Outlook Improving for Kids with Sickle Cell Disease

Children of African descent can be prone to sickle cell disease, an inherited disorder in which red blood cells become misshapen and cause painful, sometimes fatal, complications.

Children of African descent can be prone to sickle cell disease, an inherited disorder in which red blood cells become misshapen and cause painful, sometimes fatal, complications.While the disease cannot yet be cured, a new study finds that fewer children are dying from sickle cell disease, the average age at death is increasing, and infection-related deaths have decreased.”Research developments during the past several decades have improved the lives of many persons with sickle cell disease,” study co-author Dr. George R. Buchanan, from the University of Texas Southwestern Medical Center at Dallas, said in a statement. “Yet, the true impact of these investigations on survival of children and adolescents with sickle cell disease has not been clear until now.”The findings, which are reported in the medical journal Blood, are based on a study of 711 Dallas-area children with sickle cell disease who were followed for an average of about 7 years.Twenty-five deaths occurred during that period. This translated to a survival rate of 85.6 percent at 18 years of age, the investigators note. In contrast, 30 years ago, only about one half of children with sickle cell disease were expected to reach this age.The average age of death in the current study was 5.6 years, and five of the children who (20 percent) succumbed to infection. By comparison, in a 1994 the average age of death was between 1 and 3 years, and 50 percent of deaths were due to infection.”This work gives us contemporary and accurate data confirming the success of our research,” Buchanan noted. “It was only a half-century ago that very few persons with sickle cell anemia and related conditions survived beyond 21 years of age,” he added.(Source: Blood, Reuters Health, April, 2004.)