phaware global association

@phaware | partner
Show the world you are #phaware of a rare, life-threatening lung disease. Join our mission to create global pulmonary hypertension awareness through engagement and innovation to forge a new course to a cure.
Erica Huntzinger

What Family Illnesses Taught Me About Meeting the Moment

My mom’s mom, my grandma, used to save money and she would jokingly say that it was for a trip she would take to Bermuda. Then when an emergency would come up, if she had to use her vacation money, she would say, “There goes my trip to Bermuda.” I was little, she was ill and I didn’tknow what or where Bermuda was or what it meant. Now I know she was talking about meeting the moment. Meeting the moment can mean different things. It can include how we face the social, spiritual and financial changes that serious illness and caregiving bring to our lives. Grandma was always sick, so my mom let her live with us. Later, when I was still little my grandma was diagnosed with end-stage cancer. She had a huge family that lived in the area, and no one helpedwith anything. Everyone had a reason why they couldn’t help out or even come visit.  This was in the ’70s before hospice was an option like it is today. My mom was Ms. Career Woman so she hired a neighbor to stay home with grandma while I was at elementary school, partly for grandma and partly so I would not come home and find grandma in a bad way. My mom was in her late 20s, looking back it is amazing to me that she handled things the way she did. My mom took a lot of criticism from people (the same people who could never come by to visit), about hiring someone to sit with grandma during the day. I did not think much of the neighbor being there at the time, because my mom always seemed to find a way to make things work out. Looking back, it seems like a brilliant idea, long before “caregivers” were an option like they are today. When mom was home, she helped grandma with whatever she needed, and sometimes when she was in the hospital we would go to the hospital every day and visit.  It’s just what we did. Usually, insurance does not pay for private caregivers, especially non-licensed (non-RN) so people choose to pay for that out of pocket if they have to. Like paying for a babysitter or a nanny or private daycare for small children. Home health nurses are not caregivers. They visit the patient to perform specific tasks and then they leave to see another patient. The role modeling for independence and caregiving started early for me as I watched my mom handle things and as she allowed me to help with tasks I could do. My mom was an excellent daughter and an excellent mom. When it was mom’s turn, my dad and I did everything for her. While it wasn’t easy, in time I came to see this as a way to repay a debt of gratitude to her. I also came to realize that everyone is not cut outto do this. I let go of any expectations of those who could not find time to come around and visit or offer to help out. I knew it would not do any of us any good to hold on to negativity, and I had to accept the fact that everyone was not going to meet the moment the same way. I didn’t want to have regretsabout not being there for my family so I rearranged my life and priorities accordingly and stepped into the moment as best as I could for however long it was going to be. At the time of my mom’s diagnosis, I was already working full-time and in college full-time. Rearranging the little free time I had meant delegating certain things like housework or cooking to others, and limiting my social life. I asked my friends to please understand that I needed the time with my family. Those years taking care of my mom were so important for us. It brought our family closer and we found ways to enjoy the time together. Sometimes people would tell me to take more time for myself, and I realized I felt guilty just at the thought of doing something fun out of the normal routine and notbeing there if an emergency came up. I felt terrible guilt. I felt I would be judged for doing something for myself. People will judge regardless but I felt I would be judged more than usual. I had to make my peace with this. Managing the day-to-day became easy. What was difficult was not knowing how long an illness would go on, and watching your loved one struggle with all the unknowns of pulmonary hypertension. This was the difficult thing. The unknown is not easy.  

