Five Quarts: A Personal and Natural History of Blood (21 page)

“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.”

Much of her present-day passion for mentoring was inspired by the man who’d started the support group, Wes. “He taught me a lot of important things—about treatments and disability benefits and working the system.” Wes, who passed away five years ago, was also a buoy during some rocky emotional times. In the same spirit, she’s now mentoring a fourteen-year-old fellow factor I in Ohio, keeping in touch through e-mail and phone calls. Further, Cindy sees it as part of her role with the Web site to facilitate a mentor matchmaking of sorts. “Usually I’m able to link people pretty well. If you contact me and say, ‘I have factor VII deficiency, I want to talk to somebody,’ I’ll make a couple of calls and get you connected with another woman who has your disorder.” She’ll make sure, too, that you receive written information and, if need be, a referral to an accredited hemophilia treatment center.

“That’s the biggest problem with the rare disorders,” she continued. They’re often not seen as falling under the umbrella of hemophilia, so these patients don’t get referred to the best treatment. Sad but true, “There are all these independent docs out there winging it on old, old information.” Cindy told me about a fifty-year-old factor I with whom she’d spoken not long ago. “She was only being treated with plasma, not cryo—very inefficient, because you’re getting a lot less of the clotting factor and a lot more volume.” Though Cindy has grumbled about how dated her cryo infusions are, this woman’s care was cutting edge, 1940.

“That’s like going to your AIDS doctor and only getting a prescription for AZT instead of a multidrug cocktail,” I observed.

“Yeah, it’s pretty scary.”

The amount of misinformation is sometimes daunting, Cindy acknowledged. Not long ago, for instance, she had to swoop in and set straight a nice woman from Maine who, for reasons unknown, believed that bleeds “were only serious if they occurred below the knees!” Cindy actually went and met this woman and her husband in person. She arrived at the restaurant they’d chosen to witness the husband rearranging all the furniture in the waiting area, so that his wife’s knees would not be imperiled.

At this point, although Cindy’s stories hadn’t run out, her cryo had. Carrie returned and removed the empty pouch as Cindy, like the patient schoolteacher she used to be, provided a careful narration of what followed: “Now she’s going to flush the line with saline to clear it, then put in a little heparin—that’s an anticoagulant—to keep the port from getting clotted.” Carrie then unplugged the plastic tubing, bandaged the tiny hole, and Cindy was free. “If everything goes right, if I don’t fall or something,” she said matter-of-factly, “I won’t need to come back here till next Monday.” And with that, we said quick goodbyes. It was 11:30
A.M.
and Cindy Neveu had much more to fit into her superhuman day.

E
LEVEN

Blood Drive

IN A BODY AT REST, A SINGLE BLOOD CELL COMPLETES A full circuit of the circulatory system in just about thirty seconds. Blood bursts from the heart at its top speed, around one mile per hour, and shoots through the tough plumbing of the arteries outward to the body’s extremes. On its return, venous blood—now depleted and slogging waste—often must work against gravity and, at best, will only reach half its starting speed. In other words, the second half of the trip is more arduous than the first, which I suppose could be said of life as well.

At an approximate rate of one million per year, my blood’s clocked just over forty-three million circuits and, barring catastrophe, I anticipate another forty million or so. Long-livedness runs in my family, though I can expect the expected “infirmities of old age,” as my eighty-seven-year-old great-grandma Bridget’s obituary described her last years. Already though, as all people do, I’ve outlived my blood many times over. I contain, for instance, no red cell older than four months, no platelet over ten days. Some of my white cells survive less than six hours. Other blood cells are longer-lived, it is true. The lymphocytes called memory cells, for example, are ferried about the circulatory system for decades. But these, too, eventually fail. Such deaths go unnoticed by me but not by my body. Individual blood cells are constantly being replenished or replaced through a remarkable system of inner housekeeping. That being said, it is still a disquieting notion that my blood retains none of its original parts.

Once blood is removed from the body, the cellular life spans plummet. Forestalling the death of blood is the major clinical function of any modern blood bank, although, granted, you wouldn’t find such dark phrasing in an annual report. A blood bank gives the impression of being less a bank—as in, a place to stockpile—and more a hospital, wherein blood is on continual life support. I quickly came upon this realization during my tour of the main branch of Blood Centers of the Pacific, a state-of-the-art facility here in San Francisco, where I’d come to see how blood products are made. As Richard Harveston, the director of hospital services and my genial host, explained to me, blood must be carefully housed, nourished, and tended. “Blood is a living tissue,” he said. And therein lies the challenge.

“One of the greatest advances in blood banking came in the early 1970s with the advent of plastics,” continued Richard, in what at first seemed like narration from a different tour.

“Plastics?”

