I'm Aware That I'm Rare: the phaware® podcast

Former pulmonary hypertension patient, Sarah Sizemore discusses life 1 year post-lung transplant surgery.

My name is Sarah Sizemore. I'm 22 now, and I live in South Carolina. My connection with pulmonary hypertension is I was diagnosed with idiopathic pulmonary hypertension since age four until I was transplanted on October 31st of 2022. I was on IV Remodulin. I was on several oral pills for pulmonary hypertension. My symptoms started to become unmanageable, and I was maxed down on all therapies. My PH specialist said the next option is really lung transplant, as there wasn't really much to do anymore, as I was already on so many aggressive therapies. So it was kind of time. Around early 2021 is when I really started to decline. So we started that process. 

The process for a lung transplant was really fairly easy and quick, as I was declining so rapidly. So the MUSC transplant team tried to make things go very quickly. I think within two months, I was listed, and then I received my double lung transplant, I think, a month and a half on being on the UNOS list for organs. I think the decision for me was pretty quick. I would take it as if there's any slim chance that I could have a life without pulmonary hypertension, no matter how risky a transplant is, to me, that was a risk worth taking. As I've had pulmonary hypertension in my entire life, there was this option that could provide a life without PH, and I really wanted to pursue it. It's not an easy journey. It's difficult. I mean, it's a major surgery. 

When I would talk to post-transplant patients before I got my transplant, they describe it as trading one illness for another. It's kind of like that, just because of all the risks that come with transplant. But every single person that I talked to pre-transplant said that they would do it again in a heartbeat, just because the amount of life that they were able to live post-transplant. That really made the decision for me. So when I was told that it was time for a lung transplant, my immediate decision was that I wanted to pursue it, just due to the fact that I could have a life without pulmonary hypertension.

My recovery was fairly easy and smooth. I was out of the hospital within two weeks. I really didn't have any complications. It was a big surgery, so there was some hard recovery. But I really didn't have any complications. We thought I was going to need ECMO due to how severe my pulmonary hypertension was. I ended up not needing that at all. I was breathing on my own within two days post and then walking like within three days post, and then, I was discharged at two weeks. Lung transplant patients have to go through pulmonary rehab and physical therapy post-transplant due to just how big of a surgery it is. It takes a toll on your body.

I've had PH my whole life, so I had to relearn how to breathe. I was used to taking short, shallow breaths, but with new lungs, you need to expand them and really take deep breaths. So pulmonary rehab really works on breathing and helping you adjust to exercising with these new lungs. With new lungs, you really have to exercise every day to keep them in shape and keep them physically fit and open. So it's pulmonary rehab. You do it for three months post-op, but I haven't stopped doing pulmonary rehab since my transplant due to just some complications. I love it. It's a controlled environment. Everyone knows me, and they really care about me.

Then, around March, I ended up getting COVID. I had that for seven weeks due to not having an immune system. We have to be on immunosuppressants so we don't reject our lungs. So I had COVID for seven weeks. I really didn't get sick at all. My lungs were just that strong to handle it, but unfortunately, the risk with getting an illness is your body wants to fight it. So it gives antibodies, but that can also wake up your lungs and your body, and your body say, "Well, these aren't my lungs." So I unfortunately went into acute rejection in May and respiratory failure, having rejection due to the COVID. That caused some scarring and damage, just to being such a traumatic event. So that damage is unfortunately permanent, and so I will need another transplant soon due to the scarring. But transplant has changed my life in just 10 months, so I would do it again in a heartbeat.

With having COVID, it decreased my lung function, which is 36%, and so that unfortunately isn't going to get any higher, just due to the scarring and damages. I have some new conditions with these new lungs. It's not a problem with the pulmonary arteries anymore, like with pulmonary hypertension. It's now with the vessels and the airways. So that has caused scarring and damage to that. That is what's causing low lung function. It's just only going to get worse over time. My function has to drop a little bit more to be listed for a second lung transplant, just due to UNOS's rules and everything.

One year post, the journey has been incredible. It's been a year full of getting to experience things that I've never been able to, like swimming, because I was always on IV. I was running five miles a day, just so many things. I mean, and breathing, I felt what it feels like to be able to breathe normally, which was huge. Just so many things that you never think you would get excited to experience. I've fallen in love with exercise and actually being able to do that. So, I mean, each month has been something new that I've discovered I can do. This transplant has just been life-changing. 

October 31st will be a year. Then the year ahead is probably a second transplant, which I'm completely fine with, as I would do it again in a heartbeat. It's just another chance to get to experience those things all over again, which I'm looking forward to.

So I have a golden retriever, Hudson. He's two. I got him around the time when things started to decline. He has completely changed my life. I mean, dogs can save people's lives, and he definitely saved mine. Those years were rough, and then going into transplant, he was such a comfort and everything. He's a golden retriever, so he has so much energy. He loves playing ball and swimming. He made sure to take me out and make sure I got exercise to keep strong for transplant. Now, post-transplant, I've gotten to take him on big adventures that we've never been able to do, which is such a blessing. I call him my furry soulmate. I mean, he's my soul dog. He's my whole life. I mean, I couldn't imagine doing this journey without him. He loves life and loves adventures. 

The advice I would give is to trust your instincts. I know it's terrifying, and it's scary. I mean, I felt all of those things. It's such a big journey, but each second and minute that you get to experience a life without whatever disease you have, is so worth all of the hard milestones.

The biggest thing for me was getting to carry on my donor's legacy in life. Now, I am living for myself and my donor. They have given me this chance, and that's the biggest thing. Then, just talk to other transplant patients that have gone through it, because that was the biggest support for me, was just speaking to other transplant patients. They gave me tips for surgery, how to make it comfortable post-transplant. They gave me so many tips that now, being out, I was like, "Wow. That really helped."

Really take care of yourself, too, because it is a big journey. You want to be strong mentally, really mentally and physically, but mostly mentally, because it is a big journey. Just take care of yourself. Prioritize yourself. Take time for yourself to process and everything. Trust your medical team. It's scary, but they do what they do best. I was really at peace when I had my transplant, because I trusted my team. I was just so ready to have a second chance at life. I think that's the biggest thing I would say. 

My name is Sarah Sizemore, and I am aware that I'm rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

Former pulmonary hypertension patient, Sarah Sizemore discusses life 1 year post-lung transplant surgery.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

Kristina Kudelko, MD, is the Director of Education and Stanford's Vera Moulton Wall Center. She discusses the 23rd annual #RaceAgainstPH, how it raises awareness and funds for this devastating rare disease and the importance of PH Courage Award winners. 

Don't miss the 23rd Annual Race Against PH at November 5 hosted by the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford. Click here to learn more. #raceagainstph @phaatstanford

My name is Kristina Kudelko and I’m a clinical professor of medicine at Stanford University. I specialize in pulmonary hypertension and therefore I have a very strong relationship with the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford.

I’ve been here since 2008 when I started my fellowship in pulmonary vascular disease, and I stayed on as faculty until now. I’ve been able to work with pulmonary hypertension patients and the Wall Center for all of those years. I am also the director of education at the Vera Moulton Wall Center and a member of the Steering Committee. And I really love my job!

Today, I am here to talk about the Race Against PH, which is a race that was started by a very devoted patient and her family member all the way back in 2001 to raise awareness and funds for this very devastating, progressive, and rare disease.

I’ve been attending the race every year and actually participating in the run of the race every year since 2008. It’s one of the highlights of my year every year just to be able to interact with PH patients, providers, drug suppliers, researchers. It’s a place where we all come together and celebrate patients who have this disease, celebrate the progress they’ve made, and remember those that we have lost to this disease.

This year, it’s happening as it does every year, on the first Sunday in November, November 5th at 9:00 AM. Please remember if you decide to register to register at www.med.stanford.edu/raceagainstph. We are still taking registrations even at this late hour. If you decide to come to the race, we always like to remind people to please remember the daylight savings time change. It actually allows you an extra hour of sleep before you come and support our cause!

One aspect of the Race Against PH is that we get to devote the PH Courage Award to one pediatric patient and one adult patient who has lived with this disease. Once a year, the Vera Moulton Wall Center for Pulmonary Vascular Disease strives to acknowledge a patient with pulmonary hypertension who has demonstrated exceptional determination, honesty, and resolve in battling this disease. A patient who validates the journeys of hundreds of others by being truthful in his or her own journey, acknowledging the hardships, cherishing the poignant moments of hope, looking forward to the promise of therapies on the horizon. A patient who surfaces as a natural leader and mentor and inspires all of us to press on fighting, press on teaching, press on researching, press on healing. A patient who serves as a powerful beacon of optimism for fellow patients, families, clinicians and scientists. A true PH hero.

This is a relatively new award that was created a few years back. It was a way for us as providers and researchers to give back to patients very directly. We enjoy, every year, nominating a few wonderful patients who have not only showed their own resolve with battling the disease, but have been really active in the PH community and have served willingly as mentors with starting the new therapies that might be amid the future of PH patients recently diagnosed, joining support groups, being active online.

Of course, we could nominate several to hundreds of patients a year to probably receive this award, but we really enjoy selecting one to focus on and allowing that person to tell their story. We really do find that patients are inspired and humbled and appreciative to receive this award, acknowledging what they’ve had to deal with dealing with this disease. It’s really sort of a wonderful give and take between our community and the patient community in that way.

I will say it’s always a very happy event, the Race Against PH, even for families who have lost members of their family or friends to this disease. They show up every year. Sometimes they sing and dance and run the loudest, because they’re still very much supporting the memory of the loved one that they’ve lost to pulmonary hypertension. They feel even more empowered in some way to sort of keep the race going in order to promote more research, hopefully more future therapies and more options for patients who are facing pulmonary hypertension.

Of course, we try to do some fundraising by participating in the race every year, but I do think that the most important part of what the race gives is a sense of community to the patients, to the doctors, to the researchers, to the people who help us develop drugs that battle this disease. We’re all able to unite every first Sunday every year and come together and participate in something with a common cause of raising awareness and remembering people that we have lost, celebrating people who have done well, and kind of regrouping so that we all leave with the idea that we’re making progress in this field.

I’m so proud to be part of the Vera Moulton Wall Center for Pulmonary Vascular Disease, which sponsors this race every year. Even through a pandemic, they were able to set up a virtual Race Against PH. We’re really committed to the cause and to the sense of community that this event provides.

