Abstract
Background
Sickle cell disease (SCD) is an inherited hemoglobinopathy due to a defectively structured hemoglobin S leading to red blood cell malformation, hemolysis, and recurrent vaso-occlusion crisis. Sickle cell disease is a global public health concern with the rampant spread of the disease in sub-Saharan Africa, the Middle East, and African descent population.
Objective
This report includes the overall pathophysiology of SCD and the practice of treatment, including curative, supportive, and pharmaceutical treatment.
Methods
Pathophysiology and treatment of SCD were searched for in published literature. Molecular mechanism, complication, and therapeutic intervention information was obtained.
Results
SCD results from polymerization of deoxygenated HbS into sickle morphology with resultant vascular occlusion and organ damage. Progress has occurred through increased hydroxyurea treatment, stem cell transplant, and gene modification techniques but with continued disparities in access.
Conclusion
Effective management of SCD is founded on an integrated, multidisciplinary disease-modifying therapy, care, and curative technology development approach. Access to therapy with correct diagnosis forms the foundation towards reducing the disease burden.
Key Words: Sickle cell disease, pathophysiology, hydroxyurea, vaso-occlusion, stem cell transplantation, gene therapy