This week the ramblings from red shift looks at a disease which used to be a very common occurrence for me in London, often seen once or twice a week, but while not as common here, I do seem to be coming across more patients in crisis than I used to.
Sickle cell disease is a hereditary condition affecting the haemoglobin contained within red blood cells. These red cells are prone to assuming a permanently sickle shape when exposed to a variety of factors including hypoxia, cold or dehydration – referred to a crisis. These cells are prone to mechanical damage, hence haemolytic anaemia which can lead to occlusion of the microvasculature resulting in tissue hypoxia, pain and organ damage.
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These are high risk patients who normally present with normal vital signs but can rapidly deteriorate.
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There are different types of sickle cell disease mainly found in people of African or Afro-Caribbean origin, but these can also affect people of Mediterranean, Middle Eastern and Asian origin.
![](https://www.paramedicine.education/wp-content/uploads/2020/07/sickle-ed-1024x618.jpg)
For further reading please visit the links below, the first describes Perth Children’s Hospital’s Sickle Cell ED pathway while the second includes a podcast with relevant reading.