MSUD -- or maple syrup urine disease -- is a rare metabolic disorder that affects 1 in every 225,000 people. MSUD patients lack the natural enzymes necessary to break down the amino acids leucine, isoleucine and valine. These amino acids occur naturally in many foods, and if eaten, will accumulate in the blood because they cannot be broken down and used. Because of this, diet is an important factor in the treatment of MSUD. Without treatment, MSUD can lead to urine that smells like maple syrup, severe mental retardation, coma and death.
MSUD Diet Overview
To keep blood levels of leucine, isoleucine and valine under control, you will need to follow a strict diet. You must follow the diet for life, as there is currently no cure for MSUD. For most cases of MSUD, a special routine of medical formula will be prescribed by your doctor or dietitian to allow you to get all of the nutrition you need without any leucine, isoleucine or valine. You will be allowed limited foods, but the foods are special, low-protein foods, and you will need to record and count the amounts of each of these amino acids each day.
About Counting Protein
You will need to avoid protein foods, as they contain all three amino acids. Your doctor or dietitian will give you a specific amount of protein you can have each day, which is generally 10 g or less. It is important you do not exceed this restriction. Use resources, such as the USDA Nutrient Database or the book "Bowes and Church's Food Values of Portions Commonly Used," to help you count protein and amino acid content of foods.
High-Protein Foods
Completely avoid foods such as meats, eggs, dairy, soy and legumes, as they are very high in protein and also high in isoleucine, leucine and valine. Additionally, avoid products containing ingredients that come from these foods, as they also provide too much protein. Remember that your medical formula provides all of the daily protein that you need, so your diet should only contain minimal amounts.
Low-Protein Foods
Low protein foods include fruits, vegetables and most grains. However, just because these foods are low in protein does not mean they have no protein at all. It is important to count the protein in all foods in your diet, even if it only provides 1 g. Pay attention to portion sizes to make sure the low-protein foods are not adding up to too much protein.
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