Research Centre on Public Health (CESP), University of Milano-Bicocca, Monza, Italy.
Neurology, Public Health, Disability Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Haemophilia. 2024 Mar;30(2):449-462. doi: 10.1111/hae.14926. Epub 2023 Dec 26.
A huge amount of data about psychosocial issues of people with haemophilia (PwH) are available; however, these materials are fragmentary and largely outdated, failing to reflect the impact of current treatment strategies.
Describing the influence of illness on psychosocial aspects of adult PwH (≥18 years) and caregivers of children with haemophilia (CPwH) without inhibitors, in Italy.
Surveys (for adult PwH, CPwH and haemophilia specialists) were developed by a multidisciplinary working group and conducted from November 2019 to June 2020.
A total of 120 PwH without inhibitors and 79 CPwH completed the survey. Adult patients reported a significant impairment in many psychosocial aspects, including working activities, relations with family members and social relations. Caregivers generally reported better scores in all aspects of the survey. Mobility, Pain and Mental health domains of EQ-5D were the most frequently impaired in both patients and caregivers, reducing the perceived quality of life. Genetic counselling was an important issue, 53% of CPwH declaring unawareness of their carrier status, as well as the psychological support offered by the reference center, 67.0% of respondents reporting that no psychological support was provided at the time of diagnosis communication.
This study provides information about PwH's and CPwH's point of view in the current scenario of continuous innovations in haemophilia treatment and management furthermore, updated insights on psychosocial problems faced by patients and caregivers are reported.
有大量关于血友病患者（PwH）心理社会问题的数据；然而，这些材料是零碎的，而且在很大程度上已经过时，无法反映当前治疗策略的影响。
描述意大利无抑制剂的成年 PwH（≥18 岁）和儿童血友病患者（CPwH）照顾者的疾病对心理社会方面的影响。
由一个多学科工作组制定调查问卷，并于 2019 年 11 月至 2020 年 6 月进行调查。
共有 120 名无抑制剂的 PwH 和 79 名 CPwH 完成了调查。成年患者报告在许多心理社会方面存在严重障碍，包括工作活动、与家庭成员的关系和社会关系。照顾者在调查的所有方面普遍报告了更好的分数。患者和照顾者中 EQ-5D 的移动性、疼痛和心理健康领域受损最频繁，降低了感知生活质量。遗传咨询是一个重要问题，53%的 CPwH 表示不知道自己的携带者状态，以及参考中心提供的心理支持，67.0%的受访者报告在诊断沟通时没有提供心理支持。
本研究提供了关于 PwH 和 CPwH 在血友病治疗和管理不断创新的当前背景下的观点信息，此外，还报告了患者和照顾者面临的心理社会问题的最新见解。
