Five Quarts: A Personal and Natural History of Blood

by Bill Hayes

  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 a normal cycle. Oral contraceptives have since become standard protocol for many women with bleeding disorders.

  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 a colorful group, she admitted, drawing individuals from the Lafayette area who have a disease in common but sometimes little else. In addition to folks with heart disease, diabetes, and bipolar disorder, “We have people who are coinfected with HCV, hepatitis B, and HIV. You think you have problems, you should talk to a person like that. That is really, really tough.”

  For peer support regarding her bleeding disorder, Christine turns to what is sadly becoming a lost art, letter writing. One longtime pen pal has been Cindy Neveu, the Bay Area woman who’d introduced me to Christine. Like members of the Pullums’ group, Cindy is multiply diagnosed. She has HCV and HIV, yet these don’t even top the list. And it’s literally a list. Her multiple medical conditions far exceed the surface area of her MedicAlert bracelet, so, as Cindy showed me one day, flashing the bracelet with a mock QVC flourish, it details only her most immediately perilous condition: her fibrinogen deficiency, a blood disorder that’s believed to affect just one in forty-three million people.

  Cindy, with whom I’d spoken by phone several times, had invited me to her weekly cryoprecipitate infusion session. The hour-long procedure was just getting started when I arrived at the infusion center in Berkeley’s Alta Bates Hospital at ten thirty on a Monday morning. I pulled up a chair as the nurse, Carrie, swabbed the skin just beneath Cindy’s left collarbone, the area where your index fingertip would rest if you were saying the Pledge of Allegiance. Here was Cindy’s venous port, a rubber device the size of a nickel implanted under her skin. Since her last infusion, the skin had barely had time to heal over, and now Carrie punctured it again. The infusion needle looked like an extra-large thumbtack. Blood immediately swirled up the attached tubing—a good sign, the port was still viable—and Carrie started a saline drip, which shooed Cindy’s blood back into her body. A nu
rse materialized just then bearing a plastic pouch containing what looked like melted orange juice concentrate. “Ah, here it is,” Cindy said, “here’s my cryo.” Within seconds, the bag was dangling from the IV pole and the infusion under way.

  Sometimes the session is not so flawlessly choreographed. Kept frozen at the local blood bank where it’s produced, the cryoprecipitate must first be slowly thawed before being couriered here to the hospital. Traffic snarls can hold things up. The window for delays is short, however; cryo loses its efficacy within four hours of thawing.

  Carrie promised to check back in a few minutes, and Cindy settled back in the infusion bed, a kind of rose-colored La-Z-Boy in permanent recline position.

  “I’ve been doing the cryo forever,” Cindy immediately confided to me, putting a playful exasperation into her words. “I was diagnosed at birth with the fibrinogen deficiency when my umbilical cord wouldn’t stop bleeding. The doctors put it together pretty quickly, though, since my older brother also had it.”

  Unlike hemophilia A or B, where inheritance is linked to the X chromosome, a fibrinogen deficiency is autosomal recessive, which means both her parents carried the faulty gene but neither Mom nor Dad had bleeding problems. In other words: “Prior to my brother and me, there was no family history.”

  Within the body, fibrinogen (also called factor I because it was the first of the thirteen clotting factors to be discovered) is the last step in the coagulation cascade, the “glue” that holds a clot together. By contrast to people with hemophilia, Cindy explained, those with a factor I deficiency are more likely to “have bleeds” in muscle tissue and the mucous membranes than in the joints. Treatment options also differ, which is something of a bone of contention for the thirty-six-year-old. A hemophiliac today can get a prescription for cutting-edge formulations of factor VIII or IX—genetically engineered, not distilled from human blood. These come in a powdered concentrate that a person simply reconstitutes and injects. Cryo, by comparison, is made from a decades-old recipe: You freeze a healthy donor’s plasma, then defrost it. The solid material that collects at the bottom is the cryoprecipitate, rich in all the clotting factors, including the fibrinogen someone like Cindy needs. From this point on, the blood factors aren’t further separated. So to raise her fibrinogen levels sufficiently, Cindy needs to infuse a bag containing five donors’ cryo. Her brother, Dave, has to receive five times that amount on a weekly basis.

  The transfusion isn’t cold, Cindy explained. It’s not the body slam of chemotherapy or the head rush of too much caffeine. Nor does it hurt, which made sense once she’d said it. This was replacement therapy, after all—simply adding something the blood is missing. But there are risks. “With any blood product you can always have a reaction,” Carrie had told me earlier as she checked Cindy’s blood pressure and temperature. While donated blood is now elaborately screened and tested for hepatitis and HIV, for instance, sometimes a bug or bacterium can slip through. Unlike other blood products, cryo cannot be heat-treated or “washed,” to borrow the nurse’s word. Though a safe, genetically engineered factor I concentrate is manufactured overseas, it has not been approved by the FDA and therefore cannot be legally obtained in this country.

  “I would love to have the concentrate,” Cindy mused, easily envisioning how it would simplify her life. “I could do it at home. I could treat prophylactically much more efficiently. I could travel!” As she sees it, however, the reality is that the profit potential just isn’t great enough for an American pharmaceutical company to create a comparable product. There may be as few as seven “factor I’s” in the United States, according to one statistic. Hence cryo, the standard treatment, which, as Cindy wryly put it, is “cutting edge, 1972.”

