Life With PH: Saying No, for Now, to Being a Parent with PH

"I went to a doctor’s appointment in March to see a rheumatologist, who placed me on another medication that causes birth defects. Again, I was warned not to get pregnant. I did something I try not to do at doctor appointments. I began to tear up, and I told her that I wasn’t supposed to have children anyway and I am too scared to adopt because of my dreaded life expectancy. At that moment, she began to talk to me a way none of my other specialists have..."

Please visit Pulmonary Hypertension News to read more.

The Worst Advice You Can Give to a Pulmonary Hypertension Patient

I’ve spoken previously about the day that I was diagnosed with pulmonary hypertension. There are a few moments that stick out from that day that will probably stay engraved in my mind for eternity. When I was first diagnosed, the doctor promptly gave me a life expectancy. It couldn’t have come at a worse time. I was 25 and just starting my career after working less than ideal jobs since the age of 15. I was obviously devastated to hear that my life was ending during a time it felt like it was just beginning. It felt like someone crumpled up everything I worked so hard for and threw it in the trash. As I started to cry my doctor said, “Any one of us could die at any time. I could die in a car accident.” This was the first time I heard this expression, but sadly, it wasn’t the last time.

As the days, weeks, months, and even two years have dragged on, numerous people have told me that anyone could die at any point, that the future is promised to no one. While I am aware of everyone’s mortality, living with a fatal illness is a very different experience than the advice I have been given. I know that the people who have said that “anyone could die at any moment” and “the future is promised to no one” are attempting to comfort me. However, these pieces of advice are often coming from a very privileged source. The doctors who have told me this are able to work full days and have a family. The people my age who have told me this are purchasing their first homes and traveling.

The advice that “the future is promised to no one” is often said in an attempt to equalize my situation with the rest of the population. Unfortunately, I am not like the majority of the population. I had about a one in a million chance of developing idiopathic pulmonary hypertension. Because the disease is considered rare, Canada does not have access to less invasive medications that have been available on the market for nearly a decade. Because PH is so rare, medications are very expensive, meaning that new medications are not always introduced in Canada, or are not approved for funding.
Pulmonary hypertension causes damage to the heart and lungs, eventually leading to organ failure and death. It is a progressive disease that leaves many people breathless and disabled. I myself am disabled because of pulmonary hypertension, and am oxygen-dependent for certain activities. I was on oxygen 24/7 for over a year after diagnosis. Knowing that PH is progressive, and that I might be that sick again, is very frightening.

While death is promised to us all, there is a difference between living with a fatal illness and living without one. Although I try my best to live in the moment, there is often a dark cloud hanging over me. I worry about what will happen to me. I worry about the slow progression of the disease leading to further disability and death. Life is filled with difficulties and tragedies. However, not everyone will understand the feeling of the dark cloud that living with pulmonary hypertension can put over your life. Able-bodied people who say that “everybody dies” in an attempt to over-comfort are forgetting one of the scariest things of living with a fatal disease: the progression.

In some ways, fatal illnesses are almost romanticized by popular culture. There are so many movies who have used this plot to write a love story. While I am very lucky to have someone who has stood by me through all of this, I know that having a fatal illness is not a selling point. It can create a lot of chaos and complications. My life revolves around medication, doctors’ appointments, tests, restrictions, limitations, disability, and oxygen. I know that not everyone is as lucky to have the kind of love I have in my life. Life-threatening diseases are scary, and sometimes they chase people out of our lives.

I often feel like I am living a fine line between quality and quantity in my life because I choose to do what I can while I can still do it. While all of us will someday die, not all of us will have to live with the burden of having a fatal illness, especially as a young adult.

What’s the worst piece of advice someone has given you about living with pulmonary hypertension?

*This article was originally posted to Pulmonary Hypertension News

Pulmonary Hypertension and "The Spoon Theory"

Do you know what the Spoon Theory is? Have any of your friends called themselves a Spoonie?
I didn’t know what the Spoon Theory was until a few months after I was diagnosed with pulmonary hypertension. I found out that I was Spoonie after reading more about the theory. If you have a chronic illness, like pulmonary hypertension, you might relate to the Spoon Theory as well.

Read more about Pulmonary Hypertension and "The Spoon Theory" over at Life with PH

Who Should Raise Awareness for Pulmonary Hypertension?

I am excited to announce that I have a weekly column called "Life with PH" over at Pulmonary Hypertension News. Please be sure to visit the source below to read my first entry about my experience wearing oxygen in public, and whether it helped raise awareness for PH.

Who do you think should be responsible for raising awareness for PH?

How My Diagnosis is Hurting Me

"What's wrong with me?"

This was a question I found myself asking only a couple of months before being diagnosed with Pulmonary Hypertension. Upon diagnosis an expiration date was stamped on my back like a carton of milk. I was officially diagnosed with Stage 3-4 'Idiopathic Pulmonary Arterial Hypertension.' The word idiopathic is a fancy word meaning that the doctors have no idea why I developed this disease. There was no rhyme or reason.

The word 'idiopathic' attached to my medical files has almost felt like a curse.

Upon diagnosis, blood work was drawn to see if the PH was caused by a secondary condition. But this only weeds out diseases that are detectable through routine blood work. Often times, PH is caused by a secondary disease. If the secondary disease is managed, it can potentially help stabilize the condition of the PH.

I have tried very hard for over two years now to seek the help of specialists outside of the PH scope. I have all these loose ends and puzzle pieces of different symptoms and clues. They all seem to overlap into a grey area, none of which really fit into the diagnosis of PH.

