Red Blood cells are round and flexible. Red blood cells carry enormous amounts of oxygen in your blood. Red blood cells carry food or nutrients throughout your body and keep you healthy. The red color in the blood cells comes from hemoglobin. Specifically hemoglobin A. Red blood cells can live 120 days. Then the cells die and are replenished by the body. You may notice that is why you cannot give blood but every quarter. However, in sickle cell anemia the red blood cells are hard and shaped like a crescent moon or a sickle, hence the name, sickle cell anemia. These red blood cells only live 10 to 20 days, which causes chronic fatigue and lack of oxygen to the body and cells. There are never enough red blood cells to feed the body. Persons with sickle cell anemia do not make hemoglobin; rather they make hemoglobin S, which of course, stands for sickle cell anemia. It is an inherited disease and is usually diagnosed around four months of age. One of the first signs may be yellowing of the skin or whites of the eyes known as jaundice. Jaundice occurs because the liver cannot process all of the red blood cells that are dying so rapidly. Another sign is swelling of the hands and feet also due to the abnormally shaped cells blocking flow of blood to the extremities.
The worst part about this disease is pain. Because the blood cells are abnormally shaped they are stuck in tight passages causing blockages where blood and thereby oxygen cannot get through to the rest of the body. The worst episodes of pain occur when the hard sickle cell blood cells become stuck in joints, abdomen, chest and bones. Sometimes the episodes of pain are fleeting and sometimes it lasts for weeks.
Persons with sickle cell anemia are also subject to frequent infections because just as the liver is affected by the rapid breakdown of dead red blood cells so is the spleen. The main purpose for the spleen is to help fight off infections so when it is compromised the risk of increasing infections also rises.
Because in sickle cell anemia the red blood cells are not able to provide proper nutrients to the body there is a delayed growth in infants and children as well as delayed puberty.
Vision is affected again due to the inability of red blood cells to get through the tiny vessels and provide nutrients to the eyes.
Complications that develop because of sickle cell anemia include infections, strokes, jaundice, increased temperature, abdominal tenderness, chest pain, difficulty breathing, shortness of breath, and ulcers of the skin, blindness and paleness of skin or nail beds. If any of these signs of complications are noted, take the patient immediately to the physician or hospital.
There is no cure for sickle cell anemia but there are treatments. Medications such as hydrea, which is a cancer drug can help decrease pain and may decrease the need for blood transfusions. Blood transfusions will become necessary and the physician will carefully monitor the hemoglobin and hematocrit of the person’s blood cell count on a regular basis. Blood transfusions not only replenish the supply of red blood cells but also will decrease the risk of stroke. This is because the blood transfusion will have normal red blood cells and these cells will in turn carry more oxygen to the brain without clogging in the tiny vessels. Antibiotics are given on a regular basis until the child is at least five years old to help prevent infections. This is called prophylactic medication use. Pain medications will also be ordered due to the severe to debilitating pain episodes that are likely to occur. Oxygen use through a nasal cannula is often necessary again because there is not enough oxygen in the blood to nourish the body’s cells and organs. A test called a pulse oximeter is used to test for peripheral amount of oxygen in the blood. In children numbers in the low 90% are cause for alarm and anyone with a pulse ox of less than 90% will of course require oxygen. Lack of oxygen makes thinking difficult, causes increase fatigue and makes walking and any movement a tiresome task.
There are experimental treatments for this disease such as bone marrow transplants but this is extremely risky and is experimental. The main thing is to treat the complications and try to keep them from occurring. Information and frequent trips to the physician and the hematologists will be required for the rest of the person’s life.
barbara bethard
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