CurrentWellcare HawaiiPolicy CP.PHAR.449

Crizanlizumab-tmca (Adakveo) Coverage Criteria

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This policy governs medical-necessity and prior-authorization coverage for Adakveo for sickle cell disease and related indications across Commercial, health insurance marketplace, and Medicaid lines of business, subject to applicable benefit documents and state Medicaid provisions.

Policy Summary

PayerWellcare Hawaii

PolicyCrizanlizumab-tmca (Adakveo) Coverage Criteria

Policy CodePolicy N/A

Change TypeMaterial revision

Effective Date03.01.20

Next Review DateN/A

Key ActionSubmit office chart notes, laboratory results, and other clinical information documenting that all approval criteria are met.

SourceLink

POLICY UPDATE CHANGES

The policy added a requirement for a trial of L-glutamine at maximally tolerated doses before initial Adakveo approval.

The policy was finalized for the health insurance marketplace line of business and aligned with Centene medical drug policies.

5 mg/kgMaximum Adakveo dose per administration

2 VOCsMinimum qualifying VOCs for initial therapy

6 monthsInitial approval duration

12 months

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CurrentWellcare HawaiiPolicy CP.PHAR.449

Crizanlizumab-tmca (Adakveo) Coverage Criteria

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Policy Summary

PayerWellcare Hawaii

PolicyCrizanlizumab-tmca (Adakveo) Coverage Criteria

Policy CodePolicy N/A

Change TypeMaterial revision

Effective Date03.01.20

Next Review DateN/A

Key ActionSubmit office chart notes, laboratory results, and other clinical information documenting that all approval criteria are met.

SourceLink

POLICY UPDATE CHANGES

The policy added a requirement for a trial of L-glutamine at maximally tolerated doses before initial Adakveo approval.

The policy was finalized for the health insurance marketplace line of business and aligned with Centene medical drug policies.

5 mg/kgMaximum Adakveo dose per administration

2 VOCsMinimum qualifying VOCs for initial therapy

6 monthsInitial approval duration

12 months

Continued-therapy approval duration

16 yearsMinimum age for FDA-approved indication

Medical Necessity and Coverage Criteria

Initial Therapy — Sickle Cell Disease

Covered for initial therapy when all of the following are met.

Initial sickle cell disease criteria

Qualifying sickle cell genotype
- Homozygous hemoglobin S.
- Hemoglobin Sβ0-thalassemia.
- Hemoglobin Sβ+-thalassemia.
- Hemoglobin SC.
Age: Member is at least 16 years old.≥ 16 years
Specialist involvement: Prescribed by or in consultation with a hematologist.
Hemoglobin level meets the minimum threshold.≥ 4 g/dL
Hydroxyurea-related VOC requirement
- VOC despite hydroxyurea: At least 2 VOC within the past 6 months while on hydroxyurea at up to maximally indicated doses.≥ 2 VOC in past 6 months
- Hydroxyurea intolerance or contraindication: Hydroxyurea is contraindicated or not tolerated and the member has experienced at least 2 VOC within the past 12 months.≥ 2 VOC in past 12 months
  Myelosuppression alone does not qualify as hydroxyurea treatment failure; the policy recommends a 6-month trial at the maximum tolerated dose before considering discontinuation for treatment failure.
L-glutamine trial: Failure of L-glutamine at up to maximally tolerated doses, unless contraindicated or associated with clinically significant adverse effects.
Baseline VOC documentation: Baseline incidence of VOC over the preceding 12 months is documented.
Concurrent hydroxyurea: Adakveo is prescribed concurrently with hydroxyurea unless hydroxyurea is contraindicated or causes clinically significant adverse effects.
No concurrent Oxbryta: Adakveo is not prescribed concurrently with Oxbryta.
Dose limit: Requested dose does not exceed the policy dosing regimen.5 mg/kg on Day 1 and Day 15, followed by 5 mg/kg every 4 weeks

Continuation Therapy — Sickle Cell Disease

Covered for continued therapy when all of the following are met.

Continuation sickle cell disease criteria

Existing coverage eligibility: Member is currently receiving medication through the Centene benefit or previously met all initial approval criteria.
Positive response: Documented improvement in VOC incidence from baseline.
Concurrent hydroxyurea: Adakveo is prescribed concurrently with hydroxyurea unless hydroxyurea is contraindicated or associated with clinically significant adverse effects.
No concurrent Oxbryta: Adakveo is not prescribed concurrently with Oxbryta.

