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Medications for urea cycle disorder.

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.

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