Zokinvy — Blue Cross Blue Shield of Texas

Initial criteria

• ONE of the following: (A) BOTH of the following: (1) Diagnosis of Hutchinson-Gilford progeria syndrome (HGPS) AND (2) Genetic testing confirms a pathogenic variant in the LMNA gene that results in production of progerin (medical record required) OR (B) The patient has a processing-deficient progeroid laminopathy AND ONE of the following: (1) Genetic testing confirms heterozygous LMNA mutation with progerin-like protein accumulation (medical record required) OR (2) Genetic testing confirms homozygous or compound heterozygous ZMPSTE24 mutations (medical record required)
• If the patient has an FDA labeled indication, then ONE of the following: (A) Patient’s age is within FDA labeling for the requested indication for the requested agent OR (B) There is support for using the requested agent for the patient’s age for the requested indication
• Patient has body surface area (BSA) ≥ 0.39 m²
• Prescriber is a specialist in the area of the patient’s diagnosis (e.g., cardiologist, geneticist) OR has consulted with a specialist in the area of the patient’s diagnosis
• Patient does NOT have any FDA labeled contraindications to the requested agent
• Alternative approval path: Member resides in Ohio AND plan is Fully Insured or HIM Shop (SG) AND BOTH of the following: (A) Patient does NOT have any FDA labeled contraindications AND (B) ONE of the following: (1) Patient has another FDA labeled indication for the requested agent and route of administration OR (2) Patient has another indication that is supported in compendia for the requested agent and route of administration OR (3) Prescriber has submitted TWO articles from major peer-reviewed professional medical journals supporting the proposed use(s) as generally safe and effective (case studies not acceptable; compendia levels acceptable as defined)

Reauthorization criteria

• Patient has been previously approved for the requested agent through the plan’s Prior Authorization process
• Patient has had clinical benefit with the requested agent
• Prescriber is a specialist in the area of the patient's diagnosis (e.g., cardiologist, geneticist) OR has consulted with a specialist in the area of the patient’s diagnosis
• Patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

12 months