Is a milk formula containing soy protein safe for infants with Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency?

Milk Formula with Soy Protein is Safe for Infants with G6PD Deficiency

Soy-based, sugar-free formula is safe and actually recommended for infants with Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency. 1 In fact, the American College of Medical Genetics and Genomics specifically recommends soy-based, sugar-free formula as an appropriate feeding option for infants with G6PD deficiency.

Nutritional Management for G6PD Deficiency

Recommended Formula Characteristics

• Soy-based formula that is free of:
  • Sucrose
  • Fructose
  • Lactose
• Should be fed on demand every 2-3 hours in infancy 1

Why Soy Formula is Appropriate

• G6PD deficiency makes red blood cells vulnerable to oxidative damage, increasing susceptibility to hemolysis 2
• The primary dietary concern in G6PD deficiency is avoiding substances that trigger oxidative stress
• Soy protein itself is not among the known triggers for hemolysis in G6PD deficiency 3
• Fava beans are the only food conclusively linked to hemolytic risk in G6PD deficiency 3

Feeding Recommendations for Infants with G6PD Deficiency

Feeding Schedule

• Feed formula on demand every 2-3 hours during infancy
• As the infant grows, overnight gastric feedings may be necessary to prevent hypoglycemia 1
• Continuous monitoring of blood glucose levels is important to maintain levels ≥70 mg/dl 1

Introduction of Solid Foods

• Follow normal timeline (4-6 months)
• Begin with infant cereals, followed by vegetables and then meat
• Avoid fruits, juices, and other foods containing sucrose, fructose, and lactose 1

Important Considerations and Precautions

Monitoring Requirements

• G6PD deficiency is a major risk factor for severe hyperbilirubinemia 4
• Regular monitoring of bilirubin levels is essential, especially in the neonatal period
• Plot total serum bilirubin (TSB) levels on hour-specific nomograms to assess risk 4

Substances to Avoid

• Fava beans (most significant dietary trigger) 5
• Certain medications including:
  • Dapsone 1
  • Methylene blue 4
  • Diclofenac sodium, ibuprofen, acetylsalicylic acid 5
  • Co-trimoxazole and nitrofurantoin 5

Nutritional Supplementation

• Complete multivitamin with minerals is essential due to dietary restrictions 1
• Calcium with vitamin D supplements if not using calcium-fortified soy milk 1

Practical Implementation

• When selecting formula, ensure it is specifically labeled as soy-based and free of sucrose, fructose, and lactose
• Maintain consistent feeding schedule to prevent hypoglycemia
• Educate caregivers about:
  • Signs of hemolysis (pallor, jaundice, dark urine) 5
  • Foods and medications to avoid
  • Importance of prompt medical attention if jaundice develops 4

Soy formula is not only safe but recommended for infants with G6PD deficiency. The primary concern is avoiding known triggers of oxidative stress, particularly fava beans and certain medications, while ensuring adequate nutrition through appropriate formula selection and supplementation.