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

Raj Parikh, MD, MPH - phaware® interview 517 04/22/2025

Raj Parikh, MD, MPH - phaware® interview 517 Dr. Raj Parikh from Hartford Hospital discusses the development of the PH-ILD Detection tool, a screening tool designed to help detect pulmonary hypertension (PH) in patients with interstitial lung disease (ILD) at an early stage. Early detection is critical, as there is often a significant delay in diagnosis of PH in ILD patients, leading to worsened outcomes. This Special Edition episode is sponsored by . My name is 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 want to talk to you about the PH‐ILD Detection tool that Hap Farber, MD and I have been working on for a couple years. Now, it's in the community. We're encouraging all interstitial lung disease, ILD specialists, general pulmonary docs who see ILD patients to really use this as a way to work on early detection of pulmonary hypertension in interstitial lung disease patients. The reason behind developing the PH‐ILD Detection tool was to really focus on early detection and early screening for pulmonary hypertension that's associated with ILD. The impetus behind that was the development of the first and only FDA approved therapy for PH-ILD, which is inhaled Treprostinil. That was FDA approved back in 2021 after the INCREASE clinical trial. The PH-ILD Detection tool, the real spirit behind it is patients with interstitial lung disease that develop pulmonary hypertension carry extremely poor prognosis. They need more oxygen. Their quality of life is worse. Their longevity of life and their mortality is worse, as well. These are things we know. We've studied the PH-ILD population for years and years. This is well-established stuff. The key now that we have a therapy that can A, improve the patient's walk distance and therefore their quality of life. B, keep them from having any sort of clinical worsening like ending up in the hospital or having more right heart failure. These are all findings based on the INCREASE trial. So the key really is to detect the pulmonary hypertension as soon as possible in ILD, not just screen for it once a year, or test for it when you meet the patient for the first time and then test for it again every five years. This is not like colon cancer screening or lung cancer screening. This is active assessment every time you see an ILD patient, you should be thinking, “Okay, do they now have signs of pulmonary hypertension? Should I now be concerned about pulmonary hypertension or should I not be?" There is no standard protocol or standard practice when it comes to screening and detecting for pulmonary hypertension in ILD patients. Back in 2022, Hap and I really developed eight parameters for PH-ILD detection. We base these parameters off of things that you should routinely already be using to assess a patient's interstitial lung disease. We're talking about 6 minute walk tests, physical exam, pulmonary function testing, assessing whether the ILD patient needs oxygen or not, blood tests, CAT scans, and their prior history and comorbidities. If we take these eight parameters and we assess, okay, which parameters have a higher weight compared to lower parameters that have maybe not as much of a weight in terms of predicting for pulmonary hypertension? We took these eight parameters and basically created a 12 score tool. The score can be anywhere from zero to 12. Four parameters have a weighted score of two points. Four parameters have a weighted score of one point, therefore equaling a max score of 12 points. Basically, depending on what you score from zero to 12, you are stratified into either low risk for pulmonary hypertension ILD, intermediate risk for PH-ILD, or high risk for PH-ILD. A score of three or below is low risk. A score of four to five is an intermediate risk. A score of six or higher is actually high risk for PH-ILD. What does that mean? What does it mean if you're low versus intermediate or high? Well, that should really dictate what are your next steps. If you're low risk, you should reassess the patient at your follow-up visit, whether that's at three months or six months or whatever. If you're intermediate risk, you should get a screening echocardiogram for that patient to make sure there's no signs or development of pulmonary hypertension or right ventricular disease on that echo. If you're high risk, that's a score of six or higher on the PH-ILD Detection tool, you should not only order an echocardiogram, but you should be calling your nearby your local pulmonary hypertension specialist or pulmonary hypertension center and say, "I have a patient with interstitial lung disease. I have a high suspicion for pulmonary hypertension, can you get them into your clinic? Can you get them in for a right heart catheterization for a definitive diagnosis?" When we developed this score, we not only first developed it internally retrospectively, but once it was initially published, we discussed it and presented it at an oral presentation at CHEST back in Nashville, so that was a couple of years back. We then actually had seven centers used the score prospectively, and we validated the score by doing a multi-center validation cohort study. That study actually upheld a very significant area under the curve, very significant sensitivity and specificity for a score of six or higher to really strongly predict pulmonary hypertension in interstitial lung disease patients. As a result of this, we've been working on promoting the use of this PH-ILD Detection tool with local ILD specialists, local general pulmonologists. We use this score not only to predict or increase suspicion for pulmonary hypertension, we've done some other data and we actually presented this information at CHEST, this past year at Boston, where the higher the score you have, the worse your right heart cath hemodynamics are, and therefore the worse your prognosis may be. Some of that is almost common sense, but it's nice to kind of see pen to paper and see that correlation, as well. We've actually been working on seeing if we can create an app for this score. Now, Hap and I are creating an abbreviated version of the PH-ILD Detection tool to take it instead of eight parameters with 12 points, can we make it three parameters? Can we simplify it even further and make it as simple as possible so that the community pulmonary doctor, the community ILD specialist -- all of them can really focus on how simple it can be to detect and screen for pulmonary hypertension. Mostly because we know that once patients develop PH with ILD, the prognosis is so poor that we want to really act fast. We want to get them diagnosed soon. If we can diagnose them quickly, we can treat them with inhaled Treprostinil. We can mirror some of those outcomes that we saw with the INCREASE clinical trial, and get these patients having a better quality of life, better walk distance, lower proBNP scores, keep them out of the hospital, so on and so forth. We know from PAH pulmonary arterial hypertension and the REVEAL registry, there's almost a three year delay in diagnosis from the time a patient develops cardiopulmonary symptoms to the point B, which is when they actually have a formal PAH diagnosis. Three years. Those three years are met with seeing different specialists, being in and out of the hospital, having a worsening quality of life because they’re always short of breath and overall just poor more prognosis. If we take that, do we have that exact number when it comes to PH-ILD? No, but we do have an extremely wide range of epidemiology when it comes to PH existence with ILD. PH can range anywhere from 12% to 86% in ILD patients. Why do you have such a wide range? That's because we don't know when to look. We don't know how to look. A lot of times we don't even look and we're getting a lot of that data that 86% is sometimes showing up on autopsy results and transplanted patients. Then, we're looking back at those autopsy or transplanted lungs and saying, "Oh wow, those patients not only had ILD, they actually had PH as well." That's not something to necessarily be proud or happy about. So it kind of is in the same boat as, what is our delay in diagnosis? What is the window of opportunity that we need to really diagnose and intervene so we can make a difference? There will be a point of no return when it comes to ILD patients with PH. The same way there is sometimes a point of no return with PAH where the patients get so advanced that they're meeting them in the intensive care unit and they're meeting them really on what we call rocket fuel. These patients are looking for the Hail Mary to get better. You're not meeting them in the office, you're not trying to do a 6 minute walk test on them these patients can't even do a 6 minute walk test when they're sometimes at that point of no return. So really what is the sweet spot? I think it is trying to mirror what we went through with PAH several years ago. Developing a registry, understanding more of the referral patterns for PAH and we're trying to extrapolate all of that information and bring it now into this PH-ILD space, as well. I know I'm speaking to a lot of the providers and doctors who are involved with taking care of ILD patients, but really switching gears and thinking about it from the patient perspective. If you're a patient who has been diagnosed with any of the many subtypes of interstitial lung disease, I think it's important to be aware of this concomitant process that can occur and that can develop, which is pulmonary hypertension. It is difficult to tease out when you have worsening cardiopulmonary symptoms, worsening breathing symptoms. Is that the interstitial lung disease? Or is that something new such as pulmonary hypertension? I think it's important to think about that and be open with your provider, with your specialist, with your pulmonologist and say, "I'm now feeling this way, and I'm now using this much more oxygen, and wasn't my 6 minute walk distance this far last time and now it's down to this time? Could this be something else? Is this just not necessarily worsening interstitial lung disease or is this a second process?" A second process that now we know we can treat and we can make better. I think that's important to sort of think about. You can be your best advocate when it comes to going into your provider's office and really telling them exactly how you're feeling, and what you're feeling, and how it's different from prior. 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 #PHILD /episode/index/show/phawarepodcast/id/35840550

