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Clinicians caring for children with cystic fibrosis (CF) should now take note; despite your best clinical care, lung disease in CF infants develops surreptitiously and ill-defined by early symptoms. By the time lung disease has symptomatically declared itself, it's probably structurally established and too late to reverse. What you don't know can hurt you.
Hoo and colleagues in this issue provide evidence of early lung disease in patients with CF diagnosed by newborn screening.1 At a mean 3 months of age, 34% of CF patients had abnormalities of pulmonary function (including lung clearance index (LCI) and/or forced expiratory volume in 0.5 second (FEV0.5)) when compared with healthy controls. The presence of symptoms, sometimes aggressively treated, did not reliably identify those with abnormal pulmonary function. This study, by the London CF Collaborative, used contemporaneous healthy controls and adhered to treatment protocols from diagnosis. The research examines with great clarity the vital question of how early lung disease starts in those born with CF. The children studied were provided with every opportunity for optimal health, by the use of newborn screening, regular chest physiotherapy and prophylactic oral flucloxacillin. Disappointingly, our current best care is not good enough to prevent lung disease. Of even greater disappointment is that those with lung disease do not stand out from the crowd.
This is not isolated data. Studies over the past decade have shown that lung disease is established very early in life with bronchiectasis and ventilation inhomogeneity preceding both symptoms and a notable decline in FEV1. Children …
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