What foods should you avoid if you have G6PD deficiency?

G6PD deficiency is a hereditary disorder that results in altered metabolism of certain substances.
G6PD deficiency is a hereditary disorder that results in altered metabolism of certain substances.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency (G6PDD) is a genetic (hereditary) disorder. Therefore, you cannot prevent it. However, you can prevent the more serious G6PDD symptoms by avoiding certain foods.

  • Consumption of fava beans
  • Consumption of food cross-contaminated with fava beans 
  • Aniline dyes

You should be cautious about the consumption of food colored with a reddish orange agent (1-phenylazo-2-naphthol-6-sulphonic acid), which is even banned in many countries.

You should also avoid certain drugs that may trigger G6PDD, including 

What does G6PD deficiency mean?

Glucose-6-phosphate dehydrogenase (G6PD or G-6-PD) is an enzyme (a catalyst that regulates chemical reactions in the body) that is essential for assuring a normal lifespan for red blood cells and oxidative processes (chemical reactions that damage vital molecules in our cells).

G6PD deficiency (also known as favism or G6PDD) is hereditary, meaning it is passed down in families. It is caused by an abnormality in the activity of red blood cell enzymes. This enzyme deficiency may provoke the sudden destruction of red blood cells and lead to hemolytic anemia with jaundice. This may be triggered by consuming fava beans, certain legumes and medicines.

How do you get G6PD deficiency?

Glucose-6-phosphate dehydrogenase (G6PD or G-6-PD) deficiency (G6PDD) is a sex-linked trait, which means that the structure of the G6PD enzyme is carried by the X chromosome (an encoded hereditary material in the egg cells or womens’ reproductive cells). Therefore, this disorder is transmitted through the faulty genes of mothers (who are usually healthy carriers) to their son (or daughter who again becomes another healthy carrier ). Hence, men are more likely to suffer from this disorder than women.

There are more than 400 genes (units of heredity) that may cause this deficiency disorder. This disorder is most commonly seen in African countries (20 percent of the population is affected), around the Mediterranean (four to -30 percent of the population is affected) and Southeast Asia. More than 400 million people worldwide and about one in 10 African American men in the United States is affected by this disorder.

G6PD deficiency (G6PDD) can be determined by a simple blood test carried out at specialized genetic laboratories. To check hemolysis, your doctor may order

  • A complete blood cell count to evaluate your overall health
  • Lactate dehydrogenase enzyme levels to check for tissue damage
  • Serum haptoglobin levels to check red blood cell destruction
  • A urine analysis to check for blood cells in the urine
  • Bilirubin levels to check for the yellowish waste product in the blood
  • Urinary hemosiderin to check for excessive red blood cell destruction
  • Peripheral blood smear to check for hemoglobin damage

What is the problem and what can go wrong?

If glucose-6-phosphate dehydrogenase (G6PD or G-6-PD) enzyme is not available in your body, strong chemical products or toxins will not be neutralized and they may damage the hemoglobin (a protein that transports oxygen in the blood) inside the red blood cells. 

If toxins or chemicals build up in your body, it may cause breakdown of red blood cells and destroy them, which is known as hemolysis. This process may reduce the level of hemoglobin and red blood cells in your body, which is known as anemia.

Most individuals are asymptomatic. However, this hemolysis may make you severely anemic, which sometimes occurs very quickly. You may get

  • A sudden increase in body temperature
  • Jaundice (yellowish discoloration of the skin and mucous membranes)
  • Dark yellowish urine
  • Shortness of breath
  • Paleness, fatigue and malaise
  • Worsening of physical conditions
  • Weak and rapid pulse and heartbeat

Some infants may also be affected by jaundice due to G6PD deficiency (G6PDD), which is quite risky.

Your risk of hemolysis and severe anemia may increase if you have G6PDD with infectious diseases such as

Sometimes, it may lead to hemolytic crisis due to rapid breakdown of red blood cells in your body. This may require hospitalization and immediate blood transfusion.

Can G6PD be cured?

Yes. Fortunately, once you remove and avoid the foods and medicines that are triggering these symptoms in you, hemolysis usually stops within a short period of time. 

Your doctor will provide a detailed list of medicines and substances that may trigger hemolysis that you should avoid. 

If you have mild deficiency symptoms, then transfusion is usually not recommended. In case of a severe hemolytic crisis, your doctor will give you a blood transfusion of either whole blood or packed cells. In areas where glucose-6-phosphate dehydrogenase (G6PD or G-6-PD) deficiency (G6PDD) is very common, the doctor will avoid giving G6PD-deficient blood to patients.

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Medically Reviewed on 11/19/2020
References
Medscape Medical Reference

NIH


Indiana Hemophilia & Thrombosis Center


British Journal of Clinical Pharmacology