Sunday June 19th was World Sickle Cell Day to raise awareness about the Bone Marrow Registry and Sickle Cell Disease. Dr. Ann Mellott, a Hematologist and Oncologist with UCHealth Memorial says "Sickle cell disease is the most common inherited blood disease in the United States. Specifically it affects the hemoglobin molecule that carries oxygen to molecules in our body. The normal hemoglobin molecule is shaped like donut, it’s very flexible and can get through blood vessels easily. When someone has sickle cell disease the blood cell is not very flexible and it there is a little crescent shaped sickle cell in the blood that causes clotting of the blood vessels. Sickle Cell results in complications to the brain, heart, liver, spleen kidneys and bones."
The most common symptoms of Sickle Cell Disease in adults, or young adults are fatigue, bone pain, some could experience unexplained shortness of breath, or unexplained chest pain. Dr. Mellott says "Children are affected as early as 3-6 months of age, so this is a disorder that needs to be diagnosed and treated early. Sickle cell is a chronic disease and it's life long so it needs to be managed from infancy forward."
The good news about sickle cell disease is that you don't need to be worried about catching it. Dr. Mellott adds "It's all genetic, it's not inherited so you can't catch it, you can't develop it over time you either have it, or you don't"
The bad news is if you do have Sickle Cell you have a long road ahead. Dr. Mellott says "There are approximately 80,000 - 100,000 individuals in the United States with Sickle Cell disease. Groups most at risk are those of African descent, also Hispanic, Southern European, also Mediterranean, Asian descent. There is one medication used to treat symptoms, it's called hydroxyurea it's a chemotherapy agent. That drug has been shown to increase survival, decrease pain symptoms, and lessen the frequency of pain crisis. The only cure is a bone marrow transplant, and that is typically reserved for young children with severe disease. Unfortunately only 1 in 10 children with Sickle Cell will actually have a donor that is appropriate "
Because you can't develop Sickle Cell, the most important thing to know is if you’re a carrier or not, so you can be aware of the risks of passing this genetic disease onto your offspring. If you have any questions about Sickle Cell disease contact your doctor to discuss them.