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Understanding the Pathophysiology of Sickle Cell Anemia and Its Impact on Treatment Options

  Pathophysiology of Sickle Cell Anemia The pathophysiology of sickle cell anemia : Genetic mutation and the hemo-globin molecule Source: Restriction site mutation, 11 β -globin gene. Findings: 1 Valine residue at 6-position in the 8 globin protein has replaced a glutamic acid residue. Effect: They don’t produce normal adult haemoglobin, they produce sickle haemoglobin.” Polymerization of Hemoglobin S When oxygen is released, HbS polymerizes into a long, rigid rod. That causes red blood cells to be comet-shaped or sickled. Red Blood Cell Sickling Sickled RBCs are: Hard and less flexible Susceptible to hemolysis (breaking up prematurely) Have a shorter life span (~10–20 days compared to 120 days for healthy RBCs) Vaso-occlusion Sickled cells: Become trapped in small capillaries Induce obstructions (vaso-occlusion) in blood vessels Lead to tissue ischemia, pain (sickle cell crises), and organ injury Hemolysis and Anaemia Progressive destruction of sickle cells results in: Levels of h...
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Sickle Cell Anemia and Pregnancy: Risks, Complications & Safe Delivery Options

  The pregnancy of a woman who has Sickle Cell Anemia is considered to be a high-risk pregnancy though with adequate medical care, most women have been able to do a successful pregnancy and birth healthy babies. Both the mother and the child should be aware of the risks involved and any complications that are likely to be experienced and the safe ways of delivery. The Pregnant Women having Sickle Cell Anemia: What happens? Sickle cell anemia influences the red blood cells and makes them stiff and in the shape of a sickle. Already increased oxygen requirements and alterations in the blood volume increase the complication of: The low oxygen levels in the baby. Greater frequency of acute pains. Higher stress on organs Risks to the Mother Women carrying the sickle cell anemia virus can meet the following health risks in pregnancy: Increased Pain Crises Bouts of acute pain (vaso-occlusive crisis) can increase in frequency. Initiated by dehydration, infection or stress. Severe Anemia Red...
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Location: Kenya
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