G6PD

Conditions We Treat Additional Blood Disorders G6PD

A genetic deficiency that can lead to anemia

Glucose-6-phosphate dehydrogenase (G6PD) is a substance that helps the body use sugar for energy. It is an enzyme,
which means it speeds up chemical changes when the body converts sugar to energy.

The G6PD enzyme is found in all the body’s cells, but it is especially important in red blood cells. These are the cells that
contain hemoglobin and carry oxygen throughout the body. The G6PD enzyme protects the red blood cells from
harmful chemicals or toxins, which can build up in your body during certain illnesses or after taking some medicines.

People with G6PD deficiency do not have enough G6PD enzyme to neutralize certain chemicals or toxins. Normally,
there are only tiny amounts of toxins in the body, and so it is not usually a problem for the G6PD enzyme to make them
harmless.

So, what is the problem?

If G6PD is not present, strong chemical products are not neutralized and they can damage the hemoglobin inside red
blood cells. A build-up of toxins or chemicals can cause the red blood cells to break apart and be destroyed (also known
as hemolysis). This makes a person anemic (reduced levels of hemoglobin and red blood cells). Sometimes the anemia
can happen very quickly.

Hemolysis may lead to severe anemia and can cause jaundice (a yellowing of the skin) from the build-up of hemoglobin
breakdown products in the blood. Some infants who experience jaundice have G6PD deficiency.

The IHTC clinical team will provide a detailed list of medicines and substances that are known to “trigger” hemolysis and
which should be avoided.  Fortunately, once the cause or trigger is removed, hemolysis usually stops within a short
period of time.

CAUSE OF G6PD DEFICIENCY

G6PD deficiency is a genetic condition that is caused by an alteration (a mutation or a change) in the G6PD gene. This
mutation prevents the production of the normal enzyme. The mutation is passed on in families in an X-linked recessive
pattern. This means that the gene that codes for the G6PD enzyme is located on the X-chromosome; males have one X
chromosome and females have two. When a male inherits a gene for G6PD that is not functioning properly, he will have
G6PD deficiency. When a female inherits a gene for G6PD deficiency she is referred to as a carrier because she has a
second normal copy of the gene (from the other parent) that can often compensate for the altered gene. Female
carriers may or may not experience symptoms of G6PD deficiency. 
Carriers of G6PD deficiency have a 50% chance of passing the altered gene on to each of their children. Each son of a
carrier has a 50% chance of having G6PD deficiency and each daughter has a 50% chance of being a carrier.  Men with
G6PD deficiency do not pass the gene on to any of their sons; however, all of their daughters will be carriers. Often a
mother has no symptoms and has no way of knowing that she is a carrier of G6PD deficiency. Carrier testing in families
who are known to have G6PD deficiency is available at the IHTC if desired.

G6PD deficiency can occur in a person from any ethnic group. In the United States it is most commonly found in people
of African American, Mediterranean (Italian or Greek), Southeast Asian, or Middle Eastern descent. 

DIAGNOSIS OF G6PD DEFICIENCY

The only way to know if a person has G6PD deficiency is to perform a special blood test as G6PD deficiency does not
usually cause problems, except in rare circumstances (see section below under “chronic hemolysis”). Many people can
go through life without any symptoms. It only becomes a problem if the person is exposed to certain chemical
compounds or toxins, or experiences certain infections that can cause hemolysis.

People with G6PD deficiency have an increased risk of hemolysis and severe anemia if they have certain infections such
as Fifth disease (parvovirus), infectious hepatitis, infectious mononucleosis (mono), pneumonia, or a blood infection.

CHRONIC HEMOLYSIS
In some people with G6PD deficiency, a small amount of red blood cell destruction occurs all the time. This process is
known as “chronic hemolysis”.

Usually a person with chronic hemolysis makes more than the average amount of red blood cells to make up for the
ones that are being constantly destroyed. This increased rate of red blood cell production can be measured using a
blood test called a reticulocyte (or “retic”) count. The retic count is usually elevated in a person with chronic hemolysis.
This means the body is keeping up with the demands for red blood cells and hemoglobin.

