Zokinvy — Blue Cross Blue Shield of Montana

Initial criteria

• ONE of the following:
• A. BOTH of the following: 1. Diagnosis of Hutchinson-Gilford progeria syndrome (HGPS) AND 2. Genetic testing confirmed pathogenic variant in LMNA gene resulting in progerin (medical record required)
• OR B. Patient has a processing-deficient progeroid laminopathy AND ONE of the following: 1. Genetic testing confirmed heterozygous LMNA mutation with progerin-like protein accumulation (medical record required) OR 2. Genetic testing confirmed homozygous or compound heterozygous ZMPSTE24 mutations (medical record required)
• AND If patient has FDA labeled indication, then ONE of the following: A. Patient’s age is within FDA labeling for requested indication OR B. There is support for use at patient’s age for requested indication
• AND Body surface area ≥ 0.39 m^2
• AND Prescriber is a specialist (e.g., cardiologist, geneticist) or has consulted with a specialist in area of diagnosis
• AND Patient has no FDA labeled contraindications to requested agent
• ALTERNATIVELY, approval if ALL apply: 1. Member resides in Ohio AND 2. Plan is Fully Insured or HIM Shop (SG) AND BOTH: A. Patient has no FDA labeled contraindications AND B. ONE of: (1) Patient has another FDA labeled indication; (2) Patient has another indication supported in compendia; (3) Prescriber submitted two major peer-reviewed journal articles supporting proposed use and safety/effectiveness (acceptable study designs include randomized, double blind, placebo controlled trials; case studies not acceptable; appropriate compendia levels specified)

Reauthorization criteria

• 1. Patient previously approved for requested agent through plan’s Prior Authorization process
• 2. Patient has had clinical benefit with the requested agent
• 3. Prescriber is a specialist (e.g., cardiologist, geneticist) or has consulted with one
• 4. Patient has no FDA labeled contraindications to the requested agent

Approval duration

12 months