62 - The Lung in Sickle Cell Disease

Abstract

Pulmonary problems in children with sickle cell disease (SCD) cause significant morbidity and mortality. Acute chest syndrome (ACS) is the leading cause of death, and recurrent ACS episodes are the major risk factor for sickle chronic lung disease, for which only supportive treatment is available. Whereas adults with SCD tend to have restrictive lung function abnormalities, children more frequently have obstructive abnormalities. Children with SCD, as a consequence of their chronic anemia, have an elevated pulmonary capillary blood volume that significantly correlates with their increased airways obstruction. Whether more aggressive treatment of anemia would improve lung function and long-term outcomes merits testing. Children with SCD experience a decline in lung function that is most rapid in younger children in whom ACS episodes are most common, highlighting the importance of identifying effective strategies to prevent and optimally treat ACS. A physician's diagnosis of asthma has been significantly related to an increased rate of ACS, but whether antiasthma therapy reduces ACS episodes has not been definitively evaluated. Furthermore, wheezing in SCD may be due to a number of causes, and affected children should be appropriately assessed before a diagnosis of asthma is assumed.