Sickle cell disease Flashcards

1
Q

how would you manage a sickle cell crisis?

A
oxygen 
hydration (IV fluids)
analgesia (NSAIDS, opiates)
consider antibiotics
blood transfusion 
exchange transfusion if neurological complications
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2
Q

what prophylaxis is given for sickle cell crises?

A

vaccine - pneumococcal every 5yrs
penicillin
folic acid

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3
Q

at what P02 does homozygotes and heterozygotes experience symptoms?

A

homozygotes sickle at P02 2.5-4 kPa

heterozygotes sickle at P02 5-6 kPa

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4
Q

how does hydroxycarbamide work?

A

switches on gamma chains to produce foetal haemoglobin (HgF)

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5
Q

what investigation is carried out to diagnose sickle cell anaemia?

A

haemoglobin electrophoresis

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