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

I'm Aware That I'm Rare: the phaware® podcast is devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. Through this series of impactful, insightful and, most importantly, hopeful stories from members of the global pulmonary hypertension community, we hope to further the global #phaware conversation as well as to capture, engage and enable misdiagnosed and undiagnosed PH patients because early diagnosis and treatment can mean the difference between life and death. Topics range from the importance of early diagnosis and global PH awareness, to the impact of clinical trials to how patient support and advocacy are paramount to battling this disease. Learn more about PH at www.phaware.global

Drs. Kevin and Heather Shannon - phaware® interview 510 03/04/2025

Drs. Kevin and Heather Shannon - phaware® interview 510 Co-Founder and Board Member, Drs. Kevin and Heather Shannon share the inspiring story of — a transformative summer camp for children with heart disease. Hear how one patient’s journey ignited a movement to create a safe, empowering space where kids can embrace their scars, form lifelong friendships, and experience the joy of camp without limits. From the challenges of the first year to the life-changing moments that keep them going, the Shannons share how Camp del Corazon has touched countless lives and redefined what it means to live fully with a medical condition. The Shannon’s will be honored at on April 5th, 2025 at the Skirball Center. Get your tickets or participate in the online auction . #galadelsol #campdelcorazon #heartcamp #camplove Kevin Shannon, MD: My name is Kevin Shannon. I am a pediatric cardiologist and the co-founder and medical director of , a residential summer camp for children affected by heart disease. Heather Shannon, MD: I am Heather Shannon. I am also known as Dr. Heather Vandeweghe. I am a pediatrician, married to Kevin Shannon. I am now retired… happily. Kevin Shannon, MD: I finished my pediatric cardiology fellowship in 1993, and had an interest in treating abnormal heart rhythms, something called electrophysiology at the time. It was a very new subspecialty in pediatric cardiology. My boss happened to be the person who did that, Tom Klitzner, who became Director of Pediatric Cardiology in 1995. He was an electrophysiologist and had recruited me into his program. Around the time that I was finishing my fellowship, Tom had asked me to see a patient for him, and it was this patient who triggered the start of Camp del Corazon. It was a young man who had a fairly common valve abnormality, his version of it was fairly severe. He had deteriorated fairly rapidly from the age of about five to the age of about eight. At age five, he was pretty normal, played soccer, ran around, was in normal school. By age about eight, he had dropped out of school because he was so fatigued with any kind of exertion. He'd reached a point where he was being carried from his home to the car to go places. His doctor, at the time, was very old school and didn't believe in heart replacement therapy for children, because just a decade prior, the survival of that was very poor. He allowed this young man to get very limited, to the point that he developed arrhythmias. He got referred to my boss, who asked me to see him. I saw this young man and my first impression was, this is a bad valve and we're not going to make any progress in him until we fix the valve. But the parents clearly were resistant to that. So, we focused instead on the arrhythmia. We did a procedure called an ablation, where we eliminated his arrhythmia, where he happened to meet Lisa Knight, who was what we called the “arrhythmia nurse” at the time. She scheduled all of our procedures and then assisted with them. He did a little bit better with the arrhythmia. I saw him several times, and eventually convinced the parents he should have heart surgery and get this valve replaced. I had them meet with the surgeon and they finally agreed. He was in and out of the hospital in a week. He just flew through the surgery, because his heart was so much better having a functioning valve that the fact that he'd needed heart surgery and been on bypass was of no consequence to him. Then, every time I saw him after that, the parents were like, "Oh my gosh, he can do this now. Oh my gosh, he did this now." He had his surgery in January of 1994, and then in September of '94, he was back in school. He was playing soccer and was doing great. I'd literally been seeing him every few weeks, because I didn't have that many patients, and I was very worried about him. But then, come September, he was doing really well, so I didn't see him for a while. I saw him for his one-year follow-up in January. I went to listen to him and he wouldn't take his shirt off. Instead, he pulled his arms inside and just held the shirt out so I could reach up underneath and listen to him. I asked him, "Are you doing that because you're self-conscious about your scar?" Before he could answer, his mother said, "I haven't seen his scar since he was well enough to put his own shirt on." So, his own mother hadn't seen this scar in months. I asked, "Is that because kids at school tease you?" He said, "No, no one at school has ever seen it except my best friend, and he begged me to show it to him." I said, "So how did you change your clothes at school?" He proceeded to show me how he could change his clothes by putting one shirt on over the shirt he had and then pulling the previous shirt out through the neck hole, never exposing his chest. As I watched him go through this contortion to do this, I was kind of devastated, because I thought I'd done so much for this child and made him so normal. What I really did is I gave him a different problem that he saw every single day in the mirror and was just afraid to show anybody else, including his mother. That was a very discouraging event for me, because I was so proud of myself up until then, and then I realized, hey, I didn't make this child as normal as I thought. Around that time, I got an email from a listserv. Emails were brand new in 1993, and I had just signed up for this thing called PediHeart, where you would get email blasts from people who posted on it. I got this post from someone about a camp in Louisiana called Camp Bon Coeur. A lot of people responded to the post about how great the program was and how much it helped kids feel better about their scars. Most of the talk was about kids who get to see other kids with scars, they feel better about it. I wrote away for information. I set everything up for this kid to go to this camp. I had the local Mended Hearts to sponsor him, I had the application, I had everything all lined up, all I had to do was get the mother to fill it out and we would be on our way. They came to see me around April of that year, I had a big packet of stuff. I brought it to clinic and I was going to give it to them. I explained what it was and where it was, and the mother looked at me and she's like, "Louisiana's 1,500 miles from here!" There was no way she was going to send this child, who a year earlier, she'd been carrying from the car to the house. There was no way she'd let him go to Louisiana for a week. So, they didn't have any interest in it, didn't want any of the stuff. So, I had all this stuff and I brought it back to my office and put it on my desk. Lisa Knight saw it there when she came in to talk about scheduling patients, and I explained to her why I had it. She had a relationship with the people who own Catalina Island Camps, or the people who ran it. She looked at me and she said, "We can do that," and she picked up the phone. She called Tom and Maria Horner, who were the directors of Catalina Island Camps at the time, and told them we wanted to start a summer camp for children with heart disease, what did they think. They needed a community service component for their lease on Catalina. So, they were like, "Oh, yeah, we can do that. Let's do it over Labor Day," which was Labor Day of 1995. So, in April of '95, we were Camp Directors planning to go to the island in August. That was the start of what was a much more complicated process than I thought. But the short version is we were able to raise money asking vendors and people who sold products to the hospital, in particular, cardioplegia, because one of the surgeons got involved. We raised $15,000 and we arranged to take a boat to Catalina Island, use their site for three days, and then take it back. We recruited kids by going through the database at UCLA and finding children who were still alive and who definitely had heart disease, because I didn't want to invite anybody who'd lost a child and I didn't want to invite anybody who'd been told their child doesn't have a heart condition. The thing I remember most about that process was when we wrote the letter inviting people, we said, "There are two equally important goals of this program. The first is to allow children with heart conditions to experience something that other children take for granted, a residential summer camp experience. The second is to allow children with heart conditions to interact with peers, children who have the same experiences and concerns that they do." That was the birth of Camp del Corazon. Heather Shannon, MD: When he came home from work and mentioned this, I was just over six months pregnant and had a one and a half year old. I was like a deer in the headlights and said, "Sure, whatever." My father had started summer camps, mainly so he knew exactly where his children were going when I was younger, so I was not unfamiliar with being part of a family that started a camp and were Camp Directors, but really had no idea that we were stepping into such a lifelong process. The best part about it was that we were going to a campsite that was willing to support us in the sense that they kept all their counselors to do the activities, so we didn't have to find counselors, which was huge that first year. Basically, our job was just to raise enough money and then get the children from UCLA Medical Center on a bus to the dock, get them on the boat, get them through the boat ride to get to Catalina Island. Once there, the Directors of Catalina Island Camps pretty much took over and did a lot of the legwork. Those three months, four months, that we worked on it didn't seem that hard. Lisa Knight did most of the recruiting of campers and getting the information she needed, which, at the time, was a lot less than what we were require now. We obviously didn't realize the amount of work it takes to deliver all the medications for these kids through a three-day stay on the island, but we learned very, very quickly. Kevin Shannon, MD: There’s lots of serendipity with camp. The biggest one was obviously the first year, we had a site willing to give us staff, who knew how to do all the things that counselors have to do. To be honest, Tom and Maria Horner, every time we'd sit down and talk to them about philosophies of child raising and discipline and running camps and things, we are spot on on the exact same page with everything. Having them as partners in this was huge. The first year, we had no idea what we were getting into, to be honest. We knew we needed to make this safe. We had as much medical information as we could possibly gather about these kids. We actually took any medical person that was willing to come. We said, "Fine." We told them they could bring their kids, because at the end of the day, we only had 49 campers who got on the boat. We only had 84 who filled out applications. We knew we needed more than that number to have momentum in groups of kids. So, we let any staff that wanted to come with their kids that were in the age range. We put them in cabins with these other kids the first year. It was kind of a whirlwind from we'd get them all on the bus, we had to drive to Newport Harbor, because the only boat we could get was a charter out of Newport Harbor. We had to carry all the luggage three blocks from the bus. Then, that was '95, there was a hurricane off Hawaii, but it was sending 15-foot swells all the way to Santa Monica Beach, so we rode out in these huge swells and almost everybody got seasick. So, we got through those three days. Those are really, in all honesty, still a blur in my mind. My most silly memory is I remember wandering around between cabins late at night after everybody had gone to bed just listening to see if anybody was seizing, because I had in my brain, if anybody had a problem, the first thing they would do is seize and make noise. I don't know why. But nobody ever has had a problem at night, knock on wood. Heather Shannon, MD: After the first year, many friends of Lisa Knight came on as counselors. We realized that we had to start having our own counselors. The first year, which would've been the second year of camp, finding enough people was a little bit of a struggle. Catalina Islands Camps gave us some more staff, and we mustered through. Then, once word got out about their experiences, these counselors, we became inundated with people who wanted to be counselors at this camp. Counselors of other camps that had ended by Labor Day, they wanted to come. They wanted to be a part of it. It just flourished to the point where we started to have to have a real application process and a waiting list for counselors, and that has continued to this day. Fortunately, after about 10 years of camp, a lot of the campers grew to an age, because we require them to be over 20, where they could be counselors. That was a whole other process and place to get counselors. We've enjoyed that tremendously, to see these children grow up and become great people and do great things, which normally we envision them thinking that they can't do anything. Then, they come back and are counselors and such great support systems. So now, probably about 30% or 40% of our counselors are former campers of some sort. Kevin Shannon, MD: I've had many parents tell me, "The hardest thing I've ever done is watch my child get on that boat." Then they say, "But after they got back, I knew it was worth it." For parents, it's hard because this is a child that they've seen go through surgeries, that they've slept in the hospital at the bedside, that they've gotten permission slips and paperwork to the schools and not really let them out of their sight for very long. For about 70% of our campers, their first night at camp is their first night with anybody but a relative. So, it's a pretty big ask, and I tell our counselors all the time, the thing we market isn't a summer camp, it's trust. We ask these parents to give us their most prized possession, who's 1,000 times more fragile than anybody else's most prized possession, for four nights and five days, and to make sure that not only are they getting their medicine and they're being watched carefully, but that if something goes wrong, we're prepared. That's really what we want to let people know or feel. We don't market it in the sense we don't tell anybody that, but we try and give that impression and make sure people know that we do training. We find out as much as we need to know about their children. We have emergency action plans. What we want them to know really is their child's going to come and have a summer camp experience, it's not going to be about their heart disease. Heather Shannon, MD: As all children are, they're very conscientious about the scars and the marks and such, partly because I think it brings back memories of what they went through getting those scars. But we found at camp, there is no hiding scars. After about an hour or two, there are scar contests, there's by number how many scars you have, who has the worst scar, who has the biggest scar, et cetera, et cetera. Now, they enjoy that with the other counselors who have been campers prior. I think that they don't stare at each other's scars, because they are used to seeing those scars and such. It makes them feel very comfortable that they can be around other children and see that other people have these scars. I think many of these children think they're the only one in the world that has this scar and they haven't seen anybody else with it. That just puts them part of the group and children want to belong. I think the scar becomes a fairly large component of this whole process at camp. Kevin Shannon, MD: The first three days of camp were a whirlwind for me, I don't remember doing anything. I know I kayaked, I know I helped kids in and out of kayaks. I know that I had been a lifeguard. We had lifeguards, but I was on the waterfront a lot. I was wandering around a lot, just checking on things. We come back and I was exhausted. We got all the kids back to their parents. We, of course, had one child that the parents were four hours late. So, finally got him off with his parents, go home, get some sleep, and then Tuesday morning, I went back to work. I remember very distinctly, I got a call from a mom, she says, "Dr. Shannon, I just had to call and tell you that you wouldn't believe Carly when she came back! You brought back a different child. She was so excited about her heart disease and so excited about her scar and so excited about all the things she got to do. We can't wait for next year!" That was the first time it dawned on me that we were going to have to do this again. That kind of enthusiasm for “you brought back a different child,” really struck me. The next event like that I recall is I got an email from the mother of one of my patients. It was a very long, well-written email. It basically said, "Dear Dr. Shannon, I just want to let you know this summer camp was amazing. We sent you a child who was ashamed of his heart disease, who looked at the ground all of the time, who didn't see a future, didn't look forward to a future. You brought back a child who held his head up high, who is proud of what he's been through, who really saw a future and an exciting one. We can't thank you enough for doing that for our child. It's something we never could have done." That has been the raison d'etre of camp, is to have that kind of transformative change on children. There's lots of benefits to summer camps Even children without special conditions benefit from them. The biggest one probably being the friends that you make. For a lot of these kids, they feel a little bit outcast or so different from other children. They don't make friends easily, but they come to camp and they make friends that they then keep in touch with the rest of the year and they look forward to seeing every year when they come back. Heather Shannon, MD: I remember the first year, Kevin running to the ATM machine to get money out of our account to pay for the bus. I remember the buffalo walking through camp and everybody going, "Well, what do we do with a buffalo in camp? How do we get rid of it? It's awfully big." I'll have to say, most recently, the hurricane that came through, there was lots of parents very frightened about that, but we had a great time on the island. Rained a little bit, there were water streams and mud everywhere. We had an extra day on camp, so we had to come up with an activity for that rainy day and the counselors did the most amazing thing and I would say one of the best days at camp we had with the kids. The counselors are amazing. They make things happen. There's just been so many magical moments, things that we do every year, disco bingo. It's just something that is loved by everybody. We used to do something called a snipe hunt with the little kids, where they went out in the evening and we usually had Kevin and some other leadership were out there with two flashlights with red over the ends of it like the eyes of these little monsters. The kids would be screaming and such. Every year, there's always something special and fun to watch. Kevin Shannon, MD: From the very start, we had this recognition that if you have a child with heart disease, regardless of your financial situation, it's a huge burden. For many families, the vast majority, it's a huge financial burden. Even if you have insurance that covers all the medical costs, there's lost days at work, there's extra travel, there's nights in hotels, there's copays, there's all kinds of expenses and loss of income associated with it. The last thing we wanted to do is create any kind of financial burden for these parents, so from the get-go, it was free. The model for Camp Bon Coeur was you would get local Mended Hearts organizations to sponsor it, which for one kid, $1,500, our local Mended Hearts could do that pretty easily. $15,000 was a... /episode/index/show/phawarepodcast/id/35098600

