Nutritional care in children with cystic fibrosis: Are our patients becoming better?

Abstract

Over the last 30 years, major advances have occurred in our understanding of the disorder cystic fibrosis (CF) with the discoveries of the underlying chloride transport defect and the 'CF gene', the CF transmembrane conductance regulator gene. Equally important from a clinical and patient perspective are the improvements in median survival from less than 10 to 20 years prior to 1980, approaching 30 years during the 1980s and over 45 years more recently. Improved antibiotic regimens and lung clearance therapy contributed to the enhanced survival, but a key factor accredited as adding a further 10 years to the median survival was improving and then maintaining normal growth and nutrition. In the main, the latter were achieved by adherence to a 'high-fat high-energy' diet rather than the advocated virtually universal policy of the 'low fat', which was associated with wasting and linear growth failure. The high-fat diet in conjunction with better control of malabsorption due to microspheric pancreatic enzyme replacement therapy, attention to adequate fat-soluble vitamin supplementation and newborn screening has ensured that at least 80-90% of children with CF will achieve better health and survival through their adult years. © 2013 Macmillan Publishers Limited.