How Pax died

The unspoken question is "how did Pax die", only 3 years old, in India.

He suffered from a genetic disorder which is relatively rare. His condition is called "Deficit in G6PDD". It causes acute hemolytic anemia. I didn't understand much about it in those days, and it was before the internet where you can find out information at your fingertips.

We first discovered he had it when travelling through France (spring 1980). He was about 15 months old. It was 1980. He appeared to be suffering from anemia, so we took him to the hospital in Aix-en-Provence, and they kept him in for what seemed like ages. He was given a blood transfusion. In the end, they gave us the diagnosis and told us he should avoid eating fava beans and some other substances. He had a mild bad turn a few months later, and then he was fine. Catherine was born, we ended up travelling back through Europe and then going to India. As far as I knew, we had it completely under control; I guarded his food intake with a vengeance.

Here's Pax a couple of months before he died, on a trip with his father and a few friends to Goa. (Another bit of life's symetry: we're going to Goa after we visit Bhopal.) As you can see, he looked fine and we weren't worried about his health at this point.

However, in May 1982, while on a train journey from Lucknow to Bombay, he started to go yellow. That was the indicator he was having a hemolytic anemia episode. (From my understanding, his white blood cells were destroying the red cells.) We got off the train at the next big city: Bhopal. We rushed him to the hospital, but despite a transfusion, it was too late. His blood count had gone too low, and we lost him.

I was too heartbroken for words. Due to circumstances (first husband being a major factor), I did not work through my grief and it was only after I lost my daughter Catherine that I started facing what had happened with Pax--29 years later!!

So last year I finally did some research into this disorder.

I got in touch with an expert and here's some of what he wrote to me.

"When I was 8 I hemolyzed badly and I do not know why to this day. We were getting out of church when I

complained of not feeling good. My mother knew what to look for and told my father to get me to the hospital, which was 30 miles away.

By the time we got there, I was too weak to walk and had to be carried into the hospital. My mother physically forced the doctor to give me blood and, obviously, I lived, but it was a very close call.

Hemolysis can be very severe and quick when the conditions and trigger are right. Moth balls are a huge trigger and hemolysis is usually very severe and quick. My cousin died before they could get him to the hospital from moth ball exposure.

Fava beans are another huge trigger. Most other legumes are less severe and some are rather mild triggers, but triggers none the less. Infections can also be triggers, and when coupled with another trigger can be very severe.

G6PDD was first recognized and named in 1956, the same year I had my first episode.

The [person]  who discovered it was bought out by the pharmaceutical company who financed his work and published a very watered down version of what to avoid and how many people were affected. . . . The real number of people who have died from G6PDD or its complications is unknown because many of the people who die are misdiagnosed or the cause of death is reported as  unknown. We don't even know how far reaching G6PDD really is and what all it affects. All the medical industry will admit to is hemolysis from triggers. But G6PDD is in every cell in our bodies and we don't know what all it affects.

Twenty nine years ago we still believed what Beutler published and we believed that he was an honest man. [This is referring to when we lost Pax.] We had no idea how many things cause hemolysis. ... Your son had three or four [episodes]. Because of our lack of knowledge, he could have had a reaction to something no matter where he was. In fact, the probability of problems is more likely in the civilized world because of all the junk we put in our food."

Knowing more about the condition doesn't bring Pax back, but at the least, now I understand a little more of  what happened.