Episode 517 - Raj Parikh, MD, MPH 04/21/2025

Ron Zolty, MD - phaware® interview 516 04/15/2025

Ron Zolty, MD - phaware® interview 516 Dr. Ron Zolty delves into groundbreaking advancements in treating pulmonary arterial hypertension. From the historical use of vasodilators to the revolutionary promise of sotatercept and seralutinib, Dr. Zolty unpacks how science is moving closer to halting—and possibly even reversing—PAH. This Special Edition episode is sponsored by . My name is Ron Zolty. I'm a cardiologist working in advanced heart failure and heart transplantation. In my training, I had to do a lot of catheterizations in patients with pulmonary arterial hypertension. I trained at the University of Colorado in Denver, which is considered one of the best centers in pulmonary arterial hypertension. Working with patients in the cath lab, I fell in love with PAH, now leading a pulmonary hypertension program here in Omaha, Nebraska. Today, I would like to speak about some innovations in the field of pulmonary hypertension. As you may know, pulmonary arterial hypertension is a progressive, lethal disease. Patients, unfortunately, die from pulmonary hypertension if not treated. We should go back a little bit to the pathogenesis at the mechanism of what we believe causes pulmonary arterial hypertension. We know that when patients suffer from pulmonary arterial hypertension, they have what we call endothelial disruption. Endothelial is the inner layer covering the vessel of any vessel and especially the pulmonary arterial tree. If you have any damage of this endothelial layer, you are going to make some small thrombi, some small clots. One of the initial treatments of pulmonary hypertension was in the eighties to treat these patients with anticoagulation. Unfortunately, there was never any prospective study looking at the benefit of anticoagulation, only retrospective studies. Most of them showed that there is some benefit of anticoagulation blood thinner. However, because there was never any prospective study, it's just a 2b indication, not everyone needs to be on anticoagulation because again, it hasn't been shown really to improve survival in patients with PAH. The second main phenomenon that patients have with pulmonary hypertension is vasoconstriction. Vasoconstriction, meaning that the vessels tighten when you do have the disease. That's the reason for the three classic pathways we treat today. Pulmonary arterial hypertension, we know that patients with PAH have too much endothelin, which is one of the most potent vasoconstrictors we have in our body. So we block this endothelin causing less vasoconstriction and vasodilator effect. We also know that patients with PAH don't have enough prostacyclin, so we do substitute this lack of prostacyclin with some prostacyclin. We do have IV, inhaled, and oral prostacyclin to give to patients. The third pathway, which is also a vasodilator pathway, is the lack of nitric oxide. Patients with pulmonary hypertension have a lack of nitric oxide, and therefore we give them a PDE5 inhibitor like sildenafil or tadalafil or a SGC stimulator like riociguat to increase the nitric oxide pathway, increasing cyclic GMP. Cyclic GMP, really vasodilates. But all these therapies, these classic therapies do not stop the progression of the disease. They slow down the progression of the disease without stopping the disease. We also know, and that's the last point and the newest point I want to make, when patients do have pulmonary arterial hypertension, the wall of the vessel gets thicker. If the wall gets thicker, the lumen where the blood travels gets smaller and smaller. That's the reason why patients get less blood, less oxygen, less hemoglobin, less oxygen, more shortness of breath. This thickening of the wall gets what we call an increase in the resistance, leading to right side heart failure. About 15 years ago, there was a paradigm shift in the understanding of pulmonary arterial hypertension thinking, why do these patients have a thickening of their wall? It's because there is proliferation of endothelial cells, smooth muscle cells like neoplasia, like a tumor. Maybe if we block this progression, this proliferation, we may stop the disease and maybe even regress the disease in making the wall thickness less thick and smaller. Therefore, about 15 years ago, there was a very interesting clinical trial with a drug called Imatinib or Gleevec. Gleevec has, for patients with chronic myelocytic leukemia has been a revolution. Patients with CML in the past needed to have a bone marrow transplant. With the administration today of Gleevec it’s been a revolution and patients can live with CML, with this chronic myelocytic leukemia for years and years with absolutely no progression of their disease. Similarly to CML, the idea was maybe if we give an agent like Gleevec or Imatinib to these patients, we may stop the proliferation of endothelial cell and smooth muscle cells in the wall of the vessel and stop or even regress the disease. This trial, called IMPRES, was done about 13 years ago. The results of the study were absolutely beneficial. However, more than 30% of patients quit the study because of side effects, and also patients on anticoagulation, on blood thinners developed some intracranial hemorrhage. The FDA, because of the side effect, decided not to approve this drug for pulmonary hypertension. Just to give you a background that there is really a paradigm shift in the understanding of the disease. The new drug that has recently been approved by the FDA is being called sotatercept. That's another mechanism. We know that in our system, every cell has like a yin and a yang. There is activin, a system which stimulates the gene while the bone morphogenetic protein system is slowed down. The activin slowed down the proliferation of cells. In our system, in a normal system, you do have a balance between BMP and activin. We also know in patients with pulmonary hypertension, they do have too much activin. This new medicine, sotatercept blocks the activin. Activin is like an accelerator. It does promote proliferation of cells on the gene. If you block the activin, you recalibrate the BMP and the activin, reducing this proliferation of cells. Sotatercept was shown really to improve patient pressure as well as improving their six-minute walk distance as well as their quality of life. Sotatercept has been approved by the FDA for the last year and really has changed the field of PAH. Another group similar to the Gleevec, but as I said, Gleevec was not approved by the FDA because of the side effects. The idea was maybe if we could give a Gleevec as an inhaler without systemic effect, patients would absorb the drug into the lungs without having any side effects. There was a clinical trial with inhaled imatinib, inhaled Gleevec if you want. Unfortunately, the study was not positive, because most likely Imatinib was not absorbed by the lungs. However, another company was able to produce a super imatinib, which is being absorbed by the lungs called GB002 or showing a significant improvement in the pressure, as well as the PVR. Currently, there is a with this drug as an inhaler. So, in summary, I just want to say the drugs we have currently until now were vasodilators, they do slow down the progression of the disease but don't stop the disease. Now, we do have one drug approved by the FDA. Hopefully, a second one will be approved. These two drugs stop the proliferation that occur in pulmonary hypertension and hopefully will stop the disease and potentially even regress the disease. We are getting closer to maybe a cure of the disease with agents like sotatercept and . Thank you for listening. My name is Ron Zolty 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 #clinicaltrial #PROSERA /episode/index/show/phawarepodcast/id/35842570

