PHighter Friday: Meredith

In February 2015, I received a diagnosis that I long feared.   I was diagnosed with Pulmonary Arterial Hypertension.

At a Blue Lips Foundation's kickoff event

I was 12 years old when I lost my Mother, Bonita, to the same disease.  In 1992, little was known about PH.  The first FDA approved therapy was more than 2 years away.  It was just as common to be diagnosed with the disease upon autopsy as alive.  My Mom was admitted to the hospital on a Wednesday after fainting at a grocery store.  She died the following Monday.  An autopsy revealed she died from right heart failure associated with primary PH.  I was devastated. I no longer had my mother’s guiding hand and heart to lead me through my life’s journey. My mom was 31 years old when she passed away – far too young – to lose her.  To lose her to a disease I had never heard of, and didn’t understand, was unfair and cruel to a young girl.

As I grew older, I dug into PH.  I needed to know how someone so young and vibrant like my Mom could unexpectedly succumb to a disease so unknown and misunderstood.  I researched the disease religiously throughout my high school and college years.  I often wrote research papers on PH.  I allowed myself to wander whether one day I too would be struck by PH.  And then it happened.

In February 2015 I was 8 months post-delivery of my second child.  Following his birth, I was eager to return to the gym, my normal workout and diet routine, and get back to “me.”  The moment I was released by my doctors, I re-started my high intensity workout routine.  I suffered mightily during the cardio segments of my workouts. I experienced extreme fatigue, shortness of breath, dizziness, and nausea – all classic signs of PH.  For months I chalked it up to the “baby.”  By February 2015, however, I could no longer ignore what my body was telling me.  I knew from the years I spent researching PH that the disease may have a heredity component.  I also knew the symptoms I was experiencing were signs of PH progressing throughout my body. 

I will never forget the moment I could no longer ignore my reality.  I awoke in the middle of night to cries from our young son.  I went to his room to rock him.  When I returned to bed, my heart was pounding out of my chest.  I could feel the beats travel up through my neck and down my arm.  My chest was beating so hard and so fast that I felt as though my heart was going to explode out of my chest.  I knew I needed to see a doctor immediately – and I was terrified of what I would hear.
I was placed under a battery of tests to rule out a heart attack and heart disease.  The doctors were pleased when those tests returned negative.  Instead of joy, I was attempting to prepare to hear news I had long feared.

I told the physicians about my family history. Within three days of my initial ER visit, I underwent an echocardiogram.  The cardiologist called my husband and advised him of the results – confirming to him that my heart was grossly enlarged and that a consultation with a pulmonologist was needed immediately.  We were then told the news – “you suffer from pulmonary arterial hypertension.”
My world stopped right then and there.  23 years of fear, agony and prayer for a better result all crashed together from those six words.  I was devastated. I instantly thought of my Mom, my two young boys, my husband and all those people I care desperately about.  I remembered losing my Mom at such a young age, and I didn’t know how to cope with the prospects my boys not having me.  I was inconsolable. 

While my universe was spinning, my husband and doctor immediately began to discuss treatment options.  What I didn’t realize at the time was that I was diagnosed as a Class II patient. Although I was feeling the effects of the disease, I was diagnosed early enough that options were available to me that weren’t so invasive and life-altering.  My local doctor started me on a regimen of oral medications.  I received a second opinion from the Mayo Clinic and a third from Harbor-UCLA.  Fortunately, I have responded well (to present) to the oral regimen, and I remain on those therapies today. 

As time has moved forward, I began to realize that I can manage PH.  Yes, there are days when PH wears me down and beats me up.  I feel it everywhere in my body – aches, pains, chest pressure and fatigue.  I no longer dwell on those days.  I have all the motivation in the world to beat this disease when I look at my family.  They support me.  They love me.  They give me space when I need a break.  Most of all, they tell me every day I am beating PH.  I believe them.  My tests results are steady.  My markers tell my doctors I haven’t regressed.  I beat PH by believing I can, and knowing that the science to treat and manage the disease has advanced beyond measure since 1992. Progress is being made every day.  I believe the scientists who tell us they are finding new and improved ways to treat PH.  The proof lies in the FDA approval of 14 therapies since 1995.  There were zero before that time.