I’m also forever grateful to the Ewing family whose generosity and innovation were so key in developing the event at the start. The Ewing family is here every year to celebrate their family member that they have lost, but also to carry the tradition forward.

So if you are in the Stanford area on Sunday, November 5th and feel inspired to come and support the Wall Center and the Race Against PH, please do so. All are welcome. You can register now at www.med.stanford.edu/raceagainstph. We’d love to see you there.

My name is Kristina Kudelko and I’m aware that my patients are rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

Kristina Kudelko, MD, is the Director of Education and Stanford's Vera Moulton Wall Center. She discusses the 23rd annual #RaceAgainstPH, how it raises awareness and funds for this devastating rare disease and the importance of PH Courage Award winners. 

Don't miss the 23rd Annual Race Against PH at November 5 hosted by the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford. Click here to learn more. #raceagainstph @phaatstanford

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

Pulmonary hypertension patient, Steve Smith discusses the importance of a good support team and how he tackled anxiety and his passion for musical theater.

My name is Steve Smith. I'm from Sterling, Colorado, a really small, rural community in the northeast corner of the state. I was diagnosed with PAH, (pulmonary arterial hypertension), in 2018. The process of the diagnosis took a long time. I think I was really frustrated and searching for answers as much as five years earlier. I would tell my doctor, "Something is not right," test after test after test. Finally, I got referred to a cardiologist. Long story short, they found a hole in my heart that had probably been there since childhood, or even birth. We don't know. It really, really explained a lot about the things that happened to me over the course of my life that I was never really sure about. By finding that hole, they discovered that my right heart chamber was considerably larger than the left, and it had just pumped very, very strenuously through most of my life. As a result, it was weakening.

They determined that the lungs had really done a number as well. That was a life-changing time. To understand, "Wow, this is what's been wrong with me for not just five, not just 10 years, but markedly, through most of my life. Granted, when I was younger, it was not as much of a problem. I did experience shortness of breath quite a bit. I was a cop. I was in law enforcement for 13 or 14 years, just outside of Tampa, Florida, in Hillsborough County. We had physicals, and I was able to run and do the physical things that were required, but sometimes they would fail me because they said my heart rate was too rapid. I said, "Well, I feel fine." "Well, your heart rate shouldn't be that rapid, running a mile." It was, and I never really understood why I had such a rapid heart rate.

Again, 2018, the lights came on, and it was all very, very clear. I've been going through treatment with various medications since 2018, some with success, some not so successful, and adjusting things. Right now, with regard to this condition, I think I'm managing it very well. I'm at a very, very good place with the disease. Some of the side effects of the medications bother me. Congestion is a huge one. It bothers me immensely, but I'm able to continue to work. I'm a college administrator at a community college here in Sterling. This is my 24th year in this position. I look to retire probably in a year and a half or so. So I'm glad I was able to continue to work as much as I wanted to, and to get involved in activities as much as I'm able to.

We moved to Colorado in 1997. We live in the plains. So the plains are not nearly the altitude that the mountains are, but we are two hours away from the mountains. We love going to the mountains. We absolutely do. I struggled in the mountains. There was a really vivid memory in my mind when I first felt like something was seriously, seriously wrong. I was with a group of men, a group of friends from my church. We were snowshoeing together up in Rocky Mountain National Park, in the winter. So we're high, it's cold. We're basically marching, because I've never been in snowshoes before. I felt like something was terribly wrong. I took my coat off, and I had to breathe really, really deeply. I got very, very afraid, because all those elements gave this condition a real workout that I was not expecting.

Walking, on a normal day in the community that I live in, where it's flat, I wasn't experiencing those things. At that point, I just attributed it to altitude. But I knew something was wrong. When I went to my doctor at the time, we talked and he did some heart tests, simple things. He said, "There's no indication that there's anything wrong with your heart." But he hadn't done those in-depth tests that a cardiologist did, to discover exactly what was wrong. He was looking at my vitals. My oxygen was good. My heart rate at that time, during the test, was normal. It was a regular beat. They weren't finding anything. But yeah, it was that trip up into the mountains that made me realize, "I've got to get to the bottom of what's wrong."

It was a relief to have a diagnosis that was solid, and certain, a whole lot better than people saying, "Well, we can't find anything wrong." Or, "Maybe it's just anxiety." There was one time I was treated for what was believed to be asthma. "Well, maybe you just have asthma. You're struggling with breathing." So I used an asthma inhaler, not knowing I didn't need one, and it didn't really help. So I went through a lot of those things, where it was trial and error. In 2018, it was absolutely a relief to be told by my cardiologist, "You have PAH." And I said, "What is that?" And he said, "Pulmonary arterial hypertension." Again, "What is that?" He was sure of what it was. I was clueless.

What do we do? We go home and we Google. I thought, "Oh my gosh, what in the world is this?" Then I read about the medical treatments, and ultimately the fact that there's no cure, it's managed. So yeah, I got a little scared. My wife and I talked. I said, "I have a really good support system. I have a really good medical team. I'm just going to take this journey. If they try medication on me, and it doesn't work, I'm going to tell them, 'This isn't working. This is making it worse,' and we're just going to do it a day at a time."

I had a really, really good mindset. My attitude was really positive. I was still doing a lot. What happened with me, every time I ran or went up an incline, or like I said, hiked in the mountains, maybe was working out in the gym, I'd feel the heart beating really fast. My heartbeat and my breathing would get out of sync with each other. It really, really scared me. That's the condition that I kept experiencing. So when they gave me a diagnosis, I thought, "Well, good, it can be treated." I didn't panic as much, and have the fear factor hit me, when I did experience those things. I learned, "You're going to have to pull back. You can't run anymore."

I'm six feet, five inches tall. I don't take stairs one step at a time, I take them two at a time. Guess what? I need to slow down on the stairs. I need to take them one at a time. And if I get to that landing on the stairs that turns, and I have to catch my breath, I can do that. So by understanding what was happening with my body, and knowing what it was... I mean, I used to always fear I was having a heart attack, or I was just on the cusp of having a heart attack. Now, I don't feel like that's what's happening if I experience those sensations. But I also know what to do to prevent them. If I'm really having a bad day, I can take the elevator. But most of the time I want to say, "No, I want to take the stairs as long as I can."

I've always been interested in musical theater, whether it was modern musicals or old musicals from the '60s. I just really enjoyed musical theater. I think it started, seeing musicals on the big screen with my parents, growing up. My wife is a musician and she loves musicals. In our early years of marriage we’d go to some Broadway musicals together. We both really had a liking for musical theater. Never did I imagine myself to be a part of musical theater. I wasn't in musical theater at all in high school. It didn't really surface and show itself to me in my life until my adult years.

Many, many, many, many years ago, there was a local theater group, a community-based theater group in my area. It's pretty much run as a nonprofit. One of the first productions was Grease. Grease was one of my favorite musicals, productions, soundtracks, if you will, going back to high school with the musical on the screen. I auditioned for a specific role, the Teen Angel. He had one song that he sang, and that was it. I got that role. It was just a very small. I'm on stage. I sing, I'm off. So I didn't have lots of experience with acting, minimal dancing. Most of mine was always singing. I really enjoyed singing, and I enjoyed pop music.

Several years passed and I wasn't involved in other productions. The director decided she was going to do Mamma Mia! She decided she was going to do that in 2018, and she called me. Mamma Mia! has several adult male parts. She said, "Steve, I would like you to consider trying out for Mamma Mia!."

I didn't know the story of Mamma Mia!, but I knew the ABBA music. So I said, "Yeah, I like ABBA Music. I'll do it." I got a role in that. It was a pretty significant role. Well, that's right when I got my diagnosis. So I had been on medication for a little while, but I was still cautious. I didn't do a lot of dancing at that time, because I wasn't real sure what I was capable of doing. I pulled off Mamma Mia! pretty well, had a few scares. There was a time I was lifting something heavy, I felt that sensation in my heart and lungs again. And I thought, "Oh, I can't do that." I couldn't move real fast, off stage. I had to move slowly or I'd get short of breath. My medicine had not all kicked in yet, so sometimes my pulse rate would skyrocket to 120, 130.

I was very nervous during 2018, during that production. I was not comfortable with it, initially. Got through it, but again, told people, "That was really, really hard. I don't think I can do that again." The next year she did Something Rotten. My wife loves the music from Something Rotten. So I told her, "I'll try out for a very, very small part and Something Rotten." I got a small part, one song, maybe two, and that was it. The next year was COVID. We didn't do anything. The next year she did Chicago, I told my wife, "No, I'm not auditioning for anything. I'm not doing this anymore."

This year. She did Chitty Chitty Bang Bang. I ran into the director in town and she said, "Well, I hope you're going to audition for Chitty Chitty Bang Bang." I said, "I hadn't thought about it," but I said, "we'll see." I decided I would. Again, I said, "A small part," and I listed a few roles that I thought I would be good at. After it was cast, I got the cast sheet, and she had given me the lead! I thought, "I can't do this." I watched some YouTube clips, and I watched the movie and there was so much dancing. I said, "I cannot do all that dancing."

I talked to her. I said, "I believe I can sing the songs. Singing doesn't wear me out." But I said, "If I'm having to sing and dance, I can't do it." I confided in her about my PAH. I told her, "This is my condition. You won't know what it is, you can look it up. But it really does cause difficulty with breathing, fast heart rate, sometimes a feeling of suffocation. I told her, "I am being treated. I am being medicated. I am being monitored, and it's managed." I said, "I'll do this lead," but I said, "I don't know about the dancing." She said, "We will simplify the dance moves for you."

Little did I know the hardest thing in the production was memorizing all the line!. There was a lot of lines. I was on stage almost the entire first act, and scene after scene after scene. So throughout the course of the summer, I developed a little more confidence, but I was always nervous, nervous, nervous. I said, "What happens when these performances come? What if I have a bad night, where I don't feel good?" I talked to my treatment team and we did a six-minute test. I did it as fast as I could, and I passed it with flying colors. I did very well with that .They gave me their vote of confidence.

My medical team said, "Steve, you can do this. You can." My doctor told me, "Your regimen is one of the most successful that I'm dealing with right now in my practice. You are on the right drugs, you're keeping your weight down. Your diet is right. You're exercising." He says, "You're doing all those things we want our patients to do so they can do the things they really want to do. In your case, it's theater. You want to do this production. They've cast you in this production. You're capable of doing this production."

