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

I'm Aware That I'm Rare: the phaware® podcast is devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. Through this series of impactful, insightful and, most importantly, hopeful stories from members of the global pulmonary hypertension community, we hope to further the global #phaware conversation as well as to capture, engage and enable misdiagnosed and undiagnosed PH patients because early diagnosis and treatment can mean the difference between life and death. Topics range from the importance of early diagnosis and global PH awareness, to the impact of clinical trials to how patient support and advocacy are paramount to battling this disease. Learn more about PH at www.phaware.global

Kayla Dunlop - phaware® interview 363 11/23/2021

Kayla Dunlop - phaware® interview 363 Kayla Dunlop is a pulmonary hypertension patient from Southern California. In this episode, Kayla discusses being born with a rare condition called giant omphalocele (GO), the challenge of adjusting to life on oxygen 24/7, and how she is using her creative spirt to make jewelry to raise funds and awareness for pulmonary hypertension. Hi, my name is Kayla Dunlop, and I'm 28 years old. I'm from Southern California, and I'm a PH patient myself. My childhood was different because I was born with actually a rare condition besides PH, that was later on, and it was called giant omphalocele (GO). So that's where all your organs are outside of you in a jelly sac, and they had to push it all back in. So that caused a lot of health issues throughout my childhood, like club foot, ASD, those were just the major ones, scoliosis, so I have rods in my back. So all this stuff, they had to correct throughout my childhood. So I was in and out of the hospital all the way probably until middle school. Then when it hit middle school, is where I had a seven year gap of hardly any medical issues. If there were, they were very small that I could deal with day to day. But when it hit August 2013, it was when I got diagnosed with pulmonary hypertension. Then that's where it all began, my PH story. It was hard. I'm very thankful that I had great family support. My family has always been close, and probably because of my medical issues, we became closer because we had to stick together. I have a younger brother, three years younger. He actually had to wait in the hospital from time to time while my parents were visiting me. So it was like we were all like a support system for each other, for which I'm thankful. My friends, they come and go, but I did have some friends throughout my childhood that stood by my side, so that helped a lot. I always had a positive outlook on life. Probably, I get it from my parents, that you kind of just have to learn to try to adapt and push through. That was always my mentality, that I need to get through this because I need to do something next, the next stage. So that's how I thought, and always had a positive outlook, and that helped a lot too. They don't know how or why I got pulmonary hypertension. I have kind of my own theories because I have restrictive lung disease from the scoliosis, so I have one lung larger than the other. So I'm thinking maybe that could've caused it, but they never really said why I have PH. How it all started was when I was walking in the mall one day, it was a hot day, and I kind of get overheated easily anyways, so I was thinking, "Oh, maybe I didn't drink enough water or anything like that." But in the mall, I was walking around with my mom. And all of a sudden, I got shortness of breath, like I couldn't get a grasp on my breath. I've never felt that before in my lifetime, so that kind of scared me. I was like, "Maybe I'm hungry, thirsty." So I sit down. It got a little better, and I was able to walk back to the car, and so that was fine. Then that night, I actually woke up in that morning, gasping for air again. I've never had that happen to me while I was sleeping. My mom was like, "Something's wrong. We need to go to the doctors." My mom's very hyper aware of that kind of stuff because of my medical issues. So she was like, "I'm going to take you to your primary doctor and we're going to get this solved." And this is at age 20. So we go to the primary doctor, and they're like, "Oh, you have low oxygen stats," and they're like ... But I'm talking, I'm walking, didn't really have shortness of breath, but it was just like trying to catch my breath a little bit, but not as bad as I felt like before. But they're like, "No, you're in the 60s. The oxygen stat's in the 60s." They rushed me to the ER in an ambulance. But I was still walking, talking, which was really weird. So I get to the hospital, and they think it's an asthma attack. So they're giving me all these breathing treatments, and the nurse is like, "This is not working." They don't know what's going on. They're like, "It's not helping you at all." So the doctor that was on call there is like, "Well, we're not sending you home until we know exactly what's going on." So I was there in the hospital for about a week, doing all sorts of different tests to kind of narrow down what it was. Then they did an echo cardiogram, and they saw some of the pressures were higher in my heart. Then to finalize what they suspected was PH, they did the right heart catheter, and that determined that I have pulmonary hypertension, and they put me on oxygen. They sent me home with oxygen. Ever since then, I've been on oxygen 24/7. At first, I really didn't understand the severity of it. I thought maybe I would be on oxygen, oh, maybe a week or two, and the medicine would kind of solve the issues. You know? So I was like, "I'll just be back to normal," kind of thing, because I never heard of pulmonary hypertension before, or what it was, anything like that. I was kind of in denial, which I've never really been since the other medical issues because they were kind of straightforward. This was kind of more confusing of how to deal with it. So I kept asking my parents after I got out of the hospital, I kept asking my parents, "Is this really happening to me? What is this?" I had to keep asking them, "Why do I have this? What's happening? I thought my health issues subsided already. Why am I dealing with this again, another thing?" So it did take a toll on me, for probably I would say about a month of trying to get adjusted to it, because I was on oxygen. I had to go back to college, and I knew if I stopped that and waited maybe next semester to go back and kind of get it adjusted, I knew that I wouldn't go back, probably because for myself, I have to keep reaching those goals. Even if they have to change a little. So it just helped to kind of get back in that normal routine, even though I had to change it because I had to drop two classes out of three. I had to be on oxygen 24/7. I had to carry the portable oxygen concentrator with me. Thankfully, my parents helped me pay for that because the insurance would take forever to get one. It was just a whole new way of life of looking at it, and especially in my 20s, because I thought all the health issues were kind of over with. The hard part was kind of probably being around my peers, because I felt different. So I had to gain my confidence back a little bit of, yeah, I am different, but I still have the right to be in school, and I still have the right to accomplish my goals. So that took a little time to kind of get over that. Also, the noise of the oxygen machine kind of bugged me at first, because it was a little bit louder and something that's always with you, so in those quiet situations, like when the professors would give lectures and people are super quiet, or test time, I kind of felt a little self-conscious. So it did take time to kind of get used to that. By keeping going to school, it did help, and be in those different types of situations helped me kind of overcome that over time. It did take time. I still have some instances of going out in public and be like, "Oh, people are staring," or stuff like that with the oxygen because you can't hide the oxygen. It's right there. It was just a learning curve for sure. I would say my self-confidence has gotten a lot better over time with the support and just living with it, basically. I would say about six months after, I wanted to kind of turn the negative into a positive and give back to the PH community, so I decided to start a small jewelry fundraising line. I wanted to spread the awareness and raise funds for the PHA, since I knew that was an organization that funded research. So I started that, I went to some craft fairs, tried to spread the awareness about PH. And I met a lot of great people along the way. They were open to share their own stories. I actually did meet a couple people that were touched by PH too. So it was very interesting to go through all that, and know that I'm kind of doing my part on trying to give back to the PH community in some way. So that has really helped too, because it is a creative outlet to make the jewelry. I know it's raising funds and giving awareness. Now I'm at Cal State San Bernardino, so I'm getting my BA in child development. I got my AA in early childhood, and now I'm doing my BA in child development, so I have about a year left of that, so I'm really happy about that. It's been a long road, but it's well worth it, so that's what I'm focusing on now. I'm really happy that I'll be accomplishing one of those big goals of mine that I've set for myself way before having PH. One thing is of course, is to try to have that support system with you. I know that's hard for some people to have that. So if you do have to reach out and create your own support system, I would advise you to join your local PH support group. I did that right after I got diagnosed. My doctor has been part of it, my PH specialist. I started with them right after I got diagnosed, and I've been involved ever since. I help run the activities for them, so the PH patients can have a creative outlet and not always focus on the medical side of it. I really enjoy that, and that's where some of my funds go from the jewelry line too, to help fund the activities. I would definitely say try to join your local PH support group and see if that's the right fit for you, because it does create a good community for you to have people that understand what you're going through. Also, I would say look for Facebook PH support groups online. Those have helped a lot. I met a lot of young PH patients through that, that I still keep into touch with today. You can get really good advice from them, and it's been a really great part of my support system too. Also, try to find a hobby that you enjoy, because then that kind of takes your mind off of the medical condition that you have. It kind of just clears your mind, and try to make sure you focus on something fun that you're doing. Also, if you have a chance, I started with pulmonary rehab, and that has really taught me how to have a good exercise routine to exercise safely, because they teach you how to do that. I did that right after I got diagnosed too because my dad actually researched on what you can do to help have better heart and lung functions. So he actually found out about pulmonary rehab, that they offered that. Then I asked my doctor about that, and he signed me up for it. That was a really good program because now I can do the treadmill on my own, and make sure I do that safely, and then walk around my neighborhood. Any exercise helps too. If it's little, whatever works for you, it helps a lot to keep your body moving. My name is Kayla Dunlop, 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 /episode/index/show/phawarepodcast/id/20843549

Episode 363 - Kayla Dunlop 11/22/2021

Doug Evans - phaware® interview 362 11/16/2021

Doug Evans - phaware® interview 362 Doug Evans is a Pulmonary Hypertension patient from Florida. In this episode, Doug discusses his time in the military as a bomb loader, why he had to move from Colorado to lower altitude due to his PH, and how his treadmill and passion for Team PHenomenal Hope helped him lose over 80 pounds by simply walking. My name is Doug Evans. I'm a 20-year veteran of the United States Air Force and retired from Comcast as an engineer. In late 2014, I was diagnosed with pulmonary hypertension. Now I’m living in Florida where I no longer have to wear oxygen, which if your doctor says to move to a lower altitude, by all means, if you can do it, go. It was a lifesaver. I'm originally from western New York near Buffalo. In the mid-'70s, there was actually no jobs in the western New York area. So myself and almost all my buddies from high school enlisted in the military. I enlisted in the Air Force and ended up loading bombs for a living. It's one of those jobs that very few people volunteer for. You end up getting assigned to it, because of a lack of manning. I ended up actually enjoying the job once I learned it. I spent 20 years loading bombs. I was in Germany, Italy, Spain, Turkey, stationed in North Dakota on B-52s for quite a long time. Korea, Guam, Hawaii, New Mexico, where I finally retired. Imagine my shock when I discovered that there were very few airlines hiring bomb loaders. So I had retired and rejoined my family in North Dakota, and it was getting near Christmas time and there was an opportunity to work for Fisher-Price toys as a computer technician. They were going to provide all the training that you would need since Fisher-Price is originally from my hometown area, I said, "What the heck, I’ll give it a try." It was some interesting computer products for little kids, and I was doing phone support for them. They were really good products. We got very few calls. So I hung out with other contracts that were in the same building, the same call center, and one of them was Gateway computers. I would sit in on their training, and I probably got more training on Gateway than the Gateway technicians. I ended up working there and ended up a senior lead, and it just kept compiling. I would move to a different contract, and eventually when that call center closed, I ended up moving back to the western New York area and ended up working for Adelphia cable. After Adelphia cable went bankrupt one winter, we had seven feet of snow in one storm, and I was really not happy with all that shoveling. A friend called up from Denver and said, "Hey, do you want a job with Comcast here in Denver? They'll move you." I was there before she could hang up the phone. I remained in Denver for close to 15 years until I got laid off, and I had enough time in to convert that to retirement. By that time, I had already contracted pulmonary hypertension. I was stationed in Denver in the early part of my career in the Air Force. I noticed the altitude did affect the human body, because the altitude in Buffalo is like a hundred feet and you'd go up to 50 to 80 feet, you've run out of breath really quickly. In the Air Force they would... For the first two weeks when you stationed there, you'd just do a lot of marching and walking to get used to the altitude. I eventually did get used to it. So when I moved back, I expected that, and it was just a lot harder to get used to it. By this time, my folks had moved to Florida. Whenever I would go visit them, of course, in Florida, I'd feel great. Then going back to Denver after a week or two vacation, it was really, really tough to get used to it. I lived on the second floor of an apartment building and climbing those stairs was just awful, and it just slowly got worse. One day I had to move a server in my workspace from a cubicle to the warehouse. It wasn't a big distance and it wasn't real heavy servers, so I just picked it up and carried it. By the time I got to the storage area, I couldn't breathe. Well, I could breathe physically. I could feel the air going in and out of my lungs, but it was like it wasn't doing any good. I remember my boss just staring at me saying, "What's wrong with you?" I said, "I don't know. I'm just not breathing right. Something's wrong." Eventually, one day not long after that, we had a big family occasion at the house for the holidays. I moved from the kitchen, walked through the house to the living room, and I almost didn't make it. I was so out of breath. When I got to my recliner and I sat down and I bent over and I'm trying to get my breath. I was almost ready to faint. My wife says, "You okay? Do you want to go to a hospital?" And I said, "Yes." And she thought I was going to die because I never want to go see a doctor. So yeah, she really freaked out. She almost called an ambulance. That's the day I found out I had pulmonary hypertension. I ended up in the emergency room at the local hospital, and they also found a congestive heart failure, which has never reoccurred. That was like a one-time thing. I went through all kinds of stress tests. Boy, I never want to do some of those again. They punch you full of chemicals and you just suddenly feel like you've ran a dozen miles. All kinds of CT scans. They said, "Yeah, you got pulmonary hypertension." I said, "What's that?" "Ah, it's a condition." That's about it. At the same time, my wife was going to National Jewish Health, one of the top respiratory hospitals in the nation. She has asthma. So she set me up with an appointment with her doctor, Dr. Amy Olson. Went to see her for what they suspected was sleep apnea. I also signed up for their pulmonary hypertension clinic. When Dr. M. Patricia George took over, that's when I finally started to understand what was going on. She really took a lot of time to sit down and explain to me exactly what was happening. She was really a blessing to have in my life. My recovery, if we want to call that, really started when we got a treadmill for Christmas. I had more fun putting it together than I really thought I'd ever have fun with a model kit. It was a really nice treadmill, and you could program a route anywhere in the world, and it would show you through Google Street View where you're walking. I just started walking. I programmed my old route I walked to school, and I was really upset that I couldn't do that. So I kept practicing over and over until I could walk that three-quarters of a mile to school. I had a high output oxygen concentrator, and I turned it up higher than my prescription said, and that really helped. I'm going longer and longer routes. At this time, it was only a year ago that the real estate market in Colorado was just going crazy. People were buying houses as fast as they could get them. The equity in my house shot up through the roof. I remember at one time, Dr. George said, "It would be good if you move to a lower altitude." My wife and I talked about it, and the friend who got me the job in Colorado was now living in Florida. She and my wife were very good friends, and we decided let's move to that area. We ended up finding a real nice house in Hilliard, Florida. The whole time I'm on the treadmill. I'm keeping track of every workout, and I was over 300 miles total. That was only about five months. I just started out just a little bit every day, and just tried to add a little bit more with every workout, if I only added five minutes. In pulmonary hypertension, we have these bad days where we we're just exhausted, and they just come out of the blue. I did everything I could to work on those days. I call them crash days. We eventually just said, "The heck with it." We got a good price on our house. We packed up and moved. We couldn't take the treadmill with, so I got down to Florida and I just started walking on the streets, and it's so much easier to breathe down here. I went to a local pulmonologist. She said, "Yeah, you don't need to wear oxygen in the daytime. You're responding so well to the lower altitude." So I started walking maybe a mile a day outside. It's so much different than a treadmill. I just kept increasing every day, a little bit more, a little bit more. I found that those crash days come further and further apart, and sometimes they're not as bad as others. So that's a big plus. When I got moving, I found that I didn't have those days where the exhaustion just overwhelmed me. I track every step I take. I got a smartwatch and a smartphone, and that does a great job tracking your activities and stuff like that. For 2020, including the time on the treadmill and everything, I logged 1,141 miles. In 2021, I'm still going. I'm up to five miles a day. Now, I do have back issues and hip issues from my service. That really kind of limits me to five miles. The arthritis starts kicking in. So I will try to walk a second walk in the evening, to extend that five miles daily. As the weather gets better down here, I'll be doing that more often. The one thing I'd say is just get up and move. I know it's difficult, especially on those exhausting days, but get a phone or something where you can play music, put on your headsets and just go for relaxing walk. For me, it was the best thing I can do, and I hope everyone can give it a try. If you can't go outside, if you're stuck up in the north, see if you can find a treadmill. Don't set out to walk a thousand miles. Set out to walk a mile. Set out to walk a half mile, and then tomorrow just do an extra 20 feet. But yeah, it really helps. I've lost 80 pounds just walking, no real major change in my diet other than I'm doing a lot better on portion control now. When you get pulmonary hypertension and you get those days you're so exhausted, the only thing you can do is watch TV and eat, at least for me. I gained a lot of weight and I'm sure a lot of folks in my position do. It not only helped me, it helped my dogs. My dogs were getting really chubby too. So I walked them with me. I was walking every day, a little bit more each day. I never really concentrated on doing it faster, but I did concentrate on doing it better, trying to keep the cadence the same and always talking to my doctor about what I'm up to. Not only am I talking to my doctors, I'm talking to my past doctors. I'm always in contact with Dr. George back in Denver. I mentioned to her what I was up to, and she said, "Well, the Team PHenomenal Hope is having a Tour of PHenomenal Hope. It's going to be like a bicycle race. It's set up in stages, but you can walk, you can ride a bike, you can swim. You just track all your activities on this free application called Strava. I joined up as an athlete. I didn't really tell anybody except Dr. George that I was a PH patient. Being retired, I could put in as much time as I wanted. That was one of the things that we're measuring was total time. I was putting in about almost 15 hours a week, just walking, occasionally riding a bike, not too often, because it is getting to be winter down here. That put me in the lead, and I wonderful for the total time invested. Then, that's when I told the organizer that I was a PH patient, and she was just ecstatic. And next thing I know I'm doing a podcast. My plan for 2021 is to try to get as many of my brothers and sisters doing this. I would like to see the number of patients outnumber the number of doctors in 2021's Tour of PHenomenal Hope. We don't have to win. We just have to be there. Go for a walk, 20 minutes a day. Just let them know that we are trying. In my personal life, my wife has been injured, her mom has been injured, so I am now the caregiver in the house. That's going to eat up a lot of time, but I'm going to continue to walk. I want to spend more time on my bike, and I want to spend as much time as I can encouraging my PH brothers and sisters that we don't have to sit down and take this. We can actually physically do something. My name is Doug Evans, 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 /episode/index/show/phawarepodcast/id/20843573

