Children's National Health System.
George Washington University School of Medicine and Health Sciences.
J Pediatr Psychol. 2018 Sep 1;43(8):882-894. doi: 10.1093/jpepsy/jsy019.
Complications that can arise from sickle cell disease (SCD) have the potential to negatively affect health-related quality of life (HRQL). SCD manifests in varying degrees of severity, but effects on HRQL are not uniform. Cognitive abilities influence HRQL in other pediatric groups, potentially through variability in treatment adherence and psychological coping. This study examined the effect of SCD severity on HRQL and explored cognitive abilities as a moderator of this relationship.
A total of 86 children and adolescents with SCD (ages 7-16 years) completed a cognitive assessment (Wechsler Scale of Intelligence for Children, Fifth Edition), and primary caregivers rated their child's SCD severity and HRQL (PedsQL Sickle Cell Disease Module). A hierarchical linear regression was conducted to evaluate the interactive effect of SCD severity and cognitive functioning on HRQL.
Caregiver-rated SCD severity predicted HRQL and cognitive abilities interacted with disease severity to influence HRQL. Youth with milder SCD and cognitive abilities in the average range or higher demonstrated significantly better HRQL compared with youth with mild SCD but below average cognitive abilities. Youth with more severe disease appeared to exhibit similarly low levels of HRQL, with only a minimal influence of cognitive abilities.
Cognitive factors modify the effect of SCD severity on HRQL, particularly among youth with milder forms of SCD. Future studies are warranted to clarify the role of cognitive abilities in determining HRQL. Clinicians should monitor youth with milder forms of SCD and limited cognitive abilities for worsening HRQL and opportunities to provide support around disease self-management.
镰状细胞病（SCD）可能引发的并发症会对健康相关生活质量（HRQL）产生负面影响。SCD 的表现程度不同，但对 HRQL 的影响并不一致。认知能力会影响其他儿科群体的 HRQL，这可能是通过治疗依从性和心理应对的差异来实现的。本研究考察了 SCD 严重程度对 HRQL 的影响，并探讨了认知能力作为这种关系的调节因素。
共有 86 名患有 SCD（年龄 7-16 岁）的儿童和青少年完成了认知评估（韦氏儿童智力量表第五版），主要照顾者对其孩子的 SCD 严重程度和 HRQL（PedsQL 镰状细胞疾病模块）进行了评分。采用分层线性回归分析评估 SCD 严重程度和认知功能对 HRQL 的交互影响。
照顾者评定的 SCD 严重程度预测了 HRQL，认知能力与疾病严重程度相互作用，影响 HRQL。轻度 SCD 且认知能力处于平均水平或更高的青少年的 HRQL 明显优于轻度 SCD 但认知能力低于平均水平的青少年。病情较重的青少年似乎表现出相似的低水平 HRQL，只有认知能力的微小影响。
认知因素改变了 SCD 严重程度对 HRQL 的影响，尤其是在轻度 SCD 的青少年中。需要进一步的研究来阐明认知能力在确定 HRQL 中的作用。临床医生应监测病情较轻且认知能力有限的青少年的 HRQL 恶化情况，并为其提供疾病自我管理方面的支持。
