Nag Heidi Elisabeth, Hoxmark Lise Beate, Nærland Terje
Frambu Resource Centre for Rare Disorders, Norway; University of Stavanger, Norway.
Frambu Resource Centre for Rare Disorders, Norway.
J Intellect Disabil. 2019 Sep;23(3):359-372. doi: 10.1177/1744629519847375. Epub 2019 May 2.
The experience of having a rare disorder was summarised in a large study as 'falling outside the vast field of knowledge of the professionals'. Parents (31 mothers and 17 fathers) of 32 persons with Smith-Magenis syndrome (SMS) participated in this study. A phenomenological approach was used to analyse the data into topics and themes. Four themes emerged: behavioural challenges displayed, parents' strategies for meeting the challenging behaviours, parents' experiences of their own competence and parents' experiences of professionals' competence and understanding regarding children with SMS and their behaviour challenges. We found that parents of children with SMS experience that they are exposed to severe challenging behaviours from their child. The parents believe that they experience more misunderstandings with professionals and that the challenging behaviours increase because there are some specific characteristics of SMS that professionals are not aware of or do not consider in their support services.
一项大型研究将患罕见疾病的经历总结为“超出了专业人员的广泛知识领域”。32名患有史密斯-马吉尼斯综合征（SMS）患者的父母（31位母亲和17位父亲）参与了这项研究。采用现象学方法将数据分析为主题和次主题。出现了四个主题：所表现出的行为挑战、父母应对具有挑战性的行为的策略、父母对自身能力的体验以及父母对专业人员在SMS患儿及其行为挑战方面的能力和理解的体验。我们发现，患有SMS的儿童的父母经历了孩子表现出的严重挑战性行为。父母认为他们与专业人员之间存在更多误解，并且由于SMS有一些专业人员未意识到或在其支持服务中未考虑到的特定特征，具有挑战性的行为有所增加。
