From “Untreatable” to Life-Changing: The New Era of CTEPH Care

What happens when patients are told everything looks “normal” but they still can’t breathe? Dr. Jenny Yang explains how new testing and team-based care are solving one of pulmonary medicine’s most overlooked mysteries and changing what’s possible for CTEPH patients.

This Special Edition episode is sponsored by Liquidia.

My name is Dr. Jenny Yang. I’m an Associate Professor of Medicine at the University of California, San Diego. I did all my training at UCSD. I did my residency and fellowship and graduated in 2021 and then joined the faculty at UCSD with a focus on pulmonary vascular disease. So, I do both PAH, CTEPH, and acute PE. I was lucky to have a lot of really great mentors who introduced me to people within CTS, ATS, CHEST. I was able to find these opportunities to do these speaking engagements and give presentations. So through those, I was able to meet other people and sort of create this network. It’s been really nice to be more involved in these organizations and get to meet other people and sort of bring the collaborations and things I learned here back to UCSD, also.

Sometimes, I’ll attend a fair bit of the sessions and do some of my own learning. I think there’s a lot of new research and sort of case series, case reports of interesting findings. I’ll go to learn more myself and see if that applies to some of our patients. Then, some of them are sort of myself giving presentations and sort of passing on what I know to others and hopefully doing the same. At the recent CHEST Conference in Chicago, I was part of a panel talking about chronic thromboembolic disease. My focus was also speaking about the diagnosis and the utility of exercise testing after an acute PE.

So after an acute PE, there’s sort of this wide spectrum of outcomes that can occur and we all sort of have gained a better understanding of CTEPH, but there’s also patients who can have these chronic PEs or chronic thromboembolic disease without pulmonary hypertension. Their resting tests might look normal. Their echoes and right heart catheterization might all be normal, but they are still quite symptomatic.

At UCSD, we’ve started doing more invasive cardiopulmonary exercise tests. What that is we end up combining a right heart catheterization with a cardiopulmonary exercise test. These cardiopulmonary exercise tests, it’s tests that you might see some like athletes do to sort of really see how great their endurance is. But for our test, these patients will have nose clips and sort of a mouthpiece on, and that’s really to measure their gas exchange during exercise. They’ll have all sort of little stickers on their chest to measure heart rate and things like that. But we also add in the right heart catheterization part of it. We’ll do these exercise tests in the cath lab, where they’ll get a regular right heart catheterization. So they’ll have the catheter in the neck and then we actually get them up off the table onto an upright bike in the cath lab and have them exercise with the Swan-Ganz catheter in place so that we get a mix of their hemodynamics while they’re exercising and also all of their gas exchange parameters while they’re exercising also. We get a wealth of information on sort of what’s limiting them with exercise.

Normally, with our regular right heart catheterization when we’re sort of diagnosing PH, we sort of put it in, get the numbers and then immediately take it out. But for these, we’ll leave it in for the duration of exercise. The treatment strategy for CTEPH has really evolved into this multi-modality approach, where for many years surgery was the only answer. If patients weren’t surgical candidates, there was really nothing else that could be offered. Over the last 10, 20 years, there’s been a lot of advancements in both medical therapy and also balloon pulmonary angioplasty. There’s a lot more options for patients now. Having this team of providers who are experts in surgery, the PTE surgery, balloon pulmonary angioplasty, pulmonary hypertension in general, and finding ways that each individualized patient can be approached is sort of the key takeaway.

Beyond the physicians on the team, the really important aspect are also our ancillary staff, our nursing, our respiratory therapists, everyone involved in the team, our pharmacists. They play a huge role in taking care of these patients. Our nurses do a lot of hard work to get these medications approved for our patients, contacting them weekly to make sure that they’re titrating these medications up appropriately if they’re having side effects, getting through all the insurance approval processes of obtaining these medications. Then, on the inpatient side, after a PTE surgery, for example, having the respiratory therapist and the nursing staff who know how to take care of these patients are also very important in the success of how these patients do.

I think it’s really unique in CTEPH that there’s a lot online that you can see. A lot of time patients will come to us with sort of preexisting ideas of what they want or don’t want for this disease state. We only know who comes to us. I would guess that there are a lot of people who never make it to us, whether that’s because they physically can’t make it because of financial reasons or sort of other social barriers or because they’re just not interested in the idea of a surgery or some sort of intervention. So the referring provider then never sends them because they’re just not interested. We sometimes do get patients who are willing to come and meet with us who come with no interest in getting surgery and they sort of say that very upfront during their clinic visit. They don’t want surgery even though they clearly have operable disease. Depending on the patient, we will lay out all the risks and say the benefits of surgery. Sometimes, patients will realize that maybe they do want surgery. I think it’s always worthwhile if they can. We can at least meet them and discuss it with them. If they still choose no surgery, then at least we’ve been able to discuss all the risks and benefits with them.

One of the big concerns that some of our patients have is with age, patients who are sort of in their 70s, even early 80s, who we recommend to undergo this big open heart surgery, they have a lot of concerns about their quality of life afterwards and that they’re doing sort of okay right now and is it really worth this big surgery? We’ve had some patients in their late 70s, early 80s that after meeting with us, discussing everything, they decide to undergo surgery. It’s been life changing for them. We sometimes hear back from them. They’ll send us pictures that they’re now able to sort of pick up their grandkids, can go out and actually play with them and spend a whole day out with them as opposed to before they were maybe using the wheelchair more or not wanting to go out just because it was so exhausting to spend a day out.

We’ve done these PT surgeries on a wide range of patients. Average age is probably somewhere in like 40s to 50s or so, but we’ve done as young as children, so like nine, 10 year olds, and then as old as in their mid to even slightly later 80s. There’s no strict contraindication to age. It’s sort of we take the whole thing all together and discuss as a group whether or not we think this patient would benefit from surgery. When they’re sort of younger than 14 years old, we usually don’t actually take care of them at the adult hospital. They go to the children’s hospital. We don’t take care of those ones as much, but the 14, 15, 16 year olds, they can get their surgery at the UCSD campus and the discussion, everything is obviously with the parents. Their journey to getting to us is oftentimes a little bit different because you don’t necessarily always think about CTEPH right away in a 14-year-old. Most of them get asthma diagnoses and sort of have a little bit of a longer journey to getting to UCSD.

Our surgeon actually will go to the children’s hospital and operate with the pediatric cardiac surgeon. Our surgeon’s the one who’s mainly doing the endarterectomy set portion of it, but then the care of these patients afterwards is at our children’s hospital. I think a lot of it is the equipment that we have at our hospital just is not feasible in an eight-year-old. But overall, this is very uncommon to have this in pediatrics this young.

Every three years, the International CTEPH Association does a big CTEPH conference. It’s in Warsaw, Poland this year, but it’ll be in June. That’s a pretty big conference that the International CTEPH Association sets up. It’s a couple of days focused purely on CTEPH and sort of any updates and changes over the last three years since the last conference.

Hopefully, at this conference, there’ll be a lot of sort of new and exciting things that we’ll talk about in CTEPH. I think there’s still a lot to be learned. We’ve come a long ways in terms of how we manage these patients and our approach to these patients and gaining further understanding and awareness. But I think there’s still a lot of work to be done in the field. Hopefully, at this conference, we’ll be able to learn a lot more about these CTEPH patients and how best to care for them.

Thanks for listening. My name is Dr. Jenny Yang, and I’m aware that my patients are rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com. #phawareMD @UCSDHealth #CTEPH @JennyYangMD

Why “The Walking Guy” Trusts His Therapist as Much as His Cardiologist

For PAH patient, Eric Borstein, walking started as a way to survive medication side effects. Now, it has become a way to survive himself. Each step pulling “EB” further from an ICU bed and closer to an identity that isn’t defined by illness. Mental health has reshaped his recovery, and has become one of the lifelines he believes should be prescribed alongside life-saving therapies.

I’m Eric Borstein. Five years ago, I was diagnosed with pulmonary arterial hypertension. I collapsed and nearly died. I spent 16 days in the intensive care unit where very early on I learned that walking helped mitigate the very powerful drugs that I was put on, the lifesaving drugs. When I left the hospital, I continued to walk as a form of therapy. It allowed me to titrate very quickly with the intense drugs that I was on, which now, looking back, saved my life. When I left the hospital, the diagnosis was very dire. I was given two to four months to live. I was put on a pump. They never thought I would get off the pump. The walking helped. The walking not only helped mitigate the side effects, but I learned very early on that it helped with my mental health.

Right out of the hospital, I started talking to a psychologist once a week, who I still talk to this day. He’s become not only a very trusted personal friend, but somebody that I rely on equally as much as my cardiologist who treats me for the PAH and as important as the drugs that I’m on today that I know are life-saving drugs. Without those drugs, my health would rapidly decline.

I learned that positive mental health was critical in somebody living with a disease like pulmonary arterial hypertension. I was diagnosed, again, with idiopathic PAH. We didn’t know why I got it. Unfortunately, there is no cure for this disease. So, there are things that a patient living with a chronic, and I’ll say terminal disease, deal with that the everyday person doesn’t have to deal with. That portion of my recovery, the mental health, has been critical. As time has passed, I’ve learned more and more about why positive mental health is so important and why… really tying it back to walking.

For people that don’t know me, my nickname is “The Walking Guy.” I’ve not only used walking as a form of my recovery, but it has kind of become my identity. It’s become a way of not only being recognized, but a way to bring awareness to this disease, this very rare disease. The more time that has gone on, I’ve started asking the question, “Why does walking make me feel good?” I know why it makes me feel good as far as mitigating the side effects (of the medications). It helps metabolize them through my body and it makes me feel good physically, but why does it make me feel good mentally? My psychologist has done an amazing job at teaching me these things and has taught me both in technical terms and in layman terms, why. It really comes down to three different reasons. The first, when I walk, and I walk 13 to 15 miles a day, I walk between 4,500 and 5,000 miles a year, which is amazing for anybody, let alone somebody living with a lung condition.

The first reason is it distances myself psychologically from the intensive care unit. I spent 16 days in the intensive care unit and I was really sick. When I left, walking was difficult. When you’re living with PAH, before I collapsed, I was suffocating. I was dying. I tell people that when I was diagnosed, it didn’t come out of left field. I was sick. I was really sick. If I would’ve walked into a doctor’s office anywhere between six months to a year prior to collapsing, they would’ve seen I was in heart failure, but I was afraid to find out what was wrong. I was scared to death. I was one of those people, especially males, who when they are critically ill or sick with something, they don’t go to the doctor. That almost cost me my life.

When I was at my sickest, I couldn’t walk 20 feet without being out of breath. I couldn’t walk up three stairs without feeling like I was suffocating. I couldn’t walk from the bedroom to the bathroom without having to hide in the closet, because I felt like I was suffocating. When you’re suffocating, you’re alone. So, when I started to get better, and even to this day, when I go walk, whether it’s five miles, 10 miles, 20 miles, or 205 miles from LA to San Diego, I’m distancing myself from the ICU. I’m distancing myself from when I was at my sickest. So, that’s subconscious. That’s not something you think about. You don’t think about where you were five years ago. I don’t think about the intensive care unit anymore. But subconsciously, when I’m able to go up that hill or when I’m able to push through those miles or when I’m able to go on an amazing walk, I’m not sick. So, that was the first reason, it’s distancing myself from the ICU.

