Sickle- cell disease or sickle-cell anemia is a blood disorder characterized by red blood cells that assume as abnormal, rigid, sickle shape. Sicking decrease the cells’ flexibility and results in their restricted movement through blood vessels depriving downstream tissues of oxygen. The disease is chronic and lifelong; individuals are most often well, but the lives are punctuated by periodic painful attacks and a risk of various other complications. Life expectancy is shortened, with older studies reporting an average life expectancy of 42 and 48 years for males and females respectively
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 earlier published reports of the disease involved black patients living in the US.
Africa tribal populations 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 Nwiiwii (from the Fante tribe).Many tribe names were also imitation of the cries and moans of the sufferers and exemplified in such phrases as “body chewing ‘” body biting”, describing the terrible torment. In one West African tribe (the Igbo’s of Nigeria), children who die soon after birth were called Ogbanje meaning children who come and go. The tribe believed that an evil spirit was trying to be born into their family and the babies bravely died to save the rest of the family from the demon. Some tribes have as many as 40% of the people carry the sickle cell gene. Among the Yoruba’s, such children are called Abikus, that is children who keep’ coming and going in order to torment their parents.
In the US in the 1846, 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”. The first formal report of sickle cell disease came out of Chicago about 50 years later, in 1922, after three more cases were reported, the disease was named” sickle cell anemia”. In 1904, Dr. James Herrick reported “peculiar elongation and sickle shaped “red blood cells in “an intelligent Negro of 20”. These sickle 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 pain 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 sample of his blood and found the red blood cells to be sickled.
By Loveth Ezeugwu