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

Amanda Howard phaware® interview 369 04/05/2022

Amanda Howard phaware® interview 369 Pulmonary hypertension caregiver, Amanda Howard, discusses her 17-year-old daughter Abby's lifelong battle with PH after being diagnosed in the PICU at 8 months old. Amanda Howard: My name's Amanda Howard. I'm connected to PH through my 17-year-old daughter, Abby. Abby was diagnosed with PH when she was eight months old. We thought she had outgrown it when she was almost six and then a routine echo on August 31, 2017 showed that it was back. That brought us back to being connected with PH again. I currently live in Perry, Iowa with my husband, Chris. We have two other children named Grace. Grace turns 14 on the 20th of this month. And then Blake is eight. When she was eight months old, we almost lost her, but it was found that she had PH in the ICU. We thought it went away because she was supposed to go to Texas to get a double heart and lung transplant shortly before she turned two. At the time, her cardiologist said, let's wait. So we waited. Fast forward to when she was almost six. In August of 2010, she had an echo and her cardiologist thought she had outgrown it. So she wasn't taking any more meds or anything. Unbeknownst to us, she had been having chest pain with exertion and stuff, but she didn't tell us because she thought it was just normal. Then in July of 2017, her cardiologist happened to call us in and said, "Hey, let's bring Abby in for a routine echo because we haven't seen her in a while.” That's when we found out it was back. It was hard. She actually ended up in the PICU before she was eight months old. I had been taking her to my primary doctor, her pediatrician at the time, saying something's wrong. She stopped eating. She stopped drinking. She would cry all the time and he would tell me, oh, I was just being a worrywart. Well, my mom is a nurse and I made the comment to my mom. I said, "Mom, something's still going on with Abby. I'm going to take her to my pediatrician and demand that he admit her." She made a comment to me. She's like, "Do you want me to go with you?", I'm like, "No, I got this." So I went to my pediatrician and I demanded he admit her and he said, "Let's do this. Let's have you take her home and push fluids and if she's still not eating or drinking by 5:00, I'll admit her to the local hospital." I said, "Okay." So 5:00 rolled around. I had been trying to get her to eat and drink all day. She still wouldn't eat or drink. So he admitted her. It was around 8:00 the next morning, I made the comment to the nurse. I said, "Hey, I'm going to go home and get some breakfast because I'm hungry," because I was a single mom at that time. I ran home to get breakfast because I only lived a couple miles from the house. As soon as I walked in the door, my phone started ringing and it was her pediatrician. I remember thinking, "Well, this is odd." So I answered the phone and he says, "Hey, Amanda, this is Dr. Jones. Can you sit down?" I'm like, "Well, why do you want me to sit down?" He's like, "I just need you to sit down." Abby's lungs were full of fluid. I remember him telling me that she's going to be going by ambulance up to Omaha Children's Hospital. I need you to get back here as soon as you can. I did. It's kind of a blur after that, but once we got to Omaha Children's Hospital, I remember the pulmonologist, which we grew to love, ordered a CAT scan of her lungs. She came back upstairs after that. At that time I didn't understand what retracting was. I remember making the comment to my mom. I said, "Mom, Abby's stomach's doing something weird." So my mom asked me to hand her Abby and I did. The next thing out of my mom's mouth is "Amanda, go get the nurse." When I got the nurse, the nurse came in, took one look at Abby and called for a rapid response team. Shortly after that is when we started the quest to pulmonary hypertension diagnosis and everything. I was going to college. I gave up college to stay home with her. I lived with my mom. Abby was on 24/7 oxygen at that time. She was on a feeding tube. She wasn't allowed to have anything by mouth. She was on an assortment of medications. It was closer to when I met my husband. He helped me with Abby. We moved in together. He helped me with Abby's oxygen, her feeding tubes, her medications. That went on for a few years, just routinely. Then in August of 2010, that's when the doctor told us she had outgrown her PH. We thought, great, this is good news. She didn't have to wear oxygen anymore. She wasn't on a feeding tube anymore. She was able to eat my mouth. She got the feeding tube removed. She was off all the medications. It was amazing. Looking back, I wish I would have done things differently. I wish we would have went by the echo. I wish we would've been like, "Hey, no, this is good news, but let's just do a heart catheterization to make sure," because Abby since sustained some lung damage as a result of it being untreated for those years, but hindsight's always 20/20. At the time we thought it was great news. She's doing pretty good right now. She doesn't have to wear oxygen anymore. She takes meds obviously. Her last echo looked good. They said she was still stable. She has a heart catheterization coming up. She has a CT scan coming up, but they said that's just preliminary or whatever to make sure she's okay, because she hasn't had those in a while. Overall, she's doing amazing now. She still gets short of breath, but she has a few limitations because of the lung damage she suffered. But compared to overall, she's doing good compared to what could be. Because of how bad her lungs look, her pulmonologist said she could be doing worse since she is, but she's adapted and she's found a way to work around it. I'll be honest. I trust him. They've been Abby's care team since she was little. We have a new pulmonologist that's been taking care of Abby and he's great. Her cardiologist has been in the picture since she was little, and they've been great. I was a little upset with them at first, because I felt like they dropped the ball, but I'm past that now. Now that I realize things can happen and people make mistakes, we have a great relationship. I trust them. Abby can ask them anything. I can ask them anything. They will go to bat for Abby. So it's a good relationship now. Abby's doing great. She's been through elementary. She's been through middle school and she is a junior in high school now. She's been dealing with this since she was little and she knows how to handle it. Her teachers have been great in helping her when she needs help. She knows to let them know if she's having problems. It gives me a lot of faith that I know she'll take care of herself when she is off on her own, because she is 17 now. She's going to be a senior next year. I know that she knows how to listen to her body and she knows when, to say "Hey, I need a break." It gives me faith and hope that I don't have to be there to make sure she's taking care of herself, because she knows how to take care of herself, herself. Abby's 17, and her team told us that she won't live past the age of six. So it's just amazing to see how far she's come and everything. If I had to offer any advice for any caregivers or parents out there, take one day at a time. Sometimes, maybe one step at a time. I know in the beginning, I was taking one breath at a time thinking, "I can do this. I can do this for my child". You can too. Don't be scared to ask for help if you need it. Don't be scared to go to a counselor. Don't be scared to take depression meds. They help. I'm glad I do it. It's made me a better mom because of it. Things will seem hard. They were hard for me in the beginning. I remember how hard it was sitting in that cold PICU room when Abby was eight months old and thinking, "This can't possibly be what is going to happen to my child." I remember sitting in the one room again after Abby was diagnosed again, thinking, "No, we cannot go down this journey again," but now that we've done it, there is hope. There are amazing support people out there. Amazing people that have been right where you're at. We are willing to help and lend a hand. Your medical team is there. Just reach out. I have found that talking to my counselor helps me give some of the stuff that I'm having a hard time with off my shoulders. I live in the present. Down the road, can something happen? Yeah. But right now I live in the present. Don't be scared to get a counselor. Don't be scared to take depression meds if you need them to help you function. There's nothing wrong with that. My name's Amanda Howard and I'm aware that my daughter, Abby, is 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/22635695