The second reason is that when I walk, I’ll walk by a building or I’ll walk by a window or I’ll get home and I’ll look in the mirror. I don’t see myself as Eric Borstein, the PAH patient. I see myself as EB, the guy that’s kicking ass beating this disease. Unfortunately with PAH, there is no cure. I know that this probably isn’t forever, but as long as I’m able to do this and I’m able to break through these barriers and I’m able to walk 500 miles in a month, or I’m able to get out of bed on days that are really hard, and I’m just able to go walk two or three miles, and I look in the mirror and I like the way I look. I’m not Eric Borstein the patient, I’m EB, the guy that’s beating this disease. So, that’s number two.

Then, the third reason is it was explained to me that positive mental health is really as simple as finding something that you love to do. For me, that’s turned into walking. For other people, that can be filmmaking. It could be playing chess. It could be learning how to play the classic guitar. It could be bike riding. It could be swimming. It could be anything. For me, it’s walking, but positive mental health is as simple as finding something that you love to do. When you’re living with PAH, finding something you love to do can be that outlet that you’re not this patient.

I also tell people, when you’re a PAH patient, you’re reminded of that every day. You wake up, you take your medication, you weigh yourself. For me, I live by a strict diet. I do have to walk because it makes me feel better. If not the medications, the side effects are very difficult. You go through your day, before you go to bed, you take your medication again and you go to bed. So every day you’re reminded that you’re a patient. But when I can go out and walk or when somebody else can go out and find something that they love to do, it removes you from that constant reminder that you’re living with this disease that there is no cure for. Those are really the three main reasons that it’s been taught to me why positive mental health, why walking for me makes me feel better.

But as time has gone on, as the months and the years go by, and I’m now able to share my story with people, not only my community and my friends and my family, but the world. The amazing thing is, I’m becoming recognized and more and more known in the rare disease world. But the more I share my story, the more I’m learning, it’s transcending just PAH. It’s really relatable to anybody. The messaging really is not only somebody battling a rare disease, but somebody that in the face of just the biggest adversity that anybody can ever go through. Lying in your bedroom, unconscious for 23 minutes, dying, there’s not much more a person can go through in that moment. What I’ve learned is everybody faces their moment of adversity. Whether it’s losing a loved one, battling a different disease, going through a divorce, losing a home in a fire, losing a home in a flood or a hurricane, everybody at some point in their life faces that adversity. For me, positive mental health has just become a critical tool in the face of that adversity on that recovery.

Again, bringing it back to PAH, the disease isn’t going away. It’s how I deal with it on a daily basis that’s keeping me alive. Again, along with the life-saving drugs and other tools that I’ve learned help, positive mental health has just become that critical portion and that critical part of my recovery. I talk about it as much as I can to everybody. It’s just something that I will always depend on my psychologist who’s an amazing man, because I’ve learned that, at least for me, it’s an equal part of my pie. I firmly believe it’s one of the main reasons why I’m still here today.

I don’t do this because I want anybody to feel sorry for me. I do everything that I’m doing because number one, it helps me feel better. But then knowing that I’m helping others is a positive. I want to help other people. That is my mission and my goal and everything that I’m doing with this second chance is for my family. It’s for my friends. It’s for those that are close to me. It’s for my community, but it’s really to help others. I’ve been given the second chance. I told my wife in the hospital, if I could help one person when I’m dead, I’ve done my job. But what I’m finding is I’m helping more than one person. To me, everything is a positive.

It’s hard. Living with this disease is difficult. It is a struggle. I make it look easy. Being able to walk the distances I do and do the things that I’m able to do are not normal. I understand that and I don’t take that for granted. I look at it as a positive and I want to continue doing so. Stepping outside of my body and looking back at it, it is a positive boost. I’ve only been sharing my story publicly for a couple years now. It’s amazing how fast this is taking off and how many people it’s affecting. I’m so happy and blessed. But when I first started talking about it to doctors and sharing my story and talking about the importance of positive mental health, it was only a couple years ago, but they were saying, “You got to talk more about this. You’ve got to get out there and you’ve got to talk about the importance of mental health. We want to talk more about this.” Just in the last two years, it’s becoming more widely talked about.

I know mental health has kind of been that taboo subject for decades, but now it’s more widely talked about, which I think is really exciting. What people are going to find is the more you can talk about it, the more it’s going to help people and the more it’s going to save people’s lives, both inside pulmonary hypertension and outside pulmonary hypertension, but really coming back to the rare disease world and pulmonary hypertension and PAH, it is such a critical part of my recovery, but it’s really, it needs to be talked about more because it is super, super important and it isn’t taboo and it isn’t something that people should shy away from.

I come from a generation where you talked about your feelings… you did and you didn’t. Then, I learned in the years leading up to collapsing, when I was sick, I tell people it was hard on our marriage. My wife didn’t understand why I was kind of pulling back. I went from a very social person. For anybody that knows me now, social, outgoing, friendly, I pulled away. Again, I take it back to when you’re sick with PAH and you’re suffocating, it’s hard to be around people. You can be around 100 people, but you’re alone. So it was hard on our marriage. My personality changed. I just wasn’t who I was. In those two years leading up to me collapsing, we started seeing a therapist. So, I learned before I collapsed to learn to trust a therapist. So, when I collapsed and left the hospital, it was very easy for me to get into therapy and to trust therapy and to know therapy works because therapy saved my marriage in a time where I was dying. I know therapy works. I am that person. I will be the spokesman for positive mental health and the importance of it living with a chronic disease. But I’m so happy that it’s being more widely talked about and more widely discussed.

I’m just hopeful that we get to a point where it can be part of almost prescribed therapy. When somebody is diagnosed with pulmonary hypertension or pulmonary arterial hypertension and you’re given a prescription for your drugs, that you’re given a prescription for a therapist. I think it’s so critical. It’s not a drug you put in your mouth, but sitting across from somebody or on a Zoom with somebody and them learning who you are and talking to you about why you feel the way you feel, not necessarily physically, but mentally, and knowing that what you’re doing might not be a bad thing. Being upset, being angry, being in pain, you’re living with a disease that doesn’t have a cure. That’s hard. So, to be able to give somebody the ability to talk to somebody is critical.

The other thing that I do talk a lot about is I’m extremely fortunate, and I don’t take that for granted. I’m fortunate that I live in a city that has amazing doctors and hospitals, and I was able to get to the hospital as fast as I did, and they were able to put me on the lifesaving drugs as quickly as they could. I’m fortunate that I have an extremely loving family, both immediate and extended, who has been there for me from day one. I have a community. I have friends. I have a neighborhood. I have people in the PAH world that I’ve met. I’m extremely fortunate. I’m not isolated. Then, I’m fortunate that I have the means to have the best health insurance and to get whatever care I need and to be able to go around the country and speak and help people. I understand all of that. Most people aren’t, and it is heartbreaking. I’ve met people along the way who aren’t as fortunate and they’re no less of a person than I am.

They are the most loving, caring people. I’ve met people who don’t have anything who would give the shirt off their backs to help me being a fellow patient. So, when I meet these people and they don’t have the same care and they don’t have the same opportunities and they don’t have the same support group, but then they barely get the medications to help them survive, but then they don’t get this extra part that I have and they’re not getting that same care, it’s heartbreaking. I really do hope that this portion of therapy becomes widely accepted and is something that everybody has the opportunity to get one day.Thank you for listening. This is Eric Borstein, and I am aware that I’m rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com. @teamphhope @where_is_eb

Don’t Let PH Ground You: A Real-World PH Travel Playbook

For Nicole Dempsey, travel has always meant freedom. After being diagnosed with pulmonary hypertension, that freedom suddenly felt fragile. Nicole shares how she continues to explore the world, raise her family, and find freedom beyond fear, proving that even with a rare disease, life doesn’t have to stop.

My name is Nicole Dempsey. I’m from Cambridge, Ontario, Canada. And I have pulmonary arterial hypertension. I was diagnosed 12 years ago. Today, I want to talk to you a little bit about traveling with PH. When I was diagnosed, I had been a heavy traveler and I knew that having a diagnosis like pulmonary hypertension, traveling might be greatly affected. I didn’t want it to, so I just decided that I was still going to travel. I knew there would be barriers, but we would work around that. That’s exactly what I did.

Some of the barriers that we need to overcome when traveling as a PH patient are obviously the medications. Especially, if you’re on IV medication or infusion medication, there’s a lot of medications to travel with. It’s just bearing in mind that you put them in a carry on and you get a letter from your doctor, if you so wish, that identifies what the medication is and you travel with them. It’s no different than being at home. Other barriers might be oxygen. For myself, I’m not on oxygen, but I know that a lot of PH patients are. That’s another barrier. But all the barriers can be overcome in order to travel.

For me, one big thing is when I travel, whether it’s internationally, locally, I typically rent a car, and I find this helps a lot to get around places. I know a lot of people like to walk to places. That’s just not something that I can do as a PH patient. Even though I’m stable in my disease, I still rely on having a car and being able to get around. It’s a reassurance for me to know that if I need a break, I just go sit in the car. We get to actually see more places. When we’re out and about. In Europe, for example, we’ve gotten to see a lot more landmarks in different places. If we would’ve had to rely on public transport, let’s say, it would’ve been much more difficult to get to all these places. So that’s a huge tip for me.

I know some people can get really, really intense on looking up the hospitals, looking up PH centers. My pulmonary specialist, he knows a lot of people in the world that work with pulmonary hypertension. Sometimes I’ll say to him, “I’m going to Ireland,” and he’ll say, “I know Dr. So-and-so in Dublin, and you’ll be in good hands if something were to happen.” Or he’ll say, “Okay, you’re going here in the Caribbean. I know there’s a clinic in, I don’t know, Barbados.” So you know that you’re not going to be in trouble. You’ll have some clinics around. I don’t get too intense about that. I just know that I’ll be okay in some capacity if there’s a clinic within a certain radius.

Prior to being diagnosed with PH, I always had the urge to travel. Growing up without the means to travel meant that I did not travel. So once I got older, I knew that I wanted to travel the world and see a lot of different places. When my kids came along and the diagnosis, I thought I really needed to expose them to as much as possible at a young age and to see whatever we could. If it meant that we could also go to Europe or further places than Canada, then that’s what we would do and just explore new places and have them enjoy where we’re going, as well, and have them have the urge to travel lifelong because it’s amazing.

