How is UCD Treated?
Treatment is a lifelong process that doesn't cure the condition, but it can effectively manage the symptoms. Frequent blood tests are done to monitor ammonia levels. Doctors in the areas of pediatrics, genetics and nutrition will work together to develop the child's treatment plan.
The child's treatment could involve:
- A low protein, high-calorie diet. Protein in the diet is lowered by avoiding protein-rich foods. Examples of foods that provide calories without loading the body with protein are fruits, vegetables and starches. Protein restriction must be done with advice from a healthcare professional. A dietitian will plan and update a protein-restricted diet as the child grows. Some children need to take a special formula to make sure they are getting proper nutrition.
- Medications. Some children will need to take medicine to help get extra ammonia out of their body. Medication by mouth is given that binds to ammonia and carries it out in the urine.
- Amino acid supplements. Depending on the type of UCD, amino acid supplements such as arginine or citrulline may be added to the diet. This helps give the body what it needs to make proteins that are important for growth and tissue repair, since children with UCD can't make arginine on their own.
- Liver transplantation. Because the production of urea cycle enzymes takes place in the liver, a liver transplant can be an effective treatment for urea cycle disorder.
Your doctor will talk to you about the risks of transplantation. If a transplant is the best treatment option, your doctor and members of the care team will focus on preventing complications. The care team will treat symptoms while your child waits for the donated liver.
Illness, fever, surgery or an accident, can cause ammonia levels to rise. Care must be taken at these times. Extra calories will be needed to provide the stressed body with fuel.
Visits with your doctors and other specialists will be needed to make adjustments to diet and medication. Having your child follow the diet your doctor gives is important for your child's health, growth and development.