coverage considerations include clinical situations that may preclude approval. Adakveo (crizanlizumab-tmca) is not covered when it is being used after disease progression on the same regimen. Members receiving chronic anticoagulation therapy other than aspirin (for example, warfarin or heparin) are excluded from coverage. A baseline hemoglobin threshold also applies: the medication is not covered when the member has a hemoglobin level of less than 4.0 g/dL. Requests that exceed the policy’s dosing limit — a single dose greater than 5 mg/kg — will be denied. These situations should be documented in the authorization request to avoid administrative denial.

Use of Adakveo (crizanlizumab-tmca) for investigational or off‑label indications is considered not medically necessary when the supporting evidence is insufficient or not generally accepted by the medical community. Insufficiency is defined by the absence of support in CMS‑recognized compendia or acceptable peer‑reviewed literature and includes circumstances such as inadequate representation of the patient or regimen in published evidence, study outcomes that are not clinically meaningful, inappropriate experimental design for the question under study, reliance solely on uncontrolled case reports, or conclusions based only on abstracts without a full peer‑reviewed article. Requests lacking adequate evidence per these criteria will be denied.

Provider Actions and Authorization Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required through Evolent for Adakveo; approval when member meets clinical criteria (see coverage module).

Documentation Required

Hydroxyurea Requirement

If receiving hydroxyurea, member must have been on it for at least 6 months and on a stable dose for at least 3 months prior to approval.

Documentation Required

Required Clinical Documentation

Clinical evidence supporting diagnosis and prior therapy history (including hydroxyurea use and stability), documentation of number of sickle cell pain crises, etc.

Denial Risk

Denial Triggers

Denial may be triggered for requests that exceed the single dose limit of 5 mg/kg; use after disease progression on the same regimen; use while on chronic anticoagulation (other than aspirin); hemoglobin < 4.0 g/dL; or investigational/off‑label indications lacking sufficient supporting evidence (not supported by CMS‑recognized compendia or acceptable peer‑reviewed literature).

Single dose limit: Adakveo 5 mg/kg
Use after disease progression on same regimen
Receiving chronic anticoagulation therapy (e.g., warfarin, heparin) other than aspirin
Hemoglobin level < 4.0 g/dL
Investigational or off‑label use without sufficient supporting evidence

Background on Crizanlizumab (Adakveo)

Background: Crizanlizumab (Adakveo) is used to reduce the frequency of vaso‑occlusive crises in patients with sickle cell disease. Coverage for Adakveo is defined by specific clinical criteria and exclusions; the policy references FDA prescribing information and recognized clinical evidence sources to establish accepted uses. When considering therapy, reviewers will apply the exclusion criteria above (including anticoagulation status, hemoglobin threshold, dosing limits, and evidence sufficiency) in addition to the coverage criteria for initial and maintenance therapy.