Sickle cell disease

Question 1

how would you manage a sickle cell crisis?

Answer 1

oxygen 
hydration (IV fluids)
analgesia (NSAIDS, opiates)
consider antibiotics
blood transfusion 
exchange transfusion if neurological complications

Question 2

what prophylaxis is given for sickle cell crises?

Answer 2

vaccine - pneumococcal every 5yrs
penicillin
folic acid

Question 3

at what P02 does homozygotes and heterozygotes experience symptoms?

Answer 3

homozygotes sickle at P02 2.5-4 kPa

heterozygotes sickle at P02 5-6 kPa

Question 4

how does hydroxycarbamide work?

Answer 4

switches on gamma chains to produce foetal haemoglobin (HgF)

Question 5

what investigation is carried out to diagnose sickle cell anaemia?

Answer 5

haemoglobin electrophoresis