Cystic fibrosis (CF) is an autosomal recessive hereditary disease caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Mutations of CFTR lead to non- or mal-function of all exocrine glands and mucosal surfaces of the human body. Thus, the disease affects various organs including intestine, pancreas and lung (1). Life expectancy of CF patients is severely decreased and this is nowadays mainly dictated by the pulmonary phenotype: CF patients suffer from thickened respiratory mucus causing mucus plugging of the airways, chronic inflammation as well as increased incidence of pulmonary bacterial infections. Infections are of polymicrobial nature yet H. influenzae, S. aureus, and P. aeruginosa are clinically important pathogens in CF lung disease (2). Bacterial airway infection and inflammation associated with reduced mucociliary clearance mediate progressive lung damage and a decline in lung function over time, finally resulting in death due to respiratory failure.