Sickle-cell anemia comes in a couple of forms, but in general SC disease is more trait-like, and SS disease is more severe, with pain crises, strokes at young ages, autoinfarction of the spleen, and so on. So, typically we're more careful with SS patients than with others.
A 13 year old girl with SS comes in with a sore throat for about six hours and feeling a bit tired. She has friends who have strep. Her exam isn't scary; she does have a fever, and a bit of a fast heart rate (116), but otherwise, her throat isn't bad, she's not in respiratory distress, she's appropriately alert. This is not like a normal crisis. She's having absolutely no chest pain or shortness of breath. Her neuro exam is normal. By protocol we sent off CBC, reticulocyte count, and blood cultures, but only by protocol.
Her anemia was so profound that her count of new blood cells being created in order to make up for her losses was almost higher than her blood count. Her hematocrit was 18 (normal is above 35) and her reticulocyte count was 17 (normal is, like 2). Oh, and her O2 saturation was 80.
We only got an oxygen saturation because she had sickle cell disease.
This kid scared me good. Respect the SS disease. She looked fine! No respiratory distress! Yes, she had a murmur--but a hematocrit of 18?!? And a pulse ox of 80%?!?
Oh, by the way, the strep was negative.
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