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

Kevin Allen - phaware® interview 552 12/24/2025

Kevin Allen - phaware® interview 552 The Pulse of a Fighter: Survival, Stigma, and Shaking Up HealthcareThey told him he had months to live. He used them to rewrite the rules. Kevin Allen survived near-death, systemic medical neglect, and an invisible disability most physicians don’t understand. Today, he’s pushing policy, pressuring universities, and pioneering new models of PH patient-centered care. My name is Kevin Allen. I am a psychological science pre-med major at Texas Tech University in Lubbock, Texas. I was diagnosed with pulmonary hypertension on June 15th, 2020 when I was flatlining in ICU at 3:00 AM. I woke up to a man named Dr. Victor Test, who's a world-renowned pulmonary hypertension specialist. Shortly after that, I went on to spend a month in the hospital. I was on dialysis. I thought I had two hours to live. They just played it by ear from days to weeks. By the end of that month, I told him I was going to dedicate my life and education and career to helping patients with pulmonary hypertension. So far, I've lived up to that. Almost immediately, once he got me when I could walk again, because I couldn't walk for a while, he had me visiting patients. I created a support group in Lubbock. I reached out to the Pulmonary Hypertension Association and said, "Here's what I do. Here's what I've been doing. I would like to have a relationship here." They adopted Lone Star Lungs as one of their support groups on their website. That's helped me a lot, because I can arrange support groups online and advertise it a lot easier than I could just on my own. That's how I'm connected. I live with pulmonary arterial hypertension. Right now, I'm 33 years old. When I was 25, I was really big into working out, wanting to try to push into bodybuilding. I was real big on supplements. I would work out every night. I had a partner whom I was very serious and committed to at the time. In the gym, when you're building muscle and you do more reputations and you add weight, the idea is to gain muscle and lose weight. I started doing the reverse. Instead of adding weight, suddenly I couldn't do as many reputations. Then, it went from I can't do as many reps to I'm having to decrease the weight that I'm using. I was like, something is very wrong. That should not be happening. My next indicator, later that year, at 25 years old I started experiencing problems keeping up with my partner intimacy-wise. That was very odd to me. Once you get past the whole ego thing of this is embarrassing, it's concerning. 25-year-old males should not be experiencing those problems, coupled with the workout regression. Shortly after that, I just got to where I couldn't lift weights. I couldn't cycle. I couldn't do cardio. I just started gaining more and more weight and I developed a cough. For a long time, I didn't know what that cough was. What it was was fluid building up. Eventually in January of 2020, I started passing out. At first, I would be walking and I would feel like a tickle, like you've got a cold or something. I would cough. Eventually, that cough turned into three or four coughs. Then, I'd black out. I could be standing up, walking around, black out. It was fluid building up in my neck and everywhere. I was gaining more and more weight. For a little bit, I hit a threshold where I didn't gain. I just kept coughing and coughing. I gained something like 40 or 50 pounds in a month. It was just rapid water weight. My lips were purple. I worked at a school called South Plains College, a community college, during the pandemic. So everyone's freaking out, oh God, this guy's coming in here, coughing his head off. I was hiding in bathrooms coughing, because I knew I would pass out. I was like, no, they're going to send me to the hospital and think it's COVID. It's not COVID. I went to eight different doctors trying to tell them, "Something's going on, something's wrong." I begged one female doctor. She ran a couple of tests on me and a CT. She said, "I'm not saying this, because I'm not a cardiologist, but you have, to me, all the indications of someone who's fixing to have a massive heart attack. I'm going to refer you into a cardiologist.” During that process, I tested negative for strep, COVID and flu six or seven, eight times. They were convinced it was something else. I was like, "No, it's definitely not, but okay." I went to the hospital. I see a cardiologist. He said, "Oh, well, you're young and all these problems you're talking about, you're just overweight and that's what's going on. I don't see anything here, but I'm going to order a medical vest and have you wear it. We'll see you in a month and see what happens." That month didn't happen, because that same week I hit my 169th pass out right as I was going into the hospital. All I remember from that point is coughing. I was paralyzed with my mouth stuck open. The doctors and nurses, they thought I was joking around playing. I'm sitting there paralyzed, unable to move with my mouth stuck open. To be honest, all I wanted to do was go to the bathroom. Then sometime that night by 10:00 or 11:00, Dr. Test was involved. They're fixing to hit me with defibrillators. They had a line of nurses and doctors. They brought my family in. I said goodbye to everybody. They were sure I was fixing to go. They didn't know if it was COVID or what. All their tests were coming negative. They had 13 IVs coming out of my neck. Somewhere in there, Dr. Test, he didn't know what to do, because I wasn't responsive. Grabbing him and saying, "Help me please," was the only thing I had done in all those hours. Don't know how I did it. Don't know where that strength came from, but the moment I did that, I could watch him around the room doing everything he could trying to figure out what was going on. He took control of the room. Right as I was about to flatline somewhere around 3:00 that morning, he tried a medicine called epoprostenol, which is Veletri. I'm so close to flatlining, I like to say I flatlined. I didn't quite get there, but it was like half a second there. My heart started beating again. In fact, I think one of the doctors grabbed the nurse with the defibrillators, because they were afraid they were going to shock me and undo everything. They went on to pull off 144 pounds of water weight from heart failure. My God, they did more procedures than I can honestly remember or even count. I was on a table multiple times with doctors terrified to touch me, and Dr. Test would have to tell him, "If we don't do this, we're going to lose him. Go ahead and do it. He's stable. He's just really sick." Just a lot of procedures that I don't fully remember everything about anymore. It took a whole month to get me out of the hospital and get me stable enough where I could leave, I could walk on my own, followed by about a year's worth of recovery at home to where I could walk around, and I could be what for me is normal as far as living and walking around-wise. During that point, once I recovered enough, I had a bunch of professors reach out to me. I'm assuming during the pandemic, a lot of people disappeared from school and everything went online across the US. They reached out to me. They were like, "Where'd this student go? He had all these high grades, all these scholarships, and he just goes off radar." One of them, she reached out to me. She recently passed away this year. She was a dear friend of mine, Dr. Christina Garrett. She took it upon herself to figure out what happened to Kevin Allen. Once she found out, she started networking. She got me plugged into a host of different professors. When you're that sick, you lose a lot of skills. I had to relearn algebra, I had to relearn how to write papers. All these professors came together and they said, "You worked really hard when you were at this school to give back. Now you need help. We're going to help you." They really taught me and re-educated me and mentored me and got me back up to the collegiate level I was at where I was ready to attend a university. I piddled around for about a year, just taking psychology classes, majoring in psychology. While visiting patients of Dr. Test, I think we had a conversation. He was like, "What do you want to do?" I said, "Well, I like the idea of being a psychologist, but I really like working with patients." I said, "But here's the thing. I believe the biomedical model and the biopsychosocial model don't belong separate. I believe there's a way to bring those together." What that means is healthcare should be structured around the patient. You talk about palliative care and stuff like that, but it's going a little bit further. We decided, "If you go to Texas Tech and you major in psychology and you take your pre-reqs and you decide that you want to go to medical school, we'll try to get you into Duke University. That way you can be one of the first patients with PAH that also becomes a doctor specializing in PAH, and you'll have an advantage most physicians won't have, but as a patient that really understands it." That's where psychology really came into it, because I think it's very important that we don't get so caught up in healthcare and medicine that we lose sight of the humanizing aspect of these patients are people. They're just like you and me. It doesn't matter who's got what education or what financial level you're at. There's an interpersonal level there. I have been able to really connect with them in ways that a lot of doctors at the hospitals here have not been able to. They'll talk to them. The moment I go in the room, a few minutes into hearing that I have it and talking to me, I walk out of that room knowing their life story and their plans for the next 10 or 20 years going home. It gives them a lot of hope, a lot of stuff to look forward to. My hope is that having the disease I can provide a level of care that they normally wouldn't get anywhere else wherever I end up, and also to contribute to research for the disease as a patient, and that may be limited. Whatever I can do, I'm willing to do it and I've dedicated my life to doing it however long that takes. Originally, my idea was I visit a lot of patients for Dr. Test. I'm in a unique position, so I said, "Well, I'm going to create a support group." I wanted to find a therapist or psychologist that would be willing to lend some free time to come and be there when needed. In fact, I even wrote a paper when you have support groups and they become so large, if you don't have a mental health expert, you go from having a support group to a regression group. As a pulmonary hypertension patient, the simple explanation for that, I experienced so many near-death experiences that I became unfazed. When I would be close in a situation, near death and, “Oh, I need to change my pump. I'm just going to go take a nap.” Don't do that. I got in a situation where for a little bit I was on the fence about forming a support group, but I went ahead and I did it. I thought, there's a lot of young adults with pulmonary hypertension. I live in West Texas, and that happens to be the medical hub of West Texas. There was a lot of opportunity to do some good there. I just had to figure out is this the right thing to do? Eventually, I decided for the patients, bringing them together, having each other's support was great. I just always emphasize, I'm not a mental health expert, none of us here are. If you do have these issues, I have resources available. Or if you want to reach out to a therapist, I provide a list of information at those support groups. Prefacing it that way, I was more comfortable doing it as a psychology major. When you're in West Texas and you're in the medical hub, what that looks like, people from Amarillo, Midland, all across Texas, they come here to see Dr. Test, straight to his clinic. A lot of our members are scattered across West Texas. Texas is very big, so not all of us are in Lubbock like I am. The more I looked into it, I saw support groups in Dallas and Houston, San Antonio, Corpus, El Paso. So I started Lone Star Lungs, and it's just a pulmonary hypertension support group for West Texas. Dr. Test comes, and he'll talk to patients and visit with them. He'll educate the public on what I'm doing. I visit patients of his. Some of them come from California, some of them come from Florida, some of them come from New Mexico. I invite them to be part of it. If I have a meeting, not everybody can afford to just leave and come right back. A lot of times they can structure their appointments on the day of those support group meetings. That way, we get to be around each other throughout West Texas, because you can drive a long time in Texas and not see anything. It brings all of us together so we're not as isolated as a lot of people that try to form support groups are. I'm big on in-person support groups and bringing card games and board games to patients, their families and their kids, just to get people out of their heads, to encourage social interaction, to really encourage camaraderie with each other. I like to invite doctors and medical professionals and ADA professionals into that, because it humanizes them and gets them out of the clinic. They can really sit down and interact and eat and play games and socialize with their patients. It becomes a little more personal and less clinical at that point. There's a time and place for the clinical and medical and healthcare, but then when you humanize them, it's a lot easier for patients to become comfortable with their medical experts and their healthcare providers. I think it brings an aspect of palliative care to the physician, which usually, physicians are normally too busy to take that on. When you open that door and you bring it to them and structure it as something they can just come into when they have time, that gives back to the community in a way that's very rare. You're not going to really find that. A lot of doctors don't have time to do that. Dr. Test is very good about attending those meetings, talking to people, educating the public. With that support group, I visit everyone from teenagers that are diagnosed to elderly people that have been diagnosed. I try my best to bring them little books on pulmonary hypertension, what they could and couldn't eat. I give them my number, my email. I let them know I'm not a medical professional, so there's certain questions I won't answer, but I'm here. There's support here. I'll put them in touch with other members if they need that type of support. I tell them about PHA. A lot of patients are diagnosed after flatlining. Then, you wake up and get told, "Hey, you have an incurable disease. It's not only incurable, it's progressive. It's only going to get worse." Psychologically, that does a number on a lot of patients. A lot of patients give up. I'm very adamant don't sugarcoat things, but at the same time, don't go in there and crush their hopes and spirits. There is definitely hope with this disease. It's the best time to be diagnosed with it with all of our medical advancements. A lot of the pain we go through, if you're on remodulin, playing card games and board games, tabletop trivia and being around people, that's a great way to get out of your head and not really think about or realize you're in pain. I play a card game called Magic: the Gathering. I've played that game for years. It's one of the most popular games in the world. It's not for everybody, but for me it's great to get out of my head with, just like other board games, Terraforming Mars, Catan. It can be real simple stuff, UNO!, Skip-Bo, Yahtzee. You could argue that bringing that stuff to patients is a type of care or a form of therapy, in a way, that really helps people cope with pain, with depression, anxiety, doubts they may have, living with the disease in general. To this day, I encourage mental health experts also to come to our support group meetings. That way, I can offer patients as many resources as I can possible because of how big West Texas is. Bringing in mental health experts, diagnostic experts, your ADA experts, I've invited elected city officials to be a part of it to come and learn, because a lot of cities, they don't know, hey, we have these really rare medical professionals here. We need to support them and their clinics and uplift them, because this is very unique and not everyone has this. We need to be promoting this and bringing attention to it the same way they do with other diseases and conditions and disabilities. In fact, I'm in a couple of pushes with my support group. I'm pushing Lubbock, Texas to endorse the document that I call The Pulse Heartiness Initiative. What that is a document of policy, measures, systems, operations, not for higher education institutions to automatically adopt, but that they could to help patients with pulmonary hypertension and like diseases and conditions. That came into being when I asked my support group and then different support groups around the US, if you're a student, what accommodations would benefit you? What do you wish was an accommodation or considered one that isn't? Be it starting class early. I will argue as a patient, pulmonary hypertension necessitates being able to start class ahead of other students, because you don't know when you're going to get sick. You can think of higher education as a treadmill and it's going to keep going. If you have a couple of days where you're down and you're sick, or in my case a week or two, that semester keeps going. You're expected to learn everything and catch up while simultaneously learning new material. You can't do that without learning the old material, so the new material piles up, you've got to get it done. Suddenly, you can't study and you're failing quizzes and tests and before you know it, the semester's over. You're having to take an incomplete. You finish the course not really learning anything and go on to the next course not ready for it. There's a lot of accommodations I've put in there that I think would help people if institutions were to adopt it and find a way to create some of these unique accommodations that I've created. In that document, I talk about a shuttle service with golf carts, because a lot of institutions, they'll argue, "Well, we have buses." People don't think about the burden it is to be a student with pulmonary hypertension carrying medical equipment, your oxygen machine, batteries for the oxygen machine, on top of your books, on top of that, you get fatigued and heat-fatigued. Then you get brain fog, you get sick, you're in severe pain. They don't consider a lot of these things. I think shuttling students from classroom and across campus on a golf cart when they've got a complex and advanced cardiovascular pulmonary disease is a great thing that a lot of schools don't think about. I've asked different universities, including the one I go to, please let's pilot what it would look like to wholly and adequately accommodate a pulmonary hypertension patient. They've been very unresponsive with that, which is why I created that document and have pushed the city of Lubbock to look into endorsing it at some point just to encourage them to look at it and say, "This is something we should take seriously." If healthcare often needs to be structured around students, well then so does education, in my opinion. There's no reason that healthcare and academia cannot merge and come together. Especially, when you live in a city like I do, where you have Texas Tech University and Texas Tech Physicians, Texas Tech Health Science Center, deep medical ties where everyone's conjoined together. There's a lot of opportunity for disability innovation there. Even with my support group, I'll tell patients, "If you need me to pick up the phone and make a call and try to serve as a liaison, because you're not getting accommodations you need, I'll do it. They're... /episode/index/show/phawarepodcast/id/37711055

Episode 552 - Kevin Allen 12/23/2025

Russel Hirsch, MD - phaware® interview 551 12/17/2025

Russel Hirsch, MD - phaware® interview 551 Why Families Belong at the Table: PHriends of the PPHNet Dr. Russel Hirsch breaks down the core pillars of the , from shared research to consistent care standards, and explains why the next chapter in pediatric pulmonary hypertension care must be written with families, not just for them through the family-led committee. My name is Russel Hirsch. I'm a physician, pediatrician, pediatric cardiologist, and I work out of Cincinnati Children's Hospital. I've been in the United States now going on about 33 years, actually. I grew up in South Africa and trained at the University of Cape Town. Then, knew that I was probably going to be leaving and trained for a further year and a half in the United Kingdom before moving to actually St. Louis Children's Hospital where I did my pediatric training and my pediatric cardiology training and actually specialized at that time in interventions. My initial appointment was actually doing interventional cardiology. At St. Louis Children's Hospital, they had a very large lung transplant program. In fact, the biggest in the country at the time for pediatrics. They were doing a lot of pulmonary hypertension work, so I had a lot of background in pulmonary hypertension, cardiac catheterization, and also nascent interest in the therapies and the drugs for pulmonary hypertension. After I'd been there for about five or six years, I was recruited to Cincinnati Children's Hospital where I've been ever since. That's going on about 22 years now, where initially, I work mainly in the cardiac catheterization laboratory, but from about 2003, 2004 onwards, it became apparent that nobody was really looking after pulmonary hypertension patients and they were all coming to the cath lab. I was cathing them and once they were cathed, they were all going to the cardiac intensive care unit or the general cardiology floor. By default, they simply started falling in my clinic. That was how the pulmonary hypertension service started. It was around about 2005 that we actually formed it into a formal pulmonary hypertension service. Now, I've got a training program and it's grown into something really quite amazing and quite big. I've been incredibly fortunate to be very well supported by those around me, because I work with incredible people and my hospital has been very supportive. Our initial involvement in the came after I first met Steve Abman, actually. He came to give a lecture for a conference at Cincinnati Children's Hospital. It was a BPD chronic lung disease neonatology conference, and I met Steve there and he introduced us to the Pediatric Pulmonary Hypertension Network. He was the Director at the time. It was very interesting to me, but I didn't think we were ready and part of the readiness was that I didn't feel like we had our research and academic and scholarship credentials quite in line where they needed to be. So, we waited about two or three years before we put in for a formal membership. I think that was probably around about 2015, 2016, maybe a little bit later, 2017, 2018, but we became a member of the network. Now, the Pediatric Pulmonary Hypertension Network is a consortium of currently 14 pediatric hospitals around the United States and Canada. It is not necessarily the largest of the sites, but it's the sites that have established themselves as creating lots of work in the academic sphere, in the clinical care sphere, and also in the advocacy sphere, and what it's allowed us to do is to combine all of those attributes into a network that is now very, very strong and serves for teaching advocacy, consistency of care, industry relationships, which are now getting larger and larger. My involvement in that organization, initially, was to sit in the back of the conference room and listen, because it was quite intimidating. These were the biggest names in pulmonary hypertension, but a wonderful group of people who all had one thing in common and that was the care of the pediatric patient. How could they do better? How could we as individual centers combine our efforts to actually advocate and do better for pulmonary hypertension? It was remarkable, because the other thing which I recognized was that everybody at these meetings, at the PPHNet meetings, were voluntary. Nobody was being paid. They were all doing it on top of their academic and their clinical and their own institutional research activities. Nobody got any stipends. Nobody got any payment. They were doing it because of the love of children with pulmonary hypertension. It was remarkable to me because there was a need for some kind of financial background. Quite remarkably, at the second meeting that I was at, it just dawned me and I asked a question. I said, "Is there a finance committee for this organization?" Everybody looked at me and pointed at me and said, "Yes, there is now." That's how I became the treasurer of the PPHNet. As I progressed through the organization and started understanding the chart and the bylaws of the organization, one thing became completely apparent to me. As much as that there were periodically involvements of families and parents, there wasn't a concerted family involvement. This was just before the pandemic. I had started developing some ideas as to how we could change that. In actual fact, I had started registering a completely separate organization as a nonprofit that was aimed at raising funds for pediatric pulmonary hypertension research, which I was going to use as a vehicle to involve families to actually contribute their time, their efforts, their own advocacy for their children into bake sales and golf tournaments and all other kinds of ways that we could start raising money to support families and children with pulmonary hypertension. Long and short of it is everything came to a crashing halt during the pandemic. As much as it was registered as a nonprofit in Ohio and I'd put through the application to the federal government, the IRS, I decided actually to stop there. What I ended up doing was I actually took the framework of that organization and turned it into a subcommittee of the PPHNet, which was accepted by the organization, the executive committee. That's called the . The whole point of the PHriends Network is to further involve families and parents and aunts and uncles and grandparents and anybody, neighbors, into the organization so that they could have an equal voice in the organization. They could actually contribute, they could come with their ideas. In reality, there are lots of needs that we could help those families with. Not everybody lives in a big urban area where they have access to medical centers. Not everybody has oxygen concentrators or the insurance companies pay for oxygen concentrators that allow the kids to be more mobile and get around rather than taking big oxygen tanks with them. Not everybody has access to understanding what research is out there for children with pulmonary hypertension. It's all of those and some other ideas that formed the basis for the PHriends Network. So far, the PHriends committee has been in effect now for two years. It's been slow but steady, as we've grown. We started off just with an initial virtual meeting to get families together. We recognize that there is privacy and there's confidentiality that we want to be very, very sensitive of because some families don't want to know that they're out there, so we've taken that into account. I think that has been maybe a slight impediment to getting some families to become involved, but the Zoom meetings are open and they're open to the aunts and the uncles and the brothers and the cousins and everybody else. Having gone through surgery, pot shunts or lung transplant doesn't preclude families from contributing. I'm going to be very, very honest. We've got some families who've lost children to pulmonary hypertension who become members of the PHriends Network because they are incredible and they want to continue to contribute. Having said that, we've extended the actual organization to now starting to do educational seminars. Eric Austin, my colleague from Vanderbilt spoke about the genetics of pediatric pulmonary hypertension. Rachel Harper spoke about medicines in pediatric pulmonary hypertension. We have a number of series that are planned coming forward. The dates are yet to be determined. We plan to have the next session, which will actually be myself talking about cardiac catheterization as it relates to pediatric pulmonary hypertension all the way from the newborn, all the way to the older adolescent that's transitioning to adult care and needs that last cardiac catheterization at a pediatric institution before they move on to the adults. I want to talk about the anesthesia. I want to talk about the risks, but in actual fact, what I plan to do is to go through a cath report so that everyone can understand what is the need for reliable, valid information and how that impacts care. The actual hemodynamics. The measurement of the pressures. What the tracings look like, what the environment looks like, show you a picture of a cath lab. It's really important for everyone to see that. In that context as well, one of the things that I am, of course, proud of is we have started a program to try and work on decreasing the anesthesia exposure at the time of cardiac catheterization. We do know that particularly that first cardiac catheterization, there is a huge amount of anxiety both from the parents, but most importantly, for the child involved. We've invoked a program that is becoming more and more active to use our child life specialists to actually be not just in the room at the time, but to actually walk through the cath lab the day before with the child, the adolescent, to show them the environment, show them so they can ask questions so that it isn't a shock and surprise when they walk into that very clinical environment the next day with a lot of strange people. It gets them to say "hi" to everybody, "hi" to the nurses. We've done this now with a couple of patients and we've actually avoided any sedation or any anesthesia whatsoever. That doesn't work for every single child. These are ideas which I would like to be able to put out into the PHriends Network so that they can continue to advocate for their children, because not every program obviously has the means to do this, but I'd like to be able to do that in the future as we go forward. The hope is that the actual office holders for the PHriends committee are filled by parents. The ideal is for families to be front and center in the PHriends Network. I serve as the Chief Facilitator and leader of the committee at the moment, but I have no plans to do that forever. As soon as I can move into the background and serve as a facilitator, a communicator, that would be better, because not only will that mean greater advocacy and personal advocacy for the families, but it gives them a seat front and center in the PPHNet on the steering committee. There is a chair waiting on the steering committee for the chair of the PHriends of the committee who can come to the meetings, can understand what we're doing as a network and can actually change us from just being the hardcore science, registry, advocacy, teaching [organization], to put the softer edges because it's the softer edges that really count. At the end of the day, unfortunately, we're dealing with very sick children and we want to make sure that we are catering to every single need. Until such time as we have families deeply involved and embedded within the organization... and I'm not trying to imply that there aren't any families. There are some families involved in the organization, but just that greater advocacy, that greater visibility, ability to also just move the organization in slightly different directions other than what we see from big academic institutions, 14 big centers. Hopefully soon to have more network sites in that organization, because we will be expanding shortly. I'm Russel Hirsch, and I'm completely 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 /episode/index/show/phawarepodcast/id/37710965

