cholic acid (Rx)

Brand and Other Names:Cholbam
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

capsule

  • 50mg
  • 250mg

Bile Acid Synthesis Disorders

Indicated for bile acid synthesis disorders due to single-enzyme defects (SEDs)

10-15 mg/kg/day PO qDay or divided BID

Peroxisomal Disorders

Indicated for adjunctive treatment of peroxisomal disorders (PDs), including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea, or complications from decreased fat-soluble vitamin absorption

10-15 mg/kg/day PO qDay or divided BID

Dosage Modifications

Familial hypertriglyceridemia

  • Patients with newly diagnosed or a family history of familial hypertriglyceridemia may have poor absorption of cholic acid from the intestine and require a 10% increase in the recommended dosage to account for losses due to malabsorption
  • Recommended dosage range: 11-17 mg/kg/day PO qDay or divided BID
  • Determine adequacy of the dosage regimen by monitoring clinical response, including steatorrhea, and laboratory values, including transaminases, bilirubin, and PT/INR

Dosing Considerations

Safety and effectiveness on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs have not been established

Monitoring

  • Monitor AST, ALT, GGT, alkaline phosphatase, bilirubin, and INR every month for the first 3 months; every 3 months for the next 9 months; every 6 months during the subsequent 3 yr; and annually thereafter
  • Monitor more frequently during periods of rapid growth, concomitant disease, or pregnancy
  • Administer the lowest dose that effectively maintains liver function
  • Discontinue treatment if liver function does not improve within 3 months of the start of treatment or complete biliary obstruction develops
  • Discontinue treatment at any time if there are persistent clinical or laboratory indicators of worsening liver function or cholestasis
  • Concurrent elevations of 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
  • Serum or urinary bile acid level using mass spectrometry is used in the diagnosis of bile acid synthesis disorders due to SEDs and PDs, including Zellweger spectrum disorders
  • The utility of bile acid measurements in monitoring the clinical course of patients and in decisions regarding dose adjustment has not been demonstrated

Dosage Forms & Strengths

capsule

  • 50mg
  • 250mg

Bile Acid Synthesis Disorders

Indicated for bile acid synthesis disorders due to single-enzyme defects (SEDs)

<3 weeks: Safety and efficacy not established

≥3 weeks: 10-15 mg/kg/day PO qDay or divided BID

Peroxisomal Disorders

Indicated for adjunctive treatment of peroxisomal disorders (PDs), including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea, or complications from decreased fat-soluble vitamin absorption

<3 weeks: Safety and efficacy not established

≥3 weeks: 10-15 mg/kg/day PO qDay or divided BID

Dosage Modifications

Familial hypertriglyceridemia

  • Patients with newly diagnosed or a family history of familial hypertriglyceridemia may have poor absorption of cholic acid from the intestine and require a 10% increase in the recommended dosage to account for losses due to malabsorption
  • Recommended dosage range: 11-17 mg/kg/day PO qDay or divided BID
  • Determine adequacy of the dosage regimen by monitoring clinical response, including steatorrhea, and laboratory values, including transaminases, bilirubin, and PT/INR

Dosing Considerations

Safety and effectiveness on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs have not been established

Monitoring

  • Monitor AST, ALT, GGT, alkaline phosphatase, bilirubin, and INR every month for the first 3 months; every 3 months for the next 9 months; every 6 months during the subsequent 3 yr; and annually thereafter
  • Monitor more frequently during periods of rapid growth, concomitant disease, or pregnancy
  • Administer the lowest dose that effectively maintains liver function
  • Discontinue treatment if liver function does not improve within 3 months of the start of treatment or complete biliary obstruction develops
  • Discontinue treatment at any time if there are persistent clinical or laboratory indicators of worsening liver function or cholestasis
  • Concurrent elevations of 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
  • Serum or urinary bile acid level using mass spectrometry is used in the diagnosis of bile acid synthesis disorders due to SEDs and PDs, including Zellweger spectrum disorders
  • The utility of bile acid measurements in monitoring the clinical course of patients and in decisions regarding dose adjustment has not been demonstrated
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Interactions

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            Adverse Effects

            1-10%

            Diarrhea (2%)

            Reflux esophagitis (1%)

            Malaise (1%)

            Jaundice (1%)

            Skin lesion (1%)

            Nausea (1%)

            Abdominal pain (1%)

            Intestinal polyp (1%)

            Urinary tract infection (1%)

            Peripheral neuropathy (1%)

