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Excessive lung inflammation is a major factor in the morbidity associated with cystic fibrosis. Despite the lack of evidence of convincing benefit,1 2 corticosteroids are being increasingly prescribed to children with cystic fibrosis to reduce this inflammation, whilst ibuprofen has also been advocated as a useful anti-inflammatory agent. We wished to ascertain current prescribing practice in the UK, so carried out a postal survey of all specialist cystic fibrosis centres treating children under 18 years of age.
Surveys were returned from all 31 centres that collectively care for around 3500 patients and the results are shown in fig 1. Within centres, regular oral steroids were prescribed to a median (interquartile range, IQR) of 2.5% (0–7%) patients. There was a wide range (0–23%) and in seven centres over 10% of patients were prescribed them. The main indications were allergic bronchopulmonary aspergillosis and asthma/wheezing, although 83% of centres also prescribed them non-specifically for severe lung disease. Inhaled steroids were more commonly prescribed (median 44%, IQR 18–59%) and again there was a marked variation between centres (range 10–93%). Most centres (87%) gave them for troublesome wheezing whilst 65% prescribed them as anti-inflammatory therapy. Only eight centres (26%) reported ever having prescribed ibuprofen for lung disease and in five this had been prescribed to a single patient only. The main reasons given for not using ibuprofen were concerns over safety and practical difficulties with assaying blood levels.
This survey has revealed wide variation in the practice of treating lung inflammation in cystic fibrosis with corticosteroids, whilst ibuprofen is hardly ever used. The figures are similar to those reported by Walters in her survey of adult patients in the UK,3 and is also consistent with data from the (European) Epidemiologic Registry of Cystic Fibrosis which found that 36% of 3433 patients of all ages in the UK were prescribed inhaled steroids.4 Further evidence is needed to remove the uncertainty surrounding the use of anti-inflammatory therapy in cystic fibrosis and this needs to be informed by well defined clinical trials.
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