Erica Huntzinger

Making Connections at the Pulmonary Hypertension Support Group

I had never been to a support group before. When my mom was sick, she opted to create her own support system and forego joining a support group. She chose to spend her time with her family and friends as much as possible. We had attended a large, organized event for pulmonary hypertension (PH) patients, which she found a bit overwhelming, and she decided it just wasn’t her thing. Years later, my friend Steve asked me about going to the local support group. He was surprised to learn I had never been to one. I told him my mom decided to forego that. My mom passed on by the time we were having this conversation, and I wondered what it was like and what happened there. Somehow my impression of a local support group had some resemblance to a therapy session, like on “Frasier,” “The Sopranos” or reaching way back, “The Bob Newhart Show” of the 1970s. As a daughter of a patient that passed on and not having PH myself, I felt like I shouldn’t go. Steve said, “I really want you to meet these people.” “OK, I’ll go if you go.” The group met at a restaurant that looked out on the ocean. The agenda went something like lunch, guest speaker/medical topic presentation, announcements, formal close and then everyone hung out and socialized. The group leader was Tiffany, the quintessential California girl in her jeans and a sweater and her O2, with her hair and nails done. I also met Ornah, an attorney with a megawatt smile and a spring in her step, and Phaedra, Ornah’s BFF, quiet and dignified but also steadfast and no-nonsense. Ornah seated me at a table next to Bruno and Bill, young men who looked like GQ models, and across from her in-laws who were visiting from out of town. As I looked around the room of about one hundred guests, there were people of all ages and ethnicities. At a glance, it was hard to tell who was a patient and who was a caregiver. Some might have had their O2 with them, and some might have brought a rollator or more. Some brought their kids because families were welcome. Aside from the medical presentation, there was not much about this gathering that was blaring “rare disease” at you. It was a group of pleasant people enjoying each other’s company. As the afternoon progressed, I said to Steve, “all the cool kids are here.” A PSA we had worked on just launched on television, and many of the people in attendance had seen it. Steve introduced me as the person who had gotten the PSA on television during announcements. True enough, I had called PSA directors all over the country and pitched the PSA to them. I smiled and waved a little as the room erupted with applause and cheers. Not for me specifically, but because patients and their families want to see more about awareness for PH, especially in the media, they were excited to see it happening on television. I told Tiffany and some of the others that I felt kind of weird being there, not being a patient myself. “No, we really want you here,” they said. Someone took me by the hand, sat me down, and told me their story of having both PH and scleroderma. Others wanted to know my connection to PH. Most of all, they wanted me to feel comfortable coming back to future group meetings and to connect on social media. Driving home, I thought about how this was not really what I expected. No Tony Soprano. No Dr. Frasier Crane. No Bob Newhart. No going around the circle talking about your feelings. And that’s OK. Instead, I had lunch with a hundred new friends. Many cities don’t have enough people to have a local support group. That’s why online support groups are so important. Patients all over the world are welcome in any number of PH support groups online, and family members are welcome too. It’s a place for moral support, learning, sharing, celebrating and sometimes a place where we grieve. It’s the friends you didn’t know you needed. It’s where you don’t have to explain anything anymore about what you go through because they get it. Some people have found love and romance in a support group, meeting their significant other, and some have even gotten married. As we celebrate World PH Day, let’s make sure we recognize the vital contributions of support groups that show us what being cool is all about.

Erica Huntzinger

What I Would Tell My Younger Self About Caregiving

When I was trying to be a helpful kid taking on caregiving tasks, I didn’t know any other kids my age in similar circumstances. I was not sure how to fully express what our family was going through, and I did not think of or label myself as a caregiver. I was just the daughter. A recent article in Forbes states there are 3 to 5 million kids under 18 who are caregivers in their families in the United States. Those statistics are from a national survey that was done just before the pandemic. Now with the COVID-19 global pandemic, that number will likely become much higher. The short- and long-term effects of COVID-19 on health and its impact on families are not yet known, the health care system is overburdened, and the economy is in recession. Families will have to do more to take care of their loved ones, and inevitably the younger members of the family will start to share in the responsibilities. This perfect storm of events helped me decide to do my first podcast episode with PHaware Global Association because I felt it was time to share how our family was not defeated by illness. In the episode, I share some of my experiences as the young daughter of a pulmonary hypertension patient, taking on caregiving tasks from middle school onward, and eventually caring for both of my parents as an adult daughter. I’ve been thinking about how our family somehow navigated through the years my mom did not have the correct diagnosis, and the twisting and turning road that took us there. I wondered if I knew then what I know now, would caregiving have been any different for me, and what would I tell my younger self if I could? This is what I would tell my younger self about caregiving: Enjoy and appreciate the people you love so much. You might have them longer than you think, but not as long as you would like. Make the time with them valuable. Tell them you love them, tell them how much, and tell them often. There is a long road ahead. There may be moments of uncertainty. There may be times you will wonder which ER visit will be the last, which doctor will have all the answers, and why we have to go to the hospital again. You may wish there were a magic wand that could fix everything. Instead, take it step by step. You will need to be flexible. Your capacity will expand beyond what you thought was possible. Ask questions and learn as much as you can. Keep good relationships with the doctors and others involved in the care and treatment of your family. You have all the abilities to meet the challenges ahead and be successful. It takes a certain kind of person to be a caregiver. Everyone might not understand or fully appreciate what your family is experiencing. They may not relate to things you choose to do or responsibilities you have. Don’t waste time waiting for people to understand. Ask for help if you need it. Create your own support team for the times you may need some direction. No one can do this alone. Let people earn your trust, and listen to your instincts. Don’t give up on what you want for your family or for yourself. Continue taking steps towards your own goals and ambitions, no matter how big or how small. The concept of work-life balance is overrated. You can best decide how to spend your time. Do not let others try to rearrange your priorities for you. Do not let others make you feel bad because you have family responsibilities that they don’t. Be at peace with your decisions. There will be times you may wish things could have been different. It is not easy to watch your loved ones experience health difficulties. You have a special purpose in your family to be there for them at this time. Someday you will experience the loss of a loved one. When the grief comes, you will have to be strong and not let it consume you. Everyone has their own way of dealing with grief. You will discover yours as well. Have faith, including faith in yourself. No illness will ever defeat you. Your life is precious and valuable. You are loved more than you know. This is dedicated to caregiver kids, caregiving youth, and family caregivers of all ages.

