Mortality in children and adolescents with sickle cell disease

• Published in: Pediatrics (Evanston) Vol. 84; no. 3; pp. 500 - 508
• Main Authors: LEIKIN, S. L, GALLAGHER, D, KINNEY, T. R, SLOANE, D, KLUG, P, RIDA, W
• Format: Journal Article
• Language: English
• Published: Elk Grove Village, IL American Academy of Pediatrics 01-09-1989
• Subjects: Anemias. Hemoglobinopathies; Biological and medical sciences; Diseases of red blood cells; Hematologic and hematopoietic diseases; Medical sciences; Mortality; Patient outcomes; Pediatric diseases; Prognosis; Sickle cell anemia; Sickle cell anemia in children; Human; Hemoglobinopathy; Sickle cell anemia; Prognosis; Follow up study; Mortality; Adolescent; Hemopathy; Child; Statistical study
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.84.3.500

A study of the natural history of sickle hemoglobinopathies was begun in March 1979. By August 1987, a total of 2824 patients <20 years of age were enrolled. There have been 14 670 person-years of followup. Seventy-three deaths have occurred. Most of the deaths were in patients with hemoglobin SS. The peak incidence of death was between 1 and 3 years of age, and the major cause in these young patients was infection. Cerebrovascular accidents and traumatic events exceeded infections as a cause of death in patients > 10 years of age. There was limited success in identifying risk factors for death. Comparison of this study's overall mortality of 2.6% (0.5 deaths per 100 person-years) with previous reports indicates improvement of survival in US patients <20 years of age with sickle hemoglobinopathies. This improvement is most likely due to parental education and counseling about the illness and the early institution of antibotics in suspected infections.