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

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

Kari Gillmeyer, MD, MSC - phaware® interview 537 09/10/2025

Kari Gillmeyer, MD, MSC - phaware® interview 537 Delays in diagnosing and treating pulmonary hypertension (PH) can have life-altering consequences. In this episode, Dr. Kari Gillmeyer, discusses her groundbreaking study on PH care networks. She explores why so many patients experience delays, how geography and provider access play a role, and what can be done to improve timeliness of care. This Special Edition Episode Sponsored by: My name is Kari Gillmeyer. I am a pulmonary and critical care physician and a clinical and health services researcher at Boston University and VA Boston. I've been doing pulmonary hypertension research pretty much since the start of my research career, probably six or seven years ago. I am excited to be here today to tell you about a study that we did on exploring care networks for pulmonary hypertension and some of the implications that our findings may have on improving timeliness of pulmonary hypertension care. Our motivation for this study really came from an awareness and an appreciation of the substantial documented gaps in both the timeliness and the quality of pulmonary hypertension care. For example, we know that the time from symptom onset to diagnosis for folks with pulmonary hypertension, specifically pulmonary arterial hypertension, is two to four years. For those with chronic thromboembolic pulmonary hypertension, it's more than a year. Unfortunately, over the last two decades or so, those delays in making a diagnosis for pulmonary hypertension have not improved, despite the efforts, including the efforts of people like phaware® and this podcast to improve the awareness of the disease. We know that these delays in making the diagnosis for pulmonary hypertension have significant long-term impacts and outcomes for patients. The delay to diagnosis is certainly not improving, and we would expect to see improvements over time given all the efforts to improve the awareness of the disease, all the efforts in the research realm to try to improve our understanding of the disease, and yet it's not improving. I think that we can kind of hypothesize what some of these reasons are for the delay. I think we know some of them and we can hypothesize other reasons. For example, we know that patients often present with very nonspecific symptoms, especially earlier in their disease. They may come in feeling a little short of breath. They may have some lightheadedness. These types of symptoms can be seen in a whole host of other diseases. I think that's one reason why this disease gets missed. Then, I think some of the other reasons include just a lack of awareness and a lack of comfort with managing this disease, particularly among general practitioners and primary care providers. But I would say even among some specialists, cardiologists, and pulmonologists who don't see folks with pulmonary hypertension that often. A lot of those factors, a lot of those, what we call social determinants of health broadly, certainly have an impact on delays in care. Not this specific study, but in parallel to this work, we're looking at trying to identify what those specific factors are. For example, we know that patients who are on Medicaid experience greater delays in getting into care. That's been shown in other disease processes as well. These folks have fewer options for who they can receive care from. They have greater barriers to overcome from an insurance perspective. That's just one example, but certainly a lot of these issues are at play here. Recognizing these significant delays in care and recognizing that we don't really have a full grasp on why these are happening, we sought to conduct a population level study to try to evaluate care networks for pulmonary hypertension, to try to understand where patients are receiving their pulmonary hypertension care, from whom they're receiving their pulmonary hypertension care, meaning what type of specialists are providing the care, and then to look at kind of referrals for patterns of care as sort of a first step to understanding where some of these gaps in getting patients into expert care might lie. We used a database called the all-payer claims database, which as the name implies, includes claims data for all payers, meaning commercial payers, Medicaid, Medicare. This is a really strong and powerful type of database where we can kind of look at the entire population and see where patients across the entire population are receiving their care. Importantly, this data set also includes something called the National Provider Identification Number from the provider standpoint, so we can connect this claims database with provider data so that we can understand very specifically what types of providers are caring for patients with pulmonary hypertension. Then, using that database, we used a tool called Social Network Analysis, which for those who have not heard of this term before, is basically a way to look at relationships within a network. A network can be made up of any type of people. In this case, we're interested in looking at providers in the network. Then, the relationships between those providers are patients that they share. For example, if provider A saw a patient with pulmonary hypertension and provider B also saw that same patient with pulmonary hypertension, those two providers would be connected in the network. By seeing what providers are sharing patients, it allows us to kind of understand where these referral patterns might be happening, and importantly, where there might be gaps, where people who are seeing, for example, a primary care doctor who aren't getting in to see a specialist or who aren't getting in to see a PH expert, where those gaps might be happening across the population level. We found about 9,000 patients in our database that have pulmonary hypertension, and we found about 5,000 providers who take care of those patients. We created these networks from these patients and providers. Some of the main things that we found were that in the state of Massachusetts, which is where this database came from, we found only 35 pulmonary hypertension experts. That represents one pulmonary hypertension expert to every 250 patients with pulmonary hypertension. This data set only captured about four years of data. so we think that that number is significantly higher, meaning that there are far more patients with pulmonary hypertension that we identified. So, we think that ratio is even higher. Obviously, pulmonary hypertension experts are a limited resource. There are far too few of them to be providing care for patients with pulmonary hypertension. That's one of the main findings that came out of this work. Most patients did see a specialist for their pulmonary hypertension, meaning that they saw either a cardiologist or a pulmonologist at least once for their pulmonary hypertension. However, a number of patients, nearly a quarter of patients, only saw a primary care provider and had no contact whatsoever with any relevant specialists. No cardiology visits, no pulmonary visits. Importantly, among a subset of patients who are on PH therapies, in this case, we define these as pulmonary vasodilators, about 8% of patients never saw a cardiologist, never saw a pulmonologist, were only cared for by primary care providers who we know don't have the knowledge and the expertise to be taking care of this very complicated and very complex disease. Then, in terms of patients who saw a pulmonary hypertension expert over the study period, only about 8% of patients in the entire network saw a PH expert, which is not surprising given how few PH experts there are. But even among patients who are on therapies, even among patients who are on pulmonary vasodilators, fewer than half of those patients saw a pulmonary hypertension expert during our study period. In summary, I think a lot of these patients are being cared for by folks who don't have the expertise and the knowledge necessarily to provide the really complicated care that is required to care for these patients. We already talked about how pulmonary hypertension experts are a limited resource. I think there definitely needs to be really concerted efforts in the PH community to try to build that capacity. I think that needs to happen probably on multiple levels. We need to improve the pipeline of providers going into this specialty. So, increasing the number of training programs, for example, that can train pulmonary hypertension experts. I think there also needs to be a building up of the PH expert centers to be able to accommodate more patients. So, that might be building more infrastructure, bringing in support staff who can help PH providers see more patients. At the end of the day, a PH expert only has so many hours in their clinic that they can see patients. We need to think a little bit more broadly about how we can provide high quality care to these patients, recognizing that PH experts are a limited resource and they can't see everybody. So, does that mean creating some sort of hub and spoke type model where PH expert centers can help support maybe the cardiologists and the pulmonologists who don't have specific PH expertise in the community to help support them care for these patients in kind of a collaborative fashion? Maybe some of this is patient preference with them wanting to stay kind of with their local provider, but I think more of it is just an awareness on the provider level that they ought to be referring these patients. I think we need to be disseminating clearer referral guidelines for folks in the community. For folks, meaning providers out in the community who are not within an expert center, I think we need broader efforts to try to disseminate to these folks who don't see pulmonary hypertension as often, what patients they need to be referring, and importantly, when they need to be referring. We also know from prior work that patients, when they ultimately end up seeing a PH expert in one of these PH care centers, they often have very advanced disease at the time. They're often like what we call a functional class III or IV, meaning that they are very symptomatic, they're very short of breath. We know that patients, when they present late like that, it affects their long-term outcomes. I think we need do a better job of making sure that folks out in the community know when and how and for what patients they need to be referring into expert care. Some of the other findings that we found from this specific study looking at care networks was we found significant kind of geographic patterns to referral patterns. When we looked visually at the networks of providers taking care of patients with pulmonary hypertension, and this tool that we use, Social Network Analysis, allows us to visually graph out what these networks look like. We found that patient sharing between providers tended to land pretty squarely within geographic regions of the state. So, providers within the Greater Boston area, for example, primary care providers, cardiologists, PH experts, they all shared patients really readily. But then when you look out to the western part of the state, Western Massachusetts, which is much more rural, there was far fewer sharing of patients across providers across these geographic boundaries. We saw that visually in the network graphs that we created and we also saw that sort of quantitatively in some of the analytic work that we did. You can imagine that Massachusetts is a fairly small state. There are many PH expert centers or many PH experts. Most patients in our sample actually lived within 25 miles of their nearest PH center. This is a state that geographically is probably less challenging for patients to actually get to a PH expert or get to a PH care center. You can imagine that there are a number of states across the country, a lot of rural areas across the country where there are zero PH centers or zero PH experts. So, you can imagine that some of these gaps in referrals to PH experts are probably even much more pronounced in states across the country that are more rural, in states that don't have as many PH experts and PH care centers. I think the importance of getting patients into expert care cannot be understated. Not only do they get the expertise that comes with being seen by somebody who has specific training and experience in pulmonary hypertension, but they get all of the resources that come along with it. They get access to pharmacists, for example, who would have more knowledge about pulmonary hypertension therapies and can help these patients navigate the very complex issues with dealing with specialty care pharmacies, with dealing with insurance companies. They get access to clinical trials, they get access to other research opportunities. They get better connection to patient associations and other patient support groups where community providers, primary care providers and other specialists who don't see PH may not just have the awareness of all of these resources that are out there that are available to patients. So, I think patients who don't ultimately make it into a PH care center miss out on this whole host of opportunities, not just the opportunities to see an expert and to receive higher quality care. This is a hard problem to fix. Obviously, we can't increase the number of PH providers and the PH care centers overnight. That will take time. It'll take effort on the provider level, on healthcare center levels, on health policy levels to address some of these gaps. I think we'll probably need a multifaceted approach to try to improve the access to expert pulmonary hypertension care and decrease some of these delays in care that we're seeing. This includes investing in the pipeline of pulmonary hypertension experts, as we've already talked about. This might include expanding use of technology, expanding use of telehealth, for example, to try to reach some of these folks who are a little more remote, who may have geographic or other socioeconomic barriers to getting in to a PH expert. This may include developing AI tools, using the electronic health record within some of these centers outside of PH care centers to identify patients who are appropriate for referral and maybe flag for the provider to say, "Hey, this patient meets all the criteria for referral. You ought to think about getting them in." This might include building telementoring models so that PH experts can be mentoring providers out in the community to make sure that they're providing the highest quality of care. I think that we need to think outside the box. I think it's going to take a lot of creative efforts to try to tackle this really important problem of improving access and timeliness to pulmonary hypertension care. My name is Dr. Kari Gillmeyer, 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/35538390

