For children with sickle cell disease, lung disease is part of the package

ATS 2008, TORONTO—Children with sickle cell disease (SCD) have a significantly sharper decline in lung function with age when compared to other children of the same race and age. Furthermore, that loss of function appears to be linked to a restrictive rather than obstructive pattern, contrary to previous research that has focused on obstructive or asthma-like patterns in loss of lung function with sickle cell disease. The research will be presented at the American Thoracic Society’s 2008 International Conference in Toronto on Sunday, May 18.

“The restrictive pattern of decline is supportive of early injury or inflammation resulting in progressive changes in lung volumes across age,” said lead researcher Joanna MacLean, M.D., of the department of respiratory medicine at Children’s Hospital at Westmead in Australia. “We expected that children with sickle cell disease would show greater loss of lung function than other children, but this had never been quantified, nor was the pattern of decline clear.”

To determine the patterns of loss of lung function, the researchers analyzed 1,357 lung function results that were completed between January 1989 and January 2005 on from 413 children with SCD during routine sickle cell clinical visits. Lung volume measurements were also included for 1,129 records.

They found that over time, there was a significant decline in lung function shown by a decline in the percent predicted values for all spirometry measures except FEV1/FVC ratio, a marker of airway obstruction. The pattern of decline of lung volume confirmed a restrictive pattern with an average loss of 2 percent per year of total lung capacity.