Episode 516 - Ron Zolty, MD 04/14/2025

Rajan Saggar, MD - phaware® interview 515 04/08/2025

Rajan Saggar, MD - phaware® interview 515 For decades, researchers have sought better treatments for pulmonary hypertension in interstitial lung disease, a condition that drastically impacts patients’ quality of life. In this episode, Dr. Rajan Saggar dives into the latest advancements, including new inhaled medications, upcoming clinical trials, and the push for more personalized treatment approaches. This Special Edition episode is sponsored by and . I am Dr. Rajan Saggar. I'm Co-Director of the Pulmonary Hypertension Program at UCLA, and my Co-Director is Dr. Richard Channick. We work at that program together. One of my main interests, actually, is pulmonary hypertension, complicating interstitial lung disease. Sometimes, interstitial lung disease can progress and become pulmonary fibrosis. So, along that spectrum of disease, pulmonary hypertension, which is Group 3. There's basically five types of pulmonary hypertension. There's five different groups. The one related to lung disease ... and again, that lung disease could be COPD or emphysema, or it could be interstitial lung disease - pulmonary fibrosis. Any type of lung disease is considered Group III pulmonary hypertension. The focus that we have right now is specifically with patients who have interstitial lung disease or ILD and pulmonary fibrosis. This is a field that I've been interested in for a couple of decades. There's been a lot of failed studies over the years. There's been a lot of attempts to use drugs that have clearly worked and are now FDA approved for the much rarer form of Group 1 pulmonary hypertension, which is also called pulmonary arterial hypertension. Those drugs, which are now FDA approved and thriving and doing well have been used or tried to be used in the Group 3 pulmonary hypertension setting. Unfortunately, the majority of them have failed. But the good news is recently, as of April of 2021, there is a medication that now is FDA approved, specifically pulmonary hypertension in the setting of interstitial lung disease or what we call PH-ILD. That is inhaled treprostinil. It's an inhaled medication that's taken four times a day. What's exciting now is that that started off a chain of events, which I think re-lit interest in this type of pulmonary hypertension. Now, there's several pharmaceutical companies that have shown interest, and more than that, have actually started clinical trials looking at the potential for other inhaled therapies. I say inhaled, because most of the therapies being looked at are inhaled therapies. The rationale there being you have a lung disease, specifically affecting the lung and you happen to have associated pulmonary hypertension. The idea being that if you inhale something as opposed to taking something orally or something through the vein, you might have more of an effect. You're delivering the drug to the area of interest. There's a lot of people and rationale for doing that and maybe sparing patients from the side effects of these drugs, when you inhale it, as opposed to giving it what we call systemically, either orally or through the vein. There's a lot of interest in the inhaled approach. Another product being looked at, and the reason I'm bringing it up is it's maybe close to being available and being launched, is a drug called Yutrepia. Again, another formulation of inhaled treprostinil made by a company called . We are recording this before the potential for launch, but it's possible that this drug could be launched as early as May of 2025. Again, the potential benefits of that drug, because one could ask, "Well, what's the point? You already have an approved medication that's inhaled treprostinil, as well." The touted benefits of that drug may be a decrease in side effects, particularly cough, which may allow more up-titration of that particular medication in terms of actual absolute dosing. What we do know is that more is better of this medication, inhaled treprostinil, in the setting of PH-ILD. If we can get to higher doses without having side effects, that would be the ultimate goal. Then, even a further goal would be to do that with, let's say, once a day dosing, for instance, to make the frequency less. That would be pie in the sky. But the point is there's motion here and there's movement, so we look forward to that. There's another formulation of inhaled treprostinil being looked at, which is by a company called Insmed, and they're also looking at a once-a-day formulation of inhaled treprostinil. Again, specifically in pulmonary hypertention with interstitial lung disease. That's not as far along as the Liquidia product, but that's also exciting, because again, you would be going to once a day, which would be a game-changer for a lot of patients. There's other companies looking at other formulations of inhaled treprostinil and trying to make that better, if you will. The reason I say that is because the current approved medication, inhaled treprostinil, is a drug which is four times a day and also can have cough associated with it, which is not atypical for an inhaled medication, but a lot of these patients have cough even from their interstitial lung disease to begin with. So, when we introduce a medication which sometimes exacerbates their pre-existing cough, it can become quite difficult. At four times a day, it can be a little cumbersome. There are other companies trying to improve the side effect profile perhaps, but also the frequency of how often they take it, trying to build on what's already out there. In addition to reformulating the treprostinil product there, there's also other companies looking at other mechanisms, just brand-new mechanisms. For instance, there's a drug called , which is made by . This is a drug also inhaled, a dry-powdered inhaler, which blocks certain molecules in the body called tyrosine kinases. It blocks several of these and has a lot of promise in PAH, so that's Group 1 pulmonary hypertension, but they've extrapolated that and now are looking at it actively in Group 3 pulmonary hypertension, specifically this PH-ILD, pulmonary hypertension with interstitial lung disease. That's exciting. The other medication that's being looked at is a drug made by , which is called , which is reminiscent of another pulmonary hypertension medication that we have in Group 1 disease, which is called Riociguat. But this is specifically being looked at, much like the Seralutinib product is being looked at in both Group 1 pulmonary hypertension as well as Group 3 pulmonary hypertension. Again, specifically pulmonary hypertension with interstitial lung disease. So again, you can see that the people are making newer medications not just for the Group 3 necessarily, but they're looking at it in Group 1, the more traditional pulmonary hypertension, the pulmonary arterial hypertension, but also looking at it in Group 3 pulmonary hypertension. That product with Pulmovant is also an inhaled medication. There is a protocol in motion, as we speak, looking at PH-ILD, which is exciting. It's a guanylate cyclase activator. It works in the nitric oxide pathway, which has been an old pathway used in pulmonary hypertension. It has some interesting benefits that are touted in terms of how it works in that pathway specifically. But without getting into the details, it is an old mechanism, but looking at it with new parts, if you will. That's Pulmovant. Again, that's their new medication, Mosliciguat. That's out there, as well. There's some other ones in the pipeline that are not as far along. There is a aldehyde dehydrogenase stimulator, which is being looked at. Again, very early. There's a drug that blocks hyaluronan synthesis, which is a drug that's coming out of Stanford actually, which is also very early in the mix. Nothing really ready for patients to engage in just yet. But again, the pipeline's pretty deep. It's very exciting because pulmonary hypertension with interstitial lung disease has really been a type of pulmonary hypertension yearning for a drug that we can actually give patients and actually get some relief. It turns out that this particular type of pulmonary hypertension, it causes a lot of illness, much more than the other types of pulmonary hypertension. These patients are unfortunately dealing with pretty much two lung diseases, they have pulmonary hypertension and they have this interstitial lung disease, so they tend to need oxygen. They tend to be very functionally limited. As anyone knows, living with oxygen in the house is hard enough. Leaving the house with oxygen, it becomes extremely difficult. Unfortunately, us in the respiratory world and the pulmonary world, we haven't really figured out how to best give oxygen outside of the house just from a comfort standpoint, and just making it easy for patients. It's still, I think, relatively cumbersome. I think that's another area that we should be focusing on to make living with oxygen easier, particularly outside of the home. So yeah, I'm happy to report that there's a lot going on in this field. From a clinical trial standpoint in the PH-ILD space, luckily so far, knock on wood, there's been sort of minimal overlap in terms of the trials being available. In other words, we don't have two trials or more going on at the same time for PH-ILD. For instance, I mentioned the Liquidia product, Yutrepia is farther along, so their study is finishing while the Pulmovant study with Mosliciguat is ramping up. So luckily, we haven't had the problem of having to try to figure out which study to go into, or which one to consider, or having choices in that matter. That really hasn't happened just yet, but I suspect it will, given the pipeline we just discussed. At that point, I think it's just an individual patient discussion, one-on-one discussion with the pros and cons, and probably has a lot to do with the side effects they're dealing with at some point, or inclusion/exclusion criteria. I think it gets into some of the details that we'd have to have an individual discussion with that particular patient to make the best decision with them. From a physician standpoint and trying to understand disease, any disease, not just pulmonary hypertension with interstitial lung disease, once we get more and more into it, we often start realizing that things can't just be lumped. Patients can't just be lumped into one category that you have PH-ILD and everyone will respond to drug X or drug Y. We all know that not everyone responds to available therapies. Or if there is response, there's a spectrum of response. Some are no response, some have mild response, some have significant response. But what makes this even more, I guess in some ways, difficult, but also exciting from a medical standpoint is that we are learning that there is an extreme amount of heterogeneity within PH-ILD. By that, I mean there's so many different causes and etiologies of ILD, and each of those patients with ILD has a different natural history of that specific ILD in terms of how it progresses. Some people are much more stable than others. Some people are progressively getting worse faster than others, and it has a lot to do with the type of ILD. Then, when you throw the pulmonary hypertension on top, the pulmonary hypertension itself has a heterogeneity to it in terms of how it progresses. Does it stay stable or does it tend to progress? Then, you can understand that when you're dealing with two diseases which are heterogeneous in their natural history, then it becomes a little bit more difficult to understand in terms of lumping people in one category. That's important, because the physicians in this space, and I being one of them, one of our tasks is to make sure that we understand what we call phenotypes. A patient's phenotype is not always the same with PH-ILD. Phenotype means the set of characteristics specific to that patient. There'll be patients just like that patient that fit into a certain phenotype. There's going to be multiple phenotypes in this PH-ILD space. We're learning about those. The reason it's important is because not only do they have different rates of progression or changes in their ILD and PH, but it also predicts who's going to need a lung transplant sooner perhaps, or who may be more responsive to a medication. Perhaps a patient who has idiopathic type of disease responds better to something than someone who has a connective tissue disease or vice versa. Those are the things that we need to learn about, so then we can inform patients better. Sometimes, that might trigger us off into saying, "Hey, you probably should think about this clinical trial, because the data with your type of phenotype may not be as predictable or as wonderful as we want." That's just the reality of the situation. Some people are going to have better responses than others. But that's important for us to identify that a priori so we can actually have a discussion with some real data so the patient can be informed, so we are not just making up stuff. We want to actually have real data, that hard data so we can actually help the patient make the best decision. I think that's really important. I'm Dr. Rajan Saggar, Co-Director of the Pulmonary Hypertension Program at UCLA in Los Angeles, California. 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 #PHILD #PROSERAstudy #clinicaltrial #PHOCUSstudy /episode/index/show/phawarepodcast/id/35843325

