I'm Aware That I'm Rare: the phaware® podcast
I'm Aware That I'm Rare: the phaware® podcast is devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. Through this series of impactful, insightful and, most importantly, hopeful stories from members of the global pulmonary hypertension community, we hope to further the global #phaware conversation as well as to capture, engage and enable misdiagnosed and undiagnosed PH patients because early diagnosis and treatment can mean the difference between life and death. Topics range from the importance of early diagnosis and global PH awareness, to the impact of clinical trials to how patient support and advocacy are paramount to battling this disease. Learn more about PH at www.phaware.global
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Tanya Stinson - phaware® interview 499
12/17/2024
Tanya Stinson - phaware® interview 499
Tanya Stinson shares her journey of being diagnosed with pulmonary hypertension (PH) after persistent symptoms and misdiagnoses. Her struggle highlights the difficulty of finding the right diagnosis and the emotional toll of living with a rare, chronic disease. Tanya’s experience underlines the fear, mental challenges, and hope intertwined in navigating PH, inspiring others to seek support and find solace in community, showing that even in isolation, they are not alone. My name is Tanya Stinson. I’m from a really small town in Manitoba called Saint Eustache Manitoba. It’s about half an hour away from Winnipeg. I was diagnosed about a year and a half ago with pulmonary hypertension. My family doctor thought for sure that it was a heart condition. I kept going to her and telling her, “I can’t breathe, I can’t breathe, I can’t breathe.” She kept saying, “Well, we’ll get you in to see the heart doctor.” My husband even came with me to a couple of appointments and he’s said, “It’s not her heart. She can’t breathe. It’s her lungs.” The doctor was like, “No, no, no, no, it’s her heart.” Being a woman of larger size, it’s automatically assumed it’s my heart, and I said, “It’s not my heart. It’s the fact that I cannot breathe. I can’t go from my bed to the bathroom, which is like five steps away, without having to stop halfway to catch my breath.” She said, “Well, let’s just wait. We’ll get you to the heart doctor.” I saw the heart doctor. Then, that weekend I couldn’t breathe hardly at all. I phoned her office on Monday morning and I said, “Can you please, please, please get me in? I need to see you. I cannot breathe.” So I went in. She put a pulse oximeter thing on my finger. She took it off my finger and she cleaned it and then she put it on her finger and then she put it back on my finger. She said, “I think this thing might be broken because you’re registering 71.” I said, “Well, see, I told you I can’t breathe.” She said, “Okay, well you need to go to the emergency room.” In my province, which is Manitoba, we have urgent care, and then we have three hospitals in Winnipeg. She said, “Go to what we call an urgent care, which is Victoria Hospital.” But she thought she was sending me to a regular hospital and that wasn’t the case. I got to this urgent care and they did my oxygen. Then, I had to wait for a special ambulance to take me to St. Boniface Hospital in Winnipeg. That’s where I met my doctor. The way I look at it is he knew the second he saw me almost what it was. But he said, “We have to do tests, and it’s already like 10 o’clock at night. There’s no way we’re going to run tests today, so we’re going to admit you.” I had the right heart catheterization. He came in the room. The look on his face probably scared me the most, because it was a look of very much of concern and almost sadness in a way. When I saw his face, I’m said, “Am I going to die?” That was the first thing that ran through my head. When he told me it was pulmonary hypertension, I said, “Oh, okay, so I’ll take a couple of pills and I’ll be fine.” He started me on medication right away. Within the first week, I already felt better. I thought, okay, the longer I’m on these meds, I’ll be able to breathe again. Then, I went home and I had a week off of work before I had to go back to work. It’s almost like I plateaued. My breathing wasn’t getting better and I kept thinking, I thought these pills would help. I thought they would cure me. Even though he told me it’s not curable right now, I felt at least I would be able to breathe. Now knowing, a year and a half in, that this is the best it’s going to get is scary in a way, but at least I’m not where I was a year and a half ago before diagnosis when I couldn’t even take five steps to go to the bathroom. Now, I can work without having to worry about anyone looking outside the window going, why is she stopped in the middle of the road walking into work kind of thing. I don’t have to worry about that. I can at least get myself into work and stop and take a break and then continue on. I don’t have to worry about people looking at me and saying, “Well, why is she just standing there?” I don’t have to pull out my cell phone pretending I’m taking a phone call in a parking lot so that I don’t look like an idiot stopping in the middle of the parking lot to just catch my breath. My doctor, because he works at the pulmonary clinic, he told me, don’t Google anything because it’s the wrong thing to do, but someone tells you not to Google something, you’re going to Google it. So, that was a bit scary. But then I found the website and that helped a lot. But in our province, there has to be more of us. We got more than a million people, so if it’s one in a hundred thousand, you figure there have to be other people, but you still feel really lonely because you don’t know of anyone. I was able through PHA Canada to find a PHA buddy and she has been a godsend for me. She’s answered so many questions and has been there to support me through the really difficult, hard days, whether it’s in relation to work, my breathing, just anything. If it wasn’t for PHA Canada, I don’t know where I would be as well as without my doctor. I sometimes will say to my husband, “If it wasn’t for my doctor, Dr. Christensen, I’d probably be dead,” because it was him who figured it out. It wasn’t my family doctor. It was my family doctor who said, “It’s your heart, it’s your heart, it’s your heart.” He was the only one that looked at me like a normal person, not as an overweight person, not as a female, not as somebody who’s all in our head. He actually listened and he knew. To me, without those two, Dr. Christensen and PHA Canada, I would probably be dead. Even just the overwhelming grief that comes with knowing that you’ve been diagnosed with a disease that you’re going to die from, and knowing that in the back of your head and having to deal with everything that comes with that is scary. But without those people, I don’t think I’d be here. Things would’ve been too tough. This disease is very challenging on multiple aspects of it and in multiple areas, whether it’s mental health or just with your actual physical health. Knowing that even though you’re taking the meds, I’m not going to get better. I’m going to actually over time probably get worse. So even though we’re trying the meds I’m on now, I spoke to him a little while ago and he says, well, we’re going to try the new medication that’s out, but we have to wait until we get approved. I think for me it’s the actual breathing and knowing that it’s only going to get worse, but then also having to deal with that mentally, being scared of when it does get worse. What’s my life going to be like when it gets worse? Am I going to have to stop working? Am I going to lose more friends? Am I going to lose family members, because they think I’m just faking not being able to breathe? I think it’s everything about this disease, but having the supports there from PHA Canada and from Dr. Christensen, that helps so much, because you know are not alone. There are people out there that understand you and can appreciate what you’re going through and help you get through that. Having someone who knows what you’re going through is extremely helpful. When first diagnosed, I heard the statistics is one in a hundred thousand, so you know that there has to be other people out there, but you have no way of knowing who those other people are. You have no way of knowing if you’re all alone right now, and if anyone is anywhere near you that you can talk to about what I’m going through is normal. Now, every person’s journey is different, but I think some of the same things apply to everyone. Everyone struggles with the breathing aspect. Is it normal that on these new meds that I still can’t breathe perfectly? That’s a question I had. I had at that time no one to ask that question to, and I didn’t want to appear stupid or dumb in front of my doctor. No one wants to do that. So, it’s like a question of, well, do I look stupid by asking my doctor is this normal, or is there someone around me that I can ask and not look stupid? On the medication I thought, great, I’m going to be able to breathe. But then to know that you’re not, but then to have that person there tell you that’s normal. It’s normal that your fingertips might still be blue. It’s normal that you’re going to struggle some days harder than most. At the PHA conference in 2024, it was in Calgary and I live in a prairie province. It’s very flat, very cold in the winter. Traveling to Calgary which has a lot of mountains and is very high up, I was worried about breathing. To be able to talk to my PHA buddy and PHA Canada and my doctor, they were able to tell me, it’s going to be okay. We’ll give you a little bit of supplemental oxygen and you’re going to be okay. But those are questions that if you’re all alone and don’t have that support system, you’re not going to know. You’re not going to have those answers, and it’s going to further put you, I feel, in a depression that you can’t climb out of without the support. For me, I’m hoping to be able to help other people because, in our province, there’s not a lot out there for people who have been diagnosed. What gets me going every day is knowing that I’m working with my PHA buddy and we want to set up a support group in our province to be able to help other people through this is what gets me going every day. Knowing that there are people out there that have been 20 years, even 10 years, my PHA Buddy’s been nine years. I know I’m not going to die necessarily tomorrow, and that makes me feel really good knowing that that’s not going to happen. To me, I’m still young. I’m only 50. I was diagnosed when I was 48. Knowing that I’m not going to die tomorrow is wonderful, because you do get that scary feeling. Like if you Google it at seven to 10 years. Well, I don’t want to die before I’m like 60. No one wants to die that early. Some people won’t live that long. But knowing that there are people that have is very encouraging and gives you hope, and hope is all you have to fight this disease some days it seems. Yeah, hope is important. I really hope that recording these messages helps a lot of other people. I know that there’s got to be people with PH out there that don’t live anywhere near a center, but maybe can listen to your podcast and get some sense of I’m not alone. There are other people who are out there in this world that are suffering, but hearing these words can offer them some encouragement. My name is Tanya Stinson, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Episode 499 - Tanya Stinson
12/16/2024
Episode 499 - Tanya Stinson
Tanya Stinson shares her journey of being diagnosed with pulmonary hypertension (PH) after persistent symptoms and misdiagnoses. Her struggle highlights the difficulty of finding the right diagnosis and the emotional toll of living with a rare, chronic disease. Tanya’s experience underlines the fear, mental challenges, and hope intertwined in navigating PH, inspiring others to seek support and find solace in community, showing that even in isolation, they are not alone. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Hall Skaara (Bel Air Center) - phaware® interview 498
12/10/2024
Hall Skaara (Bel Air Center) - phaware® interview 498
Hall Skaara, a resilient PH patient and founder of the Norwegian Association for Pulmonary Hypertension, recounts his 19-year journey living with idiopathic pulmonary hypertension. He shares how he transformed the challenges of the pandemic into an innovative opportunity by helping create the , a virtual global PH community space. The Bel Air Center aims to foster global connections, support, and resources for patients, caregivers, and healthcare professionals. My name is Hall Skaara. I am a patient. I was diagnosed with idiopathic pulmonary hypertension in 2005. So, I've been living 19 years with this disease, still doing very well. I founded the , because there was no association in Norway when I was diagnosed. Later, we became part of the European Umbrella Association. I started working more for them. Somebody else took over the Norwegian Association. Today, I do project work with . One of the exciting projects that I'm working on is something called the . This is a virtual PH community that we have created. Let me tell you about the name, first. Bel Air Hotel was a hotel we used to meet once a year outside Barcelona. Then, we had the pandemic, and we could not meet anymore. We thought, we need to meet virtually. We contacted a company that designed a virtual hotel for us. We made it into a virtual conference center. Here, we had our annual meeting with our general assembly meeting. We had the presentations, etc. Once the pandemic was over, we thought this was way too good to just scrap. So, we kept it and we developed it further. We removed all links to PHA Europe from it, because we want this to be a global PH community center not linked to any specific association. When you log in, you walk into a lobby in a hotel. I'm actually there to greet you as the receptionist. You can send me messages and ask me questions. Then, you move from the lobby into different areas of the hotel. We have an exhibition area for all the associations. So, each association in PHA Europe, but also other associations around the world that would like have a booth. In these booths, they have information about themselves. They can have videos. They can have documents that are uploaded. They can have links to their webpages, etc. Also, we have an exhibition area for pharma companies that work in the field of PH. You can go into their booth and see information about their products and about their company. We have live session room, where you can watch live sessions related to PH. Once the session is over, we put the recording into a recorded presentation room. Here, you can search for all these presentations by keywords. You can search by presenter name, etc. It's very easy to find a presentation that is fitting for you. We try to subtitle presentations there, but also we utilize dubbing. We use artificial intelligence technology to dub, so that the person who is presenting in English can speak Spanish or Chinese or any language. At the moment, we have Spanish, German and Croatian dubbing in there, but we can do much more. This is a little up to what the community wants and requests. We have an art room where PH patients and PH doctors have a provided us with art. You walk through a gallery and you can see artwork on the wall, and also videos. For instance, we have a PH doctor that plays piano. You can see this as an artwork on the wall. Then we have a social area where you can meet other people who are logged on to this virtual center. You can chat with them or you can have video calls with them. Also, you can be invited to meetings with other people there. For instance, after you've gone to a presentation, you can go to a social area, meet with other people that have been at the presentation, and discuss the presentation in there. We have an area for podcasts, and this is where phaware have provided us with all their podcasts. So this is a great resource for PH patients, carers and doctors. We have made this open to anyone. Anyone can create an account. You do this by going to . We've made this open to associations, too. They can create accounts in there as well as stands where they talk about their association. We have stands from all over the world. We have Japan, China, Canada, USA, most European countries, Nigeria from Africa and so on. We had our grand opening at the beginning of the year in 2024. We have had a steady increase in the number of people that joined this community. We hope to continue growing the numbers. Not only are patients welcome, but also carers, healthcare professionals, etc. We would welcome even more participants from the medical community and associations to join and make stands. Also, pharma companies. We hope to make this the leading virtual PH platform for pulmonary hypertension. My name is Hall Skaara, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Episode 498 - Hall Skaara (Bel Air Center)
12/09/2024
Episode 498 - Hall Skaara (Bel Air Center)
Hall Skaara, a resilient PH patient and founder of the Norwegian Association for Pulmonary Hypertension, recounts his 19-year journey living with idiopathic pulmonary hypertension. He shares how he transformed the challenges of the pandemic into an innovative opportunity by helping create the , a virtual global PH community space. The Bel Air Center aims to foster global connections, support, and resources for patients, caregivers, and healthcare professionals. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Cynthia Neilson - phaware® interview 497
12/03/2024
Cynthia Neilson - phaware® interview 497
Cynthia Neilson shares her journey as a mother raising her daughter Claire, who was diagnosed with pulmonary arterial hypertension (PAH) at a young age. Cynthia recounts the challenges she faced navigating the medical system, the emotional toll of dealing with an unexpected diagnosis, and the difficulties of balancing family life while ensuring her daughter received the best possible care. Cynthia emphasizes the value of connection, resilience, and advocacy for families dealing with rare diseases. My name is Cynthia Neilson. I live in Toronto Ontario, and I've been there 20 years now. I've raised two kids there. I have an older daughter, Claire, who's 14 and I have a son, Owen, who's 10. My daughter was diagnosed when she was two. I really didn't have any connection or any knowledge of the disease, who to connect with, contacts, etc. I got involved with the Patient and Family Advisory Committee at Sick Kids Hospital in Toronto, which was great. I got to meet other families, but unfortunately I'm the only PH parent on the Advisory Council, because as you know, PH is a rare disease. I really enjoyed that sort of connection with other parents. Then, about a year ago I learned about the Pulmonary Hypertension Association of Canada. I was really interested in learning more and there was an opportunity to join the board. So I did that, which was great. That's been a really fantastic opportunity to meet other families who have varying types of PH. I always in my gut knew something about her was not right, but I was a first-time mom, doctors know best, et cetera. So, I sort of ignored that inner voice. Then, I took her to emerge for an earache. I had a med student examine her and they said, “Oh, her heart sounds sort of strange.” So, we went to and had an echo done. Sure enough, the tech said, “Oh, well, obviously she has a really large hole in her heart.” This was the first time that I had ever heard anything like that. I said, “No, I actually have no idea.” You could see this tech's face sort of go pure white and leave the room sort of suddenly. From there, it was a very quick referral into Sick Kids, where she was formally diagnosed, I don't know, probably like two weeks later. Everything moved really quickly. Then, we joined that clinic. They wanted to close the hole in her heart first, but the pressures in her heart and lungs were too high. We decided to wait a year and put her on oral medication and oxygen to hope that we could get some of those pressures to come down to do the surgery. A year later, she had an open-heart surgery. They repaired the ventricular septal defect. It was a pretty rough road. We were in the hospital for a long time and there was some complications, but unfortunately the PAH didn't resolve itself and we were looking down the path of what does the life look like with PAH? We got really involved in the hospital. As I said, I joined the patient and family advisory group. We used child life support. We just sort of figured it out our way. It wasn't the easiest path. Fortunately for me, I have a very healthy family. I've never had really anyone in my family that has chronic disease. I'm not sick myself. All of this was really new territory. I had just gotten pregnant with my second. I was going through this time of surgery being pregnant, which was also sort of an emotional rollercoaster, which didn't make things much easier. About six months before I was pregnant with my son, I had a miscarriage. At that point, I started questioning like, “Oh, is this because I have a daughter who has PAH? What's going on?” When I got pregnant with my son, we did do some fetal echo ultrasounds and some genetic testing just to see there was no sort of predisposed conditions, underlying conditions, et cetera. There wasn’t. I think the miscarriage was just an unfortunate, unrelated event and my son is healthy and does not have any sort of health conditions. We were fortunate that that was the case. But we did do a bit of investigation, because I was anxious. I wasn't confident that I could take care of a child with complex medical needs and then having another child potentially sick. I was still a fairly new parent. Obviously, my husband's hugely supportive and we were in it together, but it's still not easy. Neither of us grew up in Toronto, so we had no family support close by. My family's about four and a half hours away and my husband's is about three hours. It was a trying time when I look back, dark days. So she went to preschool. Both my husband and I work full time and that was sort of post-surgery. That was fairly smooth. By that point, she was getting ready to go to school. I would say the transition to school was a little bit rocky in the beginning. Claire, at that age, was still napping. She was much more tired than other kids. So, trying to negotiate with kindergarten teachers that she still needs a nap, they wanted her to go into a preschool room. Some of that when she was in the earlier years was a little bit trickier, but fortunately for us, she's more tired and can't sort of play as hard as the other kids. Physically, she's actually quite capable of a lot. In those early years through school, we actually had few concerns or issues with her being able to do what other kids were doing. She had to take medication at lunchtime, but I think there is a lot of kids who take a lot of different medication. That's pretty normalized in the school system now. As she got to grades 5, 6, 7, it became a period of time where she started identifying that she had some limitations. She couldn't run as fast, wasn't playing soccer, et cetera. It became a little bit harder. She started asking