Episode 362 - Doug Evans 11/15/2021

Derek Henderson - phaware® interview 361 11/09/2021

Derek Henderson - phaware® interview 361 Derek Henderson is a #CTEPH patient from Toronto, Canada. In this episode Derek discusses is road to diagnosis with a rare form of pulmonary hypertension, the mental challenge of PTE surgery and the misconceptions of Canada's socialized medicine program. My name is Derek Henderson. I live in Toronto, Ontario, Canada. I'm a CTEPH patient (Chronic Thromboembolic Pulmonary Hypertension). I've had CTEPH since 2013. I'm an avid biker. I bike a lot when the weather's better. What ended up happening was that I just was losing my breath. My bike routes times were going down. Eventually, I was diagnosed with pneumonia, twice. A couple years passed after I was diagnosed with pneumonia. Then it started happening again. I went back and they did some more tests. They found it wasn't pneumonia this time, and it just kept getting worse and worse and worse. Eventually, it got to the point where I couldn't walk the length of my house, which is about 100 feet. So, that's when I went to the hospital and I was diagnosed with blood clots, and then eventually I became diagnosed with CTEPH. Initially, I was just in the hospital with blood thinners, and then I got out, and then they gradually cleared up the initial problem. Then, in Toronto, I was referred to Toronto General, to the Pulmonary Hypertension Clinic, and eventually they just ran some tests. At the time, the policy in Toronto had changed from the PTE surgery (Pulmonary Thromboendarterectomy) from going to be curative or done when there was an emergent problem, to being more preventative. So, I was one of the first patients that the policy effected. So I was done before it was needed to be done. In other words, they did it to prevent future problems. So, I was lucky in that regard. What ended up happening with me is that it went for about seven months, between the time I was diagnosed to the time I had PTE surgery. So, it was fairly fast. For me, part of the thing was that, when you're going through PTE surgery, you have to do a bunch of tests to make sure that you're doing okay for it, that you can live through it in effect. There'd been a bit of a gap between my initial review at the clinic and then the actual intake towards the surgery. I got a call that I was scheduled for a bunch of tests, which was routine, normal stuff. Then I got an email saying that, "We'd like to come to talk to you about the date of your scheduled surgery." The email was on a Monday. I had a right heart catheter test to measure the pressures in my right heart on the Tuesday. So I got the email on a Monday. On a Tuesday, my wife and I went in to talk to the surgeon about the surgery. And at the time we didn't even know we were for sure, scheduled for PTE surgery at all. I found out on Tuesday that I was going in the following Monday for surgery. It ended up that I really did not have a lot of time to get ready for it, nor did my wife or my family members have any warning for it. It was a bit of a surprise, but in some ways that might have been a bit better for me, because I didn't have time to think about it. I didn't really realize how serious the problem was until afterwards. So in other words, we had the surgery and for those of you who don't know, it's open heart surgery. You're put into hypothermia, they turn off the heart lung machine, they dissect some of the lungs. So it's pretty dramatic surgery. I didn't have really have time to look into it that much before we had it. I didn't really realize how sick and how serious the problem was until afterwards. I think that might've been a bit of a blessing in disguise because I wasn't quite as freaked out as I would have been. What I found later on was that I had surgery in August and then in December, the clots came back, because my blood thinner medication wasn't working that well. Then I had time to think and sink in a bit about what had happened, what the problem was, how bad the surgery was, how serious the problem was. It was sort of a delayed reaction to me just because I was so focused on recovering from the surgery and dealing with all the side effects of like I had lost some jobs, my employment had to come back, all that sort of stuff. Then I finally had some time to realize, "Well, I was really, really sick." It's quite a bit of a change, especially for someone like me who's really never been that sick. There was a lot of appointments. You have to understand too, that I wasn't that sick. I didn't feel sick before I went into surgery. I live about five miles from the hospital. I could have easily biked or walked to the hospital before surgery. So to come back out from that surgery and really not be able to walk around my own house, and I got better fairly quick, but still to come back out from that aspect of it, was a bit of a shock, took a bit of getting used to, just to. I had to measure my trips up and down stairs. Also people would tell me that, "Oh, you don't really look sick." Well, I was trying to cross some streets and people were on their cars at me saying I wasn't walking fast enough. I was just being lazy. To go from being able to bike 15 miles to being able to barely walk around your house was quite a bit of a shock for the surgery. What I would tell people is just to first of all, to reach out to someone that's had it before. Your program and others that are around now are a lot better to give some people some advice as to what you can expect from the surgery. A lot of people don't tell you to hug your pillow after your surgery, which is a great thing for heart patients. To try to stay active. Just try to start walking as much as you can, and to get involved with your physician and just to learn about what's going to happen, what the plan is. Actually even like when you're recovering from this surgery to have a plan of something to do, to keep yourself occupied, that's the big thing. Also to let your friends know what's going on, what it's like to have the surgery and get them to, if you need help, or if your caregiver needs help to let them know what's going on. With pulmonary hypertension, there's no fear involved when you hear the diagnosis. With cancer there is. So if you hear somebody who has cancer, oh, there's a fear involved. With pulmonary hypertension there isn't. It can be just as bad. It can be just as traumatic. It can be just as deadly. So you have to do some education to your family and friends as to what's going on with it. For me, it took a few years to completely recover after the surgery. I think part of it is I was a little reluctant to push myself physically. Certainly now you probably couldn't tell that I ever had that surgery. I’ve become a lifelong patient of blood thinners. That's one thing for me. My life is pretty well back to normal, but I don't take things for granted. Obviously we're recording this COVID world. So not able to do as much traveling as I normally would, but I travel a fair bit during non COVID times. But I also don't think things for granted anymore either. I pay attention to it, if I'm sick of it, I get it seen quicker than I used to, which is probably one of the bigger things that has changed for me. Otherwise I've been lucky. I've been able to recover back to my normal lifestyle that I had before. The biggest thing though, that I would say is that there's a misconception for people that Canadian healthcare, everything's paid for. I spend now $1,300 a month on drugs. My employer pays a fair bit of it because I have a good drug plan, but that's a lifelong payment for me, unless my drug therapies change. That's $1300 a month. That's a fair bit of money that you have to spend that someone else doesn't. So that's something that I never would've thought of before having this problem. I'm Derek Henderson 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 /episode/index/show/phawarepodcast/id/20843594

Episode 361 - Derek Henderson 11/08/2021

Kelly Lynch - phaware® interview 360 11/02/2021

Kelly Lynch - phaware® interview 360 My name is Kelly Lynch. I live in San Francisco. My rareness doesn't really start with PH. PH is probably lesser of the rare things for me, because I was supposed to be a conjoined twin and the other baby just didn't develop so I ended up with, some might call it a parasitic twin situation, where it was just kind of parts and bits and pieces. Essentially, the main thing that I ended up with is an extra spine, so it's like double-helixed. It kind of created this hump which is now hunching me over, which over time has contributed to the PH. So, I've actually probably had PH my whole life, but not to the degree where it was very severe or known. When I was younger they actually did quite a few surgeries on me, they did a series of three in one year to keep me more upright, because they had an idea that it was going to affect my breathing severely, so they wanted to keep my spine and everything as upright as possible. So they put a halo on my head and attached weights to it and then they took ribs and tried to make kind of a buttress to brace it up, like an internal brace. But that didn't work. Eventually the bones just dissolved. But a bone in my back fused together and made the whole thing just miraculously work out for quite a while. I did have several surgeries, they did remove a third arm that I had growing at the back of my neck that wasn't really useful for anything, it was just kind of there. There's only one photo of that. My mom offered to throw it away, she didn't know if I'd want to see it and I was like, "Yeah, I want to see it." I honestly wish I could have kept the arm. I'm a bit into my freaky self now. As I got older I started fascinating my own self. I would visit my family in Ireland every once in a while and the last time I had gone back I was walking around with friends and I couldn't get more than six steps without running out of breath and I was like, "This is bizarre, this is even worse than I normally am." My heart would just start racing. Eventually I just started using a wheelchair and my friends were actually quite worried about me at that time. I had to have people's hands to hold their hands when I was walking to get my arms off my chest. That gave me a little bit of leverage. I didn't know what was going on, honestly and the racing of my heart really freaked me out. I have a friend who's a cardiologist in Philadelphia. I wrote him first and I said, "I don't think I'm having a heart attack", because it wouldn't keep going for days like that. But I said, "This is kind of bizarre." And he was like, "Yeah, I would definitely go in to check on that." He was basically explaining that sometimes clinic doesn't know how sick you actually are, so just go to the urgent care, or something. I think I ended up going into the pulmonary hypertension cardiologist at UCSF at one point. They had a department just for that. That's when I got diagnosed. It took me a while to get around to this because when you hear that it's life shortening. As far as I knew nothing that I had was terminal in terms of my body shape and my conjoined twin, my scoliosis and all that. So when you hear that you do have a terminal disease, not knowing what the actual truth is in terms of life expectancy, because you're reading things on Google and you're talking to your doctors and you get 50 million answers. It's very, very depressing at first, or it was for me anyway. I'm not going to lie, I'm very happy now but I was in a dark spot for a while because one, I had this image that everybody around me would be there and they surround your bedside like in a movie. They're all there for you doing anything you want because you're about to die. I felt like a lot of people were just going about their lives and I was stuck home, because I couldn't get around as well. People don't like to call these days, it's all about texting and I hated texting. So I was just like, "I'm asking you for the courtesy of a FaceTime or a call and you can't even give me that and you don't know when I'm going to die?" I definitely inflated it way more in my head at the time and I actually had to seek counseling, because I just didn't know what to do with it all. They basically said that it gets harder for people that love you more, so the people you thought would be closer to you might retreat. I had to explain to people in general, it's like when someone is ill it's not about you, it's about them, you need to be there whether it's hard for you or not, because that person feels extremely alone. That's part of what I started my TikTok account about because I wanted that message to get out there. It's not about saying the wrong thing to the person. People give lots of dumb advice to people when they don't know what their body is, like, "Oh, you should eat celery." It's not about that, it's just sure, saying the wrong thing is still much better than not being there at all. But moreover, to say the right thing you can just be like, "What can I do to help you? How can I be there? Do you want to just watch a movie? Talk about regular things?" At one point I joined a Facebook group, it's a different one than I'm in now, but I actually did have to leave the Facebook group for a period of time because there seemed to be a string of people that were passing away at that time, around November of that year. I was like, "This is depressing, I can't be in here, this is making it worse." So I got out of that for awhile, until I came to terms with everything. I didn't want to wear my oxygen at first because I'm young, I'm 37, I was very vein and I was like, "Nope, I want to look cool, I don't need to look more disabled than I already am." I was very ableist against myself in general even before the PH. I would never get the disabled parking placard because I didn't want the little wheelchair anywhere near me in the car or the desk at school if it had the little sticker on it I was like, "No, not sitting there." I did everything I could to just completely deny being disabled in any way, shape or form. After awhile, I realized that's not really a good thing to do, it's a disservice to other people as well and it's a disservice to yourself, because when you push your body in ways that you shouldn't necessarily when you could just go with the flow and be happier and lighter and spend less time worrying about things and just enjoy the stuff that brings you joy. So it was kind of like a release. I definitely don't like taking my meds still because they make me feel bad. But I try to do them, especially now with COVID, I'm trying my darnedest to not end up back in the hospital. It was like September of the year before that my niece showed my TikTok. She showed me a funny sound, a funny video and it was a little funny whistley, squeaky sound and I said, "Oh that's hilarious, I want to make one." So she's like, "Yeah, you should do it." Another friend of mine, she's a little bit younger than me too, she's like, "Oh yeah, everybody would think your videos are hilarious." So I just put a few videos out. I would say for the better part of the year maybe I had a few hundred people that followed me. It's the @ sign and then it's toolowfrontrow, it's T-O-O low front row. That is because I'm very short and I love going to concerts and if I'm not in the front row I see nothing but butts. I had posted a few things that were funny. One kind of went mini viral at one point, because usually what happens is something will just pop off and then that's where you get all your followers and your likes and all your stuff. At one point I did a general disability joke kind of thing, basically teasing at the, "Oh, you're so inspirational", and I'm just living my life getting a soda, kind of thing. So I made that and that one went viral and then nothing for awhile, just here and there. Then at one point I just decided to talk about my disability and I showed myself, I said, "This is what I've got going on.” It just went bananas. I ended up getting hundreds of thousands of views on it, I got the majority of follows from that and one subsequent other video and from then on it just kept going and then I got quite a few new people from the last video. Actually that was how I was discovered as well by the “Born Different” people. There are so many bits of advice I could give. One thing that helped me initially to get over the vanity of the oxygen, I know people probably heard of the Chloe Temtchine. When I first got diagnosed I was trying to find cute oxygen tanks or cute canulas or something. She said, "Give your oxygen tank a name.” I guess hers was Steve Martin and so I was like, "Okay." Then I had my friends name my little mini one and that's how we came up with Roxygen. So she's got a little personality and that made her less of a medical device. I guess another thing is just speak up, don't take no for an answer. If you're not satisfied with your treatment, if you're not satisfied with your team, if your friends aren't getting it, just keep talking to them. Just keep looking, keep looking for new people. If you need to invite more people into your circle. Don't let yourself be pushed aside because that was what happened for me and there's no reason for that. I finally came around to being joyous, because you could deal with all the physical ramifications of PH and be sick and bloated and tired and what have you and also be sad and miserable about it, or you could distract yourself and be happy about the things that bring you joy. So at least you're not sad on top of being sick. I think that's where I finally made a turning point. My name is Kelly Lynch 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 /episode/index/show/phawarepodcast/id/20843540

