Sickle Cell Anemia (SCA)

Sickle cell anemia is a hereditary disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent. More than 70,000 Americans have sickle cell anemia. And about 2 million Americans - and one in 12 African Americans - have sickle cell trait (this means they carry one gene for the disease, but do not have the disease itself).

Symptoms include pain, cardiovascular changes such as risk for high-output heart failure, priapism, low-grade fever, and organ damage due to poor tissue perfusion. Preventative measures for SCC include proper hydration, abstaining from cigarettes and alcohol, seeking medical attention at first sign of infection or illness, staying current on vaccines including a yearly flu vaccine, avoiding extreme temperatures and unpressured cabins on airplanes, and engaging in low-impact exercise three times a week (Byar,

The sickle cell disease affects about 100,000 people in the America. The most common ethnic group the sickle cell anemia is seen in is African Americans and Hispanics. Approximately one in every ten African American and one in every one hundred Hispanic Americans have the sickle cell trait. Approximately two million people have the sickle cell trait in America. Approximately one in five- hundred African Americans and one in one thousand to one thousand and four hundred Hispanic-Americans have sickle cell disease. No universal cure has been found for sickle cell anemia (“Facts About Sickle Cell Trait And Disease,”n.d.). Sickle cell anemia affects many Americans and a universal cure needs to be found.

A sudden worsening of anemia resulting from infection or enlargement of the spleen is a common reason for a transfusion. Multiple blood transfusions, however, might cause health problems because of the iron content in the blood. Iron overload, called hemosiderosis, can damage liver, heart, pancreas and other organs also leading to diseases such as diabetes mellitus. Iron chelation therapy should be started in patients with SCD receiving regular blood transfusions to reduce excess iron levels. Infections are treated with antibiotic medicines and sometimes blood transfusions. At the first sign of an infection, such as a fever, it is important to see a doctor right away as this may represent a medical emergency for people with SCD (Hurston 2018). Early treatment of infection can help prevent problems and even save

Prevalence of Sickle Cell Anemia: Sickle cell anemia is the most common inherited blood disorder in the United States, affecting about 72,000 Americans or 1 in 500 African Americans.

The term sickle cell infection (SCD) depicts a gathering of acquired red platelet issue." Normal red blood cells are shaped like discs or donuts. They are soft and flexible so they can easily move through very small blood vessels"(anonymous, 2015).Individuals with SCD have anomalous hemoglobin, called hemoglobin S or sickle hemoglobin, in their red platelets. Hemoglobin is a protein in red platelets that conveys oxygen all through the body. The most well-known sort is known as, sickle-cell frailty (SCA) and there is A few Types of Sickle Cell Sickness: Hemoglobin SS, Hemoglobin SC, Hemoglobin SD. In the Unified States, a great many people with sickle cell illness (SCD) are of African family line or recognize themselves as dark. About1 out of 13 African American children is

Sickle cell anemia (SCA) is an inherited disease of the blood that is characterized by the production of abnormal hemoglobin S causing the cell to acquire a sickle shape that prevents the smooth flow of blood to a major organ (Shea et al. 2017). The stress is usually caused by fever, infection or cold temperature, which lead to sickle cell crisis caused by hypoxia, dehydration, and acidosis (Barranger, 2017).

All of the cells within the tissues of the body need a regular supply of oxygen to function. The hemoglobin found in red blood cells (RBC’s) are essential for the proper transport of oxygen molecules. They bind oxygen in the lungs and carry to every tissue within the body. Due to the structure of hemoglobin, normal RBC’s are donut shaped with a concaved center. This flexible shape is what allows the BRC’s to move freely into very small spaces such as capillaries where they will deliver their oxygen. Sickle cell anemia or other diseases that fall within the broader group of disorders characterized by SCD describe conditions where the protein (hemoglobin) has misfolded and altered the shape of the cell. Sickle hemoglobin allows for the formation of stiff rods within the red blood cell giving it a sickle, or crescent shape. This new shape is not flexible and can block or slow the flow of blood. When this occurs, oxygen cannot reach its intended tissues. This results in severe pain called crises, and these attacks occur

