Orphacol

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

Allergies

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Pediatric

Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of cholic acid in children 3 weeks of age and older.

Geriatric

Although appropriate studies on the relationship of age to the effects of cholic acid have not been performed in the geriatric population, no geriatric-specific problems have been documented to date.

Breast Feeding

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Interactions with Medicines

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. When you are taking this medicine, it is especially important that your healthcare professional know if you are taking any of the medicines listed below. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following medicines is usually not recommended, but may be required in some cases. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

• Cyclosporine

Using this medicine with any of the following medicines may cause an increased risk of certain side effects, but using both drugs may be the best treatment for you. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

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

Interactions with Food/Tobacco/Alcohol

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other Medical Problems

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

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

• Liver disease—Use with caution. May make this condition worse.

This section provides information on the proper use of a number of products that contain cholic acid. It may not be specific to Orphacol. Please read with care.

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.

It is very important that your doctor check your progress at regular visits. Your doctor will do blood and urine tests to make sure that cholic acid is working properly and to check for unwanted effects.

If your condition does not improve within 3 months, or if it become worse, check with your doctor.

Check with your doctor right away if you have pain or tenderness in the upper stomach, pale stools, dark urine, loss of appetite, nausea, vomiting, or yellow eyes or skin. These could be symptoms of a serious liver problem.

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

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

• 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
• stomach pain, continuing
• unpleasant breath odor
• unusual tiredness or weakness
• vomiting
• vomiting of blood
• yellow eyes or skin

• 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 occur 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 about ways 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:

• Burning, numbness, tingling, or painful sensations
• chest pain
• difficulty with 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.