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CrenessityBlue Cross Blue Shield of Montana

classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency

Initial criteria

  • The patient has a diagnosis of classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency as confirmed by ONE of the following: (1) Positive infant screening with secondary tier 2 confirmatory testing OR (2) Elevated serum 17-hydroxyprogesterone level (17OHP) above the upper limit of normal (ULN) OR (3) Cosyntropin (ACTH) stimulation test OR (4) Genetic testing for mutation in the СΥΡ21Α2 gene consistent with CAH AND
  • If the patient has an FDA labeled indication, then ONE of the following: (1) The patient’s age is within FDA labeling for the requested indication for the requested agent OR (2) There is support for using the requested agent for the patient’s age for the requested indication AND
  • The patient is currently treated with glucocorticoid replacement therapy (e.g., hydrocortisone, prednisone, prednisolone, dexamethasone) AND
  • The patient will continue glucocorticoid replacement therapy in combination with the requested agent AND
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, 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

Reauthorization criteria

  • The patient has been previously approved for the requested agent through the plan’s Prior Authorization process AND
  • The patient has had clinical benefit with the requested agent AND
  • The patient is currently treated with glucocorticoid replacement therapy AND
  • The patient will continue glucocorticoid replacement therapy in combination with the requested agent AND
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, 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

Approval duration

12 months