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Pediatric Nursing (NUR1310) Sickle Cell Disease

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Sandra Watson
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Sickle Cell Disease – Professor Rounds-Stanley

Professor Rounds-Stanley

University

Broward College

Course

Pediatric Nursing (NUR1310)

Sickle Cell Disease (Anemia)
Definition: Sickle cell disease is a hereditary hemoglobinopathy characterized by the partial or
complete replacement of normal hemoglobin with abnormal hemoglobin S (Hb S) in red blood
cells. This causes occlusion of small blood vessels, ischemia, and damage to affected organs.
Pathophysiology:
 Hgb in the red blood cell acquires an elongated crescent or sick shape
 Any condition that increases the body’s need for oxygen or alters the transport of oxygen
may result in sick cell crisis
 The sickled cells are rigid and obstruct capillary blood flow
 Microscopic obstructions lead to engorgement and tissue ischemia, this local tissue
hypoxia causes further sickling and large infarctions
 Sickled cells can resume a normal shape when rehydrated and reoxygenated (although the
membrane becomes more fragile, decreasing their lifespan to about 10-20 days)
 In response to the shorter life span, bone marrow spaces enlarge to produce more red
blood cells

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Pediatric Nursing (NUR1310) Sickle Cell Disease

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