G6PD Deficiency

 

Glucose-6-phosphate dehydrogenase deficiency

Glucose-6-phosphate dehydrogenase deficiency is a genetic disorder that occurs most often in males. This condition mainly affects red blood cells, which carry oxygen from the lungs to tissues throughout the body. 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.

The most common medical problem associated with glucose-6-phosphate dehydrogenase deficiency is hemolytic anemia, which occurs when red blood cells are destroyed faster than the body can replace them. This type of anemia leads to paleness, yellowing of the skin and whites of the eyes (jaundice), dark urine, fatigue, shortness of breath, and a rapid heart rate. 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).

Glucose-6-dehydrogenase deficiency is also a significant cause of mild to severe jaundice in newborns. Many people with this disorder, however, never experience any signs or symptoms.

Causes

G6PD deficiency occurs when a person is missing or doesn't have enough of an enzyme called glucose-6-phosphate dehydrogenase, which helps red blood cells work properly.

Too little G6PD leads to the destruction of red blood cells. This process is called hemolysis. When this process is actively occurring, it is called a hemolytic episode. The episodes are usually brief, because the body continues to produce new red blood cells, which have normal activity.

Red blood cell destruction can be triggered by infections, severe stress, certain foods (such as fava beans), and certain drugs, including:

• Antimalarial drugs
• Aspirin
• Nitrofurantoin
• Nonsteroidal anti-inflammatory drugs (NSAIDs)
• Quinidine
• Quinine
• Sulfa drugs

Other chemicals, such as those in mothballs, can also trigger an episode.

In the United States, G6PD deficiency is more common among blacks than whites. Men are more likely to have this disorder than women.

You are more likely to develop this condition if you:

• Are African American
• Are of Middle Eastern decent, particularly Kurdish or Sephardic Jewish
• Are male
• Have a family history of the deficiency

A form of this disorder is common in whites of Mediterranean descent. This form is also associated with acute episodes of hemolysis. Episodes are longer and more severe than in the other types of the disorder.

Symptoms

Persons with this condition do not display any signs of the disease until their red blood cells are exposed to certain chemicals in food or medicine, or to stress.

Symptoms are more common in men and may include:

• Dark urine
• Enlarged spleen
• Fatigue
• Pallor
• Rapid heart rate
• Shortness of breath
• Yellow skin color (jaundice)

Exams and Tests

A blood test can be done to check the level of G6PD. See: G6PD screen

Other tests that may be done include:

• Bilirubin level
• Complete blood count, including red blood cell count
• Hemoglobin - blood
• Hemoglobin - urine
• Haptoglobin level
• LDH test
• Methemoglobin reduction test
• Reticulocyte count

Treatment

Treatment may involve:

• Medicines to treat an infection, if present
• Stopping any drugs that are causing red blood cell destruction
• Transfusions, in some cases.
• Avoiding foods and medications that can trigger the condition.
• Reducing stress levels

Nutrition Therapy

Some of these foods to avoid are commonly eaten around the world and can cause people with G6PD . Deficiency to hemolyze and can even be deadly (like fava beans). Some others can cause low level hemolysis, which means that red blood cells die, but not enough to cause the person to go to the hospital. Low level hemolysis over time can cause other problems, such as memory dysfunction, overworked spleen, liver, kidney and heart, and iron overload. Even though a G6PD Deficient person may not have a crises when consuming these foods, they should be avoided.

Fava Beans and other legumes

Our list of legumes contains every legume we could find, but there may be other names for them that we do not know about. Low level hemolysis is very hard to detect and can cause other problems, so we recommend the avoidance of all legumes.

Sulfites

and foods containing them. Sulfites are used in a wide variety of foods, so be sure to check labels carefully.

Menthol

and foods containing it. This can be difficult to avoid as tooth paste, candy, breath mints, mouth wash and many other products have menthol added to them. Mint from natural mint oils is alright to consume.

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.

Ascorbic acid

Artificial ascorbic acid commonly put in food and vitamins can cause hemolysis in large doses and should be avoided. It is put into so many foods that you can be getting a lot of Ascorbic Acid without realizing it.

See Ascorbic Acid for more information about iron absorbtion and ascorbic acid.

Vitamin K

This is from drugbank: “Menadione (vitamin K3), which is not used as a nutritional supplemental form of vitamin K for humans, has been reported to cause adverse reactions, including hemolytic anemia. Large doses have also been reported to cause brain damage. Vitamin K administered to newborns with G6PD Deficiency has been known to cause adverse outcomes including hemolytic anemia, neonatal brain or liver damage, or neonatal death in some cases.”

Tonic water

(contains quinine, a contraindicated drug which causes hemolysis in G6PDD people).

Gluten Free Products

Some of these, like flour, etc. have fava beans in them. Be sure the check the label for legumes before you purchase of eat them.

Bitter Gourd and Garden Egg

Bitter Gourd is also known as Bitter Mellon. These are common foods in some parts of Africa and Asia.

Some Chinese herbs

particularly Rhizoma Coptidis (huang lien), Calculus Bovis (neu huang), Flos Chimonanthi Praecocis (leh mei hua), Flos Lonicerae (kam ngan fa) and Margarita or anything containing them.

Black and Green Tea and extracts

Research has shown that these products significantly reduce reduced glutathione levels among other things in people with G6PDD. See article 55 on the references page.