Seth Tulika, Udupi Shashank, Jain Suman, Bhatwadekar Seema, Menon Nandakumar, Jena Rabindra Kumar, Kumar Ravindra, Ray Shomik, Parmar Bharat, Goel Anil Kumar, Vasava Ashvin, Dutta Anupam, Samal Priyanka, Ballikar Riya, Bhat Deepa, Dolai Tuphan Kanti, Bhattacharyya Jina, Shetty Disha, Mistry Manish, Jain Dipty
Department of Hematology, All-India Institute of Medical Sciences, New Delhi, Delhi, India.
Department of Oncology Medical Affairs, Novartis Healthcare Private Limited, Mumbai, Maharashtra, India.
Br J Haematol. 2025 Jan;206(1):296-309. doi: 10.1111/bjh.19829. Epub 2024 Oct 28.
Sickle cell disease (SCD) with vaso-occlusive pain crisis (VOC) significantly impacts patient well-being and often results in extensive healthcare resource utilization. This study assessed the VOC burden, its management and its impact on patients' quality of life (QoL). A cross-sectional observational study was conducted between November 2021 and June 2022, including 1000 SCD patients from high-prevalence states in India. Data on demographics, clinical characteristics, VOC severity, management and QoL were collected. The study revealed that 33.5% of patients reported at least one VOC episode during the study period. In the year prior to their enrolment, 836 (83.60%) patients reported at least one VOC episode, with an equal proportion of 407/487 (83.6%) adults and 429/513 (83.6%) paediatric patients, reducing their QoL across all domains compared to patients without VOC. Of these, 469/1000 patients (46.9%) experienced ≥3 VOC episodes. Additionally, 764/1000 (76.40%) patients managed their VOCs at healthcare facilities, with 501/1000 (50.1%) requiring inpatient admissions. Further, 71.80% of patients received Hydroxyurea (HU) therapy. The study depicts the severity of the Arab-Indian haplotype in Indian SCD patients visiting healthcare settings based on high VOC burden. This highlights the urgent need for better management strategies and resource allocation for these patients.
镰状细胞病（SCD）伴血管闭塞性疼痛危象（VOC）严重影响患者的健康状况，并常常导致大量医疗资源的使用。本研究评估了VOC的负担、其管理方式及其对患者生活质量（QoL）的影响。2021年11月至2022年6月期间进行了一项横断面观察性研究，纳入了来自印度高流行州的1000例SCD患者。收集了有关人口统计学、临床特征、VOC严重程度、管理方式和生活质量的数据。研究显示，33.5%的患者在研究期间报告至少发生过一次VOC发作。在入组前一年，836例（83.60%）患者报告至少发生过一次VOC发作，其中成年患者407/487例（83.6%）和儿科患者429/513例（83.6%）的比例相同，与未发生VOC的患者相比，他们在所有领域的生活质量均有所下降。其中，469/1000例患者（46.9%）经历了≥3次VOC发作。此外，764/1000例（76.40%）患者在医疗机构接受了VOC治疗，其中501/1000例（50.1%）需要住院治疗。此外，71.80%的患者接受了羟基脲（HU）治疗。该研究描述了基于高VOC负担的印度SCD患者在就医时阿拉伯-印度单倍型的严重程度。这突出了迫切需要为这些患者制定更好的管理策略和资源分配方案。
