Sickle Cell Disease

Introduction       

Sickle cell disease is also known as sickle cell anemia and is defined as a disease in which an individual's body produces sickle or crescent shaped red blood cells (Buchanan et al. 2004). The shapes of sickle-shaped red blood cells differ greatly from the normal blood cells which are disc-shaped. Red blood cells are important in human body since they contain hemoglobin which carries oxygen molecules from the lungs to all parts of the body. Sickle cells develop a crescent shape because of the type of hemoglobin they contain that is referred to as hemoglobin S or sickle hemoglobin (David, 2006). The sickle cells are characterized of being short-lived sticky, and stiff. Therefore, they tend to get stuck in the rumen of blood vessels thus blocking normal flowing of blood. The flow of sickle-shaped blood cells in the blood vessels causes pain, organ damage, and serious infection (Jones, 2008).     

Causes of sickle cell disease

Sickle cell anemia is a genetically inherited disease. Those individuals suffering from the disease genetically acquire two genes for hemoglobin S - one gene from each parent (David, 2006). In case an individual genetically acquire a gene for sickle hemoglobin from one parent and a gene for normal hemoglobin from the other parent, is said to have a sickle cell trait (Jones, 2008). Individuals with sickle cell trait experience few symptoms and therefore lead normal lives. Some individuals with sickle cell trait may develop medical complications. Individuals can inherit hemoglobin S from parents with a sickle cell trait. If both parents have an abnormal gene and a normal gene, the offspring have a probability of 25 percent to inherit two normal genes; a probability of 50 percent to inherit one abnormal gene and one normal gene; and a probability of 25 percent to inherit two normal genes (Buchanan et al. 2004). Sickle cell disease exhibits a number of signs and symptoms in individuals who have it.  

Signs and symptoms of sickle cell disease

The symptoms of sickle cell disease vary from one individual to another. Some individuals experience mild symptoms while others experience severe symptoms which necessitate hospitalization for medication (David, 2006). Most infants start to experience the signs and symptoms of sickle cell disease after they have reached the age of four months. The signs and symptoms of sickle cell disease are usually associated with pain and anemia. Symptoms related to anemia include fatigue, dizziness, shortness of breath, coldness in feet and hands, jaundice, and pale skin (Jones, 2008). While the symptoms associated with pain include sickle cell crisis which is suddenly experienced all over the body since it affects lungs, bones, joints, and abdomen. The crises usually take place as blood flow to organs is blocked by the sickle shaped red blood cells, which causes pain as well as organ damage. The sickle cell crises increase when the affected person's body undergoes dehydration. Therefore the tendency of sickle cell crisis can be reduced by drinking plenty of fluids. Sickle cell crises can bring about many complications including the crisis of the spleen, hand-foot syndrome, pulmonary hypertension, acute chest syndrome, delayed puberty and growth in children, eye problems, stroke, unwanted erections in men, multiple organ failure, and gallstones (David, 2006).  

Diagnosis and treatment of sickle cell disease

According to John (2008), sickle cell disease can be diagnosed by use of simple blood test which can detect whether an individual has hemoglobin S. It will be of prime importance to have the disease diagnosed earlier because treatment measures so that severe signs and symptoms cannot be experienced. The diagnosis and treatment of sickle cell disease is done by hematologists. Treatments of the sickle cell disease are meant to prevent organ damage, infections, and strokes; control complications; and relieve pain. It has been found that stem cell transplantation can perfectly treat some people (Austin & Jennifer, 2008). If sickle cell disease is detected earlier, the individual should be treated with antibiotics to keep away infections. Plenty of fluids should be taken to avoid dehydration because the sickle cell crisis becomes severe on dehydration. Blood transfusion is necessary when sickle cell anemia becomes worse to avoid some complications like stroke and acute chest syndrome (John, 2008).