Unfortunately, as soon as another specialist reads the words 'idiopathic' and 'fatal' on my medical chart, the appointment ends. I saw an endocrinologist a few months after diagnosis. She told me that I had probably about 5 years to live, and that there was nothing she could do for me. That was the end of the appointment, without even asking about my symptoms or looking through my files. Since then I have had many failed attempts with other specialists, but I continue to keep trying to find answers.

All my medical files from the first year of my diagnosis start with the same line. "Serena refuses to take her one medication as she believes it makes her worse. Serena has improved since her last visit." I look back at the months leading up to diagnosis and I started taking a medication that can interfere with the autonomic/sympathetic nervous system- although this is extremely rare. (PH- by the way, is also extremely rare.) The autonomic nervous system controls certain functions such breathing and your heart rate.

I explained to every doctor that this medication made me ill. It would make me bedridden within a matter of days after taking it, even after I started making progress. Each doctor told me to continue taking it, but I could feel it killing me. I was prescribed nearly 12 different variations of this medication until I had enough.

I stopped taking it. I switched medical teams. My 6 minute test improved by 100 meters. (This typically only happens in PH 3 months after starting a new medication.) The last time I had started a new medication at this point was a well over a year ago.

There are handfuls of puzzle pieces to my diagnosis that don't add up.

The PH progressed at an alarming rate, which isn't impossible, but very unusual for PH. It takes about 2-3 years for most patients to receive a diagnosis of PH, which unfortunately, allows the disease progress to a later stage. (Sometimes the progression of the disease can be escalated by something like pregnancy.) For me, I developed symptoms in a matter of months after starting this medication and a plane ride. Specialists tried to convince me that I have had it for years, but before June of 2013 I was working at the busiest bakery in town lifting heavy boxes up a rather large stair case- something that would be very difficult for someone to do with PH. At one point in my life I was carrying drum kits and guitars, and loading them into a van. I worked out regularly with no symptoms up until October 2013.

A PH specialist that I was seeing said that I may have POTs because of a few symptoms I show. My regular bloods pressure is 80/50 which is abnormally low. (My blood pressure was this low before starting PH meds, which can lower your blood pressure.) My blood pressure also drops when I stand. My heart rate increases by over 30 beats, and my o2 levels drop. So many of my symptoms overlap with other invisible diseases, making them harder to diagnosis. POTs can also be caused by a nervous system dysfunction.

One of the main causes for PH is a congenital heart defect. Upon diagnosis I learned I had a valve open in the chamber of my heart, and that the hole was rather large. I've heard conflicting opinions over and over again about this hole. It has been so confusing to hear one specialist say I will die quicker without oxygen use, while another one said I am fine because of that hole. A cardiologist said that if I got the hole repaired there is a chance I could be in better shape. I was never given a cardiologist after my diagnosis despite the abnormalities that appeared in various tests. Despite the hole in my heart, I was still given the diagnosis of having an 'idiopathic' disease.

I also had a fistful of other unexplained symptoms that are typically tied to sympathetic nervous system. I remember laying in the hospital bed after my right heart cath telling the doctors that my feet and arms were tingling. They told me that it had nothing to do with PH and that it was fine.

I've had night sweats, muscle weakness, the inability to regulate my temperature, intolerance to the heat and cold, nerve pain in my thighs and head, along with having my blood pressure drop upon standing. My blood work has also came back irregular for  markers for various things, but again, the word 'idiopathic' stops any specialists from trying to put together these puzzle pieces. I sometimes feel like they see me like a lost cause.

In March of this year I finally saw an rheumatologist. She took a look at the blood work done from the very day I was hospitalized and diagnosed, nearly two and half years ago. She laughed a little and pointed something out to the student doctor. She then told me that I wasn't crazy, that something was definitely abnormal. I had a high amount of white blood cells. She told me that she had a gut feeling that I had Lupus, and I was immediately put on some heavy duty imunno-suppressants. I received a phone call three weeks later informing me that I didn't have Lupus, or any other rheumatoid diseases. I am still on the Lupus medication, just to see what happens.

I have come into my appointments with research papers, along with written timelines and events leading up to my diagnosis. I bring up my strange reaction to do different medications. They are a little bread crumbs leading me to somewhere. Unfortunately, my desire to be proactive in my treatment has not been with an open mind from the specialists I have seen. Several times I have heard "you have idiopathic PH, sweetheart" as if that is a concrete diagnosis.

The word idiopathic relates to any disease that arises spontaneously with an unknown cause. To have it treated as if it a concrete diagnosis that answers to all of my symptoms, and has been diagnosed through blood work, is beyond frustrating.

Thankfully, my family doctor is a wonderful and patient man. From the very start of my diagnosis he has said that he would do whatever he could to help me. I recently went to him with my concerns, and he is happily referring me to several different specialists. He even suggested that I research different specialists to see what one would have the most interest in my case. I have been referred to other specialists before, I think they feel scared to over step over any other specialist's work and diagnosis.

It is discouraging, but I will keep trying until...

Truthfully, a large part of me is frightened that my PH will not be managed in an optimum way if my other issues are not addressed. It is clear to me that they over lap, and it is terrifying to think I could get sicker because of this. I hate thinking that I might get sicker, or even worse, all because no one will listen to me. All because they see the word 'idiopathic.' That giant question mark has made it impossible to get a real second opinion, or another diagnosis from another specialist.

I have already had one specialist tell me that I am "not crazy." I try to stay hopeful that someday my puzzle pieces will make sense to a specialist, and that I can get better treatment to help me stay here and healthy for as long as possible.

Kawaii Box Review and Giveaway

My best friend Melissa got me a 3 month subscription to Kawaii Box back in February. It was such a great gift idea because I kept getting presents well after my birthday passed. (Who wouldn't love that?) As someone living with Pulmonary Hypertension some days, weeks and months are more challenging than others. Having a little present arrive at random has certainly helped brighten some of my more challenging days.

