Version May 2024
Bile acid synthesis disorders: Oral: 10 to 15 mg/kg (once daily or in 2 divided doses); administer 11 to 17 mg/kg (once daily or in 2 divided doses) in patients with concomitant familial hypertriglyceridemia.
Peroxisomal disorders: Oral: 10 to 15 mg/kg (once daily or in 2 divided doses); administer 11 to 17 mg/kg (once daily or in 2 divided doses) in patients with concomitant familial hypertriglyceridemia.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling. Discontinue if hepatic function does not improve within 3 months of starting treatment, if complete biliary obstruction develops, or if there are persistent clinical or laboratory indicators of worsening hepatic function or cholestasis; continue to monitor hepatic function and consider restarting a lower dose when parameters return to baseline.
(For additional information see "Cholic acid: Pediatric drug information")
Bile acid synthesis disorders or peroxisomal disorders (eg, Zellweger spectrum disorders): Infants, Children, and Adolescents: Oral: 10 to 15 mg/kg/day once daily or in divided doses twice daily; round dose to nearest available capsule sizes (50 mg or 250 mg). Allow 3 months of treatment to assess therapeutic effect (eg, clinical response parameters: LFTs, bilirubin, PT/INR, steatorrhea); use the lowest dose that effectively maintains liver function; discontinue if hepatic function does not improve within 3 months of starting treatment (Ref).
Dosing adjustment for familial hypertriglyceridemia (including newly diagnosed or family history of): Infants, Children, and Adolescents: Oral: 11 to 17 mg/kg/day once daily or in divided doses twice daily; round dose to nearest available capsule sizes (50 mg or 250 mg). Note: Patients with hypertriglyceridemia have malabsorption of cholic acid requiring 10% higher doses.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer's labeling. During treatment, if complete biliary obstruction develops, or if there are persistent clinical or laboratory indicators of worsening hepatic function or cholestasis, discontinue treatment and continue to monitor hepatic function; consider restarting a lower dose when parameters return to baseline.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
>10%:
Gastrointestinal: Cholestasis (≤13%, including exacerbations)
Hepatic: Increased serum bilirubin (≤13%, including exacerbations), increased serum transaminases (≤13%, including exacerbations)
1% to 10%:
Dermatologic: Skin lesion (1%)
Gastrointestinal: Abdominal pain (1%), diarrhea (2%), intestinal polyps (1%), nausea (1%), reflux esophagitis (1%)
Genitourinary: Urinary tract infection (1%)
Hepatic: Jaundice (1%)
Nervous system: Malaise (1%), peripheral neuropathy (1%)
Postmarketing:
Dermatologic: Skin rash
Gastrointestinal: Abdominal distension, cholelithiasis (3β-HSD deficient patient), constipation, gastrointestinal distress, vomiting
Hepatic: Severe hepatotoxicity (in patients with cirrhosis)
Miscellaneous: Fever
There are no contraindications listed in the manufacturer's labeling.
Disease-related concerns:
• Hepatic impairment: Monitor liver function tests (eg, AST, ALT, gamma glutamyltransferase [GGT], alkaline phosphatase, bilirubin and INR) monthly for the first 3 months. Discontinue if hepatic function does not improve within 3 months of starting treatment, if complete biliary obstruction develops, or if there are persistent clinical or laboratory indicators of worsening hepatic function or cholestasis; continue to monitor hepatic function and consider restarting a lower dose when parameters return to baseline. Concurrent elevations of GGT and ALT may indicate cholic acid overdose. Cases of severe hepatotoxicity have been reported in patients with cirrhosis.
Other warnings/precautions:
• Experienced physician: Treatment should be initiated and monitored by an experienced hepatologist or pediatric gastroenterologist.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Capsule, Oral:
Cholbam: 50 mg, 250 mg
No
Capsules (Cholbam Oral)
50 mg (per each): $350.85
250 mg (per each): $1,055.76
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Cholbam is only available through an exclusive pharmacy provider, Dohmen Life Science Services, Inc. For additional information, call 844.CHOLBAM (844.246.5226) or visit https://www.cholbam.com/support/.