They gave me the vote of confidence. I approached the rehearsals, all summer long, with a sense of hope. I said, "I absolutely know I can pull this off. I can do this. They've modified the dances for me, the singing is in my range." Much of the musical, I was sitting in the car, pretending like I was driving. So a lot of it, I was sitting down while I was singing. So I really looked at every aspect of that.

Mid-September we performed it, first night was great, which gave me more confidence for the next night. Which was great. Then the third performance, and the fourth performance. So I was really, really, really pleased with the production, my ability to perform in the production, my accomplishments when it was done, and the vote of confidence that both the director gave me, and my support team gave me.

I do recognize that everybody's diagnosis is different. People's level of abilities are different. I'm not at a stage in life yet where I'm on oxygen. I may be one day, and that may limit my abilities. The direction my doctor often gave me was, "Do what you feel like you can do. If you feel like you can hike, hike." He told me one time, "Don't hike a trail with a super steep incline, because you know you can't do that. Read the trail guides, and find the easier trails to do."

I'm 61 years old, so I don't have to do what I did when I was 25. I can't. But I can do the things at 61 that I want to do right now. That's what I tell people who talk to me about their situations. "Do you think you can walk a block? Do you think you can walk around the block? Do you have to stop? It's okay to stop." There are days that are bad. There are days when I go down into the basement to do something at my house, and I come back up the stairs, and I struggle.

There are days that are not bad. I just have to realize, on those days that are not bad, do what I think I can do. And the days that are bad, it's still the same message. Do what I think I can do. It might not be as much as the good days, but what can I do? And do it. Sometimes I mow the lawn with no problem. Other times I mow the lawn, and it's difficult. I still mow the lawn. I don't say, "Well, I had a bad day mowing the lawn, therefore I'm not going to do it anymore." No. "I had a bad day mowing it, and I had to stop. And I had to sit down and I had to catch my breath. But that's not every day." So I keep doing it until I can't. When I know I can't do it anymore, I'll have to say, "Well, it was fun while I could."

About a year and a half, two years ago, I was dealing with anxiety about the condition, not depression, but anxiety. I was nervous about, "What comes next? What happens if I do get worse? What happens? What happens? What happens?" I was nervous about things that hadn't happened yet. I told this to my primary care physician. He said, "Steve, why don't you talk to somebody about that?" He recommended a therapy group. I'd never pursued therapy. And you know what? I found somebody whose specialty was helping people with transitions in life. He dealt with older generational people. I began to talk to him about the anxiety.

And you know what? If I hadn't done that, the year prior to Chitty Chitty Bang Bang, I might not have been able to do Chitty Chitty Bang Bang. But by the time Chitty Chitty Bang Bang came around, I'd gotten a real good grip on anxiety. How to deal with it, how to face it, and realized what it can and can't do to me. That's important to me.

My name is Steve Smith, and I'm aware that I'm rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

In this episode, pulmonary hypertension patient, Steve Smith discusses the importance of a good support team and how he tackled anxiety and his passion for musical theater.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

Pulmonary hypertension patient and Stanford's Race Against PH 2023 Adult PH Courage Award recipient, Kendra Meneghetti, discusses her lifelong battle with PH, the challenge of receiving a transplant during Covid, and how her band provides a unique outlet by releasing her trauma through music.

Don't miss the 23rd Annual Race Against PH at November 5 hosted by the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford. Click here to learn more. #raceagainstph @phaatstanford

I'm Kendra Meneghetti. I reside in Martinez, California. I was originally born in Mexico City. My mom is Oaxacan, so I speak both Spanish and English. My connection to PH is that I was actually diagnosed at one and a half by an amazing doctor named Howard Rosenfeld at Oakland Children's Hospital. He was the one who told my parents that it might be this rare disease. I was put on Flolan, which was still pretty experimental at the time. Dr. Hickman of the Hickman catheter was the one who placed my central line, so I was immediately started on IV therapy. My parents were mixing my medicine up until I was maybe 15 years old, and then I learned. I was on a bunch of oral meds as well on top of the IV meds. I actually got my heart lung transplant in September of 2021.

Definitely there's been changes over time. I started with the larger pump, we used to call it the “brick.” Flolan at the time in the '90s used to have to be on ice 24/7. I was on that pump for a very long time, and then I moved to the CRONO Five. It's a small pump. It was nice because it was much smaller. So as I was entering my teen years, I could put that one in my pocket and it was less noticeable. Then I went back to what we called the “brick.” I was on that large pump until my transplant. I was always on the IV meds. I tried a lot of different oral meds. I was on REVATIO pretty much my entire life, as well. Then Letairis, I started when I was about 17 maybe. Compared to now, Flolan had to be constantly on ice, which I giggle at the thought now just because I was this tiny little kid carrying around this chunky little bag. It was constantly cold and leaking from the ice.

There's been a lot of changes, so I feel very grateful and lucky to have been in that position. Growing up my parents and I were always one unit. I'm very grateful for their organization and consistency and their teaching and how important it is to mix on time. I was in a very, very lucky position with them and with my nurses and with my doctors, both in the pediatric field and then moving into the adult field, too. I started having a lot of syncope episodes in my early to late teens as well as episodes of supraventricular tachycardia. So the disease was progressing, which I knew. I knew that growing up. I knew that that would be a prevalent situation. I sort of came to terms with that. The syncope was definitely difficult, but around 16, we met with some doctors at Lucile Packard. We visited the transplant team. They said that this was something that may come to fruition in time. At that age it didn't, so I transferred completely to Stanford.

As a new patient, they ran all the preliminary testing and told me I was a good recipient for a transplant. I was put on the list on and off, because I've struggled with my weight all my life, not only from the right heart failure and the pulmonary hypertension, but my dad's side of the family is incredibly thin. So I had my genes working against me, as well. But in 2021, I finally did have my transplant. It was hard. I met amazing people though. The entire pulmonary hypertension team that monitored me while I was in the hospital for three months was incredible. I became really close with all my nurses.

Prior to being admitted into the hospital, I had a lung collapse. I was admitted to Stanford for about a week, and then was sent home for maybe 12 hours until I felt it collapse again. So it was very difficult in terms of both physically and mentally. The physical aspect being multiple chest tubes being placed and then having them fail and then having a new one put in and then just waiting three months in the hospital. But I feel very lucky and grateful that I have and had an excellent support system, both with my family, friends and the medical staff.

My husband told me something that stuck with me the night before the transplant. He said, "It's out of your hands now." That kind of calmed me down. I feel like I’d been fighting for a long time. I’d gotten a second chance, and at that point it was out of my hands and in the hands of the incredible surgeons and nurses who were able to give me that second chance on life.

It was also during sort of an uptick in COVID when I was staying in the hospital. So at the time, the visitor policy was changing every day. I had two people that were allowed to come in and out to see me. I had a lot of family and friends come to Stanford and wave to me from outside while I was on the seventh floor. That was just so special. My family came from Mexico just to wave to me from the ground. It still makes me want to cry today, but it was just so special and things like that made me feel both physically and mentally stronger. I’m just beyond grateful and thankful that I had such strong and empathetic and kind people around me to constantly build me up.

It seems cliche, but I always tell people that it's the very tiny things that I feel most grateful for. My absolute favorite thing is to be able to take a shower and then not have to change my central line site and clean it. Just taking a walk around the neighborhood without being in pain and short of breath and without worrying that I'm going to faint is beyond special. I sometimes just start to weep while I'm taking a walk around the block, because it's not something that I've ever really experienced before. I talk to my family and my husband a lot and my friends about how the world has gotten so much bigger, and I'm grateful for that every day, even when I'm in a bad mood, I still feel grateful that I get to experience being cranky, if that makes sense. It's vastly different. I don't think that I realized how difficult and how sick I was when I had PH. it wasn't until after the transplant where I realized how much pain I was in every day, how much fatigue I was actually feeling all the time.

Then, just looking at photos of myself when I had PH, like when the disease progressed pretty heavily is not shocking to me, but there's a distinct contrast to how I look now. I just feel very grateful, and again, it's a second chance on life. I always have to remind myself of that, but it really is the little things that I'm most grateful for. Another one is just being able to dance around the house and not being out of breath and being able to run up the stairs. Sometimes I just want to run up the stairs just because, so it's pretty incredible.

Growing up, I was sort of quiet about my pulmonary hypertension and my chronic illness, something that I never wanted to have define me. However, I wouldn't ever change it about myself, because it made me into who I was and who I am. I have met some incredible people and friends that I still talk to today from the age of seven to now we're both 30 who have PH. My journey was maybe a little bit different from some other people who were diagnosed in the sense that I was more reserved about it, but it doesn't make me any less grateful or proud to be somebody who had PH and somebody who has received a second chance at life with a transplant.

My reservation about telling everybody growing up not only had to do with the struggles of being a regular adolescent. Kids can be really mean. I grew up with obviously a large pump and always had to carry a purse. I had rashes from the Flolan. So, it didn't only come from that, but it came from just a place of wanting to protect my own personality and not have it define me. However, it has defined me in the sense that I am much more empathetic and I have a perspective that I'm grateful for in terms of showing up for people. I feel really lucky to have gained that strength from having PH and having a chronic illness. I've just really met some incredible people along the way.

My husband and I are actually part of a band, and we have a very talented bassist and drummer. We are a four piece band, and the band started when I still had had pulmonary hypertension, and we're still going post-transplant. We sing a lot about chronic illness and hospitals, both from the perspective of a patient and as a caregiver. It's a pretty unique outlet and it feels really good to be able to put our pain into songs as most musicians do. That has always been mostly really special to my husband. He has been one of my number one caregivers. It's something we do together and enjoy and a way that we can release our trauma through song and lyric. Shameless plug, our band is called Box Of Matches, and we are on Spotify. If you want to feel a little bit sad or maybe have a release of stress, you can listen to our music.

Another thing that I'm incredibly grateful for and I feel really beyond proud and I feel truly honored, is to be the 2023 Adult PH Courage Award winner for Stanford's Race Against PH. It's not something that I ever thought would happen to me and I truly feel honored. The Race Against PH is an event that takes place at Stanford. It's a 5K longstanding event for doctors, patients, such as myself and staff. It takes place on Sunday, November 5th at 9:00 AM at Stanford. Of course, community supporters and listeners, if you'd like to learn more or register for the race, you can do so at med.stanford.edu/raceagainstph.

My name is Kendra Meneghetti, and I'm aware that I'm rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

In this episode, pulmonary hypertension patient and Stanford's Race Against PH 2023 Adult PH Courage Award recipient, Kendra Meneghetti, discusses her lifelong battle with PH, the challenge of receiving a transplant during Covid, and how her band provides a unique outlet by releasing her trauma through music.