The Blue Lips Foundation was born out of this urgency to provide hope.  I first had to find my own.  I did that.  I then turned my attention back to my time as a child.  I lost my Mom because the disease was not diagnosed in time to allow her to receive a heart and lung transplant.  The doctors didn’t know what they were looking for.  Times have changed.  The medical community now has an understanding (albeit not widespread enough) of how devastating PH is.  Unfortunately, early diagnosis rates remain poor.  It simply infuriates me that with the all the scientific advancements of PH that early diagnosis rates have not improved in 20 years.  That is unacceptable.

PH had bit my family twice in my 34 years of life.  I knew I needed to do something to change the course of PH for other families.  Blue Lips was founded by my husband and me to do just that.  74% of PH patients are diagnosed in advanced stages (Class III, IV) of the disease.  Generally, PH patients will see 3 or more doctors before an accurate diagnosis is made.  The time span between symptom onset and diagnosis is typically 2.8 years.  These statistics are harrowing.  Along with our team, we work every day to change them.

Blue Lips is the only organization in the world dedicated solely to advancing PH early detection and diagnosis rates. We work tirelessly to educate the public and the “gatekeepers of medicine” as to the signs and symptoms of PH, to raise their awareness and understanding that otherwise common symptoms may just be the signs of this medical “silent killer.” Additionally, we fund researchers committed to developing novel diagnostic and screening tools that can be used by the everyday practitioner to “rule-in” or “rule-out” PH during routine medical examinations.  We believe arming family practice physicians, pediatricians and geriatric specialists with easy to use tools will forever alter the course of PH by allowing those who harbor this disease an “earlier” diagnosis. 

It is well understood that those who are diagnosed early in the process, and begin a monitored treatment regimen, stand a far greater chance to be a long term survivor of PH. Until a cure is found, PH patients require as early a diagnosis as possible to continue to live full and productive lives.  My dream is that we, through our work at Blue Lips, are able to give others the same hope I have developed – that even with PH, you can continue to live your life.  Early diagnosis is essential to give others this belief. 

Today, I play with my boys.  I respect and appreciate the precious time I have with them.  Although modified, I work out at the gym and with my trainer.  I travel with my husband. I enjoy “girls’ nights.” I manage our house and home.  I no longer fear that my fate is the same as my mom’s, or that my boys’ fate is the same as mine as a 12 year old girl.  Yes, I have PH, but I am me.  I intend to keep it that way.

You can learn more about the Blue Lips Foundation at www.bluelipsfoundatio.org. Follow us on facebook, twitter (@BlueLipsORG) and Instagram (Blue_Lips_Foundation).

Social Media Detox

Looking forward to finding more
zen time with Sammy.

When I was diagnosed with Pulmonary Hypertension I began a journey of wellness. I sought the help of nearly every alternative therapy available to me. Some of the alternative therapies I have tried have been pretty wacky. Others have felt really rewarding because the practitioners helping me supported me so deeply.

In continuation of trying alternative ideas to improve my health, I will be making the transition to  going vegan starting in March. I hope to try it out for a month and see if I notice any changes. I was vegetarian with a limited dairy intake before I was diagnosed, so I am not sure what to expect. I am also going to get a juicer, which I feel pretty excited about! I asked my friends on Facebook to share any tasty vegan recipes they might have with me. This solidified the bigger change that I wanted to make; a social media detox!

Why I Need a Social Media Detox

I started this blog a few months after I was diagnosed. A lot of people with PH reached out to me when I first started the blog and told me that it took them years to get involved in the PH community. I don't think I jumped into creating the blog too soon, but I wasn't prepared for all of what would come with it.

By nature, I am ambivert. I enjoy being social, but on a small-scale with my fellow quiet friends. I also enjoy my alone time and my privacy. I have made some really great friends through social media who I cherish very much. However, it can be a little overwhelming for me to deal with the attention the blog has brought to my personal life. I get daily Facebook adds (often from people that I do not know, and have never interacted with.) I get messages from people that I do not know. "Weird" guys who have some kind of fetish for disabled ladies have also tried to add me to contact me. Cringe. Barf. No thanks. Delete. BLOCK.

My goal of with this blog was to create a sort of "safe space" for people with PH. I realized that it was so sorely needed. I am so grateful for all of the amazing people that I have connected with through this endeavor. I could not have created this without the help of other people, and their willingness to share and discuss aspects of their life. I have also written personal posts and have publicly shared very difficult topics. I eventually rewrote and shared some of these articles on other websites, and was confused by some of the comments I received from strangers. Which leads me to the issues that I have now, and why I so sorely need a social media detox (among with other things!)