Other Diagnoses or Indications

For other diagnoses or indications, coverage requires one of the specified pathways.

Other diagnoses or indications

Existing therapy with response: Member is currently receiving medication through the Centene benefit and documentation supports a positive response to therapy.
Approval duration is the duration of the request or 6 months, whichever is less.
Off-label review: If the diagnosis is not specifically listed among diagnoses or indications for which coverage is not authorized, refer to the applicable off-label use policy for the relevant line of business.

Non-FDA-approved indications not addressed by this policy are not authorized unless sufficient documentation of efficacy and safety is available under the applicable off-label use policy or evidence of coverage documents.

Concurrent use of Adakveo with Oxbryta is not authorized under the initial or continued-therapy coverage criteria.

Diagnosis Codes and Clinical Parameters

ICD-10-CM Diagnosis Codes that Support Coverage CriteriaICD-10Covered

D57.0*	Hb-SS disease with crisis
D57.1	Sickle-cell disease without crisis
D57.2*	Sickle-cell/Hb-C disease
D57.4*	Sickle-cell thalassemia

Age

Minimum age≥ 16 years

Covered populationAdults and pediatric patients aged 16 years and older with qualifying sickle cell disease

ApplicationThe age requirement applies to initial Adakveo approval for sickle cell disease

Hemoglobin

Minimum hemoglobin≥ 4 g/dL

AbbreviationHb means hemoglobin

ApplicationThe hemoglobin threshold is an initial approval criterion for sickle cell disease

Prior Authorization and Provider Requirements

Prior Authorization

Adakveo Prior Authorization and Approval Duration

Prior authorization is required. Submit documentation supporting that the member meets all applicable Adakveo approval criteria. Initial approval is for 6 months; continued approval is for 12 months when continuation criteria are met.

Adakveo (crizanlizumab-tmca) approval duration: 6 months initially.
Continued-therapy approval duration: 12 months.

Step Therapy

Hydroxyurea and L-Glutamine Trial Required

Document prior treatment with hydroxyurea and L-glutamine before initiating Adakveo. Hydroxyurea should be used at up to maximally indicated or tolerated doses, and L-glutamine at up to maximally tolerated doses, unless the relevant agent is contraindicated or clinically significant adverse effects occur.

Hydroxyurea treatment failure generally requires a 6-month trial at the maximum tolerated dose; reversible, dose-dependent myelosuppression does not qualify as treatment failure.

Medication and Indication Background

Crizanlizumab-tmca (Adakveo) is a selectin blocker used to reduce the frequency of vaso-occlusive crises in people with sickle cell disease. The policy’s FDA-approved indication applies to adults and pediatric patients aged 16 years and older with specified sickle cell genotypes, including patients with recurrent vaso-occlusive crises despite hydroxyurea or who cannot use hydroxyurea and have completed the required L-glutamine treatment pathway.

Clinical Definitions and Abbreviations

Vaso-Occlusive Crisis (VOC)

TermVaso-occlusive crisis (VOC)

Clinical eventA previously documented episode of acute painful crisis or acute chest syndrome for which there was no explanation other than VOC

Treatment requirementThe episode required prescribed or healthcare-professional-instructed analgesics for moderate to severe pain

SCD

AbbreviationSCD

MeaningSickle cell disease

Policy useSCD is the covered disease indication addressed by the Adakveo criteria

Adakveo Initial Therapy

Covered for initial therapy when all of the following are met.

Initial Adakveo therapy

Qualifying sickle cell genotype
- Homozygous hemoglobin S.
- Hemoglobin Sβ0-thalassemia.
- Hemoglobin Sβ+-thalassemia.
- Hemoglobin SC.
Age:

Adakveo Continuation Therapy

Continued therapy requires all of the following.

Adakveo continuation criteria

Established eligibility: Member is currently receiving Adakveo through the Centene benefit or previously met all initial approval criteria.
Documented response: Member is responding positively to therapy, as evidenced by documented improvement in the incidence of VOC from baseline.
Concurrent hydroxyurea: Adakveo is prescribed concurrently with hydroxyurea unless hydroxyurea is contraindicated or causes clinically significant adverse effects.

Required Prior Therapy

Step	Required prior therapy	Requirement before Adakveo
1	Hydroxyurea	Must be tried at up to maximally indicated doses. The member must have experienced at least 2 vaso-occlusive crises within the past 6 months while receiving hydroxyurea, unless hydroxyurea is contraindicated or not tolerated.

