Bos-Roubos Anja, Wingbermühle Ellen, Biert Anneloes, de Graaff Laura, Egger Jos
Centre of Excellence for Neuropsychiatry, Vincent van Gogh Institute for Psychiatry, Venray, Netherlands.
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, Netherlands.
Front Psychiatry. 2022 Jun 28;13:897138. doi: 10.3389/fpsyt.2022.897138. eCollection 2022.
Prader-Willi syndrome (PWS) is a potentially life threatening, genetic developmental disorder that requires lifelong medical treatment and behavioral management. PWS has a major impact on the patient's social environment. In this study, we have explored traumatic life events and symptoms of posttraumatic stress disorder (PTSD) in family members of individuals with PWS. We have also assessed quality of life in relation to trauma manifestations. In addition, we have evaluated demographic characteristics such as living setting of PWS patients as well as PWS symptom severity.
Data of this observational study were obtained by means of the Life Events Checklist DMS-5, the Posttraumatic Stress Disorder Checklist DSM-5, the abbreviated World Health Organization Quality of Life questionnaire, the Lancashire Quality of Life Profile questionnaire, and a short demographic inventory. The study sample includes 98 adults aged 19 to 80 years ( = 49, = 15), who are relatives of 69 individuals with PWS aged 0 to 58 years ( = 19, = 13). Participants were recruited via the two Dutch patient associations PWS and the Dutch Digital Center of Expertise PWS.
Life time prevalence of traumatic events (93%) was higher in family members of PWS patients ("PWS relatives") than in the general Dutch population (81%). Of those who reported any traumatic event, almost half reported events. The prevalence of probable PTSD was higher in PWS relatives (12.1%) than the general lifetime prevalence of PTSD (worldwide, and in the Netherlands 7.4%). Predominant trauma symptoms in PWS relatives were "negative changes in arousal and reactivity" and "negative changes in cognition and mood;" both significantly negatively related to quality of life. Symptom severity of PWS individuals, as well as the associated trauma symptom severity of their relatives increased with age of the PWS individual. The presence of trauma symptoms was less frequent among relatives of PWS individuals living in a care facility.
Having a relative with PWS is associated with higher prevalence of traumatic experiences and greater vulnerability to PTSD. Raising awareness in health care professionals of trauma symptoms in PWS relatives may contribute to effective treatment of their psychosocial stress. In addition, timely interventions might prevent family members from developing psychopathology like PTSD.
普拉德-威利综合征（PWS）是一种潜在危及生命的遗传性发育障碍，需要终身医疗治疗和行为管理。PWS对患者的社会环境有重大影响。在本研究中，我们探讨了PWS患者家庭成员的创伤性生活事件和创伤后应激障碍（PTSD）症状。我们还评估了与创伤表现相关的生活质量。此外，我们评估了人口统计学特征，如PWS患者的生活环境以及PWS症状严重程度。
本观察性研究的数据通过生活事件清单DMS-5、创伤后应激障碍清单DSM-5、世界卫生组织生活质量简表问卷、兰开夏郡生活质量概况问卷以及一份简短的人口统计学调查表获得。研究样本包括98名年龄在19至80岁的成年人（男性 = 49，女性 = 15），他们是69名年龄在0至58岁的PWS患者（男性 = 19，女性 = 13）的亲属。参与者通过荷兰两个PWS患者协会和荷兰PWS数字专业中心招募。
PWS患者家庭成员（“PWS亲属”）中创伤性事件的终身患病率（93%）高于荷兰普通人群（81%）。在报告有任何创伤性事件的人中，近一半报告了多个事件。PWS亲属中可能患PTSD的患病率（12.1%）高于PTSD的总体终身患病率（全球以及荷兰为7.4%）。PWS亲属中主要的创伤症状是“觉醒和反应性的消极变化”以及“认知和情绪的消极变化”；两者均与生活质量显著负相关。PWS个体的症状严重程度以及其亲属相关的创伤症状严重程度随PWS个体年龄的增加而增加。生活在护理机构中的PWS个体的亲属中创伤症状的出现频率较低。
有一名患有PWS的亲属与创伤经历的较高患病率以及对PTSD的更大易感性相关。提高医疗保健专业人员对PWS亲属创伤症状的认识可能有助于有效治疗他们的心理社会压力。此外，及时干预可能会防止家庭成员发展为如PTSD等精神病理学问题。