COVID-19: A Doctor on What Pulmonary Hypertension Patients Can Expect

In this special edition episode, Roham Zamanian, M.D., from Stanford University Medical Center discusses the coronavirus (COVID-19) and gives specific advice for the pulmonary hypertension community. My name is Roham Zamanian. I’m the medical director of the Adult Pulmonary Hypertension Program at Stanford University Medical Center and I’m here talk to you a little bit about what’s going on with the coronavirus and the specifics of what our pulmonary hypertension (PH) community and pulmonary hypertension patients should be thinking about. I think the first place that we have to start with is the patients in the community should know that there’s a lot of concern out there and there’s a lot of stuff in the media and there’s a lot of stuff on social media. The first place that they should really refer to for information, in general, should be the CDC website and their own county healthcare system website. Those will be the most up to date on the CDC website, if they look up coronavirus, CDC or COVID-19 CDC, they’ll be able to find very specific information, both for patients and physicians. I think that the second thing that patients should realize is this is a viral infection and it is one that is moderately contagious and moderately impactful on their health. It seems to be one that is probably more dangerous for susceptible patients and therefore that’s why PH patients should be careful about it and also the elderly. There is a lot of panic out there to some extent, but I think patients and caregivers, everyone, all of us, the physicians, should start off with the point where taking responsibility for your own hygiene and hand washing is of utmost importance. Hand sanitizers are good, but they’re not necessary. The basic are good hand washing techniques. There’s plenty of videos online that they could look up that and just basically employ it as often as possible. The second thing I’m telling my patients to do is to avoid unnecessary environments. Whether you visit a friend, it’s up to you or not, but really I think that at this point in time, there’s universities that have changed their classes to online. If you’re planning on taking a trip, it’s worthwhile looking into delaying that trip. If you’re thinking of going to a large event where you won’t have a lot of control over who and how you come in contact with other people, it’s wise to rethink that. I’m doing that for my own family, I’m doing it for my patients and I think that’s the first place we have to start off with. Some of the other things to think about are who are the points of contact for you for your healthcare. I’m sure each patient who is part of a pulmonary hypertension center should have access to their PH experts. Really, I think what we would begin with, is the idea is, do have an upper respiratory tract symptom? Do you have fevers and have you had any recent travel or contact with people who have either traveled to areas that are suspicious? The international destinations that are currently listed by the CDC The hardest thing about this is it isn’t just a common variety flu and upper respiratory tract infection. But I think being vigilant and making sure to communicate with the healthcare system. Getting your information from reputable sources is a good place to start. It’s always good to have hygiene products available. The hand sanitizers have become ridiculously difficult to find and they’re not necessary. First of all, the hand sanitizers that you have to find are the recommended ones that have 60% or more of alcohol. It’s obvious, but patients should not be using drinking alcohol like vodka or any of those spirits to make solutions to wash hands with. The reality of it is you don’t need that. You could really use antibacterial soap along with lukewarm water, and hand-washing techniques. Again, you could look up the duration and how to do proper hand washing out there. So I think a supply of a product like that. Patients may be told by their physicians to isolate themselves. They may be told to self-quarantine in their home, for either their own safety or other people’s safety. If that happens, obviously they’re going to have to have supplies that could last them seven to 14 days, whatever duration that they’re told to do so. But make sure you have a plan, both a communication plan and a support plan. A lot of the PH patients that we take care of have family that support them as well. So I think you should be thinking about some of those support mechanisms that could help you get through it. There’s nothing magical. You don’t need to run out and buy all the toilet paper in the world. You don’t need to go buy all the water bottles in the world. It’s more that you should have some plans in case you are required to quarantine. Then I guess the bigger thing is avoidance of any ill contact or situations where they can get exposed to other people who they don’t know if they have had ill contact. The Stanford team is inclined to say that our stable patients with pulmonary hypertension who haven’t had any change in their medications or change in their symptoms for a very long time, we are considering asking them not to travel to the clinic and come to a healthcare environment where there’s potential exposure. But again, I think it’s very difficult to get specifics on this and I think that the patients return to their primary pulmonologist or primary care physician, whoever’s caring for them for their pulmonary hypertension to decide on that. In terms of the flu shot and flu vaccine, while the vaccine is not for this strain of coronavirus, I still encourage my patients to seek and get flu shots if they have not done so already. We have to think that there are multiple viruses that are traveling around, and this happens to be something that we’re very focused on, coronavirus, but there are other pathogens, other viruses that are respiratory viruses that we still want to make sure our patients are vaccinated against. So our recommendation stays the same. But the other aspect of this is that patients who are on routine medications through mail order that need to be filled, they should stay on top of it. Again, not giving any specific advice, but it’s good habit and good practice to make sure you plan ahead for your refills and have those in hand. I think the only other thing to say is that the situation continues to be evolving. Patients should continue to stay up to date with, as I mentioned, appropriate sources and make sure that they follow the advice of their primary or pulmonary cardiology physicians. This is a dynamic situation, things are changing and as protocols and procedures change or if there are any significant updates, patients should continue to be engaged and look at the CDC. Obviously, we’re glad to have another follow-up interview and give you any updates, whether we have some at Stanford or in the broad sense for patients with pulmonary hypertension. My name is Roham Zamanian, I am a PH physician and I am aware that my patients are rare and lovely. Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials . Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin. Engage for a cure: www.phaware.global/donate #phaware #phawareMD #Coronavirus #COVID19

