9 Cystic fibrosis: Nutritional consequences and management

Malnutrition is an adverse prognostic factor in cystic fibrosis, influencing the course of pulmonary disease and correlating inversely with survival. A positive energy balance between energy intake and the combination of total energy expenditure, energy losses and growth-related energy cost is essential to maintain normal nutritional status. Before starting nutritional supplementation, it is important to rule out pathological conditions that may have a deleterious effect on nutritional status: persistent exocrine pancreatic insufficiency, chronic bacterial pulmonary colonization, impaired glucose tolerance, specific nutritional deficits and associated disorders leading to a decrease of energy intake. Several methods are available, ranging from boosted oral nutrition to behavioural intervention, oral supplementation, enteral nutrition and, rarely, parenteral nutrition. The use of elemental nutrients for either oral supplementation or enteral nutrition seems of no nutritional benefit and is more expensive than conventional polymeric nutrients. Provided that the goals of the nutritional supplementation are fulfilled, simpler is often better.