18 May 2008

The old, familiar sting . . .

Hemophilia can be a truly nefarious condition.

One of its more nefarious aspects is that it can lie low for generations, quietly getting passed from mother to daughter but not calling attention to itself by taking any side trips into the male branches of the family. (Yes, I know it's all governed by genetics and chance, and the condition does not, indeed cannot, take any active interest in how and when it manifests itself. It's just sometimes easier, for me at least, to talk about it this way.) The family goes along minding its own business, and with each generation one or two of the daughters are carriers, who grow up and have children and again the luck of the draw gives them a son who is not hemophiliac and a daughter who will quietly, secretly, unknowingly pass on the genes. Eventually so many generations pass that the family forgets about great-great-great-great grandfather Sheldon who was said to be a cripple and died young bleeding into his stomach.

Then it happens.



A beautiful little son is born, but after the circumcision he doesn't stop bleeding. The doctors, and the nurses, and the social workers (oh, god! the social workers), quiz the family about passed generations' health history, but nobody remembers Sheldon, and they keep saying, "I just don't know where it came from." Sometimes it is even more nefarious than that. I once met a man (this was in the early 60s) who didn't find out he had a reasonably mild form of hemophilia until he went in for some minor surgery as an adult. I thought at the time that he must have led a fairly sedentary youth, and went in more for stamp collecting than football; but that was his story, and I've seen weirder things happen, so who am I to doubt him.


And from what I've observed over the passed sixty some years, that's when the guilt and the fear really take hold. The mother puts herself through agonies of guilt and remorse. "I gave this horrible thing to my son." If they are of a religious frame of mind they might spend eternities wondering what terrible sin they committed to be punished in this manner. Either way they then live a life of martyrdom to atone for what they did to their son. Unfortunately, the father will all too often (once is too often, but the percentage is sadly much, much higher, or at least was in the 50s and 60s) also blame the mother for giving him a defective son.


All they've ever heard about the condition are some old wives' tales about someone who knew someone whose second cousin's sister's boyfriend's uncle bled to death after popping a pimple. Then the parents, usually the mother, will do some research and discover that uncontrolled bleeding from a cut is not, as serious as it can be, the truly dangerous/debilitating aspect of the condition. It's those pesky internal hemorrhages that can be triggered by a bump or a twist or for no discoverable reason that are the true nightmares.


In the old days (that is to say my youth—say before 1960 or thereabouts), what would often happen next was a total redesigning of the family's lifestyle to "protect" little Tommy or Edgar or whoever who might die because of an excessively firm run in with some object harder than a fresh marshmallow. (Stale marshmallows can become dangerously hard and must be removed immediately.) Every room would be carpeted. Tables would be wrapped in foam rubber. Tommy et al would be barred from the kitchen where there was too many hard things and sharp things. One family went so far as to keep their son in a bath tub filled with luke warm water for most of his waking hours. (That, unfortunately, is neither an exaggeration nor a product of my imagination. I was always surprised he didn't drown.)


Naturally any activity more physical than chess was forbidden. Running, jumping, climbing (breathing!) might cause a knee bleed. In the 1950s even swimming was looked at with suspicion. At one local Hemophilia Association meeting my parents were told that contrary to their evidence (my parents' evidence that is), swimming was too dangerously "active" and the exertion would cause untold joint hemorrhages. And, the pressure of the water might cause the lungs to bleed or something. It goes without saying that baseball, playing tag, etc were considered just plain suicidal.


My brother and I were truly lucky. My mother's father had hemophilia, and she knew what to expect and, more importantly, some of the challenges, and nightmares, we would have to face and that they could be overcome. My father had total faith and love for my mother, and that meant he had total love for his sons.


It's at this point that some of the other fathers all too frequently disappeared. Sometimes it would be because he couldn't love a woman who couldn't give him a "real son." Sometimes it would be because he couldn't bear the shame of fathering "something that would never be a real man." Sometimes it would be because he was just too afraid of what he might have to witness.


Like I said, hemophilia can be a truly nefarious condition.


この道や行く人なしに秋の暮
No one/Walks this road—Autumn evening.

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