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Clinical Practice Guidelines

Sickle cell disease

  • See also

    Acute pain management 
    Blood product prescription

    Key points

    1. Acute presentations may be due to infection, vaso-occlusion or anaemia. Life threatening complications include splenic sequestration (and subsequent shock), chest crises and stroke
    2. Children presenting to the emergency department should be assessed promptly, with timely initiation of analgesia and fluids
    3. Acute painful episodes in sickle cell disease (SCD) can be severe and require analgesia within 30 minutes of presentation. Escalate analgesia from what has been instituted prior to presentation
    4. Children with SCD should be managed with the assistance of a paediatric haematologist and all emergency presentations discussed
    5. Recommend haematology consult prior to sedation, anaesthetic and, or surgical procedures in children with SCD

    Background

    • Sickle cell disease (SCD) is caused by structurally abnormal haemoglobin (HbS) that polymerises with shape change when deoxygenated, resulting in obstruction of blood flow and manifesting as sickle cell “crises”. The most common types of sickle cell disease include HbSS, sickle ß Thalassemia, haemoglobin SC disease, all of which are treated in the same way
    • Acute crises may occur spontaneously or may be precipitated by infection, dehydration, hypoxia or medications (including sedatives, and general anaesthetics)
    • All children with SCD become functionally asplenic and are at increased risk of infection, particularly from encapsulated organisms eg pneumococcus, meningococcus, Haemophilus
    • Children with SCD may develop the following acute complications
      • Vaso-occlusive crisis (VOC) (painful crisis)
      • Serious bacterial infection
      • Acute chest syndrome (also known as chest crisis)
      • Acute splenic sequestration
      • Aplastic crisis
      • Acute haemolytic crisis
      • Stroke
      • Priapism

    Assessment

    History

    • Pain location (eg bone, back, abdomen, chest), duration and if similar to previous episodes
    • Has any analgesia been administered prior to presentation and what was the response
    • Shortness of breath, fever, pallor or lethargy
    • Acute neurological/stroke symptoms
    • Precipitating factors eg fever, exposure to extreme temperatures, long flights, surgical procedures, recent hospital admission
    • Past history of SCD complications and management required, eg ICU admission, ventilation, exchange transfusion
    • Usual treatment and adherence eg hydroxyurea, chronic transfusion program and management plan
    • Comorbidities such as asthma

    Examination

    • Vital signs
    • Pallor or jaundice
    • Hydration
    • Signs of infection
    • Respiratory examination
    • Splenic and liver size (compared to child’s baseline)
    • Neurological examination (increased risk of stroke during aplastic crises/severe anaemia)
    • Bony tenderness including hips (eg avascular necrosis), joint swelling, swelling of hands and feet in younger children (dactylitis)
    • Penile examination (if priaprism a concern)

    Management

    Investigations

    • FBE, reticulocyte count
    • Blood group and cross match
    • Blood and urine culture if febrile
    • UEC and LFT
    • CRP
    • Chest x-ray if febrile, respiratory symptoms or chest pain
    • Neuroimaging and code stroke is indicated if clinical suspicion for acute stroke
      • Seek advice from a haematologist and neurologist
      • MRI is the modality of choice: discuss with a haematologist prior if GA required
      • If not available, CT without contrast is appropriate (risk of hyperviscosity with contrast)
    • Parvovirus serology if concerned about aplastic crisis

    Treatment

    Approach to initial treatment

    CPG Flowchart Sickle Cell Disease_Sep2026

    Specific management as per type of crisis

    Presentation Overview Ongoing management
    VOC 
    (painful crisis)    		 Obstructed small blood vessels causing intense pain and inflammation
    Most commonly affects bone or gastrointestinal tract (the latter may present with acute abdomen and/or paralytic ileus)
    Younger children (<4 years) may present with pain and swelling in the hands and feet (dactylitis)
    Chest pain may indicate an acute chest syndrome rather than a vaso-occlusive episode if associated with respiratory symptoms    		 Parents and children will often know what pain medications have been effective in the past
    Escalate analgesia from what has been instituted prior to presentation
    See Acute pain management
    Those admitted to hospital with VOC should have twice daily reviews to allow for early detection of progression to acute chest syndrome
    Stroke SCD is a leading cause of paediatric stroke
    Can occur suddenly or as a complication of acute chest syndrome or aplastic crisis    		 Contact haematologist and neurologist for urgent referral, or call a code stroke with haematologist consult
    Management of stroke differs in the setting of SCD. Primary management is transfusion ie exchange transfusion or initial simple transfusion to Hb 100 g/L
    Early discussion and transfer to PICU with exchange transfusion capabilities
    Acute chest syndrome (ACS) Acute illness related to infarction of the lung tissue. Usually associated with lower respiratory symptoms, hypoxaemia and a new infiltrate on CXR. Chest pain and hypoxaemia may be the only signs
    ACS may be preceded by painful VOC (any location)

