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
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Tina Proulx - phaware® interview 478
07/23/2024
Tina Proulx - phaware® interview 478
Tina Proulx was diagnosed with pulmonary hypertension at the age of 19. She experienced difficulty breathing and chest pain, leading to a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), a condition caused by blood clots in the lungs. Tina eventually underwent transplant and celebrated eight years post-transplant, surpassing the average lifespan post-transplant. Tina emphasizes the importance of advocating for oneself in the healthcare system and encourages others to trust their instincts and fight for their needs. My name is Tina Proulx. I'm originally from Sudbury, Ontario, which is a northern city, but I've been living in Ottawa, Ontario, Canada for quite a while now. I've built roots here. I call this my home now. I used to have pulmonary hypertension. I had it for many, many, many years. I was actually diagnosed at a very young age. I was diagnosed at the age of 19. It all actually started when I was living in Toronto with my boyfriend, who is now my husband, but I was living in Toronto. We were going to college together. I noticed that my walk back and forth to school was getting a lot more difficult to do. I thought that was weird, given the fact that I do it every day. I thought it's got to get easier in some way. It struck me as being odd, and I was a little concerned. So I brought it up with my husband, and the amazing person that he is, and quite the joker, he thought perhaps I was just out of shape, and decided to take me for a jog. I could easily tell you that that jog was an epic fail. I barely made it out of the parking lot of our building, when I told him I couldn't, I was completely out of breath. At that point in time, I had decided, "Well, first, I'll take the first step and I'll talk to my family doctor," who sent me for a chest X-ray and diagnosed me with a mild case of asthma, which I'm aware of a lot of PH patients get diagnosed with asthma to start off with, so I'm not very uncommon there. He sent me off with some puffers and a few medications to manage asthma. It didn't take very long, I was probably on that medication for a few months before I realized that, "This is not working. In fact, I feel a little bit worse." I brought it up with my family doctor, once again, who told me, "Okay, well, if it's not working, stop." As a young person, I didn't really know too much about advocating for yourself in the healthcare world. I probably should have pushed the issue a little bit more, but he just took me off the medications and didn't look further into it. I walked away from that, just thinking that I probably had a mild case of asthma and that was it. One moment, in particular, that was the kickstart to a lot of stuff was quite a few months after that when I woke up in the middle of the night with some serious chest pain. I told my husband I couldn't breathe. I couldn't lie flat, I couldn't lie back down. It was very scary for me. I had told my husband, "I think I need to go to the hospital," and he agreed, and so we went that night. After several few tests that night, I think they called my lungs to be over-inflated, and so that seemed very strange to them, and so they decided to refer me to a respirologist. He's the one who thought out of the box, and I'm very happy that he did, because he was the one who actually decided to send me for a heart echo, in which they were able to determine that I did, in fact, have pulmonary hypertension. He, however, did not believe that I had primary hypertension, which is what we called it at the time. He thought that it was secondary to something. He started looking more into it. He had the impression that it was probably due to mesothelioma, a cancer, which struck me as being very odd, given the fact that I had never been exposed to asbestos, and it's called the asbestos cancer. He really did think that that's what it was, and so he decided to send me to see a surgeon where they could do a biopsy of my lung in order to confirm this theory of his. I was very fortunate that the surgeon had his own theories on what was going on, and before he decided to do a very invasive surgery, he decided that he wanted to send me for one test, which was a CT scan with dye. Lo and behold, they found hundreds of little micro clots, is what they call them. They filled up my lungs entirely, which is the reason why I couldn't breathe and the reason why I had pulmonary hypertension, and so it was him, at this point in time, who had finally diagnosed me with my full diagnosis of chronic thrombolic pulmonary hypertension. I always say that it's a mouthful to say. Chronic thrombolic pulmonary hypertension or CTEPH, what we like to call it for short, it's a version of pulmonary hypertension in which blood clots are in the veins and the arteries of the lungs, and are causing blockage, which is making it very difficult for the blood to easily flow through, forcing the heart to work a lot harder than what it was designed to do. It also makes it very difficult to push oxygen throughout the rest of the body. I always said that pulmonary hypertension or CTEPH is a lung disease that affects the heart, and so, essentially, my lungs were slowly killing my heart. As far as the PTE surgery, at that point in time, it was not something that I was being evaluated for. They wanted to first manage the blood clotting issues. They had no idea where the blood clots were coming from, so that was a whole other mystery. The PTE surgery didn't come into play until later on. At that point in time, they, immediately, once they discovered that I had this, sent me down to the thrombosis clinic, in which they were able to put me on blood thinner treatment in order to, at least, manage it from worsening or for any other blood clots to form and make their way. Unfortunately, that took years for us to finally come to a treatment that worked well for me. Many emergency hospital visits where I got new clots that came to my lungs, which made it even harder and even worse. So it took quite a bit of time before they finally found a treatment that actually worked for me as far as blood thinners go. The PTE surgery did finally come into play. I was evaluated more than once, and the reason why I did not qualify was because my clots were baby clots, and so they were very, very deep into my lungs, and almost impossible to get to. On top of that, I was told that even if they were capable of getting the clots, because they had been there for such a long time, there was so much scar tissue that had built around it, that it probably wouldn't have helped me as far as my pulmonary hypertension went, because there was a lot of scar tissue there that would still have caused blockages and still would have caused some issues for my heart. I'm actually very fortunate. I was pretty stable for a very long time, aside from them trying to figure out my blood thinning treatment. I didn't try too many treatments as far as pulmonary hypertension goes, because there weren't a whole lot that were approved here in Canada for CTEPH, in particular. Ao there weren't a whole lot of options for me. As far as like what it was like as a young person, obviously, very overwhelming, to the point that once I was officially diagnosed, and once I graduated from college, and I do got to say, my boyfriend, at the time, who is now my husband, we've been together for a very long time, we're high school sweethearts, and so he's been through it all with me, he was the sweetest person in the world, starting at a very young age, because he actually quit school, so that he could help me finish my schooling, because he knew it was getting very difficult for me. I finally graduated. We decided that it was probably best, because we were young, to move back home with our parents, so we could figure stuff out, which is what we did. We stayed there for a few years until we came to a point where we're like, "Okay, this is our life. I'm stable. We need to start living, and start learning how to live with pulmonary hypertension." Not always the easiest thing but we, certainly, weren't going to let pulmonary hypertension get in our way of still achieving the kind of life that we were hoping for ourselves. Right? That's when we decided to move to Ottawa. Yes. It was very overwhelming, but, at the same time, I always say that everything that I've been through made me the person that I am today. I'm very proud of that person. I think, obviously, I would have preferred not to go through that, but, at the same time, it made me a stronger and a better person. When you live with a chronic illness for such a long time, you're accustomed to not feeling all that great, and so you just push on, which is pretty much what I was doing until it was my husband and my mother who had told me that I didn't seem right, that I seemed like I was a little worse. So it was under their direction that they had suggested I go see my hematologist, because in the past, the thing that had always been the issue was the blood clotting issue, and so I thought that going to see my hematologist would be the best bet. It was her, who had sent me for a few tests. She had discovered in doing those tests that my heart was actually much larger than it was from the last test, and that it seemed like the pulmonary hypertension had gotten worse. She immediately contacted my PH team here in Ottawa, and I was able to see him shortly after. They started to get the ball rolling with regards to being able to figure out what was going on, how could they help me, and so, at that point in time, I was told that my lung pressures were at 125, at this point. They told me that, at this point, it was a progressive disease, and that I was getting worse, and that my only hope for survival, at that point, was a double lung transplant. People always ask me what it was like to be told that. Honestly, it's very overwhelming. t's even hard to describe but you feel very helpless, because, at this point in time, you've reached the point where there's nothing that you can do in order to help this situation. You can't eat healthy. You can't do any exercise. There's really nothing more that you can do. You have to go for the surgery, if you want to survive. It's a very emotional period, but I would say the best way to describe it is I felt very helpless. In any case, I've always considered myself to be a fighter, and I certainly was not going to stop at that point. Of course, when they asked me if I wanted to pursue going through with transplant, the answer was a hard yes, because I was not ready to die. I think it's important to note that when you're waiting for transplant, you have to be close to the hospital. There's no center here in Ottawa that performs lung transplants, and so we actually had to move to Toronto, back where it all started, in order to wait for transplant. When you're already dealing with so much, you have to deal with this additional thing where you have to find a place to stay and you have to figure out how you're going to pay for that place, because we both took leaves of absence from work, unpaid. There's a lot of the additional stresses that come with transplant. Just thought I'd mention that. While waiting for transplant, obviously, as it's a progressive disease, I got worse, so it came to the point in November of 2015 where it was no longer safe for me to be out of hospital. At this point in time, my husband was pushing me in a wheelchair everywhere. I couldn't tie my own shoes. I always say if I dropped something on the ground, it stayed there, so our house was a little messy here and there just because I couldn't bend over, because I would be at-risk of passing out if I did that. Sleeping was becoming beyond uncomfortable. Everything. I was beyond uncomfortable. I know my husband had said, "Near the end," which means near the end before transplant. My husband said that my heart was beating so fast and so hard, that he could hear it when he was at the other end of the room. Just to paint a picture as to how bad it actually was. I was not in a good place at this point. The doctors had decided that it was best to admit me, obviously, and what they did was they wanted to initiate what they called Plan B. Plan B, they had mentioned, to me, when I got listed, that it might be something that they would have to do. I was hoping we wouldn't have to, but, unfortunately, we did. They had to hook me up to ECMO, which is an artificial lung. They said it was important to do this, because my heart needed a break. I was under the impression that it was going to go through the groin or in the neck, but my doctor said not for me. What they had to do was they actually had to hook up the ECMO directly to my heart, and so that required open heart surgery. So the ECMO was pretty much attached to my heart, and then I had a giant bandage here while the machine was attached to me. I was on that machine for six days when they finally came into my room to tell me that they had found a match for me. I wasn't on the machine for very long, but it was still quite the experience, and because of that, I always say I have two scars, one that goes horizontal and one that goes vertical. A T for Tina as I say. I may have an additional scar, one that I wasn't expecting, but both these scars saved my life, and so I keep them on me and I'm very proud of them. I just actually celebrated eight years post-transplant, which, to me, is quite the achievement. When I was first told that I needed a lung transplant, they had told me that the average lifespan post-transplant is five years. Now I believe they tell people eight years, which is great. We're moving up. Hitting eight years, to me, is like a milestone, because I know that that's what they tell people now. I would like to continue, year after year, hitting these milestones, because, to me, it's just a representation of the years, the days, the hours that I never would have had had I not had a donor. To me, it's very, very important to recognize those moments. I also just celebrated my 40th birthday, which, again, to me, very, very exciting whereas a lot of people, they may be scared of that number but, for me, it was something to rejoice in, because I didn't even know whether or not I would see that birthday. To me, it's like I thrive in growing old, because I never knew that I would be able to, and so every year that I get older is just something exciting for me. Since my transplant, I can easily say that I've been able to do so much more, even so much more than what I was able to do when I had pulmonary hypertension and I was stable. I've been able to do something that I thought I would never be able to do, because when I was diagnosed with pulmonary hypertension, I was told I wasn't allowed. I love rollercoasters. So post-transplant, I was finally able to go to Wonderland, which is a theme park in Toronto. I was finally able to ride the rollercoasters again. I'm not going to lie, I bawled the entire time. They were happy tears, but it's like I celebrate in those little moments, those moments that so many people take for granted, and don't realize how lucky and how precious those moments are. I'm also an adventurous person. I am extremely happy that I've been able to let that adventurous side of me out. Since I've had my transplant, I've walked the CN Tower, the Edge Walk of the CN Tower, I've been ziplining, I've been white water rafting. I'm trying to convince my husband into going skydiving but we'll see. I've always been that kind of person, and so, to me, it's a very exciting thing to be able to live up to that little girl in me that always wanted to do those things. Now my husband and I, in our spare time, we run the Ottawa Gift of Life, which is an advocacy group here in Ottawa, in which we advocate for organ and tissue donation. We run that in our spare time. I do a lot of charity work outside of that. I'm part of Ontario Health patient and family advisory board. I'm doing my best in order to give back. I think it's important to take the time, and help others. I believe I was always that kind of person, but I think going through something like this only emphasizes that person in you, and that person just wants to come out. Right? I so desperately just want to be able to help as many people as I possibly can. When I look back, and if I could tell my younger self something, I think it would be if you're unhappy with something, then just speak your truth. Just say what you need to say, because nobody knows you better than you. If something is wrong, then make sure you trust your gut and you fight for that. Advocating in the healthcare system is so important. I think many patients struggle with that. Don’t be scared. Speak your truth and fight for the things that your gut is telling you, because too many people ignore that gut feeling and sometimes that gut feeling is actually telling you something that you need to know. My name is Tina Proulx, and I'm aware that I am rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 478 - Tina Proulx
07/22/2024
Episode 478 - Tina Proulx
Tina Proulx was diagnosed with pulmonary hypertension at the age of 19. She experienced difficulty breathing and chest pain, leading to a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH), a condition caused by blood clots in the lungs. Tina eventually underwent transplant and celebrated eight years post-transplant, surpassing the average lifespan post-transplant. Tina emphasizes the importance of advocating for oneself in the healthcare system and encourages others to trust their instincts and fight for their needs. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Marilyn Hanft - phaware® interview 477
07/16/2024
Marilyn Hanft - phaware® interview 477
Marilyn Hanft, a CTEPH patient, shares her experience with the condition. In 2011, she initially mistook her symptoms for asthma but later discovered she had clots in her lungs. Despite her health challenges, she remains determined to live life to the fullest. Hanft discusses her love for sailing and her current plan for writing a book about her sailing experiences. My name is Marilyn Hanft. I'm a CTEPH patient. I live in St. Marys, Georgia, but I'm from Maine and lived most of my life in New England. In 2011, I was around all this foliage that was all blooming, and I'm not used to that. So I kept saying, "It's my asthma. It'll get better." It didn't get better, so I finally gave in and went to the emergency room and discovered that it wasn't my asthma. It was clots in my lungs. It showed up. They did an echocardiogram, and the high PA pressure showed up on it. My doctor was not good. He didn't even tell me about it when I was discharged. Because of that, I went home. I was home exactly a week. Woke my husband up and told him to get me an ambulance, and he was kind of, "Well, how do I do that?" I said, "911," and he sat up and said, "For real?" I'm like, "Yes." Was about two and a half minutes before they got here. All in my head I thought, "Just give me some oxygen, and I'll be fine," which they did right away. I evidently looked really bad, because we went to the hospital, lights, and sirens, and weaving in and out of traffic. They took me into the code room when I got there. I was about probably 15 minutes or so from respiratory arrest. They did a chest X-ray when I first got there, and I had more clots in my lungs. My right lung was what they call a whiteout on the X-ray with pneumonia on that side. They shipped me up to the ICU. I remember getting in the bed, and that's all I remember that day. They put me on the BiPAP, and when I took a breath once they had it all set, my reaction was, "I can breathe." That night, the doctor that was on came in the room, and that was when I kind of checked back into reality. She told me that I wasn't getting worse, but I wasn't getting better. They thought I'd need a ventilator, so she needed to ship me up to Brunswick, Georgia, which is about thirty-five miles away. It's the bigger hospital. They had the pulmonology group who took care of me up there. When the doctor said I might need a respirator, I was an RN for 40 years, and I did a lot. My major areas of practice were neonatal intensive care, ICU, CCU, and the emergency room. When she said put me on a ventilator, and I'm thinking, "You're going to put one of those tubes down my throat. No way I will do this." You know? That was when they started giving me, number one, told me that I had pulmonary hypertension. They couldn't figure out why it wasn't stopping. I was still having clots. Finally, they did a, I don't know, whatever genetic testing they have to do, and one of the docs came in, "We figured it out." I said, "Well, what is it?" He answered a question with a question, said to me, "Who in your family died of a stroke?" I said, "I think maybe my mother's mother did." My grandfather had multiple TIAs, my mother's father. He actually died of a pulmonary embolus. My mother had started having the same TIAs, which affected her more and more as far as speech and cognition. She had to have pureed foods, which she hated. She finally decided that that was it. She didn't want to be around anymore. She died three days before her 84th birthday. The TIAs didn't cause her death, but everything was related. I was in the hospital up in Brunswick for 11 days and got the kind of care and discharge, et cetera, where they were like, "Oh. You're going to need a walker. You're going to need this," and they kept saying to me, "But your Medicare will pay for it." I said to them, "No. It won't, because I'm only 63." Finally, we figured it all out. I came home and had to deal with massive frustration, because I couldn't do much. I had been out of the house basically for the month of June. My oldest sister came and helped. One of the most important things to me, my older sister's five years older than me, and since we've been adults, more than when we were kids, we are extremely close. The first night that I was in the hospital, she called, and she has called me every night since. That was 2011. It's been beneficial for both of us, because she was going through a lot with her husband. I had a husband who dealt with my diagnosis with anger, so things were a little difficult. But she was the first one that got me to realize that I almost died, because she said it. At one point, I was crabby because I can't do this, and I can't do that. She calls me May, and she said, "May, you almost died. Give yourself a break." It stopped me, and I said, "I never thought of that." It never occurred to me that I might die. It was, “Give me some oxygen, and I'll be fine”. That really kind of stopped me, because I hadn't thought about it. I said, "Please, God, I'm not ready yet. I've got things I