Obviously, when you’re traveling, there are barriers. With a disease like this, it can be so unpredictable. You might feel like, “Yeah, I can do this today.” Sometimes you’re like, “I may not be able to do that hike that we had planned.” Hiking for a PH patient is quite daunting. So for me personally, I have never said to my family, “Hey, go and I’m going to stay back,” because of the whole FOMO thing, and I don’t want them to go out and enjoy something while I’m going through site pain.

What I do is I come along and I sit if I need to and they’ll go and do what needs to be done, or I just chill out while they go and maybe climb a hill that I can’t climb. My kids, they want me to be okay. They don’t want me to miss out, and they never felt like they missed out because of maybe something I couldn’t do. If I say, “Oh, I won’t do that,” they like to say, “Oh, that’s okay, mom, we’ll stay with you,” but I encourage them to go. I don’t want them to miss out on a situation, but it’s never been a problem in my family. I think that stems from… My kids were so young when I was diagnosed, they were three and five, and that’s all they knew. So it wasn’t anything lacking for them that mom is tired, mom is sick, mom may not be able to walk this distance during our vacation. They just really easily go with the flow.

As far as PH is concerned, and especially if you’re on the IV or the infused medication, packing for that and planning takes a bit more time. You should always plan to bring more than what your travel time is going to be. If you’re going for a week, I would plan to bring for a week and a half or even two weeks if you really want to be very conservative. With your oral medications as well, I always bring a little bit more than what I require. Always, always keep your medication in your carry on. I think most PH patients know that, but it’s a good reminder sometimes.

Some of the places I was fortunate to visit was South of France. The South of France is beautiful and hot in the summer. So I guess a tip for someone who might want to travel and see the South of France might not be to go in the summer. It’s a beautiful place, but someone with our disease and not adapting well to humidity and intense heat is not ideal. I’ve seen France. I’ve seen Portugal. I’ve seen Ireland. I’ve seen some islands in the Caribbean down south and Mexico. I’ve always got on quite well managing everything, managing the barriers.

Of course, if you have the means to travel, by all means, do it. This disease is all day every day, 24/7. It doesn’t leave us. Traveling sometimes lets you leave and escape for a minute. I know it’s always with you, but when you’re away from home, you don’t have it right in your face constantly. Whereas at home, your thoughts are a bit different. When you’re away, you can just divert yourself a little bit, explore, and realize that you can travel while being sick. But yes, I think it’s important to carve out time for yourself other than just being home and being saturated with disease. Especially if you’re not working, if you’re just home and you’re sitting and you’re thinking about this disease all the time, and it’s consuming. I just think having a vacation on the horizon gives me will to live because sometimes it’s hard. It’s hard, the medication, the side effects. When I know I have a vacation on the horizon, that just gives me hope.

A lot of people worry about going through customs with all the medication that you travel with, especially again with the IV and the infusion. A lot of people will get doctor’s notes. Personally, I’ve never been asked in 12 years for the letter that explains the medication that I’m traveling through. For all the travel I’ve done, which is a lot, my medication has maybe been flagged twice. It’ll be flagged. They’ll look at it quickly and then they close it and you’re on your way. I walk through the metal detector with my pump on. Some opt to not do that. I walk through it. It very rarely sets it off, as well. I don’t know if that’s because my pump looks like a diabetic pump and they just assume it’s diabetes. Sometimes, I get pat down, but it’s very rare that it does that.

I think a lot of the travel component, that’s what makes it daunting for some patients and why they might not want to fly places. They’d rather drive if they can do that. I know it can be scary for people who don’t travel frequently. So for the first time, if you can talk to someone who’s a seasoned traveler and that can give them peace of mind.

I was in a travel session recently at the Pulmonary Hypertension Conference in Canada. While I’m not personally on oxygen, that’s another thought that people have. Those who need to travel with oxygen will often travel with a POC, which is a personal oxygen carrier. There’s a special sticker that they might need to put on it so that they don’t get flagged. There’s patients who’ve also specified that they need to increase the amount of oxygen while they’re in the air. As I said, I’m not on oxygen myself, and it’s obviously something that you’re going to speak to your doctor about if you are on oxygen and how to manage that, but there’s absolutely a way to do it with oxygen. Don’t let your disease limit you.

If travel is something you want to do, hope that you can do in the future. Don’t let the disease deter you. If you’re not well, wait until you’re well and then give yourself that vacation to look for on the horizon. Because sometimes with this disease, we don’t always have things to look forward to. Going on vacation and have something to look forward to is definitely a way to live well with the disease.

My name is Nicole Dempsey, and I’m aware that I’m rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com. @phacanada

He Sent 100 Emails to Save His Wife… Only 3 Doctors Answered

From a devastating diagnosis to a lung transplant that changed everything, Florin Dumitrescu shares how mindset, persistence, and community carried his family through their darkest moment and how survival transformed into advocacy for pulmonary hypertension patients across Europe. #WorldPHDay2026

My name is Florin Dumitrescu, I’m 56. I’m from Romania, currently living in Austria, in Vienna. We are a family that entered the pulmonary hypertension influence four years ago when my wife got this diagnosis. Unfortunately, in Romania without any hope, because at that moment when the doctors said, “This is bad news. You have pulmonary hypertension. There is no cure for the pulmonary hypertension, but your disease condition is so tough that your life expectancy is six to nine months.” We were told at that moment that, “You have to find solutions anywhere else, but not in Romania.” We decided and we tried to understand what’s going on and what would be the best path to follow. We both said we have to find a solution.

I wrote an email. I put all the information in it. I wrote it from my heart. I searched the internet for all the people, key speakers in the conferences related to PH all over the world. I sent more than 100 emails, waiting for the answers. I got only three answers. One was from Canada. One was from Germany. The third one came from Professor Irena Lang, who’s the head of the department in AKH, the public hospital in Vienna. The doctor said, “You have six to nine months.” The answers from Canada and from Germany were like appointments in six months. That was too late. But we were very lucky that Professor Lang here in Vienna said, “I’m waiting for you in 10 days. I have COVID now, so the earliest moment I can see you is in 10 days.”

We packed, traveled to Austria, met her in a private institution. She said, “You have an appointment at 6:00 PM. Let’s wait till 9:00 when the last appointment ends and we can sit together. We have the night in front of us to see what this is all about and what are the best ideas and the best strategy to deal with.” We waited. We spent almost more than half of the night because it was after 2:00 AM when we left the office. She called one of her colleagues in Japan. They did an assessment at that moment and she started to build a strategy. She said, “There is no cure. We can try something to see whether it fits or not with your condition, but I think that a lung transplant will be the only solution for you. “

We went back to Romania. We shared nothing with our family because we have a daughter, she was 12 at that moment. We wanted to keep her safe and sound, and sane, because she’s a very fragile and very sensitive girl and we wanted to keep her safe. We started our journey with monthly visits to Austria where the doctors took care of Nicoleta, my wife. They’ve recorded all her parameters, seeing that all were going down or degrading.

One year later, in 2023, they said somewhere in the summer, “You have three months to move to Austria because the transplant is imminent. We have to be prepared in the next three months for this.” They were able to extend survival almost a year, but what we had to do was the lung transplant. We packed everything. That was the moment when we told our daughter that we have to move to Vienna, to Austria. She froze, having no idea about what was going to happen with her life. But I think someone up there was not very busy at that moment and took care of everything. Like magic, we found an apartment. In Vienna, it’s quite difficult to find an apartment, especially when you’re a first timer. If you’ve been living here in Vienna, you have the credentials for a landlord to give you an apartment. Otherwise, it’s quite impossible.

I don’t know how and why they trusted us. Probably because we had good jobs in Romania. We said we would keep our employment in Romania. My wife, she’s a lawyer. I was a senior manager in a large international corporation. So, we got an apartment. After that, we needed a school for our daughter. We found a place we had been accepted in a private school. The apartment was 600 meters away from the hospital. In a week, we packed everything, rented a truck and moved to Vienna. Long story short, this is our journey.

In December, Nicoleta got the new pair of lungs, and with those lungs a new life. Now, we have, let’s say, a normal life under the conditions after lung transplant with the immunosuppressors. But otherwise she’s working as a lawyer, she started exercising. We enjoy life together.

No hospitals in Romania have a transplant protocol implemented. They don’t have the knowledge how to take care of somebody who went through a transplant. It’s something very special. Once you are a transplanted person, you don’t have an immune system. You are completely immunosuppressed. Therefore, any infection, any disease, it’s life-threatening. So, here in Vienna, they have a dedicated line, when you call for emergency for the ambulance, for instance, and you say, “I’ve been through a transplant, I’m a transplanted person.” They send a dedicated team of paramedics. They know the procedures, how to handle somebody who went through a transplant. We decided that in order to have our family safe, leaving in Romania for good was the best for us.

When we got the PH diagnosed, this had a tremendous effect, not only on us as adults, but also on our daughter. She had to deal with this tough information, because she was not aware of it, of the severity of the illness. She had to find her own resources to process this information. She also got the support of her colleagues, of her teachers, of a very special doctor she met, a psychologist who taught her how to deal with such a big issue in life. It was very tough for her. They are quite straightforward. They said, “This is going to be a lung transplant. Nobody can guarantee that it’s going to be 100% safe. Anything may happen. It’s a major intervention, it’s not like appendicitis or something like this.” We were also very lucky that in the school where she is, they made a team of professors and children to take care of her. She was surrounded by a group of friends and teachers who went through this, and it was very, very, very special for her because she felt that she’s not alone in this. But it was very tough.

Being EU citizens, we are not required for any kind of work permit or anything else, but it was right after COVID. All the health system in Austria were under special supervision, so no foreigners were allowed to benefit of the services in the public hospitals. Therefore, we needed to became Australian residents. For us in EU, it’s not complicated, because you have the right to work, to relocate your family here. You only have to be registered as self-employed. She’s a lawyer, so it was quite easy for her, she had to go through the relocation process in the bar, but it’s not complicated as long as, as I said. For me, it was a little bit tougher, because I was working as an employee, but right here, right now, I’m working as a consultant. I’m self-employed, a little bit harder being in a different market, but we can manage it.

The most important thing is that we are a family, we are still a family, we are all of us. We can enjoy living together. We built a connection with the Austrian branch of PHAEurope. We were enrolled in the Austrian system, the people from the PH Austria have contacted us and said, “We have this association here in Austria. We are taking care of the patients in need. If there is anything else we may do to help, just let us know.”

We decided when we went through this process, that our knowledge that we gathered these years would be important or very useful for people in need in Romania. We decided to help anybody, any person who might need help. The idea of going in the Romanian Association and helping the Romanian Association came when they started to prepare the first patient doctor’s meeting in Romania. I went there, I met the guys in the Romanian Association, and that’s how I started working for both of them, bringing my knowledge as a former manager and these two years, making things happen also in Romania for the Romanian patients.

My wife, she’s a very, very resilient, very tough person. She never considered failure as an option in anything she does. When she got the diagnosis, she said it from the beginning, “We have to find a solution, there is no other option.” I have the same mindset, I think that’s why we have such a great relationship and a great family, I may say.

