Sickle Cell Anemia is disorder of the blood in which our red blood cells become sickle shaped and their age is 10 to 20 days. The normal shape of red blood cells is soft and flexible so that they can pass through our blood vessels efficiently. Sickle shaped blood cells can stuck in the blood vessels and block them.
Causes:
Sickle cell anemia is a genetic disease and occurs due to mutation in beta hemoglobin chains in red blood cells. Due to changes in normal hemoglobin, abnormal hemoglobin develops. These mutated molecules are unable to carry oxygen smoothly. When the oxygen supply reduces, red blood cells develop sickle cell shape and become rigid and stiff.
Symptoms:
In this condition, body parts are affected in different way. If it happens in chest or abdomen, it causes pain. If sickle cells become in feet or hands, they get swelling, if they are in eye, visionary problems occur. Due to brittle nature of sickle cells, oxygen delivery to the organs is delayed and fatigue is caused. Other conditions include kidney problems, jaundice, stress, poor hydration and frequent infections.
History:
When we talk about history of this disease, it was discovered in Africa five thousand years ago. But officially it was described before 100 years ago. In 1910, a student named Walter complained of pain and was discovered with sickle cell anemia. According to survey, in 400 African American births, one person is affected with Sickle cell anemia.
The people who have prevalent malaria are usually carrier of sickle cell anemia. These people are usually from Africa, India, Mediterranean and Saudi Arabia. It occurs mostly in black Americans and black Africans. In Americans, this disorder occurs in one individual out of 500 births.
Treatment:
There are different treatments available for sickle cell anemia depending upon its severity:
In order to regain hydration, intravenous fluids are taken and they bring red blood cells to normal state. If RBCs are dehydrated, they deform and adopt the sickle shape.
It is important to treat the infections otherwise the patient may go into stress and that results in sickle cell crisis.
Blood transfusions from the healthy persons also improve the oxygen transport. In case of oxygen deficiency, oxygen is given through oxygen masks. It helps to ease breathing and increase oxygen levels in the blood.
In severe cases of pain during sickle cell crisis, medication like morphine is used to treat pain.
In children of age less than 16 years who are suffering from severe complications, bone marrow transplant is done after finding matching donor.
Reference:
1- https://www.emedicinehealth.com/sickle_cell_crisis/article_em.htm#sickle_cell_disease_symptoms