Don't miss the 23rd Annual Race Against PH at November 5 hosted by the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford. Click here to learn more. #raceagainstph @phaatstanford

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com 

Harrison "Hap" Farber, MD discusses PHenomenal Hope 2023.

Join renowned experts as well as rising young investigators and allied healthcare providers as they share original research.

This symposium offers a combination of oral presentations, expert panel discussions, and poster sessions in an environment that encourages collaboration and a deeper exploration of patient-centered research. 

This event takes place December 15, 2023 Omni Boston Hotel at the Seaport For more info and to register, visit: www.PAH2023.com

Hi everybody. I'm Hap Farber. I am a pulmonary hypertension doctor at Tufts Medical Center in Boston. I'm also the President and the Chairman of the Board of Team Phenomenal Hope. In both of those capacities, I would love to invite everybody to come to Boston on December 15th for the first annual Team Phenomenal Hope PAH Research Day.

 So what is it you ask? It's going to be a day with research presentations, posters, and other presentations of topics that are not usually covered in most symposia or are covered in most registries. For example, we will talk about diversity and inclusion in registries and clinical trials. Not only that, but how do we get people who are not usually included in either registries or clinical trials into said registries and clinical trials? We need strategies to diversify our field, because if you look, all of the data so far, and this has actually been published of PAH registries and clinical trials are overwhelmingly Caucasian. There's very little representation of any other ethnic groups, et cetera.

Other things are unmet needs for patients with pulmonary hypertension. This can be anything from an air conditioner to a way to get a ride to go to a transplant evaluation. These are not covered in most cases by any form of insurance. They're certainly not covered in any symposia you will ever listen to.

Other things we can talk about, which are covered to some degree, but we're going to delve into them, are substance abuse and PAH, especially with the meth epidemic on the West Coast and the cocaine epidemic on the East Coast and somewhere in between both of them. We're now running into people in Boston who have both. It turns out, I was reading something very interesting, I believe it was in the New York Times, that currently in the United States, drug abuse and drug fatalities are the leading cause of death for people under the age of 45, which is frightening if you think about it.

We're also going to talk about a hot topic at the moment that probably can't be talked about enough is what is exactly a disease modifying agent in PAH? What does it mean? Do we have one? Are we going to have some on the horizon? If we do, how do they work and what do we expect from them?

Other things would be clinical trial designs and improving data quality. I mean, obviously we're dealing with a disease entity in which the number of patients we can recruit is relatively small compared to other diseases. For example, you can do a cardiology trial and get 20,000 patients in a week. We can't get 20,000 patients in the whole history of PAH. So how do we use these patients, especially in the age of presumably sotatercept, to find clinical trials and ways to design clinical trials that will be meaningful and that will add to our drugs or other treatments that will improve the outcome of pulmonary hypertension?

The other parts of this that are important is this symposia is open not only to physicians, but also to ancillary people who are interested in PAH, such as nurses, nurse practitioners, respiratory therapists, medical students, and fellows.

Another sort of unique aspect of this symposium is the fact that each one of these subtopics will generate a white paper that hopefully will be published, or at least for sure will be kept as an archive, as a resource for everybody involved. Not just the people who were in the symposium, but anybody who wants to have access to that.

This symposium will take place on December 15th. It's at the Omni Hotel in the Seaport of Boston, so it's very close to the airport. It's a great location. The other thing of importance, is it takes place the day after the Tufts Symposium, so you can go to both of them. They're both in the same hotel.

If you go to sign up, there are multiple ways to sign up. One is you can go onto the Team Phenomenal Hope website, look under events, and it'll be there. If you sign up for the Tufts Symposium, at the bottom of your registration, there's a link to sign up for the Team Phenomenal Hope Symposium. Or you can go directly to www.PAH2023.com. 

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.global @teamphhope

In this episode, Harrison "Hap" Farber, MD discusses PHenomenal Hope 2023.

Join renowned experts as well as rising young investigators and allied healthcare providers as they share original research.

This symposium offers a combination of oral presentations, expert panel discussions, and poster sessions in an environment that encourages collaboration and a deeper exploration of patient-centered research. 

This even takes place December 15, 2023 Omni Boston Hotel at the Seaport

For more info and to register, visit: www.PAH2023.com

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.global @teamphhope

Canadian pulmonary hypertension care partner, Joan Gibson discusses her daughter Jane's path to diagnosis, decision to embrace surrogacy, and her road to recovery after two PH related strokes.

My name is Joan Gibson. I live in Ottawa. I became involved with pulmonary hypertension six and a half years ago when my daughter was diagnosed with idiopathic pulmonary arterial hypertension. PH is a disease we knew nothing about. I noticed in the summer that we were moving something heavy in her house. She was really out of breath. I suggested that she check with her doctor. So she went to check with her doctor. I asked, "Well, how did the appointment with the doctor go?" She said, "I went to an allergist, asthma doctor, and he gave me a puffer and it's no good, it doesn't do anything." I said, "Okay. Well, maybe we should go back to the doctor. There's something that's not right here." I said, "If you want, I can come with you." I went back with her. The doctor said, "Well, usually doctors have a plan A and a plan B, so maybe you could go back to the allergy doctor and say, Hey, plan A didn't work. What do you suggests for plan B?"

He said, "We'll do a few more tests. One of them will be a stress test." I went with her to have the stress test done. I was sitting in the waiting room and we waited and waited and waited. They weren't able to progress with the stress test too far because her pressures were too high. Basically, at that point in time, he determined that she had pulmonary arterial hypertension, even though there was no other indications other than the stress test and the results. He referred her to the clinic in Ottawa. They called us the next day. We were lucky in that it was diagnosed really quickly. So even though it was severe, her heart wasn't damaged too much at that point in time.

In the initial timeframe when we heard about it, this is the point where I bring my Kleenex, because even though it's been six and a half years, it was devastating. I remember we were waiting for Jane to come back from her final test, and the nurse was in the room with me and said, "We're pretty sure this is what it is." She started crying. I thought, "Whoa, if she's crying, then this is pretty bad," so I started crying. She had a daughter the same age. She knew a lot about the disease and the progression. It's progressive and it's chronic and there's no cure, so there's an end to it. It was pretty devastating actually. Then when we got it confirmed on Tuesday, by then the shock, I think it's the shock of it all.

We're basically a healthy family. We don't do interventions. We have a lot of Parkinson's on both sides of the family, so I'd been working with that group thinking, "Okay, this is our future. I know what it's like. I know how to handle it. I know what to expect," but this was like, wow. I never even heard about this. I knew enough not to go on Google, because there's just terrible things on there. We had really good support from the clinic. It was hard to hear the doctor very honest and open. When she talked with him she said, "I'd like to be a mom. Is that possible?" He said, "No, because it takes energy for a baby and you don't have energy, and you can't get pregnant because it's hard on your body, and you have to be around to bring up the child." That just tore at my heart.

It's been a hard journey, and sometimes I do well and sometimes I don't do well. She did really well for six and a half years on oral meds. Then, we had a bump in the journey a few months ago where her pressures were very high. The doctor wanted to have an aggressive treatment and recommended IV therapy. She's very smart, and she figured out how to use this pump and used these drugs. It was going pretty well, and then the drug was doing well. Her blood pressures were going down a lot in her heart, and she was feeling a bit better. Then, she had a stroke. She actually had a couple of strokes, that was devastating. The first stroke was very upsetting. The second stroke was devastating, because we knew what was ahead with the hospital visits and the treatments, and lack of treatment. Well, not lack, but the difficulty in treating, because there were four or five medical specialties involved with her who coordinated well, but it's difficult.

A lot of the messaging was, "Well, it's rare. We don't know. She has a complex case. We think this should work, but we don't have any data. We don't think this is a risk factor. We don't know why she had this stroke. We don't think the medicine and the IV is causing it." More really difficult times and information from the doctor. We said to the doctor, "Well, we'd never heard of strokes with people with PH." His response was, "Well, they don't usually live." It was hard to hear that information and realize how sick your daughter was because my approach for her first few years was just to pretend she was okay. She looked okay, and she was doing okay. I just figured there was going to be a cure before she wasn't okay. That approach didn't work too well. Now, we were dealing with she is sick and she's got a very serious disease, and they know a lot, but there's so much they don't know. Because it's interacting with other parts of her body, which I think is not uncommon with PH patients, I think there's other parts of their body that are sick at the same time. So all the interactions of the drugs and how the body's responding, plus everybody is individual, so her body responds in its own individual way. It's been really, really difficult.

We're three months from the last stroke. The last month has been good. There's no symptoms. So she's gaining more confidence in her body and in her ability to live, so we're gaining more confidence. I've been living with them to provide support for her and her husband and the baby. Now, I go home on the weekends, so that's good for them to have some independence. So that's the goal. Our new normal will be she's got PH, she's had strokes, but her meds are good and we're looking forward to another number of good times.

With the baby, she did the surrogacy journey. She wanted to have a baby. She and her husband thought about it, because it's difficult when you have a disease like this. Should you have a baby? Well, not just physically, but they thought about it a lot and they chose life. They decided yes, they would have the baby. We lined up behind that with all the risks that inherent with it. A baby's always something to be celebrated. He's a beautiful little boy. He's a year old now. He brought a lot of joy and love into their life, and they were really lucky.

Jane has a spiritual side. We have a spiritual side. I guess, part of the decision making was if the stars line up and it occurs, it's meant to be. The stars lined up really well. The surrogate that they found through word of mouth, which is really unusual, actually had the same name as my other grandchild and had a dog whose name was the same as my son's. I mean, they're just really little silly things, but they just give you, "This is interesting, these coincidences.” It worked out really well. The lady lives not far from where they live, so it's in the same city, and she was very open to having them involved in the pregnancy and still very supportive. She's an honorary aunt. She's the godmother. She has a family. I think her seven-year-old daughter was probably more excited about having this baby come than... Well, obviously Jane and Craig were the most excited, and then this little girl was just pretty excited about the baby. He's not little. He's big. He's a handful. He's a wonderful little guy. He's brought a lot of joy into our lives.