Everything that came… When she entered into the OR in the hospital in December 2023. She was laughing with the doctors when she entered in the operating room. She made jokes with them and she laughed. After the operation, eight hours later when the doctors went out, they said, “We have never seen a patient laughing and enjoying the moments, because she knew that after the surgery, she will be a new person. Usually patients are afraid, they’re anxious, we have to calm them down, but this was not the case.” She had a quite tough recovery. She’s been for 24 days in the coma, because her recovery after the surgery was quite tough. After that, she started building her strength again every day, every day. She went through a three weeks recovery process somewhere in the mountains with physical activities, with mental preparation, with medication management and so on. When she came back, she said, “I have to go back to the physical status and the mental status I was at before.” And she achieved it.

One year later after the surgery, she started running, she started doing her daily routine in exercises, physical exercises. But everything comes from the mindset. If you have the right mindset, you can go through easily. The most important thing for people in this situation or with these kind of issues, your status, your mental status is very important. This is something that everybody’s supposed to know and everybody’s supposed to try to achieve it. Be prepared and be sure that the stars will be aligned for you.

My name is Florin Dumitrescu and I’m aware that I’m rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com. @EuropePHA

She’d Never Been Sick — Until a Rare Disease Hit Like a Brick Wall

Terri Keplinger went from healthy and unstoppable to fighting for breath after unexplained swelling and a shocking ER visit led to a pulmonary arterial hypertension diagnosis. In this episode, she shares how denial turned into purpose, and how she transformed her fight into advocacy, support, and a mission to help others get diagnosed before it’s too late.

My name is Terri Keplinger. I’m from Johnson City, Tennessee. I am a patient. I was diagnosed June 15th, 2021. It took me probably a year and a half to accept that I had a disease. I had never been sick in my life. I started swelling pretty bad. About 50 pounds of fluid later, I couldn’t mow my yard. I was used to mowing my yard. I couldn’t walk from my car to the front door of my workplace, which I was the manager of. A customer actually looked at me. She said, “I just want you to know I’m a nurse and you have something really, really wrong with you.”

I shrugged it off. My family begged me to go to the doctor. I just had never been sick so I didn’t think that there was really truly a whole lot wrong with me that I couldn’t fix myself. For about a year and a half, things just kept getting worse and worse. Finally, my family said, “If you don’t call the cardiologist,” he was our friend, they said, “If you don’t call him and get an appointment to see what’s wrong with you, we’re going to take you ourselves.” I ended up giving him a call. I went to see him, and the next day, he called my work and he said, “Meet me at the emergency room within two hours.” I said, “Okay.” He said, “We’ll talk about it when you get there.”

I got there before him and the nurses were like, “Why are you here?” I said, “Well, I don’t know. I don’t know why I’m here. My cardiologist said to meet him here, and I’m not sure exactly what’s going on.” About seven doctors started filtering in to try to figure out what was going on with me. Finally, my specialist from Knoxville had come in. He said, “Oh, she has pulmonary hypertension.” I had no earthly idea what that was, what meant. I thought it was a joke. I thought they were all kidding and it was a horrible, practical joke, to be honest with you.

Four days later, I get out of the hospital and they give me these list of things that I need to do and all these medications, and I’ve never taken any medicine in my life. It was all just really new. I was a gymnast growing up, and hiker, avid outdoors person, and they’re telling me not to do a whole lot. It was just surreal. I couldn’t wrap my head around any of it.

So fast-forward about six months and I decided I needed a second opinion, so I go to Cleveland Clinic and same thing. 10 days in the hospital there and numerous tests and they were like, “You have pulmonary arterial hypertension.” I couldn’t believe it. I couldn’t accept the fact that was what was going on in my body. I came back to Tennessee and I go back to work, and in the meantime, my owners had asked me to step down from my manager position because they knew I would just die at the counter. I was broken-hearted about everything. The doctors had said, “Don’t Google it,” so I didn’t. I didn’t do any research. I was just blindly going through the motions of just taking the medicine and trying to keep these symptoms down from this disease that I have that I don’t even know anything about.

I got back from Cleveland and I needed some more medicine. I ended up calling my original specialist whom I deemed that I needed a second opinion from because I just didn’t believe anybody. I called him and I just apologized. I was like, “Look, I’m really sorry.” He said, “I don’t know how you feel.” He said, “I couldn’t imagine what you’re going through right now, so we’ll just forget everything from the past and we’ll just move forward.”

He had put a seed in my head about the support group because he is two hours away in Knoxville and he didn’t have the means to have the support group here, so he had tried to make a huge support group between Knoxville and Johnson City area. He had just been such an awesome doctor, I was like, “You know what? I’m just going to jump in. I’m just going to do this. Let’s just do this. I don’t know anybody with this. I would love to talk to somebody that actually has this issue.” That’s how I became a support group leader. He had just made mention that there was a lot of people in the area that he wanted to reach out to but just didn’t have the means. So I said, you know what? I think I can do that.

I started the support group here in the area, and we don’t have many people, which is good. It’s definitely given me a lot of different things, to be honest with you. Not only the friendship, but the camaraderie, the support. It’s been life-changing to accept this and start to move forward in how can I help others? I’ve not ever been one to take care of myself, so to have to spend every day thinking about me, it’s different. It’s different. I feel selfish a lot, but having the support group and helping with people who have this, it’s really helped keep me going. I’ve never had to deal with any sort of illness, and then to be smacked with this kind of illness, it was like hitting a brick wall.

I have a guy that I knew from high school and we got together about two years before I was diagnosed, and he has been an absolute rock. I know he was putting in my life for a reason. I used to question, why would this happen to me? Why did this happen to me? Well, now I know. It was all just to lay the foundation for everything to happen up to now. It’s actually been really crazy. I didn’t know why I had my children then and why did my daughter have her children then? Well, because I had to get sick later. I am at least well enough now that I can play with them and love them. My children are amazing. I have two kids and I’m very close with my family. They have been everything for me. I wouldn’t be here right now if it weren’t for them.

I just recently had to quit. I had taken over a store that was going bankrupt and I had built that up to a profitable store. It was my baby, basically. I was there all the time, I was working my hind end off. At first, I didn’t think that it was out of love and concern from them, but later on, I realized that it was because I was just so angry. But they asked me to step down out of concern for my health because it’s a very stressful job and they knew I loved it, and they knew that I wouldn’t step down otherwise. Again, there, I got to go back to my original store and work with a gentleman who helped save my life, as well. I’ll never forget those guys. They really cared. They didn’t want anything to happen to me so they let me continue to work, but in an environment that if I needed to leave, they let me come and go as I pleased. If my body let me do three hours of work that day, then I could. If it didn’t, I didn’t have to.

So, it was amazing for about two years that I was able to work when I could and not if I couldn’t, so I was very fortunate to still be able to do that. I’ve gotten a little bit worse, so I decided that it would be in my best interest to go ahead and quit. It’s been different. Worked my whole life and to just kind of take care of me all day long is a bit different.

I ask God to speak through my mouth because I know I was put on this Earth to help people my whole life, and that’s what I do. The day that I was told that I had to step down from my position, I was leaving for St. Augustine for my birthday. I go to St. Augustine, I was angry the whole week, even though it was wonderful. The last day, and I kept saying, “I just want to talk to somebody that has what I have.” The very last day, I begged my guy to stop at the farmer’s market, because I didn’t want to leave. I knew when I went home, I would have to go to the sales job and I didn’t want to do that yet. So, I was stalling as much as possible, and we’re walking around this farmer’s market and I’m just kicking rocks and stuff. I heard this woman say something, and I just blew her off.

The next thing I hear her say was pulmonary hypertension, and I whipped my head around and I said, “Excuse me? What did you just say?” She said, “You have pulmonary hypertension, don’t you?” I said, “What? Yes. Do you?” She said, “Yes. It took me seven years to get diagnosed.” It was so crazy. We sat and talked for probably two hours. I’m dumbfounded every time I speak with people about how long it’s taken for them to be diagnosed.

One of my support group girls, her brother passed away last year, and they said it was from pulmonary hypertension but he wasn’t diagnosed. It’s now my passion to educate, educate, educate, because so many people are being misdiagnosed or taking forever to get their diagnosis, and they’re getting worse and worse and could potentially die, and it’s not right. I’m dumbfounded. I get it that it’s a rare disease, but we’ve got to educate people so people will quit dying. It’s horrible.

So my dream is to travel the country. I love rocks. I love rocks. They are just fascinating on how they’re made. They’re made with heat and pressure and time. Just with a small change in a mineral, it could make the craziest rock. It’s amazing that the Earth makes that kind of beauty through pressure, heat, and time. It just blows my mind. We’ve been to Oregon and South Carolina. We’ve been in a lot of different places to dig for rocks, and I’m going to do this one day. It’s going to be called Digging for Change. I’m going to travel around the country and my guy and I are going to dig for rocks, gemstones, minerals, and we’re going to educate the United States on pulmonary hypertension and just travel around the country digging and educating, because it’s imperative that people know so people can live and have a better quality of life. It has to be, it has to be.

My name is Terri Keplinger, and I’m aware that I’m rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com.

From Newlywed to Newly Diagnosed

Six months after saying “I do,” Rey Adam Rodriguez was told he had pulmonary arterial hypertension. Rey shares his whirlwind journey through misdiagnosis, a new baby, and finding hope through expert care and peer support.

My name is Rey Adam Rodriguez. I was diagnosed with pulmonary hypertension in 2022, 6 months after I just got married. The time of my diagnosis was a whirlwind for my family. I’ve always been a healthy young kid playing every sport, baseball, basketball, football, soccer growing up. A little after high school, I started running, noticing I couldn’t really run as far as I used to. I thought, “Okay, I’m gaining weight.” I pushed it off as cardio. I started working out a lot more, taking the pre-workout. One time with my wife, I went to work out and I felt a sharp pain in my chest. I went to the emergency room and they said I just had high blood pressure. One month before I got diagnosed, I was actually in Florida playing slow pitch softball. It was one of my hobbies that I do now.

I was running around, working fine, but the time I got diagnosed, I had just started a new job so I had to walk to a shuttle. I walked a few steps up and just got dizzy. I felt like I was about to black out. I got lucky. My wife, me and her, we drove in together, so I had to call her, tell her, “Hey, come on back. I don’t feel so well.” She looked at me and she saw I was pale, sweating. At first, we thought it was my blood pressure, because I was 26 on blood pressure medications. She said, “Okay, let’s go take you to urgent care.” From the medical center to our house was about 30 minutes. We drove all the way back towards our little area, urgent care. They took me back real quick and they said, “There’s really nothing we can do with you. We suggest you go to the ER.”

I went to the emergency room that we have in our little town. They rushed me back. They freaked me out, because after they did the EKG, they brought in a crash cart. They suggested I was having a heart attack. I was like, “What was going on?” A little backstory about us, when I say whirlwind of the month, my father-in-law had just went into the hospital and my wife just found out she was pregnant with our first son. My father-in-law was diagnosed with Stage 4 cancer. He beat it and got the surgery. A week after he came home, that’s when I went into the hospital.

