A Brief History of Sickle Cell Disease
“Peculiar elongated and sickle-shaped” is how sickle cells were first described in 1904 by intern Ernest Edward Irons when examining the blood of Walter Clement Noel, a 20-year-old first-year dental student from a wealthy Black family in Grenada. Noel had been admitted to the Chicago Presbyterian Hospital suffering from anemia and was readmitted several times over the next three years before completing his studies and returning to Grenada to successfully practice dentistry. He died of pneumonia in 1916.
Iron’s supervising physician, James B. Herrick, wrote a paper published in 1910 in the Archives of Internal Medicine documenting the first known case of sickle cell disease in the United States.
Although this was the first known US case, elements of the disease had been recognized earlier: African medical literature reported this condition in the 1870s, when it was known in some areas as ”ogbanjes” (“children who come and go”) because of the very high infant mortality rate caused by this condition. There are reports of the condition tracking back to 1670 in one Ghanaian family.
While sickle cell disease primarily affects persons of African descent, it is also present in Portuguese, Spanish, French Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks and Cypriots. Sickle cell disease also appears in Middle Eastern countries and Asia.
Linus Pauling and colleagues were the first, in 1949, to demonstrate that sickle cell disease occurs as a result of an abnormality in the red blood cell. This historical finding was the first time a genetic disease was linked to a mutation of a specific protein.
The origin of the mutation that led to the sickle-cell gene derives from at least four independent mutational events, three in Africa and a fourth in either Saudi Arabia or central India. These independent events occurred between 3,000 and 6,000 generations ago, approximately 70-150,000 years.
A significant figure in Sickle Cell history is Dr. Charles F. Whitten. He was a trailblazer in medical education and sickle cell disease awareness. During the early 1970s, he realized that services for people with sickle cell disease and their families were sorely lacking. Out of his pioneering efforts to advocate for the needs of people with sickle cell disease, the Sickle Cell Disease Association of America (SCDAA) was born.
In 1960 Dr. Whitten started a fund to help minority medical students with financial and academic assistance. He wrote many educational materials and was the recipient of many honors and awards. His papers are archived in the National Institutes of Health Library of Medicine. He was received in the Oval office by three sitting U.S. presidents.
Dr. Whitten died in 2008. He is survived by his wife, Eloise (Culmer) Whitten; his daughters Lisa Whitten, PhD and Wanda Whitten Shurney, MD.
Despite these and many other advances that have greatly improved and helped lengthen the lives of those with sickle cell, there is still no universal cure. In the United States it is estimated that over 80,000 people have sickle cell disease. Over 1,000 babies are born with the disease each year in America. We have hope for a universal cure through the bone marrow transplant which has been successful with a few patients, including our first teen spokesperson. Bone marrow transplants are not yet widely available due to the risk.
With your help. We can make history and change that.
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