PHighter Friday: Steve Van Wormer on becoming #phaware

My son Lucas was born a happy and healthy child but life as we knew it changed when he turned four years old. Lucas was misdiagnosed with asthma, so for months doctors put him on nebulizers, but nothing seemed to help and he was on a downward spiral. Nine long months later, after an unrelated routine chest X-ray, his pediatrician recognized that he had a slightly enlarged heart. We were referred to a pediatric cardiologist, who gave my wife, Marina, and I the devastating news – our only child was diagnosed with ideopathic pulmonary hypertension.

About a week later, as we waited to meet with a PH specialist at UCLA, around 4am, Lucas stumbled into our bedroom, struggling and moaning. He had chest pain, he couldn’t breathe and his heart was pounding. He was rushed to UCLA in an ambulance to be stabilized.

Thinking back to that time (nearly ten years ago), I realize how lucky Lucas was. Only weeks earlier, combination therapy had come into play to treat pulmonary hypertension. That coupled with his relatively speaking early diagnosis, has contributed to Lucas being a robust responder to low dose oral therapy. Tragically, not every PH patient responds to treatment as successfully as he did (and continues to do).

As I write this blog entry, I’m thrilled to report his 14th birthday was yesterday and he’s never felt better with regards to his PH!

Lucas’ greatest wish is to find a cure for all the types of PH so no one young or old will have to go through life breathless. This is the reason why our family has worked so hard to raise global PH awareness - in honor of patients like Lucas and in memory of all the patients who have lost their battle with this deadly disease.

GIVING A VOICE TO PH

From day one of Lucas’ diagnosis we knew that if there is to be cure for this rare, life- threatening lung disease it needed to start with awareness.  Awareness for what PH is… Who it impacts… Where people can find information… and most importantly, how can people help? That process started with collaborating with talented graphic artists, editors, music designers at FOX to craft a series of awareness videos and PSAs that, over the past few years, have aired across multiple national networks, played literally a million times in taxi cab TVs, have been translated to over 50 languages and distributed to every global PH organization on the planet. The Lucas voiced pediatric PH research PSA even went from “rare to Time Square” when it graced the NASDAQ tower (the largest free standing screen in the world)!

FROM AN APP TO AN .ORG

With each of these awareness successes, with every email received from countries all around the world from patients responding to a #phaware tweet or reaching out because they saw an ad on TV, we realized the community was looking for new ways to connect.

And so phaware global association was born out of a simple hypothesis: “If we capture a single patient… if we save one life… what will it be worth?” That hypothesis has been proven repeatedly as phaware™ continues to discover diagnosed and undiagnosed patients, connects medical professionals, educates the general population, enlightens new investors and supporters, informs congressional leaders, and activates government and industry decision makers.

One of the prime objectives of our new 501(c)3 organization, which was founded by a group of pulmonary hypertension awareness activists, is to leverage innovative technology and use it to capture, engage and enable diagnosed and undiagnosed PH patients, caregivers, and medical professionals by providing them with state of the art tools and creative content focused on education, resources and knowledge.

GLOBAL DISEASE. GLOBAL AWARENESS.

From Burbank to Buenos Aires... Barcelona to Beijing. At phaware our focus is to inform and educate the masses, using impactful and innovative methods. phaware aims to make the reality, a day when no PH patient goes undiagnosed or misdiagnosed. Our goal is to reach and discover patients and supporters across multiple touchpoints. Our initiatives and awareness activations engage audiences through a variety of screens, mediums and platforms.

This exposure allows us to tell patient stories in ways never before imagined. This opportunity will be the catalyst to create greater impact on the discourse of healthcare and disease prevention not only in this country, but across the globe and bring activated individuals into the fold to help forge a new course to a cure.

BECOME #phaware365: A DAILY DOSE OF PH AWARENESS

Of course, we celebrate and honor the global PH community on Rare Disease Day (February 29th), World PH Day (May 5th), and during PH Awareness Month (November), but at phaware, we’ve created a new way for you to raise global PH awareness 365 days a year…It’s a snap with our phaware365™ app.

Show the world you are #phaware of this rare, life-threatening lung disease.

●      Snap a selfie.

●      Decorate your photos.

●      Customize them with PH facts.

●      Add awareness stickers.

●      Participate in global phaware activations.

●      Share across social media and on our phaware365 Global Selfie Stream.

