Grant: Travel Awards - Institute Community Support

Cystic fibrosis (CF) patients develop thick and sticky mucus in their lungs, which can result in flare-ups of lung infection, known as pulmonary exacerbations (PEx). PEx greatly reduce quality of life for these individuals and are major drivers of health care costs. These PEx are characterized by worsening cough and breathlessness, and are often treated with antibiotics because bacteria are often thought of as the sole culprits. However, conditions such as asthma or fungal infections, which do not respond to antibiotics, can also cause PEx in the CF population. Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity (allergic) reaction to the fungi, Aspergillus fumigatus, and can cause similar signs and symptoms to PEx caused by bacteria. ABPA has been shown to be associated with an increased decline in lung function as measured by FEV1 as well as a risk factor for poorer outcomes after PEx. Furthermore, it is not clear if CF-asthma overlap patients have worse PEx outcomes as making this diagnosis is also problematic. Up to a quarter of patients experience permanent loss of lung function even after treatment of a PEx with antibiotics, suggesting that our current approach to the diagnosis and treatment of these flare-ups are not optimal, and that treating the inflammation might be more appropriate. CF patients with ABPA and/or asthma could benefit from systemic corticosteroids alone (instead of antibiotics) or in addition to antibiotics to improve their response to PEx treatment. The diagnosis of ABPA and asthma in individuals with CF is difficult because of shared symptoms, however, ABPA specifically, has been associated with accelerated lung deterioration, so timely diagnosis and treatment is essential. Therefore, the objective of this study is to identify markers of fungus-induced lung inflammation in the blood of CF patients.