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

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.