“Yep, just like that guy says in the movie
The Graduate:
‘Plastics.’ ” He smiled, adding, “Before technology for making plastic bags was perfected, blood was drawn in glass bottles,” which caused a lot of headaches. Bottles took up lots of storage space and trapped air, fostering bacterial contamination. By contrast, plastic bags provided a slew of advantages, including being virtually unbreakable, lightweight, airtight, and malleable. In addition, Richard explained, “Plastics made possible the era of component therapy.” This last sentence rose to a deliberate crescendo. In component therapy, blood is separated into its various parts, which then become highly efficient, targeted interventions, such as the present-day replacement therapies for hemophilia. Cindy Neveu’s cryoprecipitate, although a dinosaur when compared to other treatments, would also fall under this heading.

We were standing on the periphery of the blood center’s collection area, where five donors were giving blood. To illustrate the procedure, Richard pulled out a “blood collection set”—three connected clear plastic bags (a large and two smalls) trailing a tangle of tubing. The whole mess looked like a jellyfish, the kind of thing a kid on a beach would poke with a stick. “Blood flows into here,” he said, pointing to the primary collection bag. This pouch already contained a small mix of fluids: an anticoagulant, a phosphate to maintain the pH, and a nutrient to keep the blood cells alive. He then traced the tubing to the second bag, which would later be used during the blood processing phase to hold the plasma, and to the third, a pouch for platelets. “If you’ll notice here,” he said, inviting me to feel the last pouch. “This is a different plastic, this has a different porosity,” allowing gases to pass in and out. “Just like we do, platelets have to breathe.”

Leaving behind the homey atmosphere of the collection area, Richard and I entered the factory-like environment of the Component Lab and stepped around what could’ve been a beverage cart from an airplane, heaped with fresh pints of whole blood. It looked kind of disorderly, truth be told, but each unit, Richard hastened to point out, was bar-coded, its every movement through the facility tracked. For each bag here, a tubette of the donor’s blood had also been collected and affixed with a matching bar code. These samples were already on their way to a lab in Arizona, where each would be comprehensively tested for HIV, hepatitis, syphilis, and so on, and so forth.

Within six hours of being drawn, a large portion of the pouches are spun in a centrifuge. The technician working this machine allowed Richard to demonstrate. The centrifuge, whose interior is chilled to just above freezing, has six pewter buckets. He stuffed each with a bag of blood, the one full and two empty pouches sandwiching nicely. Blood naturally separates, Richard noted, but this device speeds up the process. “Once it gets started, the buckets spin out, like being in a Tilt-A-Whirl.” The speed of the “ride” can be varied, he added. A light spin, for instance, is necessary if you’re harvesting platelets. Richard then closed the lid and flipped a switch. The procedure would only take a few minutes.

In the interim, he steered me to an adjoining room, a kind of platelet pantry. On floor-to-ceiling shelves, small bags of the straw-colored cells lay like flat pillows on undulating metal racks, the rocking movement driven by grinding motors. “Platelets are very fragile,” he said in a raised voice, “not hale and hearty like red cells.” But they’re very eager to clump, which is their pivotal role in the coagulation cascade. Once clumped, though, they don’t unclump. “So,” Richard concluded, “you have to keep them in constant motion.” You also have to keep them exactly at room temperature, a fact I found quite odd. Ironically, once removed from the 98.6 degrees Fahrenheit of the human body, platelets no longer thrive at that temperature. He picked up one of the bags for me to peer at, holding it up to the ceiling light. The platelets were just a swirl in a shallow bath of plasma.

These cells remain functional for only five days, Richard said. They’re the shortest-lived products produced at the center, and biting into that time is the thirty-six-hour wait for test results. I quickly did the math in my head. Subtract the day it takes to process the blood, minus the day and a half for testing: “So half their shelf life is spent here on the shelf,” I observed.

“You got it,” Richard said with a nod. “Which is why we always, always need new donors.”

Red cells, he went on to say, can last forty-two days if refrigerated and years if frozen. Plasma is more finicky. If not frozen within six hours, the essential clotting factors “will disintegrate” or break down. Frozen plasma will keep for no more than twelve months.

Back at the centrifuge, Richard gently withdrew a spun bag of blood, now displaying neat layers of amber, white, and burgundy. We took a giant step to the right, at the same time moving from high tech to low. At this workstation each bag of separated blood is hung by its edges to the “plasma expresser.” Anyone who’s worked an old-fashioned orange juicer could handle this device. Pulling down a simple lever applies pressure to the lower portion of the bag, thus squeezing the plasma at the top through the tubing and into the second collection bag. The only trick is knowing when to stop pulling.

On the other side of this counter, an IV stand held several fat red pouches of plasma-less blood that were now being stripped of white cells, a slow process that appeared to depend mostly on gravity. Blood snaked down a length of narrow tubing, passed through a white-cell-catching filter about the size of an ant trap, and pooled in a bag near the floor. The white cells would be discarded. Watching this procedure brought up a question that’s nagged at me for many years: If a healthy person’s immunity is largely contained in his or her white cells, couldn’t an ill person benefit from them? Or, coming at it another way, why throw them out? Wouldn’t transfusing them be useful?

