These guidelines are intended for use by clinicians providing care to children with Sickle Cell Disease (SCD)
who present to the Emergency Department, to ensure that these children are expediently and correctly
assessed and managed according to best practice guidelines.

This guideline is intended for use by all clinical staff treating paediatric patients with SCD.
SCD is an autosomal recessive disorder caused by a structurally abnormal haemoglobin (HbS) that
polymerises with shape change when deoxygenated, resulting in obstruction of blood flow.
There are 3 common types of SCD
• Sickle Cell Anaemia (HbSS)
• Sickle Beta Thalassaemia
• Sickle haemoglobin C disease
Patients with SCD may present with the following acute problems:
• Painful Vaso-occlusive crisis (VOC)
• Fever
• Acute chest syndrome
• Acute splenic sequestration
• Aplastic crisis
• Stroke
• Priapism
Acute crises may occur spontaneously or may be precipitated by infection/ dehydration/ hypoxia or anaesthetic/
surgery.
Investigation
• FBC including reticulocyte count
• Blood group and cross match (if suspected anaemia, acutely enlarged spleen, neurologic or respiratory
symptoms). If new patient or has never had previous transfusion request extended red cell phenotyping
given high incidence of alloimmunisation developing in this group.
• Blood and urine cultures as well as C-reactive protein (CRP) if febrile or respiratory symptoms
• Electrolytes, Urea, Creatinine and Liver function tests (ELFTs/CHEM20) if dehydrated or jaundiced (or send
if IV hydration required)
• Chest X-ray (CXR) if respiratory signs / symptoms, chest pain, hypoxia, or febrile / toxic appearance
• Lumbar Puncture (LP) only if would be indicated as per usual work up of a child with “fever and no focus”
• Consider abdominal Ultrasound (US) for right upper quadrant or epigastric pain (risk of cholelithiasis and
intrahepatic sickling)
• Urgent Computer Topography (CT) brain without contrast (or Magnetic Resonance Imaging (MRI) if
available without delay) in a child with new neurologic symptoms or signs
Management (see below for management of specific presentations)
• For urgent presentations (chest crisis, stroke, septic child) do not delay commencement of IV fluid or IV
analgesics while awaiting topical anaesthetic cream
– Intranasal fentanyl 1 to 1.5micrograms/kg (maximum 100 micrograms per dose) may be considered for
severe pain whilst IV access is being established
• Supplemental oxygen should be provided if O2 saturation <95% 
• Start analgesics promptly (within 30 minutes of arrival):
– Mild pain – oral paracetamol 15mg/kg (maximum 1g) 6 hourly and ibuprofen 10mg/kg (maximum 400mg)
8 hourly
▪ Reassess at 20-30 minutes and consider oral oxycodone 0.1-0.2mg/kg (maximum 5mg) 4 to 6 hourly
if pain persists
▪ If pain worsens of is not controlled by oral medication manage as per Moderate to Severe pain
– Moderate to severe pain – oral paracetamol, ibuprofen (doses as above), and morphine
– 3 months to 11 months – use morphine 0.025 mg/kg/dose
• Dose calculation 0.025 mg/kg/dose (25 micrograms/kg/dose).
• If required morphine dose can be repeated after 15 minutes, maximum three (3) times, until pain
is controlled (e.g. three (3) doses over a 30-minute period).
• Recommended minimum timeframe of 15 minutes (time to peak concentration) between
morphine doses.
– 1 year and older – use morphine 0.05 mg/kg/dose (max. 2.5 mg/dose)
• Dose calculation 0.05 mg/kg/dose (50 micrograms/kg/dose).
• If required morphine dose can be repeat after 15 minutes, maximum three (3) times, until pain
is controlled (e.g. three (3) doses over a 30-minute period (max. 7.5 mg)).
• Recommended minimum timeframe of 15 minutes (time to peak concentration) between morphine doses.
• For older children and in patients with severe pain, higher doses of morphine may be required and
previous admission history and dosing should be viewed as used as guide for adequate analgesia.
This should be discussed with the on-call haematologist.
• Early consideration of notification of admission and referral to the Acute Pain Service and
commencement of a PCA (Patient Controlled Analgesia), nurse-controlled analgesia (NCA) or
continuous opioid infusion for severe pain (particularly if a repeat bolus of opioid is required within 2
hours).
• See CHQ-PROC-01003 Intravenous Opioid Infusions - Patient Controlled Analgesia, Nurse Controlled
Analgesia & Continuous Opioid Infusion and CHQ-PROC-01050 Morphine Intermittent Intravenous
(IV) Bolus Injection
– For moderate pain which responds rapidly to initial analgesia a combination of regular paracetamol,
ibuprofen and oxycodone orally as above may be more appropriate than ongoing intravenous opioid
therapy (regular reassessment is required) and may give opportunity for early discharge.
– Where intravenous opioids are used patients must have pulse oximetry applied and a sedation score
monitored as per the paediatric analgesic infusion monitoring tool, or, when this is not available,
according to local hospital policy (see ID 01003 & 01050 above)
• Fluids
– Encourage oral fluids
– May require IV fluids
• Electrolytes should be ordered prior to IV fluid commencement 
• For simple painful VOC, IV fluids (0.9% sodium chloride) at 2/3 maintenance should be commenced
• If clear signs of an acute chest crisis, abdominal or back pain, do NOT give a bolus and ensure a
maximum total fluid intake of not greater than maintenance rate.
• If there is clinically apparent dehydration a small bolus of 5-10mL/kg of 0.9% sodium chloride
(Maximum of 1L) may be considered, followed by maintenance IVF with 0.9% sodium chloride and
5% glucose
• Fluid balance monitoring should be commenced for all patients
– Blood transfusion
• Not recommended to be given routinely during acute painful VOC
• Is not required for severe anaemia that is chronic, asymptomatic and well-compensated (within 10 g/L
of the patients normal baseline Hb)
• “Over-transfusion” (Hb >100 g/L) should be avoided as higher Hb leads to increased plasma viscosity
and potential for increased vaso-occlusion
• Standard products are appropriate (irradiated or CMV negative blood product modifications are not
required), however all patients with Sickle cell disease should receive extended red cell phenotype
matched blood – please liaise with blood bank to ensure they have appropriate units available,
and if there is any issue contact the haematologist on call.