Episode 510 - Drs. Kevin and Heather Shannon 03/03/2025

Jen Cueva - phaware® interview 509 02/25/2025

Jen Cueva - phaware® interview 509 Jen Cueva, a pulmonary hypertension patient of almost two decades, discusses her diagnosis, her advocacy work, and how she uses writing to support the pulmonary hypertension community. My name is Jen Cueva. I from San Diego. I am a pulmonary hypertension patient of almost two decades. Throughout my journey with pulmonary hypertension, I have met some incredible healthcare providers, friends and family and loved ones and others in the pulmonary hypertension community. Before I was diagnosed with pulmonary hypertension, I was a hospice nurse. But, unfortunately, when I was diagnosed for a variety of symptoms, no one had a clue about, and, obviously, I went to nursing school way back and I never heard of pulmonary hypertension. I think back when I was in school, there was maybe a sentence. It said the word pulmonary hypertension, but it never elaborated and it was never a focus or anything. So, of course, when I was diagnosed after having syncope episodes, fainting, I also was having fluid retention and I looked like, as my boss would say, the Michelin man. I was short of breath, had to crawl up my stairs every night to get to bed. Thankfully, I was diagnosed pretty rapidly compared to many people. I only waited a few months. Definitely, when I was diagnosed, my world was flipped upside down as well as my families, because I worked as a hospice nurse and I was a mom, a wife, a sister. I was the actual caretaker in the family and within my friend group. Now, I'm the patient. So, I struggled mentally and emotionally quite a bit with trying to ask for help. I know so many people out there do, but for me, it's still not easy. Today, I try to remind myself how I feel when I'm able to help someone, so just think of that. Also sometimes you just can't do it without help. For me, I thought this is not a "terminal diagnosis." It wasn't cancer like most people think or like end-stage anything. I thought originally like, "Oh, I'm going to just be in the hospital. They're going to fix me up. I'll get back home to my normal life." Unfortunately, that's not the case. But my hospice nurse experience has helped me emotionally and mentally and physically, because I have learned through the years how I educated families, loved ones, and my patients. I think it makes me be the person that's like, "I'm all about having to improve quality of life. Do what you're going to do. Enjoy life. Savor every moment. Create new memories," and things such as that. I definitely went online to research, which is not always the best, especially if it's unaccredited, because I was told by one doctor who actually said, "No, you don't have pulmonary hypertension, but it's good because it's a death sentence.” I was like, "Okay, you're fired. On to the next doctor." I found an incredible knowledgeable doctor to confirm yes, I do have pulmonary hypertension. No, I am not going to be gone in three to five years. There's no cure for this rare disease, but there are medications and treatments that can help the symptoms, and it did. I was grateful. I went home on oxygen, which I didn't want to wear out in public because I'm a young adult. I didn't want anyone to see me in my oxygen. I fought that oxygen for the longest time, but wearing it at night and then finding how much benefit I got of it in my body, I started to do it slowly. I've lost friends. I've gained so many other friends in the PH community and other areas, as well. The doctor that I found, he was a pulmonary hypertension specialist, which I always suggest and recommend for new patients if they're seeing their local pulmonary or cardiologist. Those doctors may not have ever worked with a pulmonary hypertension patient. They may have one or two, so they tell you that, yeah, they're knowledgeable. I also had that happen to me. The medication they wanted to put me on at that time, the doctor had no clue. He gave me a pamphlet and sent me home to research on my own and say, "You can schedule an appointment next month and we can start you on this IV medication." For me, as a nurse and an advocate already for my patients and everyone else, I had to advocate for myself and go to the next doctor. Sometimes that happens. I was very fortunate to find the PH specialist. They are well-trained, educated. They also know the newest treatments. They've treated thousands probably of pulmonary hypertension patients. Most of the time, their whole team's incredible, knowledgeable of the medications, what is needed. I always say it takes a village and my healthcare team is almost like a football team because without them I wouldn't be here today. We are the expert in our bodies, but we need that backup team as I say, the strong and knowledgeable healthcare team, such as a PH specialist. Not only just loved ones and family and friend support, we need it all. When I was diagnosed in 2005, of course, I thought I would be fixed up and get back to my patients and everyone. I was told otherwise. I was not allowed to go back to work. I was told I needed to file for disability. At my age, I thought that was the end of the world. I love nursing and caring for others. What am I going to do? Also, financially, my income was part of a two income family. Now we're down to one, not to think about the added cost of all these hospital visits and tests and medical expenses. It took a toll on me mentally. Of course, that affected me physically. I did go into a dark area at times and I just stayed closed off from the world for a little bit, which was very unlike me because I'm very talkative. I like to go and do things and help people enjoy life as we all should. I always thought I would get back to work in some type of capacity. I continue to have my nurse's license and do the upkeep with that, because I worked hard for that license. It was about a year off and on that I thought my life was over. I couldn't drive. That was another thing that took my independence away. I was on oxygen. I couldn't drive. It was hard for me to get to my daughter's events at school and things such as that. Sometimes, I missed those activities because I was in the hospital or I was sick. I couldn't be around the kids at school. That took a toll on me mentally as well, because of course I thought she went without, and it was because of my sickness, my diagnosis of pulmonary hypertension. So I felt guilty about that, as well. About a year later, I went online. I had heard about Pulmonary Hypertension Association from my PH team. I started volunteering with them. I was doing a lot of advocacy work. I would do writing if they needed anything such as that, share my experience, talk to other patients that were maybe new or isolated and felt alone and overwhelmed, of course, at diagnosis. I started to have a little bit more hope and thought, "Oh, well, I am doing something." At that time, I had limited energy and oxygen, because I was still on oxygen full time. After a while of that, I found , which is a part of , a company that focuses on 50 plus rare diseases. At Pulmonary Hypertension News, we had forums for patients and caregivers that I started going into. They reached out to me and said, "You're on here so much supporting people. We want to hire you. We want to give you a few hours a week. You can moderate the forums. You're in there nonstop anyway and supporting people." A few months after that, I had the opportunity to write for them, a column. I share my experience with pulmonary hypertension, the ups and the downs. At first I was determined I'm only going to share things that are fun and hopeful and not to bring people down, but then I'm like, "You know what? I'm not going to do that. That's a disservice to the PH community, because they really need to know the trials and tribulations and things we have to go through to know that, 'Oh, I'm not the only one going through this.'" In the forums and in my writing, my column at , for me, that was my way of nursing and giving back. I did a book, a collaboration of rare disease stories for Rare Disease Day 2024, that's called . The proceeds to that book went to a nonprofit that focuses on rare disease. I have worked on a new book, , that is a collection of 22 other featured writers and myself. We each wrote a chapter on our rare disease and how the rare disease has impacted our lives, because we all know no matter what rare disease you have, I feel like the people in the rare disease communities just come together. We just want to be this huge community of support, and if we can help someone else, it's all worth it. That book was released in November for PH Awareness month in 2024. I share a little bit of my experiences with pulmonary hypertension, but my main focus is my transition from a nurse to a patient and how that evolved through the years and some of the struggles and challenges that I face. Tou can go to Amazon and find that book, Positively Rare, as well as Kaleidoscope, the stories of the rare disease patients. At first, when I started writing, I thought that I would never have enough information to share. I'm like, "I'm not that creative. I just write." I journaled it a lot as a kid, so I have always written in some form. For me, if there's a certain challenge or triumph, I go through, I am like, "Oh, maybe I should write about it" so other people in the PH community would understand. And if it happens to them, they can relate and say, "Oh, I'm not the only person that's going through this. Oh, I'm not crazy as some people have told me." They will say, "Oh, you read my mind. I can agree with everything you just wrote here. It's so crazy that you understand what I'm dealing with." For me, if I can be that voice, that validation, to make sure that others in the community are heard, at the end of the day, that's all I'm going to do. Through my diagnosis, I was journaling every day about my symptoms and things for my doctors to keep up with, so I found writing. I didn't have time to do that when I was working and being a mom and working full time. Writing for me has been therapeutic, but I've also found that I was much stronger than I ever thought possible, because some days I was like, "I'm not going to get through this." So it's not the end of the world. I met people, they do crafts and arts, and they sell them online and to other people. Some do jewelry and different beadwork and things focused around pulmonary hypertension. That is therapeutic for them. They had never had the time to do that before, but they're like, "Oh, I'm crafty." Or they sew or cook more. I knew one lady that started her own business. She's like, "This is what I was born to do." So despite the pulmonary hypertension diagnosis thinking that life is over, there's so much more out there. No, I can't run. I can't do exercises I would love. I still use a wheelchair for distances, but I weaned myself off a full-time oxygen. I use it at night and as needed when I'm up walking around. I'm trying to get past that point, but we have to keep working. There's just so many little things like that. I've had more time to spend with my daughter as she was growing up. Now, she's a young adult. We have time to go on trips sometimes overnight. We can do that. Before, we couldn't. It's just mom and daughter time and that bonding time. So when you're diagnosed, I always say, "You need to allow yourself to feel all the emotions that you have. Just don't let them stick with you." One of my late PH friends would say, "You can sit on the pity pot, but make sure you get up and flush it and move along." That's stuck in my head. I have days where I think, "Oh, it's not a good day." I may be on the couch all day and there's plans that I had, but I know my body is telling me to listen to my body more. Unfortunately, I learned the hard way because I push and push until it just gives out. When those times happen, I can write more. I can email, check on others who have reached out and see if I can help them as they learn about this new diagnosis, or maybe they have had the diagnosis and just now decided to try to find others and learn more about it and how they can improve their symptoms and what things they can do versus, "Oh, it's the end of the world." That's my purpose. That's why I do it. As my husband says, "You can't save the world," but I can try. That's why I do what I do. My name is Jen Cueva, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/34400985

Episode 509 - Jen Cueva 02/24/2025

Stephen Gariepy - phaware® interview 508 02/18/2025

Stephen Gariepy - phaware® interview 508 Canadian resident, Stephen Gariepy, was diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) in 2018. After initially being active in senior hockey, his condition worsened, leading to difficulty breathing and performing daily tasks. Gariepy continues to live with this rare and terminal disease, adapting to the limitations it imposes on his daily life. My name’s Stephen Gariepy. I’m from Edmonton Alberta in Canada. I was first diagnosed with CTEPH in 2018. What had happened was I had just recently retired and was beginning to enjoy retired life. I had joined a senior hockey league and was very involved in that playing hockey three times a week. Of course, everything came crashing down with this diagnosis. Every September I’d go out and do some recreational skating and preparation for the season openers, in October. I had started my skating program. There was a large clock on one of the ends of the rink, and I always use that to guide how my times were doing. I was noting there was a little improvement. I’m going, “Wow, I must have really got out of shape this summer.” So I continued my skating, was getting nowhere with it, so I figured, “Oh, well, I’ll be like the other guys and I’ll just play into shape.” Fortunately, I was able to do a couple of games, but my breathing was very compromised. I didn’t know what was going on. Then, I got a shoulder injury and that pulled me out of hockey and then I could concentrate on why I wasn’t breathing. This progressively really got worse to the point… I live in a two-story house and I was barely making it up the stairs. It was just ridiculous. I didn’t know what was going on. Fortunately, my GP I’ve had for 30 years, so the familiarity with me and him was very fortunate, because I went in to see him explaining what was going on. He could visibly see I couldn’t even breathe. So we set up a bunch of tests. He was able to pick off right from the original chest X-ray that it appeared that I might have pulmonary hypertension. What he did with that was completed the rest of the tests, sent in the referral to the Pulmonary Hypertension Clinic in Edmonton. I was able to get in there within the usual four to six weeks. I got my appointment there. I met with the specialist. They did a CT scan during the appointment and an ECG. I got lost in emergency somewhere, because they couldn’t fit me in anywhere. I had to go through the emergency system. During my appointment, finally, around 9:00, I was able to get it done. Fortunately for me, it proved the dedication of the staff at the Pulmonary Hypertension Clinic. It was a Friday night and the doctor waited until it came through. He was going to Disney World the next morning. I don’t know how he did it, but he stayed. Then, he came and got my wife and I from the emergency back to his office, back to the clinic. And he quickly looked at the CT scan and he advised me that yes, I had chronic thromboembolic pulmonary hypertension. At that point, he told me the only option was if I had an operation, which would have to be done in Toronto, because they had lost their search and had Edmonton. They went at it right away. There was another test that was required for the surgeons in Toronto, which was the heart catheterization and some other test. I’m not sure what they all were. I was getting tested so much I couldn’t keep track anymore. Then, they had to set up a video interview with the surgeon in Toronto. That was Marc de Perrot. Great man, very impressive person. He determined that I was a candidate for a pulmonary thromboendarterectomy. They also noted that after the heart test and all that, they should probably do a bypass while I was opened up and that would allegedly make my heart stronger to survive the endarterectomy. So from October when I was diagnosed, I was able to get down to Toronto for the surgery. Of course, that came as quite an expense. The government did provide airfare and, of course, I had to travel with somebody, so I brought my wife. Then I had the surgery. It worked out to be, I think it was a little over 13 hours, because they had to do the bypass initially, and then they went in and did it. I was septic. I developed pneumonia twice. The real kicker here was that I turned out to have allergic reaction to the Heparin and then I developed a blood clot in my leg. The Heparin reaction like that is unbelievably rare. The only way they confirm it is to send your DNA to a laboratory in Hamilton, where then, of course, it was confirmed. To make it a long story short, ultimately it ended up the thromboendarterectomy being futile because I instantly re-clotted because of the Heparin allergy. With all the complications and everything, I wound up in Toronto in the hospital there for 45 days. I came out no better than when it went in, actually. Possibly a little worse. As I did not get better, I continued on with the Pulmonary Hypertension Clinic and they re-referred me back to Dr. Perrot in Toronto to look at other options. Ultimately, what was concluded was that I was too high of a risk to endure another pulmonary thromboendarterectomy, because of existing scar tissues and things of that nature just made the procedure too risky for me to have. But they did conclude that they could refer me to a vascular surgeon who could look at doing what’s called a balloon pulmonary angioplasty. That was arranged for me to go back to Toronto. That doctor said until he got in there, he couldn’t tell. Whether or not he could proceed with it, because if it was 100% blocked, he had nothing to work with, he couldn’t get a catheter around it. But he said he wouldn’t know that until he went in. But he saw some kind of backflow with the blood and figured he could attempt it. So I was scheduled to have it done again the next day for both lungs. He was able to get through, inserted a balloon in one lung. That worked in opening up one of the arteries. Then, he went in to the other lung and successfully opened that one as well, which was great. Then, we flew home. I felt much better. I was still on oxygen, but that didn’t last very long. At some point in the future, if I meet the requirements, I will be subjected to a lung transplant, but that’s down the road. I just take it every day by day, wake up in the morning and appreciate that I’m awake. On a humorous note here, what pulled me through the last time through the first endarterectomy was I said, “Please let me live so I can watch…” One of my daughters was just finishing off a degree, and I just wanted to be around for her to graduate. I take this as an omen. She was accepted for a post-graduate degree, but she started in September. So, I figured that’s got to give me two years because I have to see her graduate again! My wife just recently retired in June, which is fantastic because being alone with this thing, you’re just sitting at home dwelling on your illness, so it’s great. I have my partner back. We’re guardedly proceeding with our retirement and seeing what limitations I have. There’s all the flying restrictions. I have to go through all that kind of crap, but that’s just part of the disease and learning to live with it. I finally after years and years through the , I was finally able to get hooked up with a PH buddy. I talked to him about twice, because he had had endarterectomy 10 years previous. After the 10 years he was deemed PH-free, so he kind of disappeared. I got to start working on that again. When I was diagnosed with CTEPH, the first emotion is, “God hates me. What did I do?” You get a diagnosis like this. First thing you do, like everybody does, you look it up on the internet. It says one to three years, you’re dead. God. You go through the five stages, right? Anger, denial, until ultimately you’re at peace with it. I know what I’m up against. I’ve been through it all and living day by day. I wake up, I’m happy I woke up. I do everything I possibly can. Of course, there’s good days and bad days. There’s days I can barely get out of bed, and there’s days I feel okay. Of course, if I go to cut the lawn, I’ll throw the oxygen tank on and get out there. I can’t do anything physical just the way it is, at least without oxygen. The surgery is a major surgery. It is huge. You’re made hypothermic. They stop your heart. They stop your brain. You’re clinically dead for 20 minutes for three periods, so you’re dead for an hour. The reality is that I’m living with a terminal disease. I realize that. I’ve gone through all the stages of grief and everything, and I just carry on. I know there is possibly a chance of getting a transplant down the road. By the time that happens, I wouldn’t even be eligible till I’m 70, and then the whole process will have to be reevaluated again. Until such time, I’m just going day by day, living with it, going through every emotion known to mankind. I got nothing left. I’m just living. It’s such a rare disease, you almost get tired of trying to explain it, so you say, “I’m sick.” There’s no denying it’s hard, what I’ve been through. I don’t think anybody’s been through, but I’m still here talking to you, so I’m winning. You just adapt and do what you can do. My name is Stephen Gariepy, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/34285185