Episode 537 - Kari Gillmeyer, MD, MSC 09/09/2025

Eric Borstein - phaware® interview 536 09/03/2025

Eric Borstein - phaware® interview 536 A 200 Mile Journey from LA to San Diego to Raise Awareness for PH When Eric Borstein collapsed in his bedroom in 2020, doctors gave him just months to live. Instead, he fought back with walking, mental health, and the support of his community. Now, he’s leading a movement, raising hundreds of thousands for pulmonary hypertension research, and walking 200 miles down the California coast to prove hope has no limits. Learn more about the September 7, 2025 Steve Van Wormer: Welcome to I'm Aware That I'm Rare: phaware podcast. I'm Steve Van Wormer from Phaware Global Association. I'm here with Eric Borstein, a patient who you may recognize from the podcast who did an episode with us last year. Eric lives in Los Angeles. He's a pulmonary arterial hypertension patient diagnosed in 2020, and he's got some exciting updates to what has happened in his life since the last time he appeared on the show over a year ago. Eric, thank you so much for being here today. Eric Borstein: You're welcome. Thank you, Steve, and thank you for having me back. Steve Van Wormer: Last year you did a walk called, " Basically, you walked from Santa Monica to San Diego over the course of a week. Before we get into that, I wanted to see if you could give viewers the overview of what happened to you back in 2020 that led you to that first Where is EB walk. Eric Borstein: So, September 21st, 2020, I collapsed in the bedroom and almost died, was taken to St. John's Hospital here in Santa Monica where I was stabilized and was diagnosed with Class 4 pulmonary arterial hypertension. I had collapsed from severe right ventricular heart failure. After a few days and being stabilized, I was transported to Cedars-Sinai and I spent a total of 16 days in the ICU. (Doctors) told my wife I had three to six months to live. I didn't know any of this. I had made a decision very early on that I was going to beat this disease, and I didn't know what that meant. But I looked my wife straight in the eyes, tears rolling down my face, and said “I'm going to beat this disease,” and she had tears rolling down her face and she said, "I know you will." But I didn't know how sick I was. I came home and started walking, which helped mitigate those side effects (of the medications). A block turned into two, turned into three, eventually a mile, and two and three. Walking just became a big part of my life. I also, very early on, started talking to a psychologist and realized that living with a terminal disease like PAH, positive mental health was very important. So, through positive mental health, exercise, strict nutrition, and relying on family, friends, my neighbors, my community, what I call my "village", I've made nothing short of a miraculous recovery. I was put on a pump. They never thought I would get off the pump. After a year, my right ventricle made almost a complete recovery and I was taken off the pump. I've been on oral medications ever since. Obviously, there's no cure for PAH, so I live with it. But I've adopted this, what I call "pieces of my pie", which again are medicine, positive mental health, nutrition, exercise, and my village. That's what I live by. Now that I'm better, I'm feeling better, I've dedicated my second part of my life not only to my health and my family, but to advocacy and raising awareness for the disease, and then also for people underprivileged who need it most. When I was diagnosed with PAH, we have a family foundation and we got involved with National Jewish Health in Denver, and we got involved with funding research. This is a disease that nobody in my world had ever heard of before I was diagnosed. We looked around the country, found NGH. On one visit back there, about probably three years ago now, I was introduced to Patty George who was one of the founders of Team PHenomenal Hope. They said, "You've got to meet Patty." We met and her energy was infectious. The mission that the organization had aligned with the lifestyle I was living. Right around that time, my oldest daughter came to me and said, "Dad, I want to do a fundraiser for the disease that you live with," and Where is EB was born. What better organization to raise the money for than Team PHenomenal Hope? I thought, okay, let's do a walk. So, we did a 5K walk and fundraiser, and I'm like, you know what? What better way to raise awareness for a rare disease than to go big? I planned a walk from LA to San Diego. A 170 mile walk. I would go down the coast, end in Del Mar. So, we started planning Where is EB, and what I thought would be 150 people coming out to a walk, maybe we would raise $75,000 through friends and family and people that we know. We raised close to $200,000 all for PH, PAH research and helping provide for people living with the disease that need it most. The first Where's EB I walked from, like you said, Santa Monica. We headed down the coast. Once we hit Huntington Beach, I just went down pretty much Route One. The biggest hurdle was Camp Pendleton. It turned out through the help of Carl Hicks, another board member, but somebody who I've become very close with. It's a very close-knit community because the disease is so rare and I've met so many amazing people along the way. Carl, who lost his daughter, Meagan. Ashlee Gambino, who I had the privilege to meet on a trip back to Las Vegas, who's a patient who has just an amazing story. And I met you and I learned about your story with Lucas. It is just such a tight-knit community, because when you're touched by this disease, whether it's losing a loved one or, like myself, being a patient, it is a family. So, Carl helped us through Camp Pendleton. That was a big hurdle because you can't go from LA to San Diego without going through Camp Pendleton. The US Marines could not have been more supportive. That day, going through Camp Pendleton was the highlight of my week. I was being escorted by the Marines. They brought in somebody to do an interview, and I'm being interviewing and we're walking, and I share my story. The interviewer stops and he looks at me and he said, "Sir, can I ask you a question?" He said, "Are you a Christian?" I knew immediately why he was asking me this. I said, "I'm not, but what I can tell you is I was given a second chance in life, and that second chance was to provide hope for those who need it most." He stops, and I'm abbreviating the story, but he puts his hand on my shoulder and he said, "Sir, that's probably one of the most meaningful things I've ever heard in my life, and that transcends all religion. Thank you." I realized right then and there that by sharing my story and by doing Where is EB and raising awareness for the disease, you never know who you're going to help. So, we get through Pendleton. I'm with Hap Farber. He was accompanying me by bike down the coast. Hap is another Team PHenomenal Hope board member, an expert in the field, and has become a friend. We probably got into the Del Mar area, and I had already calculated. I'm feeling great, there's no way we're going 175 miles. In Hap's head, he already had figured this out. He had already gotten to know me. Because I was pushing the whole way, and I'm living with PAH. Here I am pushing going down the coast, and we get into Old Town San Diego, and we go to bed and we wake up and I turn to Hap and I'm like, "We're not going 180 miles." He goes, "I know. We're going 200." So, we ended up going all the way down to Imperial Beach, if anybody knows Southern California. Almost down to the border. We were like three miles from the border. Came and then went up through Coronado, ended in Del Mar. It was 202 miles in six and a half days, all raising awareness for PAH and PH. That was last year, last September. The story just started to take off. I've met some amazing people along the way, developed relationships with people along the way, and it's evolving. It's evolved into a movement, is what I call it. I couldn't be luckier. People ask me, they say, "Do you have any regrets through this whole process?" I realized that I put people through some hardship in those years leading up to collapsing. I knew I was sick, but I wasn't going to the doctor. I was afraid to find out what was wrong, and I learned later on from my psychologist that upwards of 75% of men who are very sick don't go to the doctor. So, I wasn't alone. But I wasn't going to the doctor, and it was really creating hardship in my family, and for my wife and my kids, and it was creating issues in the house. Through the whole process, I have two regrets. One is that I put my family through that in those four or five years, because living with PAH is very alienating. You can be surrounded by 20 people, but you're alone because you're suffocating. That was one of my regrets. My second regret is that one of my kids saw me collapse, and then my second daughter... It was during COVID, so everybody was home, and my second daughter came up and sees me lying in a pool of blood. That is the hardest thing for me and that is my biggest regret. But besides that, I tell people if it didn't happen so dramatically, with stress that was going on in my life I might've been still dealing with that, but maybe I solved my health issues. But not everything would've been resolved. When I collapsed, it was like breaking through a sheet of ice. Everything went away. All problems, all stress, anything I was dealing with. I decided right there and then I was going to get help, I wasn't going to die, and I headed down a completely different path and I haven't looked back. That's my only regret. The last year has just taken on this amazing life of its own. I look at this as a second chance. So, through speaking engagements and then this very special project that we're now working on. I was approached about doing a full feature documentary and hired an amazing production company who's both familiar with obviously very good at