In an earlier post I wrote about how subscription boxes are a great gift for someone living with a chronic illness. It also makes a great gift to give yourself. Now you have the chance to get your very own Kawaii Box!

Kawaii Box was kind enough to send me their May 2016 box to review. Kawaii Box also wants to send one of their boxes to one my readers, so please be sure to check the bottom of the post for giveaway details!

What is Kawaii Box?
Kawaii Box is a monthly subscription box. Each box contains about 10-12 handpicked kawaii items from Japan and Korea. Kawaii Box often includes a mix of Japanese candy, accessories and stationary.

How Much Is Kawaii Box?
Kawaii Box is $19.80 USD a one month subscription. It also includes free shipping world wide!

Kawaii Box May 2016 Review

Above is a picture of the outside of the Kawaii Box. Even the shipping box is very 'kawaii' (a Japanese word for cute.)


Here is glimpse of what is inside the May 2016 Kawaii Box. Everything arrived in perfect condition, and each box comes with a 'thank you' card that describes each item in the box. Items are wrapped in tissue paper covered in hearts. The box also includes details about how you can take a picture of your box for the chance to win another Kawaii Box!


The May box included the following stationary items:
- Lucky Star Origami Papers: the back includes small pictures how to create star shaped origami with the papers.
- Puffy Dessert Stickers
- Hello Kitty Pencil Sharpener
- Animal Icon Stamp Set
- Pastel Bear Pen: after having received several Kawaii Boxes, I can attest to how much I love the pens they include in their boxes. They are perfect for writing letters and doodling. (The ink comes out very smooth!)

I have several pen pals and the stationary items in the Kawaii Box are always great for creating personalized letters and mail.

For snacks, the box included:
- Anapanman Corn Rings
- Kasaugai Chibi Vege Remune Candy
The above picture also includes heart shaped silicon bracelets that were also featured in the May box.


The fun accessories included in the Kawaii Box for May 2016 are:
- Cute Animal Socks
- Kawaii Girl Coin Purse
- Kitty Doughnut Squishy: My favorite item! It resembles Hello Kitty and is perfect to squish if you feeling nervous or anxious....or you can squish it just for fun too!


The last item included in the box is this Fluffy Pom Pom Key-chain. I tried to take a picture that would should off the shape and puffiness of the pom pom.

Kawaii Box has been one of my favorite subscription boxes. The stationary items are perfect for anyone who has pen pals, or likes enjoys being creative. The snacks are always very fun as well. Some of the previous Kawaii Boxes I received included interactive snacks that you make, which is really cool. It makes for a fun activity with a friend or by yourself.

As mentioned before, the Kawaii Box ships for free anywhere in the world which is a really good deal for a subscription box. It can take several weeks to receive an item because the box ships directly from Singapore. I have contacted their customer care about some of my boxes because some took longer than receive than others, and their staff are very helpful and friendly.

Kawaii Box Giveaway

There will be a Kawaii Box give away on my Facebook Page, but you may also enter here. To win a Kawaii Box of your own please enter the contest here: Kawaii Box Giveaway

Good luck!

Are We Missing the Point? How Gord Downie Helped Change the Way I Face My Illness

On May 24^th Gord Downie and his doctor from Sunnybrook Hospital in Toronto confirmed that he was diagnosed with terminal brain cancer. Their report stated that Downie had been dealing with the very serious disease privately since December 2015. Later that week, the Tragically Hip, a band that Downie has been performing with for over 20 years, announced that they plan to have a tour this summer.

Long-time fans were obviously heart-broken to hear about Downie’s diagnosis, making purchasing tickets for their summer tour a more difficult endeavor. Presale tickets have sold out in less than two minutes. In fact, presale had sold so quickly that The Tragically Hip added four more tour dates to accommodate to the predicted madness that will happen as I write this article- the public sale of their tickets.

Fans are obviously upset over what has happened over the sale of The Tragically Hip’s 2016 summer tour. Other ticket outlets have been purchasing large quantities of the tickets available during the presale, only to resell the tickets at more than 5 times the cost of the original price of tickets. Ticket scalpers are obviously taking gross advance of a sensitive situation that should be treated with the utmost respect. To make matters worse, a portion of the original ticket sales is being donated to Sunnybrook Hospital. Who is profiting from the resell of tickets at nearly 5 times more their original value?

There have been on going news stories regarding the foul play made by the ticket scalpers. Fans have flooded social media to express their anger at ticket scalpers. Some have gone so far to make online petitions to protest the resale of tickets at such a high price. Tickets are going for over $8,000 USD- making purchasing a ticket from a scalper nearly impossible for the average Canadian. Scalpers are trying to profit off of someone’s illness, and are using bots to take away tickets from the average hard working fan. It is wrong- so outrageously wrong.

But are we missing the point? 

Reports on the resell of tickets by scalpers flooded social media and news outlets almost immediately after Downie shared his diagnosis. While it is iniquitous that ticket scalpers are profiting more than the band, and the hospital they were donating money to, this fiasco has overshadowed everything else.

For anyone who is familiar with my blog, I was given about 5 to 10 years to live about two and half years ago, after being diagnosed with Pulmonary Hypertension. I think that Downie’s diagnosis might have struck a different chord with me, and perhaps other people in my situation.

Two years ago I was given a life expectancy, and was severely disabled requiring supplementary oxygen 24 hours a day at the age of 25. Although I am still living with a very serious life-threatening illness, I am doing better than I was two years ago. However, I found that I became afraid of living for fear of making my condition worse. Going to concerts and shows was one of my most cherished past times in life, but I stopped going to them after my diagnosis out of fear.