questions. As much as she knew as she had a hole in her heart, the doctor sewed it up and now she's better. She just takes medication to keep her better. I think that was sort of the age-appropriate conversations we had had. But by this time, she's starting to ask questions like, I'm still taking oxygen at night and like, oh, now I have another new medication and I'm tired and what is going on? I think that made things a little bit harder to just have some of those conversations. I would say that for the most part the teachers have been pretty responsive. I do an annual meet with the teachers, the gym teacher to say, when you say keep running and she stops, you can't be yelling in her face to keep running. Some of those conversations. For the most part, it's been pretty straightforward and easy. If I fast-forward to today, she started high school. I think the game is changing a little bit. I think with high school kids are just exposed to more, whether it's vaping, smoking, edibles, alcohol, et cetera. We're having to have some of those conversations around what is an issue. I mean, all of those things are an issue for everyone, don't get me wrong, but I think with someone with PH, inhaling smoke, it's a bit of a different game. I think having some of those conversations has been a learning curve for sure. One of the things we also tried to navigate was trying to keep her in activities and doing things like her friends and not being different but still being able to manage it. A good example is summer camp. She wants to go to summer camp like every kid. Day camps were never an issue. Once she got older, she wanted to do overnight camp. Overnight camps that allow oxygen are a few and far between. I must have called, I don't know, 10 camps and only one would allow us to bring oxygen. It was great, but she was the first time in the history of this camp that's been in existence for a hundred years that's had someone use oxygen or bring oxygen. So again, it's making sure that she has the right tools to be able to talk to her cabin mates about why she needs the bottom bunk, why she needs an extra plug. I think it's just trying to get her to a place where we can kind of shift some of that sort of conversational responsibility away from us and to her and that's a hard space to be I think right now. This particular camp that we ended up going to does have a full-time doctor and nurses on staff that are there 24 hours. They obviously have electricity in the cabins and were frankly quite accommodating. It was good. It's been a really good experience. She's gone for the last three years, but it's a really expensive camp. We're fortunate we can afford to send her there, but lots of parents can't do that. I think about what are the opportunities for those kids and being able to have some of those experiences? Fortunately for us, we've always had a very sort of open relationship with Claire. So no question's a bad question. She's just coming to us and still having those conversations. I'm really happy about that, because I think otherwise I don't even know if I would really know at what point I'm having what conversations. She was very open about is it safe for me to vape? Can I drink alcohol? We were able to give her lots of great... well, what I think is great advice, at least health advice. But I think some of those conversations, I don't know, I think are just going to evolve and I'm just going to keep my fingers crossed that she just keeps asking the questions. I think we're lucky Claire has had the same nurse practitioner her entire life, so it's definitely a safe space where the two of them can have a conversation and she can ask all the questions she needs to. I think we also rely on mental health support in the community. Having a person that she can connect to that's not one of us, and ask these questions, allowing her to have that space, as well, hopefully will mitigate some of these issues as they come up. There's no rule book to raising kids, whether it's a kid with PAH or a perfectly healthy kid. I think parents just have to kind of go with the flow and deal with what comes. PAH is a rare disease and the social networks are not like some of the other chronic diseases. I finally came to a place that I was ready to connect with other people who are living the same experience as me. Visibly, Claire looks like she's not sick. PAH can be a very invisible disease or an extremely visible disease depending on the severity. I was very worried that coming to some of these events or conferences or getting involved, it would be a look into my future and thinking this is what Claire's future holds. Very much keeping her protected from the community as well because I was she'd have the exact same experience. I remember when Claire was about five years old, I went to a provincial conference that was held. It was all heart conditions. I went and I saw all these visibly sick children and I had to leave right away. I said to my husband, “I will never set foot in another one of these places again. I just can't see this.” I think with time, actually getting involved in the community has just raised my level of awareness and understanding that I think I'm just ready. It just took me a while. I think I also came to the realization that Claire is now the age that she needs to make these decisions. Maybe for her actually connecting with kids who have something similar to her is quite therapeutic. I have up to this point made that decision for her, but it's time. She's older. I think that was part of it. I've been so fortunate. Our medical team and the community has been so great that it's just another way to give back. If my story or my advice or even just being there for someone else can provide support, then I'd love to be able to do that for someone else or another family. My name is Cynthia Neilson and I'm aware that my daughter is rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Episode 497 - Cynthia Neilson
12/02/2024
Episode 497 - Cynthia Neilson
Cynthia Neilson shares her journey as a mother raising her daughter Claire, who was diagnosed with pulmonary arterial hypertension (PAH) at a young age. Cynthia recounts the challenges she faced navigating the medical system, the emotional toll of dealing with an unexpected diagnosis, and the difficulties of balancing family life while ensuring her daughter received the best possible care. Cynthia emphasizes the value of connection, resilience, and advocacy for families dealing with rare diseases. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Natalie Roy - phaware® interview 496
11/26/2024
Natalie Roy - phaware® interview 496
Natalie Roy is a double lung transplant survivor living in Canada. She was diagnosed with pulmonary arterial hypertension (PAH) in 2005 and received her transplant in 2017. . After her successful transplant seven years ago, Natalie experienced a difficult initial recovery but felt amazing once she started healing. The first two years were cautious, but she was able to achieve many milestones she never thought possible, including getting married, playing sports, and changing careers. My name’s Natalie Roy and I’m living in Victoria, BC, Canada. I am a double lung transplant survivor. I was diagnosed with pulmonary arterial hypertension in 2005, and I received my double lung transplant in 2017. I did this podcast shortly after my transplant about six years ago, and I just celebrated my 40th birthday and have celebrated my seven-year lungaversary. I started fainting out of nowhere when I was about 19 and was diagnosed in the emergency room in Vancouver. I was diagnosed with idiopathic pulmonary hypertension at the ripe age of 20 and started oral treatment and did quite well with that right up until about 2010. My disease started to progress and I went on subcu Remodulin. Shortly after that, I transitioned to IV Remodulin and had that journey for about five years and slowly declined and was listed for transplant in 2015. I did wait for about 19 months for my transplant and got to experience a dry run. For people who don’t know, that is when they call you and tell you that they have lungs for you and prep you for surgery and you discover that your lungs aren’t viable and they send you home. That was quite the experience. I did quite well on my oral medication for many years. I was a chef for about 13 years. Just before I got listed for transplant, I worked pretty much my whole diagnosis. I did quite well on treatment. I participated in a lot of support groups. I found a lot of community amongst other patients in the PH world. I don’t think I would’ve survived without many of the other patients throughout Canada and the world. I think that community is really important when you are suffering. It wasn’t until I started Remodulin that I actually really, I think that was the grieving process actually really hit me square in the jaw. I was like, “Wow, I’m really sick. I am connected now to a device 24 hours a day and I need support.” That’s when I reached out and found the local support group in Calgary at the time. I was surprised with how almost desperate I was for that connection when I went to my first meeting. I didn’t realize how isolated I actually was and I was just kind of going through the motions and kind of really just living my day-to-day in denial of my disease and minimizing it, pushing through, and not really allowing myself to be somebody who was unwell. I think the support groups give you a space where you’re able to connect with other people who actually, obviously, understand what you’re going through. There’s only so much your caretakers and loved ones can relate. They sit beside you, but they don’t experience it with you. The online is amazing, but if you can find other people in person, I think there is something really beautiful to going and grabbing a coffee with somebody or talking on the phone or meeting up with somebody. I did run the one in Vancouver with Tarya, which was really amazing, and that was one of the most enriching experiences that I think that to date I was involved in. I think you need to be prepared to experience somebody else’s grief, that is part of showing up and holding space for somebody else and meeting somebody where they’re at, because somebody who potentially isn’t as progressed or as sick as you are, it could impact them. They might not be able to cope with it in a way that you’re able to cope with it. That was one of the things that I did notice, was that everybody has different coping levels. What you go through in your life, how you cope, how you move through things, some people haven’t gone through a lot of hardships in their lives and they get diagnosed with this disease out of nowhere and it completely rocks their world and they do not know how to manage. They find these support groups and they go there and their emotions are very big. I think that’s part of the rooms and part of the experience. If you are an empath or if you’re somebody who is sensitive, I think you do leave those rooms feeling quite exhausted. When you only have so many spoons, you definitely walk away feeling quite tired. When I got the call for the dry run, I was absolutely terrified and not ready. Sitting, waiting outside of the OR and I realized that it was a dry run, I felt extreme relief. When I was able to go home to my dog and eat a sandwich and go to bed, I was like, “whew.” I was very devastated the next day when I had to wake up and I had to mix my meds and I had to live life unwell and hooked up to a machine and my line got infected and I just had to continue on living life with PH and being sick. But by the time that I actually got the call from my lungs, I was ready. I felt safe and I felt cared for, and I think that was part of it. For me, I had always told myself like, “No matter what,” and I think I said this in my last interview, “do it scared. If it’s your choice, doesn’t matter. If you’re scared, do it anyways.” Just get in there and do it, because it is honestly the best gift I’ve ever received in my life. My initial recovery was quite difficult. I was in the hospital for about two months, but once I started recovering, that was it. I just wanted to live and my body wanted to live, and I felt amazing almost immediately. I was up and moving and working and back in the world. I think the first two years, if I’m being realistic, were cautious. I followed all the rules. I had a few bumps. There was a few complications. My donor lungs had a few issues. I had to do a few treatments along the way, but for the most part, I think for anybody who has been quite sick and has progressively gotten more and more ill, you can’t compare. Once you have the opportunity to take full breaths and you’re not in chronic fatigue and you’re not actively dying, no matter what you have, it’s no problem. You’re up, you’re moving, and you can push through it. When I’m asked like, “Oh, how was it?” I can’t even remember what complications or what issues I had. I actually can’t remember, because it really feels like it wasn’t a big deal. It just really was such a blessing and such an honor. I got married. I’m learning how to drive. I moved to cities. I’m doing things in my life that I didn’t ever think I would accomplish. I hit a milestone, which was turning 40. That alone feels like such a privilege. I think aging for people who are chronically ill is such a privilege. There’s so many things that have happened in the last seven years. I mean, every year, I think because my lungaversary and my birthday are a week apart, not even they’re a couple days apart, that it’s such a tender space for me and it’s such a dual celebration. It’s definitely a big honor to be aging. Every year, another year with my lungs, another year on this planet, another year celebrating and living and embracing life for whatever it is. Even the mundane stuff just feels like the best stuff. For instance, when we got married, it was like I was having before my transplant, we were having this huge wedding, everybody was invited. I had this whole thing planned out. Then, I had my transplant and I was like, “No.” We eloped. It was my husband, it was me, it was my best friend, and that was it. We were in the forest. I didn’t want anything else. It was interesting how before everything had to be big, involved, passionate, intentional. I absolutely live my life with intention now, that is the name of the game, but I feel like there was a desperation attached to everything I did before. Now, there is an ease, if that makes sense. What I get to experience now, even if it’s a fraction of what I tried to cram in before is enough based on the quality of my life. It doesn’t matter how much I get to do now, it’s how good it feels. Right now, I changed careers, so that’s been very all-encompassing. I went from working as a chef and now I am working in a corporate office. That’s been overwhelming at times. I feel like an imposter coming from somebody who couldn’t walk from the bed to the bathroom. Now, I’m running a little financial center and that feels strange to me, and sometimes I’m like, “How did I get here?” So I feel like that at the moment is like I am upgrading my education, which wasn’t an option for me. Yeah, right now I’m 40, but I feel like I got sick so young and then I was sick for 19 years. I got diagnosed 19 years ago, so a big chunk of my life was being chronically ill. I wasn’t able to make plans for myself, because my body was like, “No, we don’t get to do that.” Anytime I tried to make plans for myself, I would get unwell or something would shift and it just wasn’t feasible. So right now, I am working. Right now I’m trying to upgrade my education. We are buying our first vehicle and we joined a softball league. Who does that? I joined a softball league and played a season, and we got third. We went to the playoffs. I did a sport. I’ve never played a sport in my life. I’ve been sick forever. Those little things. So yeah, I don’t really have any plans. I really am just going with the flow, but the flow feels pretty good. I stayed connected to the PH community throughout. I didn’t lose touch at all. I do feel like I didn’t stay as closely connected as I wanted to. Going to the this year reconnected me closer, which felt really good. I have linked back in a little bit closer. I’ve joined their support group, so I’ll be going to that. I have my friends in the community and keep in close contact with them, but I really would like to do a little more volunteer work. I’d like to participate a little bit more in what they got going on with , but I feel like that’s where I want to put my energy moving forward, because that’s where I have the energy to do it. I had a hard time where I had identity crisis because I didn’t know where I fit into the community anymore after my transplant. What I did feel going to the conference this year and the feedback I did get was, “It’s really nice to see you here,” because a lot of transplant patients kind of move on with their life and they don’t really stick around, which is a beautiful thing, you go live your life. But those people saved my life and kept me alive and filled my cup and continue to do so. They are a family to me. I want to do a little bit more where I can. My name is Natalie Roy, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Episode 496 - Natalie Roy
11/25/2024
Episode 496 - Natalie Roy
Natalie Roy is a double lung transplant survivor living in Canada. She was diagnosed with pulmonary arterial hypertension (PAH) in 2005 and received her transplant in 2017. . After her successful transplant seven years ago, Natalie experienced a difficult initial recovery but felt amazing once she started healing. The first two years were cautious, but she was able to achieve many milestones she never thought possible, including getting married, playing sports, and changing careers. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Maureen Harper - phaware® interview 495
11/19/2024
Maureen Harper - phaware® interview 495
Maureen Harper, a CTEPH patient from Canada, shares her journey with this rare condition. She initially thought she had an infection in her leg, but further tests revealed enlarged pulmonary arteries and multiple blood clots in her lungs. After being diagnosed with pulmonary hypertension, she underwent a complex surgery in Toronto to attempt to remove the clots. Maureen continues to work full-time as a pharmacy technician, remains active with her family and Girl Guide unit, and maintains a positive outlook, choosing to focus on the positives rather than dwell on the negatives. I am Maureen Harper, and I’m from Olds, Alberta and I’m a CTEPH patient. I had a lump on the front of my leg, on my shin. It looked like an infection, so the doctors put me on antibiotics. So I did six weeks of oral antibiotics, six days of IV antibiotics, and it kept growing. They decided it was not an infection, so they sent me to a dermatologist. The dermatologist was like, “Well, that’s weird.” And by this point, of course the lump is gone because that was six months later. So, he’s like, “I think it might be something else.” He sent me for an X-ray. The X-ray showed I had an enlarged pulmonary trunk, so he’s like, “Oh, you’re not mine.” He sent me to a cardiologist. The cardiologist put me on the treadmill to do a stress test, and my heart did great and my oxygen just kept dropping and dropping and dropping. So, they pulled me off the treadmill and I’m like, “Just give me a minute. I recover really quick. I can pop back on and we can finish this. She’s like, “No, no, you can’t.” The cardiologist was like, “Wow, that’s weird. Let’s do a V/Q scan and just rule out pulmonary embolism, so make sure you don’t have blood clots in your lungs.” We went into the V/Q scan. My husband and I were sitting there together and we’re waiting for results. They’re like, “Yeah, the radiologist wanted to talk to you. We’re going to move you to this waiting room.” They moved us to a waiting room by ourselves. Then, some other people got moved into our waiting room. They moved us to another waiting room, and some more people got added to that waiting room. So, they moved us to a third waiting room. I looked at my husband and went, “Something’s wrong.” He was like, “What do you mean?” I’m said, “They just keep putting us in waiting rooms by ourselves.” He was like, “Oh.” The radiologist came out and said, “So you’ve got embolisms in your lungs and a lot. How are you feeling?” I’m like, “Fine.” I got to go see my cardiologist. He said, “Yeah, you’re not my patient. We need to treat you now.” He put me on a blood thinner and said, “You need to see a pulmonologist.” That’s how I ended up in the PH clinic. I had one meeting with them and they immediately wanted to do a right heart cath to see what was going on. I was diagnosed with pulmonary hypertension. Then, a little while later, we did some more testing and that’s when we figured out it was CTEPH. My doctor in Calgary actually didn’t think I would qualify for the surgery, just where all my clots and stuff were, but they sent my stuff off to Toronto anyway. It came back saying, yeah, they think they could help. I was originally booked into surgery at the end of August. I’m said, “Oh my God, I can’t do the end of August.” My daughters had just graduated. They were starting college, and I’m like, “They can’t start college without mom.” I still need to be there in case they need some support or the paperwork didn’t go through or whatever. So, we bumped it until October. It all kind of goes really quickly. You get flown out to Toronto from Alberta, and we flew out Friday, spent Friday night in a hotel. Saturday, I got admitted into the hospital to start heparin therapy and blood thinning therapy to help make sure I’m safe for surgery. I was there Saturday and Sunday. Monday morning around 6:30, they came in and said, “It’s time to go.” They roll you down. The surgery eight to nine hours. Then, you’re sedated for 24 hours just to give your body a chance to recover. My husband did not enjoy that 24-hour period. I did not wake up from sedation really well. They took three different tries to get me to wake up. Then, you start the recovery process. We were in Toronto for nine days, flew home. I did really well for the first week or so, and then my daughter came home from work one day and looked at me and said, “Mom, you don’t look very good.” I said, “I don’t feel very good.” I’ve got a headache, and I’m just off. She said, “All right, let’s go.” She packed me off to the hospital and they took one look at me, went, “Well, get comfy, you’re staying.” We were in a small hospital. I spent a week there and came home for a couple of days and got sick again. That’s when I ended up in the Peter Lougheed for a week. It was a bit of a bumpy ride, but at least I was home for the bumpy part of it. The part in Toronto went pretty smooth. After we did the couple of weeks in and out of hospitals, I actually did really well. I was still working. I worked through the entire process. I would work an eight hour a day and then I could walk four kilometers at night. I was feeling great and doing really well for about 18 months, and then I couldn’t walk anymore. They did another right heart cath and discovered that my pressures actually had gone up and instead of CTEPH being a cure for me, they were able to get