So, at this time, we’re both freaking out. My wife was answering all the questions for me because I was just shocked. The first EKG was abnormal. The second EKG, they said, “Okay, you’re fine, but something is going on.” They did a CAT scan. They told me, “Hey, we’re going to send you to a hospital down the street from us.” I went to that hospital. I stayed in there for a week. I was passing every single test until the stress test.

When I did the stress test, by the third phase, when it started going up higher, I almost passed out in front of the doctor there. They rushed me to go get a heart cath. Again, I’m 20 something years old, I was freaking out. My wife was at work, she rushed back home. After that heart catheterization, they said, “Okay, you do have something called pulmonary arterial hypertension.” We didn’t know what it was. We looked at Google. Don’t look at Google for this diagnosis, that’s what I’d say. We were freaking out, but the doctor told us, “Hey, we already talked to a team in the Texas Medical Center in Houston. They have a team waiting for you. We’re just waiting for a bed for you.” Within that next day, I was in the medical center. I give Dr. Sandeep Sahay thanks for everything that I go through, because he came in so professionally. By 10:00 that next morning, he answered all our questions. He told me, “You’re young, you’ll do great with the medication. You caught it early. But if I’m worried, then you should be worried, and I’m not worried at all.” That’s what calmed me down after that.

I’ve been diagnosed for now three years. At that time, we looked at Google. Life expectancy for pulmonary arterial hypertension on Google was three to five years. Both of us freaked out. We were having a little one. My wife was really about to be a single mother in that short timeframe. I was thinking, can I do the normal activities I can do? Can I go work? Can I even lift anything? I liked to lift weights. Dr. Sahay came in and he told us, “Do not ever look at Google.” He told me that from day one, to stay off of Google.

He told me, “Everything will be fine. There’s new medication nowadays. If you stay on track, it will keep you going. You’re healthy. Keep on losing the weight, keep on doing what you’re doing.” But one big thing is I could not work out. I don’t work out, I just do it in other ways, walking, playing slow pitch softball. Thankfully, I still play. I test my limits sometimes, but I know my limits not to push, especially running-wise. Another thing, when my son was born, he told me, “Don’t lift too heavy.” I couldn’t see my baby’s eyes, I just had to lift them. I take my time. He knows that there’s sometimes I can’t run as fast as he can. He knows I’m easy with him. He still plays with me and everything.

My wife, at that time that I was diagnosed, like I said, her father was just diagnosed with Stage 4 cancer, renal cell. We didn’t know if he’ll make it either. At that time, she’s thinking, “Am I going to lose both my father and my husband at the same time?” Thankfully, we’re both still here and we’re both still pushing.

How I face each day is different. There’s days I wake up where I’m scared. There’s days I wake up where I feel great. The days that I’m scared, for example, one time I went to go work out, I tested my limits trying to go on a StairMaster. I woke up, got dizzy, never went to that gym again. I’m never doing that StairMaster again.

I didn’t know who to reach out to. I’m thankful for pulmonary arterial hypertension groups that are out there, because I reached out to people, they messaged me real quick saying, “Oh, yeah. This is this. You might think you have the regular body that you used to have, but you got to remember, you’re going through something.” That’s what really helps me, because there’s days I’m scared where I’m like, “Is this the time I have to go on another medication? Is this the time I have to go back to the hospital?” I worry about that because I’m missing my son’s time. What helps me get through those days is to take a deep breath and seeing my son smile. Every single morning, he’s either looking for me or his mother, and to us, that makes our day. I got to keep on fighting for him.

The days I feel real anxious, stressed out, I just sit back and take a moment for myself, take a deep breath and reflect. Even though I’m going through this, there’s other people going through this and we’re all going to get through it together one way or the other. Either support groups or just even talking to somebody that has it, that really helps me. Even though other people are going through it, they have the will to keep on going, that makes me have the will to keep on going. I listened to a person saying every day, they walk a few miles a day, they feel better. Okay, you know what? Let me go walk with my son just a few blocks a day. It makes me feel better. It shows that even though you’re doing something alone, you’re not really alone. If you need to reach out to somebody, someone in those support groups are going to be there for you.

There’s times where I’ll come home freaking out in the middle of the night. My wife, I love her to death, but she just doesn’t know the feeling of, “Okay, we have this.” There’s days I’ll go scanning through Facebook or the internet about this disease that I freaked myself out about. I just go look in the support groups I’m in on Facebook. Other people are going through this but they have the answers I’m looking for.

My biggest thing was can I reproduce after this if me and my wife wanted to have another child. My doctor said, yes, I could. I’ve seen on my groups, few men have had other children. I was so panicky about my son having it. We went through the doctors, and we did the genetic test and he doesn’t. We were lucky. Seeing that everyone else can keep on continuing with their life shows me I can keep on continuing with my life. Don’t hold back on anything. Don’t miss out on little things. Right now, we’re getting ready to go to Destin, Florida, me and my whole family. There’s things that are like, “Ooh, should I really?” but I’m like, it’s life. Keep on going. Keep on making your day a little bit happier than you are.

My wife, I say she’s my rock. She’s the strongest person I’ve known, because at the time of my diagnosis, her father had cancer. She pulled through where I would never think she would’ve. She held strong. She was there for me. Every time that I cried, the times in the hospital where I just did not know what was going on, that I felt that blank stare, what’s next, she just held my hand and said, “We’re going to get through this together. We’re going to get through this together.”

A year and a half ago, my mother found out she had cancer. The first person she asked for to be in there with her was my wife. Not me, not my brother, not my sister. It was my wife. My wife stood strong and she stood there right there by her, and even though my mom didn’t want her telling me the stuff that was going on, she still told me. She told me, “Relax, it’s going to be okay. We’re in the right care team.”

In my defense, Houston Methodist is one of the greatest hospitals because they helped my father-in-law, my mother, and they’re helping me, so we owe a lot to them. But yeah, my wife is my rock. She holds down the fort with my son. The days that I just come back and I’m like, “Okay, I’m tired. Give me five, 10 minutes.” The days that I just need reassurance, she just tells me, “Okay, it’s all going to be all right.” In the beginning of our relationship, in 2016, I wasn’t a church person. When I met my wife, I knew that, okay, I’m going to church. I believe in God. I changed my whole life around, from going out to playing all day to just staying at home and going to Sunday church.

Growing up, I hated going to doctors. I never wanted to go to the doctor. I always pushed everything aside. Before I was even diagnosed, I kind of pushed it to the side saying, “I’m going to be all right. It’s my blood pressure. I already have medication.” Just walking a few steps, that’s when I started feeling dizzy. I said, “I’ll go to the doctor in a month, I can push through it.” I kept on going, “Okay, I got a few weeks before I go to my next doctor’s appointment.” I’m still feeling that little loss of breath. I seen stars until I finally told my body, “Okay, it’s time to go to the doctor.”

Doctor Sahay and his team at Methodist, once I got introduced with them, they’re very hands-on. I got all my doctors through him. If I have a question, I message him. Within 24 hours, they’re messaging me back. One of my first questions I remember, because I love going deer hunting, “Can I go deer hunting?” He said, within the next couple of hours, “Go deer hunting. Enjoy your life. Just remember, bring a pulse oximeter. Don’t go too high.” He told us the first day that he met us, “This is my card. You have a question at midnight, you call and text me. You have a question you want to put on MyChart, MyChart. My whole team’s going to be there.”

Anytime I have any little feeling wrong, I message him. Real quick, they message me. If I’m having digestive issues, I told him, I felt heartburn sometimes, “Hey, I got you appointment with a GI doctor,” all ready for me. He said, “Everything comes through me. If another doctor wants to prescribe you medication, make sure they ask me first, because I want to make sure everything going in your body is right,” so that’s why I give him everything.

Every time I message him or see him, I just get happy, because I know he’s a great guy. He asks about my son every single time. He asks about my wife. Anytime I go to a doctor’s appointment without my wife, he’s like, “Hey, call your wife. Put her on FaceTime so she can hear us.” His support team was great too.

I always keep on pushing the limits to know my body and know what I can and cannot do, but at the end of the day, I want to make sure I’m here for my son. One of the things I told my wife when I was diagnosed was I want to beat this thing. Even though you can’t technically beat it, I want to show that you can live with pulmonary arterial hypertension longer than the three to five years that Google says.

My name is Rey Adam Rodriguez, and I am aware that I am rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com.

She Was Prepared for Breast Cancer Not for a Rare, Incurable Disease

At 30, Sherry Rouse went in for a breast cancer screening. What doctors found instead wasn’t cancer, but was something far rarer, and potentially fatal: idiopathic pulmonary arterial hypertension Sherry recounts the emotional rollercoaster of being blindsided by a rare illness and how she turned that chaos into advocacy and hope.

My name is Sherry Rouse. I am from the Bay Area, California. I was diagnosed with idiopathic pulmonary arterial hypertension in 2016. I feel like my story is a little different than many of the stories of people that I’ve connected with over the years. I went in for a routine breast cancer screening. I was 30 years old. My mom had breast cancer really young, and so I was on a really intense breast cancer screening schedule. I got an MRI and the doctor called me a few days later and said, “You don’t have breast cancer, but you do have an enlarged pulmonary artery.” I was like, “What is that?” I had no idea. He said, “I recommend that you go into the ER right away.”

I was working. I was actually getting ready to go to a field trip with my fourth graders. I’m a teacher. From there, I checked myself into the ER. They ran all the tests right there that day. They didn’t diagnose me that day, but had a pretty clear idea of what my diagnosis was probably going to be at that point. They sent me home and about a week later I got a call from a pulmonologist. The pulmonologist asked me to come in and he gave me the diagnosis of pulmonary hypertension.

I was totally caught off guard. I feel like I had for quite a few years mentally prepared for a possible early diagnosis of breast cancer, because my mom got breast cancer before she was 30. She did pass away. I was mentally prepared for an early diagnosis and, “we’ll take care of it.” To get the call saying I had an enlarged pulmonary artery and just being totally floored, totally taken back and really confused.

My pulmonary hypertension is idiopathic. It’s not something that runs in my family. My family is generally pretty healthy. It was incredibly confusing and obviously really jarring once I learned what was going on.

The early days were just really confusing, really anxiety inducing. I went to the pulmonologist. He was wonderful. Shout out to Dr. Gupta at Kaiser back then. He really clearly gave me a diagnosis. He walked me through what pulmonary hypertension is. He asked me all the questions about my history and about my lifestyle, ran additional tests. I got so much blood work done during that time. Then, we came to the diagnosis of idiopathic pulmonary arterial hypertension. I was diagnosed with severe pressures. They were, I believe, close to a hundred at the time of my diagnosis.

Luckily, my parents who were living out of town at the time, they happened to be in town through this whole process and extended their stay, so I had their support through the whole diagnosis and going on medication. Juggling that kind of anxiety and then also still needing to go to work, really being again, just incredibly confused, like, “How is this my life? What just happened to me?”