Episode 360 - Kelly Lynch 11/01/2021

I'm Aware That I'm Rare: the phaware® podcast Trailer 01/15/2021

Raymond Benza, MD - phaware® interview 359 12/29/2020

Raymond Benza, MD - phaware® interview 359 Dr. Raymond Benza is the Director for the Division of Cardiovascular Medicine at “THE” Ohio State University. He has been in practice for more than 20 years. In this episode, Dr. Benza discusses and explains what differentiates GB002 from other investigational drugs in development. Raymond Benza, MD: Well, good day, everyone. I hope everyone is staying safe in these crazy days. My name is Dr. Raymond Benza and I am currently the Director for the Division of Cardiovascular Medicine at “THE” Ohio State University. I am also a PH Clinician and Researcher. I've been working in the field of pulmonary hypertension for over 20 years and have been involved in the majority of the main clinical trials, exploring new therapeutics for the treatment of this very severe disease. I’ve treated many patients with this disease, personally. I've seen what patients have suffered through and really have come to be very close with this community and I'm very passionate about treating this disease process. Over the last 20 years, we've seen a remarkable improvement in the way we manage patients with pulmonary arterial hypertension, so much so that we've actually tripled the life expectancy for patients with this deadly disease. Now, overall, that sounds like a good thing, but when you recognize that the majority of the patients who have this disease are young women, an extension of life from three years to nine years may not be enough. So we are all dedicated trying to find new therapies that really, really act at the heart of this disease in order to make a better impact on survival and to keep patients out of the hospital and make them feel better. Unfortunately, the currently available therapies do not really address the proliferative process leading to pulmonary arterial hypertension. What I mean by proliferative is that this is a disease in which the cells of the blood vessels divide in an unchecked manner. These are the cells of the middle lining of the blood vessel called the media. By the expansion of this media, by these proliferating cells, the vessels become smaller and smaller such that blood cannot traverse the lung to get oxygen into them. This causes a series of problems, ultimately leading to heart failure and to patient demise or death. So our patients need better options. They need therapies that directly attack this proliferative process in order to reverse the course of the disease, improve their survival and their quality of life. This is going to be one of the medications that we will be talking about today called GP002. Now I am a member of the steering committee and helped design the Phase 2 trial for this particular therapeutic in pulmonary arterial hypertension. I'm very familiar with the therapeutic and very excited about this. The main difference between available therapies for pulmonary hypertension and GB002, and what makes GB002 such an exciting clinical drug candidate, is that this is a new chemical identity that is developed specifically for the treatment of pulmonary arterial hypertension, that targets that underlying proliferative mechanism that we just spoke about. That really leads to the development of PAH and its ultimate progression. Remember, the three pillars that drive pulmonary arterial hypertension are inflammation, fibrosis or scarring and proliferation. This is all occurring in the blood vessels or pulmonary arteries in the lungs. GB002 has demonstrated effects on several of these underlying mechanisms and pathways, suggesting that GB002 really has the potential to be disease modifying. Again, a drug that is doing much, much more than just dilating the blood vessels, but actually working on the mechanisms in the cells that cause these blood vessels to close down. Interestingly in a healthy volunteer study, GB002 was shown to be very well tolerated with no significant adverse events that were observed. It is also being evaluated in patients with PAH in a Phase 1b trial. Now, this I believe will be completed by the end of December of this year. So we might have some really more exciting information to talk about, perhaps in a later podcast. The other unique thing about GB002 is that it is delivered by a dry powder inhalation twice daily, and it can be self-administered by the patient. This delivery system, we'll talk about a little bit later, because it's pretty cool. I think most people will really like the way it works. So where is GB002 now? Let's talk a little bit about the Phase 2 trial that I mentioned a little bit earlier. Again, I am part of the steering committee and helped design this . This particular study, Phase 2, is also referred to as the Torrey Study. It's a randomized, double blind, placebo controlled, multi-center clinical trial, to evaluate the efficacy and safety of oral inhalation of GB002 for the treatment of WHO Group 1, pulmonary arterial hypertension. Now remember, Group 1, pulmonary hypertension is the type of pulmonary hypertension where the disease is intrinsic to the arteries of the lungs. It's not caused by some other secondary problem like heart failure or chronic lung diseases or blood clots in the blood vessels. It's actually an intrinsic problem of the blood vessels itself. This study will be enrolling around 80 patients with confirmed diagnosis of idiopathic pulmonary arterial hypertension, heritable pulmonary arterial hypertension, or pulmonary arterial hypertension related to connective tissue diseases. Now, the patients who enrolled in this study are functionally impaired. They know something is going on, but their symptoms can be minimal and really affects them only when they're really exerting themselves, or it can actually be affecting them so much that they have symptoms at rest. Particularly breathlessness. Now study sites are going to be located all over the United States, as well as in Canada and across the European Union. Now, the good thing about this trial, is that unless a patient is on an inhaled prostacyclin like TYVASO or iloprost, the patients do not have to come off their currently approved PH therapies in order to participate. That's really great. Now the study duration will be 24 weeks in duration and here are some of the study requirements and visits for the patients. We're trying to keep it simple. In clinic visit spaces will be about 12 weeks apart. We wanted to make sure that we kept this simple because of the ongoing issues with the pandemic. There will be opportunity for a home health nurse for certain visits too. Again, to simplify things. There really are only two mandatory clinic visits at week 12 and 24. And again, this is an attempt for us to be COVID conscious in these days. Now, let's talk a little bit about the administration because I mentioned earlier, this is really cool. The drug is going to be administered two times daily using a dry powder inhalation. Now, this dry powder inhalation is delivered by a very novel inhaler. I think the actual inhaler is the cool thing. It's very discreet and it can fit in the palm of your hand. It could fit in your pocket or in your purse. The size of the inhaler is similar to the bottle of hand sanitizer, which I hope you have all in your pockets right now. And smaller than the size of a computer mouse. If you just pick one of those up, you'll see what the inhaler sizes is like. So again, it's compact easy to use, and it has a well-established track record already. The FDA has approved this for other inhalational therapies. Now the study objective, the primary one, is to determine the effect of GB002 on improving pulmonary hemodynamics. That means, the pressure in the lung or how well the heart circulates blood and subjects with WHO Group 1, pulmonary arterial hypertension. Which we explained early what that classification is, who are functional Class 2 or 3. Again, we explained the level of intensity of the disease a little bit earlier. Now the secondary objective is to determine the effective GB002 on improving exercise capacity in this population. And lastly, obviously, and very importantly, we want to evaluate the safety of GB002 in this population. Now again, the specific pulmonary hemodynamic that we're interested in is the change in pulmonary vascular resistance using right-heart catheterization, which many of you are familiar and probably have had in the past from baseline to week 24. So two different heart catheterizations. Now, remember the resistance is the actual hemodynamic that reflects the degree of arterial remodeling in these blood vessels, in the lungs that we talked about earlier. Our secondary end point, which again is a measure of your exercise capacity as how far you can walk in the six minute walk tests between baseline and week 24. And again, everyone is very familiar and has probably been in these six minute walk tests in clinic, and we know how easy they are to do. Following the participation in the study, there will be an open label extension of the study that is also planned. So people can continue on this drug, which is very important, particularly if you have a really good benefit from the medication and we want to keep it going. So to conclude, those interested in participating or potentially participating or learning more about the GB002 Phase 2 study, I encourage them to talk to their physicians and you can find out more details by using the following link on ClinicalTrials.gov and search for the or the . We will also have this cited at the end of this podcast today. Remember GB002 targets a new pathway. It really acts on the cells that are in these blood vessels that's causing the disease. Given that PAH currently has no cure, it's important for patients to participate in clinical trials. This is how we push the needle forward, both by allowing researchers to study new pathways, like I just described, as well as helping them to discover and develop new and more effective treatments for this very deadly disease. My name is Dr. Raymond Benza 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 /episode/index/show/phawarepodcast/id/17026712

Episode 359 - Raymond Benza, MD 12/28/2020

Roz Jones - phaware® interview 358 12/22/2020

Roz Jones - phaware® interview 358 Rosalind Marshall-Jones is an innovative speaker with more than 15 years of experience in the caregiving industry. She is a seasoned health care provider, who runs Jacksonville’s Best Caregivers, an organization that provides short and long-term health care. In this episode, Roz discusses the impact #covid19 has had on home healthcare from anxiety, depression to feelings of isolation. Hi, I am Roz Jones, I'm the CEO and owner of Jacksonville's Best Caregivers, and we are located in the Sunshine State of Jacksonville, Florida. My company, we are a nonmedical home healthcare business, and we provide four levels of service to your loved one: sitter, homemaker, companion, home health aid, and certified nursing assistant. Our mantra is, "If you can't do it all, you give us a call." Today I just want to talk to you all a little bit about caregiving during #COVID19, what has changed, is anything still the same? As many of us know, it's not the nursing homes that many of our loved ones may be in or may eventually be in, were not built for isolation. So with them being isolated in the nursing home, it's caused many problems. It's caused depression. It's caused anxiety, as well as death. Some people have died from loneliness. So how do we reverse this? How do we make sure that this does not continue on? We have to make sure that either you have to make sure you stay in contact either by phone, or by writing letters, or sending cookies. I'm going to give you a good example, the client that I'm taking care of today, her great nieces and nephews and friends during #COVID19, the children drew pictures, someone baked cookies for her, someone sent her a really nice bottle of scotch. Yes. So they have sent her all kinds of wonderful things to keep her going, and they have called, and that is so important. The isolation, even though it stops the physical contact, you still can contact through letter writing and these other things. So that's part of the way #COVID19 has impacted the nursing homes. Now, so far as doctor's visits, that's another impact, because many of the people living in facilities have doctors come in. Well, now they're doing the telemedicine to where they're doing it over the phone. So the lady that I'm taking care of, we have had to talk to the doctors by phone, and we have actually had to do the actual assessment of her body for the doctor, or for the nurse, or whoever, because either they're not able to get in because they didn't take the #COVID19 test, or they won't come in because of the high risk to the population here in the different facilities. So that's another way #COVID19 has impacted. Also too, #COVID19 has impacted when it comes to advanced directives. Many people did not, during #COVID19, they didn't have their advanced directive. So when it was time to make decisions to move people, they didn't have that information already taken care of, so they didn't know how [the patient] wanted to be treated, do I want a DNR? Do I want to be incubated? These were decisions that were not discussed nor prepared for prior to #COVID19. So now, since #COVID19, you have to sit down and have this conversation. I know people don't want to talk about death and all of this, but it's a conversation that has to be had. There's no way to get around it, none of us can get around it. So make it easy for your family. Have everything in order, your advanced directive, your will, your trust, so that this stuff does not go to probate. Let's not make the courts rich. Let's leave a legacy for your family by getting your life insurance, make sure it's up to date, make sure all of this stuff is taken care of because now #COVID19 is pushing us… is forcing us, to have this conversation that we didn't want to have before. One of my clients, she was supposed to have hip surgery, and for many of any type of major surgeries or minor surgeries, they pushed it back. The reason why they pushed it back was because the hospitals needed either the ventilators or the PPE to take care of people with #COVID19. So that was a priority. So if your surgery was not life-threatening, you had to postpone it. But now since you postpone it, you have to do different things for pre-op, during the surgery, and post-op that you didn't have to do before #COVID19. Also too, during the #COVID19 crisis, we asked people and I beg, "Please, ma'am, please, sir, go and get tested." Not only for your peace of mind, but then to keep the people that you are around, your loved ones, to keep them safe. Not only get the #COVID19 test, but get the antibody test to see if you have had come in contact with someone in the past, or if you currently have it, because the antibodies will tell you instantly whether or not you have been in contact. So those are just some of the ways that #COVID19 has impacted caregiving. And another way it's impacted too, is that going to the grocery store is different. We have to go during non-high peak hours to the grocery store, or they can't go at all and I have to go out and do the shopping, or we have to use Instacart or some of these other shopping companies to bring the food in. Then in some of the facilities, they have cut out dining completely, and they deliver the food to the resident because of #COVID19. Now some of the facilities here in Florida, and I'm not sure about everywhere else, but here in Florida, they are just getting to stage one to where they are allowing them to come back out and have some type of interaction with one another. But for months, for six months, many of us have had to take care of the person inside of the facility, or, I'm blessed with my client, she has a car. So we go out and do what we call field trips or excursions. We ride around the city or we may take a trip. Many of you may be familiar with St. Augustine, so we ride through St. Augustine. We may go somewhere else, but getting out once a week, those are the things that you have to remember when you have a loved one in a facility, and not even at a facility, at your house because of the isolation, get them out of the house. That is so important. Then also too, another thing that we've had to do is Amazon has been huge here in a lot of the facilities. They deliver here at least four or five times a day because they work with Whole Foods, so they bring the food, they deliver undergarments, they deliver the bed pads, medicine, pill boxes, all these types of things that we took for granted that we used to go to the store for, now we've had to alter our shopping because we cannot go out like we used to in the public. Even me as a caregiver, I have had to alter a lot of my life. I, too, only go to the stores here in Jacksonville. They allow essential personnel to come in on Thursday mornings and Friday mornings before anybody else to do shopping, which is nice. So that means that we don't come in contact with the general public that much, other than the employees at the store. I used to take my uniforms to the dry cleaners. I don't do that anymore because I don't know who may or may not have #COVID19 that's cleaning the clothes, so I do everything myself. When I go home, it's a different process. I Lysol my shoes before I come in. I change my clothes in the garage, and I'll wash them separate from everybody else in the house. So #COVID19 has taken a significant impact, not only on the people receiving care, but also on essential employees. I bathe with a different soap. I don't bathe with Dove or Dial. I bought a special surgical soap that I use to make sure that I'm clean. These are the precautions that I take since #COVID19. Then on a weekly basis, I go and get a #COVID19 test. Every week, I have a standing appointment to where I go and get a #COVID19 test. The new normals are, I'm going to give you an example, at the end of our shifts, before I leave out, we have to wipe down the doors with Clorox wipes. All the high touch areas. We make sure that we have it clean, the refrigerator door, the microwave, the toilet seat handle, the guard rails that they pull up with. All of that is things that of course we would clean, but now we clean it more often to reduce the opportunity for #COVID19. So it's a new norm. Are we going to go back to the way we were? No, no, I don't think so. I really doubt it. One of the first things when #COVID19 first came out, the concern was because of my age if I go to the hospital with #COVID19, will they treat me or would they let me die? That was heart clenching for me to hear one of the clients say that. It wasn't even a client, it was just one of the residents, excuse me, walking in the hallway and having a conversation, "Will they treat me or will they let me die because of my age?" So that was the mentality for a lot of them until it started coming out that they're treating the elderly as well as the young, and everybody was being treated the same. But initially when it came out, that was scary. They were saying that we weren't going to treat the elderly and they were going to let them die. That was real scary. Even with the veterans, a lot of them, again, isolation. So we're sending them back through another trauma, it's almost like another war. My parents, my dad is a Vietnam vet. It's been very hard on them because they are so isolated as well. So we haven't even been able to visit my parents. Now, my client here, she's very lucky. We get to go and see her daughter and her granddaughter, but some people aren't that lucky where they get to actually go and see their loved one. But we still take the precautions when we go out and visit. We have the mask. We have the hand sanitizer, we do all of that, but it's important to her. She always says, "I want to see my babies. These are my babies. That's my only grandchild." So we make sure that we take her on a field trip to go see her children, because they're so isolated and they can't get out like they want, they're not coping well. A lot of them have lost weight. A lot of them don't eat. They don't have an appetite. That's when depression sets in. So some of them have had really gone to a deep depression. Now here it is six months later, they're starting to get out, and it's almost like a flower being blossoming that they're able to get out. Just like this weekend, I will make sure that we get out at least two or three times in the next couple of days to make sure that they see each other. That's part of it is to make sure that we try to get back to some, normal way of visiting and some normal way of being able to see family and friends. So those are the things that they don't have. Because when they were able to visit during #COVID19, they had to visit on the other side of a screen or look outside their window to visit with their loved ones. That was horrible. That was horrible. Or they had to do a drive by. With my client, her daughter has done drive-bys, and we've sat outside and spoken from a distance, but that's all we could do. That's all we could do. We are a country of human touch and touch is so important in thriving, and healing, and living. When you lose that touch, they lose a lot of their identity and who they are. So we try our best to make sure that if they can't do a hug and a kiss that at least they can see them, at least from a distance. But now, they're going to allow some hugging and things like that. So hopefully it's going to be a turnaround for a lot of them. This isolation has been horrible. This has been horrible. I can't wait for them to be able to get back out and mingle with their community. These facilities were built for them to be able to still thrive in a community that's similar to what they had when they're at home. And we shut that off for six months. It was like taking six months of their life away. My optimism for 2020, I think we are going to be able to start having more interaction but we have flu season coming up. That's the scary part. So still we have to take those precautions, even in flu season, just remember that. This is not over, so we have two things that's going to butt heads. We have #COVID19 and we have the flu season. Take the same precautions. And get your documentation in order. That's my biggest thing. Please, ma'am, please, sir, sit down with your family members and get your documentation in order, and don't let it go to probate. My name is Roz Jones, and I am aware that I am 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 /episode/index/show/phawarepodcast/id/17166467