Episode 515 - Rajan Saggar, MD 04/07/2025

Eric Austin, MD, MSCI - phaware® interview 514 04/01/2025

Eric Austin, MD, MSCI - phaware® interview 514 Eric Austin, MD, MSCI discusses early detection among people who are at risk of pulmonary arterial hypertension (PAH), particularly those who would have genetic susceptibility, or who may have congenital heart disease, or who may have connective tissue disease or other features that would make them more at risk of developing pulmonary arterial hypertension than the rest of the population. My name is Dr. Eric Austin. I'm a physician and physician scientist at Vanderbilt University Medical Center. I direct the Pediatric Pulmonary Hypertension Program at Vanderbilt and have done so since 2012. I have studied children and adults with pulmonary hypertension, in particular pulmonary arterial hypertension, but other forms as well, for nearly the last 20 years, with a particular interest in people who are at risk of pulmonary arterial hypertension for various reasons or are resilient to its development for reasons. I have great interest in the topic at hand today – early disease detection among people who are at risk of pulmonary arterial hypertension, particularly thinking about people who would have genetic susceptibility, or people who may have congenital heart disease, or people who may have connective tissue disease or other features that would make them more at risk of developing pulmonary arterial hypertension than the rest of the population. When you think about people who are at risk of developing pulmonary arterial hypertension, the most commonly thought about group in the current era are individuals who actually have familial disease; that is to say they have pulmonary arterial hypertension, or PAH, that occurs in families. This would be two or more family members with truly documented pulmonary arterial hypertension. We and others here at Vanderbilt and others around the world have been interested in studying families who are impacted by pulmonary arterial hypertension as a way to investigate the factors that lead to disease expression or presentation of PAH among people who are at risk. Years ago, in 2000, the initial genetic discovery was the association of bone morphogenetic protein receptor type II, or BMPR2 gene, with PAH. This association was discovered by several groups working in concert and several groups working independently, who discovered that single-gene mutations or rare variations in the BMPR2 gene can associate with pulmonary arterial hypertension. I intentionally said associate, because while I think they cause PAH, we don't actually definitively know that those mutations, ”cause pulmonary arterial hypertension.” We just know that beyond a shadow of a doubt, families with BMPR2 mutations are much more likely to have people with PH in them than the general population. Other genes have emerged over the past 20 years associated with pulmonary arterial hypertension, including mutations in the gene TBX4, mutations in ALK1 and endoglin, which actually also associate with hereditary hemorrhagic telangiectasia, SOX17, and several other genes. There's a smattering of genes that are now known to put a person at risk of developing pulmonary arterial hypertension. You may ask, "Well, what's the risk?" Well, that's the hard part. If you look at families who have individuals who have mutations but are healthy, it does appear that not everyone with a mutation in the genes that we think essentially “cause” pulmonary arterial hypertension will ever develop pulmonary arterial hypertension in their lifetime. Stated another way: a person with a mutation in one of the ‘PAH genes’ will not necessarily develop PAH. Most of this data actually is from BMPR2 mutation-carrying families, because mutations in this gene are by far the most common--about 75% of families that have PAH due to BMPR2 gene mutations. So most of our data emerges from those people. We know that on average, about 20% of all people who carry one of those mutations will develop PAH in their lifetime. But that's not a yearly risk; that's in their lifetime. We also know that mutation-associated PH can develop not only at any point in the life; but, it's actually different from men and women. People of female sex are much more likely to develop PH than those in male sex who have BMPR2 mutation. In fact, it's about a 42% lifetime risk for a female to develop PH if they have a BMPR2 mutation and about a 14-16% lifetime risk for men. For reasons we don't totally understand yet, although a lot of work has gone into this, as you can imagine, BMPR2 mutations don't equally cause PH between biologic females and males. That's a point of interest that makes you think, "Well, are the people of male sex resilient in developing PH, or are the females more susceptible, or somewhere in between?" It's probably somewhere in between, but we don't know for sure. You may then ask, "Well, what is the risk of a person to develop it in a given year if they have a mutation?" I told you there's a lifetime risk of about 42% for females and about 16% for males, but what is the yearly risk? Some of that work has now emerged from groups in Paris and in the Netherlands who have been studying individuals who have pulmonary arterial hypertension in families. Recent data from the French registry of these individuals suggests that about 2 to 3% of people per year in their cohort convert and develop PAH every year. If you take a group of people and they say to you, "Well, what's my likelihood of developing PH in the next year," it's probably about 2% to 3%. We don't know specifically yet. That's the best indicator we have. Here at Vanderbilt, we follow about 250 families who have pulmonary arterial hypertension in their family, and many of those have a BMPR2 mutation, and that's probably about the data that we have as well, though we have not published that. We also are now excited to be studying individuals who have BMPR2 or other mutation-associated PAH and then their family members who have those mutations but are healthy. We call that the RARE study, R-A-R-E. It's funded by the National Institutes of Health. I'm pleased to work on this with my collaborators and friends, Drs. Anna Hemnes and Evan Brittain, where we're studying people who have PH in the family, looking at healthy and unaffected as well as PH-affected people. We're interested not just in what makes the person who has the disease develop the disease, we're interested in what helps the person who is healthy stay healthy. By studying genomic and transcriptomic information, which tells us about their DNA and RNA, and then protein-based factors in their blood that may ultimately associate with whether they develop the disease or whether they stay healthy, we hope to learn more about ‘resilience’ to and ‘susceptibility’ to PAH. We hope that one day if we can figure out what factors are more present in the healthy people who should be sick and are less present in the diseased people who should not be sick, we can harness that to both prevent disease, but also maybe someday develop treatments built around that. You can imagine if there's something that... We find a protein that really helps someone stay resilient to or prevents them from developing PH even though they ought to have it, what if we could harness that as a therapy in the people who already have the disease? We're really interested in this notion, both here at Vanderbilt and around the world, of using people who have genetic susceptibility to understand the disease better and to understand how to prevent it and how to treat it better. You see a great example of this in the recent approval of sotatercept in the EU and in North America by the FDA. The drug sotatercept really emerged because of the genetic discoveries around BMPR2 and the associated pathways that go with it. We think that there's a lot more opportunity to learn a lot more from our genetically susceptible and genetically resilient people. There are also other forms of disease that associate with PAH. We know that individuals who have an illness known as HHT, or hereditary hemorrhagic telangiectasia, we know those people also are at higher risk of developing PAH in their lifetime. It's still quite rare for those people. It's not a common presenting symptom, but all of our PH clinics have multiple people with PAH and HHT, so it's common enough that we see at a fair amount. There are actually two different genes that most strongly associate with HHT, and that's endoglin and ALK1, or ACVRL1, and a few others. You probably don't care the names about them per se, but just to know that there are families of individuals who have HHT that courses through the family, and those people are also at risk of PAH. For people who have genetic susceptibility, whether it's HHT families or families with BMPR2 or other gene mutations, there's a little bit of controversy onto how often to screen the healthy people for pulmonary arterial hypertension. You may ask me, "Well, what do you do?" Most people would recommend that first-degree relatives, that is a mom, a dad, or a son or daughter, or a sibling, of a PAH patient due to a mutation like BMPR2 or HHT PH be screened every one to three years with a clinical evaluation and an echocardiogram for pulmonary arterial