I wouldn't say that I am in the public eye, by any means. However, I think there is the assumption that people have the right to be unfiltered with me because I have put myself out there. Perhaps this is a side effect of social media, and it is unavoidable on some levels. I think people forget that I too, am fragile and need support. I am human. I am going through something difficult and challenging on so many levels.

When I first started this project nearly two years ago I was contacted by several PH organization. They did not offer me support even though I was newly diagnosed. Instead, they asked me for help on projects. This blog has given me a lot of great opportunities, but I also think it gives people the perception that I must have everything all figured out. People assume that I am immune to all the emotional and physical complications of PH, when that is simply not the case.

I received a lot of unwarranted advice from both strangers and friends on social media. The “advice” I have received has ranged from birth control (which is sadly, what most took away from xojane article) and my diet to oxygen and my spiritually/religion (or lack there of.) Other people (who do not suffer from a life-threatening illness) have tried to tell me what will “fix me.” I find a lot of this “advice” to be very privileged.

For example, a lot of people not in my situation have told me that they would go on "eat, pray, love" mission if they had PH. Having PH can make it difficult to travel for a variety of reasons that differs from patient to patient. Another issue is that I can't have salt because it can cause heart failure. That is two out of the three components of their asinine idea. Others have made comments on me becoming a parent/adopting saying whether I am too sick to do it, or that I should just do it anyways! I realize that I did write about this topic openly, but I didn't expect people to feel comfortable enough to say whether I was healthy enough or too sick to do something. Some of the comments were very cruel, but it is fine. I knew that this was a high possibility when I began writing on my blog and freelance writing else where.  The issue is that these comments and “advice” have slowly made its way over to my personal social media accounts, the place where I wanted to maintain a sense of normalcy. I have slowly lost my “safe place.”

It is fine if people want to comment on my posts here or my freelance work. I always appreciate the fact that someone took the time to write a comment, regardless of what it is. I want to emphasize that I do want people who read my blog to feel as if they have a friend in me, and that they have my support. I always feel very honored when people take the time to send me an e-mail about an article or blog entry that I wrote. My business e-mail address is always there for anyone who wants to reach out to me about anything, whether it pertains to a freelance writing, or someone has just been diagnosed and is looking for support. I truly, truly, truly want to offer my support to anyone who reaches out to me. I know how lonely and isolating this disease can be, and I have made some of the best friendships with people that I have not met through this blog.

My birthday just passed and I was surprised by how many people mentioned the fact that I was sick in the same breath as wishing me a Happy Birthday. I am sure these people meant well. I do appreciate the birthday wishes that I received, but I think sometimes people need to take a moment before they hit “send.” Did I want to think about having PH on my birthday? No- but sadly there hasn’t been a day yet where it hasn’t consumed me. Do I want people to only view me as someone with PH? No. I am so much more beyond all of this, and have a life and interests beyond it. I happen to have PH, but it isn’t a word that describes me.

So, this brings me to my much-needed social media detox! In March I will try juicing and making the process of going vegan for a month. I also plan to deactivate Facebook for the month and limit my computer and cell phone use each day. I will still be on Instagram (because there are pictures of cute dogs there...) My hope is that I will have more time to meditate (and to become better at it) each day. I hope having more spare time that is free of distraction will also encourage me to read, write and draw more.

I really just want to live a better life. I feel so disconnected from people sometimes because of technology. How many times do we check our phones during dinner or a conversation? I know I have been guilty of doing this. While social media can be really create for friendships, I think it can also give a false sense of what is important in life. I just need to balance having a life beyond the blog, and hope that my desire to have a personal/private life in addition to the blog can be appreciated.

xo Serena

*Note: The PHight or Flight Project will continue during the month of March. Please be sure to follow me on Twitter for the latest updates, or subscribe to my posts by entering your e-mail in the tool located in the menu to the right. You can also view updates directly on your phone by downloading the phaware app. Click "more" and scroll down to find my blog.

PHighter Friday Follow Up: Haley

In almost just a year and a half since I last wrote for Miss Serena (Haley's previous post can be found here,) Pulmonary Hypertension is quickly becoming a closeted thing in my life. Like a favorite sweater or coat you buy, wear the hell out of it, and after so many years it spends more time in a closet because you're somewhat discovering other fun coats? That's currently my situation.

