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Sickle Cell Anemia (Sickle Cell Disease)


Sickle cell anemia, also know as sickle cell disease is a genetic disorder that effects the structure of red blood cells within the body.  Although there is no cure for this disease, it being genetic, there are ways of treating the complications due to sickle cell anemia, and as this article progresses some of these treatments and complications will be addressed.  The purpose of this article is to provide a general and brief overview of sickle cell anemia and as such it should be considered only as a starting point in an effort to gain further education about this genetic disease.  As always, should you require immediate medical help with this or any other disease or disorder, or would like definitive medical advice, you should contact a trained and certified health care professional such as your family physician.


Sickle cell anemia is characterised by the unusually “sickle” shaped red blood cells found in the blood stream.  Normal red blood cells more flexible that sickle cells and are roughly circular in shape, thicker at the edges than in the center.  For those who are unfamiliar with the term sickle in reference to the shape of the blood cells, it might help to think of a crescent moon, which is also sickle shaped.  The complications due to sickle cell anemia have to do with the red blood cells circulating throughout the body, which as a result of their unusual shape can become caught in blood vessels – a painful condition.  Sickle cells are also much unhealthier than regular blood cells and as such do not last as long as they might otherwise do so, which leads to problems with the amount of red blood cells available in the blood stream.  The unusual sickle shape is a result of a complication in the haemoglobin in the red blood cells.


History of Sickle Cell Anemia


The history of Sickle cell anemia begins for the most part in the early beginnings of the twentieth century when James Herrick and Ernest Edward Irons in 1910 discovered the unusual sickle cells, so named for their sickle like shape in a patient who was suffering from anemia.  However it was not until some twelve years later that sickle cell anemia was actually named by Vernon Mason.  This is not to say that the disease was not in fact prevalent long before it was identified as such by Western medicine.  Rather, this had been a common disease with a long history.  The next stage in the history of sickle cell anemia was the 1949 proof by Linus Pauling that proved that the unusual shape of the red blood cells in sickle cell anemia was due to an alteration of the haemoglobin within them.  It is unclear where the genetic mutation, as sickle cell anemia is indeed caused by genetic factors, originated from, though it is hypothesized by some that it has its roots in Africa, the Middle East and India many generations ago.


Symptoms of Sickle Cell Anemia

Sickle cell anemia, being a genetic disorder, is ultimately characterised by the genetic mutation that causes it which, according to the Mayo Clinic, effects one in twelve black Americans.  As the name of the disease would suggest, one of the characteristic aspects of sickle cell anemia is anemia itself, which is a result of a lack of viable red blood cells leading to a lack of oxygen circulating in the body, characterised by a feelings of fatigue or lethargy.  In addition, the unusual sickle shape of these blood cells can cause them to become stuck in blood vessels throughout the body leading to intense feelings of pain that may require hospitalization.  The pains themselves may last between a few hours to a few weeks at a time, varying in severity, location and frequency based on the individual experience of each discrete case of sickle cell anemia.  Another symptom of sickle cell anemia is a swelling in the hands and feet, which most often characterised the presence of the disease in infants and is known as Hand-foot syndrome.  There are a number of other symptomatic difficulties for those suffering from sickle cell anemia and these include vision problems, frequent infections, stunted growth and a yellowing of the skin known as jaundice, which stems from liver damage.


How to Treat Sickle Cell Anemia


The only way in which sickle cell anemia can be potentially cured is through a bone marrow transplant, which in of itself is a very difficult procedure to undergo at the very least because there are so few suitable donors for each individual who requires them.  In general, individuals who have sickle cell anemia spend more time attempting to alleviate the symptoms due to complications.  As a result it is not uncommon for those who are suffering from sickle cell anemia to have to regularly consult with a physician in order to monitor the progress of the disease and the quality of the red blood cells in their blood stream.


There are a few types of medications that are regularly prescribed such as antibiotics which are used to prevent bacterial infections as a result of the sickle cell anemia.  In addition, the painful episodes caused by the blockages due to the sickle cells becoming stuck in small blood vessels may require the use of analgesics, including at times stronger, prescription pain killers.  There is also a drug called hydroxyurea (Droxia, Hydrea), which stimulates the production of haemoglobin in order to ensure that there is a sufficient number of healthy blood cells in the blood stream; however, long term usage of this drug is in some studies considered to be detrimental to the health of the patient.


In some cases blood transfusions are also undergone in order to improve the quality of the red blood cells circulating in the blood stream.  There are also a number of other treatments which due to the limitations of this particular article cannot be gone into in much depth.  However, they do include such procedures as bone marrow transplants and genetic therapy.  It is recommended that should you be considering any of the mentioned treatment options for sickle cell anemia that you only do so under the advice of your physician.