Laffan Mike, Benson Gary, Farrelly Cathy, Gomez Keith, Jones April, Maclean Rhona, O'Donnell James, Lavin Michelle
Centre for Haematology, Imperial College London, London, UK.
NI Haemophilia Comprehensive Care Centre, Belfast City Hospital, Belfast, UK.
Haemophilia. 2023 May;29(3):819-826. doi: 10.1111/hae.14765. Epub 2023 Mar 6.
Von Willebrand Disease (VWD) is the most common inherited bleeding disorder. However, recognition of the disease by both the public and healthcare professionals lags behind that of other bleeding disorders, leading to delays in diagnosis and treatment for patients. Updated national guidelines are needed to highlight an appropriate pathway for managing VWD patients in a timelier manner.
To identify ways in which care for VWD can be achieved on a more equitable basis.
Using a modified Delphi approach, a panel of VWD experts developed 29 statements across five key themes. These were used to form an online survey that was distributed to healthcare professionals involved in VWD care across the UK and Republic of Ireland (ROI). Stopping criteria comprised 50 responses received, a 3-month window for response (February-April 2022) and 90% of statements passing consensus threshold. Threshold for consensus for each statement was agreed at 75%.
A total of 66 responses were analysed with 29/29 statements achieving consensus of which 27 attained ≥90% agreement. From the high degree of consensus, eight recommendations were derived regarding how detection and management of VWD can be improved to provide equity of care between men and women.
Implementation of these eight recommendations across the VWD pathway has the potential to raise the standard of care for patients in the UK and ROI by reducing delays to diagnosis and treatment initiation.
血管性血友病（VWD）是最常见的遗传性出血性疾病。然而，公众和医疗保健专业人员对该疾病的认知落后于其他出血性疾病，导致患者诊断和治疗延迟。需要更新国家指南，以突出及时管理VWD患者的适当途径。
确定以更公平的方式实现VWD护理的方法。
采用改良的德尔菲法，一组VWD专家围绕五个关键主题制定了29条陈述。这些陈述被用于形成一项在线调查，该调查分发给了英国和爱尔兰共和国（ROI）参与VWD护理的医疗保健专业人员。停止标准包括收到50份回复、3个月的回复期限（2022年2月至4月）以及90%的陈述通过共识阈值。每条陈述的共识阈值定为75%。
共分析了66份回复，29条陈述全部达成共识，其中27条达成了≥90%的一致意见。基于高度的共识，得出了八项关于如何改进VWD检测和管理以实现男女护理公平性的建议。
在VWD诊疗路径中实施这八项建议，有可能通过减少诊断和治疗开始的延迟，提高英国和爱尔兰共和国患者的护理标准。
