Baumann Kimberly, Hernandez Grace, Witkop Michelle, Peltier Skye, Dunn Spencer, Cutter Susan, Frick Neil, Haugstad Kimberly, Guelcher Christine, Frey Mary Jane, Rotellini Dawn, Clark David B, Iyer Neeraj N, Cooper David L
Center for Bleeding and Clotting Disorders, University of Minnesota Health, Minneapolis, MN, USA.
Center for Comprehensive Care and Diagnosis of Inherited Bleeding Disorders, Orange, CA, USA.
Eur J Haematol. 2017 Apr;98 Suppl 86:25-34. doi: 10.1111/ejh.12852.
The psychosocial impact of hemophilia on activities was recently investigated in the Hemophilia Experiences, Results and Opportunities (HERO) study (675 people with hemophilia and 561 caregivers of children with hemophilia in 10 countries). The impact of hemophilia B may not be accurately reflected in the HERO results, as ~75% of respondents described issues affecting males with hemophilia A. To address the needs of this population, the Bridging Hemophilia B Experiences, Results and Opportunities Into Solutions (B-HERO-S) was developed as a pilot study in the United States in collaboration with the hemophilia community. The analysis reported here assessed engagement in recreational activities and changes to treatment regimens around activities as reported by 299 adults with hemophilia B and 150 caregivers of children with hemophilia B. Nearly all adults with hemophilia B (98%) experienced a negative impact on their participation in recreational activities due to hemophilia-related issues, and most caregivers (90%) reported that hemophilia B had a negative impact on their child's engagement in recreational activities. One of the main reasons identified for discontinuing past activities was the risk of bruising or bleeding (adults/children with hemophilia B, 49%/41%). In particular, adults with hemophilia B reported a history of activity-related bleeding, and most adults decreased their participation in high-risk activities as they aged. Substantial percentages of adults and children with hemophilia B (including mild/moderate severity) altered their treatment regimens to accommodate planned activities. These findings may help inform guidelines for individualizing treatment regimens around participation in recreational activities based on hemophilia severity, baseline factor level, and activity risk and intensity.
血友病对活动的心理社会影响最近在血友病经历、结果与机会（HERO）研究中得到了调查（该研究涉及10个国家的675名血友病患者和561名血友病儿童的照料者）。B型血友病的影响可能未在HERO研究结果中得到准确反映，因为约75%的受访者描述了影响甲型血友病男性患者的问题。为满足这一人群的需求，“将B型血友病经历、结果与机会融入解决方案”（B-HERO-S）作为一项试点研究在美国与血友病群体合作开展。此处报告的分析评估了299名成年B型血友病患者和150名B型血友病儿童照料者所报告的娱乐活动参与情况以及围绕活动的治疗方案变化。几乎所有成年B型血友病患者（98%）因与血友病相关的问题在参与娱乐活动方面受到负面影响，且大多数照料者（90%）报告称B型血友病对其孩子参与娱乐活动有负面影响。确定的停止既往活动的主要原因之一是瘀伤或出血风险（成年/儿童B型血友病患者，分别为49%/41%）。特别是，成年B型血友病患者报告有与活动相关的出血史，且大多数成年人随着年龄增长减少了对高风险活动的参与。相当比例的成年和儿童B型血友病患者（包括轻度/中度严重程度）改变了他们的治疗方案以适应计划中的活动。这些发现可能有助于为根据血友病严重程度、基线凝血因子水平以及活动风险和强度围绕参与娱乐活动制定个体化治疗方案提供指导。