hypertension. I told you earlier, I direct the Pediatric PAH Program here at Vanderbilt, and here at Vanderbilt we actually tend to see the children who are at risk about every year with a clinical exam and an echocardiogram. I also like to get spirometry with DLCO to look at their diffusion capacity. If they seem at all impaired, we'll do a full six-minute walk test and lung volumes as well. But what we're doing is we're trying to screen them and perhaps detect PH earlier. If their echocardiogram is abnormal, you can imagine we would activate a full PH workup, including ultimately cardiac catheterization, because we think that earlier therapy will probably lead to better outcomes. We don't actually have that definitively proven, though, in clinical studies. But we also know that every year we have people who have syncopal events or pass-out events or other dangerous events that occur with their pulmonary arterial hypertension. I would be devastated to know that someone was at risk and we never looked for PH and then they died or had a bad event in the community because they passed out. So we screen our people who are children every year. In the adult world, you may see it more commonly every one to three years of screening those at risk. Another form of at-risk population would be the individuals who have connective tissue disease. The most common connective tissue disease that we know associates with pulmonary arterial hypertension is scleroderma or systemic sclerosis. But you can actually see PH in other forms, including lupus, mixed connective tissue disease and the like. So individuals, and they're usually but not always adults, who have connective tissue disease but are not known to have PH should be screened for pulmonary arterial hypertension every one to three years as well with a clinical evaluation and echocardiogram. Now, the reality is if you have connective tissue disease and you're well-connected, you're probably seeing your provider every three to six months, and part of the evaluation at every visit is considering progression of disease; is it possible the person has a new diagnosis of PAH? It's not extremely common in those groups to develop PH, but it certainly can happen. They can also develop pulmonary hypertension due to what we call Group III PH, which would be more lung-disease-driven, perhaps due to interstitial fibrotic changes in their lungs that can cause low oxygen tension and can cause PH, as well. So it's not always a pure pulmonary arterial hypertension Group I phenomenon. That's a quick once-over of the main types. The other things you see in children but not as much in adults, although you certainly can, is congenital-heart-disease-associated pulmonary arterial hypertension. So people who maybe had profound severe congenital heart disease that was operated on when they were young, such as a tetralogy of Fallot, or people who have less severe congenital heart disease, such as an atrial septal defect or a ventricular septal defect that's being monitored or was repaired, can all develop PAH at any point in their lifetime, as well. There's not strict guidelines for pulmonary arterial hypertension or pulmonary hypertension screening for repaired individuals who have had congenital heart disease in the past, but certainly if you were a provider and you saw a person with a history of congenital heart disease repaired that presents with unexplained shortness of breath or difficulty in breathing or other features that could be suggestive of pulmonary hypertension, then you would certainly want to activate some sort of more aggressive screening evaluation for pulmonary arterial hypertension for those people. Lastly, this is a group that may be less likely to seek care until they're very sick, or they may be depending on the scenario, would be people who are using recreational or other forms of drug use, such as amphetamines or cocaine. Those people are at higher risk of developing pulmonary arterial hypertension. So too are people who use the dietary drug fenfluramine, which has largely gone away in the United States and in Europe, but is known to associate with pulmonary arterial hypertension. This is not the diet drug that you're seeing now that's so popular across the world right now. Ozempic would be a common name for semaglutide and related GLP agonists. Those drugs do not, to our knowledge, associate with pulmonary arterial hypertension. But a traditional weight loss drug called fen-phen, or fenfluramine, did associate with pulmonary arterial hypertension. We still see it's used sometime among people with seizure disorders, by the way. I recently had a patient with this exposure, and we decided to stop that for safety reasons for that person. If you think about people who are at risk of developing pulmonary arterial hypertension, as I mentioned before it's pretty rare. Thankfully there's a lot more common diseases out there than pulmonary arterial hypertension. But it's really challenging to develop that disease because pulmonary arterial hypertension can significantly impact someone's quality of life and their survival over time, and so we like to be vigilant about it. If you ask the question, "How would one use the information we know about people at risk in a day-to-day basis," what I would say is that certainly for those individuals who are in a family known to have people with PAH in the family, two or more people in particular, of pulmonary arterial hypertension, it would be great for the family to be aware of it as a group and understand that, “Hey, cousin Jimmy died of pulmonary arterial hypertension and his son or his daughter also has it. I should think that if I ever feel short of breath, or if my daughter ever feels short of breath or has difficulty with walking or not really the same cardiovascular fitness that one would anticipate, that person should go be screened and seen by a medical professional.” One great way is to be aware of it on your own. It can be hard for families, because sometimes some of the people who are impacted in the family may be multiple branches of the pedigree or multiple branches of the tree away from the person who's actually at risk; that can happen. For individuals who have other risk profiles, like if they have connective tissue disease, if they have congenital heart disease, it's that partnership with your provider or your physician or your nurse practitioner or your PA or whoever you're working with for your care, and both of you together being aware that not only are you being cared for your current problems, but being vigilant for the associated conditions that can occur in the setting of your current diagnosis. If you have connective tissue disease, having a good discussion with your provider about, "Hey, Dr. so-and-so, thank you so much for your care of my rheumatoid arthritis, but what other things should I be thinking about as we move along through life? Do I need screening for pulmonary hypertension? Do I need screening for such and such?" Having that dialogue with your provider can be really helpful. Obviously, we're hopeful that would never actually come about and you would not be impacted by PH, but we would want to be on top of it in the event that you did have that happen. A thing that we're finally becoming a little more aware of in the PH community is the incorporation of people of all backgrounds, shapes, and sizes. For whatever reason, for many years, a lot of the individuals who we were studying at least, and even the people in our clinic, skewed toward a Caucasian population. But we've recognized in the last 10 years in particular that that needs to be rethought and more sensitively thought about. There are people in disadvantaged groups, individuals who are native people of the region, individuals who have African ancestry, individuals from LGBTQ+, who may not be seeking care or receiving care with the frequency with which other populations are. Many of us have worked with many groups to improve our distribution of information about pulmonary hypertension across all providers and into populations of individuals, as well, because that is a really important thing. I know Dr. Vinicio de Jesus Perez at Stanford has worked very closely with disadvantaged people across the world, which is another issue that's important. The PVRI (Pulmonary Vascular Research Institute) has been working on this ,as well, spreading the information about screening and about notification of PH and awareness both within North America, but also around the world in places that may be less able to access care or less able to access screening. Right here at home, I think we've done a much better job in the last 10 to 15 years of being aware of this and made sure that we are getting into all communities. Just like any other medical condition, if you don't have access to the care or you're not receiving care that's aware of the problem, you can miss it. I think through the years we've been not as aware of that as a group as we can be, and we've seen a real improvement of that over the last 10 years or so. I absolutely know that I have learned a lot about that, and so that's a really important point and it’s something that we, as providers, need to be thoughtful about. My name is Dr. Eric Austin, 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/34719895

