Treating Most Types of UCDs With RAVICTI

For some patients, diet and supplements alone are not enough to manage elevated ammonia levels caused by urea cycle disorders (UCDs). These patients may be prescribed a type of medicine called a nitrogen scavenger, sometimes called an ammonia scavenger, to keep their ammonia levels under control. Two such ammonia scavengers are RAVICTI® (glycerol phenylbutyrate) and BUPHENYL® (sodium phenylbutyrate). They both contain the same active ingredient—phenylbutyrate—and help remove ammonia from the body. But there are some differences. See the chart below to understand more.

RAVICTI is for patients at least 2 months of age whose UCD cannot be managed by diet and supplements alone. RAVICTI must be used with a low-protein diet and, in some cases, with dietary supplements. In the chart below, you can compare taking RAVICTI with taking BUPHENYL.

Along with a low-protein diet that may include supplements, RAVICTI has been proven to help manage ammonia levels for babies, toddlers, children, and adults with urea cycle disorders. RAVICTI should not be used in babies younger than 2 months because they may not be able to digest it.

RAVICTI is a liquid medicine that is easy to take. It is odorless and nearly tasteless. Watch a video to see how RAVICTI works.

RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crises) or N-acetylglutamate synthase (NAGS) deficiency. The breakdown of RAVICTI produces the byproduct phenylacetate, which may cause nervous system side effects.

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What are the differences between RAVICTI and BUPHENYL?

Dose

BUPHENYL

up to 40 tablets per day

RAVICTI

up to 17.5 mL per day

People switching from BUPHENYL to RAVICTI should get the dose of RAVICTI that has the same amount of the active ingredient (phenylbutyrate) as their dose of BUPHENYL

Delivery

BUPHENYL

  • Tablet or powder form
  • Taken by mouth
  • Can be taken by feeding tube

RAVICTI

  • Liquid form
  • Taken by mouth
  • Can be taken by feeding tube
Taste

BUPHENYL

  • Strong odor
  • Salty taste

RAVICTI

  • Odorless
  • Nearly tasteless

Take RAVICTI exactly as your doctor has prescribed it. Babies and toddlers may be prescribed up to 5 doses per day. Do not change your dose of RAVICTI without a doctor's permission. If you take too much RAVICTI, call your doctor or go to the nearest hospital emergency room right away. The maximum approved daily dose of RAVICTI is 17.5 mL for both adults and children.* If you miss a dose, talk to your doctor.

*Total daily RAVICTI dose:

  • Adults and children age 2 years and up: given in 3 equally divided doses, each rounded up to the nearest 0.5 mL
  • Babies and toddlers aged 2 months to younger than 2 years: given in 3 or more equally divided doses, each rounded up to the nearest 0.1 mL

Switching to RAVICTI: Making a change may be easier than you think

Living with a urea cycle disorder can be stressful. Choosing a medicine that requires little preparation might make it easier. If you are taking BUPHENYL tablets or powder to treat your UCD, you should know that liquid RAVICTI is taken differently.

  • You take RAVICTI as few as 3 times a day with formula or fluid
  • RAVICTI is odorless and nearly tasteless
  • No pills to swallow or powder to mix

Once you and your doctor decide to switch, your doctor will tell you the right amount of RAVICTI to take. Always take RAVICTI exactly as your doctor prescribes. RAVICTI is for patients with UCDs who are at least 2 months old and have not been able to control their ammonia levels 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 crises) or N-acetylglutamate synthase (NAGS) deficiency. It should not be used in children younger than 2 months because they may not be able to digest it.

How RAVICTI Works

The active ingredient in RAVICTI is released slowly in your body, helping to control your ammonia levels all day.

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How RAVICTI Works Video
Read transcript

A urea cycle disorder, also known as a UCD, is a genetic disease that people are born with.

As the body digests protein in food throughout the day nitrogen is released and it circulates in the body in the form of ammonia, or NH3.

Normally most of the ammonia is converted to urea in the liver and the body gets rid of it via the kidneys in the urine.

However, in people with a UCD, ammonia is not as effectively turned into urea and can build up to toxic levels. And spread throughout the body, including the brain.

An ammonia-removing medicine may be needed to help bind to ammonia so it can be safely removed from the body.

RAVICTI may help.

RAVICTI is an oral liquid ammonia removing medicine that is taken 3 times daily. And keeps working in your body throughout the day.

When a dose of RAVICTI travels through the body it is not released right away.

A naturally occurring enzyme found in the digestive tract slowly unlocks RAVICTI so it can be absorbed and start working to bind and remove excessive ammonia.

Once unlocked, RAVICTI is slowly released and converted to the active substance that binds nitrogen, so that it can control ammonia levels when given as indicated.

IMPORTANT SAFETY INFORMATION

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.

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.


Detailed Important Safety Information

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.

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.

Starting RAVICTI – Amy and Mya

When Amy's daughter Mya was diagnosed with a UCD at age 6 months, Amy learned to be an advocate for her daughter. Talk to a UCD patient or caregiver

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Starting RAVICTI: Amy and Mya's story
Read transcript

VOICEOVER:

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.

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.


Amy:

Mya was diagnosed at 6 months old. Our first step was low-protein diet. And she was just on that until about a year. At a year, we added some supplements. At 2 years, we had to add a medication, an ammonia scavenger.

The ammonia scavenger she was on could leave a bad taste in the mouth.

It was, uh, challenging to just flat out get her to take it.

And I thought, "There has to be another treatment."

I was willing to try something new.

So I went to the internet, and I searched and I searched, and I found RAVICTI.

And I thought, I have to get my child on this.

I did talk to my doctor about starting RAVICTI.

I had found most of the information online, so I pretty much sent him a link and said, "Here's this information." And he responded, "Great. I support you."

RAVICTI may not be a fit for everybody. For Mya specifically, it is convenient,

because it's tasteless, it’s odorless. Mya really doesn’t mind taking it.

For us,

starting RAVICTI was pretty easy.

It was easy for her to take,

and the routine was the same, 'cause

she still got the RAVICTI at the same time every day that she got the other one. It was just a

different medication.

Every family's experience is different, but for us,

Mya has been on RAVICTI for almost 6 years,

and we continue to like the convenience

VOICEOVER:
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.
It is not known if RAVICTI is safe and effective for children younger than 2 months.


Detailed Important Safety Information

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.

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.

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Important Safety Information And Approved Uses For RAVICTI® (glycerol phenylbutyrate) Oral Liquid
Who Should Not Take RAVICTI: Children...Children younger...

+ close -

Important Safety Information And Approved Uses for BUPHENYL® (sodium phenylbutyrate)
 

+ close -

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.

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 (ASS)

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 Prescribing Information, including Patient Package Insert, for more information.

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
BUPHENYL

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 (ASS)

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 Prescribing Information, including Patient Package Insert, for more information.