National Sickle Cell Disease Awareness Month
September is National Sickle Cell Awareness Month. Because it is “back to school” month for most children, the Sickle Cell Disease Association of America wants the public to reflect on the children and the adults whose lives, education and careers have been affected by this disease. The observance originated in 1975 when the Association and its Member Organizations began conducting month long events to call attention to sickle cell disease and the need to address the problem at national and local levels.
The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. “Inherited” means that the disease is passed by genes from parents to their children. SCD is not contagious. People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person’s body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD.
In the United States, approximately 100,000 people have sickle cell disease, and 2 million people have sickle cell trait. Sickle cell disease is thought to have developed in areas of the world where malaria is present, because sickle cell trait provides some protection from malaria. Sickle cell affects people of many ethnicities, including those of African, Middle Eastern, Latino, Asian, Indian, and Mediterranean decent. However, it disproportionately affects African-Americans. Twenty-five years ago, a person with sickle cell disease was not expected to live to adulthood, and the average life span was 21 years. Today, the outlook is much more optimistic, and many people are living beyond age 50.
The most common symptoms are pain and problems from anemia. Anemia can make you feel tired or weak. In addition, you might have shortness of breath, dizziness, headaches, or coldness in the hands and feet. A blood test can show if you have the trait or anemia. Most states test newborn babies as part of their newborn screening programs. Sickle-shaped red blood cells can be seen when a blood sample is examined under a microscope. But sickle cell disease is diagnosed by a blood test called hemoglobin electrophoresis, which measures the amount of the abnormal sickle hemoglobin. The amount of sickle hemoglobin determines whether the person is a carrier (sickle cell trait) or has sickle cell disease.
Sickle cell anemia has no widely available cure. Treatments can help relieve symptoms and lessen complications. Researchers are investigating new treatments such as blood and marrow stem cell transplants, gene therapy, and new medicines. Scientists are making progress in the development of new medications that prevent sickling of red blood cells and improve blood flow and delivery of oxygen to the body. Some of these new treatments are already being tested in patients with sickle cell disease.