Effects of crisis

Obstruction of flow
Regional hypoxemia and acidosis (metabolic or
respiratory).
Further sickling, tissue injury and pain.

Cause of pain
Pain due to increased intra-medullary
pressure (within juxta-articular areas
of long bones). Caused by an
inflammatory response to marrow
necrosis caused by sickled RBC.

Hydroxyurea also
incrases risk of infection
and some concern that it
may cause tumours or
leukemia

Blood transfusions
RBCs removed from a
supply of donor blood
and given
intravenously to
patient. Increase
number of normal
RBCs

Treatment
Blood transfusions
Hydroxyurea (drug)
reduces frequency of
painful transfusions +
may reduce need for
transfusions
Transcranial used for
children

Higher pressure in bone

marrow/spinal cord. Especially
near or in a joint. This is
caused by an immune
response to the death of bone
marrow through sickled red

Transcranial ultrasound
machine used to check for risk
of stroke. High risk of stroke
means they are treated with
regular blood transfusions