Gearing up to find the cure

BASYA LAYE

Thousands of Canadians were inspired by the poignant story of 25-year-old Eva Markvoort, a woman from New Westminster with cystic fibrosis, who was the subject of 65 Red Roses, a documentary on CBC’s Passionate Eye earlier this year. The film focused attention on CF and mobilized people from all over the country to take part in fund- and awareness-raising projects organized by the Canadian Cystic Fibrosis Foundation and its Vancouver chapter.

Those pushing for a cure will soon be embarking on the fifth annual GearUp4CF bike ride. It leaves Vancouver on June 19, winds its way through the Rockies and reaches Banff on June 27. Fifteen riders, several of whom are Jewish, are making the trip this year.

CF is the most common fatal genetic disease affecting Canadian children and young adults for which there is no cure. It is a multi-organ disease, primarily affecting the lungs and digestive system, and is most found in people of European ancestry. For Jews, it has a similar incidence rate as Tay-Sachs, another inherited disorder that predominately affects Ashkenazi Jews.

Among the riders this year are Jewish community members Leona Pinsky and Stan Shaw.

Five years ago, Pinsky, whose daughter, Rina, has CF, was approached by Dr. David Speert, a leading CF researcher at B.C. Children’s Hospital and University of British Columbia, who came up with the idea for the ride.

“Speert came to me and said that, the year before, he’d done some crazy ride from Minnesota to Maine and he wanted to do the first half, so that he could say he’d ridden across the continent.... It was like a vision quest thing,” Pinsky said. He told her, “‘I want to raise money for CF, so why don’t we see if we can get together a group to ride to Calgary?’ I said, ‘David, don’t be crazy. No one is going to ride to Calgary.’ By the time she and Speert had finished listing family members who might like to participate, Pinsky said, she had agreed to help organize the ride and, soon, they had 11 cyclists for that first year.

Seeing the first year’s riders off, Pinsky said, “I thought, I just have to do this.... The next year, we organized it again and we had even more riders and, by the following year, between Dave’s relatives and my friends and relatives, I’m sure it was like 30 percent Jewish,” a percentage, she said, that has not wavered.

Shaw came to CF through his relationship with the Pinsky family and through his wife, Dr. Hilary Vallance, a medical biochemist at Children’s Hospital, who is responsible for the province’s Newborn Screening Program. Shaw said, “Last fall, her lab began testing newborn children across British Columbia for cystic fibrosis. This has helped to pave the way for earlier detection, which has enormous benefits in improving outcomes for children with this inherited genetic condition.”

In an e-mail, Shaw wrote, “I first met the Pinskys through Vancouver Talmud Torah, where Rina and my youngest daughter, Naomi, have become friends. Two years ago, Leona and [her husband] Max rode the same massive ride on behalf of their daughter to help raise research funds for CF.... Knowing I was an avid cyclist, they asked if I would ride in their place last year. The ride was unforgettable. I documented it in an online journal, filled with spectacular photos and wonderful stories.”

Limited to 20 riders, Pinsky and Shaw said the ride is singular in the disease-fundraising roster. “We’re not trying to compete with the Ride to Conquer Cancer. This is a whole different thing,” Pinsky said. “GearUp is for people who want something a lot more, I think, personally meaningful, where you’re really connected to the cause, one where everyone on that ride is deeply connected and you really get to know people intimately and you really challenge yourself. It’s physically challenging, but it’s emotionally challenging too. A lot of getting up those hills is here,” she said, pointing to her head, “I just think about people with CF who operate on 50 percent lung function all the time. I can get up that hill – I have 100 percent lung function. You just think about people we’ve lost, people who are ill, the hope you have for young people now ... and you just keep pedaling.”

Most people don’t know much about CF and that contributes to some of the urgency in raising awareness. Because babies born in previous generations with CF didn’t survive very long, there hasn’t been a good way to chart long-term mortality statistics, meaning that, “you’ve got that whole cohort that’s not here,” said Pinsky. “There are only about 3,500 people in Canada with cystic fibrosis. Even though it’s one in 3,000 births, traditionally, they didn’t live very long. That’s changing, thank God, and we’re now in a place where, last year, we passed a very important milestone. We actually have more adults than children in Canada with CF.”

