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Existing drug therapies

Table 1: Treatments for CF lung disease Disease stage Pulmonary status Pre-infection

Aim Early

Mucus clearance Prevent infection


Airway clearance techniques (physiotherapy and adjuncts). These include exercise and mucolytics eg. rhDNase], hypertonic saline, inhaled mannitol)

Segregation and cohorting to prevent cross-infection Prophylactic antibiotics controversial Influenza vaccination

Intermittent isolation of Pseudomonas aeruginosa


Chronic infection with usual organisms (Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae)

Surveillance for and early eradication of infection

Suppression of bacterial load/ limitation of inflammatory response

Most clinics would use combination therapy with nebulised and oral or IV anti- pseudomonal agents. The optimal regime is not clearly identified.

Depends on organism: Pseudomonas aeruginosa: nebulised high dose tobramycin (300 mg bd) or colomycin. Nebulised aztreonam lysine more expensive, but now available, and other nebulised antibiotics are in development. Use the new, faster nebuliser devices for example the e-flow and iNeb, or the newly available dry powder devices

Treat infective exacerbations

Reduce inflammation Oral or intravenous antibiotics Culture results usually guide choice, but no evidence that this improves outcome

No evidence for a role for corticosteroids except in treating ABPA, because of efficacy but adverse side-effect profile (oral) or lack of benefit (inhaled)

Ibuprofen not much used in most of Europe; beware synergistic nephrotoxicity with intravenous aminoglycosides

Azithromycin is useful, but mode of action unknown

Infection with less common organisms (Burkholderia cepacia complex, Stenotrophomonas

maltophilia, Achromobacter xylosoxidans)

Allergic bronchopulmonary aspergillosis (ABPA)

Non-tuberculous mycobacterial infection

Reduce allergic response Prevent bronchiectasis

Eradication or suppression (Mycobacterium abscessus may be very difficult to eradicate)

Lobar or segmental atelectasis (may be seen at any stage of CF)

Late Major hemoptysis Re-inflation of the lung

Oral corticosteroids (long course often required), consider pulsed methyl prednisolone Addition of an antifungal agent common but evidence limited

Infection with these organisms appears to be becoming more common. Diagnosis and management difficult; seek specialist advice, especially for Mycobacterium abscessus infection.

Prolonged courses of multiple chemotherapies will be needed: ethambutol, rifampicin, azithromycin, amikacin, ciprofloxacin, moxifloxacin are among the agents used

Intensive physiotherapy, with rhDNase and hypertonic saline as appropriate Intravenous antibiotics Fibreoptic bronchoscopy, consider endobronchial instillation of rhDNase if conventional management fails

Prevent or halt acute bleeding

Pneumothorax End-stage respiratory failure

Control air leak, prevent recurrence

Optimise conventional treatment

Refer for lung transplant assessment

and did not result in a reduction in the primary outcome, infective exacerbations. However, a previous study from the same group demonstrated an improvement in the sensitive physiological outcome, lung clearance index (LCI), in CF patients with normal FEV1

.2 Recombinant human DNAse is

or carers. At this stage, additional inhaled agents may or may not be used. Hypertonic saline has recently been studied in children under the age of six years1

used in many centres for patients with evidence of more established lung disease, but data clearly demonstrating a reduction in neutrophilic inflammation in very young children receiving this drug support its use at the presymptomatic stage.3 (ii) Early infection +/- prophylaxis: the commonest infecting organism in early CF life is Staphylococcus aureus. Opinions differ globally on the use of prophylactic antibiotics, much of the UK and parts of Europe favouring long-term

prophylaxis with agents such as flucloxacillin/co-amoxiclav, but most of the US avoids such strategies. There are some data supporting a link between the use of broad-spectrum cephalosporins and the acquisition of Pseudomonas aeruginosa, which led to concern, although this has to date not been the case with more specific anti-

staphylococcal agents. A well-conducted, randomised controlled trial is needed to address this fully. Any infection that is 15

Admit for intravenous antibiotics and clotting studies; bronchoscopy not useful. Bronchial artery embolisation for on-going bleeding; can consider the use of tranexamic acid. Lobectomy is a last resort

Conservative management for trivial pneumothoraces, otherwise tube drainage. Early surgery and pleurodesis if does not respond rapidly

Oxygen therapy (no survival benefit demonstrated, unlike for COPD) Consider nasal ventilation as a bridge to transplantation

Eradication if early; suppression of bacterial load most commonly

Confirm diagnosis in a reference laboratory; treat on an individual basis with specialist microbiological advice

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