documentaries, but also familiar in the rare disease field. It's owned by a woman by the name of Christie McCaffrey, amazing person, mom. She lost her mom to scleroderma, so she understands rare diseases. We're working on this amazing film. It's going to educate people on PH and it's going to educate people on PAH, but it's really a movie about overcoming adversity and providing hope, and through the adversity, what people do. It's going to feature myself and my story, but it's bringing in Carl Hicks and he's going to share his story, it's going to bring in yours truly (Steve Van Wormer), and it's going to bring in Ashlee Gambino. It's going to share their stories, but it's going to share what each individual has done through their adversity, through their moment and what they've done with it. The hope is that it educates people on the importance of mental health, and village, and education, and exercise and diet. But really for anybody going through adversity, whether it's living with a rare disease, losing a home in a fire, going through a divorce, losing a loved one, being diagnosed with anything, taking that first step and utilizing these tools, and how have we gotten through it and what are we doing with it? We've all decided to take that first step in our own way. My hope is by sharing my story and sharing these other stories that other people can see that and they can see if we can do it, they can do it. So, that's a really special project that I'm working on. Then, we're putting together the second annual Where's EB? That's going to be on September 7th. After the event, after the 5K, which is going to be an amazing, fun morning with food and giveaways and music and speakers, everybody's going to bid me adieu and I'm going to head down the coast again and walk 200 miles in six and a half days from LA to San Diego, and we're going to do it all over again. All with the hopes of raising as much awareness for the rare disease that I live with, which is pulmonary arterial hypertension and pulmonary hypertension. Steve Van Wormer: It's interesting, when you describe what happened to you five years ago, when you decide to pick yourself up and to move forward, whether you be the patient in your case, or a care partner, or whatever it is, a spouse of someone inflicted by this disease, can you tell me a little bit about how in your brain you kind of go from this selfish to selfless kind of role? If that's an accurate portrayal. I feel that for myself, and it's just like how you are pulling at threads in the community, and relationships, and people, and being blessed with having access to doctors and other patients and support and all that. How do you reconcile that in yourself? Just in your daily life or when you're singularly walking the streets or the highways of Los Angeles halfway to San Diego. Eric Borstein: Helping others came naturally to me. I have a philanthropic background. It was instilled in my brothers and I very early on that we're fortunate, and because we're fortunate, we should give back. It really wasn't a choice. We're going to give back. I've always volunteer coached. Even before I had kids I volunteered places. So, I always gave my time even before I had the means to give and help others financially. I've also been a very outgoing person. We always had people over at our homes. I always wanted to be around friends. I was just a very upbeat, outgoing, friendly person, giving person. So in those, I'll call it the four years, five years leading up to collapsing and pulling away, other than suffering health-wise and it getting worse and then to the point where I couldn't walk and breathe, the hardest thing was pulling back and it sent me into depression. I was depressed. I didn't see it. My wife saw it. But I was depressed. Now looking back, it's because I wasn't me. That was a really difficult time, and I'm the least selfish person you'll meet naturally. I give. I don't like to take, I give. I didn't understand it going through it, but one of the reasons why I was becoming depressed was because I wasn't myself. I wasn't happy. I wasn't wanting to be with people. I was really pulling back. After I collapsed and I was stabilized and I was able to start to look out for others again, it was just so easy and natural to want to help others. Sharing my story was very therapeutic, and to this day is very therapeutic for myself. It helps me. It is a reminder of where I've come in such a short period of time when I'm able to tell people I just walked 30 miles today. Living with a disease is very difficult. People look at me and they're like, "You look great." But it's a grind. It is a grind living with PAH. It's every day you've got to deal with really bad side effects. Again, one of the main reasons why I walk. You deal with the fact that you're going to live with this disease for the rest of your life, as long as you're alive. In the back of your mind it's always there. Are my lungs going to fail? Is my heart going to fail? It's always there. So, it is a battle. It is a daily battle both mentally and physically. But when I was able and I was healthy enough to help others, it was just very natural. It's something that's just in me. It's something that is very important to me. When I realized that my story was touching people both inside and outside of the PAH and PH world, it's something that I feel is my responsibility. I am very fortunate that I'm able to dedicate my life to my health. I'm very fortunate that I have an amazing business partner that takes on most of the day-to-day operations of our business. I'm fortunate that I've got the best doctors, and I've got great insurance, and I can afford the co-pays, and I can have a psychologist. I am very aware of that, but I'm also very aware that most people aren't so fortunate. Especially in the rare disease world, there's just not a lot of attention and dollars at the national level spent on these diseases, and the underserved are underserved. I feel like it's my duty to help those. It's my duty to provide toward research to hopefully find new therapies and extend lives. I've rededicated the second part of my life not only to myself and to be as healthy as I can, and to my wife and kids and those around me, but to help those others that need it most and to raise as much awareness for this disease as I can while I'm here. Steve Van Wormer: For me, I feel I was always very lucky. We were always very lucky, my wife and I, with our son, Lucas. He was diagnosed relatively early. We had great access to insurance. We had good doctors. And so, when you find people, when you know there's people in the community that don't have that access to services or they can't afford a clinic or they're just lost in the wayside... Right? I think that finding people like yourself, like Carl Hicks, another great friend I've known for two decades, that especially ones that have lost somebody, I feel like it would be easy to kind of just let the next generation figure it out. But I think this disease in particular is really built on the backs of all the families and community and the doctors that came before it. You mentioned this other patient, Ashlee Gambino. She's someone that's going to be a speaker at a December conference that's being held in Boston. So, the listeners have not heard her story yet, but they will. But it's another powerful story of a patient who went through a lot of tragedy, and has really picked herself up and is really working to help herself, first and foremost, and helping many others beyond that. Eric Borstein: One of the things that I've found being a patient, another highlight of my year this past year was I was invited to go to Washington on World PH Day, and meet with Congressional Delegates and meet with US Senators and their offices and staff, and share my story, and advocate for hopefully no cuts to NIH funding, Medicaid and the like. With everything going on, I felt, again, it was so important to do that, and it was a highlight of my year. What I'm finding in moments like that, I was elected Board Chair of Team PHenomenal Hope this year, and being an advocate for the disease and through Where is EB, what I'm finding is because I'm a patient and I'm living with this disease and this disease almost took my life, it's a different perspective but it's a very effective perspective. It's something that I don't take lightly. Again, yourself included, Carl, amazing advocates and voices for the disease. Ashlee, she struggles. She's much more newly diagnosed, and she'll share that in her story, but we're at different stages. But because I am able to, again, I feel it's important and it's effective being a patient. My livelihood depends on these things. My livelihood could depend on the next therapy, and those therapies come from NIH funding, and they all start somewhere. Being that patient voice is something I don't take lightly and something that I'm really embracing. Again, I've always been passionate about philanthropy, and I sit on other boards and all of them are so important. But living with this disease and advocating for this disease is something that I've never felt so passionately about. Steve Van Wormer: That's wonderful. We are blessed to have you. I think we would be remiss if we didn't say, and I trust you would agree, that a patient specifically who might be listening to this, while neither of us are doctors, the importance of pulmonary rehab and all that is a good thing. But I think we should tell patients, if you're inspired by a person like yourself, Eric, they should definitely talk to their healthcare team before embarking out on doing walks and all that stuff. Mental health on the other hand, I would say that's a pretty large part of your pie. Would you agree? Eric Borstein: It is. I tell people I wouldn't be alive without the medications, obviously. I'm extremely lucky that my body reacted so quickly to the medication that I was on. That's number one. Then... /episode/index/show/phawarepodcast/id/37892885

