5599344 IMPACT OF HOSPITAL ADMISSION FOR VASO-OCCLUSIVE CRISIS ON HEALTH-RELATED QUALITY OF LIFE IN CHILDREN WITH SICKLE CELL DISEASE

Abstract

Background: Sickle cell disease (SCD) is the most common inherited hemoglobinopathy characterized by chronic hemolytic anemia, vaso-occlusive crises (VOCs), sensitivity to bacterial infections and multi-organ failure. Painful VOCs may severely impair the life of children with SCD and their families, resulting in affected health-related quality of life (HRQoL). Currently, we lack insight in how HRQoL changes over time following hospitalization for VOC in children with SCD. Aims: The aim of this study is to evaluate the longitudinal impact of hospital admission for VOCs on the health-related quality of life in children with SCD up to one year after hospitalization. Methods: In this retrospective cohort study, children between the age of 8 and 18 years with SCD treated at the Amsterdam University Medical Centers were included. As part of standard care, once a year, these children completed patient reported outcomes measures on the KLIK PROM portal (www.hetklikt.nu). HRQoL was measured with the Pediatric Quality of Life Inventory (PedsQL). This questionnaire consists of 23 items covering the following 4 domains: physical, emotional, social and school functioning. The score ranges from 0-100, and a higher score indicates better HRQoL. Data were analysed from the electronic medical records of patients, who filled out at least two PedsQL questionnaires between 2012 and 2021. The effect of the occurrence and frequency of hospital admissions for VOCs on the HRQoL was evaluated 3, 6, 9 and 12 months following hospitalization using linear mixed model adjusted for age and SCD genotype. Results: In total, 94 children were included with a median age of 11.8 years (IQR: 9.3 – 14.1). The total follow-up duration was 451 patient-years with a median follow-up time of 4.0 years (IQR 2.0 - 6.0). The PedsQL was completed 455 times in total. The majority of the included patients was male (64%), while 57 patients (61%) had the severe genotype: HbSS or HbSβ0 thalassemia. The median total PedsQL score was 78 points (IQR 71-85). During follow-up, 37 patients (41%) had been admitted to the hospital for a VOC. Hospitalization for VOC in the last 12 months led to a decrease of 3.2 points (p=0.053) in HRQoL as compared to patients without hospitalization. Recurrent admissions for VOC in the previous 12 months further decreased HRQoL with 2.3 points for every additional admission (p=0.035). The significantly affected domains were physical functioning at 6, 9 and 12 months, and school performance 12 months after hospitalization. Conclusion: The negative effects of hospital admission for VOC on HRQoL aggregate and persist for up to 12 months after hospitalization. These findings highlight the importance of using patient-reported outcomes in children with SCD to screen for the need for extra support after hospitalization for VOCs to optimize HRQoL.