Sickle cell disease is a group of hereditary blood disorders that affects hemoglobin, a protein in red blood cells that carries oxygen throughout the body. The abnormal hemoglobin causes red blood cells to have a crescent or “sickle” shape. These sickle cells are rigid and sticky, causing them to get stuck in blood vessels and block blood flow. This can cause a wide range of symptoms and complications including organ damage and severe pain. Sickle cells also die faster than normal red blood cells, often leading to a low level of red blood cells in the body (anemia).
The types of sickle cell disease are based on the specific genes a baby inherits. The three most common forms of sickle cell disease are:
Sickle cell anemia is the most severe form of sickle cell disease. HbSC and HbS beta thalassemia are often milder, though some types of HbS beta thalassemia may also be severe. There are also several, rarer types of sickle cell disease.
Sickle cell disease is usually diagnosed at birth during routine screening. The severity of symptoms can vary greatly depending on the type of sickle cell disease your child has.
The most common symptoms of sickle cell disease are anemia and pain crisis. Pain crisis is caused by sickle cells that get stuck in blood vessels and block blood flow. Pain can range in severity and duration.
Sickle cell disease can affect all major organs and cause a wide range of complications including:
Sickle cell disease is caused by a genetic mutation in hemoglobin, the main component in red blood cells. It occurs when a child inherits two sets of genes for abnormal hemoglobin. The type of sickle cell disease a child has depends on which sets of gene variants are inherited.
There are several treatment options to manage symptoms of sickle cell disease and prevent complications. Treatment will depend on the type of sickle cell disease and the severity of your child’s symptoms. Your care team at Arkansas Children’s is experienced in treating all types of sickle cell disease, and we’ll work with you to create the best treatment plan for your child’s specific type of sickle cell disease and symptoms.
In some cases, a bone marrow transplant (also called a stem cell transplant) may be used to cure sickle cell disease. This procedure uses healthy stem cells from your child or a compatible donor, usually a sibling. A bone marrow transplant is currently the only known cure for sickle cell disease.