Episode 508 - Stephen Gariepy 02/17/2025

Jolie Lizana - phaware® interview 507 02/11/2025

Jolie Lizana - phaware® interview 507 Jolie Lizana is a pulmonary hypertension (PH) survivor and advocate. Despite enduring severe heart failure and lengthy hospitalizations, Jolie credits her medical background, determination, and the love for her child for pushing her to survive and adapt. Jolie now raises awareness through her website, , and has contributed to creating EMS protocols for PH patients, showing her dedication to empowering others with hope and resources. My name is Jolie Lizana. I am in Slidell, Louisiana, and I have been diagnosed with PH since 2013. I went six years from doctor to doctor to doctor to doctor trying to find out what was wrong with me, and they gave me every diagnosis under the sun. But even before I was bouncing from doctor to doctor, as a child, I remember, "Why can't I run that well, I don't get it. I don't have the breath." I always played sports. I was very active, but just for some reason I didn't feel like I was breathing as well as everyone else was. In my late 20s, early 30s, I actually started running because I knew that I wanted to have a child one day, and I knew something was wrong. I finally got up to four miles a day. I was just so excited to have this be something that I shared with my child. What a wonderful moment to share. What a wonderful thing to give to a child. That would be something they would love. So I thought it was just such a great thing to do and it saved my life. By the time my diagnosis came, I was really bad off. I think a few weeks before I had left the ER and the doctor told me, "I don't know what to tell you. Everything's fine," said I'm fine, "Everything's fine." And I'm just looking at her like, "Okay, I'm leaving this hospital cause nobody's helping me, and I think I'm going to die." At the time, I had a five-year-old son. I was working as a sonographer, so I would have to push patients sometimes or the machine, and I would have to stop mid-hallway or mid whatever and pretend to be reading over their chart so that I could catch my breath. I didn't want anyone to know that I was about to pass out all the time. I would lose my job. Nobody was helping me anyway to try to find out what was wrong with me. I had an appointment to see my regular doctor. I was supposed to go to work afterwards, but I went to see him, and he's like, "I don't know, Jolie. Everything seems to be fine. All your blood work is incredible. You're in great health. I don't understand." And he's like, "But you don't look nervous. You don't look anxious." I'm like, "Just let me walk up and down this hall. Take my vitals. Let me walk up and down the hall, and I'll let you know when I'm ready." And he's like, "Well, okay." So finally, somebody did it. It took me all of maybe three or four minutes and I went back in. He's like, "Oh, my gosh, are you okay?" And I'm just shaking my head, "Yeah," because I'm used to it. I'm trying to catch my breath. They took my pulse and everything, and they're like, "Okay, you need to go straight to the hospital right now, Jolie. Something's wrong with your heart and there's been something wrong for a long time." All this happens. I end up going to get an EKG where they tell me, "Ohm goodness, this says you have heart failure. This machine's messing up. Let me try it again. Oh, goodness, it looks like you have heart failure. Hold on just a minute." And she comes back, "Okay, the doctor signed off on it. You're fine. I told him that you look so healthy. There's nothing wrong. This machine's messing up." I'm laid there wanting to choke her, just not saying anything. I'm like, "Just get me out of here," and they did the echo. And next thing I know, some students and them are over there clamoring about my results. Like I said, I did sonography, but I did every part of the body except for the heart. That's a whole other beast of its own, and I don't know what's going on over there, but I know it's not good. And I'm looking, I'm like, "Okay." And they're like, "Oh, you could go." So I leave and I'm barely able to make it to the car. And this is on Friday. Come Sunday, I couldn't even sit up to breathe. I had to lay down and my aunt screaming at me, "You're going to the hospital, Jolie. You're going, you're going." I'm like, "Take me to this particular hospital because they are good with hearts. Let's go there." By the time I got there to get the diagnosis, I had right-sided heart failure, diastolic, systolic heart failure, a BMP of 467, a blood clot in each lung and PH pressures over 125. I don't remember exactly what it was, but it was extremely high. And I was in the hospital that year altogether for eight months. I was put on IV Remodulin. I was not expected to make it through the week, through the month, through the year, through the, here I am on year 11. And I credit me being alive to having ran before all this hit me to get my lungs healthy and to having that child to give me all the strength and energy I need to know that life is worth living. And just to I think having a medical background understanding completely as soon as they tell me what's happening, although I had never heard of PH before, clearly, as soon as they tell me what's happening, I get it. I understand it. Wrapping my head around still now, "Okay, I've lost my identity. I'm disabled, I this, I that, whatever. Who am I now? This is just crazy." I lost everything I had. So that was the hard part, having to admit, "Okay, this is what it is. You can't change this." I had more than one doctor tell me, "You can't push through this, Jolie." When the doctor says, "This is very serious, Jolie. Something's wrong with your heart." And from there, that's how my diagnosis just came about, being very pushy. I do believe that I had issues all my life with PH. I talked to my coaches and running, why couldn't I keep going for as far as fast? It wasn't my muscles, it was my breathing. So what was wrong? And I remember doing that several times. So I had this already, but yes, whenever I had a child, everything inside just gets pushed, and it's really pushed and it makes everything extremely hard. I couldn't run anymore while I was pregnant because I was having a lot of pain, I would keel over. Although I think I am pretty sure I had this all of my life from things that have happened, I definitely think having a child, expanding everything, pushing on it, I do believe that that kicked it into high gear. Having the diagnosis after everything I went through trying to figure out what was wrong was absolutely not a relief at all. Other people said that, "Wasn't a relief to know." No, absolutely not. Perhaps it was the way that I found out. I literally couldn't sit up to breathe, so I almost died. So it might've been a relief if the situation were different, but for me particularly, it was not a relief. It was a mixture of being pissed off because I told these people countless times, "I can't breathe. I can't walk. Something's wrong with me." No one took me seriously, and so it was anger. And then there was also this resentment within myself like, "Why didn't I push harder?" But really, I don't know what more you can do if no one's really listening to you. I think that being in the medical field helped me in the long run, but initially it didn't. Initially, it was, "Okay, I'm going to find out how I can fix myself. I'm going to find out what gave me this, what caused it." And I had my medical records. 3:00 in the morning, I'm surrounded by papers that I'm reading and looking stuff up online. And my doctor comes in, he's like, Jolie, you have to rest. Your body has to rest. Please stop looking at this stuff and let me do my job." It's the hardest thing to say, "Okay, I trust you. After six years of not being diagnosed, how do I suddenly trust this doctor?" And so I didn't necessarily just drop everything and stop looking. But when I was diagnosed, it was rough. I was trying to find out the cause, what was going on, and my head was just spinning all the time. Even though I knew that, well, I probably had this all along, it was still this, "No, no, no, no. Something had to have caused this." You just search and search for an answer, something that can fix it, not what they're telling me I have to do. There's got to be another way. There's got to. I can get through this. And so it was a lot of digging and a lot of just messiness; not sleeping well, staying up all night in the hospital. They'd give me medicine to make me sleep because my anxiety was just so bad. It was rough. But as soon as I finally calmed down with all that, the medicine started helping. During the first year, it was just about making it through the day. There were many, many nights I didn't think I was going to wake up. A little over a year in, I hadn't stayed in the hospital. I didn't go for an entire month. That's the longest time I had been out. And I'm like, "That's it. I'm getting better. We're going to celebrate. There was a fair, the world's largest free fair is right where we lived at the time in Franklinton, Louisiana. So I'm like, "We're going to go to the fair." And then it just hit me, "You know what? Let's not just go to the fair. Let's get a booth at the fair. Let's give out information about this to anyone and everyone that'll take it." So that's what happened. My sister helped me and we made business cards, got stuff from PHA and all these different places to give information out to anybody and everybody that would listen. I was out there with my IV pump sitting on a stool. I couldn't stand much, but I was there stopping anybody that I could on the way through. It was really great. I started having some family give me things like a guitar to a raffle off and various other things and so we did an awareness event. I know some people in a band and some people that are DJs, so we had light and sound and all this stuff, and it was great. It was the first awareness event. I had to set up a website for that because we were selling T-shirts for the walk. And then it was like, "Well, I guess I'll do a Facebook page." And so then it just grew into more. And this website I did. I started doing a blog on it, and I wrote anything I could think of that helped me. What helps you take a shower? What helps you go grocery shopping? What helps you do your laundry? Just anything I was writing down and it was helping me and hopefully helping others. I don't know if they even knew about it. I was doing it. I figured if it helps one person, then it's all worth it. So it started out like that, and suddenly, I don't know how, but I was approached by a couple of people to do various panels. It was just about a little over a year ago where I saw the thing with Johnson a& Johnson's where they have all these advocates go to Healthy Voices. They pick about 100 advocates a year. It's mostly nationally, but it's also internationally. I'm like, you know what? "Deadline's tomorrow, whatever. I'm going to send something in." So I just typed up something, "This is who I am," and whatever. I didn't think for a second that I get chosen, but I did. And I'm like, "Whoa, wait." And I'm looking at all these people that are going to be there, and I'm like, "These guys are real advocates. I'm just some person." Well, it took me going there to realize that, "Hello, you've been advocating for 10 years, Jolie." It's like it didn't even sink in what all I was doing. I was making T-shirts, just all these various things, but I never considered myself to be an advocate. So after going there and learning how to be a better advocate, which I definitely think is an ongoing process. But learning how to be a better advocate really just kind of sparked a whole new thing. So now I'm like, yeah, I'm all in. As an advocate, it's important not to only help others, but it's also nice to have a way to get out to express yourself with everything that's going on to people that understand and can relate and possibly really need to hear it. So the website that I have is . On there, there's information for people newly diagnosed and beyond that. There's more information to go to. Whenever I was first diagnosed, it was completely overwhelming. All these websites had so much information. I stagger it out a bit, and I also have a blog there and new information that pops out about medications, that sort of thing. And , it is also breathtakingawareness and it has my name, Jolie Lizana, attached to that. And the Instagram I have up, that is the same as well, breathtakingawareness. Having PH for a lot of people means finding a new identity. You lose some of your old identity, which is very difficult to deal with. So what do you do? Where do you go from there? I had to find things that I can do, things that maybe I would enjoy and I've done various art things; resin, paints, acrylics, never oils. Everything is pretty safe. I've redone furniture, at some points, make jewelry. I have a ton of things that I do, and I stay learning just anything to do with art. My favorite is painting. I spend a good bit of time painting. It's almost like my meditation, it's takes me out away from everything else, and it is an extremely powerful outlet. Whereas years ago, I used to run and that was my outlet. To find a new outlet, it took me a really long time, but it's extremely important. Being an advocate has given me opportunities to do a lot of things, and I was in EMS. I did that basically to get into school for sonography. It was just a bonus I hoped to get into school. But having that background, the medical background, I was invited to a round table event at the EMS Expo in New Orleans. I sat with paramedics, lawmakers, PH nurses. It was quite incredible. We wrote the first-ever pulmonary arterial hypertension protocols for EMS, and it's just passe Senate right now, and it's going to Congress. It went through House, through Senate. They're going to teach EMS protocols for all these rare diseases so that they can give patients the medication that they have on them. They can't do that right now. You have to wait until you get to the hospital so they can assist with patients' medication and just various things for PHers. And so I'm really, really excited to have that all come out. It's supposed to be 2.1 million every year, extended education for EMS. I'm very excited about that. When you're first diagnosed with PH, it's a lot. You really don't know what to expect, just sticking in there and doing what you can to find the new you and being just adamant about not giving up on yourself and not giving up on having a life. It may not be the life I chose for my trajectory, as I always say, just went straight off the cliff. But it doesn't mean that I have to just stay there. I can decide what I do with my life. And starting advocacy is something that fulfills me in so many ways, and it's definitely something I'm going to keep doing. I was completely overwhelmed, but now I am quite knowledgeable and just see myself sharing as much as I can with whomever I can. What gives me a lot of strength right now and hope for the future is knowing that I'm going to be helping people. It's not that I might, it's that I'm going to be a force and do all I can to help others. And in many ways, actually, as far as advocacy goes, I'm about to start advocating for a few more things because as advocates, we learned to be vulnerable, and I have a lot to share. So I'm just really looking forward to it. And I couldn't imagine finding someone whenever I was first diagnosed that could just pull me through this and say, "This is what happened, and this is the story, and you're going to be okay, because there's definitely hope." My name is Jolie Lizana, and I am aware that I'm rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/34157550

Episode 507 - Jolie Lizana 02/10/2025

Allison Fenstra - phaware® interview 506 02/04/2025

Allison Fenstra - phaware® interview 506 Allison Feenstra shares her transformative journey from being an active, independent young woman to navigating life with pulmonary hypertension (PH). She recounts how a sudden blackout led to hospital visits and the discovery of the condition, stemming from complications related to prolonged bed rest and birth control medication. Allison candidly discusses how she underwent a significant surgery and persevered through a high-risk pregnancy, ultimately becoming a mother. My name is Allison Feenstra. I’m from a tiny town called Nobleford. I was born and raised there, lived there all my life. My whole family’s still there. I came into the world of PH in 2019 when I was diagnosed. They actually found it because I blacked out on the sidewalk and somebody found me there. They had to bring me to hospital. I was in and out of hospitals for about a year before they figured out what was wrong. It was a lot to cope with, because I was in school to get my education degree. I had to stop that, because I wasn’t able to continue and work at the pace that I wanted to. I wasn’t able to work. I basically had to quit my job. I moved back in with my parents. I was definitely very unsure. I had no idea how I could go from being completely healthy and running 5K races to literally not being able to walk up the stairs. I went from one normal to a completely different normal in the span of three months. It all spanned from my doctor putting me on birth control, which caused the clots, and then the clots turned into the scar tissue, which turned into the pulmonary hypertension. I was in the 1% of people that happens to. It was one thing from being completely normal to completely rare really. I got pneumonia. It would’ve been November of the year that I was diagnosed. They had to put me on prolonged bed rest. Little did I know that the birth control I was on, one of their side effects was blood clots if you were on prolonged bed rest. Nothing was in the pamphlets that I was reading when I was given it, so I had no idea that this would happen. Then, I started getting more and more breathing issues and my doctor was like, “Oh, it’s just asthma. It’s just this. It’s just that,” but it actually turned out to be clots that had formed in my lungs, because of the pneumonia, because I was on prolonged bed rest while on this birth control pill. Like I said, I had to quit my job. I was working in a daycare at the time. It was deemed that I was unfit to work. Because of everything that I was going through, I was traveling to one from Calgary every six weeks normally. I couldn’t do the things that I wanted to do in life anymore. My life had completely changed. It wasn’t something that I was expecting at all. I think had I had some sort of, “Oh, well, I was sick,” and then it happened instead of rather than I was fine and then I wasn’t. It was a lot for me to deal with, especially personally. I was like, “I’m a young woman. I still want a family. I want the rest of my life,” and now here they’re telling me, “Well, you’re going to have this forever and you’re always going to not be okay. There’s nothing that we can do that’s going to 100% cure you.” I was in university for my education degree. I wanted to be a teacher. I wanted to work with kids. It’s something that I had always known I wanted to do. I was in year three, so it’s not something that I could just stop and then go back to, because you miss all of that. You have to do your practicums. You have to go into a school. You have to do all of these things in order to continue. It’s not something I could just be like, “Oh, well, I’m kind of better now so I could go back to that.” I had to completely reroute my life. After it all happened, I just laid low for a bit. I couldn’t work during COVID because of it. Now, I work full time. I’m a supervisor at my job. I honestly don’t know where I would be if it wasn’t for the team of doctors I had. They did put me through my pulmonary endarterectomy in the middle of COVID. Going out there by myself was completely weird for me, because I had nobody that I could have with me. I had no support system. Now, I’m in the workspace, but before that, I didn’t do anything. I very rarely left the house, because I was so worried that, well, what happens if I have a drop out while I’m with somebody and they don’t know how to deal with it? I was very cautious. Anytime I went out, I was with my family. If I didn’t go out, it’s because my family was staying home. I found out I was pregnant with my daughter eight months after I had surgery. My pressures were kind of back to something that wasn’t super bad but not advisable. They told me that there was a very high risk that I would end up hospitalized for my pregnancy. Because of the blood thinners I was on, they actually had to put me on injections, because the warfarin isn’t safe for kids. Especially when you’re pregnant, it’s not safe for them. because it can cause deformities and stuff like that. I had to stop a couple of my other medications that they had me on, because they wanted to make sure that my pregnancy stayed viable, because that was something that I told them I wanted. I told them there was no negotiating for that. I’ve always wanted to be a mom, and I wasn’t going to let this stop me from that, especially because I am so pro-life. I was like, “Why would I stop something that I have the power to say?” We went through it all. I was very high risk. I saw OBs in Calgary, I saw OBs where I lived. It was constant doctor’s visits. For me, it was normal. It was nothing out of my normal. It was just now I’m growing this extra life. I went through the pregnancy. It was pretty seamless except for a couple things that they had to rework. My daughter was born exactly a year after I had my surgery. It was honestly something I never thought I’d have, because they always told me, “Now, you have to be super careful.” But I also wasn’t allowed to be on birth control, because of the hormones that they say caused the clots, so it was like, “Oh, well, I’m pregnant and I’m not getting rid of the kid. She’s mine and I have a connection to her, and I’m not going to let anything stop me.” I’m a very resilient person, so for them to tell me basically, “This could make you worse,” I was like, “Well, if that happens, then it happens, but I’m not going to stop what I want in life because of something that I had no control over. I have control over this, so this is what I want.” Now I have a healthy, happy three-year-old, so I don’t think I would’ve changed anything that I did. I waited almost a year for them to get back to me about this surgery. I found out that my pressures were high enough that they figured she’s a candidate. If it’s going to help her, let’s do it. The pulmonary endarterectomy, the way that they’ve always said it to me is it’s basically open heart surgery but on your lungs. They go in, they go into your lungs. They basically scrape out the scar tissue that’s there, then they try and make it so that you have more lung capacity than you did before. My lung capacity at the time wasn’t great, so they did extensive amounts. Again, I’ll never be 100%, but even the amount that they got out, they didn’t think they would be able to get out as much as they did. I was super scared going into it. It was like, “Well, I don’t want this scar for the rest of my life.” I don’t want to have to tell people or have people ask me all the time. They actually let me go home early, because they say it’s a two-week recovery time in hospital. They sent me home in a week and a half, because I went above and beyond what they wanted because I wanted to get home. I didn’t want to be stuck there. I hate hospitals, so the fact that my normal was constantly going to hospitals, it was something that I was like, “I’m going to get out of here on time. I want to go home. I want to be with the people that have supported me through everything.” Then, I got to go home and my dad and my little brother were there for me. My dad came, flew out to Toronto because he had to fly back with me afterwards. They just poured everything into me. My little brother is 17. He’s still in high school, absolutely adores Aria. We only live four blocks away from each other. Normally, when I work, she’s with my dad and my stepmom all day. They take care of her. Every once in a while, if I work late, they keep her overnight. That way, I’m not having to wake her up. My mom and I are super close. She was the first person that I called when everything happened, but my dad was the one that was always with me in hospital. My grandparents, actually, have also been there for me all the time. Our family’s very close-knit. We’re a very family-centered family, which I love. I love that now I get to raise my child in that too, but my family was one of the biggest deciding factors for me through everything. It was always, “Oh, well, something’s wrong. We need to do something.” The primary people that took care of me during all of this was my dad and my little brother. My mom wasn’t at home a whole lot, but when she was, she was there. Having lived away from home for so long, it was something that I realized I’m allowed to rely on these people again. I didn’t have to be alone. For the first little bit, I kept to myself. I didn’t want anybody to worry about me. I didn’t want to bring anybody else into what I was going through because they didn’t understand. They’re like, “Oh, well, you don’t look sick so you’re not sick.” It was like, “Well, you can’t see the internal struggle.” I’m like, “But there’s definitely a struggle. It’s like my lungs are pretty much not working.” I stuck to my family, because they were the only ones that understood, because they were there when everything happened. I’ve always been a super independent person, even young pre-PH. I’m the middle child. I’m the only girl. I had to learn how to do things on my own, because my older brother wanted nothing to do with me. Once my little brother came along, my parents shoved me to the side, because I wasn’t the baby anymore. I learned from a pretty young age that if I wanted something, I’d just do it myself. Going into adulthood, it really strengthened itself. Before, because I moved 13 hours away from home, I was then essentially alone for my first year out of high school. I was like, “I don’t need anybody.” But then having to backtrack from where I was, it was really hard to let people in, because I was so used to doing everything on my own. Having to rely on somebody else after I was diagnosed with PH and basically going from doing everything for myself to not being able to walk up the stairs by myself was a complete 180 in my head. I had to retrain myself to be okay with asking for help, because it was never something that I enjoy doing. I liked doing things on my own. I have goals to be an assistant manager where I’m working now. I absolutely love what I’m doing, and I don’t think I’m going to change that. I just want to be able to live my life as normally as possible at this point in time. I’m not going to let anything slow me down. I’ve already let it happen. Now, it’s just more so focusing on my life, and making sure that my daughter’s taken care of, and being able to give her that life that I want to. Being able to lean on the people around me a little bit more is definitely something that I’ve done a whole lot more now than I ever did and being able to just focus on life after it all now, because my doctors say I’m getting better. I’m not getting worse, so I’m progressing rather than regressing, which I loved hearing. Being able to go back to that normal life where, hopefully, I can do the things I used to do. I used to love being outside and I used to love doing all the things outside that I used to do. I just hope now that I get to show Aria all of those things too. Don’t shut down. I shut down, and it was something that I never should have done. I pretty much shut out the world. I hated what had happened. I hated the doctors for putting me on the thing that caused my condition. Lean on the people that are around you, because if I would’ve done that from the get-go, I don’t think I would be in the situation I was in when it happened. Just be able to try and adapt. Don’t close your mind off to what everybody is telling you. Adapt your mindset to what you’re going through, because it’s going to be different for everyone. You’re rare, but that rare also makes you special and it gives you something to live for, because eventually, you could get better and you could live to be able to tell your story. It might bring somebody else hope that they’re not at rock bottom. My name is Allison Feenstra, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946707

