Sickle Cell Flashcards
1
Q
Presentation of sickle cell crises
A
Majority diagnosed during antenatal 10wk screening
Painful episodes - blood vessel occlusion
Infections - due to splenic dysfunction (need to breakdown more RBCs)
Anemia - fragility haemolysis
2
Q
Pathophysiology
A
Autosomal recessive => Hbs
Carrier => sickle cell trait
Sickle cells are fragile and haemolyse easily
3
Q
Investigations needed for diagnosis
A
Haemoglobin electrophoresis
4
Q
Management of crises
-initial
A
Initial
- Analgesia - opiates
- AKI => Rehydration
- O2
- ABx if evidence of infection
- osteomyelitis in femur, tibia, humerus - salmonella
- Blood transfusion if needed
Prophylaxis - hydroxyurea => increase HbF
-pneumococcal vaccine
5
Q
Triggers of crises
A
Acidosis Infection Psychological stress Extreme exercise Cold Pregnancy Dehydration Reduced O2
