Generic alternatives to RAVICTI® (glycerol phenylbutyrate) are expected to become available for patients before the end of this year. Your treatment coverage and costs may be impacted.
For some patients, a low-protein diet and supplements alone are not enough to manage elevated ammonia levels caused by UCDs. These patients may be prescribed a nitrogen-scavenger medicine (sometimes called an ammonia scavenger) to help keep their ammonia levels under control. RAVICTI is an ammonia scavenger that must be used along with a low-protein diet that may include supplements. RAVICTI is not indicated for treatment of acute hyperammonemia in patients with UCDs, and it is not known if RAVICTI is safe and effective for the treatment of NAGS deficiency. RAVICTI has been proven to help manage ammonia levels for newborns, babies, children, and adults with UCDs.
Two ammonia scavengers are RAVICTI® (glycerol phenylbutyrate) Oral Liquid and BUPHENYL® (sodium phenylbutyrate) Tablets and Powder. Both medicines help remove ammonia from the body. Review the chart below to see some of the differences between these two treatment options.
Living with a urea cycle disorder can be stressful. RAVICTI is a liquid medicine that you can take by mouth, as few as 3 times a day. It is nearly tasteless and nearly odorless, and there are no pills to swallow or powders to mix.
You may take RAVICTI as few as 3 times* a day with formula or a liquid *Children less than 2 years of age may need more than 3 doses per day.
RAVICTI is nearly tasteless and nearly odorless 
No pills to swallow or powder to mix If you and your doctor decide to make the 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 have not been able to control their ammonia levels with diet and supplements alone. RAVICTI must be used with a low-protein diet and in some cases dietary supplements. RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crises). It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency,
Hilary decided to switch to RAVICTI to help manage her UCD. Watch as she explains why she chose RAVICTI.
Read transcript
We have protein guidelines, and we take our RAVICTI regularly. We can enjoy our foods, but not have to worry or be stressed out.
Of course, we only have protein mainly for one meal, whether it’s lunch or dinner, just to keep within the guidelines from the doctor, but we can still enjoy most things.
We take it once in the morning when we wake up. Ah, and I take a dose with me to work...and since I don’t need to mix it with anything, I can just take it.
I have a pretty big support system. I have my mom. She’s very helpful to me. She helps to make sure that Myiah gets her RAVICTI every morning before she gets on the school bus. Myiah’s father is very supportive. He makes sure Myiah takes her medication every time she’s with him. I have my boyfriend. He’s been a big help with me, also.It’s very helpful to know you’re not alone when dealing with things like this.
I really enjoy getting my hair done, doing my nails…I think it’s very important to focus on taking care of myself as an individual, so I can remind myself, I’m not just a mom taking care of people, paying bills.
I think that’s really important, to take time for yourself and do things that you enjoy doing…I believe that it genuinely makes you a happier person, more relaxed person…So you can give your best version of yourself to everyone else. The people you love and care about.
I kind of always want to put my kids first, so I feel guilty any time I do things for myself, but you can’t do things for others, if you can’t take care of yourself…and my kids are much happier when they know their mom is happy and taken care of, for sure.
USE and IMPORTANT SAFETY INFORMATION
What is the most important information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, 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:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
The most common side effects of RAVICTI in adults include:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional important safety information, see the
available at RAVICTI.com and discuss with your doctor.
Read transcript
Hello, today I want to talk to you about Understanding Urea Cycle Disorders and Your RAVICTI Treatment. First, let's talk about Urea Cycle Disorders. What is a Urea Cycle Disorder?
A urea cycle disorder, or UCD, is a rare inherited condition where toxic levels of waste called ammonia build up in the blood and cause damage throughout the body.
Ammonia is released when the body digests protein from food.
Normally, the liver makes enzymes that break down the ammonia into urea. The body then gets rid of urea through urine.
In people with a UCD, these enzymes don’t work right, so ammonia builds up in the blood.
High levels of ammonia in the blood can make you very sick and cause serious health problems. A low-protein diet and supplements can help keep ammonia levels down, but sometimes this isn’t enough.
Adding Ravicti can help keep ammonia at a safe level in the blood.
Ravicti is slowly broken down by a naturally-occurring enzyme found in the digestive tract.
Ravicti passes through the stomach and starts to slowly break down in the intestine. Once it breaks down, it can start getting to work.
A part of Ravicti then binds to ammonia molecules to form a Ravicti/ammonia complex that is excreted in the urine.
When taken correctly, Ravicti works to keep removing extra ammonia throughout the entire day. RAVICTI may cause side effects. The most common side effects of RAVICTI in adults include: diarrhea, gas, headache, abdomen (stomach) pain, vomiting, tiredness, decreased appetite, indigestion or heartburn.
So how do you take Ravicti? Ravicti is a liquid that you take by mouth with a syringe or dosing cup 3 or more times a day with a low-protein diet and in some cases dietary supplements.
It’s important to take Ravicti exactly as your doctor tells you in order to keep the
ammonia in your body at a safe level.
Set reminders so you don’t forget to take your dose ........ and keep track of when you do
take it.
A UCD is a lifelong disease that can cause organ damage and other very serious health problems over time without proper treatment. So even if you’re feeling fine, it’s important to continue taking your Ravicti to help prevent toxic buildup of ammonia in the blood. Talk to your doctor for more information and to help create a routine that works for you.
USE and IMPORTANT SAFETY INFORMATION
What is the most important information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, 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:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
The most common side effects of RAVICTI in adults include:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional important safety information, see the
available at RAVICTI.com and discuss with your doctor.
*According to data sourced from IQVIA and Amgen, between 6/01/2018 to 5/31/2019. Based on the total number of unique patients who had at least one dispense for RAVICTI in the United States.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, 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:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.
What is the most important safety information I should know about BUPHENYL?
BUPHENYL may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of BUPHENYL, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking BUPHENYL:
What is BUPHENYL?
Do not take BUPHENYL if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you experience an allergic reaction while taking BUPHENYL.
Before taking BUPHENYL, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of BUPHENYL?
BUPHENYL may cause serious side effects, including:
See “What is the most important information I should know about BUPHENYL?”
The most common side effects of BUPHENYL include:
These are not all of the possible side effects of BUPHENYL. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Patient Package Insert and discuss with your doctor.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, 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:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, 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:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.