want to do." I've been doing them pretty much for, what is that, 12 years, something like that? Unfortunately, my asthma flared recently. It's been getting kind of worse. I'm allergic to oak trees. I have a huge live oak in my backyard, which I love. I love the tree. I have a couple of laurel oaks in the front yard. There are a bunch of other live oaks around. So when that's shedding its leaves and stuff, I have trouble with my asthma. Recently, a year or two ago, I developed an allergy to pines. I have 13 Southern yellow pines in my front yard. I've been having a little trouble with my asthma, and because of that, back in 2019, I had to go on oxygen all the time. I'd been using it at night most of the time, but I had to use it 24/7. I've never gotten off it. It annoys me. It frustrates me, because I can't go as fast as I want to, and I can't do things a lot of times that I want to do. I've had to deal with that. That's been kind of hard. I can't do any of the things that I loved, hiking, camping, that sort of stuff. I already couldn't ski anymore, because of my knees and stuff. I suppose I would not have been able to do it because of my breathing. The biggest thing and the most difficult to deal with was that we came to St. Marys on our boat. It was a thirty-six-foot motor sailor. It means it has full sails, but it has a ninety-horse diesel engine. We came down the Intracoastal. We had friends here, so we stopped to see them. We kind of fell in love with St. Marys. It's a beautiful little southern town. It's on the St. Marys River. The waterfront there is all older homes and stuff like that. It's very nice on the waterfront. Although Irma tried to destroy it, but we really, really liked it here. So anyway, my husband bought a house. We pulled into the dock at 6:30 in the morning on December 31st, 2010. What we didn't know was that was the last time we used the boat. It had repairs that needed to be done. I got sick. My husband's hips gave out on him, and we just never could do anything with it. It spent about 10 years up on land. My husband was going to try to work on it, and he just never could. He died in 2021, so that was hard. That was the biggest thing that my health has taken from me. We had a lot of good times on that boat. I've just started on a book about sailing. It's kind of a memoir/guide. It's a lot of stories about things that happened to us, good and bad. The working title is Three Boats, Four Dogs, and a Cat: 38 Years of Sailing the New England Coast. I've always wanted to write a book, so now I'm writing a book. I have to remind myself to keep at it, because I get caught up in doing housework and that stuff, and I get tired. What most people don't realize is writing is hard work. It really is. I belong to the Yacht Club here in St. Marys. We are a small group, and we're getting older. There are quite a few of us who no longer have boats, but we have get-togethers. We have coffee every Friday morning. In talking to them and telling stories and that as we all did to each other, there are some people that kept saying to me, "You tell the stories so well, you ought to write a book." Finally, I said, "Yeah. I ought to write a book." So I actually read a couple of books on how to get yourself going, and then I took a two-day seminar. It was wonderful. I learned so much. It just reinforced that idea of, "Yes. I want to do this." It is basically stories, things that happened to us, how we dealt with them, the times we had lobster right off the boat and cooked it a couple hours later. Oh my God, it was good. A lot of stories, basically. We'll see what happens. I never expected to do it when I was 76. However, it's a bucket list item that maybe I can get done. Wish me luck. My name is Marilyn Hanft, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 477 - Marilyn Hanft
07/15/2024
Episode 477 - Marilyn Hanft
Marilyn Hanft, a CTEPH patient, shares her experience with the condition. In 2011, she initially mistook her symptoms for asthma but later discovered she had clots in her lungs. Despite her health challenges, she remains determined to live life to the fullest. Hanft discusses her love for sailing and her current plan for writing a book about her sailing experiences. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
/episode/index/show/phawarepodcast/id/30375638
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Scott Fleetwood - phaware® interview 476
07/09/2024
Scott Fleetwood - phaware® interview 476
Scott Fleetwood, a former pulmonary hypertension patient from Buffalo, New York, shares his journey with rare diseases. He initially experienced shortness of breath while playing hockey and was later diagnosed with pulmonary hypertension and scleroderma. Scott received a double lung transplant in 2017. He discusses the initial fear he felt upon learning about his condition and the importance of finding a doctor you trust and following their instructions. He acknowledges the challenges and risks associated with lung transplants but highlights the positive outcomes he has experienced, such as walking his daughter down the aisle and becoming a grandfather. My name is Scott Fleetwood. I am from Buffalo, New York. I am a former pulmonary hypertension patient following a lung transplant in Pittsburgh. I also have scleroderma, so I have a couple of rare diseases that I'm aware of. I was playing in a media hockey game and one of the guys asked me to stick around so he could take shots on me because I was a goalie. After a while, I was really slow to get up and having a hard time catching my breath. He said, "Hey, Fleets, you're out of shape. You got to lose some weight or something." One thing led to another. I saw a doctor. Eventually, we got around to seeing a pulmonologist and he then suggested the upper right heart catheterization and that's when I found out I had pulmonary hypertension. Matter of fact, one of the guys I play with, his dad was a heart surgeon and actually had looked at my chart. He was the one who said I do have scleroderma. I said, "well, yeah, I know, but I'm just losing my breath all the time. I can't catch it. I can't walk from my truck to the locker room without huffing and puffing." So that led to seeing a pulmonologist and going through the battery of tests and things that your pulmonary doctor will do with you. I was out of breath. I was also overweight and I didn't eat so great. But all things considered, I look back at it now and I'm going, "Well, maybe I should have picked up on it." But it's not something that's in the front of your head that it's something terrible that's lurking. After I saw the pulmonary doctor, he recommended that I see a PH specialist here in Buffalo. I saw Dr. Gibbons. He then eventually recommended that I get a lung transplant. It was up for me to decide. I ended up going with my gut feeling and choosing Pittsburgh. When I got to Pittsburgh, I spoke to Dr. Marc Simon, who is a cardiologist. He gave me a bunch of drugs to try while I was getting upper right heart caths. He found that a lot of these different families of drugs that I was trying just weren't working on me. Eventually I ended up getting on the list and receiving my double lung transplant on August 30th, 2017, which is a trip of about five and a half years from me going, "Oh, I'm really out of breath," to getting the lung transplant. I remember when I first got my phone call from my pulmonologist telling me that he got the results back from the upper right heart cath and he told me that I had pulmonary hypertension. I did the thing you're not supposed to do. I Googled it. The funny thing about Wikipedia is that it's all in the way you phrase things, especially when you're scrolling on your phone. They said the life expectancy of a person with pulmonary hypertension is one to two years, but I didn't scroll to, "If left untreated." So I didn't see that when I first read it and I go, "Oh my God, I'm going to die." Just for your own information, that's been fixed. It's backwards now. If left untreated comes first. I got that initial scare and I was really surprised at myself that it really didn't bother me a lot. I've lived a full life already. I've had a hundred different jobs that I loved where you didn't actually have to work for a living. I've had a great life, a wife, a daughter. I guess it was I was more concerned for their wellbeing than my own, you might say. After I got that initial reaction, I started working with Dr. Gibbons and immediately got hope that this is something I can deal with. This is something that's manageable. On the other hand, it's that cannula hanging off your nose all the time, the leash you're on when you're home, because you're tied to an air compressor. They're not a big hassle, but you go traveling, you got to bring six batteries for one air flight. It's an inconvenience more than anything. I got kind of used to it, maybe a little annoyed with it. We got to the point where the drugs just weren't working and my weight was 290. My doctor actually while I was in Buffalo, he transferred me to UPMC and I took a nice two hour long ambulance ride to Pittsburgh. You're sitting there in the back of the ambulance and talking to the guys that are with you and so on and so forth. Then you get there and then it's like one of those point of view scenes in a TV show where it's like you're almost like you're in St. Elsewhere or something where you see the ceiling and you don't really see anything. So, that kind of is a little bit on the scary side. Then eventually when they got around to doing the diagnosis and recommending that I get a transplant, that's when the reality of things really kicked in, because I never really thought of a transplant as being something that was going to be in my future obviously. I mean, even I remember when my doctor told me I had pulmonary hypertension, I said to him, "So, how long do you think I can keep working for? He said, I'm surprised you lasted this long. You're going on disability right now. You're done. I'm pulling the plug on you." A lot of it was I didn't really have a lot of choice in the matter. A lot of it was this is the way it is. You're going to retire. You can't work anymore. Even after the transplant, the doctor said, "You're done playing hockey." I was hanging on for hope that she was going to let me play again. Then finally she said, "Okay, as soon as COVID is done, you can go back to playing." Then I took a bone density test and found out that I had osteopenia and she said, "You're never playing hockey again. Get rid of your equipment." So, I did. I'm going to be a cheerleader here for UPMC. This is kind of like the place you go to and nobody else will look at you or talk to you or discuss with you your situation. I met a lot of incredible people. It's kind of like the last resort , I guess you could say, for anybody who needs to get this done. How they do things there is they'll bring you in on a Monday morning and sometimes you'll have tests lined up. You'll have tests lined up for Monday through Wednesday. You're going to get breathing tests. You're going to get a tube shoved down your nose and they're going to check for acid reflux. There's this whole battery of tests and things that you go through that's all part of the process of the evaluation. One of the things they also do is they have this little meeting on the first day and they take you to this big conference room, and you're there with other patients and other caregivers. Some people, there's four or five people for one person. For me it was just me and my wife. They will spend the next hour and a half, two hours scaring the bejesus out of you by all the ways that you're going to die because of this lung transplant you're going through, everything from rejection, to things happen in the operating room, to infection, to kidney failure and all the other things that come along with it. They make you well aware of all the things that can go wrong. So, then they do a little break and they break for lunch and afterwards about half the people don't come back. It's not for everybody. And as a matter of fact, just before we broke for lunch, my wife said to me, "Are you really going to go through with this? I mean, this sounds pretty scary." And I says, "Well, let's put it this way. They can give me a perfect set of lungs that fit perfect. They don't have to shave anything. They just drop them in. It's a perfect fit. They can sew me up, not even a scar. I go outside and I get hit by a bus." Anything can happen to anybody at any time. For me, it's just a matter of what are you willing to accept as a quality of life. For me, I didn't want to do that anymore. I didn't want to have the hose in my nose anymore. I didn't want to be restricted on what I can do and so on and so forth. I'm still restricted. I mean, everybody's got restrictions, but not like it was when I had PH. There's no signs of PH, but there's no guarantee it's gone forever. There is always a chance that it could come back. I was a difficult case, obviously. I'm not exactly a poster child for pulmonary hypertension or scleroderma. First of all, I'm the wrong sex. I'm too tall. All the criteria, I'm just on the edge or fringe of everything. For somebody who's 6'1", 6'2" to get a double lung transplant, they get excited about those things because now they've got something to do with these big lungs they get. Because you can always put smaller lungs into somebody. You can't put bigger lungs into somebody. It's also an advantage too if you're getting a lung transplant. Numbers are in your favor, so to speak. As far as the scleroderma goes, it's a factor that my transplant team is aware of. As a matter of fact, my transplant doctor, she recently left UPMC to go to run the program at University Hospital in Cleveland. She obviously, I still see Dr. Domsic in Pittsburgh for my scleroderma, and I see Dr. Kilaru in Cleveland for my transplant. So, I stay in touch with both of these ladies, tele-visits once a year and in-person visits once a year and things like that now. But we stay on top of the scleroderma. I'm on two or three drugs that, like many of the drugs PH people are on, it wasn't developed for PH, but it ended up helping people with PH, so, that's why we get on these drugs that we get on. To sum it up for people, I would say make sure you get a doctor you trust. Make sure you get a doctor that you're comfortable with and you can work with. I've been very lucky with my doctors. They've been one to recommend to somebody next in line. Like the one pulmonologist recommended, Dr. Gibbons, they're not even in the same group. It wasn't a matter of recommending some PH guy. It was like he recommended the PH guy, that sort of thing. So, I've been pretty lucky with all my doctors in that way. First and foremost, once you find a doctor that you're comfortable with, do what they say. Just do what they say. Don't argue. You can question, you can discuss, but just do what they tell you to do. Because I see so many things on Facebook and stuff where people just decide that they want to do things their own way because they think they know better. That 12 years of education that they go through doesn't mean anything to anybody apparently. So, I would recommend highly that you follow your doctor's instructions. The other thing is get into a group. I'm involved with there's a UPMC group for post-transplant people. There's a bunch of groups for PH. Find a group that you're comfortable with. I like the PH Men's PHight Club and I'm involved with the Western New York PH group. Even though I don't have it anymore, I stay there in case anybody does have questions about lung transplant or anything else. I just started a transplant group for University Hospital in Cleveland for their program. Get into a group on Facebook or into a group where maybe they meet. I know here in western New York, they meet in public. Sometimes they meet on Zoom. Sometimes they meet face-to-face. Use those people to your advantage. I know when I was talking to a couple of PH guys, whether it be in England, or in Ireland, or wherever, where we would just talk and chat. Some of us are still friends. Some are gone. But it's good that we can talk and we learn from each other. I hope it makes the journey better for everybody, especially to have somebody who has taken that route. It makes the journey maybe not better, but a little easier. It is scary. I understand. It's not for everybody. Not everybody can get a lung transplant. That's definitely true. But if you have the wherewithal and can do it, I have walked my daughter down the aisle and I'm going to be a grandfather in May. None of this would've been possible without the care of the transplant teams and to be honest with you, the other transplant recipients before me. Because if it wasn't for the people that took those big steps back 30 years ago, we wouldn't be where we are today, whether it be with pulmonary hypertension or lung transplants or scleroderma or whatever. It's huge. My name is Scott Fleetwood and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 476 - Scott Fleetwood
07/08/2024
Episode 476 - Scott Fleetwood
Scott Fleetwood, a former pulmonary hypertension patient from Buffalo, New York, shares his journey with rare diseases. He initially experienced shortness of breath while playing hockey and was later diagnosed with pulmonary hypertension and scleroderma. Scott received a double lung transplant in 2017. He discusses the initial fear he felt upon learning about his condition and the importance of finding a doctor you trust and following their instructions. He acknowledges the challenges and risks associated with lung transplants but highlights the positive outcomes he has experienced, such as walking his daughter down the aisle and becoming a grandfather. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Robert Frantz, MD - phaware® interview 475
07/02/2024
Robert Frantz, MD - phaware® interview 475
Dr. Robert Frantz, a cardiologist at the Mayo Clinic, discusses current and upcoming clinical research in pulmonary arterial hypertension (PAH). He emphasizes the low participation rate of PAH patients in clinical trials and encourages patients to consider participating, as it not only benefits themselves but also helps advance medical knowledge. I am Dr. Robert Frantz. I'm a cardiologist at the Mayo Clinic in Rochester, Minnesota and Director of the Pulmonary Hypertension Clinic there. Today, I'd like to talk a little bit about what's in the pipeline in clinical research in pulmonary arterial hypertension. The concepts include inhaled therapies that aren't really acting as vasodilators, but maybe anti-proliferatives with maybe some anti-inflammatory effect. This includes inhaled imatinib. Imatinib is a tyrosine kinase inhibitor that is known as GLEEVEC in the cancer world, but which had been shown to drop pulmonary vascular resistance in patients with pulmonary arterial hypertension years ago, but never came to market for that indication, because there were too many side effects with the oral administration, and there were rare cases of cerebral bleeding in patients that were on anticoagulation. However, the concept of inhaling the imatinib, where then you're delivering the drug right to where it needs to be, will hopefully lead to fewer off-target effects, because the blood levels are quite low. So, the IMPAHCT study is looking at this inhaled imatinib and just finished the Phase 2 study and has sort of a seamless integration of the Phase 3 that launched immediately after the Phase 2 completed. That's very interesting and it will be exciting to see where that goes. Continuing on the theme of inhaled therapies to try to avoid systemic side effects, there's inhaled seralutinib like que sera sera. What will be will be, but we want to change PAH. This is a compound that was designed to have some of the effects of a drug like imatinib, but even more powerful at some of the receptors that imatinib hits, and also to hit a pathway on activated macrophages, which are around the blood vessels and can cause inflammation. So, that's the colony stimulating factor 1R inhibition aspect of seralutinib. Seralutinib was shown in Phase 2 studies to reduce pulmonary vascular resistance. So, if that could occur in patients with PAH in the Phase 3, then it should be associated with improvement in six-minute walk. So, the Phase 3 study, PROSERA, is randomizing patients with PAH to either inhale seralutinib or a placebo. The end-point is six-minute walk. See if we can make people function better that way. So that's also a really exciting thing that's going on. Now, to change tack a little bit, some patients have what we call combined pre and post-capillary pulmonary hypertension, where the pulmonary artery pressures are high, but maybe the left side of the heart is stiff in how it fills, what we call heart failure with preserved ejection fraction. That then backs blood up into the lungs and back into the pulmonary arteries. Some people, they have more severe pulmonary hypertension that's causing the right ventricle to fail, as well. So, the left side of the heart is stiff, the pressure's high in the pulmonary arteries, and the right heart's also impaired. Very hard to treat with pulmonary vasodilators, because it's sort of like opening up the faucet if the sink is plugged up. You can increase flow into the lungs with a pulmonary vasodilator, but it has trouble getting out and you just cause pulmonary congestion. The CADENCE study is taking sotatercept, which is at the FDA now for consideration for approval for pre-capillary pulmonary arterial hypertension, and the date for that review is March. The concept with using sotatercept in patients with combined pre and post-capillary PAH and a stiff left heart would be if they're suffering with right heart failure and backup of fluid on the right side, as well, that you may be able to drop that pulmonary resistance without necessarily forcing a lot more blood through the lungs, but making it easier for the right heart to pump. So, in those patients, they'll be treated with optimal therapy to make the left side of the heart fill as well as possible, which are currently therapies like SGLT2 inhibitors, which are sodium glucose transporter type 2 inhibitors that make people dump fluid and salt and glucose in the urine and have been shown to be helpful for a stiff left heart syndrome. So, you might optimize a patient on that to try to get the left heart filling pressures down as much as possible, and then maybe put them into a study of sotatercept to try to lower pulmonary resistance to make it even easier for the right side of the heart to pump. So, unmet needs in the PAH world that are going to be met in the years to come. I think it's really exciting that this is happening. The percentage of patients with pulmonary arterial hypertension that actually participate in clinical trials is remarkably low. Partly there may be exclusion criteria for which they're not allowed into the study. Partly it's a lot of back and forth to the center in order to have follow-up testing. So, things of that type can sort of reduce the number of patients coming in, but it's also an opportunity to get a therapy that's not yet available. If it turns out to be a therapy that's effective, you might be a couple years ahead of the game by going in a clinical trial. So, I encourage patients to consider it carefully, talk to their physicians and providers about it, and think about getting involved, because being in a clinical trial, you're not only doing something maybe good for yourself, but you may be helping another person, as well, which is a pretty cool thing. Whenever we're seeing a patient with medical issues that aren't adequately controlled, we're thinking about the whole range of options. What do we know could be effective that's not being optimized right now, and what can we do about that? That's the low-hanging fruit. Sometimes, in pulmonary hypertension, it's recognizing that oxygen levels are low at night and detecting that and then giving people oxygen at night or treating sleep apnea. The prevalence of sleep disordered breathing resulting in low oxygen levels at night and pulmonary hypertension is remarkably high. There's no salesperson knocking on our door saying, "Hey, look for this because then we can sell you this drug for it." It's like, "Oh, you have to think of it." Maybe a problem is that the oxygen levels are low at night and that's contributing to the pulmonary hypertension and right-sided heart failure. I'm struck at how often we just do overnight oximetry and pick that up. Little things like that that are clearly within the mainstream of currently proven medicine are sometimes overlooked. Then, if there are clinical trials, then we have to talk to our patients about what about their situation makes us think this might be or not be a good fit for them. Fortunately, most of the drugs in pulmonary hypertension that have been developed work in the majority of patients to some extent, so it's not necessarily that we know how to look at a patient say that probably won't work for you. But on the other hand, there are very specific subgroups of patients that some clinical trials are trying to find, and if the patient doesn't fit that criteria, then it doesn't make sense to go there. So, a careful discussion and looking at the options. A neat thing people can do to take a little empowerment themselves is to look at . It's a website where you just type in pulmonary arterial hypertension or if you heard about a drug, put in the name of that drug and boom, you'll see what clinical trials are going on, often with some inclusion/exclusion criteria for you to look at and centers that might be doing the study. So, I think it behooves patients who have any kind of disease that's not adequately managed to be thinking about what's out there. Sometimes you have to be proactive to make it happen, if that study's not being done in your local area. My name is Dr. Robert Frantz, and I'm 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