Episode 536 - Eric Borstein 09/02/2025

Akshay Muralidhar, MD - phaware® interview 535 08/27/2025

Akshay Muralidhar, MD - phaware® interview 535 Dr. Akshay Muralidhar, Co-Director of the Pulmonary Hypertension Center of Excellence at Arizona Pulmonary Specialist in Phoenix, discusses the importance of having a specialized center for pulmonary hypertension. Dr. Muralidhar also highlights the challenges of managing patients with substance abuse issues, particularly methamphetamine use. Dr. Muralidhar emphasizes the importance of access to care, particularly for underserved communities, and the need for outreach programs to ensure that all patients have access to treatment options. This Special Edition Episode Sponsored by: My name is Akshay Muralidhar. I am a Co-Director of the Pulmonary Hypertension Center of Excellence at the Arizona Pulmonary Specialist in Phoenix, Arizona. I joined this group in 2019. We have been a Center of Excellence for nearly a decade now. We have been managing and caring for pulmonary hypertension patients in the Phoenix area for almost two decades, managing complex care across the spectrum of pulmonary hypertension disease process. I did my training at the University of Colorado at their Pulmonary Hypertension Center of Excellence. I graduated in 2019. I am a native of Phoenix, Arizona. I was brought up here. I was looking for a reason to come home. It was a nice coincidence that my passion was explored more here at the Pulmonary Hypertension Center of Excellence at Arizona Pulmonary Specialist. The importance of having a pulmonary hypertension center of excellence is something that I really felt deeply about, and that's something I looked for when I wanted to start my career as a new attending and a new graduate of a fellowship specialty in pulmonary hypertension. So what makes a pulmonary hypertension center of excellence important? Pulmonary hypertension is a rare disease in the general population, but is an incredibly complex disease requiring multidisciplinary care across a spectrum of organs in the human body. As anyone who suffers from pulmonary hypertension knows, the one effect of worsening pulmonary hypertension can affect every single organ in the body. The specialty care offered by a Pulmonary Hypertension Center of Excellence, I feel, really allows for that multidisciplinary comprehensive care that a pulmonary hypertension patient needs with the expertise of someone who has seen hundreds of patients with pulmonary hypertension. That is important because being a rare disease, pulmonary hypertension sometimes gets overlooked or is difficult to diagnose in patients who might present with what we call non-specific symptoms. These are symptoms that don't really tell you what the disease is, but are damaging and tend to affect patients significantly, such as shortness of breath, leg swelling, lightheadedness, symptoms often experienced by pulmonary hypertension patients. It takes an experienced diagnostician and management specialist to be able to correctly diagnose disease and manage it in the future. Pulmonary hypertension is a disease of the right side of the heart and the lungs themselves, which results in damage to the right heart and the pulmonary vasculature that goes to it. When people present with shortness of breath, leg swelling, and chest pains, oftentimes routine testing in the hospital or at your general cardiologist or general pulmonologist office involves an echocardiogram. This often shows severe dilation and dysfunction of the right ventricle with potential involvement of the elevations in pulmonary pressures. In that situation, people often get alarmed about the presence of pulmonary arterial hypertension. While it is important to be concerned about this, it is also important to take the next step in beginning diagnostic testing and the referral process to a Pulmonary Hypertension Center of Excellence. The reason for this is because uniformly pulmonary arterial hypertension was a fatal disease in the 1990s. Thanks to expert care and expert medication and treatments, it is now well managed and completely treatable if the diagnostic tests indicate pulmonary arterial hypertension. The most important thing is having a low threshold for suspicion. Whenever we see patients with evidence of right heart failure, which include leg swelling, shortness of breath, lightheadedness, things of that nature, we have a low threshold for getting an echocardiogram just like any general cardiologist, pulmonologist would. If there's evidence of right-sided heart failure, a referral would be indicated to a pulmonary arterial hypertension center. In our center, we will do additional diagnostic testing and screening questions to be able to identify potential causes for this and then do confirmatory testing with right heart catheterization, which is the diagnostic test of choice to determine if the patient has pulmonary arterial hypertension. That allows an experienced physician to be able to formulate a management plan based on the severity of disease and the experience the patient is having. At a Pulmonary Hypertension Center of Excellence, the goal is to provide a comprehensive care management strategy for the patient. This includes appropriate diagnostic testing, determination if the patient truly does have pulmonary arterial hypertension, and then classification into one of several groups of pulmonary hypertension that are currently described including: Group 1 pulmonary hypertension, which is pulmonary arterial hypertension, Group 2 pulmonary hypertension, which is due to left heart disease, Group 3 pulmonary hypertension, which is due to lung disease and hypoxia, Group 4 pulmonary hypertension due to chronic thromboembolic pulmonary disease and Group 5, which is pulmonary hypertension due to other etiologies. Now, any experienced pulmonary hypertension physician will tell you that many patients fall into multiple categories. In this case, it takes an experienced physician to determine which is the predominant factor that is affecting the right heart in the pulmonary vasculature, and to be able to identify appropriate treatment strategies. Treatment strategies vary depending on the type of pulmonary hypertension you have. That is why it is very important to make sure that patients have appropriate diagnosis and classification of their disease process. After this, a comprehensive strategy to determine treatment is indicated. This includes meeting with an experienced nurse coordinator to be able to assist with management strategies as well as close follow-up routine lab testing to determine how treatment is progressing. If treatment involves therapies such as continuous infusions or injections, appropriate training is necessary to make sure that our patients are able to manage their disease properly as well as the side effects. As many patients who suffer from pulmonary hypertension know, side effects from medicines often are just as bad if not worse than the symptoms of the disease themselves. It takes very important coaching by experienced clinicians and nurse coordinators to be able to manage the side effects of the medications so that patients are able to take them without too much discomfort. Over time, many of these side effects are manageable and the true benefit of these medications are observed in the appropriate patient. Furthermore, being able to determine which classification of pulmonary hypertension a patient has is important, because medications that work for some groups of pulmonary hypertension such as Group 1 and Group 4 disease, may not work for other groups of pulmonary hypertension such as Group 2 disease. It is extremely important to have an appropriate interview with a pulmonary hypertension trained physician to be able to determine which classification predominates. When Centers of Excellence are identified, patients have been referred to us from all over the state and sometimes even across the country. I've had patients from rural Arizona, which is a large swath of area as well as many Native American reservations. I've also seen patients from Southern Nevada and Western New Mexico who make the sometimes eight-hour trek to get to our center. So, we have a very large referral basis to be able to seek a Center of Excellence so that they can get comprehensive care for their pulmonary hypertension patients. Outreach is incredibly important for any Center of Excellence dealing with rare diseases such as pulmonary hypertension, because it is very important to identify those that don't have access to care, to be able to get them to a Center of Excellence in an appropriate and timely manner. I personally do a month of pulmonary clinic at one of the Native American reservations. While it is a general pulmonary clinic, I've already identified several pulmonary hypertension patients in that area that I have then ensured that they are seen and managed by our Comprehensive Center of Excellence. It's incredibly important that not only common diseases, but also rare diseases that are life-threatening such as pulmonary hypertension be identified with a low threshold for referral and ensuring that these patients have the appropriate resources to be able to do that. Pulmonary hypertension centers, as I mentioned, also provide a comprehensive management strategy to these patients. This not only includes access to nurse coordinators, physicians who are trained in pulmonary hypertension such as myself, but also access to things like support groups as well as symposia and learning materials and access to online materials that are important for patients to be able to take away and manage their disease at home. Methamphetamine use is one of the most common drugs used in Phoenix, Arizona. In fact, Phoenix has unfortunately topped the charts in the country for methamphetamine use. Many of our patients who struggle with methamphetamine use get admitted either to the hospital with decompensated previously undiagnosed pulmonary arterial hypertension, or are seen in our clinic for progressive shortness of breath and are then found to have pulmonary hypertension. This is a particularly difficult population of patients to treat only because not only do we have to manage their medical disease as well as ensure that medications are administered appropriately, but we also want to address their ongoing substance abuse problem in a timely manner. Unfortunately, resources in general are lacking from management of substance abuse such as methamphetamines. So, a comprehensive care is only benefited many of our patients because of close follow up management strategies, support groups, things of that nature that really allow our patients to understand the lethal disease that they have because of their substance abuse problem and seek treatment before it's too late. Pulmonary hypertension back in the 1990s was a uniformly fatal disease. Thanks to the development of IV therapy, specifically IV Epoprostenol, this started saving lives initially. Since then, thanks to the advent of clinical trial research programs, pulmonary hypertension is now an extraordinarily manageable disease. It's wonderful to see the advent of therapies available for pulmonary hypertension patients thanks to clinical trials. Part of being a Pulmonary Hypertension Center of Excellence is that we have a robust clinical trial program. We understand the importance of clinical trials in improving the nature of treatment for patients suffering from pulmonary hypertension. Our patients are wonderful in actively participating in clinical trial programs. Thanks to these, we have now been able to administer adequate treatment for pulmonary hypertension to many patients that just consist of oral therapies. Thanks to clinical trials, a recent drug, Sotatercept, was approved, which is a new first-in-class drug that has been shown to be beneficial for pulmonary hypertension patients, and the field is only expanding. We have many partners in our pharmaceutical industry as well as in the clinical practice arena that developed a partnership with Centers of Excellence across the country thanks to being able to bring together many patients from a rare disease. In order to have a successful clinical trial, you need to enroll many patients and need to be able to demonstrate that your particular therapy has benefit to these patients. In a rare disease, the only way to be able to do that is to focus on centers that see these patients on a regular basis such as Pulmonary Arterial Hypertension Centers of Excellence. So, we personally have a robust clinical research program. We've been involved in most clinical trials that have come in the last decade, and ours the highest enroller in many of these as well thanks to an excellent clinical research team and most importantly, our amazing patients who are willing to participate in these trials so that they can benefit the field as a whole. One of the biggest reasons why I joined a Pulmonary Hypertension Center of Excellence is because I am able to open the door to patients who are interested in expanding treatment options for the field so that everyone can benefit by participating in these clinical trials. Being part of a Pulmonary Hypertension Center of Excellence, I think it is very important to make sure that patients have appropriate strategies in place for the therapy. This not only involves diagnosis of the disease, but also appropriate risk stratification. Many of my patients fall squarely into the category of pulmonary arterial hypertension, but many others have comorbidities that put them across several groups of pulmonary hypertension. All these patients require a specifically tailored regiment of therapy that allows for simplicity of management so that patients know what they're taking, how they're taking it, and to make sure that they're able to take it appropriately without overdoing it in terms of therapy. The advent of oral and inhaled therapy and now with Sotatercept has really made a difference in terms of being able to simplify the regimen. However, some of our sicker patients, while they require multiple therapies, 1, 2, 3, or even four therapies now with Sotatercept, many of our patients do just fine with dual oral combination therapy, which is basically standard of care at this point with close monitoring and management. However, pulmonary hypertension is still, by definition, an incurable disease. It's manageable, and management means making sure that the patients are not only on the appropriate therapy, but not overdoing it or not underdoing it. This requires close risk stratification, echocardiogram, right heart catheterization data if necessary, as well as management by an experienced clinician found mostly at a PH Center of Excellence. It really is important, I think, for patients to realize that their general cardiologists, their general pulmonologists, do an amazing job in being able to identify the disease. Most of the time, we're not the ones identifying the disease. We're the ones managing it. We get referrals because pulmonologists, cardiologists, and general practitioners understand how serious this disease is and being able to improve the awareness of the severity of this disease, especially since pulmonary hypertension is a relatively uncommon disease compared to things like diabetes and coronary disease, but just as deadly. I think is extremely important to be able to identify and send over to a fellowship trained pulmonary hypertension physician for management. This is particularly important in our underserved communities, which is why I think outreach is so important, especially in Native American reservations, across the landscape for patients in lower socioeconomic status who may not have access to not only diagnostic equipment, but treatment options too once they're diagnosed. We routinely care for patients who are not able to afford sometimes the expensive medications available that will treat their illness, requiring constant searches for sources of funding to provide for these patients to be able to manage their disease appropriately with the drugs that we know work for them. It is gratifying that pulmonary hypertension is now much more manageable than it was 20 years ago, but now it is very important to recognize the problem of access, the problem of specialty care, risk management, and treatment by Centers of Excellence because we need a comprehensive approach to management of disease. We cannot just treat the disease by itself because that will not help the patient. My name is Dr. Akshay Muralidhar, 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 /episode/index/show/phawarepodcast/id/31778022

