Sickle cell disease is an inherited blood disorder that causes red blood cells, which are normally round and flexible, to become stiff and crescent shaped. As a result, these blood cells are unable to travel through the blood vessels to deliver oxygen to the body. They become stuck, causing pain and organ damage (brain, spleen, kidney, heart, liver and bones) and increasing susceptibility to infection.
In addition, sickled cells live for only about ten to 20 days, rather than 120 days as normal red blood cells do. Because the body has trouble replacing these cells fast enough, a shortage of red blood cells, or anemia, may result.
How does a child get sickle cell?
Sickle cell diseases are inherited from both parents. A baby inherits two genes for hemoglobin, one from the mother and one from the father. If a baby inherits genes for hemoglobin S from both parents, he’ll have sickle cell anemia, also known as hemoglobin SS disease.
A baby who inherits one hemoglobin S gene and one normal hemoglobin A gene won’t have sickle cell anemia, but he will be a carrier which means that he could pass the trait to his children. This condition is called sickle cell trait.
Some children inherit one hemoglobin S gene and one for another type of abnormal hemoglobin. This results in a different form of sickle cell disease, such as S-beta thalassemia or hemoglobin SC disease.
It is commonest among those of African descent and African Americans. It is also found in other ethnic and racial groups including people from South and Central America, the Caribbean, Mediterranean countries and India.
Sever pain is an emergency called acute sickle cell crisis, infections, stress dehydration and malaria are the common triggers.
Symptoms of crisis
Chest pain and difficulty breathing
Joint pain and arthritis and bone infractions
Blockage of blood flow in the spleen or liver