For persons with the form of G6PD deficiency that is associated with chronic hemolysis, their doctor may prescribe a
medication called folic acid. This vitamin supplement helps the bone marrow keep up with the increased production
demands needed to make the extra red blood cells. The symptoms of hemolysis and the resulting anemia may include
tiredness, paleness, headache, yellow tint to the whites of the eyes or skin (jaundice or icterus), fast heartbeat,
shortness of breath, or dark urine (tea or coke colored). Individuals with these symptoms should inform their healthcare
provider promptly.

SPECIAL CONSIDERATIONS: WHAT TO AVOID

You cannot prevent G6PD deficiency, but fortunately, if you avoid exposure to certain medicines and foods, you can
prevent the more serious symptoms. Fava beans is one of the foods you should always avoid. 

Medications: What to Take, What to Avoid

Any time persons with G6PD take any medication, whether prescribed by a doctor or over-the-counter, they should tell
their doctor or pharmacist that they have G6PD deficiency to be sure that it is safe to take the medication.

The common over-the-counter treatments for fever, pain, nasal congestion and cough – acetaminophen (Tylenol),
ibuprofen (Advil), pseudoephedrine (Sudafed), and guaifenesin, (Robitussin) – are usually safe at normal therapeutic
doses for people with G6PD deficiency. People with G6PD deficiency should avoid medicines that contain aspirin
(salicylic acid).

Common Over-the-Counter Medications to AVOID:

 Anacin

Aspirin

Bufferin

Ecotrin

Empirin

Excedrin

Pepto Bismol

Common Prescription drugs to AVOID:

Antimalarials:
Chloroquine (Aralen)

Mefloquine (Lariam)

Pamaquine

Primaquine

Quinidine

Quinine

Sulfonamides and Sulfones:

Dapsone

Furosemide (Lasix)

Sulfacetamide (Sulamyd)

Sulfamethoxazole (Bactrim, Septra)

Sulfanilamide

Sulfasalazine (Azulfidine)

Sulfisoxazole (Gantrisin)

Nitrofurans
Nitrofurantoin (Furadantin, Macrobid, Macrodantin)

Quinolones and Fluoroquinolones:

Ciprofloxacin (Cipro)

Levofloxacin (Levaquin)

Moxifloxacin (Avelox, Vigamox)

Norfloxacin (Noroxin)
OTHER PRODUCTS TO AVOID:
Acetylphenylhydrazine

Beta-Naphthol

Chloramphenicol

Dimercaprol

Fava beans (a type of bean found in the Mediterranean)

Glyburide (Diabeta)

Henna

Menthol

Methylene Blue (a dye)

Moth balls (or anything containing naphthalene)

Nitrofurazone (Furacin)

Penicillamine

Phenazopyridine

Phenylhydrazine

Probenecid

Rasburicase (Elitek)

Tolbutamide (Orinase)

Toluidine Blue (a dye)

LEARN ABOUT ALL THE FOODS AND MEDICATIONS TO AVOID

HOW THE IHTC CAN HELP YOU

The IHTC is committed to providing expert care for infants, children, teens, and adults with G6PD. As a center of
excellence in the treatment of bleeding and clotting disorders, the IHTC offers a high level of coordinated care for
patients with this disorder. We also provide prompt communication with your local healthcare provider related to our
treatment plan and your progress. The IHTC team collaborates with national experts and patient participation in
research studies may be available.

At the IHTC, we have clinicians who are experts in treating G6PD and providing support to patients with this disorder. As
patients with G6PD must avoid certain foods and medicines, it is very important that you are seen by a clinician who is
familiar with the signs and treatment of G6PD. We can help you with diagnosis, testing and treatment; and can provide
expert dietary guidance suitable for your specific needs.

If you live in Indiana and need more information, please contact the IHTC.

The IHTC will not provide advice or information about G6PD to individuals who reside outside of Indiana.
 If you are in the U.S. but not in Indiana:
FIND AN HTC NEAR YOU

If you are outside of the U.S.:

FIND AN HTC NEAR YOU

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