just saw this from my files. this is an excerpt from the UP seminar leaflet.
What is G6PD deficiency?
Glucose – 6 – Phosphate dehydrogenase deficiency, or G6PD deficiency for short, is the most common enzyme deficiency worldwide. This is an inherited disorder which means that from the time a baby is born, there is already something wrong with how his body makes and breaks important substances. According to statistics, about 400 million people have G6PD deficiency, and it is most common in Africa, Southeast Asia and the Middle East.
Babies with G6PD deficiency have very little or no enzyme called Glucose – 6 – Phosphate dehydrogenase. An enzyme is a kind of protein that speeds up chemical reactions in the body. The enzyme G6PD is especially important to red blood cells. If this enzyme lacking or missing, red blood cells are easily destroyed.
Another name for G6PD deficiency is favism because some people who have it, usually those living in the Mediterranean region, react very badly after ingestion of fava beans.
What are the harmful effects of G6PD deficiency?
A baby with G6PD deficiency appears and remains healthy until he is exposed to a large amount of oxidative substances. When this happens, his red blood cells are destroyed, a process known as HEMOLYSIS.
Red blood cells carry oxygen to all parts of the body. When they undergo hemolysis, the baby will have hemolytic anemia. The signs and symptoms of hemolytic anemia are paleness, dizziness, headache, tea-colored urine, and abdominal or back pain or both. Hemolytic anemia, when very severe, can end in death. Destroyed red blood cells are brought to the liver to be broken down to smaller pieces for disposal. One of the end products is bilirubin, a yellowish substance that accumulates in different parts of the body when too much is produced. Quite often, bilirubin accumulates in the skin and causes it to appear yellowish. In worst case, bilirubin accumulates in the brain and causes mental retardation or death.
As parent, what should I do to prevent a hemolytic crisis?
Tell your child’s pediatrician that your child has G6PD deficiency. This is very important so that he will not prescribe oxidative drugs in case your child gets ill.
Keep your list of oxidative substances in a handy place. Better yet, post it in a convenient spot on the kitchen wall. Always double-check food, beverage and medicine labels against the list.
Memorize the signs and symptoms of hemolytic anemia. Bring your child to his pedia as soon as these signs and symptoms appear.
Do not ignore infections.
As your child gets older, honestly and gently tell him about his condition and teach him to be careful about what he eats.
