LONG-TERM SURVIVAL AND NUTRITIONAL DATA IN PATIENTS WITH CYSTIC-FIBROSIS TREATED IN A DANISH CENTER

Background - Adequate nutrition and optimal treatment of bronchopulmon ary infections are both of critical importance in maintaining the heal th of patients with cystic fibrosis. The cystic fibrosis centre in Cop enhagen has followed a regimen of very early and aggressive antimicrob ial treatment, especially against Pseudomonas aeruginosa infection. An unrestricted diet of low fat and high protein without hyperalimentati on was recommended before 1985 which was then changed to a high fat, h igh calorie intake. Methods - The overall impact of the treatment regi men was evaluated by a cross sectional analysis of all 223 patients wh o attended the centre in 1989. Growth and nutritional parameters were combined with lung function parameters and with a retrospective analys is of chronic P aeruginosa infection and its duration. Survival curves for all 313 patients treated at the centre since 1949 were calculated . Results - All the patients with cystic fibrosis had normal height, a lthough the final height was achieved a little later than in healthy c ontrols. Body weight was lower than normal in males above 15 and in fe males above 10 years of age. The body mass index (BMI), which was appr oximately 98% of normal in the younger patients, declined to 90% in ad ult men and to 83% in adult women with cystic fibrosis, and was strong ly correlated with lung function parameters. in 1989 the median age of survival of all patients treated in the centre since 1949 was 30 year s (32 years in males and 29 years in females). Conclusions - The overa ll treatment regimen in the cystic fibrosis centre in Copenhagen is as sociated with growth and survival rates that are at least equal to tho se in other cystic fibrosis centres in other countries.