●      Available for FREE on the App Store or on Google Play or at www.phaware.global/apps

Each time you share your phaware365 selfie on your social media, you raise awareness to your inner circle. Each time you share it to the phaware365 app, you raise awareness globally. With each post, you continue to bring PH from rare to everywhere!

THE BIG GAME. BIG 50 CONTEST

For Rare Disease Day 2016, we are kicking off our 2016 global awareness efforts during the biggest event on the planet - Super Bowl 50!

Launching January 25th through February 29th, (Rare Disease Day), phaware will be featured in a full-page ad in the Official Super Bowl 50th Anniversary Program. phaware will also be featured in the 2016 NFL Pro Bowl & NHL All Star Game programs, as well.

Go to www.phaware365.org to learn how you can help make PH history.

The Goal:

50K FANS  - Follow @phaware on twitter, facebook & instagram

50K SHARES - Visit www.phaware365.org to share their new digital awareness video

50K DOWNLOADS - Get the phaware365™ & phaware™ mobile apps

50K DONATIONS - Donate $25 to receive your FREE "I'm aware that I'm RARE" T-shirt for

Rare Disease Day 2016

These limited edition "I'm aware that I'm RARE" T-shirts are also available at www.represent.com/phaware

Accessibility of PAH Therapies in Canada: Part I - Access to Opsumit

Part I: Access to Opsumit in Canada

In March 2015, the Canadian Agency for Drugs and Technologies in Health (CADTH) suggested that all newly diagnosed people with Pulmonary Arterial Hypertension (PAH) in functional class II or III receive the same mono-therapy approach for their treatment. This approach would take the decisions for treatment out of the hands of PH experts and place it in the hands of government bureaucrats. I asked PH specialist Dr. Sanjay Mehta* to answer some questions I have regarding the accessibility of PH therapies in Canada.



Serena: I know that you are very involved and passionate about the PH community, and that you have been working very hard to ensure that all newly diagnosed people have access to the therapy that is best suited to their needs.Could you briefly explain why it is so important that PH specialists, like yourself, are able to treat PH patients on an individual basis and recommend a therapy suited to their needs instead of the stepwise, rigidtherapy approach suggested by the CADTH? 



Dr. Mehta:  It is one of the most basic principles of medicine: that each patient is an individual, and needs to be understood, respected, and treated as an individual. This includes doctors making decisions about the best medical treatment for a patient’s illness.  Although many patients may have the same illness, as in the case of PH, they are all still unique individuals. As such, there is no reason to expect that they respond similarly to PH medications, or that the same medication is the best one for each patient. Expert PH physicians need to consider many factors in deciding on the best initial and subsequent medical treatment for each PH patient. The decision is based on having expertise and experience in PH, understanding the patient and their other conditions, and the specific risks of each treatment.  Even in clinical studies of PH medications, all patient participants don’t respond similarly and, while some may respond well, others may not.  Importantly, in dealing with a serious, progressive illness like PH, a patient can’t afford to waste precious time “trying” anything other than the most effective, best treatment for them.

Serena:  The newest Health Canada-approved oral medication for PAH, macitentan (Opsumit) is not available to most Canadian PAH patients, as per the recent pan-Canadian Pharmaceutical Alliance (pCPA) decision to close negotiations with the manufacturer, with the impact that this drug is not approved for funding in most of Canada. 

I understand that Opsumit is generally well tolerated, and that it was tested in one of the largest and longest clinical studies of any approved PH treatment. Can you discuss some of the benefits of Opsumit? If the government continues to deny funding for Opsumit, what is the future for this medication in Canada?



Dr. Mehta:  In 2013, the Seraphin study reported on the benefits of macitentan (Opsumit) in what was at the time the largest, longest study ever in PAH patients. It showed that macitentan reduced the morbidity (severity of illness) in PAH patients, specifically reducing the risk of progressive worsening of PAH by 45% and reducing the risk of hospitalization by 50% over 3 years.  Moreover, treatment with macitentan significantly improved symptoms, quality of life, and exercise capacity in PAH patients. This is a unique study that showed for the first time the long-term benefits of treatment with a PAH medication, compared to all other PAH studies which only looked at benefits over 3-6 months. Clearly, PAH patients don’t want to just improve over the short-term, but hopefully remain well for many years!