Episode 369 - Amanda Howard 04/04/2022

KC Yates - phaware® interview 368 03/29/2022

KC Yates - phaware® interview 368 Pulmonary hypertension patient, KC Yates, discusses battling COVID-19 twice during the pandemic and how she relies on her inner-strength to battle her rare disease. KC Yates: My name is KC Yates. I'm from Michigan. My PAH just came on randomly. I was pretty much having headaches, where you couldn't talk to me. You couldn't make noise around me or anything. Then I moved down to New Mexico, and on November 15th, 2018 is when I first got diagnosed. I went into the hospital, because I was having shortness of breath and the headache still, and couldn't barely walk around the house. So we went up to the house and they said I had idiopathic pulmonary arterial hypertension. The first few months were scary, because me and my husband at the time didn't know what was going on, what we were dealing with or anything. We had my mom come down to help us. When we took her back, I had my initial first appointment with my pulmonary hypertension doctor. He was like, “I want you to get on the subcutaneous pump. And I want you to have that safety net of the lung transplant evaluation.” So we went through all that, the nurse came out. I got on the pump. I was scared at first to get on it. We trialed an error with. I went up and did my double lung transplant evaluation. And they told me that my lungs were too healthy to even consider getting the lung transplant. So that was relieving. I kept on asking my doctor if I could get off the pump, because it hurt so bad. I just wanted to be free again. I felt like I was tied down to something. I couldn't take showers by myself. I couldn't walk by myself. I couldn't do most things that I'm doing right now by myself with that pump on. A year later, I got off the pump. I went on the oral meds and titrated up to the same dose that I was on with the subcutaneous pump. Made myself sick a couple times to get to that right dose. Then a year after that, I got a divorce. Now I'm on Opsumit and Sildenafil. My heart is back to normal. I am doing super good. I'm considering going back to work. I'm happy where my health is, but they said that I still need the two meds to cope with everything. Then I developed some other medical issues on top of that. So just managing them all together is frustrating right now, but I'm getting there. When I moved back up to Michigan, I got a new doctor and they did the right heart catheterization. They did all my testing over again. They were like, your numbers are awesome. We want to see if you can stop all meds. If not, we'll put you back on two meds that we think are going to help you. So I stopped all meds. I did another right heart catheterization, and my numbers jumped up a little bit. So they put me back on the Opsumit and the Sildenafil and I'm only on those two right now for my heart. When COVID actually started becoming a thing and we went into the lockdown, I was not doing anything different. I was wearing my mask in the stores. Sometimes I wouldn't wear my mask because it was hard for me to breathe in them. I had to get a special cloth mask that I could actually breathe in. Around Thanksgiving time, I got COVID and I was down for two weeks. All my health problems jumped up to more intensified. I couldn't eat anything. I couldn't smell nothing. I couldn't taste nothing. Thanksgiving dinner didn't taste good at all. I got over that. Then my grandpa got it. So I got it again, unfortunately. But this time it was less of a sore throat and a headache. So I'm glad that I did get my vaccine. I'm not boosted yet. I don't know if I want to get boosted, but they told me that if I didn't get my vaccines when I did and got COVID, it would've been more worse with my PAH. So the first reaction, it was weird because my hip was numb. So I went up to the hospital for my hip being numb, I'm like, my hip's numb, and I have a headache and my chest hurts. They sent me home because nothing was broken in my hip. Then the next day I'm like, okay, more intensified symptoms. I told one of my best friends who works up at the hospital and she gave me a COVID test. Within an hour she's like, yep, you got COVID. She's like push Pedialyte. Push any meds that you can take, like cold meds. So I pushed a bunch of cold meds. I pushed a bunch of water. The only thing that I was concerned about is not eating and not sleeping. I couldn't even keep the water down. I couldn't keep nothing down. So it was scary to not be eating. I lost about 70 pounds from not eating. They were worried about me losing pounds, so I went to my GI doctor and she put me on some anti-throw up meds, which I'm still on right now. Then everything went back to normal. I have celiac vomiting syndrome. So certain foods I can't eat like pizza, tomatoes. Anything acid I can't eat because then I'll just throw it up. But it wasn't a big deal. Not seeing anybody was big. Not being able to do nothing was my biggest concern, because I was bored. The not sleeping. But other than that, it was okay. I am considering going back to work. With all that, I moved back to home. I got a divorce. I'm living with my parents, because I can't afford nothing by myself. I went back to school for my medical assistant degree. I just finished with that. Now I am waiting to hear back from two jobs that I've only applied for. If I get it, I get it, great. Then I'll go through the hoops of the social security to go back on work and all those major questions. Then, if I don't, then I'm going to focus on my associates degree. I start that for nine months, and then see where I go. I'm not pushing hard to get a job, but if I get it, I get it. I have to try at least something. If I don't try, I will never know. I would say, be patient with this disease. Try new things, because not everything's going to work out for you like it did with me. Everybody's different in their own aspect. Trial and error with your doctors, see if there's any changes that you guys can make. Some doctors are willing to help you get off the meds, and some doctors are just like, no, you can't ever get off these meds. Just try. If you don't try, you will never know. From where I was, not being able to take a shower by myself or walk around the house, to where I am, where I can walk around my house, I can take a shower. I can do anything I want right now. It's a blessing. I just went with the flow. And I knew my body was going to do whatever it wanted to do. I knew that if it was meant to be for me, it was meant to be. If I was meant to be on that pump for my whole life, it was meant to be. But I am glad that I'm not on that pump anymore. It makes you stronger. I've realized going through all these health issues and seeing where I was before, I'm the strongest I will ever be. I encourage everybody to take that strength and put it to their health, because the stronger you are, the more stuff that you can take on. I took on more health problems and it doesn't phase me, I just take it like a champ right now. There are Facebook groups that you guys can get on. I've been helping about five people so far with their pumps and with their meds and just pretty much helping them navigate what I've gone through and telling them my tricks and my tips. What you should not do or do, if this is right or that's right. So everybody's different. You've just got to navigate yourself, for yourself. My name is KC Yates, and I know 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/22583237

