Treatment is a lifelong process that doesn't cure the condition, but it can effectively manage the symptoms. Frequent blood tests are done to continue 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 probably will involve:
- 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.
However, protein is important for growth, so the 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.
- Medications. Some children will need to take medicine to help take extra ammonia out of the body. Oral medication 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 to help give the body what it needs to make proteins that are important for growth and tissue repair, since children with urea cycle disorder 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 discuss the risks of transplantation with you and a decision must be made with the consideration of the pros and cons.
If a transplant is the best treatment option, the doctor and the other members of the patient care team will focus on preventing complications and will treat symptoms while your child waits for the donated liver.
Stresses on the body, such as illness, fever, surgery or an accident, can cause ammonia levels to rise. Care must be taken at these times, and extra calories will be needed to provide the stressed body with fuel.
Visits with your doctors and other specialists will be necessary to make adjustments to diet and medication. Having your child follow the diet your doctor gives is important for the child's health, growth and development.