Wednesday, May 07, 2003

Time has no meaning when you're waking up every three hours. But this is hardly any way to celebrate one whole year of Jersey Exile updates, is it? I must make amends.

Baby Andriana is doing well and approaching three weeks here in the outside world, and aside from an occasional bout of fussiness, she's actually sleeping at night (which is a big change from the first few days!). The little peanut is definitely much more aware of her surroundings than she was even a few days ago, and I think every once in a while she's actually trying on a smile for us. As for her health, we had a minor freakout a few days ago, when we informed by our pediatrician that Adri's phenylalanine level was twice what it should have been for a newborn baby.

(Pardon me while I put on my Science Cap)

Phenylalanine is an essential amino acid for human beings, found pretty much in any food that contains protein, and most people have no problem at all in breaking it down into the compound tyrosine, which is then used as raw material for the building and rebuilding that constantly goes on inside the body. Sometimes however - the overall odds are between 1 in 10,000 and 1 in 20,000, with significant variations depending on the parents' genotypes - a child is born who lacks the ability to break down phenylalanine. Some evolutionary biologists believe that this odd mutation may have conferred a benefit to the first human beings who carried it, as the inability to break down phenylalaline is a proof against certain nasty mycotoxins than can form on stored grain in areas with moist climates, such as Scotland or Ireland, where the mutation is thought to have originated. The tradeoff is that foods that are protein-rich work as a kind of slow-acting poison to people with this metabolic condition, which is called Phenylketonuria or PKU for short, and can actually cause brain damage as the phenylalaline builds up in the bloodstream and is not broken down into tyrosine.

(Taking off Science Cap now)

Enough with the scary, though - the good news is that PKU is almost 100% treatable when caught in the first few weeks of life, before the baby's phenylalaline levels become dangerously high. Using a combination of blood monitoring, diet modification, and a special "phe-free" formula that is used to supplement the newborn's feeding, PKU complications have by and large become a thing of the past. We're not quite sure how much modification Andriana's diet is going to require, but we're relieved that we're in very good hands, and that Massachusetts law is extremely progressive when it comes to metabolic disorders such as PKU - not only does is mandate the screening tests that catch conditions such as this very early on, but it requires health insurance companies here in the Commonwealth to cover the full cost of the phe-free formula and even help parents recoup the cost of special "low-protein" foods which can round out a diet that can potentially not include such foods as cheese, milk, fish, meat, nuts, or whole-grain pastas and breads. There's even a company in Framingham (which is not far from where we live) that specializes in low-protein cuisine, and delivers to boot!

And of course it helps to be a cook with a sense of adventure. Although I don't know how things will turn out in the long run, I'm happy at least that the little pudding pop will not be denied her mother's favorite dish (and a perennial fav of my own): Potatoes with Tomatoes and Chile. And in the end the most important thing is that Andriana is going to be just fine. In fact, she's already gained back her birthweight and then some! If we end up having to go veg for our daughter's sake, well, there are definitely worse things in this world...

...such as our President and Commander-in-Chief. But more about that later.

ps. The writing continues! My initial predictions for "Bambino" being close to the end were a little off. I'm now nearing the fifteen thousand word mark, which is more than I've written on the novel at this point. This short story is turning into a novella!

pps. For more information about PKU, I heartily recommend the National PKU News Organization, whose very informative website went a long, long way in reassuring my wife and me about this eminently treatable metabolic condition.

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