sickle cell anaemia

Question 1

RBCs are

Answer 1

fragile and more easily destroyed–> haemolytic anaemia

Question 2

inheritcance

Answer 2

autosomal recessive

Question 3

abnormal gene for

Answer 3

beta-globin on chromosome 11

Question 4

complications

Answer 4

anaemia 
risk infection 
stroke 
AV necrosis large joints
pulmonary htn 
priapism 
CKD
sickle cell crisis 
acute chest syndrome

Question 5

general Mx

Answer 5

avoid dehydration + other crises triggers
up to date vaccines
antib prophylaxis
hydroxycarbomide
blood transfusion 
bone marrow transplant

Question 6

what antibx prophylaxis is given

Answer 6

penicillin V (phenoxymethylpenicillin)

Question 7

what can hydroxycarbamide be used for

Answer 7

to stimulate production of fetal Hb as HbF doesnt lead to sickling of RBCs

Question 8

sickle cell crises

Answer 8

umbrella term for spectrum of acute crises

can be triggered by infection, dehydration, cold, stress

Question 9

sickle cell crises Mx

Answer 9

low threshold hosp admission
treat infection 
keep warm 
hydration - IV fluids
simple analgesia
penile aspiration in priapism

Question 10

priapism

Answer 10

painful and persistent erection

Question 11

vaso-occlusive crisis (aka painful crisis)

Answer 11

sickle shaped RBCs clog up capillaries causing distal ischaemia

Question 12

vaso-occlusive crisis (aka painful crisis) - associations

Answer 12

dehydration

raised haematocrit

Question 13

vaso-occlusive crisis (aka painful crisis): symptoms

Answer 13

pain
fever
those of what triggerd it
pripism in men

Question 14

priapism Mx

Answer 14

aspiration of blood from penis

Question 15

Splenic Sequestration Crisis

Answer 15

RBCs block blood flow within spleen
causes enlarged and sore spleen

pooling of blood in spleen can lead to severe anaemia + hypovolaemic shock

Question 16

Splenic Sequestration Crisis: Mx

Answer 16

blood transfusions
IV fluid resus

if recurrent = splenectomy

Question 17

aplastic crisis

Answer 17

temporary loss of creation od new blood cells

leads to signif anaemia

Question 18

aplastic crisis trigger

Answer 18

parovirus B19

Question 19

aplastic crisis Mx

Answer 19

blood transfusions if needed

Question 20

acute chest syndrome: diagnosis requires

Answer 20

fever or new resp systems with new infiltrates seen on CXR

Question 21

acute chest syndrome: causes

Answer 21

infective cause

non-infective cause e.g. fat emboli, pulmonary vaso-occlusion

Question 22

acute chest syndrome: Mx

Answer 22

antib or antiviral for infection
blood transfusion
incentive spirometry
artificial ventilation