At the end of a successful Pulmonary Hypertension Awareness Month, members of the Pulmonary Hypertension Association of Canada (PHA Canada) and Scleroderma Canada (SC) have come together to thank provincial governments for providing the majority of Canadians with public funding for Uptravi (selexipag) for the treatment of pulmonary arterial hypertension (PAH). In the past month, both British Columbia and Nova Scotia have approved Uptravi for public funding for eligible patients, making this novel therapy now accessible in nine out of 10 provinces. This result stems from the conclusion of pricing negotiations for Uptravi by the pan-Canadian Pharmaceutical Alliance (pCPA) in December 2017. The PAH and scleroderma communities are relieved that Canadian PAH specialists are now able to provide optimal, individualized treatment to improve the outcomes for patients living with this potentially fatal disease.

"We are thankful that the voices of the PAH and scleroderma communities have been heard. Today, we celebrate the accessibility of Uptravi through public funding from coast to coast," says Jamie Myrah, Executive Director, Pulmonary Hypertension Association of Canada. "We have always believed that PAH patients should have equitable and timely access to optimal treatment options in order to manage their disease. We are thrilled that this is now the case for the vast majority of Canadians in need of Uptravi."

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. 

"We are so pleased to be able to tell our patients that Uptravi is now accessible through public funding in almost every province," 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. "Because of the highly complex and serious nature of PAH, specialists need the ability to prescribe optimal treatment options and offer the individualized care required to improve and extend the lives of our patients, no matter where they live in Canada."

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.

"Despite this positive news and a step in the right direction, patients living with PAH still have to manage the emotional and psychological burden associated with this rare disease. Patients face the prospect of more complex and invasive medications, possible lung transplantation, the high risk of disease progression and a shortened life expectancy," says Maureen Sauve, VP Public Relations and Advocacy, Scleroderma Canada. "Our communities will continue to urge all governments to ensure all Health Canada-approved treatment options are accessible to those in need."

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, potentially life-extending treatment options to be made accessible through public funding.

The PAH and scleroderma communities are hopeful that provincial and territorial governments will continue to support PAH patients and make all remaining Health Canada-approved treatments accessible through public funding.