Oral: Administer (once daily or in 2 divided doses) with food; do not crush or chew capsules. Take at least 1 hour before or 4 to 6 hours after bile acid binding resin or aluminum-based antacid.
For patients unable to swallow capsules, contents may be mixed with 15 to 30 mL of infant formula, expressed breast milk, or soft food such as mashed potatoes or apple puree. Stir for 30 seconds and administer immediately. Capsule contents will remain as fine granules in the milk or food and will not dissolve.
Oral: Administer with food at least 1 hour before or 4 to 6 hours (or at as great an interval as possible) after bile acid binding resin or aluminum-based antacid. Do not crush or chew capsules.
For patients unable to swallow capsules, may mix the entire capsule contents with 15 mL to 30 mL of infant formula, expressed breast milk, or soft food such as mashed potatoes or apple puree. Stir for 30 seconds. Capsule contents will not dissolve and remain as fine granules in the milk or food. Administer the mixture immediately.
Bile acid synthesis disorders: Treatment of bile acid synthesis disorders due to single enzyme defects.
Peroxisomal disorders: Adjunctive treatment of peroxisomal disorders, including Zellweger spectrum disorders, in patients who exhibit manifestations of liver disease, steatorrhea, or complications from decreased fat-soluble vitamin absorption.
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Aluminum Hydroxide: May decrease the absorption of Cholic Acid. Management: Administer cholic acid at least 1 hour before or 4 to 6 hours after administration of any aluminum hydroxide-containing products to minimize the potential for a significant interaction. Risk D: Consider therapy modification
Bile Acid Sequestrants: May decrease the absorption of Cholic Acid. Management: Administer cholic acid at least 1 hour before or 4 to 6 hours after administration of any bile acid-binding products to minimize the potential for a significant interaction. Risk D: Consider therapy modification
BSEP/ABCB11 Inhibitors: May decrease the excretion of Cholic Acid. Management: Avoid the use of bile salt efflux pump inhibitors with cholic acid. If such a combination cannot be avoided, monitor serum transaminases (eg, AST, ALT) and bilirubin closely. Risk D: Consider therapy modification
Sevelamer: May decrease the absorption of Cholic Acid. Management: Administer cholic acid at least 1 hour before or 4 to 6 hours after administration of any bile acid-binding products, such as sevelamer, to minimize the potential for a significant interaction. Risk D: Consider therapy modification
Sucralfate: May decrease the absorption of Cholic Acid. Management: Administer cholic acid at least 1 hour before or 4 to 6 hours after administration of aluminum-containing products such as sucralfate to minimize the potential for a significant interaction. Risk D: Consider therapy modification
Animal reproduction studies have not been conducted. Information related to the use of cholic acid during pregnancy is limited. Tests of hepatic function should be monitored closely.
A registry is available for women exposed to cholic acid during pregnancy. Patients or their health care provider should call 844-202-6262 to enroll.
Endogenous cholic acid is present in breast milk; it is not known if administration of cholic acid effects milk concentrations or production. According to the manufacturer, the decision to breast-feed during therapy should take into account the risk of exposure to the infant and the benefits of treatment to the mother.
Administer with food.
Monitor AST, ALT, GGT, ALP, bilirubin and INR every month for the first 3 months, every 3 months for the next 9 months, every 6 months during the next 3 years, and annually thereafter. Monitor more frequently during periods of rapid growth, concomitant disease, and pregnancy.
Cholic acid, a primary bile acid, enhances bile flow and provides the physiologic feedback inhibition of bile acid synthesis to maintain bile acid homeostasis.
Absorption: Absorbed by passive diffusion along the gastrointestinal tract. Patients with concomitant familial hypertriglyceridemia may have poor absorption.
Metabolism: Hepatic; conjugated by bile acid- enzymes. Conjugated cholic acid is secreted into bile. It is reabsorbed in the ileum, and enters another cycle of enterohepatic circulation.
Excretion: Feces
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