PURPOSE Patients with sickle cell disease (SCD) may experience sickle cell-related pain crises, also referred to as vaso-occlusive crises (VOCs), which are a substantial cause of morbidity and mortality. The study explored how VOC frequency and severity impacts health-related quality of life (HRQoL) and work productivity. METHODS Three hundred and three adults with SCD who completed an online survey were included in the analysis. Patients answered questions regarding their experience with SCD and VOCs, and completed the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) and the Workplace Productivity and Activity Impairment: Specific Health Problem (WPAI:SHP). Differences in ASCQ-Me and WPAI:SHP domains were assessed according to VOC frequency and severity. RESULTS Nearly half of the patient sample (47.2%) experienced ≥ 4 VOCs in the past 12 months. The most commonly reported barriers to receiving care for SCD included discrimination by or trouble trusting healthcare professionals (39.6%, 33.3%, respectively), limited access to treatment centers (38.9%), and difficulty affording services (29.4%). Patients with more frequent VOCs reported greater impacts on emotion, social functioning, stiffness, sleep and pain, and greater absenteeism, overall productivity loss, and activity impairment than patients with less frequent VOCs (P < 0.05). Significant impacts on HRQoL and work productivity were also observed when stratifying by VOC severity (P < 0.05 for all ASCQ-Me and WPAI domains, except for presenteeism). CONCLUSIONS Results from the survey indicated that patients with SCD who had more frequent or severe VOCs experienced deficits in multiple domains of HRQoL and work productivity. Future research should examine the longitudinal relationship between these outcomes.

中文翻译：

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镰状细胞病成人的血管闭塞性危机的频率和严重程度与健康相关的生活质量和工作生产率之间的关系。

目的镰状细胞病（SCD）患者可能会经历镰状细胞相关的疼痛危机，也称为血管闭塞性危机（VOC），这是发病率和死亡率的重要原因。该研究探讨了VOC的频率和严重程度如何影响与健康相关的生活质量（HRQoL）和工作效率。方法包括333名完成在线调查的SCD成人纳入分析。患者回答了有关使用SCD和VOC的经历的问题，并完成了成人镰状细胞生活质量测量信息系统（ASCQ-Me）以及工作场所生产力和活动障碍：特定健康问题（WPAI：SHP）。根据VOC频率和严重程度评估ASCQ-Me和WPAI：SHP域的差异。结果近一半的患者样本（47。在过去的12个月中，有2％）经历了≥4个VOC。据报道，接受SCD护理的最常见障碍包括医疗保健专业人员的歧视或信任他们的麻烦（分别为39.6％，33.3％），进入治疗中心的机会有限（38.9％）和难以获得服务（29.4％）。挥发性有机化合物含量较高的患者与挥发性有机化合物含量较低的患者相比，对情绪，社交功能，僵硬，睡眠和疼痛以及缺勤，整体生产力下降和活动能力受损的影响更大（P <0.05）。当按VOC严重性分层时，还观察到对HRQoL和工作效率的显着影响（对于所有ASCQ-Me和WPAI域，P均<0.05，但代表出席除外）。结论调查的结果表明，具有更频繁或严重VOC的SCD患者在HRQoL和工作效率的多个方面都存在缺陷。未来的研究应检查这些结果之间的纵向关系。