All our babies will be tested for G6PD deficiency right after birth and this is stated in a card along with their blood group. So, do you have idea of what is G6PD?

G6PD is short for ‘glucose-6-phosphate dehydrogenase’ which is a chemical that found throughout the body. In fact, G6PD is one of many enzymes that help the body process carbohydrates and turn them into energy which is also protects red blood cells from potentially harmful byproducts that can accumulate when a person takes certain medications or when the body is fighting an infection. Some people have less than the usual amount of G6PD in their red blood cells. This is called G6PD deficiency and it may occasionally cause problems.

It is an inheritable disease. Consanguinity plays a major role in the incidence of this disease. It is an XLinked –recessive disease, affecting males more than females, also categorized as a sex-linked disorder that usually found in the newborn babies.

Most people with G6PD deficiency don't have any symptoms, while others develop symptoms of anemia only after RBCs have been destroyed, a condition called hemolysis. For a child with G6PD deficiency that is exposed to a medication or infection that triggers the destruction of RBCs may have no symptoms at all. In more serious cases, a child may exhibit symptoms of anemia (also known as a hemolytic crisis), including:

- paleness (in darker-skinned children paleness is sometimes best seen in the mouth, especially on the lips or tongue)

- extreme tiredness

- rapid heartbeat, rapid breathing or shortness of breath

- jaundice, or yellowing of the skin and eyes, particularly in newborns

- an enlarged spleen

- dark, tea-colored urine

Once the trigger is removed or resolved, the symptoms of G6PD deficiency usually disappear fairly quickly, typically within a few weeks. If symptoms are mild, no medical treatment is usually needed. As the body naturally makes new red blood cells, the anemia will improve. If symptoms are more severe, a child may need to be hospitalized for supportive medical care.

Problems for children and adults
Most people with G6PD deficiency have completely normal health. However, problems can occur occasionally. These problems are much more common in boys and men with G6PD deficiency than girls and women. Some children and adults with G6PD deficiency may develop haemolysis and anaemia if they get a fever or if they take one of the medicines listed below. Some people may develop haemolysis and anaemia if they eat broad beans (this is called favism). An affected person feels unwell, becomes pale and jaundiced (yellow) and may have a backache and pass dark urine.

Problems for newborn babies
If a mother carries G6PD deficiency, she may pass it on to some of her children and some may get jaundiced (yellow) in the newborn period. This is particularly likely for baby boys. Severe jaundice can be serious for newborn babies if it is not treated. It is usually treated by putting the baby under a special light for a few days. Many babies with G6PD deficiency are diagnosed because they have jaundice soon after birth. Once the jaundice has passed they should not get other problems from G6PD deficiency provided they avoid broad beans.

The following medicines can sometimes cause problems for people with G6PD deficiency and should be avoided if possible
1) For malaria avoid:

Maloprim (contains Dapsone), Primaquine, Pentaquine and Pamaquine

2) For other infections avoid:

Nalidixic acid, Nitrofurantoin, Sulphonamides (some, including Co-trimoxazole = Septrin®, Bactrim®) and Dapsone

3) Avoid close contact with:

Moth balls (Naphthaline)

4) Avoid eating:

Fava (Broad) beans and Chinese herbal medicines

5) For malaria avoid:

Maloprim (contains Dapsone), Primaquine, Pentaquine and Pamaquine

In most cases, cases of G6PD deficiency go undiagnosed until a child develops symptoms. If doctors suspect G6PD deficiency, blood tests usually are done to confirm the diagnosis and to rule out other possible causes of the anemia. If you feel that your child may be at risk due to your family history or your ethnic background, talk to your doctor about performing a screening with blood tests to check for G6PD deficiency is advisable.

With the right precautions, a child with G6PD deficiency can lead a healthy and active life.