Carlton Dampier|Susan Lieff|Petra LeBeau|Sang Youl Rhee|Marsha McMurray|Zora R. Rogers|Kim Smith?Whitley|Winfred C. Wang
Health?related quality of life in children with sickle cell disease: A report from the Comprehensive Sickle Cell Centers Clinical Trial Consortium
Emory University|Rho (United States)|Rho (United States)|Rho (United States)|Rho (United States)|The University of Texas Southwestern Medical Center|Children's Hospital of Philadelphia|St. Jude Children's Research Hospital
pediatric healthrelated quality life hrqol questionnaires validated children sickle cell disease scd small sample sizes studies limited clinical comparisons used baseline clinical data collaborative data cdata project comprehensive sickle cell centers cscc clinical trial consortium perform detailed descriptive study hrqol using pedsql version 40 generic core fatigue scalesretrospective clinical data obtained via medical record abstraction staffadministered health history psychosocial health behavior interviews completed parent guardian pedsql forms completed separately child parentguardianthe study enrolled 1772 subjects 53 boys mean age 96 years sd 47 ss sbeta0 thalassemia occurred 68 32 sc sbeta thalassemia occurrences pain priapism avascular necrosis hipsshoulders avn asthma common complicationsconditions reported multiple regression models controlling hemoglobinopathies gender age suggested parent reports physical functioning sleeprest fatigue declined response pain avn school functioning scales declined response pain asthma sickle pain lesser extent asthma negatively influenced child reports almost functioning fatigue scaleswhile longitudinal studies necessary determine sensitivity change current study suggests potential utility several pedsql hrqol scales patientreported outcome measures observational interventional treatment studies children adolescents scd