Sickle Cell Anemia

Posted: November 27th, 2013

Sickle Cell Anemia

 

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Abstract

Sickle cell anemia is a blood disease that is inherited and it affects people worldwide. This research seeks to find out more on the issues concerning sickle cell anemia inclusive of its causes, complications, diagnosis and treatment. According to various studies, the sickle cell condition affects a particular protein found in red blood cells referred to as hemoglobin. An individual may experience the disorder when he or she inherits two abnormal genes from parents suffering from the disease. However, it is not possible for a person to suffer from sickle cell anemia when he or she inherits such genes from one parent. Such individuals become sickle cell carriers. An affected individual is likely to know if they have sickle cell through various signs and symptoms. These may involve swelling of hands and feet, frequent pain crises, recurrent infections, and loss of eyesight due to damages in the part of the eye that holds visual images. Credible research will be used to identify various advances noted with regard to the same.

 

Sickle Cell Anemia

According to research, anemia is a disease that is inherited affects individuals from most parts of the world especially from the African countries, the Mediterranean and Middle Eastern descent. This disorder is known to affect a certain protein found in red blood cells referred to as hemoglobin, importantly used in carrying oxygen to all parts of a human body. An individual may experience sickle cell anemia upon inheriting two abnormal genes from parents suffering from the given ailment. These abnormal genes thus interfere with the red blood cells by modifying their shape. For instance, red blood cells may become rigid and curve-in assuming the shape of the traditional farm tool called the sickle rather than attaining flexibility and a disc-shape as normally required. The rigidness and sticky shape occurs due to the clumping of the hemoglobin within the cells.

According to studies, red blood cells are supposed to be very flexible because they need to move freely and easily through the bloodstream, providing enough oxygen to all other human body cells. Research has further indicated that the sickle cell condition is a type of anemia; a condition noted when a person’s blood has few than the level of red blood cells. Note that, red blood cells are formed in bone marrow that is placed inside big bones of the body. New blood cells are always made in order to replace old ones. Supplementary research reveals that the ordinary hemoglobin cells live averagely one hundred and twenty days in the blood stream and die afterwards.

In as much as the red blood cells are responsible for providing oxygen in the body, they are also quite significant in removing carbon dioxide. However, sickle-shaped cells are fragile and tend to break down only after lasting averagely ten to twenty days hence causing anemia. Individuals with anemia are always fragile in the sense that they get exhausted easily and are prevalently weak. Such individuals may also encounter some complications due to impaired blood circulation and fighting of bodily infections. This is because anemic individuals are prone to certain infections such as acute chest syndrome that is known to occur as a result of inflammation, infection or even blockage of blood vessels in the lungs caused by the sickled red blood cell.

According to Mayo (2011), sickle cell anemia is not easily transmitted from one person to another like a cold or other similar air infections. This is because the disease is transferred through inheritance activities from parents in terms of abnormal gene computations. However, individuals who inherit sickle genes from only one parent may not suffer from the ailment although they may bear traits or symptoms of the same that may be passed on to their own generations. Unfortunately, individuals with sickle cell symptoms are always largely not aware of their status therefore, it is hard for them to discover that they have sickle cell genes. Hence, it is always important that teenagers who doubt their statuses should seek for medical attention concerning the disease.

Causatives of  Sickle Cell Anemia

Sickle cell anemia is caused through the mutation of a gene responsible for producing hemoglobin or red blood cells that are rich in the iron compound evidenced in the red blood color. Generally, sickle cell is an inherited problem that tends to occur in generational terms. According to research, sickle cell is transferred as an autosomal condition illustrating that it is not related to a sex chromosome. Sickle cell carriers inherit the same gene in an autosomal form that is portrayed as a dominant trait. Therefore, for the sickle cell condition, two autosomal genes must be inherited from the parents constituting to two sickle cell genes in the child. Sickle cell carriers do not experience many health problems thus they tend to live their lives just like other healthy people. The distortion of red cells is enhanced by the lack of enough oxygen, high levels of acidity and low blood quantity. Causes of these conditions may include tissues injury and dehydration. Some organs that have high rates of metabolism tend to increase the distortion of sickle cell anemia through their usage of more oxygen from the blood making the organs exposed to various injuries.

Signs of Sickle Cell Anemia

Commonly, symptoms attached to sickle cell anemia occur due to the abnormal shapes of red blood cells and blockages of blood circulation within the body. In fact, body tissues experiencing impaired blood circulation encounter extreme damage due to lack of enough oxygen. Signs of sickle cell disease are always realized when a child is at an age of four months old. Individuals affected with the same are known to be fragile and therefore they break down easily. The sickle-shaped cells are always weak and thus needs urgent and recurrent replacements. Studies show that red blood cells are able to live up to one hundred and twenty days but the sickle cells can barely manage this leading to chronic reduced red blood cells (Landau, 1996). It is extremely hard for the human body to get enough oxygen with fewer red blood cells present. Hence, because of this lack of enough circulation anemia leads to unrelenting exhaustion.

