What is the disorder?
Sickle Cell Anaemia.
What is sickle cell anemia?
Sickle cell anaemia (sickle cell disease) is a disorder of the blood caused by an inherited abnormal hemoglobin (the iron-rich protein that carries oxygen from your lungs to the rest of the body).
Hemoglobin is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs and releases the oxygen to all parts of the body. From this, normal red blood cells are flexible and round, moving easily through blood vessels.
But when abnormal sickle haemoglobin releases the oxygen, the oxygen sticks together and forms long rods, which damage and change the red blood cell into rigid, sticky, sickle like or crescent moon shapes.
The sickle-shaped red blood cells break apart easily, having a shorter lifespan of only 10-20 days instead of the normal 120 days and aren 't replaced as quickly as normal blood cells. This leads to a shortage of red blood cells, known as anaemia.
These irregularly shaped blood cells, unable to move around as easily, clump together get stuck in small blood vessels, blocking blood flow and the transportation of oxygen to parts of the body.
Tissue and organs that do not receive a normal amount of blood flow eventually becomes damaged resulting in episodes of severe pain called a sickle cell crisis or a vaso-occlusive crisis.
A sickle cell crisis or a vaso-occlusive crisis can last from a few minutes to several months, although on average most last
Sickle Cell Anemia is a group of disorders that cause red blood cells to become misshapen and break down. Sickle Cell Anemia affects many people all over the world; Sickle cell disease is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans (Ashley-Koch, Yang and Olney). Sickle Cell Anemia causes your red blood cells to be thin, stiff, and shaped like a sickle. But your red blood cells are supposed to be round and soft. When a person is diagnosed with sickle cell anemia the blood cells start to become clogged blood vessels, which can cause a great amount of health issues including: infections, stroke, and acute chest syndrome. People get sickle cell anemia by inheriting a mutated gene from both of their parents. On the down side, there is not currently an effective cure for sickle cell anemia, there are several different therapeutic approaches to treating and attempting to cure the disease and help people who are affected with sickle cell anemia better manage their symptoms. There have also been several great scientists that dedicated a lot of research to help find a cure for sickle cell anemia.
Sickle cell anemia occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. Instead of being flexible and round, these cells are more rigid and curved in the shape of the farm tool known as a sickle - that's where the disease gets its name. The shape
Each year in the US, an average of 75,000 hospitalizations are due to sickle cell disease, costing approximately $475 million. Sickle cell disease is also associated with significant mortality. Among children, the primary causes of mortality are bacterial infections and stroke. In adults, it is more difficult to attribute specific causes to mortality, but it appears that individuals with more indicative disease are at risk for early mortality.
The term sickle refers to the fact that the red blood cells take on a sickle cell shape, instead of their normal biconcave shape (1). The cells can switch between the sickle shape and their normal biconcave shape (2). Sickled cells lose their pliability making it hard for the red blood cells to move through the smaller blood vessels (1). In their abnormal state they become more likely to adhere to the endothelium of the smaller vessels, causing clots to form and the possibility of stroke (2).
It is a condition in which there are not enough healthy red blood cells to carry adequate oxygen throughout the body. Red blood cells are normally round and flexible the moves easily throughout the blood vessels but, in sickle cell anemia the red blood cell is sticky, rigid, and has a crescent moon shape. The irregular shape cells can get stuck in the small blood vessels, which can slow or block
Sickle-Cell Anemia is an inherited, chronic blood disease in which the body produces abnormally shaped red blood cells. When the blood cells become crescent/sickle shaped, they are unable to deliver adequate amounts of oxygen to other cells. Also, these unusual “sickle” cells block blood pathways to the limbs and organs, limiting the amount of blood flowing throughout the body. It causes pain, organ damage, and anemia (low blood count). Unfortunately, however, when sufferers are born with this disease, they live life knowing it is incurable.
Sickle cell disease is categorized as an autosomal recessive disease. That means in order for one to inherit the disease, they must inherit two recessive alleles for sickle cell disease, or hemoglobin S gene. Having only one recessive allele makes you a carrier. The disorder causes the affected person’s red blood cells to disfigure into a crescent or sickle, which differs from the normal red blood cells that shaped into a doughnut. Sometimes, cells in a sickle shape cannot get through the narrow passageways of the blood vessels as easily as normal red blood cells. This blocks blood from entering and oxygen can’t reach the affected person’s organs. It can also crystallize and block arteries and
In most cases, people with sickle cell begin experiencing symptoms at the age of 4 months’ symptoms include fatigue which happens due to the loss of red blood cells after they rupture as they are extremely fragile. Another symptom will be the pain, it develops when other is a blockage within the blood vessels in your chest, abdomen, and joints. Intensity varies and can even lead to hospitalization. Delayed growth and vision problems of blood cells blocking areas of the body. You are more prone to infections and will also experience swelling of the hands and feet.
