Quality of Life in Paediatric Sickle Cell Anaemia

Objectives. The Quality of Life (QoL) of children with Sickle Cell Anaemia (SCA) in the UK has not been examined, and a discrepancy measure based on Gap theory has rarely been used. This study investigated whether 1) child self-reports of QoL using a discrepancy measure (the Generic Children’s QoL Measure; GCQ) are lower than those from healthy children, 2) proxy reports from parents and healthcare professionals are lower than child self-reports, and 3) demographic and disease severity indicators are related to QoL. Design and Methods. An inter-dependent groups cross-sectional design was implemented. Seventy-four children with SCA, their parent and members of their healthcare team completed the GCQ. Demographic and disease severity indicators were recorded. GCQ data from healthy children were obtained from the UK Data Archive. Results. Contrary to past research, when examining generic discrepancy QoL, children with SCA did not report lower QoL than healthy children, and parentand healthcare professional proxy reports were not lower than child self-reports. Few of the demographic and disease severity indicators were related to QoL. Conclusions. Proxy reports may be used to gain a more complete picture of QoL but should not be a substitute for self-reports. The explanation for the relatively high levels of QoL reported is not clear, but children with SCA may have realistic expectations about their ideal self, place greater emphasis on aspects other than health in shaping their QoL, and define achievements within the limits of their illness. Future research should focus on psychological factors in explaining QoL.