Grant: Project Grant

Cystic fibrosis (CF) is the most common lethal genetic disease of Canadians. A vicious cycle of lung infection and inflammation develops leading to airways destruction. Understanding these infections and how to effectively treat them is key for those with CF to live longer, healthier lives. Decades of research have focused on treating a few select bacteria infecting CF lungs including; Pseudomonas, Staphylococcus and Cepacia. However, clinicians have long recognized that the response of patients poorly correlate with the presence of these bacteria (patients may fail to respond to antibiotics which kill these bacteria and/or respond to antibiotics which these organisms resist and survive). However, new techniques identifying bacterial DNA have recently demonstrated a large, and diverse collection of other organisms in the CF lung (termed the CF microbiome). Understanding the CF microbiome's role in health, disease and treatment response will enable improved patient outcomes. We propose a unique approach to better understand and treat CF lung disease based on the CF microbiome. To do so, we plan to work backwards to identify factors associated with lung disease progression and treatment response. Our group maintains a massive, one-of-a-kind collection of sputum samples (lung secretions) from individuals with CF spanning twenty years. This "biobank" is linked with patient outcomes. Historical samples can therefore be analyzed to identify factors associating with future outcomes. We aim to determine: -What organisms/populations of organism contribute to disease progression and which are protective. - If established CF treatments act on the microbiome, and whether the microbiome can be used to predict treatment response. Our group of clinician-scientists have the expertise and skill in both molecular biology and outcomes research to take full advantage of this extraordinary resource, and improve our understanding and future management of CF lung disease.