Canadian Scleroderma, PH Groups Collect Over 2,000 Letters Urging Access to PAH Treatments

Scleroderma Canada and the Pulmonary Hypertension Association of Canada have collected more than 2,000 letters signed by pulmonary arterial hypertension (PAH) patients, their caregivers and loved ones urging politicians to make all PAH treatments accessible as soon as possible through public funding.

Health Canada approved Actelion’s Uptravi (selexipag) in January 2016 — only the third such treatment to be approved in recent years. In October 2016, the Common Drug Review (CDR) recommended that Uptravi be publicly funded for patients whose disease is not adequately controlled with first- and second-line therapies.

But Uptravi is not yet accessible to patients who depend on public funding due to long negotiations through the pan-Canadian Pharmaceutical Alliance (pCPA). Except for Actelion’s Opsumit (macitentan), which is publicly funded only in Quebec, none of the recently approved PAH treatments have been made available to Canadians through the country’s publicly funded prescription system.

“PAH patients have high hopes for Uptravi as a new treatment option to slow the progression of this rare but very complex and serious lung disease that we know significantly limits life expectancy,” Anna McCusker, executive director of Ontario-based Scleroderma Canada, said in a press release.

“As we deliver these letters, there are patients in hospital fighting for their lives who are unable to access the treatment they need,” added McCusker. “We are hopeful that the voices of so many Canadians will be heard by decision-makers, and that patients will immediately have access to a treatment option that is urgently needed by some, and seen as a last resort by others.”

The two organizations are now in the process of delivering the 2,000 letters to premiers and health ministers across Canada, including the provinces of Manitoba, Ontario, Quebec and Nova Scotia.

PAH is defined by high blood pressure in the lung area, leading to enlargement and weakness of the right side of the heart. It is a common complication of systemic scleroderma (SSc), a chronic hardening and thickening of the skin and internal organs. In this case, the disease is called scleroderma-associated PAH (SSc-PAH).

“Since my diagnosis with scleroderma, and more recently pulmonary arterial hypertension, my life has become quite limited as I am not able to work or live on my own, and the treatments I have been taking are no longer enough,” said Bronwyn Lawton, 33, who lives in Winnipeg with her parents, who are also her primary caregivers. “I depend on public funding for my medication and I simply cannot afford the new treatment my doctor tells me I need now. I desperately hope that governments across Canada hear our urgent plea.”

Around 10 percent of scleroderma patients develop PAH due to the thickening of connective tissue around blood vessels. When these blood vessels are blocked or narrowed, the right ventricle of the heart must work harder to pump blood to the lungs. This can lead to heart failure, and without proper treatment, PAH can be fatal.

Even with treatment, the average adult survives five to seven years following a PAH diagnosis. Those awith SSc-PAH live on average only three years after diagnosis. Canadian patients make clear in their letters that they simply don’t have time to wait for already approved, life-extending treatment options to be made available through public funding.