Grant: CIHR Fellowship

Cystic fibrosis (CF) is the most common fatal genetic disease and 1 out of every 3600 infants in Canada is born with the disease. Due to a genetic abnormality, CF individuals develop thick and sticky mucus in their lungs, which makes them vulnerable to repeated lung infections. Flare-ups of lung infection adversely impact on the quality of life of CF individuals and are responsible for most of the morbidity and costs related to CF care. Based on 2009 Canadian CF patient registry statistics, CF individuals spent over 20,000 days in hospitals mainly due to flare-ups of lung infection. Treatment of lung infection flare-ups in hospital involves the administration of intravenous antibiotics and chest physiotherapy. Most individuals are hospitalized for 10 to 21 days and decisions about treatment response are often guided by a global assessment of improvement in patient symptoms and/or lung function. Unfortunately, this assessment can be insensitive and unreliable, providing the motivation to search for better markers of treatment response. We believe one way to overcome these limitations is to develop a companion blood test that can assist clinicians in treatment decisions by separating out those patients that are not responding to therapy so that treatment can be modified earlier, and by separating out those that are responding to therapy so treatment can be stopped earlier. This will enable more rational and timely use of scarce resources and avoid unnecessary risk of side effects from drugs. The important information gathered in this preliminary study will help in the design of future studies using blood tests to monitor and/or predict treatment response, with the ultimate goal of improving the overall health and quality of life of people with CF in Canada.