I'm Aware That I'm Rare: the phaware® podcast
A new podcast series devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. New Episodes every Monday & Thursday. 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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Carys and Paisley Dempsey - phaware® interview 309
02/21/2020
Carys and Paisley Dempsey - phaware® interview 309
Sisters Paisley Dempsey (10) and Carys Dempsey (11) discuss their mother Nicole’s PH diagnosis and their roles as caregivers. Nicole served as a PHA Canada Ambassador from 2014-16, before being elected to the Board of Directors in 2017. She was diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 2013. Prior to her diagnosis, Nicole worked as an elementary school teacher for 12 years. The Dempsey family resides in Cambridge, Ontario. Paisley: My name is Paisley, I'm 10 years old. Carys: My name is Carys, I'm 11 years old. Our mom has pulmonary hypertension and we're from Ontario, Canada. Our mom [Nicole Dempsey], she started to feel really sick. She was starting to become unable to do things. One time, it got really bad and [our parents] had to go to the doctor and she got diagnosed with PH. We didn't really know what was going on, because we were really young and they explained it to us. We still didn't really understand, but knew something was wrong. Paisley: Mom had oxygen, so it was kind of hard for her to do things so dad had to help her. It was kind of scary, because it was really hard. Carys: At first, it was very different and it was just becoming a bit more like our life. Now, it's just a normal thing. It's normal now... Paisley: We know that we're going to live like this. Carys: We tell our friends, but... Paisley: They don't quite understand. Carys: I think they don't understand as much as we do, because it's not part of their life. When we go out, sometimes people give us looks but it doesn't really bother us. It's like we don't care. Paisley: We see it a lot. Carys: Well, we don't see it a lot, [but like] with mom's parking pass... the disability pass, sometimes we'll park in the front and sometimes people would be like, "Well you don't need that." It's rare, but it happens here and there. It gets us upset sometimes when she's not feeling extremely well and it can get upsetting for us, because you know, we want her to be healthy and be okay. So, it can be upsetting and get us angry sometimes, because we don't like it. Paisley: Not angry at her. Carys: Angry at the illness. Paisley: We help bring groceries in and heavy stuff... Carys: Heavier things that she may not be able to carry, because it maybe gets her out of breath. Paisley: We help do the dishes... Carys: Because bending can be hard. So, we do the lower rack of the dishes and the top. We feed the dogs and help with the dogs when they're getting too crazy or anything that's hard for her. Advice that I would have for someone who just walked out of a clinic with PH or [to] a caregiver, I'd probably say that, you know, it's going to be okay. Paisley: You're going to have to help though a little more than you usually do. Carys: It'll just kind of adjust to your lifestyle and it'll just become more of a normal thing in your life and it won't become one of those sore thumbs. It's a pain but, you know, it won't be as difficult. PHA Canada wanted me to draw a shirt for PH [awareness] with lungs. So, I decided to do it and I drew lungs. On one side, there were flowers and then on the other there were a little hearts with blood vessels and positive words. I did that so that other people could wear it and feel happy that other people know about it. The shirt was made and now a lot of people wear it on PH [Awareness] days. We feel it's important for our family to be involved, because it can help other PH patients feel that they're not alone. Other people are struggling with it, because you know, some people feel really sad about it. [Also] it helps PH patients maybe feel better about themselves so that they don't just think that it's something that they're just living with. We just live like it's a normal life, you know, not like anything happened. [We] just try to... Paisley: Try to avoid it. Carys: We don't let it control us. We don't let it get to us all the time. Paisley: We try not to think about it a lot. Carys: It's not really acting. It's because it's just becoming a normal thing. Our mom was a teacher, but because she got sick, she can no longer teach anymore. Paisley: Because she got a little too sick. Carys: What's a good part is she has lots more time for us. She can pick us up from school and drop us off and you know, lots more time to do things on the weekends and weekdays and spend time together. So, that's the silver lining of her being like sick. Paisley: My name is Paisley. Carys: And my name is Carys. Both: And we're aware that our mom is rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 309 - Carys and Paisley Dempsey
02/20/2020
Episode 309 - Carys and Paisley Dempsey
Sisters Paisley Dempsey (10) and Carys Dempsey (11) discuss their mother Nicole’s PH diagnosis and their roles as caregivers. Nicole served as a PHA Canada Ambassador from 2014-16, before being elected to the Board of Directors in 2017. She was diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 2013. Prior to her diagnosis, Nicole worked as an elementary school teacher for 12 years. The Dempsey family resides in Cambridge, Ontario. #phaware
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Alex Cowie - phaware® interview 308
02/18/2020
Alex Cowie - phaware® interview 308
Alex Cowie lives in the South West Coast of Scotland, UK with his partner Jo. Alex was diagnosed with Hereditary Pulmonary Arterial Hypertension in August 2016. He has participated in 4 clinical trials to date. Alex's daughter, Erika, lost her battle with PH in 2016 following her 18th birthday. My name's Alex Cowie and I'm in Girvan, a little town on the west coast of Scotland. We moved here a year and a half ago just due to life circumstance, we needed a fresh start and a new beginning. Primary one of the reasons was because my introduction to pulmonary arterial hypertension or PH, was when my daughter Erica was diagnosed when she was 11 years old. So, PH was an unknown disease condition I'd never heard of. So, obviously Erica was young, she was diagnosed when she was 11, but there was a lot of toing and froing to doctors saying "Oh, it's just asthma, teenager", that kind of thing. It didn't really go any further until she actually collapsed on her eleventh birthday going round to her friends. She wasn't very well but she carried on. She wanted to go out with her friends because they were going for a nice meal that early evening. So, it's all snowballed from there. She spent a time in hospital. Eventually it got to a point that they thought it was a hole in her heart, but she actually ended up staying in a week in a hospital where I was living, which was in south of England at the time. She was then sent to another hospital in Bristol. Bristol Children's Hospital. They did further tests and stuff. Then they sent her to Great Ormond Street in London to have further tests to see what was exactly going on. That's where they gave her a right heart catheterization. That's when she was diagnosed as severe pulmonary arterial hypertension. Then, she was classed as idiopathic, because she did do genetic tests and everything else. That was my first introduction of what PH was. I never had even heard of it before. It was an unknown disease to me. You hear a lot of things about cancer, cancer this, cancer that. To be told about pulmonary arterial hypertension and how severe my daughter was, it was quite scary because they gave her two years to live. They sent her home with loads of drugs, tablets and she used a, iloprost inhaler four, five times a day. She did that for a year and then she had to go onto a Hickman line, a CADD pump. That was a major change for a young teenage girl going into womanhood, changes to body and stuff like that. It was scary for her and it was scary for all of us, because we were like "What the hell", this disease, this condition, how severe it is and how critical it is. Then, she just battled on with the PH doing different genetic tests and trips to hospitals. Then from leaving Great Ormond Street to actually going to adult services, she was still on the Hickman line with intravenous [medication]. She had lots of severe symptoms that you get with being on the Hickman line. One of the things that she had was a rash. As a young woman, it changed her life. She was very confident. She got a job and she was working and she did a lot at school and stuff like that. So she was a very, very inspirational young lady and then she saw her 18th birthday and then, unfortunately, she passed away in the 26th of September in 2016. Just six weeks after celebrating her 18th birthday. So that was my introduction to PH. But prior to Erica getting really sick and losing her battle with PH and pneumonia, I was sort of having some dizzy spells. I'd probably had a chest infection the back end of 2015, after that Christmas period in 2016. I sort of started getting dizzy spells, postural hypertension. When I stand up I'd get a little bit dizzy or if I bend down I get a little bit dizzy. So I went to my doctor and said, "Oh, you know, this is what's occurring, I am getting these dizzy spells". [He] did the normal stuff, blood pressure and all that and said, "No, can't find anything wrong, it's just postural hypertension." Bearing in mind, they knew the history of my daughter having pulmonary arterial hypertension. So this carried on for a couple of months. I used to walk up three flights of stairs and run up them, go mountain biking and I was physically active. All of a sudden, I started to get short of breath when I've climbed three flights of stairs or when I was out mountain biking. I'd hit a hill and I'd be puffing and blowing. I was thinking, "Oh here's me. Really unfit now." Because I'd always been fit and active all my life because I was in the Army, I enjoyed running a marathon, always active, playing football or as you call it, soccer and that kind of stuff. So I was always active and all of a sudden I was blowing like I'd run three marathons. I was really struggling. Then the dizzy spells continued to happen and I went back to the doctor's suddenly, these dizzy spells are becoming more frequent and that it's not like a passing thing. I'm standing and I've got to stand still or sit down for a good 20 minutes just for them to pass and I get me bearings again, because you know what I didn't want to do is end up collapsing because I'm six foot one and I'm a big chap. So anyway I went to the doctors and that again and he said we'll do an ECG and done that. Nothing came back on the ECG and he said what we'll do, we'll send you to the hospital, get an echocardiogram, see what's going on. I was all familiar with all this kind of stuff from Erica and her having all these tests and continued to have all these tests when she'd go to her PH clinic appointments. So, I was like "Oh, bit scary and that" and wondered what was going on. Maybe it was just an age thing or had something a bit more sinister going on. So we celebrated Erica 18th birthday in end of July. Then, a couple of weeks after that I was going to go on annual leave from work. A couple of weeks off, go away, catch up with friends and family and stuff like that. Then one evening, oh you went upstairs to pick up the cat litter trays, just so I could empty them and clean them out, so they had fresh cat litter. As I was walking down the stairs, I sort of got a bit dizzy. What I did was stand there holding the two cat litter trays and holding them and normally waiting for the dizzy spell to pass. Unfortunately, the dizzy spell didn't pass and I woke up in a heap in the bottom of the stairs, covered in cat litter thinking what the hell happened and realized that I passed out. Luckily, I hadn't broken anything, maybe a few little bumps and bruises. What I did is I cleaned up the cat litter and then I text my other half, said "Look, I'm not feeling very well, I've just passed out. I'm going to go to bed and sleep it off and arrange to see the doctor in the morning." She texted me back saying, "No, you're bloody not. You're going to go to A&E and get checked out." So, I went to A&E, got checked out and they just did some normal tests, blood pressure, stuff like that. I said it was an ongoing thing, because it was postural hypertension. They said, "Okay, well what we'll do is we'll just do a quick ECG to make sure there's nothing sinister going on and then we'll let you go home". So they did the ECG and it came back a little bit abnormal. They said, "Oh, unfortunately going to have to keep you in. Not sure why, but there's something going on. So, we'd like to do some further tests." I went, “okay.” So I told my partner Jo and let my daughter know that I was being admitted to the hospital and they all come and saw me the next day. I was in there for about three or four days waiting for further tests. I had an echocardiogram, lots of ECGs, lots of blood [tests], and then they said, "Oh, well what we're going to do is we're going to refer you to some further tests, because of the history of Erica having idiopathic pulmonary arterial hypertension." They just wanted to do run a few more tests. They came back and they said, "Ah, actually you've got pulmonary arterial hypertension at the age of 46.” They classed mine as hereditary pulmonary arterial hypertension, because my daughter had it. Prior to that there was no symptoms or link between us having pulmonary arterial hypertension. That was in August the 18th, 2016 I was diagnosed. Funny how you remember dates. Then six weeks after me being diagnosed, Erica lost her battle with pulmonary hypertension. Looking back now, it's like not really having that revelation of this could be PH. I think it was because you know, I've always been fit and active. I was in the Army for eight years and there was no sign of PH for 46 years in my body. How crazy is that? I've got this rare disease, but it didn't manifest itself for 46 odd years. That was the head thing I think that I struggled with a little bit to begin with. But because it was so rare, it was like, oh, the doctors, which weren't sort of saying, "Oh, I've got it". They weren't sure. There's obviously thinking maybe I might be PH, but because what is the statistics? 1 in a million people get PH? So my daughter had PH, so she was that one in that million. I was the second 1 in that million to get PH. So, that was a bit of a strange thing to happen that all of a sudden this disease manifested itself within me. But when I was diagnosed, it was like, okay, I've got PH and Erica was with me with Jo, my partner, but before prior to him actually telling me that we had it, and they'd come into the room to say, Erica turned around and said to me "Dad, wouldn't it be funny if he got diagnosed with PH?" We both laughed because it's such a rare disease. We both had a good laugh about that. Then 10, 15 minutes later the doctor come in, Dr. Jay and he said, "Mr. Cowie, I'm sorry to you've say you've got pulmonary arterial hypertension". The initial reaction was me and my daughter just laughed and went, "Okay, it's not the end of the world. We'll just fight it together." We've got up this united front, me and her to fight this disease and do as much as we can to have a normal life, if that makes sense. Now, I think I've got this disease, but the disease doesn't define me as a person. It's just I've got this disease and it's challenging. It does make your life limited, but you can work around it and that's what I found over these last few years. It doesn't define me as a person. I know I've got it, so I have to do things differently and do things that I can do without falling back into the trap saying "I used to do this, I used to do that" but that's all in the past. I can't change the past now, I can only do what's in front of me. That's I think a good thing in a way for me. Focusing on the now and not looking back. I think when I first was diagnosed it's like ah, can't do this, can't do that anymore because I've been knackered and plus it could kill me. I can't go mountain biking up in mountains and hills like I used to. I've ridden my mountain bike on the flat, but it's not the same. You don't get that sort of buzz, but also it was a lot harder, right? You know, mountain biking on the flat with PH than not having PH, if that makes sense. The reason I got involved in clinical trials, I think it's because my daughter. There isn't a cure as such for PH, it's only drugs that can stabilize it or slow it down. My daughter never had that opportunity to survive PH or have drugs that enabled her condition to improve or for her to improve. I think for me, because it's such a rare disease and there's not many people with PH, is that the more clinical trials that I can be involved in, the more data that may help somebody else's daughter or somebody else's son or just somebody further along the line when they get diagnosed with PH. Also, if I do a trial it might also benefit me. Which is a win/win. A win for me and a win for the study into PH in new medication. I think that's what's important to me is to give people the opportunity to use me as a guinea pig, to see what works for PH but also what would work for somebody else. I think that sort of strive because of my daughter, because she didn't have the opportunity to try any clinical trials and it's a horrible disease, especially somebody young like my daughter. I've seen some children younger than when Erica was diagnosed and it is a hard life for them because they seem to be different but they're not different. They're just people like everybody else. I think the only thing I can think of really as parting wisdom is you may have this disease, the person you're caring for may have this disease, but it's not the end of the world. It shouldn't define you and life is to be lived whether you've got PH or you haven't got PH. I guess you've got to live your life and I think family's important. Spend time with those that you love and care about and want to be with, friends, family. That's important. I think we get caught up in the world too much of looking for money or trying to have that normal life. When actually, the closest things that are more important to you are you, your family and making those memories with those people I think. I think the biggest thing you can do is go and enjoy your life and live it. Live it to the full. Live it to the max, whether you have PH or not. I think it's just about enjoying it and making those memories with people. So when you're not here, they have those memories of you just like, I have these memories of my daughter, if that makes sense, you know. My name is Alex Cowie and I know, I'm aware that I'm rare. Also, I'm a PH fighter, so there you go. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 308 - Alex Cowie
