Faculty of Psychology, Sigmund Freud University Vienna, Freudplatz 1, 1020, Vienna, Austria.
EB House Austria, Department of Dermatology and Allergology, University Hospital of the Paracelsus Medical University, Salzburg, Austria.
Orphanet J Rare Dis. 2024 May 21;19(1):211. doi: 10.1186/s13023-024-03163-4.
With approximately 500 people affected in Austria, epidermolysis bullosa (EB) is a rare genetic skin disease reducing the quality of life of those affected and their relatives. The intensive efforts of the patient organisation DEBRA Austria during the last decades have led to a unique situation of those affected and their relatives, with increased support and broader knowledge about the disease in the general population. The aim of the study is to evaluate the current situation of patients and their relatives living in Austria, with a focus on burdens and helpful practices.
The mixed-methods study consisted of two parts: a qualitative interview study to identify psychosocial aspects of EB in those affected and their relatives, and a subsequent online survey to further assess those aspects in a larger sample, resulting in a total of n=78 Austrian participants. The impact of EB on the quality of life of EB patients and their relatives in Austria is related to the current health status, psychological burden, mobility, visibility, financial situation as well as job prospects. Personal and social resources and external support have a significant influence on the individual situation.
The outcome is mapped to concrete implications regarding targeted support for EB patients and their relatives on an individual level and their needs in regard to the Austrian health care system.
在奥地利,约有 500 人受到大疱性表皮松解症(EB)的影响,这种罕见的遗传性皮肤疾病降低了患者及其亲属的生活质量。过去几十年来,DEBRA 奥地利患者组织的积极努力,为患者及其亲属创造了独特的环境,他们在公众中得到了更多的支持和对疾病的更广泛了解。本研究旨在评估奥地利患者及其亲属的现状,重点关注负担和有益的做法。
这项混合方法研究包括两个部分:对患者及其亲属进行定性访谈,以确定 EB 的社会心理方面;随后进行在线调查,以在更大的样本中进一步评估这些方面,最终共有 78 名奥地利参与者。EB 对奥地利 EB 患者及其亲属的生活质量的影响与当前的健康状况、心理负担、行动能力、可见度、财务状况以及工作前景有关。个人和社会资源以及外部支持对个人情况有重大影响。
研究结果针对 EB 患者及其亲属的个体需求,以及他们对奥地利医疗保健系统的需求,提出了有针对性的支持措施。