Physical training: vital for survival and quality of life in cystic fibrosis

Cystic fibrosis (CF) is a progressive, autosomally inherited, multi-organ system disease that requires qualified care by a multidisciplinary team [1–3]. Many patients suffer from voluminous pulmonary hypersecretion with chronic airway infection and inflammation, which, without adequate treatment, cause pulmonary cysts, bronchiectasis and fibrosis that sooner or later lead to lethal respiratory insufficiency. The cause of death in CF is respiratory insufficiency in ,85%. Continuous problems, such as poor nutritional status and suboptimal treatment in general, influence the course. CF used to be a paediatric disease, but nowadays patients survive well into adulthood and have the opportunity to live a life with good quality [1, 4]. 10 years ago, the mean annual mortality rate in the Swedish CF population from 1991 was 0.9% for females and males (fig. 1) [5]. The corresponding median age at death during this period was 26 years (fig. 1) [5]. Due to the fact that fewer CF patients die early in life, making long-time survival estimates is increasingly difficult. According to a Kaplan–Meier analysis, the predicted survival in the Swedish population born during the 1990s was estimated at 95% reaching o25 years of age (fig. 2) [5]. In that study, 75% of all Swedish CF adults who had finished educational studies were working and Statement of interest None declared.