Episode 506 - Allison Feenstra 02/03/2025

Jodi Berry - phaware® interview 505 01/28/2025

Jodi Berry - phaware® interview 505 On the 28th anniversary of her PH diagnosis, Jodi Berry shares her journey of living with pulmonary hypertension. Despite facing an initial prognosis of only a few years to live, Jodi’s determination and the support of her family, friends, and healthcare professionals helped her manage her condition while maintaining an active lifestyle. Over time, advancements in treatments and her proactive participation in clinical trials provided hope and improved her quality of life. I’m Jodi Berry. My connection to pulmonary hypertension is that I was diagnosed on January 27th, 1997. I’ve now been living with it half my life. I’m now 54, so it’s been a ride. If I think back now, I probably had it for about a year before I said anything to anyone. I was 27. I played baseball, I golfed, I skied. I did all the things that a 27-year-old should do. I was having fun. All of a sudden, running to first base was like a chore. I was fainting at home. We had a new puppy and I’d bend over to feed her and then stand up and be like, “Whoa, head spin,” and pass out. I went to my family doctor, and she was great, actually. She sent me to a lung specialist right away and started doing testing. I started passing out again. My lung specialist at the time, he wasn’t a pulmonary hypertension specialist. He sent me to emergency one morning and that was it. That was in January of 1997. I spent three weeks in the hospital while they diagnosed me. Sounds like I was diagnosed quickly, which I think I was. But hindsight, I should have said something sooner. I just thought I was out of shape. I was hitting the gym more and doing all these things, and none of that was helping. In the hospital, I had a right heart catheter in for two-and-a-half weeks. I was in the hospital in intensive care with a right heart catheter in my heart. They would try different medications. They would check my pressures in my right side of my heart while they tried putting saline through, while on different drugs. So, that was like, “Oh, okay, that was a bit shocking.” At 27 years old, you’re all of a sudden tied to a ICU bed. Then, they tried Adalat, which is a high blood pressure medication for the left side of your heart. I responded well to it instantly. That got me out of the hospital pretty quick. So, thank goodness. For someone to tell me that I had idiopathic pulmonary hypertension, I was like, “What?” This was the dawn of the internet, too, so you start Googling something and nothing came up. I put myself on a list that was in the states. It was a death sentence. You had three to five years to live max, and that’s what they told me. So, I was like, “Okay, I guess this is the end.” It was very isolating, I guess. Because like I said, you went on the internet and found nothing, or just the worst-case scenarios. There was these little chat boards of people saying they had it, don’t know where to go. I put my name on this list, and you put your phone number on it. This was the dawn of the internet, so you did that. All of a sudden, I was getting phone calls asking for information. People were asking me what I knew, and I was like, “Oh, I know nothing.” But so far so good. I’m on this one drug and a blood thinner. That was really all they put me on. It was something for the swelling, a LASIK. All my friends thought I was dying. My boyfriend at the time, he thought this was it. My family all came rushing to the hospital, thinking we better hold vigil, because this could be it. Really, nobody knew much more. We had a doctor in Calgary and he knew of pulmonary hypertension. He was my second opinion, I guess, at the time. He said, “Well, there’s some breakthroughs coming. We got a doctor in San Diego studying it, and he’s coming back. I’ll let you talk to him when he comes in a few years.” It’s like, “A few years? Who’s got a few years when they just gave me three to five if that?” Kind of thing. I was thinking, “Who’s going to get me a lung transplant?” All these things. It was mind-boggling, but you got to remain positive, too. That is a family motto. We SWAG everything — scientific wild-ass guess. Just go with the flow. That’s what SWAG stands for in our family. We just go with it and keep living each day as you would. I went back to work and they were great. They put a couch in my office, in case I needed a nap. I had a great community around me, and I still do. Still all the same friends, still all the same people. The doctor that was in San Diego was Dr. Doug Helmerson. He came back and he had all these new drugs to try. But I was on Adalat for 16 years, and then it just stopped working well for me. So, I’m on four oral therapies. As the years went by, thinking I’m on this drug and life’s pretty good. The worst, terrible thing that happens is you’re really pink. Your skin goes pink, because all the blood vessels are dilated. Your feet swell up. You got ham hocks all the time. Not a real attractive look. But I got married. I changed my regime. Now, I golf, I garden. No high-impact sports or anything like that. I was working full-time all the time and everything was pretty good. You’re thinking, “Well, this is manageable.” Then, my lung doctor just said, “Oh, I think we should pass you on to a pulmonary hypertension clinic.” That was at a different hospital in my city. Thank goodness I have access. I’m in this major center, so I have access to that. Things started to get, by year 16, which nobody thought anybody was making it to, or I’d have two lung transplants by then. You never know, right? All of a sudden, these new drugs were coming. I was like, “Let’s try on this one.” I was willing and able to try any of them. I just think that people have to hope for that, because there are so many new things coming around the corner, which is going to be amazing. But for some people, it’s too late, unfortunately. It wasn’t too far down the road when I did get to meet a few patients that had pulmonary hypertension, whether it be idiopathic my variety, or they had it from another situation from blood clots or something like that. Actually, it started with somebody else from my doctor’s office was diagnosed with pulmonary hypertension, which the chances are zero from that happening. Then, I got a call from a girl in Calgary as well. She phoned me and she said, “Listen.” This probably was two years after I’d been diagnosed, and she was about my age. She said, “I’ve been diagnosed with pulmonary hypertension. I don’t know what to do.” We started talking and then we formed a little group. We would have little luncheons or appetizer parties. It was mostly women unfortunately, and we didn’t have any children at that time come, but there was about six of us. Six in one major center is quite a few, I thought. I’m thinking I was the only one. We would meet and talk, but it phased out, too. Because then, once people knew and they settled down from their diagnosis, they weren’t as scared, and seeing other people manage through it and live through it. But a lot of people do not respond well to the oral medications, and they’re dragging their oxygen tanks, and that’s depressing for even a patient like myself to see all the time. I’m not a counselor to help these people through their trauma, as well. It’s good and bad in a sense, you know, to meet other people. I think I heard about I think it was through my clinic at the hospital. The PH nurse mentioned it. I’ve followed them online. I’m not a Facebook girl, but I follow them on Instagram and such, and go to their website, before Instagram, of course. But I just was living my life. I wasn’t searching for a group. I wasn’t searching for more answers, because I was my own advocate. I would ask my doctor, I’d ask my nurse, “I want the results.” I wanted to get on a drug trial. “I want to do this, I want to do that.” So, my doctors trusted me with my questions, because I wasn’t frantic. I wasn’t scared anymore. I just wanted the facts. I did try for three clinical trials. I’ve only been admitted to one. The first one I didn’t get accepted, because I had breast cancer, and I wasn’t five years out from my breast cancer, being clean from breast cancer. Then, the second one, I walked too far on my walk test, so I didn’t qualify. That was a kick in the pants. I hadn’t been that mad in a long time at something. I was like, “So, I just had to put a rock in my shoe and walk slower and I could have gone on this drug trial?” Then, the third one, I don’t even remember the name of it, but I did qualify. It was a phase two, so they’d already tested it on healthy people. There was 90 of us around the world that they tried it on. It was a tablet form. 33% would’ve been on the placebo, 33% would’ve been on one dose, and 33% would’ve been on a higher dose. I did find out I was on the placebo for the first 12 weeks, I should say. Then, they did put me on the actual drug. I still don’t know what dose that was. It did cause some gastral issues. I wasn’t feeling lousy, but then they canceled the whole trial for everyone. They found there was no benefit whatsoever. There was nothing detrimental either, so they just canceled the whole thing. I got a call one day and said, “Stop taking those pills.” I said, “Okay, I’ll bring them back.” In the interim, there was testing. Every week, I had to do something. I was getting an echo and coming to do a walk test at the hospital. I had a starting right heart catheter, one in the middle, one at the end. There was a lot of things that did happen. They also followed you very closely, so they could adjust my other PH drugs at the same time, to see if I needed to go up or down on something just with my pressures on my heart and stuff while I was doing this test. That was interesting as well, just to see the minutiae they could change just slightly with all this testing I was doing. They could see am I changing from day to day, from month to month. I think, it was about six, seven months I was on it. To be involved in a drug trial, you have to know all the facts. You got to read the fine print, because you want to make sure you can quit at any time. If you’re feeling lousy, obviously it’s not for you. You should stop instantly. There are so many benefits for it. Because a lot of them, if I’m on it and I am finding a benefit, but it doesn’t get Health Canada-approved, or covered by your insurance or your province, I’m going to be on it forever, because the company is now giving me the compassionate prescription. So, there is that. Some of these drugs can be $60,000 a year. I’m on four oral treatments, so that doesn’t come cheap. But I don’t pay anything out of pocket. So, there is that financial concern and benefit. As well, I just think if I could help find the cure to this ding-busted disease that was never on my radar, I would love to be on the forefront of that. I think for anyone new, who’s newly diagnosed, it’s so very scary. Why are they still telling people three to five years to live? Like, hey, maybe you should maybe mention that there are some long-term survivors here. And to be an advocate. If you can’t be your own advocate, then you need someone to go with you to every appointment, to write down things, to journal it. Because you go in and you have so many questions every time you go. I still have so many questions. Like, “So, if I get a lung transplant, are those lungs going to have pulmonary hypertension one day, because I have the gene?” They explained to me, “No, probably not, because they have a different DNA.” I was like, “Okay, that’s off the list. I don’t have to worry about that at night.” I think writing things down and going in and asking the questions to your doctor. If they don’t give you the right answers, maybe that doctor isn’t the best doctor for you. I know there are some people that are so remote that they can’t change doctors, or talk to somebody else. But there’s nurses that can help, and they have more practical solutions for some problem. Or if you have a male doctor and you want to ask your female nurse something, there’s always a solution to what’s eating at your brain and causing you to stay up at night, which the stress of it all can get to you, for sure. I think everyone with pulmonary hypertension should stay quite active, either physically, socially, and mentally. I do puzzles, I read books. I go for walks with my dogs, on a flat surface. No hills. I do yoga, which was a clinical trial I was on. There was a group of us all with pulmonary hypertension, that I had an instructor that taught us just exercises for pulmonary hypertension, to help stretch our chests. Activity is one of the best things we can do. Motion is lotion. You got to keep moving or things freeze up, including your brain. It makes your mood better. I think that really helps, getting outdoors. I know it’s hard, especially in our climate in the winter. Because for people, especially people with oxygen, when it’s -30 degrees Celsius, that’s not anybody’s best temperature. But I think staying active with your community, helping somebody else always takes your mind off your troubles, as well. My name is Jodi Berry, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946672

