Sickle-cell disease

Sickle-cell disease is a genetic disorder in which red blood cells may change shape under certain circumstances. This causes the cells to become stuck in capillaries which deprives the downstream tissues of oxygen and causes ischemia and infarction. The disease usually occurs in periodic painful attacks, eventually leading to damage of internal organs, stroke, or anemia, and usually resulting in decreased lifespan. It is common in people from countries with a high incidence of malaria, and especially in West Africa, or in descendents from those people.

History

The cause of this collection of clinical findings was unknown until the description of the sickle cells in 1910 by the Chicago cardiologist and professor of medicine James B. Herrick (1861-1954) whose intern Ernest Edward Irons (1877-1959) found "peculiar elongated and sickle shaped" cells in the blood of Walter Clement Noel, a 20 year old first year dental student from Grenada after Noel was admitted to the Presbyterian Hospital in December 1904 suffering from anemia. Noel was readmitted several times over the next three years for "muscular rheumatism" and "bilious attacks" while an undergraduate. Noel completed his studies and returned to capital of Grenada (St. George's) to practice dentistry. He died of pneumonia in 1916 and is buried in the Catholic cemetery at Sauteurs in the north of Grenada.

Related Topics:
1910 - Chicago - James B. Herrick - 1861 - 1954 - Ernest Edward Irons - 1877 - 1959 - Blood - Walter Clement Noel - Grenada - Presbyterian Hospital - 1904 - Anemia - Pneumonia - 1916 - Sauteurs

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The disease is very occasionally called "Herrick's syndrome" for this reason.

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The disease was named "sickle cell anemia" by Vernon Mason in 1922. In retrospect some elements of the disease had been recognized earlier: a paper in the Southern Journal of Medical Pharmacology in 1846 described the absence of a spleen in the autopsy of a runaway slave. The African medical literature reported this condition in the 1870's where it was known locally as ogbanjes ('children who come and go') because of the very high infant mortality in this condition. Also, the practice of using tar soap to cover blemishes caused by sickle cell sores was prevalent in the African American community.

Related Topics:
Vernon Mason - 1922 - Autopsy

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