Cystic fibrosis

Advancement in treatment has resulted in a dramatic increase in life expectancy of patients with cystic fibrosis (CF) to well beyond 30 years of age in most centres. What was once a fatal genetic disease in childhood now sees over a third of its CF populations in adult clinics. The improved survival is multifactorial, but most attribute the reasons to more aggressive nutritional care along with better management of the gastrointestinal and pulmonary systems. Many of the nutritional and GI issues of paediatric patients remain similar when they become adults, except that there is the added psychosocial stress associated with this transition. This chapter aims to highlight the gastrointestinal and nutritional issues manifesting at the different stages of life from infancy to adulthood, and the recommended management.