Cellular phenotyping in Cystic Fibrosis to delineate exacerbation frequency in lung disease

Patients with Cystic Fibrosis (CF) lung disease frequently experience episodes of infective exacerbation during which they are treated with antibiotics. The frequency of these exacerbations can vary from individual to individual regardless of age, gender, CFTR genotype and lung bacterial status. People with CF (PWCF) who have a greater frequency of exacerbations experience an accelerated decline in lung function and have an increased 3 year risk of death or lung transplant. Therefore, it is important to try and understand the underlying causes and reasons for increased exacerbation in PWCF. In this study, we will evaluate primary cells from both CF patient cohorts and using a combination of next generation sequencing, pathway analysis and protein analysis, we will identify differences in cellular function between both groups which may help explain exacerbation frequency differences between groups.