Scalone L, Mantovani L G, Mannucci P M, Gringeri A
Centre of Pharmacoeconomics, Department of Pharmacological Sciences, University of Milan, and Department of Internal Medicine and Dermatology, Mangiagalli Hospital Foundation, Milan, Italy.
Haemophilia. 2006 Mar;12(2):154-62. doi: 10.1111/j.1365-2516.2006.01204.x.
Inhibitors represent one major complication of haemophilia treatment, as they increase the risk of bleeding, physical disability and mortality. The Cost Of Care Inhibitors Study (COCIS) showed that modern strategies applied to manage patients with inhibitors adsorb high amounts of resources but provide satisfactory levels of Health-Related Quality-of-Life (HR-QoL). This paper focuses on determinants of HR-QoL in inhibitory patients. Fifty adult patients, enrolled by 11 Italian Haemophilia Centres, were clinically assessed and filled in two HR-QoL generic questionnaires: the EuroQol instrument (EQ-5D) and the Short Form-36 (SF-36). According to our results, bleeding frequency and inhibitor titres were not found associated with HR-QoL. Global HR-QoL, and in particular the physical component of wellbeing in these patients was found negatively associated with their orthopaedic condition: the EQ-5D Visual Analogue Scale (P<0.001) scores, the SF-36 domain 'physical functioning' and 'physical component summary' (P<0.01) scores were found significantly correlated with the orthopaedic joint score, even after adjusting for patients' age. These results were confirmed by those from the EQ-5D profile. To conclude, the COCIS study is the first study showing that HR-QoL in inhibitory patients is impaired by their orthopedic status, while other aspects do not seem to influence patients' global wellbeing. Our results suggest that while the management of this complication is satisfactory, the attention has now to be focused on the prevention of the orthopaedic problems in these patients, which nowadays constitute one of the most important aspects to be considered in the haemophilia care.
抑制剂是血友病治疗的一个主要并发症，因为它们会增加出血、身体残疾和死亡的风险。护理成本抑制剂研究（COCIS）表明，用于管理有抑制剂的患者的现代策略会消耗大量资源，但能提供令人满意的健康相关生活质量（HR-QoL）水平。本文重点关注有抑制剂的患者的HR-QoL的决定因素。由11个意大利血友病中心招募的50名成年患者接受了临床评估，并填写了两份HR-QoL通用问卷：欧洲五维度健康量表（EQ-5D）和简明健康调查问卷（SF-36）。根据我们的结果，未发现出血频率和抑制剂滴度与HR-QoL相关。发现这些患者的总体HR-QoL，尤其是健康的身体方面，与他们的骨科状况呈负相关：即使在对患者年龄进行调整后，仍发现EQ-5D视觉模拟量表（P<0.001）得分、SF-36领域“身体功能”和“身体成分汇总”（P<0.01）得分与骨科关节评分显著相关。这些结果在EQ-5D概况中得到了证实。总之，COCIS研究是第一项表明有抑制剂的患者的HR-QoL受到其骨科状况损害的研究，而其他方面似乎并不影响患者的总体健康。我们的结果表明，虽然对这种并发症的管理是令人满意的，但现在注意力应集中在预防这些患者的骨科问题上，而这在当今血友病护理中是最重要的方面之一。