Dr. Victor Tapson Shares How COVID-19 May Impact PAH Patients

In this special edition episode, Victor F. Tapson, M.D., from Cedars-Sinai discusses the coronavirus (COVID-19) and how it might impact pulmonary hypertension patients. Hi, I’m Vic Tapson. I’m in pulmonary critical care at Cedar Sinai Medical Center. Wanted to talk for a few minutes today about this coronavirus (COVID-19) and how it might relate to pulmonary arterial hypertension (PAH) and how we should be reacting and some thoughts about this. I think maybe the most important point to make first is while we’re taking this seriously, we don’t want to panic. One comment I want to make right up front is if you’re a patient with PAH, most patients with PAH are no more susceptible to this than anyone else is, but let me give a bit of background here. I don’t want to talk a lot of detail, because you can find a lot of this online. For those of you that don’t know, it appears that just like some of the earlier viral epidemics, like SARS and MERS you may have heard of, we clearly know the coronavirus, this novel strain called COVID-19, it’s clearly transmissible between people. Right now, 100,000 cases around the world, and this is early March. It’s been identified now in a hundred countries. Most of the deaths so far been in China, more than 3,500 deaths so far in China. More than 400 cases identified in the U.S. so far right now. Keep in mind, we haven’t done a lot of testing, so there may be more. 19 deaths in the U.S. Most of these have been in this Washington state cluster in a nursing home. We’re seeing some cases in California now. New York State has now declared a state of emergency. But again, I want to keep reemphasizing, this is not the deadly type virus that some of these early viruses, like SARS and MERS, which had more than 10% mortality rate. Our mortality rate right now we’re thinking is in the very low range of one or 2%, something like that. It does seem to affect certain people more than others, the elderly and immunosuppressed maybe more common. It’s around the world now. Italy has had more deaths than any other country except China now. The government in Italy’s closed all the schools. We’re certainly not there yet in the U.S., but there’s some schools that have been closed so far. I’ve missed three national meetings in the last couple of weeks because of caution with this virus. The impact, if we’re careful, I think because it is going to be pretty minimal in patients that have pulmonary arterial hypertension. Let’s talk a minute about symptoms and then how might relate to PAH. Right now, the incubation period of this virus, it may be a few days up to 14 days. Most infected people seem to show symptoms in about five or six days. In the original Chinese cohort from late January, most common symptoms were fever, fatigue and dry cough. That still seems to be the case. A percentage of patients, maybe around a third, may have muscle pain, difficulty breathing. Then a smaller percentage, sort of atypical symptoms, like diarrhea, nausea, things like that. But again, if you have PAH, if you get the flu or a bug like this COVID-19, those are the symptoms you’ll probably have, fever, fatigue, dry cough. Then maybe, we don’t know for sure, but a percentage of patients may get worse. Over 80% of patients will have very mild disease, may have minimal symptoms, if any. They may think they have maybe like a common cold, sore throat, runny nose, fever and you may get better from it. So majority people are going to do fine from this. We’re more concerned with older patients and immunosuppressed patients, like I mentioned. What I should maybe comment about now would be recovery. Again, many people that have gotten this disease, if it’s mild, may be over completely in two weeks. If it’s more severe, it may take three weeks or more to recover. There’s going to be some variability. This virus, it multiplies in the respiratory tract, so things like coughing, sneezing, may help spread this. Also, talk about shaking hands. Even people doing fist bumps now, elbow bumps now, because we don’t want to pass this virus. We want to be cautious with hand washing. We don’t know right now how long this virus might last on inanimate objects. People are very careful to wipe down your tray if you’re flying someplace. There’s certain specific things we can talk about even in terms of travel. Let’s talk a little bit about what you might do if you are exposed, depending on where you are. One of the reasons I’m a little concerned about doing any unnecessary travel is even if I get this coronavirus, I might do OK. Now, I don’t have pulmonary hypertension, but just like most PH patients, I’ll probably do fine. I’m a little worried about getting quarantined. I don’t want to get quarantined someplace for two weeks, so I’m going to be cautious travel-wise for that reason, as well. Necessary travel is one thing, but travel that really isn’t necessary probably wise to back off from. A few things to keep in mind, again, whether you have PAH or whether you don’t. If you’re going to be around other people, a lot of other people, consider wearing a mask, cover your mouth and nose with tissue to cough or sneeze, discard it in a trashcan, immediately wash your hands with soap and water for at least roughly 20 seconds. Use a hand sanitizer if you can, but soap and water are really preferred. Another important thing, again, for anybody, PAH or not, avoid touching your eyes, nose and mouth. If you don’t think you do it, then test yourself, because we all do it a lot more than we think. We all touch our faces just randomly and without realizing it subconsciously. You’ve got to try to practice not doing that. It may prevent infection. Best not to share dishes, drinking glasses, eating utensils, things like that. Wash things like that after you use them. Use a household cleaner to wipe countertops. Again, this stuff applies whether you have PAH or not. Another important key point I should make too is you should get vaccinated for flu if you haven’t. That won’t help with COVID-19, it won’t help with the coronavirus per se, but if you get the flu and you can be tested for COVID-19, you might be designated as a possible case, you might get quarantined. You can imagine how they can just confuse things. We’ve got people coming to emergency department with flu because they didn’t get vaccinated. We’re not going to know right away whether they have COVID-19 or the flu or what they have, and that’s going to create problems. So, I’d keep that in mind. I want to be a little more specific about PH now. If you’re older, elderly, and I won’t give a cutoff age, you’re a little more susceptible to viruses and to getting more severe disease. If you have any abnormality of your immune status, if you’re on prednisone, steroids, things like that, you may be a little more susceptible to this disease and getting a worst case I should say. Let’s suppose you’re a patient with idiopathic PAH, like many of you out there, or scleroderma. If you’re not on