I’m Aware That I’m Rare: Tina Proulx (478)

the phaware® interview

In this episode, Tina Proulx was diagnosed with pulmonary hypertension at the age of 19. She experienced difficulty breathing and chest pain, leading to a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), a condition caused by blood clots in the lungs. Tina eventually underwent transplant and celebrated eight years post-transplant, surpassing the average lifespan post-transplant. Tina emphasizes the importance of advocating for oneself in the healthcare system and encourages others to trust their instincts and fight for their needs.

My name is Tina Proulx. I’m originally from Sudbury, Ontario, which is a northern city, but I’ve been living in Ottawa, Ontario, Canada for quite a while now. I’ve built roots here. I call this my home now.

I used to have pulmonary hypertension. I had it for many, many, many years. I was actually diagnosed at a very young age. I was diagnosed at the age of 19. It all actually started when I was living in Toronto with my boyfriend, who is now my husband, but I was living in Toronto. We were going to college together. I noticed that my walk back and forth to school was getting a lot more difficult to do. I thought that was weird, given the fact that I do it every day. I thought it’s got to get easier in some way.

It struck me as being odd, and I was a little concerned. So I brought it up with my husband, and the amazing person that he is, and quite the joker, he thought perhaps I was just out of shape, and decided to take me for a jog. I could easily tell you that that jog was an epic fail. I barely made it out of the parking lot of our building, when I told him I couldn’t, I was completely out of breath.

At that point in time, I had decided, “Well, first, I’ll take the first step and I’ll talk to my family doctor,” who sent me for a chest X-ray and diagnosed me with a mild case of asthma, which I’m aware of a lot of PH patients get diagnosed with asthma to start off with, so I’m not very uncommon there.

He sent me off with some puffers and a few medications to manage asthma. It didn’t take very long, I was probably on that medication for a few months before I realized that, “This is not working. In fact, I feel a little bit worse.” I brought it up with my family doctor, once again, who told me, “Okay, well, if it’s not working, stop.”

As a young person, I didn’t really know too much about advocating for yourself in the healthcare world. I probably should have pushed the issue a little bit more, but he just took me off the medications and didn’t look further into it. I walked away from that, just thinking that I probably had a mild case of asthma and that was it.

One moment, in particular, that was the kickstart to a lot of stuff was quite a few months after that when I woke up in the middle of the night with some serious chest pain. I told my husband I couldn’t breathe. I couldn’t lie flat, I couldn’t lie back down. It was very scary for me. I had told my husband, “I think I need to go to the hospital,” and he agreed, and so we went that night.

After several few tests that night, I think they called my lungs to be over-inflated, and so that seemed very strange to them, and so they decided to refer me to a respirologist. He’s the one who thought out of the box, and I’m very happy that he did, because he was the one who actually decided to send me for a heart echo, in which they were able to determine that I did, in fact, have pulmonary hypertension.

He, however, did not believe that I had primary hypertension, which is what we called it at the time. He thought that it was secondary to something. He started looking more into it. He had the impression that it was probably due to mesothelioma, a cancer, which struck me as being very odd, given the fact that I had never been exposed to asbestos, and it’s called the asbestos cancer. He really did think that that’s what it was, and so he decided to send me to see a surgeon where they could do a biopsy of my lung in order to confirm this theory of his. I was very fortunate that the surgeon had his own theories on what was going on, and before he decided to do a very invasive surgery, he decided that he wanted to send me for one test, which was a CT scan with dye.

Lo and behold, they found hundreds of little micro clots, is what they call them. They filled up my lungs entirely, which is the reason why I couldn’t breathe and the reason why I had pulmonary hypertension, and so it was him, at this point in time, who had finally diagnosed me with my full diagnosis of chronic thrombolic pulmonary hypertension. I always say that it’s a mouthful to say.

Chronic thrombolic pulmonary hypertension or CTEPH, what we like to call it for short, it’s a version of pulmonary hypertension in which blood clots are in the veins and the arteries of the lungs, and are causing blockage, which is making it very difficult for the blood to easily flow through, forcing the heart to work a lot harder than what it was designed to do. It also makes it very difficult to push oxygen throughout the rest of the body. I always said that pulmonary hypertension or CTEPH is a lung disease that affects the heart, and so, essentially, my lungs were slowly killing my heart.

As far as the PTE surgery, at that point in time, it was not something that I was being evaluated for. They wanted to first manage the blood clotting issues. They had no idea where the blood clots were coming from, so that was a whole other mystery. The PTE surgery didn’t come into play until later on.

