First province begins funding new life-extending treatment for patients with rare lung disease

- Successful pan-Canadian Pharmaceutical Alliance negotiations for Uptravi lead to quick action by Quebec's public drug program, generating hope elsewhere in Canada -

VANCOUVER and HAMILTON, ON, March 6, 2018 /CNW/ - The Pulmonary Hypertension Association of Canada (PHA Canada) and Scleroderma Canada (SC) were unanimous today in their praise of the Quebec government for swiftly providing public funding for Uptravi (selexipag) for the treatment of pulmonary arterial hypertension (PAH). Effective as of March 1, 2018, this action stems from the recent conclusion of pricing negotiations for Uptravi by the pan-Canadian Pharmaceutical Alliance (pCPA). The PAH community is hopeful that all other provinces and territories across Canada will promptly follow suit to ensure that PAH specialists are able to provide optimal, individualized treatment to improve the outcomes for patients living with this potentially fatal disease.

In January 2016, Uptravi became the third new treatment for PAH to be approved by Health Canada in recent years. Later that year, the Common Drug Review (CDR) also recommended Uptravi be publicly funded in Canada.

"In October 2016, Uptravi was recommended for public funding in Canada because it provides an important option for PAH patients whose disease is not being adequately controlled with a first- and second-line therapy, and for those who are unable to receive continuous intravenous or subcutaneous infusion therapy due to the burden and risks involved. Today, we are gratified that Quebec has acted on that recommendation by providing public funding for Uptravi," says Dr. Sanjay Mehta, MD, FRCPC, FCCP, Director of the Southwest Ontario Pulmonary Hypertension Clinic at the London Health Sciences Center in London, Ontario, and Board Chair of PHA Canada. "The severe and progressive nature of PAH means that patients must have timely access to all Health Canada-approved treatments for PAH in order to individualize their treatment to improve and extend their lives."

PAH is a rare but very complex and serious lung disease. It is defined by high blood pressure in the lungs, which leads to enlargement and weakness of the right side of the heart – a type of heart failure. PAH is a common complication of systemic scleroderma (SSc), a chronic hardening and thickening of the skin and internal organs, and can be very severe in patients affected by this progressive connective tissue disease.

"I am thrilled with our government's decision to make Uptravi accessible through public funding. This is a major victory for scleroderma patients affected by PAH in Quebec," said Diane Collard, director general of Sclérodermie Québec (SQ). "Congratulations to everyone who has worked hard in support of this initiative, and thanks to the Government of Quebec for making the right decision and supporting those affected by PAH, including systemic scleroderma-PAH."

There is currently no cure for PAH and while the prognosis for patients has improved somewhat in recent years, it remains poor despite treatments available in Canada. The average survival in adults following a PAH diagnosis is estimated at only five to seven years, and only three years for those affected by scleroderma-associated PAH (SSc-PAH). This means that patients do not have the time to wait for necessary, life-extending treatment options to be made accessible through public funding.

"Today's announcement is important to me for two reasons. First, I am happy that all PAH patients in Quebec who can benefit from Uptravi will now have access to this important treatment option. Second, as a patient, I will personally benefit from this decision, which is a huge relief," says Judith Moatti, administrator of the Fondation Hypertension artérielle pulmonaire Québec (HTAPQ) and PAH patient. "The Government of Quebec has once again demonstrated its leadership when it comes to ensuring public access to innovative treatments for this rare disease. I now invite other provincial and territorial governments across Canada to follow Quebec's lead and move swiftly to provide immediate publicly funded access to Uptravi, so all PAH patients can have access to this treatment."

The PAH and scleroderma communities are optimistic that all patients living in Canada who rely on provincial or territorial drug plans will soon have access to Uptravi.

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SOURCE Pulmonary Hypertension Association of Canada

For further information: Jamie Myrah, Executive Director, Pulmonary Hypertension Association of Canada, 604-682-1036 ext. 101,; Anna McCusker, Executive Director, Scleroderma Canada, 289-396-3039,

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