PB2227: HEALTH-RELATED QUALITY OF LIFE OVER TIME IN PEDIATRIC PATIENTS WITH SICKLE CELL DISEASE ADMITTED FOR VASO-OCCLUSIVE CRISIS.

Abstract

Background: Sickle cell disease (SCD) is the most common inherited hemoglobinopathy characterized by chronic hemolytic anemia, vaso-occlusive crises (VOCs), multi-organ damage and an impaired health-related quality of life (HRQoL). Previous studies have shown that the occurrence of VOCs affects the HRQoL substantially in children with SCD. However, there is limited evidence about the HRQoL changes over time following hospitalization for VOC. Aims: The aim of this study is to evaluate the longitudinal impact of hospital admission for VOCs on the HRQOL in pediatric patients with SCD. 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, these children complete once a year Patient Reported Outcomes measures (PROMs) on the KLIK PROM portal (www.hetklikt.nu). HRQoL was measured with the Pediatric Quality of Life Inventory (PedsQL) (score ranging from 0-100, a higher score indicates better QoL). Data were collected from the medical files 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 using a linear mixed model 3, 6, 9 and 12 months after hospital admission, adjusted for age and SCD genotype. Results: In total, 109 children were included with a median age of 11.8 years (IQR: 9.2 – 14.2), including 72 patients (66%) with the severe genotype: HbSS or HbSβ0. During follow-up, 45 patients (41%) were admitted to the hospital for a VOC. If hospitalization occurred 6 and 12 months prior to the PedsQoL measurement, a decrease of 3.61 (p=0.049) and 3.80 points (p=0.007) in HRQoL respectively, was seen compared to patients without hospitalization. As for the frequency of VOC admission in the preceding 12 months, a decrease of 2.28 points in HRQoL was observed for every additional admission (p=0.012). Summary/Conclusion: The negative longitudinal association between hospital admission for VOC and HRQoL in pediatric patients with SCD gets worse and persists up to 12 months. These findings highlight the importance of monitoring using PROMS in clinical practice and extra attention and support for the negative consequences of the hospital admission for VOCs, affecting the HRQoL.