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Episode 475 - Robert Frantz, MD
07/01/2024
Episode 475 - Robert Frantz, MD
In this episode, Dr. Robert Frantz, a cardiologist at the Mayo Clinic, discusses current and upcoming clinical research in pulmonary arterial hypertension (PAH). He emphasizes the low participation rate of PAH patients in clinical trials and encourages patients to consider participating, as it not only benefits themselves but also helps advance medical knowledge. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD
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Amy Gietzen - phaware® interview 474
06/25/2024
Amy Gietzen - phaware® interview 474
Amy Gietzen, a patient diagnosed with systemic scleroderma, shares her journey and experiences living with the disease. She discusses her initial symptoms, the shock of the diagnosis, and the lack of information and resources available at the time. Despite facing numerous challenges, including pulmonary fibrosis and pulmonary arterial hypertension, Amy maintains a positive outlook and a determination to be an exception to the prognosis. She emphasizes the importance of education, self-advocacy, and being proactive in managing one's health. Learn even more about Nola and PAH at . #WorldSclerodermaDay #SclerodermaAwarenessMonth #SayScleroderma My name is Amy Gietzen. I was born and raised in Buffalo, New York. I am, first and foremost, a patient. I was diagnosed with systemic scleroderma in 2001 when I was only 19. Then, a few years later was diagnosed with pulmonary fibrosis. Then, pulmonary hypertension about five years ago. So I've been a patient for the better part of two decades for most of my adult life. Scleroderma is an autoimmune disease. It's considered rare. It's more common in women than men. But over the years, it has been known to be affected in men, in children. When I was diagnosed 23 years ago, I started to have a lot of fatigue. I was having trouble even waking up after a good eight hours night's sleep. I was having a lot of muscle pain, problems with my fingers, puffy fingers, sensitivity to hot and cold, trouble bending my wrists. At the time, I was working full-time and going to college full-time. I was going to be a nurse, so I was working in a nursing home and I was going to school full-time. I thought, "Well, maybe I'm working too hard, maybe I'm doing too much." My fingers started to turn purple and blue when I would be out in the cold. Then, I started to just feel really sick every morning. That was about six months of that and then I'd finally realized if something wasn't right. So, I made an appointment with an orthopedic doctor. I don't know why I did that at the time but I did. I went in and he asked me some basic questions, move your wrists up and down, raise your arms behind your back as high as you can, make a fist. All the things that I thought were super easy but I had trouble doing them. He did a couple X-rays of my hands and my back and then he very calmly said, "I think you have this disease called scleroderma. It is a terminal illness. There's no cure. They don't know why you get it.” He looked me straight in the face and said, "I would work as long as you can and get your affairs in order because it's a pretty nasty disease." I think I was just numb, because I had never heard what scleroderma was. I literally had to have him write it down on a piece of paper and spell it out for me. Looking back now, I was very fortunate to get diagnosed as quickly as I was. I know a lot of other patients can take years. I left the office just in a little bit of shock and disbelief that this was now going to be my life, which I had no idea what the outcome was, because he didn't really tell me what scleroderma was. After I was diagnosed, I had some blood work done to confirm. I had an ANA (antinuclear antibody) test done, a specific scleroderma test, which is an Scl-70 (topoisomerase I). It's a type of blood work. They were both positive. I did some research. I was a nursing student, so I went into my nursing books and tried to look up stuff. There was maybe one paragraph that said, "Rare illness, unknown origin, no cure, life expectancy, five to 10 years." I think finding that out and just sitting in it when I was 19, I always thought I would be the exception. I didn't feel sick at the time. I felt like I didn't look sick. I was like, "I'm going to be the exception. I'm going to be the one that isn't that sick. I'm going to be the one that beats this," and I kept that. Maybe it was naivete or maybe it was a positive outlook, but I kept that throughout the years. But as I started to get more symptoms like pulmonary fibrosis, which is the scarring of the lungs, having more difficulty breathing, then having pulmonary hypertension and some heart issues, having to have hand surgery to correct contractures, which are like when your fingers curl in and you can't move them. Losing fingertips due to ulcerated sores. Every time I got knocked down a peg in my confidence I would just inwardly look at myself and be like, "Well, I can wallow in the pity of what my life has become or I can just stand up and move forward with the life that I have and do the best I can with my situation." I've just always felt like there was a purpose for me getting scleroderma. At that time, I didn't know what it was. In fact, I didn't know what it was until probably 10 or 15 years later when I started to do a lot of my advocacy work. But it was scary because myself and my support system, which was my parents, we just were flying by the seat of our pants and doing everything any doctor would tell us, because we really weren't sure about treatment, about medications, resources. We knew nothing 23 years ago. There wasn't a lot of information out there so I just learned through my lived experiences, whether they were positive or negative. Sometimes it was really rough. So they were pushing clinical research, advocacy and then living a life that is still pretty active in living a good quality of life. My mindset back then was just, I'll be the exception and we'll just ride this ride for as long as I can do it. I am the type of person that I'm going to fight until my last breath because that's just who I am at the core. There were cracks in the armor, there were times where I just felt like, "I don't want to do this anymore." Scleroderma and pulmonary hypertension and lung disease blew up my entire life. I didn't get to graduate college and become the nurse that I wanted to be. I had to go on disability pretty early on in my adult life, I think I was 23. I was on a lot of medications and went through a lot of medications that ultimately left me without the ability to have my own kids. It was a lot to deal with at a young age. I really leaned on my parents, bless them. They just supported me in any crazy thing I wanted to do. I could have said I wanted to go to Mexico and get some kind of weird treatment and they would've been like, "Yes, let's go." They were very supportive. But for me, I dove into, I don't want to be treated as these symptoms attack my body. I want to be progressive and aggressive in the way I treat scleroderma, because I want to be that exception. I want to be the one whose story is different. I did so much research. I did almost five years of trying to find a scleroderma specialist in my hometown, a treatment center, resources, anything I could find. I finally came across a doctor in Pittsburgh that was doing some stem cell transplant research. I talked to my parents and I actually talked to my physician locally and he did not want me to go and I was like, "Yeah, we're going. I'm going to go." That trip, even though I wasn't qualified for the transplant because I wasn't sick enough at the time, that trip completely changed the trajectory of my life and my disease, because I found a place where people knew what scleroderma was. Where there was an actual treatment center and doctors collaborated together, like your lung doctor, pulmonologist, cardiologist, rheumatologist, they all spoke together. You saw them all in the same building. It was complete team care and I was at the center of it and they actually took my thoughts, my ideas, and my feelings into consideration. I probably wouldn't be here now if I didn't find them. About 10 years ago, after being settled in and accepting of what my life is going to be like with scleroderma, I really wanted to find other young adults like myself. I didn't really see any of them on social media, in any of the support groups and things I was going to. I felt like there was a disconnect and there wasn't a lot of resources for young adults living with this disease. I reached out to the and said, "Hey, why isn't there any young adult resources? And I would like to do something." So, I started a Facebook support group called Scleroderma Superstarz and within one year it had 5,000 followers. Then, it just grew into a young adult meetup that is virtual, that is called . So that's scleroderma young adults needing connection through the National Foundation. That was run by myself but now it's run by two very great young adults. I started to put together panels and webinars all focusing on young adult patients talking about their journey and their story. I think a big part of my journey and I think patients’ journeys needs to be education and resources. I learned a lot through lived experiences, but I also educated myself as much as I could because I think it helps that your own body and you know what you're going through. I wanted to give those tips and tricks to other patients so that they wouldn't feel alone like I did in the beginning of my disease. I am on the chapter advisory committee of the , which is a local chapter out of New York State. I am on their advocacy committee. I am also involved with the . I do a lot of advocacy work and social media stuff for them. My main thing is that I'm an advocate all around. I do a program with the and . They're based in Albany. We do an interprofessional education event where we take students rising healthcare professionals of different disciplines like nursing, OT, PT, et cetera. We take patients and we actually train the students by having them interview the patients. So the patients are actually educating the students. I do a whole training program with the patients on how to tell your story, how to focus on the best and maybe worst parts of your journey. I really am fixated with helping patients be educated and be advocates of their own and also guiding patients towards clinical research and participating in clinical trials. That's the basis of who I am as a patient advocate. I think the message I would give to newly diagnosed PH or scleroderma or any rare disease patient is that education is key. You have to know what it is that is happening to your body. You have to be on top of that so that you're able to tell what your body's going through. I think a lot of times doctors maybe would get sick of me, but I ask a lot of questions and I question a lot of things. I heard once, and it was so profound to me. Somebody said, "No is a full sentence." I think a lot of times we as patients don't use that word. We just say yes to everything because one, we're scared, and two, we think that doctors know all. They don't. They're human just like you and I. So I think it's okay to say, "No, I don't want to do this treatment right now," or, "No, I'd like to investigate. No, I would like to get a second opinion." I think that's okay. I think it's okay to trust your gut and go with what you feel is right. Ultimately, you're going to be the one living with this disease every day. In the beginning I said yes to a lot of things because I was focused on quantity of life. Now 23 years in, I don't say yes to a lot of things because I'm more focused on my quality of life. I wouldn't have known that if I didn't stay educated in my disease. My name is Amy Gietzen and I am aware that I am rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 474 - Amy Gietzen
06/24/2024
Episode 474 - Amy Gietzen
Amy Gietzen, a patient diagnosed with systemic scleroderma, shares her journey and experiences living with the disease. She discusses her initial symptoms, the shock of the diagnosis, and the lack of information and resources available at the time. Despite facing numerous challenges, including pulmonary fibrosis and pulmonary arterial hypertension, Amy maintains a positive outlook and a determination to be an exception to the prognosis. She emphasizes the importance of education, self-advocacy, and being proactive in managing one's health. Learn even more about Nola and PAH at . #WorldSclerodermaDay #SclerodermaAwarenessMonth #SayScleroderma Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Alvin Rocha, MSN RN CPN PHN - phaware® interview 473
06/18/2024
Alvin Rocha, MSN RN CPN PHN - phaware® interview 473
Alvin Rocha is a nurse care manager and pulmonary hypertension coordinator at Children's Hospital Los Angeles. He manages PH patients from birth to early adulthood. His main goal is to prepare these patients for the transition into the adult world. He is excited about the advancements in therapies and clinical trials and looks forward to attending the to learn more ways to help the patients and families he cares for. My name's Alvin Rocha. I am the nurse care manager or the pulmonary hypertension coordinator at Children's Hospital Los Angeles. I've been doing this for about six or seven years now. I manage the pulmonary hypertension patients that range from birth to 21, 22 old. I manage their symptoms, help them with medication authorizations, and just kind of guide them through their journey with PH and childhood and going through junior high and high school. My ultimate goal is to prepare them to transition into the adult world. It's a process. It's not something we do last minute. When you transition, you look at the patient as a whole. What is their family dynamic like? What is their learning ability, their developmental stage? I try to start transitioning patients or teaching about transition when kids are able to understand what's going on. So as soon as they're able to understand their diagnosis, their disease, and their medications, I start preparing them for when that day comes and they are old enough to transition to an adult hospital. What that entails is education that is tailored to where they are in life. If there's a patient that's in junior high or early high school, I start with the basics, what their meds are, when they should take, what their disease is and what it's going to look like in the future, what they can expect. Then, when they go to high school or end of high school into college, I talk about what they're going to experience, what kind of influences they're going to have, how certain things can affect their medications and how they feel. I just kind of have to go with the flow until they're 21, 22 years old and almost treat the visits as if they are adults and let them take the lead in asking questions and telling us about what's going on, how they're feeling. It's a process that takes sometimes a few years. It's almost like building their confidence for them to go to adult appointments on their own without feeling insecure about how they communicate with healthcare workers or healthcare providers. It's definitely a process. We see the patients until they have at least their first meeting with their new PH team once they transition to adult centers. We'll see those patients one more time, almost have a little graduation ceremony, and then hand them off to the next team that will take care of them. I let the parents know what I'm trying to do, what the process is. As the patient's mature, if the patient is very talkative and open, I will ask the parents if it's okay if I speak to the patient or Dr. Jackie and I speak to the patient without the parents in the room. Even if it has nothing to do with PH, nothing to do with their medication, it just gives the patients confidence to speak up for themselves and ask questions without feeling intimidated or nervous. Parents are usually open to that, which is nice. I think when I make it a regular part of our visit, they kind of get used to it. As long as everybody knows what the plan is or what I'm trying to accomplish, it goes really well. I haven't really had any issues with that. The patients and the parents are usually very involved in their care. They're involved with decision making together because it's so complex. Little decisions can change what their life is like at home. It's usually a team effort already right off the bat when you have a new diagnosis. School is a big thing. When we have patients that were either athletes or in clubs or playing instruments when they're diagnosed with PH, we have to really think about their quality of life and what's going to make these patients happy, what's going to keep them compliant with their meds. We really work with the schools to come up with a plan to make their life as best as they can at school. We do that by meeting with the patients and the family. What are their expectations? What can they handle? What can the patient tolerate? How much activity can they tolerate? We write a letter to the school with very specific guidelines to help with planning out what the child's day is like or what the patient's day is like. We really advocate for keeping the quality of life, but also keeping the patient safe. If we need to ask for extra assistance, kids are entitled to have an aide or a nursing aide at the school with eyes on them at all times, if that's something we request. We usually try to give the patient as much independence as possible, but also keep them safe by having someone keep an eye on them. Over time, patients that need oxygen or are on Remodulin or Tyvaso, they come up with a routine, they figure out how they're going to go through their day, and they'll let us know like, "Oh, I don't think I need my aid anymore. I think we're good without having someone follow me the whole day at school." Things are always changing as the patients mature and as they kind of go through life with PH and their meds. We have done a lot of combination therapies that we didn't do when I first started. The Remunity subcutaneous pump, that's new for kids. The treatments are changing pretty rapidly, in the pediatric world at least. We're constantly trying to figure out how to navigate training patients and families, but also training schools on a new pump, for example. With the SubQ pump, the Remunity pump is really new because it has a remote, and it's a lot smaller, which is great for the patient, because you can't really tell they're on a pump, but it's also brand new to everyone. So, we're troubleshooting problems both at home and at school. So that's a learning process in its own, and we're still learning new things about it. Kids with this new pump, there's a little more freedom with activities they can do. It's water resistant. They have a little more freedom with it. With telehealth, we're able to follow up more closely and see the patient. A lot of times, the way the patient looks and how they feel. Their color is really important for us to assess. Just being able to assess virtually is great because they don't have to find a way to get to LA if they don't live nearby. We can do things a lot quicker by assessing virtually. Over the last seven years, it's changed lot. Technology has really improved quality of life for a lot of patients. Communication has just been really key in growing and growth. PH is a scary diagnosis right off the bat. I've learned over time that helping the parents