steroids, you’re not on immunosuppressive drugs, let’s say if you’re younger, you’re stable, there’s no particular worry you’re going to have a severe problem if you get this disease. If you’re one of the rare people, just like patients without PH, you could get severe disease, but 80% plus patients are going to be just fine. Just do the same things we talked about. Wash your hands, don’t touch your face, et cetera, et cetera. Let’s suppose you’ve got scleroderma or maybe you have lupus, suppose you are on immunosuppressive drugs. Again, you want to be very careful not to get any kind of a viral illness or bacterial illness if you can help it. We’re more concerned about patients that are immunosuppressed or older, so that applies again, PAH or not. Let’s suppose you’re a patient that has PH from CTEPH, from chronic pulmonary embolism or maybe from congenital heart disease. Again, you’re no more likely to catch this coronavirus than anyone else. If you get it, if your disease is very stable, you’re unlikely to get any more ill than anyone else would. You may be one of those rare people that gets sicker, but it’s more likely if you’re older. What if you’ve got PH from IPF or pulmonary fibrosis or COPD? Again, many patients that have these diseases are older. If you’re older, you’re going to be more susceptible, and if you have abnormal lungs from severe PH and/or pulmonary fibrosis or COPD, again, you might not tolerate a severe infection as well as someone that doesn’t have lung disease. If you’re a PAH patient who has severe disease, maybe you were just started on IV treatment. You’ve got more severe disease, again, your lungs aren’t going to tolerate the insult from a virus as well as someone who has normal lungs, but again, 80% plus, most patients who aren’t older and don’t have any kind of immunosuppression going on are likely going to do fine and not get severe disease. A situation where we want to be additionally cautious would be suppose you’ve got PH and you had a transplant. If you had a transplant, then you are on immunosuppressive drugs. These immunosuppressive drugs can make you more susceptible to infections and you might get a more severe case. Again, you want to be particularly cautious about being around a lot of people at the same time, [not] being closer than six feet to someone if you can help it. You don’t want to fly any place that’s unnecessary if you can help it. But you really want to use particular cautions in that situation. In summary, in pulmonary hypertension, if you aren’t immunosuppressed for any particular reason, if you did get a severe case, you’re more likely to tolerate it better, but you’re less likely to get a severe case of this coronavirus. Again, I can’t emphasize enough the importance of hand washing, not touching your face, not being any place in crowded conditions where there’s a lot of people around, especially people who have traveled. These are some of the important things that you should keep in mind. We don’t know what’s going to happen here. I think we’re watching very closely. I’ve got a couple meetings coming up. I’ve got one in New York in a couple of weeks, I’ve got to decide whether I can go or not. In some situations, these decisions are being made for us because the meetings are being canceled. Some medical centers, I’ve heard of several now, are limiting travel. Certain medical centers are saying you cannot travel, if you’re on staff you cannot travel internationally. Some are even saying you can’t travel domestically. We need to make sure that physicians and healthcare professionals don’t get sick and get quarantined. These dynamics may change over the upcoming weeks. There’s no vaccine, as most of you know, right now. The CDC, they’re scrambling. Researchers are working on this. We don’t have a vaccine right now, but again, get a flu vaccine if you haven’t had it. Make sure you’ve had the flu vaccine, because that might prevent confusion and prevent you from getting sick also. These are some of the key points. Right now, we’re in a dynamic phase with this disease. If you’re older and you’re immunosuppressed, you’re a little more susceptible, but everyone should be cautious, using hand washing, being careful not to do unnecessary handshaking and sharing utensils, things like that. If you do get symptoms, you should report it. Many people will have mild symptoms, and if they can’t be tested yet, should still try to stay home and be quarantined, self-quarantined if you have to. Stay away from others, especially those that are more susceptible to this illness. Most people that contract the coronavirus, this COVID-19, will not require hospitalization or medications of any kind. Most patients, PAH or not having PAH, are not going to require such medical care and will recover on their own. Some will require hospitalization and more intensive care. Most patients with PAH, if they get coronavirus, are going to do fine. They’ll probably be in that 80% plus that don’t get severe disease. If you do get a more a severe syndrome, the vast majority of people will survive this, unlike some of the earlier epidemics, like SARS and the Middle Eastern Respiration Syndrome, MERS. Again, I think the bottom line should be let’s take this seriously, let’s be careful. Keep up to date on what’s going on, on the news and the online, with this coronavirus, but let’s not panic. There’s no reason for panic, let’s just stay educated. What I would say about patients who think they might’ve gotten this virus, they may have some cold symptoms, a cough, a fever. Sure, you need to contact medical personnel, but I wouldn’t just show up at an urgent care clinic. I would call ahead. If you’ve got a family doctor, let your doctor know and say, “Here’s the scoop. I think I’m getting something. I don’t know if it’s coronavirus, but I’m getting sick. Based on my symptoms, what do you think I should do?” If someone’s having a high fever, is having difficulty breathing, they’re going to need to present for help, but they should be guided. Naturally, anytime someone is suddenly very, very sick, you call 911, but if you get to that point, I think that’s going to be pretty rare with a situation. You want to let any EMS personnel know that you’ve been having these kinds of symptoms so they can take proper caution. You always want to let the medical personnel at the clinic know upfront you’re coming, let ED know upfront you’re coming. It’s better for them to be able to give you some advice about how to handle it. I think in many, many cases patients can be monitored and can stay at home, but we started to want to ignore patients who are having more severe symptoms. Well, folks, keep tuned in everybody on what’s going on with coronavirus and maybe we’ll talk again soon. Thank you. My name is Vic Tapson, and I’m aware that my patients are rare. Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. . Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin. Engage for a cure: www.phaware.global/donate #phaware #phawareMD #Coronavirus #COVID19