Episode 551 - Russel Hirsch, MD 12/16/2025

Jennifer Keeley, DPN and Mary Whittenhall, MSN - phaware® interview 550 12/10/2025

Jennifer Keeley, DPN and Mary Whittenhall, MSN - phaware® interview 550 Jennifer Keeley and Mary Whittenhall, experienced nurse practitioners in the field of pulmonary hypertension, discuss the management of cough in patients receiving inhaled therapies for pulmonary hypertension and interstitial lung disease. #GossamerBioPartner #sponsored This Special Edition episode is sponsored by . Jennifer Keeley, DPN: My name's Jennifer Keeley. I'm a nurse practitioner and I practice in a large academic institution in Pittsburgh, Pennsylvania, Allegheny Health Network, specifically Allegheny General Hospital. I am a nurse practitioner there and have been in the clinic over 10 years, and in the PH space as a nurse practitioner for over 15 years, as a registered nurse for almost 20 years. So, I have a lot of experience and I'm really excited to be here today to talk about inhaled therapies and cough. Mary Whittenhall, MSN: My name is Mary Whittenhall. I'm also a nurse practitioner. I am currently an advanced practice provider at Pulmonary and Sleep Associates in East Providence, Rhode Island. I've been in pulmonary hypertension for about 11 years now. In that time, I have worked in a variety of settings, both inpatient and outpatient, managing patients with pulmonary vascular disease, and have also touched upon patients with interstitial lung disease and pulmonary hypertension. I get very excited when I hear about new opportunities for our PH patients. I think a lot about even when I started in pulmonary hypertension and the therapies that were available to our patients. Many of these therapies had been around for a little bit of time. But also something that I think is extremely exciting is that there's just been a rapid progression in development of therapies. And now, with the focus of looking at these therapies as potentially disease modifying, not necessarily slowing the progression of disease. With the advent of all of these new therapies, there become more options for our patients, as well. Often, patients can't tolerate some of the medications that we have due to side effects and despite lots of work to manage these side effects, the patients are not always successful. One of the great things being involved in an academic center is that we have the ability to help link patients to cutting edge research, particularly looking at a new drug that is an inhaled therapy that has shown significant promise in improving the lives of patients with pulmonary hypertension. As a part of the PH community, we all do quite a bit of networking with each other, as well as with our patients and other colleagues in the space. In that time, we did network regarding the study and have participated in some activities where we're looking at the data from the part of this trial and then also looking at some of the side effect management related to the medication, which seemingly is well tolerated. However, for some patients it may not come extremely easy. I think that's where the role of the nurse or the advanced practice provider really comes in this space is that we have a real strong dedication to helping educate patients about ways to manage these side effects. We want patients to be able to continue with therapies. We don't want them to say, "Well, this isn't working for me, it's time to move on." I think that we have a lot of strategies and a lot of experience with trying to help patients really figure out the best way to manage these things and to be confident that they can continue on with obviously the biggest benefit of improving their pulmonary vascular disease. Jennifer Keeley, DPN: We actually met at an advisory board last year. It was an advisory board consistent of registered nurses and nurse practitioners who, just like Mary and myself, have vast experience with patients and therapies, not just in the inhaled space, but more conventional pulmonary vasodilator medications that have been used in our patients for many, many years. As Mary had suggested before, when we start to think about newer agents, many, many of them are not the conventional pulmonary vasodilator medications, but disease modifying agents. Now, we've acquired an armamentarium of medications. So, inhaled delivery is just a really great option to avoid systemic side effects on top of each other. Our PH patients today, many of them are on more than three therapies, many of them are on four or even more therapies, so the delivery of the medication is just one aspect. When we talk about cough and side effects, I like to think about it and explain to my patient when we talk about side effects, particularly cough, to imagine a Venn diagram with cough being in the middle and what affects cough. You see this outward circle, how we deliver it, what kind of device we deliver it in. The drug, how small, large are the particle size? Is it easy enough to use for our patients? The formulation, is it dry powdered versus inhaled aerosolized? And then finally, just the patient themselves. What's their background? What type of PAH do they have? So, we can talk a little bit more about this, but just to get us started, this is how this developed and we had a lovely advisory board meeting with seralutinib and Gossamer Bio, and this was the outcome of it. We produced a lovely poster. This is a conversation if you will, that Mary and I are going to have based on what we talked about and the poster production, that came out of that wonderful advisory board. Mary Whittenhall, MSN: Inhaled therapies are unique in a way in that they actually have direct access to the lungs. So, when you think of an oral medication, an oral medication needs to be digested in the gut and sometimes that systemic digestion takes a while. Additionally, it's also often that we see patients that have more systemic side effects when we're using an oral formulation. Intravenous or subcutaneous formulations of these medications tend to cause pretty strong systemic side effects for patients, and there tends to be a lot of management that we need to do to help make these side effects more tolerable. For most of our patients, I say to them, "You're going to think I'm cruel because I don't really want these side effects to go away." In a way, we look at them almost as if you have a cup and your cup is full of water and after the top of the water hits the rim of the cup, then the water starts to spill over onto the sides of the cup. I think of other medications that we typically prescribe for patients in that way that when we get that spill over, so to speak, it's an indication that we've actually targeted all of those receptors that we want to help with vasodilation. Now that we're looking at other medications that don't really necessarily look at vasodilation, we're looking more at treating the blood vessels in a different way or affecting the process for which those blood vessels become diseased. I think that the side effects become different and I think they become less. In working with inhaled therapies, as you can imagine, the number one side effect that most patients will complain of is cough. Sometimes we have patients who have an underlying cough already, and that's usually not related to PAH, but in PH-ILD where we now have an FDA indication to use another inhaled therapy, we've seen in treating these patients that baseline cough is something that is extremely problematic for them before they even start therapies. So, trying to find ways to improve that baseline cough, treat any underlying symptoms, things like acid reflux as well, that may cause that, treating seasonal allergies, et cetera, and then, obviously, managing any additional overlapping side effects that may occur because of the new therapy that they're on. Jennifer Keeley, DPN: I think that's a really important part, is to talk with the patient, educate the patient on these inhaled therapies. First and foremost, that cough is almost an expected side effect. These are patients particularly with our interstitial lung disease patients that have PAH, cough is a part of their daily life. It's important to document and ascertain what these patients' baseline cough is. In many, many clinics, particularly pulmonary PAH clinics, and I'm sure much like Mary's, many of my colleagues have recommended using validated cough questionnaires so that we can get a really, really good baseline of what that patient's baseline cough is. Are you coughing at night? Do you have mucus? How long have you been coughing? Does it interfere with the quality of your life? Do you cough at night? Does it keep you up? Does it interrupt your sleep? Those kinds of things that help differentiate acute cough versus chronic cough. Many of these patients cough every day. They also have other inhaled therapies such as our ILD patients that are also on corticosteroids, many of them on inhaled corticosteroid therapy that can thin the oral pharynx, the posterior pharynx, and really affect the degree of nerve innervation in the posterior pharynx in the mouth. So, just really understanding what the patient's baseline cough is and educating them on the fact that cough is likely going to be a side effect with the use of this inhaled therapy. Certainly, as we continue to use the therapy, we would hope that the cough can be mitigated either through some lifestyle modifications, some natural remedies, and even some medical remedies such as bronchodilators. But really teaching the patient about the medication and inherently that this is likely going to induce a cough, but that we have mitigation strategies to help dissipate the cough. I always like to tell my patients also in the clinical trials, particularly the Phase 2 clinical trials that are out there that patients had a lot of cough. The patients on drug that were in most of the Phase 2 clinical trials for seralutinib and even for treprostinil inhaled, 30 to 40% of them experienced cough. But at the same token, the placebo-based patients that did not receive drug in these Phase 2 clinical trials also had a lot of cough. So, what that's telling you is yes, you're going to get probably some more cough, but it's likely not going to be that much or more far advanced than the cough that you're already experiencing. I also think it's important to tell these patients, many, many patients that experienced cough did not stop the medication. Actually, in these Phase 2 clinical trials, very few stop the medication. So, that gives you a really good big picture that we are pretty good at educating our patients how to mitigate cough, and if we aren't, then we should learn how to do so. Mary Whittenhall, MSN: I think it's important for us to set some expectations for patients when we're talking about cough. We've already discussed a bit that cough can happen for people from other things outside of their lung disease, but it's important to also look at what may be causing the cough when we are giving a patient an inhaled therapy. So, any type of inhaled therapy, whether that be a dry powder, a mist, whether that's nebulized or through in actuated inhaler, there are particles inside of that medication as it's going in and those little particles, when your lungs inhale that medication, those particles are penetrating your lungs and your lungs are not accustomed to them being there. It's almost as if your lungs are saying, "I don't recognize this. I don't know why this is here," and it may feel like it's an irritant, so you may start coughing as a result of that, but the cough is not necessarily a bad thing. Those particles are there, and the job is to essentially help deliver the medicine to penetrate that lung tissue and then for your body then to absorb the medicine. Your airways and your blood vessels inside of your lungs are extremely close to each other. So, when you inhale that medication, those little blood vessels are also right next to where those airways are, and then that is how those blood vessels then absorb that medication, because they're so close to the site at which those particles come into your lungs. Jennifer Keeley, DPN: I think this is an important concept to understand. They choose the form of delivery based on the goal of delivering the most medication efficiently to the distal bronchioles. That's where the disease is. It's in the distal arteries. So, trying to formulate how we get these very powerful, oftentimes disease modifying agents into the periphery of the lungs can be very challenging. Dry powdered inhaler is one form that the variability of delivery is not as dispersed as an aerosolized. So, it's more efficient delivery to the place where the medication needs to work the best, and that's in the distal periphery of the lungs. Unfortunately, one thing you have to deal with is that oftentimes these medications, dry powdered medications, not just in the PH space, but there's a lot of other dry powdered inhalers in the COPD space, as well. Oftentimes, what happens is these powdered particles get dispersed extra thoracically. So, they get dispersed in the oral mucosa, in the posterior pharynx, on the way down into the stomach. That's wherein we have to deal with mitigating side effects. The biggest side effect of these particles, even though they're very small, is cough. So, technique comes into play. Mitigating things to coat the posterior pharynx come into play. Re-education comes into play. Show me again how you're doing this inhalation, because I don't think that you're holding this okay. In one instance, I had a patient that was inhaling dry powdered inhaler with the medication right out of the refrigerator. So, the medication was cold. It was clumping at the back of her throat. All of these things really take into consideration how we most efficiently get the medicine to these pulmonary arterial hypertension patients where their disease is oftentimes very difficult to get to, and other forms of medications that are systemic, orals, parenterals that have first pass metabolism, and so you're going to get more side effects from those medications. So, I always teach my patients, "Hey, we're a couple steps ahead because we're bypassing the type of metabolism that you get with orals and even parenterals." Mary Whittenhall, MSN: There are so many challenges that these patients face. Oftentimes, patients have never been sick before they develop this, and now we're putting them on multiple therapies, multiple modalities, telling them that there's going to be side effects and they need to learn how to manage them. It's certainly a lot to handle. But I think one of the best things that we have in our PH community is that we really work so hard to partner with the patients and their loved ones and forming this relationship, fostering that relationship as time goes on, I believe that these patients really do trust us and that what we're telling them is things are going to be okay. We are going to be there by your side. We're not going to give you this medicine and then say, "See you in six months. Hope everything goes well." We're really going to be working with them. In some cases in my specialty clinic, we have nurses, we have a pharmacist, a pharmacy tech, and then our advanced practice providers that check in with these patients quite regularly. We are actually taking the initiative to reach out to them versus the patient who may be having trouble advocating for themselves or feeling like, "Really, I don't want to be a pain, but this is challenging for me." We are really in touch with them, and that connection also helps to keep patients on therapy. So, what are some of the specific techniques to manage or mitigate cough? This is something that was a real hot topic at our last advisory meeting. We put together a bunch of folks in the room who deal with other inhaled therapies and patients that have cough and said, "Well, what do you tell patients to do?" First and foremost is to look at any other potentially underlying conditions that may be causing cough and ensure that treatment of those underlying conditions is optimized. I think cough is actually the number one referral for any type of pulmonary practice, but it is a really, really broad differential when it comes down to it. We obviously look first at things like environmental factors. If this could be seasonal allergies, then we try treating patients with antihistamines. Perhaps some of those are intranasal, as well, that may help with some things like rhinorrhea or post nasal drip. Acid reflux is actually a huge, huge reason for cough. Many patients say, 'Well, I don't get acid reflux. I don't feel that burning in my chest after I eat," but come to find out that it can actually be a silent trigger. So, treating patients with medicines that help to reduce acid or suppress acid will oftentimes help with that cough. On top of that, when we're dealing with patients that are on inhalers and now we're adding another inhaled therapy. I find that for some patients that are on actual inhalers that sometimes they do better with nebulized treatments. The nebulized treatments are slower, and may have a bit of a better penetration into the lungs and the patients tend to like it. It is one of those things that you do need to be compliant with in order to really see the benefits to it. I will say that oftentimes, again, partnering with the patient, giving them specific instructions about how to do all of this, we can really see some improvement to those symptoms. Then, there's just basic over-the counter measures and precautions, things like making sure that when you're eating that you're not laying down at least for 60 minutes after you've been eating. If you do have acid reflux, trying to sleep with two pillows or a wedge pillow, that can help to keep the head of your bed elevated. Some of our patients have those really fancy adjustable beds that are also quite helpful for that. I think that sometimes things like basic cough drops actually can be quite wonderful and helpful. Drinking very cold or very warm water or tea, adding some honey to that if a patient isn't diabetic, things like that tend to really help with cough. We reinforce these measures when we start therapies like this. Jennifer Keeley, DPN: In terms of mitigation, I think it's really important on technique. This is why, as Mary had alluded to, it's so important to follow up closely with these patients, particularly our elderly patients who sometimes don't, if they have connective tissue disease or scleroderma, have a lot of good fine motor coordination. A couple of things that I wanted to touch on with regards to that… One, these inhalers are typically high resistance, low flow. So, these are not the type of patients that need to be taking in very forceful inhalations with these inhalers and thank goodness, because we're talking about patients that have inflammatory interstitial lung disease, as well as pulmonary vascular disease. So their degree of inspiratory effort is actually minimal to disperse that medication to the distal pulmonary bronchials. It's equivalent to them taking a deep breath in when you ask them to auscultate their lungs. So it's not a big forceful breath. The other thing is too, a lot of times, sometimes more variability in the disbursement of the drug is better in compliance with some patients. Dry-powdered inhalers, again, do not take a very big forceful effort, but some of them, because they are powder, some of the medication will actually hit the back of the throat as it goes down and can cause some irritation, whereas the nebulized form does have a variability in disbursement and can be more easily tolerated in some. The other issue is the technique itself. Oftentimes, we ask them in some of the inhaled therapies to lower the device itself so that the tongue doesn't protrude and get in the way, because if medication gets on the tongue, the next swallow that they take, that medication is going to hit their posterior pharynx, and... /episode/index/show/phawarepodcast/id/36415525

