Healthcare Utilization and Clinical Burden of Sickle Cell Disease in a Portuguese Emergency Department

Background/Objectives: Sickle Cell Disease (SCD) is a rare hemoglobinopathy in Portugal, where epidemiological data remain limited. National newborn screening has revealed a higher-than-expected incidence, particularly in urban areas, underscoring the importance of understanding acute care utilization. Vaso-occlusive crises (VOC) frequently lead to emergency department (ED) attendance; however, no prior studies have characterized SCD care in Portuguese EDs. This study aimed to describe patterns of emergency care use, clinical presentation, and outcomes in a Portuguese hospital, and to contextualize these findings within international standards. Methods: This retrospective observational study reviewed SCD-related emergency visits to the ED of a district hospital from January 2021 to December 2023. Patients aged ≥18 years with SS, Sβ, or SC genotypes were included. Data collected included demographics, triage category, presenting complaints, pain scores, waiting times, treatments administered, diagnostic investigations, ED length of stay (LOS), disposition and 72-hour revisit rates. Descriptive and bivariate analyses were performed, with significance defined as p < 0.05. Results: A total of 264 ED episodes from 93 unique patients (mean age 30 years; 61% male) were identified, with a mean of 2.8 visits per patient over three years. Limb pain (31%) and back pain (28%) were the most common presenting complaints, and 83% of patients reported severe pain at triage. Most episodes were classified as very urgent (65.2%) or urgent (27.3%), yet the mean time to physician assessment was 58 minutes. The mean ED LOS was 13 hours, substantially longer than reported internationally, and 45.8% of visits resulted in hospital admission. Laboratory testing was performed in 91% of episodes and imaging in 39%. Analgesia was administered in 98% of visits, with frequent use of multimodal regimens; however, pethidine was used in 28% of episodes despite guideline recommendations discouraging its use. The 72-hour revisit rate was low (~4%), with six return visits requiring readmission. Conclusions: SCD patients in Portugal frequently present to the ED with high-acuity VOC requiring intensive evaluation and analgesia. ED LOS substantially exceeded international benchmarks, likely reflecting structural factors such as overcrowding, boarding delays and absence of dedicated acute pain pathways. The persistence of outdated opioid practices further highlights opportunities for quality improvement. These findings emphasize the need for locally adapted, guideline-concordant ED pathways to optimize the acute management of SCD and improve patient outcomes.