Episode 535 - Akshay Muralidhar, MD 08/26/2025

Raj Parikh, MD, MPH - phaware® interview 534 08/20/2025

Raj Parikh, MD, MPH - phaware® interview 534 Dr. Raj Parikh from Hartford Hospital discusses building on foundational pathways with treatment options for Pulmonary Arterial Hypertension (PAH) patients. He also emphasizes the value of clinical trials as well as the importance of a good doctor/patient relationship. This Special Edition Episode Sponsored by: My name's Raj Parikh. I'm the Director of the Pulmonary Hypertension Center at Hartford Hospital in Hartford, Connecticut. I did a lot of my training kind of all over. I went to medical school in New Orleans, Chicago, ended up in Boston, and then made my way back home to Connecticut where I grew up. I've been at Hartford Hospital for the last four years. I started the Hartford Hospital's Pulmonary Hypertension Program from scratch, and I've been building it up since then. I got involved in pulmonary hypertension really during my fellowship at Boston. I really wanted to get involved in the rare disease space of PH, where patients are far and few between, but the ones that are there, they have pretty significant morbidity, mortality, and a poor prognosis. There's limited treatment options and there's limited specialty care for these patients. I really wanted to come back home to my state of Connecticut and build up a program in sort of the area that I grew up in. Today I'd like to discuss building on foundational pathways, treatment options for pulmonary arterial hypertension patients. This is pretty exciting stuff. In the last year, we've had one of the latest agents to enter the pulmonary arterial hypertension therapy space, and that's the first and only combination pill. When we think about PAH and the pathophysiology of pulmonary arterial hypertension, we think about three foundational pathways. These three pathways basically result in too much restriction of our pulmonary blood vessels. What that does is it creates these three pathways as potential targets for therapy down the line. What we know about PAH historically is that these three pathways have created three different classes of therapeutic agents. We think about the nitric oxide pathway and the nitric oxide agents that then therefore intervene on that pathway and can treat PAH. We think about the endothelin pathway and then therefore the endothelin receptor agents that again, can intervene on that pathway. Then we think about the prostacyclin pathway. As the first and only combination pill, what it does is it takes two of these foundational pathways and develops it into one, combination, once-a-day, easy to use, easy to prescribe, cost-effective pill for patients. We just think about what that can not only do for patients in terms of compliance and adherence, but also decreasing their pill burden, making sure they're getting this dual therapy upfront, which is part of really our standard guidelines now for PAH treatment, which is using multiple pathways, multiple agents, not just one agent from one pathway for sort of standard of care. That's really what the foundational pathways and building on that and creating this treatment option for PAH, which is the first and only combination pill is really all about. A lot of what we do in pulmonary arterial hypertension, what we've learned is the more aggressive we are upfront, the better the patient's overall morbidity and mortality and prognosis will be on the back end. We've shifted away from a gradual, let's do one medication, let's assess your response. Let's do a second medication, let's assess your response. We've become a little bit more, what I would say aggressive upfront. Because what we've seen with that is that while we are aggressive, we're able to take a patient's, what we call risk score, risk stratification from something like high risk or intermediate risk and really bring them down to a lower risk score. When we do that, it's not just about creating a number of a risk stratification score of 12 to two. That two is really where the patient's quality of life improves. The patient's able to breathe better. The patient's able to do the things they want to be doing. The patient's able to live longer. The patient's able to stay out of the hospital, stay out of having to do routine invasive tests and things like that. Overall, the prognosis, the morbidity, the mortality changes as we have a lower risk stratification score. We really use our risk stratification tools. There's two or three out there. There's the European tool, there's the REVEAL score to really guide our therapy. What we've learned and what we've actually made standard of our clinical practice based on standard guidelines mentioned by the World Symposium on Pulmonary Hypertension, standard guidelines mentioned by the European risk stratification scores and European guidelines is to think about dual upfront therapy. That is basically taking a medication from one pathway, taking a medication from that second pathway and getting patients on two medications upfront right away for patients who have certain degree of risk. What that does is it improves their morbidity and mortality and prognosis by bringing their risk score down. So if we think about a medication like this, which is essentially saying, okay, well, we know standard practice is to be on two types of pathways, why not take those two types of pathways, spit it out into this one combination pill, the first and only ever combination pill in pulmonary arterial hypertension and make it easier for the patient. So now they're not taking up to 13 pills at three time intervals in a day, they're taking one pill at one interval once a day. This is cheaper for the patient. This is easier for these rare disease offices like PH clinics who have to order and get these medications approved through insurance. Now, they just have to get one medication approved instead of multiple, two or three or something like that. When you think about it from that angle and you think about rare disease in general, I think getting involved in clinical trials is almost like what you kind of have to do when you have, let's face it, limited options when it comes to pulmonary arterial hypertension therapy. I've been in this space managing and directing the PH Center at Hartford for four years. Prior to that, I've been as a fellow, really involved in learning about pulmonary hypertension for three years prior to that. In the last seven years, only one new medication, except for obviously this combination pill, has entered the PH space. There is limited options as a whole when it comes to pulmonary hypertension. Pulmonary hypertension is a rare disease. It's rare because it's overlooked sometimes. It's missed sometimes. It has a poor prognosis, and therefore there's not a lot of patients living for 15, 20 years with pulmonary arterial hypertension. That's just not the longevity of the disease. So when you're dealing with rare disease, when you're dealing with limited therapeutic options, getting involved with clinical trials is really important, especially when you're somebody who is a little bit more advanced, or you have a patient in your office who's a little bit more advanced than the common patient in PH who has a lot more deteriorated quality of life, a lot worse prognosis. Then, you're really looking at things outside of the box, outside of really these three foundational pathways, outside of what's really FDA approved for these patients and what else can you offer them? Can you get them involved in a brand new clinical trial for a medication that A, might have amazing outcomes, B, may have really neutral outcomes or C, god forbid, may have an increased signal for harm? But sometimes some of those risks really outweigh the benefits and sometimes the benefits really outweigh the risks for a patient who has poor prognosis in a rare disease space such as pulmonary arterial hypertension. When this combination pill, got FDA approved and was available in the market, what we did in our office, in our center was literally went through our list of patients and cold called them. We said, "You are already on the therapy that really this new medication contains." It contains one product from a phosphodiesterase or nitric oxide pathway, one product from the endothelin pathway, and it really takes it in and just spits it out into this one pill. "You are taking three pills once a day or you're taking 13 pills three times a day. That's really the combination that really would translate over to this new option." We called them up and we said, "You are welcome to make the transition.” It is, of course, rocking the boat. Now maybe you are fairly stable, maybe you are just freshly on these pills. Maybe you are on these pills for a long period of time, but you're not really stable and you could feel like you could get better. It's a balance between all those three types of subpopulations. Naturally speaking, the patients who are on only three pills and they're going from three to one and have been very stable and have been on that regimen for years, probably are a little bit more hesitant than, let's say the patient who's on 13 pills, three times a day and switching it over to just one pill once a day. Or if you take the patient who's on three pills, but it's for some reason for their insurance, that's a very expensive three pill regimen, and they're going to be able to really cut down their monthly costs in terms of pills and costs for the medications by switching over to this combination pill. So that's another type of patient, even though they're very stable, even though they've been on this regimen for several years, they really want to transition over. I would say that for the patients who were not treatment naive who have been on the different products that are contained into this combination pill, really anecdotally speaking, we have seen no sort of adverse or new developments or really curveballs in terms of making the transition. It's been pretty seamless for patients transitioning from being on the separate therapies into this really what we call loose dose therapies into this combination pill. My relationship with my patients is very important, and I think that focus is really because it's a rare disease space. When I'm going into my clinic day and that clinic day may have 20 patient visits for that day, 12 of them are follow-ups, patients I know. But eight of those 20 patients, almost half are brand new referrals, new patients I've never met before. They've never probably seen a pulmonary hypertension specialist before. They could be traveling from an hour, hour and a half, two hours away. Pulmonary hypertension, as we know, it's a rare disease. There's not a lot of providers who specialize in just pulmonary hypertension. There might be cardiologists who can dabble in pulmonary hypertension. There may be pulmonologists who dabble in pulmonary hypertension, but the space of pulmonary hypertension is limited in terms of how many providers are available nationwide, even worldwide. A lot of times patients who may have pulmonary hypertension, may have a concern that they may have pulmonary hypertension, may have already formally been diagnosed with pulmonary hypertension, may have to travel hours out of state, out of their area to come and visit a pulmonary hypertension specialist. With that visit comes a lot of pressure, comes a lot of pressure on the patient's end, comes a lot of pressure on the pulmonary hypertension specialist's end, comes a lot of pressure on the doctor who made the referral. That patient may have been for 30 years following with this cardiologist who is well renowned in their local town, who has managed all the cardiology issues for all of that patient's family members, their parents, their grandparents, and what have you. Now, that cardiologist is saying, "As seasoned as I am, as well as I know you, as well as I know your family, as well as I know this community, I'm sending you out of this state to a specialist in the city who is 30 years younger than I am and is not as experienced in the field in general as I am, but he or she is a pulmonary hypertension specialist." There's a lot that really comes with that. I think we sometimes don't put as much weight into all of that, but I think that's what is the pure beauty of really rare disease. That's what accompanies rare disease cases and rare disease patients and rare disease specialists. Now, you walk into their clinic room and they've traveled an hour and a half, they've been Googling about their disease state, rightfully so, doing their research. They've talked to the referring doctor who maybe is a long-term doctor of theirs, and they really have certain expectations from the provider. Now, I'm walking in and I want to make sure I can meet those expectations. I can really tell this patient, this is truly what I think is going on. These are the X, Y, or Z ways I think we can potentially make you better. And if those things don't work, this is my backup option. Or I'm walking in and saying, "Hey, you are very advanced, and if we don't do these two things, I don't think you can live this much longer." It's hard to say that to someone you've just met for two minutes who really is expecting to really make significant improvement by seeing you. It's a fine balance. I think sometimes being confident in your subspecialty disease, knowing what's available, knowing how to assess the patients, their prognosis, just having confidence in that process helps you maybe gain confidence from your patient. It's really hard. It's something that we all deal with in the rare disease space, is that how do we manage those expectations from the patient, from the referring doctor, and how do we invoke confidence in our patients? We want patients to trust us in this small field, in this rare disease field. We know what we're thinking about doing. We know what we want to do. We hope that it's going to be successful. If it's not, we know what our backup option will also look like too. When you stop and think about pulmonary hypertension patients, it's not just about the pulmonary hypertension pills that they're on. There's a couple pathways out there, like I've talked about. Those pathways yield certain types of medications. There's three or four maximal medications a PH patient can be on from a PH specific standpoint. But what about what other medications are they on? A lot of patients with pulmonary hypertension, they deal with heart failure. For that reason and that reason alone, these heart failure patients are on water pills. Sometimes they're on one water pill. Sometimes that water pill is dosed two to three times a day. Sometimes it's two pills three times a day. Now, a lot of times you're on two or three total water pills. So think about the pill burden from just the heart failure perspective. And the heart failure is simply a consequence of the pulmonary hypertension, but not treating the pulmonary hypertension itself. When we think about pill burden in PH patients, it's not just the PH pills, the three to five to 13 pills that they're taking three times a day. But now you add on the water pills, which are two to three times a day, two to three pills at every interval. Then with the water pills, you have electrolyte imbalances. So, now these patients are taking potassium supplements once or twice a day, magnesium supplements once or twice a day. Let alone if they have any other comorbidities such as diabetes or hypertension or hyperlipidemia. But let's just say just this pill burden alone is so overwhelming for these patients. So, to be able to take that pill burden and bring it down from 13 to one, from five to one, from three to one, in the large scheme of things, is a huge win in regards to patient adherence and patient compliance and cost-effectiveness, as well, for the patient. Thanks for listening everybody. A couple takeaways from my standpoint is obviously talk to your care team, talk to your provider before making any significant medication regimen changes. It's always important to be aware and ask about what active clinical trials may be going on, if that's something that you would be a good candidate for. I think that's important from just a disease perspective to know that these are the other options that are available to you out there. With pulmonary hypertension, this is a rare disease. I think the more we as a community can discuss amongst each other options that we have available to improve our quality of life, improve our prognosis, improve our medication adherence, these are all opportunities that we have to take advantage of. My name is Dr. Raj Parikh, 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/35535790