out the big clots, but there’s a bunch of little clots that are very distal, so very deep in my lungs, and they couldn’t get to those. So, those ones get to stay, and I still get to have CTEPH. Medically, they called the surgery unsuccessful. My husband and I kind of batted that around a little bit, and he said, “It wasn’t unsuccessful. They took out massive clots out of your lungs.” Even though my lung didn’t improve and doesn’t work like it’s supposed to, at least those clots are out of there. So, I might still have little ones all through, but I still have less clot that’s in there. My angiogram before surgery and post-surgery, according to my doctor, looked pretty much the same, so it really didn’t make any difference to my lungs, but at least I’m not fighting the extra clots that were in there. Medically going forward, I am on oxygen therapy. I’m on Adempas, which is made specifically for CTEPH, and I’m on ambrisentan, which also works for PH. We’re just trying to see if we can figure out a way to figure out my symptoms versus what my blood work says. My blood work says I’m doing real good, but going for a walk says I’m not doing real good. So not much changed in my life. I work a 40-hour work week. I’m a pharmacy technician, so I’m on my feet all day in a very busy pharmacy. I have three kids. They’re all technically grown-ups now. Then, I have a Girl Guide unit and we do crafts and activities. We go to camps and do everything all the other Girl Guide units can do, and we just kind of keep carrying on like we normally would. If I’m taking my Girl Guides camp or anything, we go on hikes. Previously, I was able to carry my tank in a backpack, because I didn’t need as much oxygen as I do now. Now, I have my tanks in a rolly cart and it just rolls behind me. We start at the beginning of the year with, “This is my friend, I call him Big Tom.” He comes everywhere with us and he helps me breathe, and they don’t bat an eye at it. Hopefully, from that what they learn is people with disabilities can do stuff too. There’s nothing holding us back. We can do whatever we want. I guess it was never an option for me that I wouldn’t continue working. I’ve always worked my whole life. The company I was with, I’d been with for many, many years when I got diagnosed. I’d been there about 11 years when I got diagnosed. My pharmacy team and my owner were fantastic and wanted to know what they could do to help. For a lot of the time, it didn’t make any difference. I pop a couple pills a day and they don’t know about it or they don’t do anything about it. As things have progressed, it has changed. I took time off to go for surgery and stuff like that. Then, when I got put on oxygen, we put a concentrator in the pharmacy and ran tubing. Then, when I could go to a tank, which was easier, I put it in a backpack and I carried it in a backpack and COVID had hit. So when I got put on oxygen, I was wearing a mask. I wear my tubing down my front because I trip on it because I’m a bit of a klutz. Lots of customers didn’t even know I was on oxygen until we stopped wearing masks a couple of years ago. Then, they were all really surprised to see that that’s what I had been doing, but to me, it was never an option to quit, because I didn’t feel sick enough that I needed to quit. As a support team, I’ve got my husband and I’ve got my three kids and they’ve always got my back. They’ll do whatever I need. If I can’t carry something, then they carry it for me. When I was using tanks at work, my son would bring my extra tanks in, so there was always tanks there for me to switch out. I’ve been a part of since probably 2018 or so, but I felt like a lot of those people were sicker than I was. I didn’t need their support or it felt like I didn’t need their support. I was always kind of in the background on the Facebook group, just kind of chilling and reading. Most of the people weren’t working and most of the people were having a lot of issues and I wasn’t, so I didn’t feel like I needed the extra support that they had. I’ve always been a busy person, and I find if you’re busy, then you don’t have time to kind of dwell on the what ifs. PH is not my only chronic condition. I had brain surgery 10 years ago. If you Google the stuff, if you look it up online, it’s terrifying. In my brain surgery group, everybody’s terrified of dying on the table and I’m like, “Oh, that didn’t even really dawn on me, because I trust my surgeons.” So through the brain surgery, through the lung surgery, neither time did it dawn on me or did I dwell on the fact that this is a life-threatening surgery, I could die on it. During the CTEPH surgery, the PTE surgery, they actually stop your heart and your lungs quite a few times as they clean out the clots, so you’re technically dead on the table, because there’s nothing flowing through your system. But I didn’t look at it that way. Somebody else told me, “You’re dead on the table.” And I’m like, “What? No, no, they’re just busy working. They just need a clean field.” It’s all mentally how you look at it. As a pharmacy technician, in the medical field a bit, I have some more knowledge than maybe the average person, so it didn’t scare me as much as some other people who are maybe not in the field. I’ve dealt with a lot of negative people in my life, so I find that you can get up every morning and choose to be happy or you can choose to be miserable. I just choose to be happy. I’m always kind of trying to look for the happy outcomes or the happy side of things in order to keep things positive. If you choose to be miserable, you can have a really miserable life, but I’ve decided to be happy and try to find the positive in everything. I’m Maureen Harper and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Episode 495 - Maureen Harper
11/18/2024
Episode 495 - Maureen Harper
Maureen Harper, a CTEPH patient from Canada, shares her journey with this rare condition. She initially thought she had an infection in her leg, but further tests revealed enlarged pulmonary arteries and multiple blood clots in her lungs. After being diagnosed with pulmonary hypertension, she underwent a complex surgery in Toronto to attempt to remove the clots. Maureen continues to work full-time as a pharmacy technician, remains active with her family and Girl Guide unit, and maintains a positive outlook, choosing to focus on the positives rather than dwell on the negatives. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Lynette Chambers - phaware® interview 494
11/12/2024
Lynette Chambers - phaware® interview 494
Lynette Chambers, a 9-year PAH (pulmonary arterial hypertension) patient, has also been diagnosed with pulmonary fibrosis (PF). She shares her journey, from struggling with daily activities to being admitted to the hospital with severely low oxygen levels. Lynette discusses the emotional challenges of facing a terminal illness and the difficulty of balancing work and family time. Despite the hard reality, Lynette emphasizes the importance of gratitude, making others feel better, and being the best version of oneself. She finds strength in her family, especially her grandchildren, and strives to create lasting memories and make a positive impact on those around her. My name is Lynette Chambers. I’ve been diagnosed for nine years now. I was in the hospital with congestive heart failure and they did a whole bunch of tests and decided that I had PAH. So, I’ve been going through a journey with congestive heart failure and PAH. Now, nine years later, I’ve been diagnosed with pulmonary fibrosis. I was at work, I was running a swimming pool company. I was running around getting stock and dealing with clients and stuff, and I was finding that I was really short of breath. After I was done talking to a person, it was like, I can’t get a full breath. For me, it was really scary because I thought there’s something wrong that I can’t breathe. Then, I went home, I could breathe fine. I drove home. Then, I got out of the car and went to walk up to the house. I couldn’t walk all the way up our sidewalk, which is like maybe 10 feet from the car to the house. I would have to stop, take a breath, go up our four stairs, stop, take a breath, and then go in the house and sit on the couch and try and recuperate. As the days went on, it just got harder and harder and became a lot more evident that I can’t lift stock, I can’t run from one end of the store to the other. I would recuperate on the drive home. Once I got there, I couldn’t make it into the house. It wasn’t a fluid motion. I went, okay, there’s something really wrong. I’ve always been active. I’d always taken my kids camping, done all kinds of things, water-skiing, downhill skiing. I couldn’t do any of that. I was just so fatigued the whole time. So, I went, no, there’s something going on here. I had a shower and I couldn’t get out of the shower. I had to sit on the edge of the tub. Then I got to where I felt a little bit better and I decided, no, I better go in. I went into the hospital. My oxygen level was at 48% and my heart rate was 174 over 94. So they went, “Okay, you’re admitted and that’s where you’re staying.” I was admitted into St. Boniface Hospital for two and a half weeks, while they tried to figure out what was going on. That’s when they did all the tests. At first they said, “No, it’s just congestive heart failure.” Then, they went, “But your oxygen went to 48. So that tells us there’s something in the lungs.” So they started doing more tests, like echocardiogram, all of that. Then, they went, “Okay, this definitely PH,” and that’s where it stayed. I’ve had my cardiologist put me in touch with another doctor who deals with my PAH, and then I have a cardiologist that deals with my heart. Then, I have a respirologist who now diagnosed me with PF after doing a CAT scan. I was in the hospital in and out from December to March five times with what they thought was pneumonia. My respirologist went, “Nobody gets pneumonia five times in a row.” I would go from one med to another. She said, “No, I want a CAT scan of the heart and lungs today.” I got a CAT scan and she phoned me at work and said, “Are you sitting down?” I said, “No, but I’ll take you and I to a boardroom.” So we went to the boardroom and she said, “You have PF.” I said, “Okay, what’s PF?” She said, “It’s pulmonary fibrosis, which is a scarring of the lung tissue, making it harder to breathe and suffocating the lung on its way up.” I said, “Okay, what are we doing?” She said, “Well, I’m not sure yet, because it is terminal. So whatever we do, I want you to understand you’re not going to get better.” I said, “Okay, well, we knew that with PAH.” She said, “No, this one’s different and you’re just going to find it harder and harder to breathe.” So we’re now in the testing process of finding out what meds will work for both or where we’re going to go from here, what tomorrow will look like. When I first was diagnosed with PAH, I thought, “Okay, this is it. I’m done.” But then, I started reading and reading and reading. I heard more stories about people lasting 20, 30 years. That was great. But then when I was told I have PF, and I started looking into that and I asked, “What does that look like?” Because when I was first diagnosed with PAH, my doctor said, “You’re going to be dead in two to five years.” It was left at that. There was no empathy, there was nothing. It was more of a, just so you know. Then, when I got the PF and I started looking, I didn’t find anybody that had had the disease more than seven years. So, then I started in my head going, okay, I have two grandchildren. One turned three and one is six. I’m not going to see them graduate. I’m not going to see them grow up. It’s a reality check and it’s hard, because you want to think, well, I can keep working because I have that energy and I love my job. So you want to keep doing that. But then in the back of your head is how much quality time am I missing with my family by being at work? So it’s a, could I afford to just not work? So there’s this whole piece emotionally where you’re going, what am I missing by doing this? Or what will I miss if I don’t do this? Amazon and I are best friends and I order everything I can, so my grandchildren have everything. Part of that is because I want them to remember me. I want them to know that Ama loves them, and I may not be there in person, but I will always be there. So, yeah, there’s that emotional thing that you do go through. I think when you’re being given a diagnosis of a terminal illness, there’s always that human component of this is your reality. This is where you are at. I have a husband that I love more than anything. I’m not going to get to grow old with him. We think when we get married that that’s why we marry is we’re going to grow old with our best friend and I won’t. It’s a challenge to get up every morning and know today may be your last day. You try not to think that way, and you try and be positive, because I believe positivity creates everything, but it’s really tough to put yourself back in there. I do believe that we’re all born and made for a reason and you do what you can do. I’ve been very blessed through my life. I’ve been a foster parent of 30 kids since I was 19. I have my own biological children and I’ve adopted children, so I’ve been very, very blessed in the life that I have. But it’s still hard to think that it’s going to end and I can’t do more and be more. For me, I need to be more to everyone and do whatever I can. If I can make somebody smile, I will. If I can help out in any way, shape or form, that’s who I am. This disease makes it harder to know that you’re not going to be there to make those changes. You can’t make a difference the way you’re used to. I have learned, and I’m very blessed to have learned this. One of the ladies I worked with said to me, we were talking about gratitude, and she said, “Take 15 minutes a day and write down five things that you’re grateful for. Read that at night before you go to bed. The next day you get up, have your day, take your 15 minutes and write down five things you’re grateful for.” Do you know I’ve been doing that for almost eight months? Not once have I written the same thing twice. You look back at that and you go, wow, look at all of this that I’m grateful for. That makes you go through the next step and go to the next day and see what five things you come up with at the end of that day. My family is my strength. I look at my grandchildren. If you watch the innocence in a child, it’s so amazing. I watch my grandchildren and I listen to them. It makes you want to be better. They make you want to do more, because it’s just so incredible how they look at things. People are arguing and fighting outside, and my granddaughter’s picking flowers. I said, “What are you picking flowers for?” She said, “Maybe they’ll be happy.” She wanted to take these two people flowers. She’s six years old. All she sees is two people who need something. That makes you want to be better. That makes you want to do more, realizing that the smallest token is the largest gift that you can give someone. The most important takeaway for all of us in life is be the best that you can be. Be true to you, but be the best that you can be. If you can say to someone who looks like they’re having a hard day, “I really like that blouse. That’s nice.” They smile and they feel better about themselves. It’s a two-second thing. It doesn’t mean anything to you, but it sure does to the person on the receiving end. I think that that’s the biggest thing for me is you need to try and make everybody feel better. It makes you feel better inside, knowing that you’ve made someone smile. You’ve given someone something to take from their day. If it’s part of their gratitude post, great. If not, better luck next time. My name is Lynette Chambers, and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Episode 494 - Lynette Chambers
11/11/2024
Episode 494 - Lynette Chambers
Lynette Chambers, a 9-year PAH (pulmonary arterial hypertension) patient, has also been diagnosed with pulmonary fibrosis (PF). She shares her journey, from struggling with daily activities to being admitted to the hospital with severely low oxygen levels. Lynette discusses the emotional challenges of facing a terminal illness and the difficulty of balancing work and family time. Despite the hard reality, Lynette emphasizes the importance of gratitude, making others feel better, and being the best version of oneself. She finds strength in her family, especially her grandchildren, and strives to create lasting memories and make a positive impact on those around her. Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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John Kingrey, MD and Nicole Fitzgerald - phaware® interview 493
11/05/2024
John Kingrey, MD and Nicole Fitzgerald - phaware® interview 493
John Kingrey, MD, Director of the Pulmonary Hypertension (PH) Center of Oklahoma and PH patient, Nicole Fitzgerald, discuss the importance of the physician-patient relationship as it relates to participation in clinical trials including the Phase 2 TORREY Study (now the Phase 3 ). #GossamerBioPartner #sponsored John Kingrey, MD: My name is John Kingrey. I’m the director of the Pulmonary Hypertension Center at INTEGRIS Health. I’m a pulmonologist in pulmonary hypertension in Oklahoma City. I’ve been involved in pulmonary hypertension starting in the early part of my medical training, and I chose to pursue pulmonary hypertension as a subspecialty within pulmonary medicine and have been doing that ever since. Nicole Fitzgerald: My name is Nicole Fitzgerald. I am 44 years old and I am a pulmonary hypertension patient. I’m originally from Los Angeles, but I currently live in Oklahoma City, where I have lived for the past six years. John Kingrey, MD: Today, we’re going to discuss the importance of the physician-patient relationship as it relates to participation in clinical trials. Clinical trials are an essential part of most fields of medicine, but particularly pulmonary hypertension at this time, because there is so much development of new pathways and new agents that can potentially change the landscape of the disease. At our pulmonary hypertension center, we have typically multiple clinical trials going on at one time. The nature of each clinical trial is a little bit different, and the population that we are investigating is also different, but it’s all centered around patients with pulmonary hypertension. However, it’s really important to understand that clinical trials have a very focused population for which they’re interested in, because they’re trying to investigate whether or not a certain product or a certain device could be effective in this specific disease state that they think that it will work in. Each patient that comes in, in some way, either formally or informally is considered for clinical trials. We know as a center, what our current clinical trial profile looks like and the specific population that could be potential candidates. When I’m in a clinic visit with a patient, I am thinking about what we have potentially to offer them and whether or not they would be a candidate. For a lot of our patients, I know that because of either how long they’ve had the disease or the specific nature of their disease and its severity, they may not be a clinical trial candidate, so it’s not really brought up in many patient encounters. However, when I have a patient that I think may fit one of our clinical trials and I think, more importantly, that it would be a good opportunity that would be of benefit to the patient, then that’s when I will bring that up, usually as part of a typical clinic encounter. Sometimes, depending on the clinical trial, we will have a special visit to discuss a clinical trial opportunity with myself or my partner, to see if the patient would be interested and if they would be a good fit. Nicole Fitzgerald: Because I was diagnosed so quickly before my husband and I had to make a big cross-country move, pulmonary hypertension was something obviously I had never heard of. It just was not even on my radar as something I should even know that this is something that could happen. I just had no idea that it even existed. I would say because we moved so soon after and I was consumed with trying to just find my footing in terms of the move, and then finding the right doctors in Oklahoma, because I had none when I came out here, there was a lot that I was learning to navigate. Obviously, it was a lot of change at once. For a long time, just trying to get myself with the right doctors, having all the right testing done, kind of getting a control on my medicines and what things I should take. Just getting myself stable took a very long time. The first year or so I would say was the most challenging, just because of all the newness and all the adjustments that were being made in my life in general, so it took a lot. I got worse before I got better. So, like I said, just getting stable I would say was my biggest priority for a very long time. It took a while before I started to hear from other patients that there were clinical trials available. Once those opportunities became available to me, that was something that I was very interested in, because I wanted to kind of be as aggressive as possible with treating my PH. Initially, that was not even on my radar. It was just more about getting myself to a point in my health where I was stable and can do normal things, which was a real big challenge for me, especially early on. So, it was primarily online through Facebook support groups for patients with PH. You kind of hear rumblings, just word of mouth of other patients were taking a particular trial, or participating here or there. Questions had been brought up with their physicians wherever they were from. You kind of branch out a little bit to know that there are trials taking place. It wasn’t something where I right away sought that out for myself. For me as a patient, I was just excited that research was being done. I think that’s something that all of us as patients just want to know, that there’s hope out there. I think we get kind of so bogged down in the drugs that we have available to us. There’s only so many and you are put on one thing, and you either respond to it well or you don’t, and then they take you off, and maybe you get put on something else depending on how you’re feeling. So, after a while you just kind of get kind of in that routine, but you want to know that there is hope coming, that there are better drugs coming, that there’s something else out there. So, I think as patients, we’re just all really excited and curious to know that there’s research being done, there’s trials being done. So, that was something exciting that I had heard about online, but it wasn’t anything necessarily specific where I right away brought that up to Dr. Kingrey. That was something that he brought up with me and I’m so happy he did. It worked out well for me in the end. John Kingrey, MD: I think that one thing that’s really important for patients to understand, is that clinical trial work is really, really hard work. The ability to recruit patients for clinical trial takes a lot of effort in identifying the right patient population and identifying patients who are not excluded for a number of many