Page 63 of Hell Bent
“It is. For hereditary Hemophilia A or B, at least. Look. Queen Victoria was a hemophilia carrier, OK?”
“I remember something about the Tsar of Russia,” he said. “Can’t remember what.”
“Victoria and Albert had nine children. A carrier has a fifty/fifty chance of passing the gene to each child. In their case, two daughters were carriers, and a son had the disease. If a daughter gets the gene, she’s a carrier. If a son gets it, he has the disease. Pretty simple. Victoria’s daughters singlehandedly spread hemophilia throughout the royal houses of Europe. Russia, Spain, England, Germany. Eventually, it mostly died out, because most of the men died before having kids, and people stopped marrying the women.”
“Except in your case,” Sebastian said. “Because you have it.”
“Yes. We’re one of the unlucky lines. The British and Greek ruling families didn’t have it, which is why you haven’t heard about it with Queen Elizabeth and so forth. But, yes, Alix of Hesse, who became Tsarina Alexandra of Russia, as I mentioned, was a carrier, and her son Alexei, the Tsarevich, the youngest child, had the disorder. Historians say that was one cause of the Russian Revolution, in fact. Alexandra was terrified for her son’s future and turned to this faith healer, Rasputin, for spiritual guidance, which turned out to be a bad idea, because Rasputin was majorly unpopular and so was she. Conspiracy theories abounded, anger and resentment followed, and eventually something like ten million people died, including Rasputin and the entire royal family, partly because of one kid and his clotting disorder. Of course, the hemophilia was a major state secret, because the kid was the heir to the throne and couldn’t be perceived as sick, but it didn’t turn out to be allthatsecret. Did you ever wonder why all those sisters were there to die in the basement, when the oldest two were in their twenties? The other European royal families were afraid of them bringing in hemophilia, that’s why, and didn’t want their sons to marry them, even as intermarried as all those royal houses already were. People didn’t know thescience of it, but they’d more or less figured out that it was hereditary.”
Sebastian said, “Wow.”
“The funny thing is,” I said, “or not so funny, turns out Anastasia was the only daughter who was a carrier. DNA analysis on the bones. Yet another reason why ‘Anastasia Alix’ seems like a bit of a cursed name. Two carriers. Not the family legacy you burn to carry on.”
“All right,” he said, “but most people with hemophilia aren’t royal, and you’re not a Russian princess, so again—why the secrecy? Also, if women are only carriers, how does it affect you?”
I sighed and adjusted the ice pack on my belly. It did help. “Carriers can be sick too. Internal bleeding, mostly, which hurts. It depends how much clotting factor you have. Best case, you have fifty to sixty percent of a normal person’s factor, because you have one gene for the disease and one normal one, right?”
“Right. So that makes you sick. Just notassick.”
“No. You’re pretty much fine with fifty percent. If you have less than that, you have some issues. Mostly manageable. You can’t play contact sports—the disappointment of my life—for obvious reasons. You bleed more during menstruation. You bruise more easily. Childbirth is riskier, and so is surgery, even dental surgery. And if you’re having issues, you may need more clotting factor, which you get via a slow-drip IV. I’m on birth-control pills to lessen my bleeding, but I can still have pain. I toughed it out until today, but I knew I couldn’t go to work tomorrow unless I got some help. Unfortunately, I hadn’t set things up ahead of time with the clinic up here, which was stupid. Wishful thinking. Which meant I had to do paperwork and be assessed, and I had to wait, andthenI had to get that IV. Which means that now I’m tired and still hurting some, but I’ll be fine tomorrow. Also, that’s onereason I’m in Portland, because theyhavea clinic. And plenty of work for a commercial electrician, of course.”
“Doesn’t everyplace have a clinic?” He was frowning now, his face focused, intent.
“A hemostasis and thrombosis clinic? Not so much. Eight regional treatment centers in the U.S., and it’s helpful but not necessary to be close to one. So there you go. Do you feel leveled with now? Honesty. Openness. Possible relationship. Done.”
Sebastian
I’d wanted to hold her hand since about thirty seconds into that recitation. Now, I did hold it and say, “Thank you.”
“Ha,” she said. “I told you, it’s not important, and it’s not edifying, but now you know.”
I looked at her white, exhausted face, at the shadows under her eyes, and felt … something. Not pity, but something newer that I was becoming uncomfortably familiar with. I was very much afraid it was tenderness. I asked, “Want a cup of tea?” It was the only thing I could think of.
“Oh, man,” she said with a sigh. “I do. But you don’t have any.”
“Hang on.” I climbed off the bed.
When I came back ten minutes later with a mug, she was asleep, but woke when I sat on the edge of the bed. “Sorry,” she said, pushing herself up to sit again. “What?”
“Tea,” I said.
She blinked at it. “How?”
“Borrowed a few tea bags from next door. Women generally have tea, I’ve noticed. I’ve added it to my shopping list.”
“Thanks. But this is what I don’t want. Fussing.”
“How is that fussing? Isn’t it just normal? How can youcome over and help out Ben, but it’s unacceptable for me to bring you a simple cup of tea and an ice pack?” My voice was rising, I noticed, but who could blame me? This was maddening.
“It’s fussing when it’sfussing.Why do you think I lived at my grandparents’?”
“Wait. Your mother would have it too.”
“Yes. And my grandmother. Mine’s worse than theirs, because I have less clotting factor—the luck of the draw—and my mother’s always felt guilty about it. Hence the Olympic levels of fussing. Also why it’s princesses all the way down. Nobody could risk having a son. There was an abortion or three in there, I suspect, though nobody’s ever said.”
“Oh,” I said. “No genetic testing.”
“Exactly. You couldn’t do it then. You can now, but being sure of having an unaffected child means IVF. So there you go. I’m a too-precious child, I’m slightly defective, partner-wise, and I’m more defective life-partner-wise.”