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            Warnings

            Contraindications

            None

            Cautions

            Monitor liver function and discontinue at any time there are clinical or laboratory indicators of worsening liver function or cholestasis

            Concurrent elevations of GGT and ALT may indicate overdose; discontinue (see Dosage Modifications)

            Evidence of liver impairment was present before treatment with cholic acid in ~86% of patients with bile acid synthesis disorders due to SEDs and in ~50% of patients with PDs, including Zellweger spectrum disorders

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            Pregnancy & Lactation

            Pregnancy

            No studies in pregnant women or animal reproduction studies have been conducted

            Limited published case reports discuss pregnancies in women taking cholic acid for 3beta-HSD deficiency, resulting in healthy infants

            COCOA Registry (ChOlbam: Child and mOther's heAlth)

            • Women who become pregnant during treatment are encouraged to enroll
            • Patients or their healthcare provider should call 1-844-20C-OCOA or 1-844-202-6262

            Lactation

            Endogenous cholic acid is present in human milk

            Clinical lactation studies have not been conducted to assess the presence of Cholbam in human milk, the effects on the breastfed infant, or the effects on milk production

            There are no animal lactation data and no data from case reports available in the published literature

            The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for the drug and any potential adverse effects on the breastfed infant from the drug or from the underlying maternal condition

            Pregnancy Categories

            A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

            B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

            C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

            D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

            X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

            NA:Information not available.

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            Pharmacology

            Mechanism of Action

            Cholic acid is a primary bile acid synthesized from cholesterol in the liver

            In bile acid synthesis disorders due to SEDs in the biosynthetic pathway and in PDs, including Zellweger spectrum disorders, deficiency of primary bile acids leads to unregulated accumulation of intermediate bile acids and cholestasis

            Bile acids facilitate fat digestion and absorption by forming mixed micelles, and they facilitate absorption of fat-soluble vitamins in the intestine

            Endogenous bile acids, including cholic acid, enhance bile flow and provide the physiologic feedback inhibition of bile acid synthesis

            The mechanism of action of cholic acid has not been fully established; however, it is known that cholic acid and its conjugates are endogenous ligands of the nuclear receptor, farnesoid X receptor (FXR)

            FXR regulates enzymes and transporters that are involved in bile acid synthesis and in the enterohepatic circulation to maintain bile acid homeostasis under normal physiologic conditions

            Absorption

            Cholic acid is absorbed by passive diffusion along the length of the GI tract

            Once absorbed, cholic acid enters into the body’s bile acid pool and undergoes enterohepatic circulation mainly in conjugated forms

            Metabolism

            In the liver, cholic acid is conjugated with glycine or taurine by bile acid-CoA synthetase and bile acid-CoA: amino acid N-acetyltransferase

            Conjugated cholic acid is actively secreted into bile mainly by the bile salt efflux pump (BSEP) and then released into the small intestine, along with other components of bile

            Conjugated cholic acid is mostly reabsorbed in the ileum mainly by the apical-sodium-dependent-bile acid transporter, passed back to the liver by transporters including sodium-taurocholate cotransporting polypeptide and organic anion transport protein, and enters another cycle of enterohepatic circulation

            Elimination

            Any conjugated cholic acid not absorbed in the ileum passes into the colon and may be reabsorbed in the colon or excreted in the feces

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            Administration

            Instructions

            Take with food

            Take at least 1 hr before or 4-6 hr (or at as great an interval as possible) after a bile acid binding resin (eg, cholestyramine) or aluminum-based antacid

            Do not crush or chew the capsules

            Unable to Swallow Capsules

            Capsules can be opened and the contents mixed with either infant formula or expressed breast milk (for younger children), or soft food such as mashed potatoes or apple puree (for older children and adults) in order to mask any unpleasant taste

            Mix with soft food or liquid

            • Hold the capsule over the prepared liquid/food, gently twist open, and allow the contents to fall into the liquid/food
            • Mix the entire capsule contents with 1-2 tablespoons (ie, 15-30 mL) of infant formula, expressed breast milk, or soft food (eg, mashed potatoes, applesauce)
            • Stir for 30 seconds
            • The capsule contents will remain as fine granules in the milk or food and will not dissolve
            • Administer mixture immediately

            Storage

            Store at room temperature 20-25°C (69-77°F)

            Excursions permitted from 15-30°C (59-86°F)

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            Formulary

            FormularyPatient Discounts

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            The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

            Tier Description
            1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
            2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
            3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
            4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
            NC NOT COVERED – Drugs that are not covered by the plan.
            Code Definition
            PA Prior Authorization
            Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
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            Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
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            Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
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            Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.