Current issues in the nutritional management of children with cystic fibrosis

Recent evidence suggests that malnutrition has a negative impact on pulmonary function in children with cystic fibrosis. In the past, dietary management has aimed at high energy low fat intake, but this has recently been shown to fall far short of the 120-150% of the recommended daily allowance for energy cystic fibrosis patients require. This paper outlines the current principles of nutritional management for children with cystic fibrosis. These include a high energy, high fat containing diet (within limits of individual tolerance); high carbohydrate intake; high salt intake; replacement of fat-soluble vitamins; appropriate use of pancreatic enzyme preparations; and supplemental feeding when indicated. It is vital that a nutrition education programme be established for each child and his or her family so that the emphasis shifts from treatment of malnutrition to prevention. The long-term aim must be to promote an independent, healthy lifestyle which incorporates good nutrition and other healthy pursuits such as exercise.