reasons that patients can excluded from trials, and again that they have the right disease state. The next step is finding someone who is willing and open to the concept of a clinical trial. I think that there are a lot of people out there that look at clinical trials as a big risk. A phrase that I hear a lot and I know that other providers hear a lot is, patients say, “Well, am I going to be a Guinea pig?” That is kind of an antiquated way of looking at things, because the clinical trial space is so heavily regulated and protected, even in the hands of a provider who may not be paying it that close of attention to detail. It’s really, really hard to enroll a patient in a clinical trial and have them be subject to a real significant risk, that at least has not been properly spelled out. Many people don’t understand, for example, that when it gets to a phase of clinical trial where human beings are involved, that the medications or the devices, or whatever it might be have been trialed in both animal studies and also in healthy patients that don’t have the disease, just to demonstrate safety. That’s a barrier for some people. I think though, that probably the biggest barrier is the relationship that they have with their physician, because it doesn’t matter if I told a patient that I’ve got a clinical trial in Tylenol that’s been on the market for decades. If they don’t trust me and they don’t have a relationship with me, it’s unlikely that that person’s going to want to be part of a clinical trial. There’s two reasons generally that people end up doing a clinical trial. One is that they look at the potential benefit for their own health, which of course is a huge reason that it exists. The other is because that patient really wants to benefit others who may come after them, who will be suffering from a similar disease and now potentially have a new therapy, because of the way that was laid forward by clinical trial participants. The most important thing that I would say to patients and also to physicians, is if you want to be involved in a clinical trial, and from my standpoint, if you want your patients to be involved, you better know your patients. You better know more than just what’s in black and white. You better have a relationship with them. And if you don’t, it’s going to be really, really hard to bring up a clinical trial that’s going to potentially come across as very scary and very experimental, when in reality there’s a lot of protection built into clinical trials, particularly in the United States, that is squarely focused on protecting the patient. Another element in considering clinical trial participation is making sure that the clinical trial is going to fit within the signature that the patient needs. Another way of saying that is, consideration of participation in clinical trials is really important to tailor the needs of the patient and ensure that the clinical trial fits within the boundaries of what would be an individualized treatment plan. In pulmonary hypertension, for example, we do have some guidelines regarding treatment and how patients should generally be approached. However, it is not cookie cutter. There are several different pathways that patients can go down and arrive at a good outcome, but the considerations of how to get there are variable. They would include patient’s preferences on how they take medicines, patient’s tolerance of certain side effects that certain classes of medications have, the amount of pills that someone needs to take and so forth. So in clinical trial, the point here is, every patient has a very individualized treatment plan, and when we think about what the next thing is that the patient needs, we have to ask ourselves, does any of the clinical trials that I’m offering, assuming that the patient fits the general profile, is that going to be beneficial to them? So, severity of disease is interesting, because some clinical trials exclude patients who are doing too well, others exclude patients who are too sick. You have to be able to take that into account, as to whether or not the medication or the certain application of a medication is handled. We have to be incredibly prescriptive in identifying the right type of patient for the right clinical trial. Another element of this is helping the patient understand where the potential treatment comes into play. Many times, I will have a patient, for example, that comes to me and is doing worse. If I have a clinical trial that that patient would fit, but that particular clinical trial has a study design that is very common, where neither myself nor the patient know if they’re going to get the medication, meaning that it’s placebo-blinded, so it’s a double-blinded placebo trial, and I know that that patient needs more therapy, I don’t feel like it would be ethical. If I have something else to use that I know would help them that would be already on the market, then I’m not going to have that patient be in a clinical trial, where they could be going six or 12 months without getting anything. Or even if they did get the medicine, something that’s clearly under investigation, when I could be reaching into the cupboard and grabbing a medicine that is already available that I know would be immediately beneficial. There are a lot of considerations. That’s just one example. Other things would include patients access to coming to a clinical trial. In Oklahoma City, we have many patients who live hours away from us, and though clinical trials are incredibly good about making sure that the patient doesn’t have out-of-pocket costs for things like travel, it still can be a matter of time not available or reasonable for the patient to do it, because of travel time or perhaps they work and they wouldn’t be able to get off work. So there’s lots of things that have to be under consideration before somebody really is able to fit in a clinical trial. Nicole Fitzgerald: For me, I think a lot of consideration, obviously, was given to my health at the time. During that period, I was a little bit more stable, but not as good as I had been or as I could be. So, it was one of those things where I just kind of fit into a category where it was like, okay, well, I’m not super sick. I’m not as sick as I was initially when I was diagnosed and at various times had been, but I’m also not feeling great. I’m not feeling at my very best. I’m sure I could be doing better. So, it was one of those things where it was like, okay, well, I’m just kind of at a standstill, but not at a place where I am necessarily as good as I want to be. For me, it was obviously interesting to have something that could help me more than I was being helped already. Then, there’s also, as Dr. Kingrey mentioned earlier, you’re in a position where you want to help yourself, but then there’s also that feeling of, okay, well I can do something potentially that can help a lot of other patients. So for me, that’s obviously always a factor. There’s a lot of things that get taken into consideration when you want to participate in a trial. Obviously, you have to meet the criteria of whatever trial you’re participating in. There’s things that involve just your own personal lifestyle, the way that you might administer the medication, how often the trial visits are, Like he mentioned, Oklahoma City, there’s people that live pretty far out. I am fortunate that I live only 20 minutes away from the center where all my trial visits are, so it’s convenient for me, as opposed to other people might have a lot more difficulty just getting back and forth. There are lots of things that factor in, but for me it seemed like it would be a good fit and the time constraints weren’t anything extraordinary. So, it just worked out where I felt like, okay, this is something that could really benefit me, but also potentially help others down the road. John Kingrey, MD: Once a patient is identified as a potential candidate for a clinical trial, that’s just the beginning. Usually the next step is, I will have a conversation with the patient about the opportunity to participate in a clinical trial. With that, will be a general overview of the type of patient that the clinical trial is looking for, which obviously the patient you’re talking to should fit that. Then, I will give an overview of what the general purpose of the trial is and how it’s constructed, in very high level terms, not getting into the nitty-gritty at that point, but just to explain generally what we’re doing. The next thing that I do is if the patient has interest, then, I will talk to my clinical trial coordinator and they will give the patient a phone call. Or, if the coordinator is available, I’ll have them come down to the clinic right there in real time, if both the coordinator and the patient has the time. Either way, there’s a conversation that takes place between the clinical trial coordinator and the patient that involves additional detail to kind of put a little bit more meat on the bones, so to speak. At that point, the patient says, “Yes, I’m really interested in this.” Then, typically the clinical trial coordinator will go through exclusion and inclusion criteria to make sure that the patient is the right fit. Sometimes that happens independently. Sometimes the clinical trial coordinator will have questions, and they will come to me and we’ll make sure that yes, the patient is the right fit. Once we’ve cleared that step and there isn’t anything obvious, then, starts the process of an official screening. Screening is when the patient comes in and provides consent to participate in the clinical trial. Then, a series of tests and questions happen to make sure that there isn’t anything else right before the patient would go into the clinical trial that would potentially exclude them. The cool thing is, is that patients with pulmonary hypertension are not going to be unfamiliar with most of what is required. Every trial is different, but most trials are going to have some combination of requiring a six-minute walk, lung function tests, imaging, sometimes a CT scan, sometimes perhaps an MRI, chest x-ray. Some clinical trials will require a repeat heart catheterization within a certain period of time of enrolling in the clinical trial. Not all of them. Some of them just want to demonstrate that at some point, yes, the patient was properly diagnosed with pulmonary hypertension of whatever variety the study is looking at. Always blood work and then a questionnaire. Now, that is not necessarily a comprehensive list. There could be a few other things, but it’s pretty rare that there’s something that the patient has never heard of before that needs to be done as part of a clinical trial evaluation for pulmonary hypertension, because most of what we’re looking at are things that we’re already doing in the evaluation of a patient. Once you go through all of that, it’s kind of like a final system of checks and balances. Okay, we did the screening testing, everything seems to be checked out, and yep, you are a go. Everything is good. There’s nothing that has excluded you. You’re exactly the right patient. Once you clear that hurdle, then the patient becomes enrolled officially in the clinical trial. Of course, at that point, it depends on how the trial is designed. There could be a medication involved or perhaps a different application of a known medication, perhaps a device or something that the patient will need to have with them, or inserted or whatever. There’s lots of different possibilities, but that process of screening really starts well before an official screening. Then, you go through the testing to make sure that everything still checks out. Then, you go through the clinical trial. Clinical trials are of various duration. Usually in pulmonary hypertension, it’s a pretty common interval is usually at least six months. There have been certainly clinical trials that have lasted shorter than that. There are some that go beyond that. Obviously, the patient is going to know that before they would ever even sign consent, what the duration of the trial is. So, you go through the clinical trial. At the end of the investigation period, many clinical trials then have an opportunity to go into what’s called an open label extension. If it’s a drug trial and the drug is not approved on the market yet, then the only way to get access to the medication is within the confines of a clinical trial. So the cool thing is, and it’s really kind of a fringe benefit of participating in the clinical trial, is the patient many times, and the patient would know this before entering the trial if this were an option, it’s not held out as a mystery until you get done with the trial period. So let’s say the trial is six months. At the end of the six months, you and the physician are unaware if you’ve got the drug, if it was a drug trial, for example. At the end of the six months, many clinical trials are structured so that you can enter into an open label extension, which means the patient is guaranteed to get the drug, not a placebo, but the actual drug, for a...
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Episode 493 - John Kingrey, MD and Nicole Fitzgerald
11/04/2024
Episode 493 - John Kingrey, MD and Nicole Fitzgerald
John Kingrey, MD, Director of the Pulmonary Hypertension (PH) Center of Oklahoma and PH patient, Nicole Fitzgerald, discuss the importance of the physician-patient relationship as it relates to participation in clinical trials including the Phase 2 TORREY Study (now the ). #GossamerBioPartner #sponsored Learn more about pulmonary hypertension trials at . Engage for a cure: #phaware Share your story: Like, Subscribe and Follow us: .
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Kathy Downey - phaware® interview 492
10/29/2024
Kathy Downey - phaware® interview 492
Canadian patient, Kathy Downey, recounts her four-and-a-half-year journey with pulmonary hypertension (PH). Kathy shares the challenges of living with the condition, including reliance on high-flow oxygen and the emotional toll it has taken. Despite these obstacles, she has become a passionate advocate, educating others about PH and serving as an ambassador for PHA Canada. Throughout, Kathy highlights the crucial support from her husband and the difficulties caregivers face. My name is Kathy Downey. I am from Calgary, Alberta. I have been a PH patient for four and a half years, and it's been a fun ride. When I first started feeling that something was wrong, I was in San Diego. I was there on a medical conference. We were at a nice little Italian restaurant and we had to climb over all those canals that San Diego is famous for. Everybody was pulling away from me. I thought, well, I didn't drink that much, I didn't eat that much, so what's the problem? I am so out of breath. So, I spent most of that conference just trying to fake it till I make it, because even though I'm with all these doctors, they're all forensic pathologists, so I don't need their help yet. I thought, okay, Kathy, when you get home, you are going on a diet. You are going to exercise until you get this fat ass in control, and there ergo, your breathing will be better. We went back home. I talked to my family doctor. Of course, she was on board with the losing weight and all that. As time progressed, things progressed even more. I got to the point I was no longer working for the medical team. I was working for the faculty of nursing at the University of Calgary. I was trying to walk from building to building as part of my position. I would be taking my phone with me, not to call an emergency, but to be able to catch my breath, and hopefully nobody would notice. One of the worst times was when we had construction going on in the little area where our buildings were, so we had to walk outside in minus thirty-something degree weather, in Celsius. The wind was blowing and it was so cold. I couldn't catch my breath for love nor money. I thought, well, I'm just going to lay down here and die. I'm done. Of course, being a woman, we also have other issues that might be a problem. I was of the age of perimenopausal. I was bleeding a lot, so the tiredness and the short of breath could be attributed to that. Finally, when I was able to find a gynecologist that would actually do surgery for me, I thought, oh, I'm going to get this out of my body and I'm going to be a-okay, I'm going to be back to the land of the living. But it didn't help. So, I went to the ER. Not multiple times, I tried not to go, but when you're not breathing and you think, well, I'm going to go to the ER and they're finally going to figure it out. But I would get doctors saying, "Yeah, you've got something, but I don't know what it is." Or before I had my hysterectomy, one doctor said, "You can't buck the system by coming into emergency and hope that we're going to pull your uterus out of your body." He didn't even attribute any of my concerns of, “I wasn't caring about this, I was caring about my breathing.” I went back to San Diego the month of February 2020. I still was having troubles. I got onto Santa Monica Beach. I got halfway across the beach and I thought, well, here we go again. I'm going to just sit down in the sand, at least it's warmer, and I'm going to die here. It kind of put a damper on that trip. I mean, anybody who's been to San Diego and seen the zoo and the hills in that zoo, let's just say I did a lot of sitting on benches while my daughter and my husband went and did all the other stuff. When I got back, I started having pains in my arms. I couldn't figure out what's that from? Is that because I flew? It's a three-hour plane ride. I got in there, I got a ER doc that actually listened. He finally did all sorts of tests on me. Meanwhile, (my husband) Don had to go back to San Diego. He was staying with our friend who's a forensic pathologist. I was writing, ask Evan, our friend, what should I be asking this ER doc, because I have his attention? Evan says, "See if you can get a copy of your medical records." I got medical records, took pictures and sent them to Don and Evan. He says, "Yeah, ask them why you have pulmonary hypertension." He's a doctor, it's great that he knows a little bit about this stuff, but I'm saying, "No Evan, it's not pulmonary. It's cardiology, because my blood pressure is bad," and everything like that. While I was waiting for Evan and Don to write me back, I was looking through my medical records and down in the corner, in 2017, and this is 2020, it says, “pulmonary hypertension?” Nobody had gone any further in the speculation that I may have had pulmonary hypertension three years prior to when I finally got diagnosed. The short end of the rest of the story is that within a week I was back to see my respirologist, who was a great person of, "Lose the weight and you'll feel better." All of a sudden, he was eating his words and he had me in the PH clinic. Within a week, I was a patient of Dr. Jason Weatherald. I just went through the system really fast. Unfortunately, by the time I had my right heart catheterization, he came in and said, "You have this. You should be in the hospital, but you can't stay in the hospital, because the world is shutting down this evening for Covid." We went back home and it's like, okay, now I've got to deal with this pulmonary hypertension thing. Don't Google it. Covid, what is this Covid? Covid wasn't even on my radar, because I was so busy working with what's going on with me, and oh, I think I see a glimmer of hope that somebody knows what's actually wrong with me. It was hard for the first few months of dealing with not being able to ask the questions that I wanted to ask, and trying to navigate both of the huge issues that were in my life at the time. But I am very grateful to . I've become an ambassador. The Facebook page, the Canadian PHriends Facebook page is a wonderful place to go, because you're with friends. It's private. We did a lot virtually for two or three years, which was also nice to be able to have that camaraderie. About a week after I got diagnosed, I was going back for an assessment and I said to Dr. Weatherald, "I am still finding it really hard to breathe.” I already was put on, I think, two oral medications by that time. He took me for a little walk around the office and he said, "Yeah, you are quite low." He gave me a list of oxygen providers in our city. I went home and I picked one. I phoned them and they came in, and their assessment had me on eight liters per minute already. I have been on relatively high flow since the very beginning. I just celebrated my four and a half year anniversary. I am now currently at 22 liters per minute, because my condition is steadily getting worse. The responsibility of oxygen, is knowing or being able to estimate, how long are you going to be out? How many cylinders of oxygen do you need? What will you do if you get into a snare in the traffic and you're at a standstill for… 20 minutes, could even be detrimental. All these things are going through your mind as you're going out. You're not able to totally relax. In the early days, I was able to go out and have coffee with my friends. I used to drive. I used to go to my appointments by myself. Then, as the oxygen started mounting and mounting and mounting, I found that I would get dizzy. It was a lot lugging the tank in and out of the car. Trying to do that again in -20, -40 degree weather was not fun. My husband ended up taking up that duty and he's been great. But, the one time I had to do it of late, my son and I were going to the appointment and I'm so used to Don preparing everything for me, that we took what we thought would be enough. But then, we got unsure of where the appointment was, a little lost. Then, by the time I got to the appointment, I was already half done with all the oxygen that I brought. When I was on eight, it would be scary, but I knew I could get home even if I ran out of oxygen. At 15 plus, there's no chance. I've gotten to the point now that if I'm without oxygen for even three minutes, I think I'm going to blank out. I always prided myself, they always ask in a clinic situation, "Have you ever fainted?" I say, "No, I'm not a fainter. It must take a lot to make me faint." Well, I found out five minutes without oxygen can be that level that makes me pass out. I've been blessed with the greatest caretaker in the world, and many people in the Calgary PH community know us as the couple, the powerhouse couple. Not because we'd wanted it to be, it just happened that way. I don't know how I could do it on my own, all this, with the appointments, the hospital stays, the oxygen and everything. I have needed Don along the way, mental support, emotional support. Caregivers get a bum rush in many ways that they are not supported by our government. They are only supported if they had a job and had to leave it, and can go on unemployment benefits. But when your husband, or partner, or caregiver has to start looking after you and they're self-employed, and no longer can do their job, there's diddly squat for them. Most people on PH, for me anyway, I was too far gone by the time I was diagnosed, to be able to ever work again. You feel that emotional tug of, I want you to go and work and I want me to go to work, but I want you home in case something happens. So my hat's off to the single people who have to do this by themselves and juggle everything, because he contacts my disability for me, because sometimes they just talk above me. I don't know if it's the disease. I never felt so dumb until I got this disease. The funny thing, I guess, is when I used to have to go for blood work, because I'm on such high flow and during Covid, he couldn't come with me with the extra oxygen. He would sit out in the car and if I had to wait anytime, because even though I had an appointment, I'm still waiting an hour. I would text him and say, oh, I need oxygen. He would come in and assure everybody that he was only there for a second or two. Then, I had to go in during the winter months. I said, "Let's just go in the morning when they first open up, because then it's done and we can get on with our day." They open up at 06:30 in the morning. I'm sitting in the car where it's warm, and Don's standing in the lineup with everybody else with his toque, and his mitts, and everything. This is the sort of stuff that he does for me. Now he does all the cooking and house cleaning. I'm so grateful for him. But I'm also sad for him that he's had to do this. When I was diagnosed, before that, I always wanted to be an authority on something. We'd all like to, not necessarily die the way Matthew Perry died, but he had remembrances and accolades and all that. I don't think I'm going to get that, but I wanted to be an authority on something. When I got my disease, I said, I am going to be an authority on this. I am going to learn everything I can about this. We did that. We studied, we talked to people, we read reputable articles and all that sort of stuff. My main thing was to educate. First, it was to educate family doctors, because they're the ones that see us first and they don't have a clue. It's not their fault, because they are a general doctor. They can't know everything. Even specialists don't know everything either. Throughout the last four years, I make it a point to introduce myself to newbies on the Facebook page. I host a monthly meetup that's virtual across Canada. If somebody's in the hospital, I'm going to write to them and say, "Hey, what are you doing? Everything going well?" Blah, blah blah. Then, somebody said, "Are you interested in becoming an ambassador?" I thought, oh, I don't know if I can do that. I looked at Don and I said, "Do you want to do it?" He said, "Yes, let's do it," because then we have another way of getting the education out. So, we became ambassadors two years ago. Then, we were invited to be on the CRAVE panel, which talked about patient involvement in trials of new drugs and procedures. So, we're being sought out, which is really nice. I think I'm close to being that authority in something. So, if anything else, when I'm gone, somebody can say, "Oh, that Kathy, she knew everything." I am Kathy Downey, and I am aware that I am rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 492 - Kathy Downey