cope with it first has helped the most just because they know the patient best. They know how to navigate their emotions the best. If I can get the parents' emotions under control, help them understand what's going on, what we're trying to plan, what kind of meds we're going to use, I find that the patients do better. It's almost like the parents are taking the lead in their care, even if it's managed by a doctor and a nurse. I also find that when I answer questions and when I do teaching, right off the bat, I go into a lot of detail. Over a few weeks, I go over all the meds. Every med we could possibly start, I talk about, just so the patient and the parents are able to make decisions, knowing a little bit of information beforehand. We might not start all the medications, but knowing that it is a possibility just helps in general. I've also learned that when I talk to the patients and let them ask questions, that also helps with mental health just because their questions are completely different from parent questions. I can talk for an hour straight with parents and a patient. All the patient wants to know is, "Can I ride a roller coaster? Can I go swimming? Can I do this?" They just want to play. They just want to live their life. I just have to understand, again, where they are developmentally, where they are in terms of learning, because I find that the more they know what can happen, what is happening, the easier it is for them to cope. I also tell them that what they can read on the internet is very scary, but it's also very unique in pediatrics to have PH. Every child has a different experience. I tell them not to compare themselves to what they see on the internet, just because there's a lot of adults and a lot of older patients that aren't necessarily in the same place that kids are in. It does look very different. So, that also helps. I also found that if we have a chance to have patients meet with similar situations, let's say two younger school age patients with a Remodulin pump, if they're able to meet and just see that the other patient's okay and they look normal and they are able to play and do things, it makes things a little easier. And parents, when they're able to meet, parents from different patients, that also can help with just sharing experiences. Again, in pediatrics at least, I have to really read the family where they're at, how they cope, how much information they can handle at a time, and just kind of go from there. Just knowing more right off the bat can help with coping in the long run. I've been in this role now for going on seven years, and I'm just amazed at how much has changed over these seven years. We've seen a lot of change with medication and therapies for pediatrics. I know there's other things in the works with clinical trials in adults, so I'm excited to see how patients do with those medications. I'm attending the in June, which I'm really excited about in Spain. I look forward to seeing what the rest of the world has been doing and what they've been seeing with different therapies and different combination therapies. Then, I look forward to seeing what will soon become available for kids. We're usually the last group of patients to get therapies just as they do trials with adults, so I'm excited to see the results from those studies. I'm just excited to share with families what I learned, just because it gives them hope, it gives them motivation to stick with the therapies we have now. We don't always have the most convenient therapies. Some therapies are multiple times throughout the day. So hopefully, in the future, there are just therapies that are easier for kids to manage and easier for families to manage and just to have better outcomes with these therapies. I pretty much have decided that the rest of my career is going to be in PH. I've been a nurse for 12 years and I just don't see myself going anywhere else. The PH community is so strong, the doctors, all the scientists that are working on these therapies. Things just keep getting better for PH patients. It is a very close knit community, people that really care, people that are excited about the future of PH and how we treat it. I just kind of hold onto that while I do my everyday job. My name is Alvin Rocha, 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:
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Episode 473 - Alvin Rocha MSN RN CPN PHN
06/17/2024
Episode 473 - Alvin Rocha MSN RN CPN PHN
Alvin Rocha is a nurse care manager and pulmonary hypertension coordinator at Children's Hospital Los Angeles. He manages PH patients from birth to early adulthood. His main goal is to prepare these patients for the transition into the adult world. He is excited about the advancements in therapies and clinical trials and looks forward to attending the to learn more ways to help the patients and families he cares for. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Dunbar Ivy, MD - phaware® interview 472
06/11/2024
Dunbar Ivy, MD - phaware® interview 472
Dr. Dunbar Ivy, a pediatric cardiologist at the University of Colorado, discusses the upcoming where experts from around the world will gather to discuss the progress made and future directions. Dr. Ivy highlights the work of the pediatric pulmonary hypertension task force, which aims to develop expert opinions on various topics, including the role of different therapies for patients who have failed all available medical treatments. The task force also focuses on understanding genetic abnormalities and developmental lung disorders, defining pulmonary hypertension in neonates, and addressing the challenges of clinical trials for pediatric patients.The task force plans to publish their findings in 2024 and welcomes feedback from the medical community. Hi, I’m Dunbar Ivy. I'm a pediatric cardiologist at the University of Colorado in Denver, Colorado. I've had an interest in pulmonary hypertension for very close to 30 years. For me, it's been a very exciting journey to see things move from really, no therapies, to one therapy to over 12 therapies. That's really been exciting to be able to use some of those medicines off label, but I think they really have helped children and improve survival. This year will be our next meeting of the . It's a meeting every five years or so. The goals of the meeting are to get experts from around the world in all aspects of pulmonary hypertension to bring together and develop ideas as to where have we come since the last meeting certainly, and also where should we go? What are the things that we need to keep working on? It's exciting for us. I believe this is our third year to have a pediatric pulmonary hypertension task force. I think it's really wonderful to see how far pediatrics has come. There are eight clinicians chosen by the steering committee to meet and develop our own ideas and to write them down and to develop a paper so that we can share some knowledge. It's important to note that they're not guidelines. They're sort of an expert opinion kind of document. The group is going to focus on several things. One is in patients who have failed all available medical therapies, what is the role of the Potts shunt versus an atrial septostomy versus lung transplant? Which patient would benefit most from which of those therapies? And is there a preferred order? Some of this I think really depends on the capabilities of the center, but we'll try to provide some guidance in terms of which therapy for which patient. Other things that we're very interested in is there's been really an explosion of interest in knowledge in developmental lung disorders from genetic abnormalities. The TBX4 mutation has raised a lot of interest in which patients have this, and it's the second most common gene mutation and is interestingly found even in adults sort of later on or in newborn babies. So, there's some exciting pathobiology that we need to learn about, for example, for that mutation, but also others. We're going to work on the definition of pulmonary hypertension in neonates. I think that's well established in adults that they have a mean pulmonary pressure greater than 20 millimeters of mercury in an elevated pulmonary vascular resistance. But many of the patients that we take care of are neonates and we're not able to perform cardiac catheterization on them. So, we're trying to define some criteria that would be suggestive of an elevation of pulmonary artery pressure. Then, the key really is to say why is the pressure elevated? Is it from a left heart problem? Is it a right heart problem? Is it from pulmonary veins? Is it from intracardiac shunt, for example? I think there's a need for that for some definition of pulmonary hypertension in neonates. It's a well-known and well respected conference. These are proceedings, again, they're not guidelines. But I think they're very well-read and I think that people take these recommendations seriously. From being on the committee for the last two sessions, we do get challenged on our thoughts, which is totally fine. But I think again, it sort of helps people to focus a little bit and to have a more clear definition of some things and directions. One very, very important area is clinical trials for approval of medications in children with pulmonary hypertension. The approach is similar but very different between the US and Europe. I think there's a higher standard showing a drug is effective in the United States, whereas in Europe, they tend to rely more on the adult trials for the efficacy. Then, they look at the pharmacokinetics, how the drug is metabolized and what the drug levels are in safety. Now, certainly we still do that in children in the States, but we'd like to have a common pathway for Europe and the US for drug approval. One of the things that we will discuss is the challenges of enrollment. In order to get a drug approved in the United States, we are going to have to have enrolled trials. Right now, a lot of the trials are stopped early because they can't enroll. So, there's been a lot of thought about why that is. The design of the trials has changed so that I think families were very nervous about repeat heart catheterizations, for example. So those are not allowed in the trials anymore. Many of them will have a heart catheterizations as an entry criteria just to be sure that we know the right disorder. But then we're looking for non-invasive ways to show that the drugs are helping the children. I think the trickle down is that we always learn a lot from our adult colleagues. They have more evidence-based guidelines than we do. Ours are more experience-based. So, when I go to that meeting, I try to attend every session I can to learn from our adult colleagues. There's 14 other sessions and one pediatric, so we're in the minority, but it's a very passionate group. I think all of us like going to the whole meeting and not just to the pediatric session. These are the ideas that we have. We will continue to meet at least monthly to go through these ideas and work them out and see if we're thinking about things correctly. Then, we're pretty sure the manuscript will be published this year in 2024. So, we'll be excited to get our ideas out there and also to get feedback. My name is Dr. Dunbar Ivy, and I'm 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 #PPHNet
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Episode 472 - Dunbar Ivy, MD
06/10/2024
Episode 472 - Dunbar Ivy, MD
In this episode, Dr. Dunbar Ivy, a pediatric cardiologist at the University of Colorado, discusses the upcoming , where experts from around the world will gather to discuss the progress made and future directions. Dr. Ivy highlights the work of the pediatric pulmonary hypertension task force, which aims to develop expert opinions on various topics, including the role of different therapies for patients who have failed all available medical treatments. The task force also focuses on understanding genetic abnormalities and developmental lung disorders, defining pulmonary hypertension in neonates, and addressing the challenges of clinical trials for pediatric patients.The task force plans to publish their findings in 2024 and welcomes feedback from the medical community. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD #PPHNet
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Nola Martin - phaware® interview 471
06/04/2024
Nola Martin - phaware® interview 471
Nola Martin shares her experience with pulmonary arterial hypertension (PAH). Nola describes the physical and mental challenges she faced, including fatigue, weight gain, and the need to ask for help. Nola emphasizes the importance of self-advocacy and fighting for one's own health. She recounts a situation where she had to push her doctor to listen to her concerns and change her medication. Despite the limitations, she has a better quality of life and encourages others to be their own advocates. Learn even more about Nola and PAH at . #SclerodermaAwarenessMonth #SayScleroderma My name is Nola Martin. I'm from Philadelphia. I was diagnosed with pulmonary arterial hypertension about 14 years ago. When I was first diagnosed, I had no idea where pulmonary hypertension was, had never heard of it. I thought it was high blood pressure, and my doctors said, "No, it's kind of different. Whereas with this one, the blood vessels in your lungs are constricted." But what I can tell people about pulmonary hypertension is, it is definitely a lifestyle adjustment. How I was diagnosed was, I had been an avid walker. I love to walk. I noticed one day at work I tried to walk two feet to go to the printer, and it felt like all the oxygen had been sucked out of the room. I didn't know what was going on. Of course, it was a panic attack. I called my primary care doctor. She said, "Okay, are you able to come in?" Sure, I can come in. I didn't tell her I was an hour away. I had no idea how severe it was. I tend to rationalize things away, because looking back now, there were so many telltale signs of what was going on. For instance, at that time, I had gained a whole lot of weight, but my meals were one orange a day. For some reason, I don't know why it was an orange. But that's all I could eat was one orange a day. But I was still constantly gaining weight. Simple tasks like taking a shower, walking up the steps, trying to make my bed, became very difficult for me. I would get short-winded. When I finally went in to see my primary care doctor, they put a pulse oximeter on me and said, "Well, let me see if you can walk around the nurse's desk what would happen." I took a couple of steps. The next thing I knew, it was like the oxygen really got sucked out of the room. They put me into a wheelchair and whisked me over to the ER. They were like, "Your oxygen level plummeted to 85. Your heart rate went up to 125 and was rising." That was the first time, once I was admitted, that I learned what pulmonary hypertension was. My diagnosis came from a right heart catheter. I had so many echocardiograms, too. I was told at that time that I had so much fluid, too, around my heart. They were like, "We don't even know how you walked in here." I went in the hospital weighing 213 pounds and came out weighing about 180. They were like, "That's how much fluid you had on you." I take diuretics daily because the fluid will come back. You have to watch salt intake. I've never been one that was big on salt anyway, so that wasn't the issue for me. My biggest issue was I was limited to the amount of fluid intake. I was only allowed to have 64 ounces of fluid a day. That included anything that had water in it, like fruit. That included ice cubes. In the summertime, I can go through 64 ounces of water in the morning. So, having to limit my fluid intake, that was the biggest obstacle for me or so I thought until later on when it came to trying to do those chores. Like I said, something simple as taking a shower became very difficult because of the steam in the shower. It would make it difficult for me to breathe. I was on two liters of oxygen that I had to wear 24 hours a day. I never knew how strenuous vacuuming could be, but that would also make my oxygen level plummet. Trying to make my bed, just getting dressed sometimes in the morning, in your mind, even with an illness like this, you just go on about doing your daily routine and you think it's fine. Okay, I can do this. But the moment I would start to move, that's when my oxygen levels would plummet, and my heart rate would increase. Dealing with the physical aspects, I'm very independent. I'm used to doing everything myself. Knowing that I had to ask for help, that was hard for me, because I've always been the caregiver in the family. I've always been the one helping out other people. I don't ask other people to help me. For me, going to the grocery store wasn't an option by myself anymore because first of all, I had to ride around in that cart. I couldn't walk around and carry the oxygen tank with me. For me, to have to ride in that cart mentally, it was like, no, this is for people who's really, really sick. Even though I was that person who was really, really sick, in my mind, I wasn't ready to accept it. When you hear a diagnosis that you're unfamiliar with and then you're being told all of these things that you can't do anymore, all these things that you can't eat, and there's so many limitations put on you, mentally, it's very trying. You go from being angry like, well, what did I do? I do the right thing. I help people. Why did this happen to me? Or did I do something that caused this? Because no one else in my family had this, it is like, well, it had to be me. I had to be doing something wrong, because no one else in my family that I knew of had this illness but me. You get judged by the way you look, because with pulmonary hypertension isn't something that they can see what is going on in the inside of your body, you're judged by what they can see on the outside of your body. When people look at you and they see the weight gain and the different things going on, the first thing they say, "Well, diet and exercise. If you diet and exercise, you'll be fine." Well, it wasn't just the weight gain, it was also the fatigue. It was you get exhausted from getting up in the morning and taking a shower. I could wake up and say, "Oh, I feel great." In my mind, I'm going to do this, this, and this, but by the time I go and brush my teeth and take a shower, I'm exhausted. I don't feel like doing anything. Sometimes plans with family and friends had to be canceled, Then you stop getting invited to certain things because it's, "Well you're not going to show up anyway, so we just stopped inviting you." That gets to you, too, mentally because it's like, well, wow, now I feel like I'm letting people down or people don't want to be around me. Or it's a thing where it's, "Oh well, she's going to be sick, and nobody wants to be around a sick person that we have to watch what we say and what we do." It's like, well you smoke, but you can't be around me smoking. I'm wearing oxygen. There's a lot physically and mentally that comes along with this that you just have to make an adjustment for. Joining a support group helped me with that because one of the things that is explained to you in a support group and that I also had to wind up explaining to one of my doctors is, you may know medicine, but I know my body. I tell everyone, you have to fight for yourself. I've had that conversation with my lung doctor. I don't care how many letters you may have behind your name, this is my body. I've had it all my life. I know the changes that are going on. I know what's working and what's not working. If you're giving me something or telling me my body should be doing this but it's not doing that, I have to stand up and fight for myself. You only get this one body to work with and whatever happens to it, it's up to you to make sure that you speak up for yourself. I had to learn to do that because I come from an era where the doctor is always right. If your doctor tells you this is wrong or you need to do this or take this, this is what you do. I had to learn that no, because this medicine you've given me, it's not working, or you're telling me I should be walking more or I should be doing this, and my body is responding differently than what you're telling me. I always tell everyone, even in our support groups, when I tell my story, my story is my story. PH affects everyone differently. You know your body. If something isn't right, you have to stand up for yourself and let them know. Don't let anyone silence your voice when it comes to your health or anything else. My self-advocacy started three years into my diagnosis with pulmonary hypertension. I was taking a medication that I was on for about three years, and it stopped working. I felt as though it stopped working because even though I was following my routine of what I was supposed to do, taking the medicine, eating the foods that I'm supposed to eat, keeping my water intake down, no salt, things like that, I was starting to gain the weight again. I was starting to be tired more, have more fatigue. My iron level had dropped. My iron was so low, I was getting IV infusions, and it wasn't raising it any. I said, "Something is wrong," and my doctor at the time felt as though it wasn't. He's like, "No, the medicines that you're on, it's been working all this time." I said, "Yeah, but I'm not a textbook case. Medicines don't always work. They do stop working. Your body sometimes adjusts to the medicine that they take, and it may not work anymore. I feel as though that's the case for me." He didn't want to listen, so I said, "Listen. Well, we're at a standstill right now. Either you're going to have to change, give me some more tests, do whatever you need to do, or else I just have to go somewhere else, but I physically know there is something wrong with my body, because it's telling me. I ignored it in the beginning when I was first diagnosed. I kept rationalizing away my symptoms I was having. I'm not doing that anymore." Just at that time when we were going through that, I received a letter from the pharmaceutical company of the medicine I was taking. It said that if you have this symptom, this symptom, this symptom, that you should stop taking the medication. I had every single symptom on that list except low sperm count. I said, "I think if I was a male, I would've had the low sperm count." So I took that to my doctor and I said, "This is what I received from the pharmaceutical company, and I'm having every last one of these symptoms on here." The low iron was one of them. I said, "You can see that I've been doing iron infusion and it's not working." Then from that, that's when we had more tests done. A new medication was induced. That's why I tell people, you have to fight for yourself. If you know that something is wrong with your body. Stick up for yourself. I am still with that doctor. He loves me. I love him. We give each other little jabs every now and then, but I love him. I trust him now. He trusts me. He told me, "Nola, you are one rare patient." I said, "Well we all are. We have a rare disease. We're all rare patients." He said, "Well you're special because, boy, I tell you. You're the one that told me 'Doctor,' he was like, 'You say, 'You can't treat all patients the same. Everybody is not a textbook case.'" He said, "You drilled this and drilled that." He said, "I get it. Sometimes doctors know best, but I appreciate you sticking up for what you really believe. Something was wrong with you and you stuck with it." I'm like, "Well that's one thing you've learned about me." So 13 years later, he told me I abandoned him for his nurse practitioner, because I see her more now, but I said, "Because you're more on the research side." But he's still my pulmonologist, and I really enjoy going to see him now. My journey with PH today is a little different from what it was when I was first diagnosed years ago. For one, I'm no longer on oxygen, which is great. I enjoy the freedom of moving around without that. But I also have to still limit myself, because even though I'm no longer on it, and there may be days when I feel like, oh, I can do this, I can do that, it'll easily shut you down and let you know, okay, nope, I'm still here. You're doing too much today. But what I also just found out from a test that I had is that I now have to take two medications for my pulmonary hypertension. I was just on one. They found that my lungs had a little scarring. It's moderate, as they call it. Now, I have an inhaler that I take along with my regular oral medication. I do find as time goes on, I'm getting more fatigued. I'm not as active as I used to be, which is hard to believe. I was so sick I could hardly move around, but in between those years, there were some good years where I was a little more active. I still can't master, though, walking in the sand on the beach. That's still challenging. I never realized how challenging it would be to do something as simple as that. But I can do my daily tasks. I just have to limit everything that I do and not try to do everything at once, but in stages. But I do have a better quality of life now than I had in the beginning. I always tell everybody, be your own advocate. If you have any problems and you feel like you need a little encouragement, call me. I’ll help give you a little encouragement. I'm actually, co-president of the support group that we have at the hospital that I attend, so I enjoy it. I really do. Speaking to other people, talking to them about it. I can go on and on all day about PAH, but the only important thing I like to share is, remember, my story is my story. We're all different. PAH affects people differently. It also depends the other illnesses you may have, because I have scleroderma, as well. So like I said, my story is my story. My name is Nola Martin, and I'm aware that I am rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 471 - Nola Martin