Another sign of sickle cell disease includes frequent periods of pain, which are termed as pain crises. This pain occurs mostly when red blood cells block blood flow in small blood vessels, notably on a person’s chest, arms, legs and other joints. The pain may stay for a few hours or even weeks and varies in intensity depending with the person and blockage intensity. In some circumstances, the pain may be so severe that the patient may require to be hospitalized. Some individuals experience hand and foot syndrome and this is easily noted in children through hands and feet swellings. The swelling is caused by the sickled red blood cells that inhibits blood movement form the hands and feet.

Ever occurring infections are another significant sign of sickle cell anemia. This is because sickle cells are in position to cause great damage to the spleen, which helps in fighting infections (Monroe, 2008). This always leaves the affected populace vulnerable to infections. To help young children in coping well with the condition, health providers offer antibiotics that help in preventing potential life threatening infections like pneumonia. Individuals with the sickle cell ailment may also experience delays in growth. This is because of lack of red blood cells that help in providing the body with enough oxygen and nutrients that the body needs in order to grow. Some people also may loose their eyesight because the tiny blood vessels that distribute oxygen in the eyes may be plugged with sickle cells hence damaging the part of the eye that holds visual images.

Problems associated with of Sickle Cell Anemia

Various complications may occur as a result of sickle cells anemia. These include stroke that occurs after the flow of blood has been blocked by sickle cells to specific parts of a person’s brain. Symptoms of stroke include difficulties in speech, loss of consciousness, and seizures (Lin-fu, 2009). Therefore, it is important to identify such symptoms early enough and seek medical attention because stroke is a very dangerous problem that needs early treatment. Another complication includes acute chest problems that lead to fever and breathing difficulties. This problem may be as a result of an infection in the lungs or the blocking of blood vessels in a person’s lungs by the sickle cells. Acute chest syndrome is dangerous and thus requires urgent medical attention to avoid further complications.

Individuals suffering from sickle cell anemia are most likely to experience high blood pressure in the lungs and breathing difficulties hence leading to pulmonary hypertension. Sickle cells may also lead to the damaging of body organs such as the spleen, kidney, and liver due to blockages of blood flow in body vessels hence reducing the amount of blood and oxygen in an organ or tissue. The affected may also loose eyesight and skin ulcers; the latter may lead to the advancement of open sores especially on the legs. Additionally, one may experience gallstones and priapism leading to the blockage of male organs.

Diagnosis of Sickle Cell Anemia

In the process of performing a diagnosis to a person suffering from sickle cells anemia or being a carrier of the disorder, a blood test may be carried out to help in checking defective hemoglobin. This process is achieved through the use of a certain tool termed as the hemoglobin electrophoresis. The test is fit for both children and adults considered that the problem affects both populaces. Current research explains that most countries have implemented a law that every newborn child has to go through a sickle cell anemia test before leaving the hospital. In addition, expectant mothers may also decide to have unborn children tested for the same prior to the delivery period.

This is always procured in antenatal screening specializing in checking any inherited diseases towards sound parental decisions and counsel. For instance, in determining if a person suffers from the sickle cell disease, after the test has been performed a small level of defective hemoglobin should be used to establish whether a person has the sickle cell trait but not suffering from the actual condition. Note that, a high level of defective genes indicates that a person is infected.

Another process that takes places during diagnosis includes guidance and counseling. For instance, some mothers or parents may be upset and depressed after realizing that their unborn child is going to have sickle cell anemia. Therefore, counseling is very important to enable a couple to have the opportunity to express their feelings, understand and get more information concerning the effects of the condition as well as handling practices. The counseling process also acts as an opportunity to find for discussing the most preferable treatment approaches for prudent decisions.

Treatment

According to Beshore (1994), sickle cells anemia can be cured through a transplant of bone marrow, which is known to be the only possible cure available presently. However, identifying and acquiring a donor has always proved to be very difficult and the steps taken in performing the transplant are very complicated with many risks such as death. Therefore, the best alternative for dealing with the sickle cell disease involves avoiding pain crises, relieving notable symptoms, and preventing further complications through the use of antibiotics and regular check-ups towards verifying the level of red blood cells count. Medications are always supplied to a patient to help in pain reduction.

Young children recognized with anemia are advised to use antibiotics in particular penicillin at the age of two years and continue doing so until they reach the age of five years. In addition, it is necessary to provide such children with vaccinations to help prevent various infections. In order to reduce the rate of risky situations like stroke, blood transfusion should be utilized towards aiding with the number of the required red blood cells in circulation. Research has actually revealed that frequent blood transfusion in young children helps them a great deal especially in reducing stroke instances.

References

Beshore, G. (1994). Sickle cell anemia. Boston, MA: F.Watts.

Landau, A. (1996). Sickle cell disease: psychological and psychosocial issues. New York, NY: University of Illinois Press.

Lin-fu, J. (2009). Sickle cell anemia: a medical review. Los Angeles, MA: Bureau of Community Health Services.

Mayo, T. (2011). Sickle cell anemia. Mayo foundation for medical education and research. Retrieved from http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324

Monroe, P. (2008). Sickle Cell Anemia. New York, NY: The Rosen Publishing Group.

 

 

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