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TABLE 93.6 SICKLE CELL ANEMIA: COMPLICATIONS AND MANAGEMENT Complication Clinical features Management Comments Vasoocclusive episode Joint or bone pain Mild or moderate pain IV fluid can be Joint swelling Oral or IV hydration D5 normal (e.g., dactylitis) (bolus 10–20 mL/kg, saline or D5 ½ Abdominal pain then 1–1½ times normal saline (may mimic acute maintenance volume) solution abdomen) Analgesia NSAID may be NSAIDs ibuprofen or Oral medications ketorolac and such as oxycodone should may be sufficient continue after for some patients discharge until IV (intermittent) pain resolves doses of morphine, If delayed or fentanyl, or difficult IV hydromorphone access, Nitrous oxide plus intranasal oral opioids is fentanyl may used in some be considered centers for initial Consider admission therapy if Patient-controlled pain worsens analgesia inadequate oral (PCA) may fluid intake include a repeat emergency continuous department visits infusion of Severe pain opioid IV bolus 10–20 mL/kg followed by If using fentanyl, patient may hydration at 1–1½ require PCA times maintenance due to short volume duration of Analgesia action NSAIDs (ketorolac) IV (intermittent and frequent) doses of morphine, fentanyl, or hydromorphone PCA (early) For refractory pain: IV ketamine at subdissociative doses Intermittent Continuous infusion Admit unless pain markedly reduced and patient tolerates oral fluids Priapism Splenic sequestration Acute chest syndrome Prolonged erection lasting more than hrs Pain May experience difficulty urinating Oral or IV hydration Urology may (10–20 mL/kg bolus, consider early then 1–1½ times aspiration of maintenance) the corpora Oral pseudoephedrine Analgesia as needed to control pain Consult with urology if no relief within hrs of onset Left upper Immediate volume Onset of quadrant pain replacement symptoms is Pallor IV fluids often sudden Lethargy Simple red blood cell Usually occurs Splenomegaly transfusion before age 5, May have altered Admission to hospital but may vital signs Splenectomy in develop later in (tachycardia, refractory patients patients with hypotension) hemoglobin SC Worsened anemia disease with elevated reticulocytes and mild to moderate thrombocytopenia Chest pain Antibiotic therapy Oxygen Third-generation saturation below cephalosporin, patient’s baseline consider addition of vancomycin; if Therapy with steroids not usually needed unless patient has a history of Ischemic or hemorrhagic stroke Hemolytic anemia crisis Symptoms of respiratory distress New finding on chest radiograph Fever often present allergy to cephalosporins, consider quinolones (e.g., levofloxacin) or clindamycin, AND Macrolide Consideration of red blood cell transfusion for patients with respiratory distress Simple transfusion if hemoglobin
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