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CholbamCigna

Bile Acid Synthesis Disorders Due to Peroxisomal Disorders (PDs), including Zellweger Spectrum Disorders

Initial criteria

  • Patient has peroxisomal disorders with at least ONE of the following (a or b): a) An abnormal urinary bile acid analysis by Fast Atom Bombardment ionization – Mass Spectrometry (FAB-MS); OR b) Molecular genetic testing consistent with the diagnosis; AND
  • Patient has liver disease, steatorrhea, or complications from decreased fat soluble vitamin absorption (e.g., rickets); AND
  • The medication is prescribed by or in consultation with a hepatologist, metabolic specialist, or gastroenterologist

Reauthorization criteria

  • According to the prescriber, patient has responded to initial Cholbam therapy (e.g., improvements in liver enzymes or improvement in steatorrhea); AND
  • Patient does not have complete biliary obstruction; AND
  • The medication is prescribed by or in consultation with a hepatologist, metabolic specialist, or gastroenterologist

Approval duration

initial 3 months, reauth 1 year