Sickle Cell Anemia

Sickle cell anemia is a disorder of the blood caused by an inherited abnormal type of hemoglobin called hemoglobin S, which changes the red blood cells to become fragile and shaped like sickles. Sickle cell anemia is inherited as an autosomal recessive condition, meaning that the gene can be passed on from a parent carrying it to both male and female children. In order for sickle cell anemia to occur, a sickle cell gene must be inherited from both the mother and the father, so that the child has two sickle cell genes. People who have sickle cell trait inherit a normal gene from one parent and sickle hemoglobin gene from the other parent. Their bodies make both sickle hemoglobin and normal hemoglobin. The hemoglobin molecule has two parts- an alpha and a beta. When a mutation in a gene on chromosome 11 is present in the body, hemoglobin molecules are not formed properly, causing red blood cells to have a sickled shape. These cells block the blood vessels, leading oxygen to be transported ineffectively. Doctors screen newborns for sickle cell disease with a simple blood test; however, if the disease is not found at birth, doctors use a test called hemoglobin electrophoresis. This test determines whether a person has sickle cell disease, or whether he or she is a carrier a hemoglobin gene. When a patient has sickle cell anemia, he or she experiences painful episodes called crises. These crises last from hours today, causing pain in the lower back, leg, joints, and chest. When anemia becomes more severe, symptoms may include jaundice, paleness, rapid heart rate, shortness of breath, and fatigue. Younger children with sickle cell anemia have attacks of abdominal pain. When small blood vessels become blocked by the abnormal cells, painful and prolonged erection, poor eyesight or blindness, ulcers on the lower legs and problems with thinking or confusion may occur as symptoms. Sickle cell anemia affects South America and Africa more than anywhere else. Because malaria infects people that do not have sickle cell, many African people reproduce more kids that have sickle cell, because people with sickle cell have better chance of living than people who do not have it, those who are likely to get malaria. When babies and young children have sickle cell disease, they take penicillin, a group of antibiotics derived from Penicillium fungi, to prevent potentially deadly infections. Also, patients take folic acid, a vitamin that helps the body produce new red blood cells. When doctors treat sickle cell anemia, they advise patients to get plenty of rest, avoid physical activity, and drink lots of water. A common treatment is blood transfusions that provide a patient with healthy red blood cells. Bone marrow transplant can be performed to people with more severe cases of the disease. This procedure provides the patient with healthy red blood cells from a donor. In the past, people who have sickle cell disease died between ages 20 and 40. The life expectancy has been reduced. People now can live to the age of 50 and beyond. Most patients with sickle cell anemia suffer intermittent, not continuous, pain crises, bacterial infections, fatigue, and progressive tissue and organ damage. Children with sickle cell anemia experience impaired growth and development along with physical and emotional trauma. Bacterial infections are the most common cause of death, as well as stroke or bleeding into the brain, and kidney, heart, or liver failure.

Works Cited

"Sickle Cell Anemia." KidsHealth - the Web's Most Visited Site about Children's Health. Ed. Robin Miller. The Nemours Foundation, 01 Sept. 2012. Web. 14 Apr. 2014.

"Sickle Cell Anemia: MedlinePlus Medical Encyclopedia." U.S National Library of Medicine. U.S. National Library of Medicine, 26 Feb. 2014. Web. 13 Apr. 2014.

"Sickle Cell Anemia (Sickle Cell Disease) Causes, Diagnosis, Symptoms, Treatments - MedicineNet." MedicineNet. Medically Reviewed by a Doctor, 4 Mar. 2014. Web. 14 Apr. 2014.

"Sickle Cell Disease." Sickle Cell Disease. Genetic Science Learning Center, 2014. Web. 15 Apr. 2014.

"What Is Sickle Cell Anemia?" - NHLBI, NIH. NIH, 28 Sept. 2012. Web. 13 Apr. 2014.