glycerol phenylbutyrate
Ravicti (glycerol phenylbutyrate) is a medication used to treat people with urea cycle disorders (UCDs). It's usually added onto a low-protein diet. This medication is available as a liquid taken by mouth at least 3 times per day.
What is Ravicti (glycerol phenylbutyrate)?
What is Ravicti (glycerol phenylbutyrate) used for?
- Long-term treatment of urea cycle disorders (UCDs)
How Ravicti (glycerol phenylbutyrate) works
Ravicti (glycerol phenylbutyrate) is a urea cycle disorder agent. It removes ammonia (a toxic waste product) from your body. The medication attaches to it and changes it to a chemical that's removed from your body through your urine.
Drug facts
Side effects of Ravicti (glycerol phenylbutyrate)
The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.
Common Side Effects
- Diarrhea (16%)
- Passing gas (14%)
- Headache (14%)
Less Common Side Effects
- Stomach pain
- Nausea
- Vomiting
- Indigestion
- Poor appetite
- Fatigue
Ravicti (glycerol phenylbutyrate) serious side effects
Contact your healthcare provider immediately if you experience any of the following.
- Nervous system side effects: sleepiness, lightheadedness, numbness or tingling sensation, changes in taste, partial hearing loss, confusion
The following Ravicti (glycerol phenylbutyrate) side effects have also been reported
Side effects that you should report to your care team as soon as possible:
- Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
- Pain, tingling, or numbness in the hands or feet, muscle weakness, change in vision, confusion or trouble speaking, loss of balance or coordination, trouble walking, seizures
Side effects that usually do not require medical attention (report to your care team if they continue or are bothersome):
Pros and cons of Ravicti (glycerol phenylbutyrate)
Pros
- Works well to treat UCDs
- Available as an odorless, nearly tasteless liquid taken by mouth
- Can be used in people of all ages, including children as young as 2 months old
- Can safely switch from sodium phenylbutyrate (Buphenyl) to Ravicti (glycerol phenylbutyrate)
Cons
- Needs to be taken at least 3 times per day
- Needs to be used together with a low-protein diet
- Brand name only, which can be expensive
Pharmacist tips for Ravicti (glycerol phenylbutyrate)
- Before starting Ravicti (glycerol phenylbutyrate), make sure your provider or pharmacist teaches you how to prepare and give this medication to avoid wasting it. There are videos and guides available for instructions and tips on how to prepare and give Ravicti (glycerol phenylbutyrate). Contact your provider or pharmacist if you have any questions.
- Take Ravicti (glycerol phenylbutyrate) with food or formula. Use the oral (mouth) syringe provided by the pharmacy to directly place the medication into the mouth.
- If your baby needs to take Ravicti (glycerol phenylbutyrate) and is breastfed, give them Ravicti (glycerol phenylbutyrate) right before breastfeeding.
- For people who can't swallow, Ravicti (glycerol phenylbutyrate) can be given through a feeding tube. Ask your provider if you have any questions about how to take the medication with a feeding tube.
- Always check to make sure the medication is clear and colorless to pale yellow before using it. If it's cloudy, discolored, or has particles (solid chunks), don't use it. Contact your provider or pharmacist to see if it's possible to get a replacement.
- Store Ravicti (glycerol phenylbutyrate) at room temperature only. Throw away bottles 28 days after opening them, even if there's leftover medication.
- Don't breastfeed while taking Ravicti (glycerol phenylbutyrate) due to the risk of severe side effects to your baby. Ask your provider about other ways to feed your baby.
- Ravicti (glycerol phenylbutyrate) can interact with certain medications. These interactions can cause problems like raising blood ammonia levels, raising the risk of Ravicti (glycerol phenylbutyrate) side effects, or causing other medications to not work as well. Depending on your other medications, your provider might need to monitor you more closely. Before starting Ravicti (glycerol phenylbutyrate), make sure to tell your provider about all of the medications you're taking, especially if you're taking steroids, valproic acid, haloperidol, or probenecid. Also, don't start any new medications without talking to your provider or pharmacist first.
Common questions about Ravicti (glycerol phenylbutyrate)
The urea cycle is a process in your body where certain enzymes (proteins) help change ammonia (a toxic waste product) into urea. Urea is then filtered out of your blood by your kidneys and removed from your body when you urinate. Urea cycle disorders (UCDs) are a group of genetic problems where your body is missing some of the enzymes required to change ammonia to urea. This causes high levels of ammonia in your blood (hyperammonemia). Symptoms of hyperammonemia can include confusion, headache, dizziness, and vomiting, but can even cause serious problems like seizures and coma. Ravicti (glycerol phenylbutyrate) helps keep ammonia levels low by changing ammonia into a chemical that can be removed through your urine.