10/28/2024
Episode 492 - Kathy Downey
Canadian patient, Kathy Downey, recounts her four-and-a-half-year journey with pulmonary hypertension (PH). Kathy shares the challenges of living with the condition, including reliance on high-flow oxygen and the emotional toll it has taken. Despite these obstacles, she has become a passionate advocate, educating others about PH and serving as an ambassador for PHA Canada. Throughout, Kathy highlights the crucial support from her husband and the difficulties caregivers face. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Sarah Stone - phaware® interview 491
10/22/2024
Sarah Stone - phaware® interview 491
Sarah Stone shares the story of her family's journey with rare medical conditions in North Central Minnesota. Sarah's life took a turn when her daughter began showing unusual symptoms, eventually diagnosed with pulmonary hypertension, a rare condition complicated by her family's genetic predisposition to HHT. As their medical challenges unfolded with two of her three children facing life-threatening conditions, Sarah discusses the trials of managing these complex issues while living in a remote area. She delves into the lessons learned, the importance of specialized medical care, and the determination to live life fully despite the circumstances. My name is Sarah Stone and we live in North Central Minnesota. We are a family that has a few rare things going on, pulmonary hypertension as a result of HHT. We're a family of five, and this is our story. My husband and I got married and didn't have kids for a while, and we wanted to start a family and we adopted our oldest son as a newborn. Then, we had two surprise babies that came along after that that we gave birth to. Everything was great. The pregnancy was great. The birth was great. Since we live in a remote area of North Central Minnesota, we did not want to be on the side of the road trying to get to the hospital that's about an hour drive away. We just chose birth at home for safety reasons. We had a great team working with us and felt really comfortable and confident about all that. All that went wonderful. Then, about 15 months into our new life with our toddler son and our 15-month-old daughter, we were newly living up in northern Minnesota for approximately a year. I had to keep taking my daughter into the doctor, because I just felt like something wasn't quite right. She had curious breathing things going on. I thought she's breathing quite heavy today, or she seems tired. I kept bringing her in to see if there was maybe bronchitis or pneumonia or just what was going on. Each time, things checked out fine. No clinical signs of bronchitis or pneumonia is what my very uneducated, ignorant brain was thinking of, because that's all I know of when I would hear about breathing issues. I'm not a person to compare kids. Every kid is unique and has their own pathway of development within certain parameters. She just seemed kind of low-key compared to her cousin and I thought, "Oh, this poor child. Is she going to be a quiet kind of wallflower child and she's stuck with this really outgoing, people-person kind of mom?" At that point, when she was about approximately a year old, she was breastfeeding a lot more and she was really struggling with sleep at night. I was just exhausted thinking, "Oh my goodness, she's supposed to be feeding less and why is she scream every time I try to lay her down." I would end up just holding her in the rocking chair while she sat up and was sleeping sitting up. I later learned in hindsight from her pulmonary hypertension specialist out at CHOP a couple of years later once we got connected with a peds PH team, which is really important to do. Pediatric cardiologists are wonderful, but they're human and not everybody can know everything about every possible rare disease, so it's really important for families to get connected with a pediatric pulmonary hypertension specialist. I learned in hindsight that the reason it was so hard for her to lay down and she would be screaming at night is because that great pressure that she felt on her chest, which I hear from adults described as an elephant sitting on your chest. As a one-year-old, she would figure out how to communicate that to me with a very specific high-pitched frequency or pitch of squeal that I could not do anything except obey what she needed or wanted. The only way I can think to describe it to people is that it was like an ice pick. It pierced a certain part of my brain, the certain sound she would make. She was super smart. I think it's our job as the adults that have language to just figure it out and read their signs they're trying to give us. She was able to communicate to me how to care for her even though she didn't have words, because she was just a one-year-old. We would sleep sitting up in the rocking chair. She had given up pulling herself up and she had given up walking along furniture. She just would be happy to sit in the middle of the floor and play with some blocks and whatever as a one-year-old. She had the ability to walk. I was really puzzled thinking she must not have some sort of neurological involvement. If it is neurological involvement, it must be intermittent, because she is able to walk and she is able to do these coordinating things, so I don't know that it's necessarily something with her brain. So as a mom, I'm observing this, troubleshooting it and just knowing there's something wrong. To be fair, pulmonary hypertension is really tricky to correctly diagnose. My opinion is that our daughter had a very quick diagnosis, even though it might sound like it was kind of a long road from 9 months to 15 months, but that's nothing compared to what a lot of people go through being misdiagnosed or undiagnosed for many years in their life. She was feeding so often, because she needed calories. Little did I know, but her heart was pumping like crazy. I remember my mom saying something to me like, "Look at the artery in her neck." She said, "Look at how much that's pumping and pulsing." It was something where this pulmonary hypertension, now in hindsight I know, had come on gradually. I could not tell the difference, because I was seeing my daughter every day, whereas my mom saw her every once in a while, because we live about four hours away. She said, "That neck artery pumping, that doesn't seem quite right." From that, in hindsight now we know, that at that time her heart was working really hard. I took her into our local clinic and I was just like, "Something's not right with her breathing. Hi, I'm the crazy mom that's here again. Will you please check her out again?" Our doctor looked at her again. She said, "Well, I can't find anything clinically wrong. Let's just take a look at chest X-ray images." She took a picture of Evelyn's chest via X-ray. She came back to me and she said. In so many words, she was very cool and calm about it, but she was like, "This isn't good. You're going to go to higher care today. We're going to call some kind of transportation, a helicopter or ambulance. Her heart is really, really enlarged and I've spoken and consulted with a heart person who knows more about this than me, and we are going to get you to higher care now. Today, in the next hour." We live in a part of North Central Minnesota that the nearest village is like a hundred people and then the nearest town is like 400 people. But at that town, in Bigfork, Minnesota, there is an excellent rural critical access regional medical facility that we can take a helicopter or an airplane and get to higher care. Living where we live, sometimes there's challenges with things turning into a possible wilderness context, you could say, which I believe if I'm up-to-date on these terminologies is getting to higher care like an ER in two hours or less. Living up here, we're always figuring out how to think pre-emergently and to give ourselves the best advantage to get the best care that we might need. Actually, what happened at that time is there was a lot of snow and there was a snowstorm going on, so there was no helicopter or fixed-wing aircraft available. We ended up having to take an ambulance. Instead of the four-hour drive down to the Minneapolis area, we had to take an ambulance ride two-and-a-half hours to Duluth, Minnesota where we were in a peds ICU-type setting. Here's how ignorant I was about all of this kind of thing. Looking back, we're in Duluth and we had a really great young doctor there. He was sitting there with us along with a nurse, and I thought, "Wow, this is so great. They really have great customer service, because here I am one-on-one. We've got a whole nurse to ourselves and she really seems to be paying attention to only us. Wow!" I think back on that sort of humorously, because I realized, well, it's because my child was probably near death, because she had such an enlarged heart. She was in a dangerous situation. We were at that hospital overnight and they took really great care of us and then they put us on an airplane immediately the next morning as soon as they could get us out of there. We were taken down to Minneapolis to Children's of Minnesota where Dr. Charlie Baker helped us out. He was the lucky or unlucky guy that had us as a patient that day. I feel like we were very blessed to have him. He said that he had done a heart catheterization. Another thing I like to laugh about is poor Dr. Baker, his face, he looked like he had seen a ghost. I thought, "Wow, this guy needs a vacation. He needs to get out from under these fluorescent lights." Here I am concerned about his well-being. He could use some sun. Honestly, he was probably kind of pale, because he had just been the doctor in the catheterization lab performing the heart cath and was probably not able to believe this bad report that she was so bad off. Basically, the doctor said, "What we can do is go ahead and try a systemic vasodilator called sildenafil and see if she responds to that. If she does, awesome. If not, then we're looking at some sort of transplant consideration," because at the time her heart was so, so, so, so, huge, huge, and enlarged. She did show trending in the right direction of improvement. She did respond to the systemic vasodilator. This was in December, when she was correctly diagnosed. By August, essentially, she was labeled as out of heart failure. So that was awesome. We were really happy about that. She was on the oxygen 24/7. In the winter, it was really great. She would just pull a little sled. She was very independent and would just pull a sled around with her oxygen tank in it if we wanted to be outside. We live in a cold climate. She has always been really busy and active and enjoyed just living her life. We decided that this is her life, she doesn't know anything any different. My husband and I had worked with a lot of different people with special needs over the years, whether it be cognitive or physical, or both. Some of the families and kids that we really respected and really were impressed by were families that didn't put limits on their loved ones. They, within reason, allowed them to push their boundaries and figure out who they could be and to not feel sorry for them. This is their life. Something we realized is, it's kind of silly to feel sorry for people because that would be like feeling sorry for me, because I have blue eyes or feeling sorry for me, because I'm right-handed. It's just life and it's just how we are. Let's deal with that appropriately, but it's not something that needs to necessarily be felt sorry about. We found out that we needed to have a pediatric PH team would be the wisest thing. We somehow found the Families of Children with Pulmonary Hypertension Facebook group that was started by a mom out east. We joined that group. We learned from people there that it's super important to have a pediatric PH team or doctor that's managing the care of your child because there's all these, “yeah, but” situations and things that people and other pediatric cardiologists don't know that they don't know or they just haven't had the experience or the opportunity to practice because it is a rare thing. Pulmonary hypertension is rare. So we went to the pediatric doctor that we were most acquainted with and comfortable with, which was the team out at CHOP, which is in Pennsylvania. Mind you, we live in Minnesota. We chose that team. They're all really great. Way back then it was Dr. Brian Hanna. We interviewed him and he talked with us to see if we all wanted to work together, but we had decided to go with that team, because we had met Steve Walker at the PHA Conference, which at that time was in Indianapolis. Evelyn was around two years old when she started doctoring with the team at CHOP. They were awesome, and their whole team was awesome. Dr. Hanna recommended that she be on subcutaneous infused Remodulin. That was the second of three possible PH meds that she started as a toddler. Then, she was on sildenafil. About that time that we were starting Remodulin, I was really feeling sick. We were at CHOP for the first time. I just remember being very, very nauseous. Well, maybe I'm under a lot of stress. Maybe I haven't had enough water. We have just been flying on airplanes and we are dealing with this highly stressful rare diagnosis and all these decisions to make about new medication and whatnot. I didn't know. Maybe I was having some sort of acute stress reaction, ASRing in my own way or something. Turns out I was pregnant with our second surprise baby. Now, unbeknownst to us, heading towards the HHT understanding. Learning that I was pregnant, we shared that with the team at CHOP. Dr. Hanna even ordered that it'd be really wise to have certain unique checkups done in utero with this new baby that was coming. He ordered a very fancy, high-end… I think it was 3D imaging type ultrasound. So we did that. Because at the time Evelyn was diagnosed, her PH was diagnosed as idiopathic pulmonary hypertension. They did not know the cause. Idiopathic means no known cause. They did do a simple genetic test for some of the most common genetic markers that can cause pulmonary hypertension. Those results came back normal, so those were not the case. We went ahead and did all these additional tests and everything looked good. So that pregnancy was uneventful, boring, and healthy like everybody wants their pregnancies to be. Then, Josiah at about three weeks of age, I took him into his well-baby check. Dr. Harmon, our local GP, she did all the normal checking out. His soft spot on the top of his head was full. Not bulging, but it was full, and she was like, "Oh, this is strange." Given that we already have had these unexplainable things going on with Evelyn, we decided that it would be wise, the doctor said, "We're going to not mess around. You don't need to go by ambulance." But she said, "Go home and pack your things and you're going to drive down to Children's of Minnesota today, and we're going to have them do some imaging on his head." We thought, "Oh boy, here we go." That was a little bit traumatic. We had moved up here because we wanted to enjoy the outdoors and peace and quiet and live our lives and work our jobs and raise our kids in a beautiful northern Minnesota location that people save their vacation time all year long to be able to get away to and vacation in. Here we are having to take airplanes, helicopters, go home, packing your bags, and get down to the ER in the next four or five hours. We checked in at the ER down in Minneapolis and got in to do imaging right away. Sure enough, there was a malformed vein in Josiah's brain. It was this vein that we all have called vein of Galen. It was a really big tangled mess. What was happening was, this malformed vein was blocking the flow of the cerebral spinal fluid. It was causing hydrocephalus for him, thus the soft spot being full. We thought, "Okay, well, here we go." They diagnosed it as vein of Galen malformation is the official name of it, which makes sense. He did have five embolization surgeries on his vein of Galen malformation from about the time he was one and a half months of age until one and a half years of age. All those went really well. Dr. Jagadeesan at U of M Masonic Children's did an awesome job and was in great communication being guided by the wisdom and experience of Dr. Alejandro Berenstein at Mount Sinai Medical. Everything went great. Our son was very fortunate to have really great outcomes. He is great in every way. He has no complications from any of his brain surgeries. We're really thankful for that. With the blood rushing through that vein of Galen malformation and right to the heart, Josiah was discovered to have a little bit of edema, a little bit of extra fluid on the heart. But somehow his heart adapted and he did not get pulmonary hypertension as a result of the vein of Galen malformation. So then, I'm wondering, what are we doing that we've got two kids with vascular types of involvement, and so talked to our daughter's PH team and said, "Hey, can we do that full panel to test?" I said, "I think we might have HHT," thanks to something a mom named Kelly on our PH support group on Facebook had shared about her daughter and a lot of the things were the same. She was awesome to call me one day when I reached out to her and I said, "Tell me more about HHT." After speaking with her, I said to my daughter's PH team, "Hey, can we finish that entire genetic panel. I think we might have HHT in our family?" And they said, "Sure. You probably don't have HHT, that's really rare, but yeah, let's go ahead and do that. No problem." We did that at the next time we were down for Evelyn's annual right heart cath. We were over in Philly for that. They called back and said, "Guess what? You were right. It is HHT." So our family has the ACVRL1 genetic variance now is the language. Not genetic mutation anymore, genetic variance. Sounds a little nicer I guess. We have the ACVRL1 genetic variance causing HHT and it's the HHT that's causing the pulmonary hypertension. As you can see, we've got a lot of crazy different inroads of rare involvement in our family with the pulmonary hypertension, the vein of Galen malformation, the HHT. We just decided, hey, we're going to make the best of this. What you get is what you get and you make the best of it. We just have decided as a family to try to live life to the fullest. We've let our kids do whatever they need or want to do. Luckily, Evelyn's pulmonary hypertension is a type of pulmonary hypertension that she is able to be successful in self-regulating and just kind of reading her body and what she needs. She has enjoyed life learning how to ride bikes and cross-country ski, and run and play. Thankfully, her heart has been mostly anatomically sound. She did have a little bit of a PDA that needed to be closed at around age five. She got all the more efficient with functioning, and so she no longer needed naps. We've just been enjoying travel to go visit family and friends four hours away. We're kind of the oddballs of our family that we live in this far remote area in northern Minnesota. We have had to travel via Angel Flight out to Philadelphia each year to do Evelyn's annual right heart catheterizations. She does have AVMs in her lungs, which are causing some left heart enlargement. She does have some aorta enlargement, but we just try to live life to the fullest and take our situation as normal for us. It's really challenging and it's taxing as a caregiver, it can wear on you. It does cause a lot of family challenges and challenges in processing all of this and deciding what to do or not to do in life and where to invest our time and energy and how to enjoy the best life that we can. It's been a good lesson trying to navigate what's important to us and taking a look at those things and thinking about those things. We have had a really great relationship with our local clinic with the different medications Evelyn's been on. We have a pre-emergent agreement with our local ER that if something's starting to look like it might be sketchy, we go to the ER a couple of steps ahead of when your average person might decide to go to the ER, just so that they have the chance to call the helicopter, call the airplane and that kind of thing. There was a time where in the different medications Evelyn's been using, she at one point was on the sub-Q infused Remodulin. Then, she...
