If your urine smells like burnt sugar or maple syrup, you may have a condition called maple syrup urine disease, or MSUD. MSUD is a genetic metabolic condition that disables a body from being able to break down some smaller parts of proteins called branched-chain amino acids, according to MedlinePlus. If you have MSUD you can't properly digest some parts of protein and would need to eat a diet that reduces protein intake but still offers a balanced intake of nutrients.
Limits
If you have MSUD, limit your intake of protein by cutting down on high-protein foods such as cow's milk and cow's milk products, regular infant formula, eggs and all types of meat, according to the California Department of Public Health. Also cut down on or eliminate beans and nuts, peanut butter and regular flour and eat only carefully measured amounts of fruits and vegetables that contain low amounts of branched-chain amino acids.
Tracking Protein Intake
Even if you have MSUD, you need to take in some protein every day because everybody needs branched-chain amino acids to grow and develop properly, according to Kaiser Permanente online. However, to avoid eating more than your body can handle, you will need to learn methods of tracking how much protein is in the foods you eat. This typically involves looking at a food chart or typing a food into a database such as "USDA National Nutrient Database," found under Resources below, and searching for the quantity of leucine, isoleucine and valine -- branched-chain amino acids -- in the food item.
Formula
A MSUD diet by itself is unlikely to sustain optimal health. Therefore, an additional aspect of a diet for MSUD is a protein formula that contains all necessary amino acids besides leucine, isoleucine and valine, according to Kaiser Permanente. The formula is also fortified with essential vitamins and minerals to reduce risk of malnutrition. Your doctor or dietitian will prescribe a formula based on your individual needs.
Concerns
A primary concern about MSUD diets is achieving and maintaining balanced nutrition. Low-protein foods are often high in fat, sugar or both fat and sugar, according to Kaiser Permanente. If your dietitian or doctor doesn't carefully monitor your diet, you will be at greater risk of developing health problems such as obesity. Another potential problem is a lack of food variety in your diet, but specialty low-protein products can help make your diet more appetizing.
Supplementation
You may have a form of MSUD that can be fully corrected by supplementation with a B vitamin known as thiamin, according to medical journal "The Lancet." In this case, you would need to make no dietary changes but take about 10mg of thiamin per day to reduce your risk of health complications.
References
- Kaiser Permanente: Maple Syrup Urine Disease
- lowproteinLIVING by SHS International: Maple Syrup Urine Disease
- California Department of Public Health: MSUD
- MedlinePlus: Maple Syrup Urine Disease (MSUD)
- MSUD Family Support Group: Treatment
- "The Lancet"; Thiamine-Responsive Maple Syrup Urine Disease; C.R. Scriver, et al; Feb. 1971
