Abstract
Background Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin S, leading to red blood cell deformities, chronic hemolysis, and frequent vaso-occlusive crises (VOC). While advancements in medical care have improved survival rates, adults with SCD continue to face substantial challenges in their quality of life (QoL) due to chronic pain, recurrent VOCs, and various complications. This study aimed to evaluate the health-related quality of life (HRQoL) in adult patients aged 14 years and above with SCD and identify key factors influencing patient outcomes using the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me). Methods A cross-sectional study was conducted at the Prince Sultan Oncology Center, King Salman Northwest Armed Forces Hospital, Tabuk, Saudi Arabia, between December 2019 and May 2020. The study population comprised adult SCD patients attending outpatient clinics. The QoL was assessed using the ASCQ-Me Short Form, which evaluates five domains: emotional impact, social functioning, pain impact, sleep impact, and stiffness impact. Additionally, a nine-item SCD Medical History Checklist was used to evaluate complications and treatments. Data were collected through structured one-on-one interviews. Scores were transformed into T-scores (mean = 50, standard deviation (SD) = ±10) based on standardized guidelines. Inferential analysis was conducted to compare QoL domains between groups stratified by VOC frequency and severity using the Mann-Whitney U test, with a significance threshold of p ≤ 0.05. Results A total of 53 adult SCD patients were surveyed, with the majority aged 25-34 years (50%). Gender distribution was nearly equal, with 50.9% male participants. The prevalence of complications was notable, with 31% reporting chronic pain, 26% reporting gallstones, and 9% reporting avascular necrosis. Laboratory findings revealed a mean hemoglobin level of 8.63 g/dL (SD: 1.59) and an average fetal hemoglobin (HbF) level of 10.59% (SD: 5.64). The frequency of VOCs varied, with 30% of patients reporting no VOC in the past year, while 57% experienced two to three VOCs. Patients with higher VOC frequency (≥4 per year) reported significantly lower scores across all QoL domains except stiffness (p < 0.05). Higher VOC severity was associated with poorer sleep quality and social functioning (p < 0.05). Pain severity was also a critical determinant, with more than 55% of patients rating their last pain episode as severe or extreme. Conclusion The study highlights the significant burden of pain and VOCs on the quality of life in adult SCD patients. Frequent VOCs and chronic complications such as avascular necrosis and gallstones were major contributors to reduced HRQoL. Findings emphasize the need for comprehensive, multidisciplinary care approaches targeting pain management, psychological support, and functional independence. The ASCQ-Me tool proved valuable for identifying specific domains of impairment and guiding patient-centered interventions. Future research should focus on longitudinal studies to explore the efficacy of tailored interventions and address the ongoing challenges faced by this vulnerable population.
Published Version
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