“No, almost never,” Richard answered. “White cells are not a good thing, and you want to remove them.” Beneath his blanket statement were a number of powerful reasons. For starters, too great a risk exists of transmitting an infectious disease for which testing isn’t done, such as the cytomegalovirus (CMV), which may be present in white cells even if the donor has never manifested symptoms. Further, contrary to my layman’s thinking, white cells rarely see another person’s white cells as allies. Instead, they go on the attack. The recipient may, as a result, suffer a high fever or a life-threatening reaction. “There are very few indications for white cell transfusions,” Richard concluded, “one or two a year, if that.” Thankfully, for the vast majority of patients a far safer and more effective alternative exists in antibiotics.

Okay, that all made sense, but a new question now replaced the old. Of course, I began, people with HIV cannot and should not give blood. But in view of the fact that (1) HIV only infects white cells, and (2) white cells are removed from all donations, why then, speaking hypothetically, couldn’t a person with HIV give blood?

Richard’s whole demeanor said,
Ah, good question!
“Well, you’re right, HIV does only infect white cells,” he replied. But when you’re “manipulating” blood products, it’s not so cut-and-dried. He then motioned to the centrifuge. “Centrifugation is a pretty crude separation technique. You’re artificially causing trauma. White cells are very fragile; they can rupture and release the virus.” Free virus, he called it, which can then turn up in the wrong blood “zone.” In test studies with HIV-infected blood, he noted, “All of the blood products have been shown to contain virus”—red cells and platelets, as well as plasma.

As for my hypothetical, as it turns out, people with HIV do on rare occasion donate blood, although they’re typically unaware at the time of their positive status. “We get about two or three HIV positives a year out of 125,000 donations, which means the donor history and medical screening we’re conducting is actually quite effective.”

By way of explaining to me the many layers of safeguards in place, he suggested we jump ahead thirty-six hours in the processing of newly donated blood. At this point the Arizona test results have just arrived by computer. The red cells have remained refrigerated, the plasma kept frozen, and the platelets have never stopped undulating in their metal beds. Richard and I now stood in the Label & Release room, where a technician sat before a computer monitor, a box to her right filled with rock-hard plasma units. The technician swiped the bar code on the first frosty unit of plasma, calling up its results, pass or fail. (The red cells and platelets would also undergo this inspection today.) An A-OK was followed by a search of national, state, and internal databases to quadruple-check the donor’s information. Has the individual ever been deferred in the past because of, say, foreign travel or short-term illness? Did the donor make his or her donation before the mandatory fifty-six-day wait? (This waiting period allows hemoglobin levels to return to normal.) An approval label only generates if no flags go up. The rejection rate is “very low,” Richard noted. “Far less than 1 percent.”

The new label was smoothed over the old, then the unit was scanned again and officially validated. “Now this unit is, by definition, ‘Finished Goods,’ ” Richard announced, framing it with his hands.

Along with the seal of approval, each bag at this moment acquires an important quality: monetary value. The not-for-profit Blood Centers of the Pacific does, after all, have to survive financially. In the Bay Area this translates into a unit of fresh frozen plasma selling for $70; red cells for $180; and platelets for $600. (The national average price tags are roughly 20 percent lower, Richard noted.) Yearly, the center sells to forty local hospitals approximately 125,000 units of red cells, 50,000 of plasma, and 15,000 of platelets. The organization wholesales an additional 75,000 units of plasma to pharmaceutical companies for further processing, such as the making of factor VIII concentrate. (Only a small percentage of the center’s total output is whole blood, a fact I found surprising. TV medical dramas, as it turns out, vastly overplay the call for whole-blood transfusions.) At any given time about 10,000 units of special red cells remain here on the premises, 99 percent of which are kept frozen in long-term storage. In this capacity, the bank is most bank-like. Some of this store is autologous blood—donated and reserved for an individual’s own future use, such as for an upcoming surgery—but most of it is blood of the rarest types, the Château Lafittes of the blood world. To keep these red cells viable for as long as possible, each unit is infused with a preservative, Richard explained, “not unlike the antifreeze you put in your car.” The blood is not left in the bag but spread flat in “a single monolayer,” he continued, and frozen at minus eighty degrees Celsius.
That,
I thought,
should be interesting to see.

But first he led a winding route from the Label & Release room to a set of windows overlooking another work space. The sight of two white-coated scientists huddled over computers wasn’t all that fascinating, but the work these gentlemen were doing certainly was, Richard assured me. He gestured grandly. “This is our Immuno-Hematology Reference Laboratory. It is very much a part of the history of blood banking, one of the oldest in the country, and one of the most famous.” Perhaps he could tell by the look on my face that I’d gotten lost on the way through his hyperbole. Richard paused, then rewound his narration. “You know, each individual has a ‘genetic fingerprint,’ if you will, on his red cells—”

“Right. Not a DNA signature, but a kind of tag that identifies your blood group.”