Step	Required prior therapy	Requirement before Adakveo
2	L-glutamine	Must be tried at up to maximally tolerated doses and failed, unless contraindicated or associated with clinically significant adverse effects.

Adakveo Dose and Administration Limits

Crizanlizumab-tmca (Adakveo)

DrugCrizanlizumab-tmca (Adakveo)

Authorized dose5 mg/kg per infusion

Administration scheduleIntravenous infusion over 30 minutes on Week 0, Week 2, and every 4 weeks thereafter

Policy Summary

PayerWellcare Hawaii

PolicyCrizanlizumab-tmca (Adakveo) Coverage Criteria

Policy CodePolicy N/A

Change TypeMaterial revision

Effective Date03.01.20

Next Review DateN/A

Key ActionSubmit office chart notes, laboratory results, and other clinical information documenting that all approval criteria are met.

SourceLink

On This Page

Dose increase limit: If requesting a dose increase, the new dose does not exceed the policy limit.5 mg/kg every 4 weeks

Vaso-occlusive Crises With Hydroxyurea

Qualifying historyAt least 2 vaso-occlusive crises within the past 6 months

Hydroxyurea statusCrises must occur while receiving hydroxyurea at up to maximally indicated doses

Approval pathwayThis is one of the qualifying hydroxyurea/VOC pathways for initial Adakveo therapy

Vaso-occlusive Crises Without Hydroxyurea

Qualifying historyAt least 2 vaso-occlusive crises within the past 12 months

Hydroxyurea statusThe member must have intolerance or a contraindication to hydroxyurea

Approval pathwayThis pathway applies when hydroxyurea cannot be used for initial Adakveo therapy

Adakveo Dose

Dose5 mg/kg per infusion

Initial administrationsAdminister on Day 1 and Day 15

Maintenance scheduleAdminister 5 mg/kg every 4 weeks thereafter

Hydroxyurea Titration ANC

Adult ANC thresholdANC ≥ 2,000/uL

Younger-patient ANC thresholdANC ≥ 1,250/uL in younger patients with lower baseline counts

Monitoring contextCBC with WBC differential and reticulocyte counts should be obtained at least every 4 weeks during titration

Hydroxyurea Titration Platelet Count

Minimum platelet count≥ 80,000/uL

PurposeThis platelet value indicates that it is safe to increase the hydroxyurea dose

Low-count managementIf thrombocytopenia occurs, hydroxyurea dosing is held and CBC with WBC differential is monitored weekly

Failure to increase MCV or HbF alone is not an indication to discontinue hydroxyurea.

For hydroxyurea intolerance or contraindication, document the reason and the required VOC history.

Document L-glutamine failure, contraindication, or clinically significant adverse effects.

Adakveo should be prescribed concurrently with hydroxyurea unless hydroxyurea is contraindicated or clinically significant adverse effects are documented.

Documentation Required

Clinical Documentation for Prior Authorization

The prescriber must provide clinical documentation demonstrating that all approval criteria are satisfied. Supporting evidence may include office chart notes, laboratory results, treatment history, VOC records, and documentation of contraindications, intolerance, or adverse effects when an exception applies.

Include the SCD diagnosis and genotype, age, hemoglobin level, prescriber specialty or hematology consultation, and relevant treatment history.
Document baseline VOC incidence over the preceding 12 months and the VOC history used to support the request.
Include laboratory results and other clinical information supporting eligibility and safe treatment.

Documentation Required

Document Response for Continued Therapy

For continued therapy, document a positive response to Adakveo by showing improvement in the incidence of vaso-occlusive crises (VOC) compared with the member’s documented baseline.

Compare current VOC incidence with the baseline incidence submitted for initial approval.
A response must be documented for continued approval.

Documentation Required

Hydroxyurea Monitoring Requirements

Monitor hydroxyurea with complete blood counts, white blood cell differential, and reticulocyte counts at least every 4 weeks during dose titration, and document the member’s response. Dose increases should be supported by safe blood-count values.

For dose escalation, ANC should be at least 2,000/uL in adults or at least 1,250/uL in younger patients with lower baseline counts.
Platelet count should be at least 80,000/uL.
If neutropenia or thrombocytopenia occurs, hold hydroxyurea and monitor CBC and WBC differential weekly.
Hydroxyurea may be restarted after laboratory values recover.

