Former Vancouver private investigator Bill Dean was on a Panama cruise last fall when he started feeling ill. The ship’s doctor figured he’d contracted the same virus that had hit half the passengers. A week later, Dean was admitted to Vancouver General Hospital, where he spent nearly a month. He was diagnosed with thrombotic thrombocytopenic purpura (TTP), an autoimmune blood disorder that’s as mysterious as it is rare.
Now, Dean’s throwing his support behind a young Toronto woman with the same condition who’s on a mission to raise awareness of TTP across the country.
“It doesn’t matter what your age or what your gender; anybody walking the street can get it,” Dean, 80, says in a phone call from his home in Osoyoos. “It’s absolutely crazy. People know very little about it. We don’t know what causes it, and there’s no cure.”
Potentially fatal complications can accompany TTP, in which blood clots form throughout the body. A resulting low platelet count can lead to uncontrollable bleeding. And those clots can restrict blood flow to vital organs, causing kidney, heart, or brain damage from stroke. Without treatment, 95 percent of TTP patients die.
What makes the condition so puzzling—and hard to detect—is that symptoms can be vague and vary tremendously from person to person. Signs include fever, diarrhea, chest pain, headaches, bruising, and bleeding from the nose and gums.
Dean’s main complaint was lethargy. Toronto native Sydney Kodatsky, meanwhile, started noticing a change in the migraines she’d long experienced; although they weren’t as painful as those she’d had in the past, she started having vision and memory problems. She had just gotten married and finished her MBA when she was diagnosed in 2008 at age 28.
“I wasn’t seeing properly and I wasn’t thinking properly,” Kodatsky says on her cellphone. “I was calling people by different names.”
She was eventually admitted to hospital, where doctors were initially stumped.
“Two days later, they said they were 50 to 60 percent sure that I had TTP. But they said ”˜If you do have TTP and we don’t treat you, you could die.’”
She began receiving multiple plasma-exchange treatments, or plasmapheresis—in which plasma from her own blood is replaced with that of a blood donor—and stayed in hospital for three weeks. Like Dean, she also took prednisone, a corticosteroid that suppresses the immune system (thereby slowing the progression of autoimmune diseases) but has harsh side effects, such as weight gain, hair growth or loss, facial puffiness, and mood swings.
Because it’s so rare, affecting about three people in a million, TTP is considered an orphan disease. Like other unusual diseases, TTP isn’t a priority for pharmaceutical companies—which fund clinical trials—because there’s little profit to be made. No money means no motive.
To bring TTP to the attention of health professionals and the public alike, Kodatsky founded Answering TTP. The volunteer-driven, nonprofit organization provides information, resources, and support to patients and caregivers plus raises funds for research.
Kodatsky recently travelled throughout western Canada on the TTP “road show”—which she’ll take out east next—connecting with others with the disorder (including Dean in Vancouver). Despite the shared diagnosis, they all had wildly different stories. One 30-year-old woman knew something was wrong when she discovered tiny red spots all over her legs, chest, and arms (petechiae) and started having trouble breathing. Another woman, in her 50s, had vertigo and couldn’t understand what people were saying to her.
Triggers for the disorder, according to Answering TTP, include pregnancy, cancer, infections, live vaccines, surgery, stem-cell transplant, chemotherapy, and hormone-replacement therapy. Some cases of TTP involve a deficiency of an enzyme called ADAMTS13.
Besides plasma exchange, other treatments include rituximab, a drug commonly used in a chemotherapy regimen for certain types of lymphoma, and the removal of the spleen.
Since her first medical crisis in 2008, Kodatsky has had two relapses. For all of the plasma-exchange treatments she’s needed, she estimates she’s used blood from more than 500 donors. Dean says he’s required about 400.
No wonder, then, that part of the message they want to convey is gratitude to people who give blood. They emphasize the ongoing need for donations.
“We’re begging people to donate blood,” Dean says.
Kodatsky explains it like this: each person with TTP needs, on average, about 15 plasma-exchange treatments to achieve remission, which works out to approximately 150 units of blood. Because people have to wait at least 56 days between blood donations (that’s how long it takes for red blood cells to be replaced), it would take a single donor more than 20 years to donate 150 times. “We’re so thankful to donors,” she says.
Answering TTP is a member of Canadian Blood Services’ Partners for Life program, through which corporate and community organizations can donate blood as a team.
Although Kodatsky is feeling better now than she has in a while, she says she still worries about a future she used to take for granted. But she’s determined to see support for people with TTP grow.
“I’m channelling that nervous energy into the TTP foundation,” she says. “We might be losing people who either aren’t being diagnosed or are going to the doctor too late. By working together we can change the prognosis.”