06/03/2024
Episode 471 - Nola Martin
Nola Martin shares her experience with pulmonary arterial hypertension (PAH). Nola describes the physical and mental challenges she faced, including fatigue, weight gain, and the need to ask for help. Nola emphasizes the importance of self-advocacy and fighting for one's own health. She recounts a situation where she had to push her doctor to listen to her concerns and change her medication. Despite the limitations, she has a better quality of life and encourages others to be their own advocates. Learn even more about Nola and PAH at . #SclerodermaAwarenessMonth #SayScleroderma Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Eric Austin, MD - phaware® interview 470
05/28/2024
Eric Austin, MD - phaware® interview 470
Dr. Eric Austin, a pediatric pulmonologist and pulmonary hypertension doctor, discusses the upcoming which will focus on various aspects of pulmonary hypertension research and clinical care. Dr. Austin is part of the task force on genetics and genomics, which aims to explore the genetic landscape of pulmonary hypertension and identify opportunities for future research and therapy development. He highlights the significance of the discovery of the BMPR2 gene and its role in pulmonary arterial hypertension (PAH), leading to the development of the gene-informed therapy sotatercept. I'm Eric Austin. I'm a pediatric pulmonologist and pulmonary hypertension doctor at Vanderbilt University Med Center at the Children's Hospital there. I direct the Pediatric PH Program. I'm a proud member of several groups that affiliate with pulmonary hypertension research and clinical care, including the . Today, I'm going to talk to you a little bit about the , which occurs this summer, 2024, in Barcelona. This is a conference that's occurred intermittently since its initial meeting, I believe, in the 1970s. The most recent meeting was about five years ago. So, we're reconvening as a field to think about different aspects of pulmonary hypertension in children and adults and different forms of both research, clinical care, and opportunities for the future and needs that we still need to fill. For example, I'm part of the task force on genetics and genomics, which I'm leading with Dr. Wendy Chung. We have several fantastic people in our task force. We're charged with thinking about where we've been in terms of the genetic landscape of pulmonary hypertension, what discoveries have been made, and of course providing an overview to the ultimate readers of the documents that come out of the conference. But also, to think about what's the next phase? Where do we need to go with genetics and genomics? We have a lot of gene discovery now that has occurred. We've had some progress over time in terms of impacting clinical care, but not as much as one would hope based upon other fields that have profound genetic discoveries. However, we've got this exciting new line of study that has been translated from cell-based studies and animal studies and then into clinical trials with the drug sotatercept that is emerging. I'd remind people that that drug and its progression, which was led by several wonderful investigators and then a partnership with industry, they really pursued that because of the initial genetic discovery that the bone morphogenetic protein receptor type two, BMPR2, was not only mutated in a lot of families that have pulmonary hypertension - children and adults with what we used to call primary pulmonary hypertension, but is also not as robustly expressed as a protein in people of other forms of pulmonary arterial hypertension. Because of that initial genetic discovery of pulmonary arterial hypertension associating in families with BMPR2, it was then discovered that BMPR2 is found in other forms like idiopathic PAH, in particular. Then, people started to look at other types of PAH, and they found that even if a person does not have the mutation, they have low expression of the gene and they probably have some degree of depressed function of pathways related to that gene. So, that triggered many, many years of study that ultimately are culminating now in the first gene associated or gene informed therapy, the sotatercept therapy, that will hopefully be approved by the time we're meeting this summer. So, what's exciting about genetic and genomic therapy to date is that while it hasn't had a profound impact on our care to this moment, we understand more and more about patients and families who are impacted by gene mutations, but we've also had investigators around the world looking at the genes and pathways related to not only BMPR2, but other genes that are now known to be mutated and associated in families. That investigation, that gene informed investigation, has really what has propelled the field toward new therapy opportunities. In particular, as you may or may not know, BMPR2 expression is reduced in the tissue of many forms of pulmonary arterial hypertension. Because of that reduced expression and probably reduced signaling in the lung of people who have PAH, investigators started to say, "Okay, well, let's use our genetic information that we understand and let's try and think of ways that we could improve the function of that pathway or correct its balance in a way." So the gene discovery informed therapeutic development in a really exciting way that of course now come the meeting this summer in 2024, it's likely that there will be people getting FDA and EU approved sotatercept, which is a gene informed therapy, I would argue, to correct the balance at the molecular level that BMPR2 is so intimately involved in. So, an interesting question you might ask is, "Well, so you're part of the genetics and genomics task force?" There's lots of task force by the way. There's a pediatric task force, there's a risk score task force. There's many other aspects. There's a pathobiology task force. The genetic and genomic task force to my recollection, I think this will be the fourth time it's been included in the conference. What really prompted its initial participation as a task force is because of the genetic discoveries that were happening and people wanted to get informed. So originally, BMPR2 was discovered in the year 2000, published in 2000 by two different groups working across the world. There was actually, truthfully, a little pause thereafter in genetic discovery from about 2000 to about 2008. People were working hard trying to make new discoveries, but they weren't really emerging as quickly as one would hope. Then, we started with the emerging landscape of new technologies to survey people's genome, starting with whole exome sequencing and then going with whole genome sequencing. Of course targeted discovery efforts based upon what we knew about the genes related to BMPR2, including alkaline and enhancin, which of course did come shortly thereafter. I think around 2008 was the first time there was a genomic task force included in the process. The goal at that time was really to just update people and say, "Okay, look, what genes are available? What are on the horizon?" The other thing that's challenging for clinicians is that we now think about genetics and genes in our patients much more commonly than we certainly did a decade ago. But it's hard to know sometimes when you're boots on the ground in the clinic or in the hospital, which panel do I order for someone? What company or institutional resources do I engage to not only send out the blood and get it evaluated, but also understand the information that comes back? Because unless you're a trained geneticist, it can be a little confusing. I'm sure patients and families have also seen their reports at times. You'll have reports that will say, "Okay, we found these seven genes have variations in them that might be classified as mutations." A mutation, by the way, means there's less than 1% of the global population who is your ancestry would have that variation in their DNA. But there are so many variations now with this explosion of DNA analysis that you get a report back as a clinician and a family member or a patient and it will say, "There's these 12 genes that are different than they should be or mutations." But the clinical significance of that has yet to be published or determined. So you could have a person who's maybe the first person who's ever been discovered with PAH to have that mutation. Does that mean it caused the mutation? Does that mean it was associated in some way that made their PAH develop? Where is the connection there? So, it's really difficult for doctors and patients and families to understand sometimes those reports. So, long-winded way to say, one of the charges that we're going to take is helping people to understand the resources that are available to interpret those findings and do our best to say, "Okay, here are the genes that we really believe are truly, if not causative of PAH, extremely tightly associated with it." To the point that if you have a mutation in that gene, you should take it very seriously. What we may not be able to do in that task force is go through every mutation in every gene and publish, "Okay, if you get this variation in your DNA and this gene, this means this." That's probably a level of detail that will be hard, but try and provide resources for people. Then, the second thing that we've really tried to do, and I've talked to Dr. Chung a lot about this, and as our senior lead, she's really interested in going beyond just gene description, PAH or not, done. So what we're going to try and do is think about what is the next horizon that needs to be done to use the knowledge that we have already to get to more drug therapy, to get to more understanding of clinical outcomes? Because we do tend to, in our field, tend to make these discoveries but due to limitations of sample size and other things, we've not always made the connection. We know that individuals who have certain gene mutations, including BMPR2 probably are diagnosed earlier, probably have worse hemodynamics when they're diagnosed, probably have a higher risk of right ventricular dysfunction and failure before a given other person, but we don't have a lot of great data beyond BMPR2 for that information. One of our tasks is going to be to collect what we do have, analyze it together, and then look to challenge the field for what needs to be done between now and the next world symposium, wherever that is going to be in four to six years. When we have a new diagnosis, that's the most common time to initially think carefully about genetics and genomics. We could come back to that, but one thing we've started to begin to do is think about revisiting genetics and genomics when we didn't find anything five years ago. But say you have a new patient and they present unfortunately with what we think is idiopathic pulmonary arterial hypertension, and they have no family relationships that would suggest that they have familial disease, we still in the current era, at least in pediatrics, offer them genetic testing for a few reasons. One, to make sure that we understand to the best of our ability why they have the disease. Number two, there may be major family-based impacts to have a diagnosis that comes with a risk of heritability or the risk that someone may have received it because they got a gene that was variant from their mom or dad that could have repercussions for other siblings or for future childbearing and whatnot. So, in pediatrics we kind of grew up as a field even beyond PH, thinking about genetics very carefully. We have that discussion every time with our patients. But what we don't do a great job of is actually thinking about the cost of that. For our region in the mid and southeast, we have very limited difficulty getting it approved, currently. Almost every insurance company approves the genetic testing interestingly. Generally speaking, aside from the copays or the additional burden of any other test, it's not an added measure of financial cost to families. Of course, it's still part of the giant cost of having to deal with an individual who has PAH or other medical problems. To do the current era, a panel is probably about $1,000 charged to the insurance company. It varies by company and whatnot so I don't know specifically. If you're going to look for a panel of genes, and that's an important point, also. Generally speaking, we have a new individual diagnosed, we offer them genetic testing, we would send off probably a targeted panel looking at the genes that we know are associated with PAH. Within that targeted panel, not every company offers exactly the same panel. So, your physician and you may want to think carefully about which panel we're doing. The truth is sometimes you don't have much choice. Oftentimes, there may be negotiated contract relationships by both insurance and your medical facility that force, but that panel tends to be about $1,000. If you're in a family that has known PAH and known gene already that's variant. So, say you're in a BMPR2 family, maybe you don't know the mutation, but someone in your medical care world does, that's a cheaper test, that's a targeted test. You could say, "Well, we're not going to bother to send the whole panel out, because we know this family has BMPR2. This is so rare, it'd be weird for them to have another gene." That's about 250 to $300. So, some families, especially in the previous era, used to elect to spend that themselves out-of-pocket, because they didn't want their genetic information for their child to be already dictated at they're four years old and they already have PAH, which is a challenge, and then we're going to label them as having a, quote, "mutation". That stigma has begun in the last five to seven years to really dwindle, thankfully. There are protections for people genetically. Genetics has become so ubiquitous in our care that we don't have that happen very often anymore that someone just pays out-of-pocket. So, I'm really excited to continue working with our group on the task force for genetics and genomics for the World Symposium on PH, and also just for the collaborative opportunities and processes that we have going on in North America and beyond already. I think if you're a family who is struggling with this, one thing you can hold onto with hope is that we are learning so much every day about genetics and genomics. It's not just about yes, no mutation. It's now about what are the variations and the pathways associated with these genes and how can people in the bench, and then ultimately those of us translating into the clinics, how can we take either novel therapeutic developments or existing therapies that, oh, it turns out that therapy that has been on the shelf for 50 years actually influences the activity of genes related to the gene that causes my kid's PH. There's a lot of work going on to try and figure that out. Sotatercept, as I said, is just one example of that. I think and hope we're going to have many more soon. So, this summer is a great opportunity to kind of regroup, think about where we're at, lay groundwork for what we want to challenge the field to do in the next five years, and really set the stage for the next era of PH therapy, which I think is coming quickly. That's going to be beyond relaxing the blood vessels, what we call vasodilation, but into modifying the underlying causes. So, we have great hope. Genetics and genomics are just one component of that. I'm Eric Austin, and I'm aware 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 #PPHNet
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Episode 470 - Eric Austin, MD
05/27/2024
Episode 470 - Eric Austin, MD
In this episode, Dr. Eric Austin, a pediatric pulmonologist and pulmonary hypertension doctor, discusses the upcoming , which will focus on various aspects of pulmonary hypertension research and clinical care. Dr. Austin is part of the task force on genetics and genomics, which aims to explore the genetic landscape of pulmonary hypertension and identify opportunities for future research and therapy development. He highlights the significance of the discovery of the BMPR2 gene and its role in pulmonary arterial hypertension (PAH), leading to the development of the gene-informed therapy sotatercept. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD #PPHNet
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Victoria McKinnon - phaware® interview 469
05/21/2024
Victoria McKinnon - phaware® interview 469
