“Sickle Cell Disease (SCD) is an inherited, blood disorder that causes individuals to produce abnormal hemoglobin, causing their red blood cells to become rigid and sickle-shaped (SCD Coalition, n.d.).” According to Sickle Cell Disease Association of America (2022), hemoglobin helps red blood cells carry oxygen from the air in our lungs to all parts of the body. Normal red blood cells that contain hemoglobin A are soft and round and can squeeze through the blood vessels; however people living with sickle cell conditions make a different form of hemoglobin A called hemoglobin S (S stands for sickle). Typically, red blood cells live for about 120 days before new ones replace them, but when an individuals red blood cells contain mostly hemoglobin S, they normally last about 16 days. They also become stiff, distorted in shape, and have difficulty passing through the body’s small blood vessels; therefore when the sickle-shaped cells block small blood vessels, less blood reaches that part of the body. Due to the lack of normal blood flow, parts of the body eventually become damaged. This is what causes the complications of sickle cell disease, although some individuals may be eligible for a curative bone marrow transplant (Sickle Cell Disease Association of America, 2022).
An individuals eye color, hair color, blood type, and other physical traits are inherited from their parents in a similar way that a person inherits sickle cell conditions. “The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets, one from each parent (Sickle Cell Disease Association of America, 2022).” According to the American Sickle Cell Anemia Association (2021), individuals who inherit one sickle cell gene and one normal gene have something called sickle cell trait (SCT). When a person has SCT, they usually do not have any of the symptoms of sickle cell disease (SCD), but they can pass the trait on to their children (SCDFC, n.d.)
There is no universal cure for sickle cell disease, however a medication called hydroxyurea has been found to significantly reduce the number of painful crises (Sickle Cell Society, 2021). Similarly, the Sickle Cell Disease Foundation states that with effective treatment and appropriate medical care, survival rates and the quality of life for persons with sickle cell disease has been greatly improved.
Depending on the child’s stage of development, here are some key words you can use when explaining sickle cell disease to children:
• “Sickle cell disease is a disease of the blood. Red blood cells usually look like round discs. But in sickle cell disease, they are shaped like crescent moons, or an old farm tool known as a sickle. A round disc is the healthiest shape for red blood cells because they can move easily through the blood vessels of the body to get oxygen where it needs to go. When red blood cells are shaped like sickles, they can get stuck, especially inside smaller blood vessels. This keeps blood from flowing as it should, which can cause pain and damage to parts of the body.”
• Red blood cells – cells that bring oxygen to all parts of your body
• Blood vessels – the pathways in your body that move your blood from one area to another
• Cells – building blocks or the basic components of your body
According to Sickle Cell Disease Association of America Inc, there are several types of sickle cell disease. The most common are:
• Hemoglobin SS Disease, Hemoglobin SC Disease
• Sickle Beta-Plus Thalassemia
• Sickle Beta-Zero Thalassemia
Sickle Cell Disease is most common among Black or African American individuals in the U.S. However, people with heritage from the following areas may also be affected (ASCAA, 2021):
• Sub-Saharan Africa
• India
• Spanish-Speaking Regions in the Western Hemisphere (South America, the Caribbean, and Central America)
• Mediterranean Countries (Turkey, Greece, and Italy)
According to the Sickle Cell Disease Coalition, symptoms of sickle cell disease range from mild to severe enough to require frequent hospitalizations. The possible signs and symptoms of sickle cell disease include the following:
• Anemia (looking pale)
• Dark urine
• Yellow eyes
• Painful swelling of the hands and feet
• Frequent pain episodes
• Stunted growth
• Stroke
Sickle Cell Disease Facts
• Nearly 100,000 people in the United States have SCD (SCD Coalition, n.d.).
• “According to the Centers for Disease Control and Prevention, around one in every 365 African American babies is born with SCD in the U.S. Furthermore, approximately 300,000 babies are born with SCD each year worldwide. (ASCAA, 2021)”
• Sickle Cell Disease is the most common genetic disease in the world (SCDFC, n.d.)
• Children with SCD are at increased risk for stroke, the risk is highest between the ages of 2 and 16 (Sickle Cell Society, 2021).
• Episodes of pain may occur in SCD and are generally referred to as a “crisis” (Sickle Cell Society, 2021).
• SCD is inherited from both parents; sickle cell trait is inherited from one parent (Sickle Cell Society, 2021).
Resources
- Websites
• https://ascaa.org/
• https://www.sicklecelldisease.org/
• https://www.scdfc.org/
• https://www.sparksicklecellchange.com/
• https://www.sicklecellsociety.org/
• https://www.embracekids.org/
• http://www.scdcoalition.org/ - Books
• Hope & Destiny: The Patient and Parent’s Guide to Sickle Cell Disease and Sickle Cell Trait by Allan F. Platt Jr. P.A., James Eckman M.D., and Lewis Hsu M.D. Ph.D
• My Brother has Sickle Cell by Dr. Erica D. Gamble
• I Only Cry At Night: Living with Sickle Cell Disease by P. Allen Jones
• Living with Sickle Cell Disease: The Struggle to Survive by Judy Gray Johnson and Jr. Leroy Williams
• A Sickle Cell Coloring Book For Kids: A Creative A to Z guide on growing up with Sickle Cell Disease for Children Ages 5-8 With Over 26 Coloring Pages by Elle Cole and Kate Hamernik - Applications
• SCD Companion
• iManageSCD
• Sickle-O-Scope
• SCD Warrior
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References
About sickle cell. Sickle Cell Society. (2021). Retrieved from https://www.sicklecellsociety.org/about-sickle-cell/
About sickle cell disease. Sickle Cell Disease Coalition. (n.d.). Retrieved from http://www.scdcoalition.org/sickle-cell-disease.html
Sickle cell inheritance. Spark Sickle Cell Change. (n.d.). Retrieved from https://www.sparksicklecellchange.com/sickle-cell-genetics/inheritance
Sickle cell disease resources: ASCAA. American Sickle Cell Anemia Association. (2021). Retrieved from https://ascaa.org/sickle-cell-disease-resources/
What is sickle cell disease (SCD)? Sickle Cell Disease Association of America Inc. (2022). Retrieved from https://www.sicklecelldisease.org/sickle-cell-health-and-disease/types/
What is sickle cell disease? Sickle Cell Disease Foundation (SCDFC). (n.d.). Retrieved from https://www.scdfc.org/what-is-sickle-cell-disease