Haematology 6 - Sickle Cell Anaemia

Question 1

sickle cell disease

Answer 1

should be picked up on 5 day heel prick test

HbS

Question 2

7 complications of sickle cell disease

Answer 2

stroke 
avascular necrosis of joints
pulmonary hypertension
priaprism
chronic kidney disease
crises
acute chest

Question 3

management of a sickle cell crisis

Answer 3

supportive only

keep warm + hydrated
analgesia (can be morphine)

penile aspiration if priaprism
treat concomitant infection

Question 4

vaso-occlusive sickle cell crises

Answer 4

capillaries blocked + distal ischaemia

assoc with dehydration

get pain fever + infection often triggers it

an emergency!

Question 5

splenic sequestration crisis in sickle cell

Answer 5

acutely enlarged and painful spleen

severe anaemia and circulatory collapse

splenectomy + manage fluids/bloods

Question 6

aplastic crisis in sickle

Answer 6

parvovirus B19

1 week of supportive care and recover

Question 7

acute chest syndrome sickle diagnosis

Answer 7

fever or resp signs
+ new infiltrates seen on CXR

emergency

give treatment for cause
consdier ITU