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).
Sickle cell anemia is a blood disorder that is inherited from both parents in which the body produces abnormally shaped red blood cells. In sickle cell anemia, the hemoglobin in red blood cells links together; resulting in the red blood cells to become rigid and a C-shaped. These deformed cells block blood and oxygen flow in blood vessels. Sickle cells deteriorate quicker than normal red blood cells, which results in anemia.
Sickle-cell anemia is an illness in which the shape of red blood cells is altered to a sickle-like shape (Peachley, n.d.). It is basically the sickling of erythrocytes. The protein molecule, hemoglobin, is the reason for this altered shape. Hemoglobin is found on red blood cells and it is used to provide oxygen to tissues (Peachley, n.d.). In sickle-cell anemia the altered shape of the red blood cells prevent them from passing through blood vessels and this will result in an inadequate amount of oxygen being delivered to tissues in the body (Peachley, n.d.). Sickle cell anemia was discovered because of gel electrophoresis, which is a method that evaluates the charges of the hemoglobin. Gel electrophoresis, the inheritance pattern, and hemoglobin
Sickle cell anemia affects millions of people around the world, with about 70,000 Americans inheriting the disease, and many others carrying the sickle cell trait (Genetics). This disease mostly affects people that have African and Mediterranean roots, mostly because this disease provides protection for people from malaria which kills 3,000 African children daily (Malaria). Malaria is a deadly infectious disease that is transmitted by to humans when mosquitoes sting them (Learn). Sickle cell anemia is the most common blood disorder that is a result of a mutation in the genes causing a mutated form of hemoglobin, the protein that helps red blood cells transport oxygen to the body from the lungs. The mutated form of hemoglobin, hemoglobin S, causes the red blood cells to alter their shape into a fragile crescent or sickle shape, which is the main cause for any negative effects a person with sickle cell anemia might have.
INTRODUCTION — Sickle cell disease (SCD) is an inherited group of disorders characterized by the presence of hemoglobin S (HbS), either from homozygosity for the sickle mutation in the beta globin chain of hemoglobin (HbSS) or from compound heterozygosity of a sickle beta globin mutation with another beta globin mutation (eg, sickle-beta thalassemia). The hallmarks of SCD are vasoocclusive phenomena and hemolytic anemia. Sickle cell trait is a benign carrier condition characterized by heterozygosity for the sickle hemoglobin mutation.
Sickle cell disease is caused by a mutation in the hemoglobin gene. In healthy condition, normal RBCs are circular and flexible. The hemoglobin molecules inside the cells are free moving, which help them to maintain their shape and flexibility. Due to their flexible shapes, RBCs are able to move through the blood vessels without any hindrance. In case of a certain mutation in the hemoglobin gene, abnormal hemoglobins called ‘Hemoglobin S.’ are produced. The mutated hemoglobin molecules form chemical bonds with each-other creating long chains. These chains result in a stretching force from the inside of the cell causing the cell to stretch abnormally. The cell then takes an elongated, ‘crescent-moon’ shape and loses its flexibility.
Sickle cell disease is an inherited disease where the red blood cells in the body are produced abnormally by bone marrow as crescent shaped red blood cells. Unlike normal red blood cells, sickle shaped cells are unable to deliver much oxygen to other parts of the body due to the abnormal hemoglobin. Sickle cell’s are stiff and sticky and tend to clump together between blood vessels that can cause pain, damage to the organs, and infections. If a child inherits this disease they can be healthy throughout their life or need special care. “In the United States, SCD is most common among blacks and Hispanics. SCD affects about 1 in 500 black births and about 1 in 36,000 Hispanic births in this country. SCD is also common among people with family from Africa, the Caribbean, Greece, India, Italy, Malta, Sardinia, Saudi Arabia, Turkey or South or Central America (March of Dimes)”.
Cell plays a big role in our body. According to Campbell biology, a cell is a basic unit of life and all organisms are made up of cells. The book states that some complex organisms like plants and animals are multicellular. Furthermore, cells plays different functions in living things. Cells transport proteins to different parts of the body, they transport waste product, Provide energy in form of ATP, protects our body by making sure that blood is not exposed to germs. Cell are also composed of nucleus which contains the DNA used for reproduction and protein formation. The normal red blood cells are round in shape to increase the surface area for oxygen transportation whereas sickle cell anemia cells are sickle shaped and stiff. This makes the cells unable to carry oxygen because the surface area is reduced in size. Furthermore, when the cells becomes sickle they only last for a couple of days and die. This makes the cell unable to move and they stick in the small blood vessels; as the result, body tissues and organs don’t receive enough oxygen required to stay healthy. This is why many people affected by sickle cell anemia have difficulty in breathing, feel weak and tired. It also causes the damage of organs like lungs and liver. Sickle cell anemia affect respiration system in the body. Even though the study in HRQoL is not promising that people with sickle cell anemia will have
“In the United States, it's estimated that sickle cell anemia affects 70,000–100,000 people, mainly African Americans” (NHLBI, NIH, Who is at risk for sickle cell anemia). SCD is a disease that is a serious disorder in which the body can make normal blood cells and sickle shape cells. Sickle shape cells can block the blood flow in your vessels and cause pain or organ damage also put you in risk for infections. SCD has no cure available but there are many treatments out there to deal with the complications of it. From over years treatments did get better from way back in the day doctors have learned. Sickle cell disease has lack of attention and funding because it’s only affecting African American the most.
Sickle cell anemia (SCA) is one of the most common inherited genetic diseases. It is frequently found in tropical regions of the world. It primary affects people from African descend, followed by people of Arab, Indian and Hispanic descent. SCA changes the conformation of hemoglobin, the globular protein inside red blood cells that helps transport oxygen throughout the body. The articles titled “Pathophysiological insights in sickle cell disease,” “Sickle Cell Anemia: Progress in Pathogenesis and Treatment,” and “A Review on Sickle Cell Anemia” all agree that SCA occurs when a person inherits two copies of the 11th chromosome from each parent containing the receive trait. If an individual inherits only one copy of the chromosome with the mutation, he will only develop the sickle cell trait because only one copy of DNA with the sickle cell trait is not enough to cause the disease, but the homozygotic disease can still be passed on to his children with a 50% chance. SCA originates from an abnormality in the Beta amino acid chain of hemoglobin. The normal hemoglobin is made up of two beta-globin protein chains and two alpha-globin chains. The structure of the B-amino acid chain of hemoglobin at position six, in Beta chain 2, is expressed as GAG, that codes for glutamic acid, but on SCA patients the Beta amino acid chain structure replaces the adenine for a thymine nucleotide, the GTG changes a hydrophilic glutamic acid (GAG) for a hydrophobic valine (GTG) still at position 6, a position that is at the surface of the amino acid chain and it is usually exposed to water. The hydrophobicity of valine reduces the solubility of the molecule. This small, simple change of one letter on the Beta amino acid chain of hemoglobin generates the sy...