Grant: Project Grant

Cystic fibrosis (CF) is the most common lethal genetic disease of Canadians. In CF, a vicious cycle of infection and inflammation leads to lung destruction. Understanding how this process contributes to disease progression is key for those with CF to live longer, healthier lives. Cytomegalovirus (CMV) causes a chronic viral infection in humans. The immune system acts to control CMV but it is never cleared from the body. Its prevalence increases over time and >50% of adults have evidence of CMV infection. CMV reactivation and invasion in to various organ systems causes tremendous disease specifically in immune-suppressed individuals. However, an increasing body of literature has demonstrated that CMV may have negative consequences even in general populations. CMV positive individuals have increased rates of chronic inflammatory diseases including cancers, dementia and heart disease. Indeed, CMV positive individuals live shorter lives. How this occurs is unknown - but it is suspected that CMV replicates in already inflamed tissues - exaggerating resultant tissue damage. CF, a disease of chronic infection and inflammation, is a perfect candidate for exploitation by CMV. Provocative preliminary data from the University of Calgary has confirmed the presence of CMV in the lungs of individuals with CF and that CMV positive individuals progress to end-stage lung disease ten years earlier then CMV negative. We propose a multi-centre observational study in several leading Canadian CF centres to determine the role of CMV in CF disease progression. In this study we will determine; the rates and risk factors for CMV infection, correlate disease burden and progression rates with CMV status, and identify how CMV reactivation associates with this process. As potent therapies and vaccines to treat and prevent CMV infections exist - our study has the potential to provide immediately actionable information and improve the quality of life of those living with CF.