Nutrition in adults with cystic fibrosis

Improved survival has been associated with better nutritional status in pat ients with cystic fibrosis (CF). In this study we examined the relationship between nutritional state and other measures of clinical severity in adult patients with CF, attending a regional centre. Eighty-one patients (median age 21 years) were studied. Patients with CF were significantly under weig ht, compared to healthy individuals but were of similar height. Measurement s of lung function, FEV1 and FVC were significantly related to body mass in dex. Lung function was poorer in patients with chronic pseudomonal infectio n but body weight and body mass index were not significantly different comp ared to those without such infection. In 53 patients who were alive 4 years later, FEV1 had declined by -10.5 (2.1)% (P < 0.001) but there was no sign ificant change in body weight 1.5 (6.5) kgs. In 23 patients who died or had lung transplantation the change from 1994 to the date of death or transpla ntation the FEV1 was reduced by -7.9 (11.2)% (P = 0.004) and body weight -2 .8 (4.4) kgs (P < 0.01). In 12 patients who had supplemental enteral feedin g, the median increase in body weight was 7 kgs over a period of 12 months. This study confirms that young adult patients with CF are significantly un der weight and declining health is associated with significant weight loss. In patients with severe malnutrition significant improvement can be achiev ed by enteral feeding.