History of sickle cell disease

Although the Hbs gene 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 disease 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 report of the disease involved black patient living in U.S.A.

African tribal population
were all too familiar with the disease and created their own names for it. It
is interesting to note that the tribal names all carry repeating syllables,
possibly to symbolize the repeating painful episodes. Such names include,
ahututuo (from the Twi tribe); Chwecheechwe (from the Ga tribe); nuidudui (from
the Ewe tribe) and nwii wii (from the fante tribe). Many tribal names were also
imitations of the cries and moans of the sufferers or formed such phrases as
“body chewing” or “body biting” which described their terrible torment. In one West
African tribe, children who died soon after birth were called “Ogbanjes”
meaning children who come and go. The tribes people believed that an evil
spirit was trying to be born into a family with Ogbanje children, but the
babies bravely died to save the rest of the family from the demon. Some tribes
had as many as 40% of the people carrying the sickle cell gene.
The first sickle cell
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 hospitalization for his
illness, Walter Clement Noel completed his training along with his classmate,
three years later he returned to Grenada and practiced dentistry until he died
of pneumonia at the age of 32. Although the disease does not distinguish
between the rich and the poor, it does single out those from the tropical and
subtropical climate of the old world.
One long held theory as to
why it was so common in the tropics was its association with malaria. In the
1940s, Beet a British Medical Officer stationed in Northan Rhodesia (now
Zimbabwe), observed that blood from malaria patient who had sickle trait had
fewer malaria parasites than blood from the patients without the trait.
Following this observation, a physician in Zaira reported that there were fewer
cases of severe malaria among people with sickle cell trait than among those
without it.
In 1954, Allison continued
to build on these observations and hypothesized that sickle cell trait offered
protection against malaria. He suggested that those with the trait did not
succumb to malaria when they did, the disease was less severe. It is now known
that, when invaded by the malaria 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. This apparent ability of a genetic condition
to protect carriers is particularly important in infants. Those in regions
repeatedly devastated by malaria, people who carry the sickle cell trait will
have a greater chance for survival than other individuals.
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