As a result, treatment with macitentan has been strongly recommended by the most recent 2014 PH Clinical Practice Guidelines jointly published by the European Society of Cardiology and the European Respiratory Society.  Moreover, macitentan has been approved for funding in the US, and in many European countries, and many PAH patients are currently being treated with macitentan.  Notably in Canada, Quebec approved public funding of macitentan in October of 2013, such that Quebec PAH patients have complete access to macitentan as a treatment for their PAH, should their expert PH physician decide it is the best treatment for them.  Similarly, several large private insurance companies across Canada have approved macitentan coverage for their clients.  However, the pCPA recently denied funding for macitentan for the rest of Canadians (PAH patients living in all the other provinces and territories who do not have access to private health care), and indeed, has broken off negotiations with the pharmaceutical manufacturer, Actelion.

This is a very concerning development for Canadian PAH patients, most of whom have been denied access to public funding for treatment with macitentan. Moreover, this establishes a dangerous precedent whereby any and all future new PAH therapies (for example, the new oral **selexipag or Uptravi, which was just approved by the FDA in the US) may similarly not get approval for funding for Canadian PAH patients.  It would appear that Canadian and provincial governments are saying that PAH patients are doing just fine with the therapies they have available today!  Clearly, PAH patients and their physicians know otherwise;  despite treatment with the many PAH medications we have, many PAH patients remain seriously ill, limited in everyday life, and their disease continues to progress until it takes their lives, on average 7-10 years after diagnosis.  Is it reasonable to accept that?  Should we not try to further improve the health and lives of PAH patients?  Most definitely, all Canadian PAH patients and their caregivers would want us to continue to develop, test, approve and make available newer and better PAH treatments. Lack of government understanding of this critical issue is already leading to less than optimal treatment of Canadian PAH patients, as currently demonstrated by lack of access to macitentan, and likely to reoccur with lack of availability of future new PAH treatments. 

Serena:  Thank you for sharing your thoughts on this complex issue. Is there anything that you would like to share going forward?

Dr. Mehta:  All Canadian PAH patients and their caregivers should be heartened by the incredible progress we’ve made in the treatment of PAH, since the 1st medication, intravenous epoprostenol (Flolan) became available in 1997. Currently, 9 different medications are approved and generally available for the treatment of PAH in Canada. As a result, the quality of life and survival of most PAH patients have significantly improved. This is important to keep in mind and we should remain hopeful that, thanks to the joint efforts of dedicated advocates, members of the medical community, and PHA Canada, new treatments such as Opsumit will be made available to all PH patients in Canada. This is what we must continue to strive for.

** selexipag/Uptravi received approval by Health Canada on Tuesday, January 26th, 2016 for the treatment of PH.

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.

My Experience: Depression and Pulmonary Hypertension

Facing a serious and potentially life-threatening disease is no easy feat. When I look at my life, it is now divided into two parts. Before diagnosis and post-diagnosis. I am also divided between who I was before this all happened, and who I am now. It has affected every aspect of my life, such as ending my career, to complicating my relationships with not only my partner but my parents and friends.



I was diagnosed with Pulmonary Hypertension at the age of 25, and it completely unraveled me. I had lead my life thinking that I had my whole life ahead of me, only to find out it may end out much sooner than I would have hoped. I was certainly aware that I wasn’t invincible, but being a young adult gives you a false comfort of time. Throughout my youth I was a bit of a loner, and worked very hard to balance a part-time job while attending university. I was preparing for later, for when I thought life would really get good. I was saving up to move out, started my first real career job, dreaming of getting married and thinking about having kids when I found out that I have PH. I feel like I was treating life as a lay-away plan, hoping to enjoy it once I reaped the fruits of my labour. What I really wanted was a simple life, and that is what I was waiting to enjoy. It feels like my diagnosis has taken many of those plans away from me. As you can imagine, it became very difficult to find anything to look forward to.



I have struggled with depression my entire life. I remember being as young as four or five years old the the first time I had dark thoughts. I was a very shy child and also suffered from crippling anxiety. I was often ill as a child and had spent some time in and out of the hospital. I learned in school that being sick and hospitalized often as a child can contribute to depression and other mental disorders later on in life. I began to really struggle with depression as a teenager. I was eventually offered medication from a family doctor. I politely declined, feeling it wasn’t the right choice for me. 



I was very hard on my self, and I never felt I was good enough. I am not sure where this feeling came from because my parents have always been incredibly supportive. I put an unreasonable amount of pressure on myself. Depression affects everyone differently. I had a hard time finding value in myself, or value in the life I was trying to make for myself. I felt like such a loser no matter how hard I tried at things. I felt unlovable and unworthy (even though I had incredible love from the people in my life.)

