A Halifax woman's battle with a rare, incurable liver condition

Sarka Kralicek didn't know what was wrong with her at first. 

In January 2011, she began feeling a persistent, tormenting itch.

"It literally felt like I had a million mosquito bites underneath the skin, 24 hours a day. It was all over my body with no reprieve," Kralicek, 48, told CBC's Information Morning.

"You go from a point where you're itching a little bit to where you're tearing your skin apart."

Soon after, exhaustion and joint pain began setting in.

"I was always tired and couldn't seem to get in front of the fatigue," she said.

It would take nine months after she first started showing symptoms for Kralicek to be diagnosed with primary biliary cholangitis (PBC) — a little-known chronic liver disease.

Condition difficult to diagnose

Before her diagnosis, Kralicek's symptoms baffled doctors, who conducted MRIs, ultrasounds and biopsies. She eventually became jaundiced, indicating the problem was with her liver and not her skin.

Finally getting that diagnosis changed Kralicek's life.

"It's scary because it wasn't something that I actually knew … but it's also something that gives you an answer," she said. "So you actually know where you're going, what to do about it, and looking for the best optimal treatment."

For the first few years, Kralicek's treatments weren't working. She was on her way to needing a liver transplant, but began a new treatment last spring and has since been doing much better.

Now, she's trying to raise awareness for PBC so others might be able to recognize the signs early on.

"They may not know what it is, or what's out there to help them … the symptoms are so broad that you really need to keep on digging," she said. 

"PBC is not something that you find right away. You really have to investigate because there could be so many answers."

Many patients are asymptomatic. According to the PBC Society of Canada, 60 per cent of patients are diagnosed without symptoms.

Disease disproportionately affects women

Dr. Magnus McLeod, who treated Kralicek and teaches at Dalhousie University's medical school, said PBC attacks the bile ducts within the liver, impairing the flow of bile.

"Bile is a toxic substance, so if the flow is impaired within the liver, it slowly causes damage over time, and that damage leads to inflammation and scarring," he said.

"That scarring, if let go long enough, will develop into cirrhosis and will lead to death unless a liver transplant is done or unless treatment is started."

The rare condition affects women four to five times as much as men, he said.

About 11,000 patients are thought to have the disease in Canada, and McLeod said about one in 1,000 women over the age of 40 are living with it. 

In Atlantic Canada, he said the prevalence rate is higher, though experts can't say why.

"We know that genetics are a factor and the environment is a factor, but we don't have a specific trigger that we know about," he said.

McLeod noted it's difficult to come up with an exact number of people who have the illness because many more could be undiagnosed.

The disease is incurable, but treatments are available. 

For the past two decades, the only option was a synthetic bile acid that helps remove the damaging effects of the bile from the liver.

Within the last two years, a new agent has been made available that improves the bile flow. This agent is used on patients who don't respond to the first treatment option — including Kralicek.

Other treatments focus on relieving PBC's side effects.

More awareness needed

McLeod also said PBC needs to have more recognition inside the medical community.

"Family physicians are not as aware of PBC as they probably should be," he said. "It is taught in medical school, but it's not seen very often."

He said anyone who has common symptoms like fatigue, itching and joint pains, and who has also had abnormal liver tests for more than six months should be investigated for PBC.

By going public with her story, Kralicek hopes to help others who may unknowingly have PBC get the treatment they need.

"For me now, I've been six years without itching. It's been the best present I possibly could've gotten," she said with a laugh. "I'm actually in control now. I know I won't need a transplant."