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Cholbam (cholic acid)CareFirst (Caremark)

Bile acid synthesis disorders due to single enzyme defects (SEDs)

Initial criteria

  • Diagnosis confirmed by mass spectrometry or other biochemical testing, genetic testing, or enzyme assay
  • Member has liver dysfunction (elevated transaminases, elevated bilirubin, or presence of cholestasis) at baseline

Reauthorization criteria

  • Member is currently receiving the requested medication through a paid pharmacy or medical benefit
  • Member has achieved and maintained improvement in liver function from baseline (reduced transaminases, reduced bilirubin, no evidence of cholestasis on liver biopsy)

Approval duration

Initial: 6 months; Reauthorization: 12 months