How to manage advanced lung disease in cystic fibrosis

Patients with cystic fibrosis (CF) who have advanced lung disease require aggressive therapy to maximize lung function and minimize pulmonary complications, which include colonization with resistant organisms, allergic bronchopulmonary aspergillosis, hemoptysis, and pneumothorax. In addition to traditional manual percussion, methods of enhancing airway clearance include a high-frequency oscillatory device and devices that create resistance to exhalation or provide positive expiratory pressure (PEP) and percussive ventilation or vibratory PEP. Mucolytic agents have been a mainstay of therapy. More recent advances include recombinant human deoxyribonuclease and inhaled tobramycin, which can decrease the rate of decline in lung function and prevent pulmonary exacerbations. The use of azithromycin as maintenance therapy for patients with CF is becoming more widespread because of its combined antibacterial and anti-inflammatory effects.