Sickle cell anemia is a genetic condition whereby red blood cells are unable to effectively carry oxygen throughout the body. The disease produces red blood cells that are crescent-shaped instead of the normal round shape, which makes the blood cells stick to each other and not carry enough oxygen. Red blood cells contain hemoglobin, which is a protein that carries the oxygen. Sickle cell disease makes the hemoglobin develop abnormally due to a defective gene called Hemoglobin S, making them fragile and causing them to break easily. Diagnosis consists of a genetic test using blood as well as observing blood under a microscope for the sickle shape. The condition is common in people who are of African and Mediterranean descent who may carry the gene and pass it on to their children. If a child receives a defective gene from both parents, then the child will get the disease. The effects of sickle cell anemia differ between individuals and may require specific treatment.
Time Frame
Sickle cell anemia symptoms usually show up in infants around the age of four months, and effects vary between individuals. Pain symptoms or crises occur over periods of time and may last days or even weeks. Some patients may experience pain a few times a year while others may experience it more than a dozen times. Severe cases may require hospitalization and intravenous treatments. Sickle blood cells also have a short life span, which ranges between 10 to 20 days while normal blood cells live up to 120 days, according to the Mayo Clinic.
Complications
According to the Mayo Clinic, sickle cell disease may cause several complications. Stroke and organ damage are very common complications and are caused when blood cells block oxygen flow to organs like the brain, eyes and kidneys. Sickle cells also carry less oxygen, which can damage organs over time. Acute chest syndrome occurs when sickle cells block blood vessels in the lungs, making it difficult to breathe and causing pain. Sickle cells cause high blood pressure in the lungs, which can also make it difficult to breathe and may lead to heart failure. Gallstones are common and are caused by the breakup of red blood cells. Painful erections, or priapism, may occur in men due to blockage of blood vessels in the penis and may cause impotence.
Symptoms
According to the National Institutes of Health, common symptoms include ulcers, fever, breathlessness, abdominal pain, rapid heart rate, paleness, jaundice, frequent urination, chest pain, stroke, excessive thirst, painful erection, poor eyesight, delayed growth and puberty, and pain in the bones. Patients should seek medical attention if they experience swelling of the abdomen, hands and feet; paleness in the skin or nail beds; jaundice or yellowing of the skin and eyes; fever; or signs of a stroke, which include paralysis of half the body, confusion, numbness and vision problems.
Prevention of Effects
Infections may be prevented by regular immunizations against diseases such as influenza, hepatitis B, Haemophilus influenza, meningococcal and pneumococcal disease. To prevent cells from becoming sickled, it is important to treat infections as quickly as possible, and to get enough oxygen and fluids in the body. Preventing crises in children is also possible with lifestyle changes. Children should be immunized regularly as well as wear a Medic Alert bracelet to alert others of their condition. To prevent oxygen loss, it is better to prevent strenuous physical activities, smoking, low oxygen environments (high altitudes) and emotional stress. Dehydration can be prevented by drinking fluids constantly and reducing exposure to the sun, according to the National Institutes of Health.
Treatment of Effects
Treatments usually depend on the effects of the condition. Patients should take folic acid supplements regularly because they are needed to make red blood cells. Hydroxyurea and pain killers should be taken to reduce the pain symptoms of crises. Dosages depend on the severity of the pain. Antibiotics may also be used to treat bacterial infections, which may be short or long-term in duration. Blood transfusions may also be used to prevent strokes and crises and can be performed regularly. Surgery or organ transplant may be required for organs that are mildly or severely damaged, according to the U.S. National Library of Medicine.
