From the Guidelines
Individuals with Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency should avoid specific medications and foods that can trigger hemolytic crises, and screening for G6PD deficiency is recommended before starting therapy with an oxidant drug in patients with a predisposing racial or ethnic background. Management of G6PD deficiency primarily involves avoiding triggers that can cause hemolytic crises. Individuals with G6PD deficiency should avoid specific medications including antimalarials (primaquine, chloroquine), antibiotics (sulfonamides, nitrofurantoin, ciprofloxacin), analgesics (aspirin in high doses), and other drugs like rasburicase and methylene blue, as noted in the study 1. Certain foods should also be limited, particularly fava beans (which can cause favism), and in some cases, high doses of vitamin C. During acute hemolytic episodes, treatment includes discontinuing the triggering agent, maintaining hydration, monitoring hemoglobin levels, and providing supportive care. Severe cases may require blood transfusions when hemoglobin drops significantly (typically below 7 g/dL) or when symptoms of anemia become severe. Patients should carry a medical alert card or bracelet identifying their condition and maintain a list of medications to avoid. Regular follow-up with healthcare providers is recommended, especially during illness or when new medications are needed.
- Key points to consider in management:
- Avoidance of trigger medications and foods
- Screening for G6PD deficiency before starting oxidant drugs, as recommended by guidelines 1 and 1
- Discontinuation of triggering agents during acute hemolytic episodes
- Supportive care and monitoring of hemoglobin levels
- Potential need for blood transfusions in severe cases
- It is essential to prioritize the avoidance of triggers and to be aware of the potential for hemolytic crises when managing individuals with G6PD deficiency, as supported by the most recent study 1.
From the FDA Drug Label
WARNINGS Hemolytic anemia and G6PD deficiency Due to the risk of hemolytic anemia in patients with G6PD deficiency, G6PD testing has to be performed before using primaquine. In case of mild to moderate G6PD deficiency, a decision to prescribe primaquine must be based on an assessment of the risks and benefits of using primaquine If primaquine administration is considered, baseline hematocrit and hemoglobin must be checked before treatment and close hematological monitoring (e. g. at day 3 and 8) is required. Adequate medical support to manage hemolytic risk should be available. Discontinue the use of primaquine phosphate promptly if signs suggestive of hemolytic anemia occur (darkening of the urine, marked fall of hemoglobin or erythrocytic count)
For individuals with G6PD deficiency, the management and treatment recommendations are:
- G6PD testing must be performed before using primaquine.
- In cases of mild to moderate G6PD deficiency, a decision to prescribe primaquine should be based on an assessment of the risks and benefits.
- Baseline hematocrit and hemoglobin checks are required before treatment, along with close hematological monitoring.
- Adequate medical support should be available to manage hemolytic risk.
- Primaquine should be discontinued promptly if signs of hemolytic anemia occur. 2
From the Research
Management and Treatment Recommendations for G6PD Deficiency
- The most effective management strategy is to prevent hemolysis by avoiding oxidative stressors 3.
- Management for pain and anxiety should include medications that are safe and have not been shown to cause hemolytic crises, such as benzodiazepines, codeine/codeine derivatives, propofol, fentanyl, and ketamine 3.
- Patients with G6PD deficiency should avoid exposure to certain diets, drugs, and diseases that can trigger hemolysis 4.
- Medications that should be avoided in individuals with G6PD deficiency include dapsone, methylthioninium chloride (methylene blue), nitrofurantoin, phenazopyridine, primaquine, rasburicase, and tolonium chloride (toluidine blue) 5.
- Large neonatal screening programs can help detect cases of G6PD deficiency before the occurrence of acute hemolysis 4.
- Point-of-care testing for G6PD deficiency can provide opportunities for screening and inform care of newborns and malaria case management over their lifetime 6.
Safe Medications for G6PD-Deficient Patients
- Benzodiazepines, codeine/codeine derivatives, propofol, fentanyl, and ketamine have been found to be safe for use in G6PD-deficient patients 3.
- Nitrofurantoin, ciprofloxacin, glibenclamide, ofloxacin, phenazopyridine, sulfamethoxazole/cotrimoxazole, sulfasalazine, hydroxychloroquine, glimepiride, mesalazine, and sulfacetamide have been prescribed safely to G6PD-deficient patients in a real-world study 7.
Precipitating Factors for Hemolysis
- Diets, especially fava beans, falafel, chick pea, broad bean, green pea, pea nuts, lentil, and black eyed peas, are common precipitating factors for hemolysis in G6PD-deficient patients 4.
- Infections, such as pneumonia, tonsillitis, typhoid fever, hepatitis A, and urinary tract infection, can also trigger hemolysis 4.
- Certain medications, such as diclofenac sodium, ibuprofen, acetylsalicylic acid, co-trimoxazole, and nitrofurantoin, can cause hemolysis in G6PD-deficient patients 4.