Cystic Fibrosis—from Lung Damage to Symptoms

Respiratory symptoms in cystic fibrosis are both local and systemic. The local symptoms include cough, sputum, wheezing, haemoptysis and breathlessness, while systemic symptoms of malaise and fever occur with pulmonary infection. There are also interactions between respiratory and gastrointestinal systems in producing symptoms of malaise and weakness and these also contribute to the secondary psychological and social problems that a number of patients with cystic fibrosis experience. These local respiratory symptoms can be attributed in part to lung damage, but are also a manifestation of the CF defect itself. Similarly, lung damage, allergy, haemodynamic and nutritional changes all contribute to the symptom of breathlessness. Further improvement in symptoms in the future will come not only from limiting the lung damage but also from therapy aimed at reversing the CF defect Itself.