Sickle Cell Disease ED Pathway quick reference.
Sickle cell disease (SCD) is a haemoglobinopathy where abnormal haemoglobin (HbS) “sickles” causing blood flow obstruction.
Sickle cell crises can be spontaneous or precipitated by:
A child with SCD presenting to ED with fever or pain should be assessed within 30 minutes of arrival or earlier if clinically indicated.
Evaluate for complications and begin urgent treatment after discussion with the Oncology/Haematology Fellow on-call.
A child with SCD presenting to ED with fever or pain should be assessed within 30 minutes of arrival. Consult on call Haematology Fellow/Haematologist early.
Do not wait for EMLA for commencement of IV fluids or analgesia.
Start analgesics promptly:
Repeat as needed – may need opioid continuous infusion.
Consider other imaging as clinically appropriate
Precipitated by dehydration, hypoxia or infection.
All episodes of pain should be treated initially as vaso-occlusive disease as per general management above.
NB Chest pain may indicate an acute chest syndrome rather than as a vaso-occlusive episode if associated with respiratory symptoms.
Patients are functionally asplenic and at greater risk for invasive disease particularly by encapsulated organisms (eg. Haemophilus, Meningococcus, Streptococcus).
Chest crisis is a life threatening condition and patients can deteriorate rapidly.
Suspect if respiratory distress, hypoxia or chest pain
Incidence is up to 1 in 10 patients with HbSS disease.
Can occur suddenly or as a complication of acute chest syndrome or aplastic crisis.
May be intermittent or prolonged (>4 hours increases risk of impotence)
An acute illness with a decrease in haemoglobin without a reticulocyte response (usually <1%). Usually associated with acute infection including parvovirus. Present with pallor +/- shock
Anaemia (drop in Hb >20g/L) with mild to moderate thrombocytopaenia and acute splenomegaly. May have co-existent aplastic anaemia if reticulocyte count is low. May present in shock.
Autotransfusion will occur if haemoglobin is increased excessively or too quickly. This increases risk of stroke due to hyperviscosity.
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