Episode 505 - Jodi Berry 01/27/2025

Janette Reyes, NP - phaware® interview 504 01/21/2025

Janette Reyes, NP - phaware® interview 504 Nurse Practitioner Janette Reyes from the Pulmonary Hypertension Program at the Hospital for Sick Children shares her extensive experience caring for pediatric patients with pulmonary hypertension. Reyes discusses the unique challenges and developmental considerations involved in treating children from infancy through their teenage years, as they transition to adulthood. Reyes also touches on the emotional and social struggles these young patients face, such as bullying and anxiety, and highlights the role of the multidisciplinary team in supporting their mental well-being. Hi, I am Janette Reyes. I'm a Nurse Practitioner in the Pulmonary Hypertension Program at the Hospital for Sick Children in the cardiology department. My connection to PH is not just seeing patients in the clinics, but also when they're an outpatient and inpatient, thus promoting continuity of care for the patients and families. Whenever the parents and patients have questions, I'm there for them. Today, I want to talk about the range of ages of our patients and how pulmonary hypertension can happen as early as birth. We follow these patients until they're 18 years of age. I've been in this role for quite a while, so I have had the experience of and privilege of caring for these patients as they grow older, from infancy sometimes to their teens and transitioning to adulthood. So, I've had to also consider their developmental stages that they're at when providing care. It's also been a pleasure and exciting to see them grow and change and have different types of concerns and issues, particularly as they get older and they become more trusting of the medical team and you start to observe the changes in their moods during the appointments. So, you have to be cognizant of what they're going through. Perhaps there have been changes in their school or interaction with their peers or how they're coping with their PH as they change schools as well, making new friends and the challenges that they go through. They're a bit more different in a sense that they can't do all the things that other children and teens can do. They're a little bit more limited, let's put it that way, at times. But, we discuss that there are other positive activities or issues that can come out of their current situation, as well. One concern is sometimes they somehow with our interaction with each other, something at school may bother them and so we would focus on that. When I ask them how they're doing, it does come up that perhaps something happened at school and they're feeling sad about it. Sometimes the parents inform me that there's teasing or bullying. One patient was experiencing some bullying at school, because he couldn't keep up with his friends. There's also psychological issues that could make them feel sad as they report. We're fortunate to have a multidisciplinary team of social worker, psychologist-- and psychiatrist -- that can help us with such issues, including anxiety and how they're dealing with their illness, family and friends. For some patients, I start to see them at a young age. As they go through their stages of growth and development they go from being very chatty at their clinic appointments. They're not aware of their diagnosis, so they come to a clinic visit happy because most of them will get a treat after their appointment and they're not really understanding what's going on. They're eventually get used to taking their medication on time. They hear from us that “You have to take this in the morning and then at night and they understand that it is good for their heart. It interesting to witness the change in their disposition as they grow older, where the chattiness starts to become less. This mood start to occur around 11 or 12 years of age, the tween years, when they are quiet during the appointments. They sit in the room and not being very verbal. You have to really try to get information out of them, so the parents are very much helpful and involved in letting me know what they’ve observed and how their child's feeling. Then, as they patient becomes older, and mature, they start to understand their health and illness. At around age 14 years old, I will start more in depth teaching by discussing and illustrating, “This is what's wrong, or this is what's happening to your heart. This is pulmonary hypertension.” Then, once they start to understand, then they start to engage more in conversation, become more responsible and involved in their care over the years. There are still some patients that require more teaching and there are others that understand and being to ask more in depth questions and discuss what they're going through. With respect to the transition process, as the patients get older, they become more responsible in taking their own medications, reporting more of their symptoms to me. This is the one of the goals. I also encourage them to communicate directly to me rather than having their parents inform me and the doctors. The parents start to release some of their, interaction with me. They start to understand that they know what they're feeling, they know their symptoms, they know what their concerns are more than anyone. A typical interaction would be when the parents will wait and remain quiet, allow the child talk and answer the questions and sometimes when the child doesn't... Or the teen forgets something, the parent(s) will say, "Oh, remember you did have chest pain last week." Just sort of as a reminder. The parents and the PH Team help prepare the teen towards transitioning to adult care as they leave Sick Kids. The way I deal parents who remain very involved with the care and responses to my questions, where when I ask the question to the patient and then the parents will right away answer, I turn back to the patient and say, "What is your description of how you feel? What is your pain like? How often?" I turn the conversation back to the patient. However, if the teen is not able to further provide a detailed response, then I'll turn it back to the parents. We discuss goals. I would typically say, "When the next time you come, I want you to tell me how much have you done." For the older patients, I would say, "I want you to call your prescription to the pharmacy. Your parents can be there, but I want you to make a phone call. Then, when you come back, you tell me if you did that." I discuss little goals to help them with that independence. I let the parents know it is also for the best, because you're not going to be there with their child at all times when they're in university or college. We do need to prepare them to be a bit more independent." I've been in my role over 20 years. In the 20 years, we went from just Flolan, intravenous Flolan with the emergence of sildenafil in the 2000s. Then, came the bosentan. Then, what was wonderful was the treprostinil, which then was an option, because Flolan as many of you know, you have that six minute half-life. It's a very demanding, stressful therapy. So with the four-hour half-life of Remodulin, that was something that was positive that came out of these medications. Then, more oral medications started coming to a point where there was even an oral prostacyclin, where we could at least prolong the subcutaneous and the IV medication therapy. Sotatercept, with that coming up, that's another exciting new pathway of medication. So everyone's certainly looking forward to that. We've come a long way, and my conversations with families, it's still the same in the beginning where it is overwhelming. I tell them not to Google, although they will still Google, which is understandable. But now we have so many resources in the PHA Canada website and PHA Association in the US and other countries that we could ensure them and say, "there is no need to Google. Go to these websites, and that's where you'll find your information." That's the change in helping these parents in the beginning who are so overwhelmed with the diagnosis, particularly with idiopathic pulmonary hypertension. Then, over the years, it has been easier because there are medications. We'd no longer say, "Your child has three to five years to live." Now we don't know. These children are becoming adults, growing up to be adults, perhaps only on one medication, maybe on just two oral medications. The types of conversations have certainly changed more in a positive way. It's an exciting future up ahead for even new patients who are being diagnosed. So, hopefully there's going to be more medications and maybe a cure. A cure is what we're looking for. We're fortunate we have different options and now a different pathway of medication, but let's hope a cure is coming soon. I think one thing that I'd like to add is that parents should be open to allowing their child to grow up as normal as possible and allow them to experience life. These children can do many activities of daily living and be happy. They know their limits. If they want to go out with their friends, just set out some guidelines in case there's issues or emergencies. That way they can hang out with their friends. It's okay. It's okay if they want to do a little bit more, physical activity such as sports and employment. Talk to the PH team. Together, we can help the child grow up as normally as possible and experience life to the best that they can live. One last thing that I'd like to add is that I'm very fortunate and privileged to work with children. Each child is different and unique in their own way. So, the way we treat them and my interaction with each and every one of them is unique. The care comes from the heart. My name is Janette Reyes, and I'm aware that my patients are rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946612

Episode 504 - Janette Reyes, NP 01/20/2025

Jennifer Howard - phaware® interview 503 01/14/2025

Jennifer Howard - phaware® interview 503 Jennifer Howard’s inspiring story is one of resilience and hope in the face of immense challenges. Diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in July 2019 after a long and frustrating path to answers, Jennifer faced the daunting reality of living with a chronic illness requiring continuous IV medication through a pump. Despite the overwhelming nature of her diagnosis, Jennifer discovered an inner strength she never knew she had. With a focus on living life to the fullest, she learned to navigate both the bad days and the good, embracing moments that brought her happiness and refusing to let despair take root. My name is Jennifer Howard, and I'm from Calgary, and I was diagnosed in July of 2019 with IPAH. The day after I was diagnosed with my right heart catheterization, I immediately went on to the IV medication Caripul (epoprostenol). So I'm on a pump 24/7. I complained to my family doctor, "I'm breathless when I do stuff," and she's like, "Here's an inhaler." I was like, "Great." Then, I was like, "It doesn't work." Then, she said, "Try another inhaler." "It doesn't work." Then, New Year's Eve day 2018, and I was walking to Walmart and I fainted. My thought process was if I make it to that light, I will be okay because I can brace myself, catch my breath. But I passed out. Some lovely people called the ambulance on me. I said, "No, no, no, I don't want to go to the hospital, because they're going to charge me $250 for me to be told I didn't eat enough food." But then they took a EKG and they're like, "You are looking a little weird. You should see your family doctor. That took a couple of weeks. Then she said, "I'll send you for a stress test." Nothing happened with that. The cardiologist wasn't speedy, so about March, I finally got the stress test and they're like, "Nothing's alarming." Then, they said, "Oh, we'll do an MRI." They're like, "You'll hear from us in five days." The next day, I got a call. "You have pulmonary hypertension." I'm like, "What is pulmonary hypertension?" They're like, "We don't know." So we're both Googling at the same time. I'm like, "Great. I'm dying, because Dr. Google is not accurate." Within a week, I was sent to the Calgary clinic. Within days, I had my right heart catheter and I was put on the pump. From the time I fainted until my diagnosis, it took six months. But before that, it was just years of my doctor saying, "Here, have an inhaler," because she didn't care, which is a sad thing to say, but she really didn't. I even had the thought process, before I was diagnosed, was I'm not going to see the end of the year because I was just feeling crappy. The doctors here saved my life. I love my pump, keeps me alive. I didn't have a lot of process time, because I went from meeting my then doctor on a Tuesday to having my right heart catheter on the Thursday, to him saying, "We need to immediately admit you." By Friday morning, I had my new accessory. I was numb to the situation. Then, Sunday hit. We had this book to read about all our instructions. I had read it, but I didn't process it. Then, I'm like, "I'll read it again." I just started to cry in the hospital. One of the nurses came in and she's like, "Now is not a good time." I only let myself have that one day of just, why me? But I couldn't let myself focus on that because I was like, "I don't want to become my illness." I came out swinging, and I tell my mom. I'm like, "I never knew until I was diagnosed that I was a fighter." Because I'm not. I'm the introvert. I'm the book nerd. "Oh, no. No people." But I was just like, "I'm coming out swinging." I took a lot of inspiration from my uncle who had a massive stroke and he had this cane, and his physiotherapist is like "Not that cane, because it makes you look sick, so we're going to give you this cane." That stuck with me once I was diagnosed. I spent a lot of time when I was in the hospital, because I had nothing else to do for two months, shopping on Amazon for things that were cute or pretty or things that just didn't make me look or feel sick ,because I was like, "No, no, no, no, no." In the book it said something about a cooler to carry your medication because the Caripul cassette needs to stay cold. You can't just throw it in a bag and away you go. It needs to be kept cold, so that means ice packs or a cooler. That's what really broke me. I was like, "I don't want to walk around with a cooler." But I found the baby bottles that you can use to keep baby bottles cold. I was like, "My ice packs fit in it perfectly." So I was like, "I don't look sick." I have my pretty pink backpack, but just is my cold bag. I really took inspiration from him. His doctors had no hope for him, but he and my aunt just kept fighting. I was like, "Well, if he can fight to walk and talk, I can fight to breathe." I just really used him as my barometer of doing the best I can. We all have bad days, but it's just not drowning in my bad days. Every day I get up and get dressed, even when I feel crappy, because I don't want to... Well, first of all, I have to because I have to change my cassette every day, but if I were to stay in bed because I'm a wallower, I would never get out of bed. I know my weaknesses, because I'm a little bit of a bummer. So, I was like, "I can't wallow in my own personal weaknesses because I just want to keep going." I had two young nephews at the time. I was like, "I'm not ready to say goodbye to my nephews, to my brother, to my mom, to all my friends." I was 46, so I was like, "I'm not quite ready to go yet." I kind of kept that mentality through everything. PH is not the same for everybody. None of our journeys is the same, and so I know I could say like, "Oh, I came out swinging," and somebody would be like, "I just don't have the energy to swing." I try not to judge how my journey has been based on theirs. Don't base your idea of swing on my idea swinging, because maybe yours is just waking up that day is your idea of fighting. I turned into a gamer girl, so I started gaming. I'm terrible at Fortnite, but I have a lot of fun. I found people to game with. It helps because it gives me a social aspect, because I don't work. I'm on disability because of the side effects of this drug, and all my friends are working. But when I game, it's with people. I have a full-time mom who games, so I get a social aspect I didn't have before, and it's fun. I'm terrible, but I have fun. I turned into a gamer girl in my 50s. It's funny because I never considered myself positive or even a fighter, and so that's what my illness has taught me, that I'm a fighter, that I am far more positive than I was before. I think you need to find something that makes you happy, not even passionate or... I'm not saying run out and write a book. If you can, that's awesome, but if that doesn't make you happy, don't do it. If numbers make you happy, find something that makes you happy. It makes it easier to not dread every day thinking, "I'm sick today. I'm sick today." Some days it's just like, "I'm headachy, I'm tired. I don't want to do it." So I don't do it. We have to also let ourselves be permissioned to have our bad days, so then we can embrace the good days a little bit more. But just find something that brings a smile to your face and makes you forget for even five minutes, "I'm a sick person," because I think if it were me, if I were to spend so much time thinking, "I have a chronic illness that will probably kill me," I can't let myself think that way. It's so weird because teenage me would've moped in this. I'm surprised at what this illness has taught me and what other people with this illness have taught me. Everybody facing any chronic illness were just freaking amazing. The first time after I got on my pump and I started feeling better, I walked across that same spot I passed out on, because I have to walk there every time I go to Walmart. I always look at it and I go, "I see you." I just keep going, because I remember what it was like to be like, "Am I on the ground? My pants are cold." So, in five years, there's so many changes happening with medications. I know we're all staring intently at Sotatercept. We're like kids waiting for Santa. That's how I get, and every question is about it. I'm hoping that drug or the next drug, and that one day I don't need the pump and that nobody else needs a pump, that friends don't have to be on oxygen, that I don't have to lose more friends. I just can't wait to see what comes down the pipe. But for me personally, I don't know. I hope I get better at Fortnite, and I'm actually writing. But I don't know. I just keep swinging. My name is Jennifer Howard and I am aware that I am rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946557

Episode 503 - Jennifer Howard 01/13/2025

Kaitlyn Salonga - phaware® interview 502 01/07/2025

Kaitlyn Salonga - phaware® interview 502 Kaitlyn Salonga shares her journey of resilience and advocacy while living with pulmonary hypertension (PH). Kaitlyn recounts her fight for personal autonomy, including a successful battle to secure surgery for her reproductive health after initial denials due to weight and health concerns. Through her triumphs, she highlights the importance of advocating for oneself in the face of medical and societal challenges. Her story is a powerful testament to embracing one’s identity, breaking down stigma, and showing that life with PH can be fulfilling and bold. My name’s Kaitlyn Salonga. I also go by Kate, or on social media as , which is how I like to describe my disease of pulmonary hypertension to those who might not be so familiar with it. I was diagnosed in December, 2018. For my particular treatment, I’m on triple therapy, so two oral pills and IV Caripul. Last time I chatted here, it was mostly about how I had recently gone through a breakup in my relationship of 11 years. Unfortunately, we were the example of what can happen negatively once a patient gets diagnosed. That resulted in me being a single and having to navigate a new version of my life where I was living on my own and having to be my own caretaker and having to mix everything by myself. Once I got comfortable in all of that, I was able to start dating again, but that still brought up the issue of pregnancy versus PH, which do not go hand in hand as most of us know. I brought up the topic of birth control again to my doctors. I was fighting to have my tubes removed or something that was a hundred percent proof, because the IUD was not a great option. My birth control pills were half as effective, because of my PH meds, so I wanted something that was foolproof. Unfortunately, the first doctor that I had spoken to about three years ago had said I was too overweight and too sick to operate on, which is a harsh thing to hear when you’re trying to get an operation that could save your life or be preventative in something that could harm your life. I asked the doctors again, “Let’s have a second opinion.” I was very lucky that a doctor in Richmond was happy to do the surgery. I had a date, I had signed forms, only to be told that the anesthesiologist disagreed and they canceled the surgery. So, I was back at square one. I was very grateful to her because she understood the risks that were to be taken if I didn’t have the surgery, which is what a lot of people were not kind of open to, which is basically if I get pregnant while I have PH, my heart’s going to explode. Obviously, that is something that we want to prevent. She understood my concerns as a patient, that I wanted to take charge of my sexual health. It wasn’t a situation where I could ask a long-term partner, “Why don’t you get the vasectomy so I can have control of my body?” This is something that I wanted to do for myself and my future. She actually referred me to another doctor and we set a date for March, and I finally got my fallopian tubes removed. The surgery was successful. I had no complications in it other than some allergic reactions to the face mask that they used, which was completely unrelated to my pulmonary hypertension. I’m happy to report that all went well, and that is an option, if your PH is stable, that you can bring up to your doctors. I want people to know that this is something that they can do to take charge of their sexual health. And if you want to learn a little more about my experience, you can actually check out the article that I wrote in PHA Canada’s Connections Magazine about . I had the pleasure of rejoining a community choir that I had spent a lot of time with in my twenties. I wanted to kind of prove to myself, can I still sing with a lung disease? That was a very daunting question because that was part of my identity for a very long time. To be burdened with this lung disease and to have this thing that I worked so hard on for so long kind of taken away from me, there was a lot on the line to see if I could actually handle rehearsals and learning music and putting effort into all of that, and standing for a whole concert to see if I could perform again in front of the stage and doing what I love to do. I’m happy to report that after eight weeks of very compacted learning and rehearsal and very grueling work, I was actually able to do it again. I was filled with so much joy and a personal accomplishment after that experience. Unfortunately, that kind of led to a different experience. Being somebody who has mobility needs and considers themselves disabled and is also attached to an IV pump, I was having conversations with the directors about the accessibility of this space and how I had to climb up and down stairs just to get to rehearsal. That winded me. I couldn’t get up to use the bathroom because of those stairs, and simple things like that that a lot of able-bodied people don’t necessarily think about, especially in the frame of a younger person. Unfortunately, with that organization, we had some photos taken on concert night. I was feeling good, only to realize that when those photos were shared later, my site, my dressing, and my tubes that were connecting me to my life-saving device were photoshopped out of the picture. I asked them to take it down. I asked them to remove it from all marketing materials, and I told them I do not consent to this form of ableism. The organization has apologized for the actions that were taken by the photographer, not by the instruction of the organization. But that’s an example of the subtle things that might happen when you are in this situation of kind of approaching this world of ableism and disability awareness. It’s little things like that that can really frame your perspective of how far we have to go still in our work with ambassadors. On the flip side of that, I’ve actually had the pleasure of working with a photographer, Tilly Nelson, who was the photographer in residence at Langara. She asked me to be part of a project called . This project highlighted the fact that disabled people are all around us, people with visual differences are all around us. It’s time that society acknowledges our contributions. I was very flattered be her tube representative. I was rocking the pulmonary hypertension devices and the little inscription on my poster that was hanging up in this college, in this art exhibit for about a month said, “I deserve a seat at the table.” I think what I’d love for people to take away from this is not everybody with pulmonary hypertension considers themselves disabled, and that’s okay. That’s an identity that I would like to put on myself, and I’m proud of it. I think it has given me a lot of bravery and opportunities to speak up on people of other differences. We do have a lot of work to be done, but my hope is that people with pulmonary hypertension or any set of tubes specifically don’t feel so isolated that they don’t feel like they’re such this rare commodity. I want to see tubes in advertisements. I want to see tubes in leadership roles. I want to see more medical devices being understood as a common presence in our lives, because they are all around us. People do hide them, but they don’t need to hide them. They can actually use them. It’s part of their everyday lives. I just want to point out that it’s no different than somebody who uses a pair of glasses or a hearing aid. We just don’t see it as much. If we put more of it out there, then people are going to start feeling better about themselves. I’ve had good and bad luck traveling with my condition. Obviously, at the airport, you are usually instructed by security to take off your belt, take off all your bags, your jackets, and all of that, but I’m attached to my medical device, and I’m not about to stop my IV and unhook it just to walk through the security gates. Most recently from flying from Vancouver to Calgary, I had the best airport experience that I’ve had so far. It was acknowledged that I have a medical device. It wasn’t just me telling the people. It was them communicating to each other that there is somebody with a medical device that they cannot take off. That has been such a huge improvement from where it was. In earlier times, when I was traveling, they would think it was a weapon. Somebody actually was ready to pull their taser on me just because I was gesturing to my fanny pack just to say, “Hey, listen, I can’t take this off.” I’m very, very impressed with how far airport security has come, at least at YVR. I hope anyone out there who is on a pump device or is considering joining us, that you realize that it’s not the end of the world. It can actually be extremely beneficial, and that you can lead a really full and exciting life despite being attached to an external device. I hope this has opened your eyes a little bit as an insight to pulmonary hypertension and my life as . I hope that we can look forward to less invasive therapies in the future. I hope everybody on the outside looking in can realize this is part of our life and it’s our everyday norm, and that they can accept it, because we already are on our way there. I’m Kaitlyn Salonga, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946517

