Vuong Caroline, Moussa Ibtissame, van Muilekom Maud M, Heijboer Harriët, Rettenbacher Eva, Haverman Lotte, Twisk Jos, Fijnvandraat Karin, Eckhardt Corien L
Department of Pediatric Hematology, Amsterdam UMC - Emma Children's Hospital, University of Amsterdam, Amsterdam, Netherlands.
Department of Child and Adolescent Psychiatry and Psychosocial Care, Amsterdam UMC - Emma Children's Hospital, University of Amsterdam, Amsterdam, Netherlands.
Pediatr Blood Cancer. 2023 Dec;70(12):e30691. doi: 10.1002/pbc.30691. Epub 2023 Sep 25.
Sickle cell disease (SCD) is characterized by vaso-occlusive crises (VOCs) that impair the health-related quality of life (HRQoL). The aim of this study is to evaluate the impact of hospitalization for VOCs on HRQoL in children with SCD over time.
In this longitudinal cohort study, children aged 8-18 years diagnosed with SCD at the Amsterdam UMC were included between 2012 and 2021. HRQoL was annually measured as part of standard care using the Pediatric Quality of Life Inventory. The impact of hospitalization for VOC on HRQoL was evaluated using linear mixed models 3, 6, 9, and 12 months after hospitalization. The effect of frequency of hospitalization for VOC on HRQoL was evaluated over the last 12 months.
In total, 94 children with SCD were included with a median age of 11.8 years (interquartile range [IQR]: 9-14). Thirty-seven patients (39%) had been hospitalized for a VOC. Hospitalization for VOC led to a decrease of 3.2-4.8 points in total HRQoL compared to patients without hospitalization, most pronounced 3 months after hospitalization. Recurrent admission for VOC in the last 12 months was associated with a decrease of 2.3 points in total HRQoL (p = .04). The most affected subscale was physical functioning.
The adverse effects of hospitalization for VOC in children with SCD persist up to 12 months after hospitalization. After hospitalization for VOC, extra attention and support for its negative impact on HRQoL are recommended. This study also underlines the importance of systematically measuring HRQoL, allowing clinicians to intervene accordingly.
镰状细胞病（SCD）的特征是血管闭塞性危机（VOCs），这会损害健康相关生活质量（HRQoL）。本研究的目的是评估因VOCs住院对SCD儿童HRQoL随时间的影响。
在这项纵向队列研究中，纳入了2012年至2021年间在阿姆斯特丹大学医学中心被诊断为SCD的8至18岁儿童。作为标准护理的一部分，每年使用儿童生活质量量表测量HRQoL。在住院后3、6、9和12个月，使用线性混合模型评估因VOC住院对HRQoL的影响。在过去12个月内评估因VOC住院频率对HRQoL的影响。
总共纳入了94名SCD儿童，中位年龄为11.8岁（四分位间距[IQR]：9 - 14）。37名患者（39%）因VOC住院。与未住院的患者相比，因VOC住院导致总HRQoL下降3.2 - 4.8分，在住院后3个月最为明显。过去12个月内因VOC反复入院与总HRQoL下降2.3分相关（p = 0.04）。受影响最严重的子量表是身体功能。
SCD儿童因VOC住院的不良影响在住院后长达12个月持续存在。因VOC住院后，建议对其对HRQoL的负面影响给予额外关注和支持。本研究还强调了系统测量HRQoL的重要性，使临床医生能够据此进行干预。
