Paediatric origins of adult lung disease bullet 7: Cystic fibrosis

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Abstract
Newborn screening for cystic fibrosis has been adopted in some centres as a way of identifying asymptomatic patients and initiating early treatment with the aim of minimising long term changes. Proponents of this method would suggest that this means a much more aggressive approach to management of lung disease in the early years as opposed to patients born in unscreened populations where clinical evidence of respiratory disease is a frequent cause of presentation. The natural extension of this claim would be that early aggressive and often preventive treatment of lung disease will slow the deterioration of lung function in the long term. Waters and colleagues have studied long term lung function in a group of infants born during a period of population based newborn screening and have shown that those with CF born during this period have better lung function and improved growth parameters compared with children with CF born in the same population in the period before the introduction of screening.2 We have recently examined similar data from our clinic but have excluded those patients in the non-screened group who presented before the average age of diagnosis of the screened CF infants.3 We reasoned that those infants would not have benefited from the existence of population based newborn screening as their clinical symptoms led to their diagnosis earlier than would have occurred on the basis of screening. When these early diagnosis infants with CF were excluded from the unscreened group, we found no difference in lung function or nutritional parameters at the age of 9 years between the unscreened (n=29) and the screened (n=31) groups. Further work is required to identify whether population based newborn screening will result in better lung function in later life. While the answer to this question is still unclear when considered on the basis of population based screening, there is no question that limitation of lung disease, particularly in infancy and early childhood, is the major factor in determining the adult consequences of CF lung disease.