Glycerol phenylbutyrate binds with other substances in the liver and kidneys to help eliminate nitrogen from the body. Excess nitrogen can cause hyperammonemia (HYE-per-AM-moe-NEE-mee-a), a build-up of ammonia in the blood. Ammonia is very toxic when it circulates in blood and tissues and can cause permanent brain damage, coma, or death.
Glycerol phenylbutyrate helps prevent a build-up of ammonia in the blood in people with urea cycle disorder. glycerol phenylbutyrate is used in adults and children who are at least 2 months old. Glycerol phenylbutyrate will not treat hyperammonemia.
Glycerol phenylbutyrate is usually given together with a low-protein diet and sometimes dietary supplements.
Glycerol phenylbutyrate may also be used for purposes not listed in this medication guide.
You should not use glycerol phenylbutyrate if you are allergic to it.
Glycerol phenylbutyrate should not be given to a child under 2 months old. Do not give glycerol phenylbutyrate to a child younger than 2 years old without medical advice.
To make sure glycerol phenylbutyrate is safe for you, tell your doctor if you have:
- liver or kidney disease;
- a pancreas disorder;
- stomach or intestinal disorder,
- a condition called NAGS (N-acetylglutamate synthase) deficiency; or
- if you use other medicines.
It is not known whether this medicine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.
If you become pregnant, your name may be listed on a pregnancy registry. This is to track the outcome of the pregnancy and to evaluate any effects of glycerol phenylbutyrate on the baby.
It is not known whether glycerol phenylbutyrate passes into breast milk or if it could harm a nursing baby. You should not breast-feed while using this medicine.
Follow all directions on your prescription label. Your doctor may occasionally change your dose. Do not take this medicine in larger or smaller amounts or for longer than recommended.
Glycerol phenylbutyrate is usually taken 3 times per day. Take with food or infant formula.
Measure liquid medicine with the dosing syringe provided, or with a special dose-measuring spoon or medicine cup. If you do not have a dose-measuring device, ask your pharmacist for one.
If you are unable to swallow, you may take glycerol phenylbutyrate through a nasogastric (NG) or gastric feeding tube as follows: After measuring the correct dose in the oral syringe, attach it to the feeding tube and push the plunger down to empty the syringe into the tube. Then flush the tube with 2 teaspoons of water and allow it to drain. Follow this with 2 more teaspoons of water to wash the contents down.
If you switch from sodium phenylbutyrate to glycerol phenylbutyrate, your dose will not be the same. Follow your doctor’s dosing instructions very carefully.
Glycerol phenylbutyrate is only part of a complete program of treatment that may also include a special diet and other medications. It is very important to follow the diet plan created for you by your doctor or nutrition counselor. You should become very familiar with the list of foods you should eat or avoid to help control your condition.
To be sure this medicine is helping your condition and is not causing harmful effects, your blood will need to be tested often. A buildup of ammonia in the blood can quickly cause brain injury or death. Do not miss any follow up visits to your doctor for blood tests. Every person with a urea cycle disorder should remain under the care of a doctor.
Your name may be listed on a Urea Cycle Disorder registry. This is to collect information about people with these disorders and to evaluate the effects of treatment.
Store at room temperature away from moisture and heat.
Along with its needed effects, glycerol phenylbutyrate 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 while taking glycerol phenylbutyrate:
- Continuing nausea or vomiting
- increase in the frequency of seizures
- loss of appetite
- swelling of the face
- tiredness and weakness
Some side effects of glycerol phenylbutyrate 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:
- Bloated or full feeling
- excess air or gas in the stomach or intestines
- passing gas
- upper abdominal or stomach pain
- Acid or sour stomach
- stomach discomfort or upset
Keywords: Glycerol phenylbutyrate.