Diets for patients with cystic fibrosis

Cystic fibrosis (CF) is a disease caused by a genetic disorder which is associated with an impaired transport of electrolytes in the secretory epithelium of excretory glands. This autosomal recessive hereditary disease is a multiorgan disease, characterized mainly by respiratory but also by digestive malfunctions and often linked with malnutrition. Malnutrition is due to a disturbed energy balance caused by reduced energy supply (maldigestion and malabsorption as a result of exocrine pancreas insufficiency, lack of appetite, nausea and vomiting) and an increased energy need (infections, fever, increased respiration). Nutritional therapy and supplementation of pancreatic enzymes are effective means of influencing the extent of malnutrition and deterioration of lung function. The diet of CF patients should be well balanced and fat-supplemented. in addition, the status of vitamins, minerals and trace elements should be checked regularly and individual nutrients be supplemented, if necessary. The use of nutritional supplements may be beneficial for undernourished patients to significantly increase their weight index. In severe cases of malnutrition enteral or parenteral nutrition should be considered.