Episode 368 - KC Yates 03/28/2022

Dawn Meador - phaware® interview 367 03/15/2022

Dawn Meador - phaware® interview 367 Long-term pulmonary hypertension patient, Dawn Meador, discusses navigating her PH during a pandemic as well as why she and her husband chose to adopt after her PH diagnosis. Dawn Meador: Hi, I'm Dawn Meador. I am from Springfield, Tennessee. I was diagnosed with PH in March of 1998, so I've been living with PH for 24 years. I had a nine month old daughter at the time, and I had noticed I'd been having a lot of trouble breathing. I went to Walmart one day with just me and her. She was so small, she was still in the pumpkin seat. So I carried the pumpkin seat into Walmart and I got into the store and thought, "Boy, I can't breathe. I'm really out of shape." Carrying the pumpkin seat, she doesn't weigh that much. I got the seat set in the buggy, walked back to the dog food aisle. My heart was just racing, beating hard enough that you could kind of see my shirt moving and I could not catch my breath. I knew something was wrong. The next day, she had a baby well check at our family physician and I questioned him about it at the time. When I asked him about it, he looked me over and did some tests and immediately sent me to a heart specialist. From that heart specialist, they did an echo and several other tests and discovered that it was PH. I had never heard of it before and I was basically told, "Hey, you're one in a million." So, this wasn't really the lottery I wanted to win, but okay, it's the cards I was dealt. So what do we got to do to deal with it? Because at the time, I had a five year old son and a nine month old daughter and I thought, with everything that they were all telling me about it. Then they were giving me the statistics of ... back then it was, life expectancy now that you've been diagnosed is only like three to five years. I'm like, "No, God's going to let me live to see my kids grow up." I was on oral meds for the first 14 years and then all of a sudden they just stopped working. So they put me on the subcutaneous pump with Remodulin®. I was on it for about a year. And then they tried me on the pill form of the Remodulin®. After about eight or nine months, they put me back on the pump. So I've been on the pump for almost eight years now. I thought, "Well, I've made it 14 years. Is this the point that everything starts going south?" The pump is inconvenient, it's painful. But my feelings on it is, it's doable and it keeps me around just a little bit longer. So I'll do what I have to do to be able to spend time with my family. They did a lung biopsy to see if they could pinpoint what caused my illness and determined it to be idiopathic. So there was no known cause, it wasn't hereditary. It wasn't caused by the pregnancy or anything like that. It was just a fluke, that one in a million. For a lot of years, I kind of dealt with it on my own. And then I finally got into a support group on Facebook and got in touch with other PH patients. At that time I thought, "I'm the only one dealing with this. Nobody truly understands what I'm going through." Then I started talking to other people that do have it and realize there are more of us out there. There's not a lot of us, but there are more of us out there. We kind of got to lean on each other because we all know what the other one's going through and nobody else really does truly understand. When we moved to Virginia, I got in with my new doctor there. And they talked me into going to one to the support group meetings. The very first meeting I went to, it was meant for me to go that meeting. There was a young girl that was 19 years old that had just been diagnosed like a couple of weeks before I got there. She was totally devastated. The whole meeting, she just was in tears the whole entire meeting. Something told me, "Go up and talk to her after the meeting was over with." So after the meeting was over, I went and talked to her and her mom, and we have stayed extremely close ever since. That's kind of what has kept me going is if I can give somebody else support and let them know, "Hey, yes, it is scary when you get this news that, this is what you've got. But if you go online and start reading everything that the web MD says, it terrifies you even more." I'm usually telling everybody, "Don't go read the website. Talk to somebody else that has it because they can give you better information than what you're going to get off those website. When you read the websites, it's going to scare you to death." If I have the opportunity to give somebody a little bit of, "Hey, it's going to be okay, you're going to make it through this. It's going to be right." Then that's what I'm going to do. At the beginning, it probably brought us closer together. After a few months, the newness of me being sick wore off and it actually kind of ... we had problems to begin with, but it kind of ended the relationship. We ended up divorced. About a year later, I met somebody else. He swore that he could handle it. We got married, nine years later after realizing that he really didn't support me whatsoever, along with other issues, we got divorced. I had kind of given up on anybody else and decided it was just going to be me and my kids and that's all that this was going to be, because nobody else could handle dealing with me being sick. Then I met my husband, which was a total fluke because at the time, I had swore I was never getting married again, I was never going to date again. I wanted to do with it, I was over it. I needed to take care of my health and my kids and that's all I cared about. A friend of mine talked me into just meeting him. On the very first date I told him, I said, "I have two kids. I'm in the middle of my second divorce, because I have a health condition that is called pulmonary hypertension. It's a lung disease and it will one day either require a double lung transplant or take my life. One of the two. If you can't handle this, then we're just going to call this friends and we'll go on with our dinner." His next response was, "Does the illness define who you are as a person?" I was like, "Well, no." He said, "Then why should I have a problem with it?" He said, "You can't help that you develop to this illness, so why should it cause me to not want to be around you or with you because of it?" We had been together ever since, because he was kind of the blessing that I needed at the time. We have a child together, she is actually adopted. Everybody always asks me, "If you've had PH for 24 years, how do you have a 10 year old?" She is adopted. We actually finally told her last year that she was adopted and she's completely fine with it. It was ordeal It terrified me, had me stressed out, because I had two kids, he didn't have any kids and he wanted kids. He wanted to be able to raise one, as well. My two, by the time we met, my oldest was 17 and my youngest was 13. They were kind of pretty much grown. And we got blessed with a brand new baby. We were there, watched her come out and everything at the delivery and she was handed straight to us. She's been ours ever since and been ours from the get go. It's not always easy, especially with him being in the Navy, because there's a lot of times that he's not here and he's not here for several months. You just kind of have to deal with it. It's hard because there are days that you think, "Okay, I can't do this. I'm sick and I feel like somebody's just ran over me with a truck." But then you stand up, you smile and you say, "I got this." You suck it up and you go on and you deal with what you got to deal with. None of us asked for this disease, we just developed it. The only thing you can do is you can sit and wallow and self-pity over it. Or you can live life to the fullest and do what you can while you can. At the beginning of COVID when they first started coming out, talking about it and how everybody was getting sick. For like the first three months, my husband was bound and determined, I wasn't leaving the house, because he didn't want me catching anything. Because with PH you are immunocompromised. For a PH patient that already has issues with their lungs to develop something such as COVID, you're pretty much in the hospital and more than likely on a vent because your lungs already compromised and that's the first thing an illness with your body is going to attack, is your lungs. Because it's the weakest point. For the first three months, he pretty much was, "Okay, make me a list. I'm going to the grocery store. I'm going to Walmart. I'm going to go do this." Everybody got to laughing at me because I would tell them, "Oh, I got to go for a ride, I'm starting to know what it's like to be a dog." Everybody was like, "What do you mean?" I said, "Well, I'm at home all the time. Just to get in the car and go for a car ride. I just wanted to just sit there in the car and go for a ride, because it got me out of the house." For the first few months I was a little stir crazy. Then finally I got him to realize, this isn't going to go away anytime soon and I can't stay confined in a home until all this is over with, because it's going to drive me insane. So I put on my masks and I was very cautious. If we went anywhere, it was in and out pretty quick. It's not quite as bad as that now. I'm cautious, but I'm not crazy. I don't go overboard like some people do. Which everybody has their own way of dealing with it. It's not something that's ever going to actually go away. It's here. But we have to learn to live life and be cautious, but don't be stupid. But enjoy life, too. If we sit still and wait for it to go away, you're never going to enjoy life for who knows how many more years. Who knows how many more years until it actually goes away. My advice is just have the faith and stay strong. Know that whatever comes your way, how you decide to face it is going to depend on how the results come out. But for me, I'm a full believer that there is a higher power that's in control of everything. My faith is truly the only thing that has kept me going through all of this, between the illness, COVID, anything that's ever come my way. Living with PH is not the easiest thing in the world. You have so many things thrown at you between the medical issues and the medical bills, that's a whole another story in its own. Then the prescription costs, that's another whole other story. It's a struggle and it's very stressful. For a PH patient, the stress is the worst thing they can deal with, because the stress just makes the PH that much worse. I have always just put my trust in faith in Him and just told myself, "It's going to work out, no matter what it's going to work out. It's going to be okay." That's how I deal with all of it. I'm Dawn Meador and I'm aware that I'm rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials /episode/index/show/phawarepodcast/id/22434587