I worry how the bass will affect my heart (something I have always had an issue with.)
I worry that I will need to wear my oxygen as the night goes on and I get tired.
I worry that I might have to wear a medical mask to protect me from germs.
I worry that people will stare at me because of my medical equipment.
I worry that strangers will ask, “what is wrong with me?” Which is always funny to answer, because I don’t feel like anything is “wrong” with me. I just happen to be sick.

Living with a terminal or life-threatening illness has sometimes made me feel like I am staring down a barrel of a gun. When I heard about Downie’s diagnosis, and how he planned to go on one of his best tours yet, it really encouraged me. Hearing the way he faced his diagnosis made me want to change the way I was facing mine. This man was also staring down a barrel of a "diagnosis gun" and he’s going to do what he loves to do anyways. Why wasn’t I doing the same? I realized I couldn’t keep letting life pass me by out of fear. Maybe it is time to test the waters a bit. Maybe I cant’t know what I can or can’t do without trying. I was lucky enough to score presale Hip tickets (please don’t hate me!) I also plan to go to a smaller local show next week just to test out the waters. The idea of going to a venue that houses nearly 20,000 with lots of stairs really freaks the heck of me, but I am hoping I will have a great time with no ill consequences to my health.

If only for a moment, can we shift our focus from how what the ticket scalpers are doing should be illegal, to how great The Tragically Hip and Gord Downie are?

Jack Chamber’s painting 401 Towards London No. 1

The Hip to me, are classic Canadiana, comparable to Jack Chamber’s painting 401 Towards London No. 1. When I listen to their music I can picture the Canadian landscape that surrounds me. This is mostly because of their phenomenal story telling. Some of The Hips most loved songs tell the stories of news events that took place in Canadian towns. Fifty Mission Cap tributes former Toronto Maple Leafs player Bill Barilko, and his mysterious disappearance due to a plane crash, and the eeriness surrounding the retrieval of his crashed plane. 38 Years Old recounts the fictional account of some 14 inmates that escaped from a prison in The Hip’s hometown of Kingston. Their lyrics and songs remain a vivid backdrop for Canada and its stories, both big and small. Real and fictional.

I want to thank The Tragically Hip and Gord Downie for lending their music to become apart of the soundtrack to so many people’s lives. I remember how excited a bar full of college students got when someone sang New Orleans Is Sinking at a Karaoke bar, in Waterloo, Ontario, just a few years before my diagnosis. I remember watching the music videos for Bobcaygeon and Ahead by a Century back when Much Music still played music videos, on early Saturday mornings before my parents were awake. Whenever Fireworks comes on the car radio my boyfriend, Spencer, sings along and squeezes my leg extra hard at “you said you didn’t give a fuck about hockey…” This will always be one of my favorite memories.

While I think that we can all agree that what has happened with the ticket scalpers in this situation is very disappointing and in bad taste, please don’t let it outshine what is really important. Gord Downie shared a very personal diagnosis with the public, something that I know is very difficult to do. I am sure that he and his family are facing something that is very hard to imagine unless you have experienced it. By letting the ticket scalpers cause so much anger, we are letting them place value on the wrong things. There is so much media attention surrounding this. Wouldn’t it be more beneficial to raise funds and awareness for brain tumors? Or discuss about the accessibility of drugs and treatment of diseases in Canada?

The way Gord Downie is facing his diagnosis has shown me how I want to face mine. It is also a good reminder for what is truly important in life, because I know that sometimes we all forget. Instead of focusing all of our attention on the negative, lets celebrate what was, what is and what will be.


Thank you so much, and I look forward to seeing you in August.

6 Tips for Facing Retirement as a Young Adult



In December of 2013 I was diagnosed with Idiopathic Pulmonary Hypertension. Although my symptoms came on quite quickly, I was only diagnosed once I was in severe right-sided heart failure and required supplementary oxygen 24/7 a day.  Months prior to my diagnosis I had finally started my first adult job after years of pushing tea and cupcakes. This was not as fun as it sounds, and finding a job with a Fine Arts degree in a tech city is no easy feat. 



I went back to work about 6 months after my diagnosis, which in hindsight, was way too soon to return to work. I was eager to return back to the job I loved, interact with people, and feeling like I was contributing to society again. More than that, I wanted a distraction from what was going on in my life. I managed to work part-time a few months short of a year, but realized I was in no condition to work. Unfortunately, it became clear that the stress and physical demands of working was too much for me and I had to give up my newly found career.

I really do have a bunch of 'island time' themed
outfits.

As such, at the age of 27 I had to retire, and I have been retired now for nearly a year. 

I wanted to share what I have learned in the past year about being retired in my 20’s. Retiring as an early adult is uncommon. Therefore, very little support and information is available for this kind of transition.

So aloha- welcome to the island. Grab your beverage of choice and your favourite Hawaiian print shirt. (If you don't have one yet, now is the time to invest in one!)



1) Netflix and Nap



Chances are if you have a chronic illness or a condition that is preventing you from working, you will need some down days. Don’t feel guilty for needing some time to take it easy on your body; this is part of the reason why you are no longer working. If your body is telling you that you need a down day, reach out and take it. Take naps as needed. I know my body needs extra TLC compared to most people my age because it works so much harder just to do simple tasks (like breathe.) 

It is okay to find a good show on Netflix and slowly marathon your way through. Just be sure that watching iZOMBiE isn’t the only thing you do for a month straight. Although majority of your family and friends will be at work or at school during the day, it is still important to use and limit your TV time wisely.



2) Stay Sharp



According to a study found here 42% of college graduates never read another book after university. After I stopped working for a bit I noticed that I no longer felt as mentally 'sharp.' In order to try and maintain my brain I read several books a month and write.