Episode 502 - Kaitlyn Salonga 01/06/2025

Lisa Mielniczuk, MD - phaware® interview 501 12/31/2024

Lisa Mielniczuk, MD - phaware® interview 501 Dr. Lisa Mielniczuk, a cardiologist at the Mayo Clinic in Rochester, shares her personal journey of becoming interested in pulmonary hypertension and the challenges faced by patients in accessing care. Dr. Mielniczuk emphasizes the importance of building connections with patients and involving them in decision-making. She discusses the CRAVE study, which aims to test novel and repurposed therapies for right heart failure using a platform trial design. She highlights the importance of patient involvement in the trial design and its upcoming feasibility study. My name is Lisa Mielniczuk. I am a cardiologist and Sr. Associate Consultant at the Mayo Clinic in Rochester, formerly from the University of Ottawa Heart Institute in Ottawa, Canada for many years. I am a specialist in what's called advanced heart disease. So, my area of interest and specialty is looking after patients with very serious and complex heart disease. That includes things like heart failure. I look after patients who have or have had or need a heart transplant, as well as patients who require mechanical circulatory support or heart pumps to help to keep them well. I would say quite honestly that one of my biggest interests and passions in my career thus far has been being involved in the pulmonary hypertension community and looking after patients with pulmonary hypertension. I'm really excited to talk to you a little bit more about some of the research that is launching here in Canada to help to improve the outcomes of our patients with pulmonary hypertension and right heart failure. Before I do that, though, maybe I'll tell you a little bit about myself in terms of why I got interested in the field of pulmonary hypertension. That was many, many years ago when I first started my clinical practice, I was on staff probably about a year or less, and I was working in the intensive care unit. We had admitted a patient with heart failure and she was a very young girl. She was only 19 years old, very nervous, appropriately so. It was quite obvious to all of us that she was very sick. Now, initial echocardiogram or ultrasound of her heart showed that she had significant right heart failure and she had severe pulmonary hypertension. She was very unwell and a very high-risk patient. Immediately, all of us as a team rallied and wanted to do everything we could to help her in this journey of diagnosis and management. In fact, actually very quickly, it became apparent that this wasn't the first step in her diagnosis. In fact, she had had symptoms for many, many months before she came into the intensive care unit. Like many patients with pulmonary hypertension, her symptoms were attributed to other things, other things that might be more common in a woman with shortness of breath. That, like for many of our patients, led to a delay in her diagnosis. So, by the time she had her diagnosis, unfortunately, she was very ill. That was an important realization for us that we needed to do better. Then, there was a second realization that happened in the care of this patient. That was that, at that time, many years ago, in the infrastructure in Ottawa, we did not have a Pulmonary Hypertension Clinic. In fact, we didn't have a PH program at all. So, all of our patients that were diagnosed or needed treatment for pulmonary hypertension had to either go to Montreal, which is about two hours away, or to Toronto, which is about four and a half hours away. We ended up transferring the patient to the excellent care of the Toronto program, and she did very well. But can you imagine being told that you have a very serious disease and then being told that you now have to be transferred away from your family, away from your home, to an external institution to receive care and treatment? It was right then that I knew that, in Ottawa we needed to do better. We needed to do better for our patients and our community. So, along with some incredible collaboration with a colleague of mine from the pulmonology side and some excellent collaboration with nursing support from the Heart Institute and even support from industry partners, within a year, we were able to start a PH Clinic here in Ottawa. Our little program grew. I'm really happy to say that now we are certainly one of the larger programs in the country and really actively involved in research, support, and education. We've really been fortunate to make a huge difference to the care and the quality of our patients with pulmonary hypertension. That's a little aside, but it tells you a little bit about our story, and why I'm here, and why I think being here is so important. One of the things that I think is just as important as the medical therapy of pulmonary hypertension is the connection that we build with our patients. Myself and my colleagues have been really lucky to work very closely with incredible patient partners and organizations to unite our community. I've been working with Pulmonary Hypertension Association of Canada for many years now, previously as vice chair. I can tell you quite honestly that it is probably the most fun part of my work. In everything we do, we realize how important it is that we have our patients and our caregivers first and foremost with all the decisions we make. With that as kind of an introduction, I'd like to tell you a little bit about some of the research that we do. Now, my area of research interest for many years now has been in the field of right heart failure. Now, if you haven't heard of that before, and I tell my patients all the time, "Heart failure sounds like a terrible thing, right. My heart is failing," and we need to come up with a better term for that. But essentially, what that means is almost you can think of it as a supply-demand mismatch between what your heart is able to give in terms of cardiac output and blood flow through your body and what your body needs to feel well to do the things that we love to do, whether it's exercise or even walking, talking sometimes just eating and feeling comfortable at rest. Whenever there's an imbalance there where the heart is unable to provide the needs of our body with what we need to feel well, we develop the symptoms of heart failure. Those symptoms can include things like shortness of breath, fatigue, chest discomfort, fluid retention, exercise intolerance. Unfortunately, many of our patients that live with pulmonary hypertension are at risk of developing right heart failure. Now, why is right heart failure so important? It's very important for a number of reasons, but I'm going to share with you probably two that have really inspired me to do the research that I'm doing. The first is that right heart failure is one of the most important predictors of what we call prognosis. How we determine how we need to worry about a patient in the short or long term? We know that if patients develop symptoms of right heart failure, that they're going to struggle a lot more. So, it's a very, very important thing to identify. The other thing that's known about right heart failure is it's not so much the severity of the pulmonary hypertension or how high the blood pressure is in your lungs that determines whether or not you get right heart failure. It's a much more complicated algorithm. In fact, we don't even know all the pieces of what makes somebody predisposed to right heart failure and what makes somebody relatively spared from right heart failure. But those are some of the things that in my research I've been trying to answer over the last few years. The second thing that I want to share about right heart failure is that unlike left heart failure, which is a very common type of heart failure that I also look after in other clinics, in other areas of my job, the treatments for right heart failure are very, very limited. In fact, we have great medications that work really, really well on failure of the left heart to improve quality of life, improve survival, and actually sometimes even improve pumping function of the heart. Yet, those same medications, when applied to the right side of the heart, are either shown to be not effective or, in some cases, dangerous. So, there's a lot of uncertainty about how we identify right heart failure, how we predict right heart failure, and importantly, how we treat right heart failure. This is where we are focusing on our energy. My colleague, Dr. Jason Weatherald, and I have been talking about this for quite a few years now, because we want to develop a research line of focus that can help us to move forward faster and more efficiently, potential treatments for right heart failure. One of the things that we think is very, very important when we're talking about clinical research is when we used to do clinical research many, many years ago, it was really thought about from the level of the healthcare professional. The study was designed by the healthcare professional. We enrolled patients who participated in the clinical study, and then we analyzed the results and came up with conclusions. We hoped that the patients would agree with our conclusions, but they historically weren't given a lot of information or attention into how those results may impact them. I can say very gladly that the landscape for this has changed quite dramatically. We now understand that for medication to be successful, for patients to take medications and feel good about medications and be engaged in the whole interaction of their clinic encounter with their healthcare team, they have to be at the table. Not only do they have to be at the table, but they are the most important part of the conversation. So, one of the things that we're trying to do with our CRAVE study is engage our community partners, our patient partners, our people with lived experience, and their caregivers at every step of a clinical trial design. What that means is we want to know what matters to our patients. What clinical endpoints in a clinical trial are relevant to them? What type of clinical study would they feel comfortable participating in? What type of clinical study would they feel very uncomfortable with? What sort of result would be meaningful to them, so meaningful that they might actually feel very good about taking a particular therapy, or what sort of result would be not consistent what they think is in their best interest? So, with CRAVE intertwined into the design of our project is going to be partnership, and we've been so grateful to have excellent partnership through PHA Canada to really engage our patients at every step of the trial design. The last thing I'd like to say is just to tell you a little bit about the trial design. As you might imagine, from everything I've been saying, CRAVE is designed to look at novel or potentially repurpose therapies for right heart failure. By repurposed, I mean treatments that have been shown to be effective in one clinical condition to see whether or not they're effective in patients with chronic right heart failure. One of the things that we've also struggled with in the land of pulmonary hypertension and right heart failure research is it's very hard to do large clinical trials, because we know that there are not hundreds and hundreds and hundreds of thousands of patients with pulmonary arterial hypertension. Oftentimes, clinical trials are smaller as a result, and their endpoints or their results may be limited because you may not have enough power in the study to find a meaningful result. One way around that is a novel statistical design called a Platform Trial. That's what we are trying to build with CRAVE, whereby not only are we testing one treatment at a time, but we may, in fact, be testing multiple treatments at a time in different scenarios and over different arms. By following things very closely, we might be able to identify, early on, a clear winner of these early studies, a drug that shows a lot of promise, and we can shift the design and the nature of the study to preferentially enroll patients in a treatment that might be showing good promise. Alternatively, in a treatment that may not be showing very good promise, we can stop studying that treatment earlier on and shift our focus without having to end a study and start over again and, therefore, lose time. We had meetings in Edmonton, Alberta, recently of a number of engaged stakeholders and community partners to sit down for a day and a half and really just talk about what would this CRAVE trial look like. What sorts of things are important to us, what therapies might be good candidates to trial, and how might we launch this as we go forward? I'm really excited about the next steps. We are in the process of launching what we call a Feasibility Study, which is sort of a mini-scale version of what we anticipate the larger trial may be, and we're very, very excited about launching this in Canada in 2024. But we're also really hopeful that we'll be able to partner with some excellent colleagues and friends in the United States and potentially consider this a North American initiative, because we do feel that it is so important. We are really excited about this new day of clinical trial research, where research is not just on the patients, but the patients and the caregivers, and everyone are working collaboratively together, all at the same table all at the same time. My name is Lisa Mielniczuk, and I am aware that my patients are rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD #HeartWorksApp /episode/index/show/phawarepodcast/id/30380828