Unfortunately, I couldn’t see how good my life was for a long time. 

It took the brink of death to help me really appreciate life. This isn’t to say that life after diagnosis is a blessing, or that I no longer struggle with depression. After diagnosis I was recommended medication to treat my depression. I explained to my doctor’s that of course I was depressed, look at the news I had just received. I politely declined medication for treatment for depression once again.



The depression I now faced was paralyzing. I cried before bed and when I woke up, and sometimes I cried throughout the day. I barely spoke or ate. I became withdrawn. It was hard to find a reason to get up most days. That is the weird thing about living with Pulmonary Hypertension. I want to live so badly, but not under the conditions the disease offers to my life.

 I decided that I was tired of wasting my valuable time feeling so terrible- so I started seeing a life coach, and spoke to my alternative health practitioners about my depression.

Living with a life-threatening or chronic condition can understandably place a damper on one’s life. Suddenly it feels like we are in a world we no longer belong in because of accessibility issues, or the need for medical equipment. Sometimes it can be hard to enjoy aspects of life because of the all of the side effects of medications, the new equipment we need to stay alive, the pain we feel, the emotional burden we carry, and whatever else comes along with a disease.


I found myself having a hard time knowing who I was anymore. The disease weighed so heavily on my self-esteem. Suddenly, I felt and looked like a different person and it wasn’t a version of myself I liked. I would cry sometimes looking in the mirror, seeing how frail I looked, and having a nasal cannual attached to my face made me self-conscious. Prior to PH I knew who I was, and I had already created and obtained my own identity. After diagnosis I had to figure all of that out again. I am still trying to figure out who I am (and who I can be) now.



Unfortunately, there is still a stigma around mental illness and chronic conditions (like PH.) To the naked eye people with depression, PH, or both, can look ‘fine.’ A lot of symptoms are chalked up to being in our head, or easily fixable (which is often not the case.) What outsiders fail to realize is that we would much prefer not to have these problems. I didn’t decide to have Pulmonary Hypertension, but life had other plans.

 If I had my way, I would have preferred to have a life less complicated by disease.

I hope that this can bring light to the fact that depression, and other mental illnesses like Post Traumatic Stress Disorder (PTSD,) are very common among people within the Pulmonary Hypertension, (and chronic illness) community. We are constantly exposed to triggering environments. Such as the hospital, where routine tests are often performed. This environment, the treatments and the tests are often associated with negative memories and experience. I experience vivid nightmares where I wake up because of the sound of my scream, covered in sweat.  I also have panic attacks when I have to go back to the hospital where I was diagnosed. They left me in a wheel chair facing the wall while I cried.  This hall way is where I used to do a walk test. Needless to say it was hard to get good results when all I was reliving the worst experience of my life. Luckily, these nightmares and attacks rarely happen anymore- but of course I still struggle. It would be very difficult to go through many of the experiences of having a life-threatening illness and come out of unscathed.



If you are struggling, please do not be ashamed to find the right treatment for you. There are lots of ways to seek treatment. Talk to a doctor, family member or friend that you trust. When finding professional treatment, do not settle. Find someone who you have a connection with, someone who you trust and helps you feel a little safer. There are not only doctors you can talk to, but psychologists, social workers, life coaches, alternative health practitioners and so on. These qualified specialists can help discuss various treatment options with you as well, and help you develop coping skills.



Ironically enough, my depression is probably the best it has ever been in my entire life. I have found a regimen for myself that works well for me. I practice different alternative therapies along with meditation and yoga. I know that I am human and still allow myself a few days if needed to be sad. This usually happens after a PH appointment. It is very difficult to get used to, but I try very hard to live in the current moment. Even though things may suck (for lack of a better word,) there are still things around me that I can enjoy. There are also many things that I am grateful for. This experience has really taught me to appreciate things more, including myself. When I was first diagnosed I thought I shouldn't enjoy things until I got "better." Now I have learned to try and enjoy things while I can.

My life isn't perfect, or what I had hoped it would be. However, my quality of life was so bad before that it has helped me appreciate my health now. Sometimes I get caught up thinking about the future, which can be pretty scary. It is human to be scared, but you have to be able to find the right balance. I give myself a few days to deal with the grief, but try to let deal with my emotions and let them go. What I am going through isn’t easy, so I have learned to be more patient and kind with myself. This has also helped me learn how to be more compassionate towards others.  I am no longer hard on myself. I have also learned how to love myself, which I desperately needed to do in order to take care of myself and truly love those around me.



