Nutrition

Derangement of metabolic equilibrium is manifested in multiple organs in RTT. This often has a nutritional origin. There is very high-energy expenditure through increased motor activity, forceful breathing, Valsalva’s manoeuvre type of breathing, hyperventilation and perspiration. Daily energy and water requirements may be much higher than is often provided to persons with RTT. It is apparent that Forceful Breathers and the RTT population with the Valsalva’s manoeuvre type of breathing will require more than the normal amount of daily energy intake. It is also apparent that there is extra need for DNA and cell repair due to increased catabolism. This is usually carried out through the complex pathways of intracellular and extracellular membrane transport systems that are affected by the Reduction-oxidation (REDOX) status of the cell. The deranged carbon dioxide metabolism in RTT affects the REDOX status of the cell and therefore influences these cellular processes. 


Nutritional management in RTT must include evaluation and calculation of daily intake of food and energy requirements by a dietician. Measurements of Body Mass Index (BMI) and skin folds are useful for monitoring the progress of treatment. Clinical monitoring of blood total protein, albumin, protein electrophoresis, urea, creatinin and electrolytes like Na+, K+, Cl¯, Ca2+ and PO4 are useful in the assessment of nutritional status in RTT. Nutritional treatment must include high and condensed calorie diets. Poor responders may eventually require Percutaneous Endoscopic Gastrotomy, later replaced by small Mickey button, for supplementary feeding. Food supplements and extra micronutrients like glycoproteins, glycolipids and essential fatty acids are required. Glyconutrients are necessary for cellular and nucleic acid repairs .