UNASSIGNED: The access to replacement therapy and prophylaxis has driven a dramatic reduction in mortality and resultant increase in life expectancy. Beyond this, the abolition of bleeds and preservation of joint health represent the expected, but rarely attained, goals of haemophilia treatment and care. These outcomes also do not address the complexity of health-related quality of life impacted by haemophilia and its treatment.
CONCLUSIONS: Capitalizing on the major potential of therapeutic innovations, \'Normalization\' of haemostasis, as a concept, should include the aspiration of enabling individuals to live as normal a life as possible, free from haemophilia-imposed limitations. To achieve this-being supported by the data reviewed in this manuscript-the concept of haemostatic and life Normalization needs to be explored and debated within the wider multidisciplinary teams and haemophilia community.
■获得替代疗法和预防疗法已导致死亡率的急剧下降和预期寿命的增加。除此之外,消除出血和保持共同健康代表了预期,但很少达到,血友病治疗和护理的目标。这些结果也没有解决血友病及其治疗影响的健康相关生活质量的复杂性。
结论:利用治疗创新的主要潜力,止血正常化,作为一个概念,应该包括使个人尽可能正常生活的愿望,没有血友病施加的限制。为了实现这一目标-在本手稿中审查的数据的支持下-需要在更广泛的多学科团队和血友病社区中探索和辩论止血和生活正常化的概念。