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Ctexli — Blue Cross Blue Shield of Kansas

cerebrotendinous xanthomatosis (CTX)

Initial criteria

  • ONE of the following:
  • A. The requested agent is eligible for continuation of therapy AND ONE of the following:
  •  1. The patient has been treated with the requested agent (starting on samples is not approvable) within the past 90 days OR
  •  2. The prescriber states the patient has been treated with the requested agent (starting on samples is not approvable) within the past 90 days AND is at risk if therapy is changed OR
  • B. 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 for CTX [e.g., xanthomas in lungs/tendons/bone/CNS, infantile-onset diarrhea, childhood-onset cataract(s), adult-onset progressive neurologic dysfunction (dementia, psychiatric disturbances, pyramidal and/or cerebellar signs, dystonia, atypical parkinsonism, peripheral neuropathy, seizures)] 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 AND
  • 2. The patient has had a baseline liver transaminase (ALT and AST) and total bilirubin level prior to initiating the requested agent AND
  • 3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, geneticist, hepatologist, endocrinologist, neurologist) or has consulted with such a specialist AND
  • 4. The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

  • 1. The patient has been previously approved for the requested agent through the plan’s Prior Authorization process AND
  • 2. The patient has had clinical benefit with the requested agent

Approval duration

12 months