Denial Risk

Initial Clinical Criteria Not Met

An initial request may be denied when the member does not meet the required SCD clinical criteria or the submitted documentation is insufficient. All applicable criteria must be satisfied, including genotype, age, hemoglobin, hematology involvement, VOC history, baseline documentation, and required prior treatment.

SCD must have one of these genotypes: homozygous hemoglobin S, hemoglobin Sβ0-thalassemia, hemoglobin Sβ+-thalassemia, or hemoglobin SC.
Member must be 16 years or older and have an Hb level of at least 4 g/dL.
Medication must be prescribed by or in consultation with a hematologist.
Member must have at least 2 VOC in the past 6 months while receiving hydroxyurea at up to maximally indicated doses, or at least 2 VOC in the past 12 months when hydroxyurea is contraindicated or not tolerated.
Baseline VOC incidence for the prior 12 months must be documented.
A VOC must be a documented acute painful crisis or acute chest syndrome requiring prescribed or professionally instructed analgesic use for moderate to severe pain, with no explanation other than VOC.

Denial Risk

Concurrent Oxbryta Use Not Covered

Adakveo must not be prescribed concurrently with Oxbryta. Concurrent use creates a denial risk for both initial and continued-therapy requests.

Review the complete medication regimen before submitting the request.
Document discontinuation of Oxbryta when applicable.

Denial Risk

Dose Exceeds Policy Limit

Do not request or administer Adakveo above the policy dose limit. Excessive dosing or a continuation dose increase beyond the allowed regimen may result in denial.

Initial regimen: 5 mg/kg IV on Day 1 and Day 15, then 5 mg/kg every 4 weeks.
For continuation dose increases, the new dose must not exceed 5 mg/kg every 4 weeks.
Maximum dose: 5 mg/kg; administer by IV infusion over 30 minutes on Week 0, Week 2, and every 4 weeks thereafter.

Denial Risk

Non-FDA Indication Requires Supporting Evidence

Coverage is not authorized for non-FDA-approved indications that are not addressed by this policy unless sufficient efficacy and safety documentation is submitted under the applicable off-label-use policy or evidence of coverage.

For commercial members, reference CP.CPA.09.
For health insurance marketplace members, reference HIM.PHAR.21.
For Medicaid members, reference CP.PMN.53.

AbbreviationHb

MeaningHemoglobin

Policy thresholdThe initial approval criterion requires Hb ≥ 4 g/dL

Myelosuppression and Hydroxyurea Treatment Failure

MyelosuppressionDose-dependent and reversible toxicity associated with hydroxyurea

Treatment-failure distinctionMyelosuppression alone does not qualify as hydroxyurea treatment failure

Treatment-duration contextA 6-month trial at the maximum tolerated dose is recommended before considering discontinuation for treatment failure

Additional response measureA lack of increase in mean corpuscular volume or fetal hemoglobin is not an indication to discontinue therapy

Member is at least 16 years old.

Specialist involvement: Adakveo is prescribed by or in consultation with a hematologist.

Hemoglobin level meets the minimum threshold.≥ 4 g/dL

Hydroxyurea and VOC requirement

VOC despite hydroxyurea: At least 2 VOC within the past 6 months while receiving hydroxyurea at up to maximally indicated doses.≥ 2 VOC in past 6 months
Hydroxyurea intolerance or contraindication: Hydroxyurea is contraindicated or not tolerated and the member has experienced at least 2 VOC within the past 12 months.≥ 2 VOC in past 12 months
Myelosuppression alone does not qualify as hydroxyurea treatment failure; the policy recommends a 6-month trial at the maximum tolerated dose before considering discontinuation for treatment failure.

L-glutamine trial: L-glutamine has failed at up to maximally tolerated doses, unless it is contraindicated or associated with clinically significant adverse effects.

Baseline VOC documentation: Baseline incidence of VOC over the preceding 12 months is documented.

Concurrent hydroxyurea: Adakveo is prescribed concurrently with hydroxyurea unless hydroxyurea is contraindicated or causes clinically significant adverse effects.

No concurrent Oxbryta: Adakveo is not prescribed concurrently with Oxbryta.

Dosing requirement: The requested dose does not exceed the policy dosing regimen.5 mg/kg on Day 1 and Day 15, followed by 5 mg/kg every 4 weeks

Initial approval duration is 6 months.

No concurrent Oxbryta:

Adakveo is not prescribed concurrently with Oxbryta.

Dose increase limit: If a dose increase is requested, the new dose does not exceed the policy limit.5 mg/kg every 4 weeks

Continued therapy approval for sickle cell disease is generally 12 months.