Episode 550 - Jennifer Keeley, DPN and Mary Whittenhall, MSN 12/09/2025

Ashlee Gambino - phaware® interview 549 12/03/2025

Ashlee Gambino - phaware® interview 549 My name is Ashlee "The Mobwife" Gambino. I'm a retired professional fighter. I've acted. I've done a lot of different things. I am now diagnosed with idiopathic pulmonary arterial hypertension. I'm in the severe category, so it's pretty far along. I can no longer fight, unfortunately. I have a central line pumping life-sustaining medication directly into my heart right now. I performed on Fremont Street for two years when we first moved to Las Vegas. As a fighter, I would hand people a pair of boxing gloves, provided they weren't belligerently drunk, and they agreed to the rules, which where we weren't supposed to actually hit each other. We would shadow box each other. Of course, that turned real more often than not. That was a lot of fun. From there, I debuted in MMA for here in Las Vegas. Then, I became pregnant with my second daughter. Seven months after I had her, I debuted in professional boxing for a short notice fight to help them save a card. I have two kids. I have Carmella, she's my oldest. She's nine years old. My youngest is Carmine, he's three. We lost Katana, she would be five right now. I was going to have a baby when I was diagnosed with this. I was very pregnant and unfortunately I lost her due to the complications of this disease. I lost my daughter Katana to the incredibly rare disease called acute leukemia. It can be very fast acting. In her case, it was very fast acting and it took her very suddenly from us. It happened almost exactly a month right before my diagnosis. She passed away right after Thanksgiving and I was diagnosed a month later. I was having these issues where I was trying to be an Uber driver. The shortness of breath is the biggest thing. I was also pregnant at the time. I would drive for one day and then I would sleep for literally two or three to make up for my energy loss. We had no idea what it was. Nobody here knew what it was. The very first sign of the pulmonary hypertension was after I had my son Carmine. They found that my oxygen was dropping at an abnormal rate. So doctors had extensively tested my lungs and my heart to see what was going on. At the time, the only answer I had gotten is that my heart and lungs looked perfect and athletic, and that there was nothing wrong. Obviously, there was though, because my oxygen was dropping. Now, in retrospect, that I know what it is I feel like there was signs before that also. Before Carmine, I think it was happening too. That was around the time that I had debuted professional boxing. I believe that I was showing signs of that too, because I accepted the fight on short notice and I was out jogging and I just couldn't even make it to the end of the street. That wasn't normal for me. I would usually be able to take a jog no problem. It was really difficult to complete even just a jog, so I believe that that was trying to show me a sign that I had the pulmonary hypertension, initially. We flew to Ohio from Las Vegas for our daughter's funeral. That's where most of our family is at. During that time period, I was having a very hard time making it up a flight of stairs. I would have to take three breaks, even just to get up one flight of stairs. My oxygen would drop all the way into the eighties, moving from one room to the other. I had the oxygen test due to recognizing that my oxygen was dropping after I had Carmine. I went to the hospitals seeking help knowing that I at least needed oxygen if nothing else. I didn't know what else to ask for at the time. They had sent me home, told me there was nothing they could do for me. I didn't even get oxygen. That took three tries of going to different hospitals before I finally got transferred to a bigger hospital, and then even that hospital almost sent me home, too. But luckily, there was one doctor that had saw my echo and recognized that my heart was enlarged. If you know something's wrong with your body, then that means something's wrong with your body. Even if a professional disagrees with you and you know something's wrong you have to keep advocating for yourself, because if you don't, it's impossible to get the proper help. The immediate plan of action was a whole team of doctors came in looking at me sad, because they had to tell me that I had to lose my daughter in order to survive. I didn't know it was a little girl yet I had to find out it was a little girl right before I lost her. That was probably the hardest decision I've ever made in my life. When you have pulmonary hypertension, getting pregnant is basically a death sentence. I feel very lucky to have survived Carmine's birth, possibly Katana's through pulmonary hypertension. I would not have survived with our new baby, baby Rain, we named her. If I would've even waited another week to try and get her to be able to survive, they weren't confident at all that I would survive. They couldn't even believe I was alive by the time I got into hospital. They wouldn't even put me into labor. So, I didn't even get a chance to hold her, because I wanted to just be put into labor. I didn't want to go any other route, but I had no choice unfortunately. I had to be stoic as I possibly could in order to survive that situation for my husband and my two children that are still here with me. So here I am. When it first happened, they tried to just start me out on pills, and that wasn't helping fast enough. They felt like if I didn't switch to the IV therapy that I would not make it. We chose to go ahead and let them put the central line in my chest and very quickly I lost even the ability to go swimming, one of my favorite things to do. I wasn't able to train anymore. I mean, I couldn't even do the dishes when this first happened. I would try to walk three rooms down to a room that was private for me and my lips turned blue. It was a very serious matter. All of a sudden, I had to learn how to do surgical things and mix my own medicine that would be needing to be mixed every 48 hours to keep me alive and learn how to work those pumps and change my dressing. It was such a shock to me. I knew that I was strong. I'm no quitter. That's why I planned on becoming a World Champion in martial arts. I really had to muster up some strength through this situation. I found myself really overwhelmed. The first day that my nurses came into the hospital room and they started my lesson on teaching me how to mix my life sustaining medication, I had just gotten out of my first real shower after being in the ICU and everything. It was just very hard to deal with, but I did. I also had a completely torn apart knee. I don't really talk about that very much. I was pregnant. I couldn't fix it. My plan was to wait until after I had her to fix it. I had my knee brace in the hospital while I was moving around during all this too, so that was also a struggle for me. I was in the hospital for almost a month. There was a lot of talk going on outside my room that they thought that I didn't know about, but they didn't think that I was going to make it. I really shouldn't have made it with how severe my situation was. When they found it my heart was twice the size as it should have been the right side of my heart that is, my pressures were in the hundreds. It was very, very severe. I mean, even now you can tell that my pressure is very high. I'm even getting out of breath trying to talk. It's a very crazy situation to know that I have to deal with this, but if I don't, then I just lose my identity. My identity is to be strong, to be stoic, and so I had to push through it. Then in the end, my team of doctors walked in and explained to me that I had inspired them in many different ways, including medically. I realized at that point that I needed to keep doing that, that's why I started fighting in the first place so that I could reach a position of influence in order to inspire people to be and do better, and that's my mission. I'm going to keep following it. Even if I have to take a different route at this point, which I do. It's definitely a continual battle. I have used my skills from fighting to help save me during one of my right heart catheterizations, which is by the way, the only way to read the actual pressure in your heart. My heart wasn't happy. I was conscious. I only requested the local anesthetic. I refused any type of sedation, because I don't feel like my heart can handle it. I had to use my fighter skills to calm my heart down, and I did. I used the same technique as I would in between rounds of a fight, for example. But this whole situation, losing my daughters, being put on life-sustaining medication and having to deal with this on a day-to-day basis is definitely taking my toughness to a whole new level. I have to evolve daily with it. It's something that has not been easy to get through. I know that I'm a tough individual, but I have been fighting so very hard to get through this and to get to this point to even do these podcasts, because it's just a really humbling experience. I went from being a pro athlete to not even being able to walk across the street to the gas station without needing help in a wheelchair. Now, I'm getting back to the point where I can walk a little bit more, but it's also progressing again, so now I have to go up on my medicine, because this is a progressive disease. It doesn't stop, just keeps going. They don't technically have a cure for it. The medicine that I am on is meant to ease your symptoms as much as possible until the inevitable, whatever. I don't want to believe that that's going to happen to me. I'm probably the most stubborn individual you'll ever meet. So, I plan on being around until they find some kind of a cure and then still sticking around. I don't believe that my body is strong enough to get through the double lung transplant that is an option for people in my situation. I don't believe that I could even sustain being under any type of sedation, let alone them doing something as severe as the double lung transplant. So, it's not really in the talks for me at the moment. I'm kind of just stuck between a rock and a hard place. I have 15 pills I have to take every day, which is also new to me because I wouldn't even take a headache pill while I was breastfeeding or pregnant, so I hate pills. I have to take these. You can hear my pump going off during this podcast. I have to have this pump pumping directly into my heart every day, all day. If it goes off even in the middle of the podcast, I have to stop and fix it, because it's life sustaining. Everything comes with their own challenges that you have to face and be strong in order to survive with. Yes, the inevitable of it making me pass away eventually. My husband and my kids are very supportive. Even my toddler, he understands what I have to deal with and is very cute and very sweet, how well he handles it, and my daughter too. Even with support groups and meeting other patients and this and that, no matter what, ultimately you have to reach inside to find that kind of strength to get through something like this. You have to reach inside and pull that strength out from your heart, because if you don't, you'll just fall down this terrible slippery slope and you don't want to do that. You don't want to do that. Even in a situation like we're in with life sustaining medication, you have to find purpose somewhere. You're still here for a reason, so you have to keep fighting and keep yourself here and find that reason and exploit it. I've had a goal for years to make appearances and do speeches. I'd like to go to schools and do it. I'd like to do it in any public setting, really. I just want to share my story and awareness of what happened to me, maybe what could be happening to one of your family members and a way that you could help save them, because if it gets ignored, then it'd ultimately end up killing somebody. It could have easily killed me and my daughter if I didn't keep trying asking for help. She would've suffered a lot more than she had to. It would've been just a terrible situation for my family. So you have to advocate for yourself. You have to reach out. So that's my goal. I want to do motivational speeches. I want to go out and do speeches to the best of my ability, even though they'll probably knock me on my butt for the next two days after that speech. I want to keep doing this. I want to keep sharing my story. The most powerful thing that I have powering me and any type of courage that I have right now is my love for my family. That is the ultimate power that I have is my love and their love for me. Besides that, I also have a desire to reach out and share that with other people. I feel like we've lost our message of treat others as you wish to be treated in today's world, in society. Things are just slipping in such a bad direction. I want to reach out and help change that for us to be in a better way. If we would just become more positive as humans, we could easily evolve so much faster. Right now, we're just stuck in this lull space of not knowing what we're doing, because everybody is so negative and we need to get away from that. So, I want to try and help spread that message, and that is also powering me to be courageous in this and to keep pushing forward. My name is Ashlee Gambino, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . /episode/index/show/phawarepodcast/id/37709365

Episode 549 - Ashlee Gambino 12/01/2025

Sophia Esteves - phaware® interview 548 11/26/2025

Sophia Esteves - phaware® interview 548 Scars and Survival: Rebuilding Life After a Devastating Diagnosis After countless hospital stays, new medications, and near-breaking points, Sophia Esteves found the reason she wanted to live—and it wasn’t just for herself. This episode dives into her spiritual and emotional transformation from patient to powerhouse advocate, and how she now helps others reclaim hope through honesty and grit. My name is Sophia Esteves. I am living in San Antonio where I grew up. My connection to pulmonary arterial hypertension in the community is I'm a support group leader and I'm also a patient advocate. I was experiencing syncope. I had a couple episodes where I passed out, and the doctors were telling me, "I don't know what to tell you. I could keep you here all night, or I could let you go." The next week I passed out again, and I said, "Okay, we have to figure something else out here, because I can't get any more of these bruises on my head." I said, "Okay, some doctor has to notice something." I went to every doctor you could imagine and then they finally said, "I'm going to send you to a doctor in Houston, because I think you might have this rare disease, and if you do, you might not have that long to live." It was that whole story that they tell you when you're on the cusp of being diagnosed. So of course, I went up to Houston, and I had a right heart catheterization. They said, "Yeah, you have pulmonary arterial hypertension. You have about four years to live, and you need to find someone to raise your daughter, and this is not a joke, and we need you to stop working because your job now is trying to stay alive." That was 22 years ago. I was super ambitious. Well, I'd say I still am, but sometimes the fatigue gets the best of me. But at that time, I was working two jobs and going to school and trying to raise my daughter. I mean, I was like a functioning zombie. I was just determined to make things work for her and I. She was five at the time. AI justified all of my symptoms that I had, like, "Well, obviously I'm sleepy because I'm always at work, and I'm tired." Everything I experienced, the swelling of feet, maybe I need to elevate my feet more. My red hands and my red feet, maybe getting too much sun. I mean, I came up with the reason for everything, because I didn't want to slow down, really. I was 21. Well, back then there were no pump options presented to me. The only thing I had was a calcium channel blocker and a blood thinner and high hopes. That was about it. At that time, fortunately, a study drug was presented to me. With much research, I determined that that was going to be the best option for me. That was the best route. There was a clinical trial. Actually, there were two side-by-side that were available. I looked for the one that, I guess, fit for me a little bit more in my life, because it was going to be held at a PH clinic which is three hours from my house. Sometimes when you're doing studies, they require you to be there quite often in the beginning. I just made sure it was something I could commit to. I didn't really have that many other concerns except for maybe some side effects and things like that. It was the study that I ended up joining that became one of the best decisions that I made. It was like that whole fight or flight or sink or swim type of mentality for me, because you're telling me all of these heavy things about the direction of my life now, and the rest is going to be good decisions and high hopes, like I was saying earlier. I said, "Okay, I need to be really serious about this even though I'm young. If I want to be here long enough to see my daughter graduate, then I really need to make sure that this is my top priority." The big old packet that they give you when you're about to join a clinical study, I went through every single page like if it was a research paper. I highlighted and I had questions. I've always been super thorough when it comes to my own personal health. I want answers. As long as you can give me those answers and let me know that if at any time I'm not happy with this study or maybe I'm not feeling so well and I want to come off of it, I want to know that am still in control and I still have the option to do that. And of course, the doctors were very good about saying, "Hey, look, this is something that you're trying. Even though you're committing to it, it's something that you're trying. And if it doesn't work at any time, you can come off of it." That was what made me decide to go forward with being on a trial, being on a study. It was a commitment that I learned a lot from. I think I made three trips to Houston every week. So it was like Mondays, we would wake up at 5:00 AM and drive three hours, get to the clinic, take my pill, and then sit for a six-hour period in which I was watching VHS tapes, I think, if that takes you way back, these classic movies in the little office in my doctor's office. I was just tucked away. Then, I would go eat lunch and come back and take the next batch of my medicine. Then, that was done for the day. I kept telling myself, "Sophia, this is the least that you could do for yourself, right? You want to live. You're just coming over here and watching movies. You've got this." With clinical trials, it just depends, are they double-blinded where the staff and the patient don't know what you're taking? You're just optimistic. That's kind of the study that I was on. Then, as you progress through it, you have less visits and you learn how to be a thorough patient for the success of the trial and, of course, your own personal journey. Coming off of that, it was just like, "Hey, I feel pretty good. If I'm supposed to be feeling something different, I don't." Then, I was like, "What else do you have for me? I'll take it. I'll try it." I was just so ready to use my body as this vessel to try anything that I could, because, of course, I had a great experience. From that point on, then other medicines became available, IV therapy. I said, ""Okay, if this is what you think is best for me, let's do it." Then, that just became my whole objective with trying to treat myself and be an advocate for myself was, whatever you have, I'm going to try it. I learned that each individual on this planet is completely different. We all have different contributing factors that affect us overall, which means that any type of medicine you put in any person is going to probably have a slightly different effect. So I can't take it if it's a movie review where you said, "That movie sucks. Don't go watch it." I'm like, "No, I want to try it for myself. I might like it." So that's what I did. I tried oral therapy. I tried inhaled therapy. I tried intravenous therapy and subcutaneous therapy. Now, the injection therapy. I'm trying everything across the board to make sure that I'm being fair with myself. It was something that was a big learning process. Every time you take a new medicine and you put it in your body, your body is going to have some sort of reaction, otherwise the medicine wouldn't be working. I had to learn what those were. So yes, my face might be red, and I might feel flushed, but guess what? That means the medicine's doing what it's supposed to do. It was kind of one of those things where I learned to treat the side effects from the medication as just a worst case scenario. That way, once I learned how to deal with those side effects, I could try to get back on track to have a more normal life. I knew that I loved working, going to school, taking care of my daughter, all of those things, and I wanted to continue them so bad. I was like, "I'm going to do everything I can to get this under control enough so that I can have some sense of normalcy, whatever that looks like to me in that chapter." Of course, as any PH patient may experience that you go through a lot of hospital stays. Of the two decades, I feel like I've spent maybe half of that time in a hospital. I mean, I have scars all over my body that tell the most beautiful story of how I'm still here. I had to find a way in all that just to make it okay to continue doing those things, using my body in those ways, like, okay, take this medicine or heal from this scar and all that so that you can continue to have the other side of it, which is the time that you're not in the hospital because that's what makes it all worth it. It was just this really beautiful balance that I was learning while I was taking all of these medicines. Another part of it was being realistic with myself. If my goal is to take this medicine and have a better quality of life, do I feel that? Do I feel like it's better, I'm better or worse? Without saying too much, I'll say that one of the treatments for me that I was on was just an absolute no-no. I did not enjoy that treatment, out of everyone that I've ever been on. It was the most painful thing for me, and I had to make a decision that I didn't want to be on that anymore. Fortunately, now we have this rainbow of different options that we can pick from. I went back to something else that I was more familiar with, and then it created the stability that I needed to get back to that more balanced life. I believe personally, we all need purpose. Sometimes when you're faced with a diagnosis, like whatever you want to call it, chronic illness or a long-term disease or life-threatening illness, it kind of feels like everything you ever worked for, which is also your identity, your career, your education, your family life, all your social groups bundled into one is who you are. But then they go, "Hey, you can't do that anymore. You can't work. You can't be over there. You have to be over here." It's almost like you're searching again for a new identity. You are focusing on what you can do and what you can't. Once you do that, then you start identifying more of who you are in that phase, in that chapter. As I was going through it, I will tell you, these very heavy moments... Because I'm an empath, so I feel everything so deep. I remember coming home one time from the hospital, and I had my arm outside of the passenger window. My dad was driving me back, and it was covered in bruises. I remember just crying, filtering it out. I said, "I don't know that I'm okay with this. Am I okay with this? Do I want to keep going through this?" Those were all the really truthful raw questions that would go through my mind every time I would look at myself. I would look at myself in the mirror as if I was someone else. I would say, "I just want to hold you. I just want to take away some of what you're feeling." It was all of those things I had to dissect and understand why, because of course I want to be here. But why? Then once I decided why, it's, "Okay, what are you going to do about it?" When I finally decided, "Hey, yes, this is what I want to be doing. Yes, it's going to look like this sometime, but why? Why are you going to do this, and who are you doing it for?" I learned that every time I went into the hospital and I got a new scar, it wasn't for me, it was for someone else, because that story that I told someone else about that experience that I had shifted their life in some way. Maybe they needed to go on IV therapy, and they didn't want to live anymore, because they didn't want to go on IV therapy. Then, here I come into their hospital room and I'm telling them my last experience. "Look, this is why I'm here in the hospital. You can do this. It can look normal. It can get better." Then, all of a sudden this person calls me a week later and says, "Thank you for convincing me to get on this medicine. I'm alive and I'm with my family now." Things like that. So I understood that's my purpose. That's why I'm here. That's what makes it okay. It's like waiting in a really long line. Yes, it sucks. Without the pretty words, it sure does suck sometimes, but not all the time. Then, I'm like, "I didn't wait in this line all this damn time just to get out of it. I am going to keep going forward, and when I get there, I am going to live it up and I'm going to do as much as I can, whatever that looks like." One thing I did notice going through this is that a question would pop up a lot in my mind. "Am I depressed or am I tired?" I had to understand that my body just went through something enormous. Even though we normalize it because we have to, because it's a sense of survival, it doesn't mean that it's any less of a huge experience for our body. Our poor little bodies are delicate little flowers. We put them through all this stuff. We have to learn in that process to give ourselves that grace and to say, "Hey, it's okay to rest. It's okay if you sit at home all day and watch Netflix.” It doesn't mean you're depressed, because I guarantee, if you felt well, the minute you are feeling better, you get up off of the sofa and you run out into the sunshine, and you do all the things your little heart wants to do. That clarification, every time I would get up from being tired and resting told me, "Hey, that wasn't depression, that was what resting looked like." Sometimes they can look similar. I just want to throw that out there, because I'm sure a lot of us go through this little questioning moment where we are wondering that. But again, it's just part of our recovery process. We hit the reset button and then we keep going. I'm Sophia Esteves, 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: /episode/index/show/phawarepodcast/id/37711655

