Cystic fibrosis (CF) is a genetically inherited multi-system disorder that primary affects the lungs and the digestive system. Ireland has the highest incidence of CF in the world. The main contributor to morbidity and mortality in CF is lung disease, which is characterised by persistent inflammation of a specific type of immune cells called neutrophils. Matrix Metalloproteases (MMPS) are a group molecules that speed up the breakdown of proteins which provide structural and biochemical support to neighbouring cells, extracellular proteins. There is emerging evidence that MMPs that could play a role in CF lung disease and impact patient outcomes. The CF-ABLE score is an clinical scoring system, validated by the Cystic Fibrosis Registory of Ireland, that predicts the risk of death or requirement for lung transplantation in patients which CF by using commonly available information such as age, BMI, etc. This study aims to further validate the CF-ABLE score by demonstrating a relationship between CF-ABLE score and a series of MMPs in both airway secretions and plasma.
