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The Devastating Effects Of Sickle Cell Anemia

Posted Apr 01 2009 3:02pm
Sickle Cell Anemia (SCA) is an autosomal recessive genetic disorder, which means that one is born missing a chromosome, which is what shares DNA with another. And SCA causes chronic hemolytic anemia, which unlike anemia itself, actually destroys the red blood cells within a SCA patient, and not simply is depleted of such cells as is the case with anemia in general. SCA affects almost entirely the black population for reasons unclear as far as the race goes, but the disease is in fact due to a mutated gene assigned to regulate hemoglobin within red blood cells. The red blood cells (RBCs) take on a rigid and sickle shape in the bloodstream of those affected.   SCA was first discovered around 1950, and researched as well afterwards. SCA typically presents itself in one’s childhood, as this anemia is rather ruthless in the damage it cause various areas of the human body. The average life expectancy of A SCA patient is in the mid 40s presently.    The low life expectancy is due to the multiple clinical consequences that occur due to SCA.   The hemolysis that occurs causes excessive destruction of one’s red blood cells.    As a result the spleen of the SCA patient becomes impaired due to the overload of red blood cells in now has to remove from the blood stream of the SCA victim.  And the SCA victim has to rely on his own bone marrow to reproduce additional red blood cells destroyed because of this sequence of events. Approximately 70 thousand people are with SCA in the United States presently, and all experience this self destruction within them due to SCA. Symptoms that happen due to SCA include what is called a pain crisis, anemia, infections, spleen inflammation and damage, and infections due to decreased blood flow to various parts of the body.     With SCA children, they are particularly at a high risk for both a stroke and lung disease as a result of this disorder. Treatment options for SCA include hydroxyurea, which is an anti-metabolite that has the ability to reduce the number of sickle cells present in a SCA patient as well as increases their hemoglobin levels- both of which are needed with the SCA patient. SCA patients report a deceased number of pain attacks on hdroxyurea as well. Blood transfusions occur at times with SCA patients- perhaps every few weeks.    Pain management is needed for the SCA patient often at times. There is ongoing research regarding sickle cell anemia, and there is hope that better treatment options, if not a cure, will be created sooner rather than later.   Because in this certain disease, anemia does not only mean one who is without blood.    This anemia means the SCA victim is also without comfort, and often, without peace. www.saonline.org 

Dan Abshear

 

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