Adult Dosing
Bile acid synthesis disorders due to SEDs and peroxisomal disorders including Zellweger spectrum disorders
- 10-15 mg/kg PO qd or divided bid with food
- In patients with concomitant familial hypertriglyceridemia : 11-17 mg/kg PO qd or divided bid with food
Note:
- Refer package insert for number of capsules that should be administered daily to approximate a 10 mg/kg/day and 15 mg/kg/day dosage using the available 50 mg and 250 mg capsules alone or in combination
- Take cholic acid at least 1 hour before or 4 to 6 hours (or at as great an interval as possible) after a bile acid binding resin or aluminum-based antacid
- Administer the lowest dose that effectively maintains liver function
- The safety and effectiveness of cholic acid on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs including Zellweger spectrum disorders have not been established
Monitoring- Monitor serum aspartate aminotransferase (AST), serum alanine aminotransferase (ALT), serum gamma glutamyltransferase (GGT), alkaline phosphatase (ALP), bilirubin, and INR every month for the first 3 months, every 3 months for the next 9 months, every 6 months during the subsequent three years and annually thereafter
- More frequent monitoring is advised during periods of rapid growth, concomitant disease, and pregnancy
- Discontinue therapy if liver function does not improve within 3 months of treatment initiation or if complete biliary obstruction develops
- Discontinue therapy if clinical or laboratory indicators persistently suggest worsening liver function or cholestasis
Pediatric Dosing
Bile acid synthesis disorders due to SEDs and peroxisomal disorders including Zellweger spectrum disorders
- <3 weeks: Safety and efficacy have not been established
- 3 weeks or older: 10-15 mg/kg PO qd or divided bid with food
- In patients with concomitant familial hypertriglyceridemia : 11-17 mg/kg PO qd or divided bid with food
Note:
- Refer package insert for number of capsules that should be administered daily to approximate a 10 mg/kg/day and 15 mg/kg/day dosage using the available 50 mg and 250 mg capsules alone or in combination
- Take cholic acid at least 1 hour before or 4 to 6 hours (or at as great an interval as possible) after a bile acid binding resin or aluminum-based antacid
- Administer the lowest dose that effectively maintains liver function
- The safety and effectiveness of cholic acid on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs including Zellweger spectrum disorders have not been established
Monitoring- Monitor serum aspartate aminotransferase (AST), serum alanine aminotransferase (ALT), serum gamma glutamyltransferase (GGT), alkaline phosphatase (ALP), bilirubin and INR every month for the first 3 months, every 3 months for the next 9 months, every 6 months during the subsequent three years and annually thereafter
- More frequent monitoring is advised during periods of rapid growth, concomitant disease, and pregnancy
- Discontinue therapy if liver function does not improve within 3 months of treatment initiation or if complete biliary obstruction develops
- Discontinue therapy if clinical or laboratory indicators persistently suggest worsening liver function or cholestasis
See Supplemental Patient Information
- Monitor liver function and discontinue therapy in case of exacerbation of liver impairment
- Discontinue therapy if clinical or laboratory indicators are suggestive of worsening liver function or cholestasis
- Concurrent elevations of serum GGT and ALT may indicate overdose; continue to monitor laboratory parameters of liver function and consider restarting at a lower dose when the parameters return to baseline
- Discontinue treatment if liver function does not improve within 3 months of treatment initiation or if complete biliary obstruction develops
Supplemental Patient Information
- Advise patients that they will need to undergo laboratory testing periodically while on treatment to assess liver function
- Advise patients that cholic acid treatment may worsen liver impairment and that they should immediately report any symptoms associated with liver impairment to their health care provider
Pregnancy category: Not rated
- Inform patients about the pregnancy surveillance program that monitors pregnancy outcomes in women exposed to cholic acid during pregnancy
Breastfeeding: Safety Unknown. Manufacturer advises caution.
