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: 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 glycerol phenylbutyrate, your dose will not be the same. Follow your doctor's dosing instructions very carefully.
Glycerol phenylbutyrate doses are based on body surface area (height and weight). Your dose needs may change if you gain or lose weight or if you are still growing.
You may need to follow a special diet while using glycerol phenlbutyrate. Follow all instructions of your doctor or dietitian. Learn about the foods to eat or avoid to help control your condition.
A buildup of ammonia in the blood can quickly cause brain injury or death. You will need frequent 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.