Even though Ravicti (glycerol phenylbutyrate) helps get rid of ammonia from your body, it's important to continue to limit your protein intake while taking this medication. This helps the medication work as best as it can to keep your blood ammonia levels low. Your body breaks down protein from foods and beverages into ammonia. But with a UCD, your body can't get rid of this ammonia. Eating or drinking too much protein while taking Ravicti (glycerol phenylbutyrate) might counteract what the medication is trying to do.
No, don't take more Ravicti (glycerol phenylbutyrate) than what's prescribed to you. If you're experiencing symptoms of high blood ammonia levels (hyperammonemia) like confusion, dizziness, headache, vomiting, or tiredness, get medical help right away. Ravicti (glycerol phenylbutyrate) isn't used to treat short-term hyperammonemia, so you'll need to go to the hospital to receive appropriate treatment.
Both Ravicti (glycerol phenylbutyrate) and sodium phenylbutyrate (Buphenyl) contain the same active ingredient (phenylbutyrate) and work well to treat UCDs, but there are several differences between the medications. Sodium phenylbutyrate (Buphenyl) is available as a tablet or powder and tends to have a strong odor and salty taste. On the other hand, Ravicti (glycerol phenylbutyrate) is an odorless, nearly tasteless liquid. Additionally, with sodium phenylbutyrate (Buphenyl), you might need to take up to 40 tablets per day, whereas with Ravicti (glycerol phenylbutyrate), you might only need to take up to 17.5 mL (or 3.5 teaspoons) per day. Talk to your provider to see which medication is right for you.
Ravicti (glycerol phenylbutyrate) hasn't been well studied in human pregnancy, so it's not known whether this medication is safe or works well in pregnant females. However, some animal studies have suggested that Ravicti (glycerol phenylbutyrate) could harm babies and sometimes mothers during pregnancy at doses higher than what's typically used for humans. If you're pregnant or thinking of becoming pregnant, talk with your provider about the risks and benefits of taking Ravicti (glycerol phenylbutyrate) during pregnancy. If you're pregnant and are prescribed Ravicti (glycerol phenylbutyrate), let your provider know. It's recommended to report the pregnancy to the manufacturer (Horizon Therapeutics) by calling 1-866-479-6742.
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Risks and warnings for Ravicti (glycerol phenylbutyrate)
Ravicti (glycerol phenylbutyrate) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Nervous system side effects
Ravicti (glycerol phenylbutyrate) can cause nervous system side effects, such as sleepiness, fatigue, lightheadedness, and headache. Other related side effects can include a numbing or tingling sensation, changes in taste, partial hearing loss, and confusion. In clinical studies, these side effects were typically reversible and may have been due to high blood levels of Ravicti (glycerol phenylbutyrate). Contact your provider if you have any of these symptoms. Your provider will likely measure your ammonia level to see if your symptoms are due to Ravicti (glycerol phenylbutyrate), high ammonia levels, or some other cause. If they're due to Ravicti (glycerol phenylbutyrate), you might need to take a lower dose of the medication.
Use with caution in people with pancreas or intestine problems
Ravicti (glycerol phenylbutyrate) might not work as well in people with pancreas or intestine problems, because the medication might not get fully absorbed into your body. Certain proteins in your pancreas help your body absorb Ravicti (glycerol phenylbutyrate) from your intestines, but pancreas and intestine problems can interfere with this process. Make sure to tell your provider about all of your medical conditions before starting Ravicti (glycerol phenylbutyrate).
Ravicti (glycerol phenylbutyrate) dosage
Typical dosage for Ravicti (glycerol phenylbutyrate)
This medication is taken by mouth with each meal or feeding.
- If you haven't taken either Ravicti (glycerol phenylbutyrate) or sodium phenylbutyrate (Buphenyl) before: The typical starting daily dose is based on your body surface area (BSA), which takes into account your height and weight. It also depends on how much protein you eat or drink and how severe your UCD is. Your provider will calculate the correct dose for you.
- If you're switching to Ravicti (glycerol phenylbutyrate) from sodium phenylbutyrate (Buphenyl): Your total daily dose of Ravicti (glycerol phenylbutyrate) will be based on your previous treatment with sodium phenylbutyrate (Buphenyl). Your provider will help find the right dose for you.
Children younger than 2 years should take the total daily dose of Ravicti (glycerol phenylbutyrate) in 3 or more equal doses (rounded up to the nearest 0.1 mL).
People 2 years of age and older should take the total daily dose of Ravicti (glycerol phenylbutyrate) in 3 equal doses (rounded up the nearest 0.5 mL).
The maximum dose is 17.5 mL per day. Your provider will adjust your dose depending on your diet, how your ammonia levels respond to the medication, and if you're having side effects. Your dose also might differ if you have liver problems.
Ravicti (glycerol phenylbutyrate) contraindications
Contraindications are specific health conditions or situations in which a person should not take a medication due to safety concerns. If you have any of the following conditions or if any of the following apply to you, let your healthcare provider know because Ravicti (glycerol phenylbutyrate) will not be safe for you to take.
- Allergy to phenylbutyrate
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