I don't know how I've had such a great recovery, but I really do believe that recovery dwells within the mind, and spirit; The body will eventually follow. I've gone to therapy, explored medications (whoops - not such a great idea), explored natural healing for my mind, and I really do believe that because I was so focused on how I felt mentally and spiritually that my lungs are now keeping up quite well. While Pulmonary Hypertension will always be an unfortunate huge puzzle piece to my life it doesn't necessarily take the spotlight anymore, and for me that is quite healthy.

Knowing this information, and craving change, I moved myself up into the mountains and into high altitude as well. I came armed with my medication, oxygen, and it's been lovely. While some might think it's negative to have to slow yourself down because of your disease, I think it's wonderful to force myself to slow down, and catch my breath. Life up here is breathtaking in so many ways - not just because of the low oxygen, but because of the beautiful scenery, and all of my lovely animal friends (deer, and more!) I've pushed myself into jobs that are extremely physical, forcing myself to really break out of boxes that my disease put me in. While I know my limits, I'm discovering silly ones that my mind set for itself; something I believe we all unintentionally do. I love visiting my doctors, and watching the surprised look unfold on their face when they see that my breathing is almost normal, and I'm "looking" better.

As a patient of a rare disease, it's hard to force yourself into change. You get used to your doctors who know you inside, and out (quite literally), supportive friends, stores that you can shop in, pharmacies that you may really enjoy the customer service there...it's just hard to pick up and move. Not only do you have to move your furniture, but you have to scope out a whole new medical area, and if they will even be prepared to deliver what you need. I moved to a state that is so behind on two major things; education and medicine. Being an educator and seeing some of the schools in my surrounding area is just cringe worthy. Education is not pushed because of poverty, and how families function daily. Also, the nearest hospital is a little over an hour away from me, and even then it's a tiny hospital that barely has the means to go. It's been quite challenging in both these areas of my life to adjust to these lower standards. However, I love a challenge.

I think my overall theme for these last months of my life has been "challenge". We box ourselves in without even knowing we are doing it, and create kind of false realities of what we can and cannot do because of our disease. Don't be that person. Instead, be a safe person who knows limits, and is careful. A person who also wants to try new things, and do exactly what people think you cannot do. They said I wouldn't live, and I did. They said I wouldn't be able to work, and I have so ridiculously much. They said I wouldn't be able to dance, and I danced my ass off in ceremonies. They said I could never live in altitude, and I'm living quite healthy, and happy. Who knows what is next...maybe I'll soon be on the mountain snowboarding!

We think we know our bodies, and even doctors think they do, but for some reason I'm still the "mystery" patient, as my doctor calls me. Try things, grow, be safe, and actually live. Surviving is no longer an option.

-haley.
phenomenalhaley.wordpress.com

PHighter Friday Follow-Up: Colleen

On January 2, 2016 I hit my eight-year anniversary since diagnosis. This makes me a long-term survivor, I have officially defied the odds.

It’s a curious milestone to celebrate. Of course, I am deeply grateful to be here. There is so much living left to do. The flip side of the statistic, however, is that half the people diagnosed the same time I was are no longer here. It’s a fact I am acutely aware of far too often… watching others with this disease suffer and pass on is  something that has never ceased to cut through me.

Although incredibly hard some days, I can’t stay in that dark space – I think that knowledge is perhaps the most valuable lesson (and skill) I have learned these last eight years. It’s a balancing act between weathering the really hard parts and then getting back up and focusing on the good. In fact, this lesson has been so deeply ingrained in me, I wrote a book on it.

“Defining the New Normal: A Guide to Becoming More Than Your Diagnosis” is the book I published in November 2014. By 2015 it was on the Amazon bestseller list, a feat that not only shocked (okay, and thrilled) me, but one which left me really humbled. I knew when I set out to write this book, that everyone knew somebody who would need it (it’s for all people rocked by a major diagnosis, not just PH). I just didn’t know how many people would resonate the way they have. It’s just… well, it’s just the best thing knowing that this really crappy PH thing can be turned around for good. I am nothing but grateful for how it has been received, and grateful to the Caring Voice Coalition and PHA for supporting my efforts.  And I absolutely love traveling to speak to other patients about how they too can define a life they love, no matter the diagnosis.

In addition, I’ve been given the opportunity to speak to the FDA, Women in Government and PH related industry several times. I really believe it is the patients who have to take our stories right to the change makers, and the policy makers. They need to hear how their every decision impacts our wellbeing and survival.

A book is fun, but the most significant thing in my world as of late has been the adoption of our daughter this past July. We started with fostering the tiniest sweetest little four-pound bundle in November of 2014 (oh yes, she was placed with us the same week my book launched – no pressure there!). Eight months later, in a little courtroom, a judge ruled her ours. Forever.