So as a caregiver, it's a bit of a funny role because I'm a mom and a grandma, and she has a wonderful husband who's a live-in caregiver. When she was first diagnosed, she had just moved out. I was dealing with empty nest syndrome, which was great, because I didn't want dependent kids. My kids are independent. My head was very happy with that. My heart was not quite as happy, but I knew she was in a good place. Then a couple of months later, she got diagnosed with idiopathic pulmonary arterial hypertension. Then I wanted to call her every morning just to make sure she was okay, but that's not really what you do with a daughter who's 26 years old and has a partner. Anyway, it wouldn't have been good for her confidence for me to be calling and checking in every day. So it's been a caregiver, but respecting that she's in a partnership. They got married a few months later. Even after her diagnosis, her partner said he didn't care. He just loved her to pieces and wanted to be married, so we loved him to pieces. It's been in support of them as a couple and because I want to respect that they are a couple.

So the caregiving roles weren't really so onerous except the heartstrings and going to the medical appointments. I'm happy to do that, especially when the information's good, but when the information's not good, it's really bad. It seems that when I go, it's bad information, so we've got this thing now, I'll go to the doctors where I'm not going to get such bad information, because it could be a bit superstitious. Is me being there, going to give bad information? My husband or her husband will go to the other ones.

After the baby was born, I noticed she was getting more tired, so I'm retired, so I would go out a little bit more often and just spend the afternoon just so that she get a little bit of rest. But I was thinking it was the baby and babies are tiring, but after she had the stroke, they couldn't manage on their own. She was in the hospital for I think about two weeks the first time, and they needed help at home with the baby and going into the hospital to visit her. It's pretty heavy duty. Then, after the second stroke, the same thing. Again, we were just starting to loosen up a little bit. They were getting a bit more confident and independent, but then the second stroke brought us back to actually before square one I think, because you know what you're getting into. It's very scary. There were more side effects from this stroke. Then, there's the little guy who, it broke my heart to have them separated for two weeks in the hospital the first time and a week and a half the second time. The little guy would go in to visit her a little bit, but there was COVID on her floor one time. So it's hard, as a caregiver, to see your daughter not being the mom she wants to be.

I came to be more involved with PHA Canada, because after a year or two of pretending things weren't happening, it didn't really work very well. I thought, "Well, maybe I'll throw myself into it, get more knowledgeable, know more people. Maybe that'll help me deal with the fears and the sadness about it." At that point in time, there was a campaign for UPTRAVI. It was a drug that Jane was already on and was covered for by her company, luckily, but lots of people weren't covered for it. Advocacy is something I had done with Parkinson's. I used to be involved with the Parkinson's Association. Plus, I worked in the federal government for 30 years and had worked in the Privy Council Office. I had a fairly good understanding of how governments worked and wasn't overwhelmed by the whole government stuff. It interested me.

PHA Canada had put together a great resource for us through a consulting service who would provide us with the information we needed to meet the MPPs to advocate for the access to UPTRAVI on the public formulary so everybody could have access to it. That was great. I felt like I was able to do something. I visited three MPPs, one with Jane, one with my son, because he had a different MPP. It's really important that you bring somebody in the MPP. MPP is a member of provincial parliament in Ontario. It's important to bring somebody who's actually in the constituency, then you get better traction. Then, we were able to go with some people in the PH support group to another MPP who we were able to make the link with. I think that, that helped, and I was able to engage my sisters who live in Southwestern Ontario to meet with their MPP, something that they really didn't feel comfortable with it, but because they loved Jane so much, they did it.

We moved the dial on that. It was approved on the formulary, so that was a great thing. I think because I became involved in that and was interested in it, I was able to help out a bit more with PHA Canada, then they asked, "Would we like to be an ambassador?" Jane wanted to be, but because she has PH and it's not always able to depend on her energy, she thought maybe if two of us did it together, we could be together ambassadors, but she doesn't need my help. She's very out there. She's involved in lots of different initiatives to try to support the PH community. She's a co-chair of the support group here in Ottawa. So it's been interesting being an ambassador for a year.

To be a support system for my loved one, I think the child is the part that gets me. I think you obviously don't want anybody to be involved in it, but if it was an older person like my husband, we've had a life, but for your child, it's just not the way things are supposed to be. My experience is specific to that. It's six and a half years and I still struggle, so it's just the way it is. In terms of suggestions, I think boundaries are okay. Sometimes I can't do everything I want to do. Sometimes it's okay to put your head in the sand and pretend it doesn't happen. It's okay to cry. It's okay not to be okay. It's okay not to have the answers. As I was mentioning earlier, I just don't go to some doctor's appointments, which is not like me, because I'm usually a pretty good patient advocate to go and take notes. People like me there with them, but I can't handle the information. It's okay for me to say, "No, I can't go to that appointment. You have somebody else."

I found that yoga's good for me. I find being around positive people is good for me. Sometimes part of my boundaries are, I can't be on that Facebook page. There's just too much going on there right now that breaks my heart. Not because people are mean or nasty, just because sad things are happening. So I can say, no at that point in time. I can't be with this group of friends because there's too much drama or there's too much whatever. I need to find a friend who's just positive about things. I have a friend who has a daughter with MS, so we have a common thing there that's another chronic progressive disease for which there's no cure, and her daughter's looking at surrogacy. So there's a lot of things that we can relate to each other on.

Therapy is good. It's hard to get these days. The psychologists are few and far between. I rely on some spiritual direction. I find that, that's helpful, so sort of a bit of an inward calming. It's hard in terms of tips for caregivers, especially if you're a parent, it's just hard. When your new normal has been established and you're running smoothly, it's good. Then when you hit a bump, it's not good. Then, you just work that through and you know that you're going to come through on the other side again, and it'll be good for a while again. So you just hope for the positive. You celebrate the positive, and you find the joy in each moment. I heard that I think on the radio, I don't know, a couple of months back, and I thought, "Oh, for heaven's sakes, how can I be joyful? It's not joyful." Then, I thought, "Well, I can be joyful for a moment."

So I just focused on a bird, or I focused on the flower. I listened to something and thought, "Oh, that's joyful." Then it was kind of relieving to have a moment where I had joy. Then I didn't really want to get back to my sadness too much after that, so it was a good eye-opener for me. I think it was interesting that, that bit of information I paid attention to at that point in time, because sure, I've heard, "Look for the joy," many, many times, but for some reason at that point in time, it stuck in my head and it was helpful.

My name is Joan Gibson and I'm aware that my daughter is rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

Canadian pulmonary hypertension care partner, Joan Gibson discusses her daughter Jane's path to diagnosis, decision to embrace surrogacy, and her road to recovery after two PH related strokes.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

Pulmonary hypertension patient, Jo-Anne Mainwood, is a Canadian school teacher who couldn’t keep up with her students, friends and family. After a long journey of misdiagnosis, Jo-Anne details why PH is not a one-size-fits-all disease.

My name is Jo-Anne Mainwood. I'm from Ottawa. I've had diagnosed pulmonary arterial hypertension, the cause being idiopathic. I was diagnosed in 2009. About four or five years ago, it was my 40th birthday. I wanted to go and do the aerial park where you go up, climb the trees and swing across wonderful different obstacles. I could not keep up with my friends. I'd start climbing up the ladders, and I was just completely out of breath. I'm also a teacher of grades seven and eight. My classroom was on the second floor. So I'd be walking up the stairs and be completely out of breath. Grade seven and eight, that's 13, 14-year-olds would say, "Hey, miss, you okay? You a little out of shape there?" I was a little bit mortified, but I thought, "There's something going on here. This is not normal, I shouldn't be out of breath just walking to my classroom."

I could not keep up. It was embarrassing. I had two young children at home. It got to the point where I was so fatigued and so tired. I just didn't want to go out anymore. I just wanted to hide with the covers over my head, because when I did go out and when I did a lot of the things that I used to really enjoy, it was so difficult. I was breathless and embarrassed and just felt out of shape and not like myself.

It was a long journey getting to the point where I was actually diagnosed. I went to my family doctor. I had two children. I put on a bit of weight. And so it started with the usual, "Oh, you're out of shape, you're out of breath." Then, there was an asthma diagnosis and there were allergies that were diagnosed. Finally, when I just said, "No, there's something severely wrong," I got sent to see the asthma specialist, Dr. George Chandy, who was also a PH specialist coincidentally.

What's important to note is that he is a PH specialist, and he was my doctor who diagnosed asthma. He missed my PH at first. So it is really hard to diagnose. But once I really started telling him how debilitating simple things in life had become, he just took the bull by the horns and said, "We're going to give you every test. They're going to get progressive, and hopefully we won't have to go through all the different medical diagnoses, but let's get to the bottom of this." So he was absolutely amazing and fabulous. He solved the mystery.

First, I was hospitalized after doing one of the tests because it looked like blood clots on my lungs. But even then, they still were unsure and he pushed forward for another test. When he said, "You don't have blood clots," which to me was such a relief because I'd been injecting my stomach with blood thinners, and that was a horrendous experience. The first thing I said was, "Well, can I stop doing this?" "Yes,” he said. It was like, "Oh, okay. Well, it's pulmonary hypertension. I don't have to give myself needles." At first, I was almost relieved, because I just heard the word hypertension and thought, "Well, lots of people have hypertension. It's not a big deal." But once the disease was explained in detail to me and I looked it up on Google, I did not feel as reassured or good about the diagnosis. I think that's common for patients.

The journey is going okay. I was really lucky because I have excellent communication with my doctor and nurse at the pulmonary hypertension clinic. At one point, he had brought up that they were going to do a new trial, a stem cell study. I said, "Okay, sign me up." He said, "Well, you have to meet all these criteria. It's hard to get into." I was really, really lucky, because I did meet every single criteria for the study. I was able to become a participant in that study. I really feel that the stem cells helped me, because I felt better and had more stamina since I had the trial.

Well, first of all, with pulmonary hypertension, it's not a one-size-fits-all. It's also your treatment can work and work, and then suddenly it stops working. That was my experience. I was on a drug called Revatio, and my pressures had been under control. Then suddenly, it was getting higher and higher and higher. Dr. Chandy had said, "I don't really like the direction this is going." I said, "Do whatever. I'm game, let's take whatever treatment." When he brought up and explained the stem cell study for me, I said, "That sounds perfect," because you're using your own body's resources. They take your blood, then they take it to a lab, they alter it, they create the stem cells out of your own blood and then inject it back into you. It was a really good treatment, because it was nothing foreign. It was actually my own cells that were working to repair some of the damage from pulmonary hypertension.

