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The mainstay of treatment of these disorders is PROTEIN RESTRICTION. As protein is the source of nitrogen-containing chemicals in the diet, limiting protein will limit the amount of nitrogen-wastes.
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This reduces the stress on the hampered urea cycle. It is imperative that protein restriction be done with guidance from a health care professional who can make certain to balance the amount of protein needed for growth with that which will cause symptoms.
It is extremely important for people with these disorders to AVOID FASTING.
Patients with urea cycle abnormalities must also be very careful under times of stress, such as infections. Stress, such as fever, can cause the body to break down its own proteins and exceed the capacity of the abnormal urea cycle to dispose of the by-products.
A sick-day plan should be formulated with your doctor to avoid all protein, consume high carbohydrate drinks, and stay hydrated.
Most patients with urea cycle disorders require hospitalization at some point in their illness. During such times, they may be treated with medicines that help the body dispose of nitrogen-containing wastes. Dialysis may be required to help rid the body of excess ammonia during extreme illness.
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