Episode 548 - Sophia Esteves 11/25/2025

Richard Channick, MD - phaware® interview 547 11/18/2025

Richard Channick, MD - phaware® interview 547 The Silent Rise of Chronic Pulmonary Embolism and the Tech That’s Changing Everything From portable ECMO to catheter breakthroughs, the treatment of pulmonary embolism has come a long way. Dr. Richard Channick takes us behind the scenes of cutting-edge interventions and shares the empowering truth: even community hospitals now have lifelines to expert centers. The care you need might be closer than you think. Hello, I'm Rich Channick. I'm Professor of Medicine at UCLA Medical Center and Co-Director of the Pulmonary Vascular Disease Program at UCLA. I've been there for the last six and a half years, but have been involved with pulmonary hypertension for over 30 years from my fellowship in the '80s onward. I've gotten to see all the evolution and changes that have occurred in this exciting field. Today, I'd like to talk to you about a session at a recent CHEST meeting in Boston, where we discussed, among other things, chronic thromboembolic disease and the best treatment for those patients. These are people who've had a blood clot or pulmonary embolism as we call it, but for some reason those blood clots have not gone away and they become chronic or more like scar tissue inside the arteries of the lungs. Depending on how much clot there is and how the patient is doing, there may be intervention needed. The good news is that they're very effective treatments for patients with chronic thromboembolic disease. Those treatments have been well known. There's a surgery called a pulmonary thromboendarterectomy or PTE surgery that can be curative. There's catheter procedures. There's even medication. What about patients who have some blood clots but they don't have pulmonary hypertension? So they have these chronic blood clots, but it's not enough to cause elevated resting pulmonary pressures. That's an interesting area, because those patients may have symptoms and they may be limited by that chronic blood clot, but it's not to a severity where they have right heart enlargement or really high pressures. But still, there may be some patients that treatment is warranted. That's kind of what we discussed. What would be the reason to do a surgery for instance on somebody who had chronic thromboemboli with no pulmonary hypertension? There's some ways to look at that using some more sophisticated testing like cardiopulmonary exercise testing, where a patient gets on a bicycle and you see how far the person can pedal and measure a bunch of things when you collect the gas, if they're wearing a mask and whatnot. Those are ways to evaluate patients to determine if for instance surgery would be an option for somebody who has chronic thromboembolic disease without pulmonary hypertension. It's also important because these are more common than we think. CTEPH as we call it, chronic thromboembolic pulmonary hypertension, is relatively rare, but CTED, or chronic thromboembolic disease, is probably quite a bit more common. It also points out something very important that I've always pushed is that after a patient has an acute pulmonary embolism and close to a million people by the way have pulmonary emboli in a year in the US, there needs to be a very structured follow-up six, eight weeks after that acute pulmonary embolism to really evaluate has the patient returned to baseline, are they fully recovered, or do they have any persistent symptoms? Those are patients you're going to want to follow a lot more closely. A lot of what I do is to really develop a system for systematic follow-up after an acute pulmonary embolism. The system from when I first started doing this, which was much more haphazard for following patients after acute pulmonary embolism, has really been revolutionized. I was fortunate enough, when I was in Boston in about 2011 at Mass General Hospital to develop something called PERT or pulmonary embolism response team. This was something that we came up with to try to do just that, organize how patients are not just managed acutely, but followed up after the acute pulmonary embolism; to have a multidisciplinary team and an organized process for treating these patients optimally. PERT concept is something that's really taken off around the world, actually, where these teams are in hundreds and hundreds of hospitals. We have a consortium of PERT teams that we meet every year. It's really kind of been a revolutionary change since how I remember it in the '80s to where we are now, which is very satisfying. One of the changes or evolutions we've seen in pulmonary embolism is really the availability and application of advanced technologies. I mentioned surgery, the catheter procedures, even medication. I think that this is an example where the technology has really made a big difference. For instance, ECMO or extracorporeal membrane oxygenator, it's almost like a heart-lung machine, which has been around for many decades, but with improvements in technology it's been made more portable, lower complication rate. Now, we're using it acutely to treat life-threatening pulmonary emboli, not uncommonly. That was another thing we talked about is what are the indications? When would we put a patient on an ECMO machine in the setting of pulmonary embolism and how we would do that? It's not available at every hospital. So, like a lot of high-level technologies, small community hospitals may not have access to it, whereas big hospitals do. Actually, that's another advantage or value of the whole PERT concept is that a hospital that may be smaller, a community hospital, may in many cases have a relationship with a large PERT center. We've set up this sort of hub-and-spoke model and actually now have just initiated an accreditation program for the pulmonary embolism response team consortium. Similarly, what we did with PHA many years ago, (The Pulmonary Hypertension Association), where we can look at different centers and seeing what they can offer patients for pulmonary emboli and do they have a relationship with surrounding hospital? Things like transfer of care for patients. So, if a patient has a big pulmonary embolism and is close to a small hospital and it's a life-threatening situation, there's a mechanism for transferring those patients to larger places. The centers of expertise in pulmonary embolism in terms of the ability to perform open-heart surgery to remove these blood clots has really expanded over the years. We do have to distinguish the acute blood clots from chronic blood clots. CTEPH for chronic blood clots is a little more difficult, because these clots are kind of grown into the wall of the vessel. You really need a surgeon with a lot of experience and that's why, for instance, UC San Diego where the surgery is pioneered, still remains still the very largest program by far, but there definitely are now programs around the country that have developed that expertise. I think that that's really a good thing because back in the day, and I was in San Diego for over 20 years, patients aren't always in a position to fly across the country to have a procedure. They're too sick and they don't have the means, whatever. It's really been critical that these regional centers developed. We built one in Boston when I was there, which served a huge need in people in the New England area, for instance, to have a center. Now, it's all around the country I would say. 10, 12, maybe, at least, regional centers. Pulmonary emboli can present in a number of ways. Certainly shortness of breath, chest pain, lightheadedness. You may think about a pulmonary embolism. There's certain risk factors. Blood clots form usually in the legs and then they break off and go to the lungs. Things that may make it a risk to develop a blood clot in the leg like prolonged immobility. So, you're lying in bed because you're sick or you just had a recent surgery. There are certain things like orthopedic procedure, specifically, where you have a very high risk of getting a blood clot in the leg. Most of the time, orthopedic surgeons, they'll give you what we call prophylactic medications, either shots or pills, to prevent blood clots from developing. Certainly, you want to make sure that your doctor has addressed that and hasn't overlooked it for instance. You need to really be sure that if you have orthopedic surgery or any prolonged immobility, you should at least discuss having blood thinners to prevent blood clots. Certainly, when there are symptoms that develop; swollen leg, the things I mentioned, you need to seek medical attention, because, not to scare people, but the mantra is that if a patient makes it to the hospital with acute pulmonary embolism, they have a very high likelihood of doing fine, of surviving. It's the next one that you worry about. You don't want to sit at home thinking you're going to get better and then the next one happens, because then that can be a very bad situation. If you have these symptoms and they're not easily explained, you should go to the hospital. My name is Dr. Richard Channick 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 #CTEPH /episode/index/show/phawarepodcast/id/37719875

Episode 547 - Richard Channick, MD 11/18/2025

Jenny Yang, MD - phaware® interview 546 11/12/2025

Jenny Yang, MD - phaware® interview 546 The One Type of PH You Can Actually Cure Dr. Jenny Yang dives into the rare but life-changing world of CTEPH, the only form of pulmonary hypertension that may be curable. From early warning signs to surgical options, this episode explains why ruling out CTEPH should be step one for any PH diagnosis. My name is Jenny Yang. I am currently at UC San Diego. My background is in pulmonary and critical care. I did all of my training at UC San Diego and was exposed to pulmonary hypertension there throughout my training and really fell in love with the disease process of PAH and CTEPH. So, when I finished up my training, I decided that that's what I wanted to pursue with my career. I am currently still working at UC San Diego and focused on pulmonary vascular diseases, so I do some PAH and CTEPH with a really big focus on CTEPH and the different management options that we have for that now. Today, I think it'd be a great opportunity to talk to you all a little bit more about CTEPH. The most common way that a patient presents to us with CTEPH is after they get an acute pulmonary embolism. The vast majority of patients have had a prior PE, or pulmonary embolism, and afterwards they don't recover back to how they were feeling. After three to six months or so of being on blood thinners most people should feel back to how they were before that PE, but a small percentage of people won't. Then they'll get worked up by their local doctors and eventually will get referred to us. It is a pretty rare disease, CTEPH. It only happens in about probably 3% or so of patients after PE. So, sometimes the journey and process of getting to us can take a while, but hopefully most people will find their way to a CTEPH center and can get the appropriate workup and then treatment for that. I think there's more awareness of the disease, which has definitely helped improve provider's awareness, patient's awareness of what CTEPH is and that if they're not feeling back to normal that something is going on and something is wrong. A lot of CTEPH centers have popped up all over the country now. The important thing is that you go to a center that has experience with this disease. CTEPH is unique in that it is potentially curable. It's sort of the only form of pulmonary hypertension that is potentially curable. Anyone who gets a diagnosis of pulmonary hypertension, I think it's important to rule out CTEPH as the first step because if you miss it, you sort relegate someone to being on medical therapy for the rest of their life when potentially either a surgery or other procedures could cure or prevent the need for any further medications. The process of getting to that diagnosis can be tricky. Our screening test of choice is something called a ventilation profusion scan. It's not widely available everywhere and it's seen as an outdated technology in certain places, but it really provides a good initial test to know if someone might have CTEPH or they won't. It's essentially a test where it looks at blood flow in the lungs and if it looks like your blood flow is completely normal, then it basically rules out CTEPH and that's not your diagnosis within pulmonary hypertension. But if it's abnormal, then it's a possibility and it means that you need further testing and confirmatory testing to see if you do have CTEPH. Once they're referred to our center for possible CTEPH, we have this sort of dedicated pathway that they'll go down. They'll meet us in clinic and they get a slew of testing. They'll get an echocardiogram, a chest X-ray, the V/Q scan, and then also a right heart catheterization to assess their hemodynamics and a pulmonary angiogram, as well. We meet as a group once a week, our multidisciplinary CTEPH meeting with pulmonologists, our cardiothoracic surgeons, our BPA interventionalists and chest radiologists, and we all review imaging and history together to make sure that we think that surgery is the way forward. Once we've decided that surgery is the way that we're going to go, most of these patients already have a surgical date penciled in for them since a lot of them are coming from across the country. Then, they will get admitted and go through surgery. The surgery itself, it's a long surgery. It's about eight to 10 hours, an open heart surgery. Most of these patients, they spend about an average of 10 days or so in the hospital. I think the soonest that we've had patients get discharged is on day six after surgery. Most of these patients will fly through and the recovery after they get discharged is about one to two months for that sternotomy to really heal. Then, a lot of them can get back to their lives and feel much better than before and are able to get back to doing marathons, hiking, rock climbing, and sort of all the things that they couldn't do before surgery. We do roughly 180 surgeries a year or so. Then, we've also started a balloon pulmonary angioplasty program about 10 years ago now. That is really reserved for patients who aren't surgical candidates or who still have some residual pulmonary hypertension after surgery. So, we see those patients as well on top of it. It ends up still being quite a lot of patients that we're seeing. Even though there are other centers that are starting all over the country, there's still a big need for CTEPH care and I think a lot of patients that those other centers are potentially serving are ones that couldn't travel or wouldn't travel. We're one center and one part of the country and can only see sort of those patients that can travel, so the select few. There are probably thousands of CTEPH patients that we don't see and still need care. There's definitely a lot of questions and some people will come in with preconceived notions that they don't want surgery or they don't want the balloon pulmonary angioplasty procedure. There's a lot of information online and in support groups that they get that information already and so they can come in with those ideas. When we meet them in clinic and when we talk with them after they complete all their testing, if they have disease that we think is technically operable, we really try to explain to them why surgery is the best option for them. If they really don't want surgery, we won't, obviously, push them towards it, but we'll explain what the potential other options are and sort of the pros and cons of going that way as opposed to surgery. But we've had people definitely who have had what we consider to be operable disease who ended up going with balloon pulmonary angioplasty for personal reasons and still do well, and that sort of fits their schedule and their reasoning better and they're able to travel back and forth to San Diego easily. That is with the balloon pulmonary angioplasty procedure. It does take multiple trips out to San Diego as opposed to the surgery, which it's a longer visit, but it's sort of one time you get the surgery, the hospital stay and then you're done. But a lot of it is explaining and the reasoning behind why we recommend one procedure or the other, and then sort of having that shared decision-making process. Let me give you a brief overview of how PTE surgery works. So it's an all-day surgery. It's eight to 10 hours, and so it's an open-heart surgery, and so they go through the breastbone and really sort of the key tenets of the surgery is that we will do the endarterectomy on both sides. Even when it looks like there might only be disease on one side, a lot of times we'll find disease on both sides and it requires cardiopulmonary bypass. Then, the unique part of the surgery is that it requires this deep hypothermic circulatory arrest. So, we'll cool patients to about 18 to 20 degrees Celsius. Then, when they have adequately been cooled, we completely stop blood flow. So, they have this circulatory arrest period and that is essential so that the surgeons can see exactly what they are dissecting out in the pulmonary artery so that they have this completely bloodless field. We limit that period to about 20 minutes or less. Then, they'll allow blood to recirculate and then they'll do the same thing again for the opposite pulmonary artery. After that's completed, then they slowly rewarm the patient and then usually by the mid to late afternoon they're done with surgery and are closed up. But that's sort of the unique part of that surgery. Now, I'll do the same for balloon pulmonary angioplasty and why we may proceed with balloon pulmonary angioplasty rather than the PTE surgery. With balloon pulmonary angioplasty, we work with our interventional cardiologists for. It requires a multiple trips out to San Diego to perform an adequate number of balloon pulmonary angioplasties. Essentially, what happens is it's done in the cardiac cath lab and patients will start with a right heart cath, very similar to sort what is done during their diagnostic workup for CTEPH, but this time we use femoral access. All of the access is through the groin and a larger sheath is placed so that through there then all of the balloon pulmonary angioplasty equipment can be placed. It takes about roughly two hours or so. The procedure starts with a right heart cath and measuring the pressures. Then, we do a setup for the balloon pulmonary angioplasty. During the procedure, essentially we perform selective angiography. In select areas of the lung where we think that there's disease, which is usually we start with the lower lobes, we will inject contrast to highlight what the vasculature looks like and identify target lesions. Once those target lesions have been identified, we'll pass a wire through that lesion and then inflate a balloon. The goal is to sort of push aside that disease, that clot, and restore blood flow. The goal of the BPA procedure is different than PTE. Since nothing is being removed, it's simply pushing the disease aside to restore blood flow. That's one of the reasons why we really prefer PTE is because we're actually physically removing a lot of that scarred and disease, if possible. So, BPA is really reserved for the patients who have disease that's too distal and that cannot technically be operated on or patients with residual pulmonary hypertension after PTE surgery. That is really it's for disease that's too distal for our surgeons to remove. People can still have residual pulmonary hypertension after PTE surgery. What we've come to realize over the last several years is that CTEPH is this spectrum of proximal and distal fibrotic obstructive disease, but as well as this microvasculopathy component. There may be patients who may benefit from multiple modalities of treatment. If it looks like patients have what is considered to be operable disease, we would still recommend PTE surgery knowing that they may potentially still need BPA afterwards because it looks like they may have some disease that's quite distal that our surgeons won't be able to get everything out. Even when the endoterectomy looks like it was very successful in terms of being able to achieve a lot of that clot out, that component of microvasculopathy can what we think contributes to some of the progression of symptoms and residual pulmonary hypertension after PTE surgery. It does happen that patients will still have symptoms and some degree of residual PH afterwards and may need either BPA medical therapy or oftentimes a combination of both. Riociguat is the only FDA approved medical therapy for inoperable CTEPH or residual PH after PTE. We typically reserve it for patients who are not operable candidates or who have residual PH after PTE surgery. If patients are going towards BPA with sort of pretty moderate to severe hemodynamics, it's become standard of care that patients will get on Riociguat to try to improve their hemodynamics going into BPA. It's been shown to reduce complications of the BPA. So, that's really sort of the main role of Riociguat and medical therapy in general is for patients who aren't surgical candidates or prior to BPA to help reduce complication rates. We do see a lot of Riociguat use before surgery, and we don't have any data to support the use of medical therapy as a bridge to PTE surgery, but it does happen. It's still unclear whether or not that is helpful or not and that data just doesn't exist yet. We had a patient prior to his pulmonary embolism, was a very avid ice climber and hiker and would sort of hike all over the state, all over the country and doing these very amazing trips to tops of mountains. He had a blood clot and afterwards was really unable to do a lot of his normal hiking and really even just walking up a hill started to become taxing for him. This had a huge impact on his quality of life and the things he enjoyed to do. He came to us and had PTE surgery. He lives not too far from us. About probably 200 miles or so. About six months later, he came for his post-PTE visit and ended up biking his way down from his home to San Diego and felt great. He had no limitations on his bike ride. At that visit sort of told us that he'd been back to hiking again. Really at two months is when we sort of lift a lot of our sternotomy precautions. He had already started hiking. At that six month mark, he was doing so well that he got the green light to go back to ice climbing and rock climbing. I think he climbed Mount Whitney and sort of all mountains around California and has now sort of expanded that to the Canadian Rockies and is back sort of living his dream, his life. That part of the job is incredibly gratifying and he sends us pictures routinely of all the various trips that he's done. But that's really what makes CTEPH I think so unique and that people are able to be completely cured. He is not on any medical therapy, has never needed any procedures afterwards and is back to sort of doing what he wants to do with his life. Thank you for listening. My name is Jenny Yang and I am aware that my patients are rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD #CTEPH /episode/index/show/phawarepodcast/id/37709040

Episode 546 - Jenny Yang, MD 11/11/2025

Victor Test, MD - phaware® interview 545 11/05/2025

Victor Test, MD - phaware® interview 545 The Diagnosis Delay That’s Still Hurting Patients Despite better tools and more awareness, people with PH are still being diagnosed too late. Dr. Victor Test opens up about the biggest barrier in PH today: getting diagnosed on time. Learn why shortness of breath is often dismissed and how both patients and doctors can work together to catch pulmonary hypertension earlier. I'm Dr. Victor Test, the Chief of Pulmonary Medicine and Critical Care at Texas Tech University in Lubbock, Texas, and Director of the Pulmonary Vascular Program. I've been a pulmonary hypertension doctor since the year 2000. I began my journey as a pulmonary hypertension specialist as a fellow at the University of California at San Diego, where I was influenced by Dr. Lewis Rubin and Dr. Richard Channick, as well as Dr. William Auger and Dr. Peter Fedullo, and developed a love for pulmonary hypertension and pulmonary hypertension patients. I'd like to take a little bit of time to tell you about the current state of pulmonary hypertension. We are in a very exciting time in pulmonary hypertension. From a therapeutic standpoint, we have a new medication that was recently approved that may alter our future methods and tactics in treating this complicated disease. We're hopeful that in the very near future, we'll have even more choices than we have today. When I started in pulmonary hypertension, we had one medication. The conversation about that medication was long, but the time to choice was pretty short. You either got it or you did not, and fortunately that medicine was a life-saving medication. Rapidly after I became a PH doctor, we'd had one medication after the other to their current state where we are now over 14 medicines to treat pulmonary hypertension. We're hopeful there'll be more. We hope that it will change the landscape in the future for PH patients forever. My own personal crusade is that we will make the recognition and early treatment of the disease a continued priority because we continue to struggle with time to diagnosis and inadequate access to appropriate diagnosis and medications. When I started in medicine, it was so long ago that, for example, one of the training facilities I worked at as a surgical trainee, we did four echocardiograms every Wednesday, and now echocardiogram is widely available. It's difficult for me to understand why the time to diagnosis has changed for the negative. It seems like we should be getting better at that. I think physicians and healthcare people are more aware of the disease, but I think we all have run into the issue where patients can present with shortness of breath, and there's a lack of realization of the importance of that, both from a patient standpoint, where they tend to associate that with their weight or conditioning or asthma or some other diagnosis. Physicians, of course, do the same thing, so they tend to treat the much more common disorders. I think the biggest pitfall there is if you treat for a common disorder and the patient doesn't improve, you have to start looking for a different diagnosis. That's what I preach or at least try to spread the word about when I go out and speak to physicians about our disease of pulmonary hypertension. When we started, we saw people with very advanced state of disease. We still see people with very advanced state of disease at the time of diagnosis. Now, we see a broader spectrum of patients, so whereas we would traditionally see people who had pulmonary arterial hypertension, now we see people with lung disease-associated pulmonary hypertension. Thus, the outcomes and the treatment that we have made a significant difference in decreasing the need and progression to transplant. But transplant is still a big part of what we do, particularly for people with advanced pulmonary hypertension and heart failure. The access to transplant is even more complex than the access to pulmonary hypertension therapies, which is challenging. We're all looking anxiously for more information, particularly about pulmonary hypertension associated with lung disease and what are the best ways to diagnose and treat that disorder, which patients benefit, which patients do not. Do patients with pulmonary fibrosis respond the same as patients with obstructive lung disease? Because if we think about the diagnosis of Group 3 pulmonary hypertension, which is lung disease associated, we now have an approved therapy for interstitial lung disease. But what about those patients with obstructive lung disease, which are quite common. We're hoping that this (CHEST) meeting or in future meetings, we'll have clarity about how to approach those patients. When I started in pulmonary hypertension, we really only used pulmonary hypertension therapies for patients with pulmonary arterial hypertension or Group 1 PH. In the last several years, with the evolution of our therapies and studies that have shown that we can help outcomes in patients with pulmonary fibrosis to have improved quality of life and exercise capacity with pulmonary hypertension therapies, that has changed everything about how we do things. Over time, I've come to realize that patients are always more complicated than I thought they were when I was younger. Pulmonary hypertension patients and particularly pulmonary hypertension patients with lung disease are clearly in that category. The pulmonary hypertension expert, the future, the physicians that we're training now, their outlook is going to be much broader than what mine was. Again, when I started, we had one therapy, it was for one class of pulmonary hypertension. We're now seeing what I hope is to be an explosion of new therapies, which give us all kinds of alternatives about how we manage other groups of pulmonary hypertension. The physician coming up and training today is far more facile, and bedside echocardiography, for example, is a way of diagnosing heart and lung disease, including pulmonary hypertension. I think that the future is bright for those physicians that are interested, intrigued, and dedicated to the treatment of pulmonary vascular disorders. I think for our patients now, we do have more hope than we did. We went for a number of years where we had essentially the same medications. Things are changing rapidly, and that does give us hope that perhaps if we aren't getting closer to a cure necessarily, that we're getting closer to better and more patient-friendly therapies than we've had in the past. I believe a patient today, what I express to them when I see them is, while we would never choose for you to have pulmonary arterial hypertension, that we have more choices than we could ever have, and so there is hope for the future. I'm Dr. Victor Test, and I'm aware that my patients are rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . #phawareMD /episode/index/show/phawarepodcast/id/37719950