That moment was literally a dream come true. I had wanted to adopt ever since I was in college. For a long time it looked like PH was going to derail that dream. Questions about how sick I might get, and how bad the outcome might be, kept my husband and I from moving forward.

Finally though, it seemed I was going to be pretty stable for a good long time. I have a kick-ass team of doctors and each one of them was 100% behind the idea of adoption – which was incredibly reassuring.

She and my nine-year-old son keep me very busy. They get most of my energy these days. Adopting was the best decision, but caring for an active toddler and a boy on the move is no joke! I’m writing a lot, and coaching clients all over the country to better health on their terms. I don’t think this journey is ever going to be an easy one. But in choosing whether I PHight of take Flight… I choose to stand and PHight… or sit down on the couch and PHight from there if I have to – because let’s be honest, PH fatigue is no joke either. Either way, I’m defining this life on my terms and it’s a life I love.

*This entry is a follow up to Colleen's original #PHighterFriday, which can be viewed here.

PHighter Friday: Kris Ross

The road to diagnosis was incredibly long and frustrating. I was mostly symptom-free as a child. I would get blue lips when I was cold which was attributed to being so skinny. In my later teenage years, I started having breathing difficulties when I would do physical activity and when it was cold out. I was told I had a reactive airway and an intolerance to cold so I should use an inhaler before going to the gym and before going out in colder weather. My blood work indicated high hemoglobin so I was sent to a specialist who did some nuclear medicine tests. He diagnosed me with “couch potato polycythemia” --- basically, my hemoglobin was high because I was lazy and didn’t do very much physical activity.

Over the next decade, I signed up for gym memberships I couldn’t use, despite my best efforts, and tried different inhalers. I had high hemoglobin results on my blood work during this time period but I was assured I was fine and my doctor told me he wished all of his patients could be so healthy. Finally, after a couple of my colleagues told me I didn’t sound like I had asthma or a reactive airway during a basketball game, I started questioning things and decided to go to a walk-in clinic to explore things further.

In July 2002, I went to see a doctor at the clinic. We had never met before but he listened to my concerns and sent me for a chest x-ray and scheduled some cardio-pulmonary tests and an arterial blood gas. The x-ray was deemed normal but my lips and fingers turned a bluish-purple shade during the cardio-pulmonary tests and I had pain between my shoulder blades. My arterial blood gas was 44. I was sent back to the clinic immediately and, once I convinced him I had never been a smoker, the new doctor told me the only things that would give me these types of results were a major heart or a lung problem. I was scheduled to see a respirologist the next week. He determined my x-ray was not normal because my pulmonary artery was enlarged. He felt the most likely reason was an undiagnosed hole in my heart.

Within two weeks, I met with a cardiologist and had my first echocardiogram. It revealed a large ventricular septal defect (hole in the wall between the two pumping chambers of my heart). Over the following three weeks, I had a number of tests, culminating with a right-heart catheterization. It was determined I had pulmonary hypertension caused by the hole in my heart. It was left open for so long that my lungs were irreparably damaged. To say this was devastating isn’t enough to really capture how I felt. The future I had imagined for myself was gone and, in its place, I was left with fear, anger and uncertainty. The road to a diagnosis took a decade to travel and there was some thought that had I been diagnosed when I first began exhibiting symptoms the hole could have been repaired and the lung damage minimal. My body was my enemy.

There were very few specialists at the time so I was sent to Toronto for assessment. I was told, untreated or unresponsive to treatment, I might have a couple of years before I would need a heart-lung transplant, if donor organs were available. Thankfully, the medication worked well, stabilizing things, and I was able to return, mostly, to the life I’d known before. There were things I would never be able to do and there were things I had always wanted for myself that would never happen. I mourned them and had to move on, living life as fully as possible. My family and friends were great supporting me, adapting things so I could be included in their activities and events. Eventually, they opened a Pulmonary Hypertension Clinic in Ottawa at the Heart Institute, closer to my home, and I starting being treated by the fabulous team there.

I was stable and leading a relatively normal life for years until many bumpy months last year after a nearly fatal case of pneumonia. I had excellent care at the Heart Institute and from family and friends. With some medication changes, months off of work and a staggered return, I was able to get back to my pre-pneumonia state --- even better in some ways.

It’s 2016 and I continue to be relatively healthy with the support of family, friends and the team in Ottawa.

