When Renee Higginbotham’s wrists started hurting last year, she figured it was because her work-from-home setup didn’t have the proper ergonomics.
Eventually, the Cornwall, P.E.I. resident decided to see a doctor. In October, she was diagnosed with a form of scleroderma, a rare autoimmune disease that causes the scarring of her skin.
“The movement in my wrists is very limited by my skin thickening,” she said. “Opening doorknobs is a little bit more challenging than the average individual.”
Higginbotham’s lung tissue is slowly thickening, making it harder to breathe.
She’s 46 years old, and now uses elevators instead of the stairs.
“I look at how little I can do now compared to what I used to do,” she said. “If I try to take the stairs, even just one flight, I’m out of breath.”
She’s been told that eventually she may need a lung transplant, but she’s determined to avoid it.
“It can also go on to affect how I eat and swallow and digest my food.”
Scleroderma is incurable.
Higginbotham is taking what she describes as a regimen of medication, but she’s seen a lot of change in her condition in the last year.
Her doctor told her there was a possible treatment. A new drug is being hailed as a possible game-changer for patients with Higginbotham’s condition because it slows down the progression of the scarring.
The drug is nintedanib, but is commonly referred to by its brand name, OFEV.
Until now, it’s been used for other lung diseases — but not scleroderma.
It has been approved by Health Canada for scleroderma patients, but it is still not covered by any of the Atlantic provinces.
Higginbotham offered to pay for it out of pocket after her private insurance also denied her the medication.
“They quoted me a monthly cost that was astronomical and then I got off the phone and had a big cry,” she said.
“You start doing the math on that, that’s $42,000 a year, that’s a lot of people’s paycheques.”
Higginbotham has since joined Scleroderma Atlantic. The group formed just over a year ago to help spread the word about the little-known disease.
Jason Doucette, the president, estimates there could be 500 people in the region who have it.
“Many people who live with the disease live in rural areas and live with not knowing anyone else who has the disease who lives near them,” he said.
Doucette, who is based in Halifax, started advocating for scleroderma patients after his mother was diagnosed.
He says it took her years to get that diagnosis because so few general practitioners know about the condition.
“She lived with that for a number of years and then the disease progressed,” he said.
His mother died of complications from scleroderma in 2012.
Doucette says OFEV is the first medication that has given patients hope.
Scleroderma Atlantic has written to the four provincial health ministers, asking them to expedite coverage for the drug.
“Without access to this drug, certainly their quality of life would suffer and there’s also the potential that it would be fatal for them,” said Doucette.
Scleroderma Atlantic also has the support of Dr. Gokul Vidyasankar, an academic respirologist with Memorial University in St. John’s, N.L.
He focuses on interstitial lung disease, which is part of Higginbotham’s diagnosis.
Vidyasankar says until now, patients have had few options for medications.
Steroids don’t work because scleroderma doesn’t cause inflammation, but scarring.
“What this drug has been shown to do is slow down the rate of scarring by about 50 per cent,” he said.
“Although it can’t reverse the scarring that’s already in place, it does have the potential to slow down the disease and potentially change longer-term outcomes for people with scleroderma.”
Vidyasankar said Higginbotham is not alone.
He said several of his patients are frustrated that a treatment exists, but at a horrendous cost.
“Some of them have chosen to pay out of pocket which can be very expensive for them.”
CBC News reached out to the four health departments in the Atlantic region. All but P.E.I. replied.
They say the Canadian Drug Expert Committee has approved the medication, but now it comes down to cost.
Private negotiations are happening between the pan-Canadian Pharmaceutical Alliance and the manufacturer.
If they agree on a lower price, Newfoundland and Labrador’s Department of Health and Community Services says it plans to add the drug to the provincial formulary.
Both New Brunswick and Nova Scotia said it will wait to make a decision. There’s no word on how long the price negotiations may take.
Meanwhile, Vidyasankar is applauding the efforts of Scleroderma Atlantic for trying to raise awareness.
He said that can only help pressure the provinces to act faster.
“We can’t forget about the fact that there are some rarer diseases that can cause a significant impact on the individuals that are dealing with them, even if the numbers are smaller.”
For Higginbotham, every month that goes by leaves her anxious for an update.
“It’s super important. If it can improve my life, of course it’s something I need and want.”
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