Simpson Amy, Ross Richard, Porter John, Dixon Simon, Whitaker Martin J, Hunter Amy
Genetic Alliance UK, 49-51 East Road, London, N1 6AH, UK.
Department of Oncology & Metabolism, EU12, The Medical School, University of Sheffield, Sheffield, S10 2JF, UK.
J Genet Couns. 2018 Dec;27(6):1447-1458. doi: 10.1007/s10897-018-0278-9. Epub 2018 Jul 7.
Research into adrenal insufficiency (AI) and congenital adrenal hyperplasia (CAH) in children has focused largely on clinical consequences for patients; and until recently, the wider experience of the condition from the perspective of other family members has been neglected. In a mixed methods study, we captured the experiences of parents of young children affected by AI/CAH, including their views on the psychosocial impact of living with and managing the condition. Semi-structured interviews were carried out in the UK and an online survey was developed, translated and disseminated through support groups (UK and the Netherlands) and outpatient endocrinology clinics (Germany). Challenges associated with diagnosis, treatment, support and the future were identified. For UK parents, the diagnosis period was characterised by a lack of awareness amongst healthcare professionals and occurrences of adrenal crisis. Parents reported burden, anxiety and disruption associated with the intensive treatment regimen. Parents adjusted and gained confidence over time yet found delegating responsibility for medication difficult and worried about the future for their child. Access to psychological support and contact with other families was reported as highly beneficial. The findings of the study provide critical context for future studies and for informing how parents and families can be better supported. Prenatal genetic counselling for parents who already have an affected child will include an explanation of recurrence risk but should also focus on providing information and reassurance about diagnostic testing and care for their newborn.
儿童肾上腺功能不全(AI)和先天性肾上腺增生(CAH)的研究主要集中在对患者的临床影响上;直到最近,从其他家庭成员的角度对该疾病更广泛的体验一直被忽视。在一项混合方法研究中,我们了解了受AI/CAH影响的幼儿父母的经历,包括他们对与该疾病共处和管理该疾病的心理社会影响的看法。在英国进行了半结构化访谈,并开展了一项在线调查,该调查经过翻译后通过支持小组(英国和荷兰)以及门诊内分泌诊所(德国)进行传播。确定了与诊断、治疗、支持和未来相关的挑战。对于英国的父母来说,诊断期间的特点是医疗保健专业人员缺乏认识以及肾上腺危象的发生。父母报告了与强化治疗方案相关的负担、焦虑和生活干扰。随着时间的推移,父母进行了调整并获得了信心,但发现很难将用药责任委托他人,并且担心孩子的未来。据报告,获得心理支持以及与其他家庭联系非常有益。该研究的结果为未来的研究以及为告知如何更好地支持父母和家庭提供了关键背景。对于已经有患病孩子的父母,产前遗传咨询将包括对复发风险的解释,但也应侧重于提供有关其新生儿诊断检测和护理的信息并使其安心。