Sunflower Health Plan ADAKVEO, Crizanlizumab-tmca Form

Crizanlizumab-tmca (Adakveo®) is a selectin blocker. FDA Approved Indication(s) To reduce the frequency of vasoocclusive crises (VOC) in adults and pediatric patients aged 16 years and older with sickle cell disease (SCD). Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria. It is the policy of health plans affiliated with Centene Corporation® that Adakveo are medically necessary when the following criteria are met: I. Initial Approval Criteria A. Sickle Cell Disease (must meet all): 1. Diagnosis of SCD with one of the following genotypes (a, b, c, or d): a. Homozygous hemoglobin S; b. Hemoglobin Sβ0‑thalassemia; c. Hemoglobin Sβ+‑thalassemia; d. Hemoglobin SC; 2. Age ≥ 16 years; 3. Prescribed by or in consultation with a hematologist; 4. Hb level ≥ 4 g/dL; 5. Member meets one of the following (a or b): a. Member has experienced at least 1 VOC within the past 6 months while on hydroxyurea at up to maximally indicated doses (see Appendix D); b. Member has intolerance* or contraindication to hydroxyurea and has experienced at least 2 VOC within the past 12 months (see Appendix D); *Myelosuppression and hydroxyurea treatment failure: Myelosuppression is dose-dependent and reversible and does not qualify for 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. 6. Failure of L-glutamine at up to maximally tolerated doses, unless contraindicated or clinically significant adverse effects are experienced; 7. Documentation of baseline incidence of VOC over the last twelve months; Page 1 of 7 CLINICAL POLICY Crizanlizumab-tmca 8. Adakveo is prescribed concurrently with hydroxyurea, unless contraindicated or clinically significant adverse effects are experienced; 9. Adakveo is not prescribed concurrently with Oxbryta®; 10. Dose does not exceed 5 mg/kg doses on Day 1 and Day 15, followed by 5 mg/kg every 4 weeks. Approval duration: 6 months B. Other diagnoses/indications (must meet 1 or 2): 1. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or 2. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. II. Continued Therapy A. Sickle Cell Disease (must meet all): 1. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B); 2. Member is responding positively to therapy as evidenced by a documented improvement in the incidence of VOC from baseline; 3. Adakveo is prescribed concurrently with hydroxyurea, unless contraindicated or clinically significant adverse effects are experienced; 4. Adakveo is not prescribed concurrently with Oxbryta; 5. If request is for a dose increase, new dose does not exceed 5 mg/kg every 4 weeks. Approval duration: 12 months B. Other diagnoses/indications (must meet 1 or 2): 1. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): Page 2 of 7 CLINICAL POLICY Crizanlizumab-tmca a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or 2. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. III. Diagnoses/Indications for which coverage is NOT authorized: A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policies – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid or evidence of coverage documents. IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key FDA: Food and Drug Administration SCD: sickle cell disease Hb: hemoglobin VOC: vaso-occlusive crises Appendix B: Therapeutic Alternatives This table provides a listing of preferred alternative therapy recommended in the approval criteria. The drugs listed here may not be a formulary agent for all relevant lines of business and may require prior authorization. Drug Name Dosing Regimen hydroxyurea (Droxia) hydroxyurea (Siklos) L-glutamine (Endari) Age ≥ 18 years Initial: 15 mg/kg/day PO single dose; based on blood counts, may increase by 5 mg/kg/day every 12 weeks to a max 35 mg/kg/day Age ≥ 2 years Initial: 20 mg/kg/day PO QD; based on blood counts, may increase by 5 mg/kg/day every 8 weeks or if a painful crisis occurs Weight > 65 kg: 15 g (3 packets) PO BID Weight 30 to 65 kg: 10 g (2 packets) PO BID Weight < 30 kg: 5 g (1 packet) PO BID Dose Limit/ Maximum Dose 35 mg/kg/day 35 mg/kg/day 30 g/day (maximum dose based on weight) Therapeutic alternatives are listed as Brand name® (generic) when the drug is available by brand name only and generic (Brand name®) when the drug is available by both brand and generic. Page 3 of 7 CLINICAL POLICY Crizanlizumab-tmca Appendix C: Contraindications/Boxed Warnings None reported Appendix D: General Information • A VOC is defined as a previously documented episode of acute painful crisis or acute chest syndrome (ACS) for which there was no explanation other than VOC that required prescription or healthcare professional-instructed use of analgesics for moderate to severe pain. • Myelosuppression and hydroxyurea treatment failure: Myelosuppression is dose- dependent and reversible and does not qualify for 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. • Hydroxyurea dose titration: Members should obtain complete blood counts (CBC) with white blood cell (WBC) differential and reticulocyte counts at least every 4 weeks for titration. The following lab values indicate that it is safe to increase dose. o Absolute neutrophil count (ANC) in adults ≥ 2,000/uL, or ANC ≥ 1,250/uL in younger patients with lower baseline counts o Platelet counts ≥ 80,000/uL If neutropenia or thrombocytopenia occurs: hydroxyurea dosing is held, CBC and WBC differential are monitored weekly, and members can restart hydroxyurea when values have recovered. V. Dosage and Administration Indication SCD Dosing Regimen Administer 5 mg/kg by intravenous infusion over a period of 30 minutes on Week 0, Week 2, and every 4 weeks thereafter. Maximum Dose 5 mg/kg VI. Product Availability Single-dose vial for injection: 100 mg/10 mL (10 mg/mL) VII.