    Children presenting with chest pain should be evaluated as an acute chest syndrome and not simply as a VOC

    This is a life-threatening illness and patients may deteriorate quickly

    		 Supplemental oxygen (or respiratory support) to maintain saturations ≥95%
    CXR (should not delay commencement of treatment)
    Consider antibiotics if pneumonia strongly suspected. See Local antimicrobial guidelines
    Treat concomitant conditions such as asthma exacerbation
    Early discussion and transfer to PICU with exchange transfusion capabilities if significant hypoxia or respiratory distress
    Acute splenic sequestration Haemoglobin (Hb) drops at least 20 g/L below child's baseline level with an acutely enlarged spleen Mild to moderate thrombocytopenia is often present
    Most common in children ≤5 years    		 Contact blood bank and haematologist regarding transfusion volume and rate
    While waiting for blood give un-cross matched blood, or if unavailable, 0.9% sodium chloride (10-20 mL/kg bolus) to treat hypovolaemia
    Suggest initial transfusion to aim for Hb of 50-60 g/L initially to manage shock/haemodynamic instability
    Initial transfusion volume of 5-10 ml/kg with ongoing reassessment of haemodynamic status, in consultation with haematologist (rapid rise in Hb increases risk of stroke)
    Aplastic crisis An acute illness associated with Hb below baseline and a substantially decreased reticulocyte count (usually <1%)
    Usually associated with acute infection including parvovirus
    May also be associated with splenomegaly (see acute splenic sequestration)    		 Transfuse red blood cells if patient is symptomatic with anaemia or Hb <50 g/L
    Priapism Prolonged painful erection of the penis often starting in the early hours of the morning. Most common in adolescent years
    Occurs in 2 forms
    Intermittent, self-resolving “stuttering” episodes which last 2-4 hours but are often recurrent and may precede a severe episode
    Severe attack lasting longer than 4 hours and can result in impotence    		 Priapism lasting more than 4 hours can result in ischaemia and is a urological emergency. Consult general surgery (urology) and haematologist early
    Initial management includes having a warm bath or shower, moderate exercise, eg running up stairs
    Encourage emptying of the bladder and consider catheterisation if unable to empty bladder
    Commence hydration, oxygen, analgesia and alkanisation of the urine as soon as possible
    Do not use ice
    If surgery is required, transfusion support may be needed to reduce risks associated with the procedure. Discuss with haematologist

    Blood transfusion principles

    • Rediscuss with haematologist prior to administration
    • Children with SCD are at risk of developing hyperviscosity with over-transfusion of red cells. Post-transfusion Hb targets should generally not exceed total Hb >100 g/L
    • Children with SCD are more likely to develop clinically significant red cell antibodies with red cell transfusion
      • If phenotypically matched blood is not available in the emergency setting, transfusion should not be withheld in life-threatening situations
      • The preference is for the provision of phenotype-matched blood (minimum of RhCE, Kell matched)
      • Most children with known SCD will have a red cell phenotype +/- genotype performed at diagnosis (for children admitted to peripheral centres, this information may be obtained from the child’s treating hospital)
    • Acute transfusion is not recommended for uncomplicated pain crisis, asymptomatic anaemia or management of priapism

    Venous thromboembolism (VTE) prophylaxis

    • SCD is associated with an increased risk of VTE and prophylaxis should be considered
    • Consideration of VTE prophylaxis in conjunction with local hospital policy (if no contraindication) in: 
      • post pubertal children with SCD admitted to hospital 
      • children with SCD following CVC insertion (limited duration)

    Consider consultation with local paediatric team and haematologist

    • All children presenting with acute SCD-related presentations
    • Any concerns for acute stroke: urgent consultation and/or Code Stroke
    • When transfusion is being considered
    • All children with SCD requiring sedation, anaesthetic, or surgical procedures
    • Priapism lasting >2-4 hours: also consult surgery/urology

    Consider transfer when

    • Child with suspected acute chest crisis or stroke
    • If child may require surgical treatment (ideal to occur in sickle treatment center due to risks of GA and need for transfusion support)
    • Child requiring care above the level of comfort of the local hospital

    For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services

    Consider discharge when

    • Pain controlled with oral analgesia (able to mobilise freely)
    • Maintaining adequate oral hydration
    • Maintaining normal oxygen saturations without supplemental oxygen therapy
    • Afebrile and cultures negative at 48 hours
    • Paediatric haematologist has been consulted

    Last updated September 2026