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Existing drug therapies


Existing supportive drug therapies


Cystic fibrosis clearly poses a huge burden to patients and families, both in terms of the life-shortening nature of the disease, the time-consuming treatments prescribed and the ongoing morbidity


Jane C Davies Andrew Bush Paediatric Respiratory Medicine Royal Brompton Hospital, London, UK


The nature of cystic fibrosis requires a co-ordinated approach to management by a multidisciplinary team based in secondary or tertiary care, liaising closely with the local primary care providers. Management strategies in developed countries with a relatively high incidence of the disease are, in general, fairly similar. Standards of Care have been published by Consensus groups and in the UK are updated regularly by the CF Trust (https://www.cysticfibrosis.org.uk/ media/82070/CD_Standards_of_Care_ Dec_11.pdf). Some of the management strategies currently accepted in clinical practice have evolved over decades without being subjected to high-quality randomised, controlled trials (RCTs), although more recently, CF has led the field for high-quality, network-driven clinical trials. There are multiple reviews of the evidence-base on the Cochrane website (http://onlinelibrary.wiley.com/ cochranelibrary/search).


Lung disease Monitoring


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Patient will be seen regularly and undergo physical examination (including chest auscultation, assessment of cough/ huff, clubbing, ENT), lower airway culture (cough/ throat swab, sputum [if able to expectorate; may be induced by nebulised hypertonic saline if not], bronchoalveolar lavage if clinically indicated) and, if old enough, spirometry. This required a forced expiratory manoeuvre and a


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degree of co-ordination, so is difficult for some, in particular, very young children. Most clinics perform a plain chest radiograph annually; some centres advocate routine CT scans, although others consider that the high radiation burden is unacceptable.


Treatment


The aim of treating the lungs varies at different stages of lung disease (Table 1). At all stages, prevention of cross-infection


is key. Patients should not mix with each other and strict infection-control precautions should be employed by all members of the clinical team: (i) Early, presymptomatic stages: the focus here is on mucus clearance and close surveillance for infecting pathogens. Airway clearance techniques should designed and overseen by physiotherapists experienced in suppurative airways diseases and should be tailored to the individual patient and/


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