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A 16-year-old male patient with sickle cell disease is scheduled for a cholecystectomy. He has been having biliary colic for 3 months; recent ultrasonography confirms cholelithiasis. Past medical history is significant for multiple hospitalizations for management of vaso-occlusive crisis (VOC), once for acute chest syndrome (ACS) at age 11, and once for splenectomy at age 3. His home medication regimen includes hydroxyurea, penicillin, and folic acid. His usual site of VOC pain is in his legs, back, and chest. His inpatient pain management regimen during his last VOC hospitalization included a morphine sulfate patient-controlled analgesia (PCA) and nonsteroidal anti-inflammatory medication. He has taken no opioid for the last 6 months.


Patients with sickle cell disease (which includes sickle cell anemia [SS], sickle hemoglobin C disease [SC], and the sickle B thalassemias) who undergo surgery are generally considered to be at greater risk for perioperative complications. Vaso-occlusion, which may involve both the micro- and macrovasculature, is the most important pathophysiologic event in sickle cell disease and explains most of its clinical manifestations. Cholelithiasis has been recognized in increasing numbers of pediatric patients with sickle cell disease.


Intensive care unit admission for close respiratory monitoring should be considered if the patient has a history of pulmonary disease (previous episodes of ACS, recurrent pneumonia). Stress of any kind may trigger the onset of a VOC.

  • Opioid prescription via PCA should be considered. PCA prescription can include a demand dose (if age appropriate) with or without a continuous infusion and clinician boluses.

  • Patients who have chronic pain and who have been on recent standing opioids preoperatively may have developed opioid tolerance and may require higher doses of opioid intra- and postoperatively; they may also be at risk for opioid withdrawal if their standing preoperative opioid dose requirement is not addressed postoperatively.

  • Laxatives should be prescribed for patients on opioids to avoid the risk of constipation. Caution should be exercised in the setting of abdominal surgery, and the prescribing of laxatives should be done in concert with surgical team recommendations.

  • Acetaminophen at appropriate weight-based doses can be given as an adjuvant for mild breakthrough pain.

  • A transition to oral opioids should be considered for postoperative pain control for a patient who has undergone an uncomplicated laparoscopic cholecystectomy once the patient is tolerating oral intake and is able to ambulate.

DOs and DON’Ts

  • ✓ Do consider the co-administration of adjuvants with the primary opioid analgesics to enhance their analgesic potential and obviate or ameliorate opioid side effects if co-administration of adjuvants is not otherwise medically or surgically contraindicated.

  • ⊗ Do not start a continuous infusion via PCA for a laparoscopic cholecystectomy if the patient can execute a demand dose unless there is established opioid tolerance, concurrent VOC, or another compelling reason to do so.

  • ✓ Do monitor for sedation and ...

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