Episode 358 - Roz Jones 12/21/2020

PAH Today: Moving Forward - phaware® interview 357 12/16/2020

PAH Today: Moving Forward - phaware® interview 357 SPECIAL EDITION: Moving Forward The PAH Initiative and United Therapeutics are excited to bring you the PAH Today National Broadcast Series. This series of virtual events is intended for adult patients with pulmonary arterial hypertension (PAH) and their caregivers. Advancements in the care and treatment of PAH are improving the lives of those impacted by this life-threatening disease. The National Broadcast Series will include insights and perspectives on today’s approaches to managing PAH from nationally recognized PAH healthcare providers. Join patients and caregivers across the nation to hear current considerations in the care of PAH. This is the 4th of 4 PAH Today broadcasts to learn about how you can be empowered to stay informed, motivated, and focused on your future. Presented by Dr. Lana Melendres-Groves the Director of the Pulmonary Hypertension Program at the University of New Mexico and PAH patients Denise B. & Lauren J. Learn more at: Dr. Lana Melendres-Groves: Good afternoon and welcome to the PAH Today National Broadcast Series. Today, we're moving forward. My name is Dr. Lana Melendres-Groves and I'm the Medical Director and an Associate Professor at the University of New Mexico. On behalf of United Therapeutics, myself and everyone joining us today, I want to welcome you. Whether you were on your own PAH journey or here to support someone you love, I hope today's series is informative and helpful. This presentation is sponsored by and made on behalf of United Therapeutics. Healthcare professional speakers such as myself are compensated by UT. Not all drugs are appropriate for all patients, so speak with your healthcare professional to determine which treatment plan is right for you. Patient experiences relayed during this program may not represent the experiences of all patients. If you've been with us throughout this series, you know that we've covered a lot of information, but today we really want to focus on helping you move forward on your PAH journey. We'll talk about three different areas. The first is going to be about getting informed and staying informed. Next, we'll talk about staying motivated, tips on how you might be able to do this because each of us, we all need a little help on certain days. Third, we'll focus on your goals, so that what you do today will help you through tomorrow. All right, so let's talk about getting informed. I think that when any of us have something new to learn, it can feel overwhelming. I always think about it in terms of my daughter who's learning to read. Now, I know I can't walk up to her and hand her a novel and expect her to read it. Instead, I have to give her the basic tools to be able to. Those building blocks she needs to learn the sounds of letters. With those sounds, be able to make a word. With those words, be able to read a sentence so that someday she will read that novel. When we think about it like this, in terms of PAH, we also need to learn the basics, those building blocks. In one of our earlier series, we talked about that. We talked about the heart, the lungs, the blood vessels, and how they all work together to keep us alive and how specifically in PAH they are working the correct way. By knowing those basics, we can then build off of that so that you're able to do your own research and find things that are important to you, or that you may have questions about. You'll be able to get those answers. More importantly even though, is to stay updated. This is a disease that we learn more and more about. Our treatments continue to expand. Over the past 25 years, how far we've come is tremendous. So knowing what may be new today can help you in your tomorrow. Make sure that you feel like you can have an open and honest conversation with your healthcare provider, so that if you have questions, you feel like they're being answered. Let's talk a little bit about where you might be able to find some of those resources. Now, I mentioned talking to your healthcare provider. For me, I think that's the first place you should go, because they really should be the individual that you feel confident and comfortable with in terms of sharing your concerns or maybe gaps in your own knowledge. You have people right there in your own home potentially, or your community who can help. Your caregivers, they may not have the medical knowledge to answer your questions, but they're there to help. I'm sure they'll help you find that information. That brings me to advocates. In some situation, the caregiver is your advocate and in others, that is a person who is helping you along your PAH journey. Now, I don't want to forget to mention the patient group meetings. These are essential to create a network for you while you're on your PAH journey, so that you can have others who may be able to share similar experiences or give you information on where they found something that was important for them. Some of the places that you might want to visit online are the PAH Initiative or the Pulmonary Hypertension Association. These are just several that have good information that may help you to learn more. I do want to spend just a little bit more time on the because this is a robust learning library with resources for all levels of patient education. I think it's wonderful because it has not only informative videos and interactive content, but continues to expand the information on a frequent basis, so important to visit it often. At this point, I think I've talked enough about what I think. Instead, I think it would be wonderful to hear what Lauren and Denise, who are two real patients on their PAH journeys, do to continue to stay informed about their disease. Denise: Hi, my name is Denise and I am a PAH patient. Lauren: Hi, I'm Lauren. I am a PAH patient. I've been diagnosed about 10 years now. Denise: As you know, there is so much happening in the PAH community and it is so important to stay informed. Lauren: I am excited to share a little piece of my story with you. Denise: I'm sharing my journey with you today. I hope you enjoy it. Thank you for listening. When I was first diagnosed, I didn't really know the difference between a cardiologist or my PAH specialist. In my personal opinion, I believe that seeing a PAH specialist is way more beneficial to somebody because they are the expertise in this field. Lauren: My relationship with my doctor is very good. I respond very well to people that are straightforward. There's no sugarcoating. She said, "I'm your specialist. I promise I will take the best care of you that I can, and this is what's happening right now." Denise: I think it's pretty important to stay on top of the research and to find out what's going on out there and you might talk to your doctor about the new things that's upcoming or different drugs that are out there. You can talk to them and see how they feel about something. Is it something that is going to be right for you? Lauren: Assessing your risk level goes hand in hand with being compliant as a patient. So when your doctor orders tests and appointments for you to show up at it is crucial that you show up and get those testing done, because based off that it shows how your PAH is in a current standing. So are you stable? Have things changed? Have your pressures gone up? Do we need to change medications? Denise: The most drastic changes for me personally, that I've had to make is that I'm not going to bend over and pull things out of the bottom of the cabinet, or you don't want to pull things off the top shelf. It's just learning your new normal and to be able to do different things. Lauren: The lifestyle modifications were quite a bit of a challenge too, like how do I hide my pump? All of that kind of stuff was more of a barrier that I dealt with on a daily basis. Denise: I see my PAH specialist about every six months. Usually when I prepare for my doctor's visits, I would just tell them if I had had chest pain or tightness, or if I feel like I was at a grocery store and by the time I got home, I couldn't even breathe. Lauren: I wear a heart rate monitor when I exercise and that way I can see what did I do that day. I can always show it and reflect to my doctor how I'm feeling based on where I'm at. You need to be clear in communication with your healthcare team and mention things way sooner than later. PAH is a very stubborn disease, and I think it is so important to stick to your plan with which you had made with your doctor in terms of medication and adherence to medications. Dr. Lana Melendres-Groves: Well, we've talked a bit about how we stay informed and become more informed. Now it's time to talk about staying motivated. Although I'm going to talk about goals, which may not seem the same as staying motivated, I think in a minute, hopefully you'll understand. A goal is something that drives you to move forward. We want to reach that goal. And in order to do that, trying to stay motivated. By making personal goals or treatment goals, it allows you to have that checkpoint in insight where it's not just one day after the next with no end. Instead it's, I would like to experience fewer symptoms like fatigue or shortness of breath. Maybe it's spending more time with family or friends or just being more active. All of those things may be an important goal for you. Then let's move over to treatment goals and these may seem completely foreign or different from your own personal goals. Often this is where I think there's a disconnect between what a provider is talking about and what a patient is talking about. Well, I hope I'm able to align those for you today, because treatment goals may be something like improving how the right ventricle is functioning or slowing down the disease progression. By achieving those treatment goals, we're also potentially achieving some personal goals where when your heart is functioning better, you'll have fewer symptoms and be able to do more. So I think it’s important to start thinking about goals for yourself that are achievable. In doing that, it's really important that one, you write them down. I think that we often forget what we could do back when we felt normal. So it's important to remember back how you used to feel and what you used to be able to do and then you really think about how you feel today. And in talking with your healthcare provider, what might it be goals of three months from now, six months or a year from now, so that you really have something to look toward and to get to. It's then important to check in often on, are you achieving those goals? Have you surpassed those goals and need to make new ones? These goals don't have to be groundbreaking. They may just be being able to bring your groceries in from the car. That is okay. That is the baby steps that turn into miles and miles of road behind you on your PAH journey. So how can we bring those PAH goals to life? Well, this is where I think everybody is very individual. For me, if I wasn't in the medical field, I think I would have been a, probably struggling artist, but I love it. I love to express myself through creative means. So a vision board may be the right thing for me. I actually really liked poetry. So potentially writing a poem that had some of those things that you would like to be able to do again. Other people are more concrete. They may write themselves a contract because they can stick to that. But it's important, whatever works for you, you write it down. You look at it often, so that you can continue to move forward toward those goals. When I was looking through all of this and preparing to talk with you all today, I thought back about my mother when I was a child and how I used to hear her talking in the other room. Now, at first I thought she was talking to somebody. But later, I realized that she was actually just saying out loud that things that she planned to get done that day and over the course of the week. I thought it was funny as a little kid and now I realize that when you say something out loud, it is so much more meaningful. So using positive language and encouraging yourself. Checking in regularly with other people so that you feel like everybody is helping you along that journey. Then, celebrate the successes. I think we're so hard on ourselves often. But we have to remember that whether it was a little one or a huge milestone, we have to be able to congratulate ourselves so that we can for at least that moment, breathe deeply and really feel as though we're moving in a positive direction. Checking in with our advocates. Celebrating ourselves. Those are things that I would really ask you not to forget to do. So we've talked about how we might be able to stay motivated, write down goals. You may already be doing a huge amount to improve your PAH, but you should never stop asking, "Is there something more I can do for my future?" This is where you need to feel confident to want more for yourself and to talk to your healthcare providers in order to get there and to achieve that. To be able to ask yourself critically, "Am I feeling better? Are my symptoms the same? Do I feel as good as I could feel," so that you continue to reevaluate those goals and make new ones for yourself. This is where I'm going to get to invite Lauren and Denise to give us a little bit more information, because they are experiencing this on a day in and day out basis. It would be lovely to hear how they continue to stay motivated. Denise: I literally think I spent the first year on the couch, not because the doctor told me to, but I was scared to literally move. I wish somebody could have told me in the beginning that you don't need to be fragile, you're not going to break. I mean, I'm not telling you to run a marathon, just do a little something more positive tomorrow than you did today. Lauren: I think it's important to set improvement goals for yourself while you're working with the different therapies and all of that. What can you do to make things better? It's all about the fight, because if the fight isn't there, then what are you going to accomplish? Set your goals and then set them higher. Denise: Generally, it is a goal of mine to try to beat the last six minute walk test. The doctor comes in and he'll say, "Oh my gosh, you walked farther than you did the time before." It's definitely a good feeling. Lauren: A lot of staying positive for me is reaching for my daily goals. So a lot of that has to do with my exercise and I also am a practicing Yogi. So I do yoga probably twice a week. I do everything in my human power to make sure I'm in the best health that I'm in. So my husband and I have adopted a low sodium diet. It took a couple years to get used to the whole idea, but it was worth it. Denise: To be able to go to the grocery store on my own or drive that two hours to an event or different things, definitely is something that I wasn't sure I was going to be able to do. It's just learning your new normal. Lauren: There was one time I remember running up the stairs to my apartment and being like, "Wow, I can breathe. That hasn't happened in a while. That's pretty cool. I think things are working." Denise: One day I went to the beach and I was by myself and walking through the sand is hard enough, but with PAH it's hard to walk. But I walked pretty far. It was pretty amazing. It was like I had no limit that day. Lauren: My support network for me has been everything. A huge chunk of my support is my husband. He stuck with me through it all, even though I told him to run. Denise: I really think you need some kind of outlet, you need some person to talk to and find something, whether it be another PAH person or just somebody. I'm definitely looking for in advancements in PAH treatment. I hope we find a cure very soon. Lauren: Never stop achieving. Never stop reaching for your goals. It is so important to have the motivation and strive to reach for something. When you hit that, reach for the next goal. Never settle, own your health, talk with your doctors and you're not in this alone. Dr. Lana Melendres-Groves: So we had a chance to talk about staying informed and staying motivated. Now we're going to shift gears just a little bit and talk about staying focused on your goals. So what can you do today to impact your tomorrow? For me, the very first thing is finding a PAH expert who's right for you. Now it's important to know that PAH is a rare disease. So not all cardiologists who are heart specialists or pulmonologists who are lung specialists have expertise in treating it. That doesn't mean that they're not great physicians or providers. This may just not be their area of expertise. So make sure to seek out a health care provider and a treatment team with experience using all of the available types of PAH medicines. This brings me to, if you find that person, feel confident that you can share information with them, good information or bad information. We understand. We all have slip ups along the way. So maybe you ate a little bit more salt in your diet and you have swelling. It's okay to talk to us about that. We're here to work together, not criticize, not critique, but instead to help make sure that your tomorrow is even better than today. So how do we move toward a better future? Well, in most things, it's important to be empowered. For PAH, it's even more so. You need to feel that if you're still having symptoms, that you can talk to your healthcare provider, so that you can come up with ideas or management strategies or treatment options that might help you do better. Then, never discount today. Even if you feel as though getting up is just too overwhelming. Just a few steps every day, a few minutes even, to be able to improve your tomorrow. Continue to make strides so that your tomorrow is so much better than today and that your goals can be reached. This concludes the 2020 PAH Today National Broadcast Series. To watch parts one through three in this series, visit . Also, consider joining the PAH Initiative at , where you can stay informed, stay motivated, and stay focused on your future. Thank you. 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 /episode/index/show/phawarepodcast/id/17067371

