Known sickle cell disease with acute pain
Adult or child with SCD (HbSS, HbSC, HbSβ-thalassaemia) and an acute pain episode. Pregnant: involve obstetrics and haematology early.
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Hematology & OncologyEmergency
Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020)
Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020): Known sickle cell disease with acute pain → Triage: very severe pain is ATS 2 → Fever ≥38.5 °C:...
Pathway Overview
14 steps
Algorithm Steps
14 total
01Start 02Action Triage: very severe pain is ATS 2
Give the first analgesic within 30 min of triage or 60 min of arrival.
- ATS 2 (10 min): very severe pain, fever with lethargy, unstable sepsis, stroke signs
- At least ATS 3 (30 min): moderately severe pain that needs analgesia
- Full observations: temperature, SpO2, RR, HR, BP, pain score
- Do not delay analgesia for IV access: use SC or intranasal route
03Warning Fever ≥38.5 °C: possible sepsis, not only VOC
Functional asplenia: high risk of pneumococcal sepsis. Treat infection and pain together. Looks septic: start the sepsis pathway now.
- Blood culture, FBC, reticulocytes; urine culture if UTI suspected; CXR if chest signs
- Child: prompt parenteral 3rd-generation cephalosporin (eg ceftriaxone)
- Adult: low threshold for IV antibiotics; admit if ≥39.5 °C or looks unwell
04Action Assess while the first dose is prepared
Look for complications and other causes of pain. Do not delay the first analgesic dose.
- Usual crisis pain? Atypical pain: look for another cause
- Opioids taken today, individual care plan, baseline Hb, genotype
- Chest pain, cough, dyspnoea, SpO2; neuro exam; spleen size vs baseline; priapism
- FBC, reticulocytes, group and screen; U&E and LFT if jaundiced or dehydrated
- Ask: transfused in the last 3 weeks? Pregnant? Renal impairment?
05Action Severe pain: opioid within 30 min of triage
Parenteral opioid, then reassess. Hold the next dose if sedation score ≥2 or RR ≤10/min (adult) or below the normal range for age (child): hypoventilation can trigger acute chest syndrome. Mild to moderate pain: paracetamol plus NSAID; no NSAID if renal impairment, pregnancy, anticoagulant or peptic ulcer.
- Use the individual care plan if there is one; if not, base the dose on home opioid use
- Adult without a plan: IV or SC morphine or hydromorphone; dose per local protocol
- Child: morphine 0.05 mg/kg IV or SC per dose, repeat as needed (max 5-10 mg per dose; max 2.4 mg/kg/day)
- Child >12 months, no IV: intranasal fentanyl 1.5 microgram/kg (max 100 microgram) first dose
- Reassess pain, sedation and SpO2 15-30 min after each dose; re-dose or raise dose by 25% until controlled
- Renal impairment: prefer fentanyl over morphine. Do not use pethidine.
- Oversedation or slow breathing: stop opioid, support airway, oxygen; naloxone per local protocol
06Decision Red flags present?
Fever ≥38.5 °C, chest signs or hypoxia, neurological signs, Hb ≥20 g/L below baseline, enlarging spleen or liver, priapism, or looks unwell.
- Fever ≥38.5 °C or looks septic
- Acute chest syndrome: chest pain, cough, dyspnoea, hypoxia or new infiltrate
- Stroke or TIA: focal deficit, severe headache, seizure, reduced consciousness
- Hb ≥20 g/L below baseline (or <60 g/L), enlarging spleen or liver, low reticulocytes
- Priapism: ≥4 hours is an emergency
- If Yes
07Action Red flag: stroke, chest syndrome or sepsis
Admit. Call haematology now. Continue analgesia with sedation and SpO2 checks. Transfuse only with haematology, Rh (C, E) and K matched: HbSS, do not raise Hb above 100 g/L; HbSC, expert decides; transfused in the last 3 weeks, blood bank first.
- Stroke signs: urgent CT, stroke team; transfuse within 2 h of presentation, exchange preferred; no exchange within 2 h and Hb ≤85 g/L: simple transfusion first
- Adult ischaemic stroke: assess for IV thrombolysis per stroke pathway, in parallel with transfusion. Not in children under 18
- ACS: IV cephalosporin plus macrolide; oxygen to SpO2 ≥95%; incentive spirometry
- ACS transfusion: simple if Hb >10 g/L below baseline; urgent exchange and ICU if SpO2 <90% on oxygen, worse distress or spreading infiltrates
- Fever or sepsis: cultures and IV antibiotics now; sepsis pathway if unstable
08Action Red flag: acute anaemia, sequestration or priapism
Transfuse only with haematology advice, Rh (C, E) and K matched red cells. Do not overtransfuse: HbSS, do not raise Hb above 100 g/L.