Episode 545 - Victor Test, MD 11/04/2025

LaRae Hacker - phaware® interview 544 10/29/2025

LaRae Hacker - phaware® interview 544 How TBX4Life Is Fueling a Global Collaboration LaRae Hacker never set out to become a rare disease advocate—but when her daughter’s TBX4 diagnosis left her feeling helpless, she found power in connection. Discover how became her anchor in uncertainty and why she believes parent-led advocacy is the key to progress in rare disease research. My name is LaRae Hacker. I am a mother of a four-year-old little girl who has pulmonary hypertension as the result of a genetic mutation in the TBX4 gene. We live in Sacramento, California, and I'm here to share a little bit about our story. Kate was born on her due date at 40 weeks, and she was healthy and perfect, and we went to the postpartum room and about six hours after we arrived, she turned blue and was rushed to the NICU where she spent about six and a half weeks of uncertainty in the NICU. She wasn't eating well, she was on oxygen permanently. The doctors would constantly come in saying, "We don't know what is wrong with your little girl." Something never felt right during that. So, I went and pursued second opinion at Stanford, which is kind of near us. That's where she was diagnosed with genetic mutation in the 17th chromosome, which is called TBX4 Syndrome. As a new mom who had just brought a baby girl into the world, it was horrifying. Nobody was able to tell us what was happening. For several weeks, they actually didn't know what was going on and why she had developed the condition that she developed, which they originally thought was PPHN, pulmonary hypertension of the newborn. It was helpless. We spent 15-something hours every day in the NICU just trying to seek out answers and reasons and figure out how to get our little girl home and out of the hospital, which we did at about six and a half weeks. She came home on 24/7 oxygen, a feeding tube, and a pulse oximeter 24/7, as well. That was overwhelming coming home with cords sprinkled like spaghetti all over the house with your newborn restricted to certain parts of the house without the help to get moving to another part of the room. It's funny, when you're going through something like this, you're just searching for an answer. Why? Why is this happening? Because if we know why, then we can fix it, right? That's what you think. When you get a diagnosis like this, this was four years ago, and we thankfully know a lot more about this genetic mutation now, but at the time, she has this genetic mutation called TBX4. By the way, we don't really know anything about it. We don't have any cure for this, and she'll never grow out of it. So, it was comfort we were seeking, and at the same time, just even more devastation that the doctors that we'd seen before at Stanford who said, "Oh yeah, babies grow out of this. She just must be taking a lot longer than your average kid, but she'll be off her oxygen and her heart will be fine." Then, when you find out the reality, it's an even bigger blow. From there, her pulmonologist, I had asked her, "What do we do? How do you get attention in rare diseases that nobody knows anything about?" She had told me that it's actually parent advocacy groups that make that difference and are able to make bigger waves than the little institutes or the research labs or whatever else. You have to make a wave and you have to make an impact. Shortly after that, I discovered a small, very tiny little Facebook group with about 20-something people in it called Families of TBX4 Syndrome. I think that next year, Anton Morkin, the CEO of , started the organization. I just couldn't get her doctor's words out of my head that parent advocacy groups were the ones who do it. So, I couldn't really let him just deal with this alone. I stepped in and tried to help as much as I could. She was on 24/7 oxygen and a feeding tube until about two and a half. Within three weeks of each other, she was freed of both of those things, which was amazing. That was incredible. It was hilarious though, because her little two-and-a-half-year-old mind, she did not even notice. Before she was off of oxygen, she would get literally clotheslined around the corner, right? She'd run around the corner like her little two-year-old self, and then the oxygen cord would get caught in the corner and she'd fly back, or she'd get caught around the coffee table and stuck and had to rewind herself. But then, suddenly, she was off of this oxygen and she literally didn't notice. The only time I noticed that she recognized it, was she liked to play with her little brother's booger sucker, for lack of better words. She would move her oxygen on top of her nose as her little routine and then play with it. She went and grabbed for her oxygen one day and didn't find it and kind of looked confused for a second, and then just went about her day. So that was pretty funny. But she's doing great now. She's four. She's in preschool. We go to church, and she's able to be there without any issue. She's incredible. She doesn't know really much about her condition. She doesn't understand that yet, other than we have breathing treatments throughout the winter to protect her a little bit more from illness. Otherwise, she just thinks she's a typical little girl, which she mostly is. The 17th chromosome is where the T-box genes live, and TBX4 is one of those genes. It's widely known as something called small patella syndrome. Up until only a few years ago, we knew of it as an orthopedic problem. So funky kneecaps and feet and hip issues. It had only a few years before Kate was born been discovered as also connected to pulmonary hypertension. This mutation or deletion in this 17th chromosome is what causes pulmonary hypertension. I think that there's been a lot of noise happening about TBX4. I think when Kate was diagnosed, it had just kind of been getting started. Thankfully, we were lucky enough to land in the lap of a pulmonologist who actually actively was researching TBX4 when we got there. But now, I think that through the work of , there has just been so much more noise and awareness about this TBX4 gene and researchers and scientists just seem to be fascinated about why this impacts the lungs and the heart to cause pulmonary hypertension. We've seen a big growth in the connections that we have with TBX4 and the clinicians and scientists who are interested in joining our movement is awesome to see. TBX4Life is a rare disease advocacy group, is kind of how we explain it. We realized that in order to actually fund clinical trials and research, you need millions, and that takes a long time and a lot of work, and sometimes just luck. Anton Morkin, our CEO, he just knew that you had to just connect people. We have institutes like Vanderbilt and Boston Children's Hospital and Stanford, and even many institutes internationally. These scientists work in collaborative groups, and they meet fairly often, and they basically share the research that they're actively studying that's not been published yet. They have this wonderful agreement with one another that, hey, you can't share my research, but let me tell you what we're doing so that you can maybe use this information I learned on your study and then we can get closer to, ultimately, our goal is a cure, right? But better treatments to prolong the lives of those impacted by not only TBX4, but this work can also impact those with lung disease and anyone with pulmonary hypertension, as well. Some of the other things that TBX4Life is championing is a that we had earlier this year in San Francisco. We had a little over 60 participants come from around the world and share their research, share their papers that they've published and the findings that they have. They did case study descriptions of different patients that they've had and the tricky patients, the ones that they can't figure out, and they had dialogue with one another. "Hey, have you tried this? Have you done that?" It was just wonderful to get everybody in a room together and humanize everyone, but also just have that collaborative effort between major research institutes who don't usually share research information. They keep it tight because Stanford wants to post that or Vanderbilt does. So, it was lovely to watch that collaborative effort across institutes. I am the Director of Communications. Basically, I help support the community of families and keep those connections strong. I run social media pages and also work on fundraising. I am obviously hopeful. It's the only thing that you really can do. You have to remain hopeful in order to survive something like this, honestly, because it is so frightening and there is still so much more unknown. Even at the conference that we had in San Francisco, there was still so much uncertainty, or we don't know the answer to this. We don't know why this child presents this way. We don't know why this specific thing happens in the lungs that then impacts the pulmonary hypertension. My hope comes from the amount of people that were involved and are involved in this organization that before it's too late for my daughter, we can find some way to prolong, extend life, or even ultimately cure pulmonary hypertension in general without the need for a lung transplant, which we all know only slightly increases the time that you have. That would be my hope. My aspiration within TBX4Life would be to keep this train and this momentum going in the medical community, continue expanding it, continue finding more families who have this genetic mutation. There are actually so many that we know are out there that they just had a genetic panel that didn't at the time include TBX4. So, they think that they have unknown causes, but then they're slowly coming out of the woodwork, these teenagers, these adults who find out, "Oh, this whole time it's actually been because of TBX4." So yeah, my hope is just to broaden our reach. I'm LaRae Hacker and I'm 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: /episode/index/show/phawarepodcast/id/37707395

Episode 544 - LaRae Hacker 10/28/2025

Anthony Carrasco - phaware® interview 543 10/22/2025

Anthony Carrasco - phaware® interview 543 How I Survived Meth Addiction, Lung Failure, and Found Purpose Anthony Carrasco was a meth addict by 15, and nearly dead by 35. Diagnosed with PH after years of drug use, his story spiraled from homelessness to hospice. But a double lung transplant and a deep surrender to recovery transformed everything. This Special Edition Episode Sponsored by: My name is Anthony Carrasco. I'm from the Bay Area, born and raised there. I'm here to discuss what my part in the pulmonary hypertension world was like. Basically, I am a bilateral transplant post-patient, so I had my lung transplant last year. I started with pulmonary hypertension. I wasn't able to breathe or walk too far without breathing heavy. It was very hard to breathe. They diagnosed me in 2005. I took medications after medications after medications. I'm still on medications because it’s post-transplant, I take the anti-rejection medicines. But before, I tried maybe 15 different medications while I was going through my pulmonary hypertension to try and bring my oxygen levels up, and my pressures down in my heart. I took them as the doctor prescribed. We did that for 19 years. Nineteen years of taking medications to try and help me with my pulmonary hypertension. They worked for a while, and then after that, they didn't work. In 2023, I was told that my oxygen levels were not going up, even with the medications and the oxygen. Still, my oxygen levels were not going up, so they made the decision to put me on the transplant list. It's pretty crazy. Four days later, they called me and said, "We have a match." I freaked out, of course, a little bit, because it's my first time ever having a major surgery. I was praying and trying to get my head in the right place with God. They got me all prepped at UCSF. I was laying on the gurney, just waiting. The last thing that the doctor has to do, the transplant doctor, is hold the lungs that are coming in for transplant, because they come in in a little ice cooler. If the doctor feels like they're good lungs and he's okay with moving forward, then we move forward. But if there's anything wrong with the lungs when he holds them, he calls the whole thing off. They prepped me for a good 12 hours. The next day, when they came in, the doctor held them and said, "Nope, I don't like these ones," so they called it off. Fortunately, I had my transplant the right time, but I had been called four other times to go in and get prepped, and they called it off four times in a row. On the fifth time, the lungs came in, and the doctor said, "It's a go." All of a sudden, I'm saying bye to my wife, and next thing I know, I wake up. I asked the doctor, "Hey, when are we going to get my transplant?" He goes, "You're already done. You were just in a 12-hour surgery." I go, "What?" They had me tied down and everything, because some patients get excited when they come out of a surgery like that. I was okay and they released my hands, and the healing started to begin. It was a very, very hard process to get up, walking, and getting used to your new lungs. Actually, I was to a point where I didn't really know how to breathe normally. I was always trying to gasp for air. It took me a good couple of days to get used to the lungs. What my brain was telling me, what my lungs were doing were two different things. Right out of surgery, I said, "I'm ready to walk." The reason why I wanted to walk and do this so quickly, because I wanted to get back to playing golf. I got up that same day and I walked a little bit, and then I was doing laps in the hospital, and they said, "After nine days, you're ready to be released, you're good." So nine days later, I'm getting released after having a major surgery like that. Here I am today, with no oxygen, breathing at 98 to 100. Before, I was breathing 78, with 10 liters of oxygen. I feel different. I feel like my skin color's back. I'm just really, really excited about life now, and it's really flowing over into my real estate company that I have. People would just say I'm excited and I'm a different person. I was a gray and purple person before. Now, my skin is pink and brown, which was my normal color. It's just crazy how changing the oxygen in your body will affect your body, your nails, your hair, everything, eyelashes. Now, I can tell you that going through this process, I look at life differently now. I definitely thank God for the breath, every breath that I take in my life. Before, I would take it for granted. We just breathe and it happens. But now that I've been through this, when I think about my breathing, it's just like every breath is a miracle from God. Every breath. I still am on a lot of medication, but that's post-transplant, but nothing to do with pulmonary hypertension. It's all just post-transplant medication, which is like the anti-rejection drugs. I want to share with you and go deeper into my story of how I got the pulmonary hypertension. I was actually raised with my mom. My mom and dad got divorced when I was five years old. My mom had to work and take care of four boys, and we were all a year and a half apart. So, all she did was work. I basically raised myself. I started hanging out with the wrong crowd. I was 13, and I started smoking weed, and it was like, "Wow, this stuff makes me feel different.” I really liked it. I found out later that anything that I like, I have the addictive personality. It's all the same thing, with every kind of drug that was out there. This all happened the same way. Basically, I smoked weed on Friday and Saturday, then it turned into Sunday, and then Monday. Then, now I'm a full-blown pothead at 13 years old, smoking weed every single day. After a year of doing that, I started experimenting with other drugs, and the same thing happened. We started with coke, and started doing that on Friday, Saturday, and it turned into Monday, Tuesday, Wednesday, Thursday, and now I'm full-blown coke addict. Then, I smoked crack for a while. I smoked crack twice in my life, once for six months and once for four months. That's how fast it took me to lose everything that I had. Crack is an addiction that it's just like you have to have more, and you'll do whatever it takes to get it. But the last drug that I did, I started doing meth, methamphetamines, when I was about 15 years old, and I loved it. It made me awake and alert, and just like a different person. I stayed with the meth from 15, all the way up until I was 35. So that long of doing meth. I never injected it. I smoked it and I snorted it. Sometimes I would eat it, depending on the situation, but it took me out, basically. When I started doing the meth and I turned 18, I pretty much hit the streets and got into a little apartment. I had a bunch of little apartments, but I lost them all during my addiction, all of them. I was still working, making great money. I was working for Cellular One when the phones first came out in 1989. So, 1989 to 1999, I was making a boatload of money. I was making $250,000 a year, and I'm a 19-year-old kid. With that money, I just went full-blown addict. I was going to Vegas and justed lived that life. It was crazy. But what happened was, in 2003, I was actually homeless at that time, living on the streets. I had a girlfriend, and we were both just living on the streets, and we'd sleep wherever we could if we needed to sleep, but most of the time we were up. If I was awake, I was loaded. Alcohol was a part of my story too. My flavor was Budweiser. I would drink Budweiser all day, all night. If I was awake, I was drinking along with the meth and cigarettes, and it all came together. In 2002, I would walk a little bit and I would pass out, and I didn't know what the heck was going on. I couldn't breathe. It happened quite a few times, and I would go to the hospital. They would give me albuterol, prednisone, just to make me feel really good and where I could breathe. I felt like a lot better than I did before. So as soon as I would pass out, they'd take me to the hospital. I'd get out and do the same thing over again. Just start with the drugs every time, even though they told me, "Don't do them because you're going to die." The drug was that powerful, where I didn't even hear those words. I just kept doing what I was normally doing. Those last three years of my addiction, it really was hard for me. I kept passing out, and I couldn't breathe. And at the end, I went in for the last time, and I said, "My ankles have swelled up really big. I was retaining a lot of water. I didn't know what was going on." I thought I had some bad dope. So, I went in there, and I told the doctor. They knew me in there, and everyone knew my name is Worm. Everybody called me Worm. Nobody knew my name, Anthony. That was like another person, really another person. When I got clean, I read about the worm, what a worm is. It's the creature, the insect that goes the lowest in the world, way down in the dirt, lower than any other species. That's what I was. I was a worm. I was at the bottom of the bottom. When I went into the hospital, and the doctor said, "Hey, man, we've been telling you for three years now that there's going to be a day where you're not going to make it, and today's the day you're not going to make it. Your heart is failed. Your lungs are shutting down. Your organs, everything's shutting down. So we're going to give you morphine to make you comfortable." Basically, they put me in hospice. They said, "Do you have any family?" I said, "Yeah, but I haven't talked to them in three years, because I was embarrassed of my appearance, and my family for what I was doing, what I chose to do." He said, "You should call your family." So, I called my brother, Jim, and he came over the hospital after I hadn't seen him for three years, and then my other brothers came. My mom came and my dad came. I can remember them standing around me. Sorry, I'm getting a little emotional. Sorry. I remember them standing around me, and they were crying. I just looked so bad. I was 110 pounds. I wouldn't eat. It's really hard to eat when you're on meth. They just sat around me and held my hand, and they gave me morphine, and just waited for me to die. I woke up 10 days later. I just came out of the morphine high that they were giving me. I was just out of it, didn't really know what was going on. I came out of it and I looked around, and I thought I had died. I looked around and I saw all this hospital stuff, and I pushed the button. The nurse came running in. "Are you okay? Are you okay?" "Yes, I'm good." In my mind, it told me that, "Let me get out of here, so I can go back out there and use again." But the doctor came in. I'll never forget him. He said, basically, "Hey, you might make it. Everything's starting to wake up again. We're start giving you a lot of medications to get you back to being okay. So, you need to make a decision, Anthony. You can go back out there in the street and use again, and die," exactly what he told me, "or you can get help for your addiction, and live a life that you should have been living a long time ago." He told me straight that you're going to die. My brain told me, "Hey, if I go into recovery, I won't be able to drink anymore." This is disgusting when I think about it, but I asked the doctor, "Does that mean I can't even have one beer?" after he just told me I was going to die. He just looked at me in disgust. He said, "Yeah, well, you figured out what you want to do. You didn't want to die or you want to live." He was an older doctor and he wasn't playing. I called my mom, and my mom had been in recovery already, so I knew a little bit about recovery. I said, "Mom, I got to go back out there and find my girlfriend," who was cheating on me anyway. It was just a horrible situation. I told the doctor I want to get clean, but I need to go find my girlfriend, which was a bad choice, because I could have easily picked up again. I went to the apartment that we were staying in, and I climbed through the window, and I saw all these pipes on the floor, and it just looked like it was a meth party. I started breaking them. I just started stepping on them. It made me sick. I just started breaking them, instead of picking them up and using, and I could have did that. I just started breaking them. I walked out of there. I got out of the house. I just went through the window. That was our entrance, and I called my mom. My mom came, pick me up, and my recovery started. The first time I went to Sunrise in Concord, I had a lot of doctor appointments, because they diagnosed me with congestive heart failure and pulmonary hypertension. They had all these doctor appointments set up for me, and they put me on a program. I started to try their medications, and everything was getting a little bit better. I felt a little bit better. I went through a 90-day program in Concord called Sunrise. Then, from there, I went to my mom's, but my mom couldn't handle me because I was still a mess. So, I went into an SLE. An SLE is a sober living environment. I stayed there for a year and a half. After six months, I became the house manager. I was the house manager for a year. With the program, they teach you how to show up and be accountable and responsible, and how to take transportation. I had no car. I had no cellphone. I had nothing. My mom paid for my SLE, and I stayed there. It was like $500 bucks a month. Then, from there, started going to all my doctor appointments, and everything started to get a little bit better. They were starting me on medications. They told me that I would need a heart and lung transplant within two years. If I didn't get it, that I wouldn't be able to make it. All those years later, I was following directions from the doctors. That was the most important part right there, because I had to do that if I wanted to live. My life was so good. I felt better than I ever had before. I walked outside. I was in Hayward. I walked outside of the SLE. I'm walking to the bus stop, and I'd hear the birds so loud, and the flowers looked so bright, and the mountains. I didn't know there was mountains in the Bay Area, because I was always walking with my head down, and I saw the mountains and I was surprised. I included God in my life and I started to go to a thing called Celebrate Recovery, a 12-step program that I'm still involved with today. I was going to AA, NA. I started sponsoring people. I'm totally blessed by God, that he took me from living down in the dirt to buying a house in Alamo, California. We used to call them pipe dreams. I would take a hit of a pipe, and then I would dream about whatever I wanted. They were called pipe dreams. That's what they call it, pipe dreams. I always wanted to do real estate. As soon as I got clean, two months later, I went and took my real estate test. I passed the first time. Nothing ever good happened to me. It was always bad. In and out of jail in Santa Rita. I've been over there 20 times. Sometimes, I would like getting arrested because I had three hot meals and a cot. Three hot and a cot, that's what they call it. I lived at Santa Rita, basically, sometimes. In and out of the street, get picked up, and that's just the way it was. The whole time, I was doing meth. It ruined my lungs from smoking meth. That's how I got pulmonary hypertension, through smoking the meth and doing the meth, and the alcohol, and just not taking care of myself. By the grace of God, I'm totally healthy today. I went from living under the bridge to living up on a hill on Alamo. It blows my mind. I have a 16-year-old son that I was able to raise. I got custody of him when he was 10. His mom still struggles with alcohol and drugs. I was able to go through a two-year court battle, and I got him when he was 10. Full legal, physical custody of him. He's still with me today, and he's doing great. He's getting straight A's in school. God has given me a second chance at raising kids, because I've had kids before that I wasn't present for. I have three other kids that I wasn't able to raise, because I didn't even know how to take care of myself. So, a lot of blessings. People that come in from homelessness, drug addicted, I'm the first one to talk to them. That's my heart. I've been there. I can relate to them. I pull over to talk to the homeless people. I'll eat lunch with them, or I'll pray with them, or whatever it is. I can remember. I just wanted them to know that there's hope out there, and that if I can do it, you can do it. I'm here to support you all the way through, show you how to change your life, like I did, and I'm passionate about it. If you know anyone that's struggling with addiction, and they're going through the same thing of not being able to breathe, please call me. I'd be happy to walk you through the steps and bring you through the whole process to get to where I'm at today. By the grace of God, I'm successful. My heart is to help people. Most of all the drug and alcohol addicted, I want them to make it, as well. That's where my heart is at. I'll do whatever it takes to get them where I'm at. So, I'm here for you. My information will be below. If you have any questions or want to ask me questions, personal questions, I'm here for you. But I'm doing great. I just celebrated my two-year lungaversary. We call it lungaversary. It's the anniversary for when we had our lung transplant. It's a big day for me, and a big blessing that I can say I'm here after the transplant, two years later, without oxygen. It is all because I am staying clean. It's crazy. When you stop doing drugs, you stop going to jail. Just remember that. I haven't been to jail since 2005, and that's because I stopped doing the meth, right? I'm trying my best to live a life the way that God wanted me to live. I just want to thank you, and thank you for having me on your podcast. It's amazing to be here with you, and I'm just so, so honored that you would ask me to share my story. Thank you. My name is Anthony Carrasco, 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: /episode/index/show/phawarepodcast/id/37706805

