chenodiol — Blue Cross Blue Shield of Illinois

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: (1) Genetic testing confirming variants in the CYP27A1 gene OR (2) ALL of the following: (A) Elevated plasma cholestanol ≥ 5 to 10 times ULN AND (B) Urine positive for bile alcohols AND (C) Clinical findings consistent with CTX [e.g., xanthomas in lungs, tendons, bone, or CNS; infantile-onset diarrhea; childhood-onset cataracts; adult-onset progressive neurologic dysfunction] OR (B) The patient has another FDA labeled indication for the requested agent and route of administration AND (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 OR (B) There is support for using the requested agent for the patient’s age for the requested indication
• The patient has had a baseline liver transaminase (ALT, 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

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, AST) and total bilirubin levels 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 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

Approval duration

12 months