Sickle Cell Disease Process

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Identify the disease process presented by the case study as well as the normal structure and function of the organ system and/or physiological process that has the alteration.
Shawn, the main character of “The Uniformed Coach” scenario has sickle cell disease (SCD) and is suffering from a sickle cell crisis. Sickle cell disease is a form of hemolytic anemia that is inherited; being caused by an abnormal hemoglobin molecule. There are several forms of SCD but the most severe is simply called sickle cell anemia; the dysfunctional hemoglobin molecule, called hemoglobin S (Hb S), assumes an unusual shape when it is subject to deoxygenation or dehydration (McCance & Huether, 2014). In a normal person, all hemoglobin molecules are shaped like
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Pain crisis is the primary symptom of SCD; it is caused by sticky, sickle cells forming blood clots resulting in ischemia. Acute chest syndrome is a potentially life-threatening complication that occurs when blood clots collect in the small blood vessels that supply the lungs. Symptoms include fever, chest pain, dyspnea, chills, and hemoptysis. Pulmonary infiltrates are common as well as pulmonary hypertension, and the majority of patients are hypoxemic (Paul, Castro, Aggarwal, & Oneal, 2011). Splenic sequestration is another common complication; rising when the sickle cells become clogged and are trapped in the spleen causing rapid splenomegaly and pain (National Heart, Lung and Blood Institute, 2015). For many patients with SCD the spleen ceases to function due to repeated episodes of sequestration. Since the spleen plays a vital role in filtering blood and preventing infection, spleen dysfunction further increases morbidity and mortality for patients with SCD (Johns Hopkins Medicine,

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