Nutrition in Cystic Fibrosis

Cystic fibrosis is the most common life shortening autosomal recessive disease among Caucasian populations which involves different organs.Growth failure is common in cystic fibrosis children and adolescents.Malabsorption, maldigestion and increase nutritional need are the combination causes of growth faltering in CF patient.Early recognition and intensive treatment of malnutrition can minimize damaging effect of it on lung disease and quality of life.Pancreatic insufficiency is an important contributor to malnutrition in most CF patient.Pancreatic insufficient patients should consume a high-calorie diet with unrestricted fat with PERT therapy that is appropriate to age and clinical status. Enzyme dosing may be done either by grams of fat ingested or by weight. Dosing by grams of fat is more likely to mimic the normal pancreatic response to a meal.Infants generally require 450-900 lipase units/g of fat, or 2,000-4,000 lipase units per 120 ml of formula or when breastfeeding and Older children and adults generally require 500-4,000 lipase units per gram of fat ingested or 500-2,500 lipase units/kg/meal, 250-1,250 lipase units/kg/snack.Other factors such as high dose target of fat vitamins, sodium abnormality, CFRD, CFLD, inflammation and importantly, chronic and progressive pulmonary infection, can effect on growth pattern of patient.According to this, multi-disciplinary team work management consist of pulmonologist, gastroenterologist and nutritionist is essential for the best result.

Keywords:

Cystic fibrosis , Malnutrition , PERT