Books have been a great source of entertainment for me. Fictional books can allow you escape reality (if only for a moment.) Inspiring and self-help books can help you cope, and find hope. Trashy memoirs are usually an easy read and thoroughly enjoyable.



If you want to continue to learn on an educational level, you can always find books in your area of interest. iTunes also offers “The Open University” which has free lectures and content on various subjects. If you are really ambitious in continuing learning, you can try and create your own lesson plans which is a great way to still set goals and deadlines for yourself. (Some of us A type personalities really miss having that kind of structure.)

Books and lectures aren't the only way to stay sharp. You always find activities that incorporate    working out that brain muscle in a way that feels more like play. Puzzles, word searches and logical/spacial thinking work books are all a great way to keep that hamster in your brain on the wheel.

Documentaries also available on Netflix and YouTube for those days where you want to learn from the comfort of your couch and sweat pants.


3) Creative Outlet

Creative outlets are important to both develop and hold onto during your transition to retirement. Hobbies and interests are often things that we naturally enjoy doing. Sometimes our illnesses might take away our ability to do certain activities that you previously enjoyed doing. As such, you may find yourself on the hunt for new interests and hobbies.

It is really important to maintain a creative outlet. Being creative and having hobbies will still provide you with a sense of being able to accomplish a goal. I know that when I stopped working I felt a sense of loss. How could I accomplish anything if I didn't have deadlines and the demands of an office to meet? I learned to set my own goals, and to create my own accomplishments. Sometimes my goal might be to do one small drawing in a day, or to work on writing an article.

Having a creative outlet can also have many beneficial side effects. It may help reduce stress and anxiety.

Drawing and writing aren't the only ways to have a creative outlet or hobby. Hobbies can range from crafts, scrap booking, sewing and playing music to volunteering, playing cards and cooking!


4) Keep Moving

It is very important to keep as active as possible. Unfortunately, some chronic illnesses can make it difficult to stay active. Pulmonary Hypertension, for example can cause disability as it leaves people breathless. It also has the potential to cause dangerously low oxygen saturations. As such, it is recommended that you speak to your doctor to discuss the best way for you to stay active.

I am disabled due to having Pulmonary Hypertension, but try to stay as active as possible. I will try to walk at least a mile each day. Walking on flat land is easier for me, so if the weather sucks or I am having a bad symptom day, I will walk around the house until I reach the mile mark. I also try to get up to walk for 5 or 10 minutes if I have been sitting for an hour.

If walking without a destination sounds boring to you, you can always go to the mall or to a museum. Both provide climate controlled environments with flat land (and elevators if you are unable to do stairs.)

At the world's worst wax museum.

In addition to walking, I try to stay active by doing a 20 minute session of yoga or Pilates from the comfort of my own home. There are great and affordable apps for yoga and Pilates, and there are also free videos on YouTube as well. Depending on your abilities, you may be able to adapt these works out by doing chair yoga.


5) Treat Yo Self

Ah, the catch phrase that might ruin this generation (and my bank account.) It is very important to take care of yourself. Try to get enough sleep. Eat as healthy as possible, but also know when it is okay to cheat. Bottom line, you should be able to feel like you can enjoy your retirement. We shouldn't be punished for not being physically capable of working.

Make plans for whatever adventures you are able to do. This can range from trying a new cafe, going to a new museum or trying a weekend get away. Find ways to make yourself smile. It can be as simple as putting a bird feeder outside of a window.

6) Find Yourself

It can be very difficult to retire during what should be the prime of someone’s life. Even older people have a difficult time adapting to the transition of retirement because of how heavily careers and professions are weighed in with our perception of ourselves. In order to get to know someone, we often ask “what do you do?”

While many of us are or were very passionate about our careers, I have learned that jobs are usually on the more superficial layer in terms of defining someone. For example, if someone heard what my former title was, they might assume that I studied business. I never took a business course in my life. (It was all learned through pushing cupcakes, baby!) My real passion has always been being creative. I went to university for fine art (drawing, painting and sculpture) but now I use freelance writing as a form of being creative. So my former career did not really define me, and my current retired status doesn’t really define me either. I have always been a very hard worker, and very career driven (hence why I started a blog a few months after facing a heavy diagnosis.)

Facing retirement so young can certainly be challenging. I still have my days where I question my self worth because I can no longer contribute in the ways I used to, or desire. However, I am proud of myself for having such a big obstacle and still accomplishing everything that I have through freelance writing. My diagnosis, as cheesy as it sounds, has taught me a lot about myself, and even other people in my life. It has also pushed me to continue to try and find myself and be the person I want to be despite everything.

To The Person Who Wondered If I Am Really Sick

A photo where my invisible illness is visible.
I sleep with oxygen every night.

As someone with the often invisible illness Idiopathic Arterial Hypertension (also known as PH,) I have had small incidents of strangers who have questioned the severity of my illness. I have had strangers question why I use an accessible parking spot. It really sucks trying to justify your illness (especially when it is life-threatening) to a stranger. It isn’t an easy thing to open up about, and often time these strangers aren’t asking you about whether you are disabled or not because they genuinely care about you. They are looking for validation. They want to be "right" about calling you out over a silly parking spot. Unfortunately, when you have an invisible illness this is bound to happen with strangers.

Recently I found out that someone I know asked if I was really sick. It felt like a punch to the gut. Life with an invisible and life threatening illness is so incredibility complicated and painful. I don’t understand how someone could question the validity of my illness. I figure if one person has questioned my illness, other people may have also wondered how serious it is, and how it impacts my life. Their comments made me feel extremely deserted.

