The woman at Lori Wagner's door introduced herself as a case worker for Child Protective Services and demanded to see Lori's son, Ian, who was 2 at the time. It wasn't the first time someone had questioned the parenting abilities of Lori and her husband, Patrick.
Lori explained that the severe bruising on her son was due to hemophilia. A quick check with Ian's physician satisfied the case worker, but Lori spent the next day going door to door to explain to neighbors that her son had an inherited bleeding disorder and would continue to be black and blue through his toddler years. Then she tried to soften their inevitable embarrassment.
"I said I was glad to be in a neighborhood where people showed concern for children," she said.
Ian is classified as a severe hemophiliac, having less than one percent of a needed clotting factor in his blood. Severe hemophiliacs can bleed frequently without noticeable cause. The biggest danger does not come from cuts and scrapes, but from internal bleeding that can damage the joints and cause severe arthritis in adulthood.
Fortunately, treatment has advanced dramatically in the past decade. Phoenix Children's Hospital is on the forefront of these advances by having the Valley's only Hemo-philia Treatment Center. The center participates in national research studies to keep abreast of new treatments. Now 11, Ian is able to prevent many bleeding episodes by taking an intravenous medication three times a week through a catheter device implanted in his chest.
The risk of HIV infection for hemophiliacs has been considerably reduced in recent years. In the past, the medication used was derived from the plasma of a pool of donors. "With every weekly treatment, hemophiliacs were exposed to the blood of 60,000 people," Lori explained. That's why so many severe hemophiliacs - about 80 percent - were infected with HIV in the early 1980s. In addition to a safer blood supply overall, hemophiliacs now have the option of recombinant DNA medication that doesn't rely on the human blood supply. Unfortunately, these advances don't come cheaply. Ian's medication alone costs $3,000 per week.
Just before his second birthday, Ian had his first severe bleeding incident. While taking a bath, he bumped his mouth and bit his lip. His parents couldn't stop the bleeding, and he was admitted to Phoenix Children's Hospital. Doctors explained that Ian had built up a resistance to the blood clotting factor he was receiving. Two units of blood were required to stop the bleeding. By that time, Ian had bled for nine days, and Lori realized how serious her son's illness could be.
Ian was given a substitute medication, but it wasn't nearly as effective. As a result, Ian had frequent bleeding episodes. After two more hospitalizations in less than two years, a PCH physician asked permission to try a new therapy. Through a central line, he gave Ian large doses of his original medication, in addition to the alternative. After about a year, Ian's body stopped fighting the factor to which he'd become immune, and he was able to go back to the more effective medication.
Ian is comfortable telling other kids about his illness when they ask about his medical bracelet. One of the most common misconceptions he has had to address are playmates' fears that if he gets hurt he will suddenly bleed to death. Hemophiliacs don't bleed any faster than anyone else, he explains. The problem comes with getting the bleeding to stop, and his medication helps his body do that.
Ian owes much of his knowledge to his mom, who is a nurse with the Hemophilia Treatment Center at PCH. He is used to being the "show and tell" portion of Lori's occasional presentations at Arizona State University's College of Nursing, demonstrating how he is able to give himself infusions.
For the first few years of her career, Lori resisted nursing assignments with hemophiliacs, feeling it was too close to home. Now, however, she realizes what an asset her experience is to others. Recently she was able to comfort a couple whose newborn son had just been diagnosed with hemophilia.
"I was handing the mom a tissue, and I flashed back to seeing myself in her chair, with someone else doing the same for me. So I told them I understood and pulled out a picture of Ian," she said. Seeing the healthy 11-year-old made them feel better.
And it made Lori feel pretty good, too.