Sickle Cell Disease (SCD) affects millions of adults and children around the world and today June 19th is World Sickle Cell Awareness Day, a great time to increase public knowledge and raise awareness about this potentially fatal disease.
Sickle Cell Disease is an inherited life-long disease that can run in families. The diease was first described in 1910, in a dental student who presented with pulmonary symptoms. Herrick coined the term “sickle-shaped” to describe the peculiar appearance of the red blood cell of this patient.
However, given the patient’s symptoms, he was not sure at the time whether the blood condition was a disease. In the next 15 years, several similar cases were described, supporting the idea that this was a new disease entity and providing enough evidence for a preliminary clinical and pathological description.
Sickle Cell Disease is one of the main causes of premature death in children under five in various African countries. SCD causes red blood cells to change their shape from the usual donut shape to a C-shape, like the farm tool called a “sickle.” The sickle cells get caught on the walls of tiny blood vessels, stick to one another and can’t squeeze through the blood vessel. This can form clumps inside blood vessels, blocking the flow of blood, which causes severe pain and other serious problems.
People with SCD are more at risk for harmful infections, so getting immunizations on time is important. Common illnesses, like the flu, can be dangerous for children with SCD and Pneumonia is a leading cause of death in infants and young children with sickle cell disease. The disorder produces abnormal crescent or sickle-shaped red blood cells that break down prematurely and can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of the red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice.
Pain crisis or episodes remain one of the most common complications of sickle cell disease. The severity and type of pain varies from person to person. Other complications can include stroke, acute chest syndrome, and infection.
Myths and Facts
Myth: You can “catch” Sickle Cell Anemia from someone who has it.
Truth: No, a person cannot “catch” Sickle Cell Anemia from another person. Sickle Cell Anemia is a genetic condition that is inherited when a person receives two sickle cell genes – one from each parent. A person can also inherit a sickle cell gene from one parent and a different kind of abnormal gene from the other parent and inherit a different form of sickle cell disease, such as hemoglobin SC disease or hemoglobin S beta thalassemia.
Myth: People with Sickle Cell Anemia can’t have a normal, productive life.
Truth: Yes, they can. They can have families, work, and enjoy hobbies and recreational activities just like everyone else. However, like all people with chronic diseases, individuals with sickle cell can best manage their health with a comprehensive, multi-disciplinary program of care and a strong, extended support system. Treatments are improving and people with Sickle Cell Anemia are living longer and more productive lives.
Myth: Women with Sickle Cell Anemia cannot have a healthy pregnancy.
Truth: Yes, they can. Women with Sickle Cell Anemia are more likely to have complications during pregnancy that can affect their health and the health of their unborn baby. During pregnancy, the disease can become more severe and pain episodes can occur more frequently. Complications can include among others, miscarriage, preterm labor, and a low birth-weight baby. However, with early and regular prenatal care and careful monitoring, women with sickle cell disease can have a healthy pregnancy.
Good quality medical care from doctors and nurses who know a lot about sickle cell disease can help prevent serious problems. If you were not screened at birth, talk with your health care provider. A simple blood screening can reveal if you have the trait. There are many online resources help you learn about SCD and learn habits to stay healthy.
With the help of scientists, researchers, and doctors, people with sickle cell anemia are living significantly longer and more productive lives than ever before. With a healthy diet and lifestyle, knowledge about factors that cause crises, and sufficient coping mechanisms, many sickle cell patients can reduce or prevent many of the complications of sickle cell disease.