Other chronic complications include pigment gallstones, dlayed growth and development, aascular necrosis, plmonary hypertension and renal disease (Dale, Cchran & Jernigan, 2009, pNearly 30 % of sickle cell disease patients experience splenic sequestration at less than six years of age. Aute splenic sequestration may be classified as major and minor episodes. Mjor episodes are characterized with rapid enlargement of the spleen and circulatory collapse requiring transfusion. Tis is life threatening compared to minor episodes which involves rapid enlargement of the haemoglobin reduction is less sever (Meier & Miller, 2012, haemoglobin (HbF) prevents polymerization of HbS therefore reducing haemolysis leading to sickle erythrocytes that have large amounts of HbF survive longer than those with lower concentration.
Ifants are from face lesser severe pain. Te pain often first occurs in small bones and hands and feet. Aproximately 50% of children with sickle cell disease start developing the pain by two years of age. I early childhood, sckle cell patients experience acute chest syndrome. Te patients develop symptoms such as cough, fver, weezing and chest pain. Aute chest syndrome is highest HbSS and HbSβothalassaemia (Meier & Miller, 2012, pDuring hypoxia, aidosis or in the setting of pyrexia or dehydration, te HbS molecules polymerize and form linear elongated fibres that distort the shape of the red blood cells.
Tis polymerisation causes chronic haemolysis with concomitant vaso-occlusion. Vso-occlusion is principal in diagnosis. Aute chest syndrome is also associated with diagnosis of sickle cell disease in children (Meier & Miller, 2012, pIn children, Tanscranial Doppler Test (TCD) screening could decrease the stroke rates in sickle cell disease as TCD is able to detect vessel disease in patients which are most likely involved in overt strokes.
Slent Infarct Transfusion Trial is used in treatment of Silent cerebral infarct (Meier & Miller, 2012, pPharmacological treatments such as hydroxyurea, sem cell transplantation or physical therapy may help improve the health of sickle cell patients. I children, te stating hydroxyurea dose is 20-50mg/kg/day and is escalated by 5mg/kg/day until a dose of 30- 35mg/kg/day is reached. I patients experience myelosuppression, hdroxyurea should be held back for two weeks...
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