Overall survival has improved dramatically for patients with cystic fibrosis (CF) since the millennium such that median life expectancy now extends into the fifth decade. However, with increasing longevity comes a range of comorbidities, some as complications of the disease itself but others as a consequence of long-term treatments required to manage CF. Chronic infection and inflammation eventually cause irreversible bronchiectasis and decline in pulmonary function, and 90% of CF deaths are caused by progressive respiratory failure.