Episode 534 - Raj Parikh, MD, MPH 08/19/2025

Robert Frantz, MD - phaware® interview 533 08/13/2025

Robert Frantz, MD - phaware® interview 533 Dr. Robert Frantz breaks down the key metrics behind risk stratification—functional class, six-minute walk distance, and brain natriuretic peptide levels—and explains how they shape treatment plans. He also explores the role of echocardiography, right heart catheterization, and cutting-edge therapies in improving patient outcomes. This Special Edition Episode Sponsored by: I'm Dr. Bob Frantz. I'm a cardiologist at the Mayo Clinic in Rochester, Minnesota, and Co-Director of the pulmonary hypertension clinic there. I have been working there on staff since 1990, so actually many decades of time of seeing patients with pulmonary arterial hypertension and seeing their therapies evolve. Today, we're going to talk a little bit about risk stratification and pulmonary arterial hypertension. I had the privilege to be a member of the at the Seventh World Symposium on Pulmonary Hypertension in Barcelona. This brought experts together from around the globe including patient representation in order to talk about the different risk stratification systems, how they can be utilized, and both discussing their use in clinical practice and also as tools and research protocols, as well. I would say that we had a very vigorous discussion at those meetings, because there are different risk stratification tools. Some people might say, "Well, if I have a really expert pulmonary hypertension provider, why do they need some risk calculator to plug in some numbers about me to say you're low risk, intermediate or high risk or whatever?" I'd say that's true to an extent, but there's a sense to which we all acknowledge that, number one, everybody seeing a PAH patient might not be a world expert. Number two, it is sort of objective and can be tracked over time and can avoid conscious or unconscious bias that can come into our thought process for whatever reason. Even things like, well, we like our patients, we want them to be doing well. It's hard to talk about maybe your prognosis is not as good as we would hope. Then, we might actually underestimate the severity of their disease because we're trying to be positive and optimistic. I feel like for me, who has been doing this for many years, I feel like, "Okay, I'm just going to objectify this set of information so that I'm keeping myself honest about where this person really is in terms of an objective risk calculator that might be a little different from how I'm viewing them today." The thing about risk calculators, they're used in many disease states in order to try to quantify prognosis with regard to risk of hospitalization or mortality. We know a couple of things. One, if you don't have very many of the components of the risk calculator, then it doesn't work very well. One thing we agreed on at the World Symposium is that there are various risk calculators, but key components are Functional Class I, II, III or IV. "Are you asymptomatic or are you short of breath at rest?" Functional Class III would be if you go up a flight of stairs you get breathless. That's one metric. So, that is one component because the more advanced your Functional Class, the more concerned we should be about you. The other thing is six-minute walk distance, like how far can you walk in six minutes? That is quite closely related to prognosis. That's why it's important for patients to be doing those six-minute walks and trying during that time to walk about as far as they think they can in that six minutes, even if they have to rest or stop a couple of times, because it's a reproducible way of assessing whether they're getting better or worse or about the same. The third component is brain natriuretic peptide or N-terminal pro-brain natriuretic peptide, which is a mouthful, but it's basically a protein made by the heart that is released to try to help our kidneys dump fluid and salt, so kind of what we call a counter-regulatory hormone, that if the pressure is going up inside the heart, in the right atrium and things, we release more of this to help the kidneys get rid of fluid and salt. We use it as a prognostic marker. You can, as a patient, know what your BNP level is next week and you could have it checked again in three or six months and might follow that over time with the idea that if that value is rising, that might be of some concern. If it's dropping, that might be reassuring. The thing about that particular metric is that there's two assays. One is called BNP, just brain natriuretic peptide, and the other is N-terminal pro-brain natriuretic peptide. Different hospitals and systems might use the different assays. It's important to know which one is being done, because otherwise a person could be rather concerned that the level's changing dramatically. It's just actually a different assay depending where you had it done. The NT-proBNP circulates at levels that are four to eight times the BNP, so they're very different metrics. I think the bottom line is the risk calculators that include a variety of things that include the REVEAL risk calculators 2.0 and what we call 2.0 Light and the European calculators, which are things like COMPERA and COMPERA 2.0 have some differences, but all of them include functional class, six-minute walk distance and brain natriuretic peptide levels, and may include other things that are things like in for REVEAL 2.0 is heart rate where if your heart rate's high and your blood pressure is low, that's concerning. So high heart rate over 96 beats per minute or low blood pressure sort of in a way that would be of concern also would go into that risk calculator. In the REVEAL 2.0, if you have renal impairment, so that when you see these calculations being done, you can think about what's in the calculator. When we think about that risk calculation, baseline when people are first diagnosed, are you low risk, intermediate risk or high risk? Almost everybody, even if they're both low and intermediate risk are getting an endothelin receptor antagonist and a phosphodiesterase 5 inhibitor, what we call dual therapy. If they're high risk, they may be getting IV or subcutaneous prostanoids plus an endothelin blocker plus a phosphodiesterase 5 inhibitor, what we call upfront triple therapy. The precision of that risk estimate just has to get your provider in the ballpark that they're going to provide you at least dual therapy or maybe triple therapy. The goal of the risk calculator is to try to get patients to low risk if possible, a good six-minute walk distance, a good functional class, not being out of breath going up a flight of stairs, having a natriuretic peptide level that's normal or low as opposed to high and rising. These risk calculators are especially useful when patients come back, because then they're already on some treatment, and is that treatment actually working or not? Patients and family members might get frightened that they're in a high risk or intermediate risk category, but need to recognize that the important thing in a way is when they come back in three to six months, is that risk lower now, are the therapy's working? So, redoing that risk calculation and saying, "Oh, the six-minute walk distance is a hundred meters farther and your brain natriuretic peptide level is significantly lower, and now you're not short of breath on stairs. We're feeling like you're now in a low risk category. Let's stay with these two drugs we're using." Or, "Actually things aren't quite where we want them to be." The beauty of the risk calculator, in a way, is its objectivity. Sometimes the patient will say, "Doc, I don't know you that well. Do I trust you, you're really expert about this? You really want me to add another drug? Is that just because you want to be aggressive or do I really need it?" Having an independent risk calculation that says, "Yes, actually you're still at intermediate risk for a bad outcome," can sometimes empower patients to understand that it's just not me as a provider saying I think they should do this or that. Any group of experts would say, "This person is still at intermediate risk. They would benefit from additional therapy and it may be worth putting up with some side effects to reduce the risk." The other way we use these risk calculators sometimes can be in clinical trials as a secondary outcome measure, that is, is your risk calculated score improving on average with therapy or not? The other way we can use them is that if we have a set of patients who went into a randomized clinical trial of a PH therapy, we can characterize those patients by low, intermediate or high risk. Then, at the end of the trial can look and see which patients benefited the most. Then, when I'm seeing a patient in a clinic and I'm saying, "Should I apply this drug from this clinical trial in this clinical situation? How does my patient compare to the patients who did or didn't respond to the therapy?" An example of that would be that analysis in the GRIPHON Trial, which is the study of selexipag, which is an oral IP receptor agonist as therapy for pulmonary