Sickle Cell Disease
Sickle cell disease (SCD) is an inherited blood disorder in which red blood cells contain an abnormal form of hemoglobin, the protein in red blood cells that carries oxygen throughout the body. This abnormality leads to anemia. Red blood cells become misshapen so that they look like "sickles" under the microscope. These sickle-shaped cells can clog the blood stream and cause obstructions resulting in serious medical problems. The most common types of abnormal hemoglobins that cause sickle cell disease are: • Sickle Cell Anemia (Hemoglobin SS) • Sickle C Disease (Hemoglobin SC) • Sickle Beta Thalassemia (Hemoglobin SBo or Hemoglobin SB )
How Do You Get Sickle Cell Disease?
To have sickle cell disease, a child must inherit two abnormal hemoglobin genes, one from each parent. They must have one gene that produces hemoglobin S and another that produces either hemoglobin S, hemoglobin C or abnormal beta thalassemia hemoglobin. If the child inherits only one abnormal gene, he or she is said to have sickle cell trait.
Sickle Cell Demographics
Sickle cell disease is the most common genetic disease in the world. Approximately 150,000 Nigerian children are born each year with sickle cell disease making it the country with the largest burden of sickle cell disease in the world.
Sickle Cell Disease Life Expectancy
The life expectancy of persons with sickle cell disease depends on many factors including the severity of their illness and complications. The life expectancy of persons with sickle cell disease is increasing with medical advances. With good management of their disease, people with sickle cell disease can live beyond 40 years of age. Early diagnosis and improved supportive care are expected to decrease death rates in children. Causes of death include: stroke, bacterial infection, and kidney, heart or liver failure.
Sickle Cell Disease Diagnosis
Sickle cell disease is diagnosed by different tests. The simplest test is hemoglobin electrophoresis. People who have sickle cell disease should see their physician at least once a year or more often if problems occur.
Sickle Cell Disease and Stroke
Strokes are frequent complications of SCD, occurring across the lifespan from infancy to old age. Children with SCD have one of the highest rates of ischemic stroke of any group of children, with an incidence of 240 per 100,000 person years. Given the importance of reaching developmental milestones, and the necessity of formal education in childhood, the lifelong adverse impact of strokes in children is tremendous.