I may look like a vibrant young woman who is just on the cusp of starting her adult life, but looks can be deceiving. Although I may not look ill, I have received a diagnosis. I have seen many specialists; I have gotten second opinions. My diagnosis shouldn’t be up for debate, but apparently it is. Pulmonary Hypertension is an invisible illness to the naked eye, but there are many indicators that I am sick to someone who understands the disease.

Majority of society doesn't know what signs and symptoms to look for to indicate that I have Pulmonary Hypertension. Mainstream media often depicts someone as being sick as looking pale, frail and bald. However, this isn't always the case for determining how serious an illness is. Many invisible illnesses can be well concealed, especially with the help of make up. Not only is my illness well hidden, but so is my disability. A symbol of someone in a wheelchair is often used for accessible parking spots and bathrooms, even though there are an array of different disabilities (including invisible ones.) Because of this, society weighs so heavily on being able to visibly see a disability in order to believe its validity. Regardless, there are still many different disabilities that do not require a wheelchair, or any visible medical equipment.

I can easily cover up how sick I am- to the point that many doctors ignored the fact that I was heart failure before being diagnosed. Pulmonary Hypertension isn't diagnosed through blood work. It is diagnosed through a process of elimination. For the person who doubts my illness, I can ensure you that I went through a plethora of invasive tests to receive a diagnosis.

At one point I had so much radiation exposure the technicians running the various x-ray, MRI and CT scans turned me down for another test, fearing it would be too dangerous. However, a specialist demanded that I had another CT scan, even though I had several in the past 24 hours, and even more in the last week and month leading up to my diagnosis. Dozens of vials of blood work was also ordered to rule out other underlining diseases, such as HIV.

The most invasive and effective procedure for diagnosing PH is called a Right Heart Catheter (which I had on Christmas Eve in 2013.) For this test, a tool is placed in a vein in either your groin or neck that leads to your heart. Mine was placed in my groin and the tool eventually made its way up to being directly in my heart to measure the pressure my pulmonary pressures. I was completely awake for this procedure, and was only given a small numbing in my leg. This test confirmed that I did indeed have a high pulmonary pressures, and I was officially diagnosed with Pulmonary Hypertension.

If you try to walk along side me, you may notice that I need to slow down, or may have to try and catch my breath while speaking. You might notice me gasping for air if we had to walk up a hill or some steps. People with PH are often out of breath by the time they reach the third step in a flight of stairs. I may look perfectly healthy, but I have a lung- heart disease. Those are two very vital organs that needed to do the most basic of tasks that are often taken for granted, such as going up the stairs, or bending down to tie your shoes.

Being short of breath was actually one of my first noticeable symptoms. I had my first symptom in Fall 2013. From there my symptoms progressed at an alarming rate. I was diagnosed quite quickly, as most people wait 2 years for a diagnosis. However, by the time I was diagnosed I was told that I was somewhere between a stage 3 and a stage 4.  Even though I was on the verge of dying, I still didn’t look sick to trained professionals (which delayed receiving a diagnosis before the progression of the disease kicked in.) If I didn’t received medical intervention when I did, I am not sure how much longer I would have lasted. In the weeks and days leading up to my hospitalization, I would wake up gasping for air. I had started to stop breathing in my sleep.

Pulmonary Hypertension doesn’t just make you short of breath; it narrows the arteries in your lungs. This makes it more difficult for oxygenated blood to be pumped throughout your body, causing an overloading in the heart as it desperately tries to pump blood. All this extra work causes heart failure. A symptom of PH is having an elevated heart rate- many people with PH experience a heart rate similar to someone who has run a marathon while doing a simple task; such as walking, or cleaning the house.  Many people with PH (like myself) also suffer from lower than normal oxygen saturations and dyspnea. Having low oxygen satuations also causes someone to tire very quickly because of how hard their body must work to accommodate.  Perhaps these aren’t visible symptoms, but that shouldn’t make the seriousness of an illness illegitimate. These symptoms are serious, and can escalate the progression of the disease, and can lead to organ damage and failure.


There is a huge difference between whether someone looks sick, and whether someone is sick. Just because someone looks healthy, that shouldn't dismiss their diagnosis nor their symptoms. I can only assume that the uninformed comment about whether I was sick or not was influenced by how I appear on social media. My Instagram account is largely photos of cafes I visit, my dog, my boyfriend and little adventures I go on. Although having a life threatening illness dictates how I live my life, it does not define me as a person. I don't want to document my illness too heavily on social media. As such, I don't include many pictures of me at the hospital (although I do include them from time to time to help raise awareness for PH.) I also don't include many pictures of me wearing oxygen, or taking medications. I don't want people to pity me, or assume I am trying to get attention through my illness. Social media is also a great way to edit your life; you can only show what you chose to share. I may have a picture of me on a hill smiling, but that doesn't mean I wasn't gasping for breath, and had to take breaks to get there.  For me, I only capture what I want to remember, and what I want my friends and family to remember.

After my diagnosis I was so terrified to enjoy moments in life. I was scared that if I laughed or smiled, that would mean that I was okay with what was happening. Now it seems like I have to be afraid to laugh or smile because of how other people will perceive that. I have my bad symptoms days where I am extremely short of breath, and become even more physically limited. I spend hours each month waiting in waiting rooms of hospitals and blood labs. I also spend hours each month commuting to specialists appointments. I often feel ill as a side effect of the medications I am on. These medications are not elective, I also do not great a break from taking them. I must take them everyday like clock work. There are currently no treatment options that will cure the disease I have. The medication I have will only slow down the progression of the disease, but unfortunately, it is still considered fatal. It is difficult being in my 20's and knowing that I have a fatal illness. I am at an age where I am watching more and more of my friends get married, travel, start careers and have children. I was diagnosed several months after starting a career job, and I loved working. I was saving up to move out of my parent's house. I worked with children throughout university and received a specialization in education. To think that I am faking this disease is ludicrous. 

