Sickle Cell Disease

Background of sickle cell disease Sickle cell disease is one of the most common diseases in African people and the Mediterranean and the Middle East. About 70,000 Americans are known to suffer from this disease and about 2 million people have this characteristic (ie, they have a single genetic variation). Sickle cell disease is an autosomal recessive disorder caused by mutation of hemoglobin β globin gene. Autosomal means that it is not associated with sex chromosomes. Therefore, any parent can pass that gene to the child.

In order for you or your child to inherit sickle cell disease, both parents must have sickle cell disease (two sickle cell genes) or sickle cell properties (sickle cell gene). There is a variant of sickle cell disease called sickle cell disease or sickle-shaped dysentery, which is a serious condition, but sometimes it is not too serious. In case of sickle cell disease, give the sickle cell gene to the child. Sickle cells are characterized by hereditary hematologic diseases accounting for about 8% of African Americans. Individuals with sickle cell properties possess only one defective gene, unlike sickle cell disease, in which the patient has two genes causing abnormal hemoglobin production, usually with no health problems associated with sickle cell, normal Live a life. In rare cases, extreme symptoms such as severe dehydration symptoms or severe physical activity can cause serious health problems in patients with sickle cell characteristics including sudden death.

Sickle cell disease (SCD) or sickle cell anemia is a major genetic disease that affects most countries in Africa. In sickle cell disease, normal round erythrocytes look like crescent shaped satellites. Round red blood cells can move easily through blood vessels, but pleated cells are connected to each other and can cause thrombosis. In this area, most children who suffer from the most severe illness die from under 5 years of age, usually with infection or severe blood loss. In Cameroon, Republic of Congo, Gabon, Ghana, Nigeria, prevalence is between 20% and 30%, but in some parts of Uganda it reaches as much as 45%.

The loss of elasticity of erythrocytes is central to the pathophysiology of sickle cell disease. Normal red blood cells are very elastic and allow cells to deform through capillary vessels. In sickle cell disease, hypoxic pressure promotes recurrent episodes of erythrocyte sickle cell and sickle erythrocyte, damaging the cell membrane and lowering the elasticity of the cell. Once normal oxygen pressure is restored, these cells can not return to normal shape. Therefore, these hard blood cells can not deform as they pass through thin capillaries, causing vascular occlusion and ischemia.