Medications for urea cycle disorder.
Enroll with us in a few easy steps
A low protein diet and amino acid supplements help keep your ammonia at a safe level. But sometimes, you may also need medication for your urea cycle disorder (UCD). Two medications available today are Ravicti (glycerol phenylbutyrate) and Buphenyl (sodium phenylbutyrate). These medications don’t directly lower the amount of ammonia in your body. They lower the amount of nitrogen that changes to ammonia. That’s why they are sometimes called nitrogen scavengers.
Ravicti comes as a liquid. Buphenyl comes as a powder or a tablet. It’s important to spread your daily dose out with your meals during the day. These medications should always be taken with food. Most people don’t like the taste of these medications. But, it’s important to take them as instructed.
UCD is unpredictable. You can eat right and take your supplements and medication as directed. Even then your ammonia levels may go up. This is why it’s important to watch for signs of rising ammonia level in your body. Neither of these medications help when your ammonia level is very high (also called hyperammonemia or hyperammonemia crisis). Hyperammonemia is always an emergency.
Talk to your doctor about any symptoms that are new or getting worse. Your CVS Specialty CareTeam is also here for you. They can help answer your questions about side effects or give you tips on staying on track.
This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about your medical condition and prior to starting any new treatment. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.
CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship, or recommendation by CVS Specialty.
Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.
This page contains trademarks or registered trademarks of CVS Pharmacy, Inc. and/or one of its affiliates; it also contains references to products that are trademarks or registered trademarks of entities not affiliated with CVS Health.