I'm Aware That I'm Rare: Candace Malson, RN (256)

Candace Malson, DNP, R.N., ANP-BC, is an adult primary care nurse practitioner in Lake of the Ozarks area of Missouri. She is currently writing a book about dealing with the diagnosis of a life-limiting condition. She also give health related talks in the community. She shares her impassioned views on advocating for oneself. My name is Candace Nelson. I’m a pulmonary hypertension patient and I am also a primary care nurse practitioner. I had an initial diagnosis back in 1992 when I first became a nurse. I had a heart valve replaced and a heart doctor at that time told me, “You have PH, but you don’t have to worry about it because your heart’s been fixed.” Now 26 years later, apparently it’s back. When I first heard about it, I did some research, got what information was there, what 26 years ago. “Oh, you’ve got a very short life span,” and he said, “Oh no, you don’t have to worry about it.” Well, of course, in the interim years I had other things to deal with like recurring atrial fibrillation and all kinds of stuff like that and PH went away until last fall when I’d gone through a heart valve replacement, a cardiac ablation, umpteen cardioversions, every drug known to mankind to manage AFib. Ended up in total heart block, got a pacemaker, was feeling better. That happened about two years ago, and then about last fall I started getting tired very easily. A flight of stairs had me panting like a puppy. I went back in and my small-town cardiologists said, “You’ve got PH.” She sent me to someone in St. Louis who did an exercise stress test and said, “We’re going to put you on the Letairis. At that point I said, “Time out,” because that month from the stress test to my last visit with him, being a clinician and working for the VA, where I had access to whatever journals I wanted, I did an extensive search on PH and found that number one right heart catheterization is the diagnostic gold standard. He didn’t do this. He didn’t do a VQ scan. He didn’t do a lot of things, but he was ready to start treatment. But I told him that I am a clinician, and if for no other reason than I am a clinician being part of the problem isn’t enough. I want to be part of the solution. I want a referral to a PH center so I can offer myself for studies, clinical trials, whatever. If they turn me down, fine, but if I don’t make the offer, we don’t know what we threw away. I learned a lot about doing peer reviewed research, not only in my master’s program, but in my doctorate program and it can be very exhaustive, but I learned that there are ways to target things so that you can be a little more efficient. My feeling was, if I don’t do this for me, nobody will. I have to do this for me, but I’m also part of this community. I have the ability to do this so I don’t just owe it to myself. I owe it to my community to be part of the greater good. I was on the table in the lab at this hospital in St. Louis, where I did this very interesting stress test where I was laying on my back on kind of a gurney that had one of those small cycles with the surgical boots attached so that my shoes wouldn’t come out and I was laying on my back and peddling. There was a nurse on my right side injecting something. It was some kind of a contrast, so they could get better pictures of the heart. There was an echo tech on my left side moving the probe here, there, and there was the physician near my feet saying, “Yeah, keep the second light lit,” and telling the technician, “Yeah, that’s good. Capture that, capture that, capture that.” When the test was done, he said, “Oh you did very well. Your pressures go way up when you exercise, but it’s okay. We’ll put you on Letairis.” That was what I heard before he walked out the door. That gave me a month between then and my follow-up appointment to do my homework and realize that I got to look out for myself. Anything you can do for yourself, do it. Call anyone you know. Talk to anyone you know. If you hear of anyone who has the same condition, see if you can connect with them and talk to them. Because I’m in the clinical world and I can ask people, who do you like for primary care? Who do you like for endocrinology? Who do you like for different things? But most people don’t. Any network you have, use it, milk it. Take as much ownership as you can. I live in this body. I own it. I know it better than anybody because I’m with it 24/7 and anybody with PH will tell you the same thing. You can get all the tests you want. People can do all the research they want to do, but 24/7 this is what I own, and my experience has as much value as anyone else’s experience does, and I have the ultimate responsibility for myself. Call somebody. Write somebody. Facebook has support groups. Get on Facebook and talk to people. Look up pulmonary hypertension. You will find all kinds of help. If you have access to clinical level stuff and you can handle it, go for it. Do everything you can to advocate for yourself because the best outcome comes from the best investigation. Every one of us has the opportunity to participate, and I don’t know what my personal outcome is going to be. Maybe it’s not even going to be so hot, but maybe some little thing that I do somewhere with someone regarding this disease will feed that great bucket of information and someone else down the road will get a benefit. We’re all part of the community. We’re all park of this world, and the best way to make it is to stick together. Life is short. Fill every minute with whatever you can. Live. I’m Candace Nelson I’m aware that I’m rare. Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: www.phaware.global/donate #phaware #ClinicalTrials @antidote_me