02/17/2020
Episode 308 - Alex Cowie
Alex Cowie lives in the South West Coast of Scotland, UK with his partner Jo. Alex was diagnosed with Hereditary Pulmonary Arterial Hypertension in August 2016. He has participated in 4 clinical trials to date. Alex's daughter, Erika, lost her battle with PH in 2016. Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: www.phaware.global/donate #phaware #ClinicalTrials
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Cheryl Wegener - phaware® interview 307
01/31/2020
Cheryl Wegener - phaware® interview 307
We are heartbroken that our friend, Madison Wegener, lost her battle with PH on January 19, 2020. In this episode, Madison 's mother, Cheryl discusses the importance of early diagnosis, the impact PH has on the entire family how journaling became a teaching tool for everyone in their lives. My name is Cheryl Wegener and I'm a pulmonary hypertension caregiver. In my opinion, pulmonary hypertension awareness is critical because it means so much to early diagnosis for people like my daughter and others like her. So many times people go so long with misdiagnosis, symptoms that go unchecked, that sort of thing, and so being aware of that just ultimately will save lives. Day one into our diagnosis, I wish I would've had a better understanding of the disease and what its implications meant for not only my daughter, but for myself, for my son, for our entire family. So tips, as far as going forward, I would definitely say you absolutely have to find a support network, whether it's an online support network, talking with other moms, other parents, other caregivers, friends that are local that get that you might be having a rough time that are there to help you. Absolutely, support is critical. I wish that the doctor would have approached it more holistically towards our family as opposed to just simply focusing on Madison. That sounds kind of backwards, but making us aware that this is not just going to be her disease, this is going to be your family's disease and this is going to impact every single one of you. And [to] be aware of that and be tuned into that and be self-reflective in that and watch that out with little brother, that sort of thing. I wish we would've had more knowledge and more awareness of that from the beginning. We have definitely been on a roller coaster on the six years that we've been in the PH club. Sometimes we've responded to it very well and very positively and sometimes our family members, myself included, have not responded to it very well. I think acknowledging that you're human and that you're going to have good days, you're going to have bad days, you're going to have positive I can do this moments and you can have other days where you need somebody else to tell you that you can do this moments. We've experienced a lot. I've experienced a lot with Madison and watching some social struggles with her at school. I've experienced some struggles in my own relationship dealing with stress, anxiety, depression, and little brother has also experienced a lot of anxiety and depression that I had no idea to look for from the beginning. My husband and I, from day one of diagnosis, we kind of started journaling about it and that became an open book for us. Our CaringBridge journal became very much a teaching tool for everybody in our lives. We also participate in PH fundraising walks. They expect us to come and to perform well each year. We have a team that's well known now at our local walk. I teach high school and I'm very open with my students about the fact that you just never know and life is short, and you need to seize the day and you need to appreciate what you have. All of those messages come out, and I'm very open about my own struggles and very open with my daughter's struggles. They are very much #phaware by the time they leave my classroom as well. My name is Cheryl Wegener, and I'm aware that I'm rare. Please join the Wegener Family for "" on May 16 in Dewitt, MI to honor Madison's legacy and making Madison's dream come true by ending the suffering of these PH kids and finding a cure. You can also read more about Madison on her page. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 307 - Cheryl Wegener
01/30/2020
Episode 307 - Cheryl Wegener
Madison Wegener, lost her battle with PH on January 19, 2020. In this episode, Madison 's mother, Cheryl discusses the importance of early diagnosis, the impact PH has on the family how journaling became a teaching tool for everyone in their lives.
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Madison Wegener - phaware® interview 306
01/28/2020
Madison Wegener - phaware® interview 306
We are heartbroken that our friend, Madison Wegener, lost her battle with PH on January 19, 2020. In this episode, Madison discusses the importance of awareness, research and why she loves PH Conference. My name is Madison Wegener, I'm a pulmonary hypertension patient. I think it's really important to make people aware so that way it's more fundraising because you kind of need the money for that, because it is kind of a lot of money and it's a hard thing to deal with. I found it kind of made me a bit stronger. I don't know, more sensitive kind of. I'd kind of say, find a support group. Find friends. It's definitely a lot more helpful. Try and go to conference if you can. I remember my first time here, it was so nice seeing someone else with it because like I said, it is a hard thing to deal with so you kind of need someone there. You have your parents but then they're also dealing with it and it's kind of nice to have someone who knows what you're going through. It's kind of easier not to be mean or anything, but they understand it in [that] you're restricting yourself. Your cousins might want to be like, "Hey, let's go run." I remember in elementary it was really hard for me, because everyone would play tag and everything and I couldn't do that. Maybe someone else who had it, they're slower and they're more my pace. That's why I love conference so much, because it's only, it's barely that much if you think about it and it's to just see your friends that much. That's not a lot. Not everyone can always come. Timing doesn't always work and stuff. Sometimes you don't even get to see them so anytime you can you want to. I remember when I was diagnosed I was really into gymnastics and I did that with my other friends. But since I got a pump and everything that threw me off balance so that was kind of off the list. It kind of forced me into learn a lot of other things. I like the woods, my grandparents own a property and we go up there a lot and do maple syrup and stuff. I've started archery, horseback riding. I haven't really settled into anything yet. I'm still kind of exploring places, but it's definitely made me more, I guess, adventurous you could say. I learned how to change my own dressing. I've tried learning how to mix my medicine, but it was over the school year and that's kind of a lot at night. I only know how to change my dressing. I know the name of some of my pills. I should probably be better at that, but I'm not. I'm working on it though. My main tip would be trying to tell people and make them understand and be like, "Hey, I really can't do this." Your friends, sometimes they understand, sometimes they don't. When you get older they do definitely. And just try and get to conference, try and make friends that do have PH because it's definitely helpful. My name is Madison Wegener and I'm aware of that I'm aware. Please join the Wegener Family for "" on May 16 in Dewitt, MI to honor Madison's legacy and making Madison's dream come true by ending the suffering of these PH kids and finding a cure. You can also read more about Madison on her page. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 306 - Madison Wegener
01/27/2020
Episode 306 - Madison Wegener
We are heartbroken that our friend, Madison Wegener, lost her battle with PH on January 19, 2020. In this episode, Madison discusses the importance of awareness, research and why she loves PH Conference.
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Harold Palevsky, MD - phaware® interview 305
12/06/2019
Harold Palevsky, MD - phaware® interview 305
Harold I. Palevsky, MD is a Professor of Medicine at the Perelman School of Medicine at the University of Pennsylvania and the Chief of Pulmonary, Allergy and Critical Care Medicine at the Penn Presbyterian Medical Center in Philadelphia. He also heads the Pulmonary Vascular Disease Program at that campus. For the past 25 years, he has been recognized as one of Philadelphia Magazine’s and America’s Top doctors. In this episode, Dr. Palevsky discusses the importance of clinical trials in patients with pulmonary arterial hypertension including a new molecule that's on the forefront of treatment for PAH called PB1046 and the positive effects it could have for the community. My name is Harold Palevsky. I'm a pulmonary critical care physician, a professor of medicine at the University of Pennsylvania, where we have a large pulmonary hypertension associated comprehensive care center that has eight physicians working in it, as well as advanced practice nurses and research coordinators and research staff. We have a lot of people who are focusing their time and their academic careers on pulmonary hypertension and pulmonary vascular diseases. I've been caring for patients with pulmonary hypertension since the time of the NIH Primary Pulmonary Hypertension Registry, which was in the mid 1980s. At the time of that registry, as a senior fellow, I was the individual taking care of the patients with primary pulmonary hypertension who were being seen at our center and entered into that registry. That was a very gratifying experience, although at that time we really didn't have any therapies for pulmonary vascular disease. We were trying to understand the disease and its natural history. The particular interest in pulmonary vascular disease was as a consequence of an epidemic of primary pulmonary hypertension seen in Austria, Switzerland, and Germany; and ultimately determined to be the result of a diet medication that was sold in those three countries. That epidemic provided the impetus for the first World Health Organization Conference on Pulmonary Hypertension, which was actually held in 1973. That led to the NIH Primary Pulmonary Hypertension Registry. I've been involved in the care of patients with pulmonary vascular disease since that time and have seen the landscape change to the point where we now have 14 approved medications, basically in three biologic families that we use for the treatment of pulmonary hypertension. So that's the good news. The disappointing component of our care of patients with pulmonary vascular disease is that we haven't had any medications in new families - medications working by different pathways - approved for the last 14 years. All of the current medications are in the same three families, the prostacyclin family, the nitric oxide pathway family, and the endothelin pathway. We have multiple medications in the three pathways and drugs that can be used in combination. And in least currently in the prostacyclin family, we have drugs that can be given orally, intravenously, subcutaneously, and by inhalation. All of these have allowed us to improve the effectiveness of our therapies over time, but we're not curing pulmonary hypertension. We are often arresting it, preventing it from progressing, but we'd like to have more options, so that we can use and choose the best drug or combination for an individual rather than our current approach, which is very homogeneous. We tend to treat patients in a very similar manner based on their functional class and functional capacity rather than on individual characterizations of their pulmonary hypertension and its causes and precipitants. So, the fact that we now have a number of drugs in development, in early phase clinical trials, moving through the process by which we develop medications that represent new pathways and new mechanisms for us is very exciting. We can't predict which of these drugs are going to ultimately prove successful and come to regulatory approval, so that we have them at hand to treat patients. Having drugs that represent new methods of treating pulmonary hypertension, impacting on different biologic processes and biologic pathways, at least in some of these cases, using new mechanisms for drug administration represents a step forward and a very exciting time in our approach to pulmonary hypertension. One of the directions of ongoing research is trying to determine how we assess each individual patient to be able to give them the best therapy for them and how we decide among the available choices - what that might be, trying to figure out how to individualize therapy within the options we have. Beyond that, there are exciting developments in the fact that there are several agents under development in various stages of clinical trials that represent totally new pathways for treatment and may be particularly important in a given individual. That's why part of what is being done in the current clinical trials are obtaining blood specimens, banking specimens. So, at the end of the period of study, when we can assess who responded very well to a specific agent, a new drug and who didn't respond as well,we can go back and look at their disease, how we assess it clinically, how we assess it functionally and how we assess it biologically by looking at some of the blood work and other things we may be able to monitor. So, that we can then try to distinguish who is likely to be a responder, who's not as likely to benefit, so that we can decide what order and when we try individual therapies in an individual patient is as informed and sensible a decision as we can make it. Progress in understanding pulmonary hypertension and in developing more effective therapies relies on clinical trials. We have a basis in our understanding of biology and in what has been learned from animal models and animal experiments as to what pathways may be important in pulmonary hypertension. But when a medication is developed that appears to work in one of those pathways, we need to go through the sequence of studies that is usually done in the development of a medication to determine if it's going to be as useful as our hope based on the biology. The three phases of study are basically, first, making sure that a proposed medication