Sickle Cell Disease: What is it?

Sickle cell disease, or SCD,  is a term that includes a number of different diseases related to the red blood cells. More specifically, the protein called hemoglobin that makes our blood red and carries oxygen around our bodies. People with a sickle cell disease have a gene that causes them to have crescent (sickle) shaped red blood cells, something that makes it hard for your blood to flow properly. In America, 1 in 13 African Americans carries a sickle cell trait, and 1 in 365 African Americans have a Sickle Cell Disease. This has led to stigma surrounding the pain that these patients experience, especially because it was previously incorrectly taught to doctors that African American patients felt less pain than their white counterparts. Patients in crises are often seen as “drug-seeking” because of racial prejudice, despite the very real pain they are in.

The type of SCD someone has depends on what genes they inherit from their parents. The most common type, and most severe, is Hemoglobin SS, also known as sickle cell anemia. It happens when someone inherits an abnormal hemoglobin trait called “Hemoglobin S” from both parents, so they have two sets of those genes. Sickle cell anemia causes “vaso-occlusive crises” that consist of sudden, sharp pain that is usually in the abdomen, lower back, arms and legs. It can also cause “acute chest syndrome” which is a common cause of death involving blood vessel blockage in the lungs. There are a number of other complications it can cause.

Other types of SCD include Hemoglobin SB 0 Thalassemia, which is also a form of sickle cell anemia. Hemoglobin SC Disease and Hemoglobin SB+ (Beta) Thalassemia are less severe forms of sickle cell anemia.

Sickle cell diseases usually become visible around 5 months of age. In the United States of America, 1 in 13 African Americans carry a sickle cell trait, and 1 in 365 African Americans have a Sickle Cell Disease. This has led to stigma surrounding the pain that these patients experience, especially because it was previously incorrectly taught to doctors that African American patients felt less pain than their white counterparts. Patients in crises are often seen as “drug-seeking” because of racial prejudice, despite the very real pain they are in. It is important to be educated on the symptoms and treatment for these patients in order for them to have their pain properly alleviated and complications avoided.

Written By Will Hazan

Sources:

• What is sickle cell disease? (2022, August 18). Centers for Disease Control and Prevention. https://www.cdc.gov/ncbddd/sicklecell/facts.html

• Types of sickle cell. (2020, January 30). Sickle Cell Speaks. https://sicklecellspeaks.com/understanding-sickle-cell/types-of-sickle-cell/#:~:text=Hemoglobin%20SS%20disease%20is%20the,called%20%E2%80%9Csickle%20cell%20anemia.%E2%80%9D

• Symptoms. (n.d.). NHLBI, NIH. https://www.nhlbi.nih.gov/health/sickle-cell-disease/symptoms

• Sickle cell anemia: Symptoms, what it is, causes & treatment. (n.d.). Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia#symptoms-and-causes