Adakveo (crizanlizumab-tmca)

POLICY NUMBER

L.5.01.555

Adakveo (crizanlizumab-tmca)

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Sickle cell disease (SCD) is the most common inherited hematologic disorder in the United States in which red blood cells are abnormally shaped in a crescent or sickle form, which restricts the flow in blood vessels and limits delivery of oxygen to tissue. This characteristic presence of sickle hemoglobin (HbS) is associated with chronic hemolysis, recurrent vasoocclusive crises (severe pain episodes), organ damage, and premature death. Vasoocclusive crises (VOCs), also known as sickle cell pain crises, are the clinical hallmark of SCD and are the primary cause of hospitalization in SCD. The key interventions to reduce VOCs include long-term treatment with hydroxyurea and regular blood transfusions; however, hydroxyurea is not completely effective in VOC prevention.

Crizanlizumab is a humanized IgG2 kappa monoclonal antibody that binds to P-selectin and blocks interactions with its ligands, including P-selectin glycoprotein ligand 1 on the surface of activated endothelium and platelets, causing a blockage of interactions between endothelial cells, platelets, red blood cells, and leukocytes. Adakveo (crizanlizumab-tmca) is indicated to reduce the frequency of vaso-occlusive crises (VOCs) in adults and pediatric patients aged 16 years and older with sickle cell disease.

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements.

Adakveo (crizanlizumab-tmca) may be considered medically necessary when ALL of the following criteria are met:

1. The individual is 16 years of age or older;

2. The individual has a documented diagnosis of sickle cell disease (i.e., HbSS, HbSC, HbSβ0-thalassemia, HBSβ+-thalassemia);

3. The individual has experienced at least two sickle cell-related pain crises (defined as acute episodes of pain requiring a medical facility visit and treatment with oral or parenteral narcotic agents or a parenteral NSAID) in the past 12 months;

4. ONE of the following:

   1. The individual is currently taking hydroxyurea and BOTH of the following:

      1. Hydroxyurea dose has been stable at the maximally tolerated and acceptable dose for at least 3 months; AND

      2. Hydroxyurea will be continued along with Adakveo (crizanlizumab-tmca); OR

   2. The individual has a history of treatment

      failure, documented intolerance, FDA-labeled contraindication, or hypersensitivity to hydroxyurea;

5. If female, the provider has confirmed the individual is not pregnant;

6. The prescriber is a specialist or has consulted with a specialist in an area of the individual’s diagnosis (i.e., board certified hematologist);

7. The individual does not have any FDA-labeled contraindication(s) to therapy with the requested agent; AND

8. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

Adakveo (crizanlizumab-tmca) may be approved for RENEWAL when ALL of the following criteria are met:

1. The individual has been previously approved for therapy with the requested agent through BCBSMS PA process;

2. The individual has documented clinical improvement (i.e., reduction in the number of vaso-occlusive crises, delay in time to crises, reduction in number of hospital admissions);

3. The individual does not have any FDA-labeled contraindication(s) to therapy with the requested agent; AND

4. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

POLICY EXCEPTIONS

State Health Plan (State and School Employees) Participants

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B. appropriate with regard to standards of good medical practice; and

C. not solely for the convenience of the Member, his or her Provider; and

D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

Medication Intolerance

Medication intolerance is defined as the inability to digest, metabolize, or mitigate the expected adverse effects of a prerequisite medication for ≥4 weeks use as evidenced by documented attempted intervention(s) to minimize sensitivity where appropriate (i.e., dose titration, dose reduction, administered with food, administration at different times of day, use of alternative formulation(s) etc.) and claims history. Experience of common side effects of medication will not be considered medication intolerance for the purpose of this review.

Medication Failure

Medication failure is defined as disease progression at generally accepted doses (for >3 months use) as appropriate for the disease state being treated. Dosages below the recommended dose for the specific condition being treated and/or experience of common side effects of medication will not be considered medication failure or lack of response for the purpose of this review.

BCBSMS determines patient medication trial and adherence by a review of pharmacy claims data over the preceding twelve months. Additional information may be requested on a case-by-case basis to allow for proper review. If member is new to BCBSMS and pharmacy records are needed to confirm medication trials and adherence, it is the responsibility of the member and/or requesting provider to obtain said records and to submit them to BCBSMS upon request. Medical records from the provider that list previously prescribed medications will not be sufficient to show medication trials or adherence.

POLICY HISTORY

05/01/2020: New policy added.

06/26/2020: Code Reference section updated to add new HCPCS code J0791, effective 07/01/2020.

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

07/01/2023: Policy Exceptions updated regarding State Health Plan (State and School Employees) Participants. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity."

06/03/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding indications for Adakveo (crizanlizumab-tmca). Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Medically necessary criteria revised to define sickle cell-related pain crises, to separate statement regarding hydroxyurea, and to add criteria regarding contraindications and dose requirements. Removed criteria regarding combination therapy with Oxbryta or Endari and long-term red blood cell transfusion therapy. Added renewal criteria and length of approval. Removed statement regarding request for non-formulary/non-covered medication. Policy Guidelines updated regarding medication intolerance and medication failure. Sources updated.

SOURCE(S)

1. Adakveo prescribing information. Novartis Pharmaceuticals Corporation. June 2024. Last accessed March 2025.

2. Institute for Clinical and Economic Review (ICER). Crizanlizumab, Voxelotor, and L-Glutamine for Sickle Cell Disease: Effectiveness and Value. Evidence Report March 12, 2020. Updated February 9, 2021. Accessed March 2025. Available at:

   https://icer.org/wp-content/uploads/2021/02/ICER_SCD_Evidence-Report_031220-FOR-PUBLICATION.pdf

3. Vichinsky EP. Disease-modifying therapies to prevent pain and other complications of sickle cell disease.  UpToDate, Connor RF (ed), Wolter Kluwer. Accessed March 7, 2025. https://www.uptodate.com/contents/disease-modifying-therapies-to-prevent-pain-and-other-complications-of-sickle-cell-disease

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Covered Codes

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