Hi,

Thanks for posting the query.

After going through your query, I would like to comment the following:

1. Your daughter's son has been diagnosed to be suffering from G6PD deficiency.

2. G6PD is the enzyme Glucose-6-phosphatase dehydrogenase.In affected individuals, a defect in an enzyme called glucose-6-phosphate dehydrogenase causes red blood cells to break down prematurely. This destruction of red blood cells is called hemolysis.

3. In people with glucose-6-dehydrogenase deficiency, hemolytic anemia is most often triggered by bacterial or viral infections or by certain drugs (such as some antibiotics and medications used to treat malaria). Hemolytic anemia can also occur after eating fava beans or inhaling pollen from fava plants (a reaction called favism).

4. Let me tell you the good news! : "A side effect of this disease is that it confers protection against malaria, in particular the form of malaria caused by Plasmodium falciparum, the most deadly form of malaria."

5. Stay away from the following chemical compounds:
-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)
* Also avoid fava beans
* Try to stay away from these ( ingestion or even touching).

6. Specific details about medicines to be avoided:
Antimalarial drugs that can cause acute haemolysis in people with G6PD deficiency include primaquine and chloroquine. There is evidence that other antimalarials may also exacerbate G6PD deficiency, but only at higher doses. Sulfonamides (such as sulfanilamide, sulfamethoxazole and mafenide), thiazolesulfone, methylene blue and naphthalene should also be avoided by people with G6PD deficiency, as should certain analgesics (such as aspirin, phenazopyridine and acetanilide) and a few non-sulfa antibiotics (nalidixic acid, nitrofurantoin, isoniazid, dapsone, and furazolidone) XXXXXXX has been known to cause haemolytic crisis in G6PD-deficient infants.

7. As far as the advanced milk formula is concerned , I would like to mention the point that Soy products trigger symptoms of G6PD deficiency. Since the formula contains soy product, it needs to be avoided as far as possible.

8. Thus food products to be avoided are:

*Fava Beans. This is the number one “no-no” food for the child and has been proven to trigger the symptoms. This is so related to the deficiency that G6PD may be sometimes referred to as “favism” in reference to this food.
*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.
*Peanuts. Peanuts (in particular) and legumes (in general) are found everywhere, including peanut butter, cereals, sauces and salad dressings. Legumes are vegetables and are, supposed to be, in general, good for ones’ health. Peanuts are known as “brain food” and are becoming more popular with recently-discovered antioxidant qualities. But for G6PD cases, avoiding these foods is highly recommended.
*Menthol-Flavored Candies and Food. People with G6PD are not advised to not only eat menthol-flavored candies and food, they are not allowed to inhale this substance as well! Menthol can be found in various candies, in mouthwashes, toothpaste and gums. For children with G6PD, it is doubly hard to avoid menthol-flavored candies but avoiding these is not a matter of choice for them but a matter of survival.
*Avoid food with atrificial colouring agents in them.
*blue berries, red wine to be avoided.

9. Please do not be disheartened as "Glucose-6-phosphatase dehydrogenase (G6PD) deficiency is the most common disease-producing enzymopathy in humans."

I hope I have answered your query. I will be glad to answer any follow up queries.

Regards
Dr. Gyanshankar Mishra
MBBS MD DNB

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Hi,

Thanks for the follow up.

After going through your query, I would li8ke to comment the following:

1. The triggers mentioned above have the potential to cause hemolytic anemia. However it does not mean that these trigger will cause hemolytic anemia every time in all affected individuals. But this does imply that one should stay away as far as possible from these after the diagnosis is at hand.

2. In the United States, G6PD deficiency is most common in African Americans.G6PD deficiency is passed along in genes from one or both parents to a child. Yes, research has shown that it tends to be milder in african american. This means that the chances of developing hemolytic anemia are less in this subgroup of patients.

3. This should be kept in consideration when planning the marriages of these patients in future. Pre-marital medical and genetic counseling is important in these patients. And also their children need to be screened for g6pd deficiency at birth.

I hope I have answered your query. I will be glad to answer any follow up queries.

Regards
Dr. Gyanshankar Mishra
MBBS MD DNB

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