Different Therapeutic Interventions and Mechanisms of Action of Antisickling Agents Currently in Use in Sickle Cell Disease Management

Abstract

Sickle cell disease is a genetic disorder caused by sickle haemoglobin. In many forms of the disease, the red blood cells can change shape upon deoxygenation due to abnormal sickle haemoglobin polymerisation. The haemoglobin proteins stick to each other, causing the cell to have a rigid surface and sickle shape and in the process damaging the red blood cell membrane, causing the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischaemia and infarction (which may cause organ damage), such as stroke. Incidences of the disease are found most commonly in people of African descent and less commonly in people of Mediterranean, Latino, East Indian, and Arab descent (in that order). In African countries such as Nigeria, Gabon, Ghana, and the Republic of Congo, the prevalence of the sickle cell trait is between 20% and 30%, with the disease affecting ˜2–3% of the population. Herbal formulations prepared from plants are known as phytomedicines and are effective in keeping the patient out of a crisis state and enabling them to live stable lives in society, even though the faulty S gene is not eradicated but instead managed. This review highlights some of the therapeutic options in use in the management of sickle cell disease with a view to inspiring future research on this subject.