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Brief History Of Sickle Cell Disease

Although the Sickle hemoglobin gene (HbS) is most common in Africa, sickle cell disease went unreported in African medical literature until the 1870s. This may be because the symptoms were similar to those of other tropical diseases in Africa and because blood was not usually examined. In addition, children born with sickle cell disease usually died in infancy and were typically not seen by physicians. Most of the earliest published reports of the disease involved black patients living in the US. African tribal populations were all too familiar with the disease and created their own names for it. Some tribes had as many as 40% of the people carry the sickle cell gene.

1846   In the US a paper entitled "Case of Absence of the Spleen" (from the Southern Journal of Medical Pharmacology), was probably the first to describe sickle cell disease. It discussed the case of a runaway slave who had been executed. His body was autopsied and found to have "the strange phenomenon of a man having lived without a spleen." Although the slave's condition was typical, the doctor had no way of knowing this as the disease had not yet been "discovered."

1904   Dr. James Herrick reported "peculiar elongated and sickle shaped" red blood cells in "an intelligent negro of 20." These sickled cells were discovered by a hospital intern, Dr. Ernest Irons, who examined the patient's blood and sketched the strange cells. The patient had come to Dr. Herrick with complaints of shortness of breath, heart palpitations, abdominal pain, and aches and pains in his muscles. He also felt tired all the time, had headaches, experienced attacks of dizziness, and had ulcers on his legs. After noting these symptoms, the doctor took samples of his blood.

1910   The first description of sickle cell disease was provided in western literature by a Chicago physician, James B. Herrick, who noted that a patient of his from the West Indies had an anemia characterized by unusual red cells that were sickled shaped. This patient had come to Chicago in 1904 to study dentistry in one of the best schools of the country and was likely the only black student there. He was a wealthy man from the West Indies; and, despite repeated hospitalizations for his illness, Walter Clement Noel completed his training, along with his classmates, three years later. He returned to Grenada and practiced dentistry until he died of pneumonia at the age of 32.

1922    After three more cases were reported, the disease was named "sickle cell anemia." One long-held theory as to why it was so common in the tropics was its association with malaria.

1927    Hahn and Gillespie showed that sickling of the red cells was related to low oxygen

1940    E.A.Beet, a British medical officer stationed in Northern Rhodesia (now Zimbabwe), observed that blood from malaria patients who had sickle cell trait had fewer malarial parasites than blood from patients without the trait. Following this observation, a physician in Zaire reported that there were fewer cases of severe malaria among people with sickle cell trait than among those without it.

1948    Using a new technique of protein electrophoresis, Linus Pauling and Harvey Itano showed that the hemoglobin from patients with sickle cell disease is different than that of normals. This made sickle cell disease the first disorder in which an abnormality in a protein was known to be at fault.

1954    Anthony Allison, continued to build on the 1040’s malaria observations of E.A. Beet and hypothesized that sickle cell trait offered protection against malaria. He suggested that those with the trait did not succumb to malaria as often as those without it; but, when they did, their disease was less severe. It is now known that, when invaded by the malarial parasite, normally stable red cells of someone with the sickle cell trait can sickle in a low oxygen environment (like the veins). The sickling process destroys the invading organism and prevents it from spreading through the body.

1956    Vernon Ingram then at the MRC in England, and J.A. Hunt sequenced sickle hemoglobin and showed that a glutamic acid at position 6 was replaced by a valine in sickle cell disease. Using the known information about amino acids and the codons that coded for them, he was able to predict the mutation in sickle cell disease. This made sickle cell disease the first genetic disorder whose molecular basis was known.

1984     Bone marrow transplantation in a child with sickle cell disease produced the first reported cure of the disease. The transplantation was done to treat acute leukemia. The child's sickle cell disease was cured as a side-event. The procedure nonetheless set the precedence for later transplantation efforts directed specifically at sickle cell disease.

1995    Hydroxyurea became the first drug proven to prevent complications of sickle cell disease in the Multicenter Study of Hydroxyurea in Sickle Cell Anemia which was completed in 1995.

Information provided in this section was obtained from UTDallas, Harvard Information Center for Sickle Cell & Thalassemic Disorders and Innvista

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