SICKLE CELL ANEMIA

Sickle Cell Anemia

Sickle Cell Anemia

Introduction

Sickle cell anemia is an inherited blood disorder, characterized primarily by chronic anemia and periodic episodes of pain. The cause of sickle cell anemia is a genetic disorder in which the hemoglobin is defective. When the hemoglobin molecules gives up their oxygen, some will cluster together. This forms long, rod-like structures which cause the red blood cells to stiffen and assume a sickle shape. With the deformity, the cells cannot squeeze through the blood vessels, depriving organs and tissues of oxygen. Normal blood cells live for about one hundred twenty day in the blood, while the defective sickled cells live only ten to twenty. Because of their shortened lifespan, the blood is constantly deficient in red blood cells, a condition known as anemia (Desai 2004).

Discussion

Sickle cell anemia is a genetic disorder that resulted from a mutation which occurred thousands of years ago in Africa, the Mediterranean basin, the Middle East, and India. At the time, deadly malaria epidemics cause great numbers of deaths. Those who inherited the sickle cell gene had an advantage: unlike children with normal red blood cells, they could survive malaria, and grow old enough to have their own offsprings, thus passing along the mutation. As the population migrated, so did the mutation. In countries where malaria is not a problem, like the United States, the sickle cell mutation is disorder, no longer providing a survival trait of value. Instead it is a serious threat. If a child inherits two of the recessive sickle cells genes, they will be born with sickle cell anemia .In the United States alone, sickle cell anemia affects approximately seventy-two thousand people, most of whose ancestors descended from Africa. About two million Americans carry the gene for sickle cell anemia. The clinical course of sickle cell anemia does not follow a single pattern; some patients have mild symptoms, and some have very severe symptoms:

Hand-foot syndrome: When the small blood vessels in the hands or feet are blocked, pain and swelling can result, along with fever. This may be the first symptom of sickle cell anemia in infants Fatigue, paleness, and shortness of breath--all symptoms of anemia, or a shortage of red blood cells. Pain that occurs unpredictably in any body organ or joint, wherever the sickled blood cells block oxygen flow to the tissues. The frequency and amount of pain varies. Some patients have painful episodes (also called crises) less than once a year, and some have as many as fifteen or even more episodes in a year. The pain can last for a few hours, or for weeks. For especially severe, prolonged pain, the patient may have to be hospitalized and treated with painkillers and intravenous fluids. Pain is the principal symptom of sickle cell anemia in both children and adults Eye problems are a common occurrence in people who suffer from sickle cell anemia(Aldrich 2007). The retina does not receive the proper blood circulation, causing it to deteriorate, sometimes to ...