100 years of sickle cell
One hundred years ago this month, Dr James Herrick, a Chicago cardiologist, published the first paper identifying sickle cell anaemia. The Wellcome Library Blog has more on the historical side of things, and the resources we hold about the disease.
A genetic diorder causing the red blood cells to develop abnormally, sickle cell anaemia affects thousands of people all over the world. According to the NHS, there are around 12,500 people with the disorder in England.
In people with sickle cell anaemia, the shape and texture of the blood cells can change. They become hard and sticky and are shaped like sickles (crescents). The cells die prematurely, leading to a shortage of red blood cells. This causes symptoms of anaemia, such as tiredness and breathlessness…. A lack of a regular oxygen supply can also result in tissue and organ damage.
Herrick’s paper was the first of many increasing our understanding of the disease. The Trust is doing its part to further this, and reduce the worldwide burden. Just a few weeks ago, researchers from the KEMRI-Wellcome Trust Research Programme in Kenya produced a global map of the disease burden. The results showed that the sickle cell gene is most common in sub-Saharan Africa, the Middle East and India. Moreover, the areas it is most commonly found in tied in with areas of historically high levels of malaria, adding geographical support to the hypothesis that, while potentially deadly, the gene avoids disappearing through natural selection by providing protection against malaria.
Other research from KEMRI has found that while the gene offers some protection, those sickle cell children who do develop malaria are more likely to die of severe malaria.
You can find out more about sickle cell anaemia from this 1998 resource produced by the Wellcome Trust.