KINESIOL 1Y03 Chapter 19.12: Sickle Cell Disease
20 Sep 2017
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Pathophysiology (figure 8 below: at low po2, deoxy hbs polymerizes, leading to rigid crystal-like rods that distort membranes -- Splenic sequestration crises usually in children, with significant pooling of blood in spleen, resulting in acute decreased hb and shock uncommon in adults because of functional asplenia from repeated infarction (adults with hbsc may not have functional asplenia) Vaso-occlusive crises (infarction) may affect many different organs causing pain (especially in back, chest, abdomen and extremities), fever, and leukocytosis (e. g. acute chest syndrome) precipitated by infections, dehydration, rapid change in temperature, pregnancy, menses and alcohol. Hb electrophoresis normal; possibly a few target cells. Hba fraction of 0. 65 (65%); hbs fraction of 0. 35 (35%) Hbss increased reticulocyte, decreased hb, decreased hct sickled cells. Other: hb electrophoresis distinguishes hbas, hbss and other variants. Treatment: genetic counseling, folic acid to prevent folate deficiency, hydroxyurea to enhance production of hbf.