is safe. Then, trying to determine what the appropriate dose of a medication will be. Then, studying it in what's called a Phase III blinded study; blinded such that the clinician and the investigator doesn't know whether the individual is getting the active drug or placebo on top of their background therapy. So, that the assessments we're making in terms of interpreting symptoms, interpreting side effects, functional status, all of those sorts of things, that we are doing them in a manner where our hopes and preconceptions don't influence the results and therefore the data that we're generating from the clinical trial. That's why Phase I trials are not really blinded. They're to prove that the medication is safe, and we generally use increasing doses in very carefully monitored settings to make sure it's safe. And then in the next phase of study, we know we're giving the active agent to individuals. We want to figure out what the appropriate dose may be to get the effect we want, not to give more than we need, but also not to give less than we need. Then comes the randomized, controlled, blinded studies [that] we're often asking our patients to participate in, so that we can really decide whether or not this proposed medication is really safe and effective. Does it really improve how our patients are doing? We can [then] determine whether or not this is really a realistic medication. In terms of pulmonary hypertension, the first drugs were studied with an endpoint of the clinical trials being an improvement in the six-minute walk after three or four months of therapy. The more recent trials have used more important long-term outcomes as their endpoints, and in particular, whether or not we prevent the pulmonary hypertension from progressing, whether or not our therapy keeps people from needing hospitalization or needing additional therapies added to what they're taking. Those are called event-based trials. We're hoping that by identifying earlier changes that might be occurring in response to therapies, that we can shorten the duration of some of the event-based trials. So, that it just doesn't take as long to develop these new medications as sometimes has been happening. There are clear challenges in the development of new medications. We currently know that we have effective medications. The clinical studies that are done, are done on top of a background of medication. So, it's not like when we did the studies of the first therapies for pulmonary hypertension where for a period of time patients either got the active drug or a placebo and nothing else. That's not appropriate. It's not ethical at this point in time, where we have approved therapies that we know benefit our patients. So, new agents are studied on top of standard-of-care background therapy. We then evaluate, in a blinded manner, whether those interventions, the new therapy, make a difference on top of our backgrounds and standard therapy. It's a little harder to evaluate, because hopefully individuals are responding to their background therapy. We really have to take a longer period of time or evaluate our patients to make sure that these new therapies are adding something to the approved agents that we currently have. We are also involved as a field in improving the families of medications that we have, seeing if we can make them easier for the patients to take. If we can advance the drugs within the family, so we have fewer side effects and fewer problems. We'd like our therapies to be relatively convenient and very well tolerated and effective. I wouldn't like to take a medication four times a day, if we can develop a related and as effective medication that can be taken twice a day. So, there are quality-of-life benefits from improving the therapies we have now and making them more convenient and better tolerated. So, that's also an appropriate direction for our research to be taking. Not only developing and looking at new drugs and new pathways, but also seeing if we could make our current therapies more convenient and easier to tolerate and just as effective or more effective within the families that we have. So, having a drug within a family doesn't mean we're going to stop looking at how we can make therapy better. But we have somewhat different goals in terms of research within a pathway and family of drugs that we already have approved agents in, and when we're looking at novel pathways and the first drug within that family or that pathway. One of the pathways that is currently under investigation as a potential new pathway for therapy in pulmonary arterial hypertension is the VIP pathway. VIP stands for vasoactive intestinal peptide. It was shown some time ago that patients with pulmonary hypertension have reduced levels of this compound, VIP, circulating in their body. Initial efforts to develop a therapy within this pathway involved inhaled dosing of the VIP. That didn't prove to be as successful as was hoped. What's now being studied, is a really fascinating investigational drug where VIP is bound to a polypeptide, a long protein. This peptide has the property of becoming a gel when it gets warmed to body temperature. So, this medication, which is a liquid, is injected subcutaneously. That means under the skin. It then forms a gel, and the VIP leeches out of this gel into the circulation. We measure the biologic availability of drugs in something called the half-life. That's really how long the drug is lasting in an individual. When VIP was given by inhalation, one of the problems is that the half-life of the drug is really quite short. It's measured in minutes. When VIP is given by this new technology, as a subcutaneous injection, the half-life is on the order of two and a half days. That's dramatically different. It allows for this version of VIP to be administered by a subcutaneous injection once a week. Now, none of us really relishes the idea of giving ourselves subcutaneous injections, but it's something that's done frequently in medicine. It's done with a lot of hormones. It's clearly the method of administration for insulin. It's how we give some anticoagulants, blood thinners, so it's something that is easy to teach patients. It's something that we can all come to accept if we're giving ourselves a medication which is improving our outcome from pulmonary arterial hypertension. An injection once a week is really a minor inconvenience to allow us to add another family of medications, another pathway that we can impact in our treatment of pulmonary arterial hypertension. This agent is now in clinical trials. The clinical trials, as I said before, go through the three phases. It's been proven to be safe. Now, we're trying to learn something about its efficacy, its effectiveness, and what the optimal dosing is for individuals. That Phase II study is currently ongoing. Until we get the results, we won't know more about whether or not this is going to prove to be effective for treatment in pulmonary arterial hypertension. But it's exciting because first, it represents a pathway which we know is of biologic relevance in pulmonary arterial hypertension, that patients have reduced levels of VIP circulating. Second of all, it represents a new mechanism for drug delivery with the once weekly administration of this polypeptide bound to the VIP, which allows it to be in the body and released in the body in a manner such that a drug which has a half-life of minutes is converted to a drug that has a half-life of days. The compound under investigation has a corporate nomenclature until the drug is done with its clinical testing, is approved and is going to be available, at which time it actually gets a name. The compound I described is called PB1046. If you look at clinicaltrials.gov or somewhere else where you might want to get a little more information about a drug under development, many of the drugs that are being worked on and investigated for pulmonary hypertension have alphanumeric names rather than common names or brand names. In this case, the PB in PB1046 stands for the pharmaceutical company that is developing this agent. That's a biopharmaceutical company called . I'll close by just saying that the field of pulmonary hypertension has evolved dramatically over my career, from starting at a time when we had no specific therapies for pulmonary hypertension to now when we have 14 approved drugs in the three families that we've talked about and many other new agents under development. If you are interested in finding out more information about the research that's ongoing, you can look for information at . You can look for information through . And importantly, you can talk to your PH physician, because they may have specific information on how any of these agents or any of these clinical trials is of specific relevance to the unique nature of your pulmonary hypertension and pulmonary vascular disease. My name is Dr. Harold Palevsky, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials #phawareMD
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Episode 305 - Harold Palevsky, MD
12/05/2019
Episode 305 - Harold Palevsky, MD
Dr. Harold Palevsky discusses the importance of clinical trials in patients with pulmonary arterial hypertension including a new molecule that's on the forefront of treatment for PAH called PB1046 and the positive effects it could have for the community.
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Dale Dedrick - phaware® interview 304
12/03/2019
Dale Dedrick - phaware® interview 304
Dale Dedrick is a retired orthopedic surgeon who was diagnosed with lupus and pulmonary hypertension. In this episode, Dale discusses how her illness forced her to retire and how her love of horseback riding helped her make the United States Paralympic Equestrian Team in 2012. My name is Dale Dedrick and I live in Chelsea, Michigan, which is a small community not too far from Detroit. I was diagnosed with pulmonary hypertension about two years ago. I'm a physician by training, but I have lupus. Because of all the problems with the lupus, I haven't practiced medicine since 1992. I've been hospitalized many times, got run over by a car at one point, blew a heart valve from infection, had a couple of open heart surgeries, but in spite of all of that I kept busy with horseback riding. Since I couldn't be a physician anymore, my other thing I love in life is horseback riding. I had continued with horseback riding, and although I was disabled, I discovered that for a handicapped rider there was a sport of Paralympic dressage. Oddly enough, I managed to make the Paralympic team and represent the United States at the Paralympics in London in 2012. I flew my horse and went over with three teammates, just had the time of a lifetime. But over the last probably 20 years, I would get quite short of breath riding. Every time I would complain about it to the cardiologist or the rheumatologist or any other physician, they'd all say, "Well, Dale, several problems here. You're not exactly athletic, you ride a horse. B, you're not a kid anymore, because I am 63, and C, you don't work on your heart health particularly. If you worked at it more you would become more efficient at using your heart and you wouldn't get so short of breath." I got tired of hearing everybody say that I wasn't athletic and I wasn't working out regularly, because I was. I got so short of breath trying to ride, which is a technically difficult sport, but it requires not athletic abilities that 70 or 80-year-olds can't do. It doesn't require tremendous fitness like running or sprinting with swimming, but it does require muscle strength and being able to raise your heart rate. Every time I tried to do that, I got so short of breath. I about lost the horse and I thought, "Well, this is ridiculous," but I got tired of complaining about it because the physicians all said, "Dale, if you worked at this you could be fitter. There's nothing wrong with you." Finally I was competing in a musical freestyle. It was a test that I was riding to music and I had picked out the music that fit the horse and I. I'd written a test that contained six minutes of riding and I knew to the second how long each individual thing I had to do and it had to match the music. My coach decided I should try something new and I should try something that was a little harder than I was used to. I was determined I was going to give it a try. I went to the competition and three quarters of the way through the test, I was really short of breath and I thought, "I should just stop and take a break for a minute." I kept going, because I knew that at that point as I got very short of breath that I only had six seconds more to do and then I could walk for two minutes as part of the test. I could do anything for six seconds, I mean, let's face it. Well, in fact I couldn't, and I passed out on horseback, in front of an audience during a test. I fell, the horse got terrified and bolted. I came to on the ground in a strange place in the arena trying to figure out how I'd gotten on the floor in the arena and there was a horse nowhere in sight and a rather stunned audience and coach. After sitting there for a few minutes, I felt fine again and the ambulance people checked me out and I was fine. I even drove home after I cleaned up the horse and took care of things. But that evening I really didn't feel particularly well and ended up in the ER. They admitted me overnight and decided there was something wrong, but they weren't going to figure out what it was at that moment. Well, after that, the cardiologist thought perhaps we're missing something. After trying a couple of different things, he sent me to another cardiologist. His specialty was pulmonary hypertension, and in fact he would do a right heart catheterization with exercise. That was how we discovered it. When he did the right heart cath, I had normal pressure just lying there, but the minute I picked my legs up to put them in the stirrups my pressure jumped way up in the air. My oxygenation plummeted, and they stopped the test very quickly thereafter and said, "No, there's a problem here. You don't need to go on any further." I said, "But I can do a lot more than that, really," and they said, "No, no, no. You don't need to, that proves the point." So that's how I discovered that I had pulmonary hypertension and it explained a lot of what had been actually going on for at least 20 years, but now it's slowly getting worse over time. But do I continue to ride? No. I took up carriage driving, because it turns out that if I sit in an old-fashioned carriage and drive the horse, I don't need my heart to race fast and I don't get short of breath. So, I've discovered a new sport that I can do and enjoy, and so I keep up my life with sports. If you're curious about a fairly wild sport that's very popular in Europe, the indoor competitive carriage driving is done at a gallop and timed, with screaming audiences, around obstacles with one horse, two horses or four. It's a lot of fun and very competitive, and the horses love it. When I first got run over by the car and my heart got infected and I ended up with a couple of open heart surgeries back to back and really fought for my life, I couldn't walk for about a year and I had to retire. As a physician and surgeon, I'd been to school forever and I loved being a physician. I was very depressed and very unhappy and cried almost daily for several years. But you know what? At the end of that, I was no better and the rest of the world really didn't care. I decided that I'd better try something different and I'd better not worry about what I couldn't do anymore and start looking around for what I could do. Can't ride? Well, there's still lots of other things to do with horses. Talking with friends, they suggested carriage driving, which I'd never tried. I tried it and it's fun. I think throughout life, if you can learn to adapt and not regret so much what you can't do, but look around and find things you can do, that you can enjoy. If you can't walk much anymore, buy yourself an electric scooter, they're not that expensive. Then you can go to the mall and go shopping, do things with friends, go sightseeing. There are parts of what you enjoy that you can still participate in, even though you've lost the ability to walk any distance or breathe too much. My name is Dale Dedrick, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 304 - Dale Dedrick
12/02/2019
Episode 304 - Dale Dedrick
Dale Dedrick is a retired orthopedic surgeon who was diagnosed with lupus and pulmonary hypertension. In this episode, Dale discusses how her illness forced her to retire and how her love of horseback riding helped her make the United States Paralympic Equestrian Team in 2012.
