From the Guidelines
Anesthetic management of patients with Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency requires careful medication selection to avoid triggering hemolytic crises, and screening for G6PD deficiency is recommended upon entry into care or before starting therapy with an oxidant drug in patients with a predisposing racial or ethnic background 1.
Key Considerations
- Avoid oxidative stressors including methylene blue, prilocaine, and sulfonamide antibiotics, as they can lead to hemolysis in G6PD-deficient patients 1.
- Safe anesthetic agents include propofol, etomidate, ketamine, benzodiazepines, opioids, and all modern inhalational anesthetics (sevoflurane, desflurane, isoflurane) 1.
- Regional anesthesia with lidocaine, bupivacaine, and ropivacaine is also safe.
Anesthetic Protocol
- For premedication, use midazolam (1-2 mg IV) or fentanyl (50-100 mcg IV).
- Induction can be performed with propofol (1.5-2.5 mg/kg) or etomidate (0.2-0.3 mg/kg), and maintenance with sevoflurane (1-3%) or propofol infusion (75-150 mcg/kg/min).
Perioperative Monitoring
- Monitor for signs of hemolysis such as hyperbilirubinemia, anemia, and hemoglobinuria.
- Maintain adequate hydration and avoid acidosis, hypoxia, and hypothermia as these can exacerbate hemolysis in G6PD-deficient patients.
Pathophysiology
- The underlying pathophysiology involves reduced ability to generate NADPH, which is necessary to maintain glutathione in its reduced form to protect red blood cells from oxidative damage, making these patients susceptible to hemolysis when exposed to oxidative stressors 1.
From the Research
Anesthetic Considerations in G6PD Deficiency
- Patients with Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency require careful perioperative management to avoid the onset of hemolytic crisis 2.
- The anesthetic management in such patients should focus on avoiding the drugs implicated in haemolysis, reducing the surgical stress with adequate analgesia, and monitoring for and treating the haemolysis, should it occur 3.
Safe Anesthetic Agents
- Benzodiazepines, codeine/codeine derivatives, propofol, fentanyl, and ketamine were not found to cause hemolytic crises in the G6PD-deficient patient 4.
- Dexmedetomidine, an anesthetic agent with antioxidant actions, may be useful for reducing the risk of hemolysis after surgery in infant G6PD deficiency cases 5.
- The combination of sevoflurane inhalation anesthesia with the addition of sufentanil proved to be safe and effective in the management of a child with G6PD deficiency 6.
Perioperative Management
- Management for pain and anxiety should include medications that are safe and have not been shown to cause hemolytic crises 4.
- The most effective management strategy is to prevent hemolysis by avoiding oxidative stressors 4.
- During the operative period, the anesthetic goal is to reduce stress and monitor if the hemolysis occurs, and of course, treat it if it occurs 6.
Recommendations
- Anyone suspected of G6PD deficiency should be screened 4.
- Exposure to oxidative stressors in these individuals should be avoided 4.
- These patients should be informed of risks along with signs and symptoms of an acute hemolytic crisis 4.
- The clinician should be able to identify both laboratory and clinical signs of hemolysis 4.
- If an acute hemolytic crisis is identified, the patient should be admitted for close observation and care 4.