History of Sickle Cell Anemia

Sickle cell disease, or sickle cell anemia, is one of a class of intrinsic hemolytic anemia diseases. Essentially that means that the blood cells undergo hemolysis, or destruction, before the end of their normal lifespan because of an intrinsic, or internal, disorder or cause. In the case of individuals who suffer from sickle cell anemia it is because the cells develop in a sickle cell shape which makes them inefficient oxygen carriers and more apt to be caught in the smaller capillaries where they die and cause occlusive painful crises.

Sickle cell disease has been known to the people of Africa for hundreds of years. The disease went unreported in the African medical literature until the 1870s, probably because the symptoms were similar to other tropical diseases and because the blood of individuals who suffered were not usually examined. Infants who were born with sickle cell disease often died before the age of one and were typically not seen by doctors.

Many of the different African tribes were familiar with the disease and created their own names for it. These names included ahututuo, chwecheechwe, nuidudui and nwiiwii. Some of these names were imitations of the cries and the moans of the people who suffered from the pain crises. Some tribes had as many as 40% of the people carry a sickle cell gene.

The earliest paper published in the US was in 1846 entitled \”Case of Absence of the Spleen\” in the Southern Journal of Medical Pharmacology. The paper described an autopsy of a runaway slave who appeared to have lived without a spleen. Doctors had no way of knowing that this condition was typical because the disease had not yet been discovered.

It wasn\’t until 50 years later, in 1910, that the first report came out in Chicago by Dr. James B. Herrick who noted the unusual red cell shape in a patient from the West Indies. In 1922 three more cases were reported and the disease was subsequently named sickle cell anemia.

In 1917 the sickling phenomenon of the red blood cells in petri dishes was noted in more than one member of a family which suggested that there was a genetic basis for the disease. In 1927 doctors Hahn and Gillespie discovered that the sickling of the red blood cells was related to low amounts of oxygen.

In 1940 the research into the way in which low oxygen affected the sickling of the red blood cells reached a new level. Researchers from John Hopkins Medical School discovered that the low oxygen altered the structure of the hemoglobin in the red blood cell.

Research continued over the next decades which revealed that the hemoglobin of those who have sickle cell is different from those who have normal cells; that the genetic coding for the red blood cells was different at position 6 for those who have sickle cell anemia; and then in 1984 researchers discovered that a bone marrow transplant would produce a cure. The original transplantation was done to treat acute lukemia and the sickle cell cure was a secondary event.

In 1995 researchers brought out Hydroxyurea which became the first drug that was proven to actually prevent complications of the disease.

The history of sickle cell anemia spans hundreds of years and millions of dollars in research. Only now, with advanced technology, are doctors and researchers beginning to piece together the puzzle and offer sufferers better pain control, prevention and treatment protocols.

RESOURCES

The Journal of Clinical Investigation: Sickle Cell Disease: Old discoveries, New Concepts, and Future Promises

Harvard University: Sickle Cell Disease in African Tradition

Howard University: A Brief History of Sickle Cell Disease