Ravicti
Generic name:glycerol phenylbutyrate [ GLIS-er-ol-FEN-il-BUE-ti-rate ]
Drug class:Urea cycle disorder agents
What is Ravicti?
Ravicti binds with other substances in the liver and kidneys to help eliminate nitrogen from the body. Excess nitrogen can cause hyperammonemi, 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.
Ravicti helps prevent a build-up of ammonia in the blood in adults and children with urea cycle disorder. This medicine will not treat hyperammonemia.
Ravicti is usually given together with a low-protein diet and sometimes dietary supplements.
Warnings
Ravicti 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 Ravicti if you are allergic to glycerol phenylbutyrate.
To make sure Ravicti is safe for you, 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 Ravicti.
How should I take Ravicti?
Take Ravicti exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose.
Ravicti 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 Ravicti liquid carefully. Use the dosing syringe provided, or use a medicine dose-measuring device (not a kitchen spoon).
Ravicti may be given through a nasogastric (NG) or gastric feeding tube as follows: Measure 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. Flush the tube with 10 milliliters (mL) of water and allow the tube to drain. Follow this with 10 mL more of water to wash the contents down.
If you switch from sodium phenylbutyrate to Ravicti, your dose will not be the same. Follow your doctor's dosing instructions very carefully.
Glycerol phenylbutyrate doses are based on bod...