So what exactly is Sickle Cell Anemia? Sickle cell is an inherited blood disorder that affects red blood cells due to the presence of an abnormal form of hemoglobin, namely hemoglobin S. Sickle cell has a lot to do with natural selection and is known as genetic disorder. You may never know who will have it in your family. It will not affect everybody. Sickle cell effect the red blood cells in your body. The red blood cells in your body begin to become deformed. They begin to look, “crescent shaped and have difficulty passing through small blood vessels, which could slow or block blood flow and oxygen to parts of the body.” The cresset shaped is where sickle cell gets its name from. When sickle cell mutates in your body, it begins to effect the muscles and even could possible effect the oxygen flow into your body. When the oxygen is blocked, this causes pain and even harm to your organs and even muscles. When this happen, this effects a person ability to be active. According to web MD, “Severe pain is an emergency called acute sickle cell crisis. A person may not know what brought on the pain, but infection and dehydration are common triggers.”
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).
Sickle cell anemia mainly affects people with African, Mediterranean, Middle Eastern, and Indian ancestry. A person with sickle cell anemia inherits two sickle cell genes, one from each parent. It cause the red blood cells to change and become crescent shaped. Normal red blood cells are disk shaped and move easy through the blood vessels. The primary problem is hemoglobin, it is a protein molecule in red blood cells that carries oxygen from the lungs to the body’s tissues and returns carbon dioxide from the tissues to the lungs. In sickle cell anemia, the hemoglobin is flawed. The cells become sickle shaped and can’t move as easily through blood vessels. Sickle cell anemia has one main cause, in order for sickle cell anemia to happen, a sickle
This disease can cause severe pain and fevers, anemia, circulatory problems, strokes, and even “sudden death.” The sickle cells are able to get caught in the capillaries and disturbs the circulatory system (Eckman 447). When a person has abnormal swelling in their feet and hands, this could be caused by a buildup of “sickle-shaped red blood cells blocking blood flow to the feet and hands.” When sickle cells damage an organ, this can make a patient more “vulnerable” to infections (Mayo Clinic Staff). For example, when red blood cells “get stuck in the spleen,” this causes a splenic sequestration crisis. When this occurs, most of the blood cells are sent to the spleen which leads to less red blood cells being able to circulate in the blood stream. Symptoms of a splenic sequestration crisis include, “shortness of breath” and “having pale
Sickle Cell Disease is a genetic blood disorder that’s passed down to a child at birth from their parents. Babies inherit two abnormal genes one from each parent which causes the red blood cells to become deformed and change shape. Genes are elements is the cell that allows certain information determining traits like eye, hair color, etc. Normal rbc’s are flat, flexible, round with dimples in the middle taking on a doughnut like shape. These cells are able to move throughout the blood vessels suppling oxygen all over the body. Sickle rbc’s are long, stiff pointed ends, taking on an old farm tool called a sickle. Which is where its name derived from. These sickle shaped cell begin to bunch together causing a backup in the blood vessels causing
Sickle cells are fragile . They break a part easily and die, leaving you without a good supply with red blood cells. Red blood cells usually normal live for about 120 days before they ruptured and die and need to be displaced and formation of new cells. But sickle cells die after an average of 20 days maximum. This results in a lasting shortage of RBCs (ANEMIA). Without sufficient red blood cells in circulation, your body cannot gain the oxygen it needs to feel energized. That's why anemia causes fatigue.
Sickle Cell Anemia is a group of disorders that cause red blood cells to become misshapen and break down. Sickle cell disease is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans (Ashley-Koch, Yang and Olney). Sickle Cell Anemia causes your red blood cells to be thin, stiff, and shaped like a sickle. But your red blood cells are supposed to be round and soft. A person that is diagnosed with sickle cell anemia, their blood cells start to become clogged, causing blood clots, which can cause a great amount of health issues including: infections, stroke, and acute chest syndrome. People get sickle cell anemia by inheriting a mutated gene from both of their parents. On the down side, there is not currently an effective cure for sickle cell anemia, there are several different therapeutic approaches to treating and attempting to cure the disease and help people who are affected with sickle cell anemia better manage their symptoms. There have also been several great scientists that dedicated a lot of research to help find a cure for sickle cell anemia.