Five Quarts_ A Personal and Natural History of Blood - Bill Hayes [86]
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 nurse 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