Grant: CIHR Fellowship

Cystic fibrosis (CF) is a genetic disorder that primarily affects the lungs and the digestive system, but lung infections are the leading cause of deterioration in health and of death. These infections are characterized by a massive influx of white blood cells and the attendant inflammation they provoke. Chronic inflammation damages and obstructs the airways and eventually claims the life of most patients. Burkholderia cepacia complex (BCC) bacteria are the most dangerous infectious agents in CF and can initiate the most profound pulmonary inflammation in this disease. CF patients show wide variation in clinical outcome to BCC infection from benign course to rapid clinical deterioration with bloodstream invasion and death. The proposed study investigates the role of "inflammasomes" or regulators of inflammation in CF lung disease in response to BCC infection. A link between severity of CF lung disease and inflammasome function will be investigated as well as the bacterial components responsible for inflammasome activation. By studying the regulation of inflammation, our research will lead to a better understanding of the inflammatory process and how this may affect lung disease course in CF patients. Identifying sources of disease variation is crucial to improving therapies and care regimens for individuals with CF.