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Raymond Benza, MD - phaware® interview 303
11/29/2019
Raymond Benza, MD - phaware® interview 303
Dr. Raymond Benza is a cardiologist in Pittsburgh, Pennsylvania and is affiliated with multiple hospitals in the area, including Alle-Kiski Medical Center and Allegheny General Hospital. He has been in practice for more than 20 years. In this episode, Dr. Benza discusses the importance of clinical trials in patients with pulmonary hypertension including a new molecule that's on the forefront of treatment for pulmonary arterial hypertension called PB1046. My name is Dr. Raymond Benza. I'm a cardiologist who works at the Allegheny Health Network in Pittsburgh, Pennsylvania. The Allegheny Health Network is one of the largest health care centers in Pittsburgh and includes facilities throughout Central and Western Pennsylvania. I have a lot of responsibilities here, but the most important role I have is serving as the director for the system for advanced heart failure, which includes cardiac transplantation and mechanical circulatory support, as well as run the pulmonary hypertension programs for the system. Today, I'm going to talk a little bit about some unmet needs in pulmonary arterial hypertension including giving a brief description about the disease and some of the current limitations of the current drugs that we have to treat the disease. Then, go into a little bit about a new molecule that's on the forefront of treatment for pulmonary arterial hypertension called VIP. Also, I'll talk a little bit about the clinical trial utilizing this new therapeutic. Then, tell a little bit about what we're doing in the trials and what variables we're measuring. Then, I will talk a little bit more about the long-term extension trial for this particular molecule. As many people are familiar, pulmonary arterial hypertension is a rare disease. We see it in about 50 per million people. It involves a variety of different types of subsets. The main one that we're interested in is called Group I pulmonary hypertension. This form of pulmonary hypertension is where the disease modifications are directly related to changes in the arterials and capillary structures within the lung, so that there's not a secondary etiological cause for a problem with the left heart or problem with the lungs or blood clots obstructing these. These are intrinsic abnormalities with the pulmonary vessels themselves. This disease affects a number of people, including those who have heritable components. So, patients can inherit certain mutations in genes that affect the way blood vessels remodel themselves. The most infamous are the defects in the bone morphogenetic protein receptor type II. There are also other disease entities like connective tissue diseases; patients with cirrhosis; those who have HIV; and very importantly in an ever-expanding a number of therapeutics, other drugs that can actually damage these arteries and cause a very life-threatening condition. Now the problem with pulmonary hypertension is not only the remodeling that we see in the pulmonary vessels, it's what that remodeling does to the right side of the heart. That is essential in really trying to understand the prognosis in patients who have this disease. The right side of the heart as opposed to the left side of the heart is not built to be a very muscular chamber. So, it's not used to pumping against very high pressures. When these high pressures develop as a cause of the disease, the right ventricle starts to enlarge to try to counteract this pressure that it sees coming back at it from the lungs. Unfortunately, when the right heart expands and dilates, it loses its ability to contract. So, right heart failure is really the main leading cause of death in patients with this very terrible disease. I am old enough to really have experienced the full spectrum of the treatments for this disease. When I started learning about pulmonary hypertension and treating patients with this disease, when I was a trainee many years ago, there was no treatment for pulmonary hypertension. [Patients] were all coming to the heart and lung transplant list. That's really where I first met these type of patients, because transplantation was really the only cure treatment for this terrible disease. Then luckily in the late 1990s, a drug started evolving and coming to the market. The first [drug] was intravenous epoprostenol, and that became a life-saving drug. Because now we had a treatment for the disease, there was intense interest from both the PH community and the pharmaceutical community to develop other drugs that can treat the disease. So, learning a lot about the pathways and how the disease propagates, we were able to develop really now a full armamentarium of therapeutics that have really changed the course of this disease from a rapidly progressive, deadly disease to a more chronic and progressive disease. We've doubled the life expectancy of these patients. So, these therapeutics have really helped, but we have not found the magic bullet. We don't have the Holy Grail yet, and patients will continue to progress, and they will continue to die from this disease, because we have not found the right combination of molecules to really put the disease at bay. The type of patients that we see with pulmonary arterial hypertension, I think, has also evolved over the last several decades. Typically, when I was a trainee and early faculty, the patients we were seeing were young women, and that was the classic phenotype that we would see. As we know, pulmonary arterial hypertension occurs in women five times as frequently as it does in men. When we started learning more about this disease, we recognize that other disease subsets could also develop this disease. We began seeing in patients with scleroderma, lupus, HIV and cirrhosis. We were seeing a whole mix of different clinical phenotypes now coming to us with this same deadly disease. So now we're seeing, typically, people present in their 40s or 50s. The mean age of patients that we see has moved from the 20s and 30s to now the 40s and 50s, and in some cases in the 60s. So, it really has been an evolving landscape. As people present with the disease at an older age, we're seeing a whole mix of other comorbidities that come along with the disease; hypertension, diabetes, renal insufficiency. It really has been a dynamically changing landscape. That's very important, because that has helped us grow the drug therapies to tailor to this growing and changing population of patient. There are very good guidelines that we've developed to teach clinicians how to both diagnose the disease, and once it's diagnosed, how to treat it. One of the most important things that we do in contemporary algorithms to treat pulmonary arterial hypertension is we risk stratify people. So, when someone is newly diagnosed with the disease, we want to know where they are in the spectrum of the disease. Are they in the early portion, the mid portion, or these very late presenters who are almost in the terminal portion of the disease? That initial risk stratification enables us to use the right combination medicines upfront to battle the disease as aggressively as we can. There are a lot of contemporary tools now that we use to risk stratify people. One of the most well used and probably the one with the best discrimination is the REVEAL score. But there have also been very good scores developed in Europe, including from our colleagues in France, Germany and Sweden, that have also enabled us to profile these patients to match [a] patient with individual drugs such that a low-risk patient may be good with an oral drug - one, maybe two. But a high-risk patient should always be started on upfront combinational therapy including parenteral prostacyclin. Then the intermediate risk [patient], somewhere in between those. So, risk stratification and then marrying the appropriate risk with the appropriate drug or combinations of drugs including a parenteral drug is ultimately the most important thing that we do now. Many of the medications we have now are not truly disease-modifying drugs. They mitigate the course of disease primarily by ensuing changes in their regulatory and vasodilatory status of the blood vessels. By doing so, they allow the right side of the heart to operate more efficiently, but they do not cure the disease. They don't stop the smooth muscle proliferation; they don't stop the unchecked endothelial proliferation or the adventitial remodeling in the blood vessels. So, the disease is still there. That's why new drugs that actually modify the disease, that actually can stop the pathological processes, that are causing the arteries to remodel, those are drugs that we're looking for now. We have great Band-Aids, and these drugs do great things to patients. As I mentioned earlier, we have improved mortality with this disease, but it will continue to progress. So, we need disease-modifying drugs, and we need drugs that can push people back from the high-risk profiles to the low-risk profiles consistently and steadily. That's where the explosion in these new molecules come into a very important role. Clinical trials really run at the heart of what we do in medicine. We can't use drugs on people until we prove that they are safe and effective. That's what clinical trials do for us. Enrolling patients in clinical trials, particularly those who have unmet needs - they're not cured from the disease, they're continuing to suffer from the ramifications of the disease, they're still short of breath. These are the patients that we need to enroll in clinical trials to test these new therapeutics to make sure that they drive this disease down, and they make people have a better quality of life. We want people to function and feel better about the way they carry on their daily activities of living. Obviously, being a clinical trial investigator, I get to work on the forefront of seeing new molecules coming through the clinical trial arena. PB1046 is one of those molecules. It's interesting. This is a molecule that we have tested before, not in this specific chemical composition, but in other forms. We found that it was a very potent dilator [of] the pulmonary vasculature. Here's a new substance and a very unique molecule that we can give once a week, that can have long-lasting effects for the entirety of that week, and it will help with clinical efficacy in that manner. That affords the patient a lot of free time - [to] not carry pills with them, to set timers or take their drugs. The important thing to remember is that it's a new kind of treatment for PH that targets the disease process itself rather than just opening blood vessels like some of the older drugs. The hope is that it can be a more effective drug than these older therapies that focus mainly on dilating the blood vessels. The real name for PB1046 is a vasoactive intestinal peptide analog or VIP analog. Even though it's called vasoactive intestinal peptide, it does work directly in the lungs and heart, but it was first discovered in the intestines of some animals, and that's why they call it that. I think I was the first clinician to actually try this therapeutic, and I had three patients try it. In two of them, it made them feel better, but sometimes the patients want to feel better, so it's not really clear whether it worked or not. That's a very strong placebo effect. However, when I measured the pressure in their lungs during treatment, it seemed to have helped that. It's interesting, because we utilized this new compound in patients who had an indwelling hemodynamic monitor, and we saw very impressive changes in the pulmonary artery resistances and the way their right heart reacted to that drop in resistance by improving its stroke volume. Now, the injection causes some redness on the skin. Patients need to be aware of that and not be startled by it. I think the company says that it's due to the blood vessels that are expanding in the skin that's caused by VIP and [it] doesn't usually bother the patients too much. My patients got used to it, and they were able to stay on the drug for six to 12 months. There definitely needs to be more research to test it in more patients. The company that makes PB1046 is called PhaseBio. I work with the company as a paid member of their scientific advisory board, and they're currently conducting a larger clinical trial, to test whether PB1046 is safe and effective in slowing down or reversing the pulmonary arterial hypertension disease process. They need to test the drug in about 60 patients to see whether it works or not. It's a randomized double-blind trial, which means that patients will either get a high dose of PB1046 or a low dose. The high dose is what the company thinks may be more effective. Patients won't know which dose they're getting, obviously, until the trial is over. The treatment periods are about 16 weeks long, so patients will get 16 doses, once a week. After the 16-week study period is finished, patients will get to join the extension trial, which means that the patients can all get the high dose until the overall study results are known. Patients will have their lung pressures checked during the study, they do multiple six-minute walk tests, and have a variety of other assessments that are basic in many of our clinical trials that we do now in this disease process. We're involved in this study, and the company is opening study sites at multiple clinics around the United States and in Europe, also. They seem very committed to bring this new treatment to PAH patients around the world. To learn more about this study, you can visit , or or even better, I would use the phaware global association® clinical trial matching tool at . I really thank you for letting me be part of this today. My name is Dr. Raymond Benza, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . 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Episode 303 - Raymond Benza, MD
11/28/2019
Episode 303 - Raymond Benza, MD
In this episode, Dr. Raymond Benza discusses the importance of clinical trials in patients with pulmonary hypertension including a new molecule that's on the forefront of treatment for pulmonary arterial hypertension called PB1046.