Episode 514 - Eric Austin, MD, MSCI 03/31/2025

Kristin Highland, MD - phaware® interview 513 03/25/2025

Kristin Highland, MD - phaware® interview 513 Kristin Highland, MD, from the Cleveland Clinic discusses the importance of shared decision-making and patient preferences when treating patients with pulmonary hypertension. Dr. Highland, emphasizes that the patient is the true expert on their own body and that it is crucial to understand their perspective when making treatment decisions. She highlights the need to consider factors beyond just clinical outcomes, such as side effects, costs, and the patient's ability to manage the therapy. She underscores the evolving role of patient-reported outcomes in medical decision-making and the need for open communication between providers and patients to ensure individualized and preference-aligned care. I'm Kristin Highland. I'm a pulmonary critical care physician at the Cleveland Clinic in Cleveland, Ohio. I'm also trained in rheumatology. My focus has been rheumatic lung disease. In that space, I see a lot of scleroderma patients, and pulmonary hypertension is a frequent complication seen in scleroderma. I started at the Medical University of South Carolina and saw that we had a need for helping patients with pulmonary hypertension. At that point, back in the early 2000s, there was really only one therapy and it was a new therapy at that. I feel like I almost got into pulmonary hypertension on the ground floor. I started the pulmonary hypertension program in Charleston and then moved to Cleveland and have just been touched by the pulmonary hypertension community. It's a small community. You get to really know your patients well and their family members and other providers who have similar passion for this disease. Today, I'd like to talk to you about shared decision-making and patient preferences. When I think about taking care of a patient with pulmonary hypertension, I've come to realize that the patient is the true expert of their body. It is so important to meet the patient where they are, to understand their perspective when making decisions that are going to affect them. It's not uncommon that physicians and providers get caught up in the literature and which drug has the greatest survival benefit or is the gold standard treatment. We forget that there's always a cost to these therapies. There's side effects, expenses, there's pill burden, there may be self-image issues. There may be just the ability to execute the therapy, because you don't have good social support for some of these complicated therapies. I think there's a lot more that goes on in making these decisions. We have to make the right decision for the right patient. Sometimes, the enemy of good is better and sometimes we shoot for better and we make things worse. Decisions are trade-offs. I find it easier to think about these trade-offs when I think about cancer. Everybody can kind of think about the old-fashioned way of treating cancer where you get this horrible chemotherapy and your hair falls out and you barf your guts out. Would you do that for an extra week of life, an extra month of life, an extra year of life or an extra 20 years of life? Your tolerance of that type of treatment is going to vary based on what you get out of it. Would you do it if you're 99 years old versus if you are 25 and you have a two-year-old kid? Those decisions might influence what you're willing to tolerate and what you're not willing to tolerate. Patients are making these decisions all the time. They also are in the context of what else is going on. Often, pulmonary hypertension isn't their only problem. The average patient with pulmonary hypertension is on nine other medications. That's a lot to manage, a lot to deal with, a lot of side effects, potentially. I think it's important to really understand their perspectives, because I can prescribe medications all day long and if the patient doesn't want to take them for whatever reason, I'm not helping my patient. A medication cannot make you better if you don't take it. You have to find out what's going to work for that individual patient. I've also learned the hard way that patients don't read the textbook. They don't fit into these neat perfect diagnosis with zero other comorbidities. What works for one patient might not work for another patient, so treatment needs to be very individualized. I also think when we talk about severe diseases like pulmonary hypertension, there's no cure. People will eventually die from pulmonary hypertension and we have great therapies that are extending life, but we don't have a cure. I think as providers, we get so caught up in offering hope and offering our best therapies that we forget to address symptom management, exercise capacity and overall goals of care. Addressing the elephant in the room. Addressing that you're going to die from this disease, which is a really scary statement and is still an important one to have. We can have that conversation and say, I want you to have as many days on this earth as absolutely possible, and I want you to have the best quality of life with all of those days. But when you don't have a quality of life, what is your preference? And to meet the patient where they are, because that's a pretty individual decision, what the patient wants and what the patient doesn't want. It should not be my decision. It should not be my personal decision. I can say as I have gone through different stages of my life from being a college kid to a mother of a teenager, my decisions about my life have changed. Some patients want to do everything no matter what forever, and they are true fighters. I support those patients 100%. That's not the right answer for everybody. I think it's so important to meet the patient where they are and to remember that it's not just treating this disease with medications, but the goal is treating the disease with these medications, but with an emphasis on the outcomes that are important to the patient, which generally means giving them the quality of life that they want. I try to think about decisions that way. I think it's important to educate patients on what's out there. One of the things is that there has been a lot of great developments in pulmonary hypertension over the years. When I started, we had one therapy and now we're up to like 16 plus available therapies. Patients aren't dumb. They are on the internet. They are on this blog. They're members of PHA and other patient advocacy groups. They're wanting information. It is our job as providers to provide them that information and to provide it in a balanced way. When a drug is studied, generally, it's a very carefully curated population of study patients that are put in a clinical trial, which doesn't necessarily translate to all patients. We need to say this drug was studied in this way, in this particular population of pulmonary hypertension patients, and this is what we found. These are also the side effects that patients who were in the population experienced. Based on what I know about you, I think this might be a good drug to try, but maybe patient X has a certain comorbidity where I can say, because you have this other thing going on, it's possible that you don't tolerate this medication as well as we would like. So, we need to keep in contact. I need to know what you're going through when you take this medication, so I can either help you with side effects or we can decide that this medication is not a perfect fit for you. When we think about data that is on average the study of 1000 patients or the study of 200 patients or however many -- this drug improved hemodynamics or improved walk or improved time to clinical worsening. But that is a group of patients that this drug affected on average. There are patients in that one study that do great on that medication, and there's patients that do less great. There is a spectrum. I think it's important to say you're just one patient. You are an individual. These are things about you that I think might make this medication work or this recommendation of therapy, but here are things that we have to look out for. When I approach a patient, I always say, let's do what I call an N-of-1 trial, your own unique trial of this therapy. It sounds like a good idea. We're all on board. We're going to try this therapy and we're going to follow the results together to see if it's worth it. I learned this the hard way. My daughter has ADHD and I kept thinking her ADHD wasn't controlled, so I had just talked the pediatrician into increasing her dose and increasing it and increasing it. Well, she was hiding her ADHD medicine, because she didn't like the way they made her feel, which is why her ADHD wasn't controlled, which is why she needed escalating doses, because she never took the medication. I made that mistake with my own daughter. I could tell she was focused when she took her ADHD medicine, but she hated taking it, because it made her feel bad. Now, in pulmonary hypertension, we have 16 plus available therapies, clinical trials, all sorts of different things that we can try. It's really important to find the right combination of things that makes the patient feel better as opposed to what I'm reading about in a journal article. I try to think about that. In regards to clinical trials, it is so important for us to have clinical trials so that we can move the needle and the disease. This is how we're going to come up with a cure and we can't have clinical trials without patients. We do a lot of clinical trials at the Cleveland Clinic. I offer these trials as an option for patients. I make sure that they understand if you're participating in a clinical trial that is always voluntary. You can decide you want to do the trial today and tomorrow you can say, I don't want to do it anymore, and you can quit. Clinical trials are voluntary. Whether you participate or not should not affect your care with your healthcare provider. It certainly does not affect the care they get at the Cleveland Clinic, whether they participate or not. So that needs to be very clear. I also say, the reason it's a clinical trial is it’s an experiment. We do not know if it works. It might not work. It might make you feel worse. We have no idea. That's why it's a clinical trial. It needs to be studied. But then I say, I am so grateful for the patients who do participate in clinical research so that we now have more and more options for our patients with whatever disease, but participating in a clinical trial, that's an individual decision. Sometimes, it's just a pain in the neck to come in as often as you have to for clinical assessments in a trial. Some patients work and they just can't take that amount of time off work or they live too far and they don't feel well enough to travel that far, that frequently. Some people don't want to feel like they're being experimented on. That is 100% understandable. Other patients say, I don't know if this'll benefit me, but if I can help contribute to the knowledge about pulmonary hypertension, that's my way of giving back. A lot of patients want to participate in clinical trials for altruistic purposes. They just hope that someone else can benefit from the science. I'm certainly grateful and amazed by the number of patients that want to do that. Whether a patient participates in a clinical trial or not, again, I think is 100% their decision. I just put it out there as this is also an option. Just the same way I say, and so is participating in pulmonary rehabilitation. Shared decision making, I think has evolved over the last 20 years. The FDA, for instance, seems to have put more emphasis on what we call patient reported outcomes, how a patient functions and feels. Not just do they live longer, do they walk farther? Does their PVR drop? The FDA recognizes the importance of therapies. Not only do they want to have a biological improvement, but they need to have an improvement that the patient cares about. We're getting more and more data. Although there's some alignment between the providers and the patients, it's not complete. For instance, I've recently been on a project where we've looked at the provider recorded functional class of a patient. So WHO functional class is something that is recorded in pulmonary hypertension. Functional Class I patients don't have symptoms. Functional Class IV, they're more or less symptomatic at rest. Function Class II is they have some symptoms. Function Class III, they have more. Function Class IV, they're at rest. I was involved in a project where providers, which is the usual way of assessing functional class. But then, we had patients report their own functional class, assess it themselves, say, “Well, I think I'm a Functional Class II or I'm a Functional Class I. What we found was about 50% of the time the provider and the patient matched, but 50% of the time they didn't. The providers both over and underestimate functional class. The cool thing was that the patient reported functional class had better alignment with quality of life measures like emPHasis10, which is a pulmonary hypertension quality of life tool, and the SF-36, which is like a general health quality of life tool. What the patient says about their functional class aligns more strongly with these other measures than what the provider thinks about the patient's functional class. Although there is some alignment, it's not perfect. I think we're getting more and more data that patient perspectives matter. I also think patients have more ready access to information at their fingertips, because of the internet and blogs and websites. They are coming in educated, asking questions about various therapies. They want to know about them. They want to know if they're a candidate. They're hearing about other patients' experiences, because patients are blogging and saying, “Well, I tried this and this happened and it was great,” or “I tried this and this happened and it was horrible.” They're hearing all these stories. Patients certainly come in with a lot of questions. I think that's the challenge to make sure that we are spending enough time with our patients to answers those questions, to discuss the options, to provide them fair, balanced information. This is what the drug can do, but here's also the side effect profile of the drug. And to think about, you have to take this drug three times a day or four times a day or once a day, or it's a continuous infusion, what does that look like? Or you can take this drug and oh, by the way, your copay is humongous, because these drugs are expensive. It's going to be challenging to get prior authorization. There is a very, very unfortunate financial piece to these decisions. Also, I find as a provider, sometimes it's really hard to discuss. You're talking about therapies. You want to help your patient. You want them to live as long as possible and have the best quality of life as possible. It's hard to have a conversation around hope and then also have a conversation around goals of care and what are your thoughts about death and dying, or do you have a living will or palliative care, which I think are really important topics. Sometimes, these are topics that scare patients that they don't want to talk about, they're not ready to talk about, but sometimes they're topics that patients do want to talk about, but they don't know how to bring the question up. There's this elephant in the room that nobody's talking about. We're doing a disservice to our patients if we don't bring that part up of care. Often, patients hear the word palliative care, and it's scary. Our palliative care group prefers to be called quality of life doctors. Just because you have this disease doesn't mean we can't really work on addressing your symptoms the best way possible. Treating someone with pulmonary hypertension medications and also better managing their symptoms are not mutually exclusive. You can do both at the same time. I think providers have a hard time balancing those conversations, because you certainly want to leave the patient with hope. Always want to leave the patient with hope. But honesty and addressing the scary topics is super important too. I try to figure out where the patient is and how receptive they might be. Sometimes, these harder conversations have to build with time and patients have to be ready to have them, but they have to be given permission that the conversation can be had when they are ready. It's hard. It's hard to get through all of this in a short follow-up clinic visit. That's the challenge of taking care of the patient, but probably the most important part of taking care of the patient, in my opinion. I'm Kristin Highland, 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/34697530

