Severe bone/chest/back pain; fever; noncompliant with hydroxyurea
ABCDE
Analgesia FIRST (within 30–60 min of triage); O₂ only if hypoxic (SpO₂ <95% or below baseline); IV fluids only if hypovolemic — target euvolemia, avoid overload (ACS/pulmonary oedema risk); septic screen if febrile; watch for chest syndrome → exchange.
FIRST — before anything else: Analgesia first (within 30–60 min of triage); O₂ only if hypoxic; IV fluids only if hypovolemic (euvolemia goal, avoid overload); antibiotics for fever
Then: Analgesia (opioids)
Acute chest syndrome not responding (worsening hypoxia) → Exchange transfusion (fastest)
Prevent: Hydroxyurea 15 mg/kg/day + folate + pneumococcal vaccine + penicillin prophylaxis
Order set
- O₂, IV fluids
- Analgesia
- FBC, reticulocytes
- Cultures if febrile
- Cross-match
- CXR if chest signs
Escalate / ICU
- Acute chest syndrome with hypoxia
- Worsening despite therapy → exchange transfusion
- Stroke, priapism, splenic sequestration
Criteria
AdmitCrisis needing parenteral analgesia, fever, or complications
ICUAcute chest syndrome with hypoxia, stroke, sequestration, multiorgan
IntubateSevere acute chest syndrome/respiratory failure
TransfuseSymptomatic; exchange for chest syndrome/stroke
DischargePain controlled on oral, afebrile, SpO₂ normal, hydrated, follow-up
Clinical detail — differentials, red flags, pitfalls, disposition
Red flags
- Hypoxia/new infiltrate (chest syndrome)
- Neuro deficit
- Priapism
- Sequestration
Differentials
- Vaso-occlusive vs infection vs sequestration
- Acute chest syndrome
Common mistakes
- Under-treating pain
- Missing acute chest syndrome
- Delaying exchange transfusion
- Overhydration (risk of ACS/pulmonary oedema) and oxygen without hypoxaemia (ASH 2020)
- Skipping incentive spirometry in chest/back-pain admissions (prevents ACS)
Disposition & follow-up
Admit for crisis; severe → exchange transfusion, ICU.
Discharge package
MedicationsHydroxyurea, folate, analgesia plan, penicillin prophylaxis
Follow-upHaematology; crisis action plan
VaccinationPneumococcal, meningococcal, Hib, influenza
LifestyleHydration, avoid triggers (cold, hypoxia, dehydration)
Warning symptomsChest pain/SOB, fever, priapism, neuro symptoms
💊 Treatment detail — doses & preparation
0.9% Sodium chloridecrystalloid
DoseBolus 500 mL over 15–30 min, reassess; maintenance 1–1.5 mL/kg/h
Preparation500/1000 mL bags; add KCl only after urine output confirmed
MonitorHyperchloraemic acidosis with large volumes — prefer balanced (LR) for resuscitation
Ceftriaxone3rd-gen cephalosporin
Dose2 g IV OD (meningitis 2 g q12h); gonorrhoea 500 mg–1 g IM once
PreparationIV: 2 g in 50–100 mL NS over 30 min; IM: reconstitute with lidocaine 1%
MonitorBiliary sludging; avoid with calcium-containing IV fluids in same line
Morphineopioid analgesic
Dose2.5–10 mg IV q1–2 h PRN; PCA 1 mg bolus, 5–10 min lockout
Preparation10 mg/mL ampoule — dilute to 1 mg/mL for titration
MonitorRR, sedation, constipation; histamine release (hypotension/itch)
Hydroxycarbamideantimetabolite
DosePV/sickle cell: 15–20 mg/kg OD titrated to counts
Preparation500 mg capsules
MonitorFBC 2–4 wkly during titration; leg ulcers, skin cancer risk long-term
📖 NHLBI / BSH Sickle CellReviewed July 2026