Sickle cell anemia (SCA) is a classification of the most common form of sickle cell disease. This class of sickle cell disease is genetic and is caused by the homozygous mutation of the beta-hemoglobin gene (beta S allele) in red blood cells [2]. Individuals who are diagnosed with sickle cell anemia have deformed (sickle shaped) red blood cells that have a lower lifespan [3]. This causes irreversible tissue damage and causes a vaso-occlusive crisis, which is pain in the abdomen, bones and chest and lungs. According to the Sickle cell Disease Association of Canada, there are currently an estimated 5000 individuals who are living with sickle cell disease and 1 in every 2500 babies will be diagnosed [4]. The WHO indicates that SCA affects 72,000
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This alteration in the amino acids, which is in the 6th position of the beta-globin, causes a distorted shape of red blood cells [1]. The mutation causes the binding of beta 1 and beta 2 of two hemoglobin chains, forming a crystalized structure. The binding of the two chains is caused by the hydrophobic motif created from the mutated 6th amino acid in the hemoglobin [1]. The resulting red blood cell becomes dehydrated and deoxygenated and loses it’s biconcave shape. By using transmission electron microscopy, it is evident that sickle shaped cells have groupings of fibers that run along the long axis of the cell [9]. The fibres, which have a rope like structure, are made of 14 polymer strands [9]. Unlike erythrocytes, sickle cells differ in density. The least dense sickle cells are more predominant because there are a greater number of reticulocytes (immature red blood cells) that have less hemoglobin, which means there are less Hbs within the erythrocyte [9]. However, the more dense a sickle cell is, the more likely it will obtain a irregular shape and contribute to vascular occlusion aspects of SCD [9]. The most dense sickle cells cannot return to a the normal shape of a blood cell even when oxygenated due to the irreversible damage in the membranes [8]. Apart from the fact that it contributes adhesion of vascular tissue, it also …show more content…
HbS polymerizes in environments with lower oxygen concentrations, which causes deformation in erythrocytes that have HbS [9]. The deformation of erythrocytes clog blood vessels and stop oxygen from moving into tissues where it is essential for organ functions and survival [6]. The inflammation of the body is a response to the polymerization of HbS in erythrocytes. Cation channels open in order to prevent dehydration of erythrocytes and reduced
This alteration in the amino acids, which is in the 6th position of the beta-globin, causes a distorted shape of red blood cells [1]. The mutation causes the binding of beta 1 and beta 2 of two hemoglobin chains, forming a crystalized structure. The binding of the two chains is caused by the hydrophobic motif created from the mutated 6th amino acid in the hemoglobin [1]. The resulting red blood cell becomes dehydrated and deoxygenated and loses it’s biconcave shape. By using transmission electron microscopy, it is evident that sickle shaped cells have groupings of fibers that run along the long axis of the cell [9]. The fibres, which have a rope like structure, are made of 14 polymer strands [9]. Unlike erythrocytes, sickle cells differ in density. The least dense sickle cells are more predominant because there are a greater number of reticulocytes (immature red blood cells) that have less hemoglobin, which means there are less Hbs within the erythrocyte [9]. However, the more dense a sickle cell is, the more likely it will obtain a irregular shape and contribute to vascular occlusion aspects of SCD [9]. The most dense sickle cells cannot return to a the normal shape of a blood cell even when oxygenated due to the irreversible damage in the membranes [8]. Apart from the fact that it contributes adhesion of vascular tissue, it also …show more content…
HbS polymerizes in environments with lower oxygen concentrations, which causes deformation in erythrocytes that have HbS [9]. The deformation of erythrocytes clog blood vessels and stop oxygen from moving into tissues where it is essential for organ functions and survival [6]. The inflammation of the body is a response to the polymerization of HbS in erythrocytes. Cation channels open in order to prevent dehydration of erythrocytes and reduced