心理韌性於兒童罕見疾病主要照顧者 親職壓力與心理健康之角色：以小胖威利症為例

Abstract

目的：小胖威利症（Prader-Willi Syndrome, PWS）是第十五對染色體突變之罕見疾病，導致出生後肌張力異常、腦下垂體生長激素分泌不足；除生理及智能發展遲緩，學齡期開始有摳皮症、多食症等行為問題，罹患自閉症類群障礙症（ASD）、注意力不足/過動症（ADHD）、情感性疾患、精神症狀的風險也較高。因此，家長在照顧上面對較多的困難與挑戰。為了解家長在長期照護下的壓力與心理健康，以及長期壓力下仍能忍受、適應、恢復、維持功能的心理韌性，本研究建構照顧者壓力模型，研究家長的負向心理結果和正向適應，以及心理韌性在此模型中扮演的中介和調節角色。 方法：共收集52份PWS家長自陳式問卷，包含兒童行為檢核表、多食問卷、親職壓力量表、心理韌性量表、WHO-5幸福指標量表、生活滿意度量表、正負向情感量表、簡式症狀檢核表。 結果：（1）驗證中文版多食問卷為信效度良好之測驗工具，探索性因素分析及驗證性因素分析較支持二因素量表結構。（2）9-18歲PWS患者在多食問卷渴求向度高於3-8歲者，18歲以上在行為及嚴重度向度顯著較高。（3）PWS行為問題與親職壓力顯著相關，兩者皆與兒童年齡及其他社會因子未有顯著相關。12-18歲PWS家長在親職壓力的親子失功能互動向度之得分較高。（4）負向心理結果部分，以家長心理韌性為前中介變項，親職壓力為後中介變項，兒童行為問題預測家長負向情緒的序列中介模型成立；然而家長心理韌性為後中介變項，親職壓力為前中介變項，兒童行為問題預測家長負向情緒的序列中介模型成立。（5）正向適應部分，家長心理韌性為後中介變項，親職壓力為前中介變項，兒童行為問題及PWS多食症狀預測家長生活滿意度的序列中介模型成立。家長心理幸福感及正向情緒則受兒童內化行為問題影響較大。 討論：家長親職壓力來源為PWS的行為問題及多食症狀，而非年齡等社會因子，且青少年時親子失功能互動加劇。心理韌性在親職壓力模型中扮演重要的角色，同時具有補償及保護作用，且會根據後果變項的性質，影響其與親職壓力在序列中介模型的順序，意即，心理韌性可能作為資源而影響親職壓力，繼而預測負向情緒；而家長的心理症狀及生活滿意度，則是心理韌性在緩衝壓力後，長期調適之結果。根據本研究隻發現，建議減少兒童問題行為，並培養PWS患者與家長之關係及溝通模式。另外，納入心理韌性觀點的評估治療取向，能對長期照護壓力下的罕病家長有所幫助，提升其整體心理健康。

Introduction: Prader-Willi Syndrome (PWS) is a rare disease caused by mutation on chromosome 15q11-q13, resulting in postnatal hypotonia and insufficient growth hormone secretion. In addition to delayed and deficient physical and intellectual development, PWS patients often display behavioral problems such as skin picking and hyperphagia in school-age years. They are also at higher risk for Autism Spectrum Disorder (ASD), Attention Deficit/Hyperactivity Disorder (ADHD), mood disorders, and psychiatric symptoms. Thus, their parents may have faced more challenges in caregiving. This study aimed to understand parenting stress and mental health in the context of long-term caregiving, as well as their resilience to tolerate, adapt, recover, and maintain functioning under chronic stress. A parenting stress model was developed to examine both negative psychological outcomes and positive adaptation, with a focus on the mediating and moderating roles of resilience. Methods: A total of 52 parents of PWS children completed self-report measures, including the Child Behavior Checklist, Hyperphagia Questionnaire, Parenting Stress Index - short form, Resilience Scale, Word Health Organization – Five Well-being Index, Satisfaction with Life Scale, Positive and Negative Affect Schedule, and Brief Symptom Inventory - Revised. Results: (1) Satisfactory reliability and validity were confirmed in Chinese version of the hyperphagia questionnaire, with exploratory and confirmatory factor analysis supporting two-factor scale structure. (2) In Hyperphagia Questionnaire, individuals with PWS aged 9-18 showed higher scores in Drive subscale than those aged 3-8 years old, while those aged 18 and above showed higher score in Behavior and severity subscale. (3) Parenting stress positively correlated with PWS behavioral problems, though both of them were not significantly correlated with child’s age or other social factors. Parents of adolescents aged 12-18 reported higher scores on dysfunctional parent-child interaction. (4) For sequential mediation model of negative psychological outcome, child behavioral problems predicted parents’ negative emotions through parents’ resilience (first mediator) and parenting stress (second mediator), whereas child behavioral problems predicted parents’ psychological symptoms through parenting stress (first mediator) and parents’ resilience (second mediator). (5) For sequential mediation model of positive adaptation, child behavioral problems and hyperphagia symptoms predicted parents’ life satisfaction through parenting stress (first mediator) and parents’ resilience (second mediator). In addition, parent’s psychological well-being and positive emotions were more influenced by children's internalized behavioral problems. Discussion: Parenting stress was influenced by behavioral problems in PWS rather than social factors such as age and social status. Moreover, parent-child dysfunctional interactions were exacerbated while PWS children entered into adolescence. In the model of parenting stress, resilience plays an important role, both compensatory and protective, and in the serial mediation model of parenting stress, its order may depend on the nature of outcome variables. That is, resilience may act as a resource to influence parenting stress, and then predict negative emotions, whereas for long-term psychological health of parents, such as psychological symptoms and life satisfaction, resilience may reflect its role in the process of adjustment, buffering the impact of stress. According to the findings of the present study, it is suggested to reduce children's problematic behaviors and to cultivate the communication patterns and relationship between PWS patients and their parents. Furthermore, incorporating the resilience perspective into assessment and treatment will be helpful for PWS parents psychological health in face of long-term caregiving stress.