At that point in time, they, immediately, once they discovered that I had this, sent me down to the thrombosis clinic, in which they were able to put me on blood thinner treatment in order to, at least, manage it from worsening or for any other blood clots to form and make their way. Unfortunately, that took years for us to finally come to a treatment that worked well for me. Many emergency hospital visits where I got new clots that came to my lungs, which made it even harder and even worse. So it took quite a bit of time before they finally found a treatment that actually worked for me as far as blood thinners go.

The PTE surgery did finally come into play. I was evaluated more than once, and the reason why I did not qualify was because my clots were baby clots, and so they were very, very deep into my lungs, and almost impossible to get to. On top of that, I was told that even if they were capable of getting the clots, because they had been there for such a long time, there was so much scar tissue that had built around it, that it probably wouldn’t have helped me as far as my pulmonary hypertension went, because there was a lot of scar tissue there that would still have caused blockages and still would have caused some issues for my heart.

I’m actually very fortunate. I was pretty stable for a very long time, aside from them trying to figure out my blood thinning treatment. I didn’t try too many treatments as far as pulmonary hypertension goes, because there weren’t a whole lot that were approved here in Canada for CTEPH, in particular. Ao there weren’t a whole lot of options for me.

As far as like what it was like as a young person, obviously, very overwhelming, to the point that once I was officially diagnosed, and once I graduated from college, and I do got to say, my boyfriend, at the time, who is now my husband, we’ve been together for a very long time, we’re high school sweethearts, and so he’s been through it all with me, he was the sweetest person in the world, starting at a very young age, because he actually quit school, so that he could help me finish my schooling, because he knew it was getting very difficult for me.

I finally graduated. We decided that it was probably best, because we were young, to move back home with our parents, so we could figure stuff out, which is what we did. We stayed there for a few years until we came to a point where we’re like, “Okay, this is our life. I’m stable. We need to start living, and start learning how to live with pulmonary hypertension.” Not always the easiest thing but we, certainly, weren’t going to let pulmonary hypertension get in our way of still achieving the kind of life that we were hoping for ourselves. Right?

That’s when we decided to move to Ottawa. Yes. It was very overwhelming, but, at the same time, I always say that everything that I’ve been through made me the person that I am today. I’m very proud of that person. I think, obviously, I would have preferred not to go through that, but, at the same time, it made me a stronger and a better person.

When you live with a chronic illness for such a long time, you’re accustomed to not feeling all that great, and so you just push on, which is pretty much what I was doing until it was my husband and my mother who had told me that I didn’t seem right, that I seemed like I was a little worse. So it was under their direction that they had suggested I go see my hematologist, because in the past, the thing that had always been the issue was the blood clotting issue, and so I thought that going to see my hematologist would be the best bet.

It was her, who had sent me for a few tests. She had discovered in doing those tests that my heart was actually much larger than it was from the last test, and that it seemed like the pulmonary hypertension had gotten worse. She immediately contacted my PH team here in Ottawa, and I was able to see him shortly after.

They started to get the ball rolling with regards to being able to figure out what was going on, how could they help me, and so, at that point in time, I was told that my lung pressures were at 125, at this point. They told me that, at this point, it was a progressive disease, and that I was getting worse, and that my only hope for survival, at that point, was a double lung transplant.

People always ask me what it was like to be told that. Honestly, it’s very overwhelming. t’s even hard to describe but you feel very helpless, because, at this point in time, you’ve reached the point where there’s nothing that you can do in order to help this situation. You can’t eat healthy. You can’t do any exercise. There’s really nothing more that you can do. You have to go for the surgery, if you want to survive. It’s a very emotional period, but I would say the best way to describe it is I felt very helpless.

In any case, I’ve always considered myself to be a fighter, and I certainly was not going to stop at that point. Of course, when they asked me if I wanted to pursue going through with transplant, the answer was a hard yes, because I was not ready to die. I think it’s important to note that when you’re waiting for transplant, you have to be close to the hospital. There’s no center here in Ottawa that performs lung transplants, and so we actually had to move to Toronto, back where it all started, in order to wait for transplant.

When you’re already dealing with so much, you have to deal with this additional thing where you have to find a place to stay and you have to figure out how you’re going to pay for that place, because we both took leaves of absence from work, unpaid. There’s a lot of the additional stresses that come with transplant. Just thought I’d mention that.

