Early lung surveillance of cystic fibrosis: what have we learnt?

Newborn screening (NBS) for cystic fibrosis (CF) provides an opportunity to commence management and therapeutic interventions significantly earlier than indication by symptoms alone. While NBS has provided strong benefits in terms of nutritional status, related improvements in lung health have not been as clear [1]. Defining the natural history of CF lung disease following NBS and identifying the most appropriate outcome measures to track disease progression and to serve as end points for clinical trials are critical for clinical care to maximize the benefit of an NBS diagnosis. The two most prominent early surveillance programs are the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) and the London Cystic Fibrosis Collaboration (LCFC). The LFCF initially recruited clinically diagnosed infants from 1999 to 2002 prior to the introduction of NBS in the UK and a second cohort of infants diagnosed by NBS from 2009 onwards. The LCFC performed lung function at 3, 12, and 24 months with bronchoalveolar lavage (BAL) and chest computed tomography (CT) at 1 year in the NBS cohort [2]. The AREST CF program [3] commenced its early surveillance program in 1999, initially using annual BAL and infant lung function before subsequently expanding to include preschool lung function in 2003 and chest CT in 2005, with bronchial brushing added in 2007 to obtain primary airway epithelial cells. Data from these early surveillance programs have provided important insights into drivers of development and progression of early CF lung disease.