Victoria McKinnon shares her personal experience with pulmonary hypertension, specifically her son Owen’s eight week battle with the condition. Owen was diagnosed with alveolar capillary dysplasia (ACD), a genetic disease that causes pulmonary hypertension. Despite initial improvements, they ran out of treatment options and ultimately had to make the difficult decision to remove care. Victoria emphasizes the importance of communication and honesty from healthcare professionals, as well as the support and knowledge gained from joining online support groups. She also discussing her fundraising efforts to support research and find solutions for children with PH. Learn more about Victoria’s fundraising efforts: My name is Victoria McKinnon. I'm from Boulder, Colorado right now. My connection to pulmonary hypertension is my son died from pulmonary hypertension two and a half years ago after eight weeks of life-fighting pulmonary hypertension. Owen arrived within an hour of his due date. There was no indication that anything was going to be wrong. There was nothing wrong during the pregnancy. He actually arrived seemingly healthy. He weighed a lot. He was long. He was healthy. He came out with a big scream. It was only over the course of the first 24 hours, that we realized something was wrong. He went into respiratory distress and was intubated. We got transferred to the local children's hospital in Colorado. They assumed that it was pneumonia. We progressed. We were treating it. After a week, it wasn't getting better. He all of a sudden crashed. I'll never forget him crashing on ECMO. It was when a nurse ripped off a medical tape. We now know with pulmonary hypertension, these sort of events can precipitate a pulmonary crisis. It was extremely traumatic. At that point, after he went on ECMO, they suspected ACD, alveolar capillary dysplasia, a genetic disease that causes pulmonary hypertension due to the misalignment of the pulmonary veins. We waited for a diagnosis from the genetics, but unfortunately, we didn't get an answer. They came back positive the genetics were totally fine. Before removing care, we decided to go ahead and do another echocardiogram. The echo actually showed positive improvement. So that kind of bought us time. We thought, "Man, this is a miracle." We had our voodoo candles out. We were so excited. Then we spent weeks three through seven trying to decide what medicine to do next. We were on Riociguat, Sildenafil, all the big hitters in pulmonary hypertension for neonates. Unfortunately, we ended up running out of options. Even though he came off ECMO, he was extubated and he was able to be on a high-flow nasal cannula. Ultimately, there wasn't any more we could do. He was definitively dependent on NO (nitrogen) and was not stable enough for a lung transplant. So without a diagnosis and without knowing what to do and what this even is, we ended up getting really good advice from our pulmonary hypertension doctor. I will forever be grateful for this, because I do think that he kind of stuck his neck out on the line and gave us his true human empathetic answer. For us, it was removing care. I didn't want to incite any more pain on Owen that he was already in. So we did remove care and it was only after his death that we learned that Owen and myself actually lack the FOXF1 promoter that causes ACD that was found in the regulatory region postmortem that was not tested. They only tested the exome when he was two weeks old, so that's how it was missed. We went from, "Oh my goodness, which onesie am I going to have his newborn photos taken in?" I had a bunch screen printed with his name on it. I had a newborn photographer all lined up. I was so stressed, should I get my hair done? Should I not? The silliest things in your life. It all just came crashing down where we are just like, okay, is he going to keep breathing another minute? It was a really challenging eight weeks, but you're just single-threaded on survival for him. But it was really challenging. There were a few things that brought us enormous comfort. At along every step, we joined a Facebook group or a community. So when we were just in the NICU and it was pneumonia, I joined the NICU moms group. Then, when it was like, oh, there's pulmonary hypertension, then it's ACD, and then it's interstitial lung disease, I actually joined a Facebook group in all of those and learned what would life look like with this diagnosis? When we joined the lung transplant group, what would it look like? What are our options? All of that sort of stuff. Ultimately, the only group where we were a member of now was the ACD group, but I think having something like would've been really helpful. Granted, in those eight weeks, it's hard to find all of these things, but these sorts of networks bring so much comfort and also knowledge. My husband Daniel has a PhD in material science. I think that that carried over to him reading every single PubMed article related to this. We were reading about clinical trials. Dan on the NICU glass wall that slides and opens up to get in the room, had taped 40 different papers all highlighted, did a tree, had written the PubMed numbers on it, trying to ask questions. He would interrogate them at rounds for an hour. All of the nurses would walk away. They're like, "This is a waste of my time." But we just went into what can we do to help? Maybe they're missing something. Maybe there's something else. So to go from, should I do my hair curly to is there a clinical trial and a rat that might save my son was just really horrible. Right now, we're at this for pediatrics and neonates. I was asked to give a 10-minute talk. While I could talk for hours about Owen, I wanted to just monopolize on an opportunity to give them something to work with. So yes, this is a really sad story. Yes, there's horrible things in the world, but how can you make our horrible days less bad? So that's kind of how it came about. As a call to action for nurses, respiratory therapists, all of the doctors and physicians that are helping us, I have five asks from parents. I've consulted other parents in compiling these asks. Through that task, I did realize the breadth of requests from parents. I will caveat this list of five asks with, I think the best practice is to just ask how a parent wants to be communicated to, but these are my takeaways and what I would ask from my personal experience. The first ask is the hardest. We found that during the ambiguous end-of-life decisions, we were given a lot of information, but we didn't have the context surrounding it. This was my first baby and only baby, and we haven't seen what is a survivable sick kid and what is not. During those decisions, only Dr. Kinsella offered his compassionate perspective on what he would do if it was a family member. I'm asking as a human, as a mom to another doctor what they would do. I am asking specifically, because I trust them and I am curious. I know that this may not be shared with all of the parents to want to hear a personal opinion, but when directly asked, please go ahead and share because as a mother, I couldn't possibly have made the most impossible decision you could think of without the recommendation of someone who has seen infinitely more sick kids than I have. So his opinion was the ultimate gift of freedom for me from rumination or any guilt. So that's the first ask. The second ask is honesty. I'm going to tell a little story, and it sounds very silly, but I actually think he was really incredible. So we call him the “Pizza Tie” doctor. He's a very cheery pulmonologist. We had never met him and he shared his point of view with the most hysterical fashion. We asked if he thought Owen had ACD and he said no. We asked if he thought Owen had another interstitial lung disease and he said yes. We thought that this was good news. He said no. We asked if he thought Owen would live, and he said no. He said all of this with a smile like recommending a soup. While this sounds very unusual, I think “Pizza Tie” doctor provided us with that direct clarity that we really needed to prepare for the worst. So I think the direct answers in a way were the most compassionate and most informative. He didn't know the answers, but he gave us his opinion. I think that that was great. So second one is honesty. The third one is very unanimous along parents. If you're listening to this, you've probably received it, you're probably in the weeds. But we want to learn the diagnostic algorithm. What this means is what is the decision tree of options. What are the options of diagnoses? What are the pieces of information we need to go through to eliminate those options? We loved knowing this. I'm sure that as a physician or a nurse or a caretaker, your options are more vast and maybe you want to give us a pared-down list so we don't have unnecessary worrying. But I think, for us, the information and the education was incredibly empowering. The fourth one is parents network and Facebook. As you guys all know, there's NICU groups, there's interstitial lung disease groups, there's ACD groups, there's lung disease Facebook groups. There's so much activity in these Facebook groups. During our research in the hospital, we not only use these Facebook groups to learn about the disease, we learned what is the quality of life going to be with these diseases. Then, we also connected with other parents. I think that that has been the most impactful. There's a child in Florida that's a few years old and I am friends with the mother. While we were in the hospital, she was a few months ahead of us and she gave us so much advice about NICU, questions asked, things like that. Thank heaven her son is still alive and breathing. It's such a miracle and I am still so grateful for her. Another parent, an ACD parent, ended up moving down the street. Another ACD parent ended up being the DJ at our Children's Hospital fundraiser. Lastly, the last ask for number five is when you are escalating intervention. Let's say you're on high-flow nose cannula or you're intubated, preparing us for what would it look like for the next intervention if the worst were to happen would really help remove some of the trauma. For me, one of the darkest memories through Owen's life was the day he crashed and went on to ECMO. I will never forget when the nurse ripped off the band-aid, he wasn't breathing, he started to turn blue. I couldn't do anything. And all of a sudden, the room filled with 30 people. It was so traumatic. Whenever I'm in a doctor's office or in a hospital room, unfortunately, I've had this experience again with my dad and nurses flood in. I completely lose it and panic. I've had to do a lot of EMDR therapy for it. But I think what would really help, and my ask for physicians and doctors is to help prepare us. If you are at intervention intubation, tell us what would happen, what are the conditions that would be required for us to go to ECMO. Or if we're on an high-flow nose cannula, what oxygen do we go to intubation or things like that. I think talking it through, maybe even getting the paperwork ready so it's not a last-minute decision with ECMO would really change it for the parent, because it is something that I've continued to have to work through to be able to go to a doctor's office or if there's a lot of people running somewhere quickly. I know that you can't stop these emergent situations, but preparing us is that. Owen had three moments of joy. Well, hopefully, more than that, but there are three call out moments of joy and they would not be possible without the physicians and the nurses and the research that has made this all possible. Without that ECMO bridge to more time, we wouldn't have got this. So I'm just so thankful for the medical staff and doctors. So thank you to any of those listening. The first moment of joy was actually planned by one of his nurses, and it was night staff and our on-call attending, Dr. Paul Roseanne even made an appearance at 1:00 AM for his two-week birthday party within the hour. A nurse brought hats, balloons, and one respiratory therapist was a trained clown and made blow-up balloon animals, which escalated to sword fights in the hallway. Night shift is way more fun. We spent every night at the hospital sleeping with Owen in the room. So we were able to take part in all of that fun. ECMO, albeit a bridge to nowhere, let us reclaim a traumatic departure and have these happy moments like this birthday party. The nursing staff was just absolutely incredible and thought of everything, and we're so grateful for that one. The second moment of joy wasn't really a moment, but a series of moments. Dr. Kinsella, our attending ordered physical therapy while balancing that we're in palliative care decision-making. I think that the physical therapy brought increased connection, engagement, and positive interactions to Owen and to us. He had a little maraca, he would shake, at least I would claim that he would shake. Maybe he was accidentally shaking it. It's unclear. He was only a few weeks old, but I would say he was very advanced for his age. He also had this little light up crab and that was all gifted from physical therapy. I think when you're a little baby and all the physical touch you're having is pain, being able to introduce some positive movement let us take control back from the situation. The last event, and I don't know if everyone is going to be able to do this at their hospital, but we might have broken some rules. It's unclear, but we were able to get Owen onto the helipad to see the son before we removed care and he passed away. Owen was so captivated by the surroundings and the sun. I remember him just turning his head looking back and forth up at the sky. He was a little cold and he did not like the wind, but the sun and the sky, he was just enamored with it. It just rejuvenated us all. The photos of us on the roof being happy, we were singing to him, it was just the most cherished memories of my entire life. I think that that is something that doctors can help enable in the future, is allowing these sort of special moments to come through, whether it's outside or whatever. After Owen passed, we were a little lost. We knew we wanted to have kids. Man, having Owen and just experiencing that unconditional love, it got us hooked. We really wanted to have a family. We love Owen and we want to have more kids. That has not been in the cards for us yet. We were in Boulder, Colorado. We had bought this house for Owen. Within three weeks of knowing that I was pregnant, we were like, "We need to get a house." We bought a house for him. We just couldn't live in this five-bedroom house alone anymore, so we're renting it out. We're moving back to San Francisco. But what we're doing while we're waiting as a positive is we're doing a lot of fundraising, hearing what doctors are doing and the research. If we can help fund more private investigators to write more papers or to travel to these conferences and learn more, I want to make that happen. So, we are fundraising, we're just going through our local and that they're distributing it to things like this conference to get more speakers at this conference and things like that. We host an epic rager where it's in Owen's honor, but we really want it to be a fundraiser and a positive thing. We had a band, we had it catered, we had glow sticks. We're really trying to bring joy and happiness into making a change and to finding answers to finding solutions and helping the kids that haven't been born or the kids that are still here with us make their lives better. So that is my passion. That's what I'm working for. We thought about trying to switch and become doctors or something, but we've decided we're just going to keep working and just donate money. So that's what my passion is. That's what we're doing. It's really an honor to be here and to get to hear the research that they're doing. I hope to further that. My name is Victoria McKinnon, and I'm aware that my son was rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 469 - Victoria McKinnon
05/20/2024
Episode 469 - Victoria McKinnon
Victoria McKinnon shares her personal experience with pulmonary hypertension, specifically her son Owen’s eight week battle with the condition. Owen was diagnosed with alveolar capillary dysplasia (ACD), a genetic disease that causes pulmonary hypertension. Despite initial improvements, they ran out of treatment options and ultimately had to make the difficult decision to remove care. Victoria emphasizes the importance of communication and honesty from healthcare professionals, as well as the support and knowledge gained from joining online support groups. She also discussing her fundraising efforts to support research and find solutions for children with PH. Learn more about Victoria’s fundraising efforts: Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Wolf Haley - phaware® interview 468
05/14/2024
Wolf Haley - phaware® interview 468
Haley (aka @Wolf Haley), who was diagnosed with pulmonary hypertension (PH) at 18 years old, shares her journey with the disease and how she has found healing and purpose through art and advocacy. Haley discovered her passion for art as a form of therapy, using watercolor to express her thoughts and emotions about living with PH. Haley also works as a TikTok manager for a nonprofit organization and has become involved in advocacy work, using her personal experiences to advocate for healthcare bills and share her story with legislators. She graduates from the community health worker program at UNM this month, and plans to continue studies in radiology technology. I'm Haley. I was diagnosed with PH in 2010, a fresh 18-year-old. I'm from Texas, but I relocated to New Mexico, which truly worked in my favor, because now I'm a patient at one of the specialty clinics out here, UNMH. When I transferred to UNMH, I was a pretty angry non-adherent patient. I just hadn't been well-educated, honestly. I hadn't been taught about the disease process. Dr. Melendres was the first doctor to tell me that this was a progressive disease. They had a different approach. They had a social worker and a whole team of people that were geared to help me deal with everything beyond PH. I was really resistant to trying new therapies, especially the pump. They were begging me, but then also respecting my decision as a patient, because they were trying to get me to a place of acceptance. I survived being put on the pump. It was an emergency admin. I can't believe I lived through that, because I was in really bad shape. But I caved and got on the pump. They totally, completely transformed me as a patient, that medical team. So, University of New Mexico is wonderful. At least, wonderful for me. They worked really well for me. I was put on the pump seven years ago, but I've been off two years. So, I was on the pump for a little over five years. Honestly, I was totally prepared to be on it for the rest of my life, because that's what we knew at the time. One day I went in for an appointment and they said, "Hey, we have a new therapy we think you should try, because line infection risks and whatnot." My numbers had come down. I was doing really, really good, very consistent with my therapies and whatnot. It looked like my body was responding well enough to take the risk of moving onto an oral therapy. Because it's a calculated risk. Everything's a hypothesis, and they don't want to risk something if your body doesn't look like it's ready to do that. Mine was, and we transferred to an oral therapy. It was an ICU stay. So, I've been on that. Did my body absolutely love it? No, I mean, the pump's super strong. Now, we've been adjusting therapies around that and participating in clinical trials. I'm still doing well. I'm still stable, so I hope we can keep going down that route. But I know if things progress, whatever, I'm totally willing to go back to the pump. I'm familiar with it, and it is what it is. I moved to where I am now, a super isolated town in New Mexico. I just had really nothing going on. I had been on the pump for almost a year. I was processing really heavy, awful things. I had a patient friend pass away. I had a massive panic attack, actually. I thought I was having a PH episode, because my heart rate and blood pressure was so high. It was a panic attack. I started going to therapy. I would come home and I was doodling. I was drawing different things, different ideas I had. I always enjoyed art, but I never knew much about it beyond that, never educated in it. I started doodling. I found a watercolor palette in my house. It was really old and awful. I started having fun with that and getting into watercolor. The first one I ever did, I had a couple of doodles, but the first one that really launched everything I did, sitting in the chair with the pump. Then, I had a sign behind it that said, "There is no normal." Then I would start heavy phrases that I couldn't get out of my mind, "Do I have worth?" Or, "Be present," and different things like that. I would start drawing them. It was like I was putting thoughts I had or feelings I had in a visual form. It was really subconscious, basically. Then, I realized what I was doing way later. My therapist realized it too, and so now she encourages me. Now, I know that it's therapeutic, but that's how it starts. It starts as a phrase or something that's bothering me and I'm like, "What does this look like?" Because it's a big concept, usually. Like mortality, I have a painting about that. I literally said to my therapist in a session, "I scare people because I'm so comfortable with death. It has its own place in my house, I feel like." She's like, "Is that your next painting?" I'm like, "Actually, yes." It's concepts like that that I'm working on. It totally happened subconsciously. I'm glad, because now I don't think I'm linear enough to write as well anymore. I think art is a little bit healthier for me. I started sharing it on social media. I didn't really care about the reaction as much. It's so wild. People who are not patients do not have a good response to it, quite honestly. Most of the pictures are nudity, which I know is a lot for people, but I never knew why I was doing that. Then, I had a friend go, "I think you're just showing the vulnerability. It's just the ultimate vulnerability of who you are as a person and how you are in these medical settings." We have a cloth draped over us, and that's about it, our hospital gowns. But people who are not PH patients, nine out of 10, or unfortunately who are just healthy beings, do not have a good response to it. They don't understand it. PH patients, those are the people who like my artwork. They love it. They connect with it. They share it. They reach out because of it and they purchase the pieces. I had a patient who bought two pieces from me, and she has a really intense story about her diagnosis and things she went through. I could not be happier that she bought the piece she bought, because it was so personal and emotional for me. Then, to know her story and why she wanted that particular piece was overwhelming. I've had other people who are not PH patients, who are just chronic patients or healthcare providers, so that's social workers, nurses, healthcare providers and patients. Those are the people who appreciate my work, which makes me feel good. I feel like I am going through phases. So, I feel like those are my introductory pieces of when I started my work and I was introducing myself and what I was dealing with and working through. It's a lot of watercolor and being on the pump and different things. So, I compiled some words, because they are individual puzzle pieces, and they're fine on their own, but every time I've hung them all together and displayed them at a gallery or just my studio in my house, people are like, "Oh my God." Seeing it all together, it's like puzzle pieces coming together. It's powerful. A friend who's a professional artist was like, "This needs to be in a book. You're a storyteller with these pieces." So, I took my early pieces, the watercolor and what I had to say about it and how I got to this point in my life and my work, and I put it all into a little coffee table book and just did the beginning. So, I called those first set of pieces that are in the book, There Is No Normal, because it was introductory of there is no normal. This is my normal. We all have our different normals that exist. Now, all the pieces that I'm currently working on, I call it, Transition, because I literally spent the last two years working on transitioning off the pump, keeping my health away from the pump, and then also missing the pump. Sometimes, I still hear it beep or sometimes I get up and I still think it's attached to me, and I've been off it two years now. So, it's been a process. Also, learning how to trust my body not having the pump. I didn't realize the emotional impact it would have of learning how to trust my new therapies, because I saw what the pump did for me and how it did keep me alive. I had such trust in it. So now, I'm developing a whole new trust. So now, I'm painting about that. Maybe I'll compile all my newest paintings, Transitions, into another book. So, it's like they're their own categories or series. I've always been an advocate. I saw that this nonprofit was hiring, and I applied. As we all know, being on SSDI, I have strict income limitations. They were like, "Oh my God, that's incredibly low." It's very hard to find a job on SSDI. So, they literally created a job for me managing their TikTok, because I do TikTok on my own. They were like, "Hey, you should do TikTok for us, and we can match your income limitations." It's great. I literally just got involved, because I needed a job and this felt like a good fit. I just tried my best and they actually hired me. I've learned how to argue professionally, because you're at the Capitol. And during legislative session, which is two months long in New Mexico. You're at the Capitol and listening to people argue these bills. You're learning different