Last Review Date: 01/04/2024

Date of Origin: 12/16/2019

Dates Reviewed: 12/2019, 01/2021, 01/2022, 01/2023, 01/2024

1. Length of Authorization

Coverage will be provided for 6 months initially and may be renewed annually thereafter.

2. Dosing Limits

A. Quantity Limit (max daily dose) [NDC Unit]:

• Adakveo 100 mg/10 mL single-dose vials: 6 vials at weeks 0 and 2 and every 4 weeks thereafter

B. Max Units (per dose and over time) [HCPS Unit]:

• 120 billable units at weeks 0 and 2 and every 4 weeks thereafter

3. Initial Approval Criteria ¹

Coverage is provided in the following conditions:

• Patient is at least 16 years of age; AND

Universal Criteria

• Therapy will not be used in conjunction with voxelotor (Oxbryta) or L-glutamine (Endari); AND
  • Patient has not received prior treatment with gene therapy (i.e., lovotibeglogene autotemcel, exagamglogene autotemcel); OR
  • Patient failed to respond or lost response to treatment prior gene therapy (i.e., lovotibeglogene autotemcel, exagamglogene autotemcel); AND

Sickle Cell Disease ^1-3 † Ф

• Patient has a confirmed diagnosis of sickle-cell disease, of any genotype (e.g., HbSS, HbSC, HbS/beta⁰-thalassemia, HbS/beta⁺-thalassemia, and others) as determined by one of the following:
  • Identification of significant quantities of HbS with or without an additional abnormal β-globin chain variant by hemoglobin assay; OR
  • Identification of biallelic HBB pathogenic variants where at least one allele is the p.Glu6Val pathogenic variant on molecular genetic testing; AND
• Patient had an insufficient response to a minimum 3-month trial of hydroxyurea (unless contraindicated or intolerant); AND
• Patient experienced one or more vaso-occlusive crises (VOC)* in the previous year despite adherence to hydroxyurea therapy

*VOC is defined as an event prompting either a visit or outreach to the provider which results in a diagnosis of VOC being made necessitating subsequent interventions such as narcotic pain management, non-steroidal anti-inflammatory therapy, hydration, etc.

† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug

4. Renewal Criteria ^1,3

Coverage can be renewed based upon the following criteria:

• Patient continues to meet the universal and other indication-specific relevant criteria identified in section III; AND
• Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: severe infusion related reactions (e.g., pain in various locations, headache, fever, chills, nausea, vomiting, diarrhea, fatigue, dizziness, pruritus, urticaria, sweating, shortness of breath or wheezing), etc.; AND

• Disease response compared to pretreatment baseline as evidenced by a decrease in the frequency of vaso-occlusive crises (VOC) necessitating treatment, reduction in number or duration of hospitalizations, and/or reduction in severity of VOC

5. Dosage/Administration ¹

6. Billing Code/Availability Information

HCPCS:

• J0791 – Injection, crizanlizumab-tmca, 5 mg; 1 billable unit = 5 mg

NDC:

• Adakveo 100 mg/10 mL (10 mg/mL) single-dose vial: 00078-0883-xx

7. References

1. Adakveo [package insert]. East Hanover, NJ; Novartis Pharmaceuticals, Inc., September 2022. Accessed December 2023.
2. Bender MA, Carlberg K. Sickle Cell Disease. 2003 Sep 15 [Updated 2022 Nov 17]. In: Adam MP, Everman DB, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1377/.
3. Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease. N Engl J Med. 2017 Feb 2;376(5):429-439. doi: 10.1056/NEJMoa1611770. Epub 2016 Dec 3.
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 Sep 10;312(10):1033-48.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

The preceding information is intended for non-Medicare coverage determinations. Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCA/LCD): N/A