To the anyone who isn't sure if I am ill; please consider that not all illnesses are visible from afar. Sometimes illnesses are very easy to mask with make-up, or to filter out through social media. Just because an illness or disability is not visible does not mean that it cannot be serious or life threatening. Its visibility should not equate to its validity.

Cross-posted: When a Doctor Said 'Hope Won't Help' My Chronic Illness'

An article I wrote is up on The Mighty​ today. Below is a clip of the article. Please feel free to read more at the source.

"The day that I was diagnosed with pulmonary hypertension, a doctor waltzed into the room and announced that I had five, maybe 10 years left to live. He had spoken to my parents first about the news. I looked over at my parents to gauge their reaction. Was this really happening? My mama said very strongly, “It will be OK, because we have hope, and we will get through this.” The doctor seemed almost angry by what my mama said. He nearly cut her off to say, “This isn’t cancer. Hope won’t help you.” That was pretty much the end of the conversation. Well… actually, it wasn’t.

I was going to leave this part out, but it didn’t feel authentic. After what the doctor said I threatened to end my life. If hope couldn’t help me… if there was no hope for me, how could I possibly face each day? I was instructed to go have more blood work and go home after my parents convinced the doctor I would be safer at home with them than at their ward. It was a few days before Christmas. None of this felt like the movies."

Please follow the link below to read more:

http://themighty.com/2016/03/pulmonary-hypertension-how-having-hope-helped-me-get-better/

The Side Effect That I Wasn't Warned About

"oh brother"

When you are first diagnosed with something, you are usually warned about side effects. Upon the diagnosis of Idiopathic Arterial Pulmonary Hypertension I was warned about side effects of the disease and side effects of the medication. PH can cause a variety of symptoms, or side effects, such has shortness of breath, chest pain, swelling, and heart failure. Some of side effects of some of the medications I take? Liver failure, cancer, anemia, low blood pressure (in all the wrong places,) head aches, stuffy nose. General malaise is probably a side effect of both the medications and the PH- and maybe life. I was prepared for all of that, but then something else set in.

Guilt.

I have suffered from both external and internal guilt as a side effect of PH. I feel such incredible guilt, although the feeling has faded as the years have gone by. I sometimes still find myself plagued by it. It is difficult to see how such a catastrophic that I had such little control over runs down hill. It effects so many other parts of my world.

I feel guilty because my life isn't the only life effected by all of this. Sometimes I find myself saying "sorry, I am sick." Apologizing for something that I have very little control of. Saying sorry, even though I know these situations will happen again. Unable to work, I still live at home and rely on my parents. I am sure they planned to retire soon, and I feel like I messed up their golden years because my golden years currently overlap with theirs.

Me living at home is also expensive for my parents. There are obvious medical costs, plus my crazy expensive food (because I eat mostly fresh and organic foods.) I see how my side effects so deeply impact them in various ways from socially to their well being. I hate that something that happened randomly hurts us all so deeply. I try to handle this human experience as graciously as possible, but it isn't always possible to sugar coat things. A positive attitude will help get you through the day, but it won't change the fact that I struggle going up a flight of stairs. I also think that you have to admit when things suck. It wouldn't be healthy to ignore your challenges, or to try and dress up a wolf as unicorn and hope that it doesn't eat you.

I feel guilty because the life I planned with my boyfriend is now very different. Sometimes I feel like I have very little to offer to the relationship. On paper, I feel like I kind of sound like a lemon. "Free to a good home: 28 year old girl-woman who can't drive, work or breathe on her own (for certain basic activities.) Very expensive special diet. Comes with Boston Terrier that you must also take care of." Hopefully from that line you can tell that I have a sense of humor, which is one of my selling points, beside my really cute dog.

Spencer has stuck by my side through my diagnosis, which I am so grateful for. (But it also has made me feel guilty.) He has so much life a head of him, and I don't want to stop him from following his dreams. This experience has really shown me what is valuable. Through all of this proverbial crap I have become a more supportive and grounded person. However, that doesn't change the fact that I probably won't have biological children. Or the fact that I am not sure if I will be comfortable adopting because a few doctors gave me the life expectancy of a hamster at the start of all this. I worry that my situation will effect what jobs he will want. I don't want him to take the job with benefits over a dream job because of me. I don't want him to have to take a high paying job that he hates because I cannot work. We were supposed to be partners. Equal partners with different strengths and weaknesses to balance each other out. I am strong as hell- but are these new found strengths valuable for "real life?" I feel like I am not apart of real life, but a mere by stander watching from the outside. I know he doesn't view me as an invaluable counterpart, but sometimes I wonder what I truly can contribute to our relationship.

I also feel a lot of internal guilt. At the very beginning of all of this I blamed myself for getting sick.

"I must have done something wrong."
"Healthy people don't get sick."
"Was my diet really that bad?"
"Did I really stress that much more than my peers?"
"What did I do so differently than other people my age that this happened?"
"Am I being punished? Did I get sick because I am a bad person?"
Those are some of the thoughts that I really struggled with. I felt as if that I must have done something wrong to get sick, especially because I was diagnosed with a rare disease. Overtime I have tried to accept that the perfect storm must have been going on in my body that allowed the disease to develop. I didn't do anything to "deserve" this, it just happened.

I believe that holding on to and hoarding negative emotions are not healthy. I have made progress releasing a lot of the guilt that I feel over being sick. Obviously life happens, and the feelings bubble up from time to time. However, it is important to handle those feelings appropriately. I accept that I didn't do anything wrong to get sick. There was probably nothing that I could have done to prevent it from happening.

