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Sickle Cell Disease - Coggle Diagram
Sickle Cell Disease
Management
Immunisations (pneumo, Men, Hib)
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avoid exposure to cold, dehydration, excess exercise, stress, hypoxia
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For vaso-occlusive crises give analgesia (sometimes opiates requireed), fluids, +/- antibiotics +/- O2 if needed
exchange transfusion for acute chest syndrome, stoke, priapism
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Clinical features
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vaso-occlusive crises
hand foot syndrome: dactylitis (swelling), pain
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can be precipitated by cold, dehydration, excess exercise, hypoxia, infection, stress
acute anaemia
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causes
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sequestration crisis (sudden spleen/liver enlargement, abdo pain, due to accumulation of sickled cells in spleen)
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splenomegaly
common in young children, less frequent in older children
Intro
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common in afrocaribbeans, middle east + N Europeans
Types
Sickle cell anaemia
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small amounts of HbF, no HbA
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HbSC disease
HbS from 1 parent, HbC from other (different point mutation)
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Sickle B-thalassaemia
HbS from 1 parent, B-thalassaemia from other
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Sickle trait
HbS from 1 parent, normal B-globin from other
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no risk with GA, just avoid hypoxia
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