Cystic fibrosis revisited

Cystic fibrosis (CF) is characterized by a progressive decline in pulmonary function secondary to chronic lung infections, exocrine pancreatic insufficiency leading to malnutrition, liver disease, and growth impairment. Pathological, functional, and imaging studies all support the presence in early life of significant abnormalities in the small airways in individuals who have CF.[1] CF lung disease can vary widely in its severity and symptoms and can mimic other diseases such as asthma or bronchitis, making diagnosis and management challenging.[2] The natural history of CF lung disease is one of chronic progression with intermittent episodes of acute worsening or “pulmonary exacerbations.” These impact on survival in CF, reduce health-related quality of life due to hospitalization, and increase health costs.[3]