Sickle Cell Anemia and Red Blood Cell Physiology, Annotated Bibliography

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.

Sickle cell disease (SCD) is a genetic disorder, characterized by defected hemoglobin (Hb) synthesis with production of an abnormal form, known as sickle hemoglobin (HbS). Under low oxygen concentration or increased body oxygen demand, red blood cells that are sickle and rigid shaped fail to pass smoothly through capillaries, with multiple clotting and thrombosis. Eventually, this leads to frequent hemolytic crisis and progressive organ damage. Transfusion is the key therapy in SCD. However, transfusion has been associated with many side effects in SCD patients, mainly iron overload and alloimmunization that cause severe hemolytic transfusion reactions.

What is sickle cell anemia? Sickle cell anemia is an inherited disease of red blood cells. Normally red blood cells contain a protein called hemoglobin A, which carries oxygen to all the organs in the body. With sickle cell anemia, however, the body makes a different kind of protein, called hemoglobin S. The problem is that when a red blood cell with hemoglobin S releases oxygen, the cell changes from the usual doughnut shape to a sickle or S shape, and becomes stiff rather than soft and flexible like normal red blood cells. This "sickled cell," which resembles a crescent moon, can't continue to glide through the small blood vessels as usual.

The 'Sickle' reference is in relation to the disordered shape red blood cells take. The way the disease is acquired is through hereditary genes. The disease is primarily characterized by chronic anemia, and frequent episodes of pain. Hemoglobin, an important element in the red blood cell is found to be defective. The job of hemoglobin molecules is to transfer oxygen from the lungs throughout the body, and then transfer carbon dioxide from the body back to the lungs, then finally out of the body altogether.

Every day the body produces new red blood cells to replace old ones, but sickle cells become destroyed so fast that the body cannot keep up. The red blood cell count drops, which results in anemia. This gives sickle cell disease its more common name, sickle cell anemia. The only true way to determine if a person has sickle cell anemia is through a blood test, and if a person does have sickle cell anemia there are ways to help avoid crisis and the pain associated with the disease. There are medicines that help to prevent attacks.

The production of white blood cells affect the blood clotting process which means patients who suffer leukemia may present abnormal bleeding or brusing. The white blood cells over produce during the disease are dysfunctional and have a lack of defensive capacity which makes the body vulnerable for infection and finally the red blood cells deficit produces anemia. There are other symptoms presented before the disease is spread through other parts of the body like : fever, weakness, fatigue, loss of appetite, headache, paralysis, seizures, bone or joint pain. There are two types of leukemia : myelogenous or granulocytic and lympathic. This terms are divided depending on the types of blood cells involved in the disease (lymphoid cells or myeloid cells).

Sickle-cell anemia is a genetic disorder that makes your body produce red blood cells that are abnormal in shape. This disease is also widely known as hemoglobin SS disease. Unlike normal red blood cells, sickle cells are rigid and tenacious. Due to their shape and rigidness, they can block blood flow. In turn, this could cause organ damage to the body.

Sickle Cell Anemia Sickle cell anemia is caused by a defect in the gene that controls the production of normal hemoglobin, which is an iron-containing protein in red blood cells that transports oxygen from the lungs to body tissues. The defective gene results in the production of abnormal hemoglobin known as hemoglobin S. If you have the disorder, you inherited one gene for hemoglobin S from each of your parents. The gene is recessive, so if you received a copy of the gene from just one parent, you are a carrier of the sickle cell gene. Under certain conditions, after hemoglobin S releases its oxygen, its molecules clump together forming rigid and elongated crystals. The crystals settle to one side of the cell, which collapses the other side.

These large numbers of immature or incompletely developed cells do not function like healthy red blood cells leading to oxygen deficiency throughout the organs and tissues of the body. Since these cells are underdeveloped they also have a shorter life expectancy. Pernicious anemia has been studied by many different researchers, starting in the late 17th century. Between 1876 and 1877 William Osler and William Gardner studied a patient they suspected of pernicious anemia. Their findings were that the patient had the following symptoms: numbness in the fingers, hands, and forearms, the gastric mucosa was atrophic, and high levels of red bloods cells containing nuclei were found in the bone marrow.

This knowledge would be helpful for administration and parents of children with cancer, to better understand future expenses. The research question is clearly stated as finding the patterns of cost and research utilization for children with leukemia and CNS tumors for 3 years after being diagnosed (Hendrickson, Rimar, 2009). This is consistent with the problem about lack of knowledge in costs that was stated earlier. Later in the research, Hendrickson did expand her question to include comparisons between children with leukemia and CNS tumors and likeliness to survive based on admission in the PICU. Sampling size is sufficient with 223 children.