  I can’t imagine anyone more deserving of greater simplicity in her life than Cindy. But it may not be in the cards. On top of the fibrinogen deficiency, she has an unrelated clotting disorder. This means that, while she has to infuse once weekly to, in essence, thicken her blood, she must also take medicine twice daily to thin it. In addition, and unrelated to these blood disorders or to HIV or HCV, she has a rare neurological disease, transverse myelitis (TM), a condition similar to multiple sclerosis. I don’t think there’s a satisfying expletive that sums up having five life-threatening illnesses.

  “My system doesn’t know if it’s coming or going some days,” Cindy added. In fact, her medical history is so complicated that she carries with her at all times a single document that spells out the entirety of her conditions. “Here, I’ll show you.” She pulled her wallet from her purse and removed a fold of paper. Regardless of the emergency, she noted, “The first thing a provider wants to know is, who’s going to pay for this? So that’s at the top.” Underneath this insurance info was a summary of her medical history, single-spaced. On the flip side: the names and numbers of her various doctors as well as a listing of her many meds. “The day you don’t feel good is not the day you want to be explaining all this.”

  Cindy also carries a MedicAlert card—call the number and you’ll get her health history over the phone in a choice of languages. “And for airports, I’ve got this,” another ATM-like card. “It says that I’m bionic.”

  “Bionic? You mean, from your brace?” A metal brace provided support for her lower right leg, weakened by the TM.

  No, Cindy answered with a laugh. To control leg spasms, she had to have a catheter implanted in her spine to deliver antispasmodic medicine automatically. “Very cool! But now I beep in metal detectors.”

  Next, Cindy handed me a letter from her doctor, its edges showing signs of frequent use. Unlike the densely typed medical history, this page consisted of a single brief paragraph. “To whom it may concern,” it read. “Miss Neveu is very knowledgeable about her symptoms and the care required to treat her disorder.” In case of an emergency, “PLEASE LISTEN TO THIS PATIENT.”

  “That sure says a lot about the people you have to deal with.”

  “Yeah, this is good to have,” she said. “Nobody believes me when I start telling them what I’ve got.” Her light blue eyes sparkled. “I’ll flash it now and then, and say, ‘Do not mess with me.’ ”

  Cindy seemed to be drawing to a close her show-and-tell when I, like a nosy brother, prompted, “So, what else you got in there?” Her eyebrows lifted as she grabbed her purse: What indeed? She then rummaged and pulled out a cell phone—“Only for emergencies,” she explained. The phone company offers an inexpensive monthly plan for disabled people. Next out, an antique cough drop and a floaty pen, in which a BART train floats between San Francisco and her hometown, Oakland, followed by several old appointment cards for doctor visits—Cindy sometimes has six appointments a week. “You get really nasty hands driving a wheelchair,” she confided, now flashing a packet of Wet Ones. It was only then that I noted the wheelchair folded up and stowed to her left. Though she can walk with a cane, the wheels provide a speedier transport. One thing conspicuously absent from the purse of a woman Cindy’s age, though, was a tampon or two. Well, she explained, it’s too dangerous for her to bleed, so she takes an “industrial-strength” birth control pill that keeps her from ever having a period. This has been necessary since puberty.

  The final stop on the pocketbook tour was a small case, imprinted with GIRLS RULE!, that contained business cards for her Web site, the sublimely named Shemophilia.org. Cindy explained that a former hemophilia treatment nurse here at the hospital had affectionately called all the female patients shemophiliacs. As Cindy was preparing to launch her site in 1999, the nurse graciously allowed her to, as Cindy put it, steal the name. Already in existence were informational sites focused on some of the more prevalent bleeding disorders, such as von Willebrand’s, but Cindy felt that “too many factor I’s and II’s were falling between the cracks.” The potential for feeling disconnected is far likelier when you’re just one of a handful of women in the entire country with, say, a fibrinogen deficiency or classic hemophilia. What was needed was an online “community center�
� for these individuals. Cindy hoped the site would become a forum where someone such as Christine Pullum could share her wisdom.

  With the playful name Shemophilia, Cindy also sought to convey the user-friendliness rarely found in a pamphlet picked up at a doctor’s office. I think her instincts were spot on. FibrinogenDeficiency.org just wouldn’t have the same zing. Among people with bleeding disorders, she added, the term hemophilia is a kind of collective shorthand. In fact, she said with a laugh, when she’s tried to explain to someone her specific disorder, their eyes have tended to glaze over. “It’s often just easier to tell people I have hemophilia.”

  In my experience, I’ve found that people who are strong advocates for peer support either never enjoyed it themselves or are taking the opportunity to pay it forward. Cindy is clearly in category two. In December 1991, two years after learning she’d been infected with the AIDS virus from tainted cryoprecipitate, she arrived at an HIV support group in nearby Pleasanton. As she took a seat in the host’s living room, she realized she was the only woman in a small circle of gay men. “At first, I was kind of afraid because I’d lived such a sheltered life and didn’t have a lot of experience with the gay world,” she recalled. “But from the start, they were so welcoming and wonderful. I called them My Tuesday Guys, ’cause that’s when we’d meet. And we’d always go out afterward—this chick with a cane and eight really good-looking gay men going out to Bakers Square.” She hooted with delight at the memory. “I’m sure people had to wonder.”