When Renee Higginbotham’s wrist started hurting last year, I realized it was because the work-from-home setup didn’t have the right work environment.
In the end, the people of Cornwall, a resident of Prince Edward Island, decided to see a doctor. In October, she was diagnosed with a form of scleroderma, a rare autoimmune disease that causes scarring on her skin.
“Movement in my wrist is very limited due to the thickness of my skin,” she said. “It’s more difficult to open the door handles than the average person.”
Higginbotham’s lung tissue slowly thickens, making breathing more difficult.
She is 46, and now uses elevators instead of stairs.
“I look at how little I can do now compared to what I was doing,” she said. “If I try to climb stairs, even if it’s only one flight, I get upset.”
She was told she might eventually need a lung transplant, but she was determined to avoid it.
“It can also continue to affect the way I eat, swallow and digest my food.”
Scleroderma is incurable.
Higginbotham takes what she describes as a regimen of medication, but has noticed a lot of change in her condition in the past year.
Her doctor told her there was a possible treatment. A new drug is being hailed as a potential change agent for patients with Higginbotham’s condition because it slows the progression of scars.
The drug is nintedanib, but is commonly referred to by its brand name, OFEV.
So far, it has been used for other lung diseases – but not scleroderma.
It is approved by Health Canada for scleroderma patients, but is still not covered by any of the Atlantic provinces.
Higginbotham offered to pay for it out of pocket after her private insurance deprived her of the drug.
“They charged me with a monthly cost that was astronomical and then I left the phone and screamed so hard,” she said.
“You start doing the math on that, and that’s $42,000 a year, that’s a lot of people’s salaries.”
Higginbotham has since joined Scleroderma Atlantic. The group formed a little over a year ago to help spread the word about the unknown disease.
Jason Doucet, president, estimates that there could be 500 people in the area who have it.
“A lot of people with the disease live in rural areas and live with not knowing anyone else with the disease who lives near them,” he said.
Doucette, who is based in Halifax, began advocating for scleroderma patients after his mother’s diagnosis.
He says it took her years to get that diagnosis because so few general practitioners know about the condition.
“She lived with that for several years and then the disease developed,” he said.
His mother died of complications from scleroderma in 2012.
Duckett says OFEV is the first drug to give patients hope.
Scleroderma Atlantic has written to the health ministers of the four counties, asking them to speed up coverage of the drug.
“Without access to this drug, their quality of life will definitely suffer and there is also the potential for it to be fatal to them,” Doucet said.
Scleroderma Atlantic also has the support of Dr. Gokul Vidyasankar, Academic Respirator at Memorial University in St. John’s, NL
Focuses on interstitial lung disease, which is part of Higginbotham’s diagnosis.
So far, Vidyasankar says, patients have had few options for medication.
Steroids do not work because scleroderma does not cause inflammation, but rather scarring.
“What this drug has been shown to do is slow the rate of scarring by about 50 percent,” he said.
“Although it cannot reverse existing scars, it has the potential to slow the disease and possibly alter long-term outcomes for people with scleroderma.”
Vidyasankar said Higginbotham is not alone.
He said many of his patients are frustrated that there is a cure, but at a terrible cost.
“Some of them chose to pay out of their pocket, which could be very costly for them.”
CBC News has reached out to the four health departments in the Atlantic region. Everyone answered except for Prince Edward Island.
They say the Canadian Pharmaceutical Experts Committee approved the drug, but now it’s a matter of cost.
Private negotiations are underway between the Canadian Pharmaceutical Alliance and the manufacturer.
If they agree on a lower price, the Newfoundland and Labrador Department of Health and Community Services says it plans to add the drug to the regional formulary.
Both New Brunswick and Nova Scotia said they would wait to make a decision. No word on how long the price negotiations might take.
Meanwhile, Vidyasankar praises Scleroderma Atlantic’s efforts to try to raise awareness.
This will help pressure the provinces to act faster, he said.
“We cannot forget the fact that there are some rare diseases that can cause a huge impact on the individuals who deal with them, even if the numbers are lower.”
For Higginbotham, every passing month makes her anxious for an update.
“It’s so important. If it can improve my life, it’s something I need and want, of course.”
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