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Episode 491 - Sarah Stone
10/21/2024
Episode 491 - Sarah Stone
Sarah Stone shares the story of her family's journey with rare medical conditions in North Central Minnesota. Sarah's life took a turn when her daughter began showing unusual symptoms, eventually diagnosed with pulmonary hypertension, a rare condition complicated by her family's genetic predisposition to HHT. As their medical challenges unfolded with two of her three children facing life-threatening conditions, Sarah discusses the trials of managing these complex issues while living in a remote area. She delves into the lessons learned, the importance of specialized medical care, and the determination to live life fully despite the circumstances. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Maria Morais - phaware® interview 490
10/15/2024
Maria Morais - phaware® interview 490
Maria Morias, a registered nurse from Canada, shares her journey of managing multiple autoimmune diseases, including her recent diagnosis of pulmonary arterial hypertension. Previously dealing with scleroderma, Raynaud's phenomenon, and a rare liver disease leading to a liver transplant, Maria's ongoing symptoms of shortness of breath were eventually linked to pulmonary hypertension. Her story reflects on the psychological impact of chronic illness and the empowerment gained from community support and self-advocacy. My name is Maria Morias and I live just outside of Toronto Ontario in Canada. I'm 54 years old and I’m newly diagnosed with pulmonary arterial hypertension. I have other autoimmune diseases. So, to sort have another disease added to my list has been overwhelming to understand, but also it's given me an answer to my shortness of breath. My pulmonary hypertension is considered Group 1, associated with scleroderma. I do have mixed connective tissue disease. I've been seeing a rheumatologist for a number of years. In my early 20s, I was first diagnosed with Raynaud's phenomenon and circulation issues of my hands and feet that have been severe throughout my life course. Then, in 2018, got diagnosed with a major rare liver disease, autoimmune rare liver disease called primary biliary cholangitis. I developed several severe symptoms of fatigue, itchiness, brain fog, and shortness of breath. So, the shortness of breath I sort of figured, at that time, was due to ascites and a massive fluid buildup in my abdomen, where towards the end of my liver disease, just pre transplant, my liver became cirrhotic and not effective in what it was needing to do. So, fluid is backing up into my abdomen. I'd gain about 20 pounds a week, go in on a Friday morning and have fluid drained, 10 liters of fluid drained. I thought there was a lot of compression on the diaphragm causing a lot of shortness of breath. Pulmonary hypertension was never on the radar of any of my physicians, my hepatologist, my rheumatologist. Post liver transplant in 2021, I started to recover. That was a pretty intense recovery of regaining my energy and short walks, but I was still having severe shortness of breath. Slowly along the way, I had the respiratory sort of check out with a six-minute walk. I’ve been doing that every six months with a respirologist and pulmonary function test. It wasn't until last probably December of 2023, my rheumatologist said, "Let's do some more testing." I did have interstitial lung disease as a general diagnosis from the respirologist, but this rheumatologist thought, "Let's check some other things." So he sent me for an echocardiogram, which I thought, "Well, how does this work?" The rheumatologist looking after respirologists sort of work. It was a good thing, right? So, never discount somebody's professional opinion as an option. Of course, I wanted to inform my respirologist and he said, "Sure, no problem." So good thing that there wasn't any territorial or issues there. My pressures came back pretty high on echo. So, that led to sort of the next investigation. Then, I was referred to a respirologist that actually deals more, and specifically with pulmonary hypertension. That was earlier this year in 2024, to then go in for the gamut of testing. So, I started off with a sleep study and I thought, "Well, what does a sleep study really have to do with my shortness of breath?" I am a registered nurse. I work in public health. I've been a nurse for over 32 years, but more from a health promotion angle. So, it's kind of ironic that I'm on the other end lately as that patient and trying to advocate for my own health. It's really important, right, to navigate through the system, to understand the various healthcare provider roles and where we sit as a patient, how you're feeling, what you're feeling, all your appointments and putting everything together. It's quite overwhelming even with someone that can have ownership over your own health and control. However, you sort of lose that control when you leave it to others to kind of put your story together of coming up with what you really have. I did go for the sleep apnea test, and it was about, well, if you feel more rested then you're better able to cope with your day. So, there was some mild apnea with about seven episodes per hour. But then when I went into REM, it was 24 episodes per hour. I thought, "Okay, so there might be something there." I have not progressed yet to use a PAP machine, so that's kind of on my radar of consulting with others to see if I really need to invest in that or not. Then, I went for a bubble echocardiogram, which was pretty interesting to see these little effervescent bubbles go through my heart. I was watching the screen just out of curiosity. It did show that I did have a small hole in my heart. Apparently, that's been there since birth. I said, "Well, is that the problem of my shortness of breath?" The respirologist said, "Well, you've lived with it for 54 years, so that probably is not the problem." Then, the next sort of definitive exam was the heart catheterization, which sounded pretty scary, right? Having a tube put in right through to your heart can be very scary. The image of something touching your heart, what could happen. So again, went in. It was very simple. I think I was on a relaxation sort of medication. I wasn't put under. It was quick. There we had it, I think it was 30 milligrams of mercury. Certainly I think the cutoff is in the 20s. I’ve now started on a couple of medications. We had to go through a lot of insurance hoops to get that approved. Thankfully, for the Toronto UHN Hospital, they have patient navigation mechanisms that made it very easy. On the back end, they did all the paperwork. Then following that, it was kind of a leveling up, see if I had any major side effects that they listed off, swelling, some discomfort, some cramps in the legs. I had a little bit of that, but then tolerated it. Now, just a couple of weeks ago, have added an additional medication. I still have yet to feel any sort of changes. I understand that it could be six months to a year with a repeat of the heart catheterization. Having a diagnosis is important because then you ask, "Well, how did this happen? Why did it happen?" Leading it all back to, in my case, the autoimmune and scleroderma. Scleroderma was always something that was kind of query on my medical record. I said, "Well, how does that express itself? What am I going to see over the years?" Here it is, the starts of that as well as the interstitial lung disease. I have some start of kidney disease, as well. Also, autoimmune. I'm at stage 3A, which there are five stages, so I still have room. But knowing that how quickly my liver disease progressed to be needing a liver transplant, of course one of the things that goes through my mind is lung transplant. I try to sort of keep in check with that and take one day at a time. Right now, I'm very, very early in the diagnosis, second, third month in. I’m reading a lot, I connected with the to get credible information and to start to connect with people that are also experiencing it. I’m in Facebook groups and just learning. One of the things that I do with my liver disease is I belong to the , and I do a lot of patient advocacy there. I'm actually the VP patient advocacy. It is a totally volunteer organization across Canada. I have met some really wonderful people, lots of great peer patients. I do a lot of mentoring. I'm hoping maybe I can explore some opportunities into the pulmonary hypertension world and learn for myself and help each other sort of cope with all our uniqueness of all these rare diseases. As a nurse, one of the things that I always keep in mind is this psychological component of disease and healthcare challenges. Being in the nursing role, you have the resources, you have the referrals, you have the connections. But then finding myself in a patient role, it's very much different, because it's being done to you and upon you. What I found is that I had to bring my voice forward and have my symptoms be heard. A symptom like fatigue is something that is easily described by a mother, a working mother, children, balancing work life care provider of parents, multiple things. We're always tired. We're a society that's tired because we're doing it to ourselves. Technology makes things easier. Therefore, we do more. To really be as a patient describing what that tiredness is and how that affects the quality of life, but also then having it tied to an actual diagnosis and understanding that, I think it is a totally different situation being a patient than actually nursing that patient. Having a liver transplant for my situation, I still have PBC, primary biliary cholangitis, which is a rare autoimmune disease that affects the small bile ducts of the liver, that can lead to cirrhosis. It's rare in this population group of PBC patients. There are several stages. I unfortunately was part of the less than 30% of patients that ended up with end-stage liver disease. Having said that, it does not take away my PBC. I still live with PBC. It just says that I now have a new liver that my body will live with and eventually may, could develop cirrhosis again. My autoimmune could attack this new liver. However, I'm now 54, so by the time it attacks my liver again, if it does at all, I'll be that much older or not here. It doesn't preclude that I need to be taking care of myself, watching what I eat, my quality of life. I have a lot of medications to maintain the quality of my liver and to prevent that cirrhosis from happening. Whereas I probably lived with the disease without knowing, because that's a lot of patients do. By the time you get diagnosed, it's because something is off. Typically, your liver enzymes, but that's something we don't test daily. The fact is, I still have PBC as that autoimmune liver disease, which may or may not complicate other autoimmune disorders that are evolving in my body, which pulmonary hypertension being that example. Having the lived experience of several autoimmune diseases, and a lot of them sort of rare, it's really helped me to be a better and broader healthcare practitioner. I always understood that we're complex human beings, that factors influence the way we are and how we take care of ourselves. But the added bonus or blessing out of my health issues in the last three years is that I'm now better positioned to speak forward for patients, to bring their voices, to amplify their voices, to listen to what the community of these diseases, those that are living with these autoimmune diseases, in particular, the rare ones, where to give voice, whether it's advocating for better treatment options, like in my PBC situation, being in situations with industry and pharmaceuticals and impacting on the kinds of science and treatments that are being thought about. It's actually a really interesting time where a lot of pharmaceutical companies and physicians are really trying to hear and receive the end user or the patient perspective into their treatment. I'm fortunate enough to find myself in that healthcare system or the credibility of being a nurse. And having that lived experience, the doubling up effect, fortunate, unfortunate, has made me that much more confident. I feel like there's a calling in me to do this. That's why when I was diagnosed with pulmonary hypertension, I thought, "Well, I've done this in the liver world and the living organ donation world, why not?" It's the same sort of mechanisms, just a different disease. My name is Maria Morais, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 490 - Maria Morais
10/14/2024
Episode 490 - Maria Morais
Maria Morias, a registered nurse from Canada, shares her journey of managing multiple autoimmune diseases, including her recent diagnosis of pulmonary arterial hypertension. Previously dealing with scleroderma, Raynaud's phenomenon, and a rare liver disease leading to a liver transplant, Maria's ongoing symptoms of shortness of breath were eventually linked to pulmonary hypertension. Her story reflects on the psychological impact of chronic illness and the empowerment gained from community support and self-advocacy. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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David Lake - phaware® interview 489
10/08/2024
David Lake - phaware® interview 489
David Lake a retired jet pilot, discusses his experience with pulmonary arterial hypertension, initially misdiagnosed as exercise-induced asthma. Lake also manages hemophilia, COPD, and irregular heartbeats, impacting his health significantly. Despite these challenges, he remains hopeful and active in his community and church. His story is a testament to resilience and the quest for better treatment through clinical trials. My name is David Lake. I am from Oakley, Utah, which is a little community just east of Park City at an elevation of 6,500 feet. I have pulmonary arterial hypertension. I've been treated for several years with a pulmonologist for exercise-induced asthma. I was in the VA, because I used to fly jets, and the doctor just off the cuff said that she thought I might have pulmonary hypertension. I'd never heard of it. She says, "You need to call your pulmonologist, and ask him to check you for it." So I contacted my pulmonologist, and he said he did not deal with that, but he knew where to send me. He sent me to Intermountain Health, to the heart and lung team. In two days I was in there. They tested me. Then, Brandi Bolton, who was the nurse practitioner said, "We've got this study for a new drug." And she said, "I think you might qualify for the study." I also have hemophilia. My heart's in constant irregular heartbeat. Then, I have COPD. I've struggled with my breathing basically all my life. It's ironic that I went and flew jets, because I didn't know I had any of this. How do you get in and fly jets in the Navy with hemophilia? So they did the testing. They told me it was a two-year study and that there were three groups. There was the placebo and then the half dose and the full dose. I am 99.9% sure I was in the placebo, because I went downhill. In fact, it was just kind of like holding on by the skin of my teeth to make it six months, because I was getting sicker. They put me on a lot of diuretics and that would make me sick. In fact, at one point in the morning, they pulled me out of the study, and in the afternoon they put me back on, because I said I'd like to stick it out. Because I kind of felt with a bunch of the underlying things I had, it could be some valuable information. So just two weeks ago, it was the end of the six months. I was guaranteed either a half or a full dose. So I got the first shot. I've noticed an improvement. I haven't put my Superman cape on, because I still can't leap buildings and can't run, but I don't feel like I'm going down anymore. It's kind of like it stopped me, maybe even took me back a little bit. I get the next shot a week from today. We'll see what happens as we go through. I have always thought, because I use a nebulizer, I always thought it was this asthma. Now, I realize even when I use the nebulizer, I still struggle breathing. My oxygen content is always up in the upper 90s, so it's not a lack of oxygen. However, they've told me to use supplementing oxygen, because it makes your heart so it doesn't work so hard. What it does, it just makes you feel like when you get up and you take a couple of steps, you feel like you've run a marathon, and you're just huffing and puffing. It's a very, very uncomfortable experience to have that, because you just can't breathe. There's not much you can do for it. I can lay down. I can put on my CPAP and that helps the oxygen a little bit, but it's basically when I try to do anything, it's just really hard. Even to bend over and tie my shoes, which I don't do anymore. I can't get the weight off. I've been gaining weight. I’ve never been like this before, and I can't get it off. They thought it was water. You can't fix this. You're dealing with your mortality now. I hadn't read about the drug. I mean, it's just been approved in March. Then, just in June, it was just approved for PAH, and so the testing is going through. But the relief is I know kind of what's going on, but the forecast was six and a half years and maybe up to 20. So you deal with the mental that this is kind of a terminal disease and that it is a heart disease. That's a mental adjustment you have to go through. Of course, I'm older and I'm not afraid of death and what happens after. It's just, I don't particularly look forward to the process, but it's okay. It's just what we've got. I'm not upset. I just have hopes that this drug might stabilize me, that I'm not going downhill as much. That it takes some of that pressure off my right heart. They just did a right heart catheter again when I went and got the real shot. My nurse said there was just a slight change, but not much change in it. There's two doctors that I deal directly with, and the one is the one that gave the right heart cath. They did an echo and a EKG. I have a watch that's got all that junk on it, too. I've kind of come to the acceptance that you lay in bed or you lay down, and you rest and you think about all these things you want to go do. You think, yeah, I'm going to go do that. So you step up and take two steps and you say, "Oh, I don't think that's going to happen." Physically I just can't do it. I have a farm. I have tractors. It's gotten the point that I have to have my son-in-law help me hook the equipment on. I can run the tractor after I get in it, but if I break down, I’ve got to bring it in and he's work on it. I can't do it anymore, because I'd have to lay flat on my back and then I just can't get off the ground. But a lot of it's age too, because I am older. I'm 78. So these things are all right. It's just that adjustment in your life to what you do. I've been doing a service mission for the Church of Jesus Christ of Latter-Day Saints. I do a lot of stuff on the computer. I've gotten the point I don't really want to do the computer stuff. It's just kind of hard, and I have to force myself. That's a different thing too. So there's a lot of mental going on with this disease, because you're sick, and you're sick basically all the time as far as breathing. I get out of bed, and it used to be I could walk a little ways, but now it's a couple of steps and I'm breathing hard. I can't carry the groceries in anymore. It's kind of hard to watch my wife. I've gotten so that when I go to the grocery store, I use the little electric cart. That's kind of an adjustment, because you're embarrassed. I started carrying oxygen to church, and that's kind of embarrassing. I never saw myself that way, but that's where I am. I found it's nice to have a support group that you can go to, to meet other people that are like you. I'm kind of almost looked upon almost like the chaplain in there, because I talked pretty straightforward about staring the death in. This last one we had, which was a week or so ago, it was interesting that when I mentioned that I've been diagnosed with exercise-induced asthma, this other lady says, "That's what they did to me." Same thing. She's going on the medicine that I've been taking experimentally, and she's hoping that'll make a difference in her. But same thing, she had gained so much weight. It was nice. The one thing is you see other people, even though there's no two people that are exactly alike, right? But they're all dealing with the same kind of general thing. That's been kind of interesting to talk to them about that. The other thing is, I didn't know anything about this. So, a couple of the presentations, they really went into what the disease is. I've read lots of pamphlets and booklets and everything I could find on it. Probably the best I found though was when I went and looked up the medication that I'm on. They had more information in there that I'd never seen before. That was very informative. It really was only about maybe a month ago that I really accepted the fact that this was my heart and lungs and not asthma. That this needed help to at least slow it or get better. But it wasn't going to get better with just what I'd been doing. Before that, I just kept thinking, "Well, I'll just go use my nebulizer and try to exercise." But about a month and a half ago, I turned to my wife, and I said, "This really is a heart disease, and it is really my heart and lungs that are struggling here, not just the breathing." That stress on your right side of your heart is real. About a month ago, a little more than that is when I finally clicked in my brain that that's really what's going on. Maybe that was a good thing about not getting the real drug right away, because I might not have experienced that because the real drug seems to slow that progression. Being on this trial, I've held on to it with the hope that it would help me and help me feel better, but at the very least the results they gain from me can help others. So, the last month waiting for the shots, I mean, I was literally counting the days, because I was just getting so sick. I knew there were other drugs out there that if I would take them would help me, but it wouldn't help the trial. So, that's why I've hung onto it. Then, it was very encouraging, the last thing they told me that they've had very, very good reports that this drug is helping probably better than any other drug they've got. So that's encouraging. I keep that in mind. I'm optimistic. What you do is you plan to live and prepare to die, but you don't plan to die. Your life is going on, so you do all that you can, and you just don't give up. My name's David Lake, and I'm aware that I'm rare, but it's nice to know there are others that are rare too. Learn more about pulmonary hypertension trials at . 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Episode 489 - David Lake