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Dawn Meador - phaware® interview 302
11/26/2019
Dawn Meador - phaware® interview 302
In this episode long-term pulmonary hypertension patient, Dawn Meador discusses PH diagnosis as well as her decision to adopt a child, post dx with her third husband. My name is Dawn Meador. I currently live in Virginia. I was diagnosed in April of 1998, so I have had pulmonary hypertension for a little over 21 years. When I first noticed that something major was wrong, I had a nine month old at the time, and I was carrying her in Walmart in her little pumpkin seat and I got to thinking, "I can't breathe. What is going on here?" You could literally see my shirt moving, my heart was beating so hard. So, the next day she had a doctor's appointment and I mentioned something about it to the doctor, because it was our family physician. From there, he did testing and then sent me to a heart specialist. They basically tried to tell me I didn't have a chance. I wasn't going to make it. I was going to have to have a transplant. There was no other ifs, ands or buts. I got a second opinion from another doctor who did know more about pulmonary hypertension, who put me on medication and treated me for 14 of the 21 years before we moved out here to Virginia. However, I honestly think I had it longer, because I had spells where I would just pass out for no reason and they couldn't figure out what was going on or why I was doing it. Like during PE, there was one time we would do little stations and do exercises at the stations, and then run around the gym once or twice, and then go to the next station. My PE teacher looked at me and just screamed my name and told me to go outside because, he said, "You're as white as a ghost." He said, "From looking at you, I can tell you can't breathe, so go outside and get some fresh air." After that, needless to say, I never ran anymore in PE as long as I had him as my teacher. So, I really think I had it longer. But it took me getting with the right doctor to actually figure out exactly what was going on. At the time, I actually had a five year old and the nine month old. I guess the first thing that popped in my head was this can't be real. This can't be happening. You know, I have a five year old and a nine months old and they both need me. I've got to stay here for them, so I've got to do what I've got to do to keep fighting through all of it. I guess it was not long after my husband went on deployment, everything shifted and it was a drastic change. I think I was more nervous for him to come back than he was, because we had to decide via email whether we was going subcutaneous or intravenous [for my treatment plan]. So through email, him and I are emailing back and forth about it and I'm like, "Great." We'd been together at that time, three and a half years. I just thought this is going to freak him out. You know, he's going to come home and I'm going to have this tube hanging in my stomach or out of my chest, and oxygen in my nose, and how is he going to react? His reaction to me was it doesn't change who you are as a person so why would it bother me? It's keeping you here with me so it's all good. After my diagnosis, about a year and a half, two years later, my husband honestly just could not handle the medical issues and I was hearing, "Why do you have to go all the way to St. Louis?" Because it at the time I lived in Southern Illinois, so it was a two and a half hour trip to my doctor. "Why do you have to go all the way there? You should be able to find somebody around here that can take care of you." We all know with PH, the good doctors are few and far between. I guess that kind of started leading to the downfall of the marriage. We ended up divorcing and I met another man. He swore to me that it wasn't an issue to him, and after about eight or nine years, I started seeing that it was an issue to him, because I was having more problems. He just emotionally could not be there for me. So that marriage fell apart. So, I was very, very hesitant when someone told me, "Oh, you need to meet this guy." I'm like, "No, no, no, no. I'm done. I'm not doing this again. They can't handle the medical problems." Our very first date we went out to eat and we sat down at the table and he said, "So tell me about yourself." And I said, "You know, I have two kids. I've been divorced twice. I have a lung disease that is going to one day either kill me or require a double lung transplant, and if you can't handle that, we're just going to call this friends and leave it at that." His next words to me is kind of what probably made me think, "Okay, he's a keeper," because his reply was, "Does the disease define who you are as a person?" "Well no." He said, "Then why would it be a problem?" I said, "Well, it was for the other two." And he said, "I'm not like the other two." He has more than proven that he was right, because the week of our wedding, five days before our wedding, I was admitted into the hospital, because I was having a lot of issues and they had to do a right heart catheterization. During the right heart cath, they had to give me more anesthesia than they normally do. When I came out, I'm laid there, sicker than all get out. The anesthesia usually never ever bothered me, but because they had to give me so much more I was very sick. So here he is holding my hair back with one hand and the bucket with the other hand, and I looked at him and I said, "If this ain't going to make you run, I think we're good." He didn't have kids when we got together and that was one of my concerns because I told him, I said, "You know, I can't have kids and you don't have kids, and I know you want kids." That is how the adoption kind of came about. We started talking about it. We got married in August of 2011, and at the very end of November of 2011, we had sat and prayed and thought and tried to figure out how in the world we were going to be able to do this adoption. [Going through] adoption agencies is 30 to 40,000. I don't know how people can even adopt spending that kind of money. A friend of ours sent me a text message out of the blue the very end of November. We were out of town. He had an awards banquet or something with the Navy that we were out of town for. I looked at this text message and I looked at him and he's like, "What?" I said, "One of our friends is asking me if we're still wanting to adopt a child." I sent her back and I said, "Well yeah, if we can ever figure out how we can afford it." She said, "The girl that's been living here at my house with my daughter, who is pregnant, they have decided to put the baby up for adoption. Would you be interested?" We were like, "Yeah, we'll be back tomorrow. We'd love to meet with them tomorrow and talk, and then meet us and decide whether they want us to adopt the baby or not." So the next day we met with them and we talked for quite a long time. On the following day I spoke with a lawyer and we got all the paperwork started. We found out the very end of November, and she was due December 28th. So, everything was going to have to move really quick. She ended up being born January 5th. So in less than a month and a half, we went from not ever thinking we were ever going to get a child to having a newborn baby that we watched at the delivery as soon as she was born. We are the ones they handed her to. She actually has a birthmark that is in the exact same spot as my husband's. She's got my husband's color hair, my husband's color eyes, and acts and looks just like both of us. So it's kind of unique how our story comes about. There's lots of people that question us and ask us about it all the time. You know, "How did you think you were going to be able to handle a newborn with all your health issues?” My response was, you know, "If God gave us this baby to take care of, he's going to keep me around to me take care of him." So far he did it with the first two, so I think he's going to do it with this one too. [My husband] treated my kids like they were his from day one. When he and I got together, the oldest was 17 and my daughter, the one that was nine months old when I was diagnosed, she was 13 and a half at the time when we met. So they were kind of pretty much grown and everybody's like, "You guys are going to start all over with a baby?" "Yeah. Why not? This is what we want." There were a lot of people that had doubts and worries and concerns, and I totally understand. I have been divorced twice. I have this lung disease. We never knew what was going to happen next, but we've been together nine years. To see us together, we're not one of those couples that you can tell have been married for a long time. We're that couple, that 10, 15, 20 years down the road, you still think we're newlyweds and get along. We don't fight, we don't argue. We just don't. I dealt with all that with the first two and I told him right from the start, "I'm not going to argue, I'm not going to fight. That's not a marriage. If we can't talk about things and get along we need to figure out how to, otherwise it's not going to work." We communicate very well. My advice would be just don't give up. Don't look at it as I'm never going to be able to have kids because there are other options out there. There's surrogacy, there's adoption, there's so many different routes that you can go. Just because you've got this disease does not mean you can't be a mom. It's not easy being a mom with the disease, but it is totally worth the fight to keep going when you look at that child and you know this is my child to raise. This is my child to take care of. It's the biggest blessing. I mean everybody asks us all the time, "Would you do it again?" Oh, I'd do it again in a heartbeat. I don't care how old I am. I would take four or five more babies. If somebody was to say, "Here, would you raise these kids for me?" I'd do it in a heartbeat, because I love it. It's what keeps me going. It's what keeps me staying positive and it's what keeps me knowing that no matter how many road bumps that I hit with this disease, it's okay because I've got these babies at home to take care of and I've got to get back home to them. If anybody ever has questions or doubts, I'd be more than happy to talk to any of them. My name is Dawn Meador and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 302 - Dawn Meador
11/25/2019
Episode 302 - Dawn Meador
In this episode long-term pulmonary hypertension patient, Dawn Meador discusses PH diagnosis as well as her decision to adopt a child, post dx with her third husband.
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Hall Skåra - phaware® interview 301
11/22/2019
Hall Skåra - phaware® interview 301
Hall Skåra is a pulmonary hypertension patient from Norway. He became part of PHA Europe’s staff after his diagnosis. He has helped develop their “White Spots” program, which establishes new PH patient associations in countries where they don’t exist. In this episode, Hall discusses the first ever PAH Patient Charter that was launched on November 12, 2019 to coincide with PH Awareness Month. The Charter was facilitated and sponsored by Actelion, a Janssen Pharmaceutical Company of Johnson & Johnson and a PAH-expert Steering Committee made up of patients, patient advocates and healthcare professionals. #PAHPatientCharter #phawarenessmonth My name is Hall Skåra and I live in Norway. I was diagnosed with PH in 2005. At that point, there was no patient association in Norway, so I started one a couple of years later. A couple of years after that, I joined the European PH community, (PHA Europe). I've been working as a staff member there. During this last period, I was the interim president for PHA Europe. Today, I would like to talk about the PAH Patient Charter, which I think will be important for the global PH community. But let me just talk very briefly about pulmonary hypertension first. PH is also referred to as the other high blood pressure. You measure the blood pressure on your arm, which is the regular pressure, but then also you have blood going through your lungs, and sometimes this blood pressure is elevated. [If so] then you get a disease called pulmonary hypertension [PH]. This can be caused by many different things. In my case, they don't know why I have this, so they call it idiopathic. It is really difficult to detect. It does require an invasive procedure. You have to put the instruments inside your heart and lungs in order to get 100% accurate diagnosis. This is called a right heart catheterization. One of the reasons why it's difficult to detect is that the symptoms can easily be confused with similar symptoms to less serious illnesses. The symptoms could be one or more of, for instance, shortness of breath, water retention, fatigue, chest pains, dry cough, blue lips and fingers, et cetera. When a patient goes to a doctor and complains about shortness of breath, the first thing the doctor will think about this, that this patient is out of shape, maybe has asthma, maybe he has COPD, et cetera. So that's why it often takes a long time to get an accurate diagnosis. Unfortunately, [for patients,] their disease gets worse and by the time they get their final diagnosis, they are often in quite bad shape. I was invited by Actelion, which is one of the pharmaceutical companies, to take part in a meeting. I was there as the patient but also as a representative of PHA Norway and PHA Europe. Together with me,[there] were other patients. There were representatives from the PH Association communities around the world and also healthcare professionals. We were invited there to make a PAH Patient Charter. This Charter that's based on the ESC/ERS international guidelines for diagnosis and treatment of pulmonary hypertension. It was developed from the output of this meeting. The reason we developed this is that we want to create an easy-to-read overview of what you can expect as a patient when it comes to diagnosis and care of PH. This PAH Patient Charter can also be used by the patient to enable simpler communication with their healthcare professional so that you together can make decisions and agree on a care plan that is appropriate for you as a patient. I think this PAH Patient Charter will be important for many reasons. Today we are moving towards a more patient-centric care, and the patients themselves, they play an increasingly bigger role in the treatment and management of his or her illness. This PAH Patient Charter will empower the patients since the Charter outlines the standard of wholistic care that everyone would PAH should receive. So, I think this is a first and very good step towards the universal standardized care for people suffering from PAH. I think there are different ways to use this PAH Patient Charter. One way is, as I mentioned, is for the patient to enable him to talk better to the healthcare professionals. In Europe, there are many countries which struggle a lot when it comes to the treatment of PH. For instance, in some countries they don't even have access to medication. I think maybe the patient associations in these countries can use this, with the support of PHA Europe, to talk to the health authorities and show them what expectations the patient will have to the treatment of his and her disease. In the past, the doctor would give the patient a pill, and then that's it. We also see when it comes to PH, that other aspects of treatment it’s very important for the outcome and also quality of life of the patient. As this PAH Patient Charter outlines, you need a wholistic approach when dealing and treating with PH patients. This is something that is described in the document. We talk about, for instance, rehabilitation. We talk about what kind of food or diet people should be on. We talk about psychosocial support, guidance when it comes to traveling, et cetera, et cetera. My hope for this PAH Patient Charter is that it will be to the benefit of the patients and that it will enable them to get even better care that they get today. My hope is also that in some countries where they don't receive optimal care, that this document can be used to achieve better care for the patients. My name is Hall Skåra and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials
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Episode 301 - Hall Skåra
11/21/2019
Episode 301 - Hall Skåra
Norway's Hall Skåra discusses the first ever PAH Patient Charter that was launched on November 12, 2019. The Charter was facilitated and sponsored by Actelion, a Janssen Pharmaceutical Company of Johnson & Johnson and a PAH-expert Steering Committee made up of patients, patient advocates and healthcare professionals. #PAHPatientCharter #phawarenessmonth
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Iain Hess - phaware® interview 300
11/19/2019
Iain Hess - phaware® interview 300