Episode 357: PAH Today - Moving Forward 12/16/2020

David Harris - phaware® interview 356 12/15/2020

David Harris - phaware® interview 356 David F. Harris founded Insight & Measurement and provides research, training and speaking to the industry.   In this episode, he discusses the qualitative and quantitative work that he conducts with PAH patients, so that people who get invited to be part of these types of studies have some understanding of what actually the process is like and what gets done with those results. My name is David Harris. I run a company called Insight & Measurement, and we do a lot of work in rare diseases and in healthcare. My background has always been in healthcare. I worked in a hospital many years ago and I was director of research methods at the big pharma company called GlaxoSmithKline followed by another company called Merz North America. I've done a lot of work in the area of pulmonary arterial hypertension. I've had the good fortune of being able to work with the people at to really help advance the perspective of patients for pharma companies and for device companies. Today, I want to talk a little bit about the qualitative, and to some extent, the quantitative work that we do with patients, so that people who get invited to be part of these types of studies have some understanding of what actually the process is like and what gets done with those results. What I like about what I do, and it's probably my favorite part of the work, is when I get to talk to patients, and I also talk with nurses and with doctors too, about their real experience with the disease, with getting diagnosed, with treatments. Very often, companies will contact me because, one, they want to know what is the experience really like and that can range from what was the process like in terms of getting diagnosed to how do you manage your disease to issues around caregivers or care partners. How do you manage your social life, and all of the experiences that people have with the various medicines that get used. I think when we do this really well, we develop these things called discussion guides. I like to call them with my clients, listening guides, because it gives them an up-close and really personal feeling and view about what it's actually like living with PAH. Sometimes, our studies may have one particular objective, but when we're talking to somebody, they might start talking a lot about cooking and what it's like cooking when you're on a pump and when you're on oxygen. That could take on a whole different discussion which can be enormously useful to the company. But largely these things are around what the overall experience is like and it's helpful to them, because they're spending their careers and lives developing medicines and devices for people with PAH. We also do a lot of work around designing things. If you think about designing a clinical trial, if you think about the actual details around the design of a device, whether it's a nebulizer or a pump or something like that. We're always much better off when we go out and we talk to people who would use these devices and get as much feedback as possible about how to fix them and how to improve them. I probably don't need to tell you all the different things that might not be right about them. Some of these things have sounds. Some of these things have lights. Some of these things might be too heavy. Some of these things might tip over. Really, the lived experience of what it's like using these various devices or these medicines. The other category that we work on a lot has to do with, in the industry we call it messaging. So that's really, what do we say and how do we say it? It's a real challenge for people in the healthcare world, because the healthcare world has its own jargon and that sort of thing. My goal, when I talk with patients is to help the company get the messaging and the creative to make as much sense as possible, right? To make it just as clear as possible to a broad spectrum of people. In this case with PAH, but if it were another condition, then that other condition. So, I think in summary, what I would say is the reason why we go out and we want to interview patients and caregivers is to really understand how to adjust things so that they work better. We also do this work with doctors and nurses too. Very often we need their perspective also. That can often be really, really helpful, particularly around some of the issues that they deal with when they're working with families. So you could imagine the process of talking to a patient about being on a pump. What does that process look like? It's a really tough conversation. A tough process. We need the perspective of patients, as well as the perspectives of the doctors and nurses on how to make that effective, comfortable, and sensitive to all the different emotional issues that are going on with people. Over the years, and particularly in the past, I don't know, few years the FDA has made it really clear that they want the patient voice included in the development of medicines, devices, and processes for patients. So, when I go out at , when I work with and we talk with patients about their experience. First of all, let me say, we follow pretty strict ethical guidelines. We assure you that your identity will be kept confidential. It's completely voluntary. You can just stop at any point, if you wanted to. Usually, these things are done over the phone, so you can do them at the time and place of your choosing so that they're comfortable. We almost always compensate people for their time too. We call that an honoraria or a payment, that sort of thing. What ends up happening is we develop our discussion guide or our listening guide. We might spend 45 minutes on the phone, it might be an hour, that sort of thing. Sometimes it's survey-based. We'll get the survey information. What we do is we write that study up for the, let's say, pharma company. It could be a device company. I've done work for advocacy groups too. It could be any group that's interested in this and we present that. We present that with our numbers, major themes. I always put together quotes so that they can really read and feel the lived experience of people. In the ideal world, and this is usually the case, what ends up happening is we're going to do the study. We're going to listen to these 20 patients, talk about X, whatever it is. Then they know a lot more about how to further develop that initiative, whether it's a clinical trial or whether it's a device or a medicine, or whether it's materials on how to communicate all that needs to be said about that particular treatment. Let me talk for a minute also about the timeline for these particular studies and I'll talk about it from, let's say, the patient perspective. Usually, you get recruited for a 45 minute, hour long interview that gets put on the schedule for a time that's convenient for you. If anything comes up, we always reschedule. I always say, "Life comes first," right? So that usually gets done, I don't know, within a couple of weeks of when you first get contacted. There's another type of study that we do at Insight & Measurement a lot, which are these online studies and we have a platform where people log in. Those will often last a week where you log on for 20 or 30 minutes, once a day for five days. Obviously, if something comes up and you need another week, of course. But those will be online activities where you log in and we use things like, we call them projective techniques, because, well, that's what they are. We might do a task where you write a letter to a hypothetical patient that just got diagnosed with your condition, what advice would you give that person? We might have you select an image from an image bank or upload an image that represents how you felt when you were first diagnosed and describe that. Or represents the role that your condition has played in your life and discuss it. We also build collages. I hear that they're rewarding for people, like build a collage, select a set of images that represent your relationship with the healthcare community. So people will put the images together and that sort of thing. Those are longer studies. They pay a lot more, but that's another common type of study that we do. Again, we just do this all around what's convenient for you. The timeline for the company is usually a lot longer. So, for example, we might do the exploratory work, the online work where we have the projective techniques, the write a letter, the images, early on, because they're just entering this category. By category, I mean this particular disease state or coming out with a new medicine or device. A little bit later on when they're developing the messaging around it and we're testing creative and let's just say that's a 45 minute or hour long interview, the company might be six months away from getting approval from the FDA. Their process is a lot longer. I will tell you at the end of my conversations with patients, I almost always ask them, "What inspired you to participate?" What I hear over and over again is, "I just want to help the next patient who has this." A lot of them, it's not about the honoraria, although we usually pay people appropriate amount for their time, but for a lot of them they want to just help the next person who gets diagnosed with what they have. I have to say, I talk to so many patients with PAH and with other diseases that are either rare or sort of near rare, and it's really moving for me, because I feel like I get to know people pretty well in that process. I think the kind of thing that I think is so helpful to these companies are the big themes about being misunderstood, judged, maybe blamed, minimized, not being seen. There're issues around feeling isolated, hiding the condition for fear of being misunderstood and judged and that sort of thing. There are a lot of issues around how the healthcare community communicates with people with PAH. Underneath a lot of that is just the desire for people with PAH to have hope, right? So when you put that together, particularly with the quotes, the things that people say about their experience around being misunderstood or judged or shamed, and you put that together with the quotes around issues around wanting new medicines and wanting to have hope, it moves people. We've also done some work with the images where people selected images that represented how PAH has affected their life and what life was like on IV infusion or Sub-Q infusion. We've published work. For even people who have been around the disease and treated it for a decade or more, sometimes it brings tears to their eyes, because they're right up against the truth. The truth really around living with this condition. That's really rewarding to me. I know it's really rewarding to the people that we interview that they really get heard. That's probably the most important thing. Well, thank you everyone. My name is David Harris 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 /episode/index/show/phawarepodcast/id/17067065

Episode 356 - David Harris 12/14/2020

PAH Today: The Pathways To Treatment - phaware® interview 355 12/09/2020

PAH Today: The Pathways To Treatment - phaware® interview 355 SPECIAL EDITION: The Pathways To Treatment The PAH Initiative and United Therapeutics are excited to bring you the PAH Today National Broadcast Series. This series of virtual events is intended for adult patients with pulmonary arterial hypertension (PAH) and their caregivers. Advancements in the care and treatment of PAH are improving the lives of those impacted by this life-threatening disease. The National Broadcast Series will include insights and perspectives on today’s approaches to managing PAH from nationally recognized PAH healthcare providers. Join patients and caregivers across the nation to hear current considerations in the care of PAH. This is the 3rd of 4 PAH Today broadcasts to learn about how medications work to treat PAH and how finding the right combination of medications could help improve your symptoms. Presented by Dr. Lana Melendres-Groves the Director of the Pulmonary Hypertension Program at the University of New Mexico and Dr. Sean M. Studer Chief of Medicine at NYU-Woodhull Medical Center in Brooklyn, New York. Learn more at: Lana Melendres-Groves, MD:Good afternoon and welcome to the PAH Today National Broadcast Series: The Pathways to Treatment. My name is Dr. Lana Melendres-Groves, and I'm the medical director for the pulmonary hypertension program, and an associate professor at the University of New Mexico. On behalf of United Therapeutics, myself, and everybody who's able to join us today, I want to welcome you. Whether you were on your own PAH journey or you're here to support a loved one, I hope that this program enlightens us about the pathways to treatment, and we have the opportunity to really delve into some of those questions that you may have toward the end. This presentation is sponsored by and made on behalf of United Therapeutics. Healthcare professional speakers are compensated by UT. UT does not provide medical advice. Not all drugs are appropriate for all patients. Speak with your healthcare professional to determine which treatment plan is right for you. Patient experiences relayed during this program may not represent the experience of all patients. So today we're going to cover two areas. The first is going to be understanding treatment pathways. I think before we can really understand the pathways, we really need to go over some of the basics of pulmonary arterial hypertension. Then, we'll move into an update on a treatment pathway, which specifically we'll dive just a little deeper into one of those pathways for treatment called the prostacyclin pathway. We'll talk about what it does, where it has come from over 20 years ago when it was developed, and how much we've learned and how we can treat our patients better as we move into the future. So let's start with those treatment pathways. Now, as I mentioned, I think before you can dive into talking about treatments, we have to have a little bit better understanding about really what is PAH? Now, if you had the opportunity to join us on one of our previous broadcasts, you would have heard about and learned about how the heart and lungs work as a team. Don't worry. If you didn't get to that broadcast, we're going to do it again. Hopefully this time, everybody will have a little bit better understanding of how the heart and the lungs work. So the first thing I always like to start with with my patients is just a discussion of what the heart is. It's a muscle, but it has four chambers to it. We often talk about it as having a right side of the heart and a left side of the heart. We may also describe it as having two small muscle chambers that sits toward the top of the heart, which are called the atriums, or the two larger chambers that sit below, which are called the ventricles. Now the left side of the heart receives blood that has been oxygenated by the lungs. It's depicted in red in this picture, and it pumps that oxygenated blood out to the rest of the body. The oxygen is then delivered to the tissues and the organs. Once that oxygen has been depleted, it returns to the right side of the heart, depicted in blue. Now the right side of the heart has the job of getting that low oxygenated blood into the blood vessels of the lungs, where more oxygen is picked up. Unfortunately in PAH, things start to change. Those blood vessels begin to narrow and thicken. As those blood vessels in the lungs become thick and narrow, the resistance for that blood to get through the lungs is more difficult. It increases, and it causes a back pressure on the right side of the heart. As that pressure increases and the heart has to work harder, the right ventricle begins to enlarge. It becomes dysfunctional, and it just can't keep up with the job it's being asked to do. This is often the time when patients really start to feel those symptoms of shortness of breath or fatigue. Now, I know we've talked a lot about the heart and the lungs, but really I think it's the blood vessels that need more explanation. We haven't talked about why those blood vessels start to narrow and start to thicken. That is where these three substances come into play. The nitric oxide, the prostacyclin, and the endothelin pathway are what become dysfunctional in pulmonary arterial hypertension. Now, your body naturally produces these three substances and it helps keep the blood vessels open and the lungs healthy and everything working properly. In pulmonary arterial hypertension, or PAH, those substances start to change. They're not produced in the same amounts. Because of that, we're not able to have the same reaction within those blood vessels. In the nitric oxide pathway, this substance helps to keep those blood vessels open, letting blood move through them freely. The prostacyclin pathway also helps to keep blood vessels open, but it has a couple other jobs. It prevents clotting and it slows the thickening of blood vessels. In the endothelin pathway, this sort of acts in the opposite way. It starts to cause the narrowing and thickening of blood vessels. In PAH, one or more of those substances may become out of balance. When the nitric oxide substance is not available, you start to have this closure of those blood vessels, that narrowing. When the process cycling substance is not there in an abundance, again, those blood vessels start to narrow. You start to see clotting. When we talk about endothelin, too much of it is produced. So now we have a speeding up of the thickening and narrowing of those blood vessels. You may have one, two, or all three of these substances out of balance. Unfortunately, at this point in time we don't have a test to figure out which substance or substances may be out of balance in you. What you might feel if those substances are not appropriately produced, you may feel new symptoms, or you may notice that your symptoms are not improving, despite getting therapies. So let's talk a little bit about not just the pathways, but how we have been able to develop medications to affect those pathways and improve the outcomes of our patients. When we talk about nitric oxide, we have two different classes of medications in that pathway. We have the phosphodiesterase five inhibitors, or we call them PD5s, or the soluble guanylate cyclase stimulators. In the next pathway, the prostacyclin pathway, we have an abundance of therapies available that can be taken in oral forms, inhaled forms, or in a pump form. And the third is the endothelium pathway. We have what are called endothelin receptor antagonists. So I guess that brings us to the question of how does your healthcare provider determine which combination of PAH medications may be right for you? The answer is partially determined by your risk status. Now, in one of our previous broadcasts, we talked a lot about risk status. Essentially your risk status helps predict your chances of survival over the next five years. Now, your risk status is determined by multiple variables and tests that we often ask you to get. That will help us to decide if you are a low-risk, intermediate-risk, or high-risk for your disease progression, or for survival over the next five years. Knowing your risk status helps your health care provider, adjust your treatment plan to find the right combination of medications for you. Today's PAH treatment guidelines recognize the importance of risk assessment to help determine your treatment plan. That brings me to a very good friend of mine, a fellow pulmonologist, Dr. Sean Studer. He's here to discuss PAH treatment guidelines. Sean Studer, MD, MSc: Thank you, Dr. Melendres, and very nice presentation of the first part of this as you always do. I found it always informative. So I'm going to talk a little now about the pulmonary arterial hypertension treatment guidelines, or the PAH as it's abbreviated, treatment guidelines. So the first question is what are these things? Well, they're documents that guide healthcare providers on diagnosing and treating a disease. They're not so much like a recipe. A recipe might be something you do when you're baking, you add the same ingredients over and over. Same temperature in the oven, you cook for the same time, and you get something that's very predictable. Guidelines accept the fact that people are a little bit different. They're not exactly like baking a cake. You can't have the same recipe for every person, but it does give us some guardrails and some ways to say, "This is the practice patterns that are suggested and likely to achieve the best outcome based on what we know." On the right side there, you see an example of the 2015 European Society of Cardiology, ESC/European Respiratory Society, ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Now, who develops these guidelines? Well, top experts in the world who stand for and about research often involving thousands of patients. So it's not just their own experience of who they're treating in their own group or their other own center, but it's what they're reading. It's often the research that they themselves are involved in. Examples of meetings that have reduced these guidelines are the World Symposium on Pulmonary Hypertension, the European Society of Cardiology, European Respiratory Society, as shown over there on that right panel, and the American College of Chest Physicians. Now for someone like myself practicing on the East Coast, if I ever wonder, “I wonder what Dr. Melendres would say? I wonder what one of my colleagues in California who practices an expert center would say about this,” I can't reach them on the phone immediately. I can't catch them by email in front of a patient, but looking at the guidelines and gives me their impressions as a consensus with others of how people are thinking about a specific area. It's also a good touch point. If the patient says, “well, is there a reason you're taking this approach,” I can tell a little bit about my personal opinion and what I think of the medical literature, but citing a set of guidelines gives it the weight of a group of my colleagues who are very knowledgeable about pulmonary arterial hypertension, and I can explain that to them as the reason why I'm making a recommendation for diagnosis and/or treatment. So why do we have them? I've explained a little bit of it already, but guidelines help us ensure that you receive consistent effective care, to help us stay on top of advances in care and best practices. You see over there on that right panel another example from the American College of Chest Physicians, and these guidelines are produced at slightly different periods of time. So there might be one meeting that occurs last year, one that occurs this year, and one that occurs next year. So just that slight variation in the research occurs between each year might give us a little different insight into how this group of experts answers a question. It's not like the Supreme Court, it's not the same group of people each time that do this. It's a group of experts that often changes a bit, with a lot of overlap. So when you add in a few other voices, you often get a little nuance of opinion. I wouldn't say the guidelines are really ever in conflict, but slightly different nuances or variations in approach are sometimes seen between the two guidelines. So especially read by someone outside of the field, it might seem like there isn't any consensus on some of these items, when I think actually what you're seeing is slightly variations on a theme. So what do these guidelines say? Well, the 2018 World Symposium on Pulmonary Hypertension Guidelines said something very important, which is that most newly diagnosed patients with PAH at low or intermediate risk status should be treated with oral combination therapy. So something that can be taken by mouth, the oral piece, in combination two medications upfront as part of their treatment regimen. You see a little asterisk right there. It has the footnote at the bottom that according to the treatment guidelines, initial monotherapy may have a role in treatment for certain patients. So if someone was started on one medicine that's not in violation or inconsistent with the guidelines, but it doesn't describe our approach to treating the majority. So if you talk about a practice like mine, or many experts with whom I speak with, our goal for many of these patients is to start most newly diagnosed patients on an oral combination upfront, or meaning in the early period. That's if they're low-risk or intermediate-risk. If they're high-risk patients, an infused prostacyclin class medication, one of those that's given my pump often with a catheter or a tube in the vein, or a needle or some device into the skin, that medication class should be included in those patients because they've been shown to be effective for patients in a state to improve their functional class and their state to a lower-risk state. That is from high-risk to intermediate or low. Now it's important that whatever point at which you start, so let's say you're on a dual oral combination. You're taking two medications by mouth, that every three to six months to the right side of that panel, a risk assessment is performed. Now if you were an intermediate-risk status, which is not our goal, our goal is always low-risk status, and you took dual oral combination therapy and you improve to low-risk status, that suggests that we're on the right track and the effectiveness of the medications for you is moving in the direction we want it to be. If on the other hand, you're an intermediate risk status and at a three to six month follow-up, and when you first started medications, I tend to weigh a little bit earlier on that range. I don't want to go more than six months, but I’d certainly go closer to three months, when I can, to stay in that range, that visit, if it shows you're still an intermediate-risk, I might be making decision whether or not, like the bottom says, you're not yet at low-risk. We may need to change to dual or triple combination therapy. So my example, if you start in the dual combination, which is where most patients are going to be treated, and you've not yet achieved low-risk in the follow-up, I might want to add a third agent and you would then be on triple combination therapy. So why these multiple medications? Well think of it like if you had to bring your car in for repairs, and you might say, "There are several things that aren't working well. It's not running at its best." You could certainly decide you're just going to start with one thing and see how the car is running. But if it's recommended you need spark plugs, an oil change, and a brake adjustment, you might decide all those things are important to keep the car running well. So you'd ask the mechanic to adjust all of those things at once to keep things rolling smoothly. Multiple medications in PAH take advantage of the fact that we know that there's multiple pathways. Research has shown that taking multiple PH medications can keep patients healthier longer. As you see there in an analysis of 17 studies involving more than 4,000 adult patients found that those taking multiple PH medications had a 35% lower overall chance of getting worse than patients who were taking only one medication for PH. So if your risk status was up here, taking multiple medications lowered it by more than one third. That is 35% reduction in the chance that you're going to get worse. Certainly if we have a disease such as PAH, that's incurable, we want to keep you in low-risk status and not progressing. So that's an important thing to make sure that you're not getting worse, and try to give you the best treatment regimen when we can. That analysis quoted also showed that multiple PH medications helped improve the six-minute walk distance, that hallway walk test back and forth sometimes in the hospital in the physician's office. That helps reflect for us whether or not your functional capacity, your ability to walk and have your breathing work well and not have you be too winded are all moving in the right direction. So a longer walk distance suggests that you're functioning better and may improve Functional Class. Sometimes abbreviated as New York Heart Association, NYHA, or World Health Organization, WHO, the Functional Classes are I, II, III, IV, again. FC IV is the worst. If you were say a Functional Class III, we would like to see you improve Functional Class to a Functional Class II, or if possible to a Functional Class I. Multiple medications improved the chance that that could occur. We also want to reduce the risk for PAH related hospital admission. No one likes to be hospitalized, and in PAH, that suggests that we can't manage you effectively as an outpatient, or just through the office, that you need to be hospitalized. So minimizing that chance, or at least reducing the risk of it is an important thing we are trying to achieve. Certainly today we have multiple treatment options that offer us quite a bit of flexibility. It's much more than we had five years ago or even 10, 15 years ago. So you see the types of medication listed there. You have phosphodiesterase type five inhibitors, or PDE5Is, soluble guanylate cyclase stimulators, SGCS. I don't suspect you say most of those two phrases very often, but sometimes the abbreviations are used. Prostacyclin class therapy, often abbreviated PCY. And endothelin receptor antagonist therapy, or ERA. How do we give these medications? Well, there's multiple ways they can be taken in some classes, and not so many for others. So in the PDE5 or SGCS, there's only medications that can be taken orally, or that is by mouth. In the prostacyclin class, the medications can be delivered by all three routes for which we currently deliver pulmonary arterial hypertension medications. That is, they can be given orally, they can be infused, or they can be inhaled where the medication is delivered directly to the lungs. Then there's endothelin receptor antagonists, or ERA, that are again, only oral therapies. That is those taken by mouth. So why is the right combination of those treatments so important? Well, the right combination may help you improve symptoms. That may help you feel more confident about running errands. It may help you feel more functional doing things in your home, whether it's chores, things you enjoy. It may help slow disease progression. Very important. PAH is unfortunately currently an incurable disease, so if we can slow the progression of that disease, we are moving in the right direction in trying to keep you as healthy as we can, for as long as we can. That's why the right combination is important. It might also enable you to do more in your day. So you can be out there with family and friends. Certainly in the present environment, the amount you can get out and about and the risks involved are somewhat of a challenge. But the idea is... /episode/index/show/phawarepodcast/id/17066534

