Abstract
Introduction: Survival of cystic fibrosis (CF) patients is better with improved treatment options. However, it may be very different in individual patients because of a variable clinical course.
Therefore, models for predicting the risk of death and decline of lung function are studied, especially with respect to lung transplantation timing. Methods: Factors affecting survival were evaluated in a 10-year study of adult patients with CF and pancreatic insufficiency treated at the Department of Pneumology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital in Motol, the Czech Republic.
The studied parameters included chronic airway infection, lung function, Northern chest X-ray score and Shwachman-Kulczycki clinical score, exercise tolerance and nutritional status. Results: Fifty-eight subjects with a mean age of 25.3 +- 4.4 years were included in the study.
Infection with Burkholderia cepacia complex was present in 30 cases and represented the main risk factor of death (p < 0.001). The median survival time was 9.9 years, with its main predictor being the FEVi value (p = 0.007).
Conclusions: The study confirmed infection with B. cepacia complex as the most important risk factor of death and the FEVi value as the main predictor of survival in adults with CF.