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Nutrition

Patients with mitochondrial disease have many reasons for not eating well, including GI issues, neurologic disease, and metabolic instability.  The result is the same - an inappropriately low intake of calories and nutrients.  This can result in fatigue and lethargy; when persistent, the patient's weakened state can appear as if the disease course is progressing or accelerating.  Furthermore, a malnourished or undernourished condition can be associated with an exacerbation of symptoms (such as muscular pain, headaches, and/or migraine).  

Perhaps the most important and effective aspect of the management of mitochondrial disease is ensuring an adequate nutrient and calorie intake.  Our experience is that any potential benefit from supplemental vitamins and cofactors cannot be fully realized in an undernourished state.

Patients may have problems with swallowing due to muscular or neurologic incoordination.  Oral muscle fatigue can interfere with the ability to feed; patients may avoid foods that require a lot of chewing, and prefer mushy or soft foods, or liquids.  A change in texture preference or choosing liquids should not compromise a patient's calorie intake; a patient can take in his/her full calorie requirements in a liquid form (although the input of a dietitian is advisable).  If a trial of a nasogastric or nasojejunal tube documents the patient's ability to meet his/her dietary goals, placement of an enteral tube may be indicated.

It is always preferable to use the gut to guarantee nutrition.  However, when gut motility is severe enough that nutritional needs cannot be met enterally, consideration of parenteral nutrition must occur, even to provide a part of the patient's overall calorie intake.  However, the risks associated with having a central venous line and the use of parenteral nutrition need to be acknowledged, notably liver disease, vascular complications (e.g., clotting), and sepsis especially when gut dysmotility is severe and there is a real hazard from microbial translocation from the gut.

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