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This tool guides emergency care of acute complications in children with sickle cell disease. Clinicians pick the presentation and get steps and weight-based doses.
The presentations covered are fever of 38.5°C or higher, vaso-occlusive pain crisis, acute chest syndrome, splenic sequestration, stroke symptoms and priapism. Each has its own key thresholds. Fever needs a blood culture and IV ceftriaxone within 1 hour because of functional asplenia. Pain needs analgesia within 60 minutes of arrival. In acute chest syndrome, oxygen keeps SpO2 at 95% or higher, and simple transfusion is used if hemoglobin is 1 g/dL or more below baseline. Splenic sequestration is a fall of 2 g/dL or more with an enlarging spleen. For stroke, the exchange transfusion goal is HbS under 30%. Priapism lasting 4 hours or more needs emergency urology.
Doses include ceftriaxone 50–75 mg/kg, intranasal fentanyl 1.5 mcg/kg, IV morphine 0.1 mg/kg, ketorolac, azithromycin and red cell volumes. It is based on the NHLBI 2014 Expert Panel Report on sickle cell disease and its JAMA 2014 summary (Yawn et al.).
Give IV or IM ceftriaxone 50–75 mg/kg (max 2 g) within 1 hour of arrival, after a blood culture. Clindamycin 10 mg/kg is the option for severe cephalosporin allergy.
Give analgesia within 60 minutes of arrival, such as intranasal fentanyl 1.5 mcg/kg or IV morphine 0.1 mg/kg, reassessing every 15–30 minutes. Ketorolac can be added if kidney function is normal. Use incentive spirometry and avoid routine fluid boluses.
Simple transfusion (10 mL/kg) is advised if hemoglobin is 1 g/dL or more below baseline. Urgent exchange transfusion with hematology is advised if the child is deteriorating or SpO2 is under 90% despite oxygen.
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