Cystic fibrosis (CF) is the most common inherited life-limiting genetic condition in Caucasian populations and is a chronic debilitating disease that affects around 70,000 individuals worldwide.
Cystic fibrosis is caused by mutation of the gene that encodes for the CFTR membrane protein. CFTR is required for correct ion transport and fluid regulation across the body’s epithelial barriers, such as the linings of the respiratory and gastrointestinal tracts. The mutation leads to abnormal accumulation of thick mucus secretions which can impair pancreatic function, food absorption and reduce lung respiratory function.
At the lung level, the mutation of CFTR induces defective bacterial killing and bacterial clearance. Indeed, the CFTR mutation modifies the natural defenses of the lung against bacteria and the abnormal quantity of mucus creates an ideal environment for bacteria growth. Both factors lead to chronic and recurrent lung infections and a continuous inflammatory state that increases lung injury. CF respiratory infections have such a serious impact on lung function and long term health outcomes for patients that successful treatments or eradication of these microbes (often multi-drug resistant because of long-term exposure to conventional antibiotics) are of prime importance to improving prognosis and quality of life.