Table 2.

 Management of cystic fibrosis lung disease

Disease stage	Pulmonary status	Aim	Management	Comments
Early	Preinfection	Mucus clearance; prevent infection; maintain good lung function	Segregation and cohorting to prevent cross infection*; airway clearance techniques (physiotherapy and adjuncts, mucolytics such as rhDNase,†10 hypertonic saline†11); prophylactic antibiotics (usually against Sa†12; most commonly flucloxacillin or co-amoxiclav in UK); influenza vaccination usually recommended	Segregation of patients with organisms such as Bcc or epidemic strains of Pa is common (practice more variable with regard to other strains of Pa, Sm, or Hi); for both rhDNase and hypertonic saline evidence favours short to medium term benefit (no long term or survival data); prophylactic antibiotics decrease incidence of infection with Sa (long term benefits not well defined); increase in infection with Pa seems limited to trials including broad spectrum cephalosporins
	Intermittent isolation of organisms	Eradication of infection	Pa eradication protocols† include both topical (nebulised) and systemic (usually oral ciprofloxacin)	Eradication achieved in 80-90%,13 but uncertain long term benefit
Intermediate	Chronic infection with usual organisms (Pa, Sa, Hi)	Suppression of bacterial load and thus limitation of inflammatory response	Depends on organism (Pa: nebulised tobramycin or colomycin)	Pa: medium term benefit,† uncertain effects on survival; new, faster nebuliser devices (such as e-flow and iNeb) available
		Treat infective exacerbations	Oral or intravenous antibiotics appropriate for culture	Elective v symptomatic use*
		Reduce inflammation	Ibuprofen*; macrolide antibiotics (azithromycin)†14	Ibuprofen: limited use in much of Europe15 (used more often in US); azithromycin: good evidence for short/medium term benefit, but mechanism of action uncertain (anti-inflammatory properties thought likely); no evidence supporting a role for corticosteroids except in treating allergic bronchopulmonary aspergillosis
	Infection with less common organisms (Bcc, Sm, Ax)	Eradication if early; suppression of bacterial load most commonly	Treat on an individual basis; seek specialist microbiological advice	Confirm diagnosis in a reference laboratory
	Allergic bronchopulmonary aspergillosis	Reduce allergic response; prevent bronchiectasis	Oral corticosteroids; consider addition of an antifungal agent*	Long course often required
	Non-tuberculous mycobacterial infection	Eradication	Usually prolonged combination treatment: ethambutol, rifampicin, azithromycin, amikacin	Can be difficult to determine whether isolates are contributing to disease manifestations; most would treat if recurrent positive cultures
End stage with complications	Severe haemoptysis	Prevent bleeding, which may be fatal	Bronchial artery embolisation (rarely lobectomy)
	Pneumothorax		Drainage. Pleurodesis if persistent/recurrent	May affect suitability for transplantation in future
	Respiratory failure		Lung or heart and lung transplantation16

Bcc=Burkholderia cepacia complex; Pa=Pseudomonas aeruginosa;Sa=Staphylococcus aureus; Hi=Haemophilus influenzae; Sm=Stenotrophomonas maltophilia; Ax=Alcaligenes xylosoxidans.

*Strategies for which consensus is lacking.

†Strategies based on randomised controlled trials or meta-analyses.