Investigators: Graham Hall, Lidija Turkovic, Stephen Stick, Tim Rosenow, Sally McCappin, Alana Harper, Grace Pettigrew
External collaborators: Harm Tiddens (Erasmus University Medical Centre), Peter Sly (University of Queensland), Robert Trengrove (Murdoch University), Conor Murray (Perth Children's Hospital), Sarath Ranganathan (Murdoch Children's Research Institute)
Advances in our understanding of early cystic fibrosis lung disease led by the Australian Early Surveillance Team for Cystic Fibrosis (AREST CF) have also indicated the potential utility of a number of biomarkers for monitoring disease severity. These include measures of lung structure, lung function and airway inflammation. The gold standard measure for structural disease is chest computed tomography (CT). Radiation exposure limits frequent use of CT to monitor disease, however, recent evidence suggests that important aspects of structure and function be accurately determined using magnetic resonance imaging. The lung clearance index is an important functional measure that appears to be a sensitive marker of lung disease in school age children but relationships to structural changes during an exacerbation have not been investigated.
This study aims to
- Evaluate variability of biomarkers on a time-scale representative of clinical care protocols.
- Assess relationships between MRI (structural damage/inflammation), LCI and intrabreath FOT outputs (ΔR & ΔX) (function), and EBC metabolomics (inflammation/lung damage) during an acute clinical deterioration
- Better understand whether an exacerbation is associated with persistent, unresolved lung damage and/or inflammation when clinical stability is achieved.