WHAT IS SICKLE CELL ANEMIA?
Sickle cell disease (sometimes referred to as sickle cell anemia) is an inherited blood disorder that affects hemoglobin, the molecule in red blood cells that deliver oxygen to cells in the body. 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.
When sickle-shaped red blood cells cluster together they become unable to pass through small blood vessels and prevent the flow of oxygen-carrying blood. This can cause a sickle cell crisis – an episode of severe pain, infection, and even permanent damage to body tissues and organs such as the brain, heart, bone, lungs, kidneys, liver, and spleen. The symptoms, frequency, and severity of sickle cell vary from person to person. Likewise, the treatment for a sickle cell crisis varies. Unfortunately, there is no widely available cure and there is only one drug with FDA approval for the treatment of adults with sickle cell disease. However, early diagnosis and daily prescriptions for prophylactic penicillin; blood and marrow stem cell transplants; oxygen therapy; pain medication; and medicines such as hydroxyurea are helping to prevent painful crises and reduce the mortality rate.
There are different types of sickle cell disease. The most common types of sickle cell disease are:
Hemoglobin SS: The sickle cell hemoglobin S gene is inherited from both parents.
Hemoglobin SC: The sickle cell hemoglobin S gene is inherited from one parent and another type of hemoglobin C is inherited from the other parent.
Hemoglobin Sickle Beta Thalassemia: The sickle cell hemoglobin S is inherited from one parent and another anemia, beta thalassemia gene, is inherited from the other parent.
Sickle Cell Trait.
Sickle cell trait is a condition in which there is one normal hemoglobin and one sickle hemoglobin. An individual with the sickle cell trait does not have the disease but can pass the sickle cell gene on to their children.
MYTHS AND FACTS
Myth: Sickle Cell Anemia only affects African Americans.
Truth: Sickle Cell Anemia affects millions of people throughout the world and is most common in people whose families from Africa, South or Central America, Caribbean islands, Mediterranean countries such as Turkey, Greece, and Italy, India and Saudi Arabia.
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. A person who inherits only one sickle cell gene and a normal gene from the other parent will inherit the sickle cell trait, but not the sickle cell disease.
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. However, 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. Tests can also be performed to determined if the baby will have sickle cell disease, sickle cell trait, or neither one.
Sickle cell anemia affects 70,000–100,000 people in the U.S., mainly African Americans.
The disease occurs in about 1 out of every 500 African American births.
The disease occurs in more than 1 out of every 36,000 Hispanic American births.
More than 2 million Americans have the sickle cell trait.
About 1 in 12 African Americans have the sickle cell trait.
Resource: National Heart, Lung and Blood Institute, National Institutes of Health