遗传服务调查--爱尔兰共和国罕见病患者及其家人获得遗传服务的经验。

IF 1.5 Q4 GENETICS & HEREDITY
Journal of Community Genetics Pub Date : 2023-12-01 Epub Date: 2023-08-26 DOI:10.1007/s12687-023-00664-w
A J Ward, D M Lambert, D Butterly, J J O'Byrne, V McGrath, S A Lynch
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引用次数: 1

摘要

爱尔兰卫生服务目标规定,罕见病患者应能及时获得基因组诊断,并得到适当的检测前后咨询。然而,在研究期间,由于人员配备水平仍然较低,临床遗传学门诊预约的等待时间长达两年。2022 年 1 月,我们开展了一项有针对性的公众在线调查,以了解爱尔兰共和国罕见病家庭试图获得基因检测和临床基因门诊服务的经历。爱尔兰患者在获得临床遗传服务方面经历了漫长的等待时间,并自我报告了与延迟获得诊断、明确复发风险和后续管理有关的焦虑和压力。这对有关计划生育、教育和就业的个人决定产生了负面影响,并对寻求明确自身风险的家庭成员产生了重大影响。主流基因检测活动非常重要。患者家属对安排和提供基因检测结果的医护人员的能力表示担忧,并担心无法及时获得临床遗传学专家的帮助,以了解其临床影响。及时获得临床遗传学专业知识对于确保罕见病家庭适当了解遗传诊断的医疗和生殖影响以及获得相关护理途径非常重要。制定一个国家框架,培养医疗保健专业人员的基因组知识能力,包括建立一个国家基因检测目录,可能会有所裨益。临床遗传学团队需要持续的支持和投资,以确保为爱尔兰罕见病家庭提供安全有效的服务。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Genetic services survey-experience of people with rare diseases and their families accessing genetic services in the Irish Republic.

Irish Health Service objectives state that patients with rare diseases should have timely access to genomic diagnostics with appropriate pre and post-test counselling. However, waiting times for clinical genetics outpatient appointments, during the study period, were up to two years as staffing levels remain low. A targeted public online survey was conducted in January 2022 to capture the experiences of Rare Disease families trying to access genetic testing and clinical genetic clinics in the Irish Republic. Irish patients experience significant waiting times to access clinical genetic services and self-report anxiety and stress, related to delayed access to diagnosis, clarity around recurrence risk and follow-up management. This negatively impacts personal decisions around family planning, education and employment and has a significant impact on family members seeking clarity on their own risk. Mainstream genetic testing activity is significant. Families report concern over the competency of health care professionals arranging and delivering genetic results and delays in accessing clinical genetics expertise to take them through the clinical implications. Timely access to clinical genetics expertise is important to ensure families with rare diseases have an appropriate understanding of the medical and reproductive implications of a genetic diagnosis and access to relevant care pathways. A national framework to develop competency in genomic literacy for health-care professionals including a national genetic test directory may be beneficial. Clinical genetics teams require ongoing support and investment to ensure the delivery of a safe and effective service for Irish families with rare diseases.

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来源期刊
Journal of Community Genetics
Journal of Community Genetics GENETICS & HEREDITY-
CiteScore
3.30
自引率
5.30%
发文量
54
期刊介绍: The Journal of Community Genetics is an international forum for research in the ever-expanding field of community genetics, the art and science of applying medical genetics to human communities for the benefit of their individuals. Community genetics comprises all activities which identify persons at increased genetic risk and has an interest in assessing this risk, in order to enable those at risk to make informed decisions. Community genetics services thus encompass such activities as genetic screening, registration of genetic conditions in the population, routine preconceptional and prenatal genetic consultations, public education on genetic issues, and public debate on related ethical issues. The Journal of Community Genetics has a multidisciplinary scope. It covers medical genetics, epidemiology, genetics in primary care, public health aspects of genetics, and ethical, legal, social and economic issues. Its intention is to serve as a forum for community genetics worldwide, with a focus on low- and middle-income countries. The journal features original research papers, reviews, short communications, program reports, news, and correspondence. Program reports describe illustrative projects in the field of community genetics, e.g., design and progress of an educational program or the protocol and achievement of a gene bank. Case reports describing individual patients are not accepted.
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