10/07/2024
Episode 489 - David Lake
In this episode, David Lake a retired jet pilot, discusses his experience with pulmonary arterial hypertension, initially misdiagnosed as exercise-induced asthma. Lake also manages hemophilia, COPD, and irregular heartbeats, impacting his health significantly. Despite these challenges, he remains hopeful and active in his community and church. His story is a testament to resilience and the quest for better treatment through clinical trials. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Lori Myers - phaware® interview 488
10/01/2024
Lori Myers - phaware® interview 488
Lori Myers, shares her experience living with VSD and Tetralogy of Fallot, which caused her to be born as a "blue baby" and have various health issues throughout her life. After multiple surgeries and diagnoses, she was eventually told she had pulmonary arterial hypertension (PAH). Despite the prognosis, Lori is now in her tenth year since the diagnosis. She emphasizes the importance of listening to one's own body and following medical advice. Lori finds strength in her family, particularly her grandchildren. My name is Lori Myers. I'm originally from Upstate New York, Adirondack Mountains. I have lived in South Carolina since 1987. I was born with VSD and Tetralogy of Fallot. I was one of a set of twins, the only surviving one. When we were born, we were blue babies. We were three months premature. I had a lot of health issues the whole time growing up. I had congestive heart failure. My pulmonary valve has been replaced four times and repaired twice. After the last one in 2014, I started having more breathing problems than normal. They've diagnosed me with bronchial asthma, with just asthma, with COPD. Everything under the sun except for actually knowing what it was. After my surgery, the pulmonary doctor I was seeing sent me to a specialist for a second opinion, because nothing was working for my asthma at all. He looked at previous heart catheterization that would show him the right side of my heart, my lungs. Without even examining me, he came into my room and said, "You have what's called pulmonary arterial hypertension, PAH." That's when he told me it was a terminal illness. They gave you three to five years at that time. I'm like, "Oh my God, my son is in high school." I couldn't believe it. I was worn out. I was tired. I had no energy. I felt like I was always breathing through a straw. I am so tired of always being tired. Some days I wish I could just pull the covers over my head and make a big wish and wake up and I'm a totally different person. I'm the only one in my family that has ever had a health issue from birth to now. Nobody's ever been born with something they've had to deal with their whole entire life. They all say, "Well, I understand how you feel." Unless they've walked in my shoes, other than the people that I know have this, no one knows how we feel. It doesn't only affect our health physically, but emotionally, psychologically. Not only do I have a terminal illness with my heart but now with my lungs too. It's like a racing game to see which one's going to get me first. When my heart is stable, my lungs act up. When my lungs are stable, my heart acts up. Neither one of mine will survive the other surgery. Even doing them together, my lungs wouldn't make it through the heart and the heart wouldn't make it through the lung surgery. My chest cavity isn't big enough for an actual sized heart and lungs that I should have. My son kind of ignores it because he knows what the realization is. He calls me daily, "How are you doing? Are you all right?" I take care of his two kids. My husband, if I don't answer the phone, he freaks out because he's worried. It's emotional for them as well as us. Three years ago they told me I had three months. My son was engaged, I had a grandchild on the way. The only thing I've ever wanted is to be a grandma. I wanted to be the grandmother like my grandma was. My doctor said, "Lori, I promise you, if you listen to every word I tell you, if you do every single thing I tell you, you'll see that grandbaby." I said, "Okay, I'm ready. Whatcha you got for me?" I followed his direction to the tee. I didn't vary it. I didn't eat anything I wasn't supposed to eat. I didn't drink more than 64 ounces a day. I didn't eat anything processed, nothing canned. I prepared everything. I absolutely ate no salt unless it happened to be in it from some other means than me, but nothing out of a can. I love canned green beans. I could sit down and just open a can of green beans, drain them and just eat them plain. I love vegetables, but nothing processed. I lived on Mountain Dew. I mean, that was my drink of choice. I didn't drink alcohol. Well, I gave up that and went to Sprite Zero. Loved it. I drank Sprite Zero. He said, "You need to get to this weight. You need to do this and just think what you'll look like for your son's wedding." I said, "Okay." So I did all that. My son got married in November. I was as big as a house, because I had walking pneumonia and congested heart failure and didn't know it, because I was planning a wedding and planning Thanksgiving. They got married a week before Thanksgiving. I wasn't paying attention to myself, which I know is the first rule that we're supposed to do. If we don't look out for us, nobody's going to, but my son came first. He always has come first. Now it's my grandchildren, then my son, then me, and my husband's in the mix. Right after that, the day after Thanksgiving, I was admitted in the hospital. I got better from that, went home. My daddy passed away February 2nd. I thought my world had collapsed. I just wanted to crawl right into bed and pull the covers over and say, "Okay, I'm ready, God, take me." My two grandchildren are what gets me up daily and keeps me going. My doctor told my son after my granddad was gone, "You ever take her away from her you might as well forget it." I've seen her every day since. I was diagnosed 2014. They said your average [survival] is three to five, maybe seven [years] if you're lucky. It's 2024 now. 10 years since then and five years more than what they ever thought I'd have. If I could give any advice to a newly diagnosed patient it would be listen to your own body, listen to your doctors. Everybody says listen to your doctor first. You need to listen to your body before you can even tell your doctors anything, because your body knows before a doctor does. Just follow what they say. Seeing the smiles on your family, that's what matters. Not a piece of cheese or the turkey sandwich or the hot dog at the ball game. It's a smiles on your grandchildrens’ faces. My name is Lori Myers and I'm aware that I am rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 488 - Lori Myers
09/30/2024
Episode 488 - Lori Myers
Lori Myers, shares her experience living with VSD and Tetralogy of Fallot, which caused her to be born as a "blue baby" and have various health issues throughout her life. After multiple surgeries and diagnoses, she was eventually told she had pulmonary arterial hypertension (PAH). Despite the prognosis, Lori is now in her tenth year since the diagnosis. She emphasizes the importance of listening to one's own body and following medical advice. Lori finds strength in her family, particularly her grandchildren. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Sue Liss - phaware® interview 487
09/24/2024
Sue Liss - phaware® interview 487
PAH patient and Chicago-area support group leader, Sue Liss, discusses her pulmonary arterial hypertension diagnosis. Sue is involved with multiple PH related advocacy groups. Through these groups, she offers support and guidance, particularly in navigating medication funding challenges and accessing the right care, emphasizing the importance of community and shared experiences in managing this rare disease. I'm Sue Liss. I was diagnosed with pulmonary arterial hypertension in 2007 when I was living in Colorado. I am now living in Chicago. I had had cancer. I had a reaction to the chemotherapy and they had to start me on heparin. They found out I was allergic to heparin. I had clots in my lungs and arteries and they cannot say 100% for sure that that's why the PH developed, which is why I'm considered idiopathic. But they do believe that those clots in my lungs may have led to it. I was diagnosed when I was living in Colorado. They did tell me at that time that I should move to a lower altitude, because I was diagnosed severe. I was still working full time and I said, "Well, you going to buy my house and find me a new job?" So I stayed. I moved to Chicago in 2019 to take care of my mom, who just turned 99. I was here for a visit in 2018 and said, "I think mom needs more help than she's telling us." About three months after I got here, I could tell a significant difference in my PH. I went from being on six liters at rest in Colorado and eight to 10 liters of oxygen with activity. Here, in Chicago, I'm at three liters at rest and four liters, five if I'm carrying something, but I've never gone over five since I'm in Chicago. So I definitely can feel the improvement from that perspective. We're still working on which medications would be the best for me, because I've had some really bad reactions to a few of them that other people have had no problems with. That's another big thing with PH and the medications, we all react differently and what works great for one person doesn't necessarily work right for somebody else. So talking to my doctor regularly and seeing him regularly is how we then choose, and I do mean we, because I work with the doctor. I believe I am responsible for my health, to figure out which medicines to try. After I came here to Chicago, the doctors I was seeing here in Chicago were one of the ones that sponsored a PH group, and eventually the nurse practitioner who was running the group had asked me if I would do it. That's how I really became much more involved with the Pulmonary Hypertension Association. As a result, I have become aware of many of the other PH groups that are out there helping. I think that's another thing that's important is to know that there's more than one place. So if one place doesn't seem to fit the bill for somebody, reach out to other ones. If they call the support line for pulmonary hypertension, I know I will even tell them about other ones where they may have more interaction. But I definitely do think that everybody should join a support group. It is a rare disease and we don't always realize just how rare. I know sometimes I've said to my mother, "I'm so involved in it that to me it seems like everybody's got it." Then, the doctor I was seeing in Chicago closed the local practice that was nearby, because they only had five patients, and I had to go out to one of their suburban offices, which I had no problem with. Now, they're closing that one. So they had referred me to Northwestern and that's the doctor I'm now seeing. So he's doing a lot of baseline work again. I know there will be changes coming for me, changes in medication, because there's new ones out there. Many of the medications that we have available today did not even exist when I was diagnosed. My path was probably a little bit different in that I was being treated for cancer. I had a reaction to heparin and develop blood clots. I was in the hospital, they were going to amputate my left arm at the shoulder. They called my family out, because they didn't think I was going to make it through until the morning. Here we are in 2024, that was in 2001. It took a few years and I knew I was having breathing problems, which I have found is pretty common. The pulmonologist I was seeing at the time just kept saying, "Lose weight." That's always the first answer that I think any of us get that are even a little bit overweight. I did lose 40 pounds and my breathing was getting worse. I finally said to my primary doctor, "I don't get it. At 40 pounds, I know I'm not where I'm supposed to be, but shouldn't it at least be the same or if not a little bit better, not worse?" I literally had a hard time moving around in the house even I was breathing so hard. My mother was out for a visit and she was sitting upstairs, I had a split level, and she said she could hear me trying to breathe upstairs from me being downstairs. At that point, my primary doctor is the one who then sent me to the University of Colorado Hospital where they did the right heart catheterization, which is the gold standard for determining if you have pulmonary hypertension. I did the right heart cath, came back and was diagnosed as severe. They can't say for sure exactly what was the cause, because of the time length between when I had the blood clots and when I was finally diagnosed with the pulmonary arterial hypertension. I had some very strong reactions to the medications that they gave me, so it was not unusual when they tried me on a new medication to keep me in the doctor's office until they felt I was stable enough to drive back home. Sometimes that took hours. Thankfully, I have been on some stable medications for some time. Probably my biggest issue has been trying some of the other medications and having some very severe reactions and having to come off of some of them pretty quickly. That's one thing I can identify with along with a lot of other patients and it's that kind of thing that led me to looking into PH more. I didn't do that right at the beginning. I really was trusting and talking with my doctors in Colorado, so I didn't do a lot of looking into support groups and PH, the different groups that are out there. I did that more after I came to Chicago, so that was many years after being diagnosed. At that point I realized just how much those groups could have helped me early on. That's one of the reasons I started volunteering with the Pulmonary Hypertension Association. I also belong to the myPHteam, just trying to reach out to as many people, because I still find to this day the support is very important for me personally. I believe that that's true for anybody that's going through this, because it changes and what medicine you're taking one day you might find you have a response to it that you didn't expect. That's okay, they can change the medications. Staying calm and staying focused and writing down your questions for your doctor are all things that are really critical for getting the health that I need. The number one call we get is looking for assistance in paying for medication. By far, that is the highest number of calls. Come January, we all have to reapply for our grants, because this medication is very expensive for most of it. There are a number of grants out there and when you call the support line, we can tell you which ones are open, which ones are doing funding right now, and in January, like I said, we all have to reapply and it gets really crazy. It takes a while for these different organizations. They have to get their money in, they have to review all of those, so it takes a while and people do get panicky whether they're going to get their funding again. Very honestly, many of us cannot afford to take the medication if we don't get those funds. I actually had the experience one year where my funding did not come through and I was without my medication for two months. It was significant. I mean, I could definitely tell the difference in my health. There were a lot of things I physically could not do while I did not have that medication to help me breathe better. The second most common call that we get is people who have just been diagnosed and are literally panicking because they Google it. It depends on what site you get to when you Google. Most of the sites still will even as of today tell you that the outlook is a max of 10 years once you've been diagnosed, that the mortality rate is still only about 10 years. That is absolutely not true, as I can tell you from having been diagnosed in 2007 and it's now 2024. I am still very active and going. I was talking to someone the other day who is 31 years since they've been diagnosed. One of the things we try to remind people is those are all numbers, and numbers can be spun for different things for different reasons. Don't look at that because none of us have an expiration date on the bottom of our foot. Nobody can tell us when that date is. Do what you can do and work with your doctor and reach out for help and support, because the mental part of this can also be very challenging. I know for myself, there are days, if they've tried me on a new medicine, I'm not having a good reaction, I need to reach out and talk to other people who are living through this and say, "Did you have a problem with it? What kind of things did you do?" Especially if you have the subcutaneous type of medications. It's not unusual to have a lot of soreness around the area where that's attached to your body. Some people have had no problem with it, and some people like myself have had very serious problems with it. So, having people living through it with you, some of them made some really good suggestions for me as to things I could do to try and help with the site pain. Unfortunately, it still didn't work for me. I had an allergic reaction to it, so all of those other things weren't going to help, but that again, is where talking to each other is probably the biggest help. Probably the third type of call then we get is, "Where can I find a PH doctor?" You need to have a pulmonary hypertension specialist, not just a pulmonologist. I have nothing against pulmonologists, I love them, I have one that oversees my sleep apnea. It is a very specialized area, because it involves both your heart and lungs and you have to have somebody who specializes in it. We can help find those doctors for them. We know which sites have been accredited for the pulmonary arterial hypertension, so reaching out again to the support line with the Pulmonary Hypertension Association is just a really great place to know that you're getting the right doctors for the right care. Some of the other groups that I belong to are really good for emotional support questions, but I haven't found any of those that have the same level of information for the accredited centers and that. I am a Googler. I am the first to admit somebody says something I don't know, I'm on Google. I have found that especially when you have a rare disease, get to the association that supports that disease, because they will have information that you are not going to find in other places. They have information that you can trust. It's already been reviewed, certified. It is a great place to start. It doesn't mean it's the only place. I definitely think that phaware and myPHteam are places that are there to help with other things and help give the general support that we need just for living with a rare disease. If you're listening, maybe this is your first time hearing about it, maybe you've heard about it before, but we're not aware that there are many places to reach out to, regardless of how long you've been diagnosed, regardless of what experiences you've had, please reach out, because you can help somebody else and they maybe can help you. This is Sue Liss, and I am aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 487 - Sue Liss
09/23/2024
Episode 487 - Sue Liss
PAH patient and Chicago-area support group leader, Sue Liss, discusses her pulmonary arterial hypertension diagnosis. Sue is involved with multiple PH related advocacy groups. Through these groups, she offers support and guidance, particularly in navigating medication funding challenges and accessing the right care, emphasizing the importance of community and shared experiences in managing this rare disease. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Scott E. Olitsky, MD - phaware® interview 486
09/17/2024
Scott E. Olitsky, MD - phaware® interview 486
