Improved survival has been associated with better nutritional status in patients 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 weight, compared to healthy individuals but were of similar height. Measurements of lung function, FEV1 and FVC were significantly related to body mass index. Lung function was poorer in patients with chronic pseudomonal
infection but
body weight and body mass index were not significantly different compared 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 significant 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
transplantation 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 feeding, 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 under weight and declining health is associated with significant
weight loss. In patients with severe
malnutrition significant improvement can be achieved by
enteral feeding.