Treating Urea Cycle Disorders

Your UCD care team

People with urea cycle disorders (UCDs) usually work with a healthcare team that has several members. You may see a primary care doctor as well as a team of specialists at a metabolic center. These specialists may include a metabolic geneticist, a dietitian, nurses, a genetic counselor, a nurse practitioner, and a social worker.

Metabolic geneticists are specialists who treat disorders like UCDs. The metabolic geneticist, or one of his or her team members, will do ammonia level tests, create a UCD management plan, and make any changes to treatment. Your metabolic geneticist will help coordinate with other specialists, and can be the main point of contact for your primary care doctor. Your care team will also help with things like understanding genetics, UCDs, and family planning.

Managing your UCD

The dietary goal of managing a UCD is to control the amount of ammonia that is produced as the body breaks down protein. One way to produce less ammonia is to eat less protein. When living with a UCD, it's important to understand your protein limitations, especially since the right amount of protein is different for everyone. Limiting it too much may cause your body harm, too, because we need protein to grow and for other bodily functions. A dietitian will help you manage your low-protein diet. He or she will help you make a plan that works for your personal needs and restrictions.

Before making changes to your diet, you should speak with a dietitian to decide the right foods and portion sizes for you. A dietitian can help plan low-protein meals that meet your or your child's needs, and change them as your child grows. For recipes to help balance your daily protein needs and make it easier to prepare meals, sign up for our free low-protein cookbook.

Your care team will work to develop a customized treatment plan that's right for you or your child. This can include:

Low-protein diet icon
  • A low-protein, high-calorie diet: Protein in the diet may be lowered by limiting or not eating protein-rich foods like meat, nuts, eggs, and cheese. There are also some fruits, vegetables, and grains that may have too much protein for some UCD patients. You should speak with your dietitian or medical team to develop a diet that is best for you. Special low-protein foods in moderation can also help keep protein within your limits. These can include low-protein pastas and breads, and special formulas. Your dietitian can teach you how to weigh or measure foods to make sure you eat the right amount of protein.
Amino acid supplement icon
  • Amino acid supplements: Supplements like citrulline and arginine may be added to your diet to help your body make protein for growth and tissue repair.
RAVICTI liquid syringe icon
  • Medicines: Sometimes, a low-protein diet and supplements aren't enough to keep ammonia levels low. In these cases, medicines called nitrogen scavengers, sometimes called ammonia scavengers, help remove ammonia from the bloodstream, and may be added to the UCD treatment plan. These medicines carry ammonia out in the urine. RAVICTI® (glycerol phenylbutyrate) is one nitrogen scavenger treatment option. Learn more about RAVICTI. You should not take RAVICTI if you are allergic to phenylbutyrate.
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Managing Ammonia Levels Caused by UCDs

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Important Safety Information And Approved Uses For RAVICTI® (glycerol phenylbutyrate) Oral Liquid
Who Should Not Take RAVICTI: Children... Children younger than 2 months should not take RAVICTI because they may not be able to digest it. Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest...

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Important Safety Information And Approved Uses For RAVICTI® (Glycerol Phenylbutyrate) Oral Liquid

Who Should Not Take RAVICTI:

Children younger than 2 months should not take RAVICTI because they may not be able to digest it.

Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have symptoms of an allergic reaction, such as wheezing, shortness of breath, cough, low blood pressure, flushing, nausea, or rash while taking RAVICTI.

RAVICTI May Cause Serious Side Effects:

The breakdown of RAVICTI produces the byproduct phenylacetate, which may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI: sleepiness; lightheadedness; change in taste; problems with hearing; confusion; problems with memory; worsening of numbness, tingling, or burning in your hands or feet; headache, tiredness; nausea; or vomiting.

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, and dizziness.

The most common side effects of RAVICTI in children ages 2 to 17 years include stomach pain, nausea, vomiting, diarrhea, decreased appetite, and headache.

The most common side effects of RAVICTI in children ages 2 months to younger than 2 years include decreased level of a type of white blood cell, vomiting, diarrhea, fever, decreased appetite, cough, nasal congestion, runny nose, and rash.

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. Breastfeeding is not recommended during treatment with RAVICTI. Talk to your doctor about the best way to feed your baby if you take RAVICTI.

Approved Uses and Important Safety Information for RAVICTI® (glycerol phenylbutyrate) Oral Liquid

RAVICTI is a prescription medicine for people with UCDs who are at least 2 months of age and cannot manage their UCD with diet and supplements alone. RAVICTI must be used with a low-protein diet. RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crisis) or for NAGS deficiency.

Indication for RAVICTI Oral Liquid

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

Limitations Of Use

RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crises) in people with UCDs as they may require rapidly acting medication.

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 for children younger than 2 months.

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 1-855-823-2595 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 healthcare provider or pharmacist. The FDA-approved product labeling, including the Medication Guide, can be found at RAVICTI.com.

Important Safety Information And Approved Uses For RAVICTI® (Glycerol Phenylbutyrate) Oral Liquid

Who Should Not Take RAVICTI:

Children younger than 2 months should not take RAVICTI because they may not be able to digest it.

Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have symptoms of an allergic reaction, such as wheezing, shortness of breath, cough, low blood pressure, flushing, nausea, or rash while taking RAVICTI.

RAVICTI May Cause Serious Side Effects:

The breakdown of RAVICTI produces the byproduct phenylacetate, which may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI: sleepiness; lightheadedness; change in taste; problems with hearing; confusion; problems with memory; worsening of numbness, tingling, or burning in your hands or feet; headache, tiredness; nausea; or vomiting.

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, and dizziness.

The most common side effects of RAVICTI in children ages 2 to 17 years include stomach pain, nausea, vomiting, diarrhea, decreased appetite, and headache.

The most common side effects of RAVICTI in children ages 2 months to younger than 2 years include decreased level of a type of white blood cell, vomiting, diarrhea, fever, decreased appetite, cough, nasal congestion, runny nose, and rash.

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. Breastfeeding is not recommended during treatment with RAVICTI. Talk to your doctor about the best way to feed your baby if you take RAVICTI.

Approved Uses and Important Safety Information for RAVICTI® (glycerol phenylbutyrate) Oral Liquid

RAVICTI is a prescription medicine for people with UCDs who are at least 2 months of age and cannot manage their UCD with diet and supplements alone. RAVICTI must be used with a low-protein diet. RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crisis) or for NAGS deficiency.

Indication for RAVICTI Oral Liquid

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

Limitations Of Use

RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crises) in people with UCDs as they may require rapidly acting medication.

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 for children younger than 2 months.

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 1-855-823-2595 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 healthcare provider or pharmacist. The FDA-approved product labeling, including the Medication Guide, can be found at RAVICTI.com.