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什么是“严重”的遗传疾病?患有遗传疾病人群的看法。

What is a 'serious' genetic condition? The perceptions of people living with genetic conditions.

机构信息

Warwick Medical School, University of Warwick, CV4 7HL, Coventry, UK.

出版信息

Eur J Hum Genet. 2022 Feb;30(2):160-169. doi: 10.1038/s41431-021-00962-2. Epub 2021 Sep 27.

Abstract

Despite no consensus on the definition of 'seriousness', the concept is regularly used in policy and practice contexts to categorise conditions, determine access to genetic technologies and uses of selective pregnancy termination. Whilst attempts have been made to create taxonomies of genetic condition seriousness to inform clinical and policy decision-making, these have often relied on condition appraisals made by health and genetics professionals. The views of people with genetic conditions have been largely under-represented. This study explores the concept of seriousness through the perspectives of people with a range of 'clinically serious' conditions (fragile X conditions, spinal muscular atrophy, cystic fibrosis, haemophilia, thalassaemia). Attitudes towards suffering, quality of life (QoL) and selective pregnancy termination were elucidated from 45 in-depth qualitative interviews and 469 postal/online surveys. The majority of participants reported good health/wellbeing, and the capacity for good QoL, despite experiencing suffering with their condition. Notably, participants with later-onset conditions held more negative views of their health and QoL, and were more likely to view their condition as an illness, than those with early-onset conditions. These participants were more likely to see their condition as part of their identity. Whilst most participants supported prenatal screening, there was little support for selective termination. Moreover, social environment emerged as a critical mediator of the experience of the condition. The complex and rich insights of people living with genetic conditions might usefully be incorporated into future genetic taxonomies of 'seriousness' to ensure they more accurately reflect the lived reality of those with genetic conditions.

摘要

尽管人们对“严重性”的定义尚未达成共识,但这一概念在政策和实践中经常被用于对各种情况进行分类,以确定获取遗传技术和选择性终止妊娠的途径。尽管人们已经尝试创建遗传状况严重性分类法,以告知临床和决策制定,但这些分类法往往依赖于健康和遗传学专业人员对状况的评估。具有遗传状况的人的观点在很大程度上被忽视了。本研究通过一系列具有“临床严重”状况(脆性 X 综合征、脊髓性肌萎缩症、囊性纤维化、血友病、地中海贫血症)的人的观点来探讨严重性这一概念。通过 45 次深入的定性访谈和 469 次邮寄/在线调查,阐明了人们对痛苦、生活质量(QoL)和选择性终止妊娠的态度。大多数参与者报告健康状况良好/幸福感强,并且有能力获得良好的 QoL,尽管他们正在经历疾病的痛苦。值得注意的是,与早发性疾病相比,患有晚发性疾病的参与者对自己的健康和 QoL 持更负面的看法,并且更有可能将自己的状况视为一种疾病。这些参与者更有可能将自己的状况视为自己身份的一部分。尽管大多数参与者支持产前筛查,但对选择性终止妊娠的支持甚少。此外,社会环境成为影响体验状况的关键因素。具有遗传状况的人所具有的复杂而丰富的见解,可能会被有益地纳入未来遗传“严重性”分类法中,以确保这些分类法更准确地反映出具有遗传状况的人的真实生活状况。

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