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From constantly coughing to clearer lungs: a CF patient’s journey on a new treatment

Toronto, May 25, 2015

By Melissa Di Costanzo

Chris Macleod and Dr. Elizabeth Tullis
Chris Macleod lives with CF and can breathe easier thanks to novel treatment changes largely introduced by Dr. Elizabeth Tullis, director of St. Michael’s Adult Cystic Fibrosis Clinic. (Photo by Katie Cooper)

Chris Macleod, a Toronto lawyer who lives with a rare form of cystic fibrosis, experienced a flare-up in the summer of 2012 that sent him to 6 Bond for the better part of four months.

When in hospital, Macleod remembers choking and gasping for air. He had been forced to carry an oxygen tank and considered a lung transplant.

That all changed in the fall of 2012 when Macleod began taking Kalydeco, a groundbreaking medication that helps a small number of CF patients breathe easier. Kalydeco is the only drug that treats the underlying cause of CF: a faulty gene and its protein product.

Since starting on Kalydeco, Macleod has married Gloria, an elementary-school teacher, travelled to Albania, the Philippines and Mexico, and is back to work as a commercial litigator.

Macleod hasn’t been hospitalized since. His lung capacity has doubled and he no longer needs an oxygen tank.

“And we’re still progressing,” Macleod said. “That’s the difference Kalydeco has made.”

  May is Cystic Fibrosis Month
#CFAwarenessMonth

Health Canada approved the drug in 2012 for use in people who carry the G551D mutation. Ontario agreed to fund the drug last fall. It carries a staggering annual price tag ($300,000), and is licensed only for use in those three per cent of CF patients with specific CF mutations.

Dr. Elizabeth Tullis, director of the hospital’s Adult Cystic Fibrosis Clinic, the largest in North America, helped to ensure Kalydeco was available to Canadians.

“I’ve never seen anything like this,” Dr. Tullis said. “People with CF on Kalydeco don’t need hospital admission, are able to work or attend school, and they feel better than they have in years. It’s very overwhelming as a CF physician to see how this has changed people’s lives.”

St. Michael’s was the only site in Canada to participate in the early Phase 2 clinical trials for the drug and had the largest number of patients enrolled in the Phase 3 trial.

Dr. Tullis said the next challenge for researchers to tackle is the common mutation seen in 90 per cent of Canadians with CF.

  Quick facts about CF
  • Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system
  • A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food
  • CF is the most common fatal genetic disease affecting Canadian children and young adults
  • The CF gene was discovered in Toronto at the Hospital for Sick Children in 1989

Fixing this mutation may require more than one medication. Based on results of Phase 2 and 3 studies, a combination of two drugs has already been submitted to Health Canada. If approved, this combination would likely carry a price tag similar to that of Kalydeco.

There may be other options. Some drugs that have been approved for use in different diseases could also correct the CF protein defect and one such medication is being studied at St. Michael’s – the only site in Canada.

“Our responsibility is to participate in as many of these studies on new drugs as possible,” said Dr. Tullis. “We don’t know what the right medication is going to be for these patients but the more quickly these studies are done, the faster we will find the best solution.”

Dr. Tullis is also celebrating $500,000 in new annual funding from the province, which has allowed her to hire another nurse, a social worker, a physiotherapist, a pharmacist and a clerical position.

About St. Michael's Hospital

St. Michael’s Hospital provides compassionate care to all who enter its doors. The hospital also provides outstanding medical education to future health care professionals in 27 academic disciplines. Critical care and trauma, heart disease, neurosurgery, diabetes, cancer care, care of the homeless and global health are among the hospital’s recognized areas of expertise. Through the Keenan Research Centre and the Li Ka Shing International Healthcare Education Centre, which make up the Li Ka Shing Knowledge Institute, research and education at St. Michael's Hospital are recognized and make an impact around the world. Founded in 1892, the hospital is fully affiliated with the University of Toronto.