Being newly diagnosed was an incredibly tumultuous time. My now husband and I, we were only about six months into our relationship. The second time that my husband met my parents was after I got out of the hospital on my day of diagnosis. It was a very new relationship. We had to navigate my PH diagnosis and what that means for us and our future, essentially from the beginning, which has its pros and cons, but I think it’s made us stronger in the end.

My husband and I had the opportunity to adopt our daughter recently, so she’s two and a half now. I was also told at that time that I wouldn’t be able to carry my own children, which was something that I always dreamed of doing, and that was taken off the table pretty immediately. I remember at the time being less worried about that. I was 30. I was in a new relationship, but I was more worried about living than I was about carrying my own children. I think the grief around that came much later. I was just like, “I don’t care. I just want to live. Just tell me how I can stay alive and we’ll figure out all the rest later.”

PH has really put a lot of things into perspective for me around what matters and what things to let go. My diagnosis was close to 10 years ago now, so I’ve been through a lot of ups and downs in terms of mental health. When I was first diagnosed, I just had my eye on how am I going to live.

I did the infamous Google search that I think everyone newly diagnosed does where it says that you have about three to five years to live. That put me in a really, really dark space. I remember, I think it was my sister that said, “How do you feel now? Are you okay now?” And I was like, “Yeah, I actually feel totally fine now.” So she said, “Just focus on that. Focus on today you got up. Today you feel fine. You’re going to go and do your thing and live your life. And if that changes, we’ll figure it out.”

That’s kind of just been my MO since. I feel okay and I’m really lucky. I am in a very, very lucky space that I did have an early diagnosis, that I wasn’t feeling a whole lot of symptoms before I was diagnosed. I’ve been doing really well on medication where I live my life pretty normally at this point. I just know that that is such a privilege.

In the days that I do feel anxiety or, “woe is me,” because of my diagnosis, which isn’t often at this point anymore, I really just think about one step ahead of me, like, “Today I feel okay. Today I’m going to go take a walk in the sun. Today I’m going to go do yoga,” and remind myself that I’m living and I’m okay.

Moving my body has been a huge reprieve for me post-diagnosis in a way that I wouldn’t have imagined. Before my diagnosis, I had a lot of shame around what my body couldn’t do. I’ve never been the fastest runner or the most athletic person on the team, and I had a lot of shame around that growing up.

Post-diagnosis, getting on medication, it helped take away a lot of the shame. It’s a shitty disease. It’s devastating to get this diagnosis. But it also has the ability to bring a lot of perspective. Something that has been just a huge shift for me, post-diagnosis, is the way that I view my body and the way that I view my body’s abilities. It’s not attached to shame anymore. I’m proud of what my body can do. Even if that means I go hiking with friends and I’m the slowest one out on the trail, I’m like, “Heck yeah, I’m out here.” It makes me focus on being strong instead of looking a certain way or getting skinny. It has been more of like, “I want to be strong. I want my heart and lungs to be healthy. Exercise and movement is the best way I know how to do that.” It’s been such a huge tool for me with my mental and physical health. Even on really hard days, keeping movement central to my day and keeping it a priority has been just a game changer for me.

At one point, I went on a medication that gave me a lot of side effects. I’m not on that medication anymore. It was challenging, and some days I could barely function. My headaches were so bad. My flushing was so bad. As long as I could get up and ride the Peloton for 15 minutes or get out on a walk or just do some stretching, I would call it a win. It just has totally reframed my experience of myself and my experience of PH. That’s not something that I would trade at this point, because I really think that it’s been a huge tool for me.

I’m in elementary education, so it’s a pretty physically demanding job. I’m on my feet all day. I’ve been working full-time since diagnosis. I’ve never stopped. I’m also in grad school right now to hopefully become an administrator. So that will keep me on my feet all day every day. I’m not planning on letting PH slow me down yet. Hopefully not for a while. My daughter also keeps us real busy and on my feet, so I’m constantly on the move.

I have dipped my toes in a little bit into the support group space. I think if we’re being honest, I wasn’t able to relate to a lot of other people. PH is so different and it impacts people in so many different walks of life. When I was diagnosed, I was 30. The idea of carrying my own children being off the table for me was, at one point, really devastating. In my small dabbles into support groups didn’t find a lot of support with the things I was going through, specifically.

But I have found a lot of support actually in the social media space. Mostly on Instagram. I’ve been able to connect with such an interconnected group of women that are similar to my age that really have helped to support me. I think we support each other. If I have a new medication that I’m adding on, I’ll reach out to some people that I know are on that one already. I’ve had phone calls, I’ve had message exchanges with a lot of people that are going through PH in a similar way that I am that I found really, really, really helpful and empowering.

With this disease, research is so important. Right now, we’re at a critical space in time where funding for research is being pulled. I just think about the devastating impacts that it’ll have, not just on the PH community, but on the chronic illness community. I think that it’s important now more than ever for people to dip their toes into advocacy spaces in the ways that they feel comfortable doing to raise awareness, because when I was diagnosed 10 years ago, I am currently on a medication that wasn’t available then. My numbers are back down to normal at this point. So, the constant research and innovations in the medical space are so important. There’s a lot to be said for the funding of the research. We need to keep advocating for research and innovations for the pulmonary hypertension community.

I think the advice that I would give to my younger self, first of all, don’t Google, please don’t Google. Along with that, find a doctor that you trust. Obviously, advocate for a second, third opinion as needed, but find a doctor you trust. Everybody is so different and responds so differently to treatments. So, listen to the advice of your doctor rather than going down Google rabbit holes.

I find that when I talk to my doctor, she brings me a lot of hope, realistic hope. She was like, “If this medication doesn’t work for you, we have more medications that we can try. We have other routes that we can take. There are new medications coming up the pipeline that we can try. There’s always something that we can do to help maintain your quality of life. You don’t have to live a miserable life.” That was just so powerful for me to hear, because you think of this disease as life-threatening, progressive. Those words are really scary. But there is a lot of options out there.

Then, find a community that brings you hope. I have, again, connected with a lot of people in the social media space. We all are going through challenges. We all try medications that don’t work or that make us feel like shit. But people are out there living really joyful, fulfilling lives. So, to connect with people that are going through some of the same struggles, but also finding joy in their own lives has been hugely empowering for me.

My name is Sherry Rouse, and I am aware that I am rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com.

The Insurance Plan You Never Knew You Needed

What does a lung transplant have in common with car insurance? More than you’d think. Dr. Nicholas Kolaitis shares why seeing a transplant team early, even if you’re stable, can help protect your future.

I’m Nicholas Kolaitis from the University of California, San Francisco. I’m a pulmonologist who does lung transplantation and pulmonary hypertension. The thing that I want to talk about today is lung transplant, and some of the issues surrounding lung transplant, as well as, why it’s important to engage with the transplant team as a patient with pulmonary hypertension.

First thing, is a couple of disclosures. One is that, as I said, I’m a transplant doctor and a pulmonary hypertension doctor, and I’ve lost people, patients of mine, who were referred to transplant late. Patients who maybe could have had an opportunity for transplant had they been referred earlier. That is not something that I want for anybody. I want transplant to be an option if people need it.

The second disclosure is that getting referred for transplant, talking to the transplant doctors, being evaluated for transplant, thinking about transplant, does not mean that you’re getting transplanted. So that, I think, is a big misconception is that when somebody brings up transplant, it’s not that you need the transplant right away, at least it shouldn’t be. If the pulmonologist or the cardiologist who’s taking care of you says that you need a transplant now, it’s probably too late for you to actually go forward and be evaluated. You probably can still do it, but it makes the road much more challenging.

When we think about transplant, the question is what is transplant and why do we do transplant?

There’s sort of two aims of transplant. One is to improve somebody’s quality of life, and the other is to improve their survival. Transplant is quite effective for both of these things. So, if you look at people who’ve undergone transplant at my center, for example, at UCSF, our median survival post-transplant is about 11 and a half years post-transplant, which means that these are people who you would think would not have been living anymore without the transplant. We typically only think about putting people on a transplant list if we worry that they’re going to die within the next two years without transplant. So, 50% of them are alive 11 and a half years after the transplant. That’s pretty good at extending your survival. Our longest survivor is over 30 years out of her transplant. That’s not an uncommon thing that we see people throughout the world that are decades out of their lung transplant.

The other is that it improves your quality of life. So, you don’t need to be on parental therapy anymore, if you are. You do need to be on a bunch of different medicines. But people who’ve undergone transplant, we’ve looked at this at our center, they’ve got dramatic improvements in quality of life, they improve their physical quality of life, they improve their mental quality of life, they improve their respiratory quality of life, depression scores get better, frailty gets better, cognition gets better. All these things get better after transplant. So, transplant can be really effective for the right person.

So, sounds pretty great, right? Well, why do we wait?

We wait as a transplant team to do it until we actually need to because we want you to live as long as possible, right? No transplant center wants to transplant somebody too early. Getting referred to your transplant center and talking to your transplant team about transplant early, that’s great. That’s not a bad thing. Transplant is scary though. It’s a major surgery and although it sounds great, it’s not always perfect. There’s complications to major surgeries, there’s risks to major surgeries. It can be problematic.

So, how do you make it as safe as possible? Well, you time it right.

You make it as safe as possible by being seen early, getting evaluated early, learning about transplant early, getting all your ducks in a row early. I say this a lot to our patients that I see in the transplant clinic is — think of me like an insurance plan. Think of our transplant team like an insurance plan. I don’t want to pay my insurance. I don’t want to pay my house insurance. I don’t want to pay my car insurance. I’m sure none of you do either, but you do it because if you get in a car accident or if your house burns down, you do it because you would actually recoup the loss. So, consider your referral to transplant like an insurance problem. I don’t want to be referred to transplant, I don’t want to talk to the transplant doctors, I don’t want to go do these tests for transplant. But do it because if something goes wrong, they’re ready and you’re not starting from day one to try to fix things. That’s the reason to get referred for transplant.

So who should get referred for transplant?

We’ve looked at this in a number of different ways. There’s different societies that have put out various statements on who should get referred for transplant and when we should get referred for transplant. Those are mostly sort of consensus, sort of expert opinions from people that are in the field. We also recently did this Delphi process through the Pulmonary Vascular Research Institute looking at, there’s about 30 of us around the world that answered questions about transplant. We recently published this looking at who should start conversations about transplant, who should get referred. Basically, the short end is if you want to get referred for transplant, you should get referred for transplant. If you are not doing well despite being on appropriate therapy, if you remain sort of in the higher risk category or in the intermediate high risk category, despite being on appropriate therapy, you should get referred for transplant.

Getting referred is, sort of again, do it earlier. The worst thing that can happen is that you’re not referred or you’re referred too late. You’re referred at the time that you’re so sick that it makes transplant nearly impossible and then it’s not an option. Again, we don’t want to transplant anybody if we don’t have to, as a transplant doctor. Our goal is not to transplant people. Our goal is to transplant people that need it though, and to make it so that’s as safe as possible.

