Sickle Cell Flashcards
Pathophysiology behind sickle cell disease
Genetically inherited from gene HbA From both parents
Occurs due to decreases O2 tension and decreases 02 from infection disrupting haemolysis
These are distorted and take on sickle shape by release of O2 causing molecules to stick together and form long chains which distort the cell causing it to bend out of shape
When return to lungs become reoxygenated and regains normal shape but cyclic alteration in shape of molecules damages Hb and erythrocytes
Cells are rigid and fail to love through circulation properly blocking blood flow to tissue resulting in hypoxia and pain
RBCs can catch eachother bc of their shape and clump together further blocking boos flow
Shape abnormality reduces lifespan from 120 days to between 17-20 days
Low blood Ph can occur from result of reduced clearance of CO2 by lungs
Pathophysiology of sickle cell anaemia
Person inherits HBs gene fro each parent
Condition effects beta globulins on chromosome 11
Causes abnormality in formation and quantity of HB caused by error in amino acid sequence
Characterised by slow movement of RBCs and exacerbated when O2 levels reduced
Cause vascular obstruction especially in spleen and bone
Jaundice is a symptom bc of breakdown of RBCs
Signs and symptoms of sickle cell disease (13)
Chronic anaemia (HB 70-100) Metabolic acidosis Unpredictable pain sickle cell crisis Jaundice Capillary stasis Increased blood viscosity Occlusion of blood vessels Embolism Ischaemia necrosis of tissues Risk of ischaemia heart disease Fever Sudden death Chest/abdo/joint/back pain
Triggers for sickle cell (6)
Tiredness Stress Infections Dehydration Sudden changes in body temp Some types of drugs eg anti malarial
What would your pre conceptual and antenatal labour and PN care be for someone with sickle cell?
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