Episode 513 - Kristin Highland, MD 03/24/2025

Jimmy Ford, MD - phaware® interview 512 03/18/2025

Jimmy Ford, MD - phaware® interview 512 Jimmy Ford, MD discusses the 7th World Symposium on Pulmonary Hypertension and his work on the in PH Task Force. My name's Jimmy Ford. I work at the University of North Carolina at Chapel Hill where I direct the pulmonary hypertension program. I got into pulmonary hypertension when I was a pulmonary fellow about 17 years ago. At our institution, there was not really a formally organized program or cohort of patients being followed by a pulmonary hypertension specialist. Also at that time, there were a number of new therapeutics coming online and being developed, so it was a very exciting time. I managed to cohort together an increasing number of patients throughout my fellowship to the point that once I was done, they didn't want me to leave. I managed to stay on and continue to grow the program, and here we are today. I'd like to discuss really a new, important task force that was included in the World Symposium on Pulmonary Hypertension. At the previous event, in 2018 in Nice at the Sixth World Symposium, we had the first patient perspective task force. Then, back this past summer, in Barcelona, now the second . It's really, I'd like to think evolved quite a bit since the first one, and appropriately was positioned as the first task force presented at the World Symposium fittingly, since everything should be centered around the patient. It really helped to set the stage for a lot of important discussions about patient care, and brought to the forefront a number of new issues with regards to patient's perceptions of care, how they access care, how they are or are not included in important shared decision-making approaches, and making sure that their individual treatment goals are being addressed and met. I think it's a really important topic that we'll see increasing focus on as the years go by. I think as providers we tend to get tunnel vision sometimes about we have a disease and we must treat the disease, but all the while forgetting there's a patient who's dealing with that disease. I put a quote up by Sir William Osler when I presented our recommendations at the World Symposium, and he stated that a good physician treats the disease, a great physician treats the patient who has the disease. I think that's really the spirit of this. Every patient's journey and experience of pulmonary hypertension is different. Every patient's goals for therapy and what they want to achieve in terms of treatment are different. I think if we just take a sledgehammer approach of we must treat this disease, must treat this disease, and don't consider the individual patient's journey and how they're dealing with it or struggling to deal with it and what they want to achieve with therapy, then we're really doing them a disservice. I think that's why we're seeing this percolate ever more to the top of the agenda on the World Symposium and other important pulmonary hypertension meetings, as well. We'll really need to lean on patient associations and patient advocacy groups increasingly as time goes on to really get their input collectively, as well. They really serve as an important avenue for patients to collectively advocate for their voice to be heard, and also a mechanism for their voice to come through in cooperation with industry and provider professional societies. One area that we talked about was patient reported outcome measures, or PROMs, as we call them for short, which are tools that have been systematically developed to essentially capture patient's quality of life. There are a few different tools out there, some of which are specifically developed for pulmonary hypertension, some of them are more generic. At the end of the day, the intention is to capture not only symptoms of the disease, but also how the symptoms affect the patient's life, and also how the effects of treatment affect the patient's life. Trying to find ways to get clinicians to adopt a more systematic inclusion of the use of those tools in clinical practice is really something that we advocated for. Not really coming down to preferring any one particular instrument over the other, but simply just to try to encourage patients to do this systematically. There are a lot of potential benefits. It helps to facilitate the shared decision-making approach, in that you are collecting quality of life information from the patient in a structured manner that is repeated hopefully in serial encounters over time, such that you can compare and contrast how the patient may or may not be achieving a better quality of life with therapeutic interventions or having their treatment goals met. And really thinking about, well, how can we better facilitate their use in clinical practice? The one thing we always hear from providers is that there's just not enough time in the clinical encounter to do this, right? It's a tool you've got to give to the patient, they've got to fill it out, it takes some time. Ideas like electronic versions of these tools, which are in development, and can be provided to the patient ahead of a visit. Patient can fill them out, it doesn't take time away from the clinical encounter. We can set reminders to have the patient fill them out or to remind us to have the patient fill them out at certain intervals. They also can be tools which help to determine whether quality of care that's being delivered at a particular center is at a certain desired level, and hopefully improving over time, as well. I think as these are developed further and developed into formats that are more readily digestible and can be completed more easily in the clinical encounter or even in advance of it, we will see better adoption of them. Another issue is, thinking more globally, how many languages are some of these translated into? If you're looking at the pulmonary hypertension-specific PROMs, I think the most that any one of them is translated into is about 20 to 30 languages, whereas some of the more generic ones are available in well over 100 languages. We prefer to use a pulmonary hypertension-specific PROM, that was our recommendation. But we still have to do a lot more research about things like, how often should patients be filling these out? And do they potentially have some value in conjunction with the risk assessment tools to help actually inform the risk profile of the patient too, not just their quality of life? We do have a few studies out there that have shown that the PROM scores, particularly for EmPHasis-10 and the SF-36 tool ,at the time of diagnosis, have translated with outcomes over time. We still have a lot to learn and explore about how to best utilize these tools, but I think at minimum we need to find a way to start using them more routinely in clinical practice. One thing we did is we polled a variety of patients, providers, and patient advocates throughout various areas of the world across almost every continent. They were distributed all throughout South America, Africa, Europe, Oceania, Asia, so a really varied sample of patients in terms of social situations and geopolitical situations. There were a lot of similar recurring themes that we saw from these survey responses, including generally an impaired lack of access to expert pulmonary hypertension care in centers. In conjunction with that, relatively poor access to diagnostic procedures, particularly the most important one in pulmonary hypertension, the right heart catheterization. If we're looking at this globally and we can't get a significant number of patients to right heart catheterization, the discussion about the disease is moot because we don't even have a diagnostic foundation to begin with. We talked about some initiatives that have been employed in the past and that might be expanded upon to improve access to the right heart catheterization, whereby medical centers throughout Europe, South Africa, United States, have partnered with underserved areas, particularly two in Africa, Uganda, and the Morning Star Clinic in Zambia, where cardiology teams have actually traveled to these places and provided expertise, equipment and ability to get those sites set up to do right heart catheterizations. But again, that's just a few places, and there's still large swaths of the world where access to that remains poor. Similarly, distance that patients are traveling to pulmonary hypertension centers can be quite far either in time and or distance. Clearly, we need more expert centers, more expert providers throughout the world in underserved areas. It's interesting, even in the relatively underserved or global south areas of the world, even beyond those in fairly well-resourced nations within them, we see significant disparities in healthcare access and delivery. It's really a complex problem, which certainly is not limited to just pulmonary hypertension, but I think when you've got a serious chronic, relatively rare disease where the therapeutic regimens can be quite complex, these obstacles become all that much greater to overcome and, more importantly, to address. Another few areas that we addressed in our task force included, for example, climate change. In general, the idea of adverse humanitarian situations, which can include climate change, armed conflicts, pandemics, all of which we are seeing unfortunately throughout our world with increasing intensity and frequency. So recognizing what are some of the challenges that each of these adverse situations brings, particularly to a pulmonary hypertension patient. For example, with the topic of climate change, we know that poor air quality, increased air pollution, extreme heat and weather events leading to storms, flooding, et cetera, can really cause a lot of issues for patients with cardiopulmonary diseases, can increase symptoms of dyspnea, cause acute exacerbations. There's the potential for waterborne and vector borne illnesses with flooding, all of which on top of a complex serious disease like pulmonary hypertension can really spell a lot of trouble. Then, with regards to armed conflict, like any individual, and especially those dealing with chronic rare complex diseases like pulmonary hypertension, life is disrupted significantly, right? People are displaced potentially, their access to medications and care is interrupted substantially due to their likely geographic displacement or to the destruction of that healthcare infrastructure or medications. Really the best way that we could think of to try to address or mitigate some of these things is with advanced contingency planning of multiple different types, forming cross-border partnerships, really educating the patient about what to do in these situations, making sure that they are maintaining a supply of their medications, that they know where to go in the event that they cannot reach their usual care team, that they have access to telecommunication devices if telemedicine or e-health is necessary to be employed. Every situation is different, of course, with these adverse humanitarian situations, but we thought it was important to call them out as they're becoming increasingly problematic, and to try to come up with strategies to preemptively deal with them. Unfortunately, methamphetamine use is becoming an increasingly noted cause of pulmonary hypertension that we see in a lot of pulmonary hypertension clinics across the US and even the world depending on where you are specifically. We didn't address that specifically from the context of the patient perspective, but it certainly is an emerging problem, which does have some geographical predilection. In my part of the US, it's not so much of an issue. I know in many parts of California and Western US up to and including regions around the Mississippi River it is a huge problem, and probably other urban centers throughout the eastern US too. I think it really depends on where your practice is located. Meth use really plays into the idea of social determinants of health. The reasons that people get into the use of methamphetamine are likely related to a lot of what we call allostatic load metrics that affect one's health. Being poorly, financially resourced, having low general and or health literacy, and having a lot of life or psychosocial stressors which lead one down the path of substance abuse. It is relevant in that regard in terms of addressing the social determinants of health. So probably should be something that is addressed in the next patient perspective task force, particularly if it continues to be an ever-increasing problem in pulmonary hypertension clinics. One other important aspect is increasing the diversity of patients that we include in our registries and our clinical trials. For the longest time, those cohorts have consisted largely of non-Hispanic white patients, if you look at the percentage. It's not getting any better as time goes on from when we really started doing pulmonary hypertension trials a few decades ago, to even our most recent trials with Sotatercept, for example, we're still seeing overwhelmingly non-Hispanic whites being enrolled. That's problematic in the sense that we sort of take the findings and results of these trials as generalizable to our entire patient population that we treat in the real world outside of clinical research. That may not be true. As it is, consider that we already see significant variability in treatment responses to pulmonary hypertension therapeutics between individuals even within the same race. You can imagine that genetic pharmacogenomic differences certainly exist on a racial basis that we're not capturing in our clinical trials. I think we need to be much more intentional about enrolling certain percentages of races and ethnicities in clinical trials so that we can stand on a better and firmer ground when we say that we can truly generalize these findings across our clinic patients. This is really just a top-line look, a 30,000 foot view, if you will, of the world of the patient perspective and pulmonary hypertension. I think it's a really challenging area because so many factors inform it, right? We'll continue to uncover more, and we should continue to uncover more as time goes on. But I think it's important to really tackle the ones we've identified now head-on, and be ever mindful of emerging ones like methamphetamine, as I mentioned. My name is Dr. Jimmy Ford, 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/34683465

