Five Quarts_ A Personal and Natural History of Blood - Bill Hayes [84]
With so few cases of female hemophilia A on record, it would be a stretch to call any one instance typical. Still, experts say it most likely would manifest in a daughter born of a male hemophiliac and his carrier wife. Not so with Christine. “Our family didn’t have the usual pattern,” she told me. “I got it directly from my daddy.” In a genetic fluke, the good X chromosome she got from her mother failed to compensate for the defective one, making Christine what’s called a symptomatic carrier. Her two sisters are also carriers but have never been symptomatic. The shared disease between Christine and her father created a unique bond. Throughout her childhood, he would tell her that their hemophilia could be traced back to the czars of Russia, referring, I gathered, to the doomed Romanov family, all of whom—Nicholas, Alexandra, Olga, Tatiana, Marie, Anastasia, and Alexis—were assassinated in 1918 in the wake of the Russian Revolution. Christine has never seen any documentation of this tie, she acknowledged, and I had to admit I couldn’t see how this branch fit onto the Romanov family tree. Even so, it sounded like a sweet way to make a daughter with a scary disease feel special.
To help build a picture of who she is now, I asked Christine how her friends would describe her, which, after a moment’s embarrassment, led to an interesting cavalcade of disclosures. “Well, I’m the opposite of a hyper person,” she told me, “and I’m going to be on the petite side in the dress department.” She used to be five-three, she added, but has lost two inches to osteoporosis. Describing her ethnicity as “a half-breed,” she chuckled and elaborated that her father was from Greece, her mother, American.
“My daddy knew when I was a year old that I was going to be a bleeder.” One day she’d bitten down on the edge of a coffee table, something not unexpected in a teething child, and cut the inside of her mouth, a wound that was slow to stanch. This was Jackson, Mississippi, in the late 1930s, she recalled, a time when you couldn’t convince a doctor that a girl had hemophilia and, even if you could, there wasn’t a heck of a lot anyone could do about it. So her father, who was born in 1903 and lived to age seventy-five, taught her what would be the secret of his longevity—being very, very cautious. The first modern medical treatment for hemophilia didn’t arrive until the early 1940s in the form of blood transfusions. These weren’t a perfect solution. Receiving another person’s whole blood or plasma did not provide what was most needed, a concentrated dose of clotting factors (not until the late 1960s was such a transfusion possible). But it was enough to save lives. With that in mind, Christine could look back with no nostalgia whatsoever at what her father had faced when he was small. “Daddy’s treatment was spiderwebs,” she marveled, “the soot from the chimney, things like that”—folk remedies used for crudely plugging breaks in the skin.
My telling Christine that I grew up with five sisters seemed to free up some thoughts on the bane of her teenage existence, menstruation. “Of course I had the very heavy periods, where I couldn’t even go to school.” This was back in the day when it was deemed inappropriate for women to wear pants, she explained. “We always wore skirts, and blood would come all the way through.” Pants, at least, would’ve kept the problem a little more hidden, but, without that option, “I knew what I had to do: I stayed home.” Her doctor was hardly a lighthouse in the storm. He suggested, she recalled, that heavy bleeding might simply be perfectly normal for women in her family. So, chin up, not to worry. Only after the birth control pill became available in 1960, when she was in her early twenties, did Christine experience