What is Sickle Cell Disease?
Sickle cell disease (SCD) is an inherited disorder of hemoglobin, which gives red blood cells their color and carries oxygen to the tissues.
In SCD, the abnormal hemoglobin (Hgb SS) makes the red cells rigid and distorted from the usual round shape into sickle shapes. These stiff, crescent-shaped sickled red blood cells can plug up small blood vessels and decrease blood flow to various parts of the body. This leads to anemia, organ damage and painful episodes, sometimes called a “crisis.”
Sickle cell disease is an inherited genetic disorder, meaning it is passed down in families through information in the cells called genes. People with two sickle cell genes have sickle cell disease. People with one sickle cell gene are carriers and have sickle cell trait. Sickle cell trait is not a type of disease.
Pain is the hallmark of sickle cell disease. Signs and symptoms of SCD may vary in frequency and severity because of the nature of the disease. SCD can affect everywhere blood flows in the body (lungs, liver, kidneys, spleen, heart, brain, intestines, bones, etc.). Symptoms such as painful swelling of the hands and/or feet (called “dactylitis”) can be seen in children as early as 4 months of age.
Children with sickle cell disease need regular visits with their hematologist. The sickle cell clinic team provides disease-specific healthcare and education for the child and their family. This includes topics key to the child’s health, such as hydroxyurea, vaccinations, fever and pain management, stroke prevention, growth and development, and help with school issues. During these visits, self-management topics such as the importance of taking medications as prescribed, avoiding temperature extremes (very cold and very hot weather), getting enough rest and hydration, keeping warm in cold weather or when swimming and tips for eating habits are discussed.
Pain episodes can vary in severity, how often they occur and how long they last. Children often have pain in the arms, legs, chest and back. Individualized pain management plans can help parents manage pain at home. However, sometimes hospitalization is required.
A blood transfusion can help lower the risk for sickle cell-related complications during illness, surgeries, tests and procedures. Some children receive monthly blood transfusions because of other severe complications of sickle cell disease, such as stroke, acute chest syndrome, and/or pain episodes.
Call Your Doctor If:
- Your child develops a temperature of 101 degrees or higher.
- You can feel that your child has rapid swelling of the spleen.
- You notice signs and symptoms of a stroke.
- Your child has difficulty breathing.
- Your child has swelling of hands and/or feet.
Sickle cell disease is a chronic illness requiring lifelong medical care. Regular visits with a pediatric hematologist to treat sickle cell disease, and visits with a primary care physician for routine childhood care, including immunizations, are important.
Caring for a child with sickle cell disease requires education about how the disease works and how to prevent complications. Knowing what to do when a child has painful episodes and recognizing the signs and symptoms of other life-threatening complications is important.
What to Expect
Some children with sickle cell disease may experience serious health issues such as stroke, delayed growth, enlarged spleen, pneumonia and severe pain episodes. However, other children lead relatively normal lives with occasional episodes of pain and few complications. However, the course of sickle cell disease varies from person to person. Hydroxyurea is a medication that may be prescribed to help reduce these complications.
Children with sickle cell disease should set their own limits on physical activities. During organized physical activity at school, the teacher should monitor the child's activities to ensure adequate rest periods and hydration.