Accessibility of PAH Therapies in Canada: Part II

Part II: Treatments for PAH That Are Not Available In Canada

As someone with Pulmonary Hypertension, I was very disappointed to learn that a couple of potentially life altering medications that have been available for many years in the United States of America are not available here in Canada. I had assumed that because Canada offers free “universal” health care, that it also had the best interest of its patients in mind. Needless to say, I was completely shocked to learn that some prostanoid-family medications like Tyvaso (inhaled treprostinil) and Ventavis (inhaled iloprost) and Orenitram (oral tablet form of treprostinil) are not available here in Canada. These medications are a less invasive form of intravenous prostanoids such as epoprostenol (Flolan, Caripul) and subcutaneous treprostinil (Remodulin). I asked PH specialist Dr. Sanjay Mehta to answer some questions I have regarding the accessibility of of PH therapies in Canada.

Serena: Could you explain the benefits of inhaled and oral versions of treprostinil over the intravenous and subcutaneous prostanoid treatments? Do you believe that medications like Tyvaso and Orenitram should be available to Canadians with PH? Could you briefly explain why these medications are not currently available here in Canada?  Is there anything that we can do as a community to have these medications available here?



Dr. Mehta:  Treprostinil is a very effective treatment for PH. It is currently approved and available in Canada both for intravenous and subcutaneous administration as Remodulin.  Both of these approaches require an external pump and a cassette or syringe to be filled regularly, usually every 1 or 2 days. 

Treprostinil has been modified for patients to be able to take it by either breathing it in (inhaled Tyvaso in the US) or by taking a pill (oral Orenitram in the US). Both of these approaches have been shown to be effective in treating PH. Moreover, the inhaled and oral routes of drug administration clearly simplify the treatment compared with much more complex intravenous/subcutaneous treprostinil administration.  However, inhaled and/or oral treprostinil are not the best treatments for all PH patients. Inhaled treprostinil may not be as effective as intravenous/subcutaneous treprostinil, and is typically only used as an addition to other oral PH therapies in the US. Oral treprostinil is effective, but side-effects of nausea, abdominal pain, and diarrhea can be difficult for some patients. 

Ultimately, both inhaled and oral treprostinil are not available to Canadian PH patients because the pharmaceutical manufacturer never submitted an application for Health Canada approval. This is largely because of business reasons, as the PH market in Canada is much smaller than in the US, and it is expensive to launch a new medication in Canada.  Moreover, many PH medications cost less in Canada than in the US, because of government regulations. As such, the cheaper price for a new PH medication in Canada can lead to pressure on a company to reduce the price in the US, which would mean they make less money in the much bigger American market. 


Serena:  Are you able to speak to a trial for and the benefits of having Remodulin administered through an implantable pump as opposed to subcutaneous or intravenous methods? Do you foresee this implatable pump being available in Canada?

Dr. Mehta:  There was an exciting trial in the US that studied whether Remodulin could be administrated via an implantable pump, rather than an external pump.  Such an implanted pump has a reservoir of medication that could last for a prolonged period, such as a month. This reservoir could be refilled regularly, such as every month, in the PH clinic.  As a result, patients would not have to look after preparing medication every day or every second day at home, or changing tubing / cassettes / needles, which would clearly improve their quality of life.  This implantable pump for Remodulin may become available in the US in 2016, and then hopefully one day in Canada.

Serena: This concludes our two-part interview. Are you able to share any insight on any exciting and promising treatments on the horizon in Canada?

Dr. Mehta: Despite all the advances we have seen in the last years in regard to PH therapies, PAH is still a progressive, often fatal illness for which we have no cure.  Tremendous research both in Canada and around the world continues to better understand the disease and what exactly is happening to the blood vessels of the lungs to cause PAH.  There are many ideas for new treatment approaches, including both pharmaceuticals and potentially gene-therapies.  This is a very hopeful time that PAH patients will continue to benefit from this research and new therapies, until such a time when we can say to a patient, “You have PAH, but we can treat you so that it will not affect you in everyday life and will not shorten your life.”

The PHight or Flight Project would like to thank Dr. Sanjay Mehta and The Pulmonary Hypertension Association of Canada for their assistance on the Accessibility of PAH Therapies in Canada Series.

*This is the second part of a three part series. The first part of the series, Access to Opsumit in Canada can be found here. The third and final part of the series will share testimonials from PH patients in the US who are on the various medications for PH that are not available in Canada.