Maple Syrup Urine Disease: Treatment, Procedure, Cost and Side Effects

branched-chain ketoaciduria

This is a rare condition where your urine smells sweet and similar to that of the smell of maple syrup. This occurs when the person suffers from a metabolic crisis. The other symptoms for this disease are that you feel lethargic, have a poor appetite, muscle rigidity and of course a maple syrup smell from your urine, sweat and earwax. If the disease is untreated or unrecognized then it might lead you to have a seizure attack, coma or even death. Infants and even adults might suffer from this disease which is caused by a neurological disorder. In adults they also might arrive from a nerve disorder or from an injury or by excessive fasting. The amino acid levels in your body increase all of a sudden and cause the complications associated with it. Other symptoms of the maple syrup urine disease are unexpected weight loss, anemia (loss of blood cells), diarrhea, pancreatitis and even neurological problems. The maple syrup urine disease can also cause certain renal infections if they are left untreated. In some rare cases of maple syrup urine disease, you can develop a condition of osteoporosis which can cause many complications regarding the course of treatment as well.

The treatment procedure involves a protein free diet and fats are injected in your veins. Diet is an important factor for the course of treatment in this case. Those who are diagnosed with this condition must consume amino acid free diet for the rest of their life. If you follow the diet charge that your medical expert provides you with then it can prevent you from having neurological damages that might lead to the emergence of the maple syrup urine disease symptoms. For the treatment of this condition, you should monitor with the amino acid levels in your body. Diet control is essential to ensure better results of the course of the treatment. You should develop a diet that has less amino acid levels , limited quantity of vitamins, minerals, omega 3 fatty acids and other nutrients should be consumed. In rare and severe conditions of maple syrup urine disease you can obtain a liver transplant as now your body fails to respond to the medications and the treatment that you obtain. This procedure also carries a certain amount of risk as a number of various factors should be kept into account. Individuals with many past surgeries and heart problems may not be permitted for a liver transplant.

Usually this disease is common with the newborns and often they are screened right after birth. Those who have an odor of their urine, sweat and ear wax similar to that of maple syrup are eligible for the treatment. You might also want to consult with your medical expert if you also face fatigue, loss of appetite and dark urine.

There are no specific conditions where you are not eligible for the treatment, however, you should consult with your medical expert to be on the safe side.

Side effects to this type of condition are unknown

You should follow the protein free and amino acid free diet for the rest of your life and you should consult your medical expert to take protein injections. However, the symptoms of this disease will be eliminated if the victim is a child but you may not know when the symptoms might re occur again.

This condition is not completely curable and you can reduce the odd odor by periodically taking protein injections and follow a diet that are not rich in amino acids.

The cost depends on injections, medicines and a diet procedure. The price range in India ranges between Rs. 600 to Rs. 3,500.