Revcovi (elapegademase-lvlr) — CareFirst (Caremark)

Adenosine deaminase severe combined immune deficiency (ADA-SCID)

Initial criteria

The diagnosis is confirmed by one of the following:
• Increased red blood cell deoxyadenosine triphosphate (dATP) or trough deoxyadenosine nucleotide (dAXP) concentrations AND ONE of the following:
• Absent or very low (<1% of normal) adenosine deaminase (ADA) activity in red blood cells
• Genetically confirmed, biallelic variant in the ADA gene
Baseline values for plasma ADA activity, red blood cell dATP, dAXP levels, and/or total lymphocyte counts have been obtained.
Requested medication will only be used until definitive therapy with hematopoietic stem cell transplantation (HSCT), OR member is not a suitable candidate for HSCT (e.g., matched sibling or family donor not available), OR member has failed HSCT.
Member does not have severe thrombocytopenia (platelets <50,000/microL).
Member does not have autoimmune disease requiring immunosuppressive therapy.
Member will be monitored for evidence of treatment efficacy per protocol outlined in the prescribing information during treatment with Revcovi.

Member meets the criteria for initial approval.
Member does not have unacceptable toxicity (e.g., severe injection site reactions/bleeding, severe thrombocytopenia).
Member is experiencing benefit from therapy (e.g., maintenance of target trough plasma ADA activity ≥30 mmol/L, trough erythrocyte dAXP levels below 0.02 mmol/L, improved or stabilized total lymphocyte counts and/or immune function).

12 months