ABSTRACT
I. Introduction Patients with cystic fibrosis (CF) experience a broad range of pathophysiological and medical conditions, especially those who have CF with pancreatic insufficiency (PI). Most therapies focus on the unrelenting lung disease and loss of pulmonary function. In addition, the importance of nutrition in CF care has been established. Nutritional care provided by the CF team is a major factor in selecting the amount and type of food and specific nutrients to support optimal growth (weight, stature), development (lean body mass, fat stores, peak bone mass, puberty, reproduction in females), micronutrient status [fat-soluble vitamins, other micronutrients, essential fatty acids (EFAs)], and modulate immune and inflammatory status. Research, clinical experience, pancreatic enzyme replacement therapy (PERT) and vitamin, mineral and enteral nutritional supplements have lead to major improvements in the nutritional status of children and adults with CF. However, the result is far from optimal-children still do not grow to their genetic potential, suboptimal nutritional status is known to contribute to the pace of lung function loss, and adults with CF are underweight and have complications such as osteoporosis and reduced work and fitness capacity in excess of the degree of lung disease.
