Department of Coagulation Disorders, Malmö University Hospital, Malmö, Sweden.
Haemophilia. 2010 Jul 1;16(4):592-6. doi: 10.1111/j.1365-2516.2009.02189.x. Epub 2010 Feb 2.
Patients with moderate and severe haemophilia are evaluated on a regular basis at their haemophilia centres but patients with mild haemophilia are seen less often because of fewer problems related to their disease. The needs of patients with milder forms of haemophilia, however, are often underestimated, both by the patient and staff at healthcare facilities. This study evaluated the knowledge of disease and adherence to treatment among patients with severe, moderate and mild haemophilia. This was a prospective multicentre study performed in Haemophilia Centres in Scandinavia. A total of 413 (67%) of 612 patients aged >25 years with mild, moderate and severe haemophilia completed a self-administered questionnaire. The mean age of the respondents was 49.7 years (range 25-87 years). Of the 413 respondents, 150 had a mild, 86 had a moderate and 177 had a severe form of haemophilia. A total of 22 (5%) patients did not know the severity of their disease, and 230 (56%) patients knew the effect of factor concentrate in the blood. Of the 413 respondents, 53 (13%) of the cohort never treated a haemorrhage. Patients with mild haemophilia, P </= 0.001, were the least likely to treat a haemorrhage. The relative number of patients who were afraid of virus transmission by factor concentrate was about similar in the three groups, 27% of those with severe haemophilia, 26% with moderate and 24% with mild haemophilia. This study shows that the amount of knowledge among haemophilia patients about their disease and treatment is somewhat limited, and demonstrates the importance of continually providing information about haemophilia and treatment, especially to patients with a mild form of the disease.
患有中重度血友病的患者会在血友病中心定期接受评估，但由于疾病相关问题较少，轻度血友病患者的就诊频率较低。然而，轻度血友病患者的需求往往被低估，无论是患者还是医疗机构的工作人员。本研究评估了重度、中度和轻度血友病患者对疾病的认知和治疗依从性。这是一项在斯堪的纳维亚血友病中心进行的前瞻性多中心研究。共有 612 名年龄>25 岁的轻度、中度和重度血友病患者中的 413 名（67%）完成了一份自我管理问卷。应答者的平均年龄为 49.7 岁（范围 25-87 岁）。在 413 名应答者中，150 人患有轻度血友病，86 人患有中度血友病，177 人患有重度血友病。共有 22 名（5%）患者不知道自己疾病的严重程度，230 名（56%）患者了解血液中因子浓缩物的作用。在 413 名应答者中，53 名（13%）患者从未治疗过出血。患有轻度血友病的患者（P </= 0.001）最不可能治疗出血。对因子浓缩物传播病毒的恐惧的患者比例在三组中大致相似，重度血友病患者为 27%，中度血友病患者为 26%，轻度血友病患者为 24%。本研究表明，血友病患者对自身疾病和治疗的了解程度有些有限，这表明持续提供关于血友病和治疗的信息非常重要，尤其是对于患有轻度疾病的患者。
