For people with urea cycle disorders (UCDs), there are options to help control high ammonia levels. There are different types of UCD treatments and each person has different needs. Talk to your doctor to develop a plan that’s best for you.

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Low-protein diet

One part of a urea cycle disorder treatment that your doctor may recommend is a low-protein diet. However, our bodies still need some protein to grow and stay healthy. In fact, eating too little protein can also be harmful because your muscles can start to break down. This process, called catabolism, can also cause ammonia levels to rise.

Amino acid supplements

Amino acid supplements 

Amino acids are essential nutrients that are needed to grow and maintain your health. The body creates amino acids when it breaks down protein in your body. If you are on a low-protein diet, and eating less protein, your body may not be able to create enough amino acids. Supplements can fill the gap for the nutrients you would normally get from having more protein in your body. A dietitian can help decide which supplements may be right for you.

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Ammonia-controlling medicines

A low-protein diet and supplements may not always be enough to keep your ammonia levels low, and a treatment that gets rid of excess ammonia may be needed. Nitrogen-scavenger medicines (also called “ammonia scavengers”) help to remove ammonia from the bloodstream. Talk to your doctor about whether a nitrogen-scavenger medicine may be right for your UCD management plan.

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Liver transplant

A liver transplant can cure a UCD because a new liver has enzymes in the urea cycle that work the way they should. Like any surgical procedure, a transplant has risks. It’s also important to understand that a liver transplant can’t fix any UCD-related health problem that happened in your life before having the transplant. 

Talking to Your Doctor

Get prepared for your next doctors appointment with these 3 things to share.

Read transcript
It’s important to talk to your physician about your urea cycle disorder because knowledge is power. If you understand your illness, you can have a better outcome. There are 3 things you should share on your first visit: your questions. If you do not understand your urea cycle disorder, you should ask questions. You should also explain your fears. It is very important to have this connection and communication with your physician because you may fear some things that may not be real, and explaining that to your physician, they’ll be able to comfort you or explain to you in a better way so you can understand. And, what are your goals, so that as a team you can come up with a management plan. If you have been diagnosed with a urea cycle disorder, you should know that the metabolic team is here for you. We are here to help you. We have developed a program to make you that you and your family is well taken care of. 

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