What’s a “sickle cell”? World Sickle Cell Disease Day

Posted on June 16, 2017 by kerri
Image credit: CDC/Janice Haney Carr
Image credit: CDC/Janice Haney Carr

A sickle is a curved bladed implement often used for harvesting crops. No, farming or gardening doesn’t have anything to do with sickle cell disease, but it does explain where “sickle cells” got their name–based on their shape! If you don’t have sickle cell disease, one type of which is called sickle cell anemia, you won’t have sickle cells in your blood. People with sickle cell disease have differently shaped red blood cells, the type of cells that carry iron, known as hemoglobin S, which makes it harder to transport oxygen around the body. [1]

Sickle cell disease is a genetic disease, which means it is inherited or passed down within families. It is most common in people of a handful of ancestries including those of African, Mediterranian, Indian, Arab, and Spanish descent [1] In the United States, sickle cell disease is the most common inherited blood disorder, and affects 1 in 500 African Americans and 1 in 1,000 to 1,400 Hispanic Americans. 70 to 80 thousand Americans have SCD. [1]
Sickle cell disease can cause pain, swelling of the hand and feet, and jaundice (yellow skin). In SCD, the spleen does not work properly, which can cause a variety of problems including increased risk of infection [2]. Many people with SCD experience chronic pain, especially in adolescence, and may have acute pain crises that differ from chronic pain experienced [2].
Other complications of SCD include blood flow being blocked out of the spleen causing spleen enlargement and causing severe anemia; aplastic crisis (which manifests in red cheeks, known as fifth disease, which is common among children but can be serious for people with SCD); acute chest syndrome (a medical emergency often accompanied by lung infection, often beginning a few days after an acute pain crisis begins), brain complications including stroke or cognitive issues (“silent stroke”), eye problems including retinal detachment, heart, kidney and liver disease, and pulmonary hypertension (high blood pressure in the lungs), leg ulcers, joint problems, and pregnancy complications. [2] While not a direct manifestation of sickle cell disease, people with SCD may experience mental health problems due to the nature of their condition. [2]
Sickle Cell Disease may be treated with antibiotics for the first few years of life, or continuously throughout life to prevent a severe pneumococcus infection. Treatment for chronic pain or management in a pain crisis will be prescribed individually for each patient. [3] Severe anemic episodes may require IV treatment or blood transfusions. [3]
Stem cell transplant may also be used as a treatment for SCD—a donor within the family who does not have SCD is usually the best choice. This is the only way Sickle Cell Disease may be cured, however, other medicines will have to be taken to ensure success of the stem cell transplant. [3]
Living with sickle cell disease can be unpredictable. For this reason, a Sickle Cell Disease medical ID bracelet is important. If a person has had a stroke from SCD, or has other complications, this should also be listed on a medical ID bracelet. As well, stem cell transplant recipient medical ID is an important item for anyone who has undergone this procedure.

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