Sickle Cell Anemia: Difference between revisions
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== Characteristics/Clinical Presentation == | == Characteristics/Clinical Presentation == | ||
Individuals with this disorder may vary in their presentation of symptoms. Occlusion of the capillaries by the sickle shaped erythrocytes leads to acute and chronic tissue damage. Clinically, this can lead to widespread pain throughout the body that may last 5 or 6 days.<ref>Goodman CC, Snyder TK. Differential Diagnosis in Physical Therapy. China; Saunders Elsevier: 2000.</ref> As the name suggests, these patients are anemic, resulting in a presentation of fatigue, pallor and irritability. Acute episodes of symptoms are typical and may be brought on by physical exertion, extreme temperatures, fatigue or recent infections. Cerebrovascular accidents and acute chest syndrome may also occur as a result of occlusion of the blood vessels.<ref>Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. St. Louis; Saunders Elsevier: 2009.</ref> The sickled RBC’s may adhere to the lung endothelium and cause inflammation resulting in acute chest syndrome.<ref | Individuals with this disorder may vary in their presentation of symptoms. Occlusion of the capillaries by the sickle shaped erythrocytes leads to acute and chronic tissue damage. Clinically, this can lead to widespread pain throughout the body that may last 5 or 6 days.<ref name="Snyder">Goodman CC, Snyder TK. Differential Diagnosis in Physical Therapy. China; Saunders Elsevier: 2000.</ref> As the name suggests, these patients are anemic, resulting in a presentation of fatigue, pallor and irritability. Acute episodes of symptoms are typical and may be brought on by physical exertion, extreme temperatures, fatigue or recent infections. Cerebrovascular accidents and acute chest syndrome may also occur as a result of occlusion of the blood vessels.<ref name="Goodman">Goodman CC, Fuller KS. Pathology: Implications for the Physical Therapist. St. Louis; Saunders Elsevier: 2009.</ref> The sickled RBC’s may adhere to the lung endothelium and cause inflammation resulting in acute chest syndrome.<ref name="Goodman" /> This manifests itself as chest pain, fever, coughing, and shortness of breath. Pulmonary hypertension may also result from occlusion in the lungs. Stroke may occur in individuals with sickle cell anemia at a young age and may lead to disability or death.<br> | ||
== Associated Co-morbidities == | == Associated Co-morbidities == |
Revision as of 03:40, 4 March 2010
Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.
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Definition/Description[edit | edit source]
Sickle cell anemia is a genetic disorder characterized by abnormally shaped red blood cells due to an abnormal form of Hemoglobin within the RBC’s. The hemoglobin is able to transport Oxygen in a normal fashion, but once the Oxygen is released, the diseased molecules stick to one another and form abnormally shaped rods in the RBC’s. This in turn, causes the erythrocytes to become sickle shaped which are unable to squeeze through the small diametered capillaries, leading to occlusion of these vessels.
Prevalence[edit | edit source]
50,000-70,000 individuals in U.S. have this disease, with approximately 1000 babies born with it each year. Sickle cell is more prevalent in individuals of African descent, and affects 1 in 400 African American newborns in the U.S. African nations have a much higher incidence of this disease, with approximately 25% of individuals in Western and Central Africa positive for the sickle cell trait.
Characteristics/Clinical Presentation[edit | edit source]
Individuals with this disorder may vary in their presentation of symptoms. Occlusion of the capillaries by the sickle shaped erythrocytes leads to acute and chronic tissue damage. Clinically, this can lead to widespread pain throughout the body that may last 5 or 6 days.[1] As the name suggests, these patients are anemic, resulting in a presentation of fatigue, pallor and irritability. Acute episodes of symptoms are typical and may be brought on by physical exertion, extreme temperatures, fatigue or recent infections. Cerebrovascular accidents and acute chest syndrome may also occur as a result of occlusion of the blood vessels.[2] The sickled RBC’s may adhere to the lung endothelium and cause inflammation resulting in acute chest syndrome.[2] This manifests itself as chest pain, fever, coughing, and shortness of breath. Pulmonary hypertension may also result from occlusion in the lungs. Stroke may occur in individuals with sickle cell anemia at a young age and may lead to disability or death.
Associated Co-morbidities[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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