Schadewald Amy, Kimball Ericka, Ou Li
University of Minnesota Health, Minneapolis, MN, USA.
Portland State University School of Social Work, Portland, OR, USA.
JIMD Rep. 2018;42:89-97. doi: 10.1007/8904_2017_87. Epub 2018 Jan 4.
The mucopolysaccharidoses are a set of rare, inherited conditions that can have a catastrophic impact on those affected and their families. Because of the rarity of these disorders, little is known regarding the challenges faced by families of those affected and what coping mechanisms are commonly used. Coping is a way to manage demands that occur in one's environment or within oneself. Medical social workers historically have facilitated this process while providing support to patients who are responding to pressures of their diagnosis and the system.A questionnaire of demographics and qualitative questions, along with the Pediatric Inventory for Parents (PIP) and Brief COPE, was sent by electronic survey to caregivers of children with MPS. The results of Brief COPE showed that problem-focused coping was more frequently used than emotion-focused (p < 0.001) or dysfunctional coping (p < 0.0001). Acceptance was the most frequently used coping strategy (p < 0.05). The results of PIP showed that emotionally distressing events were the most difficult (p < 0.001), while events related to medical care occurred at the highest frequency (p < 0.001). Psychosocial support provided by medical social workers significantly increased acceptance of caregivers (p = 0.04). Guidance on what to expect provided by any member of the care team increased denial (p = 0.02) and the difficulty of emotional distress (p = 0.04). This study identified commonly used coping strategies and measured stress among caregivers of children with MPS, as well as access to and use of psychosocial support services. Results highlight the urgency to improve the coverage and quality of psychosocial support and other support services.
黏多糖贮积症是一组罕见的遗传性疾病，会对患者及其家庭产生灾难性影响。由于这些疾病罕见，对于患者家庭所面临的挑战以及他们常用的应对机制知之甚少。应对是一种管理自身所处环境或自身内部出现的需求的方式。从历史上看，医务社会工作者在为应对诊断压力和医疗系统压力的患者提供支持的同时，也推动了这一过程。通过电子调查向黏多糖贮积症患儿的照料者发送了一份包含人口统计学和定性问题的问卷，以及《父母儿科量表》（PIP）和《简易应对方式问卷》（Brief COPE）。《简易应对方式问卷》的结果显示，与情绪聚焦应对（p < 0.001）或功能失调性应对（p < 0.0001）相比，问题聚焦应对的使用频率更高。接受是最常用的应对策略（p < 0.05）。《父母儿科量表》的结果显示，情绪困扰事件最难应对（p < 0.001），而与医疗护理相关的事件发生频率最高（p < 0.001）。医务社会工作者提供的心理社会支持显著提高了照料者的接受度（p = 0.04）。护理团队任何成员提供的关于预期情况的指导增加了否认情绪（p = 0.02）和情绪困扰的难度（p = 0.04）。本研究确定了黏多糖贮积症患儿照料者常用的应对策略，并测量了他们的压力，以及心理社会支持服务的获取和使用情况。结果凸显了提高心理社会支持及其他支持服务的覆盖范围和质量的紧迫性。
