Orphacol (Oral)

Cholic acid is a bile acid that helps treat patients with bile acid synthesis disorders caused by defects in a single enzyme. This medicine is also used for patients with peroxisomal disorders (including Zellweger spectrum disorders) who show symptoms of liver disease, steatorrhea, or have trouble absorbing fat-soluble vitamins.

This medicine is available only with your doctor's prescription.

It is very important that your doctor checks your progress at regular visits. Your doctor will perform blood and urine tests to ensure that cholic acid is working properly and to look for any unwanted effects.

If your condition does not improve within 3 months, or if it worsens, contact your doctor.

Contact your doctor immediately if you experience pain or tenderness in the upper stomach, pale stools, dark urine, loss of appetite, nausea, vomiting, or yellowing of the eyes or skin. These could be signs of a serious liver problem.

In deciding to use a medicine, you and your doctor need to consider the risks of taking it against the benefits it may provide. For this medicine, keep the following points in mind:

Allergies

Inform your doctor if you have had any unusual or allergic reactions to this medicine or any other medicines. Also, let your healthcare professional know about any other allergies you have, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients closely.

Pediatric

Studies conducted so far have not shown any specific problems in children aged 3 weeks and older that would limit the use of cholic acid.

Geriatric

While studies on the effects of cholic acid in older adults have not been done, there have been no specific problems documented in this age group so far.

Breast Feeding

There are no sufficient studies in women to determine the risk to infants when using this medication while breastfeeding. Consider the potential benefits and risks before taking this medication during breastfeeding.

Interactions with Medicines

Some medicines should not be used together at all, while others may be used together even if an interaction could happen. In these cases, your doctor may need to adjust the dose or take other precautions. When taking this medicine, it is especially important that your healthcare professional knows if you are taking any of the medicines listed below. The following interactions are significant and may not include all possible interactions.

Using this medicine with any of the following medicines is generally not recommended, but may be necessary in some situations. If both medicines are prescribed, your doctor may adjust the dose or frequency of one or both.

• Cyclosporine

Using this medicine with any of the following medicines may increase the risk of certain side effects, but using both may be the best treatment option for you. If both are prescribed, your doctor may change the dose or frequency of one or both.

• Aluminum Carbonate, Basic
• Aluminum Hydroxide
• Aluminum Phosphate
• Dihydroxyaluminum Aminoacetate
• Dihydroxyaluminum Sodium Carbonate
• Magaldrate

Interactions with Food/Tobacco/Alcohol

Some medicines should not be taken around mealtimes or with specific foods due to possible interactions. Using alcohol or tobacco with certain medicines can also lead to interactions. Talk to your healthcare professional about using your medicine with food, alcohol, or tobacco.

Other Medical Problems

Having other medical conditions may affect how you can use this medicine. Be sure to inform your doctor if you have any other medical issues, especially:

• Familial hypertriglyceridemia, or a history of—Patients with this condition may require a higher dose of cholic acid.

• Liver disease—Use with caution, as it may worsen this condition.

Along with its necessary effects, a medicine can cause some unwanted effects. Not all of these side effects may happen, but if they do, they might require medical attention.

Check with your doctor right away if any of the following side effects occur:

More common

• Abdominal or stomach pain
• chills
• clay-colored stools
• dark urine
• diarrhea
• dizziness
• fever
• general feeling of tiredness or weakness
• headache
• itching or rash
• light-colored stools
• loss of appetite
• nausea
• continuing stomach pain
• unpleasant breath odor
• unusual tiredness or weakness
• vomiting
• vomiting of blood
• yellow eyes or skin

Less common

• Bladder pain
• bloody or cloudy urine
• clay-colored stools
• dark urine
• difficult, burning, or painful urination
• frequent urge to urinate
• lower back or side pain
• unpleasant breath odor

Some side effects may happen that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you how to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

Less common

• Burning, numbness, tingling, or painful sensations
• chest pain
• difficulty swallowing
• general feeling of discomfort or illness
• heartburn
• pain or burning in the throat
• skin lesions
• sores, ulcers, or white spots on the lips or tongue or inside the mouth
• unsteadiness or awkwardness
• weakness in the arms, hands, legs, or feet

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

In the U.S.

• Cholbam
• Orphacol

Available Dosage Forms:

• Capsule

Therapeutic Class: Gastrointestinal Agent

Take this medicine exactly as directed by your doctor. Do not take more of it, do not take it more often, and do not take it for a longer time than your doctor ordered. To do so may increase the chance of side effects.

Swallow the capsule whole. Do not crush, break, or chew it. Take this medicine with food.

If you cannot swallow the capsules, you may open it and mix the contents of the capsule with 1 to 2 tablespoons of infant formula or expressed breast milk, or soft foods (eg, mashed potatoes or apple puree). This mixture must be swallowed immediately.

If you are also taking cholestyramine, colesevelam, colestipol, or aluminum-containing antacids, take cholic acid at least 1 hour before or 4 to 6 hours after these medicines.

Dosing

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

• For oral dosage form (capsules):For bile acid synthesis disorders and peroxisomal disorders (including Zellweger spectrum disorders): Adults and children 3 weeks of age and older—Dose is based on body weight and must be determined by your doctor. The dose is usually 10 to 15 milligrams (mg) per kilogram (kg) of body weight once a day, or divided in two doses. Children younger than 3 weeks of age—Use and dose must be determined by your doctor.

Missed Dose

If you miss a dose of this medicine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

Storage

Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.