Signs and symptoms of too much ammonia

Dangers of hyperammonemia

Ammonia doesn't leave your body as it should when you're living with a urea cycle disorder (UCD). When there is too much ammonia in the blood, it's called hyperammonemia (hi-per-am-oh-NEE-me-uh), which can be very dangerous. Signs and symptoms of hyperammonemia that you may experience regularly without even knowing it may include:

  • Confusion
  • Vomiting
  • Being more tired than usual
  • Not being hungry
  • Being irritable/uncooperative

Signs and symptoms of hyperammonemia could mean you're experiencing a hyperammonemic crisis, which is a medical emergency that can lead to coma or death. RAVICTI® (glycerol phenylbutyrate) is not used for the treatment of acute hyperammonemia in people with UCDs.

Even if you've never had a hyperammonemic crisis, it doesn't mean you are safe from the effects of high ammonia. Ammonia levels can go up and down throughout the day, and if they go up just a little, over time, long-term damage to the body or brain is possible.

*This table is for reference only. Symptoms can vary by age.

RAVICTI helps control ammonia levels

In clinical studies, people taking RAVICTI had their average blood ammonia levels:

  • Controlled at safe and normal levels for 24 hours
  • Stay within a normal range during the 12 months of treatment

Additionally:

  • 7 out of 51 adults (14%) in the study reported a total of 10 hyperammonemic crises
  • 5 out of 26 children aged 6 to 17 years of age (19%) in the study reported a total of 5 hyperammonemic crises

Important Safety information

Ravicti® (glycerol phenylbutyrate) oral liquid indications and usage

Approved Uses for RAVICTI® (glycerol phenylbutyrate) Oral Liquid

RAVICTI is a prescription medicine used in adults and children 2 years of age and older for long-term management of high blood levels of ammonia (hyperammonemia) caused by a condition called a urea cycle disorder (UCD). RAVICTI should only be used if the UCD cannot be managed with a low-protein diet and dietary supplements alone. RAVICTI must be used along with a low-protein diet and, in some cases, dietary supplements.

RAVICTI is not used for the treatment of acute hyperammonemia in people with UCDs.

It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.

It is not known if RAVICTI is safe and effective in children 2 months to less than 2 years of age.

Detailed Important Safety Information

Who should not take RAVICTI:

  • Children less than 2 months of age should not take RAVICTI because it may not be digested in babies less than 2 months of age.
  • Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you get wheezing, shortness of breath, cough, low blood pressure, flushing, nausea, or a rash while taking RAVICTI.

RAVICTI may cause serious side effects:

Phenylacetate, a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you experience any of these symptoms while taking RAVICTI: sleepiness, weakness, lightheadedness, change in taste, problems with hearing, confusion, problems with memory, worsening neuropathy (numbness, tingling, or burning in your hands or feet), or headache.

What are the possible side effects of RAVICTI?

The most common side effects of RAVICTI in adults include diarrhea, gas, headache, nausea, vomiting, tiredness, decreased appetite, high blood levels of ammonia, and dizziness.

The most common side effects of RAVICTI in children include upper abdomen (stomach) pain, nausea, vomiting, diarrhea, decreased appetite, high blood levels of ammonia, and headache.

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects.

Before you take RAVICTI:

Tell your doctor if you have liver or kidney problems, pancreas or bowel (intestine) problems, or any other medical conditions. Tell your doctor if you are pregnant or plan to become pregnant. It is not known if RAVICTI will harm your unborn baby. Tell your doctor if you are breastfeeding or plan to breastfeed. It is not known if RAVICTI passes into your breast milk. RAVICTI may harm your baby, so you and your doctor should decide if you will take RAVICTI or breastfeed.

Talk to your doctor about participating in a UCD registry. The purpose of this registry is to collect information about people with UCDs to improve care. For more information about the registry program, call 1-855-823-7878 or visit www.ucdregistry.com.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

This information is not comprehensive. To learn more, talk to your health care provider or pharmacist. For more information about RAVICTI, please see the full Prescribing Information (PDF) and Medication Guide (PDF) for RAVICTI.