Episode 367 - Dawn Meador 03/14/2022

Mary Hale - phaware® interview 366 03/08/2022

Mary Hale - phaware® interview 366 Pulmonary hypertension patient, Mary Hale discusses the power of positivity in life post PAH diagnosis. Mary Hale: My name is Mary Hale, and I'm from Ohio. I was diagnosed in February, 2010. I had open heart surgery in January of 2010. So once I went through all the testing, then I had a diagnosis. I had PAH. I had a pain in my side, and thought pulled a muscle at work, and I was just babying it, and I finally went to the doctor. They did all this stuff. It didn't look good. I went for a heart catheterization, and they kept me, and I had open heart surgery January 25th, Monday morning, 2010. I remember it plain as day. They couldn't believe I was still alive. I was dumbfounded, because I had no clue what it was. They didn't even know what it was. The lung specialist that was brought in during the surgery, explained to me that he thought I had PAH. I'm like, "What is that?" So he said to me, "It affects the right side of your heart. It's the pulmonary artery. It affects your breathing." So he sent me to a specialist at OSU. I did all those tests, and that's when I was diagnosed. I was in grad school when it all happened. I had the open heart [surgery.] and everything. I had dreams and stuff. I don't know how many times I could say this, it overwhelmed me. I cried a lot. What is a chronic illness? Why did I have this? How did I get this? There's no answer. No one knows how they get it, unless it’s hereditary, which is idiopathic pulmonary arterial hypertension. So from that day on, when I left the hospital, up until now, I'm on oxygen. I'm on constant oxygen. I did work up until 2013. I did finish grad school. But after that, it was just hard, because when you wear oxygen, they see a red flag. It was very devastating. I cried a lot, and I prayed a lot. I spoke to mentors that I had. I was just confused, like, "Why do I have this? How in the world do I have this? Out of four siblings, I'm the middle one, how did I get it?" It's a roller coaster. Everything changes. Year after year new medicine, new doctors. It's an adventure to be honest, because you just don't know. For 17 years I taught pre-school, and 10 of those years I worked with a program called Head Start, and it's working with inner city children and parents. My degree is in non-profit. I wanted to work with the inner city families. I had a heightened desire for that. Because I grew up in the projects until I was in middle school. So having a heart for that, I wanted to go to school so I could have the background and then go back to it and say, "I want to do this." That was my desire. I didn't get a chance to do it. One thing happens for a reason. One door closes… you know how they say that. But right now I do volunteer on Tuesdays, which I haven't because of COVID, with the inner city school program in Springfield. So that's exciting to me, and I can't wait to get back to it. In 2013, my heart couldn't handle all the work I was doing at work. I was tired a lot more. My pressures were just too high. So I did go on disability, so I'm still on that. From then on, I was just a homemaker, worked with kids at church, volunteered a lot, and plus, with the PH Group out of Dayton, Ohio, I'm one of the leaders, so I do that. Then up until 2018, it was the week of Thanksgiving, it was that Wednesday, November 21st, I'd been really tired, and was going to bed early, and my husband was like, "Boy, you must not feel good if you missed NCIS." So that Wednesday, I was cleaning my Hickman, which is, if you're on IV Remodulin®, like I am, you have the Hickman [port] coming out of your chest. You have to clean it every seven day or when it gets wet. So I was cleaning that, and I end up putting clear regular tape on my line, instead of the tape they provide - the good tape. For some reason, I thought, "Well, I can't get it off, let me use these little bit of scissors." When I pulled the tape, I cut my Hickman, which about freaked me out. I called my husband at work, and I said, "You've got to get home. I cut my Hickman, I've got to get to UC." So next thing I know, he wakes me up. I'm laying on the floor. I passed out and didn't know it. So he drove like a bat out of hell, down 75 going to UC. I remember getting there, and I remember he had to have two guys helped me out, because I was dead weight because of my leg. When I passed out, I landed on my left side of my hip, which grew into a big hematoma. I couldn't walk it out, laying as long as I did, all the nerves crushed. I remember being in the ED, emergency department at UC, and I remember they put a Remodulin® IV in me, because I cut my Hickman. I remember talking to a nurse, and next thing I know, I woke up December 3rd. They called my husband that night, November 21st, and asked if they had permission to put me on life support, which was the ECMO. Come to find out, I had MRSA, that developed into pneumonia, and I was really bad off. Thanksgiving Day, that evening, they told my husband, "You might want to make funeral arrangements, because we don't think she's going to make it through the night." Lord behold, I did, and I'm here. It was just craziness, in my mind, it was craziness, because you remember going there, and then you wake up with tubes, and you're like, "What is going on?" It was just a sense of, "Wow, what did I just go through?" Because when you're on the ECMO, you're either getting a lung transplant, heart transplant, or you're just buying time, and I was buying time, but I survived it. What happened was, I stayed in ICU, cardiac ICU, for almost, oh gosh, two weeks, because I had to get to rehab, because I had to learn to walk again. Because there for a while they were afraid that I wouldn't be able to talk, or know to do anything, because I was so down for almost 10, 11 days. So they didn't know if my cognitive [functions], if I could speak, or anything, would come back. It is life-changing. I hear people, "Wow, you've only got five years to live." No, you don't. Don't read that. The medicines have changed Everything has changed. I know somebody who's going on 24 years that is living with PAH. Look at it as, "I have today." You've just got to look at one day at a time, and live life. You've got to live life, you cannot give up on life. And people that are wearing oxygen, I hear so much, "Well, I don't go anywhere, because I wear oxygen." Oh honey, hush. I have tanks. I go out to lunch with my girlfriends, my husband and I go to movies, I go shopping. You've still got to live, you've still got to live. You cannot let this win over you, you've got to be stronger in your thoughts. You've got to be stronger in how you want to proceed with your life. Even though you have this, you still are a warrior. You've got to fight. Sometimes I'll be like, "This is not where I thought I'd be. I didn't think I'd be at the spot I am." But here I am, and I can still walk. I still drive a car. I can still clean my house. I could still do things. I don't need help. I can still do it. Now, my left leg, it works, but I have like a little limp, because it's still numb on some parts. The nerves didn't come back, so it's not full strength like my right one. But I still walk, I still do things. I still live life. I still will sometimes catch myself asking the question why, why do I go through with this? When in reality, why not me? Maybe I'm being used for something, to share with others that you can do this. Your attitude depends on how you deal with PAH. I have a positive attitude, I mean, I'm not happy every day. I have down days, absolutely, but I can't let this overtake me. I've still got a life to live. I still have family, nieces and nephews, and great nieces and nephews, that come over, and we just... I still have a life. My two dogs, and my husband, I still have a life. I don't take nothing for granted anymore. I mean, you just don't. The only thing I can say is, people with PAH, yes, it's overwhelming. Yes, it's a chronic illness. Yes, in our lifetime, we will probably not see a cure. With that in mind, we've still got to get up each day, and live our life to the fullest. When you open your eyes, you have another day, be thankful. You've got to have a positive attitude, and that's what everyone asks me. My friends, they ask me, "How do you get through this?" I say, "Well, grace of God, a prayer, but attitude, you've got to have a positive attitude to get through anything." For something like this, this ordeal, it's all attitude. You've just got to be positive. It could be so easy to be a humbug, like an Eeyore or something. Be a Tigger. Be ready to bounce. Be ready to just have fun, and live life. My name is Mary Hale, 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/22358603