- Transfused in the last 3 weeks with pain, anaemia or jaundice: possible delayed haemolytic reaction; blood bank and haematology before any transfusion
- Splenic sequestration with shock: IV fluid bolus, small-volume transfusion; avoid Hb >80 g/L (sequestered cells return)
- Aplastic crisis (low reticulocytes): transfuse to a safe Hb; droplet precautions (parvovirus B19)
- Priapism ≥4 h: urgent urology for aspiration; hydration and analgesia
- Hepatic sequestration or severe intrahepatic cholestasis: urgent haematology; transfusion
09Outcome Admit for ongoing management
Inpatient haematology care. ICU if organ failure, stroke or worsening acute chest syndrome.
If No10Action No red flags: continue VOC care
Treat as uncomplicated VOC. Recheck red flags at each review.
- NSAID for 5-7 days with the opioid; avoid if renal impairment, peptic ulcer, anticoagulation or pregnancy
- Oral fluids; IV only if unable to drink or dehydrated, at maintenance rate: overhydration risks ACS
- Oxygen only if SpO2 <95% on room air
- Incentive spirometry while awake; mobilise early; heat and distraction
- Regular paracetamol; antiemetic and laxative as needed
- No transfusion or corticosteroids for uncomplicated VOC
11Decision Pain controlled within 6-8 hours?
Controlled on oral analgesia, afebrile, SpO2 at baseline, drinking, no new red flag.
- If Yes
12Action Controlled: plan discharge
Only if pain is controlled on oral analgesia and there are no red flags.
- Oral analgesia plan; adjust home opioids to prevent withdrawal; short supply
- NSAID for 5-7 days if no contraindication; regular paracetamol
- HbSS or HbSβ0: hydroxyurea via haematology (child from 9 months; adult with ≥3 moderate-severe crises in 12 months). Not in pregnancy
- Update the individual care plan; arrange haematology follow-up
- Return now if fever ≥38.5 °C, chest pain, breathlessness, weakness, pallor or priapism
13Outcome Discharge home
With an analgesia plan, return advice and haematology follow-up.
If No14Action Not controlled: admit
Admit under haematology. Give scheduled opioid or PCA, not only as-needed doses.
- PCA or scheduled opioid; with a continuous PCA infusion, review long-acting oral opioids (over-sedation)
- Monitor sedation score, RR and SpO2; hold opioid if sedation ≥2 or RR ≤10/min (child: below age range); naloxone if respiratory depression
- Daily FBC and reticulocytes; watch for ACS (new chest signs, hypoxia)
- Incentive spirometry while awake; mobilise early
- Refractory pain: ketamine infusion 0.1-0.3 mg/kg/h (max 1 mg/kg/h) in an experienced inpatient unit
- Path rejoins step 09Shared downstream outcome
Guideline Source
American Society of Hematology 2020 Guidelines for Sickle Cell Disease: Management of Acute and Chronic Pain
Clinical Safety Information
Clinical Decision Support — Not a Substitute for Clinical Judgment
Individual patient factors may require deviation from these recommendations.
Known Limitations
- Adult opioid starting doses are not given: use the individual care plan or the local acute pain protocol
- Acute complications (ACS, stroke, sequestration, priapism) are summarised only: manage with haematology and the specific protocol
- Infants under 12 months need paediatric haematology advice
- Does not cover chronic pain, disease-modifying or curative treatment
Contraindicated Populations
pregnancy_without_obstetric_and_haematology_inputinfants_under_12_months_without_paediatric_haematology_input
Applicable Regions
AUNZUSEUGlobal
AU: Triage by the Australasian Triage Scale (ACEM G24). Paediatric doses from the RCH Melbourne guidelines. Discuss every red flag with the on-call haematologist.
EU: Adapt to local opioid rules and local emergency SCD protocols.
US: Triage as ESI 2 (NHLBI 2014). Follow ASH 2020 pain guidelines.
Version 2Next review: 2027-09-30
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Frequently Asked Questions
What is the Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020)?
The Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020) is a emergency clinical algorithm for Hematology & Oncology. It provides a structured decision tree to guide clinical decision-making, based on American Society of Hematology 2020 Guidelines for Sickle Cell Disease: Management of Acute and Chronic Pain.
What guideline is the Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020) based on?
This algorithm is based on American Society of Hematology 2020 Guidelines for Sickle Cell Disease: Management of Acute and Chronic Pain (DOI: 10.1182/bloodadvances.2020001851).
What are the limitations of the Sickle Cell Vaso-Occlusive Crisis Management (ASH 2020)?
Known limitations include: Adult opioid starting doses are not given: use the individual care plan or the local acute pain protocol; Acute complications (ACS, stroke, sequestration, priapism) are summarised only: manage with haematology and the specific protocol; Infants under 12 months need paediatric haematology advice; Does not cover chronic pain, disease-modifying or curative treatment. Individual patient factors may require deviation from these recommendations.
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