arterial hypertension. On average, there was benefit in the patients who received the drug, but the patients who are actually at highest risk going in maybe benefited somewhat less. Maybe those patients actually would've been better off just going on to an IV or subcutaneous continuous prostanoid therapy as an even more aggressive therapy. We might say people at lower to intermediate risk, adding the selexipag could be very useful. If they're at high risk, then probably they'd be better off to go with even a more potent therapy. That kind of information can help us understand how to personalize our treatment for our patients based on the risk category. General consensus was that you should be reassessed clinically and with regard to risk at three months after making a treatment decision, because most of the therapies that we give are going to be effective or not as effective as we like within that timeframe. Then, at that three month point, there'll be a reassessment, and then maybe a slightly different direction. Then, another three months later should be assessed again, and continue that every three month kind of recheck until a person is at low risk or on all the therapies we have available. If they're stable and on the therapy we think is optimal, then we might back off to every six months. I think that the issue of reimbursement and coverage of medications is one that we all deal with on a regular basis. I would say that the clinical decision to add another therapy generally isn't going to be second guessed based on a person's risk categories or something like that. If the clinician thinks another drug is appropriate, then, usually, that will be accepted. It may be that there could be some pushback where if we say they're at high risk, they need IV or sub-Q continuous prostanoid and they're not yet on an endothelin receptor blocker, which is an easier oral agent that maybe the insurance companies say, "Well, you should be adding that, and they have to fail that before they go to the continuous infusion." We might say, "They're too sick to do that now. We need to move to this other therapy now and can talk later about whether to add or subtract or something later." There can sometimes be that where if you're sort of leapfrogging ahead because they are not on aggressive enough therapy, there could be some pushback that you haven't tried something simpler. You have to say, "Look, in my judgment, we can't go simpler. We need to go with a more aggressive program." So, that can sometimes be an issue. This World Symposium tends to meet about every five years. It's only one forum for our discussions about optimal strategies. There are many other forums that can include consensus documents that different scientific groups put together like the pulmonary or the cardiology societies and position papers that people might write, or just best evidence-based recommendations from a set of experts that might get published. So, there can certainly be updates to these things. I think that one component of the World Symposium Review on Risk Calculation included a couple of things for saying directions we need to go with this. One includes that there's not very much echocardiographic guidance in the risk calculators, partly because there wasn't very much echocardiographic information in the registries that resulted in development of these calculators. So, if you don't have that information in there, it's hard to incorporate it. One of the recommendations was that in addition to using these risk scores, we should also be keeping track of echocardiography in terms of how the right side of the heart is behaving, how big are the chambers, how impaired is the function of right ventricle, how much is the tricuspid valve on the right side of the heart leaking, and use that as supplemental information to our risk score. You might have somebody who's young and they've otherwise been pretty healthy, and they still can walk quite far on a six-minute walk and maybe their BNP level isn't very high, but when you look at their echo, the right ventricle is quite severely impaired. That might be something where you say, "Well, I don't think the risk calculation is actually taking to account what's happening with this patient's right ventricle as fully as I would like. So, we're going to incorporate that right heart ventricular function information, into our decision-making and that the onus is on the community to continue to try to develop better and more widespread utilization and metrics for what the right heart is doing in terms of performance." That's one thing that the field is going to continue to work on. It's like, "What on the echo should I be looking at that tells me that they should have more therapy?" The other thing has to do with hemodynamics and right heart catheterization. From the echo, we get estimates of pressure. We might get some estimate of the flow of the heart and cardiac index, but it varies in accuracy across centers and patients. There is the understanding that in situations where we're not sure which way to go, where sort of some low and some intermediate and maybe some high-risk features, that repeating a right heart catheterization in order to get very objective, accurate information about the cardiac output and the right atrial pressure and the cardiac index and things can be useful. Especially, as we start to have developed drugs coming along like sotatercept or Winrevair, which is this anti-proliferative that's scavenging growth factors in the blood, given as an injection every three weeks, that may lower the pulmonary pressure and lower the resistance, that repeating right heart catheterization and seeing how much better are things in that objective way might help us, especially if we're trying to make decisions about backing off on one of the other therapies. We know our prostanoids, the infusions, the inhaled drugs, the oral therapies, that prostanoid class of drug has the greatest side effect burden, and is maybe most hard for patients in terms of quality of life and having to deal with that. If some of our anti-proliferative therapies are successful enough, maybe we can reduce the burden of our therapies by reducing that prostanoid or maybe in some situations even taking it away. But if you're going to be doing that, you need to be really sure where things stand and maybe a risk calculator, and how far people can walk and their functional class isn't quite enough to be sure about that. Especially now, we're talking about backing off on other therapies for improving quality of life and burden of the therapies, repeating a right heart catheterization to know where we really are could be very useful. Another thing I'd say about risk calculators is number one, they can be incorporated into electronic medical records. So then, that is living in that electronic record and can be flow charted like, "Okay, this patient's REVEAL risk score was 9 three months ago and now it's 7." I can see that difference and I can track it. The other thing is that there are apps. If a person just Googles, “pulmonary hypertension risk calculator,” it can take you to a website that has all the different risk calculators. A patient could do that themselves if they wanted to, if they have that information. So it could be sometimes somewhat empowering if they choose to do so. Go there and say, "Hey, what's my REVEAL 2.0 Light risk score after this last visit," if my provider didn't tell me or didn't calculate it? It always needs to be put in the context of the patient. I think that was another message from our debates at the World Symposium about this, is that this is one modality to assess risk. It has to be put in the context of the echo, the human dynamics, patient goals, where we're talking about patient-centric medicine. So we could say, "Oh, you're at intermediate risk, we should add a prostanoid." Then, the patient has to do some thinking about that, "Am I willing to take on that side effect burden to try to reduce these risk characteristics, or will it help me feel enough better that it's worth the side effect burden?" We can get caught up in this objectification of risk and things, but realize this is a person sitting in front of us with different goals, and maybe we're not engaged in a way where we really understand what they're looking for. If we're mono-focused on reducing risk and adding more drugs, maybe we're not really meeting the patient where they are in terms of their journey. I always like what Rino Aldrighetti, the former head of PHA used to say, "Every patient has the opportunity and the ability to fight this disease as much or as little as they choose to do." We have to accept that, that we might be all in, "Do this, do this, do this." Is that really where our patient is? So, I think that's the other message, taking it back and being patient-centric, "Here's the information. Let's talk, what are our joint goals here?" Thanks so much for listening. My name is Dr. Bob Frantz, and I'm aware my patients are rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story: #phawareMD #RiskStratification /episode/index/show/phawarepodcast/id/35847280

