World PH Day and Paint Canada Purple!
May 5, 2015 marked the 4th
annual World PH Day and, throughout the country, PH supporters put on their best purple outfits in support of those living with PH. We are amazed at how much our community mobilized and came together to spread the word about this rare disease! Social media channels were flooded with photos of PH community members—pets included! —looking snazzy in purple. We collected all the photos we found and compiled a video that shows how our PHamily came together for World PH Day! Click here
to watch the video.
Our PHA Canada Ambassadors were also very active for World PH Day. Thanks to their efforts, 14 Canadian monuments were illuminated in Periwinkle for the cause. From Vancouver, BC, to St-Ferdinand, QC, monuments shone bright in purple—a gesture that both celebrated the PH community and spread awareness about PH. Thank you to our Ambassadors and community members who helped us Paint Canada Purple
and who shared their photos of the event! Here’s a list of monuments that were illuminated for World PH Day:
, Vancouver BC
, Vancouver BC
, Calgary AB
, Calgary AB
Telus Spark Centre
, Calgary AB
, Okotoks AB
High Level Bridge
, Edmonton AB
, Toronto ON
, Toronto ON
Ottawa Heritage Building
, Ottawa ON
, Montreal QC
, Victoriaville QC
Hotel de Ville
, Plessisville QC
Manoir du Lac William
, St-Ferdinand QC
As its name indicates, World PH Day was a global event and activities were organized worldwide to mark the day and spread awareness. Here’s a sample of photographs documenting awareness-raising activities that took place around the globe. For more information about these activities visit www.worldphday.org
or lookup #WorldPHDay.
Conference 2015—A Breath Away: PHocus on the Future
We were delighted to welcome 231 attendees at our 4th National Conference, which was held in Montreal from May 1 to 3. We loved seeing old Phriends connect, we were happy to meet new members to the PHA community, and we were amazed at how much the kids had grown since the last Conference! Above all, we were delighted to see the community come together to share inspirational stories and find the support of others who are also living with PH. Our hope is that Conference attendees were able to bank up on positive energy and came back home with a better grasp of PH and with tools to make living with PH easier.
We are very grateful to our 55 speakers who shared their stories and knowledge with our guests and whose contributions to our program are invaluable. We also want to express a very special “thank you” to all our volunteers, without whom the Conference would not have been possible! Thank you for having given of your time and energy to the event by making the weekend run as smoothly as possible! We are grateful for all the help we received and want to recognize the special contributions of some of our volunteers who have earned gold stars:
Conference Award Winners
- Wendy Bédard, Kendall Tracy, Lynndel Tracy, and Jeffrey Dale Barnes, whose efficient bag-stuffing system made our Conference prep a lot easier and who were available to help whenever and wherever we needed them throughout the weekend;
- Jas and Rick James and Margaret Lehmann, who were there to lend a hand throughout;
- Bill Cook, who stepped in for anything and everything that was needed;
- Amanda English, Melissa Hutchings, and Sarah Platnar, who turned the Kids’ Room into a real treat—for both kids and parents;
- Payton Snobelen, who stood in as big sister to all the kids in the Kids’ Room;
- Allistair and Kody Greenfield, who were on hand throughout the weekend;
- Lise Demers, who joined the film crew and took charge of the French interviews;
- Micheline Richard, who wore many hats throughout the event;
- Ed Raythoni, who helped us set up and pack up everything at the end of the Conference!
- All of our PHA Canada Ambassadors who stepped in wherever needed, including filling in some important speaking roles at the last minute
We’d like to take the opportunity to honor our 2015 Conference Award winners! Their courage and dedication is an inspiration to us all.
Outstanding PH Caregiver
WENDY AND ROCH LAVIOLETTE
–Nominated by Dianne Curle: “I was diagnosed with CTEPH in 2013, and chose to undergo PEA surgery for a better prognosis. Because this surgery isn’t available in my home province of BC, I made the decision to go to the Ottawa Heart Institute. I called Wendy and Roch who live in Ottawa and I told them my situation, and they said they would be more than happy to be my caregivers. I left for Ottawa in July 2014 and underwent surgery on August 6th, facing another surgery the week later because of complications. During my stay in hospital, Wendy and Roch kept friends and family abreast of my progress, emailing and delegating. When I returned to their home to recover, they fed me, supported me and brought me everywhere for tests.
I have a hard time describing how special Roch and Wendy have been through my journey with this rare surgery, taking time out of their busy lives to be totally there for me. I returned to BC on September 23rd, after an incredible healing time with my caregivers. They never wavered from their responsibility in this gigantic and serious event in my life, and thanks just seems so minimal. Their strength truly helped me heal physically and emotionally and I will never forget their total generosity.”
Outstanding Medical Professional
DR. DAVID LANGLEBEN
–Nominated by Rita Hébert and Colette Dambroise Dubé: “
We nominate Dr. Langleben because he does everything in his power so that his patients may have access to the best possible care, and to the treatments necessary to their quality of life. He extends a humane touch towards all of his patients. He also makes himself available 24/7 and in emergencies by providing patients with his pager number and email. We’re always able to count on Dr. Langleben: he’s attentive to the needs and to the particular case of each person he treats. He is extremely dedicated to patient care: he even accompanied a patient in an ambulance all the way to Toronto so that she could receive her lung transplant. Dr. Langleben also opened the very first PH clinic in Canada, and is also a professor and researcher. For these reasons, he deserves this recognition.”
