Sickle cell disease is a genetic blood disorder that affects the hemoglobin, the protein molecule in the red blood cells responsible for delivering oxygen to the body cells and throughout the body. The disease is characterized by the presence of abnormal hemoglobin that forces the red cells to assume a sickle shape. Normal blood cells are disc-shaped which allows them to be flexible as they navigate through the blood vessels. The abnormal hemoglobin forms rod-like structures immediately after unloading the oxygen. The sickle-shaped red blood cells are inflexible making it difficult to navigate the blood vessels and can stick on the vessel walls blocking or slowing down the flow of oxygen to the tissues. Also, these sickle cells die within a few days usually 10-20 days instead of the usual 120 days. As a result, the blood is always chronically short of the red blood cells which could ultimately result in anemia (NIH).
Affected Population
Sickle cell is a hereditary disease hence, cannot be transmitted from one person to another through contact. According to the Center for Disease Control and Prevention, the disease is most prevalent in individuals who have ancestry backgrounds from South America, Central America, sub-Saharan Africa, Saudi Arabia, Italy, Greece, and Turkey. Sickle cell is more prevalent in America than other parts of the world affecting nearly 100,000 Americans (CDC).
Causes
The abnormal hemoglobin that causes the sickle cell disease is caused by a genetic defect in the gene that coordinates the production of part of the hemoglobin known as beta globin. The defect caused by the gene causes that hemoglobin to work abnormally with the underlying problem in the beta globin part. Carriers of this defective gene will more likely have the complication than those who carry the trait. Individuals with sickle cell trait inherited a defective gene from one parent and a normal gene from the other. Carriers of the defective gene can easily pass it on to their offspring (CDC).
Diagnosis
A blood test known as sickle cell test is used to determine if an individual has a sickle cell trait or sickle cell disease. This is important since most sickle cell trait carriers have no symptoms but can pass it on to their offspring. Early symptoms include fussiness and fatigue, jaundice or dactylitis. Other symptoms include paleness and shortness of breath, delayed growth, frequent infections and periodic episodes of pain and hand-foot syndrome. The sickle cell test looks for the availability of abnormally shaped red blood cells instead of their normal disc shape.
Treatment
There is no single treatment for sickle cell disease every individual is treated depending on their symptoms. Most treatments involve managing the periodic pain experienced by the individuals. This originates when the sickle cells block the small blood vessels and clog the blood flow. Patients are encouraged to avoid situations or places of high altitude and try not to get too hot or cold. Individuals who develop the hand-foot syndrome can receive pain medicine as well and encouraged to increase their intake of fluids such as water. Another complication of sickle cell disease is anemia. Patients with severe anemia can be treated with blood transfusions (CDC). However, multiple transfusions might result into more health problems due to the variation of the iron content in the blood, a patient may be required to undergo additional treatment to regulate the amount of iron in the blood stream.
Works Cited
CDC. Sickle Cell Disease (SCD). 14 Sep. 2015. Web. 30 Jan. 2016.
NIH. What Is Sickle Cell Disease? 12 Jun. 2015. Web. 30 Jan. 2016
