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Sickle cell disease is a single gene disorder that was first described in 1910 by Herrick (1910).  The disease involves sickle hemoglobin, which mainly results in the sickle-shaped conformation of the red blood cells, which in turn occludes small blood vessels of the circulatory system (Mohandas and Hebbel, 1994).  Such attachment of sickle reds to small blood vessels causes the abnormal proliferation of epithelial cells in the lining of the bigger blood vessels, resulting in the decrease in the general blood flow (Hebbel et al., 1980).  Patients with sickle cell disease also present with significantly high white blood cell counts, mainly due to the generation of cytokines (Hofstra et al., 1996).  Sickle cell disease is also associated with a susceptibility to thrombosis or bleeding, which may be influenced by both genetic and environmental factors such as acid levels in the muscular tissues and body fluids, excessive loss of water or dehydration, extremely low temperatures and infections.  Sickle cell disease also causes an approximate 50% decrease in the patient’s hematocrit, resulting in anemia.In extreme cases of anemia in pediatric patients, cardiomegaly results as a consequence of the increase in blood flow and cardiac load, in addition to extensive hematopoiesis that is influenced by chronic hemolysis.  Such condition increases the amount of calories that is needed to maintain proper growth.  The anemic condition is aggravated by bone marrow aplasia and splenic sequestration among pediatric patients with sickle cell disease, resulting in the threat of circulatory failure.  The spleen serves an important role during pediatric development because it plays a major role in protecting the individual from bacterial infections.  It also produces opsonins and macrophages which are the frontline protectors against immunological insults.  It has been observed that pediatric patients with sickle cell disease experience dysfunctional spleen activity mainly due to its occlusion with sickled red blood cells.Sickle cell disease may be diagnosed at birth through the employment of hemoglobin electrophoresis, isoelectric focusing (IEF), high-performance liquid chromatography (HPLC) or DNA analysis, all depending on the availability and price of the diagnostic test in the area of the patient.  Two other testing methods, namely solubility testing Sickledex and sickle cell preparations, have been determined to be insufficient and inappropriate in ascertaining sickle cell disease because these assays only identify sickle hemoglobin but do not consider hemoglobin C and other genetic variations of sickle cell disease.  In addition, the results of solubility testing are often erroneous or misguiding, especially in newborn testing, wherein the amount of fetal hemoglobin is generally high.  More importantly, solubility testing is usually incapable of determining sickle hemoglobin in individuals presenting severe anemic conditions.The maintenance of a pediatric patient with sickle cell disease consists of preventive measures against further complications with other diseases, as well as modes of facilitating the diagnosis of sickle cell disease and improvement of their physical well-being.  The attending physician of the pediatric patient oftentimes educates and guides the parents in taking care of their child who is afflicted with sickle cell disease (Thompson et al., 1994).  The physician primarily states that a child with sickle cell disease should not be given extreme amounts of protection or disregard.  It is often ideal that the entire family participate in interacting and giving care to the sickle cell child, and that being involved with the child is important to the psychological and social development of the pediatric patient.  It is also of great importance that the parents and any other individuals taking care of the pediatric patient to quickly identify any symptoms or signs of complications of sickle cell disease.  This responsibility also includes learning, understanding and applying any necessary measures for the well-being of the child.These include the employment of antibiotics during bacterial infections and routinely bringing the child to the pediatrician for routine checkups and immunizations.  These individuals should also have knowledge of laboratory tests and values for a comprehensive understanding on how to take care of the child.  Signs that signify the need for immediate medical attention among pediatric patients with sickle cell disease include the abrupt increase in size of the child’s spleen.  Usually, the parents and any other caregivers are taught by the physician to learn how to determine the size of the child’s spleen by simple palpation.  Other signs that should be given immediate attention include increase in body temperature, loss of color of the child’s skin, lips and nails, as well as significant changes in breathing modes.  Presentations of pain and discomfort in moving arms and legs are also classified as serious signs for immediate medical attention.  Caution should also be given with regards to the child’s level of dehydration and contact with cold temperatures.Pediatric patients with sickle cell disease are generally given 125 mg of penicillin twice a day starting at the age of two months.  Upon reaching three years of age, the pediatric patient is given twice the dose of penicillin twice a day.  This antibiotic administration is continued until the pediatric patient reaches five years of age, unless the patient undergoes a surgical removal of his spleen.  In addition to penicillin, an immunization against pneumonia is given to a child with sickle cell disease at two months of age.  This vaccine is important to the pediatric patient for his protection against infection and will be boosted three years later.  The child is also administered influenza vaccinations at six months of age.  A meningococcal vaccine is also given to the sickle cell disease child when a dysfunctional spleen has been determined.  A child with sickle cell disease should be monitored in terms of growth and development.  Supplemental vitamins are generally given to the child, as well as sufficient amounts of iron, as early as infancy.  In order to have enough red blood cells circulating in the pediatric patient’s system, folic acid should also be given.  If the child weighs less than the normal range of weight at his specific age, protein supplements are also provided for nourishment.A child with sickle cell disease is regularly monitored for hemoglobin levels and hematocrit, as well as reticulocyte counts, white blood cell (WBC) counts, and liver and renal function.  The levels of iron during infancy of the pediatric patient are also monitored to make sure that the child has sufficient iron for proper hemoglobin transport in the body.  Rapid growth among children may cause iron deficiency in young patients with sickle cell disease.  The optimal setting should be determined by the physician, because children with sickle cell disease often undergo blood transfusions, hence the administration of supplemental iron, together with blood transfusions, may also result in iron overload.  Renal disease is a complication of sickle cell disease, hence it is important that regular urinalysis is performed on the pediatric patient.  Other medical conditions such as blood in the urine (hematuria) and protein in the urine (proteinuria) may also be determined from urinalysis.  There are some cases wherein the child with sickle cell disease may also suffer from retinal disease around 10 years of age.  Hence, it is also important that routine retinal diagnostics are also conducted in pediatric sickle cell disease patients.Sickle cell disease can also be present in different hemoglobin.  In hemoglobin sickle cell, the pediatric patient does not show any signs of anemia.  In another form, sickle ß+-thalassemia, anemia is not observed and in turn, hypochromia is observed instead of iron deficiency.  A specialized physician, a hematologist, may definitely diagnose any variants of sickle cell disease in children.  The analysis of the blood of the pediatric patients’ parents may also facilitate the diagnosis of sickle cell in a child.  Presently, DNA analysis serves as the most efficient method for determining sickle cell disease, especially in children.  Sickle cell disease is commonly observed among African-Americans, with a prevalence ratio of 1 in 375 (Bowman and Murray, 1990).  The hematological disease has also been observed in Turkey, India and the Arabian countries.  It has also been observed that individuals of South and Central American origins are also affected by sickle cell disease.The comprehensive study of sickle cell disease in the last century has generated a comprehensive understanding of this disease, as well as provided efficient methods in the early diagnosis of the disease, especially in children.  Such efforts have also improved the treatment of the disease, and more importantly, have increased the life expectancy of patients afflicted with this hematologic disease.  The aggressive education and genetic counseling of healthcare practitioners have also enhanced the ways of parents, family members and physicians in taking charge of taking care of pediatric patients with sickle cell disease. 

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