Cystic fibrosis research: celebrating advances, helping seniors cope

Only a few decades ago, children with cystic fibrosis weren't expected to live long enough to reach elementary school.

These days, there are more adults living with the genetic disease in Canada than there are children living with it, and the median age is more than 50 years old — according to Cystic Fibrosis Canada.

"Our adult clinic opened its doors in 1979 and at that time we only had 20 patients in the clinic and the maximum age of our clinic population was 28 years old," said Dr. Bradley Quon, a respirologist at the Vancouver adult cystic fibrosis clinic at St. Paul's Hospital.

"Nowadays we have 270 patients in our clinic and there's at least five that are above the age of 70, so we've certainly come a long ways."

On Nov. 7 another year of medical strides in cystic fibrosis research will be celebrated at Cystic Fibrosis Canada's 15th annual 65 Roses fundraising gala, emceed by CBC host Gloria Macarenko.

Advances in treatment

Dr. Quon, who also researches the disease, said that new research has led to the development of a pill that can target the specific protein responsible for causing cystic fibrosis.

This protein creates abnormally thick mucus, leading to the blockage of the pancreatic ducts, intestines, and bronchi and often resulting in respiratory infection.

"I'm confident within my career that there will be a cure for cystic fibrosis, which doesn't seem unrealistic given the progress that's been made over the last 10, 15 years," Dr. Quon said.

Treatment is taxing

Leona Pinsky, whose 17-year-old daughter Rena has cystic fibrosis, said that though she is optimistic that the life expectancy for those with the diseases keeps increasing, treatment takes an extreme amount of time and effort.

"She spends about two to three hours every day doing life-sustaining treatments, she does physiotherapy two to three times a day, she takes between 40 and 60 pills a day," Pinsky said.

"We can't deny that the treatment load is very onerous...Rena for example is only able to attend school part-time because she has to spend so much time taking care of her health just to stay well enough to enjoy that time."

According to Cystic Fibrosis Canada, Canadian cystic fibrosis patients cumulatively spent 24,619 days in hospital last year — the equivalent of four months of full-time treatment per patient.

"That's a lot of time where she can't do other things, where she can't be with her friends or she has to leave early because she has to come home and do physio[therapy]," Pinsky said.

"We don't just want to increase life expectancy, we also want to try to improve the quality of that life along the way."

That's why Pinsky keeps fundraising.

"When my daughter was young...I knew I couldn't find a cure and I knew I couldn't be her doctor, but what I can do is raise money and awareness to add...year to life, and life to years for her.

To hear the full interview listen to the audio labelled: 65 Roses gala celebrates advances in cystic fibrosis research