In Ottawa, we have an incredible group of patients and caregivers. We do have a very active Ottawa support group. We do a lot of activities together. We talk regularly. Through the support group, people knew what I was going through. There was another member of the support group that was also eventually getting the stem cells. Then one person I just talked to in clinic one day, because the clinic days in Ottawa happen on Fridays. So usually if you're in the waiting room, chances are you're with somebody with pulmonary hypertension. We just struck up in a conversation one day.

My husband is my biggest support. He is just amazing. He's been through this journey. My husband and I have known each other since we were in grade one and two. We've grown up together and he's my biggest support. Aside from that, having people that understand what I'm going through and the fatigue. When you're an atypical breather in a world of typical breathers, if your cardiac output is less than everybody else's, or your heart's working extra to do what other people just take for granted or do normally, it can be a little isolating or make you feel like you're always catching up, you're always behind the eight ball. It's really nice in the support group because there are people in there that understand exactly what it's like and have similar experiences that we can laugh about and joke about. It's a real comfort level to be amongst, I'd joke and call them my people.

When I was originally diagnosed, they recommended that I stop working. But I said that it's part of my personality to teach, and I wasn't ready to stop. So for the past 14 years, I'm still going through adolescence and teaching my grade sevens and eights, but next year will be my last year. I'm ready to retire. I don't shy away. If anybody asks me about my disease, I am right out there. I think education is power. I've met with strangers that I've been introduced to through families or friends who are having similar symptoms just to see if they fit into the box. I'm always going to advocate for this disease, and I hope that someday we do get a cure.

My name is Jo-Anne Mainwood, and I'm aware that I'm rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

Pulmonary hypertension patient, Jo-Anne Mainwood, is a Canadian school teacher who couldn’t keep up with her students, friends and family. After a long journey of misdiagnosis, Jo-Anne details why PH is not a one-size-fits-all disease.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

On the 14th anniversary of her son's life-saving lung transplant, Canadian pulmonary hypertension care partner, Jennifer Gendron discusses how the PH landscape has changed over the past 20 years and life post-surgery.

My name is Jennifer Gendron and I'm from a small town in New Brunswick, Canada. My oldest son was diagnosed with pulmonary hypertension back in 2003 when he was five years old. As a lot of people's stories go, there were some subtle hints sort of leading up to it. Nothing seemed too out of the ordinary. We thought he had asthma. Just sort of out of the blue things progressed really quickly and we got this diagnosis sort of out of nowhere. It felt like, that he had severe pulmonary hypertension and that there was really not a lot that they could do.

20 years ago, things were certainly looked a little bit different in the pulmonary hypertension world than they do now, even less medications and treatments available. He started on oral therapies and quickly ended up on Flolan. He sort of never really did great. He stabilized for a little bit and then he would get worse. We were thrust pretty quickly, I would say, as PH goes into looking at transplant options. He ended up at the age of 11 having a double lung transplant back in 2009, so 14 years ago.

Back in that day. So one of the first things that I immediately tried to do was find some other people, connect, learn a little bit more about the disease. There was really not much happening here in Canada. I live on the East Coast and I found a very active support group on the West Coast, BCPHS. It was led at the time by Liz McCall. It was the most active group here in Canada and literally on the opposite end of the country. There had been the Pulmonary Hypertension Society of Canada at one point, which had become pretty much inactive by the time that Braden was diagnosed. I found PHA in the US and got myself on a plane and got to a conference down there, met some really incredible people and started to make some connections with people that were from Canada that were also looking for support, that had started little pockets of support groups throughout the country. I got myself connected with that group, and it wasn't too many years after that that we were able to come together and form what is now PHA Canada, that just celebrated 15 years unbelievably as an association.

To me that was one of the biggest, most important things was to connect with other people and learn as much as we could about the disease, find the support that we could not only for our son but for the rest of our family. I had two other young boys that were three at the time, twin boys, that when their brother was diagnosed and certainly it's a disease that impacts the entire family and one that is very complicated to manage. Just talking with other people and connecting with other families that were dealing with this similar journey was certainly, I feel, very important.

It's interesting because over the years I'd often have people say to me... So he was diagnosed in '03, so he lived with the disease for six years before we ended up in on the transplant list. People would say, "Well, he have a lung transplant, wouldn't that fix it?" It sounds like a fix, but you're certainly trading off one set of problems for another and a lot of unknowns. As much as it sounded like this miracle cure, it's not something we wanted to rush into. We also don't live anywhere near a transplant center. Being from rural New Brunswick, the closest transplant center for us is Toronto, Ontario. So 18 hours from where we live, and we had to physically move to that area before Braden could be listed.

We also weren't sure if that was really something that he would be up for. He was 11 years old. He was very frail. Would he survive a transplant? Was a transplant the right decision? Was he up for the long process of the recovery and all of those things, because he was still a child, but he was getting to that age where how much do we involve him in the decision? How much of this decision is ours? At one point, he wasn't responding well to treatment. We had gone through the transplant process, the workup leading up to it, tons of testing, and then they got him on Flolan and he stabilized a little bit. So that kind of got parked. It was almost a relief like, okay, we don't have to deal with this right now. We don't have to think about this. Because it was a really tough decision and a decision that affected us all.

Unfortunately, he stabilized only briefly on the Flolan and so we got faced with the whole transplant scenario again very quickly. We went up and we had all of the testing done again. It wasn't just him that was testing, we all had to meet with the psychological team to make sure we were all capable of going through the process or that they deemed us to be capable of going through the process. We had to, as I say, physically commit to moving our family. That was a big decision, because at the time, again, I had two other young children at this point they were nine years old. We, I guess, decided really early on as a family that if we were going to do this and we were going to go down this path, that we were all going to do it together. So we weren't going to separate our family, because we had no idea how long we would have to be in Toronto waiting for transplant.

My husband was an RCMP officer at the time, he took a leave of absence from his job. I owned my own business. I hired someone to run it. We were still sort of on the fence if it was the right decision to put him through the surgery. I remember we were sitting in Toronto in a restaurant, my mother-in-law had come up to meet us. We had been just going through several days of tests. This was a boy who his two younger brothers played sports, hockey, which he was not able to do. But also, always, "Oh, hockey is so stupid, I'd never want to play a game like that." He was a video gamer, that was his passion. We were sitting in the restaurant having lunch and my phone rang and it was the hospital in Toronto. And they said, "We just wanted to let you know that your son, we've gotten all of his tests back and he is a candidate, we would be willing to move forward and list him for transplant." He said, "Who's on the phone?" I told him and I told him what they said and he said, "Yes." I said, "Really?" He goes, "This is great. I'm finally going to be able to play hockey." And that was that.

I had to leave the table and excuse myself and go to the washroom and lock myself in a stall and cry my eyes out for a few minutes and get it together. But I realized then, okay, this is what we're doing. This is what he wants and we're going for it. We came home and found somebody that would look after our house and packed up whatever belongings we thought we would need and got in a U-haul and drove to Toronto with our two dogs, our three kids and our worldly goods and waited. We really had no idea the wait list at that time... Well, the wait list is always... You just don't know how long you're going to be there. We didn't know if it was going to be months or a year. We arrived in Toronto at the first part of August and got a call on September 23rd, he got the call for his transplant.

Right up until they took him through the doors of that OR I kept thinking, we should just go. We should just go. We know what we have here. We know what we're dealing with. I know what his quality of life is now. We should just turn on and leave. What if he doesn't make it through this surgery? It was horrible. But he was so determined at that point that this was going to be and that he was going to have this new life, we were like there's no turning back now. But yeah, I think of all the highs and lows in this whole journey, that was one of the absolute toughest moments is just not turning around and running out the doors with him.

He's been very fortunate in terms of transplant and how well he has responded. There have been very few setbacks over the years. Early on, he did experience some chronic rejection right at the very beginning, which was really scary, because we weren't sure where that was going to lead us. I had seen and heard a lot of stories where people just chronically rejected and had to go back for a second transplant. That was fairly early on, probably six months out, he ended up with some chronic rejection and having to have high doses of steroids. You have a preteen on IV steroids, who's in this absolute emotional rollercoaster.

But after that, and when they got him stabilized, he really has done very well. We were back home in New Brunswick, he was transplant in September, we moved home in April and we technically could have come a little sooner, but I was really hesitant to leave Toronto. I felt like we were safe there and we were close to the hospital. So moving back home, I found really hard. I mean, my kids couldn't wait to get home. But it was something that I was wrestling with in that what are we going to do? What if things go badly? We had gotten used to traveling back and forth over the years. The closest PH center is also in Toronto. I was used to running back and forth to Toronto with him a couple times a year for follow-ups. But there was just something about being right there and being close to the hospital.

But we came back home and he did really well. We would go back to Toronto a couple times a year initially, and then once a year. Then the years just kept ticking by and he kept getting older. Then my new fear became, oh my God, we're getting close to the point where he's got to leave pediatric world and we got to go down the street to the adult hospital, and yikes. That's a really great problem to have when you are in this world. But that brought a whole new set of challenges with it again, because as an adult they don't want to talk to your mom and dad. They want to talk to you as the patient. As an 18, 19 year old kid, we were always the ones who were the spokesperson and the advocate for him. It was really challenging. At that point he was almost in denial about anything. He's 18 years old, he's invincible. I don't need to worry about all this stuff. I don't want to talk about it. I don't want to talk about my medications.

So we went through quite a rocky transition time, I would say, for a couple of years where he was just not as compliant as he should have been. We were trying to be the bridge, but it got to the point where the hospital didn't want to talk to us anymore. That was a challenging time. I mean, he's 25 years old now and it's gotten much better. That was probably one of the hardest times in just managing his illness, was having to turn it more over into his hands and trust that he was going to do the right things.

I never thought we would get this far. I think back to these times, well, initially when he was diagnosed, we were diagnosed in a hospital that had no experience with PH. It was basically take him home and enjoy the time you have left. We really never thought we would reach the milestones that we've reached. As he got sicker, we got to make the most of every day, because I don't know how many days we're going to have. I never thought we would get to this point. Even at the transplant point, it's like, are we going to get through this? Are we going to come out the other side of this? Am I just dropping my son... Is this the last time I'm going to see my son, as I wheel him to the OR? You just kind of live with that in the back of your mind every day.

