Sickle Cell Anemia
As an inherited condition, it is presence of hemoglobin which tends to be abnormal that brings about sickle cell anemia. In basic terms, hemoglobin is a red blood cell protein whose main function is carrying oxygen. It is this hemoglobin abnormality that informs ‘sickled’ or distorted red blood cells whose survival is compromised as a result of the distortion and fragility. Though the prevalence of the condition largely varies in accordance with the region, the Center for Disease Control and Prevention points out that of all blood disorders, sickle cell anemia has earned itself a distinction as the most commonly inherited in America. In this text, I come up with a complete and concise definition of the disorder and its prevalence. I also detail how the condition affects body systems and how it is treated.
A Detailed Overview of the Condition
When it comes to individuals with sickle cell anemia, their normal hemoglobin largely differs from sickle or abnormal hemoglobin (hemoglobin S). According to Crowley (2009), abnormal hemoglobin is brought about by the alteration of globin chain forming amino acids as a result of an abnormality in the genes which control amino acids arrangement in hemoglobin. Crowley also states that the functioning of the abnormal hemoglobin is not affected in some cases. However, in some other cases, the abnormal hemoglobin may develop some properties which are largely unusual hence effectively leading to an impairment of their functioning.
While red blood cells that are rather normal can move with relative ease within vessels and are usually round in shape, abnormal red blood cells usually have their movement largely affected (especially through blood vessels which are significantly small ) as a result of their strange crescent shape. As a result of this impairment, they commonly get stuck and effectively block other blood cells. With this blockage, it becomes difficult for the red blood cells to reach the various organs as well as tissues hence eventually damaging the same. This blockage is known to bring about what is commonly referred to as a sickle cell crisis episode. During a sickle cell crisis, severe pain brought about by the denial of oxygen to a certain body part is experienced (Falvo, 2005). Indeed, it can be noted that severe obstruction of red blood cells flow may bring about tissue death or what is also referred to as necrosis.
Further, the fragility of abnormal red blood cells commonly informs their rupture. It is this rapture or what is also known as hemolysis that brings about a sustained decrease in the red blood cells count hence resulting in anemia. According to Khanna (2009), the average lifespan of abnormal red blood cells is between ten and twenty days. However, normal blood cells have an average lifespan of up to one hundred and twenty days.
Sickle Cell Anemia: Signs and Symptoms
It can be noted that when it comes to this condition, the signs and symptoms largely vary. Hence in this sense, symptoms could either be severe or mild. However, for infants, it may take up to approximately four months after birth for signs of sickle cell anemia to show up; though the condition is present at birth. In most cases, sickle cell anemia exhibits signs which are either loosely or strongly connected to anemia with occasional bouts of pain. With that in mind, most of the other evident signs and symptoms are brought about by the complications of the condition.
In most cases, individuals having sickle cell anemia may occasionally feel rather weak and tired. The United States Department of Health and Human Services gives other symptoms of the condition as jaundice, constant headaches and occasional feelings of dizziness. Other symptoms given in this case include feet and hand coldness and short breaths. It should be noted that these signs and symptoms are also in one way or the other linked to anemia. Further, individuals with the condition may experience occasional but sudden body pains i.e. In what I have explained earlier on in this text as sickle cell crisis. The occurrence of pain in this case also largely depends on an individual with some people experiencing severe pains only occasionally while for others, the same may be more frequent. Further, sickle cell anemia can be identified based on the complications it brings about. According to United States Department of Health and Human Services, these complications may include stroke, leg ulcers or sores, erections (in males) that are painful and unwanted, eye problems and in some cases; multiple organ failure, a rather serious but very rare occurrence.
How Body Systems are affected by the Condition
Sickle cell anemia comes with a number of complications which affect the body systems of an individual. In this text, I only give some of the most common complications brought about by the condition. To begin with, sickle cell anemia may bring about hand-foot syndrome where foot and hand blood vessels (small) are blocked by the abnormally shaped red blood cells. This blockage can bring about fever, swelling and pain and is mainly common among children under the age of four. An enlarged spleen is yet another complication brought about by sickle cell anemia. In this case, it is the trapped red blood cells that should ideally be in circulation that informs the enlargement of the spleen. This occurrence is referred to as the splenic crisis. Damage to the spleen may also impair the ability of an individual to fight various infections.
The eyes of an individual having sickle cell anemia may also be affected. In this case, the eyes are denied oxygen supply as a result of the blockage of the vessels that deliver blood rich in oxygen to this organ. In some cases, this can end up bringing about blindness. Sickle cell anemia also damages the penis as a result of a condition known as priapism. This occurs when there is blockage of blood flow to the penis.
Treatment of sickle cell anemia is mainly tailored to keep the condition’s symptoms under control as well as to reduce the crises frequency. One medicine that is in most cases used to lower crises is hydroxycarbamide. This medicine facilitates fetal hemoglobin production which is a hemoglobin type that brings down the sickle cell numbers. Painkillers may also be prescribed for purposes of reducing pain. Further, to protect an individual from infections, a regular dose of vaccines may be necessary. Antibiotics may be prescribed incase of an infection.
Bone marrow transplant is another alternative in the treatment of sickle cell anemia. In this case, the bone marrow of an individual with the condition is replaced with that of an individual without the condition hence stimulating the production of healthy normal shaped red blood cells. However, this procedure is in most cases only suitable for children.
According to Arnold (2011), sickle cell anemia in most cases affects blacks as opposed to any other race. Arnold also states that of the total black American population in the U.S., 8% is believed to have the condition. The numbers of those who carry the sickle gene in the U.S. stands at approximately two million. However, the sickle cell trait is only prevalent in one out of twelve African-Americans. Internationally, the condition is mostly common in the sub-Saharan Africa.
In conclusion, it should be noted that when it comes to sickle cell anemia, there exists some special considerations for expectant women. In this case, those who have the condition should be monitored closely by a doctor and are advised to seek immediate medical attention incase of any complication, crisis or infection. It is hoped that going forward, advancements in medicine and technology will shed more light into the nature, management and treatment of sickle cell anemia.
Arnold. J.L. (July 11th, 2011). Sickle Cell Anemia: Epidemiology. Retrieved July 27th, 2011, from the Medscape Website: http://emedicine.medscape.com/article/205926-overview#a0156
Crowley, L.V. (2009). An Introduction to Human Diseases: Pathology and Pathophysiology Correlations. Jones & Bartlett Learning.
Falvo, D.R. (2005). Medical and Psychosocial Aspects of Chronic Illnesses and Disability. Jones & Bartlett Learning.
Khanna, P. (2009). Essentials of Genetics. I.K. International Pvt Ltd.
U.S. Department of Health and Human Services & The national Institute of Health (nd). What are the Signs and Symptoms of Anemia? Retrieved July 27th, 2011, from the National Heart Lung and Blood Institute Website: http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_SignsAndSymptoms.html
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