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.
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I gave birth to a perfect little baby boy. When he was two days old, he started getting very sick and having symptoms of having a UCD.
We were able to get a diagnosis and I was able to get tested. And I found out that I actually am a carrier of the genetic disorder. When I got pregnant with my second child, I was very scared. It was a very, very hard decision and at 11, 12 weeks, I found out that my baby tested positive for this same genetic disorder that my son had.
Fortunately, because we had that diagnosis, we were able to have a plan of action for bringing Myiah into the world.
When I was first diagnosed with a urea cycle disorder, I was not put on any medication. It wasn’t until after I had my first daughter, about five or six years later. I was experiencing really bad headaches and I have always struggled to gain weight. My whole life I was like 90 some pounds. It was very difficult for me.
When I started experiencing more symptoms, as I was getting older, my doctors put me on sodium phenylbutyrate. It tasted terrible. It was very difficult for me to mask the flavor at all. I did not want to take my medicine.
When she was born, she needed to be placed on medication due to her severity of her genetic disorder, so they started her on sodium phenylbutyrate and she took it orally.
Luckily, Myiah, she tolerated the medicine until she was about two years old, and that’s when we were lucky enough that RAVICTI was approved for two years and up.
My decision to start taking RAVICTI was first approached to me, for myself, actually, by my doctor. She brought it up to me. I knew about it because I had connected with multiple other parents that either themselves or they had a child that was either in the study for the RAVICTI or was actually taking it. I knew it was definitely something that I was interested in looking into.
Starting RAVICTI was a little difficult. I had to previously be prescribed the sodium phenylbutyrate to see how that medication worked for me first. It was awful to take. I wanted to throw up.
My insurance, they will require a prior authorization for the RAVICTI. If you have trouble getting coverage for the RAVICTI, there’s so many options out there.
Myiah was put on it as soon as it was approved for children her age. We started the process of getting her prior authorization approved for her to take RAVICTI and she’s been taking it ever since.
She’s done amazing on it and there was no issues with her getting coverage.
I take RAVICTI three times a day. Myiah, she’s able to take it two times a day rather than three. We take it once in the morning when we wake up and I take a dose with me to work. I put it in the syringe that it comes with, and I pack it in a little baggie and since I don’t need to mix it with anything, I can just take it and then I usually just take a sip of water afterwards. And then our evening doses are before bed.
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Voiceover:
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.
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 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.
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.