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The relation between health-related quality of life, treatment adherence and disease severity in a paediatric sickle cell disease sample


Brian Fisak Jr, PhD, Department of Psychology, University of North Florida, 1 UNF Drive, Jacksonville, FL 32224, USA. E-mail:


Background  The purpose of this study was to provide a comprehensive examination of the potential factors associated with health-related quality of life (HRQOL) in a paediatric sickle cell disease sample.

Methods  Caregivers of 78 paediatric sickle cell disease patients completed surveys, which included measures of HRQOL, treatment adherence and barriers to treatment adherence. Medical chart reviews were also conducted to assess disease severity.

Results  Barriers to treatment adherence and pain crisis frequency were found to be the most robust factors associated with HRQOL.

Conclusions  This study provides insight into the factors related to paediatric sickle cell disease and informs both clinicians and researchers interested in understanding and improving HRQOL in this population. In particular, the reduction of pain crises frequency and barriers to adherence may be particularly effective strategies to improve HRQOL.