While waiting for transplant, obviously, as it’s a progressive disease, I got worse, so it came to the point in November of 2015 where it was no longer safe for me to be out of hospital. At this point in time, my husband was pushing me in a wheelchair everywhere. I couldn’t tie my own shoes. I always say if I dropped something on the ground, it stayed there, so our house was a little messy here and there just because I couldn’t bend over, because I would be at-risk of passing out if I did that. Sleeping was becoming beyond uncomfortable. Everything. I was beyond uncomfortable.

I know my husband had said, “Near the end,” which means near the end before transplant. My husband said that my heart was beating so fast and so hard, that he could hear it when he was at the other end of the room. Just to paint a picture as to how bad it actually was. I was not in a good place at this point.

The doctors had decided that it was best to admit me, obviously, and what they did was they wanted to initiate what they called Plan B. Plan B, they had mentioned, to me, when I got listed, that it might be something that they would have to do. I was hoping we wouldn’t have to, but, unfortunately, we did. They had to hook me up to ECMO, which is an artificial lung. They said it was important to do this, because my heart needed a break.

I was under the impression that it was going to go through the groin or in the neck, but my doctor said not for me. What they had to do was they actually had to hook up the ECMO directly to my heart, and so that required open heart surgery. So the ECMO was pretty much attached to my heart, and then I had a giant bandage here while the machine was attached to me. I was on that machine for six days when they finally came into my room to tell me that they had found a match for me. I wasn’t on the machine for very long, but it was still quite the experience, and because of that, I always say I have two scars, one that goes horizontal and one that goes vertical. A T for Tina as I say. I may have an additional scar, one that I wasn’t expecting, but both these scars saved my life, and so I keep them on me and I’m very proud of them.

I just actually celebrated eight years post-transplant, which, to me, is quite the achievement. When I was first told that I needed a lung transplant, they had told me that the average lifespan post-transplant is five years. Now I believe they tell people eight years, which is great. We’re moving up. Hitting eight years, to me, is like a milestone, because I know that that’s what they tell people now. I would like to continue, year after year, hitting these milestones, because, to me, it’s just a representation of the years, the days, the hours that I never would have had had I not had a donor. To me, it’s very, very important to recognize those moments.

I also just celebrated my 40th birthday, which, again, to me, very, very exciting whereas a lot of people, they may be scared of that number but, for me, it was something to rejoice in, because I didn’t even know whether or not I would see that birthday. To me, it’s like I thrive in growing old, because I never knew that I would be able to, and so every year that I get older is just something exciting for me.

Since my transplant, I can easily say that I’ve been able to do so much more, even so much more than what I was able to do when I had pulmonary hypertension and I was stable. I’ve been able to do something that I thought I would never be able to do, because when I was diagnosed with pulmonary hypertension, I was told I wasn’t allowed. I love rollercoasters. So post-transplant, I was finally able to go to Wonderland, which is a theme park in Toronto. I was finally able to ride the rollercoasters again. I’m not going to lie, I bawled the entire time. They were happy tears, but it’s like I celebrate in those little moments, those moments that so many people take for granted, and don’t realize how lucky and how precious those moments are.

I’m also an adventurous person. I am extremely happy that I’ve been able to let that adventurous side of me out. Since I’ve had my transplant, I’ve walked the CN Tower, the Edge Walk of the CN Tower, I’ve been ziplining, I’ve been white water rafting. I’m trying to convince my husband into going skydiving but we’ll see. I’ve always been that kind of person, and so, to me, it’s a very exciting thing to be able to live up to that little girl in me that always wanted to do those things.

Now my husband and I, in our spare time, we run the Ottawa Gift of Life, which is an advocacy group here in Ottawa, in which we advocate for organ and tissue donation. We run that in our spare time. I do a lot of charity work outside of that. I’m part of Ontario Health patient and family advisory board. I’m doing my best in order to give back. I think it’s important to take the time, and help others. I believe I was always that kind of person, but I think going through something like this only emphasizes that person in you, and that person just wants to come out. Right? I so desperately just want to be able to help as many people as I possibly can.

When I look back, and if I could tell my younger self something, I think it would be if you’re unhappy with something, then just speak your truth. Just say what you need to say, because nobody knows you better than you. If something is wrong, then make sure you trust your gut and you fight for that. Advocating in the healthcare system is so important. I think many patients struggle with that. Don’t be scared. Speak your truth and fight for the things that your gut is telling you, because too many people ignore that gut feeling and sometimes that gut feeling is actually telling you something that you need to know.

My name is Tina Proulx, and I’m aware that I am rare.

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