Due to the remarkable advances in the care of children with cystic fibrosis (CF) over the past 3 decades, the traditional pulmonary function tests in most paediatric CF patients are normal. Despite this, we know that there is structural damage, inflammation and infection in the lungs of these children from an early age. In addition, new medical therapies for CF are leading to a monumental shift in how this disease is treated -- but again, if traditional pulmonary function testing is "normal" at the onset of therapy, it will not be sufficient to monitor response to treatment. For these reasons, new markers of lung health and disease are needed in this population. My research interests focus on the development of two types or pulmonary biomarkers: pulmonary function and imaging.
Multiple breath washout (MBW) testing is an old technique that has re-emerged in the literature over the past decade because it is extremely sensitive to the small airways disease that we see in early CF lung disease. There is, however, much that we don't know about this testing modality: how can/should it be used in a clinical setting? What constitutes a significant change between visits? Should a significant change in MBW outcomes prompt a change in clinical management? Through industry-initiated clinical trials and investigator-initiated studies, we hope to address these questions about this exciting new test.
Magnetic responance imaging is traditionally ineffective for imaging lung tissues, to its low proton density and high number of air/tissue interfaces. Novel techniques such as ultrashort echo time (UTE) MRI are improving the spatial resolution of pulmonary MRI, and are now being tested in children with CF. In addition, gas contrast imaging of the lungs using inhaled hyperpolarized noble gases (helium or xenon) allows for functional imaging. In collaboration with partners at UBC and St Paul's Hospital, I hope to develop a pediatric lung imaging research group at BCCH to use these emerging technologies in the investigation and care of children with lung disease.