Early detection of lung disease in cystic fibrosis (CF), combined with aggressive treatment in infants, may be the key to controlling the progression of the disease, according to a recent study. New research shows that contrary to previous scientific opinion, progressive lung damage in CF patients can begin as early as infancy even though lung function shortly after diagnosis is normal.
"We might be able to stop some of the lung function reduction we're identifying in the first months of life," said Dr. Sarath Ranganathan, Ph.D., consultant respiratory physician at the Royal Children's Hospital in Melbourne, and lead author of the paper, which appeared in the second issue for December of the American Thoracic Society's American Journal of Respiratory and Critical Care Medicine.
"We don't know what's going to work, but we have to target those patients in the first six months of life if we're going to be effective," added Dr. Ranganathan.
In the Australian study, 68 infants with CF were compared to 49 infants without the disease. The children were between six weeks and 30 months of age. Forced expiratory volume (FEV) measurements were obtained for the children at baseline, and 16 of the children with CF were measured again one year later. FEV was equivalent for all children both with and without CF at baseline. But by six months of age, the mean FEV score was significantly lower in infants with CF compared to controlsand the deficit increased with each month of age. "This finding indicates that lung function declines sooner than previously thought," said Dr. Ranganathan.
The study also found that diminished lung function occurred even in the absence of clinical symptoms and irrespective of CF genotype. But, notably, infants measured soon after birth and within the first six months of life had normal lung function. Newborn screening for CF has been in existence in Australia for nearly 30 years, but was introduced only r
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American Thoracic Society