IHTC NEWS:

Other Hematological Disorders

• Autoimmune Hemolytic Anemia
• G6PD
• Chronic Neutropenia
• Hemochromatosis
• Hereditary Spherocytosis
• Myelodysplastic Syndrome
• Qualitative Platelet Disorders
• Thalassemia
• White Blood Cell Disorders

G6PD

What is G6PD?

Glucose-6-phosphate Dehydrogenase 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 is especially important in red blood cells.  These are the cells that contain hemoglobin, which carries oxygen throughout the body. The G6PD enzyme protects the red blood cells from chemical products or toxins, which can accumulate in your body during certain illnesses or after taking certain medicines.

What does G6PD Deficiency Mean?
People with G6PD deficiency do not have enough G6PD enzyme to neutralize some chemical products or toxins. Normally, there are only tiny amounts of toxins or chemical products in the body, so it is usually not a problem for the G6PD enzyme to render them harmless.

So what is the problem?
If strong chemical products are not neutralized, damage to the hemoglobin inside tile red blood cells can occur. An accumulation of toxins or chemical products can cause the red blood cells to break apart and be destroyed. This makes the person anemic. Sometimes the anemia can happen very quickly, as if the person suddenly lost a lot of blood after an accident.

When people take certain medications, some of these toxic products can form in the body. When people with G6PD deficiency, take these same medications, their red blood cells can be damaged or destroyed by the compounds.

The process of the red blood cells with hemoglobin being destroyed is called hemolysis. This leads to severe anemia, and can cause yellow jaundice from the buildup of hemoglobin breakdown products in the blood.

You will be provided with a detailed list of medicines and substances which are known to cause hemolysis problems for people with G6PD deficiency.

How did my child get G6PD deficiency?
G6PD is usually is inherited. This means it is passed on in families from birth. It is I usually passed on from mothers to sons. Usually the mother has no symptoms of G6PD and has no way of knowing that she is a carrier of G6PD deficiency. G6PD deficiency is most commonly found in boys, but can occur in girls. It is found in many different ethnic populations.

There is no way to prevent G6PD deficiency, but fortunately, if exposure to certain medicines is avoided, it is a condition in which serious symptoms can also be avoided.

In some individuals with G6PD, a small amount of red blood cell destruction occurs all of the time. This form of G6PD is called chronic Hemolysis.

The body's blood cell producing factory is in the bone marrow. This is the red, soft, spongy part inside the bones. Usually a person with chronic Hemolysis simply makes more than average amounts of red blood cells (hemoglobin) to make up for the ones being constantly destroyed. This reved-up red cell production can be measured by 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 hemoglobin.

If your child has the form of G6PD associated with chronic Hemolysis, your doctor may prescribe a medication called folic acid. This is a vitamin supplement to help the bone marrow keep up with its high production demands.

What can I do?
The only way to know if a person has G6PD deficiency is with a special blood test. Remember, G6PD deficiency is not usually a problem. It only becomes a problem if the person is exposed to chemical compounds or toxins, which can cause Hemolysis and lead to severe anemia.

If your child is ill with certain infectious like Fifth's disease (Parvovirus), infectious hepatitis, infectious mononucleosis (Mono), pneumonia or a blood infection, there is an increased risk of Hemolysis and severe anemia.

The symptoms of Hemolysis and the resulting anemia are: tiredness, paleness, headache, yellow tint to the whites of the eyes or skin (jaundice or icterus), constant fast heart beat, shortness of breath, dark urine (tea or coke colored).

Report any of these symptoms to your doctor or nurse promptly.

Any time your child is taking any medication, whether prescribed by a doctor or over-the-counter, tell your doctor or pharmacist that your child has G6PD deficiency to be sure that it is safe.

Acetometaphen (Tylenol), lbuprofen (Advil), Pseudoephedrine (Sudafed), and Guaifenesin, (Robitussin) are OK to use for children with G6PD deficiency. These are common over-the-counter treatments for fever, pain, nasal congestion and cough.

The most common over-the-counter medicines that people with G6PD deficiency should avoid contain aspirin (salicylic acid) or phetiacetin.

AVOID:

• Aspirin
• Bufferin
• Anacin
• Pepto Bismol
• Excederine
• Empirin
• Coricidin

Other things to AVOID: Moth balls and fava beans (a type of bean found in the Mediterranean).

Prescription drugs to AVOID:

• Antimalarials:
• Primaquine
• Pamaquine
• Pentaquine
• Plasmoquine
• Quinidine
• Quinine

Sulfonamides:

• Septra/Bactrim/Gantrisin (Sulfisoxasole)
• Sulamyd
• Sulfanilamide
• Sulfapyridine
• Dapsone
• Salicylazosulfapyridine (Azulfidine)
• Sulfasalazine
• Nitrofurans:
• Nitrofurantoin (Furadantin)
• Furazolidone (Furoxone)
• Furaltadone (Altafur)

Phenothiazines:

• Theorizing
• Comparing
• Phonogram

Thiazine Diuretics:

• Furosimide (Lasix)

Others:

• Chloramphenicol
• Negram
• Acetylphenylhydrazine
• Chloroquine
• Orinase     
• Methylene blue
• Dimercaprol
• Probenecid
• Phenylhydrazine  
• Sulfoxone
• Naldixic acid
• Penicillamine