Brief about Sickle Cell Disease

What is Sickle Cell Disease?

It is inherited disease of red blood cells which can cause attacks of pain and damage to vital organs and can lead to early death. Sickle cell disease affects a protein inside the red blood cells called hemoglobin. The disease occurs when a person inherits two sickle cell genes or a combination of one sickle cell gene plus any one of several other abnormal hemoglobin genes. 

Hemoglobin in the red blood cells carries oxygen from the lungs and takes it to every part of the body. The main hemoglobin in the red blood cells of people with sickle cell disease is different from the usual hemoglobin. Red blood cells containing mainly normal hemoglobin are normal and flexible. But when oxygen is released by the red blood cells in people with sickle cell disease, the abnormal hemoglobin forces the cells to become distorted and rigid, often looking like the C- shaped farm tool called a sickle.

Sickle cell tend to become trapped and to be destroyed in the liver and in the spleen. This results in a shortage of red blood cells, or anemia, which, when severe, can cause an affected person to be pale, short of breath and easily tired. Certain conditions, such as enlargement of the spleen and infections, may worsen the anemia by speeding up destruction of red blood cells. Infections also can reduce red blood cell production.
 

What Special Problems do babies and young children with Sickle Cell Disease have?

Infants and young children with sickle cell disease are especially vulnerable to severe bacterial infections, such as those that cause meningitis and blood infection (septicemia). Infections are the leading cause of death in children with sickle cell disease. However, early diagnosis and treatment dramatically reduce the risk of infections and death.

 It is very important for babies and children with sickle cell disease to receive the regular childhood vaccinations.

What causes the pain in Sickle Cell Disease?

At Times, sickle cells become stuck in tiny blood vessels. When they get stuck, the cells pile up and block the blood vessels, this cuts off the blood supply to nearby tissues so that no cells can get through to bring oxygen to them, without oxygen, the area becomes inflamed and starts to hurt. This blockage of blood vessels is known as a sickle cell pain episode or crises. Pain episodes may be very severe and need to be treated in the hospital with pain killing drugs or, more often, they are milder and can be treated at home. If the blockage is long lasting, .it can destroy areas of tissues. Sickle Cell pain occurs most frequently in bones.

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