CHOLBAM (cholic acid)
SELF-ADMINISTRATION
Indications for Prior Authorization:
- Treatment of bile acid synthesis disorders due to single enzyme defects (SEDs)
- Adjunctive treatment of peroxismal disorders (PDs) including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea or complications from decreased fat soluble vitamin absorption
Patients must meet the following criteria for the indication(s) above:
- Prescribed by an specialist (e.g., hepatologist or pediatric gastroenterologist)
- Used to treat bile acid synthesis disorders due to SEDs, OR as asjunctive treatment of PDs including Zellweger spectrum disorders in patients who exhibit manifestations of liver diasese, steatorrhea or complications from decreased fat soluble vitamin absorption
- Baseline liver function labs (e.g., AST, ALT, GGT, ALP, bilirubin, INR) (updated labs required for renewal)
Dosing:
- Neonates at least 3 weeks of age, pediatrics and adults: 10 to 15 mg/kg once daily or in two divided doses
- Concomitant familial hypertriglyceridemia: 11 to 17 mg/kg once daily or in two divided doses and is adjusted based on clinical response
- Discontinue Cholbam if liver function does not improve within 3 months of starting tretment, if complete biliary obstruction develops, or if there are persistent clinical or laboratory indicators of worsening liver function or cholestasis; consider restarting a lower dose when parameters return to baseline (concurrent elevations of GGT and ALT may indicate Cholbam overdose)
Approval:
- Initial: 3 months
- Renewal: 1 year