Severe advanced lung disease in cystic fibrosis

Severe advanced lung disease in cystic fibrosis (CF) occurs when progressive lung injury leads to a reduction in forced expiratory volume in 1 second (FEV₁) below 30%. This is often associated with marked exertional dyspnoea and hypoxaemia at rest, with frequent cough and copious sputum production.

Patients with severe advanced lung disease in CF are closely managed by their specialist CF centre. Therapeutic options include supplemental oxygen, noninvasive ventilation and lung transplantation.