Shaw is riding for Roman, 11, and his identical twin sisters, Madison and Hailey, 8; three siblings whose story underscores the importance of early detection. “Roman was four years old before he was diagnosed,” said Shaw. “Everyone thought he had asthma. He used to have trouble breathing [and] it wasn’t until his twin sisters were born and, a year later, they were not thriving, that they got a work-up and found out that they have CF. They thought, oh my God, we’ve got to check Roman and, sure enough....”

The treatment regiment is expensive, intense, time consuming and energy sapping. It includes pancreatic enzymes to aid in nutrient absorbption and physiotherapy, which generally includes inhaled Ventolin and other medications, along with manual back-patting to aid with mucous clearing. Though difficult in its own right, Pinsky is grateful for their effectiveness: “We’ve made tremendous progress and these kids and young adults are living good, high-quality lives. It’s not just longer lives. One of the slogans [for CF] is ‘Adding years to life and life to years.’”

Since their daughter was born, Pinsky said, median life expectancy has gone up. “It’s tending towards the early 40s,” she said, “but, you know, numbers are numbers. Median means half the people make it. We buried our close friend’s daughter two months ago, at age 25.” She added, “There’s tremendous progress, but it’s really all about the money.... We have unbelievable science in Canada and, yet, you have to fund it.”

Shaw agreed: “I think we’re looking at the advances in molecular genetics but also in the way kids are being treated and improving outcomes. That’s really the goal right now. Get on top of these infections and find ways that they can be healthier and be able to absorb nutrients.”

Pinsky said organ transplant “can add some time at the end but, the thing about a lung transplant, it’s not a cure. You still have CF in the rest of your body and [now] you have transplant issues, which are difficult as many people have seen with the death of Eva Markvoort; her body rejected her lungs after two years.... Having said that, of course, thank God she had those extra two years.” Markvoort died at the end March.

Pinsky said, “Nobody wants to be an organ donor, nobody wants anyone in their family to be an organ donor, but if tragedy has to happen, then I think there would be tremendous comfort in knowing you helped someone else. Our main thrust is that we want a cure for cystic fibrosis. Having said that, a portion of our funds does go to support transplant research and transplant clinics. For a lot of people, it can add years.” Pinsky said those in the Jewish community can visit the Halachic Organ Donor Society website (hods.org) to learn more about Judaism and transplants.

Pinsky believes strongly in the local high-level research effort. “Every year, they know more and more. There’s tremendous hope for kids like Rina; in the short term, that they have better treatments and can help her have a better quality of life but, hopefully, in the long term, too, they can do something more permanent.... The [research] cascades like dominos. You do one and you can’t always predict where your discovery will lead. But it’s really important to do the basic science so that you can start that process.” She added, “Stan and I can’t do the science, but we can ride the bikes and raise the money.”

Shaw feels very connected to the ride. “There is something about riding. After nine days, you really get to know people well. I’ve done other group rides and this one is really pretty special. They’re riding not just to ride and there’s motivation there.... You realize what you’re doing is really important.”

About an experience he shared with one of his fellow riders, Shaw said, “One of the neat things about having [some researchers] on the ride was that it made it really relevant for them. [They] spend hours and hours at a lab bench, doing the testing, exploring avenues in mechanistic research [to] find out what’s really going on; it’s a huge commitment, a life commitment. So, they come out on these rides and it really brings it home for them.”

Pinsky echoed the sentiment. “I’ve seen grown researchers tear up when they meet someone with CF. They talk to them and they’re like, Oh my God. This is not, just, bacteria in a Petri dish. It’s in this little girl’s lungs and I made a difference. It’s really powerful for them to meet the families or hear the stories, even to go to the funerals. It makes a difference for them.”

Shaw concluded, “And, for us, when we’re going up those mountains and we’re breathing fresh mountain air, we think about that. How fortunate we are.”

With the ride quickly approaching, Shaw said, “we’re still working hard to raise funds. There’s lot of opportunity to help support this.”

Shaw will be blogging about the trip and readers can follow along at gearup4cf2010.blogspot.com. Each rider’s personal story, ride information and sponsorhip opportunities are at gearup4cf.org. For information on CF, visit cysticfibrosis.ca.

^TOP