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J Pediatr Hematol Oncol. 2018 Nov 28. doi: 10.1097/MPH.0000000000001363. [Epub ahead of print]

Disease Self-Efficacy and Health-Related Quality of Life in Adolescents With Sickle Cell Disease.

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Division of Pediatric Psychology and Neuropsychology, Nationwide Children's Hospital.
Department of Pediatrics, The Ohio State University, Columbus.
Division of Behavioral Medicine.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH.
Division of Hematology, Cincinnati Children's Hospital Medical Center.


Adolescents and young adults (AYA) with sickle cell disease (SCD) are at risk for poor health-related quality of life (HRQOL). Research suggests that vulnerability factors (eg, disease severity) and self-management resources (eg, disease self-efficacy) jointly impact health outcomes, including HRQOL; however, this has not been studied among AYA with SCD. This study examined the relationship between disease self-efficacy, HRQOL, and disease severity in AYA with SCD. HRQOL was positively correlated with disease self-efficacy and negatively correlated with disease severity. Disease self-efficacy and severity accounted for 35% of variance in HRQOL. Findings support the impact of disease self-efficacy on HRQOL.

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