Psychology Department, Middlesex University, London, UK.
Br J Health Psychol. 2015 May;20(2):290-304. doi: 10.1111/bjhp.12099. Epub 2014 Apr 23.
The quality of life (QoL) of children with sickle cell anaemia (SCA) in the United Kingdom 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 health care professionals are lower than child self-reports, and (3) demographic and disease severity indicators are related to QoL.
An interdependent groups, cross-sectional design was implemented. Seventy-four children with SCA, their parent, and members of their health care team completed the GCQ. Demographic and disease severity indicators were recorded. GCQ data from healthy children were obtained from the UK Data Archive.
Contrary to past research, when examining generic discrepancy QoL, children with SCA did not report a lower QoL than healthy children, and parent- and health care professional-proxy reports were not lower than child self-reports. Few of the demographic and disease severity indicators were related to QoL.
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. Statement of contribution What is already known on this subject? Children with sickle cell disease (SCD) generally have a reduced QoL compared with healthy children, but there appears to be no research measuring QoL in paediatric SCD in the United Kingdom. Proxy QoL reports from parents are often lower than child self-reports, but there is less research examining proxy reports from health care professionals. Previous research has measured paediatric QoL using measures of current health-related QoL, but this is not in line with the WHO's definition of QoL as the discrepancy between current state and expectations. What does this study add? Children with Sickle cell anaemia do not have an impaired discrepancy QoL; they may have realistic expectations about their ideal-self and define achievements within the limits of their illness. Health care professionals are able to gauge a SCA child's discrepancy QoL better than parents. The GCQ (a generic discrepancy measure of QoL) takes into account expectations about ideal QoL and does not emphasize health; it may be of use to Psychologists working with SCA children.
英国镰状细胞贫血（SCA）儿童的生活质量（QoL）尚未得到评估，基于差距理论的差异衡量标准很少被使用。本研究旨在调查：（1）使用差异衡量标准（通用儿童 QoL 衡量标准；GCQ）的儿童自我报告的 QoL 是否低于健康儿童，（2）父母和医疗保健专业人员的代理报告是否低于儿童自我报告，以及（3）人口统计学和疾病严重程度指标与 QoL 相关。
采用相互依存的分组，横断面设计。74 名 SCA 儿童及其父母和医疗保健团队成员完成了 GCQ。记录人口统计学和疾病严重程度指标。来自健康儿童的 GCQ 数据来自英国数据档案馆。
与以往的研究相反，当检查通用差异 QoL 时，SCA 儿童的 QoL 报告并不低于健康儿童，父母和医疗保健专业人员的代理报告也不低于儿童自我报告。很少有人口统计学和疾病严重程度指标与 QoL 相关。
代理报告可以用来更全面地了解 QoL，但不应替代自我报告。对于报告的相对较高的 QoL，其解释尚不清楚，但 SCA 儿童可能对自己的理想自我有现实的期望，在塑造自己的 QoL 时更注重健康以外的方面，并在疾病的限制内定义成就。未来的研究应集中在心理因素上，以解释 QoL。
