Pathophysiologyof Sickle Cell Anemia
The12 year old J.L suffers from sickle cell anemia which ischaracterized by insufficient supply of oxygen to the body cells.Hemoglobin plays an important role of transporting oxygen throughoutthe body. Sickle cell anemia results from genetic mutation in thesickle hemoglobin where adenine is replaced by thymine on the 6thcodon of the genetic structure. The genetic alteration affects thephysical properties of the hemoglobin including stability andsolubility(American Psychological Association, 2010).
Sicklecell hemoglobin are curved sickle-shaped rather the normal flat diskshape. The alteration in shape makes them rigid and less flexible andthus more vulnerable to hemolysis and may also cause blockages in theblood vessel thus disrupting the blood flow in the body(American Psychological Association, 2010).The patient may also suffer from heart attack and progressiveproliferative systemic vasculopathy becauseof the insufficient perfusion as a result of the blockage.
Asthe condition continues to develop, the membrane of the cells underpermanent damage causing them to be permanently bi-concave in shapewhich cannot carry sufficient oxygen even when blood is exposed toenough levels of oxygen(McCance & Huether, 2010). The sickle cell crisis may result in the increased blood viscosityand block formation in the blood vessels. The cells group together toform rigid mass that prevents continuous and free flow of oxygenatedblood to the body tissues, this causes severe pain to the patient.The twelve years old patient is likely to develop other types ofanemia may emerge because of the increased hemolysis of erythrocyteswith the sickle hemoglobin in the spleen. Excessive destructive ofthe red blood cells result in the accumulation of bilirubin andjaundice(Qaseem, et al., 2013).
Thepatient is likely to develop multiple organ failure and alsoexperience painful moments because of the insufficient oxygen supplyin the body. Mainorgans such as skeleton, heart, spleen, kidney, eyes and lungs mayfail because they are highly sensitive to oxygen. The spleen plays amajor role in fighting infections in the body therefore, its failurepredisposes the patients to infections because of the compromisedbody’s defense system(McCance & Parkinson, 2010).
AmericanPsychological Association. (2010). APAManual (Publication manual of the American PsychologicalAssociation).Washington, DC: Author.
McCance, K. L.,& Parkinson, C. (2010). Studyguide for Pathophysiology, the biologic basis for disease in adultsand children, sixth edition.
Qaseem,A., Humphrey, L. L., Fitterman, N., Starkley, M., & Shekelle, P.(2013). Treatment of anemia in patients with heart disease: Aclinical practice guideline for the American College of Physicians.Annals of Internal Medicine, 159(11), 770-779.