I’m Aware That I’m Rare: Sarah Brackett (232)

Pulmonary hypertension patient Sarah Brackett discusses her devastating road to diagnosis, busting CTEPH through surgery and the importance of educating medical professions in rural areas to raise rare disease awareness. My name is Sarah Brackett. I live in Manassas, Virginia. In my life, as far back as 1985, I’ve had 3 pulmonary emboluses, two of them following surgeries after a Greenfield filter was put in to prevent them. And within six months of the last one in 2004, I started getting very tired and short of breath, and I wasn’t feeling good. I had three teenagers, so I was a busy mother. I went to the doctor, a lot of doctors, for six long years trying to find out what was wrong with me. I was getting worse and worse and worse. Clueless doctors who gave me the most ridiculous theories that “you need to lose weight”, “You need to change your vitamins.” One even told me I had pulled a muscle in my neck. I could barely walk down the stairs. I couldn’t hardly roll over in bed. Every day I was getting worse. I couldn’t do the things that I was doing. It was a really bad place to be in. I can remember not being able to sleep, because I was so filled with fluid and so short of breath. You couldn’t lay down, it made it worse. I would try to get up at night and go to the bathroom, and I would just stand there. I knew I was dying. Something was clearly wrong with me, and I knew it. Why couldn’t anybody tell me? It wasn’t that I needed to lose weight or change my diet or take vitamins. Something was wrong and nobody’s telling me. And I’m scared, really scared. And that’s where it started. I got so bad I couldn’t walk 10 feet across the floor. My children, who were teenagers at the time, they were crying to their father, “Do something! Do something!” And I’m going back and forth to doctors, which just going to the doctor was the biggest effort of my whole week just to get to my car and sit there for 20 minutes until my heart stopped beating out my chest so I could drive it to get to the doctor. And then having to sit in my car for 10 minutes before I got out to try to get in the doctor’s office. All the time, planning how many steps do they have? What do I have to do? Only to come out later with another ridiculous theory. Finally, I was so sick I just wanted to die. I knew I was dying. I’m tired of fighting, I’m tired of trying to find out what’s wrong. So my husband literally picked me up and carried me to our van and took me to his doctor. I’d never seen her before. She took one look at me, and she said, “You’re in heart failure.” She wanted to call an ambulance right there to her office. I cried and begged, “Don’t do that. If I’m going to die, I just want to do it at home. I want to do it at home.” She let me go home. She gave my husband phone numbers. She said, “Look for this, this, and this. Call this number.” So she got me oxygen. It was at home by the time I got there. She setup appointments for cardiologist, and I was in bed for three months until my heart calmed down. The oxygen helped. I went to the cardiologist, they did all the tests. They are the ones that said I had pulmonary hypertension. I didn’t know what that was. One of the technicians told me when she was doing an Echo. I said, “Is everything okay?” She said, “Well, I think a little pulmonary hypertension,” she should never have told me that. But she did, and it was on a Friday. I went home and googled that term. It was terrible. It was the worst weekend of my life. I just knew from what I read that I wouldn’t live two years, if I lived that long. I’d never see my children get married, I’d never see grandchildren. She got me to good doctors. The doctor she got me to that diagnosed me, they sent me to Fairfax Hospital where I’ve met a good pulmonary hypertension specialist. They put me on Tracleer. Later, they added Adcirca. They, at first, were going to do Remodulin, but I have little pets. I was a little scared of that. I was just scared of the pet. So I asked them, “Can we do anything else?” So they did the Adcirca, they did the Tracleer. I was on that for a long time. I got a little better. They wanted me to go to pulmonary rehab, which I thought, “I’ll never be able to do that.” But I did. They were so wonderful. They let me take my daughter with me, and they were very good. They did the exercises with me. They checked my stats every time. I got stronger. It was good stress reduction, and I really needed stress reduction at that point. I needed something to look forward to. It was really good. For someone who thought I would not enjoy it, I actually did enjoy it. I got better. It took four years of getting better until the doctors at Fairfax said, “You are the best we can get you.” At that time, Adempas had come out. They took me off other medicines and put me on Adempas alone. I was still on oxygen, was on oxygen for four years. They spent four years getting me better, and then they said, “You’re as good as we can get you.” I felt great other than lugging oxygen tanks and taking handfuls of pills a day. I really felt good. I accomplished a lot at that point. So they wanted to send me to California to UCSD. I just never been to California. I couldn’t imagine having my family there with me, so I was like, “Oh, I don’t know.” My doctor, she knew Dr. Paul Forfia at Temple University Hospital, and so I went there. I spent several months doing all the testing and procedures. They found I had AFib due to the stress of the pulmonary hypertension. So we had to do