Cystic fibrosis in 2016: considerable progress, but much more to do

Abstract

Research published in 2016 contributed substantially to ongoing efforts to improve outcomes in patients with cystic fibrosis. For the first time, adults with cystic fibrosis now outnumber children; 1 Cystic Fibrosis Foundation Patient RegistryAnnual Data Report 2014. https://www.cff.org/2014-Annual-Data-Report/ Google Scholar this once fatal childhood disease is becoming a chronic disease of adulthood. Sensitive tools are therefore needed to monitor young children with cystic fibrosis and optimise paediatric care, which can in turn improve outcomes in adulthood. Airway disease begins early, as evidenced by inflammation and infection on bronchoalveolar lavage and structural changes on CT scans. 2 Grasemann H Ratjen F Early lung disease in cystic fibrosis. Lancet Respir Med. 2013; 1: 148-157 Summary Full Text Full Text PDF PubMed Scopus (76) Google Scholar However, bronchoalveolar lavage and CT scans are inappropriate for frequent monitoring; instead lung function tests are routinely done. Currently, spirometry is performed on patients with cystic fibrosis aged 4–5 years and older in most clinics; only highly specialised centres do lung function testing in infants because of the technical challenges associated with this method.