Episode 366 - Mary Hale 03/07/2022

Tarya Morel - phaware® interview 365 03/01/2022

Tarya Morel - phaware® interview 365 Canadian pulmonary hypertension patient, Tarya Morel discusses parenting in times of a pandemic. Tarya Morel: My name's Tarya. I live in Vancouver, B.C., in Canada, and I'm a mom. I have two kids, one by birth and one by foster. I was diagnosed with pulmonary hypertension shortly after the birth of my son. I've heard from so many other moms out there. That was their journey, too. I know that for women who are diagnosed with pulmonary hypertension, if we do have kids, because I know we're told often that we probably shouldn't once we have our diagnosis, but if we come to that diagnosis after having had kids, a lot of women that I've heard from have talked about having sort of subclinical symptoms before and then that getting just kicked into high gear through pregnancy, because pregnancy is no joke. It does all kinds of stuff to your body that really sort of brings those other things to light. So, I'm one of those women. When I first got diagnosed, I had this infant, I had this little baby who needed me. It was a wild experience, because you have all these ideas about what parenthood is going to look like, and then it all just gets completely flipped upside down. Suddenly, they're talking about putting me on a pump and IV meds with this attachment, and I'm picturing this little baby who grabs everything and puts everything in their mouth. I'm thinking like, "Oh my God, he's going to reprogram this thing and I'm going to be trying to decode it like an episode of 24 before the meds stop running." It was like, it was a lot to integrate in a really short period of time. At the same time, I would say at the beginning, he was my salvation. I get this life threatening diagnosis. I was severe when I first got diagnosed. I really thought that I only had like maybe a year or two left based on the statistics that I'd read online and where I was already at. I thought, "Okay, well, I get a couple good years with this baby." It really forced me to be super present with him because a baby doesn't know. They don't know that there's really heavy, serious stuff going on. I had just lost my dad the week before I got my diagnosis. I think he saved me from wanting to just go into bed and pull the covers over my head and cry all day. I got very lucky in that I responded super well to the meds and I did really well for a number of years. I've had my ups and downs. I've had to change meds a few times and had more hospital stays than I would like. I also managed to do a lot of things that I never thought were going to be possible. I went back to work part-time at first, found an amazing job, and now I work full-time from home. Well, from home for the last two years during this pandemic. That's a whole other thing. Parenting through a pandemic, I think all of us who have done it, navigating the challenges of, "Do I put my kids in school? Do I not put my kids in school? How do we navigate social interactions?", trying to keep them entertained and educated, while also trying to maintain my full-time employment. I think it's been hard for parents across the board, but then you throw a chronic illness into the mix, and it's absurd. These last couple years have been so crazy and my kids have been my salvation through it. We've had a lot of fun together, like a lot of experiences that we probably wouldn't have had because they would've been out of the house doing their own thing, and we really were forced to just be each other's best friends. You want to shield your kids and you want to protect them from the big, bad scaries of life. Sometimes when you have a disease like pulmonary hypertension, you can't do that. They just become a part of what's happening because they're here and they see it. My kids have visited me in the hospital more times than I would like, that's more normal for them than I would enjoy. I was on IV meds for a number of years. In the last year of it, I had lots and lots of problems. Lots of infections, lots of clotting, lots of issues. I remember one time, I had an issue where I had a line that was just getting constantly clotted. I don't know if you know much about how the line issues work, you probably do, but you need that medication to be running. It's very much an emergency when that stops, so you need it to happen quickly. What was happening is I couldn't get the cap off of one of the things. My niece was home and I had to ask her, "Can you go next door and get a pair of pliers from the neighbor immediately?" She saw the panic in my eyes and she went next door and she got it settled. She knew, because she'd sort of seen this kind of thing play out before, that she had sort of 911 on the standby and she was ready to go. She was, I think, 15 or 16 years old at the time. That's a lot more vigilant than a teenager I think should have to be. At the same time, I think my kids are going to grow up with a lot of empathy, because of what they've seen this disease do and what they've seen us have to deal with as a family unit. There's a good side to it. I would still not choose it given the option, but they're building some character. I'd happily go the rest of my life and never hear the term unprecedented times, but that's where we've been for the last couple of years. For the kids, they're right in that middle of that social development that is so important for them. I think at the beginning, it was actually easier, when everyone was on the same page. There was very much this social understanding of stay home, let's all do what we can to keep each other safe. My kids made a lot of use of online interactions in a really good way, I think. My youngest plays online games and manages to connect with his friend in Norway all the time, which is really kind of cool. It wasn't something that we saw a lot of happen and managed to stay connected with his school friends a lot through that, which was really nice to see. I think it was hardest for the teenager, for my older one, because she was, I guess, 15 when this started. She's 17 now. She's in grade 