G6PD Deficiency

What is G6PD?
G6PD deficiency is the lack of glucose-6-phosphate dehydrogenase, an enzyme present in red blood cells, which can cause a type of anemia known as hemolytic anemia. Red blood cells carry oxygen in the body and G6PD protects these cells from natural oxygen chemicals (oxidative substances) that may build up. This may occur when you have a fever, take certain medications like pain medicines, certain antibiotics, and drugs to treat malaria, or when you are exposed to other specific substances, such as mothballs or fava beans. If there are too many of these chemicals, they can destroy the red blood cells prematurely.

The World Health Organization classifies G6PD genetic variants into five classes, the first three of which are deficiency states.

1. Severe deficiency - with chronic (nonspherocytic) hemolytic anemia
2. Severe deficiency - with intermittent hemolysis
3. Mild deficiency - hemolysis with stressors only
4. Non-deficient variant, no clinical sequelae
5. Increased enzyme activity, no clinical

Our son Reagh has been identified as class 3 mild deficiency, meaning an improper diet or medication can trigger hemolysis.

How is it treated?
Treating the symptoms associated with G6PD deficiency is usually as simple as removing the trigger. Many substances are potentially harmful to people with G6PD deficiency. Variation in response to these substances makes individual predictions difficult. It is important to avoid the foods and drugs below.

Foods to Avoid:
The following list notes the most common foods to avoid.

• Fava Beans. This is the number one “no-no” food for the child and has been proven to trigger the symptoms. 
• Legumes. Beans, black beans, etc., this is another group of foods that must be avoided by children / adults with G6PD.
• Soya or Soy. This is an additive to some common food that we eat, including ice cream, burgers, pizzas, chocolate, doughnuts and other food products. It may go by different names but it is definitely one of those food or food additives that should be avoided if one has G6PD.
• Peas such as green peas, field peas and black-eyed peas, beans such as black beans or refried beans and edible pods such as Chinese pea pods and snow peas.
• Peanuts. Peanuts (in particular) and legumes (in general) are found everywhere, including peanut butter, cereals, sauces and salad dressings.
• Menthol-Flavored Candies and Food. People with G6PD are not advised to not only eat menthol-flavored candies and food. Menthol can be found in various candies, in mouthwashes, toothpaste and gums. 
• Artificial blue food coloring - other artificial food color can also cause hemolysis. Natural food color such as found in foods like turmeric or grapes is okay.
• Blueberries and products containing them, like blueberry yogurt.

Drugs to Avoid:

• NSAIDS (Asprin, Ibuprophen)
• Tylenol
• Quinolones
• Drugs metabolized through the liver or known to cause blood or liver related problems or hemolysis
• Sulfa drugs
• Petrochemically derived substances (This is a long list and gets longer every year. Many artificial foods, dyes and vitamins are included in this list.)
• Moth Balls and anything containing naphthalene.
• Artificial Food Coloring (Methylene and Toluidine blue)

Don't Panic.
G6PD is not an allergy, so if a child consumes any of the above in most cases it generally will not put them in any immediate danger. An improper G6PD diet is more comparable to poison, large doses will make the child ill. Regular avoidance is the best practice.

G6PD Sources: 

• http://emievil.hubpages.com/hub/Forbidden-Food-for-G6PD-Cases
• http://g6pddeficiency.org/wp/living-with-g6pd-deficiency/g6pd-deficiency-foods-to-avoid-list/
• http://www.homemade-baby-food-recipes.com/foods-for-a-baby-with-G6PD-deficiency.html
• http://www.utoronto.ca/kids/G6PD.htm
• http://en.wikipedia.org/wiki/Glucose-6-phosphate_dehydrogenase_deficiency