Causes Of Sickle Cell Disease

Sickle Cell is an inherited blood disorder that affects approximately 100,000 people in the United States. Red blood cells contain hemoglobin which is a protein that carries oxygen through the blood. Normal red blood cells are flexible and round. This allows them to travel through the small blood vessels and deliver oxygen to all of the body. Sickle cell disease(SCD) causes these red blood cells to form into a crescent shape, like a sickle. The sickle-shaped red blood cells easily break apart, causing anemia. Sickled red blood cells only live 10-20 days instead of the normal 120 days. The damaged sickle cells then clump together and stick to the walls of blood vessels, causing a blockage of blood flow. This can cause severe pain and permanent damage to the heart, brain, kidneys, lungs, bones, liver and spleen. Severe pain is called acute sickle cell crisis. Exactly what brings on the pain is undetermined, but infection and dehydration are common triggers (Powars, 2013).
Sickle cell anemia is the most severe and common form of sickle cell disease. Sickle cell anemia results from the inheriting the sickle cell gene from both parents. The most common symptoms are severe episodes of pain and anemia. There are similar manifestations that are seen in other forms of sickle cell disease but they are less frequent. In sickle cell anemia, Anemia is caused by rapid destruction of the red blood cells as they circulate due to a shortened peripheral survival time of less than 30 days (Powars,