Medical Policy

Effective Date:05/01/2020 Title:Adakveo (crizanlizumab)
Revision Date:07/01/2020 Document:BI652:00
CPT Code(s):J0791
Public Statement

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Adakveo (crizanlizumab) requires prior authorization.

2)    Adakveo is used to reduce the vasocclusive crises (VOCs) in patients aged 16 years and older with sickle cell disease (SCD).

3)    Adakveo is considered a specialty medication.

Medical Statement

Adakveo (crizanlizumab) is considered medically necessary for patients 16 years of age and older who meet the following criteria:


1)    Diagnosis of sickle cell disease (SCD) with one of the following genotypes:

a.    Homzygous hemoblogin S;

b.    Hemoglobin Sβo-thalassemia;

c.    Hemoglobin Sβ+-thalassemia;

d.    Hemoglobin SC;

2)    Prescribed by or in consultation with a hematologist;

3)    Hb level > 4g/dL;

4)    Member meets one of the following:

a.    Member has experienced at least 2 vasocclusive crises (VOC) within the past 6 months while on hydroxyurea at up to maximally indicateddoses;

b.    Member has intolerance* or contraindication to hydroxyurea and has experienced at least 2 VOC within the past 12 months;

*Myelosuppression and hydroxyurea treatment failure: Myelosuppression is does-dependent and reversible and does not qualify as treatment failure. NIH guidelines recommend a 6 month trial on the maximum tolerated dose prior to considering discontinuation due to treatment failure, whether due to lack of adherence or failure to respond to therapy. A lack of increase in mean corpuscular volume (MCV) and/or fetal hemoglobin (HbF) levels is not indication to discontinue therapy.


5)    Documentation of baseline incidence of VOC over the last 12 months;

6)    Adakveo is prescribed concurrently with hydroxyurea, unless contraindicated or clinically significant adverse effects are experienced;

7)    Adakveo is not prescribed concurrently with Oxbryta


Reauthorization Criteria:

1)    Member is responding positively to therapy as evidenced by a documented improvement in the incidence of VOC from baseline AND

2)    Adakveois prescribed concurrently with hydroxyurea, unless contraindicated or clinically significant adverse effects are experienced AND

3)    Adakveo is not prescribed concurrently with Oxbryta.


Codes Used In This BI:


1)    J0791 - Injection, crizanlizumab-tmca, 5mg

Intentially left empty

1)    Adakveo Prescribing Information. East Hanover, NJ: Novartis Pharmaceuticals Corporation; November 2019.

2)    Kutlar A, Kanter J, Liles DK, et al. Effect of Crizanlizumab on pain crises in subgroups of patients with sickle cell disease: A SUSTAIN study analysis. Am J Hematol. 2019;94:55-61.

3)    Ataga K, Kutlar A, Kanter J, et al. Crizanlizumab for the Prevention of Pain Crisesin Sickle CellDisease. N Engl J Med. 2017 Feb2;376(5):429-439.

4)    Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 Sep10;312(10):1033-48.

Application to Products
This policy applies to all health plans administered by QualChoice, both those insured by QualChoice and those that are self-funded by the sponsoring employer, unless there is indication in this policy otherwise or a stated exclusion in your medical plan booklet. Consult the individual plan sponsor Summary Plan Description (SPD) for self-insured plans or the specific Evidence of Coverage (EOC) for those plans insured by QualChoice. In the event of a discrepancy between this policy and a self-insured customer’s SPD or the specific QualChoice EOC, the SPD or EOC, as applicable, will prevail. State and federal mandates will be followed as they apply.

Changes: QualChoice reserves the right to alter, amend, change or supplement benefit interpretations as needed.