Glycerol Phenylbutyrate

Glycerol phenylbutyrate is used to manage urea cycle disorders (UCDs; an inherited condition that affects the ability to metabolize protein). Glycerol phenylbutyrate is in a class of medications called nitrogen binding agents. It works by lowering ammonia levels in the body.

Glycerol phenylbutyrate comes as a solution (liquid) to take by mouth. Take with food or formula as directed by your doctor. Give to breastfeeding infants just before feeding as directed by your doctor. Take glycerol phenylbutyrate at around the same time(s) every day.

Talk to your doctor or pharmacist about giving glycerol phenylbutyrate if you have a nasogastric or gastrostomy tube and can not swallow.

Take glycerol phenylbutyrate exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.

To use the bottle and oral syringe follow these steps:

1. Use a new bottle cap adapter with each new bottle of glycerol phenylbutyrate solution.
2. Use a new, dry oral syringe for each dose. Do not reuse oral syringes; discard after each dose.
3. Tightly close the bottle cap adapter after each use. Do not rinse the bottle cap adapter.
4. Discard the bottle and any remaining solution 28 days after opening.
5. If the solution becomes cloudy, do not use the remaining solution. Return the bottle and the contents to your pharmacy for disposal.

Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.

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

Before taking glycerol phenylbutyrate,

• tell your doctor or pharmacist if you are allergic to this medication, any part of this medication, or any other medications, foods or substances. Tell your doctor or pharmacist about the allergy and what symptoms you had.
• tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take while taking glycerol phenylbutyrate. Your doctor may need to change the doses of your medications or monitor you carefully for side effects.
• tell your doctor if you have or have ever had pancreas, bowel, kidney or liver disease.
• tell your doctor if you are pregnant or plan to become pregnant. If you become pregnant while taking glycerol phenylbutyrate, call your doctor.
• tell your doctor if you are breastfeeding. Do not breastfeed while taking glycerol phenylbutyrate.

Unless your doctor tells you otherwise, continue your normal diet.

Take the missed dose as soon as you remember it. However, if it is almost time for the next dose, skip the missed dose and continue your regular dosing schedule. Do not take a double dose to make up for a missed one. If you miss more than one dose and are not feeling well, call your doctor right away.

Glycerol phenylbutyrate may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

• diarrhea, gas
• headache

Some side effects can be serious. If you experience any of these symptoms call your doctor immediately or get emergency medical treatment:

• wheezing, shortness of breath, coughing, dizziness, flushing, nausea, rash
• nausea, vomiting; sleepiness or tiredness; lightheadedness; change in taste; problems with hearing; confusion or problems with memory, worsening of numbness, tingling, or burning in your hands or feet; headache

Glycerol phenylbutyrate may cause other side effects. Call your doctor if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (https://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

Keep this medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom).

Keep all medication out of sight and reach of children as many containers are not child-resistant. Always lock safety caps. Place the medication in a safe location – one that is up and away and out of their sight and reach. https://www.upandaway.org

Dispose of unneeded medications in a way so that pets, children, and other people cannot take them. Do not flush this medication down the toilet. Use a medicine take-back program. Talk to your pharmacist about take-back programs in your community. Visit the FDA's Safe Disposal of Medicines website https://goo.gl/c4Rm4p for more information.

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at https://www.poisonhelp.org/help. If the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services at 911.

Keep all appointments with your doctor and the laboratory. Your doctor may order certain lab tests to check your body's response to glycerol phenylbutyrate.

Do not let anyone else take your medication. Ask your pharmacist any questions you have about refilling your prescription.

Keep a written list of all of the prescription and nonprescription (over-the-counter) medicines, vitamins, minerals, and dietary supplements you are taking. Bring this list with you each time you visit a doctor or if you are admitted to the hospital. You should carry the list with you in case of emergencies.

• Ravicti^®