Sickle Cell Disease

November 12, 2012

Sickle Cell Disease

Sickly cell disease is a serious condition in which the body makes red blood cells, which are in the shape of a crescent (or sickle) instead of their normal circular or disk shape. Healthy red blood cells are able to move easily through blood vessels and contain haemoglobin, which is a protein rich in iron. In sickle cell disease this haemoglobin is abnormal and causes the cells to develop into the sickle shape also making them stiff and sticky. Sickle sells often block the blood vessels preventing blood from reaching the limbs and organs. This leads to organ damage, infection and other symptoms. The most common kind of sickle cell disease is called sickle cell anemia. Anemia occurs when you don’t have enough red blood cells or your red blood cells don’t contain enough haemoglobin. Red blood cells are essential for carrying oxygen around our bodies and removing carbon dioxide. The soft tissue inside our bones in known as bone marrow and this is where red blood cells are produced. As healthy red blood cells only have a life expectancy of 120 days, new red blood cells are always being produced. However, sickle cells only live for around 10-20 days before dying off, causing a low red blood cell count because the body cannot produce new ones quickly enough to replace them.

The sickle cell anemia form of sickle cell disease is inherited. Two genes have to be passed down, one from each parent and the disease is life-long. Those who inherit only one sickle haemoglobin gene and one normal gene will be born with a condition known as sickle cell trait. These people do not have the disease, they only have the genes that causes it meaning that they can pass it down to their children and if the gene is also passed on by the other parent then the child will be born with sickle cell anemia.

There is not yet a cure for sickle cell anemia, although there are many treatments available to control and manage the symptoms of this sickle cell disease. In some cases a blood and bone marrow stem cell transplant may be a possible cure. Sickle cell anemia has been very thoroughly researched over the years, and doctors now have a good understanding of how I can affect the body, likely complications and how best to treat it. The symptoms and the way in which sickle cell disease affects each person varies from case to case. Some suffer from chronic pain or tiredness, while others live a fairly normal life with much of it spent in good health. With the continued development of new treatments and research into cures, people who suffer from sickle cell anemia are now living into their 50’s or older and this age will continue to rise.

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