Episode 501 - Lisa Mielniczuk, MD 12/30/2024

Jason Weatherald, MD - phaware® interview 500 12/24/2024

Jason Weatherald, MD - phaware® interview 500 In our 500th episode, Dr. Jason Weatherald, a pulmonologist at the University of Alberta in Canada, discusses a project called CRAVE (Canadian Right Ventricular Failure Adaptive Platform) to address the lack of treatments for right heart failure in patients with pulmonary hypertension. He explains that platform trials, which allow for multiple interventions to be tested simultaneously, are more efficient and offer a higher chance of receiving a potential therapy compared to traditional clinical trials. Dr. Weatherald emphasizes the importance of patient involvement in research and highlights the use of , which allows patients to measure their walking distance at home, as a potential endpoint in the trial. My name is Jason Weatherald. I'm a pulmonologist in Edmonton in Canada at the University of Alberta. My main area is pulmonary hypertension in terms of my clinical focus and my research program. I also practice as a lung transplant physician in Edmonton. I've been pretty involved in pulmonary hypertension research since about 2016 and was practicing before that for a couple of years. So, I'm coming up on 10 years of being in this area. I'm starting to work on a really exciting project called CRAVE, which is the Canadian Right Ventricular Failure Adaptive Platform. CRAVE is sort of a response to, I guess a gap identified by some of us in the pulmonary hypertension space that we've been really researching treatments for the blood vessel disease in the lungs, but there haven't been a lot of treatments for the consequences of it, which is stress on the right side of the heart and right heart failure. As opposed to left heart failure, which is a very common problem for which there are many treatments that have improved the prognosis of that disease, there really haven't been a lot of therapies that target the right heart and restore right heart function or help people who have developed a dysfunction or failure of the right side of the heart. So, one of the problems we identified was that, this is a less common problem and it's harder to study rare conditions. In PAH, we've done a pretty good job of that as a community developing multicenter trials across many countries, but that hasn't been done for right heart failure yet. We learned a lot of lessons through COVID-19 about how to conduct efficient clinical trials on a large scale. We identified that there's a type of clinical trial called a platform trial, and that's where you have sort of one common infrastructure for running a trial, but you can look at multiple interventions simultaneously, and you compare them to the control arm in the trial. From a patient's point of view, this is potentially more interesting, because there's a higher chance that you get on a new potential therapy rather than being on a placebo or the control. If, for instance, if you have four treatments of interest in your platform trial, there's a four out of five chance that any given patient gets randomly assigned to being on treatment. The other advantage of this is that because these treatments share the same control arm, it's actually more efficient to run a trial. What I mean by efficient is that you can arrive at an answer quicker, and if treatments don't work, you expose fewer people to a potentially futile therapy. I became really interested in this idea and this design of clinical trials during the COVID-19 pandemic, because these were rolled out on a very large scale. In fact, much of what we learned about how to treat COVID-19 and hospitalized patients came from these platform trials. So I was really impressed by our ability to do them and to arrive at answers efficiently. My colleague and partner on this project, Lisa Mielniczuk and I sort of talked about this idea. In fact, it's been almost two years that we've been working on this idea of the CRAVE platform trial. We've managed to kickstart it a little bit and sort of develop a mini version of this trial called a pilot trial where we just make sure that we can run such a trial and we can recruit participants into this trial to make sure that it's feasible to do this on a larger scale. That's the stage that we're at right now in the CRAVE Program, with a vision in the coming months to years to develop this project across Canada and probably also into the United States, so that people who develop right heart dysfunction due to many potential causes of pulmonary hypertension may have access to this type of a research program. Recently we held a meeting here in Edmonton, called the CRAVE Planning Meeting. The purpose of this was twofold. Number one was to engage people who are important stakeholders in this type of research, specifically the patients, but also the doctors and nurses who take care of the patients that would go into this trial, to understand a bit better about how they felt about how we were designing the trial, whether it aligned with their ideas of what was important to them. In terms of the things we're measuring in the trial, what do they think would be important for us to collect. We gathered with almost 40 people to talk about different aspects of how such a trial would be designed and conducted. In the Canadian context, we wanted to make sure that it was addressing specific barriers that some people have in this country to participating in research for a variety of reasons, whether they be sort of cultural or geographic. We want to make sure that people who are underrepresented in clinical trials and who have difficulty accessing clinical trials may have better access to this trial. There's a variety of things that we can do to sort of increase accessibility for people to participating in research if they want to. We wanted to engage patients and clinicians to understand those barriers a little bit better and think about ways that we can improve accessibility to the CRAVE trial, hoping to develop ways to minimize those barriers. So whether they be making some aspects of the clinical trial conducted remotely or virtually, and doing some of the assessments outside of the hospital. Also thinking about ways that we can let people outside of the big academic institutions even know about the trial. So, getting the word out there and making it easier for people to get access to the trial. So those are some of the types of things that we've been discussing before and after the meeting, and particularly during the meeting. The timeline for this whole project can conceivably last several years. What we're working on right now is this feasibility trial. So that's like a mini version of the trial that we're aiming to get going and complete within two years. We would recruit 30 people in that trial across several sites just to show what the problems are or the solutions could be to recruiting people. What are the some of the barriers from the patient perspective, and can we capture the data and identify people with this condition and get them into a trial and run it. That timeline, although two years sounds like a long time, it's really not a long time to design and start a clinical trial from scratch and to recruit and analyze the data from 30 patients. In parallel, we will be working on a couple of other things to inform and design the larger CRAVE trial. That's a platform trial that could involve several hundred patients and could run over several years. The important thing about platform trials is that they don't necessarily have a definite ending to them as long as new therapies are identified, they can be added to the trial and sort of plugged in as like a module in this larger trial perpetually. So, it could go on for many years as long as you have patients and funding to do the trial. I think it's a really exciting way to potentially look at multiple promising treatments for people with right heart failure and pulmonary hypertension. This CRAVE trial I think is unique and innovative in several ways in that we really aim to have patients embedded in the trial as partners and not just subjects of the trial. I think that is an important example of what we call patient-oriented research, where research really has to be meaningful to patients. I think the best way to ensure that research is meaningful, is to have them as colleagues and collaborators, having meaningful input from them at all stages of the research. Even before CRAVE, this actually comes from a project we did in Canada where we asked patients and their families, what do they think are the most important priorities for future research? CRAVE kind of spawned from that exercise where we identified from the patient's perspective, what are the top 10 problems that you think future research should focus on? Things that had to do with the right ventricle and right heart failure, there were two sort of questions in the top 10 questions that had to do with the right heart. So, I think involving patients even from the beginning about what is important to you that we focus on, and then getting patient input into the design of research and how we should conduct it, makes the results more meaningful to patients. It builds a partnership so that it's not kind of us and them, but it's us together developing research and conducting it. I think ultimately that will improve interest and buy-in from patients to make sure that they think that this research is important for them. One of the things that's important to highlight is, one of the aspects of this trial that we'd like to include, and it's directly relevant to , of course, is one of the things that is typically measured in clinical trials is the six-minute walking distance. We know that this is an endpoint that's used in clinical trials that regulatory agencies like the FDA and Health Canada will accept in terms of a drug or an intervention needs to show benefit on the six-minute walk distance because that's meaningful to patients, it's a measure of how they function in regular life. That's why we care about it in the clinic and in research. Of course, there's been this app developed from phaware called , which among its features includes the ability for patients to do their walk test at home and to do it more frequently. To me this is a really interesting endpoint in a trial that we could look at. So, one of our plans is to validate this app, and its measurements further in the context of a clinical trial so that hopefully it can be something that we can record in the larger CRAVE trial and look at how these interventions affect people not just at their clinic visits six months down the road, but how does it affect their walking time and distance day-to-day. I think this app that phaware has and that our colleagues and friends in have worked on, looking in the research area, I think it's a really exciting innovation. It's a good example of what I mean about trying to decentralize clinical trial activities as much as possible to record as much of the information that you need in a clinical trial outside of the hospital, making it more convenient for patients, but actually more representative of what's going on in their day-to-day life. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD #HeartWorksApp /episode/index/show/phawarepodcast/id/30380713

Episode 500 - Jason Weatherald, MD 12/23/2024

Tanya Stinson - phaware® interview 499 12/17/2024

Tanya Stinson - phaware® interview 499 Tanya Stinson shares her journey of being diagnosed with pulmonary hypertension (PH) after persistent symptoms and misdiagnoses. Her struggle highlights the difficulty of finding the right diagnosis and the emotional toll of living with a rare, chronic disease. Tanya’s experience underlines the fear, mental challenges, and hope intertwined in navigating PH, inspiring others to seek support and find solace in community, showing that even in isolation, they are not alone. My name is Tanya Stinson. I’m from a really small town in Manitoba called Saint Eustache Manitoba. It’s about half an hour away from Winnipeg. I was diagnosed about a year and a half ago with pulmonary hypertension. My family doctor thought for sure that it was a heart condition. I kept going to her and telling her, “I can’t breathe, I can’t breathe, I can’t breathe.” She kept saying, “Well, we’ll get you in to see the heart doctor.” My husband even came with me to a couple of appointments and he’s said, “It’s not her heart. She can’t breathe. It’s her lungs.” The doctor was like, “No, no, no, no, it’s her heart.” Being a woman of larger size, it’s automatically assumed it’s my heart, and I said, “It’s not my heart. It’s the fact that I cannot breathe. I can’t go from my bed to the bathroom, which is like five steps away, without having to stop halfway to catch my breath.” She said, “Well, let’s just wait. We’ll get you to the heart doctor.” I saw the heart doctor. Then, that weekend I couldn’t breathe hardly at all. I phoned her office on Monday morning and I said, “Can you please, please, please get me in? I need to see you. I cannot breathe.” So I went in. She put a pulse oximeter thing on my finger. She took it off my finger and she cleaned it and then she put it on her finger and then she put it back on my finger. She said, “I think this thing might be broken because you’re registering 71.” I said, “Well, see, I told you I can’t breathe.” She said, “Okay, well you need to go to the emergency room.” In my province, which is Manitoba, we have urgent care, and then we have three hospitals in Winnipeg. She said, “Go to what we call an urgent care, which is Victoria Hospital.” But she thought she was sending me to a regular hospital and that wasn’t the case. I got to this urgent care and they did my oxygen. Then, I had to wait for a special ambulance to take me to St. Boniface Hospital in Winnipeg. That’s where I met my doctor. The way I look at it is he knew the second he saw me almost what it was. But he said, “We have to do tests, and it’s already like 10 o’clock at night. There’s no way we’re going to run tests today, so we’re going to admit you.” I had the right heart catheterization. He came in the room. The look on his face probably scared me the most, because it was a look of very much of concern and almost sadness in a way. When I saw his face, I’m said, “Am I going to die?” That was the first thing that ran through my head. When he told me it was pulmonary hypertension, I said, “Oh, okay, so I’ll take a couple of pills and I’ll be fine.” He started me on medication right away. Within the first week, I already felt better. I thought, okay, the longer I’m on these meds, I’ll be able to breathe again. Then, I went home and I had a week off of work before I had to go back to work. It’s almost like I plateaued. My breathing wasn’t getting better and I kept thinking, I thought these pills would help. I thought they would cure me. Even though he told me it’s not curable right now, I felt at least I would be able to breathe. Now knowing, a year and a half in, that this is the best it’s going to get is scary in a way, but at least I’m not where I was a year and a half ago before diagnosis when I couldn’t even take five steps to go to the bathroom. Now, I can work without having to worry about anyone looking outside the window going, why is she stopped in the middle of the road walking into work kind of thing. I don’t have to worry about that. I can at least get myself into work and stop and take a break and then continue on. I don’t have to worry about people looking at me and saying, “Well, why is she just standing there?” I don’t have to pull out my cell phone pretending I’m taking a phone call in a parking lot so that I don’t look like an idiot stopping in the middle of the parking lot to just catch my breath. My doctor, because he works at the pulmonary clinic, he told me, don’t Google anything because it’s the wrong thing to do, but someone tells you not to Google something, you’re going to Google it. So, that was a bit scary. But then I found the website and that helped a lot. But in our province, there has to be more of us. We got more than a million people, so if it’s one in a hundred thousand, you figure there have to be other people, but you still feel really lonely because you don’t know of anyone. I was able through PHA Canada to find a PHA buddy and she has been a godsend for me. She’s answered so many questions and has been there to support me through the really difficult, hard days, whether it’s in relation to work, my breathing, just anything. If it wasn’t for PHA Canada, I don’t know where I would be as well as without my doctor. I sometimes will say to my husband, “If it wasn’t for my doctor, Dr. Christensen, I’d probably be dead,” because it was him who figured it out. It wasn’t my family doctor. It was my family doctor who said, “It’s your heart, it’s your heart, it’s your heart.” He was the only one that looked at me like a normal person, not as an overweight person, not as a female, not as somebody who’s all in our head. He actually listened and he knew. To me, without those two, Dr. Christensen and PHA Canada, I would probably be dead. Even just the overwhelming grief that comes with knowing that you’ve been diagnosed with a disease that you’re going to die from, and knowing that in the back of your head and having to deal with everything that comes with that is scary. But without those people, I don’t think I’d be here. Things would’ve been too tough. This disease is very challenging on multiple aspects of it and in multiple areas, whether it’s mental health or just with your actual physical health. Knowing that even though you’re taking the meds, I’m not going to get better. I’m going to actually over time probably get worse. So even though we’re trying the meds I’m on now, I spoke to him a little while ago and he says, well, we’re going to try the new medication that’s out, but we have to wait until we get approved. I think for me it’s the actual breathing and knowing that it’s only going to get worse, but then also having to deal with that mentally, being scared of when it does get worse. What’s my life going to be like when it gets worse? Am I going to have to stop working? Am I going to lose more friends? Am I going to lose family members, because they think I’m just faking not being able to breathe? I think it’s everything about this disease, but having the supports there from PHA Canada and from Dr. Christensen, that helps so much, because you know are not alone. There are people out there that understand you and can appreciate what you’re going through and help you get through that. Having someone who knows what you’re going through is extremely helpful. When first diagnosed, I heard the statistics is one in a hundred thousand, so you know that there has to be other people out there, but you have no way of knowing who those other people are. You have no way of knowing if you’re all alone right now, and if anyone is anywhere near you that you can talk to about what I’m going through is normal. Now, every person’s journey is different, but I think some of the same things apply to everyone. Everyone struggles with the breathing aspect. Is it normal that on these new meds that I still can’t breathe perfectly? That’s a question I had. I had at that time no one to ask that question to, and I didn’t want to appear stupid or dumb in front of my doctor. No one wants to do that. So, it’s like a question of, well, do I look stupid by asking my doctor is this normal, or is there someone around me that I can ask and not look stupid? On the medication I thought, great, I’m going to be able to breathe. But then to know that you’re not, but then to have that person there tell you that’s normal. It’s normal that your fingertips might still be blue. It’s normal that you’re going to struggle some days harder than most. At the PHA conference in 2024, it was in Calgary and I live in a prairie province. It’s very flat, very cold in the winter. Traveling to Calgary which has a lot of mountains and is very high up, I was worried about breathing. To be able to talk to my PHA buddy and PHA Canada and my doctor, they were able to tell me, it’s going to be okay. We’ll give you a little bit of supplemental oxygen and you’re going to be okay. But those are questions that if you’re all alone and don’t have that support system, you’re not going to know. You’re not going to have those answers, and it’s going to further put you, I feel, in a depression that you can’t climb out of without the support. For me, I’m hoping to be able to help other people because, in our province, there’s not a lot out there for people who have been diagnosed. What gets me going every day is knowing that I’m working with my PHA buddy and we want to set up a support group in our province to be able to help other people through this is what gets me going every day. Knowing that there are people out there that have been 20 years, even 10 years, my PHA Buddy’s been nine years. I know I’m not going to die necessarily tomorrow, and that makes me feel really good knowing that that’s not going to happen. To me, I’m still young. I’m only 50. I was diagnosed when I was 48. Knowing that I’m not going to die tomorrow is wonderful, because you do get that scary feeling. Like if you Google it at seven to 10 years. Well, I don’t want to die before I’m like 60. No one wants to die that early. Some people won’t live that long. But knowing that there are people that have is very encouraging and gives you hope, and hope is all you have to fight this disease some days it seems. Yeah, hope is important. I really hope that recording these messages helps a lot of other people. I know that there’s got to be people with PH out there that don’t live anywhere near a center, but maybe can listen to your podcast and get some sense of I’m not alone. There are other people who are out there in this world that are suffering, but hearing these words can offer them some encouragement. My name is Tanya Stinson, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946502

Episode 499 - Tanya Stinson 12/16/2024

Hall Skaara (Bel Air Center) - phaware® interview 498 12/10/2024

Hall Skaara (Bel Air Center) - phaware® interview 498 Hall Skaara, a resilient PH patient and founder of the Norwegian Association for Pulmonary Hypertension, recounts his 19-year journey living with idiopathic pulmonary hypertension. He shares how he transformed the challenges of the pandemic into an innovative opportunity by helping create the , a virtual global PH community space. The Bel Air Center aims to foster global connections, support, and resources for patients, caregivers, and healthcare professionals. My name is Hall Skaara. I am a patient. I was diagnosed with idiopathic pulmonary hypertension in 2005. So, I've been living 19 years with this disease, still doing very well. I founded the , because there was no association in Norway when I was diagnosed. Later, we became part of the European Umbrella Association. I started working more for them. Somebody else took over the Norwegian Association. Today, I do project work with . One of the exciting projects that I'm working on is something called the . This is a virtual PH community that we have created. Let me tell you about the name, first. Bel Air Hotel was a hotel we used to meet once a year outside Barcelona. Then, we had the pandemic, and we could not meet anymore. We thought, we need to meet virtually. We contacted a company that designed a virtual hotel for us. We made it into a virtual conference center. Here, we had our annual meeting with our general assembly meeting. We had the presentations, etc. Once the pandemic was over, we thought this was way too good to just scrap. So, we kept it and we developed it further. We removed all links to PHA Europe from it, because we want this to be a global PH community center not linked to any specific association. When you log in, you walk into a lobby in a hotel. I'm actually there to greet you as the receptionist. You can send me messages and ask me questions. Then, you move from the lobby into different areas of the hotel. We have an exhibition area for all the associations. So, each association in PHA Europe, but also other associations around the world that would like have a booth. In these booths, they have information about themselves. They can have videos. They can have documents that are uploaded. They can have links to their webpages, etc. Also, we have an exhibition area for pharma companies that work in the field of PH. You can go into their booth and see information about their products and about their company. We have live session room, where you can watch live sessions related to PH. Once the session is over, we put the recording into a recorded presentation room. Here, you can search for all these presentations by keywords. You can search by presenter name, etc. It's very easy to find a presentation that is fitting for you. We try to subtitle presentations there, but also we utilize dubbing. We use artificial intelligence technology to dub, so that the person who is presenting in English can speak Spanish or Chinese or any language. At the moment, we have Spanish, German and Croatian dubbing in there, but we can do much more. This is a little up to what the community wants and requests. We have an art room where PH patients and PH doctors have a provided us with art. You walk through a gallery and you can see artwork on the wall, and also videos. For instance, we have a PH doctor that plays piano. You can see this as an artwork on the wall. Then we have a social area where you can meet other people who are logged on to this virtual center. You can chat with them or you can have video calls with them. Also, you can be invited to meetings with other people there. For instance, after you've gone to a presentation, you can go to a social area, meet with other people that have been at the presentation, and discuss the presentation in there. We have an area for podcasts, and this is where phaware have provided us with all their podcasts. So this is a great resource for PH patients, carers and doctors. We have made this open to anyone. Anyone can create an account. You do this by going to . We've made this open to associations, too. They can create accounts in there as well as stands where they talk about their association. We have stands from all over the world. We have Japan, China, Canada, USA, most European countries, Nigeria from Africa and so on. We had our grand opening at the beginning of the year in 2024. We have had a steady increase in the number of people that joined this community. We hope to continue growing the numbers. Not only are patients welcome, but also carers, healthcare professionals, etc. We would welcome even more participants from the medical community and associations to join and make stands. Also, pharma companies. We hope to make this the leading virtual PH platform for pulmonary hypertension. My name is Hall Skaara, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946442

