lonafarnib — Blue Cross Blue Shield of Illinois

Hutchinson-Gilford progeria syndrome (HGPS)

Initial criteria

ONE of the following:
A. BOTH of the following: 1. The patient has a diagnosis of Hutchinson-Gilford progeria syndrome (HGPS) AND 2. Genetic testing has confirmed 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 has confirmed heterozygous LMNA mutation with progerin-like protein accumulation (medical record required) OR 2. Genetic testing has confirmed homozygous or compound heterozygous ZMPSTE24 mutations (medical record required) AND
If the patient has an FDA labeled indication, then ONE of the following: A. The 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 AND
The patient has a body surface area (BSA) ≥ 0.39 m^2 AND
The prescriber is a specialist in the area of the patient’s diagnosis (e.g., cardiologist, geneticist), or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND
The patient does NOT have any FDA labeled contraindications to the requested agent

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

12 months