In this episode, Dr. Scott Olitsky, the Global Center of Excellence Outreach Director for , discusses hereditary hemorrhagic telangiectasia (HHT), a genetic disease characterized by abnormal blood vessel development. HHT can cause bleeding in various parts of the body, with nosebleeds being the most common symptom. In some cases, HHT can lead to the development of pulmonary arterial hypertension (PAH). Medications that dilate blood vessels can worsen bleeding in HHT patients. Dr. Olitsky shares his personal connection to HHT and PAH and highlights the efforts of Cure HHT to improve diagnosis and treatment options for patients. My name is Dr. Scott Olitsky. I am currently the Global Center of Excellence Outreach Director for , which is the International Foundation for Hereditary Hemorrhagic Telangiectasia. I also happen to be an HHT patient and a caregiver to someone in my family with pulmonary arterial hypertension. HHT is a genetic disease. It's autosomal dominant, which means that statistically 50% of all children born to a parent with HHT will have the disease. It's a disease of abnormal blood vessel development. So normally, arteries connect to veins in the body through a system of capillaries, very fine blood vessels. In HHT, the capillaries are missing, so the arteries connect directly with the veins. Because of this connection, the area where they do connect is prone to bleeding. So we see bleeding in certain sites where these abnormal blood vessels form. Typically, they form on the skin, on the mucosa of the lips, the mouth, the tongue, the nose most notably, the GI tract. Larger tangles of blood vessels can also occur in places like the lung and brain. So because of these abnormalities, we tend to bleed. The most common sign or symptom of HHT is nosebleeds. About 90% of patients have nosebleeds. These nosebleeds can range from anything from a minor inconvenience to being life-threatening. There are two forms of PH that tie into HHT. By far, the most common type occurs when these arteriovenous malformations, AVMs, form in your liver. Because of this, we can have shunting through our liver that shunts blood back to the heart before it has a chance to go to the rest of the body. This overflow of blood back into the heart is also pumped into the lungs. This can lead to secondary changes in the pulmonary artery system, which leads to pulmonary hypertension. That occurs in about 20% of patients with HHT. Far less common is the 1% of HHT patients who develop PAH, precapillary pulmonary hypertension, which behaves just like idiopathic or other forms of hereditable PAH. So as anybody listening this knows PAH is a difficult disease. Some of the drugs have some side effects that are potentially very problematic for patients, but for HHT patients in general, at this point, most but not all of the PAH drugs work by dilating blood vessels. This is problematic in HHT patients, because these blood vessels that are dilated include these abnormal blood vessels that we have. So most patients with PAH related to HHT see a significant increase in their bleeding, whether it be from their nose or very likely their GI tract, which causes iron deficiency and anemia. Of course, anemia on top of an already stressed heart is very problematic for a patient with pulmonary hypertension. By far, the most common problem patients with HHT have are nosebleeds, and they tend to run in families. Now, 90%, as I mentioned, have nosebleeds, but 10% don't. So the fact that a family member with known HHT in the family doesn't have nosebleeds, absolutely does not mean that they don't have HHT. That's very important, because these tangle of blood vessels that occur in the lung or the brain make people susceptible to stroke. We know that if you're properly screened, this risk can be maybe not entirely eliminated, but very close to it. So screening for people with HHT and these developments is very important. The most common issue that families will find is that multiple members of the family have nosebleeds. In many families that is a source of worry, but in HHT patients, we live with nosebleeds every day, multiple times a day. So many HHT patients don't really think of these common nosebleeds as being a problem. They don't think of it as being a potential sign of a disease. So one family member may have more nosebleeds than the other, but they've grown up for generations seeing this. But it's an important point because we know many patients with PAH may be on anticoagulants, they may be on oxygen, which makes their nasal mucosa more prone to bleeding, and so they may have nosebleeds, and that's not uncommon in the PH community. However, in those patients whose family members also experienced nosebleeds, this might be a warning sign that you should be looked at with a thought that HHT may be running in your family. HHT's diagnosis is made clinically, something called the Curacao criteria. There are four criteria. If you meet three of them, you have definite HHT. Two criteria, possible HHT. There's also genetic testing that can be done, which a mutation can be found in about 90 to 95% of patients. So generally what we suggest is that if a patient is concerned about potentially having HHT, they should see somebody who's familiar with the disease and see if a clinical diagnosis can be made. Generally, once the diagnosis is made, or if the diagnosis is possible, genetic testing is done. There are several mutations that cause HHT. There's one in particular which has a higher risk to develop PAH, then the genetic testing can be performed, confirm the diagnosis, also confirm the actual mutation. This is important because that mutation can then be checked for another family. So what we recommend and our international guidelines recommend is that a patient with a clinical diagnosis be tested, the mutation be found, and then all members of that family should be genetically tested for that mutation. HHT does not skip generations. If you have the gene, you have the disease. So it's important to know that. I've been involved with CureHHT for about two decades. I've been on their board for many years. I've known about my own disease since the time I was four or five. Didn't really think much about it. There wasn't a whole lot of treatments when I was younger. When I was in medical school, I remember going through the pathology textbook and seeing this disease and remembering this is what runs in our family. So I've been involved with HHT for a long time. As I mentioned, I have a personal connection to PAH. About 20 years ago, I had a cousin who was running marathons, Ironman races, and then all of a sudden started losing her exercise tolerance, misdiagnosed with exercise-induced asthma. At one point, couldn't walk down a city block. I'm sure a story that's very familiar to many PAH patients searching for that diagnosis. She was given the diagnosis, was given a very grim prognosis at the time, was put on some of the only medications available at the time and has done very, very well. That brought my awareness to what pulmonary arterial hypertension was and how it's tied to HHT. Fast-forward to about six years ago, I have a daughter who was experiencing similar symptoms and was eventually diagnosed as well. So I've seen the delays in diagnosis. I've seen the issues with some of the medications and how they affect people with HHT. At CureHHT, we talk about three goals, find, treat, cure. So we're trying to find patients with HHT so we can help treat them and eventually come up with a cure for the disease. We've been getting some terrific medications on board for patients. We're doing clinical studies to help nosebleeds, bleeding in general. We've set up international guidelines, so we really feel like we're getting places, but still, we estimate only 10% of patients with HHT are diagnosed. My personal connection, of course, comes into play here. I know that there are patients. I've helped identify patients with PAH who have HHT, and as I mentioned, their nosebleeds were thought secondary to their anticoagulants, maybe the oxygen they were on. So in order to help find those patients, help treat those patients and come up with some new therapies for patients with HHT, whether that be the best PAH medication in light of their HHT, or helping to get them on medications to control the bleeding that occurs because of the medications they're on for their PAH. We want to find those patients, and I think it's very important. Again, patients who are experiencing nosebleeds with PAH, don't just chalk it up to your medication or your oxygen therapy. Think about is there somebody in your family who has frequent nosebleeds, GI bleeding, history of an early stroke? These could all be signs that you may be a family member with both HHT and PAH. Thanks for listening. My name is Scott Olitsky, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 486 - Scott E. Olitsky, MD
09/16/2024
Episode 486 - Scott E. Olitsky, MD
In this episode, Dr. Scott Olitsky, the Global Center of Excellence Outreach Director for , discusses hereditary hemorrhagic telangiectasia (HHT), a genetic disease characterized by abnormal blood vessel development. HHT can cause bleeding in various parts of the body, with nosebleeds being the most common symptom. In some cases, HHT can lead to the development of pulmonary arterial hypertension (PAH). Medications that dilate blood vessels can worsen bleeding in HHT patients. Dr. Olitsky shares his personal connection to HHT and PAH and highlights the efforts of Cure HHT to improve diagnosis and treatment options for patients. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Eric Borstein - phaware® interview 485
09/10/2024
Eric Borstein - phaware® interview 485
Eric Borstein, who lives with pulmonary arterial hypertension, is walking from Los Angeles to San Diego to raise funds and awareness for the benefit of Team PHenomenal Hope. On September 21st, 2020, while at home, he collapsed from massive right heart failure and almost died. On September 22nd, 2024, four years after his PH diagnosis, he begin his 120+ mile walk for patients living with pulmonary hypertension. Learn more about his journey and this amazing event at . My name is Eric Borstein. I'm from Brentwood, California, in Los Angeles. I was diagnosed with pulmonary arterial hypertension four years ago this coming September. It was during COVID. I was sick. I wasn't going to the doctor. I have a team of doctors now that I rely on, one of which is a psychologist, because I've learned that you've got the physical battle with PAH, but then you've also got the mental battle. Using a psychologist has helped me get through the hard parts just with relationships and everything else. I was diagnosed in September of 2020. I was sick. I wasn't going to the doctor. My psychologist made me very aware that upwards of 75% of males don't go to the doctor. I got sicker and sicker. I was showing swelling. I was showing swelling in my midsection and my ankles. I'm very active, very athletic. It got to the point where I couldn't walk 20 feet without being out of breath. I couldn't go upstairs in the house without being out of breath. I was hiding. I started wearing bigger clothes. I started working longer hours at the office. I was hiding. It got to the point where I remember one night I was lying in bed with my wife and she said, "I really don't want to be here and wake up with you dead. You really need to get to the doctor." I was scared. I don't know if I was in denial. I wasn't seeking the help that I needed. One morning, I was lying in my home office and I just didn't feel good. I normally would have gone to bed and 12 hours later felt better, but I just wasn't feeling better. I decided to walk upstairs to go back to bed. I got to the top of the steps and everything just started closing in. It was probably the longest 30 steps of my life, most painful steps of my life. I sat down. I stood back up and with my hands at my sides just went face down in the bedroom. One of my kids saw this happen, yelled out to my wife who was downstairs getting our youngest son ready for school on Zoom, like all kids were doing back then. I was unconscious. Paramedics came. I'm assuming it was probably 25 minutes, 30 minutes later. I was dying in the bedroom. They got me to the intensive care where I spent 16 days in the intensive care unit. I was diagnosed with severe right heart failure and pulmonary arterial hypertension. I was stabilized in the hospital. I remember lying in bed in the hospital and looking at my wife straight in the eyes. Back in COVID, there was only one person allowed that was in the hospital. She was there all day as long as she could be until she had to come home and take care of the kids. I remember looking her in the eyes and saying, "I'm going to beat this." I didn't know what that meant at the time, but I knew that wasn't going to be it. I knew I was going to fight, but again, I didn't know what that looked like. I didn't know what that path looked like. Unbeknownst to me, they told my wife that they didn't know if I was going to leave the hospital. I was that sick. It turns out my right ventricle was two and a half times the size of my left. It was severe, right heart failure. I was immediately put on Veletri. Veletri's a really, really, really hard drug. The side effects are awful. I learned that walking started helping with the side effects of the medication. At the time, I only could walk up and down the hallway, but any little bit helped. I was going on 4, 5, 6 walks in the middle of the night. I was stabilized. I left the hospital. When I left the hospital, they told my wife I'd probably have three to six months to live. I came home on Veletri and again, started walking. I started walking five houses down in the neighborhood, 10 houses down in the neighborhood, a block, two blocks, three blocks. Before I knew it, I was walking a mile, I was walking two miles, I was walking three miles. As I was doing this and I'm going to the doctor, I started to feel a little better. Next thing you know, a year later I'm off of Veletri. I was put on three drugs. The side effects are bad. The side effects for these medications are bad, but I kept finding that the walking was helping. It was really helping a lot. It was helping physically and it was helping mentally. My head was clear. Between diet, exercise, medication, doctors, I was religious, and to this day, it's been four years, I've never missed my medication, never missed a doctor's appointment. I've never gone over 1500 milligrams in sodium. I've never gone over my allotment of liquids, because I'm on limited liquids because I have fluid in my heart. I've just gotten better and better and better. I now average 15 miles a day walking. Walking's just become part of my life. My heart is back to normal. My doctors say I have a mid-twenties, fully athletic heart. I'm no longer diagnosable with PAH, but I live with PAH. The doctors say if I walked into their office without biopsying my lungs, you wouldn't know that I had PAH. I understand that I'm extremely fortunate. I'm extremely lucky that I have an amazing family, an amazing support group, amazing friends that I call family. I couldn't have done it without them. I have a business partner who is just absolutely amazing and has taken on a big load. I've been able to dedicate my life to my health and getting better. I'm living proof that between all of these things, there is hope. I remember when I was getting treated, I was getting treated once a week by a nurse who was coming and helping me redress my bandages, because I was on a pump with Veletri. She had another patient that went into the hospital the same time as I did, same diagnosis, same severity, and she died five months later. It was partly attributed to the fact that the side effects were so hard, she just couldn't deal with it. People say I make it look really easy, because I'm out there walking, but it's not. It's hard. The side effects are very difficult. It's not an easy battle, but it's a battle worth fighting, because I've got three kids, 17, 15 and 11. I've got a wife of 19 years, just an amazing family and friends. That's where I am today. We have a family foundation. When I was diagnosed with PAH, we immediately started researching what organizations and hospitals there were around the country that we could get involved with to hopefully make a difference, because we don't know where my future's going to go. We found National Jewish Health in Denver. We started funding PAH research there. I was introduced on my first trip back to NJH, to Dr. Patty George. I met Patty. Her enthusiasm is infectious, to say the least. I sat down with her and was introduced to . Team PHenomenal Hope’s mission, one of their slogans is, "Let me be your lungs," pairing people who can't exercise, who are having trouble getting out of bed, who can't get to the doctor, who don't have the resources for the medications and the therapies and the education, it rang true because I was able to exercise and I'm able to do this stuff. One of the things that I've always wanted to do, and this is another thing that I told my wife and I've told my friends, now that I'm getting better, if I can help save one person's life or if I can help motivate somebody to extend their life, then one day when I'm not here, I've done my job. When I was introduced to Team PHenomenal Hope, it just hit me. They're helping people through the means that I'm actually doing on a daily basis. I told Patty, anything I can do to get involved to help motivate other patients, anything that I can do I want to do. I heard the story of Carl Hicks, how he lost his daughter. It meant so much. I had this idea in my head. I had this concept in my head, why not walk somewhere to raise awareness? Through conversations, I decided that whatever fundraiser I did, I was going to do it for Team PHenomenal Hope. I want to be a motivational speaker for them. I want to do events for them, not only on the philanthropic side, but because I'm a patient because patients can look at me for hope. In 2022, I walked over 4,700 miles. In 2023, I walked over 5,000 miles. Between the two years, I walked over 10,700 miles. Actually people in the neighborhood, they see me once, twice, three times a day walking. Sometimes they'll see me in the neighborhood. Then they'll see me down in the marina or they'll see me in Culver City or they'll see me by LAX. They'll see me all over the place a couple times a day. I've been now nicknamed, The Walking Guy. We were actually just in Europe this summer. We were in Greece. We were at breakfast, and a mother and a daughter walked up to us and she looks at me and she says, "I just have to ask, are you from Los Angeles?" I said, "Yes." She goes, "Are you from Brentwood?" I said, "Yes." She said, "Do you live off of so-and-so road?" I said, "Yes." She grabs her daughter by the arm and says, "Oh my God, it's The Walking Guy." This was in Mykonos, Greece! This has happened all throughout West Los Angeles. I'm with my kids and people do this. So I decided I was going to walk from Los Angeles to San Diego to raise money and awareness for pulmonary hypertension. I am raising the money for the benefit of Team PHenomenal Hope. We're going to put on an event the morning of September 22nd in Santa Monica, along the bike path. It's going to be a 5K walk. We're going to have some speakers. Then, we're going to go out and do our 5K walk. Then, they're going to send me on my way at the end of the event, and I'm going to start walking to San Diego with the hopes of raising as much money and awareness as we can for a disease with no cure. I'm so, so excited for it. It's the first annual. It's called, " The plan is for me to walk from LA to San Diego averaging about 25 to 30 miles a day. I'm breaking it up into segments. I'll walk 25 miles, sleep, walk 25 miles, sleep all the way down the coast. I'm going to stay as close to the coast as possible, with the goal of ending in San Diego five or six days later. My kids are in school. The plan is for me to end in San Diego on Friday or Saturday so that they don't have to miss school. They can come down. They can be at the finish line. I'm going to have other friends and family down there welcoming me at the finish line. Hopefully, they'll be nice enough to give me a ride home after the amazing adventure that I've just gone on! To learn more about this amazing event and my walk for awareness for PAH, please go to or . We would love your support, love for you to come out and be a part of this amazing day, this amazing morning and really kick this off for those living with pulmonary hypertension that can benefit from Team PHenomenal Hope. I hope to see you all there. My name is Eric Borstein, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
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Episode 485 - Eric Borstein
09/09/2024
Episode 485 - Eric Borstein
Eric Borstein, who lives with pulmonary arterial hypertension, is walking from Los Angeles to San Diego to raise funds and awareness for the benefit of Team PHenomenal Hope. On September 21st, 2020, while at home, he collapsed from massive right heart failure and almost died. On September 22nd, 2024, four years after his PH diagnosis, he begin his 120+ mile walk for patients living with pulmonary hypertension. Learn more about his journey and this amazing event at . Learn more about pulmonary hypertension trials at . Follow us on social @phaware Engage for a cure: #phaware Share your story:
/episode/index/show/phawarepodcast/id/32337022