chenodiol — Blue Cross Blue Shield of Montana

Initial criteria

• ONE of the following:
• A. BOTH of the following:
• 1. ONE of the following:
• A. The patient has a diagnosis of cerebrotendinous xanthomatosis (CTX) as confirmed by ONE of the following: genetic testing confirming variants in the CYP27A1 gene OR elevated plasma cholestanol ≥ 5–10× ULN AND urine positive for bile alcohols AND clinical findings consistent for CTX (e.g., xanthomas, infantile-onset diarrhea, childhood-onset cataract(s), adult-onset progressive neurologic dysfunction) OR
• B. The patient has another FDA labeled indication for the requested agent and route of administration
• 2. 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) support exists for using the requested agent for the patient’s age for the requested indication
• The patient has had a baseline liver transaminase (ALT and AST) and total bilirubin level prior to initiating the requested agent
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, geneticist, hepatologist, endocrinologist, neurologist) or 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
• OR The member resides in Ohio AND the plan is Fully Insured or HIM Shop (SG) AND BOTH of the following: (A) the patient does NOT have any FDA labeled contraindications to the requested agent AND (B) ONE of the following: (1) the patient has another FDA labeled indication for the requested agent and route of administration OR (2) the patient has another indication supported in compendia for the requested agent and route of administration OR (3) the prescriber has submitted two articles from major peer-reviewed professional medical journals supporting the proposed use as generally safe and effective (acceptable design: randomized, double blind, placebo controlled clinical trial)

Reauthorization criteria

• 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 patient is monitored for changes in liver transaminase (ALT and AST) and total bilirubin level AND BOTH of the following: (A) liver transaminase levels < 3× ULN AND (B) total bilirubin level < 2× ULN
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, geneticist, hepatologist, endocrinologist, neurologist), or the prescriber has consulted with a specialist
• The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

12 months