talking points. You're learning how to go about things. So, it's been really educational in that way. I'm learning about different bills, like last year, the Safe Staffing Act for nurses in New Mexico. It died, unfortunately. So, that's a bill I plan to get behind next legislative session. It's totally branched off into other things. I've been able to argue for abortion bills, which I know it sounds super inflammatory, but that goes hand in hand with women's healthcare and PAH. So, arguing for that statewide and keeping that here in New Mexico is really important to me. I've been able to tell my story to our audiences legislatively. So yeah, it's been really cool. I've learned a lot. Just seeing how all of it works, it's very overwhelming, but in a positive way. It keeps me fired up and channels my anger into something productive, I think. We didn't politicize our healthcare, unfortunately. That's just what we're living in. Whether we realize it or not, the decisions they're making do impact us. They trickle down. Whether that's abortion or prescription medications and all the healthcare bills that affect our providers, the Safe Staffing Act for nurses, there's all kinds of stuff. That impacts you as a patient and especially a specialty patient. So, anything that you can do. Know your legislative season in your state. When does legislative session begin? When does it end? Is it year round? Because some states are year round. Who are your representatives? Who can you introduce yourself to? The Capitol is your building. That's the people's house. You are allowed to show up. Know the schedule. They usually have days where they're focused on one topic. Yesterday, for us, was public health day, so I was down there. So, just go down there, know your representatives. Figure out where their offices are. Agree to sit down with them, see if they have time to talk with you. It feels like a really big separate world, and it will feel that way, but that's where you belong. That's where advocacy happens. So yeah, don't be afraid of it. It's very intimidating. I think it's meant to be that way, but no, don't be intimidated. I say go in there and blow the doors off the hinges. Professionally, and not literally. One other important avenue that I took as far as advocacy was writing for the Pulmonary Medical Journal about my experience being a non-adherent, angry, non-compliant patient. Just telling the truth about that and the process of that and how accepting my illness and working with my medical team, becoming an active participant and my healthcare saved my life. I literally wrote it when I wrote my first draft in ICU, being transitioned off the pump. I took my keyboard and just stayed busy and got it done. It was so wild having medical writers, because I write from an emotional point of view. Obviously, I'm an emotional being. Writing from that stance and then having them go in and then fix everything to medical and showing me how to do that, that was truly wild. Then, going through the whole process, editing process for the medical journal, having it peer-reviewed. I was in the hospital in July and then it was published and actually accepted in January. So, it was a process, but I loved it. It was really, really cool. So, I enjoyed that. I think all of this has been stepping stones and unveiling who I actually am, who I've needed to become. I'm constantly evolving. Right now, I'm in school. Hopefully by a year from now I'll be in radiology school. Then, the next step after that is the cath lab, which is wild to me to be shooting for that, but I really enjoy the cath lab. So, I think this was all setting me up. I think I needed to harness the anger that I felt as a newly diagnosed patient, as just a lost patient. I needed to work with that. My team did that with me. Therapy has done that. Art has done that. Now, legislation and advocacy work has done that. So, I feel like now I can actually use it productively instead of using it to actively harm myself, which is what I was doing a very long time ago. So, hopefully I'll keep evolving. I'm Haley, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . 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Episode 468 - Wolf Haley
05/13/2024
Episode 468 - Wolf Haley
Haley (aka @Wolf Haley), who was diagnosed with pulmonary hypertension (PH) at 18 years old, shares her journey with the disease and how she has found healing and purpose through art and advocacy. Haley discovered her passion for art as a form of therapy, using watercolor to express her thoughts and emotions about living with PH. Haley also works as a TikTok manager for a nonprofit organization and has become involved in advocacy work, using her personal experiences to advocate for healthcare bills and share her story with legislators. She graduates from the community health worker program at UNM this month, and plans to continue studies in radiology technology. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Dr. Jason Weatherald - phaware® interview 467
05/07/2024
Dr. Jason Weatherald - phaware® interview 467
Dr. Jason Weatherald, a pulmonologist at the University of Alberta, discusses a study on the socioeconomic burden of pulmonary arterial hypertension (PAH) in Canada. The study, conducted through PHA Canada, surveyed PAH patients and their caregivers to understand how the disease affects their ability to work and perform daily activities. The results showed that a significant number of PAH patients were unable to work or had limited career options due to the disease. The study also highlighted the impact of PAH on caregivers, with many having to assist patients with daily activities. The findings emphasize the need for healthcare professionals to discuss the implications of PAH on patients' ability to work and access support. Read the report here: My name is Jason Weatherald. I'm a pulmonologist at the University of Alberta in Edmonton, Canada. I'm really excited today to talk about a new project called the Socioeconomic Burden of Pulmonary Arterial Hypertension in Canada, which was a study that we did in the fall of 2023 that was a national survey through , looking at how pulmonary arterial hypertension can affect patient's ability to work and to execute daily activities. We also asked some questions about how the disease affects their caregiver's ability to work. We know from previous burden of illness surveys that there's a huge burden of illness and that a lot of patients are unable to work or to work full-time if they are able to work at all. We wanted to dive down and uncover a bit more about how people are affected in terms of their ability to work. We used a validated measurement tool that's called the Work Productivity and Activity Impairment Questionnaire, which contains a whole bunch of questions about employment and activity levels. Through PHA Canada, we distributed this to their members and asked participants to self-identify about what type of pulmonary hypertension they had, provide some information about what medications they were on, some demographic related factors. We then asked them to fill out some quality of life questionnaires. This specific questionnaire, which abbreviated is called the WPAI, asking questions about productivity and impairment. So really excitingly, we've submitted some of these results to be presented at the , which is coming up in June in Barcelona. As people on the podcast probably know, it’s one of the biggest events in pulmonary hypertension that only happens every five or six years. We'll be presenting some of the more detailed results there. Also the summary of the results have been posted by PHA Canada in timing with . Just to summarize the results of this study, we administered this survey again, sent out by email in both English and in French in Canada. The study was coordinated through an academic research organization based at my university called the , who provided a lot of the logistical support to this. We had quite a few responses. We had 217 patients with Group 1 PAH who answered the survey. Based on the responses, it was fairly representative of what we think about Group 1 PAH affecting... The average age was around 57, and the majority of people were women that answered the survey. We had a reasonably good representation across the country and a pretty representative breakdown in terms of how people describe their functional class. When we asked questions about how PH affects their ability to keep and find a job, there were some really interesting results. 87% of respondents said that PAH limited their career options. Furthermore, 63% said that the PH prevented them from finding a job altogether. So I think that's really huge seeing that 2/3 of patients were really not able to work at all. Digging down a little bit deeper into that, even of those people who were employed at the time of the survey, which was only about 28% of people, a lot of them were actually not able to fully participate in their workplace or they reported diminished workplace productivity. For instance, 54% responded that they felt that their activity or productivity at work was impaired because of their PAH. Furthermore, 12% of these people missed work due to PAH-related symptoms or testing. I think this was really quite profound, not only in current workplace, but a lot of other people said that their PAH interfered with their ability to move forward in their work such as getting promotion or even seeking promotion. I think that's another important factor even among the people who are able to hold down a job. Interestingly too, because there were some older people or people further along in their career that answered the survey and about half of those people said that they would have to retire because of PAH or they were planning to retire early because of being diagnosed with PAH. Almost 1/3 of the people reported that they were effectively disabled by PAH. This questionnaire allows us to drill down a little bit in terms of more specific impairments related to the disease and how it affects people's ability to work beyond just saying, “I can't work.” There's a lot of factors going on. I think there were some other really interesting insights here in terms of how their caregivers are affected. We did look at who people had as a caregiver, whether they required a full-time caregiver and whether that was a family member. Interestingly, 77% of the people that answered this survey said that their partner or spouse had to assist them with just daily activities in their home. Another 29% of people said that their children had to help them with daily activities. This was not surprisingly also related to people's functional class. People with more severe symptoms were less able to do daily activities around the home. So I think this does have a lot of impact, not just on patients, but on their caregivers. To give a specific example, when we looked at the people who reported being in functional class IV of the most severe symptoms, they reported that their caregiver spent on average about 43 hours per week related to their personal or medical care, which is essentially a full-time job. So we're able to really quantify the impacts of this disease on patient's caregivers, which I think is unique. I think there are really broad insights and implications of this survey that, as a clinician, tell me a lot about what patients are going through. But also, it started to make me think a little bit more about what type of supports we need to help patients access. For instance, when we're talking to newly diagnosed patients about how the disease is going to affect them, we typically, in clinic, spend a lot of time talking about tests and prognosis and risk and treatment and treatment side effects, but we very rarely have time or even habits of discussing how this will influence people's ability to make a living or advance in their career. We often don't spend enough time talking about the impacts of this disease on caregivers either with the patients or with their caregivers directly. I think this survey really helps us understand the magnitude of this, and it highlights the importance of talking about the implications of the disease on patient's ability to work and also their caregiver's ability to work. This can have major repercussions in countries, for instance, where health insurance is tied to employment. That could be a crucial thing for patients to maintain in order to have access to medications. Fortunately, in Canada, the vast majority of the pulmonary hypertension drugs are covered and fully reimbursed through our universal healthcare system. Not all of them are covered, but access to medications isn't as big as of an issue in Canada as it may be in other areas. But certainly income being able to support daily needs is of course strongly tied to this disease and to the ability to survive. I think as clinicians, we need to make sure the patients understand this and then we need to start thinking about ways to support patients in doing this. If there's any one benefit of the recent COVID-19 pandemic, it's realizing how many jobs or responsibilities can be done virtually or from home. For those patients who are able to do that in work, it may actually help them maintain their employment. If they're able to pivot to doing things more virtually or remotely, it certainly has the potential of being much less exhausting for them and perhaps allowing them to still participate in their job in different ways. So what are the next steps here? I think one thing we want to watch out for and see whether new treatments that are coming down the pipeline, in addition to improving things like symptoms and quality of life, it may be very interesting to see how emerging therapies actually improve patient's ability to do these other things in daily life. I think this gives us a baseline. As everyone knows, sotatercept was recently approved in the United States and hopefully will be approved in other countries soon. There's a lot of other really exciting new therapies being explored and studied in pulmonary arterial hypertension. So now we have a bit of a baseline measurement. My goal and my hope is that in a couple of years we can repeat this and look at whether new novel therapies are actually improving, at least at the population level, some of these reported inabilities with respect to being able to work and do daily activities. The other thing I think it highlights is the utility in using these types of measures, for instance, in clinical studies or in clinical trials. It goes beyond just quality of life. It's about ability to support daily life and ability to do those things that make life meaningful, like find employment and for caregivers to be able to continue their employment. I hope that this study will highlight the utility and measuring those things when we're looking at effectiveness of treatments or programs in pulmonary hypertension. I'm Dr. Jason Weatherald, and I'm 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
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Episode 467 - Dr. Jason Weatherald
05/06/2024
Episode 467 - Dr. Jason Weatherald
Dr. Jason Weatherald, a pulmonologist at the University of Alberta, discusses a study on the socioeconomic burden of pulmonary arterial hypertension (PAH) in Canada. The study, conducted through PHA Canada, surveyed PAH patients and their caregivers to understand how the disease affects their ability to work and perform daily activities. The results showed that a significant number of PAH patients were unable to work or had limited career options due to the disease. The study also highlighted the impact of PAH on caregivers, with many having to assist patients with daily activities. The findings emphasize the need for healthcare professionals to discuss the implications of PAH on patients' ability to work and access support. Read the report here: Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD
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Karen Martinez - phaware® interview 466
04/30/2024
Karen Martinez - phaware® interview 466
Karen Martinez, a mother from Eastvale, California, shares her experience with , a camp for children with congenital heart defects and lung disease. Her daughter attended camp for the first time at the age of seven and has been going every summer since. The camp offers events throughout the year and fosters lifelong connections among the campers. My name is Karen Martinez. I'm from Eastvale, California. When I was pregnant, I was diagnosed with a child with a congenital heart defect. Now, I'm the mother of a child who attends every summer. I remember getting a flyer when we were at the cardiologist's office. I took it and put it away in my desk. A few years later, I came across it and I reached out on their website. They were having one of their first annual family get togethers over at UCLA. I had missed the deadline to RSVP for the potluck. They were so kind and open to having us still join. We went out there. We met with them. They were completely amazing, welcoming, loving from day one. After that, my daughter shortly turned seven years old when we sent her off to her first camp experience. Camp del Corazon is a camp for kids that are born with congenital heart defects, but campers may also have other accompanying medical ailments. So it encompasses a lot of different medical issues, but primarily cardiology related diagnosis. The best way to describe it's a summer camp for kids between the ages of 7 to 17, but Camp del Corazon also offers events throughout the entire year so that you can build a community with other families, other children that have congenital heart defects. When you're first diagnosed with this, I personally felt lost, completely overwhelmed. It was something that we didn't understand. I had nobody to turn to. To have a community that not just embraced you, welcomed you, but also gave you the support in a desperate time of need, it's just amazing. Sending her off our first year at seven years old, oh boy, many tears involved because we kept her safe, in our opinion. At seven years old we made the decision, okay, we're going to send her off for five days. Let's hope and pray for the best. But let me tell you, the first three days I cried, the next two days my husband cried. Finally, when we went to the dock to pick her up, when we saw her get off that boat, a huge smile from ear to ear. The first words out of her mouth were "Mom, in 365 days, I get to go back!" She just lit up as bright as can be. It set our hearts at ease. We just knew this is where she needed to be. She started at the age of seven, and this will be her last year. In August, she'll be turning 17, so she'll complete 10 years total that she's been going. It was only one year she missed because of COVID, but they did online camp. For us, we look forward to it every single year because we know this is the five days that she gets to disconnect from the world completely, technology, all that good stuff. She gets to be in a place where she feels "normal". She doesn't have any limitations, any restrictions. She's with people who understand her, who love her, who accept her for who she is. She doesn't feel like she has to have limitations or she's different because of her scar. We look forward to it because we've seen this little girl grow up from the age of seven to now have this confidence, this independence that we know is hugely in part because of Camp del Corazon. There's definitely a difference when I see her with her regular school friends versus her camp friends. It's lifelong connections that she has with the kids, staff, volunteers at camp. I think it's a unique bond that they have in what they experience, in what they have in common, that drives her to have a different connection with them. Yes, she has school friends, but there's a unique connection that she has with her people from camp. Just to give you a quick little scenario. I remember her growing up she would never show her scar. It was this thing where she always was covered up. One year, coming back from camp, I noticed that she started showing the scar a little bit. It wasn't until maybe a year ago we had a discussion and she said one of her camp friends helped her understand that the scar is who she is, who makes her understand where she comes from, how she's fought to live. It makes her unique. She embraced the scar. Even though as parents, we were telling her till we were blue in the face, right, exactly what the friend told her. But hearing it from somebody else who's her age and from camp made her understand, wow, it does make me special. It does make me who I am. It's something to embrace and look as beautiful and not ugly. How other people might incorporate it when they see her. Coming across different families and different people that I've connected with throughout the years, I will for sure, for sure describe my faith as one of the most important things that have gotten us through. Initially, like I said, it was overwhelming getting the diagnosis, not knowing where we were headed. By far, it's been our faith above everything else, because of God she is here. The second thing that always automatically comes out of my mouth is talking about the greatness of Camp del Corazon, because like I said, it's not just a community, but the events and the opportunities that they allow these kids, they're overwhelmingly beautiful. They're things that I can never do for my child. I can try. I advocate for her, but in all reality, it's events, it's speaking to other kids that are going through the same things that she's going through that help her at the end of the day. So by far, it is one of the best things that I could always introduce another family to. I always carry flyers with me to share with people the greatness of Camp del Corazon. In order to find , you could obviously Google search them. Their website has all the information needed for registration for camp, which is now open. If for example, you live out of state, you're far and financially, you can't have your child travel to attend summer camp with Camp del Corazon, they have a virtual camp set up where familes are able to participate with activities. They get a beautiful special box mailed to them prior to the event. They'll be doing all of the activities online via Zoom. It's just a wonderful opportunity so that if you can't be there in person, you could do it virtually. It's just amazing as you see everybody's faces light up when you are doing the activities, engaging with the different groups, the breakout groups. It’s just overall fun. We sign up for it as well. One of the things I do want to emphasize is when I sit down and imagine the cost of camp, what would it cost me to send my child to camp? I have to take into account not just the fact that there's cardiologists there, nurses there, the feeding, the shelter, everything. It would be a crazy amount of money that would be spent sending my child here. But thank God this organization makes it where it's free for me to send my child to summer camp. And for me as a camp mom, what I feel, and I've never been told I need to, but out of my heart, every single year, I fundraise in whatever way I can to turn around and be able to give back to camp. In my heart of hearts, I feel it's important not just to spread the word, but to keep this organization going. So we've gone from selling World's Finest Chocolates to now hosting Queen Bingo Nights at Hamburger Mary's, because we've made it a priority for our family to fundraise and put the name out there for Camp del Corazon. Not because it's an obligation, but because we choose to. We truly believe in what they do and what they provide for our child. From the bottom of my heart, I will never be able to express the gratitude I feel towards Camp del Corazon, every staff member, every volunteer, every counselor, every cardiologist, nurse, etc. Ultimately, they give to my child something I could never, as a parent as I wish I could. I'm lost for words when it comes to this, because genuinely I am so grateful. I can speak days and days of how wonderful they are, and I truly appreciate every single thing they do. They are a reason my child has become who she is. This is Karen Martinez, and I am aware that my daughter is rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 466 - Karen Martinez