Episode 533 - Robert Frantz, MD 08/12/2025

Jean M Elwing, MD - phaware® interview 532 08/06/2025

Jean M Elwing, MD - phaware® interview 532 ICU & You: What Happens When Pulmonary Hypertension Turns Critical? Pulmonary hypertension is already a complex condition, but what happens when it becomes life-threatening? Dr. Jean Elwing breaks down what patients and families can expect when someone with PH ends up in the ICU. My name is Dr. Jean Elwing, and I'm a professor of medicine, and I'm the director of the Pulmonary Hypertension Program at the University of Cincinnati, and I've been there about two decades now, really focused the entire time on the care of patients with pulmonary hypertension and related illnesses. Over that time, we've built a large practice of pulmonary vascular disease-related conditions and are trying to do our best to improve the lives of people we take care of. So today I want to talk to you about how we approach a patient with pulmonary hypertension that's in a critical situation in the intensive care unit and how we manage the medications that are very helpful to you as an outpatient, but how we do assess and how we use those medications inpatient when people are very sick. So there's multiple situations that can bring a patient into the ICU. They could rapidly worsen in terms of their right heart function. If they would have an arrhythmia, irregular heartbeat, or a blood clot or something would change dramatically with their medications, or they could gradually be worsening over time, or they could be a new patient that had never been diagnosed before. Each one of those scenarios we treat a little bit differently, but the thing we can't forget is the origin of the problem is the elevated pulmonary pressures. So once we figure out why they're elevated and why that individual has right heart stress and strain and going into right heart failure, then we can intervene. In patients who have progressive pulmonary arterial hypertension or patients who are newly diagnosed, the main focus should be addressing the pulmonary vascular changes and trying to unload or relieve the stress of the right heart. And we do that through our pulmonary vasodilators. Each individual patient will be assessed based on their specific needs, but in general, we try our best to initiate medications that relieve that pulmonary vascular resistance as soon as we can. Oftentimes we start with things we can get to the patient very quickly like an inhaled therapy such as nitric oxide or inhaled epiprostenol. And then we take a few minutes, we look at our situation, we look at that individual patient and then decide is this a person that should be given infusion therapy, continuous IV medications to help reduce that stress on the right heart? And in most circumstances, that is what we do when patients are critically ill with pulmonary arterial hypertension and right heart failure. We also ask the other members of our team to help us make sure we address all the issues that could be contributing and anything else that could be worsening the situation like low oxygen. We would give you oxygen in a way that would raise the levels to a point where it no longer is causing stress on the right heart. And sometimes that's through a nasal cannula, sometimes it's through a non-invasive machine like a CPAP, and sometimes it requires patients to have a tube in their throat to be intubated and put on mechanical ventilation. So we have to figure out what need that individual patient has and then address it the best way we know how to take care of them. The other thing we'd look at is how much fluid they have in their body. Is it a low fluid state or a high fluid state? Most of the time when patients are in right heart failure, they have extra fluid and we try our best not to give additional fluid and then gradually try to help the person get rid of extra fluid through medicines called diuretics. We also have members of the team that could help with mechanical support. That is in our most severe situations, we look to people that could help us offload the work of the right heart by a machine, and that's usually ECMO. So it's a circuit that helps circulate blood, give oxygen, and get blood to the right places in the body to give the body a chance to heal. Now, that's not an option for everyone, but in rare circumstances when the medications are working but not fast enough, we have to have a bridge to give the body enough time to heal and hopefully recover from that serious situation. No one part of this equation will get the person better. It has to be a multiple intervention all at the same time working together as a team to get the body to be able to get back to the point where you can breathe independently without extra oxygen, without a machine, and get the medications up to a level where your body can heal enough to be able to go back home or go to a place where you'd have additional time for rehab. So it is a very complicated situation. We try to avoid patients getting sick to the point where they need the ICU, but this is why we have an ICU. So we can rescue people in circumstances that are very dire. So of course we never want a patient to be faced with a situation where they're critically ill, but we always want you to be prepared. This is something that we talk to our patients about oftentimes when they're first diagnosed. Then as you're on medications that are complicated and sometimes infusion therapies for years, we may not revisit that, but we want to remind everyone who's involved, the caretaker, the patient, the support individuals that we want to be ready. So be prepared in terms of having medications and your go-pack ready if you would have a situation where you'd need to go to the hospital quickly. And if you're away from your home or you're in a remote place where there's not experts in pulmonary hypertension, then make sure that doctor that sees you and the person that does the intake when you get to the emergency room knows you're special, you have special needs, and you can't be treated like everyone else. You have to be treated in a way that takes care of the underlying problem, maybe an injury, maybe appendicitis, but in the context of your pulmonary hypertension, we manage things very differently. We change how we treat you with different medications. We also change how much fluid we might give you. So those are really important things, I think, to remember and to have on your list to be ready for anything and everything because that's how life goes. The minute you think things are quiet, something comes up where you might need some extra attention. We see each patient as an individual. Pulmonary hypertension has so many different faces and so many different levels of severity. So usually the first thing that happens when you come to the hospital and you're sick and you're seen in the emergency room, the ER staff is very aware of the complexities of pulmonary hypertension and they oftentimes call a consult to the pulmonary hypertension team, which may be a cardiologist in one center or a pulmonary in another center, and they get them involved and they look how the patient is doing. If you're the individual patient being seen, they'll assess how your heart's doing with an echocardiogram. They may do labs to look how the kidneys are functioning and labs to look at stress and strain on the heart with an NT-proBNP or a BNP. They'll look at blood pressure, heart rate, and urine output and try to figure out what is exactly happening with the level of risk you're at for having worsening symptoms or for worsening right heart failure. And then we'll decide are we having a problem with medication? Was there an interruption in medication? Do we need to go up on medication or do we have to start something totally different? So really depends on what's happening.= And then of course we go through all the things that could trigger the pulmonary hypertension to be less well-controlled, meaning we think about thyroid disease, we think about infection, we think about medication interruption or anything that could set off the fine balance that you as a patient are in on a routine basis. And then once you're admitted to the hospital, you would be admitted to the location that can take care of patients with pulmonary arterial hypertension or pulmonary hypertension of other sorts. In that location we hope to be able to deliver care that's very sensitive to your needs and take that into account when we choose different therapies that we give you for whatever is triggering the episode of worsening pulmonary hypertension. One very common thing that triggers pulmonary hypertension worsening is irregular heartbeats, fast heartbeats, or what we call arrhythmias. In that circumstance, we would give you medicine oftentimes to help control that heart rhythm, but we choose specific ones that fit the needs of pulmonary hypertension patients. We would interact with our cardiology colleagues that focus on arrhythmias so we can do our best to control that, because controlling that arrhythmia many times will help you to be able to recover from that worsening event. Another thing that frequently gets patients in trouble with pulmonary hypertension is infection, and so we're very mindful of that. We try to treat infection early and treat it in a way that you tolerate, meaning not too much fluid and be very cautious about the oxygen changes that may occur in circumstances like pneumonias. So it's really complicated when you get sick and need to come to the hospital, but it really takes a team approach and to really hone in on the cause of the issue and make sure we support the pulmonary hypertension in the meantime. Of course, we always want the happy ending to every hospitalization, but sometimes things don't go that way. And sometimes there's things that are not recoverable easily with our routine medications. And as I mentioned, sometimes we look to a mechanical support with ECMO to support patients, but not everybody's a candidate for that because of various reasons. And so one thing we always have in the back of our mind is could you be a transplant candidate if you're really sick with pulmonary arterial hypertension and right heart failure in the ICU? And if so, that's a conversation we would have with you. The things we pay attention to very carefully when patients are sick in the ICU and we're thinking about transplant are a lot about your history and what might make you a optimal transplant candidate or some things may limit that option for patients. And so we look at things like social support, history of other medical illness, age, also weight plays a role, and if you're even interested in that. And if you are and you're the right type of person for a transplant evaluation, we would contact our transplant team and colleagues and see if this is the right time to complete that evaluation. Sometimes they would like us to continue to support, try to get you better before you are seen in the transplant center. And sometimes they want to start the evaluation right away. Some centers have a transplant team right there with them. In my case, I do not. I work with outside facilities for transfer, so we would work with another hospital system and then it would be a transfer to that location. So it really depends where you are, what your interests are, and what your history is to determine if transplant might be the next best step. When pulmonary hypertension patients develop a severe illness and they require urgent evaluation, we require a higher level of care to be delivered to the patient because of the complexities of pulmonary hypertension, the right heart function, and the medications that patients are on with pulmonary hypertension. So this is a circumstance where we have to be very mindful. We have to make sure all the team members are aware of the history of pulmonary hypertension, the specific medications the patient is on, because any piece of the puzzle that's left out will result in the patient with pulmonary hypertension not doing as well as we would like them to. So it is a very complicated situation. We want to take it very seriously. We want patients to be ready for these situations, and we want to work together with a team, including the ICU physicians, the ER physicians, the surgeons if we need them, the cardiologists and the pulmonologists to get the best outcome. My name is Dr. Jean Elwing, 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/36413870

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