Episode 512 - Jimmy Ford, MD 03/17/2025

Hap Farber, MD - phaware® interview 511 03/11/2025

Hap Farber, MD - phaware® interview 511 Harrison "Hap" Farber, MD gives a recap of PHenomenal Hope 2024, emphasizing its patient-centered approach to pulmonary hypertension (PH) research. He highlights patient stories, particularly Eric Borstein's inspiring journey of recovery and advocacy, and underscores the importance of patient-provider collaboration. Additionally, he addresses challenges in PH care, such as inclusivity in clinical trials, the role of AI in medicine, and the complexities of treating patients with substance use disorders. I’m Hap Farber. I do pulmonary hypertension at TUFTS Medical Center in Boston. This is the second edition of . It’s different than other symposia in that it’s much more focused on patient needs, patient opinions, patient outcomes, and what we can do in the future to improve all of those rather than just basic science or clinical science that most PH symposia cover. For example, in this year, we had a patient come talk to us about what the journey’s like for a patient, which was just incredible. People just were picking their jaws up off the floor. It was just amazing. This patient, Eric Borstein, is like the perfect patient. He’s the perfect patient in part because he has the resources to be the perfect patient. Part of the problem is the bulk of our patients don’t have the resources to be the perfect patient. Eric’s had a remarkable response to drugs, thank God. Secondly, he’s incredibly articulate about the journey he’s gone through. Thirdly, for those of us who do this a lot, he’s like the perfect PH patient that we never have, but it then sets a bar for us and our patients to try and reach that bar. As he said in his talk, he was a typical male. He disregarded or didn’t pay any attention to his symptoms, which went on for multiple years before he actually almost died because he’s a typical guy. You know, “It’ll go away.” He ended up initially diagnosed at such a severity that he ended up in an ICU, almost died from right heart failure, ended up on systemic therapy and had a remarkable response to it such that over time his right ventricle got better, he got better, and he was able to transition off onto oral meds. In this whole time, he had come to the conclusion he was going to somehow beat this disease. Through his wife, they assembled basically a team. He’s got a sports psychologist, he’s got chefs, he’s got physical therapists, but he has the wherewithal to be able to do that. Then, he started walking. By walking, he could convince himself that he was getting better and that every step he took was another step towards beating this disease as he says. In addition, for him, it seemed like that walking mitigated the side effects from the drugs. He just kept walking farther and farther. The guy walks about 12 to 15 miles a day. He doesn’t just walk, he walks four miles an hour. I mean, he’s doing a mile in like 14, 15 minutes. I mean, he chugs right along there. There’s no facade here. He realizes that he was incredibly fortunate to have survived and to have the response he did. As he says when he talks, what he wants to do now is basically devote whatever time he thinks he has left to patient education, patient advocacy, stuff like that. I think in the past, one of the biggest problems is there’s a disconnect between patients and their providers. And a lot of the talks (at PHenomenal Hope 2024), showed you that there’s a disconnect. Not only showed you, but tried to investigate why there’s a disconnect. Part of going forward with PAH is in a way to make it a more perfect patient, you need the patient to understand why you do what you do, explain to them why you do what you do, and have them work with you as a team, which is something that we at TUFTS have done for years. You can’t treat the patient as a number. Despite the fact that in a lot of practices now because of the volumes or the constraints, they’re considered a covered life or a number or whatever you want to call, they’re not. You have to think about, if I were that patient, what would I want to know? How would I want to be treated, and what input should or would I have as to what happens to me? Unfortunately, in a lot of situations, that’s not done. None of the three are done. It works better when all three aspects are done because then you get to this issue that you’re working as a team. Patients understand more of why you’re doing what you’re doing, what you’re doing, what you expect to happen, how you’re going to manage their side effects from the drugs, et cetera, et cetera. As I said at the beginning today, when we did the first one last year, I was scared that nobody was going to show up. Then this year, I looked out there, and I was like, “Oh, my God, look at how many people have actually shown up.” We actually have to keep getting better and better and upping in our game. So far, at least the feedback that I’ve gotten over the course of the two symposia has been nothing but positive. Nobody said anything negative. Part of that is the fact that we touch on subjects that most PH symposiums don’t touch. We are dedicated to patients and the advocacy and all that stuff. It’s interesting now though, since we started doing it, like at the World Symposium this year, they actually had patient input for the first time ever in their 25 years of doing it. So this first time they’ve actually asked patients to give their opinions and stuff like that. I think a lot of it goes to the idea that despite what we believe or some of us believe, patients actually want to be part of this team and want to feel like their opinion and/or their thoughts of what happens to them is actually valued, which it should be. My nurse practitioner and my fellow could tell you we go through everything with the patients, and then we go, “So what do you think?” And see what they say. Then, I’ll go, “Well, it’s okay. We can do that. But you might want to expand that horizon a little.” That’s important. The patient wants to be included. No matter what docs think, patients don’t want to be talked to. They want to be included in the discussion. It’s not happening uniformly. Some of the same problems that we had 10 years ago, we still have. You have to realize that this symposium is also very different than others in that this is driven by the abstracts that are submitted, not by my want as to who, whatever. So depending on whoever’s writing the abstracts puts in, then we decide. The one topic which I put in there because I knew it would be above everybody but within five years clearly will not be was the discussion of bioinformatics and AI. Like it or not, that’s where not only the world, but the world of medicine is headed. You can’t stop it. It’s like a tsunami. Now, the question really is going to be, how do you harness it for good and not bad? There’s going to come a point where depending on how good these machines are, they’re going to know more than we do. You have to understand that as much as you may or may not like AI and bioinformatics and all this stuff, you’re not stopping it. There’s too much momentum and too much money involved in it. In simplistic terms, medicine has tried to simplify things. You have a disease, you think you have the cause or the etiology, and you make a drug to treat it. Well, PAH is not that simple. It’s multiple different pathways that probably lead to the same node that then from that node pathology develops. The question really is, what’s upstream from that node? Are there pathways, molecules, enzymes, whatever, that you could exploit either with currently available drugs that you reposition for this or new drugs that target these pathways? That’s what it’s trying to tell you. Or the other possibility is that there’s so much overlap that you never realize between this and disease Y, that you can take the things you’ve learned from disease Y and apply it to PAH. The closest analogies are, if you look now at the neoplastic drugs, the cancer drugs, those guys are 10, 15 years ahead of us easily. But they do have the advantage of they have tissue. They can sequence tissue. You get a chunk of tumor, you sequence it, you find out where the abnormalities are, then you make a drug. When you see all those ads on TV, that’s where those things came from. They’ve got an antibody that just blasts that mutation. That’s good because it’s more specific, it reduces side effects, it decreases off-target effects and stuff like that. We don’t have that yet, but we will if they keep going like this. So since we don’t have tissue, we have to make our own tissue by computational biology and with computers and AI and all this, figure out if we had this chunk of tissue, what it would look like. One of the areas that we focus on now on both years we’ve done this as unmet needs is the patient who does meth. The biggest problem with meth, aside from the fact that their pulmonary hypertension tends to be pretty severe by the time they’re actually diagnosed, is the fact that you not only have to deal with the pulmonary hypertension, but you have to deal with addiction and other social issues that come along with addiction and things like that. Unless you can navigate those waters at the same time, it’s hard. It’s especially hard in a way because in the U.S, not so much in Massachusetts, but in the U.S, the access to mental health care, it’s just pathetic. It’s been cut and cut and cut and cut such that to get somebody into a mental health program with some expediency is really difficult. It probably depends on where you are. It’s clear that out in the West, meth is a huge issue. However, there’s meth in New England, Western Mass, Northern Vermont, Northern New Hampshire, it’s there. Part of the problem is I think they’re used to looking for it. People in the Northeast are not so used to looking for it because we don’t believe that it’s here, but it is. We’re more used to looking for cocaine whereas in the West, they have almost no cocaine. Everybody’s doing meth. In the West, if you don’t think of meth and you don’t screen your patients for meth, or God forbid, you don’t even ask them about it, you’re never going to find it. In New England, for example, I think fewer physicians actually ask about meth or actually do tox screens for meth or stuff like that. We ask every patient, every new patient about meth. We do. It’s surprising how if you ask, you’re surprised. All of a sudden this person goes, and you’re like, “Whoa, okay.” I always use this example. I saw this woman with idiopathic PH, was sent down from New Hampshire, suburban housewife, two kids, picket fence, dog, station wagon. So we’re in there, and I go through my spiel and go, “So any history of meth?” She goes, “Yeah, I used to be a total meth head.” Her husband obviously knew nothing about this. He’s looking at her like, “Say, what?” I said to my nurse practitioner at the time, would you love to be a fly on the window on that car ride on the way home? Part of the issue that’s been raised not only in PAH but in other disease states about clinical trials, clinical trial designs, registries, are the fact that they’re not inclusive. That presents several problems. One is since drugs are approved based on clinical trials, if the clinical trials are not inclusive, you then don’t know if the drug works across the board or did you just happen to hit an ethnic population in whom it works? There are multiple reasons why clinical trials and stuff have not been inclusive. We went through a lot of them. Some of it has to do with physician bias. Some of it has to do with patient bias. Some of it has to do with the economics of clinical trials. A lot of patients who work can’t just take the day off and come in and do their clinical trial visit, even if you do buy them lunch or pay for their parking, because their boss doesn’t understand. They’re risking their job. Then, there are the people who have tremendous suspicion about the medical community based on previous clinical trials to say the least unethical. A lot of these all lead up to the fact that it makes it difficult in some places to recruit minorities into clinical trials so that you get those data that you actually need to know. It’s clear now that some of the pharma companies are actually trying to, in their trials, which they never did before, report actually the ethnicity of the people who are being or have enrolled in the trials in a way to hopefully mitigate some of these issues. But, again, we still have a long way to go. Some of the stuff we’ve covered a couple years in a row, because we want to see if there’s been advances in a year. Some of them, yes. A lot of them, unfortunately, there hasn’t been any. I think should we do this again? Yes, we probably will. Most of the people who’ve at least given us feedback, enjoy it, think it’s worthwhile. Let’s think ahead to Team PAH 2025. We’ll put out the topics, but it’s up to the people who are out there to send in abstracts to fill those topics. Then, we decide who we’re going to pick, what the order, stuff like that, which I think that’s clearly unique. There’s no other symposium that does it this way. Other symposiums, which I either do or organize or whatever, you pick a topic, you go, “Okay, we’ll invite Joe Blow,” and whatever. This is very different. We’re really dependent on the abstracts that people send in. We make that pretty clear. I think I said that by closing arguments. If you don’t like the topics, blame yourself because you send in the abstracts. I’m Hap Farber. 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 /episode/index/show/phawarepodcast/id/34683220

Episode 511 - Hap Farber, MD 03/10/2025

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

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

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

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

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

Episode 509 - Jen Cueva 02/24/2025

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

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

Episode 508 - Stephen Gariepy 02/17/2025

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

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

Episode 507 - Jolie Lizana 02/10/2025

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

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

Episode 506 - Allison Feenstra 02/03/2025

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

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

Episode 505 - Jodi Berry 01/27/2025

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

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

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

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

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

Episode 503 - Jennifer Howard 01/13/2025

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

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