Foods to Avoid with G6PD Mediterranean Deficiency
Patients with G6PD Mediterranean deficiency must strictly avoid fava beans (broad beans) as they are the most potent trigger of acute hemolytic crisis, with a 10% mortality rate if untreated. 1
Primary Food Avoidance: Fava Beans
- Fava beans (broad beans) are absolutely contraindicated in all forms—fresh, dried, cooked, or processed—as they contain oxidants that trigger severe intravascular hemolysis in G6PD-deficient individuals 1, 2
- Acute hemolysis typically occurs within 24-72 hours after ingestion, with hemoglobin levels potentially dropping to dangerously low levels (e.g., 6.5 g/dL) 1, 3
- This condition, known as favism, represents one of the most important oxidant exposures for G6PD-deficient patients 1
Understanding the Mechanism
- G6PD deficiency affects over 400 million people worldwide, with the Mediterranean variant being particularly severe 2
- The enzyme deficiency causes insufficient reducing power against toxic peroxides and free radicals, making red blood cells vulnerable to oxidative damage 1, 2
- While patients are typically asymptomatic under normal conditions, exposure to oxidants—particularly from fava beans—triggers acute hemolytic crises 1, 4
Additional Oxidant-Containing Foods to Limit
Beyond fava beans, patients should exercise caution with other potential oxidant sources, though these are less consistently problematic:
- Legumes related to fava beans should be approached cautiously, though they are generally less problematic than fava beans themselves 2
- The primary management strategy is preventing hemolysis by avoiding oxidative stress from any source 2, 4
Critical Clinical Considerations
- Neonatal screening is essential in regions with high G6PD deficiency prevalence to prevent diagnostic delays and enable early parental education 3
- Parents and patients require comprehensive therapeutic education sessions focused on products to avoid 3
- The disorder is X-linked recessive, occurring much more frequently in males, though females can be affected and should not be overlooked diagnostically 3, 4
- Most G6PD-deficient individuals show no clinical abnormality under normal conditions, making prevention through avoidance the cornerstone of management 4
Practical Management Approach
- Maintain strict avoidance of all fava bean products throughout life 1, 2
- Educate family members about hidden sources of fava beans in ethnic foods and processed products 3
- Ensure all healthcare providers are aware of the diagnosis, as certain medications also trigger hemolysis in G6PD deficiency 2
- In case of accidental exposure, seek immediate medical attention for potential blood transfusion and supportive care 1