[Adults with cystic fibrosis. It's not just about longevity]

Abstract

Cystic fibrosis (CF) is one example of serious disorders for which medical progress and the integration of chronic treatment into the patients' daily routines have led to markedly better longevity. Formerly known as a 'killer disease' of childhood, CF is now considered a disorder with childhood onset, but is well known in adult medicine. Since 2009, for the first time CF adults have made up the majority of patients in the German CF registry. The drawbacks of improved longevity are long-term complications (e.g., CFRD, osteoporosis) that were rarely seen before. In particular, unwanted effects of treatments that today are performed for decades rather than years are becoming pressing problems. Unwanted effects as well as the ever-increasing treatment burden must be carefully weighed against the expected benefits of treatment. However, CF medicine has always been aware that it is not just about longevity, but that prolonged life has to have meaning. Therefore, the marked increase in longevity is also a psychosocial challenge. So far, empirical data suggest that the majority of people with CF courageously struggle for a normal life.