Sickle cell anemia is a hereditary blood disease that affects the red blood cells. It is one of the most common and usually most severe forms of a group of red blood cell disorders called Sickle Cell disease.
In order to survive, the human body needs red blood cells to supply oxygen to its tissues. Red blood cells are made inside the center of our bones, called bone marrow. Normal red blood cells are disc shaped. Each of them contains protein molecules called Hemoglobin that carry oxygen. Round flexible red blood cells flow easily through our bloodstream and pickup oxygen in our lungs. In tiny air sacs in the lungs called; Alveoli, oxygen moves into the blood and detaches to the hemoglobin, which then deliver oxygen to the cells throughout the body.
In sickle cell anemia, there is a problem with the person’s hemoglobin. This makes the person’s red blood cells sickle shaped instead of round. This is caused by a defective gene that is passed from both parents to the affected child through genetic materials in the cells called DNA. DNA is made up of sections called genes. Two of the genes called; Alpha globin and Beta globin contain the blueprint for making the hemoglobin proteins. In sickle cell anemia, the beta globin gene from both parents has changed or mutated. This mutation leads to the formation of an abnormal hemoglobin protein called Hemoglobin-S. Hemoglobin-s forms into rigid strands inside the red blood cells, which causes the red blood cell to be sickle shaped.
Sickle cells can block blood flow, and can cause pain and tissue damage. While normal red blood cells last 90 – 120 days, sickle cells only last 10-20 days. The person’s body may not be able to keep up with how fast the cells are destroyed; as a result, the person’s blood has fewer red blood cells than normal. This condition is called Anemia. The symptoms of sickle cell anemia vary from person to person, and may include; episodes of pain, jaundice, and fatigue due to anemia. Overtime, sickle cell anemia can lead to complications such as severe pain, and damage to eyes, heart, lungs, liver, kidneys, and joints. Damage to the spleen, an organ that makes immune cells, can increase the person’s risk of infections.