Experimental treatment saved life of retired Hamilton firefighter with rare disease

Tom Salisbury didn't think much of his chest cold when he got it in January 2014. He didn't know it then, but the cold was the first symptom of a rare disease that could have killed him.

The 54-year-old Hamilton man spent decades fighting fires — but he was having trouble knocking down this cold.

Antibiotics didn't help. Then, his joints started to ache — shoulders, knees and elbows.

Still, Salisbury wasn't alarmed. Although he was now retired, he worked a physically demanding job, played sports and was healthy all his life.

Five months later, in June, things changed for the worse. He was coughing up blood.

"I was concerned … your future is uncertain," Salisbury said.

His 26-year-old daughter Maddie, a kinesiology student finishing her last year in school, was worried too.

"I was shocked … it was a pretty emotional time for myself and my mom and my other sister."

The disease is a mystery

It turned out that Salisbury had a rare disease called ANCA-vasculitis. Dr. Michael Walsh, at St. Joseph's Hospital, told him that if he hadn't come to the hospital, the disease would have, almost certainly, killed him.

The illness causes one's own immune system to attack their body by targeting the blood vessels and forcing them to balloon in size.

In Salisbury's case, the disease latched onto his lungs and made them bleed. For about one in four, vasculitis causes fatal kidney failure and about one in three patients end up with a serious infection, which is the most common killer linked to vasculitis.

The sickness is a mystery for medical experts. They don't know how it starts or how to kill it, which means it never quite disappears.

While the disease affects about 20 people in a million, Salisbury's lengthy journey to get diagnosed is common among all victims of vasculitis.

"To get the correct diagnosis, first, you have to deal with the inflammation that's causing so many problems," Walsh said.

"In the old days, it was almost 100 per cent fatal in the first year … now we expect about 80 per cent of patients to have a good outcome in the first year."

Embracing uncertainty

But the day after Salisbury was diagnosed, Walsh offered him a chance to embrace uncertainty — the very thing he feared.

"Half the battle is getting over it," Walsh said.

With all the questions swirling around treating the disease, Walsh and a team of researchers decided to pursue the largest clinical trial linked to vasculitis. They wanted Salisbury to be a part of it.

The study involved more than 700 patients and 95 centres in Canada, Australia, the U.S. and the U.K.

The two prevailing treatment options for the disease are blood transfusion to exchange plasma cells and a heavy dose of steroids — the team tested them both.

First, half of the patients did a plasma exchange and half didn't.

The results showed the plasma exchange, while being a very common but expensive and tedious method, was actually more harmful than helpful. Not only did it fail to improve patients' prognosis, it also further disrupted their immune system and exposed them to the potential of bloodborne illnesses.

Then, half of patients used a regular steroid dose while the others used a reduced dose.

Walsh and the team were able to drop the prednisone dosage by 40 per cent, which caused a 30 per cent drop in serious infections.

"That was a really big win for us because everyone was using steroids but they were being used in many different ways and often times in very high doses and we were able to reduce the most common cause of death," Walsh said, adding that a lower dose also means fewer side effects.

The team published their findings in the New England Journal of Medicine on Thursday.

Seeing Walsh and his team help Salisbury fight his battle inspired his daughter Maddie to change her career path. She attended post-grad school to become a pharmacy technician which eventually landed her a job at St. Joseph's as a research consultant.

"I was so captivated by the type of life-changing research going on," she said.

Walsh said Maddie likely doesn't have the disease.

The disease returned in 2018

Salisbury entered remission not long after 2015, about a year after his first symptoms surfaced. He felt fine most days, which was all he could have hoped for.

But it was short lived.

"Last year I started sliding back into relapse, the symptoms came back stronger," he said.

The disease returned in 2018. "It brought up the old feelings again," he said.

This time, his disease wasn't a complete enigma. The team at St. Joseph's treated him and Salisbury says he's been symptom free since the summer.

But, he'll never be completely off the hook.

"It is a life-long disease so I can expect this probably to happen again maybe at some point. I hope not, but from what I understand it's quite a likely possibility."