Episode 355 - PAH Today: The Pathways To Treatment 12/09/2020

Sarah Sizemore - phaware® interview 354 12/08/2020

Sarah Sizemore - phaware® interview 354 PAH patient Sarah Sizemore discusses being diagnosed with pulmonary hypertension at a young age in Denver, her family's decision to move from altitude and how positivity helps her face this disease. My name is Sarah Sizemore. I live in South Carolina. I have idiopathic pulmonary arterial hypertension. I was diagnosed at four years old in Denver, and then we had to move to South Carolina for sea level. My dad owned his business in Colorado and my mom's an ER charge nurse. I started having seizures. Dr. Dunbar Ivy is the one who diagnosed me. He said at the time just being diagnosed, I couldn't handle the altitude. I was diagnosed in spring and that next year we moved. We sold our house. My dad quit his job. Then, my mom relocated to a hospital here in South Carolina. We moved to South Carolina, because my mom loves the beach. We did an in-between in North Carolina, a couple of hours, there's mountains so we decided it was the perfect spot. I have a sister and brother. I'm the baby. My brother's 27 and my sister's 23. I think their thought process was do whatever to keep me healthy or to try to get me stable. [My family] just decided that whatever needs to be done will be done. We've been here ever since I was four, so we've set roots. Then my siblings', also, same thought process was, whatever we need to do for me, we'll do it. Even though my mom was an ER nurse, she had no affiliation with pulmonary hypertension. She didn't know what it was or how rare it was. She did not have knowledge until I was diagnosed. I was having blackout spells and then I was diagnosed from a seizure. I think when I was diagnosed, she had a meeting with Denver to try to see what it was. Over the year when we moved, doing any research she could to find out. Now she's like another perfection of PH. It's really nice having a caregiver who also has medical training just in case anything happens at home. Early years, I remember mainly being on IV [therapy]. Flolan is what they started me on. I always remember wearing a backpack, having a central line, knowing that the medicine in the backpack was very important and to protect it. I think I was on Flolan for about five or six years. Then I switched to inhaled therapy. I went to oral pills. Then after that, I went to subcutaneous [therapy] in my sophomore year of high school. I'm back to IV Remodulin now, two years ago. The school staff, the administrators, they had meetings. Every year with school, we have meetings with the school board. Then with me, I think it was maybe open and closed. With the backpack kids could see. I just told them it was, my body needs this medicine and that was it because I was so young. Mainly closed in middle school. Then high school is when I opened up and told my whole class and everyone, all the students and made them aware about PH. Junior, senior year is when I started being in and out of the hospital. My senior years is when things have taken a toll and my PH has gotten worse. They found out from me being in the hospital and also me telling them. They were at first shocked because of course, I've been with them since seventh grade. They didn't even notice that I had a chronic illness at the least, or PH. They were welcoming. They were so loving. I wish I would have told them sooner, but they were awesome. Then they surprised me. The whole school got shirts made with #SizemoreStrong on it and surprised me one day with the whole school wearing them after they found out about my illness. I definitely cried. It was truly incredible to see that they were trying to be aware of it. My parents also cried. Pretty much everyone cried. Then I was given an award my graduation day called Tried By Fire, which is where a student, they excel dealing with a struggle or a chronic illness. They gifted that to me. The whole school was crying. Pretty much everyone was crying. I couldn't make it to that ceremony, because I was really sick, but my parents and family were there. It was truly remarkable to see. I am not in college right now. I'm a little nervous about that due to my health right now. I think right now, I'm trying to get my health more stable, trying to get the PH more stable. Any daily activities right now takes a toll on my body. I don't want to over-stress it, but I would love to go to college and study, but right now, I think my PH is too aggressive right now to do that. I think my passion right now is I work at my high school as the assistant director for the theater program. I started working for our theater program sophomore year. I was backstage stage manager. I worked all through my high school career. Then after I graduated, I wanted to officially work there as an assistant director and stage manager for the theater program. I grew up with all those kids when I started sophomore year. I hope go into theater in college. That's probably my one passion that I love. Getting diagnosed is stressful, but lean on the PH community. Ask questions. Try to get to know everyone. The PH community, for me, has been the greatest blessing with having PH due to the support and the huge family that I've gained by PH. Then just searching through therapies. I know it's definitely the hardest diagnosis that you could have, but having the PH community and just the will to fight and not to let PH win. Lean on others. Try to find therapies. Ask questions. Take a step back, try to focus and just fight. It's big, but I will never let PH win. Try to stay positive. I know that's hard, but I think, with me, what I've been trying to do is spread positivity. Fighting PH while spreading positivity or trying to make someone else's day better by trying to be positive every day. I think those are the biggest ones. My name is Sarah Sizemore and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . 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Episode 354 - Sarah Sizemore 12/07/2020

PAH Today: Shape the Future - phaware® interview 353 12/02/2020

PAH Today: Shape the Future - phaware® interview 353 SPECIAL EDITION: How Decisions Today Can Shape the Future The PAH Initiative and United Therapeutics are excited to bring you the PAH Today National Broadcast Series. This series of virtual events is intended for adult patients with pulmonary arterial hypertension (PAH) and their caregivers. Advancements in the care and treatment of PAH are improving the lives of those impacted by this life-threatening disease. The National Broadcast Series will include insights and perspectives on today’s approaches to managing PAH from nationally recognized PAH healthcare providers. Join patients and caregivers across the nation to hear current considerations in the care of PAH. This is the second of 4 PAH Today broadcasts to learn about risk status, an important part of today's approach to treating PAH. You'll hear how risk status gives your healthcare provider a new way to assess your progress, and how it could help determine whether more can be done to improve your symptoms today and down the road. Presented by Dr. Lana Melendres-Groves the Director of the Pulmonary Hypertension Program at the University of New Mexico and Nurse Practitioner Melisa Wilson. Learn more at: Lana Melendres-Groves, MD:Good afternoon, and welcome to the PAH Today National Broadcast Series: How Decisions Today Can Shape the Future. First, I would like to give a disclaimer that this presentation is sponsored by and made on behalf of United Therapeutics. Healthcare professional speakers are compensated by UT. Not all drugs are appropriate for all patients. Speak with your healthcare professional to determine which treatment plan is right for you. Patient experiences relayed during this program may not represent the experience of all patients. Today's presentation consists of three parts. The first is talking about what's beyond being stable, which at times can be a very difficult concept to understand. I like to recommend to my patients just to think about how they feel, and that's where we'll start from today. The second portion is going to be risk assessment. And again, what I'm doing is trying to present something that is hard for, often, providers to understand, but hopefully both Melisa Wilson and I can break it down in a manner that's understandable. And third, we'll talk about your risk status goals. This is what your journey is about and is very individualized to you. So let's get into what's beyond being stable. For me, I love to talk to my patients just about how they feel. Where are they now as compared to where they were when they were diagnosed or prior to starting treatment? Also, are they feeling better? Do they feel the same? Or are they just having to adjust to a new normal? And what is that new normal to them? Now please, don't get me wrong, getting to stable is an important milestone in your PAH. Being stable may show that you're doing better than you were and that your treatment is going in the right direction. You must give yourself credit. You have been motivated and invested in your own care and you've come so far. But we can't rest there, we have to continue to move forward and improve your overall quality of life. In order to continue to get there, we ask always so much of our patients, and we realize that. But this is how you can really improve your overall long-term care and prognosis so that you do well for a very long time. Many of those aspects of care have to do with adjusting your lifestyle, as may be necessary. For instance, we talk a lot about eating a low sodium diet, or how can we get you exercising a bit more? Also important in that is making sure that you see your healthcare provider in a timely manner. I know that right now that can be difficult, but it is so important that we make sure that all of these testing, that often becomes labor intensive for you, that they get done so that we understand how far someone might be walking on their walk test or how they're doing in terms of their overall functional class and exercise tolerance. There's always more to do, and we have room for improvement. So if you were ever thinking, "I wish I had more good days than bad," or, "Just a couple of more good days would really make the difference in how I feel." Are you ever declining invitations or avoiding making plans? Or do you just feel like, "I would really like to be able to do more around the house or out in my garden." This is where it's so important for us to talk about something called risk assessment. This is how we incorporate all those tests and how you feel into something called a risk status. It tells not only the patient but also the healthcare provider so much more about whether we're on the right track with your treatment plan and how we might be able to improve your symptoms even further. So, what is risk assessment? This concept is at times very difficult to understand, even for me, who's been doing this for quite some time, but it's a different way of looking at PAH. We now know that this risk assessment is so essential to how we treat our patients that it's actually become part of our treatment guidelines. It's recommended that patients receive a risk assessment from their provider on their risk status at least every three to six months. This measure here's how well your treatment plan is working for you and it informs your healthcare provider if potentially changes should be made. This is just an example of what a risk assessment looks like. It's a table that often we color code in green, yellow, and red, because I think it helps us, just visually, to understand if we're getting somebody to where they need to be. There's a lot of information in this table, but we use all of those tests that we ask you to get to be able to fill it out. And it often looks something like this, where maybe you're feeling quite good and are in a low risk category, in the green category for how you feel and what you can do. But maybe some of the blood tests we've gotten or some of the other procedures that you've done would indicate that actually you're in more the yellow zone. We take all of those together and are able to come up with a score that indicates a risk status. Knowing whether someone fits into a green zone, low risk, an intermediate zone, the yellow, which is intermediate risk, or in the red zone, high-risk, we're able to know if somebody will do better and have a better chance of survival over the next five years or if maybe there's a lower chance of survival over that timeframe. I know that I talked about this in somewhat of a scientific way, but I would really like to invite my colleague, Melisa Wilson, who is a doctor of nursing to give a little of an example of how she talks to her patients when it comes to risk assessment and risk status. Melisa Wilson, DNP:Hi, I'm Melisa Wilson, nurse practitioner and program coordinator for the pulmonary hypertension program at AdventHealth Orlando. I would like to talk to you today about doing risk assessment with my patients. In the past, when we discuss risk assessment, we used to talk really in terms of how a patient's feeling, what we call your functional class, meaning you tell me if you feel short of breath with certain activities and then I take that and put that into a category. But as humans, we tend to be very smart. If there's things that make us feel bad, we just don't do them. So instead, what we have learned over time is that there's multiple what we call variables or things that we look at, including your labs and PFT findings and echocardiogram and of course your heart cath information that we can put all together and actually come up with a composite score, which puts you into a category of a high, low, or intermediate risk patient. This is more prognostic, meaning, telling me how you're doing not just today but how you're going to do long-term. This number and this grouping is not permanent. It's not that if you were categorized as high risk that that's where you are forever. In fact, what that does is it gives me, as your clinician, the opportunity to take all of this data that we used to get in pieces or parts. I get your cath data today, I get your lab some other time, I get your six minute walk distance, and then have to put that together to say, "Hmm, am I doing enough for you as your provider?" This category or this scoring allows us to put that altogether and take a look at everything at once. And then it tells me, if you're high risk for example, that I have a lot of opportunity as your clinician to partner with you to make you better. I can do that through therapies, through rehabs, through diet. All of those things make a difference. So when I get you at high risk, my goal ultimately is to move you to low risk. Low risk is where you have the best outcomes. That's going to happen over time. We're going to go from high risk to intermediate risk to low risk. And it's our job together to get through that. I'm still going to consider, how are you doing? in all of that, but that's just now going to be one part of the puzzle. So it gives me the opportunity to look at you wholistically, as well it gives you the opportunity to have something to work towards in a notable way to notice your improvement in how you're doing overall with pulmonary arterial hypertension. So it is an exceptionally helpful tool that we didn't used to have in the past that I've become quite passionate about. In fact, with my whole clinic team, we all do this together, 100% percent involvement, and having these great conversations with all of our patients so that you understand how you're doing, you can make modifications as well. I find it changes things like your diet choices. Sometimes people, we make choices that we know aren't always the best things for us. Maybe it has a lot of sodium. Well, now understanding that sodium intake could increase what's called a BNP or an enzyme your heart gives off when it's under stress or failure, now will make many people, encourage many people to not eat those high sodium foods knowing that it can now impact how they're doing long-term. I also find that it helps with things like improvement in six-minute walk distance, because it helps with the compliance with rehab because you have a goal, something to achieve. Ultimately, our goal with treating you is to get you from high risk to intermediate risk to low risk, and low risk is where we want to keep you. Low risk we know is associated with better long-term outcomes, meaning that you're really doing very well from a pulmonary arterial hypertension perspective. So we will partner together to get you through therapies, through rehab, to get you engaged in your healthcare so that we can ultimately get you to that low risk and help you live your best life with PAH. And that's how I talk to my patients about risk assessment. Thank you, Dr. Melendres-Groves for the time today. Lana Melendres-Groves, MD:Thank you so much, Melisa. We really appreciate your insight into how you talk to your patients. It really adds to the conversation. All right. Well, we've come to the third portion of this presentation, which is a discussion of your risk status goal. Now, I know that both Melisa and I talked a bit about risk status and risk assessment. We showed you a table and we talked about some of the numbers that we'd look at to define how well we think someone will do over the next five years. Now it's a chance for us to really talk about how you individually may do, and that comes to you talking with your healthcare provider. This is essential for everybody. And often our goal is to reach the green zone, that low risk status, but I think most importantly is, are we improving your risk status? This is something that should be a goal for everyone. Overall, we know that achieving more low risk criteria improves your chance of survival. This is a graph from one of our European registries that shows your prognosis can be determined in terms of the number of low risk criteria that were looked at at their first follow-up visit. You can see that those patients who were able to achieve three low risk criteria had a 97% survival out to five years, while those who were unable to achieve that didn't do quite as well. Some other things to think about, maybe, how do you prepare for your next visit? I really ask that my patients think of questions that they can write down such as, what is my risk status? Or, is low risk status the right goal for me? I think that also understanding your walk distance and is it improving? I ask my patients to get echocardiograms frequently. So understanding what those tests results mean may be important you. Or, how can you improve your functional class? When you do improve your risk status and experience fewer symptoms, it may help you to do more in your day and build confidence about your future. In summary, today, PAH specialists perform a risk assessment and then calculate your risk status to show how you're doing today and whether you're on the right track, whether more could be done to improve your symptoms and increase your chances of survival. Risk status helps the healthcare provider determine whether changes in your medication or treatment plan could help you reach more low risks goals, lower your risk status, or improve your symptoms. Please join us next time for our third virtual event. We will be talking about pathways to treatment. You can register today or any day at . And please consider joining the PAH initiative at , where you can stay informed, stay motivated, and stay focused on your future. Thanks for joining us. 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 /episode/index/show/phawarepodcast/id/16945925

