Sickle Cell Disease: Pathophysiology, Complications, and Recent Therapeutic Insights in a low Resource Economy

Abstract

Sickle cell disease is the commonest form of haemoglobinopathy caused by elevations of intraerythrocyte and total blood viscosity that causes increased morbidity and mortality. It also and also has effect on the healthcare sector and economy of many nations predominantly in sub-Saharan Africa countries. Sickle cell disease occurs as a mutation in the b-globin chain of the hemoglobin molecule of red blood cells that leads to its characteristic sickle shape. Sickle cell disease is seen more among black Africans and African Americans and has also been linked with its occurrence with malaria dominated areas. It is one of the commonest genetic disorder in low resource economies around the world including the most populous black nation-Nigeria, which bears the largest health burden in sub-Saharan Africa. Despite this, enough is not being done in Nigeria with respect to financing of researches and advancements of drugs to battle the disease. Pathophysiology of the clinical features of sickle cell disease is often described in 3 phases namely. Vaso-occlusion is the most common leading factor to many other clinical features. Although, prognosis of sickle cell disease is dependent on age, access to care, availability of drugs, haplotype and trained personnel. stem cell transplantation, still remain the only curative management for now, although a recent therapy is undergoing trial to combat the root cause of the disease with other drugs being used to reduce the effects of pain. In this article, we offer a detailed understanding of Pathophysiology of sickle cell disease, examine common treatment of the disease in Nigeria. We also discuss emerging therapies undergoing trial to combat the root cause and manifestation of the effect of Sickle cell disease. In-depth review of literature on the study was retrieved from the relevant Hematological journals and textbooks abstracted and indexed in PubMed, Google Scholar, ProQuest and Science Direct This article connects the pathophysiology and the management of sickle cell disease alongside the psychosocial influence it has on its patients in a low resource economy like Nigeria. The study also discovered that researches on alternative drugs that provide better treatment option and better quality of life than the primary sickle cell disease drug-Hydroxyurea, has reached advanced stages. Although curiosity in sickle cell research has flourished, more clinical trials need to commence and subjected to more thorough examinations and analysis than have been used in the past especially in Nigeria.