04/29/2024
Episode 466 - Karen Martinez
Karen Martinez, a mother from Eastvale, California, shares her experience with , a camp for children with congenital heart defects and lung disease. Her daughter attended camp for the first time at the age of seven and has been going every summer since. The camp offers events throughout the year and fosters lifelong connections among the campers. #campdelcorazon #heartcamp #camplove #supportagoodcause Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Joellen Brown - phaware® interview 465
04/23/2024
Joellen Brown - phaware® interview 465
Joellen Brown shares her experience with pulmonary hypertension (PH) on the 15th anniversary of her diagnosis. Joellen was born with a hole in her heart and had her first open heart surgery at the age of two and a half. She believes she may have had PH since birth, but was not aware of it due to limited research in the 1950s. Joellen emphasizes the importance of having a supportive medical team and family, as well as being proactive in advocating for one’s own health. She encourages newly diagnosed individuals to seek out support groups and emphasizes the need for early and appropriate treatment. My name is Joellen Brown. I am in Newport Richey, Florida now. I was diagnosed 15 years in May with pulmonary hypertension. I was born in 1956 with a hole in my heart. Back then, there wasn't much information on correcting it or how they were going to go about doing it. I was about two and a half when I had my first open heart surgery and they put a patch on my heart. I went through the majority of high school, not being able to take gym, because they really didn't know if that patch was going to be pliable to exercise and all that. I was also told not to have any kids, which thankfully, I didn't listen to that. I ended up with three girls and no issues, and they were healthy. I've got three older sisters. I was the only one with the heart issues, so it wasn't anything like hereditary or anything like that. When I was about 40 years old, they told me that I needed to have another open heart surgery, because I had a tear in my aortic valve. So they went in and I have a cadaver valve. In my early '50s, I started noticing major weight gain and really breathlessness, tired all the time. I had been seeing a cardiologist all my life and nobody had ever mentioned that it was any kind of a lung or heart issue, it always had to do with the heart. I finally went to my regular doctor and she had me walk around the hall. At that time, my oxygen was 67%. I was sent home on oxygen. That's how my journey with pulmonary hypertension went. They first thought I had asthma, so I was put on an inhaler with the oxygen. That didn't work. I went to another cardiologist who was also a PH specialist. He's the one that diagnosed me with PH. But he had told me too, that I might have been born with it. But then again, back in the 1950s, there wasn't a lot of research on PH. So who knows? I might've had it all this life and not even realized it. So all that time, it's always been the heart thing. I'd always gone to the doctors. I didn't really have to worry about my weight, until after I had my kids and I had my second open heart surgery. That's when my weight started creeping up on me, which might've been back then, part of PH, I don't know. So now 15 years later, they're saying that my PH is from congestive heart failure and scoliosis, which is what I was born with. I was born with scoliosis, with the hole in my heart, so I have that against me as well. I think at the very beginning, I had a wonderful PH doctor. My team is what really got me through everything. But then again, that's well and good, but you also have to have that family support as well. Great, the treatment is helping you and everything, but you really need that mindset of having the support of your family to be there. Everything kind of fell into place when I was diagnosed. I want to think that I was one of the lucky ones. I didn't have to go through years and years and years of doctors. I was a special case, because of my open heart surgery at such a young age, on top of having the pulmonary hypertension. My cardiologist at the time, was also a pediatric cardiologist, and that kind of helped. Whenever I went in for any kind of test, they always had to work with my body and my issues. It wasn't like the same thing that they would do with somebody else. I want to think that it was all to do with the doctor I had at the very beginning. I am divorced. I'm pretty lucky that I'm able to still take care of myself. I'm a mother that I tell my kids everything. I don't want to keep it secret, because I want them to know that if anything ever happened to me that I can't speak for myself, I want them to know what I would want. So I know there are some other people out there that are like, "My family has no idea what I'm going through." I tell my kids pretty much everything. They've gone to doctor's appointments with me. They've been there when I've been in the hospital, and that has been wonderful. Right now since I'm in Florida, I have two of my daughters that live very close. That's been really helpful. But I'm not driving now too, so that's been a challenge in itself. But moving to Florida, my health has gotten so much better. My sister and I live together now. My lifestyle is much better because I do feel better. I think this was a great move for me health wise. I've lost weight finally after a year and a half. I have a great team. That's been probably the hardest part, moving. I look back at several years ago and how my health was at that time, which was not very good, and how much it is today. It's really has improved. I'm really thankful for that. I think that's a lot to do with your mindset and the team and fighting for what you need. It's definitely a journey I don't wish on anybody. The one thing that I always tell people that are newly diagnosed, because I've been on several sites on Facebook, is everybody is different. What's going to work for me might not work for you, but getting the right doctors is the best thing. But my biggest thing that I tell people is, everybody is different. You need to get that treatment right away. It's not a wait and see type of disease. It's not going to go away on its own. You need to get that right treatment. You have to be your own advocate. You have to be out there and ask questions. There's no right and wrong answer, but you need to know, because you only have this body once. You need to figure out how it's going to affect your everyday life. I had some great mentors when I first got PH, on Facebook, that are no longer here, so I try to remember what they told me to tell other people. I was born sick and I had to overcome a lot of stuff when I was little. I think that has gone on to me as an adult that is sick. I'm on oxygen now 24/7. I will always be on oxygen, so I try to be an advocate for getting the right type of oxygen I need. I don't know what would've happened if I didn't have the hole in my heart when I was little and all of a sudden, I'm 52 years old and I've got this life-threatening disease. I don't know if things would be different. It's still a shock, but I've always had people telling me, especially when I was younger, they were cruel to me at school, telling me I was going to die when I was 13. You live through that and you're like, "Okay, well, I'm 14 and I'm still here." I have more appreciation for my mother as I'm older, because I can't even imagine what she went through in the 1950s, taking me on the bus to go to the hospital and all that stuff. I can't even imagine that. What brought me here was my grandson. He lives across the street. That's my motivation to be well enough to see him grow up. That's me doing what needs to be done to keep me well. If it means staying in a lot more to stay healthy, then I'll do that. That's my motivation and just to be in spite of everybody that said, you can't do something. Just moving from Ohio to here was a trip and a half, having a friend of ours that had a horse trailer that moved all of our stuff here. Of course, I had to fly because I couldn't do the long trip. I still think we've been here two years and I'm like, "Did we really do this?" A couple years ago, I probably wouldn't have. But it's one of those that you wake up and you're like, "I'm ready. It's time." Where I used to live in Ohio, it was kind of an independent living senior place. Out of all those people, there was one lady in there that had PH at that time. I'm like, "I can't believe that I lived with somebody in this building that has the same thing that I do." She was on oxygen as well. Well, she ended up passing away. She was older than I was. One thing I would like to tell the people that are newly diagnosed, the best thing is to get into a support group. That really helped me, being around other people that have the same disease. I went for many, many years, took my mom with me. She enjoyed it. Learned a lot about pulmonary hypertension. I have not been able to find one here in Florida, so that's going to be on my bucket list for this year is to find a support group near me, or at least someplace I can get to. My name is Joellen Brown, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 465 - Joellen Brown
04/22/2024
Episode 465 - Joellen Brown
In this episode, Joellen Brown shares her experience with pulmonary hypertension (PH) on the 15th anniversary of her diagnosis. Joellen was born with a hole in her heart and had her first open heart surgery at the age of two and a half. She believes she may have had PH since birth, but was not aware of it due to limited research in the 1950s. Joellen emphasizes the importance of having a supportive medical team and family, as well as being proactive in advocating for one's own health. She encourages newly diagnosed individuals to seek out support groups and emphasizes the need for early and appropriate treatment. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Chelsea Price - phaware® interview 464
04/16/2024
Chelsea Price - phaware® interview 464
Chelsea Price shares her experience living with pulmonary arterial hypertension (PAH). She emphasizes the importance of having a good support system, including her church community and connecting with other friends with PAH. Chelsea is grateful for the good days she has and strives to be an active mom for her children. Learn even more about Chelsea and PAH at . My name is Chelsea Price. I live in St. Louis, Missouri, and I have pulmonary arterial hypertension (PAH). Shortly after I had my second son, I noticed that my endurance had decreased. I grew up playing soccer, competitive cheerleading, and I was always very, very active. But after he was born, I had trouble going up steps, just couldn't catch my breath. We'd be out in the yard and we'd be playing. The next thing you know, my husband turns around and I'm laying on the ground just gasping for air and trying to catch my breath. I kind of pawned off on being a new mom, not having the time to exercise. My doctors thought I had anxiety. It wasn't until September of 2019 when my husband and I were on a trip to New York that our view of something being wrong kind of came to fruition. We were biking through Manhattan and we came to this hill and I remember being so frustrated, because he just took off right up the hill and I couldn't keep up and I couldn't catch my breath. I just threw the bike down and went and sat and felt like I was so out of shape. When we returned, I just kind of brushed it all aside. Again, well, my doctor said, I have anxiety. There's nothing else wrong with me. I was 32 years old at the time, and 32 year-olds don't get sick. After going back to the agency that I was at previously, I started having some chest pain and some back pain. Every day when I would go up the steps at work, I would be rushing to my desk to sit down because I would be seeing spots. Again, this all went back to me feeling like I'm a new mom, I'm out of shape and I just can't keep up. I started doing yoga and my back was still starting to hurt more. I thought I was having a panic attack, one day at work, and had asked one of my good friends to just go on a walk with me. There wasn't anything to be anxious about at work. It was just again, like you have anxiety, so obviously this is a panic attack. I went to the doctor the following week, which was February of 2020. They said, "Oh yeah, you know what? It sounds like it was just a panic attack." To me that didn't feel right in my gut. Something felt more wrong than just being anxious, so I urged my doctor to do an EKG in office just to ease my mind. My family has a history of heart issues, so that's of course kind of where my head went. They had spotted an abnormality on the EKG. The following week, my doctor had scheduled an appointment for me to do a stress test at our hospital, but that was also right in the midst of COVID shutting down here in St. Louis. Monday, I stayed home from work not thinking anything of it. I mean, it was COVID, but I was working from home. Tuesday, we kept our boys home from school. They were two and four at the time. Wednesday, my mother-in-law came to stay with us for the two weeks that we were going to be hunkered down. But Thursday morning I woke up and I was in so much pain, my chest hurt so bad, my back hurt so bad, and I'd never felt any pain like that before. So my husband drove me to the emergency room where they started running tests. They noticed that my pulmonary artery was three times the size that it should have been. At the time I did not know what that meant. They ran a ton of blood work and saw that I was in heart failure. They took me up to the critical decision unit, so I was going to stay for 24 hours for them to figure out what was going on. They took me in to do my stress test and had done the echo cardiogram first. They were like, "You're not doing the stress test. You're in heart failure. You have a hole in your heart," which I didn't know I had. It was about the size of a quarter, so 80% of my blood was just circulating in my heart and lungs, and only 20% was going to my body. Then, obviously compounded all of that onto having pulmonary arterial hypertension, which was diagnosed the next day during a right-heart cath. When I was in the hospital, during COVID, it was a very scary time because, one, COVID being a new thing, but also now having this condition that I didn't know much about, but the looks on the doctor's faces we're definitely saying that it's something serious. I was by myself because my husband wasn't able to come back in because of COVID protocols. I had an amazing team that sort of walked me through everything. I was put on oxygen, because my oxygen saturation was dropping into the 70s when I was in the hospital walking just over to the bathroom and back to my bed. They started treatments right away. I was discharged a few days after. I sort of stepped back into the world of now having this rare condition alongside navigating a COVID world. With having a diagnosis, it was something for me to take action forward, which isn't something that happened initially in terms of being an advocate for myself, speaking out about what had happened. I immediately hopped on Instagram, because I was so scared because the doctors tell you don't Google, and of course you do, and then you see all of the stats of things that may or may not happen in terms of life expectancy. I had two little boys at home and a husband that I wanted to see them grow up. I was so scared that I wasn't going to see that. Sometimes those fears still come up, but at the time it was just, "Am I going to see them go to kindergarten? Am I going to see them play soccer? Am I going to see them turn into little boys?" They were babies at the time. So having a plan for treatment definitely eased my mind a little bit. Once I was able to get up on the treatments and I started feeling better and I could walk around and I was able to get off the oxygen, there was so much more hope in those moments knowing, "Okay, it's going to be a battle and it's going to be hard some days. Some days I'm not going to feel good, but I'm going to be here and I'm going to have a good life and I'm going to be there to see them grow up and to experience everything with them." That's one thing that just keeps me going on the days that I don't feel good, is that I have these two amazing little boys and a husband of 10 years now that we can live a good happy life even in the midst of something that's scary, because I've responded well to the treatments. Having gone through it during COVID was scary. But honestly, part of me is happy that it happened when it did, because I was working from home at the time at a place that was extremely understanding of what I was going through. So when I was starting up on the medications and having some really tough days with symptoms and side effects, I was able to lay in bed and to rest and to recover and to hop online for meetings if I was feeling up to it. So I'm glad COVID happened when it did. I'm glad I got diagnosed when I did, because I could not imagine trying to go into an office while battling the initial stages of the disease and getting up on treatments. For the most part, I tend to try to live in the moment, and that's been a blessing with this disease, because I am 100% type A - go, go, go. Things have to be perfect and all of that. I always wanted everything to be controlled and I wanted to be in control of everything. I think the one thing that this disease has shown me is that you can think about what may or may not happen in the future. Most times, our minds tend to wander further than things are actually going to go, or at least in my case. So I tend to focus, and actually have for the past almost I guess four years, focused on day to day, doing what I can and focusing on what I can control, taking my medications, eating good, walking when I'm feeling up to it and things like that. I don't really try to linger too much on the future, because it scares me. I think there's a lot of hope coming, hopefully with some new medications that are coming out. I really honestly, in the past couple of years, haven't thought to the future too much. There's been one time since diagnosis that I've really thought about that, and that was a few weeks ago I had some symptoms and things like that after traveling, and that's kind of where my mind went is, "Oh gosh, is it progressing? Am I going to be here?" But everything is looking good and I'm doing everything that I can to control what I can and enjoying every single moment that I have with my family and my friends. Even before I was diagnosed, we were somewhat involved with church, just different things in our past that we've been through, we've lost a child. So getting a diagnosis, I leaned into my church family, I leaned into my faith, because it's not something that we can carry on our own. So I'm thankful that I have my faith, that I have an amazing church family. My friends and my family have been incredible post-diagnosis, dropping off food. My mother-in-law ended up staying with us for months to help me and help with the kids and my husband. We just have such a good network that without them, I think it'd be a lot darker. I also, post-diagnosis, found a ton of other friends that have PH that I text with on a daily basis that when I'm having a rough day, we can commiserate. One of my friends and I actually titrate up at the same time on a medication that was really challenging to get up onto. So while I don't love that we both were going through that, I'm glad that we had each other, because I think it made it a little bit easier. We were able to cheer each other on. Community is so important, and having those friendships and people that will be around you that may not understand exactly what you're going through, but can see that you're in a moment of need and can step in and help hold you up is so important. Day-to-day now, I think sometimes I forget how sick I am. I'm able to go play with my kids and cook dinner and play soccer with them, which has always been a huge passion of mine. That was one of the big goals that I had post-diagnosis and talking to my doctor, is I want to be able to be the active mom, I want to be able to go play with my kids, because my parents were always out there playing with me and I don't want to sit on the sidelines. It took some time and I did have to change my mindset a lot when I couldn't do that initially. So again, it just goes back to just trying to focus on the day-to-day and enjoying every single moment. Right now, I'm thankful that I have way more good days than challenging days. My name is Chelsea Price, and I'm aware that I am rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 464 - Chelsea Price
04/15/2024
Episode 464 - Chelsea Price
In this episode, Chelsea Price shares her experience living with pulmonary arterial hypertension (PAH). She emphasizes the importance of having a good support system, including her church community and connecting with other friends with PAH. Chelsea is grateful for the good days she has and strives to be an active mom for her children. Discover even more about Chelsea and PAH at Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
/episode/index/show/phawarepodcast/id/29565718