Five Quarts_ A Personal and Natural History of Blood - Bill Hayes [85]
Christine grew weary over the years of having to convince doctor after doctor that she had what was unanimously considered “a man’s disease.” A formal diagnosis of hemophilia didn’t come until she was a married woman in her midthirties, following treatment at the University of North Carolina medical school. She’d returned home to Lafayette with a document quantifying her factor VIII deficiency and providing instructions to her local doctors—that, in short, when future bleeding problems occurred, she must be “treated in the same manner as a man with hemophilia A.”
“It was a relief just to have it on paper,” Christine told me, the memory of the milestone still making her voice dance. Unfortunately, the paper was put to almost immediate use. In 1975 she was hospitalized with severe hemorrhaging in her abdomen, an extreme example of the spontaneous and painful internal bleeding hemophiliacs can experience. Once again, though, doctors disbelieved her claim of hemophilia. Thankfully, Christine survived the time it took Doyle to race home to get “the proof.”
The letter of diagnosis also addressed a more personal issue that Christine had broached during her workup, the risks of having a child. What for many is a joyful decision was for the Pullums a complicated one, requiring an emotional, medical, and financial accounting of where they stood. What were the health risks to Christine of carrying a child to term? What was her own independent prognosis? Could they accept the fifty–fifty chance of a son being a sufferer or a daughter being a carrier and, possibly, symptomatic like her? And what if an affected child had a form of hemophilia more severe than Christine’s? Setting emotions aside, could they, bottom line, afford to care for such a child?
During Christine’s hospitalization, the Pullums experienced the sticker shock of a top treatment of the time, cryoprecipitate, an expensive extract of clotting factors made from fresh frozen plasma. They were also mindful of the challenges faced by Christine’s sister, whose young son had been diagnosed with severe hemophilia two years prior. Plus, for Christine, the idea of motherhood was tempered by her memories of childhood, how simple events such as losing baby teeth or falling off a bike were ordinary for everybody but her. She shuddered at the thought of her child going through what she’d faced as a girl.
Today a mother-to-be can undergo amniocentesis to learn if her baby has a genetic disorder such as hemophilia, but subtract three decades and, of course, the diagnostic tools weren’t nearly as sophisticated. In Christine’s original letter from her doctor at the University of North Carolina, he had explained in stark terms one of the few options available to her. “There is no test at present which will tell us during pregnancy whether a fetus does or does not have hemophilia. However, it is possible to determine the sex of the fetus” at about the fifteenth week, he noted. “Some couples in which the wife is a carrier choose to use this method to have only girl children by planning an induced abortion of any male fetus.” In the final balance, Christine and Doyle chose not to have a child.
As our conversation spun back to the present day, Christine reflected that the intervening years have been largely free of serious complications from hemophilia. Her extreme vigilance has paid off in that she’s rarely needed infusions of clotting factor. She is, however, of the generation for whom treatment came with disease. It took just one cryoprecipitate infusion in the mid-1970s for her to become infected with the hepatitis C virus (HCV). As she approaches her sixty-seventh year, Christine must contend with cirrhosis of the liver. She now has a diagnosis doctors will believe.
I doubt Christine would ever consider herself an activist but, in the next breath, she explained how she and Doyle founded and facilitate a twice-monthly HCV information exchange/support group. Now, this is