Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.
Department of Health Psychology, HPC FA12, University Medical Center Groningen, University of Groningen, POB 196, 9700 AD, Groningen, The Netherlands.
Orphanet J Rare Dis. 2021 Jan 30;16(1):53. doi: 10.1186/s13023-021-01702-x.
Epidermolysis bullosa (EB) is a group of rare genetic skin disorders that primarily manifest as blisters and erosions following mild mechanical trauma. Despite the crucial role of the parents of children with EB in managing the disease, studies focusing on the parent-child relationship remain a gap in the literature. To address this gap, the current quantitative study, involving 55 children with all types of EB and 48 parents, assessed the relationship between their quality of life and coping strategies. Quality of life was measured with the Pediatric Quality of Life Inventory and TNO-AZL Questionnaire for Adult's Health- related Quality of Life, and coping strategies were assessed with the Coping with a Disease Questionnaire. The majority of the analyses were descriptive and the results were interpreted qualitatively because of the small sample size.
Overall, the quality of life of children with EB and that of their parents was somewhat lower compared with the quality of life of healthy children and adults. Children with EB who more frequently used emotional reactions and cognitive-palliative strategies to cope with the disease demonstrated lower levels of emotional and social functioning, while children who showed more acceptance and distancing showed higher levels of functioning on all domains. Parents who frequently demonstrated emotional reactions reported lower levels of social functioning and experienced more depressive emotions and anger. Parents who used more avoidance showed higher levels of positive emotions. Within parent-child dyads, acceptance, cognitive-palliative strategies and distancing were positively related. Children's emotional and social functioning were negatively associated with their parents' depressive emotions. Parents' acceptance was linked to higher physical functioning in children, whereas children's avoidance was linked to a lower level of anger in parents.
Children who are able to accept the disease or distance themselves from it appear to be better off in contrast to those who tend to engage in the cognitive-palliative strategies and expressing emotional reactions. Parents seem to be better off when they are able to use avoidance in contrast to those who tend to show emotional reactions. Further research is needed to substantiate these findings.
大疱性表皮松解症（EB）是一组罕见的遗传性皮肤疾病，主要表现为轻微机械创伤后出现水疱和糜烂。尽管患有 EB 的儿童的父母在管理疾病方面起着至关重要的作用，但专注于亲子关系的研究在文献中仍然存在空白。为了填补这一空白，本定量研究涉及 55 名患有各种类型 EB 的儿童和 48 名父母，评估了他们的生活质量和应对策略之间的关系。生活质量使用儿科生活质量量表和 TNO-AZL 成人健康相关生活质量问卷进行评估，应对策略使用疾病应对问卷进行评估。由于样本量小，大多数分析都是描述性的，结果是定性解释的。
总体而言，与健康儿童和成人相比，患有 EB 的儿童及其父母的生活质量有些较低。那些更频繁地使用情绪反应和认知缓解策略来应对疾病的 EB 儿童表现出较低的情绪和社会功能水平，而那些表现出更多接受和距离的儿童在所有领域都表现出较高的功能水平。经常表现出情绪反应的父母报告说社会功能水平较低，并且经历更多的抑郁情绪和愤怒。更多使用回避的父母表现出更高的积极情绪。在亲子二人组中，接受、认知缓解策略和距离与积极相关。儿童的情绪和社会功能与父母的抑郁情绪呈负相关。父母的接受与儿童的身体功能更高有关，而儿童的回避与父母的愤怒程度较低有关。
与那些倾向于使用认知缓解策略和表达情绪反应的儿童相比，能够接受疾病或与疾病保持距离的儿童似乎状态更好。与那些倾向于表现出情绪反应的父母相比，能够使用回避的父母似乎状态更好。需要进一步的研究来证实这些发现。