I hope that this can encourage anyone who is struggling to reach out. Please know that you are not alone. Reach out to someone you love and trust, and find the best treatment plan for you. Don't be afraid to speak up about how you are feeling, and be kind enough to yourself to find help.

xo Serena

PHighter Friday: Tegan

First international trip to Japan 2010

My PH story starts when I was six, sometime in 1995. I suddenly started having fainting episodes and was quickly diagnosed with Epilepsy, having uncontrolled Tonic-Clonic seizures. 

Within a year I developed a chest infection/cough, that wasn't going away with antibiotics. The local doctor sent me and mum down the road to have a chest x-ray. I don't remember specifics but I know that it landed me pretty quickly in the Royal Children's Hospital Melbourne on the Cardiac ward. Throwing my family in to chaos with my two younger sister being looked after by my grand-parent, while mum looked after me.

Being diagnosed with "Primary Pulmonary Hypertension" Functional class 4 (now known as Idiopathic Pulmonary Arterial Hypertension) was life changing to say the least. This diagnosis didn't just effect me emotionally and physically, but my entire family, my circle of friends, my school life, after school activities and general living. I was initally started on "Diltizem", and given a 5 year life expectancy. As a 6 year old I was blissfully unaware of this.

I managed to go to school full time, partake in sedate after school activities and play with my 2 sisters. If I forgot to take my medication, or refused (as i did in the beginning), I could feel the difference, and would often have a seizure as a result.

Around the 5 year mark I got really sick. I was unable to start high school, I was in and out of hospital trying to manage the symptoms. The addition of a few medications and eventually oxygen.

Hosptial stay 2001/02, recently started
 Epoprostenol and looking much better already

I tried "Beraprost" for a short time, but this made me feel sick three times a day for about thirty-sixty minutes after each dose. It made me feel isolated from my friends as I had to go to the school sick bay to have it during class, and rest until the side effects wore off. It also didn't make me feel much better. 

This period is very vague to me and everything sort of blends together. I know I had multiple seizures at school, ambulances were called on multiple occasion, late night trips into the city to get admitted quickly and always extra drugs and bloods and tests. Mum and I spent countless hours stuck in traffic on long stretches of road on the way to the hospital, we played many cars games, but often I was vomiting or simply too lethargic to play. 

Around this time I was placed on the transplant list waiting for a heart and lungs. I was too ill to go to school at this time, nothing was working. I made it through the year using a wheelchair, oxygen and many many medications. I was in heart failure and the rest of my organs were soon to follow, fluid retention, vomiting and weight loss among the symptoms. I was palliated, something i wasn't really aware of. New teams of medical professionals were seeing me all the time to help with one symptom or another, there was a constant stream of consults, reviews and medical rounds. I had privileges from hospital, and small windows at home.

Early 2001 I was given the green light to start this magical, mystical drug from the US (Epoprostenol) as well as oral "Bosentan" (plus my cocktail of "normal" meds). Conditions applied, a fair few. Mum had to be trained in making the medications, and had to commit to making it up from powder to liquid every single day. We had to be self caring with a PICC line (a semi-permeant line directly into the heart.) I had to do any test they requested (6 minute walks, ECHO's, ECH/EKG, bloods and Cardiac catheters). I was so unwell I was not yet able to have the permeant line, so I had a temporary one inserted with light sedation. 

Kakadu National Park 2014

The temporary line proved to last quite a while. By the time the 2nd one had packed it in, I was well enough to have a bit more sedation and get my permanent line. This was a pretty good indicator that this drug was working. I made leaps and bounds from here. I came off the transplant list, I was no long made palliative and I slowly started going to school. I had missed a few months of school and had to be slowly reintroduced. Luckily with a supportive school, and some great friends I got back into it.

The combination of drugs has changed a bit over the years,. I'm no longer in any organ failure, and I take a portion of the drugs I was taking. I take 2 medications for PH, Epoprostenol and Sildenafil. However, I do take a few a preventions of to make life a little bit easier with symptoms.

It took me a very long time to come to terms with my illness and accept it as part of my identity, but not something that will define me. I can thank the use of this drug for the life I lead now.

I work full time as a paediatric cancer nurse, I ride my bike 7kms each day to work and I have the travel bug. I have it in a big way. 

I still have to undertake the same tests, just much less frequently. I can't swim in the ocean/pool, I can never be too far from a hospital and I have to carry around a pump everywhere I go 24 hours, 7 days a week. BUT I will always try to do this with a smile on my face and a positive attitude!