In our 300th episode, Iain Hess discusses his lifesaving double-lung transplant he received two years ago on January 17, 2017. Iain is a former pulmonary hypertension patient from Colorado who was diagnosed at age 5. My name is Ian Hess, and I'm here to share my story. One year ago, I was standing on top of one of Colorado's 14,000-foot peaks. It was a difficult climb to the top, but overall it was one of the most rewarding experiences of my life. It was something that I'd wanted to do for a long time, but never had the opportunity to, because for 12 years I had lived with pulmonary hypertension [PH]. Doing something like this was just simply impossible. But thanks to my physicians, nurses, parents, people at the pharmaceutical companies, friends, everyone, I was able to do such a thing. Going back to 2004, two weeks before a planned family trip to India, I was diagnosed with pulmonary hypertension. At the time, I couldn't really comprehend what my diagnosis meant, but the doctors essentially told my parents that if I were to go on a flight pressurized to 8,000 feet, it would be a big mistake. So, 10 days later I had a right heart catheterization, which confirmed that I have super systemic levels and I immediately was diagnosed and had a central line put in and was put on a CADD pump which administered Flolan. For years after diagnosis, the progression of the disease was constant, but it was not frightening. In a way, it was like boiling the proverbial frog in that I couldn't feel the difference day by day, but overall and after time, my body was taking the damage. There was a lot I could no longer do. Since I was two years old, my family had started me skiing, but as soon as I was diagnosed I could no longer go to high elevations or do anything of that sort. So initially ,that didn't really affect me too much. But as time went on and my siblings started skiing and my friends started skiing, I definitely felt like I was missing out. Being connected to a central line and pump obviously had many drawbacks. Number one, it would fail if it got wet. Number two, I had to carry a backpack with the CADD pump in it at all times. This proved difficult when it came to do activities, mainly swimming. I would have to wear a dry suit. For me, that was just one of the biggest pains because swimming was always something that I had wanted to do and always had kind of a passion for. But as soon as I had to put on that dry suit, it just took the fun out of swimming for me. That dry suit did its job perfectly, but on the few occasions I may have ripped it or something, but water did get in on a couple times, which caused the pump to fail or my Broviac to get wet. Which in many cases, which happened many times, I either had my pump fail, which meant me not getting my medicine, which resulted in the paramedics having to come and do whatever they do. The worst situation with my central line, overall, was probably when my parents were having a dinner party and the kids were running up and down the house, just having a good time. All of a sudden, my dog took one misstep, stepped on my central line and ripped it right out of my chest, basically causing blood and Flolan to spray everywhere. So, my dad called the paramedics and fortunately they came in time and took me down to the hospital. My dog didn't even bark when about 10 first responders came in just of the shock. In thinking back, my parents had to always be at the ready, so to speak, in case something like this happened because even the smallest situation could turn into a big deal. Despite many drawbacks, pulmonary hypertension came in handy once or twice during my life with it. Again, once or twice, not often. I would use it to skip a class or turn in an assignment late once in a while. But the biggest positive came from the Make-a-Wish Foundation. My dream car has always been the Bugatti Veyron. And for those of you that don't know, it was at the time, the fastest car in the world at 267 miles per hour and had a price tag of over $1 million. So, I just loved everything about it. What Make-a-Wish did for me is they sent me to France, to the Bugatti facility in the Alsace region of France in a town called Molsheim. The special thing about this trip, is that it had never been done before, first of all. Bugatti generally only lets their customers on the grounds. So, it was a very one-off experience that I got to have. Yeah, I got to ride in it, which was fun. Pulmonary hypertension is disease that progressively worsens over time. Like I was saying before, at the beginning of freshman year of high school, I started noticing that walking up a flight of stairs would leave me breathless, gasping for air. You can see in this picture, that my face is very red, number one, due to the constantly increasing doses of vasodilators that I was on. I was also turning a kind of blue color because of the lack of oxygen that I was getting. Basically, at the time I was the color of PH. This is the color of PH. I dealt with the worsening disease for about two years before my doctors in Denver decided that something had to be done. But what to do was unclear. One option was to pick a transplant facility and go through the application process and then decide based upon that. The other option was to go to St. Louis, Missouri, where they had recently adopted a fairly new procedure in the U.S. called the Potts Shunt. Essentially what this procedure is just a shunt that basically directs blue blood around your heart protecting it. After visiting various transplant teams, my family and I decided that the Potts Shunt surgery was the best option for me. I can say now that picking St. Louis was the best decision of my life. During the summer of 2016, my parents took all three kids, both my siblings on a European trip for six weeks. We went to the U.K. and saw family. We relaxed on the Coat du Soir in the south of France and went to various art exhibitions in Italy. Little did I know at the time, though, that my parents had planned this trip out as it may have been our last together. This was the calm before the storm. I went back to Colorado and then immediately my mom, my dad and I drove to St. Louis. Initially it went pretty smoothly. I had a quick but extremely painful recovery. The incision that my doctor, Dr. Eghtesady had made was called a thoracotomy. It was about a six-inch incision vertically up the left side of my back. In doing the incision there, he had to cut through tons of deep muscle, which just turned out to be extremely painful. So, I was on Oxycodone for months and months afterwards. The following months after the surgery, unfortunately, proved to be as hard on my body as ever. On one day, October 27, 2016, my memory started fogging up. It was a strange experience, because I couldn't remember, for example, what had happened 20 seconds prior. It was all just a foggy day and it's hard to explain. After that day, I woke up and I felt horrible. I felt the sickest I had ever felt before. I called my dad home. He got home and we checked my oxygen stats and sure enough, they had dropped to an all-time low of 75%. We quickly took the car down to Colorado Children's Hospital where my cardiologist, Dr. Ivy, unfortunately found out that the Potts Shunt had not worked properly. I was too late, basically. At that point, the only option that I had was to go back to St. Louis to have the double lung transplant. Two months went by and it was departure day. I exchanged emotional goodbyes with my friends at school and then later with my brother, sister, and mom. Then, my dad and I got in the car and took off for St. Louis. I can remember sitting in the car, looking back at the sunset over the Colorado Rockies and thinking man, I really hope I see this place again. For about the first month in St. Louis, I had just constant appointments. Once that was done and out of the way, we were just kind of sitting in my apartment for two months and I was bored out of my mind. I accepted it, as even the slightest activity would completely wipe me out. I ended up staying in my apartment, basically just watching Netflix or playing video games. Because at that point, I was living a miserable life, effectively in prison by a failing heart. When your body is fighting to survive, then there's no energy for you to actually live life. Your mind loses hope and your survival instincts turn on. You lose your sense of empathy for others and focus your energy on staying alive. You start to eat less because your body is physically shutting down. You know that somebody has to die for you to have a chance to live, and frankly and morbidly you wished for that. It's a depressing thought, but it's frankly the truth. After about two months, of waiting on January 17, 2017, I got the call, a new pair of lungs were on their way in. The day they called me into the hospital was a day I'll never forget, I was just sitting in my room in the apartment, my parents in the room across from me, and all of a sudden, I heard commotion in the other room. I thought, what's going on? It's like 12:00 at night. Then, all of a sudden, my parents came into my room with a video camera on saying that a pair of lungs had been found and that we had to be in the hospital by 2:00 AM. I got super excited, obviously. I got dressed, I got my shoes on, I got ready to go. I went into my parents’ room and they were still in bed. I was like what are you doing? But they had thought, and rightfully so, that the next couple of days they would be getting no sleep, so they wanted to get a little bit more sleep before we finally went in. We ended up making it out of the apartment by 2:30, and I thought we were beyond late, but it turns out we weren't really. When we entered the hospital, we found the cardiac floor and they told us to go up, where I was initially met by my first nurse, Alyssa, who put an IV in me and basically told us the same thing, just get some sleep because the next couple of days were going to be extremely long. Then the morning of the 17th, doctors made their rounds. Basically preop was initiated from the moment I woke up. Various units of the hospital stopped by and then around 12:00 PM they wheeled me to the elevators where I handed off my glasses and gave my parents final hugs, not knowing if it would be the last. Then we went down. The anesthesiologist took me up to the sleepy drugs and that was it. What happened during the next month and a half are virtually nonexistent. Well, they are nonexistent from my memory. This was due to obviously the many sedative medications that I was on, and I can only recall what my parents have recounted. Although the lungs were perfect on arrival, I ended up being on the slab for about 17 hours, because the doctors couldn't stanch endless bleeding. During that time, two massive blood transfusion protocols had to be called in, which means it's 50% of your blood replaced over a period of four hours. Only when they could stop the bleeding, which turned out to be about five days later, could they close my chest up. That was really the first battle that I faced during that time. Soon after this, I was put on ECMO and dialysis because my kidneys had failed. After that, I had pneumothoraxes, ICU delirium, strokes, a seizure. I had vagal nerve damage, which is just basically damage to [the] area around your throat. So, I was not able to talk for a while. I also had something called neuroleptic malignant syndrome, which is a reaction to a specific kind of antipsychotic drug, which basically shot my temperature up to 107 degrees. At 107 degrees, you're very close to melting your brain. And the doctor had actually comforted my mom by telling her that the brain only melts at 108. It was an intense 48 hours of ups and downs, constantly being refrigerated and unheated. Fortunately, I don't remember any of that. Looking back, my life has been a roller coaster of ups and downs, never knowing when you're going to rise and never knowing when you're going to fall. When I was initially diagnosed, I didn't understand what my life was turning into. Over the years as I grew and gained maturity and awareness, I also lost a lot of hope for the future. I didn't know how long I would live, but that's just something that kind of grew a part of me and I still have that mindset to this day. At end stage pulmonary hypertension, when my body was dying and my mind weakening, I never gave up. I just kind of accepted each day as it was. In the end, I made it through. The nurses provided miracles, the doctors provided miracles. My friends and family provided plentiful support. In the end, no one knows how long they will live for. So, my overall mindset changed through this process. I kind of asked myself why do I need to rush through life so quickly? I came to the answer -- you don't.
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Episode 300 - Iain Hess
11/18/2019
Episode 300 - Iain Hess
In our 300th episode, Iain Hess discusses his lifesaving double-lung transplant he received two years ago on January 17, 2017. Iain is a former pulmonary hypertension patient from Colorado who was diagnosed at age 5. This episode was recorded LIVE in San Francisco, CA.
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Wendy Gin-Sing, RN - phaware® interview 299
11/15/2019
Wendy Gin-Sing, RN - phaware® interview 299
Wendy Gin-Sing RN, MSc, MRes, is an advanced nurse practitioner with the Pulmonary Hypertension Service at the Hammersmith Hospital in London. In this episode, she discusses the first ever PAH Patient Charter that was launched on November 12, 2019 to coincide with PH Awareness Month. The Charter was facilitated and sponsored by Actelion, a Janssen Pharmaceutical Company of Johnson & Johnson and a PAH-expert Steering Committee made up of patients, patient advocates and healthcare professionals. #PAHPatientCharter #phawarenessmonth Read the PAH Patient Charter here: My name's Wendy Gin-Sing. I'm a Pulmonary Hypertension Nurse Specialist. I work at the Hammersmith Hospital in London. I've been looking after pulmonary hypertension patients for about 24 years, and the last 20 years as a Nurse Specialist. I've seen quite a few changes over the time. Today, I want to talk to you about the new PAH Patient Charter that's just been released. But first, let me give you a brief explanation about what pulmonary hypertension is. To understand pulmonary hypertension, you need to know what the normal circulation in the body is. You have two circulations, one which pumps blood all the way around the body, and that's the blood pressure that can be measured on your arm. And then there's a second circulation where the heart pumps the blood through the lungs so that it picks up oxygen ready to start off again. In pulmonary hypertension, the pressure in the blood vessels in the lungs goes up. Normally, this is just very low pressure, because you don't need the blood to go very far. It just goes from one side of the heart to the other by the lungs, and the oxygen moves over from the lungs into the blood vessels at low pressure. In pulmonary hypertension, blood vessels in the lungs change. The walls become thicker. They become less elastic, and the pressure goes up in the blood vessels. This then places more demand on the right side of the heart, which, over time, becomes tired and can sometimes fail. The PAH Patient Charter came about through , a Janssen Pharmaceutical Company of Johnson & Johnson. They got a group of us together: patients, patient associations, doctors, nurses. We sat down together and discussed about what we felt was really important when looking after patients with pulmonary hypertension. Both from the medical side and the patient side. We came together and we agreed on many points, and have produced the Charter. This Charter is going to be important for patients, caregivers, and clinicians looking after pulmonary hypertension. It sets out what patients with pulmonary hypertension should expect. Some of the very simple things like an earlier and accurate diagnosis, being involved in decision making, access to the therapies that are available in their country, and also, and probably the most important, the holistic care for patients so that they can actually live life well and have a better quality of life. When this is released, I think patients can use it in a variety of ways to speak to their health care professionals, and to try and ensure that they are getting the standard of care that's set out in the Charter, but also to be able to use it for lobbying politically and through the health care organizations in the countries they're in to improve the care of all patients with pulmonary hypertension. In the UK, we're very lucky that we have designated centers and access to all the pulmonary hypertension medications. But I am aware that there's many places in the world that don't have specialist centers. They have health care that they can't access all the drugs. For some patients, it takes a very long time to get a diagnosis. The optimal patient journey really starts in the very beginning, getting an early and accurate diagnosis. It still takes around two years in countries with developed health care to achieve a diagnosis of pulmonary hypertension. Whilst the first year, maybe, patients perhaps [are] not seeking medical attention, they have often seen four or five different physicians over the next year before they get an accurate diagnosis. Once patients have their diagnosis, they should have access to all available pulmonary hypertension therapies within their country. They should also have their care from a most disciplinary team so that they can look after their holistic needs. Not only their physical needs, such as rehabilitation and doing more exercise and managing their symptoms, but also the psychological care that patients, and not only patients, but their carers as well really need as they're going through the journey with pulmonary hypertension. For some patients who pulmonary hypertension therapy doesn't control all their symptoms, they need supportive care to help manage those symptoms. As the disease progresses, they should then have input from the palliative care teams, so that they have a really good quality of life throughout their pulmonary hypertension journey. I was honored to be asked to take part in the Patient Charter group. It was really good to meet up with patients, physicians, patients, advocacy groups from around the world: , the PH Association for , , , and . It was really important that advocacy groups were involved in the PAH Patient Charter. As health care professionals, we see pulmonary hypertension very much from our point of view, and the associations have many members, all of which who live with the disease or care for somebody who does. It's so important to get their views so that this PAH Patient Charter is really a whole global initiative. The PAH Patient Charter is really important, because it will help patients be more empowered so that they can be part of making the decisions about their care. Not only because they're better educated, but also they have more confidence in being able to talk to you, [the health care professionals,] but making decisions about the care that really affects them. Pulmonary hypertension can affect anybody, from little children up to people who are very old. It affects people in every country, and that's why it's important to have a global PAH Patient Charter that we use to improve quality and quantity of life with patients with pulmonary hypertension. We're excited that the PAH Patient Charter's been launched during Pulmonary Hypertension Awareness Month. To learn more, search the hashtag #PAHPatientCharter. My name's Wendy Gin-Sing, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials #phawareMD