Episode 498 - Hall Skaara (Bel Air Center) 12/09/2024

Cynthia Neilson - phaware® interview 497 12/03/2024

Cynthia Neilson - phaware® interview 497 Cynthia Neilson shares her journey as a mother raising her daughter Claire, who was diagnosed with pulmonary arterial hypertension (PAH) at a young age. Cynthia recounts the challenges she faced navigating the medical system, the emotional toll of dealing with an unexpected diagnosis, and the difficulties of balancing family life while ensuring her daughter received the best possible care. Cynthia emphasizes the value of connection, resilience, and advocacy for families dealing with rare diseases. My name is Cynthia Neilson. I live in Toronto Ontario, and I've been there 20 years now. I've raised two kids there. I have an older daughter, Claire, who's 14 and I have a son, Owen, who's 10. My daughter was diagnosed when she was two. I really didn't have any connection or any knowledge of the disease, who to connect with, contacts, etc. I got involved with the Patient and Family Advisory Committee at Sick Kids Hospital in Toronto, which was great. I got to meet other families, but unfortunately I'm the only PH parent on the Advisory Council, because as you know, PH is a rare disease. I really enjoyed that sort of connection with other parents. Then, about a year ago I learned about the Pulmonary Hypertension Association of Canada. I was really interested in learning more and there was an opportunity to join the board. So I did that, which was great. That's been a really fantastic opportunity to meet other families who have varying types of PH. I always in my gut knew something about her was not right, but I was a first-time mom, doctors know best, et cetera. So, I sort of ignored that inner voice. Then, I took her to emerge for an earache. I had a med student examine her and they said, “Oh, her heart sounds sort of strange.” So, we went to and had an echo done. Sure enough, the tech said, “Oh, well, obviously she has a really large hole in her heart.” This was the first time that I had ever heard anything like that. I said, “No, I actually have no idea.” You could see this tech's face sort of go pure white and leave the room sort of suddenly. From there, it was a very quick referral into Sick Kids, where she was formally diagnosed, I don't know, probably like two weeks later. Everything moved really quickly. Then, we joined that clinic. They wanted to close the hole in her heart first, but the pressures in her heart and lungs were too high. We decided to wait a year and put her on oral medication and oxygen to hope that we could get some of those pressures to come down to do the surgery. A year later, she had an open-heart surgery. They repaired the ventricular septal defect. It was a pretty rough road. We were in the hospital for a long time and there was some complications, but unfortunately the PAH didn't resolve itself and we were looking down the path of what does the life look like with PAH? We got really involved in the hospital. As I said, I joined the patient and family advisory group. We used child life support. We just sort of figured it out our way. It wasn't the easiest path. Fortunately for me, I have a very healthy family. I've never had really anyone in my family that has chronic disease. I'm not sick myself. All of this was really new territory. I had just gotten pregnant with my second. I was going through this time of surgery being pregnant, which was also sort of an emotional rollercoaster, which didn't make things much easier. About six months before I was pregnant with my son, I had a miscarriage. At that point, I started questioning like, “Oh, is this because I have a daughter who has PAH? What's going on?” When I got pregnant with my son, we did do some fetal echo ultrasounds and some genetic testing just to see there was no sort of predisposed conditions, underlying conditions, et cetera. There wasn’t. I think the miscarriage was just an unfortunate, unrelated event and my son is healthy and does not have any sort of health conditions. We were fortunate that that was the case. But we did do a bit of investigation, because I was anxious. I wasn't confident that I could take care of a child with complex medical needs and then having another child potentially sick. I was still a fairly new parent. Obviously, my husband's hugely supportive and we were in it together, but it's still not easy. Neither of us grew up in Toronto, so we had no family support close by. My family's about four and a half hours away and my husband's is about three hours. It was a trying time when I look back, dark days. So she went to preschool. Both my husband and I work full time and that was sort of post-surgery. That was fairly smooth. By that point, she was getting ready to go to school. I would say the transition to school was a little bit rocky in the beginning. Claire, at that age, was still napping. She was much more tired than other kids. So, trying to negotiate with kindergarten teachers that she still needs a nap, they wanted her to go into a preschool room. Some of that when she was in the earlier years was a little bit trickier, but fortunately for us, she's more tired and can't sort of play as hard as the other kids. Physically, she's actually quite capable of a lot. In those early years through school, we actually had few concerns or issues with her being able to do what other kids were doing. She had to take medication at lunchtime, but I think there is a lot of kids who take a lot of different medication. That's pretty normalized in the school system now. As she got to grades 5, 6, 7, it became a period of time where she started identifying that she had some limitations. She couldn't run as fast, wasn't playing soccer, et cetera. It became a little bit harder. She started asking questions. As much as she knew as she had a hole in her heart, the doctor sewed it up and now she's better. She just takes medication to keep her better. I think that was sort of the age-appropriate conversations we had had. But by this time, she's starting to ask questions like, I'm still taking oxygen at night and like, oh, now I have another new medication and I'm tired and what is going on? I think that made things a little bit harder to just have some of those conversations. I would say that for the most part the teachers have been pretty responsive. I do an annual meet with the teachers, the gym teacher to say, when you say keep running and she stops, you can't be yelling in her face to keep running. Some of those conversations. For the most part, it's been pretty straightforward and easy. If I fast-forward to today, she started high school. I think the game is changing a little bit. I think with high school kids are just exposed to more, whether it's vaping, smoking, edibles, alcohol, et cetera. We're having to have some of those conversations around what is an issue. I mean, all of those things are an issue for everyone, don't get me wrong, but I think with someone with PH, inhaling smoke, it's a bit of a different game. I think having some of those conversations has been a learning curve for sure. One of the things we also tried to navigate was trying to keep her in activities and doing things like her friends and not being different but still being able to manage it. A good example is summer camp. She wants to go to summer camp like every kid. Day camps were never an issue. Once she got older, she wanted to do overnight camp. Overnight camps that allow oxygen are a few and far between. I must have called, I don't know, 10 camps and only one would allow us to bring oxygen. It was great, but she was the first time in the history of this camp that's been in existence for a hundred years that's had someone use oxygen or bring oxygen. So again, it's making sure that she has the right tools to be able to talk to her cabin mates about why she needs the bottom bunk, why she needs an extra plug. I think it's just trying to get her to a place where we can kind of shift some of that sort of conversational responsibility away from us and to her and that's a hard space to be I think right now. This particular camp that we ended up going to does have a full-time doctor and nurses on staff that are there 24 hours. They obviously have electricity in the cabins and were frankly quite accommodating. It was good. It's been a really good experience. She's gone for the last three years, but it's a really expensive camp. We're fortunate we can afford to send her there, but lots of parents can't do that. I think about what are the opportunities for those kids and being able to have some of those experiences? Fortunately for us, we've always had a very sort of open relationship with Claire. So no question's a bad question. She's just coming to us and still having those conversations. I'm really happy about that, because I think otherwise I don't even know if I would really know at what point I'm having what conversations. She was very open about is it safe for me to vape? Can I drink alcohol? We were able to give her lots of great... well, what I think is great advice, at least health advice. But I think some of those conversations, I don't know, I think are just going to evolve and I'm just going to keep my fingers crossed that she just keeps asking the questions. I think we're lucky Claire has had the same nurse practitioner her entire life, so it's definitely a safe space where the two of them can have a conversation and she can ask all the questions she needs to. I think we also rely on mental health support in the community. Having a person that she can connect to that's not one of us, and ask these questions, allowing her to have that space, as well, hopefully will mitigate some of these issues as they come up. There's no rule book to raising kids, whether it's a kid with PAH or a perfectly healthy kid. I think parents just have to kind of go with the flow and deal with what comes. PAH is a rare disease and the social networks are not like some of the other chronic diseases. I finally came to a place that I was ready to connect with other people who are living the same experience as me. Visibly, Claire looks like she's not sick. PAH can be a very invisible disease or an extremely visible disease depending on the severity. I was very worried that coming to some of these events or conferences or getting involved, it would be a look into my future and thinking this is what Claire's future holds. Very much keeping her protected from the community as well because I was she'd have the exact same experience. I remember when Claire was about five years old, I went to a provincial conference that was held. It was all heart conditions. I went and I saw all these visibly sick children and I had to leave right away. I said to my husband, “I will never set foot in another one of these places again. I just can't see this.” I think with time, actually getting involved in the community has just raised my level of awareness and understanding that I think I'm just ready. It just took me a while. I think I also came to the realization that Claire is now the age that she needs to make these decisions. Maybe for her actually connecting with kids who have something similar to her is quite therapeutic. I have up to this point made that decision for her, but it's time. She's older. I think that was part of it. I've been so fortunate. Our medical team and the community has been so great that it's just another way to give back. If my story or my advice or even just being there for someone else can provide support, then I'd love to be able to do that for someone else or another family. My name is Cynthia Neilson and I'm aware that my daughter is rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33946392

Episode 497 - Cynthia Neilson 12/02/2024

Natalie Roy - phaware® interview 496 11/26/2024

Natalie Roy - phaware® interview 496 Natalie Roy is a double lung transplant survivor living in Canada. She was diagnosed with pulmonary arterial hypertension (PAH) in 2005 and received her transplant in 2017. . After her successful transplant seven years ago, Natalie experienced a difficult initial recovery but felt amazing once she started healing. The first two years were cautious, but she was able to achieve many milestones she never thought possible, including getting married, playing sports, and changing careers. My name’s Natalie Roy and I’m living in Victoria, BC, Canada. I am a double lung transplant survivor. I was diagnosed with pulmonary arterial hypertension in 2005, and I received my double lung transplant in 2017. I did this podcast shortly after my transplant about six years ago, and I just celebrated my 40th birthday and have celebrated my seven-year lungaversary. I started fainting out of nowhere when I was about 19 and was diagnosed in the emergency room in Vancouver. I was diagnosed with idiopathic pulmonary hypertension at the ripe age of 20 and started oral treatment and did quite well with that right up until about 2010. My disease started to progress and I went on subcu Remodulin. Shortly after that, I transitioned to IV Remodulin and had that journey for about five years and slowly declined and was listed for transplant in 2015. I did wait for about 19 months for my transplant and got to experience a dry run. For people who don’t know, that is when they call you and tell you that they have lungs for you and prep you for surgery and you discover that your lungs aren’t viable and they send you home. That was quite the experience. I did quite well on my oral medication for many years. I was a chef for about 13 years. Just before I got listed for transplant, I worked pretty much my whole diagnosis. I did quite well on treatment. I participated in a lot of support groups. I found a lot of community amongst other patients in the PH world. I don’t think I would’ve survived without many of the other patients throughout Canada and the world. I think that community is really important when you are suffering. It wasn’t until I started Remodulin that I actually really, I think that was the grieving process actually really hit me square in the jaw. I was like, “Wow, I’m really sick. I am connected now to a device 24 hours a day and I need support.” That’s when I reached out and found the local support group in Calgary at the time. I was surprised with how almost desperate I was for that connection when I went to my first meeting. I didn’t realize how isolated I actually was and I was just kind of going through the motions and kind of really just living my day-to-day in denial of my disease and minimizing it, pushing through, and not really allowing myself to be somebody who was unwell. I think the support groups give you a space where you’re able to connect with other people who actually, obviously, understand what you’re going through. There’s only so much your caretakers and loved ones can relate. They sit beside you, but they don’t experience it with you. The online is amazing, but if you can find other people in person, I think there is something really beautiful to going and grabbing a coffee with somebody or talking on the phone or meeting up with somebody. I did run the one in Vancouver with Tarya, which was really amazing, and that was one of the most enriching experiences that I think that to date I was involved in. I think you need to be prepared to experience somebody else’s grief, that is part of showing up and holding space for somebody else and meeting somebody where they’re at, because somebody who potentially isn’t as progressed or as sick as you are, it could impact them. They might not be able to cope with it in a way that you’re able to cope with it. That was one of the things that I did notice, was that everybody has different coping levels. What you go through in your life, how you cope, how you move through things, some people haven’t gone through a lot of hardships in their lives and they get diagnosed with this disease out of nowhere and it completely rocks their world and they do not know how to manage. They find these support groups and they go there and their emotions are very big. I think that’s part of the rooms and part of the experience. If you are an empath or if you’re somebody who is sensitive, I think you do leave those rooms feeling quite exhausted. When you only have so many spoons, you definitely walk away feeling quite tired. When I got the call for the dry run, I was absolutely terrified and not ready. Sitting, waiting outside of the OR and I realized that it was a dry run, I felt extreme relief. When I was able to go home to my dog and eat a sandwich and go to bed, I was like, “whew.” I was very devastated the next day when I had to wake up and I had to mix my meds and I had to live life unwell and hooked up to a machine and my line got infected and I just had to continue on living life with PH and being sick. But by the time that I actually got the call from my lungs, I was ready. I felt safe and I felt cared for, and I think that was part of it. For me, I had always told myself like, “No matter what,” and I think I said this in my last interview, “do it scared. If it’s your choice, doesn’t matter. If you’re scared, do it anyways.” Just get in there and do it, because it is honestly the best gift I’ve ever received in my life. My initial recovery was quite difficult. I was in the hospital for about two months, but once I started recovering, that was it. I just wanted to live and my body wanted to live, and I felt amazing almost immediately. I was up and moving and working and back in the world. I think the first two years, if I’m being realistic, were cautious. I followed all the rules. I had a few bumps. There was a few complications. My donor lungs had a few issues. I had to do a few treatments along the way, but for the most part, I think for anybody who has been quite sick and has progressively gotten more and more ill, you can’t compare. Once you have the opportunity to take full breaths and you’re not in chronic fatigue and you’re not actively dying, no matter what you have, it’s no problem. You’re up, you’re moving, and you can push through it. When I’m asked like, “Oh, how was it?” I can’t even remember what complications or what issues I had. I actually can’t remember, because it really feels like it wasn’t a big deal. It just really was such a blessing and such an honor. I got married. I’m learning how to drive. I moved to cities. I’m doing things in my life that I didn’t ever think I would accomplish. I hit a milestone, which was turning 40. That alone feels like such a privilege. I think aging for people who are chronically ill is such a privilege. There’s so many things that have happened in the last seven years. I mean, every year, I think because my lungaversary and my birthday are a week apart, not even they’re a couple days apart, that it’s such a tender space for me and it’s such a dual celebration. It’s definitely a big honor to be aging. Every year, another year with my lungs, another year on this planet, another year celebrating and living and embracing life for whatever it is. Even the mundane stuff just feels like the best stuff. For instance, when we got married, it was like I was having before my transplant, we were having this huge wedding, everybody was invited. I had this whole thing planned out. Then, I had my transplant and I was like, “No.” We eloped. It was my husband, it was me, it was my best friend, and that was it. We were in the forest. I didn’t want anything else. It was interesting how before everything had to be big, involved, passionate, intentional. I absolutely live my life with intention now, that is the name of the game, but I feel like there was a desperation attached to everything I did before. Now, there is an ease, if that makes sense. What I get to experience now, even if it’s a fraction of what I tried to cram in before is enough based on the quality of my life. It doesn’t matter how much I get to do now, it’s how good it feels. Right now, I changed careers, so that’s been very all-encompassing. I went from working as a chef and now I am working in a corporate office. That’s been overwhelming at times. I feel like an imposter coming from somebody who couldn’t walk from the bed to the bathroom. Now, I’m running a little financial center and that feels strange to me, and sometimes I’m like, “How did I get here?” So I feel like that at the moment is like I am upgrading my education, which wasn’t an option for me. Yeah, right now I’m 40, but I feel like I got sick so young and then I was sick for 19 years. I got diagnosed 19 years ago, so a big chunk of my life was being chronically ill. I wasn’t able to make plans for myself, because my body was like, “No, we don’t get to do that.” Anytime I tried to make plans for myself, I would get unwell or something would shift and it just wasn’t feasible. So right now, I am working. Right now I’m trying to upgrade my education. We are buying our first vehicle and we joined a softball league. Who does that? I joined a softball league and played a season, and we got third. We went to the playoffs. I did a sport. I’ve never played a sport in my life. I’ve been sick forever. Those little things. So yeah, I don’t really have any plans. I really am just going with the flow, but the flow feels pretty good. I stayed connected to the PH community throughout. I didn’t lose touch at all. I do feel like I didn’t stay as closely connected as I wanted to. Going to the this year reconnected me closer, which felt really good. I have linked back in a little bit closer. I’ve joined their support group, so I’ll be going to that. I have my friends in the community and keep in close contact with them, but I really would like to do a little more volunteer work. I’d like to participate a little bit more in what they got going on with , but I feel like that’s where I want to put my energy moving forward, because that’s where I have the energy to do it. I had a hard time where I had identity crisis because I didn’t know where I fit into the community anymore after my transplant. What I did feel going to the conference this year and the feedback I did get was, “It’s really nice to see you here,” because a lot of transplant patients kind of move on with their life and they don’t really stick around, which is a beautiful thing, you go live your life. But those people saved my life and kept me alive and filled my cup and continue to do so. They are a family to me. I want to do a little bit more where I can. My name is Natalie Roy, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/33692787

Episode 496 - Natalie Roy 11/25/2024

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

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