Sickle Cell CrisisEmergency

Casualty: known sickle cell disease (HbSS); severe bone, back or chest pain for 1 day, fever 38.6 °C; stopped hydroxyurea

• SpO₂ <95% or falling, new chest pain, cough or infiltrate (acute chest syndrome)
• Fever ≥38.5 °C (asplenic sepsis)
• New weakness, speech change, seizure or severe headache (stroke)
• Enlarging spleen with falling Hb (sequestration)
• Priapism >4 h
• Falling Hb with low reticulocytes (aplastic crisis)

• Osteomyelitis (Salmonella, Staph) vs bone infarct
• Acute cholecystitis / choledocholithiasis (pigment stones)
• Pneumonia vs acute chest syndrome — treat both
• Avascular necrosis of hip or shoulder
• Malaria in endemic areas
• Appendicitis or mesenteric ischaemia in abdominal crisis

• Admit: Pain not controlled with oral analgesia, any fever, chest signs, Hb ≥20 g/L below baseline, neuro symptoms, priapism, pregnancy
• ICU: Acute chest syndrome with hypoxia, stroke, sequestration with shock, multiorgan failure
• Transfuse: Simple if Hb ≥10–20 g/L below baseline or symptomatic; exchange for severe chest syndrome, stroke, multiorgan failure (keep post-transfusion Hb ≤100 g/L)
• Intubate: Respiratory failure in acute chest syndrome despite NIV

• A pain crisis is a clinical diagnosis — no test excludes it; treat first, investigate alongside.
• Acute chest syndrome is the commonest cause of death and often develops 24–72 h into a pain admission — examine the chest and SpO₂ every shift.
• Hydroxyurea: start 15 mg/kg/day (children 20), increase by 5 mg/kg every 8 weeks to the maximum tolerated dose (≤35 mg/kg); FBC every 4 weeks while titrating. It is supplied under India's National Sickle Cell Anaemia Elimination Mission (2023).
• Blood for transfusion: HbS-negative, leucodepleted, Rh (C, E) and Kell matched — alloimmunisation and delayed haemolytic reactions are common.
• Penicillin V prophylaxis is for children up to 5 years (and after splenectomy or invasive pneumococcal disease); vaccinate against pneumococcus, meningococcus (ACWY + B), Hib, influenza and hepatitis B.

• Give pethidine — norpethidine causes seizures
• Delay analgesia for tests or doubts about "drug seeking"
• Give fluid boluses to a euvolaemic patient — precipitates acute chest syndrome
• Transfuse to Hb >100 g/L (hyperviscosity → stroke)
• Stop hydroxyurea during a crisis unless the counts are low

• Under-dosing opioids
• Over-hydration
• Oxygen without hypoxaemia
• Not re-examining the chest daily
• Transfusing ABO-only matched blood
• Forgetting VTE prophylaxis and incentive spirometry

Uncomplicated crisis → ward on a care plan; acute chest syndrome with hypoxia, stroke or sequestration → HDU/ICU with haematology for exchange transfusion. Sickle cell clinic within 1–2 weeks to restart and titrate hydroxyurea.