Sickle Cell

Question 1

Presentation of sickle cell crises

Answer 1

Majority diagnosed during antenatal 10wk screening

Painful episodes - blood vessel occlusion

Infections - due to splenic dysfunction (need to breakdown more RBCs)

Anemia - fragility haemolysis

Question 2

Pathophysiology

Answer 2

Autosomal recessive => Hbs
Carrier => sickle cell trait
Sickle cells are fragile and haemolyse easily

Question 3

Investigations needed for diagnosis

Answer 3

Haemoglobin electrophoresis

Question 4

Management of crises

-initial

Answer 4

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

Question 5

Triggers of crises

Answer 5

Acidosis
Infection
Psychological stress
Extreme exercise
Cold
Pregnancy
Dehydration
Reduced O2