I also accept that my parents love me so deeply and unconditionally. My parents helping me, taking care of me and going well above and beyond the call of parents of a 28 year old child are all an act of love. I know that them taking care of me, and supporting me isn't even a question, hesitation or thought for them. They want to take care of me. I know that they would want me to feel loved through their actions, and not like a burden.

I also know that Spencer loves me, and rarely sees the disease. He understands that although being sick dictates very important aspects of my life, the disease isn't who I am. He supports me through all of this, and sees who I am truly am- which is why he sees me as an equal and valuable counterpart to the relationship. I know that his companionship is something that he chooses to offer me, and he that this is another act of love.

It has taken me a few years to slowly release all of the guilt, and to slowly accept all of the love that others want to give me. I've learned that being sick isn't the time to start denying yourself love and simple pleasures. For me, it has become a time to indulge in all the good things that I can. For me, I try and find the beauty in every day.

(Please note: I refuse to refer to it as "my disease" or "my illness." I have no ownership over it, and it is not a characteristic or trait.)

Accessibility of PAH Therapies in Canada: Part III - Patient Testimonials

Part III: Testimonials From PH Patients Who Are On A Treatment Not Available or Accessible in Canada

This is the third and final part of the series. The first part of the series, Access to Opsumit in Canada can be found here. The second part of the series about Treatments That Are Not Available in Canada Can Be found here.

Below are testimonials from PH patients in the US who are on inhaled PH medications that are not available in Canada. These testimonials are unpaid, and are shared by fellow PH patients in an effort to show the positive effect these medications can have for some PH patients.

"I started Tyvaso on the trial in November of 2004 at UCSD medical center. I was asked to go on the trial due to my high numbers.  I was diagnosed in April of 2004.  My mean pressure in my heart during right heart catheter was 96(normal is between 15 and 25).  I had gone years with a misdiagnosis of severe asthma.  I went in for another right heart cath to start the trial.  My numbers had come down, good news to 56 with the use of a medication called Tracleer.  While on the operating table, I took my first puffs of Tyvaso, I took nine.  I was the first patient ever to take nine puffs of the medication.  My mean pressure dropped from 56 to 46.  It was really a miracle for me.  At the end of the study my pressure was 35.  Darn right near normal.  Sadly though, I had to quit the Tracleer and start on Revatio due to liver problems.  But I have been on Tyvaso since starting.

I started going downhill a little November of 2013, I was needing evening O2, and usually I need only at night time.  At this time I was on Revatio and Tyvaso.  I had another dreaded right heart cath in March of 2014.  My doctor was happy with my pressure of 50.  But I was not.  My husband and I pressured him to add another medicine.  He decided to add opsumit, since I did so well on the tracleer and it is very closely related to tracleer without the liver complications we decided to give it a shot.  Fast forward to June of 2015.  I had an ECHOcardiogram.  I asked my doctor my "estimated" pressure as it is not as thorough as a right heart cath.  He said 29.  Wow...almost normal.  I have been feeling pretty good these days.  I am very reactionary.  I have good days and bad.  Without these medications I would for sure be in pretty bad shape of worse dead.  I have 3 adult children and a husband.  A grandson.  Also, raising my niece and nephew, ages 15 and 12."
- Julie, 48 years old
San Diego, California, USA

"I started the inhaled therapy, Treprostinil (Tyvaso), in 2011 after going back and forth with my doctors about IV therapy since my pressures were high and my physical activity was low.  They decided to try me on Tyvaso just to see how I would respond on it.  At first it was a bit difficult to fall into a routine of breathing on a nebulizer every 4 hours, but I wasn't too concerned after I started to feel better.  Before Tyvaso, I'd have a very hard time doing the simplest daily tasks like making my bed, or even showering, without getting short of breath.  Now after almost 5 years on Tyvaso, I'm able to exercise, walk long distances, go on bike rides, almost anything and everything that I wasn't able to do b efore the medication.  It has also improved my 6-minute walk test results, as well as my echocardiogram results.  Tyvaso, in conjunction with my oral medication, has improved my quality of life tremendously and I'm so grateful to have the therapy available to me."

-Reinee
Bay Area, California, USA

"In 2009 I was diagnosed with Familial Pulmonary Hypertension. At that time I was put on two different treatments. One was a long established drug Tracleer, and the other was an inhaler that had been FDA approved only months prior, Tyvaso. The doctor has switched my pill form of medication, but Tyvaso remains my main treatment. I always credit Tyvaso as saving my life. Before diagnosis I was not able to walk up a flight of stairs without getting out of breath, now I can go hiking and jogging. I can breathe easier with Tyvaso, if I am without it for a day I can feel the difference in my chest and my exercise capacity is diminished. "

- Raeana Rader, 28 years old
USA

"I was diagnosed with pulmonary hypertension after I passed out while walking into work. That
was simply too much exertion for my body. I was placed on I.V. Veletri and two oral medications. They got my symptoms under control but I had terrible side effects from the Velerti. The nausea, vomiting, diarrhea and headaches were the worst. I eventually improved so much that my doctor let me do a conversion from Veletri to Tyvaso. Tyvaso gave me my quality of life back. The only side effect I have with Tyvaso is flushing. I feel like a normal human being again. Because I am no longer on Veletri, I can take my kids swimming and no longer have to sit on the sidelines. I am able to exercise on an elliptical for an hour at a time and I have recently started taking a spin class. Tyvaso is also much cheaper than Veletri and because it is noninvasive, I do not have to worry about line infections which could be costly and detrimental to my health. I feel very blessed and grateful to have Tyvaso available to me."
- Ashley, USA

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. 

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