Wasting as an independent predictor of mortality in patients with cystic fibrosis

Background-Cystic fibrosis (CF) is the most common life threatening autosom al recessive disorder in the white population. Wasting has long been recogn ised as a poor prognostic marker in CF. Whether it predicts survival indepe ndently of lung function and arterial blood gas tensions has not previously been reported. Methods-584 patients with CF (261 women) of mean (SD) age 21 (7) years were studied between 1985 and 1996, all of whom were being followed up in a ter tiary referral centre. Lung function tests, body weight, arterial blood oxy gen (Pao(2)) and carbon dioxide (Paco(2)) tensions were measured. The weigh t was calculated as a percentage of the ideal body weight for age, height, and sex. Results-Forced expiratory volume in one second (FEV1) recorded at the start of the study was 1.8 (1.0) 1 (52 (26)% predicted FEV1), Pao(2) 9.8 (1.9) k Pa, Paco(2) 5.0 (0.9) kPa, and % ideal weight 92 (18)%. During the follow u p period (45 (27) months) 137 patients died (5 year survival 72%, 95% CI 67 to 73). FEV1, % predicted FEV1, Pao(2), % ideal weight (all p <0.0001), an d Paco(2) (p=0.04) predicted survival. In multivariate analysis, % predicte d FEV, (p <0.0001), % ideal weight (p=0.004), and Paco(2) (p=0.02) were ind ependent predictors of outcome. Patients with > 85% ideal body weight had a better prognosis at 5 years (cumulative survival 84%, 95% CI 79 to 89) tha n those with less than or equal to 85% ideal weight (survival 53%, 95% CI 4 5 to 62), p <0.0001. Percentage predicted FEV, (area under curve 0.83; 95% CI 0.78 to 0.87) and % ideal weight (area under curve 0.74; 95% CI 0.68 to 0.79) were accurate predictors of survival at 5 years follow up (receiver-o perating characteristic analysis). Conclusions-Body wasting is a significant predictor of survival in patients with,CF independent of lung function, arterial blood oxygen and carbon dio xide tensions.