Table 1.
Summary of recommendations
| Acute complication of SCD | Principles for management |
|---|---|
| VOE | - Evaluate pain with appropriate tools - Initiate analgesia within 30–60 minutes - Use pharmacologic, psychologic, and physical pain management. Avoid cold packs. - Administer acetaminophen and a NSAID regularly, in conjunction with opioids - Avoid over-hydration and promote incentive spirometryto prevent ACS - Consider other SCD complications (e.g., splenic sequestration, cholecystitis if abdominal pain), and differential diagnosis (e.g., avascular necrosis, osteomyelitis, septic arthritis if articular pain) - At discharge, provide appropriate prescriptions and education, and ensure follow-up |
| ACS | - Admit to hospital - Maintain oxygen saturation ≥95% - Avoid over-hydration and promote incentive spirometry - Initiate broad-spectrum antibiotics - Crossmatch for possible transfusion or ExT in severe cases (consult with hematology and intensive care) |
| Fever | - Draw blood culture and administer broad-spectrum antibiotics within 30 minutes of presentation, independent of focus for fever (do not delay antibiotics if blood culture is not yet drawn) - Treat influenza, if suspected - Consider serious bacterial infection, including osteomyelitis - Consider outpatient management for low-risk patients (Table 3) |
| Splenic sequestration | - Highest risk before age 5 (prior to auto-splenectomy) - Teach parents to palpate their child’s spleen and document spleen size at each visit - Strong indication for simple RBC transfusion - Discuss indication for splenectomy or chronic transfusion with a paediatric hematologist |
| Aplastic crisis | - Heralded by signs of anemia and low reticulocyte count - Parvovirus B19 is most common etiologic agent - Strong indication for simple RBC transfusion - Immunity conferred |
| Stroke | - Annual TCD ultrasounds (age 2–16) to identify high-risk patients - Maintain high index of suspicion, evaluate with CT scan +/- MRI - In children, AIS is more common than hemorrhagic, but either may occur - Indication for ExT - Strong evidence for chronic transfusions for primary and secondary prevention |
ACS Acute chest syndrome; AIS Acute ischemic stroke; ExT Exchange transfusion; NSAID Non-steroidal anti-inflammatory drug; RBC Red blood cell; SCD Sickle cell disease; TCD Transcranial Doppler; VOE Vaso-occlusive episode