I've been to over 20 different countries and can never wait to plan my next one. Of course I have limitations, but I'll take what I can get.

Repackaging 3 months worth of medication
for my upcoming holiday

My next trip is the biggest and the scariest and the absolute most exciting thing that I have been able to achieve since diagnosis. I have spent the last 6 months planning everything I might need for a 3 month holiday in Sweden and Europe. There is about 40 kg of supplies and 4 kgs of clothes.

This is a recent development as I switched to a more stable version of my drug. It means more freedom and less volume of medications (Thanks Veletri!). 

It has been a lot of preparation, research and challenges at every corner. I started to document my issues with travelling with a serious chronic illness and intend to continue while I travel.

I know I've had a hard life but I know its also been full of interesting moments, filled to the brim with love and most of all its been surprising. When you exceed the doctors expectations by 15+ years you learn to live life to the full.

There are so many people who help me with my challenges. I feel this is the key to my success.

Stonehenge England 2013

My blog can be found at https://www.tumblr.com/blog/tegamegadrive and my instagram at https://www.instagram.com/tegamegadrive/

PHighter Friday: Brooke

Ever since I can remember, I loved being busy. So much so, that at 4 years old my parents enrolled me in almost every sport imaginable – dancing, figure skating, gymnastics, swimming, the list goes on and on.

Figure skating seemed to stick and over the next 17 years, the sport would take me on some pretty spectacular adventures. I spent my entire childhood on the ice – 4, 6, 8 hours a day and by the age of 14, I was a national level athlete in both singles and pairs skating.  I represented Canada at several international skating events and spent hours in the gym when I wasn’t on the ice.  My aerobic capacity was second to none.

Which is why I found it alarming, in the spring of 2013 (only a few years after retiring from figure skating) that I was unable to keep pace with my Mother during a charity bike ride.
At the time, I brushed off my inability to breathe as simply being out of shape and found comfort in the idea that more training would help.

Unfortunately, it never did.

Fast forward to February 2014 when my world was forever changed.
It was the morning of the 2014 Olympic Gold Medal Hockey Game, Canada vs the US, and my sister and I were walking downtown Toronto to meet some friends for the early morning game. Out of nowhere, I had to stop. It’s hard to describe what I was feeling in that moment because I can’t really recall. I was fully aware of what was happening around me but I was struggling to even breathe. Needless to say, I visited my local ER shortly after – thinking I would be told I had asthma. Long story short, after almost every test in the book, I was discharged ten days later, with a mystery diagnosis and referrals to both a cardiologist and a respirologist.

It was on March 19th 2014, during my appointment with Dr. Granton at the Pulmonary Hypertension Clinic at Toronto General Hospital that I first heard of Pulmonary Hypertension.

With my PH diagnosis, my world was turned upside down.
Not only was I diagnosed with PH but I also learned that I have two congenital heart defects, both of which made it a miracle that I was able to compete at such a high competitive level when I was younger.

So then came the next dilemma – do ‘we’ operate on the two congenital heart defects (Atrial Septal Defect and Superior Vena Cava Displacement) in the hopes that by correcting these two defects we would stabilize the pulmonary hypertension? Or do we treat the pulmonary hypertension and the congenital heart defects as two separate entities?
Again, after more tests than I’d ever care for, it was decided that there was too much risk involved for the potential reward with regards to correcting my congenital heart defects. They would stay!

To limit the progression of my pulmonary hypertension, I am currently on Adcirca, Tracleer, and Xarelto (even though my PH is not related to blood clots, there are studies that show better prognoses while taking blood thinners). These medications are literally saving my life!
My doctors tried me on Bosentan prior to Tracleer but it was causing some less than ideal side effects.

Growing up, I was an independent, young woman who was comfortable and confidant. I don’t mean that in a cocky, over-pretentious way but in the sense that I knew who I was and was comfortable with who that was. After being diagnosed with PH, I felt as though that had been taken away from me. The body I had known and relied on for the past 24 years had failed me.

I found it hard to let my friends into my ‘new normal’ and instead of relying on my friendships to move forward, I found myself pushing them away.
I hate to burden anyone, and with the state of mind I was in post-diagnosis, I felt as though that is what I would be to my friends. Hindsight being 20/20, pushing them away was the last thing I should have done and the last thing they would have wanted.

