Buphenyl

Buphenyl is a prescription medication used to treat people with urea cycle disorders. Buphenyl helps to prevent ammonia from accumulating in the blood by helping the body eliminate substances that produce ammonia.  

This medication is available in tablet form. It is also available in powder to be administered by mouth, via a nasogastric tube, or via a gastrostomy tube.

It is taken in equally divided doses with each meal or feeding (such as three to six times a day). The tablet is swallowed whole and the powder is mixed with food (solid or liquid) or can be dissolved in water. 

Common side effects include bad taste or changes in menstrual cycle. 

Buphenyl is a prescription medication used to treat people with urea cycle disorders. People with these disorders lack certain liver enzymes which are responsible for eliminating ammonia from the body. Buphenyl helps to prevent ammonia from accumulating in the blood by helping the body eliminate substances that produce ammonia.  

 This medicine is given along with a prescribed diet that is low in protein.

This medication may be prescribed for other uses. Ask your doctor or pharmacist for more information.

Serious side effects have been reported with Buphenyl. See the “Buphenyl Precautions” section.

Common side effects include:

• changes in menstrual cycle for female patients
• body odor 
• bad taste
• stomach pain

This is not a complete list of Buphenyl side effects. Ask your doctor or pharmacist for more information.

Tell your doctor if you have any side effect that bothers you or that does not go away.

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

Tell your doctor about all the medicines you take, including prescription and non-prescription medicines, vitamins, and herbal supplements. Especially tell your doctor if you take 

• Probenecid. This medication can affect the way in which Buphenyl is processed in your body. 
• Depakene (valproic acid). This medication may increase blood ammonia levels. 
• Haldol (haloperidol). This medication may increase blood ammonia levels. 
• Corticosteroids

This is not a complete list of Buphenyl drug interactions. Ask your doctor or pharmacist for more information.

Serious side effects have been reported with Buphenyl including neurotoxicity. Tell your doctor right away if you experience vomiting, nausea, headache, somnolence, confusion, or sleepiness. 

Buphenyl tablets are not indicated for to infants or children who weigh less than 20kg.

Do not take Buphenyl if you

• are allergic to Buphenyl or to any of its ingredients 
• to manage acute hyperammonemia ( sudden, severely high levels of ammonia), which is considered a medical emergency.

Medications can interact with certain foods. In some cases, this may be harmful and your doctor will advise you to avoid certain food items. In the case of Buphenyl, there are no specific food items that you should exclude when taking this medication.

However, it is important to follow a nutritionally appropriate diet prescribed by your doctor or nutritionist. Buphenyl will be combined with dietary protein restriction and, in some cases, essential amino acid supplementation.

Before taking this medication let your doctor know if you:

• are allergic to Buphenyl or any of ints ingredients
• have congestive heart failure
• have kidney disease or kidney failure
• have swelling (edema)
• have high blood pressure
• are on a low salt diet
• have liver disease
• are pregnant or plan to become pregnant
• are breastfeeding or plan to breastfeed

Tell you doctor about all the medicines you are taking including prescription, over the counter, herbal supplements and vitamins.

Tell your doctor if you are pregnant or plan to get pregnant.

The FDA categorizes medications based on safety for use during pregnancy. Five categories - A, B, C, D, and X, are used to classify the possible risks to an unborn baby when a medication is taken during pregnancy.

Buphenyl falls into category C. There are no well-controlled studies that have been done in pregnant women. Buphenyl should only be used if the possible benefit outweighs the possible risk the unborn baby.

It is not know if Buphenyl crosses into human milk. Because many medications can cross into human milk and because of the possibility of serious adverse reactions in nursing infants with the use of this medication, a choice should be made whether to stop nursing or stop the use of this medication. Your doctor and you will decide if the benefits outweigh the risk of using Buphenyl.

Take Buphenyl exactly as prescribed. Do not increase or decrease the dose unless the doctor tells you to.

Buphenyl is available as tablets and is taken with each meal or feeding (such as three to six times a day).

It is also available in a powder form which can be taken by mouth or administered by mouth, via a nasogastric tube, or via a gastrostomy tube. When using the powder form, it can be mixed with food (solid or liquid) or dissolved in water.

If Buphenyl is mixed with food (solid or liquid), then it should be administered immediately thereafter. If Buphenyl is dissolved in water, then it can be kept for up to one week if kept at room temperature or refrigerated. 

Shake reconstituted (mixed) Buphenyl before taking. 

Make sure to follow the diet prescribed by your doctor or nutritionist. 

If you miss a dose, take the missed dose as soon as possible that same day. 

The dose of Buphenyl is based on your body weight or body mass.

The usual total daily dose is 450-600mg/kg of body weight per day in patients weighing less than 20 kg, or 9.9–13.0 g/m2/day in larger patients. 

If you take too much Buphenyl, call your healthcare provider or local Poison Control Center, or seek emergency medical attention right away.

• Store Buphenyl at room temperature.
• Keep this and other medications out of the reach of children and pets.