Oral presentation: Meindert Manshande
Sickle Cell Disease and Sickle Cell Trait on Curaçao: Incidence and Pediatric Complications. A Retrospective Cohort Study From a Single CentreOral presentation by Meindert Manshande: Sickle Cell Disease and Sickle Cell Trait on Curaçao: Incidence and Pediatric Complications. A Retrospective Cohort Study From a Single Centre
Introduction
Sickle cell disease (SCD) is a clinically heterogeneous autosomal recessive disorder that causes substantial morbidity and early mortality when not diagnosed and treated early. Despite increasing awareness of SCD in the Caribbean, epidemiological data for Curaçao are scarce. This study aimed to determine the birth incidence of SCD and sickle cell trait (SCT) and to describe the frequency of the clinical complications observed in affected children.
Methods
A retrospective, population-based cohort study was conducted using high-performance liquid chromatography (HPLC) screening data for all newborns in Curaçao born between 1995 and 2024. Confirmed genotypes (HbSS, HbSC, HbSβ⁰/β⁺) were classified as SCD and HbAS as SCT. Additionally, a descriptive review of pediatric medical records was performed to assess SCD-related complications.
Results
Among 40,231 screened newborns, 2,156 (5.6%) had SCT and 131 (0.3%) had SCD. No significant differences in incidence were observed between hospital and community births or across time. Of 74 children with SCD (mean age 10.3 years; 58.1% male), 63.5% experienced at least one complication. The most frequent were anaemia (43.2%) and transfusion requirement (21.6%), and were most common in children with HbSS.
Conclusion
SCD affects approximately 1 in 300 births in Curaçao, with a carrier rate exceeding 5%. The disease presents a substantial, yet under-recognised, health burden. The high complication frequency underscores the urgent need for a structured national neonatal screening and follow-up program to ensure timely diagnosis and care. We suggest an island-wide intervention for optimalisation of the screening and inclusion of DNA analyses for definitive diagnoses.