Sickle Cell Disease Essays - Hemoglobins, Sickle-cell Disease

Sickle Cell Disease The sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell have red blood cells that have mostly hemoglobin's, Sometimes these red blood cells become sickle-shaped or crescent shaped and have trouble going through small blood vessels. When sickle-shaped cells block small blood vessels, less blood can get to that part of the body. Tissue that does not get a normal blood flow eventually becomes damaged. This is what causes the problems of sickle cell disease. As to this day there is really no cure for sickle cell disease. Red blood cells take oxygen from the air we breathe into our lungs to all parts of the body. Oxygen is carried in red blood cells by a substance called hemoglobin (Hemoglobin ? is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body). Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Oxygen is carried in red blood cells by a substance called hemoglobin. The main hemoglobin in normal red blood cells is hemoglobin A. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them. People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S (S stands for sickle). Red blood cells containing mostly hemoglobin S do not live as long as normal red blood cells (normally about 16 days). They also become stiff, distorted in shape and have difficulty passing through the body's small blood vessels. When sickle-shaped cells block small blood vessels, less blood can get to that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC) Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia. Sickle Cell trait (AS) is an inherited condition in which both hemoglobin A and S are made in the red blood cells, there are always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy. Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical things. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets...one from each parent (Ex. If one parent has Sickle Cell Anemia and the other is Normal, all of the children will have sickle cell trait. 4 If one parent has sickle cell anemia and the other has sickle cell trait, there is a 50% chance (or 1 out of 2) of having a baby with either sickle cell disease or sickle cell trait with each pregnancy, When both parents have sickle cell trait, they have a 25% chance (1 of 4) of having a baby with sickle cell disease with each pregnancy). HOW DO YOU KNOW IF YOU HAVE THIS TRAIT A SIMPLE PAINLESS BLOOD TEST followed by a laboratory technique called Hemoglobin Electrophoresis will determine the type of hemoglobin you have. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition. This technique differentiates between normal hemoglobin (A), Sickle hemoglobin (S), and other different kinds of hemoglobin (such as C, D, E,). Medical Problems Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones. The sickle cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest and abdomen), stroke and priapism (painful prolonged erection). It also causes damage to most organs including the spleen, kidneys and liver. Damage to the spleen makes sickle cell disease patients, especially young children, easily overwhelmed by certain bacterial infections. TREATMENT Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation. Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery all backed by psychosocial support. Like all