A Prospective Investigation of Pain And Development Effects On The Health-related Quality of Life of Children With Sickle Cell Disease

Sickle cell disease is associated with numerous morbidities in childhood that may impair health-related quality of life (HRQL) (Gill, et al., 1995; Pediatrics, 2002). Previous studies of HRQL have demonstrated the utility of measures of HRQL in capturing patient perspectives of SCD (Dampier, et al., 2010; Panepinto, Pajewski, Foerster, & Hoffmann, 2008); however, much remains to be learned with regard to the responsiveness of these measures to changes in HRQL over time as well as specific factors that predict HRQL in children with SCD. The present study addressed the effects of pain and development on HRQL over time. The aims of the study were as follows: a) examine the responsiveness of a measure of HRQL, the Pediatric Quality of Life Inventory (PedsQL), to pain-related changes in HRQL over time and b) examine evidence for a general developmental decline in HRQL in children with SCD. The findings suggested that the PedsQL is responsive to pain-related changes in HRQL over time using caregiver report. The findings also supported relationships between age and physical and overall HRQL, but did not provide consistent support for a developmental decline. Limitations in the interpretation of these results, conclusions, and future directions were also described. This information is of central interest to interventionists who require measures that are responsive to changes in HRQL associated with SCD-related medical complications as well as clinicians who seek to monitor and understand changes in HRQL in pediatric patients