Sickle cell disease


These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline. 

Read the full PCH Emergency Department disclaimer.


To guide PCH ED staff in the management of children presenting to the Emergency Department with Sickle cell disease.


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:
  • Dehydration
  • Hypoxia
  • Infection
A child with SCD presenting to ED with fever or pain should be assessed within 30 minutes of arrival or earlier if clinically indicated.

Presenting problems

  • Vaso-occlusive crisis (painful crisis)
  • Fever – sepsis
  • Acute chest syndrome
  • Stroke
  • Priapism
  • Aplastic crisis
  • Acute splenic sequestration
Evaluate for complications and begin urgent treatment after discussion with the Oncology / Haematology Fellow on-call.

General management

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:
  • mild – paracetamol & ibuprofen
  • moderate to severe – oxycodone oral or morphine 0.05mg/kg IV
Repeat as needed – may need opioid continuous infusion.


  • Push oral fluids
  • May require IV fluid bolus 10-20ml/kg
  • Consider maintenance IV if unable to tolerate oral
  • Avoid excess fluids to reduce risk of chest crisis.


  • For hypoxia or respiratory distress
  • Early PICU review and commence respiratory support as soon as possible if clinical concern of acute chest syndrome.

Blood transfusion

  • May be required – discuss with on call haematology fellow / haematologist.


  • FBC including reticulocyte count
  • Blood group & cross match
  • Blood and urine cultures if febrile CRP
  • U&Es and LFTs if jaundice or dehydrated
  • Consider chest Xray if febrile with respiratory symptoms
Consider other imaging as clinically appropriate.

Specific management

Vaso-occlusive crisis

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 (e.g. Haemophilus, Meningococcus, Streptococcus). 

Specific Management:

  • Obtain appropriate cultures – blood, sputum, urine.
  • Commence IV antibiotics as per ChAMP guidelines.
  • Cover for atypical organisms (Azithromycin) if significant respiratory component.
  • Treat pain as per vaso-occlusive crisis.
  • Consider and treat for acute chest syndrome if cough or dyspnoea is present.

Acute chest syndrome

Chest crisis is a life threatening condition and patients can deteriorate rapidly.

Suspect if respiratory distress, hypoxia or chest pain

  • Oxygen to keep O2 saturation > 96% or for comfort
  • Analgesia
  • Commence IV antibiotics – Ceftriaxone and Azithromycin
  • Chest X-ray – but this should not delay commencement of treatment
  • Early referral to PICU for respiratory support if significant hypoxia or respiratory distress.


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.

Specific Management:

  • Neuroimaging required to determine if haemorrhagic or ischaemic stroke.
  • MRI is preferred.
  • CT without contrast (risk of hyperviscosity) if MRI not available.

Transfusion support:

  • Discuss with on call Haematology Fellow / Haematologist.
  • Transfusion to Hb 100g/L +/- red cell exchange.


May be intermittent or prolonged (>4 hours increases risk of impotence)

Specific management:

  • Do not use ICE
  • Analgesia, oxygen, IV hydration
  • Empty of bladder – may need catheterisation
  • Simple measures e.g. moderate exercise, take a bath or shower
  • Alkalanise urine
  • Consult on-call Haematology Fellow / Haematologist and General Surgery if priapism has lasted more than 3 – 4 hours
  • Transfusion support may be required – discuss with Haematology

Aplastic crisis

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

Specific management:

  • Intravenous fluids and oral intake to a total of maintenance.
  • Transfuse red blood cells if patient is symptomatic with anaemia or Hb <50g/L (do not increase Hb by > 30g/L).
  • Commence IV antibiotics if febrile – Ceftriaxone.

Acute splenic sequestration

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.

Specific management:

  • Fluid resuscitation – 0.9% sodium chloride 10-20ml/kg for hypovolaemia while waiting for blood.
  • Initial transfusion to aim for Hb of 50-60 g/L initially to ameliorate shock / haemodynamic instability (do not increase > 30 g/L).
Autotransfusion will occur if haemoglobin is increased excessively or too quickly. This increases risk of stroke due to hyperviscosity.
  • IV antibiotics if febrile as per ChAMP guidelines.


  1. Textbook of Paediatric Emergency Medicine 2nd Edition Cameron Elesevier 2012
  2. Fleisher, Gary R. Ludwig, Stephen. Textbook of Pediatric Emergency Medicine, 6th Edition. 2010
  3. Nelson Textbook of Pediatrics: 20th Edition Robert M. Kliegman, Bonita M.D. Stanton, Joseph St. Geme, Nina F Schor Publisher: Elsevier

Endorsed by:  Director, Emergency Department  Date:  Feb 2018

 Review date:   Feb 2021

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