A couple other things just with transplant and pulmonary hypertension in particular is that we need better access to transplant for patients with pulmonary hypertension compared to other diagnoses. In the United States, the system that allocates organs for lung transplant uses a scoring system that’s based on various clinical parameters. These clinical parameters are more relevant to people that don’t have pulmonary hypertension; people with scarring of the lungs, people with COPD, emphysema, people with airways problems but not pulmonary hypertension, because a lot of the parameters that go into here are not relevant to the blood vessels of the lung, but their relevant to the lung tissue itself. So patients with pulmonary hypertension have unequal access to transplant. Patients that are with pulmonary hypertension have the lowest likelihood of transplant and the highest rate of dying on the waiting list. That is because of the scoring system.

So, if you’re referred late, that just exacerbates the problem, because the doctors don’t have enough time to get you the transplant. We can submit exceptions to try to get patients higher scores and actually get them a transplant, but still that takes time. The system’s not fair for patients with pulmonary hypertension. So, getting referred late makes it even harder. We will do everything we can to get you transplanted and we will get you transplanted, but you got to come to see us earlier. That’s the thing.

The other benefit of it is that it demystifies what transplant is. Transplant is scary. We talked about that. But it also can be really amazing. I think that one of the things that’s really helpful is to talk to somebody who’s gone through it. Not just the doctors who are on the other side, but somebody who’s gone through it, who’s sat in your shoes. If you get referred early and you’re scared, if you get referred early and you are worried about it, we’ll set you up with somebody who’s gone through it. We’ll set you up with somebody who was you four years ago, and is now living their best life and traveling around the world. We’ll set you up with somebody who was you and is now off parenteral therapy and is swimming.

This is not to say that that’s what you want in that moment, but it is what your life can be after transplant. Transplant can be incredible and can be amazing when it’s done to the right people at the right time in the right scenario. Whatever it is, talk to the transplant team early, get demystified about transplant, and work closely with your PH team and your transplant team together for the right thing.

Getting a second transplant is not incredibly common across all lung transplant types. I would say for pulmonary hypertension, it’s probably much higher than normal because patients are young. We consider a second transplant and somebody who for whatever reason doesn’t have good lung function the first time around, or did well for the first time around and then had a rejection episode, a problematic episode that developed them to have worsening of their lung function afterwards.

When would you consider somebody for a second transplant?

Somebody that doesn’t have other organs that have gone down, their kidney is still functioning well, their liver is still functioning well, they’re not super frail. They are able to still function, do those things. What bodes best for that is younger people. We typically have a higher threshold for a second transplant in terms of the bar that a person needs to meet, but we do offer it. I would say about 10% to maybe even 15% of all transplants are probably a redo transplant. So, it does happen, but it’s primarily mostly younger people that need it, which is common in our population compared to other diagnoses where we do transplant. It can be really successful. We have people that are decades out of a second transplant and they’re doing great. The second transplant is always more risky than the first because it’s a second surgery.

I think the other thing is more unique to pulmonary hypertension is that the surgery itself is fraught with more risks as well, because when you think about transplant in general, one of the things that happens is you’re taking a donor lung and you’re putting it into a recipient. When you do that in a patient with pulmonary hypertension, the right side of the heart, which is the part of the heart that pumps blood in the lungs is used to high pressure in the lungs. So, now you replace that lung with a lung that doesn’t have high pressure, and the right heart tries to pump blood into that lung and then you can get an injury to that lung right after the transplant. So, patients with pulmonary hypertension are at risk for problems right after the transplant, which is all the more reason to try to do it as safely as possible and try to do it in the right way possible, which is all the more reason to be seen earlier and learn about it earlier because it is scary. But doing it in a controlled way is the highest likelihood of success.

Survival’s getting better and better and better. If you look at our internal cohort, our survival for the cohort that was in the early 2000s to 2010s is not as good as 2010s to 2020, it is not as good as now, so survival has gotten better over time. I think, in particular, with patients with pulmonary hypertension, we’ve gotten better at transplant, as well. I talked about this risk that they can get right after the transplant where the lung gets injured. We’ve gotten much better at supporting people through that. What we’ll typically do to support people through that is they, we will use a different kind of life support machine than we normally use after transplant. Normally, after transplant, people come out on a ventilator, which is a breathing machine that you may have seen on TV shows, things like that. But after transplant, we’ll use a different one that’s called ECMO. This is a pump that actually takes blood out of the body, puts it through this pump, and then puts it back into the body.

We’ll use that after the transplant. The idea here is that you kind of re-control some of the blood flow into the transplanted lung with this machine because you’re sort of bypassing some of the blood that is not all going right through the lung. That allows us to prevent some of the lung injury that’s happened. If you look at data globally, historically, patients with pulmonary hypertension are at the biggest risk of not making it out of the transplant. They’re actually at risk of this big problem right after the transplant. But if you follow them long-term, they’ve got the second best long-term survival of any type of diagnostic group.

What’s interesting, is in more recent years, and if you look at our center, for example, our survival for patients with pulmonary hypertension is just as good as any other diagnosis. That’s because we’re using these sort of newer ways of managing people to support them through the transplant. We can get them through the transplant much safer. So, people can do really, really well and we’re getting better and better at it. I would say that pulmonary hypertension probably should be transplanted at centers that have a lot of experience transplanting pulmonary hypertension, because you’re rare and you’re unique and you need to be taken care of by people who know what they’re doing here.

When I think about success stories, one patient comes to mind.

I had a guy who had a rare form of pulmonary hypertension called pulmonary veno-occlusive disease. He’d been seen by multiple doctors and nobody really figured out what he had. Pulmonary veno-occlusive disease is not really amenable to treatment with our pulmonary hypertension therapy. So, really the only option we have is a transplant. Between the time that I met him to the time that he was in the hospital on 20 liters of oxygen a minute was about a month and a half. He was on room air when I met him. So, he just progressed rapidly to the point that we had essentially no way to support him, and he was going to die. But what had happened was when I met him and he was on room air and people thought I was crazy, I said, “I want to evaluate you for transplant.” He goes, “Are you sure? I’m not on any oxygen.” I said, “Just trust me on this. Let’s just evaluate you. Again, let’s do it as an insurance plan.” I didn’t think he was going to deteriorate that quick, but he did. So we evaluated him, we started the work up, and he deteriorated incredibly quickly.

Unfortunately, with PVOD, you can’t really treat that. There’s not a whole lot that we have in our armamentarium to treat it. Because we evaluated him early, because he bought in to being seen by the transplant team early and got evaluated. His window was open, he made it to transplant, he’s doing great, running a company, hanging out with his kids, traveling the world. So this happens. That’s one of many, many, many stories. So transplant works.

I’m Nicholas Kolaitis and I’m aware that my patients are rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware #phawareMD
Share your story: info@phaware.global
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The Most Dangerous Phrase in Rare Disease: “You Look Fine.”

Diagnosed with idiopathic pulmonary arterial hypertension in her early 20s after years of being dismissed, Lala Juarez knows what it’s like to be told “you look fine” while fighting for breath. In this deeply honest conversation, she shares her journey from misdiagnosis and IV therapy to the shock of being told it was finally “time” for a double lung transplant. Now four years post-transplant, Lala reflects on rediscovering everyday joys, navigating loss and trauma, and why support groups became a lifeline that made her feel seen, validated, and never alone.

My name is Lala Juarez. I am a support group leader for Northern Utah. I’m also a patient. I was diagnosed with idiopathic pulmonary arterial hypertension in 2015. I had two years of being undiagnosed through, as a lot of patients know, like asthma, whooping cough, allergies. I went through oral medications. I tried the Sub-Q and we were not friends. It didn’t work out. So I went to IV therapy. I had that for about two years. I had a double lung transplant consultation. The first one I took super seriously. I brought my best friend, I brought my parents, I brought everybody and was just like, “Hey, if we have any questions, if I don’t get everything down, you guys could tell me what happened in case I forget or if it’s overwhelming.”

They ended up telling me, “You’re too healthy for a transplant.” So, I was like, “Okay.” Then, a few years later, I had another consultation. I was like, “Oh, they’re going to tell me I’m healthy again,” because I felt like I was healthier as far as mobility and stuff. I was convinced they were going to say, “You’re too healthy.” They said, “It’s time.” I was very shocked. I had my double-lung transplant in June of 2021, and now I am four years post-transplant and doing great.

I’m back at work and I didn’t realize a lot of things that I enjoyed. I didn’t realize that I would be fond of salt. I didn’t realize I’d be fond of hot showers. I don’t know, just a bunch of things, even just bending over, I’m just shook, I could do it. I’m shook, I could go back to work and I could survive a full-time job and a lot of things that I had accepted that would never happen ever again, or at least not for a very, very long time.

So it was in the middle of COVID. All my loved ones were able to be there in the waiting room. I guess I was actually fine to have my sister and my parents in the recovery room. But I’m happy it happened then and not in 2020, because if it was at a point that nobody would be able to visit me and stuff, I think that would’ve been so hard. I know that there was people who did have their surgeries in 2020. I am just happy for me everything worked out the way it did.

Just being prepared. I think I’ve been over prepared. I did go to a PHA Conference in 2018. They have so many different classes or little groups that they have. At that time, I went to a transplant group (session). I was in a very receiving place where I’m like, “I’m not going to need this at least for another decade. So, let me learn about all this stuff now where I’m not trying to pressure myself to try to get all the information in and just be calm about the whole thing.”

What I learned at the conference was there was this one girl who had said that when she had her transplant, I think her medications were at such a high level whatever, she had forgotten a lot of things like her passwords and her family. I guess she was either scared of her family or something. I don’t remember exactly her story, but it was something along those lines. I was afraid of that. So, I told them all my passwords and I was like, “You guys, if I forget, if I get nervous, play me music.” I think in that way that I was able to get that information from other people who have lived it, it made me more prepared than I would’ve been had I not gotten any of that information. Any of us that has any problems, whether if it’s PH or if it’s any other medical related problems, I think they learn to advocate for themselves and to ask questions.

I didn’t start attending any of the support groups until a year or two into my diagnosis. Maybe it was after a year and I started attending the ones at the hospital I was at. Then, I started looking into other ones where I was like, “Oh, even if it’s not quite the way that I had perceived support groups before, like what I’ve seen on TV, like AA meetings or something like that.” I was just like, “What?” I just feel like the support groups and stuff are like, “It’s not like that at all.” So, I started attending them.

The original leader for our group started needing help, she’s a little bit older. It was me and another older lady who took over and I was just going to help. Then, unfortunately, my partner had passed away in 2020 and it wasn’t COVID related. So, now it’s just me, but that’s kind of how I kind of got thrown into it. The original leader ended up just slowly but surely kind of stepped back. So, now I’m just the only leader for my group for Northern Utah. But I don’t mind, I really enjoy these groups. I enjoy even after my transplant, I feel when I do attend them and when we talk about them. It’s not always negative things. I think it’s an escape for most of us.