Fast forward almost two years later and here I am, better than I was pre-diagnosis but still learning to adapt to my new normal. I still find it difficult to talk about my journey with PH and I have moments where thinking of what the future holds paralyzes me with fear, but I try to remind myself to live in the moment. After being diagnosed, I was so closed off from the real world and guarded with friends and family – I refuse to go back to that.

As backward as this may seem, coming from someone with a chronic illness, I am one of the blessed ones. I’m surrounded by some amazing friends and family who would reach the ends of the earth if it would help. I now realize that that’s pretty priceless!

As a patient, it seems that every time I think I have found my footing some test, complication or setback rears its ugly head.
I won’t sugar coat it, adapting has had numerous challenges. I think the hardest part of my diagnosis was realizing that there was no cure; no way to make the incurable go away. Being sick is being stuck in a limbo of sorts where feelings of anxiousness and depression can creep in at any given moment, with very little notice. The future becomes one big question mark (even more so than any other average 20-something).

And although it’s easy to get lost in the negative aspects of Pulmonary Hypertension, I try to remain positive - for as much as PH has taken from me, it has given surprising gifts as well.

Throughout this journey (that I’ve hopefully only just begun) I’ve met some amazing people. PH has intensified my love of life, taught me patience where I had little, and shown me how beautiful and fragile life can be.

Like I said earlier, I love being busy – that hasn’t changed. I currently work a full-time job (that I love), a part-time job (at a local hospital) and go to school part-time (Go Marauders!).
PH definitely makes this tricky and I find that I tire more easily than before but I’m a firm believer in mind over matter, and until I’m no longer physically able to do the things that bring me comfort (like staying busy), I plan on keeping on!

I don’t know why it takes hardship to gain insight, but it does. And as much as I wouldn’t wish chronic illness on anyone, I’m grateful for the insight it has given me.

Being sick doesn’t only make you weak, it makes you strong. It gives you knowledge – you learn to stop and smell the roses. You know it’s not important to sweat the small stuff because you’ve had bigger things to worry about.

 My life may be far from perfect but I am blessed.

How to Support a Friend with an Illness

Being a friend to someone with an illness like Pulmonary Hypertension doesn't come with a set of instructions. I recognize that as a young adult it can be challenging to try and support someone through an illness like PH, cancer, MS or even depression. It can be hard to know what to say, what to do, or to emotionally handle it.

I know first hand how difficult it can be for some people to maintain a relationship with someone post diagnosis. Some friends and family members stopped talking to me after I was diagnosed. Some have admitted that it was too difficult for them to support me, and that they had no idea what to say to me. At first I found their honesty frustrating. They had just assumed that it was too hard without even trying to talk to me once post diagnosis. As a young adult, I understand that other people my age may not have the experience or wisdom yet to know how to best support a friend with an illness. Even elders can lack this wisdom. The special kind of compassion and patience it can take to support a friend through a journey like this is not universally taught.

I recognize the unique set of challenges that supporting a friend with an illness can entail. I wanted to share what I have observed through my own challenges in the chance it can help provide insight to anyone else in a similar situation. It is okay not to know what to do or say sometimes. Illness can be a very difficult thing to face.

1. It is Okay to Just Listen

I have recently learned that I have been very guilty of making this mistake. I have a friend who struggles with mental illness, and we often discuss what we are both going through. Because I want to help, I have tried to offer advice in the past. It took sometime, but I have finally learned that sometimes it is okay to stay silent and just listen. Listening is often the best way to support someone. Your friend may not be looking for advice, but just someone to share their ups and downs with. Sometimes advice, even given with the best of intentions, can be counter productive. A solution that may work best for me may not be something that works for my friend. If you find yourself stuck in a situation where you have no idea what to say, it is possible that you are in a situation where it is best just to listen and offer your support. I think it is in most of our human nature to want to make things better. We desperately try to think of words and advice to help make things better. Unfortunately, there are certain situations where words simply fail.

I have had 'healthy' people try and give me advice in an effort to help. Even with the best of intentions, their advice is often impractical for my situation. As you can imagine, it can be a little silly to get advice about being disabled from someone who is healthy, works full-time and can go up a flight of stairs without getting short of breath. I remember a lot of friends and family trying to comfort me during my lowest of lows after I was diagnosed and was on oxygen nearly 24/7 for over a year. To say I was depressed feels like an understatement. In an effort to comfort me, many friends told me how they would backpack across the world if they found out they had a life-threatening condition and how I should see the world while I could. That is an absolutely lovely idea, but it is also unrealistic for a lot of people facing life-threatening illness. If you can't climb a set of stairs, how would you backpack across the world?