12. She missed her whole grade 11 year. Did schooling from home. Did really well academically, but I think it was really difficult for her socially. Then things started to get better, and then she got a job, and now she's working in retail. We've sent her back to school, because it's grade 12, and I couldn't take that from her. But I would say that right now is actually the hardest time, I think, because we're in this place now where despite the fact that, epidemiologically, this is the worst wave. We have more people in hospital than we've ever seen before. Way more cases than we ever anticipated were possible. At least here in B.C. Everybody's kind of given up. We have very little in the way of public health restrictions. There are no online options for kids with medical vulnerabilities. Even my younger one, who I would prefer to have home right now, at least until he can get his second dose of vaccine, because we don't even have access to that yet here in B.C., because it's not just me. Yes, I am worried about getting COVID from the kids. A hundred percent. I'm as vaccinated as I can be, so I'm hoping that will keep me safe. But I would say I'm equally worried for them, because even though I know the risk is low for them to have long-term impacts, I live with breathlessness every day and I don't want that for them. I know that there's some proportion of people that have long-term symptoms of COVID. Just as a parent, I want to protect them from that. It's preventable. Sending them back into just buildings full of people where there are just so many sick kids walking around, wearing masks poorly, coughing on each other, it's terrifying to me, but that's where we're at in B.C. I find it so difficult to reconcile, because this wave is global. We've got everybody going through it at the same time. It is spreading like absolute wildfire. It's also the moment when we seem to have the least governmental and social willingness to act. I get pandemic fatigue. I get it. People are tired of it. There's a lot of protestors. It's become very political. But for people like me, that's, I think, extra scary because even with my boosters, I worry about what this will do to me and my disease progression. My health hasn't been super stable lately, and so I worry about this accelerating my need for a transplant or at the very least lining me up in the hospital. Like I said, I'm just so worried about the kids contracting this and having long-term health issues. It's just not what I want for them. The other wrinkle is that I also became single right before the pandemic. Moving into single parenthood or co-parenting, which co-parenting through a pandemic, wow is that super fun, and not necessarily always agreeing on restrictions, and travel, and school, and all the rest of that, that's a whole other piece to navigate. Again, I have so much empathy for everybody navigating this pandemic right now, because we've all got our own challenges through it and it's hard. But I think I learned through that, and through the pandemic, and figuring out my own self, is that I am so much stronger than I thought that I was. I think one of the reasons that I stayed in the relationship I was in so long was that I was really afraid, as a person with a chronic illness, that I couldn't do things for myself. All of that housework, figuring out meals and the dishes, and trying to work full-time, and get the kids to school, and all that, it all felt so overwhelming. With the help of good friends and being able to outsource some of those chores, I have a house cleaner who comes in once a month now, and I will never go back. It's amazing. I really figured out that my kids were also a lot stronger than I thought that they were than I ever expected them to be in how they helped me out, and how they take care of themselves, and how much empathy that they have in taking care of the people around them. I'm so grateful for that. For the amount of time we've gotten to spend together. Especially with a teenager, you expect that they will be out and about. When she was younger, she was. She was super independent. She knows how to get around the city on the bus, but now we're having to give her rides all the time, and we get to chat in the car. It's just an experience that I wouldn't have had otherwise. There's upsides, too. I think if I was to give advice to other parents with PH, because I know it's really challenging and it's really intimidating and it's scary, I think there's a lot of social pressure on women in general to be and do all of the things. To be the mom who does all of the birthday planning, all of the Halloween costume making, all of the homework help, all of the meal planning, all of the Christmas shopping. There are so many roles that moms fill, and on top of their work, if they work, and on top of all the family obligations, and all the rest of it. Be gentle with yourself. I would say for anyone, especially through this pandemic, trying to be that mom is impossible. Then when you throw a chronic illness on top of it, you can send yourself into an illness episode just trying to get all the things done. I would encourage everyone to ask for help a lot. Often, the people around you want to help. They just don't know how. One of the best pieces of advice I ever got was from my midwife a long time ago, who told me to keep a list of actionable tasks on a whiteboard or something on my fridge, so that when people asked if there was anything they could do, you have a concrete thing to give them to do, because people want to help. They just don't know how. If you need dog food picked up and you're talking to, a friend, "Is there anything I can get for you?" "Yeah. You know what? Can you get me some dog food?" They'll do that, and then they'll feel like they've contributed to your life, too. Right? Everybody wins in that situation. Ask for lots of help. Be gentle with yourself. This is hard and you're probably doing a lot better than you think you are. I'm Tarya Morel, 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 @TaryaMorel @phacanada #covid19 /episode/index/show/phawarepodcast/id/22256933

