What are the recommendations for anesthesia management in patients with sickle cell anemia undergoing surgery?

Anesthesia Management in Patients with Sickle Cell Anemia

Patients with sickle cell disease require meticulous perioperative care to prevent sickling crises, with particular attention to avoiding dehydration, hypoxia, acidosis, hypothermia, and pain. 1

Preoperative Management

• All patients should be screened for hemoglobinopathy before surgery, with unnecessary repeat screening avoided 1
• Patients should be reviewed in a pre-assessment clinic with input from a nominated lead hematologist who will determine the perioperative transfusion plan 1
• Schedule patients early on the operating list to avoid prolonged starvation and prevent last-minute cancellations 1
• Document baseline oxygen saturation and continue monitoring if sedative premedication is given 1
• The acute pain team should be notified in advance, particularly for patients with a history of chronic pain 1
• Preoperative transfusion requirements should be determined on a patient-specific basis, considering:
  • SCD genotype
  • Baseline hemoglobin
  • Disease severity
  • Risk classification of surgery
  • History of prior surgical complications 2

Intraoperative Management

• Pre-oxygenation is recommended before induction of general anesthesia 1
• Avoid hypotension during surgery as it can precipitate sickling 1
• Use controlled ventilation to achieve good oxygenation and normocarbia if the patient's trachea is intubated 1
• Maintain normothermia through active warming in the operating room, increased ambient temperature, avoiding unnecessary exposure, and using warmed fluids 1
• Ensure adequate hydration with careful fluid management and monitoring 1, 3
• General anesthesia may be preferred over regional anesthesia, as SCD-related complications were more frequent with regional anesthesia in some studies 4

Postoperative Management

• Administer continuous oxygen therapy to keep SpO₂ above baseline or 96% (whichever is higher) for 24 hours or until the patient can mobilize freely 1
• Continue oxygen saturation monitoring until it is maintained at baseline in room air 1, 3
• Provide early mobilization, physiotherapy, and incentive spirometry every 2 hours after moderate or major surgery 1, 3
• Consider bronchodilator therapy for patients with a history of small airways obstruction, asthma, or acute chest syndrome 1, 3
• Continue active warming until the effects of anesthesia and sedation have worn off 1
• Monitor temperature regularly as a spike may indicate early sickling 1
• Implement thromboprophylaxis for all peri- and post-pubertal patients 1
• Monitor for signs of infection and administer antibiotic prophylaxis as dictated by the surgical procedure 1
• Maintain a low threshold for admission to high dependency or intensive care units, as most complications occur postoperatively 1, 3

Transfusion Considerations

• Preoperative transfusion is associated with lower rates of SCD-related postoperative complications, particularly for SS patients undergoing low-risk procedures (12.9% without transfusion vs. 4.8% with transfusion) 4
• For SC patients, preoperative transfusion appears beneficial for all surgical risk levels 4
• Partial exchange transfusion may be considered to increase hemoglobin A percentage, with some studies aiming for levels of 40% or above 5, 6

Multidisciplinary Approach

• Communication between anesthesiologists, surgeons, and hematologists is essential to determine the risk of complications and anticipate them 7
• Daily assessment by a hematologist after moderate or major surgery is recommended 1
• Maintain a high index of suspicion for sickle complications, which may be difficult to differentiate from postoperative pain or surgical complications 1

Common Pitfalls and Caveats

• Avoid routine surgery if the patient is febrile or having a painful crisis 1
• Do not give continuous oxygen therapy unless necessary, but ensure adequate oxygenation 1
• Recognize that sickle cell patients are more susceptible to infection, including respiratory and postoperative wound infections 1
• Be vigilant for acute chest syndrome, which can develop rapidly and may require intensive care management 3
• Intravenous cannula sites should be inspected regularly for phlebitis and removed immediately if there are signs of redness or swelling 1