Normal RBCs are disc shaped in which they are able to easily travel throughout the body, especially through the tiniest blood vessels. However, with sickled shaped RBCs, they clump together while flowing through these particularly small vessels, which causes the loss of oxygen throughout the tissues and organs. This monogenetic disease is caused by a point mutation in the β- globin gene that creates abnormal Hemoglobin S (HbS). There are five different haplotypes for SCD that are classified by their geographical region

Irregular shaped sickle cells do not transport enough oxygen to perfuse body tissue, tissue not receiving enough oxygen causes more cells to sickle. An abnormal shaped sickled cell may become lodged to a blood vessel where other cells then bind to it and begin to form a clump. The blood vessels continually attach to each other and result in a blockage of small blood vessels eventually resulting in organ damage. Sickle cell disease commonly affects the spleen before other organs. Children can suffer life threatening complications when blood is trapped in the spleen, many must have the spleen removed in early childhood causing them to have a decreased immune system therefore putting them at higher risk for infections. The leading cause of death in young children with sickle cell disease is bacterial infections. Other complications can include stroke, acute chest syndrome with pulmonary infiltrate and infection, priapism, retinopathy, kidney damage, and gallstone formation (Ball, J., & Bindler, R., 2015). Sickling of the cell can be triggered by the following: emotional or physical stress, fever, or hypoxia. Precipitataing factors can include increased blood thickness, hypoxia or low oxygen tension. Sickle cell crisis may result from extreme fatigue, infection, trauma, surgery, cold exposure or dehydration, or any other condition that increases the body’s need for oxygen or that alters the transport of oxygen. Sickled cells have a

Sickle cell disease (SCD) is an inherited and noncontagious, lifelong condition. More specifically, per Ohaeri, Shokunbi, Akinlade, & Dare, 1995, SCD is a generic term for a group of genetic disorders characterized by the predominance of sickle cell hemoglobin (Hgb). Hemoglobin is a protein in the red blood cells that carries oxygen throughout the body. The disease produces significantly abnormal Hgb molecules in red blood cells (RBCs). In sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body (Ohaeri, Shokunbi, Akinlade, & Dare, 1995, p. 955.Individuals

In conclusion, a person with this blood disorder have a lower number of red blood cells and have a lower life expectancy. A patient with SCA may have chronic health problems including circulatory problems, a splenic sequestration crisis, acute chest syndrome, and brain complications. People with SCA are resistant to malaria and can be of different ethnicities including African. Despite being a painful blood disorder, there is much funding going into the research of SCA. Overall, sickle cell anemia is a chronic and inherited blood disease in which a person has “sickle” shaped blood

What is Sickle Cell Anemia? Sickle Cell disease is a blood disorder that is inherited. By inherited I mean passed down from parents to their children. Babies are usually born with sickle cell disease. When they inherit two abnormal genes, one from each parent. Abnormal genes cause the body’s red blood cells to change shape. This being the effect of having sickle cell disease.

Sickle cell anemia (SCA) is one of the most common severe monogenetic disorders in the world. (1).SCA is an inherited blood disorder caused by a single point mutation in one of the genes encoding hemoglobin (2).Hemoglobin (Hb) it is an oxygen transporting molecule, carrying oxygen from the lungs to the tissues and takinge carbon dioxide away from the cell. Hb, made of four globin protein chains two alpha and two beata chains (pic).http://themedicalbiochemistrypage.org/images/hemoglobin.jpg

It was reported that Sammy is the only person in the family to have been diagnosed with SCD. While it is not stated here, even though no other family member has presented with SCD, it can be assumed that both of Sammy’s parents have the sickle cell gene. As stated before, two parents with the sickle cell gene have a 25% chance of having a child with SCD. Sammy’s presentation is that of a six-year-old boy weighing only 64 pounds, despite the fact that he is 14-years-old. His size is directly related to his frequent episodes of sickle cell crisis. During a sickle cell crisis, the body’s ability to provide the tissue with proper oxygen and nutrients is impaired.