Nutritional management in patients with cystic fibrosis

Abstract

Cystic fibrosis is an autosomal recessive disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Nutrition as part of multidisciplinary team, plays a key role in maintaining a good nutritional status, this has been associated with longer survival and a better quality of life. Routine nutritional assessment from diagnosis and for lifetime is necessary. Malnutrition is one of the main problems in patients with cystic fibrosis and it is related to three factors: increased energy metabolism, inadequate caloric intake and energy losses due to malabsorption. Energy, protein and lipid requirements are higher in cystic fibrosis patients than in healthy people. Fat-soluble vitamins supplementation is recommended; other micronutrients supplements such as calcium, iron and sodium may be required. Antioxidants, omega-3 fatty acids and probiotics supplementation is still under study. Exocrine pancreatic insufficiency is present in 80-90% of patients with cystic fibrosis, so pancreatic enzyme replacement therapy is essential; the enzyme dosing is personalized and must be adjusted based on age, nutritional status, and clinical symptoms.