Episode 353 - PAH Today: Shape the Future 12/02/2020

Krosby Myers, BSN, RN - phaware® interview 352 12/01/2020

Krosby Myers, BSN, RN - phaware® interview 352 Krosby Myers is a registered Nurse at UC San Diego Health System. Though she works in their surgical oncology unit, Krosby had no idea that UCSD was a leading pulmonary hypertension center, until she was diagnosed with PH in her 37th week of pregnancy. In this episode she discusses her journey to diagnosis and how her nursing perspective changed in light of becoming a patient. My name is Krosby Myers. I am calling from San Clemente, California. My connection to pulmonary hypertension is that I'm a patient. I was diagnosed in 2019, when I was 37 weeks pregnant with my second child. I also actually work at UC San Diego Medical Center, where I was actually receiving my care and I still receive my care. But I work on a surgical oncology unit and we also treat patients who have had kidney and liver transplants. Throughout my pregnancy, actually, both of my pregnancies, I definitely felt short of breath and I thought it was due to my pregnancy and just being a little bit heavier with the baby weight. During the second pregnancy, every time I went to my OB appointments, my resting heart rate would be in the one teens, and my doctor just thought it was because I just went up the stairs. But I just was very short of breath, even walking from the car to the grocery store, I would have to take breaks. I still just thought it was due to my pregnancy. But thankfully my OB doctor at my 37 week appointment, she felt that I should just go into the hospital to get an EKG to be safe. Once I got that EKG that was abnormal, so that led to an echocardiogram, which showed I was in severe right heart failure. Then all the pulmonologists came in and told me how serious it was and that the next day I would have to get a right heart catheterization. That's when I was diagnosed and that started my whole PH journey. It was really scary when they first came in, when they saw my echo results. The first critical care pulmonologist that saw me, she wasn't a PH specialist and they came in to see me after her, but she definitely told me how complicated and dangerous it was. So at that point, even though I'm a nurse, I wasn't a cardiac nurse, and I just kind of broke down at that point, because I was so scared. All I knew about pulmonary hypertension was that most people needed lung transplants. All I could think about was I had a one and a half year old and I was 37 weeks pregnant with my second baby. She just kept saying, "This is so complicated." She wasn't a PH specialist. Once Dr. Papamatheakis came in to see me, he kind of gave me some hope and a different perspective. But they definitely made me very scared. They were talking about having to deliver the baby the next day. They were going back and forth with the plan, but ultimately they were trying to figure out if I needed a right heart catheterization. They decided that they wanted to go through with it even though I was 37 weeks pregnant. So the next day, I got my PICC line and then I went for my first right heart cath. They have the OB team in there, they were monitoring the baby. It was my first one, and I had to lay flat while I was pregnant. I was very anxious and I felt very out of breath, just laying flat and trying to hold still, and just not knowing what to expect. But thankfully, my pressures decreased when I got to nitrous gas, they decreased in half. So Dr. Papamatheakis was really excited about that, because I think it's only 10% of people with pulmonary hypertension respond to that gas. It's a good sign for how we respond to medication, so that kind of gave me a little bit of hope during a really scary diagnosis. Immediately, even during the right heart catheterization, they started me on Flolan in the cath lab. They decided the safest way to go was [to] start the Flolan. I was on that for as long as possible before I went into labor. The goal was to be on Flolan for two weeks. They had a set date, May 1st to have a plan to C-section. They wanted to optimize my pressures as much as possible. So every day, twice a day, I was titrating up on my Flolan. So for two weeks, I was in the hospital and I delivered at 39 weeks on my scheduled C-section date in the main OR. They had all the teams there. They had ECMO access in my groin just in case. My husband wasn't allowed in there. They had [an] arterial line. Everything was set up just in case something went wrong. But thankfully, I was awake and I didn't get intubated or anything during the C-section. It went very smoothly. I was on Flolan through a central line for four months. Then, I was able to transition to oral medications last August after my right heart cath. At first, it was really difficult. I had to stay for 25 days in the hospital and my husband had to take my newborn home with my one and a half year old. I also was learning how to mix my own medications at home and we had help, but it still was very difficult and scary being on Flolan continuously. Thankfully, now I'm on oral medications and I'm feeling a lot better. It's changed everything for me as a nurse. I have a whole different perspective, because I was a patient for so long. I'm so much more empathetic towards patients and family members, because I know what it's like to be in the hospital to be going through something that is so scary and there's so many unknowns. I wish every nurse could go through something where they could grow in their empathy, because it is scary. No matter any reason you're in the hospital, it is so scary to be there. I feel like nurses really need to grow in empathy, because these patients are going through so much. Actually, I've told one of the nurses, (one of the PH nurses at UCSD), Sandra Lombardi, I would love to mentor and encourage any new patients that come through and are going through a new diagnosis with pulmonary hypertension, because when I was a patient, Sandra brought up someone who was 15 years out from her diagnosis and was doing amazing. It really changed my whole perspective seeing that someone could survive and thrive with this diagnosis. I had no idea that UCSD was an amazing center for PH until I was diagnosed. I believe in God. I just feel like God put me there for a reason. I've worked for UCSD for six years. I started in Hillcrest and I moved to La Jolla and that's where all the PH doctors are. It's crazy that I actually work at a place and was able to receive care at one of the best PH centers in the U.S. I would just say, don't give up, never lose hope. You always have to change your perspective, because sometimes you find yourself so down, just thinking about how long do I have to live? Am I going to survive this? How am I going to leave my kids? You just have to listen to your doctors. Never give up hope, and continue to take it day by day and just live in the present. I really had to learn to do that. I had to ask in support groups for advice, see how other people were doing, because you can't Google this. You will get very depressed if you Google anything about pulmonary hypertension and see how long they give you online. You kind of have to live off the hope of other people in the beginning. I just wanted to thank my team, Dr. Papamatheakis, Cynthia, and Sandra, and everyone at UCSD that helped me get through my diagnosis and everyone that has encouraged me throughout this whole process. I would just encourage other people with pulmonary hypertension to give back because you never know when someone really needs that encouragement. My name is Krosby Myers 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 /episode/index/show/phawarepodcast/id/16990523

Episode 352 - Krosby Myers, BSN, RN 11/30/2020

PAH Today: Beyond the Basics - phaware® interview 351 11/25/2020

PAH Today: Beyond the Basics - phaware® interview 351 SPECIAL EDITION: Beyond the Basics The PAH Initiative and United Therapeutics are excited to bring you the PAH Today National Broadcast Series. This series of virtual events is intended for adult patients with pulmonary arterial hypertension (PAH) and their caregivers. Advancements in the care and treatment of PAH are improving the lives of those impacted by this life-threatening disease. The National Broadcast Series will include insights and perspectives on today’s approaches to managing PAH from nationally recognized PAH healthcare providers. Join patients and caregivers across the nation to hear current considerations in the care of PAH. This is the first of 4 PAH Today broadcasts to learn more about your symptoms and how your healthcare provider can use them to monitor your PAH. Presented by Dr. Lana Melendres-Groves the Director of the Pulmonary Hypertension Program at the University of New Mexico and Dr. George Ruiz Chief of Cardiology at MedStar Union Memorial Hospital. Learn more at: Lana Melendres-Groves, MD:Good afternoon, and welcome to the PAH Today National Broadcast: Beyond the Basics. My name is Lana Melendres-Groves, and I work at the University of New Mexico as an associate professor and medical director for the pulmonary hypertension program. On behalf of United Therapeutics, myself, and everyone joining us today, I want to welcome you and thank you for being here. Whether you're on your own PAH journey or here to support someone you love, we appreciate you being here and the time that you're taking out of your day to join us. As our disclaimer, this presentation is sponsored by and made on behalf of the United Therapeutics. Healthcare professional speakers such as myself are compensated by UT. Not all drugs are appropriate for all patients. Speak with your healthcare professional to determine which treatment plan is right for you. Patient experiences relayed during this program may not represent the experience of a typical patient. Well, I'm excited to get started, and first I'd just like to go through a bit of an overview for the presentation today. We have three separate areas. The first is going to be about PAH and the disease state overview. This will talk about how the changes in your heart and lungs cause the symptoms that people experience from PAH. The second section is going to delve just a little bit deeper into the science behind your symptoms. Don't get scared. I promise it's not going to be too bad. The third is going to be keeping track of your symptoms and why that becomes so important for you and for your healthcare providers information. All right, so let's get into it. Here's the overview for PAH. Now, when I talk to my patients, I find that it's really important for them to really understand what we're doing together and why they feel the way they feel. So to start with, we talk about how the heart and the lungs, they work as a team. They're a pretty complex team, but nonetheless, they're equally as important. I think that what I'd like to do today is start with the heart. And we'll talk about how that may be affected by PAH, and how that may lead to the symptoms that each person may feel. So first, the heart has four chambers. I'm sure many of you already know that. Sometimes we talk about it as the right side or the left side, sometimes we talk about it as the upper chambers which are smaller, or the lower chambers which are larger. I think that no matter how you talk about it, you just have to understand what the job of each side of the heart is. In this illustration, you can see that a portion of the heart, one of the larger chambers, a ventricle, is on the left side. It's depicted in red, because typically we indicate oxygen rich, or blood that's filled with oxygen as being red. On the left side of the heart, you have the left atrium, which is the smaller chamber that receives blood from the lungs. This blood entering the left atrium is going to then pump that blood through to the left ventricle. The left ventricle will then pump that blood out to the body to distribute oxygen to the tissues, the organs. It keeps us going, keeps us alive. Most of the time we hear about heart disease or heart attacks, and really what people are talking about is a disease of the left side of the heart or the left ventricle. This isn't what the problem is in pulmonary arterial hypertension. It's actually the right side of the heart. So why don't we jump over to the right side of the heart and see what's going on there? In the right ventricle, depicted in this illustration as blue because it's low in oxygen. It's called oxygen poor blood. The right ventricle is what becomes affected in pulmonary arterial hypertension, and just starts to not work the way we need it to. Overall, the body is dependent on oxygen to survive. When the blood is distributed to the rest of the body and gives that oxygen off to the tissues and organs, it then needs to return to the heart to be pumped into the lungs. That's where the right side of the heart comes into play. The blood returns to the right atrium, which is the smaller chamber, and that right atrium then squeezes and pushes the blood into the right ventricle. The right ventricle then is able to contract or squeeze and push the blood out into the blood vessels of the lungs, where oxygen is waiting, and those red blood cells are able to absorb the oxygen and now become oxygen rich and head over to the left side of the heart, where the entire process goes on again and again. In patients with PAH, things change a little bit. What we know is that the blood vessels of the lungs become affected. They start to narrow, they start to thicken. So now those tubes or vessels that blood easily flowed through to pick up oxygen are very small, narrow, and unable to do that job as well. What happens is that the right ventricle, being a muscle, says, "I'm just going to increase the pressure. I'm going to send that blood through those smaller blood vessels, because that's what my body needs." Unfortunately, what happens is that over time, that right ventricle having to work overtime day in and day out, I always describe it like running a marathon, which I could never do, but at least I could have a time point that I could rest. The right ventricle is in a situation that it continues to run this marathon without an end in sight, and over time starts to become enlarged and not work as well. This is where patients begin to have symptoms. They start to feel that shortness of breath and they start to feel the other fatiguing symptoms that often take them to a physician in the first place. But before we get there, I think sometimes trying to talk about the teamwork of the heart and lung may better be explained by a good friend of mine, Dr. George Ruiz. He has a really interesting take on something that we actually see day in and day out, and is applicable to our heart and lungs. So at this point, I would love to turn it over to Dr. Ruiz. George Ruiz, MD:Hello, my name is George Ruiz. I'm the chief of cardiology at MedStar Union Memorial Good Samaritan and Harbor Hospitals. And more importantly, I'm a heart failure physician that specializes in the care of patients with pulmonary arterial hypertension. Today, we're going to talk about something really important to not just cardiologists, but to the patients they treat. Flow. Blood flow is a very important component in being alive. It is the currency of life. You need blood flow to get around your house. You need blood flow to walk your daughter down the aisle. You need blood flow to do those things that make life meaningful. As long as we're living, there is blood flow. The analogy of a highway really can help us understand. The heart pumps blood to the lungs through a series of arteries, which we can think of as highways. These highways in the lung have a large number of lanes. As a result, the blood flow between the right side of the heart and the lungs generally flows under low resistance. The analogy here is imagine that you were going to work in the morning during rush hour and the highway you took had not four lanes, not five, but 11, 12, 15 lanes. That traffic would flow a lot easier. There would be very little resistance to flow. That's what the normal lung blood vessels look like. They're generally low resistance blood vessels, and there's a lot of flow. The reason why that's important is because we need that flow to the lungs, because in the lungs is where you take in oxygen, and where that oxygen gets connected to the blood, and then ultimately delivered to all your tissues. As a result of any one of a number of insults, the blood vessels shift, and they remodel. This is a medical term, remodeling. If we go back to our highway analogy, what remodeling looks like is imagine that there's an accident on the side of that same highway. What happens when you have an accident is that you get lanes closed. The police show up, they start closing lanes. So now imagine that same commute that you have in the morning, and instead of 15 lanes because of accidents, now you have 10, or eight, or five, or two. As you close more lanes on the highway, the resistance to flow increases. As you increase the resistance to flow, you create a backup behind where the accident is. This is stuff that intuitively we've all been on a highway where there was an accident, and what do you do? You sit there. The flow gets reduced. Sometimes the flow gets reduced to a very slow trickle. Sometimes the flow gets reduced to a standstill. As a result of this, there is less flow. And as a result of there being less flow, to patients that means there's less go. Flow equals go. You need the flow to do the things that you want. So as the flow reduces, the patients get less. They do less, they walk around less. They go to the supermarket less. Things that they used to be able to do become very challenging, because they have limited flow. So what we do is we try to assess what the resistance is in the blood vessels in the lung. We attempt to assess how much flow there is. A lot of that, we can get initial glimpses of it when we do the transthoracic echocardiogram, the pictures of the heart. We get an idea for the backup of the traffic, the right ventricle, which is the main pump to the lungs can sometimes become very dilated and distended, and that can often be a sign that the flow is being limited by resistance. Then ultimately, we send patients for a right heart catheterization, where we can actually measure the flow. By looking at the pressures in the lung and the flow, we can calculate the resistance. Now these numbers are incredibly important to me as a heart failure specialist, because they helped me understand the severity of the disease, they help me understand how limited your circulation is, how limited is your flow? It also is important to you, because it helps guide how I choose how aggressive to be with regards to medications, whether we make changes. But the most important reason why it matters is because it tells us a story about you. It tells us about where you are. It tells us about where you are with your disease. It gives us an indication about how aggressive we have to be. I hope that helps you understand a little bit better how all the plumbing is connected inside of you and how that's related to the way that you feel. Also to how I think about your disease and how I use this to help guide what we do for you. Lana Melendres-Groves, MD:Well, thanks, George. I think we'll all agree that sometimes traffic jams are definitely a mess, but I think it really is a good depiction of what potentially is going on in our heart and lungs, and why all of us can be very frustrated at times in trying to deal with it. Speaking of some of those difficulties and frustrations, that often comes to talking about symptoms. For me, this is where I tend to have conversations in great detail with my patients, because for them, they may experience things such as shortness of breath or limitations in what they can do. They may describe hard or fast or irregular heartbeats. They may find that they have swelling in their abdomen or swelling in their ankles. For them, that is most important. For me, that is something that I really need to take into consideration. It becomes important as we move forward in not just diagnosing somebody, but in how we follow them further into their treatment plan. Many of these symptoms are simply a result of the right side of the heart not being able to keep up with the job that's being asked to do. Unfortunately, because PAH is a progressive disease, we expect things to worsen over time. So, many things that we would take for granted, such as doing our laundry or going shopping, are things that we can no longer do with ease. These are opportunities for us to consider what it is we may need. It helps our providers to make decisions and recommendations on your behalf. Overall, it's important to keep track of these symptoms. Because whether they're improving or worsening, that may affect how we recommend treating you. On the other hand, maybe your symptoms are staying the same. That doesn't necessarily mean that there aren't opportunities to improve the way you feel. So it is extremely important to try to monitor those symptoms and activities that you're able to do. All right, we made it through section one. So that brings us into section two, a little bit deeper dive into the science behind your symptoms. Now I know that many of you, science may not have been your favorite subject in school. So we're going to do our best to try and make this something that is applicable to you and why certain treatments may be the right options for you, or why others may be more appropriate. All right, let's get into it. So first we need to talk about what normal is, because only when we know what normal is, can we then talk about the abnormal. In this situation with PAH and with everybody, we have three substances that the body produces. Nitric oxide, prostacyclin, and endothelium. These are all produced in normal, healthy lungs, and when working properly are produced in just the right amount. These substances keep everything moving and flowing easily through the blood vessels. So what do they actually do? Well, let's talk a little bit about that. For nitric oxide, this is a substance that is produced in the inner lining of the blood vessels of the lungs, and it helps to keep the blood vessels open or dilated. Prostacyclin is another substance that's produced in the blood vessels of the lungs, and once again helps to keep those blood vessels open so that blood can move freely and easily through the blood vessel. Prostacyclin also has a few other jobs. It helps to prevent blood clotting, it also will prevent thickening of the blood vessels of the lungs. So that brings us to endothelium. This is sort of the counterpart to these. I always describe these substances as sort of a teeter-totter. It's not a very fun teeter-totter if they're working normally, because nitric oxide and prostacyclin would sit on one side, and the endothelium would sit on the other, and they weigh the exact same and nobody gets to go up or down. That's okay when we're talking about the body. We like things to be just in balance. So what actually happens then if that isn't the case? Well, that brings us to PAH. This the time that we see these substances becoming out of balance. We see that there's a decrease in the production of nitric oxide and prostacyclin, so there's just not enough around. We're not able to hold those blood vessels open, there may be thickening of the blood vessels and endothelium, on the other side, there's too much of it. So now we start to see thickening of the blood vessels, narrowing of those blood vessels, and now all of a sudden our teeter-totter is a really large adult who has two little children sitting on the other side, and now we've tottered over to the side of constriction and narrowing of those blood vessels that puts that extra strain on the right side of the heart. These substances are also opportunities for us, because researchers over the past 25 years have worked so hard to try and improve the lives of our PAH patients. We now know that although they don't work appropriately in our PAH patients, these are also the areas that we're able to treat patients, and with medications potentially affect the amount of each of those substances. Now today, we're not planning on talking about those medications. That will actually be in our third installment of PAH Today, our National Broadcast Series. So I'm hopeful that you're able to join us again to discuss the medications at that time. Instead, I think we should get into tracking of symptoms, and why it's so important. Before we get to it, I know, my patients, they're frustrated. I'm asking them to come in. They have appointments. They have tests. Now here I am about to ask you to do one more thing when you already don't necessarily feel at your best. But I think it's important to understand why tracking these symptoms can be so helpful. Not just for you, but also for your healthcare provider. When we talk about symptoms, your providers is thinking about it in terms of your functional class or what you can do. So in order to understand my patient, I need to know what their daily life is like. Do they get tired when they're walking to the mailbox, or maybe after we've started therapy those things have improved. This can all be related back to a functional class. Many of you may have already discussed with your providers and know exactly what your functional class is. Others of you maybe haven't had that opportunity yet. But in order to understand what it means to be classified as a functional class 1 or 3, I'd like to go into the 4 groups. For our class 1, these are individuals who realistically maybe aren't effective at all. They have PAH, but they're able to go on bike rides or long walks. They're able to do all their activities and not feel as though they become breathless. Our patients who are in our functional class 2, they tend to need to pause during their activities that are a little bit more strenuous, such as climbing stairs or shopping. By the time someone is in class 3, what we find is that they're really having to stop frequently. They may actually have stopped doing some of the things that they normally would have done because of their breathlessness or symptoms that they develop. In class 4, our patients are feeling short of breath or having problems even while they're at rest. By knowing what functional class you are, it can help in terms of long-term prognosis, how you will do, and what you might need to discuss with your healthcare provider. One way that you might track your symptoms is with a form that the PAH Initiative has created. This allows you to keep track of the date and what symptom you may be feeling. I have to be honest, sometimes I can barely remember from day to day. So to be asked what I could do three months ago, that's so far away from what I'm doing right now I'm not sure I'd remember it. But if I had a quick reference, I could think, "Wow, I could do a lot more back then," or, "Look how far I've come." So I think being able to keep track of this for you, it really helps your trajectory of how you're doing in terms of your PAH. Well, thank you all for being here and joining us today. I would love it if you would join us next time, and we're going to be talking about how decisions today can shape the future. You can register today or any day at . And please consider joining the PAH initiative at , where you can stay informed, stay motivated, and stay focused on your future. Thanks for joining us. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us @phaware on Facebook, Twitter, Instagram,... /episode/index/show/phawarepodcast/id/16940585