Episode 543 - Anthony Carrasco 10/21/2025

Brandon Jakubowski, MD - phaware® interview 542 10/15/2025

Brandon Jakubowski, MD - phaware® interview 542 Treating Meth-Associated PAH Without Judgment Dr. Brandon Jakubowski takes us into the underrecognized world of stimulant-induced pulmonary arterial hypertension. From misdiagnoses to under-prescribing life-saving therapies, he lays out the systemic gaps and stigmas preventing patients from getting the care they need. This Special Edition Episode Sponsored by: My name is Brandon Jakubowski. I'm an Assistant Professor of Internal Medicine at the University of Texas at Southwestern Medical Center here in Dallas, Texas. Ever since med school, I was actually interested in pulmonary hypertension. I remember seeing my first pulmonary hypertension patient as a third year medical student. It was on the pediatrics rotation, actually. Everyone told me, "There's this patient in this room on this continuous IV infusion. If it falls out, it's really scary. Be there immediately to help the nursing staff." At the time I thought to myself, "What on earth is this disease?" Just through kind of sheer luck and happenstance, I got put on the pulmonary hypertension unit when I was a fourth year medical student, and I just fell in love with the physiology. I fell in love with the patients. I knew I wanted to dedicate my life to it. It's a disease process that if you look at the last 20 years, the amount of therapies that we have had just exponentially increased. I'm super eager to see where the next 20 years leads us. So today, I'll discuss a little bit about the historical perspectives of drug and toxin-induced pulmonary hypertension. I really want to emphasize on some of the challenges in this space, particularly access to advanced therapies and under recognition and stigmatization of drug and toxin-induced pulmonary arterial hypertension. For us as PH physicians, it was kind of in the early 2000s that we started to have increasing recognition that stimulant use was associated with pulmonary hypertension. That was really based primarily on small case reports. It wasn't until 2006 that the Center at UC San Diego published this groundbreaking article. Essentially what they found was, patients that were labeled as idiopathic pulmonary arterial hypertension, about 25% of them actually had a history of stimulant usage. When they compared it to other associated forms of pulmonary arterial hypertension that had known risk factors or had known chronic thromboembolic pulmonary hypertension, they found that there was a pretty stark difference. Only about 4% of those patients actually had a history of stimulant usage. That's really when we started to think, "Wow, this might be a definite association." Now, it should be kind of routinely part of our screening process, because we know that untreated stimulant usage or continued stimulant usage only leads to worsening pulmonary hypertension and worsening RV failure. I think the inception of the field of pulmonary hypertension is really the most fascinating one. It really started in the early '60s when there was this epidemic of weight loss medication called Aminorex that was available over the counter. One German scientist, actually, saw that he was diagnosing a lot of, what they called at the time, primary pulmonary hypertension in his own cath lab. As astute as he was, he was asking them their histories and drug histories and found out that the large majority of them are actually taking this weight loss supplement. It was over the course of the next several years that more reports of this were coming out of Austria, Germany, the surrounding area, and that actually led to the medication being taken off the market in the early '70s. The thing that's fascinating about it is, if you look at the chemical structure of Aminorex, it's very similar to the chemical structure of fenfluramine, a weight loss medication, that was commonly prescribed here in the United States in the '80s and early '90s, and also very chemically similar to methamphetamine, amphetamine derivatives that people use nowadays. I think this was a really big mistake by the medical community and by the FDA, not recognizing that Fen-phen had a definite association with pulmonary arterial hypertension. It was on the market for 16 years ultimately, whereas Aminorex was on the market only for maybe 4 or 5 years in total after its recognition. I think there was just a really large appetite for people to have access to these weight loss therapies at the time. Even, I believe, it was the CDC put out a statement that said, "The risks and benefits of untreated weight loss may be worth it if you have to take the risk of being on this medication, potentially, that can lower people's weight." But I think in my opinion, big mistake. Really did not do justice for our patients or the medical community. There's a definite geographic trend of amphetamine usage in the United States where the West and the Midwest are dominated by much higher rates of methamphetamine usage compared to the East Coast. But the statistics are actually quite alarming. It's about one and a half million Americans that report methamphetamine usage in the last year, and about a hundred thousand of those actually tried methamphetamine for the first time in that year. We're starting to see increasing rates of overlap between drug use with patients using cocaine and methamphetamines or opiates and methamphetamines. Some of the really concerning thing is, you look at just US 12th graders, 1.1% of them have reported using methamphetamine in the last year, too. I just think those are statistics that are kind of shocking to the mind, and it's something that we need to routinely screen for. I think people so often are worried about or trying to hide their drug use for fear of negative judgment or embarrassment or even risk of legal consequences, and that leads us to misdiagnosing them, ultimately. It's really difficult, on occasion. You want to be able to ask these questions in a very non-prodding manner, in a non-judgmental manner. It's usually a question that I'm saving for later in my conversation with patients. The way I like to frame it is, "These are a set of questions I ask all of my patients, and they're not meant to be judgmental. They're meant to make sure that we have an understanding as to what your disease process is." I start with things like, "Have you been tested for sleep apnea? Do you have symptoms of sleep apnea?" Before moving into what people think may be more some of those prodding questions like history of illicit substance use. But I find that waiting till later in the visit at the point in time where I've been able to establish rapport with a patient and gain their trust makes them much more likely to want to divest that information to me. I often tell patients that there's a set list of things that we know cause pulmonary hypertension, and this is at the point in time that I'm explaining to them the pathophysiology and how pulmonary hypertension progresses over the course of time. I like to tell them that if we can identify a cause of what's leading to pulmonary hypertension, we should treat that underlying cause, ultimately. If that's from chronic blood clots in the lungs or if that's from an underlying autoimmune disorder, anything that we can do to minimize the risk of PH progressing, we should undertake that. That's no different for stimulant-associated PH. I think one of the key ways of getting this information from people is just being honest, saying, "A significant portion of patients in our clinic report some history of methamphetamine usage. And I'm curious, have you ever used methamphetamines in your life?" It's really coming from that question in a manner of curiosity and understanding as opposed to an accusatory nature. Sometimes, people do not want to tell you this information. Again, that's because of fear. I think fear of the unknown, fear of legal risks, fear of being stigmatized or fear of being treated differently because of their history of drug use. But again, I think that's why it's so important to focus on developing rapport in the first 30, 40 minutes of your discussion with somebody, really sitting there and listening to how have their symptoms progressed over time? What are they dealing with now currently? That way, they can feel like you are an ally in their healthcare, working towards getting them better, ultimately. It's unfortunate that despite the life-saving potential of parenteral prostacyclins, these therapies are notoriously underutilized in clinical practice. This is true for our idiopathic PH patients, and this is especially true for stimulant-associated PH patients. Really, our guidelines say that any high-risk PH patients should be started on IV or subcutaneous prostacyclin in addition to oral therapy. Though the reality is that most patients that meet this criteria, never receive a parenteral prostacyclin. We have decent national registry data from the PHAR registry that reveal a pretty striking gap in patients that have stimulant-associated PH and that they're far less likely to be on IV prostacyclins compared to their idiopathic counterparts. It's only about 6% of stimulant-associated patients get IV therapy as opposed to 28% of idiopathic patients. That's adjusting for lots of factors. You see that stimulant-associated PH patients have an overall lower odds ratio of about 90% to get onto IV therapy. I think that just confirms that clinicians are very hesitant to initiate this aggressive therapy in this patient population. That's due to concerns about adherence and instability or active drug use. But the unfortunate consequence of that is that many of these patients are under-prescribed, despite the severity of their disease and only managed with oral medications that are unlikely to really control their disease in the long term. We have an incredible experience treating this patient population at UT Southwestern. I found that with our multidisciplinary approach we are able to utilize resources from our psychiatry colleagues and deliver compassionate care and substance abuse counseling. These patients can have dramatic responses not only to PAH therapy, but just to cessation of their drug use. We see rapid improvement in right heart function with cessation of methamphetamines. Again, this gets back to the point of why it's so important to recognize this early on, because I've seen patients who initially were diagnosed as idiopathic pulmonary arterial hypertension, and it wasn't uncovered until maybe a year or two years later that they actually had a history of active substance abuse, and it was only recognized in the workup going into transplant or things of that nature. That's something we want to avoid if we can, because we want people who are, despite sliding of their medical problems on therapy, we want them to be able to get transplanted. We have our own several success stories at UT Southwestern of patients who were previously abusing methamphetamines and are now transplanted and doing really well. There's a huge research gap in this field. I really applaud the clinicians out of Stanford for doing a majority of the work in this space. But there's no doubt there are definite challenges in this patient population with the comorbidities and concerns for active drug use. I think it's so incredibly important to have avenues for support for them. That way we can ensure that they're adhering to therapy and deriving the benefit that we see in other patient populations that have PAH. Historically, these patients have been excluded from clinical trials with active drug use. That's understandable in order to get a best quality sample ultimately for clinical trials. But we need to really, as a whole, the medical community needs to build a better rapport with this patient population. These patients have been stigmatized for so long that they don't want to seek healthcare because of concern of being treated unfairly or being treated improperly. So, we need to do a better job of categorizing and being able to follow these patients long-term with registry data and even with clinical trial data. Essentially, many patients, not just those with stimulant-associated PAH, but even those with other forms of pulmonary hypertension suffer from a lack of access to care at specialized PH centers. This is for a variety of reasons. Sometimes, there's not a specialized PH center located near them. Sometimes, they come from very underserved communities with very limited access to healthcare resources. That's unfortunate, because we know that stimulant usage is highest in lower income communities or communities that don't have the same educational resources that others do. You add on top of this, things like the socioeconomic obstacles and under-insurance, lack of transportation, all of these things make a consistent follow-up and treatment in this patient population particularly difficult. So, it's something that, as a healthcare community, we have to continue to focus on to better the lives for this patient population. Thanks for listening. My name is Brandon Jakubowski, 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 /episode/index/show/phawarepodcast/id/37705620

Episode 542 - Brandon Jakubowski, MD 10/14/2025

Nicholas Kolaitis, MD - phaware® interview 541 10/08/2025

Nicholas Kolaitis, MD - phaware® interview 541 The Face of Addiction Isn’t Who You Think It Is Once thought to be a West Coast issue, methamphetamine-associated pulmonary hypertension is now a nationwide and global crisis. Dr. Nicholas Kolaitis reveals how building trust with meth associated-PAH patients can lead to incredible turnarounds, including sobriety, trial participation, and successful transplant. This Special Edition Episode Sponsored by: I am Nicholas Kolaitis. I am a pulmonologist at UC San Francisco. I primarily focus on pulmonary hypertension and lung transplantation. I have been interested in this space for quite a long time since I was in training as a resident at UCLA where I was training with Rajan Saggar and learned a little bit about the impact that pulmonary hypertension can have on patients. I'm pretty passionate about the way that we can actually improve lives for patients with pulmonary hypertension and the field of methamphetamine-associated pulmonary hypertension is something that is quite unique in the sense that it is very regional, historically. In California, where I've done all of my training, we see a lot of methamphetamine-associated pulmonary hypertension. So, this has been something that has been ingrained in me, the care of these patients from the time that I was a medical student, because I've seen these patients from medical school, to residency, to fellowship, to being an attending and throughout my career. Seeing the impact that this highly addictive substance can have on people and seeing the way that it can cause pulmonary hypertension, but then also seeing the uplifting side of it when you can actually get people better and get people to stop and impact their lives for the better is pretty dramatic and pretty incredible. With methamphetamine-associated pulmonary hypertension, the change in the field has been that it's becoming more prevalent and it's also moving. As I mentioned, I practice primarily in California and I've been in California my entire life. I did my medical school in UC San Diego, and then I was at UCLA for residency and UCSF for fellowship and had been exposed to it multiple times throughout my training. Then when I was in fellowship with Teresa De Marco at UCSF, we honed in on this idea that methamphetamine is really a western regional phenomenon. We were able to use data from the Pulmonary Hypertension Association Registry to look at the distribution of methamphetamine-associated PH throughout the United States. We looked at this registry, which is this large 72-center registry throughout the US from patients that are seen at expert care centers. There's currently about 3,000 people in the registry. At the time we did our analysis, is was closer to about 1,500, but at the time that we did our analysis, we looked at the distribution of methamphetamine-associated pulmonary hypertension throughout the US, and we saw that 83% of all methamphetamine-associated pulmonary hypertension is in the Western US Census region. This makes a lot of sense if you sort of think about where methamphetamine has historically come from. Initially, the history of it is that the Hells Angels, which is a motorcycle gang native to sort of Northern California trafficked methamphetamine throughout the Western US, and then as we started getting more addiction to methamphetamine as a population, a lot of meth has started coming from the southern border, and so it's been come up through the southern border, then it's trafficked mostly through the West Coast of the United States. That's why we've seen so much of it here. In training, this is one of the things that I queued in with either, whether it was with Raj or with Teresa, seeing how much of their patient cohort is patients with methamphetamine-associated pulmonary hypertension. Then what I've seen over time, is that we're seeing not necessarily more of it in the Western US, because it's always been very prevalent here, but what we're seeing is that methamphetamine-associated pulmonary hypertension is becoming more common outside of the Western United States census region. It's going south, it's going to the Midwest, and it's starting, occasionally, to make its way to the east. One of our colleagues who many of you probably know, Rich Channick at UCLA, he was in San Diego then he was in Boston and then he was back in Los Angeles. I was talking to him about this and he told me that when he was in San Diego, about half his practice was methamphetamine-associated pulmonary hypertension. Then he moves to Boston and he said he asked every single patient for the years and years that he was there and not a single one had methamphetamine-associated pulmonary hypertension. He moves back to Los Angeles, and there it is again, about half his practice is methamphetamine-associated pulmonary hypertension. It is a very common thing where meth use is prevalent, but it is moving. If you look at diagnoses of methamphetamine-associated PH, whether it's in sort of the updated PHAR registry or whether it's through claims, you're actually seeing more claims in the south now than before. You're seeing more claims in the Midwest than before. Then outside of the United States, it's actually a problem. People don't really realize this because people think of this as really just, oh, this is a California or Arizona problem, but it's actually much bigger than that. We have colleagues that are in Australia and they're commenting that meth use there is rampant and meth-associated PH there is rampant. This drug is trafficked typically through Myanmar, down into Oceania. Then, we're also seeing it in parts of Europe, as well. There's a whole population in Spain that's actually using a drug that they're calling Shabu that's similar, that's basically methamphetamines. You're seeing it over multiple countries throughout the world. The problem with this is that it's incredibly addictive and it's very easy to use and it's very, very cheap. The time from first use to compulsive behavior with methamphetamine is incredibly short, and that leads to the development of addiction quite easily, which can then lead to pulmonary hypertension. I think that there's a couple of interesting aspects to it. One is the people who don't see it. There are a lot of people who don't see it in their practice. It's not that they're not looking for it, I don't think. Maybe, historically, that was the case because they didn't really know about it as much. But now, at least in the United States, people know that this is a problem, so they ask about it. I have many colleagues from the eastern part of the United States that ask me, "What do you do? Do you screen all of your patients for meth? How do you find it?" For the most part, the people that are coming to see us in the clinic are people that have understood that there's a problem that's causing them to be short of breath, understood that there's a problem that's limiting their ability to live their life as full as they can and they want help and that's why they're coming to see us. For the most part, these patients are pretty open about it. I have not had a whole lot of patients where you ask them, "Could methamphetamine be causing your symptoms or how much methamphetamine do you use?" and then they lie about it. Sometimes, it might take a coaxing to get the information out, but for the most part, patients are very upfront about it. I think there's a misconception there that I hear sometimes is that they're not going to tell me about it, so what should we do? Well, a lot of the patients will actually tell you about it because they're coming to you because they are seeking help. I think that's one thing. I think the other thing is that approaches to care for these patients is different. They're coming to us because they would need help and they're coming to us because they want to get better. They're not coming to the doctor because they don't want to be adherent to medication. They're not coming to the doctor because they are doing it for fun. They're coming because they realize something's wrong and they want to get better. Pairing with them has actually made a big difference in terms of getting them to buy into the therapy and buy into the treatment. There is some conceptions out there that we shouldn't be treating these people because ongoing substance abuse can then lead to worsening outcomes. Certainly, if you look at it from an economic standpoint, you might say that this is bad to give expensive therapies to people that are actively using drugs that are causing the disease to get worse. I'll counter with that with this is somebody who, again, is coming to you because they need help. They're coming to you because there's a problem and they're coming to you because they want to get better. If you actually focus on the fact that they are there because they are seeking care and you give them the option for being heard, number one, not brushing their symptoms under the rug and options of therapy to maybe make them better, they'll buy into you as a provider and then you have your in to try to get them actually better. I've had patients that we've seen that have been using for decades. They've been told by other people, "We're not going to take care of you because you keep using drugs." I've had patients that have been seen and they've been using for decades and they've been told, "You're going to die and there's nothing we can do about it." Then they come to see us and we tell them, "Look, I really don't care if you're using right now. What I want you to do is take our meds and here's what's wrong with you and here's why. It's because of the drugs and if you stop, we can get you better. We might not be able to fix everything, but we can get you a lot better. If you don't stop and you take the drugs, you're going to continue to get worse. I don't really care either way, but I'm going to take care of you regardless, because you're my patient and you're sitting in front of me and I want you to be better." That has worked a number of times. Examples… Patients that live for decades with meth use came to me on active drug now on therapy, stop using because they believe that this is actually getting them better. All it really takes is one or two times that they actually stop for a couple weeks and then they realize, "Hey, I'm breathing a lot better," and then they start using again and they go, "Oh my gosh, actually this is making me so much worse." I've had patients that were former meth addicts that have been in randomized clinical trials and were amazing participants in these trials, coming to every clinic visit on time, coming to every randomized clinical trial visit on time. I've had patients that have been active users of methamphetamine when I met them with horrible pulmonary hypertension that go on parenteral therapy after we get them to stop using. They manage parenteral therapy just as well, if not better than many other patients. I've had patients come and they're active meth users and we get them to stop and then we offer them therapy including parenteral, and for whatever reason they get worse and they're on maximum therapy including three, four drugs, and they continue to get worse and we offer them transplant and guess what? They do really well with transplant and they take excellent care of the organ after transplant. The option of looking somebody in the face and saying, "I'm not going to take care of you because you're using meth," is not an option in my mind. I think that we need to pair with patients to get them better, and the first step is to tell them that we are here for you and we're going to work with you to get you better, and these therapies can work. I think we should offer them to people to give them the chance to get better, because I've seen what it can do. There is a conception with substance abuse disorders that this is a certain subset of society that is not representative of many people out there, but actually it's everyone. It's soccer moms, it's grandparents. It's being used as a stimulant during romantic encounters to sort of heighten the enjoyment of the romantic encounters. It's being used in many different populations throughout all society. Again, it's incredibly addictive, so it's used a few times and then it becomes compulsive. If you go back to the historical aspect of it, meth was given to our soldiers in the World War to keep them active for battle readiness, right? That's where it came from. It was used as weight loss therapy, so people might be using some of it related to that. It was used for students that are trying to study for tests, so it can be anybody. I think that the idea that it's people that are coming from a part of the society that's constantly using drugs is sort of a fallacy here. It is the functioning members that are going to work every day just as well as everybody else. I think that you can't say it's one size fits all in terms of who the users are. For everybody that remembers their first few weeks and their first big job, it's always a bit nerve-racking. You walk in and you're hoping that everything's going to go well and you have a lot of imposter syndrome at the time. My first week, this young gentleman walks in, we did an echo on him and his heart was enormous. His right ventricle was three times the size of the left ventricle. I talked to him a little bit about methamphetamine use, and he told me that he had been using for decades. He was in his forties, and this was he'd been using since he was in high school. He was unable to walk across the room. He was syncopizing when he would walk. We talked about meth use. Since he was actively using, we elected to put him on two-drug oral initially and brought him back in a month to see that he was actually taking the two-drug oral and then put him on three-drug oral initially and told him consistently, "You need to stop." This was the approach with him, "If you stop using, I can actually use a lot more of the tools I have in my tool chest to get you better. If you keep using, these are basically the tools I have and you're on them. I'll keep you on these, but I can't do the other things." We don't give parenteral, in my practice, to active meth users. We will offer them three-drug oral, and now with the advent of a fourth pathway, we will offer them injectable therapy, but we don't offer continuous parenteral therapy just because of the risks of patients coming off of it and that causing sort of an acute RV failure. He was on three-drug oral. This was at the time we did not have sotatercept approved at the time. So, now he's on three drugs and he comes back and he tells me he's cutting back, but he's still pretty short of breath. He's no longer syncopizing, but he's having difficulty still walking and doing his activities of daily living. His kids are seeing him wither away. We sit and we have this 30-minute conversation about methamphetamine use, and sometimes in these conversations it just clicks. The light bulb turns on, and you could just see it in his eyes that in that moment it clicked. People that are trying to quit, they'll stop and they'll feel better and then they'll use again and then they'll feel worse. That was what was happening with him. He trusted me because I was the first person in his medical life that believed that there was something wrong with him and told him that his drug use was not something I cared about, but that I was going to get him better and we were going to work together. He believed me and it takes a lot from him. It's not me. I'm not the one that stopped. It's him and he stopped. Then, when he stopped and we showed a couple months of abstinence, we evaluated him for parenteral therapy and he was put on parenteral therapy. At the time that we put him on though his RV was still not functioning very well and he was having difficulty with the parenteral dosing and having difficulty with side effects, although we cranked it up, having difficulty still with his breathing being worse. So, we evaluated him for lung transplant and during the evaluation for lung transplant, he started developing issues with fluid retention despite our best efforts with therapies. Clearly, his RV was already failing at the time that I met him. We weren't going to make a whole lot of progress there. He ends up finishing his work up, but still having ongoing right ventricular failure and goes to the hospital and ends up getting transplanted. He's years out of his transplant, he's doing fantastic. He's running, spending time with his kids. I would call that a success, and this is somebody who if I had in that day when I saw him, brushed him off and said, "You're a meth user. This is from your meth use, come back when you've stopped using." I don't think he would've ever stopped because nobody bought into him as a person and nobody believed in him as a person, but here he is. My name is Nicholas Kolaitis 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 /episode/index/show/phawarepodcast/id/37522700

