Glycerol phenylbutyrate
Generic name: glycerol phenylbutyrate [ GLIS-er-ol-FEN-il-BUE-ti-rate ]
Brand name: Ravicti
Dosage form: oral liquid (1.1 g/mL)
Drug class:Urea cycle disorder agents
What is glycerol phenylbutyrate?
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 adults and children with urea cycle disorder. 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.
Warnings
This medicine can affect your nervous system. Call your doctor right away if you have: confusion, headaches, memory problems, hearing problems, vomiting, an altered sense of taste, numbness or tingling in your hands or feet, or if you feel unusually sleepy or light-headed.
Before taking this medicine
You should not use glycerol phenylbutyrate if you are allergic to it.
Tell your doctor if you have ever had:
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 glycerol phenylbutyrate 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.
You should not breastfeed while using this medicine.
How should I take glycerol phenylbutyrate?
Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.
Glycerol phenylbutyrate is usually taken 3 times per day. Take with food or infant formula. If you are breastfeeding a baby who is taking this medicine, give each dose right before nursing.
Measure liquid medicine carefully. Use the dosing syringe provided, or use a medicine dose-measuring device (not a kitchen spoon).
Glycerol phenylbutyrate may be given through a nasogastric (NG) or gastric feeding tube as follows: Measu...
