A Review of Pathophysiology, Prevalence, Diagnosis, Therapeutic Approaches, and Nutritional Considerations in Sickle Cell Disease
Rawan Mohammed Hazi Al-Ruwaili 1*, Nuha Naif Alshammari 2, Salman Ghazi Al-Faridi 3
Journal of Angiotherapy 7(1) 1-12 https://doi.org/10.25163/angiotherapy.7110144
Submitted: 09 November 2022 Revised: 08 January 2023 Published: 09 January 2023
Abstract
Sickle cell disease (SCD) is a chronic genetic disorder caused by mutations in the β-globin gene, resulting in the production of hemoglobin S (HbS). This mutation causes erythrocytes to assume a sickle shape, leading to vaso-occlusive crises, hemolysis, and multi-organ damage. SCD, inherited in an autosomal recessive pattern, manifests in various forms such as sickle cell anemia (HbSS), sickle cell-hemoglobin C (HbSC), and sickle cell-beta thalassemia, with HbSS being the most severe. The disease burden is disproportionately high in Sub-Saharan Africa, where SCD contributes to significant child mortality. Despite advances in treatments such as hydroxyurea, stem cell transplantation, and gene therapy, therapeutic outcomes remain suboptimal. The pathophysiology of SCD is complex, involving factors such as hemolysis, chronic inflammation, and oxidative stress. Emerging therapies, including voxelotor and crizanlizumab, target specific aspects of SCD, such as HbS polymerization and vascular occlusion. The role of fetal hemoglobin (HbF) induction in modulating disease severity has garnered attention, though strategies to enhance HbF production require further development. Additionally, nutritional interventions have been recognized as crucial in managing SCD, as individuals often experience nutritional deficiencies exacerbated by increased energy and protein demands. This review explores the global prevalence, clinical manifestations, and therapeutic approaches to SCD, emphasizing the need for novel treatments and comprehensive public health initiatives to address this pervasive condition. Further research is essential to improve diagnostic methods, treatment modalities, and overall patient outcomes, particularly in resource-limited regions where SCD's impact is most severe.
Keywords: Sickle cell disease, Hemoglobin S, Genetic mutations, Therapeutic strategies, Nutritional interventions
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