For the past several days, I’ve been trying to determine my earliest memory connected with hemophilia. When, exactly, did I first realize I was, as they say, a bleeder? I couldn’t think of a time when it was not part of my identity. This led me to wondering what my first memory was of actually bleeding.
My grandfather had hemophilia, so my mother knew there was a possibility her son might also have the condition. That the possibility had become reality was confirmed when I was three days old and the doctor—rather against orders, but that’s another story—circumcised me. My parents say I bled for three weeks. My memory of the incident is hazy at best, and I seem to have misplaced my notes from that period, so I am forced to take their word for it.
I know that when I was a toddler in 1948, I fell and fractured the bone behind my right ear when my head hit the marble trim in the post office. I guess it caused my parents and the local doctor some moments of anxiety. But I don’t remember any of it. Then about a year and a half later, I fell off the steps leading to my aunt’s basement and landed somewhere behind the furnace. I’m told it was all rather messy, and until I was in my 30s I had a rather interesting scar on my forehead. It’s faded away now, and all I can remember is standing at the top of the stairs and Mom telling me to get away from there. I apparently didn’t listen. My wife says those early head injuries might explain a lot.
I’m sure I had some rather routine joint bleeds along the way. I know, there’s photographic evidence: For close to a year, I had a rather large bruised lump on my forehead while I was learning to crawl and walk. The universal treatment, however, seemed to be to keep the patient as quiet and comfortable as possible. After that, your guess was just as good as theirs. The patient lived or died, and if he lived he came out of it fairly well, or crippled for life.
When my younger brother Mert was 1 or 2 (1950 or 1951—I’m pretty sure it wasn’t on a Tuesday), he had a hemorrhage that required hospitalization. I remember Mom and Mert had to take a long bus ride to Portland. While he was in the hospital, they had a lot of trouble establishing IV lines so he could be given whole blood. It was a very bad hemorrhage (oddly enough, I don’t know where he was bleeding), but the best they could do was try to give him enough whole blood to stop the bleeding, but not so much they overloaded his system and killed him.
Some hospitals at that time, I’ve heard, tried total blood replacements when the situation was grave enough, but I don’t know if they tried that with my brother. Other hospitals were experimenting with plasma from pigs and sometimes cattle, but that was, as far as I know, always a last-resort measure. They ended up doing what they called cutdowns on both of his ankles. I’m not sure about the actual procedure, but it looked like it involved making about five-inch-long incisions down the side of the ankle so they could access a vein directly. For several years after that he wanted to sleep with his shoes on, and he had the scars until he died some 40 years later, after contracting HIV from contaminated factor in the mid-1980s.
My first direct memory of seeing a doctor because I was bleeding was when I was 6. It was—surprise, surprise—a knee bleed. It was a Sunday afternoon, and Dr D. had agreed to meet us at his office. At first he was going to come to the house (doctors still did that then), but he was worried he might not have all the supplies he might need, and Dad had said we’d just come to his office. Dr D. was unusual in that even though he practiced in a small farm town 45 miles from the nearest research center, he had a small supply of AHG (antihemophilic globulin), the clotting medicine of the day. He also treated two brothers with hemophilia who were several years older than my brother and me and kept the serum on hand in case of the odd emergency.
Dr. D. decided to play it safe and give me a dose. It seemed to come in a one-size-fits-all dosage, probably because the usual plan seemed to be to give the patient a bottle and see what happened. AHG was a freeze-dried powder in, I think, a 500cc bottle that was reconstituted with sterile water. After it had been mixed, it looked kind of like weak lemonade, and using several syringes Dr D. injected it straight into the large vein on the inside of my elbow. The hope was always that one treatment was going to take care of everything, but hope sometimes doesn’t quite match reality.
After the injection, Dr D. gave Mom and Dad some instructions. I was to stay in bed or lie quietly on the couch as much as possible. He gave them a urinal so I would only have to get up to “go number 2,” and said, if possible, they should keep my leg raised on pillows. If the pain got to be too much, they could give me some baby aspirin and let me soak in a tub full of warm water. The water’s universal support would take the strains and pressures off the knee and might ease the pain. It actually seemed to work, if you didn’t mind the warm water stimulating the circulation and causing the bleed to last even longer.
He told Mom and Dad the best thing was to keep me quiet and let the AHG do its work. If there wasn’t any improvement by Tuesday, they’d bring me back and we’d try another shot. That first shot of AHG didn’t seem to have any effect, and on Tuesday I was back at Dr D.’s office for another treatment. And on Wednesday I got a third. That seemed to be the last of his supply.
The big question with the early forms of AHG was whether it was doing anything. They knew, or at least had a fairly sound suspicion, the stuff we needed was lurking in the mixture, but they were pretty much guessing at how much, the amount we needed and when, and how long it was effective. Sometimes it seemed to stop the bleeding right away. Sometimes it didn’t seem to do anything. And often you were left wondering if it had helped or if time and natural processes had done the job.
To a certain extent, I don’t think it mattered. What mattered was they were actively treating the cause of the bleeding, and the active treatment is the important bit. The doctors and my parents were no longer passively reacting to events with bed rest and the like. They were taking steps to get at the cause of the problem, and that gave them hope. And what gave them hope made me feel confident that something along the way was going to work. So far it has.
Read more Guy Boss at the Missing Factor.