If you are a parent or a caretaker of a child with a urea cycle disorder (UCD), you may have many questions on how to best manage their disease. Nutrition, activity, medicine and treatment are all important factors. Here is some information that can help make your role a little easier as well as what steps to take in case of an emergency.

A UCD can cause the amount of ammonia in a child’s blood to rise to unsafe levels. This can make him or her very sick. The symptoms of high ammonia don’t appear quickly. If you’re new to managing a UCD for your child, it may be easy to miss the subtle signs that ammonia is rising. By the time a child with a UCD appears to be sick, it usually means that medical attention is required. If this happens, you should follow the emergency protocol at the end of this section.

To stay healthy and keep ammonia levels in a safe range, following these rules is very important for you and your child:

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Stick to a low-protein diet (make sure all food intake is carefully monitored)

  • No extra food (snacks, treats, etc) unless it’s approved by parents first
  • A low-protein diet is different—and more restrictive—than a vegetarian one
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Avoid extreme temperatures (too hot or too cold)

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Stay well hydrated

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Avoid strenuous activities (for example, in gym class or at recess) and make a list of activities to avoid

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Take supplements and/or medicine every day (these should be taken at the same time daily)

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Keep a list of emergency contacts

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Know what to do in an emergency

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Watch for common symptoms of high ammonia levels, including sluggishness, staring, nausea or vomiting, irritability, aggression, lack of appetite, slurring words and headaches

If you see the symptoms listed above, it could mean that a hyperammonemic crisis is happening. This is when the amount of ammonia in the blood becomes extremely high. A crisis can be very dangerous and requires medical attention right away. While at the hospital, it is important to notify staff of your child’s urea cycle disorder.

If you care for a child with a UCD, you know that ammonia can be unpredictable. Ammonia can sometimes reach high levels with little warning. Many families keep a go bag packed with essential items for their child to help them get out the door quickly in case a trip to the emergency room is necessary. Having this bag can reassure your child and make his or her trip to the hospital more comfortable.

Use this guide to help you decide what to pack in your own go bag.

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Remember: Tell people around you about your child’s go bag. Other family members, friends, teachers, and daycare providers should know where to find the bag and what to do with the contents.

Healthy fun for children with UCDs and their families

Some activities can cause your child’s ammonia levels to become dangerously high. But there are plenty of ways to have fun indoors and outdoors while staying safe. Check out these activities and tips.

Staying safe and staying active

Diet is an important part of managing your child’s UCD. Watch these videos to find out more about what a balanced low-protein diet looks like, the role of a dietitian, navigating meals, and finding what works for your child and your family. One fun and tasty idea is encouraging your children to get involved in their own food preparation with you.

Lily’s Low-Protien Diet

Hear Leona discuss Lily’s low protein diet and her favorite foods, and how the family works together to make sure Lily feels included.

Read transcript

Regarding Lillyanna’s diet and the importance of eating a low-protein diet…part of her care is making sure that she follows that low-protein diet and sticks to it.  

Usually, I fix us up some fresh fruit such as banana, ah, and in addition, maybe a waffle for breakfast. She eats like a low-protein snack...a lot of times she sticks mainly to starches.  

For lunch…she can only have a small amount of chicken. Ah, meaning like maybe a, a fourth of a cup of chicken, ah, per day. A lot of times she sticks more to a vegetarian diet, because she says she likes the way that it tastes.  

If she could eat French fries every day, I think that she could. It’s something about the salty starch mixture that she really enjoys.   

he’s very picky when it comes to vegetables, so a lot of times we have to compromise. Whatever we can do to make sure that she eats a healthy diet, I try to, to supply and make sure that she does.  

When Lilly was younger, and I had to explain to her the reason why she...had to have a low-protein diet and her sister who, you know, is in the same household did not. I just explained to her the side effects that came with her not eating healthy…that if she did eat things that she couldn’t eat, she could have, you know, serious headaches. It could...possibly evolve into a hospital trip. And Lilly, even from a very young age, she was very understanding that she could not eat these things. She knew that these things could possibly make her sick.   

If Lilly was eating green beans, then everybody ate green beans. If Lilly was eating mashed potatoes, everybody ate mashed potatoes. So it wasn’t that she had her own meal. I made sure that she could eat it, as well as we could eat it, so she could feel included at the dinner table.  

Lilly’s, routine regarding lunch. I had …spoke to the…cafeteria management regarding different vegetarian options that Lilly could have for lunch. Sometimes children don’t always want to bring lunch to school. They wanna feel like they’re eating what their classmates are eating. So, I went there, found a letter stating foods that she could and could not eat, to make sure that they had options that were gonna help support her.   

Lilly is...very vocal. (laughs) And she’s very straightforward, and she communicates very well. So, she would just explain to them very directly, I can’t eat that because it, unfortunately it will make me sick.   

I wanted her to be aware that...these things could make you sick. So, she’s able to advocate for herself amongst her friends.  

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