MNW Consulting Group, Portland, OR, USA.
Kennedy Krieger Institute, Baltimore, MD, USA.
J Child Neurol. 2023 Apr;38(5):270-282. doi: 10.1177/08830738231172066. Epub 2023 May 9.
Rett syndrome is a rare neurodevelopmental disorder primarily affecting females. This syndrome is associated with many comorbidities and impairments related to motor function, breathing, sleep, expressive language, and repetitive hand movements. The Rett Syndrome Behaviour Questionnaire (RSBQ) is one measure used to assess changes in Rett syndrome-related manifestations or core symptoms. Little is known about how caregivers think about meaningful changes in the items that make up the RSBQ scale.
This qualitative study explored how caregivers of individuals with Rett syndrome viewed changes in the symptoms covered in the RSBQ. We conducted semistructured interviews with 40 caregivers and employed thematic analysis, identifying themes using an iterative process.
Two factors characterized caregivers' thoughts about meaningful changes in Rett syndrome manifestations. First, general features of these symptoms rendered them bothersome: the extent of bother compared to other symptoms, if or how they prevented desirable behaviors and their temporal qualities. Second, caregivers evaluated the meaning of improvements by considering the decrease in bother and the potential benefits of change. Improvements had social and psychological consequences for individuals with Rett syndrome and caregivers. In addition, implications for health, fine and gross motor skills, and communication were also substantial.
雷特综合征是一种罕见的神经发育障碍，主要影响女性。该综合征与许多与运动功能、呼吸、睡眠、表达性语言和重复性手部运动相关的共病和障碍有关。雷特综合征行为问卷（RSBQ）是评估雷特综合征相关表现或核心症状变化的一种测量方法。关于照顾者如何看待构成 RSBQ 量表的项目的有意义变化，知之甚少。
本定性研究探讨了雷特综合征患者的照顾者如何看待 RSBQ 中涵盖的症状的变化。我们对 40 名照顾者进行了半结构式访谈，并采用主题分析，通过迭代过程确定主题。
有两个因素特征描述了照顾者对雷特综合征表现有意义变化的看法。首先，这些症状的一般特征使其令人烦恼：与其他症状相比的严重程度、它们是否或如何阻止了理想行为以及它们的时间性质。其次，照顾者通过考虑减少烦恼和变化的潜在益处来评估改善的意义。改善对雷特综合征患者和照顾者具有社会和心理后果。此外，对健康、精细和粗大运动技能以及沟通的影响也很大。
