If you are a parent or the 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 steps to take in case of an emergency.
A Guide for Caring for a Child With a UCD
A UCD can cause the amount of ammonia in a child’s blood to rise to an unsafe level. This can make them very sick. The symptoms of high ammonia levels 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 their ammonia level 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 their ammonia level in a safe range, following these rules is very important for you and your child:
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.
Avoid extreme temperatures (too hot or too cold).
Stay well hydrated.
Avoid strenuous activities (for example, in gym class or at recess) and make a list of activities to avoid.
Take supplements and/or medicine every day (these should be taken at the same time every day).
Keep a list of emergency contacts.
Know what to do in an emergency.
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 UCD.
What Items Are in Your Go Bag?
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
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 level 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.
A Dietitian’s Advice for People With Urea Cycle Disorders
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 figuring out what works for your child and your family. One fun and tasty idea is encouraging your child to get involved in their own food preparation with you.