Orthopedics and Traumatology Department, Juan A. Fernandez Hospital, Buenos Aires, Argentina.
Haemophilia. 2012 Jul;18 Suppl 4:54-60. doi: 10.1111/j.1365-2516.2012.02874.x.
Inhibitors are a serious complication, considerably increasing the morbidity, mortality and cost of treatment in this patient group. The challenge of treating people with haemophilia (PWH) with inhibitors can be met by a well-coordinated multidisciplinary team specialized in haemophilia. Each treatment centre must run a screening programme to detect inhibitors within their population and develop protocols to treat these patients. The treatment centre in Buenos Aires developed a screening programme that tests all our patients twice a year, ensuring early detection of inhibitors and early treatment of complications. In 2006, we analysed the quality of life (QOL) of non-inhibitor patients and compared it with inhibitor patients detected by this programme and found no differences in QOL measured by the SF36 questionnaire and no differences in school absenteeism. When diagnosis of the inhibitor does not come from a screening programme, its presence is suspected upon a lack of response to conventional replacement therapy for musculoskeletal bleeding, losing the 'golden moment' of treatment. This complication is much more serious when facing a traumatic bleed. In this situation, the lack of early diagnosis can lead to permanent damage or even death. Due to the cost of bypassing factors and the lack of experience of the medical team in the treatment of patients with inhibitors, many treatments that would improve the QOL of patients are instituted in an insufficient manner. Therefore, patients with haemophilia and inhibitors are often untreated or undertreated in their community. Orthopaedic surgeons and physiotherapists play a key role in the treatment of these patients and should be included in therapeutic decision making and most specifically in the postoperative treatment of patients with haemophilia and inhibitors. It is important that these patients have quick access to a trained therapeutic team in order to obtain an early diagnosis and treatment plan to prevent the evolution of the pathological process. Early treatment is cost-effective in maintaining and improving the QOL of patients. Experience in patients with haemophilia and inhibitors is not very extensive. Today, this situation is changing, with several treatment centres beginning to perform surgeries in these most complex patients, giving them a chance to improve their QOL. This article presents the experience of experts from various fields involved in treating patients with inhibitors from a developed and developing world perspective.
抑制剂是一种严重的并发症，会显著增加这组患者的发病率、死亡率和治疗成本。具有抑制剂的血友病患者（PWH）的治疗挑战可以由专门治疗血友病的多学科团队来应对。每个治疗中心都必须运行一个筛查计划，以在其人群中检测抑制剂，并制定治疗这些患者的方案。布宜诺斯艾利斯的治疗中心开发了一个筛查计划，每年对我们所有的患者进行两次检测，确保早期发现抑制剂并早期治疗并发症。2006 年，我们分析了非抑制剂患者的生活质量（QOL），并将其与该计划检测到的抑制剂患者进行了比较，发现 SF36 问卷测量的 QOL 没有差异，也没有在学校缺勤方面的差异。当抑制剂的诊断不是来自筛查计划时，当针对肌肉骨骼出血的常规替代疗法缺乏反应时，就会怀疑存在抑制剂，从而失去治疗的“黄金时刻”。在面对创伤性出血时，这种并发症更加严重。在这种情况下，早期诊断的缺失可能导致永久性损伤甚至死亡。由于旁路因子的成本和治疗抑制剂患者的医疗团队缺乏经验，许多本可以提高患者生活质量的治疗方法都没有得到充分实施。因此，血友病和抑制剂患者在社区中经常得不到治疗或治疗不足。矫形外科医生和物理治疗师在这些患者的治疗中发挥着关键作用，应参与治疗决策，特别是在血友病和抑制剂患者的术后治疗中。重要的是，这些患者能够快速获得训练有素的治疗团队，以便早期诊断和制定治疗计划，从而防止病理过程的发展。早期治疗在维持和提高患者的生活质量方面具有成本效益。具有抑制剂的血友病患者的经验不是很丰富。如今，这种情况正在发生变化，一些治疗中心开始为这些最复杂的患者进行手术，为他们提高生活质量提供了机会。本文从发达国家和发展中国家的角度介绍了各个领域的专家在治疗抑制剂患者方面的经验。