Episode 365 - Tarya Morel 02/28/2022

Sandeep Sahay, MD and Jordin Rice, RN - phaware® interview 364 11/30/2021

Sandeep Sahay, MD and Jordin Rice, RN - phaware® interview 364 Pulmonologist Sandeep Sahay, MD and Jordin Rice, RN from Houston Methodist Lung Center discuss a nurse's role in informing and educating pulmonary arterial hypertension patients about clinical trials. Sandeep Sahay, MD: I am Sandeep Sahay and I'm a pulmonologist at Houston Methodist Lung Center, Houston Methodist Hospital, Houston, Texas, and I specialize in pulmonary hypertension and I'm actively involved with clinical care of pulmonary hypertension patients and also in clinical research. Jordin Rice, RN: I am Jordin Rice. I am a research nurse at Houston Methodist. I work directly with Dr. Sahay. We work with all types of PH patients near and far, and I have had the pleasure of becoming a research nurse after caring for them at the bedside. It's just been a wonderful experience. They're some of my favorite patients I've ever met. And I really look forward to talking about clinical trials and how we take care of them and manage their care. Sandeep Sahay, MD: Let me start with this. What is a nurse's role in informing or educating PH patients about the clinical trials? Jordin Rice, RN: I think first and foremost, we approach everything as a team. You and I work really closely together, looking at patients, figuring out if people qualify. You obviously know things that I don't. I might think of things about patients that you don't, like their commute, how much time it's going to take away from their normal job to participate in a trial. So nurses I feel like are really instrumental in that process in making this a realistic thing, to be enrolled in a trial. We help logistically get patients here and we talk about kind of the ins and outs of trials. Not all patients are going to qualify for something. It's up to us to really dive into a patient's medical records and talk to them directly about what it means to actually be in a trial. You have to come into clinic a lot. You have to talk to us a lot and get really close to us. So it's important that we establish a good relationship with patients, right from the beginning, to avoid any hiccups and make sure they get the best possible experience out of participating in a trial. Sandeep Sahay, MD: From the nurse's perspective or especially the research nurse perspective, because in the PH disease state, research plays a very important role and it's important to stay relevant to the newer therapies and also participate in the newer trials so that we can have more treatment options for our patients. So how do the research nurses think about the clinical trials about why it is so important for the patients to enroll? Especially if you are talking to a patient, let's say you are investigator or the PI or the physician asked you, "Hey, contact this patient, maybe for this particular trial." And now you are speaking to the patient and you feel like the patient is probably not ready. So how do you approach those kind of things in your mind? How do you think how important these trials are? Jordin Rice, RN: I think you first have to understand a patient's knowledge about clinical trials. Some people, they might work with companies in industry. They might have a family member that has participated before, or they might not know anything. They don't even know what placebo controlled means. So getting to know where a patient's starting point is, is typically how I start approaching patients. We recruit tons of different ones. It depends on what type of PH you have. We have to talk to patients why this would even be beneficial to them. A lot of patients think they might be a guinea pig and this doesn't benefit them at all. It just benefits us because we get to learn from them. But that's really not the case. Clinical trials makes medicine more accessible for patients. You can get routine testing, you can get an echocardiogram, a right heart catheterization. You can get doctors' visits, all paid for by the study. Some of our patients are uninsured, undocumented. Some of them are very wealthy. So we see a good variety of people that come in needing the same thing. Whether it's just medical care that can benefit patients, but the substances that we're actually treating them with and testing, they can be really exciting and show the first glimmer of hope of this disease actually being reversible or curable, which is exactly why I do this job. I don't have any interest in keeping up with the status quo. I want to keep advancing science. And I know, Dr. Sahay, you're the exact same way. You're an absolute pusher. So when we see a patient that is potentially eligible, meeting them where they are, making them realize that this disease is chronic, they're going to be dealing with it for their whole lives. Hopefully not. Hopefully, we find some kind of cure or treatment that's going to make it easier, but seeing what their quality of life is and what they want that to look like a few years down the road. We collaborate a lot together on talking to patients and making sure they know what they're signing up for and making sure they know they're not just an experiment. They're a person. These drugs that we're testing are exciting to us, but I hope they're equally as exciting to the patients we talk to. Whether they have the time to participate or not, I just want to open people's minds about research when I have those conversations. Sandeep Sahay, MD: In your experience, when you talk to these patients, especially about the clinical trial enrollment, have you encountered that there are some obvious barriers? In your experience, what did you find that is the most common cause for them to decline? Or if you have something like that in your mind, then how do you think we could do better in that to encourage them to participate in the trials. Jordin Rice, RN: Every single one of our patient is human. They typically have a job and some of them have kids. Some of them are living far away. So we have to be realistic in how a trial can fit into their life. Sometimes trials can be every few weeks, sometimes it's once a month. It really just depends on what trial we're talking to them about out. We're also strategic in the trials that we offer to them. I'm not going to offer a trial that you have to be here every single week for somebody that has four kids and a full-time job and is a single mom. That's not going to work for them. However, there are exceptions that we can make and work arounds we can do in order to make these trials work for patients, as well. So if a patient, they live five hours away, we can put them in a hotel room the night before or we can do an early visit, we can do a late visit. We stay well after the 9:00 to 5:00 or well before the 9:00 to 5:00. Being able to tackle those barriers with patients is instrumentally important. We can't just tell patients Monday at 8:00 AM, you have to be here and no exceptions. We really have to work with them. Also our patients sometimes are not ready for trials. I don't want to say that's a huge barrier, but if they're still getting used to being diagnosed with this disease, if they're freshly diagnosed, getting into an experimental trial might not necessarily be the first option for them. We need to get them on stable therapy. We need to get them comfortable living with their disease before we throw them into something that's unfamiliar, mostly because we have to get patients to manage their own medications and be