Sickle Cell Anemia

Overview

            Sickle cell anemia is the most prevalent form of the group of sickle cell diseases. In this disorder, the body of the individual makes red blood cells that are sickle-shaped. That is, the red blood cells are shaped like a crescent instead of the normal disc-shape. Red blood cells carry hemoglobin, an iron rich protein vital in transporting of oxygen from the lungs of the individual to the other parts of the body. The sickle-cells have hemoglobin called hemoglobin S or sickle hemoglobin. This abnormal sickle hemoglobin in return forces the red blood cells to develop that distinguishing crescent shape and they are characterized by stickiness and stiffness.  Hence, they block the flow of blood in body vessels in the organs and limbs and this may cause infections, pain and even organ damages in the long run(Institute)

Symptoms

            The symptoms associated with the disease only manifest after 4 months of age. Crises, the painful episodes affect the chest and the long bones. Symptoms include, fatigue, bone pain, abdominal pain attacks, fever, breathlessness, a rapid heart rate, delayed puberty and delayed growth, paleness, jaundice (yellowing of the skin and the eyes), paleness of the body, and in the adults and adolescents, ulcers on their lower legs. Other symptoms associated with sickle cell anemia include: frequent urination, strokes, excessive thirst, skin ulcers, chest pains, blindness or at least poor eyesight and priapism which is prolonged and painful erection and occurs in about 40 percent of the men with sickle cell anemia(PubMed Health).

Causes and the Genetics

            Anemia occurs when the blood has lower than the needed number of the red blood cells. Anemia can also occur if there is enough number of red blood cells but those red blood cells do not contain in them enough hemoglobin. The normal red blood cells are supposed to last in the blood stream for approximately 120 days and then die off. For the people with sickle cell disease, the red blood cells die only after 10-20 days and at this rate, the bone marrow is incapable of making new red blood cells to replace those dying off. Hence the patients will have low levels of red blood cells.

            Sickle cells disease is a lifelong-inherited disease. People with sickle cell anemia are born with the disease. Genetically, a patient from birth inherits two recessive genes for sickle hemoglobin from each of the two parents. For those patients who inherit only one recessive gene from one parents then a normal (dominant) gene from the second parent have a sickle cell trait. These individuals with the sickle cell trait can pass on the recessive hemoglobin to their young ones but they do not have the disease.

Population

            Sickle cell anemia is more common among people of Mediterranean and African descent. Lately however, doctors and scientists have also discovered a prevalence of the disease among people of central and South American descent, Middle East and Caribbean(MedLine Plus).

Treatment

            Since the disease is incurable, at least in the present, treatment is aimed at controlling and managing the symptoms and to a large extent to minimize the frequency of the crises. The treatment may include pain medicines, blood transfusions to prevent stroke and plenty of fluids. It has to be noted that treatment for the patients needs to be an ongoing activity even at those times when the patients are not experiencing the painful episodes of crises.      

References

Health, PubMed.Sickle Cell Anemia. 28 Feb 2011. Viewed on 4 April 2012. Web.

Institute, National Heart Lung and Blood. What Is Sickle Cell Anemia? 1 Feb 2011. Viewed on 4 April 2012. Web.

Plus, MedLine.Sickle Cell Anemia. 21 March 2012. Viewed on 4 April 2012. Web.