Sickle cell disease is a condition where normal hemoglobin commonly referred to as hemoglobin A (Hb A) is substituted by sickle cell hemoglobin referred to as hemoglobin S (Hb S). Other conditions that lead to the disease are inherited together with Hb S such as beta-thalassemia and hemoglobin C (Hb C). Hb C is a blood disorder exemplified with abnormal hemoglobin whereas beta-thalassemia is a blood disorder that is exemplified with an abnormal amount of hemoglobin. Hb C is common in West Africa while thalassemia is found primarily among people from around the Mediterranean Sea. Sickle cell syndrome causes red blood cells to change their shape (to a crescent shape or c-shape) which results in the clogging of the blood vessels. As a result, body tissue becomes damaged as a result of not receiving an adequate supply of blood (Harris, 2001).
Sickle cell disease is inherited and runs in families. It is transmitted from parent to child and can run for many generations in the family. Once a couple has been diagnosed with carrying genes for sickle cell, the probability of transmitting to their children is very high. Two copies of the disease are usually inherited, each parent contributing one. Children who take over only one gene of the illness (from either parent) develop a condition that is referred to as a “sickle cell trait”. This is a different condition from sickle cell disease and people in it cannot be said to possess the disease but have one gene that causes the disease and can pass it to their children. If a couple does not know whether it carries genes for sickle cell, it can go to the testing from the regional network for genetics services. If the trait is diagnosed in both the parents, then the baby stands at a 25% possibility of having the illness (Bloom, 1995).
The generally known symptoms of this disease include fatigue, breath shortness among others. Pain mostly occurs in the bones, abdomen, and chest. The intensity and duration of the pain, which are unpredictable, guide its treatment. For mild to intermediary pain, uncomplicated medication such as acetaminophen or codeine may provide relief, and water should be drunk in plenty. For intense and enduring pain, the physician may give stronger pain-killing drugs, such as morphine fluids. Physicians are advised to treat more than the pain because in most circumstances pain is just a symptom of a bigger problem.
Conventional management of sickle cell anemia is primarily encouraging. It is important to detect infections early and treat them with antibiotics, as these infections may trigger painful crises. General supportive care includes daily oral folate supplementation, antibiotic prophylaxis in childhood, Pneumovax among others. The primary treatment is the prevention of infectious diseases. Vaccinations including influenza A and Haemophilus influenza immunizations are indicated (Steinberg, 2001). Blood transfusion is another commonly practiced method of the treatment of anemia, the sickle cell being one of them.
The ailment affects more than 70, 000 of the US populace. It mainly affects black Americans and Hispanic Americans. It is a common genetic disease and regularly causes difficulties in pregnancy management. Patients with Hb S and Hb C are said to have sickle cell disease and this is most often accountable for unpleasant effects on the mother, fetus, and newborn. Out of every 12 black Americans, one possesses the sickle cell trait and in every 500, one has sickle cell anemia (Steinberg, 2001). The frequency of Hb S and Hb A is high in Africa. In some areas, the pervasiveness of sickle cell trait depends first and foremost on the prototype of the flow of the Hb S. Other countries that are affected are Europe, the Middle East, India, and central Asia.
Reference List
Bloom, M. (1995). Understanding sickle cell disease: Understanding health and sickness series. Mississippi: Univ. Press of Mississippi.
Harris, J. L. (2001). Sickle Cell Disease. New York: Twenty-First Century Books
Steinberg, M. H. (2001). Disorders of hemoglobin: genetics, pathophysiology, and clinical management. Cambridge: Cambridge University Press.