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Episode 299 - Wendy Gin-Sing, RN
11/14/2019
Episode 299 - Wendy Gin-Sing, RN
Wendy Gin-Sing RN, discusses the first ever PAH Patient Charter that was launched on November 12, 2019. The Charter was facilitated and sponsored by Actelion, a Janssen Pharmaceutical Company of Johnson & Johnson and a PAH-expert Steering Committee made up of patients, patient advocates and healthcare professionals. #PAHPatientCharter #phawarenessmonth
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Michelle Sweitzer - phaware® interview 298
11/12/2019
Michelle Sweitzer - phaware® interview 298
In this episode, pulmonary hypertension patient, Michelle Sweitzer discusses her illness and why she chose to adopt. My name is Michelle Sweitzer. My connection to PH started many years ago. I had a brother that was diagnosed when he was almost five and passed away shortly after. At that time, there was no treatments or anything like that. Then 11 years later, my dad was diagnosed, and as a result, the rest of my brothers and I all got tested to see if we had the disease. I had another brother that at that time was asymptomatic, but diagnosed, and myself and another brother of mine were not. We were fine at that moment, but later came to find out that when they said it's not the gene, that we in fact had BMPR2. So, with that, came different things that we needed to do to try to help if there was a way to keep the PH from activating, so to speak, being the triggers. One of those was pregnancy. So, when I got married in 1989, my husband and I decided that pregnancy wasn't something we really wanted to venture into since we knew that it could cause the PH to onset in my body. So, we looked into adoption. We started as foster parents and adopted our first son in 1992, and he's now 33 years old. Then throughout our marriage, we had other foster kids. When we moved to Washington, we ended up being blessed with another little boy at 18 months old. He is now 11. In the meantime, I was actually diagnosed two years ago with the disease, not from pregnancy, but I don't know what the trigger could have been, maybe stress or hormone changes in my body, I don't know. With that, I had to retire from teaching, although I do homeschool our son. God has blessed me with the privilege of being able to go to different pharmaceutical companies with my mom and share our story and give them the human perspective of why they sell the drugs and why it's so important to allow us to have life and better quality and longevity. I'm so thankful for that purpose. When I first started meeting people with PH, when my dad had it and we started getting involved in support groups and going to conferences and things like that, you end up meeting a lot of different people with PH. I kept hearing over and over again that there were women that that got it when they got pregnant. So, right away [it] set in my mind this idea, goodness gracious, that could be a trigger for me. That could cause me to have PH. It was actually quite an emotional thing I needed to battle with inside. I was young then. I wasn't married at the time, but I was thinking I would love children. I went into the field of teaching and I had a degree in child development. To know that I wasn't able to actually bear children just in case that might possibly onset the disease was trying. It was tough. It led me through a lot of different times of having to just really be on my knees in prayer and just asking God why or what is it that I can do, obviously definitely moments of sadness. I kind of had to work through that loss of that idea that I couldn't do that. And that if I did what the repercussions would be, not necessarily even just me dying, because I knew where I was going to go, but that would be leaving my husband having to raise a child on his own. That was definitely not something that I wished on anybody. Even though it was a difficult decision, it was a decision that I felt was the best. As I processed through it more and more, I realized there's a huge need out there for parents. There's a huge need for the kids that need a place to call home. They need a mother and a father [because they] don't have one. Or they don't have ones that are taking care of them properly. That was a need that we could fill. So, it actually gave me more hope and excitement knowing that, gosh, we could make a difference in these child's lives. When you give birth, you don't have a choice of what child you have and I imagine it must be this amazing blessing of this child that God has blessed you with and as a part of yourself. But when you adopt or you foster kids who you actually are making that choice to take in a child and to give them love and to give them a family. [You] help them know, that regardless of their circumstances or what may have happened to them or what they're feeling, that there are people that do love them and are going to be there for them and help them through life. In a weird kind of way, it actually ended up being a huge joyous thing to be able to be foster parents and to adopt these two boys. It's a huge blessing even though it started off as a feeling of almost a curse, but it wasn't. Now at this point, (I mean) it was only diagnosed two years ago, so there are so many medications out there. It's really a good time, per se, to be diagnosed, because there are so many different options out there. Whereas, if I had gotten pregnant earlier and had had the disease earlier, I don't know that I would have had that much hope at that point. There weren't that many choices of medication. In fact, when my dad was first diagnosed, there weren't any. There was just one that was experimental that he was able to get on. They gave him 13 years, which was awesome. But there was nothing for my youngest brother available at all. My middle brother, he caught the disease as a result of pneumonia. It onset and it hit too hard and there was nothing he could do. There weren't that many medications at that time either. I feel really thankful. I think it was the right decision, because I feel like I probably would have onset if I did get pregnant, which could have created all kinds of problems and not [be] as hopeful as it is now to have the disease. Everybody has their own feelings about it. For some women, that's been their dream of to be a mom and wanting to have their own child. Most people that I've talked to haven't really grieved through that process of loss of not being able to have that. They're so overwhelmed, because they're newly diagnosed that they can't see beyond that loss. I’ve tried to encourage them that with my story, just [to] say there are so many kids are out there, there's so many other possibilities. Yes it is a lot, but you could be helping so many other kids and your life could still be complete. You could have this child to have a complete family where you feel like you are a family, you have these children that you're able to love unconditionally and that love you back unconditionally. That that's still an option for you. I know other people that have also done this. They have adopted. It's been a process for them, but they're thankful too. I know a gal that she was actually really young when she was diagnosed. When she got to getting married and then an age where she wanted to have kids, she was already diagnosed. [She] knew that wasn't something she wanted to do because it could have actually terminated her life with a baby. So, it wasn't even an issue for her, because she had all those years to process it. She just immediately went after she got married into the adoption process. Now she has two little boys she's adopted. I think it's hard for some women to be able to let go of that dream of having your own child. I think when they do, look at a bigger picture and realize even though one dream might end, another one can begin. There are other doors that can be opened and other possibilities that your "traditional" family doesn't need to be a [naturally born] child. It could be one that you've adopted and still be an amazing family that way. I know this disease is hard and I know that there are challenges. I know that it's sad that you're not able to give birth naturally to a child and that's okay. It's okay to feel loss and it's okay to be sad about that. But don't stay there. Don't stay in that spot. Try to look at a bigger picture and to make the most of your life, including children. If that's something that you and your husband decide, that’s something you want to do. The need is great and you can be extremely blessed by going down that route. Don't give up hope. There are other things that you can do in life that can help you to feel fulfilled. My name is Michelle Sweitzer and I aware that I am rare. Learn more about pulmonary hypertension trials at . 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Episode 298 - Michelle Sweitzer
11/11/2019
Episode 298 - Michelle Sweitzer
In this episode, pulmonary hypertension patient, Michelle Sweitzer discusses her illness and adoption.
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Elsa Garza, DNP - phaware® interview 297
11/08/2019
Elsa Garza, DNP - phaware® interview 297
Elsa Garza, DPN specializes in Pulmonary Medicine and has sub-specialized in Pulmonary Hypertension since 2009. Caring for patients with Pulmonary Hypertension is her passion. In this episode, she discusses addressing anxiety or depression that may come with a PAH diagnosis. My name is Elsa Garza. I'm an acute care nurse practitioner. I currently work out of the pulmonary hypertension office in Chicagoland area. I actually started working with pulmonary hypertension patients many years ago when I first graduated as a nurse practitioner. I've been working with PH patients since then. These patients are very complex and they require a lot of attention in every aspect, not just in their workup, but also just getting to know them on a personal level and being able to develop a rapport and open communication with them so that you can actually talk to them as more of a human being and really help them understand what's going on. These patients many times are depressed and have a lot of anxiety, because they're worried about the medications and they're worried about side effects. A lot of these patients are younger, they're still working. They're worried about how they're going to fit in an inhaled therapy four times a day or walk around with an infusion pump, [while trying] to live out their normal daily life activities with friends and family and at work. It takes time for patients to open up to us. A lot of times the patients tell us that they're feeling anxious or they kind of start showing the signs of isolation. We open up the topic. We talk to them about how important it is to attend support groups. We talk to them about following up with their general practitioner to see if maybe there are any medical therapeutic options for them or if maybe counseling would be appropriate for them. All we can do is do our best to kind of hone in on everything that's going on with the patient and treat them completely overall, holistically, and hope that we will do a good job in doing so. It really starts with a good relationship and sometimes that in itself is very difficult to build with a patient, because maybe they're brand new to your practice. They're trying to figure out what kind of provider you are. Do they agree with what you're telling them? [Also], they're trying to digest all that information along with do I trust you, do I not trust you, should I get a second opinion? That really goes back to building a good relationship with them. Again, breaking down their barriers. Many patients, we may see that they're getting a little depressed or having some anxiety, but they're not ready to talk about it. I always tell patients we can only help them as much as they let us help them. Building a good relationship is so important. Typically, the first moment we see the patient, we give them their diagnosis and they go home and they start researching on the internet, and they come back on their follow-up and they say, "I read that this is a fatal diagnosis or that there is no cure and there's only a handful of therapeutic options that we can treat this disease with." The first thing that we say is, "You're right, number one, but number two, there are only a handful of medications that we can treat you with, but it's a lot more than we had 10, 20 years ago." So no, they do not cure this disease, but they definitely improve the quality of life, and that's really what's most important, is making sure that we're doing whatever we can do to improve your quality of life. Another trigger is typically going from an oral medication or an inhaled medication to something more advanced, whether it's continuous IV or subcutaneous infusions. Usually that just opens up another set of questions. Those are typically the two most common triggers that patients come back with, and it puts them in a very vulnerable position. It really opens up a deeper conversation. Another example would be when you're being evaluated for a lung transplant. If that is where we are in their disease progression, and we have to indulge into that conversation, we can see it in their face. [They may say], “You told me a couple of years ago I have this progressive pulmonary vascular disease and now you're telling me that I need a lung transplant and that that may be the best option for me?” It's a very sensitive subject obviously, but we tell them we have to be honest with you and we are really just looking at all options and it doesn't mean that they're a candidate right now or ever will be, but it's something that definitely needs to be addressed and should be addressed, because they have that right. We have to make sure that we do everything possible to help them, to treat them. That's why we're there. We're there to help them and to guide them, and ultimately it's the patient's decision. The patient and the caregiver or family, whoever they want include in their medical decision making helpers or the proxies or supporters. In summary, when we're dealing with patients with pulmonary arterial hypertension, there are a number of aspects that we look at when we're seeing these patients every day. It doesn't just include medications. It doesn't just include their results of recent exams or testing. It also includes addressing any anxiety or possible depression that may be coming into the picture with these patients, or even with their caregivers. Many times, we get lost in treating the patient, but caregivers need attention, as well. That's why I think support groups are incredibly important for everyone in the PAH picture. I'm Elsa Garza, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials #phawareMD @AMITAhealth
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Episode 297 - Elsa Garza, DNP
11/07/2019
Episode 297 - Elsa Garza, DNP
Elsa Garza, DPN specializes in Pulmonary Medicine and has sub-specialized in Pulmonary Hypertension since 2009. Caring for patients with Pulmonary Hypertension is her passion.
