Breakthoughs in Cystic Fibrosis, with Dr. Julian Tam & Juan Ianowski

From insects and birds, to the underwater world, Juan Ianowski's fascination with the natural world began early.

 

As a biologist, he was drawn to physiological processes, later scrutinizing the kidneys of insects, whose epithelial cells behave in similar ways to those in human lungs.

 

By 2015, Ianowski's research was focused on the pathophysiology of lung cells, and the nerve channels controlling them. He and his collaborators were working with the Canadian Light Source, to get a more accurate picture of the tiny cells involved.

 

But Ianowski had a nagging feeling he was missing the bigger picture. 

 

"I had a very large gap in my life," said Ianowski. "I had never seen a patient."

 

That summer, he fired off an e-mail to Dr. Julian Tam, who'd just moved into a new position as the director of Saskatchewan's adult Cystic Fibrosis clinic.

 

They agreed to go for coffee.

 

Tam already knew Saskatoon, after spending his residency in internal medicine here, followed by a fellowship in Respirology.

 

"I liked that I could reason out, based on various principles, how things worked in our lungs," said Tam. "There were a few patients that I looked after with CF fairly early on in my training, and I think they really left a mark on me.”

 

When they met, Tam found Ianowski's work interesting, but saw 'a bit of a disconnect' between the research and his day-to-day practice. 

Within months, the respirologist asked Ianowski to shadow him during patient visits.

 

Most CF patients take dozens of pills each day, as well inhaling nebulized saline solutions designed to loosen mucous, and reduce infections.   

 

“It can be very time-consuming and we would love for their treatments to be as efficacious as possible,” said Tam.

 

To figure out how, and why the saline solution worked — and what could make it more potent, Tam and Ianowski became collaborators on numerous projects supported by the Canadian Institutes of Health Information, the Saskatchewan Health Research Foundation, and Cystic Fibrosis Canada.

 

With the help of colleagues at the Canadian Light Source and the  Canadian Centre for Health and Safety in Agriculture, they started finding answers by doing mass spectrometry in real time, in a herd of pigs genetically modified to carry the gene for Cystic Fibrosis.

 

“This is pretty hard because the amount of fluid that we are looking for is very small. So it's about the width of a human hair,” said Ianowski. 

 

That work led to numerous publications, and to the University of Saskatchewan’s application for a patent for a better inhaled treatment to treat CF lung disease.

 

Now, Tam and Ianowski also looking at gastrointestinal disease in CF patients, and TRIKAFTA — an innovative class of drugs that’s effective for most — but not all — people with the disease.

 

Today, they’re testing those drugs in the lab to gain a detailed understanding of the effects they produce. A recent CIHR grant will help them better understand the basic epithelia of the lungs. They’re also looking for ways to better treat CF patients who cannot tolerate TRIKAFTA treatments.

 

“Our hope is that some of our work gives us a better understanding of the disease process,” said Tam, as he and Ianowski aim for better treatments, by figuring out which lung cells to target, and where.