Continued from the
previous posts.
G6PD (Glucose-6-Phosphate Dehydrogenase deficiency) is, apparently, the most common enzyme deficiency in humans, with an estimated 400 million people worldwide affected. It is most common in Blacks and inhabitants of the Mediterranean region, although Asians are also impacted.
It is sometime also called
Favism, as many people who have this condition are also allergic to fava beans.
Like
Sickle Cell Anemia, G6PD conveys a resistance to malaria, which is apparently why it became a genetic trait in the first place.
Unlike Sickle Cell Disease, G6PD usually has little impact on health. It can sit in a body for years and show nothing of itself until it is triggered by something.
The list of somethings is long, and, for me at least, unexpected: aspirin, mothballs, the above mentioned fava beans and their relations, certain antibiotics, all antimalarials, and much more can trigger an attack. See a list of drugs and foods to avoid
here. (Please see
this web page for information on Chinese herbs that should be avoided by G6PD deficient people.)
The attack itself is
hemolytic anemia that can cause hemoysis, the abnormal breakdown of red blood cells.
So, this diagnosis is, all-in-all, pretty good news.
First, it means his kidneys are functioning well. His urine is bloody because this is how his body is getting rid of the damaged red blood cells. The treatment for G6PD is blood, not only to replace damaged cells, but also to introduce a dose of the enzyme he is lacking.
The last time I spoke to his mom, he'd just finished receiving another unit of whole blood and was looking and feeling much better. The yellow aspect had dimmed considerably, and he was HUNGRY, and wolfed down a whole meal ... always a good sign in nine-year-old boys.
And the long-term outlook is good. If what triggered this bout of hemolysis can be ascertained, and avoided in the future, he should be fine. He may, however, pass the condition along to his children.
Passing the gene:
A) If the father is unaffected (healthy) and the mother is a carrier (no clinical symptoms):
• One daughter out of two will be a carrier
• One son out of two will be G6PD deficient
B) If the father is G6PD deficient and the mother is unaffected:
• All daughters will be carriers
• All sons will be unaffected
C) If the father is G6PD deficient and the mother is a carrier:
• One daughter out of two will be G6PD deficient
• One daughter out of two will be a carrier
• One son out of two will be G6PD deficient
• One son out of two will be unaffected
D) If the father is unaffected and the mother is G6PD deficient:
• All daughters will be carriers
• All sons will be G6PD deficient
•
E) If both father and mother are G6PD deficient:
• All daughters will be G6PD deficient
• All sons will be G6PD deficient
I have spent much of today finding all information I can and putting it in a form that lends itself to a simple Thai translation I found on the Internet ... I couldn't find any Thai medical sites, or not that I could recognize as such, anyway ... and emailing these to Mark in the less-than-50-word allotment the site affords. He'll print them up and take then to T's mother today.
I've been planning to get there myself, but had a call to fetch Sam from school. He's been vomiting all day now, so I don't think I'll be going anywhere.
Mark will phone me from T's bedside, and we'll speak. I'll explain as much as I can to him, and Mark will go through all the notes, English and Thai, with his mom.
What a week! And it's only Wednesday.
There is, in the real world, a simple blood test that will determine if a child has G6PD. Seems to me it would be a good idea to check for this in kids that could fit the profile, especially before giving any antimalarials if planning travel to a place where the parasite is rife.
PS: Mark just phoned from the hospital. T is up and walking around, far less yellow, eating like a horse and very ready to say
hasta la vista (his words) to hospitals. He will, however, fly to Singapore on Monday for a compete checkup, and where there are people who will be better able to explain his condition to his mother.
Mark said he was obviously disappointed that I wasn't there with him, but when it was explained that Sam was ill, he understood. In fact, when I spoke to him on the phone, he worried that Sam might have the same as him.
He's such a sweetie, and we love him to bits.