–From PHA Canada: Beyond being a tremendous support, resource and always available to his patients, Dr. Langleben has also been instrumental in building the PH community in Canada, from the very first support group in Montreal to his recent efforts helping PHA Canada secure much needed funds to make the Conference a success. Dr. Langleben is truly deserving of this award for his unwavering dedication to the PH Community.
Outstanding PH Patient
–Nominated by Cheryl Salvador: “Lynn Marie is an outstanding patient who has been a role model to all of our pulmonary hypertension patients in our program. She is compassionate, courageous and motivated. Despite her challenges with PH, she has worked hard to create the Northern Alberta Chapter of PHA Canada where she has gathered all PH patients, caregivers, supporters and medical staff to support one another and bring awareness to this disease. I am so very proud to have her as a patient due to the strength she carries to lead the Northern Alberta PH group.”
Outstanding PH Community Contributor
–Nominated by Sandy Vachon, Fayza Rifai, Hugues Boulanger, Line Ducharme, Dolores Carrier, Colette Dambroise Dubé and Judith Moatti: “Denis is the president of the HTAPQ Foundation for PH patients. He founded this association to help his daughter, Isabelle, who had been diagnosed with PH. Sadly, Isabelle passed away due to her PH in 2011, but Denis never lost his passion for this cause. Despite Denis being retired, he volunteers all of his time (7 days a week) towards a better life for all PH patients. He organizes fundraisers several times per year, as well as a weekend Conference every two years to unite all PH patients and caregivers in Quebec. 4 times per year, Denis puts together a little newsletter to keep all members up to date on important PH news and events. Denis’s Foundation provides direct financial support to those affected by PH, and funds help to pay for medications, oxygen, other necessary machines, travel and accommodation costs for hospital stays, and even meals when necessary. Denis is always there to be emotionally supportive to patients as well: whenever you need to turn to him to talk about any problems, he is always there. It’s because of his work that patients across Quebec know one another. He creates opportunities for all of us affected by PH to connect with one another and share our stories, even providing funds for patients to be able to travel and get together with one another. Finally, Denis is a devoted advocate to PH awareness and access to treatment, and he went personally to deliver letters from PH patients to the Quebec Health Minister to insist that they help fund PH treatments. Denis is an exceptional soul who is completely dedicated to PH patients in our community, and we hope that this year he may be recognized for his incredible contributions.”
PHA Canada's Pediatrics Committee has put together a list of resources for parents/caregivers of children with PH. Resources included in the School Package are specially designed to assist parent in the process of informing a school (or daycare or camp) about a child's needs and conditions. The School Package
Mohamad Taha is a PhD student under the supervision of Dr. Duncan Stewart at the University of Ottawa. Mohamad contributes a quarterly Research Corner to our Pulse e-newsletter.
- an Action Plan for parents
- a PHA Canada school letter template
- a PH information sheet for staff
- Guidelines for children at school
- To-do Checklist for Parents
- Drug information sheets
In this issue, we will address some questions regarding PH therapy, more specifically, the role of endothelin-1 in PAH and drugs used for blocking its function.
What is endothelin-1?
Endothelin-1 (ET-1) is a small secreted protein, which can bind the surface of the blood vessel cells. There are two main
“receptors” on the cell surface that ET-1 can bind, ET-A, which is abundant on smooth muscle cells, and ET-B, which is present on both endothelial cells—the cells lining the inside of blood vessels—and smooth muscle cells, the cells making the second layer within a blood vessel.
Why is ET-1 important in PAH?
In PAH, there are dramatic increases in ET-1 production and secretion, as well as increased levels of receptors on the surface of smooth muscle cells. This results in constant vasoconstriction and in the division of smooth muscle cells leading to the narrowing and blockage of the blood vessels in the lungs. The effect of this is increased pressure in the lungs and pulmonary arteries.
What treatments target ET-1 in PAH and how do they work?
Starting in the 1990s, several studies implicated ET-1 in PAH and suggested that blocking its function might be therapeutic. Drugs that inhibit ET-1 binding to its receptors—Endothelin Receptor Antagonists (ERAs)—were developed, and the first dual receptor blocker, Bosentan, was approved in PAH in North America in 2001.
Currently, which Endothelin Receptor Antagonists (ERAs) are approved for PAH?
ERAs approved for PAH patients are Bosentan, Macitentan/Opsumit and Ambrisentan. Bosentan and Macitentan bind and inhibit both ET-1 receptors (ET-A and ET-B) while Ambrisentan inhibits mostly the ET-A receptor. Bosentan is the oldest ERA approved in PAH, after which came Ambrisentan and in 2013, Macitentan was approved.
What does research show about ERAs? Which one is better?
Each of these therapies has some side effects, which could include mild liver toxicity, mild edema (fluid in the extremities) or mild anemia. One cannot say that one drug is definitely better than another since each patient is affected differently by the disease and will have a different response to drugs. Thus, it is very important to discuss with a PH specialist which drug is most appropriate for each patient.
Reference: Aversa M, Porter S, Granton J. (2015). Comparative Safety and Tolerability of Endothelin Receptor Antagonists in Pulmonary Arterial Hypertension. Drug Saf. 38: p.419.
Please always keep in mind that while I can provide you with a small insight into PH research, you should always be able to get answers from your Pulmonary Hypertension Specialist, who is more familiar with your specific case and your treatment history.
New Staff Members
We are pleased to welcome Mariane Bourcheix-Laporte on the PHA Canada team as the new Communications Associate. She is replacing Bronwyn McBride, who has taken a leave of absence.