Pulmonary exacerbations in children with cystic fibrosis: risk factors, early detection, and health-related quality of life

Cystic fibrosis (CF) is a chronic genetic disorder with a poor prognosis. This thesis demonstrates that the progression of the pulmonary disease and consequently the disorder itself is determined by exacerbations. The following factors were found to be associated with an increased risk of exacerbations: poorer lung function, previous exacerbations and the use of antacids or prophylactic antibiotics. Exacerbations also adversely affect quality of life. The detection of volatiles in exhaled breath and electronic home monitoring of respiratory tract symptoms and lung function can be useful for recognising exacerbations at an early stage. Combined with early antibiotic treatment, these tools could improve the prognosis and quality of life in people with CF.