Episode 351 - PAH Today: Beyond the Basics 11/25/2020

Natalia Maeva - phaware® interview 350 11/24/2020

Natalia Maeva - phaware® interview 350 Bulgarian pulmonary hypertension patient Natalia Maeva is a founding member and current President of the Bulgarian Society of the patients with pulmonary hypertension. In this episode, Natalia discusses her journey with PH migrating to Greece for treatment and to Austria for a double-lung transplant, her passion for PH awareness and being a positive voice for the Bulgarian PH community. My name is Natalia Maeve. I live in Sofia, Bulgaria. Right now, I am a part of the PHA Europe Board, and I am the current president of the Bulgarian Society of Patients with PH. My connection to PH was when I got diagnosed in the year 2009. In the year of 2016, I went through a lung transplant in Austria. On the year of 2007, I started feeling very nauseous, very overly tired. I fainted, collapsed. It was very scary for a young woman. So I went to get diagnosed. Until the year of 2009, I was getting through diagnostics. In the year of 2009, they diagnosed me with PH. I was treated with Revatio until the year 2011, which I paid from my own money. The country didn't support any kind of money and payments through this treatment. In the year of 2014, I started treatment with Remodulin (treprostinil), and I got it from Greece because they didn't have it in Bulgaria. In the year of 2015, the government, they didn't want to pay for my transplant, because that's when they realized that the treatment that I got didn't work in any means, and I had to get a lung transplant. Currently in Bulgaria, there are two offices of PH, one in Sophia, one in Varna. The crew is very small. It's built mainly with doctors that do it to help on their spare time. Right now, there are a lot of problems with the oxygen concentrators in Bulgaria. They don't provide it to patients at all. That's what we do in charity companies to try and help PH patients around Bulgaria to get the treatment that they need, because the government doesn't help at all. Right now in Bulgaria, there are 150 active patients with PH. We are around 7 million in this country, so 7 million citizens. But mostly the problem is because the treatment isn't right in Bulgaria, a lot of the patients, their last option is to immigrate to a different country that they could get treated from. But the problem is that it's not easy at all. First of all, you're very sick. Moving to a different country is nearly impossible physically, mentally. You have to take your family with you, because you can't cope with everything by yourself. Financially-wise, it's very hard as well, because the treatments are very expensive, and the living expenses. When you don't get any support from the country, it's very hard. That's why the Bulgarian Society, that's how we help the other patients with charity, with funds, as much as we can help them. I went to get secondary help from a doctor in London in 2010. He was a Greek doctor. Then he sent me to Greece to get treated. And again, that wasn't paid for. I didn't get any help from the country. The reason that the country didn't fund or help me and other patients, especially in the early 2000s is because it's such a rare disease. When I went to Greece to get treated, after I got sent from the Greek doctor in London, they had decided that that's the treatment that I need to get. I needed a lung transplant, because after getting the treatment that I got in Bulgaria and in Greece, nothing worked, and that was the last option. I was diagnosed with the PH in Bulgaria, started getting treatments here, moved to Greece, got treated there. They realized that it wasn't working. Then it was decided that I need to get a lung transplant. It was 11 months. For me, it felt very, very long. I just wanted to get my new lungs and start my new life. So it was a very hard waiting process for me. I was connected to an oxygen concentrator. So it wasn't that pleasant of course, but I was pretty lucky. Let's say that. I'm a very activist person. I really like to share my opinions and I always fight for my rights. If I went through such a horrible thing and such a long process that was funded by myself, I think that my role I need to give for myself, and I want the country to hear my voice. PH patients need to know that they have somewhere to go to and someone to talk to that can take care of them. When I was sick, it was very hard to me because I didn't have that. So I want, as a correction for my new life. I got a second chance. I got a new chance in life, to help and progress and raise awareness for this rare disease, especially in a country like Bulgaria. I want to help people. Especially, when this is such a rare and vicious disease, you need all the support you can get, mentally, physically, financially, everything. Bulgaria, I don't know if you're aware, isn't that... How do you say it? Not that great. It's considered one of the poorest countries in Europe. So that's why I said that a lot of patients don't want to face with it here in Bulgaria. So the last option that they have is to immigrate to a different country with better economics, with better healthcare, because here in Bulgaria, it's very, very hard. Plus, lung transplantations aren't available in Bulgaria, sadly. One of my greatest accomplishments is probably raising the awareness here in Bulgaria for PH, because when I was sick, it was so rare that no one basically in this country really knew what it is except for the people that actually had it. We also made a lot of campaigns here in the country to raise awareness. That's what I feel the most proud of, that people actually know about this disease now. The doctors also have a great knowledge about it. Always think positively, because when the mind is healthy, when the mind is thinking great things, that I can conquer this and I'm brave, and I have great family and friends around me and people that care about me, I can conquer it all. I can conquer this disease, because at the end of the day, mentally is the most important thing that you can do. Just always think positively, always think that I'm strong and I'm brave, and I can go through this. I know that it's hard and it's very tiring, but I am strong and I can do it. I'm living proof of that. Of course, even me after getting the lung transplant, I never put myself in negative thoughts, because of course the process after the lung transplant is very important, because you need to feed your body with positive thoughts and great thoughts and keep on a healthy lifestyle of course, to maintain the treatment that you got. We are really trying and pushing through getting oxygen concentrators in this country. We only have three centers of oxygen concentrators. Of course it's a large country. There are a lot of city, villages. It's not enough. We're trying to push this. We're trying to get the funds for it, because the country really isn't helping us at all with this. So that's the main goal right now. Because the awareness is happening all the time. People know about it. Doctors, of course, know about it. So that's what we're really trying to push right now. And also IV therapy. For anyone listening to this, it's really important to me to share this message that just be brave, be strong, always think positively. I know that it's hard. I went through it. I got my second chance for a second life. I'm trying to make the best out of it, not only for me, but for other people in general, not just the PH patients. Always think positively. Just know that you can get through it. It's hard, but you're strong enough and capable enough to conquer it all. My name is Natalia Maeve, 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 /episode/index/show/phawarepodcast/id/16912679

Episode 350 - Natalia Maeva 11/23/2020

Jessica Romero - phaware® interview 349 11/17/2020

Jessica Romero - phaware® interview 349 Baby Jessica Romero was born October 26, 1983 in Denver, Colorado. Jessica was born 3 months premature, weighing one pound, 7 ounces. The Rocky Mountain News crowned her the “Miracle Baby of Colorado." Now, 37 years later Jessica discusses, her diagnosis, her outlook on a lung transplant and her commitment to pulmonary rehab. Jessica Romero: My name is Jessica Romero. I'm from Modesto, California, and I have pulmonary hypertension. I was a preemie. I weighed one pound/seven ounces. I lived in the hospital for the first two years of my life. My parents had a life and death situation where we had to move from Denver to California in the Bay, because the air was too thin for me to live in Denver. So we moved to California. With that, I was diagnosed with PH in 2010, so 10 years ago. My parents, they did it for me, of course, just to move mountains. So, we moved to California to make ourselves better, and for my health, too. That was pretty much number one. It was heartbreaking to leave my family, but they did it for their daughter. They came with nothing, pretty much the stuff on their backs and just managed to live their life having a sick daughter. We just did what we could. First couple of years I know, I went to UCSF. This is not my first time around. Since I'm on the lung transplant list, I'm pretty familiar with UCSF, because I've been with them mostly half my life. I was three years old, I think, four years old when we moved. I could tell a difference, Denver's air to going to California. It was pretty much a good change. I could breathe better, considering I was on oxygen. I think we were there as a family since '86 to '95. In 1995, my parents and I moved to the Valley for a better change. Better neighborhoods. They ended up buying a house. They're all in a two story house, just a better change. I have this really funny nickname and the reason why they call me “Bones,” is because I was always underweight, since I was little. I have always been skinny, always a size two, weighing in the 80s to the 90s. So finally, I believe in 2007, 2008, I finally reached 100 pounds, and my family and I did the whole party thing, because I always been underweight for so long. So, finally when that did come, when I weighed 100 pounds, we were so happy. I grew a little bit. I ate more, eating meat and more vegetables and some meat and potatoes. Sandwiches, I love sandwiches. A year into when I was diagnosed with PH in 2010, about a year or two, after that, my primary doctor, which is a pulmonologist concerted that my PH was progressing pretty bad, and that I should consider to look into a lung transplant. I kind of was in denial, thinking okay, I can wait out my lungs just a little bit longer. We looked into it about, I would say 2011 to 2012. There was a lot of going back. I wasn't sure. Maybe I could keep these lungs as much as possible. I finally got on the list of 2016 of May. A lot of things go in the process, the blood work, what type of blood you have, the testing, seeing the doctors, just a whole lot of factors that go into it. When UCSF decided as a team, my whole team of doctors and surgeons and stuff, looked at my case and say, "Yeah, in the long run you will need a double lung transplant." So, when they decided, that year 2016, I was put on the transplant list. I was a little skeptical, kind of thrown back, like I don't know. In the long run, talking to people such as pre and post, now that I know, I think I'm a little bit more determined, because I am exercising and seeing them working as hard as they do, going what they're going through, makes me want to work a little bit harder. As soon as I got on the lung transplant list, I started exercising that year to build up my muscle. I've been wearing oxygen most of my life. There was a big gap for 10 years where I wasn't, because I was able to go to school, junior high and high school and some college. I wore oxygen till birth up until I would say 15-1/2. Then between 16 and I think my mid 20s is where I had that gap where I got to go to school and enjoy life and have friends and mostly enjoy life without oxygen. Then between, I would say the middle of my mid 20s is when I got diagnosed, at the age of what, 26 years old, is when I got put back on oxygen in 2010. COVID has kept me away from all the germs and the COVID stuff up there. I've mostly been indoors. I haven't gone anywhere since March, beginning of this year. So, I've mostly been indoors, painting and drawing and keeping myself busy, and exercising three times a week. That really keeps me going. So, I've been really indoors, keeping myself away from everything that's going on. If I do go out, which is only doctors' appointments, I wear gloves, I sanitize, I wear a mask. So that's what I've been doing. Back in 2000, I believe '13, when UCSF suggested to go to pulmonary rehab, I went there in between I think 2013 and 2014. I met this lady. Her name is Kelly Frederick. She's a home personal trainer. We met that year where I went to pulmonary rehab for two years. So, when I landed in the hospital three times in one year in 2016, I called her because I felt like I needed to do more to get myself better for the transplant. So, I called Kelly in January of 2016. She was just giving me tips of exercising, things you can do at home, the sit to stand, the bands work. But I was like, "I need your help. Can you come over and just teach me a couple things?" So, we took it from there and she was coming to my house at least once a week for only one hour. So, move forward to four years later, she's still with me. But of course, with the COVID, I haven't really seen her since March, but she gives me tips on a daily routine: what to do, what exercises and what workouts. I work out three times a week. She's been my trainer for the last four years. As a PH person, my goal is to spread awareness. If you could just do one little physical thing every day, it means a lot. In the beginning, the first year I can barely do 30 seconds and I was sitting down within 10 minutes. Now, four years later, I'm doing planks and pushups and more than I can ever do. But with ones that can't do it, you can never say I can't. There's always a will. You can. You just got to put your mind to it, physically and mentally. My name is Jessica Romero 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 /episode/index/show/phawarepodcast/id/16549838

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