image image image

High ammonia levels and risks to your health

Living with a urea cycle disorder (UCD) means being at risk of having too much ammonia in your bloodstream. And did you know that ammonia levels can go up and down throughout the day and reach dangerous levels without you even realizing it? If ammonia levels only go up a little bit over time, permanent damage to your body may occur.

In 3 clinical trials (ranging from 7 days to 4 weeks), RAVICTI® (glycerol phenylbutyrate) was shown to reduce elevated ammonia levels day and night

The short-term studies compared ammonia levels in people with UCDs receiving RAVICTI or sodium phenylbutyrate. Read the Important Safety Information and Full Prescribing Information for RAVICTI and BUPHENYL® (sodium phenylbutyrate) Tablets or Powder below.

Average blood ammonia levels were within the normal range for 24-hour control.

No hyperammonemic crises were reported with RAVICTI in these short-term studies in adults and children with UCDs.

Average blood ammonia levels were within the normal range for 12-month control.

In 2 long-term studies conducted by doctors, people aged 2 years or older who received RAVICTI had their ammonia levels controlled for a year.

5 out of 26 children aged 6 to 17 years (19%) in the study reported a total of 5 hyperammonemic crises.

7 out of 51 adults (14%) in the study reported a total of 10 hyperammonemic crises.

"RAVICTI is easy. It’s not cumbersome. It’s not inconvenient. It fits my lifestyle."

- Patricia, patient and caregiver

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.

important safety information

buphenyl®(sodium phenylbutyrate) tablets and powder indications and usage

Approved Uses for BUPHENYL®

BUPHENYL (sodium phenylbutyrate) Tablets is a prescription medicine that can be taken by mouth and BUPHENYL (sodium phenylbutyrate) Powder is a prescription medicine that can be taken by mouth or feeding tube for the long-term management of high blood levels of ammonia (hyperammonemia) caused by a condition called a urea cycle disorder (UCD). BUPHENYL should be used if the UCD cannot be managed with a low-protein diet and dietary supplements alone.

BUPHENYL only treats high blood levels of ammonia in patients with the following enzyme deficiencies:

  • Carbamylphosphate synthetase (CPS)
  • Ornithine transcarbamylase (OTC)
  • Argininosuccinic acid synthetase (AS)

BUPHENYL can be used in infants up to 28 days old who have a complete enzyme deficiency (an enzyme in the urea cycle that does not work at all). It can also be used in people 1 month of age and up who have a partial enzyme deficiency (an enzyme in the urea cycle that only works partially) and have a history of brain damage from high blood levels of ammonia (hyperammonemia). It is important to have a healthcare provider diagnose this condition and prescribe a medication as early as possible to improve chance of survival.

BUPHENYL must be used along with a low-protein diet and, in some cases, dietary supplements.

Any episode related to acute hyperammonemia should be treated as a life-threatening emergency.

Important Safety Information (ISI)

Do not take BUPHENYL if you are allergic to phenylbutyrate, or for the treatment of acute hyperammonemia in people with UCDs.

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

Talk to your doctor before taking BUPHENYL if you have heart failure or decreased kidney function, which may lead to retention of the sodium content of BUPHENYL with potentially serious consequences such as worsening heart failure, high blood pressure, and swelling. You and your doctor should decide if you will take BUPHENYL if you have these medical conditions. Do not take BUPHENYL if you have liver or kidney problems, have any other medical conditions, if your child is 20kg or less, or if you are planning to become pregnant or breastfeed, as it is unknown if BUPHENYL will harm your unborn baby or will pass into your breastmilk.

The most common side effects of BUPHENYL include absent or irregular periods in women, decreased appetite, body odor, and bad taste.

The most common side effects of BUPHENYL® seen in a laboratory setting include changes to blood pH and electrolyte levels (such as chloride and phosphate), low protein levels in the blood, high levels of certain bone and liver enzymes (such as alkaline phosphatase and transaminases), and decreased red and white blood cell and platelet count.

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 intended to replace discussions with your doctor. For additional information about BUPHENYL®, please consult the Full Prescribing Information and the Information for the Patient/Caregiver and talk to your doctor. BUPHENYL® is available by prescription only.

Please see the full Prescribing Information and Patient Package Insert for BUPHENYL.