2. "That Sucks!"

This is very intertwined with tip number one. I think having a positive attitude can help alleviate some of the suffering that we are in control of. However, I don't think it would be human to try and ignore other feelings like sadness, disappear and disappointment when set backs happen. I also think that it is important for the person who is facing an illness (like Pulmonary Hypertension) to try and find the bright side in situations for themselves.
When another person tries to point out the bright side it can sometimes feel like their struggles are being undermined.
Again, I think it is human nature to want to help and trying to find the positive can feel like the right thing to do. It can be a good way to stay positive, but it is important to know when it is okay to be positive and when it is okay to be realistic. A friend or family member certainly goes through these struggles and set backs with their loved one who has an illness, but their struggles are different than the person with the illness. It can seem easier for a 'healthy' person to try and point out the positives in a negative situation because they usually cannot relate to what is physically going on. When things suck, it is okay to admit that things suck! It is totally okay to agree with your friend about their day or situation sucking.



Being positive, and looking for the bright side isn't a bad thing but be sure to be mindful of the situation. For example, pregnancy for women with PH is strongly advised against. It is very unlikely that I will have children of my own because pregnancy for women with PH has a high mortality rate. Sometimes, in an effort to be supportive, my family will say "you never know- maybe someday." I would rather have them agree that the situation sucks like my good friends Beavis and Butthead. Having them say that it is still possible, when it isn't, sort of feels like they aren't on the same page as me which I need in order to have their support. If they still think something is possible when it isn't, they cannot understand my loss.

3. Neutral Ground Hotel

If you got this reference we should be best friends. If you didn't, lets just pretend number three is just called "Neutral Ground." This may seem really obvious to some, but you can still talk to your friend the way you did before their diagnosis.

What did you used to talk about? The Bachelor? (I think Cailia is going to win.) Making a Murderer? (I am two episodes in- don't tell me anything!) You can certainly still talk about those things. Those are your interests that you probably bonded over together in the first place. I don't always talk about being sick, nor do I want to. I have only certain people that I talk about it with or share so much with. Talking about things too much can also make it more difficult to get over what is happening, and can make me relive a bad event too many times. Sometimes I want a nice distraction! I want to go out with my friends and have a good time. For me, this means that I enjoy their company and we talk about the things we used to and have a good laugh. (A good cry is okay too!)

A lot of things can change after diagnosis. I know that I have changed, but I still really appreciate and value catching up with a good friend over a cup of tea. It is something I did before that I can still do, and really enjoy! You can still enjoy parts of your friendship that you used to have, and still have.

4. Put Oxygen on Yourself First

I have only been on an airplane twice in my life. Once to go to Montreal, once more to fly back home. (I watched Lost too many times and cried during take off, but I felt like a bad ass flying home by myself.)

It turns out flight attendants don't actually give you instructions anymore on how to survive a catastrophic event on an airplane. Instead, you watch a movie. I learned that if you want to survive a crash, you must put an oxygen mask on yourself before you can help anyone else. If you run out of oxygen, you won't be able to help other people. This might feel like the most challenging step for a lot of people, but at the end of the day everyone needs to practice self love and care for themselves.

Supporting someone through a difficult situation like illness can be very challenging. It takes very selfless, caring and special people to hold our hands through these hard times. Sometimes people feel selfish for having their own needs, and try avoid tending to them. However, you need to take care of yourself in order to take care of others and to keep yourself well. Don't be afraid to take a time out and have a bubble bath, or whatever else you may do that makes you fee good. It might be a lot on you emotionally to support a friend with an illness, and that is understandable. Be sure to acknowledge and tend to your own feelings as well.

As a friend of someone with an illness, you may have your own set of challenges. Do not be afraid to talk about about your own challenges with your friends. Friendship goes both ways, and often times we want to be there to be support you as well. I like being able to support my friends because I like helping others, and feeling as if they can still rely on me. Your challenges may be different than ours, and that is okay too. Life happens differently to everyone.

Thanks for reading, and thank you for all my good friends and family members who support me.

xo Serena

"If you have a loved one who suffers, you can be a compassionate ally for him... Play the role of a bell of mindfulness. Your squeezing the hand is like a bell, lovingly calling your friend to come back to himself. That squeeze means, 'I am here for you. You don't need to do anything but breathe.'"
 

— Thich Nhat Hanh, No Mud, No Lotus, p. 42.