compliant. That can be easier said than done. We have medications that patients have to take three times a day and be consistent with their diuretics and be consistent with so many things. That's a lot harder when you're living a real life and are discharged from a hospital. So there are several barriers. And sometimes as a nurse, I feel like I can see those or patients are more forthcoming in talking about those to me. Not that they would never talk about it to you, but if you talk about a trial to them, I've seen them say, "Yeah, yeah, sure. I'll participate." Then you leave and close the door. And they said, "What did he say? I didn't really understand that. Can you explain that more?" I feel like I can benefit patients in that way. I just have a different perspective and you are rushing around a lot and you don't have a ton of time. So I feel like I can fill those gaps a lot. And it makes us a really great team. Sandeep Sahay, MD: How do you think that nurses view enrollment in clinical trials differently than physicians? Jordin Rice, RN: That is a tough question, and it definitely doesn't have a right answer. It's going to be different for every coordinator, but what really stands out to me with trials, if I'm talking to a patient, is somebody that's just as excited as I am and just as willing as I am, because I'm willing to jump over barriers and go through hoops to get somebody on the schedule and get things that work for them. I want a patient to do that same thing for me, because we're a team once they enter a trial. Sometimes when patients are scared or they're really hesitant to enter, I find it harder to enroll them and get them excited and get myself excited for it, simply because I just want to make sure it's the right step in their care and treatment. If they're hesitant, it honestly makes me doubt that sometimes. There are definitely more issues that come up and more things that we look for in patients, but really that excitement and wanting to participate in research is something that I think is so important. I would love for my patients to think that same exact thing. Sandeep Sahay, MD: What do you enjoy most about working with or treating PAH patients? Jordin Rice, RN: PAH patients are really special, and if you work with them or if you are one, you probably know the resiliency that you have to have to one, be diagnosed with a chronic progressive disease and two, to live with it. Whether you're on oral therapies or you're doing something like subcutaneous Remodulin, you have to just be very aware of your care and very in tune with how you feel and be an advocate for yourself. Sometimes these patients are just dealt the worst cards. We've had several patients that tested positive for COVID at the beginning of the pandemic and it worsened their disease severely. It's tough to see these things happen, but sometimes they are unavoidable, but seeing patients want to get better and fight every single day to do that is really exciting. I worked with these patients when they got admitted to the hospital. They were getting heart catheterizations. Some of them were decompensating and needing treatment in ICU. I've never seen so much fight in a group of people. Especially since a lot of our patients are rather young and mostly women, it seems like a group that I can relate to, but also every other patient that we have, male or female, young or old, they all seem so just strong. That's the best word I could use. And you probably understand that resiliency yourself. I just really do love treating them so much and working with them. I wish one day I don't have a job because we cure this disease. It's easier said than done, but I hopefully, hopefully think we're going to do it one day. Sandeep Sahay, MD: I absolutely wish so. Talking to you, one thing which comes to my mind, which my mentor taught me, Dr. Frost... I guess everyone knows her in the PH world... always remember that your clinical trial should be the right fit for your patient and your patient is the right fit for the clinical trial. Every time whenever I think of a patient in a clinical trial, I just sit back for a minute and just think, "Is this trial going to benefit my patient? And is the trial going to get benefit by enrolling this patient?" These are two different things. And I'll be very honest. It took me a little time to really understand this difference. It sounds similar, but it is not actually. Jordin Rice, RN: I think whether or not you're participating in a clinical trial, you really have to take charge of your care. We see patients that do this and don't do this. The people that do it and take charge of their care and learn and absorb all of the information we give them, they do tremendously better. That goes with clinical trials, as well. You can be passive in your treatment and you can just follow through with the motions. But if you sit down and listen to everything we say and use us as resources and ask questions and navigate support groups online and use resources online, I really do feel like you'll do better, because it makes you feel like you're in control of something that's absolutely uncontrollable. I wish I could help every single patient and make everything better, but patients really need to do that for themselves, as well. They're their biggest advocates. And as much as I advocate for everybody, it's not as easy said than done. With clinical trials, if you have not participated in one or you're a patient listening, I would just look into some information. You can ask your doctors. You can ask nurses that you regularly see. Usually they're available at larger institutions. If you're not near a large institution or that's not as accessible, there can still be options with that. They really do change lives and not all of them are successful and become FDA-approved drugs. However, I still think there is value in participating in a trial, even if that's not the case, because one, you can get that medical care. And two, you form this relationship with staff that I think is instrumental to learning and being successful in coping with this disease on your own, because patients they come into the hospital for treatment and we expect them to be competent and know everything once they're discharged. Know how to manage their care outside of the hospital and mix their medications if you're on Prostacyclin, and that's not realistic. You have to have other ways of learning. You have to have mentors. You have to have people that are in your corner and supporting you. We can definitely be those people for patient. I really need patience to be those people for themselves sometimes, too. I want to support them and make everything better, but being able to learn and be a sponge is going to just change your life. We have so many different patients that either do well or they don't. There's no common denominator between all of them, but I really do think if you're always open to learning and really want to learn consistently and take charge of your care, you're going to do as well as you can. And we can definitely make that possible, especially with trials. Sandeep Sahay, MD: I hope this helps our patients understand about the clinical trials a little bit more, and I am Dr. Sandeep Sahay. Jordin Rice, RN: And I am Jordin Rice. And I'm aware that our patients are rare. /episode/index/show/phawarepodcast/id/20945651

Episode 364 - Sandeep Sahay, MD and Jordin Rice, RN 11/29/2021

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

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