Sickle-cell Anemia

The more general text about the issue of anemia can be found here.

 

Sickle-cell anemia is an inherited genetic disease, which is particularly widespread in Africa and North America, where it came with African slaves. It can not be regarded as a typical disease as it may have also some positive aspects for its bearers.

Causes

It is a congenital genetic mutation that causes the body of the affected individual to produce a different type of hemoglobin than normal healthy persons. The hemoglobin is a blood pigment located within the erythrocytes. The pathological form of hemoglobin forces the red blood cells to deform giving them a crescent shape. While passing through small blood vessels, the deformed erythrocytes may damage and tend to disintegrate causing anemia. The disease is typical by sudden bouts of disintegration of large numbers of erythrocytes. Such bouts are usually caused by stressful events, infections, etc.

 

However, there is also some positive effect. The erythrocytes with the sickle-cell hemoglobin are more resistant to the malaria and increase survival chance of the bearer in malarial regions.

 

Sickle-cell anemia

 

Symptoms

Sickle-cell anemia manifests typically with symptoms of the anemic syndrome. The patient is tired, feels dizzy and may frequently faint. The lack of hemoglobin and red blood cells causes pallor of the skins and mucous membranes – this is evident in conjunctiva. The anemic patient suffers from shortness of breath during physical exercise and palpitations as the heart muscle tries to deliver more blood with oxygen to tissues.

 

During the bouts of red blood cells disintegration, large amount of hemoglobin is released and it is processed into a pigment known as bilirubin. The excessive amount of this pigment may cause episodes of jaundice, i.e. yellowish color of skin and mucous membranes (see the related text). The bouts are also accompanied with nausea, abdominal pain and vomiting.

Diagnosis

The patient suffers from the above-mentioned symptoms and the blood test shows the presence of anemia. The examination in a hematologic laboratory may prove the deformed shape of the erythrocytes characteristic for the sickle-cell anemia.

Prevention

It is a genetic disorder and therefore, it can not be prevented. It is advisable to prevent any stressful events to decrease the risk of sudden bouts of the disease.

Treatment

The disease can not be treated. The anemia occurring during the attacks may be solved by blood transfusion.