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Sickle cell anemia is a genetic disorder that occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. Normal red blood cells are smooth and round like a doughnut without a hole. They move easily through blood vessels to carry oxygen to all part of the body. In sickle cell anemia, the body produces red blood cells that are shaped like the farm tool called a sickle (crescent shaped). These sickle cells are hard and sticky and they don't move easily through the blood vessels. They tend to get stuck and block the flow of blood to the limbs and organs. This can cause pain, organ damage, and a low blood count (anemia). Red blood cells affected by sickle cell anemia change shapes from being round and normal to sickle shaped, eventually staying abnormally shaped. Most blood cells last about four months in the bloodstream. The fragile, sickle cells break down after ten to twenty days, which usually causes anemia. People who are anemic often feel weak and tire more easily. The effects of sickle cell anemia can vary from person to person depending on the type of sickle cell disease the person has. Some individuals are relatively healthy and others are hospitalized frequently. People with sickle cell anemia can also experience complications from blood circulation and infection fighting problems. These include a higher risk of certain infections and stroke, as well as a condition called acute chest syndrome, which is caused by infection or trapped red blood cells in the lungs.
Sickle cell anemia is not contagious, so you can't catch it from someone or pass it on to another person. A person only has sickle cell anemia if they inherit one sickle cell gene from each parent. If a child only inherits one of the abnormal genes, they may have sickle cell trait, which means the person will carry the disease bearing gene, but not have any symptoms of sickle cell anemia.
Pain episodes (sometimes called crises) are the most common symptom of sickle cell anemia. In both children and adults, pain may result from blocked flow of blood and oxygen. Painful episodes may occur in any joint or organ. The pain can be acute (sudden), chronic (long lasting), or a mixture of both. Some affected individuals have only one pain episode per year, while others may have one or more pain episodes a month. Pain episodes usually last a few hours to a few days, but they may sometimes last for weeks.
The condition called sickle call trait is different from sickle cell anemia. A person with sickle cell trait does not have the disease, but carries the gene that causes the disease. People with sickle cell trait pass the gene on when they have children. Someone who has sickle cell anemia has inherited two copies of the sickle cell gene, one from each parent. Children who inherit only one copy of the sickle cell gene (from one parent) will not have sickle cell anemia. They will have sickle cell trait. People with sickle cell trait usually have no symptoms, live normal lives, and can pass the sickle cell gene on to their children.
Most states in the United States, including Wisconsin, currently test all newborns for sickle cell anemia. The test can show if the newborn infant has sickle cell anemia or carries the sickle cell trait. The test uses blood from the same blood samples as other routine newborn screening tests. If the first test shows that the sickle shaped hemoglobin is present, a second blood test is done to confirm the diagnosis. Early diagnosis of sickle cell anemia is important so that children who have the disease can get proper treatment. It is also possible to identify sickle cell anemia before birth. This is done by taking a sample of amniotic fluid or tissue from the placenta. This test can be done as early as the first few months of pregnancy.
Effective treatments exist for the symptoms and complications of sickle cell anemia, but in most cases there is no cure. Some researchers believe that bone marrow transplantation may offer a cure in a small percentage of cases. Researchers are working on developing new treatments for sickle cell anemia, including gene therapy and more safe and effective bone marrow transplants. People with sickle cell anemia need regular medical care. Some doctors and clinics specialize in treating people with the disease. The goals of treatment are to relieve pain, prevent infections, and control complications if they occur.
Painful crises or episodes are the most common reason for emergency room treatment of sickle cell anemia. The usual treatments for acute pain episodes are pain killing medicines and fluids, either by mouth or through a vein, to prevent dehydration. The pain killing medicines used most often are acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and opioids, such as morphine, oxycodene and hydrocodene. Blood transfusions are used to treat worsening anemia and sickle cell complications.
If you have sickle cell anemia, it is important to take good care of yourself and see your doctor regularly. Some important things you need to do are:
If your child has sickle cell anemia, you should learn as much about the disease as possible. This will help you recognize early signs of problems, such as fever or chest pain, and seek early treatment. Sickle cell centers and clinics can provide information and counseling to help you handle the stresses of coping with this serious chronic disease. Make sure your child gets daily penicillin up to age five to prevent infections. Children should also have a flu shot every year and a vaccination against pneumonia. Keep a thermometer on hand and know how to use it. Call the doctor if your child has a temperature above 101 degrees F. Have good communication with your child's doctor to keep your child as healthy as possible. School aged children should participate in physical education. Teachers should allow children with sickle cell anemia to rest if they tire and to drink fluids after they exercise.