Episode 541 - Nicholas Kolaitis, MD 10/07/2025

Peter J. Leary, MD - phaware® interview 540 10/01/2025

Peter J. Leary, MD - phaware® interview 540 How Stigma Blocks Meth-PAH Treatment and How to Stop It Patients with methamphetamine-associated-PAH often arrive at clinics already carrying heavy shame. But shame shouldn’t be part of their medical history. In this compelling episode, Dr. Peter Leary explains how stigma—from both society and providers—can delay life-saving care. Learn how a change in mindset, language, and compassion can transform outcomes for a neglected and growing patient population. This Special Edition Episode Sponsored by: My name is Peter Leary. I direct the pulmonary hypertension program at the University of Washington and have been taking care of patients with pulmonary hypertension, in one form or another, for about 20 years. I’m really deeply focused both on the clinical care for patients with pulmonary hypertension, but also research that is important to patients with pulmonary hypertension. My mandate here today is really to think about and talk a little bit about methamphetamine-associated pulmonary arterial hypertension. I kind of want to think about this from two perspectives. I think those two perspectives are complementary, in most cases, but in some ways can also be in a little bit of conflict, and I hope to explain really what I mean by that. I'm going to start with thinking about methamphetamine-associated pulmonary hypertension from the lens of research. This is something that a lot of us are interested in. A lot of us are devoting time to. There's a multi-institution study that's funded by the NIH, that includes several of us, to try and understand a lot of those questions in methamphetamine-associated PAH that we don't really have a good handle on. We know now that methamphetamine causes PAH. We don't know a lot of things beyond that. We don't know how much methamphetamine it takes. We don't know whether or not there are characteristics of patients that make them more susceptible or more vulnerable to methamphetamine-associated PAH when they use methamphetamine. We don't know whether or not there are specific drugs that may be more helpful or less helpful in methamphetamine-associated PAH. I think all of these questions really are fueling a number of research efforts. I think that those are important. I think that those are valuable. I think that those are going to be the seeds of tools that we may use and think about in the future. I don't, at any point, want to lessen or minimize the importance of the research around methamphetamine PAH. In fact, we partnered not only in terms of this observational registry, we partnered with patients that have methamphetamine-associated PAH in our clinical trials of H2 blockers, of Valsartan, other trials that we're working on repurposing drugs, and have found really a lot of enthusiasm in that community and excitement about trying to make the life better and improve treatments for all patients with pulmonary arterial hypertension. I think that research is good, and that's important. The other side of this, though, is the clinical care. I always feel like I'm a doctor first. I would say that one of the worries is that we have increasingly, as a field, really had this kind of research focus on methamphetamine PAH. I think that that's good, like I said, but it runs the risk of making it seem methamphetamine-associated PAH may be somehow unusual, or a different group, or requires really special consideration. I think that the reality is, in our practice, at the University of Washington, we really view methamphetamine-associated PAH like we view all of the flavors of PAH. We think about methamphetamine much like a comorbidity, something that that patient is wrestling with, like diabetes, or obesity, or whatever else the pieces are on their individual repertoire that add complexity and require consideration as we're thinking about how to move forward. We don't think about methamphetamine PAH as some kind of weird, unusual, or distinct group that requires a totally different playbook. I feel like that is really, really, really important. The reason why I say that is that, unlike some of the other comorbidities that I've mentioned, there are many places throughout society and throughout the medical system that really stigmatize methamphetamine use or drug use of any kind. I think a lot of patients, when they get to us, are worried about being open and honest about the challenges that they've face. No one would ever be worried about telling us that they had diabetes, but people are very worried, oftentimes, about telling us that they've wrestled with methamphetamine in the past. I think that that's a shame, because it reflects this experience of having been treated differently, having been stigmatized, having been pushed to the side, or otherwise felt to be lesser other than because of their addiction. I think it limits a lot of aspects of care. To be honest, it also makes research more complicated. A lot of our questions are harder to answer when we don't have those kind of open honest dialogues with each other. So we really, in our practice, spend a lot of time trying to break down that sense of difference with methamphetamine-associated PAH, trying to remind our patients coming in the door that, hey, just because your PAH may have come from methamphetamine does not mean that we're going to treat you differently. We're not going to use different drugs. We're not going to put you through different tests. We're not going to have you do things different than we would for the broader PAH population. We may suggest, if you're still wrestling with addiction, that there are extra tools that can help you live a healthier and happier life by partnering with our friends in substance abuse, and thinking about other tools that are there. Just like if somebody had diabetes, we would partner with endocrinology to help manage that comorbidity. I think it's important to recognize that our mental model is not to think about methamphetamine PAH as something different. I've had this conversation with colleagues, and I think a lot of intelligent people have a lot of thoughts, and I think that it's okay for people to disagree. I think some programs really lean into the idea that part of a treatment plan has to be stopping methamphetamine. I think that that general feeling is rooted in the idea that we are fairly confident that if a patient can put methamphetamine in their rear view mirror, they are very likely to do better over time than somebody who keeps using methamphetamine, because methamphetamine is probably a little bit like throwing gasoline on the fire when you already have PAH. I don't disagree, even for a second, with the idea that it is a hugely important part of treatment to treat the addiction, to stay abstinent from methamphetamine, and use that as one of the tools to promote health. That said, we also know that methamphetamine addiction is incredibly difficult to treat. The medical community has tried a lot of solutions, and it can be very difficult to overcome this addiction. So, our personal approach is not to make PAH treatment dependent on staying abstinent from methamphetamine. We oftentimes feel as though we can use PAH treatment as a bridge to sobriety. If we can get people feeling good enough, doing well enough, and improving from the standpoint of their pulmonary hypertension and their heart failure, we oftentimes provide them enough time to beat their addiction. I can think of lots of my patients who have come in and have really been struggling with addiction, were not able to give it up in the early stages. But as we got them onto better regimens for PAH, as they were feeling less short of breath, as they were feeling kind of more optimistic about all those aspects, they then had the time, the effort, and the support, really, to help them get through their addiction, and then have been abstinent for many, many years after that. Obviously, this is a very personal journey. Like anything else in medicine, there are some successes and there are some failures. There are setbacks, and there are pieces that move forward, but I would say our personal philosophy on this clinical care, while we think it's important to separate out methamphetamine-associated PAH for research to see whether there are things that we need to be unique and doing differently, our mindset on the clinical side really is to say, nope, this is just like everyone else we take care of. Everyone we take care of has individual nuances, pieces that fit together in different ways, but that global approach to how we care for their PAH tends to be fairly similar regardless of what the source of their PAH is. The idea that there are lots of biases that come into addiction, both on the society side, the patient side, and the provider side are huge. It is not uncommon. We probably get one to two referrals a month from an outside pulmonologist or cardiologist that kind of asks us, almost apologetically, would you consider treating this patient with pulmonary arterial hypertension, oh, even though they're still using methamphetamine? I would say there is still a perception among a lot of providers that this is a group that we wouldn't provide these standard medical treatments for. I think that some of the arguments that I hear from friends and colleagues are that PAH care is expensive, and that part of the buy-in on the patient side really are to do all the things they possibly can to support their health, and that's part of going into a course of PAH treatment. My gentle pushback on that always is yes, but we don't have these contracts with the rest of our patients. If somebody comes in with diabetes or obesity, we don't require their hemoglobin A1C to be below a certain level before we'll treat with them with PAH. We don't require their obesity to be below a certain BMI before we will treat them for PAH. While I can understand the rationale and I can understand the motivation, and I think everyone's heart is in the right place and kind of recognizes the idea that successfully treating the addiction is as important as anything we do for the pulmonary arterial hypertension. I agree with all of that. The idea of restricting care in methamphetamine-associated PAH is something that I personally don't agree with. I don't think it's really fair kind of within the spectrum of comorbidities that we wrestle with with PAH. I still like the people that have these views. I understand where they're coming from, but that certainly is not our philosophy of what to do. We've seen a lot of patients who, when you go into it with that philosophy of supporting them medically through their addiction using all the tools we have, including treating their PAH, we find a lot of people are able to pull everything together and really build the building blocks of a healthy life that starts getting better and better as all of these pieces fall into place. The other thing that comes up, and I think this is also a real question, is, what about advanced therapies? I did much of my early training in a part of the country where heroin addiction was very common. There was rightly a lot of concern, at that time, about, can you send somebody out of the hospital with an IV in place? Can you put them on long-dwelling infusion therapies? Our experience with methamphetamine, for what it's worth, is very different. Very few people have substance use disorder with methamphetamine where they use it intravenously. So, those concerns, while I understand where they're coming from, in our experience, don't apply as often. We'll normally have a frank conversation with patients, if they're in a place where they are needing advanced therapy, perineural therapy, and we will say, do you think that you can manage a pump? Have you ever used your methamphetamine IV, or is this something that's always been inhaled? Then, like we would with any other treatment, we will work with them and think about all those personal factors to think about, hey, well, what is the next best step? Does this feel like something that you can do? Should we think about IV therapy or sub-Q therapy? Or now, maybe, should we think about sotatercept? Those pieces are present in all conversations, and so I don't want to overweight them for patients that have meth-PAH, even though I understand the hesitation that many physicians have when thinking about those conversations. In our experience, we have really seen what we would call kind of an explosion of new diagnoses of meth PAH. When I first started at the University of Washington, if you had asked me this question, I would've probably said 5% to 10% of our referrals were for a new diagnosis of meth-associated PAH. What we realized over time is 5% to 10% of those referrals were for people that fit our stereotype of what methamphetamine use disorder looked like. We had to recognize our own bias in that, and we started screening everyone. So, part of our standard new patient blood work is looking for autoimmune disease, and HIV, and hep C, and all these things we've always looked for, but also doing a tox screen on everybody who comes in, and getting really, really comfortable talking about methamphetamine and the challenges it is, and the fact that it may be treating undiagnosed ADHD or anxiety, or all these other things that people may have been self-medicating for with methamphetamine. So, building kind of comfort, both with the conversations around meth, as well as screening everyone, we quickly recognized that, no, it wasn't 5% to 10% of our new referrals for PAH had methamphetamine-association. It was probably closer to 35% to 40% of our new referrals. Over time, that number is probably only creeping up. It certainly is a very big fraction of the care that we provide and has been one of the factors that has made the number of patients that we serve get larger and larger over time. It's also a part that's easy to miss if you're not thinking about it for everybody who comes in. I think it takes a little while to be able to have that conversation and kind of do that testing, because of the stigma around addiction, that doesn't make people feel devalued or judged, regardless of whether or not they've ever used meth. I think on a forward-looking basis, I think that the research piece... and this is one of the wonderful things about research, is that we're going to go into it with some ideas. I would say some of those ideas are thinking about trying to understand how meth translates into pulmonary atrial hypertension. If we can understand that pathway... And Dr. Vinicio de Jesus Perez and the folks at Stanford have implicated CESI and some of these other pathways. If we can understand that, maybe we can have these personalized targets and precision targets that help pause, or even reverse the damage from methamphetamine, even in those folks that are still working on overcoming their addiction. Those questions, I think, are ones that I can conceptualize right now. But I think the reality is, as with anything in science, as we peel back the layers of the onion, we find other layers of the onion. So, I think that the research around methamphetamine-associated PAH may have benefit for folks that have methamphetamine-associated PAH. It may have benefit for the broader spectrum of PAH. For instance, I oftentimes feel like I see patients that have wrestled with methamphetamine that have really high pulmonary artery pressures, and that tells me two things. One, they may have severe disease in their blood vessels, but two, the right side of their heart really rose to meet that challenge. So, understanding what it is that may be adaptive for the right side of the heart may have kind of ripple effects of therapies that we can think about across the spectrum of PAH. As with so many areas in science, a rising tide raises all ships. The more we understand about the pulmonary vasculature and the right heart, and all the ways that it can go wrong and can go right, the better we're going to be able to do for all patients with PAH. I oftentimes tell patients with meth-PAH that are worried about whether or not we're going to treat them differently, hey, I love you the same as all my patients, and I really do. I really, really care deeply for all of my patients, and that is totally independent of what their pathway through life has been. That's probably kind of the meat and potatoes of how we approach it. I will say the final piece is really just to recognize, and I think that this is something that pulmonary hypertension docs in the West have known here for a long time, and as the methamphetamine epidemic has spread East, is really to recognize that I think a lot of people have a picture in their mind of what methamphetamine addiction looks like. That picture in their mind may be informed by what they've seen on TV. It may be informed by patients that they've seen in the hospital. It may be informed by family members or loved ones. But I would say the reality is that, now having taken care of lots of people who have wrestled with methamphetamine addiction, there is no single picture of what that looks like. I've seen patients that have wrestled with methamphetamine that are very high powered professionals and run what otherwise, to an outside perspective, would seem like a totally addiction free life. They're going to work, they're taking care of their families, they're helping their parents who are aging, they are kind of doing all of these pieces. I've seen patients that are wrestling with meth addiction that are working long hours and doing all those pieces. I have seen patients who are wrestling with meth addiction that have a lot of social vulnerability, who may have wrestled with homelessness. So, I think all of these things are true. I think one of the biggest things, getting back to that idea that we treat patients with methamphetamine-associated PAH like everyone else, is to realize that methamphetamine addiction doesn't define somebody. It doesn't tell us what they look like or how they behave, or what they're doing in society. So, that kind of recognition and observation, I think, is a key way to kind of break down some of those barriers that stand in the way of really caring for this patient population. Yes, some people do have social vulnerability, but that's true whether or not they're wrestling with methamphetamine or whether they're not wrestling with methamphetamine. I think when we find those pieces, homelessness, all those other pieces, we, as a society, need to do better with trying to figure out how we can support patients in the setting of these vulnerabilities, but I don't think that that's something that's unique or specific to methamphetamine-associated PAH. My name is Peter Leary, 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 /episode/index/show/phawarepodcast/id/37362685

