Navon Daniel
Department of Sociology, Columbia University, New York, NY 10027, USA.
J Genet Couns. 2012 Dec;21(6):770-6. doi: 10.1007/s10897-012-9515-9. Epub 2012 Jul 21.
This paper presents a sociological examination of the role of genetic counselors as advocates, not only for patients and their families, but also for genetic conditions themselves. In becoming activists for new disorders, genetic counselors are helping to create new categories that will shape expectations and treatment regimens for both existing patients and those who are yet to be diagnosed. By virtue of their expertise and their position at the intersection of several key professions and constituencies, genetic counselors are likely to play a central role in the way the genetic testing technologies, and especially 'genotype-first' diagnosis, impacts the way we understand and categorize developmental difference. I outline some of the promises and dangers that this kind of activism holds for people with developmental disabilities, and particularly the challenge presented by systemic ascertainment bias in the face of genotype-phenotype uncertainty. I argue that new testing techniques like microarray analysis that do not need to be targeted on the basis of clinical presentation throw these challenges into sharp relief, and that the genetic counseling community should consider how to marry advocacy for new genetic conditions with an emphasis on the indeterminate developmental potential of every child.
本文从社会学角度审视了遗传咨询师作为倡导者的角色,他们不仅为患者及其家庭代言,也为遗传疾病本身发声。在成为新疾病的倡导者过程中,遗传咨询师正助力创建新的类别,这些类别将塑造现有患者以及尚未确诊者的期望和治疗方案。凭借其专业知识以及在多个关键专业和群体交叉点上的地位,遗传咨询师很可能在基因检测技术,尤其是“基因型优先”诊断影响我们理解和分类发育差异的方式中发挥核心作用。我概述了这种倡导行为给发育障碍患者带来的一些希望和风险,尤其是面对基因型 - 表型不确定性时系统确认偏差所带来的挑战。我认为,像微阵列分析这样无需基于临床表现进行靶向检测的新检测技术,使这些挑战更加凸显,并且遗传咨询界应思考如何在倡导新的遗传疾病时,同时强调每个孩子不确定的发育潜力。