I think even when we do have problems that we’re venting about, we understand each other like nobody else will understand us or like the “normies,” with people who don’t have any issues. And our caregivers, we need our caregivers, but some of them don’t understand. They could only go based off of what we tell them. They could only imagine what it’s like, but they don’t know exactly what it’s like.

I went to a group for my old hospital and I feel so seen. I feel like what I went through, especially during my diagnosis, I feel like it was almost like (not my doctors now), but doctors before, just a regular pulmonologist and a regular cardiologist. I feel like in ways that they were gaslighting me to believe that nothing was wrong with me.

I don’t know how much my age played a factor, because at that time, I think I was 22 when I started getting symptoms. Then I got diagnosed at 24. At first, they were just like, “You need to exercise more.” I tried my best to explain to them like, “I know what being out of shape feels like, and I know the more you try, it tends to get easier to do it. But the more I try, the more it hurts or makes it harder to breathe and it doesn’t end up getting easier like being out of shape does.” They were just like, “Oh, well, you’re young, you’re fine, you look fine.”

Being so young too, even cousins and stuff, they kind of looked at me like, “Wait, how can you not do this? You look fine.” Even talking to my support groups now, like I said, I feel so seen. I feel like everything that they talk about I experienced, or if I explain it in a different way, they’re like, “Yeah, that exactly.” So, me staying in this in a way makes me feel like what I went through, I wasn’t crazy. What I went through, there are other people who are going through this. I guess it makes me feel validated in a way that I didn’t just make it up.

I understand them in a way nobody else will. I feel like I can’t even say, “Oh, well, I’m not in this no more, goodbye.” No matter what, even though I am quote-unquote “healed,” and I no longer have PH, I already accepted when I first started having my illness, I will be popping pills for the rest of my life, I will still be going to the hospital and having my evaluations and checkups and stuff like that. So, no matter what, it’s not over, over.

I feel like I am different in so many different ways. In the weirdest way, I’m happy this all happened to me. I feel like I know empathy in a different way. I know how to just relate to other people who are going through issues differently than I did before. I feel like before I was more in a working mindset and too busy for everything and all this stuff. Now, I take time to be present with people.

I feel like everybody’s better off than me. A lot of people seem to be accepting of it easier and better. I feel like the way that I cope with trauma or whatever, is I was in denial about the whole thing at the beginning. It just took a while for me to accept it and be like, “Oh, I guess this is what it was.” I think part of it had to do with the two years to properly diagnose me where I’m just like, “Yeah, that’s what they say, but is it really what it is?” There’s a new normal and a different normal. I don’t have exactly that, but it’s not over, over. I’m not back to being a “normie.” I’m not back, or there’s no way of going back to where I was.

My name is Lala Juarez and I am aware that I’m rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com.

The Daily Discipline of PH: Living by the Liter and Label

Most people don’t count every sip of water or every grain of salt. Angela Brown does. This episode dives into the daily discipline required to live with pulmonary hypertension — from navigating restaurant menus to surviving a exotic vacations in sweltering heat with strict fluid and sodium limits.

My name is Angela Brown. I live in Toronto, Canada. In April of 2024, I was diagnosed with pulmonary hypertension. It started towards the end of 2022. I wasn’t feeling well. My legs would swell up and I would have problems walking and breathing. It got really bad. If I had to go to a hospital visit, my brother-in-law who was dying of cancer, when I walked with the mask on, I could barely breathe. I was hyperventilating. I knew something was not right, but because we were dealing with my brother-in-law’s illness and we knew he was going to die shortly, I didn’t tell anyone. Then, he passed away and we had the funeral. Then, I told my daughter, “I need to go to the doctor because I’m not feeling very well.” So, we went to the doctor’s office.

When we got there, I couldn’t even walk from the parking lot into the building. I had no energy. My legs hurt. I just felt like I couldn’t move and I stopped. It was raining and I just stopped in the rain and just stood there. She said, “What’s wrong with you? Come on, let’s go get inside.” I got inside the building and then tried to walk to the elevator and I passed out. That was the beginning of the journey.

They did some tests, chest x-rays. They told me that my lungs were congested. That’s all it was and I had nothing to worry about. They gave me antibiotics and that would solved the problem. I finished the antibiotics, but that didn’t solve the problem. They told me to go see my cardiologist, which I did. She did some tests. She said the pressure in my lungs was a little bit high, but she didn’t really explain what that meant. She said, “We’re going to have to send you to a lung specialist.”

They made an appointment and almost two months later, I went to see the lung specialist. When I got there, he said, “I looked at your chart.” I have a lot of chronic illnesses. He said, “But I can’t help you with what’s happening to you because I don’t work with that. I work with CPAP machines.” So, he sent me to a different lung specialist. It took another month and a half to two months. That was the first time I heard of pulmonary hypertension. He said, “Looking at your charts and everything else that I’ve seen, I think you have pulmonary hypertension, but I have to confirm it. In order to do that, we have to do some tests.” He did blood work. He did walking tests. He did chest x-rays. You name it, he did it. A month later, I went back and he said, “I still think you have pulmonary hypertension, but I still have to prove it.”

Then, he told me he needed to do a right heart catheterization. There, they told me that there’s a medication they could give me that would be able to help my pulmonary hypertension. They gave the medication and then, four weeks later, I went back and they did the test again. They said they have to wait a little bit longer to see if it would work, because so far it didn’t look like the medication was helping. So, I went back six weeks later. They gave me another medication on top of the one they gave me before. I went back six weeks later. They said they saw a little bit of improvement. So, it’s been over a year since I’ve been diagnosed.

Having pulmonary hypertension and having to take medication is not the only thing that you have to do when you have pulmonary hypertension I realized. Of course, you have to cut salt out of your diet, so no sodium or a little as possible. So, I have a low sodium diet. I can only have 1.5 liters of liquid per day, so I have to measure everything that I drink.

At first when they told me, I thought it was 1.5 liters of water per day, but when I went back they said, “No, it’s 1.5 liquid.” So, anything that produces liquid. If you have fruits, fruits produce liquid. You have to measure that. If you drink water, if you drink pop, milk, juice, whatever, it has to be measured and it cannot go above 1.5 liters per day. It is very hard. Some days are a lot harder than others, because you want to drink something and you can’t because you only have a little bit of liquid left. I’m thirsty, but I can’t drink anything. It’s very hard. No sodium is very difficult, as well, because everything has sodium in it. I didn’t even realize even a candy or a chocolate bar has sodium. So, basically, I have to look at every single thing.

I went to Jamaica for my niece’s wedding and it was crazy because it’s hot. When it’s hot, you want to drink more, but I can’t. I still have to, even though I’m in a foreign country, I’m on vacation, I still have to stick to the 1.5 liters of liquid per day. I still have to find food to eat that has no sodium or low, low sodium. It was a struggle trying to find something to eat. At a resort, every night, they had a guy who made stir fries. I told him, okay, I need stir-fry, but I need no salt.

Miraculously, he said, “Okay, I’ll get some meat from the back that’s in the fridge that’s not seasoned yet and I’ll cook it for you.” That’s what he did with the vegetables and the pasta. That’s what I ate every single night for seven days because it was the only thing I could find that I could guarantee 100% does not have sodium. I also went to a hibachi restaurant in Jamaica, as well. I told the chef ahead of time, I said, okay, no salt. Make my food first and then make everybody else’s food. That’s what he did. So, it’s always in your mind the no sodium and the liquid. It’s always in my mind. If I forget, I have my daughter who reminds me 100%. When I go to a restaurant, of course, I have to ask, “OK, what can you cook me that has no sodium?”

I try to find restaurants that cook from scratch, like right there. This restaurant, they cook it as you order it. So I can say, okay, I want salmon, please. Let me put some pepper on it. Don’t put any salt. Just put some pepper on it. Food doesn’t have the same taste it used to. I told my daughter, I said, I don’t eat for pleasure anymore. I just eat because I have to, because the food is bland. I didn’t realize that sodium makes such a big difference in food. I buy all the no-salt, seasoning that I can find. Mrs. Dash, this particular one that has no salt and other seasoning, Mrs. Dash, Clubhouse and some other ones. They make you food a little bit more tasty, but the food is a lot less tasty without sodium, but this is my life. This is what I have to go through.

Being someone who has a lot of chronic illnesses, I’ve gone through this process before many times, before getting a diagnosis. When you finally get a diagnosis, you’re like happy for a minute. You’re like, yes, but wait a minute, I’m sick. I just have something. They told me my prognosis was severe, so you have severe pulmonary hypertension. So I was happy that finally you can tell me conclusively, this is what I have. But then, I remember all the things that I’ve read and all the things that go along with it. I started reading. I remember Natalie Cole, the daughter of Nat King Cole, died of pulmonary hypertension. I never heard of pulmonary hypertension, but suddenly I can tell you stars that have died from it and all these things. Each time I tell my daughter, she tells me the same thing, “Mom, stop reading Google.”

It’s just a cycle of doctor’s appointments and letdowns and going to the doctor and hoping for a diagnosis and being let down. To just go on and on like that for two years, it’s rough on your mental health. I think anybody who has a chronic illness is resilient, because you have to pick yourself up and go every time. We go to the doctor’s appointment, you feel down, but you have to pick yourself up. You have to keep going. I would say to anybody who’s just being recently diagnosed with pulmonary hypertension, try not to Google everything and try not to read so much stuff about it, because you just scare yourself. You just put yourself into a mindset that’s not good. I was so depressed about everything after what I read, but I had my daughter to keep telling me to stop reading and just focus on only what the doctor says and not reading all this stuff out there.

Everybody is different. There’s not a one size fits all when it comes to pulmonary hypertension. When you read all that stuff, that is a possibility, but it doesn’t mean it’s a possibility for you. That’s what I learned. It’s just there. This is what has happened to other people in the past. You may be lucky and it’s not as bad as what you’ve read or it could be worse than what you’ve read. In any case, just try to keep calm. Try to focus on your doctor and what your doctors are telling you to do and not everything else that’s happening.

I know it’s scary when you’re first diagnosed, but you just got to go through the process with the doctors and trust your doctors. Celebrate the wins. I went to the cardiologist and she said everything looked good, way better than it did a year ago. That’s a win. I celebrate that. When I saw the pulmonologist and he said, “Everything looks so much better than it did when you first got here.” I celebrate that as a win. It doesn’t mean that I’m cured or I’m out of the woods, but it just means that things are progressing good and you just have to take that in and keep going with your life. You may have a few restrictions here and there, things that you have to do that’s going to make it difficult, like with no salt or counting your liquids, things like that. But it’s a win-win. The doctors smile at you and say, “Oh, things look good.” Take that as a win.

My name is Angela Brown and I’m aware that I’m rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: info@phaware.global Like, Subscribe and Follow us: www.phawarepodcast.com. @phacanada