Even now, people will say to me, "So everything's great. He's fine now." I'll be like, "Well, I mean, yeah, he's really good right now." And that's awesome. But you just don't know what's around the next corner and it's a hard thing when you really sit with it to process. But again, I mean, I just try and look at it as none of us know. He's living with something that he knows he is living with every day and none of us knows what's next in life. I just watch the things that he's been able to do. That day that in the restaurant where he is like, "Yeah, I'm going to play hockey." I was like, "Okay." And he had the transplant in September and another young fellow had a transplant around the same time, and they had a bit of a competition going, who was going to do the best in rehab and who was going to bike the longest? He used that and I started watching him.

Then he would start asking the doctor every appointment, "So can I go play hockey now?" I'm talking October. The guy still has staples in his chest... And they'd be like, "Oh, no, no, not right now." I'm like, oh God... And so every time we would go he would ask this question. So in January we'd go to the hospital and he does the same thing, "All right, so can I start to play hockey now?" And the doctor says, "Well, do you think you can find a league that has no contact?" He goes, "Oh yeah, yeah, yeah." He said, "I don't see why not." I just about fell off my chair. He's like, "All right." So the next thing I know we're sitting white knuckled in the rink watching him try and stand up on skates. He didn't even remember that he used to know how to skate, he hadn't skated since he was five years old. He was 11 and they put him on a team with, I think they were seven-year-olds with this amazing coach who was like, "We're going to get this kid skating."

He had a ball and he just went from there. I would be panicking about him wanting to do something and he would look at me and say, "Mom, I didn't go through all this just so I could keep sitting home watching everybody else do things." I was like, "How do you argue with that? All right, get out there and do what makes you happy, because that's why we did what we did."

So my name is Jennifer Gendron, and I am aware that my son Braden is rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

On the 14th anniversary of her son's life-saving lung transplant, Canadian pulmonary hypertension care partner, Jennifer Gendron discusses how the PH landscape has changed over the past 20 years and life post-surgery.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

Canadian pulmonary hypertension care partner, Don Downey 

My name's Don Downey. I'm from Calgary, Alberta. My wife, Kathy, was diagnosed with idiopathic pulmonary hypertension in March of 2020. It actually goes back way before that. She started having problems in December of 2015. We'd gone many times to her general practitioner. He sent her to specialists. She went to an internist, who basically told her she had asthma. That didn't really work. She tried puffers and all that type of stuff. Nothing of that worked. She was then sent to a respirologist, one of the top ones in the city. It basically boiled down to she was told to lose some weight, and then, hopefully, that would make things better.

Over the next couple of years, she'd do an annual visit with him. It wasn't getting better. He just kept saying during the one year she had lost like 60 pounds over the course of the year, it was still "Just lose some more weight" and everything. In February of 2020, she went to the emergency room because she was having numbness in her arms. She was talking to the ER doc. He was going through her case file and all the tests that had been done over the years and everything else. There was one that he was looking at. She asked him if she could have a copy of it, and she sent it to me. I was down in San Diego visiting a friend who's a forensic pathologist, so a doctor. She sent it to me and said, "Well, can you ask him if there's any questions I should ask this ER doc?" I asked him, and he looked at it, and I think he spent about 30 seconds, and he said, "Yeah, can you ask him why you have pulmonary hypertension?" Actually, on the report from this test that they had done two and a half years prior, September 2017, it said "pulmonary hypertension?"

Nothing had happened in between. Nobody had noted that and thought to test her for it. She asked the ER doc, and he got a whole bank of tests done right away. From there, it ended up she was sent back and ended up going back to the same internist who told her again, "I think it's just asthma," even though we already had a good idea it was PH. The following week her regular checkup with the respirologist. She had to do a stress test before it. She got about halfway through the stress test, and she just couldn't go any further. 

Then we went upstairs. Normally, we'd be sitting in the waiting area for a while, and then they put us into one of the exam rooms, which was across from his office. We would be sitting in the exam room. We could see across. He was working on his computer, finishing off whatever the previous patient was. On this day, he met us at the door. We didn't even do the waiting room. As soon as we got up there, it was right into his office. So we knew there was something weird going on. Very quickly, he had us booked in with the PH clinic in Calgary. She did her right heart catheterization and everything else.

She had her right heart catheter on Thursday, March 12th, 2020, and then we went back for the results on the 13th. So, Friday the 13th of March, which was the day that the world shut down, was the day that she was diagnosed. We had a lot of uncertainty and a lot of things where what's going to go on, because even they were trying to figure out. All of a sudden, you've got COVID. Everything's locked down. How do you go about doing all the visits and everything else that normally would happen? We were lucky enough to have a doctor that, on the one day, it took a little bit longer because he was doing stuff. So it took a few extra days before he was really able to do stuff where we would've normally done trips to the clinic. But there was one day where he spent an hour and a half on a Zoom call with us just to make sure that we were ready for it and all. 

We, at the time, didn't look back. We looked forward. But as we've gone along, we've looked back at things that might have been missed. There was this disconnect because the clinic was still trying to figure out what they were going to do. The day that we got diagnosed, that was the last time we were in that clinic for a couple of months. Normally, you'd do half a dozen visits to the clinic. It took a little while for stuff and just getting the information. And, of course, the first thing he said as soon as he said, "You have PH," is, "Don't Google it." Of course, when you're not getting information, and they could only get the information out to you so quick because they're trying to do the transition -- so we Googled it.

You're looking at now we're in this situation where we've Googled it. It says that this is the average life expectancy. We know that we had two and a half years from when it should have, in our opinion, at the time because now we know that this was questioned at that time. It's like, "Okay, so we take two and a half off. Well, that's not a lot of time." So, that was the thing. It took a while because we had to wait for the clinic to be able to give us the actual information and give us stuff that worked for her situation. They tried to keep us out for as long as they could, so it was probably, I think it was about six weeks before we did one. But even that, it was a matter of you had to go through all the protocols to get in. You had to do everything that you were safe and all that because she had severe PH right from the start.

She was on high-flow oxygen within days of her diagnosis. So it was something where they wanted to put her in the hospital to be able to try to do stuff, and they said, "Well, it's not safe." So she didn't actually get to some of the treatment and some of the stuff that they wanted to do until the hospitals were opening up a little bit more, and they could do stuff.

One of the nice things is that we actually had two adult children that were living at home at the time. So we had them. Also, we've got a house, a two-story house with a walkout basement, and there's a basement suite. Her parents lived down there. The girl that my son was going out with at the time was also with us there. So there were seven of us in the house. It was a busy place. We figured out how to deal with things. I had to figure out how to deal with the oxygen and figuring out how to change cylinders and do that quickly so that she wasn't without oxygen for any periods of time. We did a lot of grocery delivery and that type of thing, so that I wasn't going out just because of the risk.

She's had a lot of ups and downs. She's been through different therapies that try something. Some of them have worked. She's on triple therapy. So far this year, since the beginning of January, she's had three different stints in the hospital. So she's probably spent about seven weeks total. She had a gastrointestinal bleed that nobody knows what it was, but the way that her numbers were for her hemoglobin back in January when this happened, they couldn't even do a scope to check. She can't be sedated or any of that. So she ended up one of her other times that she went in, they were able to do the scope. But they weren't able to figure it out, which is causing some issues with her potential for transplant.

Kathy's the type of person that she was actually not overly outspoken. She was the type that didn't want to have anybody mad at what she said and those types of things. Once she was diagnosed, and she started looking into things, and she was pointed out by the clinic to PHA Canada. Then, she got onto the Canadian friends' Facebook page and got talking to people there, it just exploded for her where she started wanting to know more, wanting to learn, wanting to help other people learn and that type of thing. She was a research librarian in the past, so of course, she's the type that, "I'm going to look into this. I want to do that."

We could see there were things, like the amount of time it took for her diagnosis is one of the big things for me for becoming an ambassador to try to advocate for better educational situations for doctors, for general practitioners, to know enough about PH to think of it a lot sooner. That was the big thing. It just morphed out of what she was doing. I'm along for the ride, but I'm loving it. The big thing is you literally go on a day-to-day basis, because the ups and downs can happen real quick. You hope that she can keep going for as long as possible, that she can get all the stuff figured out, get her weight down that little bit, and figure out this GI bleed, and be able to look at the transplant, which would be a major thing for her because her PH was already advanced enough by the time she was diagnosed to be problematic.

It almost puts it where that is the next outcome. She also has a hole in the heart. It's one that you have when you're born that normally closes up. Hers didn't. So there are certain therapies she can't take because of it. We know that, basically, the next thing is a transplant. So it's figuring out all of the things and hoping that that's going to happen. We both want to make as big of a mark on things to try to help so that people don't have to go through what we've gone through and so that we can get it towards finding a cure or finding better therapies that'll make things better.

I'm Don Downey, and I'm aware that my wife is rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

Canadian pulmonary hypertension care partner, Don Downey, discusses his wife Kathy's road to diagnosis -- which was confirmed on Friday March 13, 2020 -- the day the world shut down and how they navigated her new normal during lockdown.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

15 years ago, after the birth of her 2nd child, Canadian pulmonary hypertension patient, Carol Doyle Ploughman was told she had only two years to live. Now, a decade and a half later, on her birthday, Carol discusses how she has maintains a healthy and active lifestyle and the importance of clinical trials for PH patients.

My name is Carol Doyle Plowman. I live in Ontario, Canada and I was diagnosed about 15 years ago with pulmonary hypertension at Toronto General Hospital. I got diagnosed after the birth of my second child, but symptoms arose right after the first pregnancy. It wasn't diagnosed. What happened was I didn't do too well with the second pregnancy. I went into delivery early. I had my second child six weeks early. About a week after my second child was born, I went into heart failure. That's when they discovered I had pulmonary hypertension. 

It took several weeks after that just to confirm the diagnosis, because when I first went to a walk-in, they said that I should go to the ER. When I went to the ER, that doctor actually told me that my swelling was the natural part of motherhood and I should just go home and deal with it. I remember leaving there in tears and I said to my husband, "Something's not right. I don't care that he's a doctor. I need to figure out what's going on." Because at that point, I was barely walking. I had pins and needles in my legs. They were swollen to the point where I could barely walk because of the pain.

My family doctor referred me to a local internist. I was in his office five minutes when he had me running a bunch of tests. For a whole week, he ran tests and then sent me to a cardiologist who told me I had two years to live if I didn't get a lung transplant. The community we have is so very important because back then, there was so much misdiagnosis, or even if you got the correct diagnosis, the information that you got may not always have been accurate. So the fact that it's 15 years later and I don't have a lung transplant, while I am on triple medication to control the disease, I'm in a far better place than I imagine myself to be. You can only imagine how it feels. I had a four-year-old, a three-week-old, and I'm told that I'm not going to live more than two years unless I got a lung transplant.

