Re: DiMauro on illness protocol

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Thank you so much for the information you are giving me. I really hope to be well armed next time I need treatment for my girls. Darla DiMauro on illness protocol Question: In a patient with mitochondrial disease, does giving extra calories, in the form of glucose, extra tube feeds or uncooked cornstarch have any effect on decompensation due to illness? Can it prevent decompensation if started at the first sign of illness, before any noticeable decompensation? D.L. Answer: If by "mitochondrial disease", we refer, as it is now customary, to disorders due to defects of the mitochondrial respiratory chain, then unfortunately provision of extra calories does not help because the problem is not in the metabolism of foodstuff (glycogen, lipids), but in the generation of energy from their terminal product, acetylcarnitine. However, many patients with mitochondrial diseases have gastrointestinal problems, making adequate nutrition difficult. In these patients, nasogastric feeding or percutaneous endoscopic gastrostomy (PEG) may be of great benefit. Responder: Salvatore DiMauro MD__________________________________________________________________There is no single answer to this question.The stomach and small intestine break food down into fats, carbohydrates (sugars) and amino acids (protein). In a healthy person, any food eaten that is needed immediately is stored in the liver as glycogen (a complex form of sugar) or as fat. Protein cannot be stored and is converted into sugar and urea. The urea is excreted in the urine. The liver can store enough sugar to run the body for about 12 hours. The ability to store fat is enormous. During times of starvation, whether this is voluntary or due to an inability to eat because of illness, we live off our stored sugars and fats. In the healthy person, the ability to survive starvation depends on how much fat is stored, and given a supply of water, people can live for months. The fat can be mobilized and quickly converted into fatty acids and ketones, which can run the body. During periods of fasting the blood sugar levels can drop to very low levels, but the body can still survive as long as it can produce and use those fatty acids and ketones.In many mitochondrial patients there is an inability to store or access any stored sugar, and an inability to convert fat into ketones, or an inability to use the fatty acids and ketones. Because there is a relative block in metabolism, the ability to process (the speed at which food can be turned into energy) calories is reduced. During an illness, giving supplemental sugar is usually necessary. This can be given as food, a beverage (Gatoraid for example), or as an IV. Cornstarch is a complex carbohydrate, which is slowly digested and releases the sugar over 6-8 hours. In a healthy child, starvation during a one or two day illness rarely causes any problems. In a child with a mitochondrial disease, where the caloric reserves are low and the ability to compensate for any stress is reduced, the risk of energy failure with resultant serious effects is heightened. This is a relative risk, and some patients are more susceptible to problems than other patients. Early fluids and sugar is helpful as a general rule. In children with fatty acid oxidation disorders, such as medium chain acylCoA dehydrogenase deficiency, each illness needs to be treated as a crisis. These children must have IV fluids and glucose. I tell parents "do not pass GO, do not collect $200,,,,,go to the ER with the first sniffle." In other children, everything can be handled at home. The protocol for this problem should be discussed with your physician beforehand. Please contact mito-owner with any problems or questions. Get more from the Web. FREE MSN Explorer download : http://explorer.msn.com