In recent years, policymakers and medical experts have expressed alarm about the common incidence of the genetic disorder sickle cell all over the world. While most parties agree that the issue deserves attention, consensus revolves around how to respond to the problem. This review paper examines the disorder, sickle cell, its signs and symptoms, mode of transmission and treatment methods. The information gathered for this paper was obtained from the work of various researchers and their findings on the disease and its effects on the various parts of the body.
A review of Literature
Sickle cell anemia is an inherited genetic blood disorder, which causes defects hemoglobin leading to the production of abnormal “sickle shaped” red blood cells. …show more content…
(1,2,3) Africans are recorded as having the most recorded cases of the sickle cell disease in the world. Hence all groups of African descent most likely have high cases of this disease. African-Americans have an incidence of about 1 in every 350 for infants born with the disorder and 1 in 12 for the trait. Commonly found among people of African and Mediterranean descent, its variability in its morbidity changes from individual to individual. It was discovered by James B. Herrick in his 1910 case report on an anemic West Indian student and since then has become one of the most common diseases in the world. The point mutation occurs in the beta globin chain of hemoglobin. Glutamic acid (hydrophilic) is replaced by valine (hydrophobic) at the sixth position of hemoglobin. This substitution of the non polar valine for a charged glutamate residue forms a protrusion on the beta chain that helps it attach to a site on the alpha chain of another hemoglobin in the deoxy state. Attachment of the deoxy alpha and beta chains leads to aggregation and eventually distortion of the red blood cell structure.(sickling) .They can also lead to blockage of circulation in the spleen (autosplenectomy), vaso-occlusive crisis which include blockage of circulation to important organs eg. brain, and Hemolytic anemia. These conditions occur when the sickle shaped …show more content…
This disease is hereditary and hence both parents being carriers increases the chances of the child being affected. These symptoms can significantly impact the quality of life of the patients. A major sign that occurs in early childhood is development of pain in various parts of the body. This occurs because the sickle shaped cells block the flow of blood to various parts of the body. This could lead to strokes if the supply the brain is blocked. It could also lead to acute chest syndrome, where the lungs are unable to take in oxygen as a result of sickled cells blocking the blood vessels. In the heart it could cause pulmonary hypertension due to increased level of blood flow. Sickled cells block the flow of blood to the heart by narrowing the diameter of the blood vessels. The body therefore increases the pressure of blood to enable flow to all parts of the body. This causes an increase in blood pressure in patients with the disease. Another sign is the enlargement of the spleen causing high reticulocyte count. The spleen is the major organ involved in fighting infections and degrading red blood cells. The sickled shapes of the cells decreases the ability of the spleen to break them down at a fast rate, and hence there is sequestration of red blood cells in the spleen causing life threatening anemia and the enlargement. The inability of the spleen to function efficiently makes the body susceptible
A review of Literature
Sickle cell anemia is an inherited genetic blood disorder, which causes defects hemoglobin leading to the production of abnormal “sickle shaped” red blood cells. …show more content…
(1,2,3) Africans are recorded as having the most recorded cases of the sickle cell disease in the world. Hence all groups of African descent most likely have high cases of this disease. African-Americans have an incidence of about 1 in every 350 for infants born with the disorder and 1 in 12 for the trait. Commonly found among people of African and Mediterranean descent, its variability in its morbidity changes from individual to individual. It was discovered by James B. Herrick in his 1910 case report on an anemic West Indian student and since then has become one of the most common diseases in the world. The point mutation occurs in the beta globin chain of hemoglobin. Glutamic acid (hydrophilic) is replaced by valine (hydrophobic) at the sixth position of hemoglobin. This substitution of the non polar valine for a charged glutamate residue forms a protrusion on the beta chain that helps it attach to a site on the alpha chain of another hemoglobin in the deoxy state. Attachment of the deoxy alpha and beta chains leads to aggregation and eventually distortion of the red blood cell structure.(sickling) .They can also lead to blockage of circulation in the spleen (autosplenectomy), vaso-occlusive crisis which include blockage of circulation to important organs eg. brain, and Hemolytic anemia. These conditions occur when the sickle shaped …show more content…
This disease is hereditary and hence both parents being carriers increases the chances of the child being affected. These symptoms can significantly impact the quality of life of the patients. A major sign that occurs in early childhood is development of pain in various parts of the body. This occurs because the sickle shaped cells block the flow of blood to various parts of the body. This could lead to strokes if the supply the brain is blocked. It could also lead to acute chest syndrome, where the lungs are unable to take in oxygen as a result of sickled cells blocking the blood vessels. In the heart it could cause pulmonary hypertension due to increased level of blood flow. Sickled cells block the flow of blood to the heart by narrowing the diameter of the blood vessels. The body therefore increases the pressure of blood to enable flow to all parts of the body. This causes an increase in blood pressure in patients with the disease. Another sign is the enlargement of the spleen causing high reticulocyte count. The spleen is the major organ involved in fighting infections and degrading red blood cells. The sickled shapes of the cells decreases the ability of the spleen to break them down at a fast rate, and hence there is sequestration of red blood cells in the spleen causing life threatening anemia and the enlargement. The inability of the spleen to function efficiently makes the body susceptible