At that point, a cardiologist referred me to Dr. Granton at Toronto General. A week later when my husband and I went down there, we had all these questions about lung transplants and he's like, "Whoa, whoa, whoa." He's like, "No, no, no, that's not where we're going. We're going to start you on medication." We kind of giggled because we thought lung transplant or nothing. Dr. Graham's like, "It's not funny." I'm like, "We're giggling because it’s a relief." They wanted to put me in the hospital because I had so much fluid backed up, but because I had the young baby at home. I didn't want that. So I said I would do whatever it took, just let me do it at home. I was on a heavy dose of diuretics. The first night, I lost seven pounds and within the first week I lost 36 pounds of fluid.

When I think back about 36 pounds, it's a huge amount of weight to lose in one week, but it was just that's how much fluid my body was retaining at that point. Dr. Granton and his team basically said, my second child is basically like an angel, because if they didn't come when they did, we may not have detected it and I could have died. At that point I was just so thankful, but I was also kind of angry because being told wrong information, and I felt that the cardiologist and the neurologist I saw before that ... because during those four years before the second pregnancy, I had seen a neurologist because they told me I had syncope and I needed to stop drinking coffee. There's just all this different things that they told me at that point. All the symptoms had another answer. It wasn't until I went into heart failure during the pregnancy that everything got figured out.

I did write an article about Dr. Browning who I felt saved my life, because he knew instantly what path to go up with the testing and if it wasn't for him, I don't know if I'd be here. That actually led to the Ontario Lung Association seeing the publication that I wrote about Dr. Browning. They asked me to do an interview for the doctors for Ontario, which I did because at that point I wanted to do whatever I could so the next person wouldn't have to go through any struggles of getting diagnosed. Not only did they run it in a commercial where I was in a commercial with other people for different reasons of dealing with the Ontario doctors, they actually posted a poster of me down around Toronto General Hospital on the subways and stuff. So at the end when they went into their next phase of doing a different interview, they actually sent me the poster, which I thought was pretty cool.

There was one point where my husband was away out of business and he was in a bar with clients, and he looked up because he is like, I hear my wife. The customer looked at him and he is like, "What do you mean?" He looked up and there I was on the TV. I like to think that that started helping people. So the first person I reached out to after Googling about pulmonary hypertension was a lady by the name of Loretta Chu. She had at that point just started a support group here in Toronto. She created the Toronto Chapter for Pulmonary Hypertension, back 15, 16 years ago. My first introduction to anyone else with the same disease was when I went to one of the support groups where Loretta was running it. It was quite the eyeopener. It's not a one size fits all disease, because while my symptoms are fluid retention and I have to take diuretics and watch my sodium. Not everyone has the same restrictions, or not everyone can take the same medications.

I started out with Revatio for the first year. They added Tracleer the year after that, and I was on Tracleer just until two months ago, because they added a third. We tried Adcirca. Adcirca did not work for me, I couldn't even get out of bed. So they switched Adcirca to Uptravi, which seems to be good for me, but they just recently, because of some symptoms that I've been having, switched it out to Ambrisentan. I've noticed a difference. Like I go walking with my husband, they usually have to push me up a hill, or I have to stop halfway up. I can't get to the top, or if I get to the top, I have to stop for a couple of minutes to catch my breath. Since they replace Tracleer with the Ambrisentan, I can get up to the top the hill without stopping. I don't stop even once I reach there. I still get winded and I can still feel it, but I don't have to stop to get it back to a normal beat.

The thing I realized it's always like baby steps. It's like I know I'm never going to ride a bike again. I've tried. I try once a year every year to test myself. The last time I did it, I passed out on it, on the sidewalk in front of my young child. It really scared them, so they asked me not to try it again, so I haven't since. But I try to find the things that I can do that will make me happier, different things I could do with my kids. I can't coach soccer, but I would always be on the sidelines watching. It's just about finding balance. I know a lot of people that have illnesses. It's like a lot of people say, I don't look sick, so I'm not sick, so they forget that you're going to do something, I can't do it. If you're going to go anywhere hiking, if there's big hills, I can't go, or I can go, but I can't climb that hill. It does impact it. It has changed friendships, but the important people are still there. My kids don't know any different. I'm very lucky that I have really good support system.

My husband picked up a lot of slack with the kids when they were younger, when they wanted to do biking and stuff like that. I try to be very optimistic, because as long as I wake up and I'm breathing, then there's something to live for. Maybe I say that because I am stable and I have been for a little while. I don't know if I think the same way, if I wasn't as stable as I am. I also make sure I follow all my restrictions and do whatever the doctor tells me I need to do.

My opinion is it's changed friendships because of the fact that I do have some limitations. So fluid restrictions, for example… I had some friends that like to drink, and maybe drink a little bit more than I can. For me to go out and have a drink or two, that means I'm going most of the day without any fluid or little fluid, and that fluid, of course, is always usually water or soda, water. Then, of course, alcohol is dehydrating, so it doesn't really play well with the fact that I have to take diuretics because of fluid retention. Then the next day, you tend to be thirsty. It's a hard thing to replenish. So I find some of those friends have gone by the wayside because I'm not able to go out and party like I'm 20 anymore. And you know what, I don't even know if I'd really want to. But again, some others would be ladies that I worked out with or did boot camps with. So different things that would make the friendships were broken because of the illness. So things changed, but there's also some friendships that I gain new friendships, or some friendships that actually made it through. I just believe that they were just true friends and they would be there regardless.

I'm actually at the point where I'm very lucky there's a lady in town who does classes and I go to twice a week and I do some interval training, which I got Dr. Granton to approve. I go at my own pace. A lot of it is chair. I know my sister-in-law does chair yoga for some people. We do stuff where we might do low weights sitting in a chair and not a lot of movements over my head. You just work it into what you're able to do instead of looking at what you cannot do.

For the first month, I detached from the baby because I didn't think I was going to be there. I didn't want the baby to get attached to me. My mother-in-law, my father-in-law pretty much, because my husband travels for work, they came to stay with me during the week and then my husband would be here on weekends. There is a bad side to the disease for sure. I just don't like to dwell on that part. I went into a bit of a dark place. I felt like I couldn't do anything, so I didn't. I didn't even try at the beginning. And it took probably a good two years. I don't know how I got the, I call it your wake-up, your aha moment, but I thought, I don't want this, I want to be able to do stuff.

So I reached out to a local lady because I saw a commercial for TRX, and I'm like, "Could I do that?" She's like, "Under the circumstances for your condition," she's like, "No." She's like, "But do you ever want to try personal training?" I'm like, "I never even thought of it." And I did. I contacted the doctor and I'm like, "am I able to try this?" Because right now I spent almost three years where I was doing nothing. I couldn't even pick something up in the grocery store from a lower shelf without holding onto a shelf to get back up, because you lose your muscle tone. You're not using your muscles and you're just sitting around all the time.

I think that was my aha moment, because when I started doing the personal trainer, I still remember the first squat I did was with a ball against a wall and I did two. I could probably do stand up and do 40 squats right now. So it's all a matter of, I try not to think any more about what I can't do. I tell my kids this all the time, you think about what you can do or what you want to do, and you try it. If you try it and you can't do it, that's great. I mean, it's not great, but at least you tried. But if you're not even trying, then how do you know? That goes for anything in life, not just people with an illness. How do you know what you're going to like or dislike, whether it be tasting different food, or travel, or any of it really. So I try to always tell them, look at the brighter side, be more optimistic and try different things.

At the beginning when the kids were younger, I did any type of trials. I remember the first trial I did, and I actually had something posted in the PH magazine about it. I did this trial and you don't know if you're on placebo or not. I'd still get the same feelings. You'd still get the fatigue, but you could do a little further. So I could walk a little further. I could last a little longer doing something. They canceled the study because they weren't getting enough positive feedback from, I guess, the people that were involved. I was devastated. Would I let it stop me from doing it again, no, but you need to be aware if you ever agree to do a study or anything like that, that there's pros and cons to it as well. I was not mentally prepared for them stopping it. They never tell you whether you're on the placebo or not. But I said to my husband, I'm like, "I know I wasn’t. There's no way I could have done that." Because when the drug went away and I'm back on my normal meds, I went back to the same where I was before the study.

I remember when I had my appointment with my doctor and I'm like, "You gave me candy and you took it away and I want my candy back." That's when they decided at that point, that's when they introduced me to the third medication, because it's like you showed me a little more, that I could move a little more, and I wanted it back. I felt so good at that point. I didn't want to not feel good anymore. So I'm like, "I want candy back." So they're like, "Okay, let's check it out."

I remember going in and doing a study. They were going to take a piece of muscle out of your leg. And I'm like, "Sure." Because Dr. Granton knows if there's a study that I qualify for, he just calls me and is like, "Are you interested?" And I'm like, "Yep." I still, to this day do that, because there's just a lot of factors that you just don't know. If they come out with new medication for it or other options for other things, then why not try? I don't lose anything from doing the volunteering, right? I have a little scar from them taking a chunk of muscle out of my leg. They actually had to do it twice, because the first time they didn't take a big enough piece. They're like, "Can we do it again?" I'm like, "Don't even ask me. Just do it and do it quick." I liked being able to do that. I haven't been asked to do anything in a study basis for a little while now, and I do miss it, because it always made me feel close to the community by doing these things.

I think the studies are important because we all want the same thing. Anyone with PH, we all want the same thing, we want a cure. So these studies help, not only with new medications, but hopefully the end result is figuring out enough from these studies that they maybe come up with this cure, because I hope to be alive for that day. Because right now, the only option we have is lung transplant, and that's not a cure because that comes with this whole gambit of things that you need to do, and medications you need to take at that point. So it'd be nice to think that there's going to be a future with a cure. I think it's doable. Because we're not all one and the same as far as symptoms and everything else, I think they need a huge array of people to do the volunteering so that they can collect as much information as they can.

My name is Carol Doyle Plowman, and I'm aware that I am rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada 

15 years ago, after the birth of her 2nd child, Canadian pulmonary hypertension patient, Carol Doyle Ploughman was told she had only two years to live. Now, a decade and a half later, on her birthday, Carol discusses how she has maintains a healthy and active lifestyle and the importance of clinical trials for PH patients.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us on social @phaware Engage for a cure: www.phaware.global/donate #phaware
Share your story: info@phaware.com @phacanada