Episode 540 - Peter J. Leary, MD 09/30/2025

Vinicio de Jesus Perez, MD - phaware® interview 539 09/24/2025

Vinicio de Jesus Perez, MD - phaware® interview 539 The Hidden Epidemic: Meth and PHMeth-Associated PAH is on the rise across the United States, yet many patients are not receiving the treatment they need. Dr. Vinicio de Jesus Perez discusses the shifting demographics of meth users, the challenges of treating addiction in cardiopulmonary care, and the need for compassionate, stigma-free screening. This Special Edition Episode Sponsored by: My name is Vinicio de Jesus Perez. I am a Professor of Medicine at Stanford University. I work with patients with pulmonary hypertension. In particular, I treat a sub-group of patients that have pulmonary hypertension that is caused by methamphetamine. Methamphetamine is an illicit drug that is being used over the last century in many ways. It was initially used as a stimulant for soldiers on both sides of the war, Americans, Japanese, Germans, as a way to keep the soldiers awake and ready for battle. Subsequent to the end of World War II, the drug found another life in the popular culture. It became a sought-after drug by artists, politicians, and other figures as a way to cope with pressures and indulge in creative bursts. Finally, the addictive potential of the drug was identified leading to its ban from pharmacies. Now, we have been seeing over the last three decades an epidemic of illicit drugs. Initially, in the '90s, we used to hear about cooks who will build meth labs in trailer parks, motels, and in their garages. But since then, this has now been replaced by a well-organized operation coming from Mexico where the drug is produced in large quantities, excellent quality, and shipped to the United States, where it enters through California. That's where I work. When I started my career back in 2004, myself and my partner, Dr. Roham Zamanian, who is the Director now of our PH program at Stanford. We were trainees and we were working with one senior physician, Dr. Ramona Doyle. Among the patients that were being referred to us as we were going through the drug history, and at that time the most common drug that we had linked PH with, of course, was weight loss agents like aminorex, fen-phen. Part of the history that we take will always include the use of both prescription and illicit substances. Time and time again, we heard that patients were either actively using meth or had used meth in the past. It was really hard not to see that there was a connection there. But whether it was causative or simply an association or risk factor, if you will, it was unclear. So, my career from that point as well as that of Roham, we began collecting information about these cases. In 2017, we were finally able to put our single center experience together. I think ours was not the first report of meth linked to PAH, but I think it comprehensively took patients who were well phenotyped. We had over 200 patients. We also had pathological samples and ultimately also outcomes. What we ended up finding was astounding. We found a very strong link between methamphetamine and pulmonary hypertension. But most importantly, we found that the clinical progression and outcome of these patients despite therapy was worse compared to the idiopathic patients. What that signals is that these patients have a different clinical syndrome compared to other forms of pulmonary hypertension. What that means for me as a clinician is that these are patients that require a different level of care than the one that we offer our other patients. In addition, this is a patient population, particularly those that are actively using that need to be convinced and assisted. This is not a straightforward task, something that we as cardiopulmonologists are not trained to do. So multidisciplinary care has become the mainstay of management of Meth-PAH, work with social workers, cardiologists, if patients have other complications associated with meth like dilated cardiomyopathy, coronary artery disease, arrhythmias, et cetera, as well as addiction specialists. Of course, it's critical also to look at random urine testing, because this is a very complex addiction. It's not unusual for patients to stop taking methamphetamine and then going back to it. It can be a few weeks, it can be a few months, it can be a few years. So even if a patient stops using the drug, we will still continue to do these random checks. It's important because the use of methamphetamine can trigger some acute effects on the pulmonary circulation that may translate into worsening of the condition. In the case of a patient with pulmonary hypertension, if I see somebody who is actually developing a progression, their functional classes getting worse or they're just losing steam in their six-minute walk, one thing is well, their disease is progressing. Or it could be that the acute use of methamphetamine again is triggering a worsening, which if the patient stops the medication it can be improved. These are some of the complexities that we deal with cases. I think as a group we have learned over decades of treating a large number of these patients how to approach it. But of course, as the methamphetamine epidemic has evolved, we have now seen that in an unpublished study we currently have that has looked at a claims database between 2018 and 2022, looking at the trends in the diagnosis and treatment of Meth-PAH across the United States. What we have found is that the map has been evolved during that period of time where you see very clearly that in 2018, most of the cases will be in the West, Southwest and the South of the United States. But with time you're seeing a clear trend in increase of diagnosed cases in the East and Southeast portions of the United States. What our study finds out, which I think is very concerning and certainly triggers a call to action, is that if you look at the treatment of Meth-PAH patients, not surprisingly, if you are a patient with Meth-PAH in the West, Southwest and South of the United States, you are getting treated for your condition. But as you look into the East, the Southeast, if you're a patient with Meth, you're not getting treated maybe. That is a cause of concern, because what we're seeing is that there's a clear trend of an increase in diagnosed cases. But what we're also seeing is that even those cases that are diagnosed are not getting the appropriate therapy. That's signals a disparity in the care of pulmonary hypertension for this particular phenotype. I think for us as a community, this is important. Why this is the case, there's a lot to speculate. We can speculate that there's a lack of awareness, there's a lack of screening. There may be bias in terms of how patients respond to recommendations about care, or how physicians feel that a patient may be compliant with care. Many things we can speculate, but ultimately, I do feel that the data clearly points to an area that needs to be properly addressed because as I mentioned previously, these patients do have a much worse prognosis compared to idiopathic even when treated appropriately. I do feel like as a community we need to be aware and we need to advocate for these patients. I think the first step is you need to ask point-blank about a history of active or previous use of methamphetamine. This is something that should be standard in the history taking of any patient with pulmonary hypertension. As we're looking to rule out potential causes, asking about former or active meth use should be a standard question. What we do in our center is that we routinely screen any new referral. As part of their workup, we screen them for drugs of abuse, not just methamphetamine but all drugs of abuse. We have been surprised to see that few patients that you will not have thought will be meth users. When we come back to discuss the results, they do admit to the use. We're talking about patients who are functional, people who have families, work. They're otherwise standout citizens, so not what you will think will be the typical phenotype of a meth user that you will see in TV shows or in the newspaper. What that tells you is that you can really not trust your own bias regarding what a meth PH patient looks like. So, I will argue if you have a patient that tells you that they've used meth in the distant past or actively using, you should definitely consider methamphetamine use. You should screen that patient and you should also ask that patient, even those who are not active users, you have to ask if they live around people who are actively using. So, that's where you're having a social worker to take a social history, where that patient lives, what connections they have to the community. These things are also important because part of what drives the success of an intervention here is knowing where the patient lives and where they work when they're not in clinic. They're responding to a more complex environment filled with pressures that can change on a day-by-day basis. These can be triggers that can lead to a patient to have a recurrence in their use or even be exposed. I think also it's important to really involve these patients early on with addiction services, especially those patients that are actively using. I think ultimately the most important thing that I think is not being done right now, especially in areas where there may not be large pockets of pulmonary hypertension patients or we're really not asking our patients straight up. I do feel that the screening of patients is critical. I don't know if at this point in time we are ready to say that we should absolutely screen everybody everywhere. I can see this being a controversial intervention, but I do feel that if you're in a geographic area where based on our map right now you're seeing that the number of meth patients is rising in your community, I think you can certainly be more aggressive and justify having standard screening as part of your battery workup. This patient population is very heterogeneous. Yes, you get the traditional patient who's unemployed, who's homeless, who hangs out with the wrong crowd, they have a criminal record, kind of like what you will pigeonhole a typical meth patients. Interestingly enough, over the years we've seen an evolution where that phenotype is no longer the dominant phenotype of meth PH patients. What we're seeing now is a transition to an older patient population. These are patients that are using methamphetamine as a way to cope with work, to cope with fatigue, to cope with depression, to cope with ADHD. This is where having that patient be evaluated and treated by a psychiatrist, not just an addition specialist but even perhaps also a psychiatrist to look for an undiagnosed psychiatric disorder becomes important. I will say right now that the typical meth PH patient that we are seeing is somebody who is using meth as part of their work-life routine in order to maintain a at work, and to cope with the pressures of life in a way that will allow them to maintain a socioeconomic status. At the same time, it hints at underlying psychiatric illnesses that are not being appropriately treated. So, that adds another layer of complexity to the management whereby it's important to help that patient not just with their pulmonary hypertension or their addiction, but also with finding proper treatment for their psychiatric disorders. The most important thing that I think is not being done which has absolutely no extra cost, is to ask the patient in a non-judgmental, non-threatening way about the use of methamphetamine. It's not just asking. It is asking in a way that the patient doesn't feel like they're being grilled, that they're being judged or they're being placed in an uncomfortable position. For me, what I'm asking patients, same as their sexual history, these are very sensitive questions. Yes, you're a doctor and yes you are in a private room, but patients can become very defensive. Again, I think that's very fair, because you are getting into a part of their life that they're not necessarily happy sharing with anyone, especially somebody that they're meeting for the very first time. They already know that there's already a society bias that goes against whatever these behaviors that they have. So, of course, they're going to be protective, they're going to be suspicious. I think it's really important for the physician to ask this question in a way where the patient feels like they're not being judged. This is part of what needs to happen in order to push forward with the right diagnosis. So, the way I normally do this is I ask the patient about drug use. I like to go down a list so I don't just jump in and say, “Have you used meth?” I start off with like, "Listen, there's a set of drugs that have been associated with pulmonary hypertension, and I'm going to ask that you give me an honest response whether it's being active or you did it years ago. I just want you to be aware that I'm not passing judgment, nor will I share this with any authorities. This stays between us. It's going to be part of the record, but I only ask because this is part of a workup that we use on every patient." With that, I go through each drugs like have you used fenfluramine? Then, have you use cocaine and have you use meth? I'm looking at the body language of patients during that meeting. Once you get to illicit drugs, by now I've done this for more than 20 years, so there is already a body language that I can read or a way that the patients take that question. Again, it's important to reassure them like, "Listen, I'm not here to judge. I'm here to help and all I need is to know, because let me tell you, this is why." Once you put it on the table, you present the patient with the data like, this is what we know about the drugs. Our group has looked at that, so that's why I am asking. So let me ask you again. Surprisingly, I think the more the patient is reassured and sharing with the patient the data that supports the question and why it's important, allows the patient to be open. I think this is also crucial because moving forward, if I'm talking with a patient who's an active meth user, I do break down with them what that will imply in terms of their management. So, the patients will receive the care they deserve. However, we're very clear in terms of what we cannot offer like intravenous therapies. We can definitely offer subcutaneous therapies, we can offer sotatercept. So, we are very aggressive. We are also very open to the patients that we really need them to be honest about whether or not they're using. They may have stopped using but they may end up using again. We're very clear that we understand that addiction is a very difficult disease to tackle. If a patient, once again, falls and starts using again, we just want to know and we want to take that into account and again, help the patient. I think where the biggest challenge come is when patients stop coming to appointments or they stop taking medications, they miss their shipments or they're not responding, because at that point in time it puts us in a very difficult position as we're responsible for these patients. We're responsible for providing them with these prescriptions and not taking some of these medications can pose a real risk where the patients can worsen acutely. And, suddenly, they may end up in cardiogenic shock in some cases, especially if they are actively using. These are the most challenging cases because you know that these are the people that as a physician, you are responsible to treat them. But when the patient is not complying, then you have to really draw a hard line and set some expectations in order to be able to help that patient. This is teamwork. One of our colleagues, Dr. Clapham talked about how the management of pulmonary hypertension associated with meth has to be multidisciplinary. I will argue that a big part of that approach has to be the patient itself. They need to know that they're part of the operation and their willingness to comply is essential for the success of that intervention. My name is Vinicio de Jesus Perez, 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 /episode/index/show/phawarepodcast/id/36534715

Episode 539 - Vinicio de Jesus Perez, MD 09/23/2025

John Kingrey, MD - phaware® interview 538 09/17/2025

John Kingrey, MD - phaware® interview 538 Dr. John Kingrey exposes the rising crisis of meth-associated pulmonary hypertension, a condition spreading fast across the U.S. He challenges misconceptions, highlights urgent research, and stresses the need for compassion in treating both PAH and addiction. This Special Edition Episode Sponsored by: My name's John Kingrey. I live in Oklahoma City. I'm a pulmonologist. I've been doing pulmonary hypertension for 10 years now. I came to Oklahoma with my wife and three kids to try to put Oklahoma in the middle of the pulmonary hypertension community. Since our time there, we've been able to build a pulmonary hypertension center. We have done that with the strength of an amazing team. Now, fortunately, I also have a partner who's helping me with that, Dr. Nick Shelburne. We're growing and we're trying to do good things in Oklahoma. I'm excited today to talk to you about a subtype of pulmonary arterial hypertension that is incredibly common and is getting more common by the day, which is pulmonary arterial hypertension due to methamphetamine use. I recently had an opportunity to present some new research at the in Boston. I’m excited to be a part of research that is really kind of peeling back the covers of a problem that's been a known for a while, but is really now becoming mainstream and center court for a lot of us taking care of PH across the country. Methamphetamine use is actually a global problem. Really since the turn of the century, it has just continued to grow in popularity among recreational drug users. We do see a heavy, heavy burden of methamphetamine use in the Western part of the United States historically, but part of the research that we have done collaborating with partners in industry have really demonstrated that it is not a Western phenomenon anymore. I think it's important to understand that it is a global problem because some people think that it just doesn't affect them depending on where they live. Certainly there are areas of the country and areas of the world where it's not nearly as prevalent. I still have colleagues on the East Coast who do a lot of pulmonary hypertension who have never seen a case of methamphetamine use pulmonary hypertension. When I moved to Oklahoma in 2014, I also had never seen a case of methamphetamine-induced pulmonary hypertension, because I trained in Ohio and we had other recreational drug problems, but meth was not the most prevalent and certainly we weren't seeing PH from that. But in my 10 years in being in Oklahoma, I have many colleagues across the country, those who are seeing meth pulmonary hypertension, though they predominantly are now currently in the Western part of the United States, it is something that's infiltrating everywhere. When I first moved to Oklahoma, I would occasionally see someone that I suspected, and so we would drug screen, and occasionally we would identify a patient that was a meth user, but methamphetamine use has been exponentially on the rise, and our diagnoses of methamphetamine-induced pulmonary hypertension have increased dramatically to the point that now, by far, our most prevalent incident population of PAH diagnoses is methamphetamine-induced. When I say the predominance, that also means, in our case, greater than 50% of all of our new diagnoses of PAH are from methamphetamine use at our center. We are taking care of approximately 500 patients with PAH or chronic thromboembolic pulmonary hypertension, most of them PAH, so we're seeing a lot of people. Probably the biggest reason for the exponential rise in the volume that we're doing is undoubtedly from methamphetamine use. Like most rare disease, we've long-struggled with the challenge of trying to identify patients sooner. We have a little bit of a different challenge in the meth population. There are some stereotypes out there, some of which are true, and some of which are not true. Typically, when people are thinking about a population that abuses recreational drugs, they think of people typically who are socioeconomically disadvantaged, and that is true. Not everybody, though. You cannot profile these people just by the way they look or walk in the room, because sometimes you'll be surprised. Because of the socioeconomic disadvantage, larger populations have state-sponsored insurance. We actually have through our claims data, which is a lot of the information that I presented at the Team PHenomenal Hope Conference is based off of that because we don't have randomized control trials in these patients, but what we know is that a larger percentage of meth PAH patients present in hospital settings in the emergency room settings because they have not tended to healthcare. They're not seeing a primary care physician. They're not doctoring frequently, and so when they finally present, it's because they're so sick that someone around them said, "You got to go," or the patients literally can't breathe or can't stand or can't walk. Many of these patients, and we do have data on this that substantiates that they're sicker when they present, they have bigger right ventricles, they have poorer hemodynamics, and are really hurting in a bad way. It's hard enough when you're trying to treat this disease that's bad and you feel like you're behind the eight-ball, but these people are presenting really, really late, large in part because they're just not seeking healthcare until they're really in dire straits. I told you that there are some stereotypes that are true and some that aren't true. Many people believe that once you identify these patients, let's say you do meet them in the emergency room or we meet a lot of them in clinic, it's not like they all present in the hospital, that you're going to see them once and you may never see them again, because maybe they finally came to you because they were so sick or someone in their family said, "You've got to go," or whatever. But it's not bearing out the prototypical way that a lot of people think, meaning that these patients do come back and see us. Now, we have recidivism rates, we have dropouts just like any population of PAH, and is it a little bit higher in this population? Yes, but anybody who's socioeconomically disadvantaged, whether it be from substance abuse or just maybe the fact that they are otherwise disadvantaged, they have similar rates of dropout, no-shows, and those sorts of things, so we do see them. But this is why, okay, and this is the part that's really critical, is that these people are coming to us with two life-threatening problems. The first, which is why they're there, is because they have pulmonary hypertension. The second is their addiction. We have to address both of those things in some way, shape, or form. Now, most people who are treating pulmonary hypertension are not addiction experts, but you have to be facile enough to understand where these people are coming from and what addiction in general means. What it means for them is that there's very few people in their lives that they can trust because they're used to being abused both emotionally, physically, sometimes sexually. They are patients largely who have very poor self-esteem and very poor confidence. The single most important thing that we have as our responsibility as a physician, particularly to this population, is to let them know that we care and that we love them, that we love them as a patient just like anybody else, that we're going to do whatever we need to do to help them irrespective of what they've done in the past. Do many of these people get bounced around? Yeah, some of them do because some of them get treated, unfortunately, by the healthcare system as people who aren't worth our time or people who "have done it to themselves>" Somehow that means that they're supposed to pay a different price. In actuality, these are the people who need us the most, more than anybody else. They need us to be friendly to them. They need us to be a friend to them in certain times and to their family, and they have to know that we've got their back. Most of the patients who present, and I think I'm speaking on behalf of all of my colleagues who deal with a large population of methamphetamine users, is that almost all of them are coming to us actively using. We do have some patients on occasion who are reformed and have gone through rehab and have put it in the rearview mirror. Even though they always have addiction, they're just not acting on it. But those patients who come to us, the majority of them are still using, we absolutely treat them. We treat them just like an idiopathic PAH patient showing up to us who needs treatment. The reason is because they feel physically terrible. They're short of breath, they're swollen, they're passing out, they're exhausted. They can't do anything because they have really sick right hearts and they're in bad heart failure. If you consider how, any of the people listening to this, how you feel when you have the flu or if you have just undergone a surgery, are you at the top of your game? No, because when you feel physically terrible, it's really hard to feel mentally good, emotionally good, and because it weighs you down. It's why we're irritable when we're sick. That's exactly what these people have, except it never goes away. So, we have to start helping their heart, both their physical heart and their emotional heart, but we have to help them with therapies. We start them on drug therapies, and are there certain considerations that you have to have with this population, things that you need to be aware of that can sometimes change the treatments that you use? Absolutely, but that's personalized medicine that we would do for anybody. But large in part, just like anybody else, we're going to treat them with a individualized treatment program that we feel is going to help them be most successful. The fen-phen comparison, there are some nice parallels there, but there are also some differences, but the mechanism of action of how fen-phen led to pulmonary arterial hypertension is very similar to methamphetamines, because it's going down the same pathway, except one was approved by the FDA, the other one obviously isn't. There was a big epidemic after fen-phen came out and quickly was taken off the market, and obviously unfortunately, we can't take methamphetamines off the market. I wasn't around in the late 1990s when the fen-phen epidemic occurred, but this much I know: they weren't being stereotyped as bad people. They were being stereotyped as people who were looking for a way to lose weight and got victimized, so to speak, by a drug that hit the market that didn't have enough data to know, at the time of its launch, that it was going to lead to both pulmonary hypertension, the mitral valve problems, and the other issues that came about. I can only imagine those people were embraced and treated accordingly, except the big difference there, as well ,is we didn't have any treatment in the late '90s, except for IV epoprostenol, which was the very late '90s. A lot of people didn't have access to that, so many of these fen-phen patients died. We do have a problem with colleagues within pulmonary hypertension and outside of pulmonary hypertension. One of the things that we have to continue to stress to people is that these people are just as valuable as anybody else, and they deserve our attention just as much as anybody else. I have people, even within my own healthcare institution, and I know that other, my colleagues, have dealt with similar biases, is I've been approached and said, "Well, what do you do with these people? I mean, I know they got a big bad right heart, but you can't treat them. I mean, they're doing meth." It's like, "Well, hold on a second. Why can't we treat them? Because they have an addiction?" In the pulmonology world, we see people every day who have smoked their whole life and have developed, as a result of that, emphysema, lung cancer, vascular disease, all the things, right? Somehow it's different because cigarettes are legal? I don't know a pulmonologist on earth who says, "I'm not going to give you any inhalers until you stop smoking," because those pulmonologists are out of a job if that's their stance. No, it's just that somehow as a society, we valued being addicted to cigarettes is somehow being on a different level than being addicted to street drugs. I maintain that there is no difference, especially if we're going to take the stance that people have done this to themselves. Well, oh my goodness, our largest burden of healthcare resources is devoted to people who have done things to themselves. Who hasn't? Whether it's smoking or obesity or having out-of-control cholesterol, because you like to eat hamburgers or whatever it is. We've done these things to ourselves except we have a street drug that is often associated with people who are dirty, who are somehow sub-societal, and then they have to pay the price for being addicted and then for getting a disease from their addiction. They are a different phenotype in very many ways. We know that they have a different hemodynamic phenotype, they're sicker. They have a different echocardiographic phenotype. Just in that, it still looks like pulmonary arterial hypertension, but often, some of the biggest right ventricles and the worst-functioning ventricles that we see are from patients who are methamphetamine abusers, but there is also a very, very different emotional and societal phenotype that has to be acknowledged with these people, this very different population. Because again, you're dealing with not only their disease, but also an addiction. None of them are proud of it. Just like most people with addiction, they're not proud of it, and they would love to get out of it. There are many, many places in Oklahoma, certainly many of my colleagues who deal with a high methamphetamine-induced PAH population see that these people come in and are disadvantaged. They want a way out, but the amount of times that I've heard a patient say, "Everywhere I go, meth is there." They live in a world where they can't get out of it. You have to be sympathetic towards that, and you have to recognize that that could have been me, right? It could be you, if you were raised in an environment like they have. I talk a lot about the glass house in this population. We all live in glass houses, so before you pick up that rock and make a judgment call on somebody, you better look in the mirror, because we all have things that we've done to ourselves. We all have things that we wish that we could erase or that we could have a redo on. I'm pretty confident that a hundred percent of my methamphetamine-induced patients wish that they would've never picked it up. We are learning a lot from what most would consider the poorest quality of data, which is claims data, but there are still things to be learned from that, because we can get general trends. In the process, we are raising awareness. What we know is that over a five-year period from 2018 to 2022, methamphetamine use is going up across the country. It's not just in the Western United States. Some of the highest percentage increases in the country are east of the Mississippi. So not surprisingly, the number of diagnoses of methamphetamine-induced pulmonary hypertension are also going up to some degree. Now, it's not completely linear because you have to identify it and you have to attribute it to methamphetamine use. I know that there are probably thousands of patients in this country who are being treated as idiopathic pulmonary hypertension simply because they never had a urine drug screen to tip off their provider. Even though I'm in the thick of it, I get duped sometimes too. As a practice, we send a urine drug screen on everybody at the time of diagnosis, but sometimes it gets missed. Sometimes the patient didn't go and get their labs or whatever, and then you kind of get into the thick of it and you think you have an idiopath or you think you have some other form of pulmonary arterial hypertension, and then at some point, you'll find out, which I think is important to recognize too. For the most part, patients are very honest. They feel horrible, and they realize that they have nothing to gain. If you set everything aside, look them in the eye, let them know that you care, they're going to tell you the truth almost always because they want to be on the same playing field as you. They want to help. My general message from the research that we've done, and some of which we presented in the conference in Boston, is that methamphetamine use is a serious problem and it is on the rise. We have well over 5,000 patients that were identified in our claims data work that were diagnosed with methamphetamine-associated pulmonary hypertension. There were more than 2,000 of them who weren't being treated, so why are they not being treated? I think that's an important question that we have to ask. I've got my ideas. I think that some of them can be directly tied to our biases and our judgmental approaches. Other people, it may just be because they presented and then they don't come back, right? There's those reasons too, but we have a lot of work to do. If you're going to be in the pulmonary hypertension space going forward, you better understand methamphetamines and you better understand drug addiction because it's here and it's not going to go anywhere for a really long time. My name is Dr. John Kingrey, and I'm aware that my patients are rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: . #MA-PAH /episode/index/show/phawarepodcast/id/35845115

Episode 538 - John Kingrey, MD 09/16/2025

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

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