Breaking barriers: Enhancing hospital support for sickle cell disease management.

Abstract

Background: Sickle cell disease (SCD) management requires comprehensive care coordination and specialised resources, particularly in endemic regions. We describe the implementation and outcomes of a unique model, the Integrated Centre for Sickle Cell Disease (CID), in French Guiana, a region with high SCD prevalence.

Design and methods: We established a comprehensive SCD care centre incorporating dedicated clinical space, specialised staff, and integrated emergency services. The model included 24/7 specialist availability, priority admission protocols, and a day hospital facility for managing acute complications.

Results: The CID currently serves 740 patients (280 children, 406 adults, including 23 elderly patients aged 60-72 years). In 2022, the centre documented 4673 patient visits, comprising 2209 consultations and 417-day hospitalisations. Of these day hospitalisations, 229 were for vaso-occlusive crises, 147 for erythropheresis, 13 for therapeutic bleeding, and 28 for plasmaphaeresis. Most notably, following CID's establishment, emergency room deaths were eliminated, compared to 2-3 annual deaths previously reported due to acute splenic sequestration. The centre maintained this achievement over eight consecutive years.

Conclusions: The CID model demonstrates successful implementation of comprehensive SCD care in a resource-limited setting. The complete elimination of emergency room deaths over 8 years, along with improved patient retention and follow-up, suggests that this model could serve as a template for other regions with high SCD prevalence. The success of this approach highlights the importance of integrated, specialised care in managing SCD effectively.