Clinically Meaningful Interpretation of Pediatric Health-related Quality of Life in Sickle Cell Disease

Abstract

Health-related quality of life (HRQL) measures provide information about disease assessment; however, healthcare providers may be reluctant to use HRQL assessments as scores can be difficult to interpret. We sought to identify levels for impaired pain-related HRQL in children with sickle cell disease (SCD). Children (n=251) completed the PedsQL™ Generic Core Scales and PedsQL™ SCD Module in a multisite study. Using children’s item scores on the Pain and Hurt and Pain Impact scales of the PedsQL™ SCD Module, High, Intermediate, and Low Functioning groups were created. We compared functioning groups to the Pain and Hurt and Pain Impact scale scores to determine levels representing high and low HRQL. These scores were compared to disease severity and the PedsQL™ Generic Core Scales. Scores of 60 or below on the PedsQL™ SCD Pain and Hurt and Pain Impact scales were associated with severe disease and met requirements for impaired functioning on the PedsQL™ Generic Core Scales. Scores of 81 or higher on the Pain and Hurt and the Pain Impact scales can be considered consistent with good HRQL in those domains in SCD. Alternately, scores of 60 or lower are cause for concern and suggest areas of HRQL impairment in SCD.

Mobile health intervention for youth with sickle cell disease: Impact on adherence, disease knowledge, and quality of life

“…These changes would likely increase youth enjoyment of the app, and were also reported by youth receiving chronic transfusions who previ- 32,43 Given the association between HU and QOL, 44 relief from illness-related impact (for example, on functional severity, activity restriction, symptom severity, and pain 45,46 ), and worry, 47-49 the ITP may have both directly and indirectly influenced QOL. Additionally, it is worth exploring whether a bidirectional relationship exists between adherence and QOL; this will facilitate targeted interventions that account for subgroup differences that may influence engagement.…”

Health‐related quality of life in children with sickle cell anemia: Impact of blood transfusion therapy

“…[3] Similar to our previous research, patients were classified as having severe SCA if they experienced additional, more severe complications of the disease – specifically, acute chest syndrome or three or more hospitalizations for painful events in the three years prior to study enrollment. [3, 25] All others were classified as having mild SCA for this analysis.…”

Greater number of perceived barriers to hydroxyurea associated with poorer health‐related quality of life in youth with sickle cell disease

“…A total PedsQL generic HRQL score at or below 69.7 (for child self‐report) and 65.4 (parent‐proxy report) can be interpreted as being at risk for impaired HRQL . PedsQL disease‐specific HRQL scores 60 and lower, particularly for pain‐related subscales, can be interpreted as impaired HRQL, while scores 80 and higher are consistent with good HRQL in youth with SCD …”

“…39 PedsQL disease-specific HRQL scores 60 and lower, particularly for pain-related subscales, can be interpreted as impaired HRQL, while scores 80 and higher are consistent with good HRQL in youth with SCD. 40…”