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.
Long-term treatment of urea cycle disorders (UCDs)
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.
Source: DailyMed
Side effects that you should report to your care team as soon as possible:
Side effects that usually do not require medical attention (report to your care team if they continue or are bothersome):
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)
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
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.
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.
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.
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).
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.
Allergy to phenylbutyrate
Long-term treatment of urea cycle disorders (UCDs)
Long-term treatment for certain types of urea cycle disorders (UCDs)
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