World Sickle Cell Awareness Day

Post

What is Sickle Cell Anemia?

Sickle Cell Anemia is an inherited blood cell disorder that affects the cell’s ability to carry oxygen throughout the body. Normal red blood cells are flexible and round, their shapes allowing them to easily maneuver through blood vessels and carry oxygen. However, like its namesake, sickle cells are shaped like crescent moons or sickles. This unusual shape affects the hemoglobin protein structure in these cells, in turn reducing how much oxygen can be effectively transported throughout the body. These cells are sticky and rigid which causes them to get caught in smaller blood vessels, slow down, or completely block blood flow, thus creating a lack of oxygen being transported to certain tissues or organs.

Heredity of the Sickle Cell Gene

This disease can only be inherited, which means, both parents must pass on the defective gene. If it’s only passed on from one parent, the child will be a carrier of the trait, but won’t show any symptoms. They can however pass it on to their own offspring. To determine the likeliness of a newborn having this disease

Brief History of Sickle Cell Anemia

It has been present for at least 5000 years and has gone by different names. It was formally discovered in 1910 when a man named Walter Clement Noel went to Dr. James B. Herrick complaining about episodes of pain and symptoms of anemia. Herrick was not interested in this case and assigned Dr. Ernest Irons, a resident, to it instead. After further research, Irons found that Mr. Noel’s red blood cells were in the shape of sickles. Then, through extensive research in 1927, Hahn and Gillespie found that the sickling of red blood cells was due to deoxygenation. In 1951, Nobel Prize-winning chemist Dr. Linus Pauling and his colleagues discovered hemoglobins and their different chemical structures when present in people with SCD.

Symptoms of Sickle Cell Anemia

Signs and symptoms of sickle cell anemia usually appear around 5 months of age, although they can vary from person to person and change over time. Some signs of this disease can include fatigue, episodes of pain in various areas of the body, low immunity causing constant infections, severe anemia, and late development or puberty. These symptoms can be related to shortness of oxygen and other common problems sickle cells are known to cause. A simple blood test is usually taken near after birth to check for the sickled form of hemoglobins among other possible diseases.

Treatments of Sickle Cell Anemia

There aren’t any known permanent treatments for sickle cells anemia as it is still considered an incurable disease at this point in biomedical engineering. An individual with SCD may take medications to temporarily relieve symptoms of pain and they may get blood transfusions frequently to make up for the loss of healthy blood cells. Stem cell transplants may cure the disease but are very risky and uncommon. Common drugs used for treatment are Adakveo, which is known for reducing pain, and Hydroxyurea, an anti-cancer, used to treat melanoma, myelocytic leukemia and such, that can be used to reduce the frequency of pain and minimize the need for blood transfusions and hospitalizations.

Sources:

• A. (2014, December 04). Monohybrids and the Punnett Square Guinea Pigs. Retrieved December 13, 2020, from https://youtu.be/i-0rSv6oxSY

• A Brief History of Sickle Cell Disease. (n.d.). Retrieved December 13, 2020, from http://www.sicklecell.howard.edu/ABriefHistoryofSickleCellDisease.htm

• Moyer, M.D., N. (2019, March 08). How Sickle Cell Anemia Affects Life Expectancy. Retrieved December 13, 2020, from https://www.healthline.com/health/sickle-cell-prognosis

• Shiel Jr. M.D., W. C. (2020, January 10). 21 Sickle Cell Anemia Symptoms, Genetics, Treatment, Life Expectancy. Retrieved December 13, 2020, from https://www.medicinenet.com/sickle_cell/article.htm

• Sickle cell anemia. (2020, January 30). Retrieved December 13, 2020, from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882

• Sickle cell anemia. (2020, January 30). Retrieved December 13, 2020, from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

---

Published June 19, 2021

Written by Fiona Xu ~ Graphics created by Tanya Gu