Abstract
PurposePatients 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.MethodsThree 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.ResultsNearly 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).ConclusionsResults 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.
Highlights
Sickle cell disease (SCD) is a hemoglobinopathy that causes red blood cells to lose their oxygen carrying capacity and is associated with severe, systemic vascular complications
Inconsistent responses were defined either as (1) different responses to 2 items regarding the number of vaso-occlusive crises (VOCs) experienced in the past 12 months in conjunction with a survey completion time of < 9 min or (2) inconsistent responses to items within the ASCQ-Me Pain Episode domain
The goal of this study was to explore the disease experience of patients with SCD, focusing primarily on better understanding the ways in which patients are impacted by the frequency and severity of VOCs
Summary
Sickle cell disease (SCD) is a hemoglobinopathy that causes red blood cells to lose their oxygen carrying capacity and is associated with severe, systemic vascular complications. VOCs are caused by multi-cell adhesion or cell clusters that block or reduce blood flow, and are a substantial cause of morbidity in patients with SCD; severe crises have been associated with increased mortality [11]. These events are unpredictable and can cause disruption and hardship in the lives of patients, sometimes requiring medical attention in emergency departments or sickle cell urgent care centers, or leading to inpatient hospitalization [12]. Previous occurrence of VOCs has been linked to deficits in domains of health-related quality of life (HRQoL) such as general health, vitality, and bodily pain [6]
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