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Connie Siskowski, RN, PhD - phaware® interview 296
11/05/2019
Connie Siskowski, RN, PhD - phaware® interview 296
Connie Siskowski, RN, PhD from American Association of Caregiving Youth discusses youth caregivers who sacrifice their education, health, well-being and childhood to provide care for ill or disabled family members. My name is Dr. Connie Siskowski. I am the president and founder of the American Association of Caregiving Youth. I was one of those kids who cared for my grandfather between the ages of about 11 and 13 and never realized how much it impacted my life as an adult. My background is in health care through a broad spectrum; everything from teaching paramedics to hospice care. It was back in 1998, when I attended the first International Conference on Family Caregiving in London that I learned about the topic of quote unquote “young carers” and how much they aren't being recognized and supported. So all my life, different seeds were planted and ultimately I remarried and my husband encouraged me to go back to school to get my doctorate. I did so to have a voice for all family caregivers, never expecting to discover the extent of this population in our country, much less in our County. The national study which estimated that there are at least 1.3 million children, ages eight to 18 and this role was released in 2005. Our data here in Palm Beach County started in middle school, so the children that we work with are from ages about 11 on to their high school graduation. So that could be 18 or it could be a little older, depending on when they started in school. Every family is different. Every child's circumstance is different. Sometimes the roles are assigned. Other times, the kids step up to the plate. For example, one of our families, the parents and son moved in with the grandma who had dementia. Dad worked and mom was home. And the student’s bedroom was right next to his grandmother. So he and his grandmother developed a close relationship and he was always there for her when his mom was not available. Ultimately, his grandmother relied on him more than anyone else. We work in partnership with the school through our Caregiving Youth project to identify the kids and then work with them in school, as well as to do a home visit and provide opportunities for the kids to have fun. Because essentially, when you're in school, it's like if you're an adult and you're at work and you're worried about your loved one at home. It's hard to focus and the stress builds no matter what the health condition is. As a result of our home visit, have we found that respite would really be helpful so that both our student and his mom could have a break. They chose to have respite on Saturday morning, so that they could as a family, go and have breakfast together and have just a little time away to work on developing their own relationship and having some of their own needs met. Some of our kids take care of a grandparent, others may take care of a parent and it may be a parent who has cancer. It could be a parent with ALS or MS or even brutal diabetes. Or it could be a sibling. As we know that there are more and more kids who are on the autism spectrum. The siblings often participate in their care as well. We identify the kids beginning in sixth grade through an eligibility process and so about 70% of our kids come that way. Parents have the opportunity to opt out. Otherwise, the kids would participate in a screening. Then we do an analysis of that data to focus our limited resources on the kids in the top three of five levels of responsibility. That's determined by how much time they spend each week and the type of tasks that they do. So for a child who is providing personal care as opposed to somebody who's doing grocery shopping, the personal care gets a higher weight in the analysis. Then we meet with the kid to let them know about the program and invite them to join if they would like to. Then they get parental consent. Others are referred to us by school staff, because the kids may be coming in late. Recently, there was the child who was coming in late several mornings a week and we found out it was because she was a high school student who is driving her mom to dialysis prior to coming to school. The issues of caregiving youth are or nothing that's taught to nurses, to school staff to guidance counselors, even to community leaders. If a child comes in late, people may easily think, oh, they don't care. Or they just want extra attention. Or they may be acting out at school, because it's the only place that they can go release some of their frustration and anger. The four ways that we enroll students in our program is through the eligibility process, through school referrals, through other agency referral or through a student self-referring. One of the things that we do at school, is the lunch and learn where we focus on a specific diagnosis each month and bring in resources related to that. It's open to all students as well as school staff. Sometimes the kids may be hearing, like a while ago, a boy who had just been with his mom the night before who had had open heart surgery, heard our family specialist talking to somebody about diabetes and he's thinking "Oh my mom has diabetes." So he went up to the table and had lunch and learn and then ultimately came into our program. In addition, in schools, we do a skills building session in group of between six and ten kids at a time. We've developed a curriculum that we use so that it's consistent to school. We [also do] the lunch and learns. During that time of the home visit, we often find out that the kids don't have a computer at home or a laptop. Kids who are homeless or who are in foster care receive special supports, because they're recognized by the state, by the government, but not yet care giving youth. So, if they go to a Title 1 school, where there's many students who are on free and reduced lunch, there's the benefit of tutoring. Our kids often have to go right home. They can't stay after school. So tutoring is something that we also provide, because we want them to succeed academically, as well as personally. Then we do some fun activities, because often our kids don't have time to do be a kid and to have fun. So we might do a picnic or go to the beach. We have an overnight camp. We do some activities with families like a family holiday celebration. We also have been creative to do something like an overnight pajama party at a local bowling alley. The range is wide, but whatever it takes it for the kids to have fun. If you compare a caregiving youth against a non-care giving youth their levels of anxiety and depression are higher. However, with support we can turn that around. The kids often just step up to the plate or sometimes there's just nobody else. For example, we have more and more grandparents raising grandchildren and they were recognized way back in 2001. President Clinton signed the National Family Caregiver Support program. That included grandparents raising grandchildren, but there wasn't any forethought to what happens if the grandparent gets sick and particularly, if it's a single grandparent and then the role reverses. The same with a single parent households where sometimes there's nobody else. There may not be a sibling. With the mobility of our society, people are moving around and around so that they have a difficulty in their roles and have so many more responsibilities. Often there are younger siblings and they may help with homework, they may be doing the cooking to make sure that their siblings are fed as well as, becoming a parent. That's why these kids really, really need support. They're precious. One of the things is that in doing caregiving, this reciprocity. So it's not all bad. The kids that we work with are such good kids and they really learn compassion and empathy. Right now there's a national effort to teach children empathy and our kids could be the teachers. There was a study done of young adults who had dropped out of school and 22% said it was to care for family members. So in terms of the individual as well as society, it's really important to support this population so that they do graduate from high school and have a future beyond their caregiving role at home. Many of them want to go and cause some form of health care. We all know that with the aging of our population, this is the labor force that's really needed. That's another benefit to supporting them. What we have done in recent years, is worked a little more closely with the aging network and services, because a lot of them are age barriers. So once a caregiving youth turns 18, and depending who they care for, they may be eligible for some additional benefits of help and resources and we want to make sure that that continues while they're in college because that's a whole other population that has yet to be fully explored. College students who are caregiving. Colleges are starting to worry about college and completion and we believe that this is one of the contributing factors to that. Sometimes it's couched in financial needs. Even if a student drops out of high school, they may say that they have to go to work to help make money for their family. But people don't tend to look at, well what's going on in your family? Why you have to do that? Well, dad fell off the roof then he can't work any longer. So every family is different and they're just a myriad of circumstances. I would say that one of the other factors is that some of our community resources and collaborating partners may not have a full understanding of the impact of health situations on the whole family. This also carries over to the military. For example, I was talking to someone who headed a kinship care program and she was telling me how there was this grandmother who was taking care of three orphan grandchildren and going to dialysis three times a week and everything was okay. But anyone who's worked with anybody who's on dialysis knows of some of the impact, and being tired. You just knew that these kids were helping grandma in some way or worried because a lot of times when children are being raised by grandparents, they've already gone through one trauma. Always in the back of their mind is, what's going to happen to me? In terms of military families, the tendency has been to focus on the veterans. But what happens if that person comes home from either being deployed and now the spouse is sick or maybe grandma's moved in and now she's had a stroke and there's children in the home. The children wind up in a caregiving role. So at some point, I believe, our healthcare system has to become family centered. If you would like to learn more, we have the website, which is simply (American Association of Caregiving Youth). My name is Dr. Connie Siskowski, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials @AACYorg
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Episode 296 - Connie Siskowski, RN, PhD
11/04/2019
Episode 296 - Connie Siskowski, RN, PhD
Connie Siskowski, RN, PhD from American Association of Caregiving Youth discusses youth caregivers who sacrifice their education, health, well-being and childhood to provide care for ill or disabled family members.
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Michael Keller, MD - phaware® interview 295
11/01/2019
Michael Keller, MD - phaware® interview 295
Michael Keller, MD is a Pulmonologist and Pulmonary Hypertension specialist with Suburban Lung Associates in Elk Grove Village, IL. He is medical director of the AMITA Health Pulmonary Hypertension program. In this episode, Dr. Keller discusses the importance of exercise in Pulmonary Hypertension. His clinical interests include the full spectrum of pulmonary vascular diseases with a focus on pulmonary hypertension in chronic lung disease. He serves as medical director of the Alexian Brothers Pulmonary Rehabilitation Program. I am Michael Keller. I am a pulmonologist with Suburban Lung Associates with AMITA Health in the northwest suburbs of Chicago. Today, I'd like to spend some time talking about the role of exercise in pulmonary hypertension. We know the benefits of regular physical activity are tremendous for all people. We all recognize that there's a significant cardiovascular benefit. Regular exercise can reduce the risk of stroke, of developing diabetes, of developing high blood pressure. There's also significant benefit in reducing the risk of certain cancers like colon cancer. We know regular exercise can help improve people's mood and reduce feelings of depression and anxiety. So there's many benefits for healthy patients and patients with different cardiovascular diseases. When we look at the body's response to exercise in the setting of a normal heart, we see that that heart squeezes out up to four to five times as much blood during a state of exercise compared to when it's at rest. The reason it needs to do that is to deliver extra oxygen to the exercising skeletal muscles. We know that the blood pressure increases to help facilitate this, as well. When you look at the blood flow through the pulmonary arteries in the lung, when the heart is pushing out more blood to accommodate the exercising muscles, the resistance to blood flow in those arteries in the lungs actually decreases to help facilitate that, and it leads to only a mild increase in blood pressure in the lungs during exercise. When you look at a patient with pulmonary arterial hypertension, the underlying problem is an increased resistance to blood flow in the arteries in the lungs. When you begin to exercise, and your muscles demand more oxygen, and your heart wants to pump out more blood, you become limited by that increased resistance to blood flow through the lungs. The right side of the heart is not able to get as much blood through the lungs to the left side of the heart, and to allow the left side of the heart to then pump that blood out to your muscles, which leads to the symptoms of shortness of breath with activity or with exercise. At a more advanced state, can even lead to symptoms of lightheadedness with exercise or chest pain. So there is another limitation in patients with pulmonary arterial hypertension, in that we know their muscles, their skeletal muscles, the big muscles in their legs, in their arms, do not function as well as somebody without pulmonary arterial hypertension. This has been looked at by taking small biopsies of those muscles. When you compare them, the muscles in a patient with pulmonary arterial hypertension are generally more atrophied and smaller. Their contractile strength is not as strong. There's a lower number of the muscle fibers that we use for endurance activities. We also know that these muscles tend to change the way they process energy, and they start making acid that builds up in your blood sooner than a patient without pulmonary arterial hypertension. If we look at our current recommended treatment algorithm for patients with pulmonary arterial hypertension, a supervised exercise training is a recommendation for all patients. There used to be concern years ago that exercise for patients with pulmonary arterial hypertension could potentially be dangerous. It's really been over the recent years that we've recognized it's not only safe, but has significant clinical benefit as well. There've been a number of small studies, usually only 20 to 30 patients, looking at the role of exercise in pulmonary arterial hypertension or in patients with CTEPH. There was a group that took all of these small studies and put them together in a systematic review, and [they] came to the conclusion that in patients who are stable on their current medication, a supervised exercise training protocol leads to a significant improvement in their six-minute walk distance. The amount that that walk distance improved is actually greater than nearly every single trial of a drug that led to an approved new therapy. It showed that the muscles were able to uptake more oxygen after an exercise training program. What patients care about most is that it led to an improved quality of life. Importantly, there were no major adverse events. In a stable patient undergoing a supervised program, we've learned that it's safe, that exercise is not dangerous. There was also another study done, in which they actually measured the function of the heart and the resistance to blood flow through the lungs before exercise and after a supervised exercise program. This protocol was fairly intense, and that the patients were in the hospital for the first three weeks, and then followed at home with a continued exercise program for another 12 weeks. Again, all of these patients were stable on their therapies, between one to three different drugs. What they showed was that at the end of that exercise training program that the heart was able to pump out a significant more amount of blood. The resistance to blood flow through the arteries in the lungs had reduced. This led to a reduction in the mean pulmonary artery pressure in the lungs as well. Additional evidence of the significant benefits of exercising and improving the condition of the skeletal muscles, not only on how patients feel and their quality of life, but actually on how the heart and lung function. Although we've shown over recent years that exercise can be safe and effective adjunctive therapy on pulmonary arterial hypertension, we do still approach each patient individually and with caution. Again, before anyone begins any type of exercise routine, they really need to talk with their PH care team to understand what is safe for them and what sort of limitations they should have in place. Some general limitations that we talk about are not over exerting yourself. I often tell my patients to follow, they listen to their bodies. We recommend sort of a symptom limited exercise program. Then if any of these concerning symptoms, such as lightheadedness, chest pain, racing heart, or excessive shortness of breath develop that that's a little too intense of a regimen. They need to take a break and slow it down. We generally recommend avoiding very strenuous exercise, such as heavy lifting, that requires you to really bear down, or again, anything that involves a very intense aerobic exercise routine. One other caution that we often bring up is to avoid exercises that involve significant movement of both your arms and your legs at the same time, because the amount of extra demand on the heart that that will create. I generally recommend an aerobic exercise training program that can involve simple walking, working on a treadmill, exercise bike, an elliptical, other forms of exercise such as yoga, Pilates. Swimming can be safe and effective as well, as well as light resistance training. Again, we recommend avoiding heavy lifting that results in you really holding your breath and bearing down, but light resistance training can help improve your muscle strength and endurance, as well. How this can be accomplished is coming up with a program that you can do on your own, or if you have access to a fitness center, or a gym where you can exercise. There are some formal exercise programs, as well, that can be offered through a hospital setting, which is a pulmonary rehab or a cardiac rehab program. The limitation for pulmonary hypertension patients is that pulmonary hypertension is currently not a diagnosis that is covered by Medicare and many private insurances for completing a pulmonary rehabilitation program. Some of our patients who have comorbid or other lung diseases, such as COPD, we may be able to qualify. Some of our patients have abnormalities on their pulmonary function test that will allow them to qualify under their Medicare guidelines, although they don't have a diagnosis of COPD or another lung disease. These formal pulmonary rehabilitation programs are nice because they are supervised by trained nurses and therapists. In addition to the exercise routine, they generally include some group education as well, which focuses on nutrition, oxygen therapy. You're meeting other patients with pulmonary hypertension, other lung diseases, and it ends up being a good support group, as well. In summary, we know now that exercise is safe for pulmonary arterial hypertension patients when done with certain limitations in place. They can lead to significant benefit in how patients feel, even in improvements in the function of their heart. Again, I'll stress the importance of being sure that everyone talk with their treating care team before beginning an exercise routine, so they do know what is safe for them and what sort of limitations they should include in their exercise program. My name is Dr. Michael Keller, and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials #phawareMD
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Episode 295 - Michael Keller, MD
10/31/2019
Episode 295 - Michael Keller, MD
Michael Keller, MD is a Pulmonologist and Pulmonary Hypertension specialist with Suburban Lung Associates in Elk Grove Village, IL. He is medical director of the AMITA Health Pulmonary Hypertension program.
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