CurrentAvMedPolicy Pub. 100-2

Adakveo (crizanlizumab-tmca) prior authorization

Requirements and clinical criteria for AvMed medical prior authorization of Adakveo (crizanlizumab-tmca) for reducing vaso-occlusive crises in patients with sickle cell disease; applies to providers requesting coverage for the medication.

Policy Summary

PayerAvMed

PolicyAdakveo (crizanlizumab-tmca) prior authorization

Policy CodePolicy Pub. 100-2

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrescriber must complete prior authorization with required clinical documentation, including ICD codes and progress notes documenting VOC events.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥2minimum vaso-occlusive crises

≥16minimum age

5 mg/kgmax dose

12 mocontinuation duration

≥90 dayshydroxyurea trial

Coverage Criteria for Adakveo (crizanlizumab-tmca)

Initial Therapy — Adakveo

Adakveo is medically necessary for reduction of VOC frequency when ALL of the following are met:

Initial Authorization

VOC frequency: Member has experienced at least 2 vaso-occlusive crises within the preceding 12 months as documented by medical records and ICD diagnosis codes (VOC defined in Part A)>=2 VOCs in 12 months
Section C
Hydroxyurea requirement: Either insufficient response to at least 90 consecutive days of hydroxyurea (defined as >2 VOCs while compliant) OR documented contraindication to hydroxyurea (severe bone marrow depression: leukopenia <2,500/mm3, thrombocytopenia <100,000/mm3, or severe anemia requiring transfusion with labs within 30 days)
Section C hydroxyurea requirements

Policy Summary

PayerAvMed

PolicyAdakveo (crizanlizumab-tmca) prior authorization

Policy CodePolicy Pub. 100-2

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrescriber must complete prior authorization with required clinical documentation, including ICD codes and progress notes documenting VOC events.

SourceLink

On This Page

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Continuation Therapy — Adakveo

Continuation approved for 12 months when ALL of the following are met:

Continuation Authorization: Member continues to meet all Section B criteria; documentation demonstrates a decrease in number of VOCs from baseline since starting Adakveo (medical claims, chart notes, and ICD codes from prior approval to current request); and there is absence of unacceptable toxicity (e.g., infusion-related reactions or interference with automated platelet counts)

Section C reauthorization requirements

Coding and Clinical Inputs

Covered HCPCS CodesHCPCSCovered

J0791	Adakveo (crizanlizumab-tmca) Injection
C9053	Adakveo (crizanlizumab-tmca) Injection HCPCS

VOC Diagnosis CodesICD-10

282.42	Sickle cell crisis code listed
282.62	Sickle cell crisis code listed
282.64	Sickle cell crisis code listed
282.69	Sickle cell crisis code listed
D57.0	Sickle-cell anaemia with crisis
D57.00	Sickle-cell anaemia without crisis
D57.01	Sickle-cell anaemia with acute chest syndrome
D57.02	Sickle-cell anaemia with splenic sequestration
D57.21	HbSC disease with crisis
D57.211	HbSC disease with acute chest syndrome

1–10 of 21

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Vaso-occlusive crises in prior 12 months

Required number of VOCs>= 2 vaso-occlusive crises in the preceding 12 months

Documentation requiredICD codes and progress notes with date, outcome, and intervention for each VOC within the last 12 months

Verification sourcesPharmacy claims and medical record documentation may be used to verify events and treatments

Hemoglobin — clinical threshold

Hemoglobin threshold> 4.0 g/dL

Timing of labHemoglobin must be documented (labs completed within applicable timeframes as requested) prior to initiation

Related exclusionsMember must not be planning exchange transfusion or be on a chronic transfusion program at time of initiation

Provider Requirements and Submission

Prior Authorization

Prior authorization required for Adakveo (J0791/C9053)

Prior authorization is required for Adakveo (crizanlizumab-tmca) Injection (J0791/C9053). The prescribing physician must complete and sign the medical prior authorization/step-edit request and provide all required clinical documentation on the form; incomplete or illegible submissions may delay authorization.

Drug Requested: Adakveo (crizanlizumab-tmca) Injection (J0791/C9053)
Prescribing physician must sign and clearly print name on the request (preprinted stamps not valid)
Complete authorization form including dosing, diagnosis, and required supporting documentation

Step Therapy

Hydroxyurea trial (≥90 days) or documented contraindication required

Member must have had an insufficient response to at least 90 consecutive days of hydroxyurea prior to approval, or have a documented contraindication to hydroxyurea (severe bone marrow depression such as leukopenia <2,500/mm3, thrombocytopenia <100,000/mm3, or severe anemia requiring transfusion). Paid pharmacy claims will be verified.

Insufficient response defined as >2 VOCs while compliant on hydroxyurea/Droxia during the 90-day trial
If contraindication claimed, submit labs within the last 30 days documenting leukopenia, thrombocytopenia, or severe anemia
Paid pharmacy claims for hydroxyurea/Droxia within the last 12 months will be verified

Documentation Required

Required submission documents and clinical evidence

Prescriber must sign the prior authorization form and submit member and prescriber information plus supporting clinical documentation, including ICD codes and progress notes documenting VOC events in the last 12 months, relevant lab results, and pharmacy claims to verify prior therapies.

Completed and signed medical prior authorization/step-edit request (physician signature required)
ICD codes and progress notes documenting VOC events (as defined in Part A) within the prior 12 months
Relevant lab results (e.g., hemoglobin, platelet counts) — labs for contraindication claims must be within 30 days
Paid pharmacy claims to verify hydroxyurea/Droxia use in the last 12 months

Denial Risk

Incomplete or insufficient documentation may delay or deny authorization

Authorization may be delayed or denied if the information provided is incomplete, incorrect, or illegible, or if supporting documentation (e.g., VOC documentation, labs, pharmacy claims) is missing.

Incomplete, incorrect, or illegible submissions can delay authorization
Missing documentation of VOC events, labs, or pharmacy claims may result in denial

Background Information

Adakveo (crizanlizumab-tmca) is indicated to reduce the frequency of vaso-occlusive crises (VOCs) in patients with sickle cell disease aged 16 years and older. The policy requires documentation of a confirmed sickle cell genotype and at least 2 VOCs in the prior 12 months (as defined by Part A and verified by ICD codes and chart notes). Prior trial of hydroxyurea for at least 90 consecutive days with insufficient response, or a documented contraindication to hydroxyurea, is required. Reauthorization is granted for 12 months when the member continues to meet criteria and shows a documented decrease in VOCs from baseline without unacceptable toxicity. Other uses outside this indication are investigational.

Definitions and Code Use

Vaso-occlusive crisis (VOC) — definition

DefinitionA vaso-occlusive crisis (VOC) is an event prompting a visit or outreach resulting in a VOC diagnosis that requires interventions such as narcotic pain management, non-steroidal anti-inflammatory therapy, hydration, etc.

DocumentationDate and outcome of the event and the interventions provided must be documented in progress notes

VerificationICD codes and progress notes and pharmacy claims will be verified within the last 12 months to confirm VOCs

VOC ICD codes

ICD codes for VOC verification282.42, 282.62, 282.64, 282.69, D57.0, D57.00, D57.01, D57.02, D57.21, D57.211, D57.212, D57.219, D57.41, D57.411, D57.419, D57.3, D57.412, D57.81, D57.811, D57.812, D57.819

Use caseCodes represent crisis while in ER/inpatient and are used to verify VOC events

Documentation requirementProgress notes with date, outcome and intervention must accompany ICD codes for verification