Dolatkhah R, Fakhari A, Pezeshki M Z, Shabanlouei R, Tavassoli N, Gholchin M
Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Haemophilia. 2014 May;20(3):376-81. doi: 10.1111/hae.12321. Epub 2013 Nov 20.
The availability of safe and effective factor replacement therapies, in persons with haemophilia (PWH), has in some countries answered the basic need for treatment of these patients. The findings suggest that adult patients who have always been on prophylaxis reported significantly better physical functioning, and thus better quality of life. This study is designed to evaluate the QoL in adult PWH, by focusing on social determinants of QoL and their relationship with health-related dimensions, in Tabriz, Iran. The survey instrument was a self-report 36 items questionnaire, 'A36 Hemofilia - QoL', which is a disease-specific questionnaire for the assessment of the health-related QoL in adults living with haemophilia. A total of 100 haemophilia A and B patients, aged over 17 years participated in this study within 1 year. QoL total score was 71.88 (±26.89 SD). Patients who treat in our Hemophilia Treatment Center, had better QoL score (P = 0.000), and education has a significant impact on the social aspects of QoL (P = 0.18). The QoL was very poor in urban area in contrast to patients who lived in the city (54.45 vs. 74.21 respectively). Single patients have a better QoL than married patients (76.56 vs. 68.50 respectively). Our results showed that low education and lack of awareness of the diseases among PWH lead to reduce of QoL and more disease complications. More and wider treatment and psychological care for improving quality of life of these patients are seriously recommended.
在一些国家，血友病患者（PWH）能够获得安全有效的凝血因子替代疗法，这满足了这些患者的基本治疗需求。研究结果表明，一直接受预防治疗的成年患者身体功能明显更好，生活质量也更高。本研究旨在通过关注伊朗大不里士地区血友病成年患者生活质量的社会决定因素及其与健康相关维度的关系，来评估他们的生活质量。调查工具是一份自我报告的36项问卷“A36 Hemofilia - QoL”，这是一份针对血友病成年患者健康相关生活质量评估的特定疾病问卷。在1年内，共有100名年龄超过17岁的甲型和乙型血友病患者参与了本研究。生活质量总分是71.88（标准差±26.89）。在我们血友病治疗中心接受治疗的患者生活质量得分更高（P = 0.000），教育对生活质量的社会方面有显著影响（P = 0.18）。与居住在城市的患者相比，城市地区患者的生活质量非常差（分别为54.45和74.21）。单身患者的生活质量比已婚患者更好（分别为76.56和68.50）。我们的结果表明，血友病患者教育程度低和对疾病缺乏认识会导致生活质量下降和更多疾病并发症。强烈建议提供更广泛的治疗和心理护理，以改善这些患者的生活质量。