procedures to take care of that. They found that I had absolutely no blood flow in the bottom of my lungs. This was by the pulmonary angiogram. You could see it. And so they spent months getting me the best I could be for that very serious surgery. I had that very serious surgery on June 1st of 2015. I did very well. I was hyped when I went into the OR. It had been a 10-year journey. I told them I was elated. I said, “Please, put me to sleep and bust the CTEPH for me.” And they said, “You’re a very brave lady.” That’s all I remember. I had the surgery, and I was home in about 10 days. I was on oxygen for a couple more weeks. But then I was able to get rid of it. I felt good. No more expensive meds, no oxygen. I felt great. I was out going and doing and trying to make up for all the things I missed. Then at three months, I went back to Philadelphia for a checkup. I did the six-minute walk, no oxygen. I’d just aced it. I was so excited to see my doctor. They did an echocardiogram. He came in, and I was so excited to see him. He said, “Well, we got a little problem.” I went, “What? What’s the problem?” He said, “Well, you have a little fluid around your heart.” I said, “Okay, what do we do? Do we take a pill for that? What do we do?” One more thing. “No,” he said, “we have to drain that off.” I went, “What?” So he explained that my heart, which was so enlarged, that had worked for such a long time beating at a marathon runner’s pace was enlarged. After the surgery, my heart started to calm down, and so it started to shrink. That area fills up with fluid, that space where it shrunk. This was not good. After that six-minute walk, they immediately put me in a wheelchair, put me back into cardiac intensive care, where they had to insert a tube in my chest. And so I was in there for another week. They drained that fluid off. I had no symptoms of distress. I felt great. They let me go home. And I’ve been great ever since. A year after my surgery, they found that my pressures were at the higher end of normal than the middle where they wanted it to be. This was a long ways from when I was very sick, and they were about 120 something. Now they were in normal range. They decided to put me back on a very low dosage of Adempas. I just take one milligram of Adempas three times a day, and I’m doing great. Now people say, “Are you cured?” Well, I have a hard time with that. I can’t say I’m cured. I can say that I am so much better. But I can’t walk away from this. It was 10 years total of my life, and I have a hard time walking away. I feel like I have to give back. I’m better, I can do more. But I know a lot of people who aren’t. And what can I do? So I am a co-leader of our Northern Virginia PH Support Group. I run a Facebook group for CTEPH, and that gives me great pleasure. I spend a lot of time every day on the computer talking to people, trying to get people to understand that if you have this, this and this, symptom… there are something that you might have. I don’t ever want anyone to be as sick as I was. It was the scariest time in my life to know that I really felt like I was dying. I went through a lot to have my children, and I was in the position that I was scared I was going to leave them. Before all of this, when I was very sick, I was so scared that I wouldn’t be here for my children. They were teenagers. I wouldn’t be here to see them get married. I wouldn’t be here to see them become the teacher and the nurse that I knew they wanted to be. How would I explain to them that I’m dying? That mom’s just not sick, I’m dying. But now, now after my surgery, now that I’ve thrown away the oxygen, I’ve thrown away the medicines, I did see that daughter become a nurse. I did see that daughter become a teacher. And I am going to be a first-time grandmother. So for all the pain and agony I went through for 10 years, I can’t walk away from it. I work every day to help others. But I’m looking forward to a future now. After all of this, so many people every day of their lives, they complain. After all of this, you learn don’t sweat the small stuff anymore. Really. It’s just being able to live to end of your day and breathe. It’s being able to walk to your mailbox without feeling like you’re going to die. It’s those little things in life you take for granted that once they’re taken away, you realize how important they are. My children and my husband probably get really tired of hearing me flap my jaws about my story, but I want to reach that one person in the grocery store thinking, “Oh, my husband sounds like that.” Or, “My sister has those problems.” That’s the one I want to reach, because I don’t want their relative to be as sick as I was. The more we talk, the more we get out in the not so much the big cities, but the small communities. Up in the hills where there’s little hometown doctors that’ve been there for 40 years. I want to get to them. I want to get to those people. I actually have a relative who’s since passed away, and she lives way up in West Virginia. She had pulmonary hypertension. Nobody did anything to help her, and she died. That’s just so sad. If we could have gotten her down to the city where good doctors are. So all the talk we do, whether it’s out of my mouth or a podcast or a piece of paper or a conference, that’s what gets the word out there. And that’s what I want to do. This mouth will work for a long time. My name is Sarah Brackett, and I am aware I’m rare. Learn more about pulmonary hypertension trials at . Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: #phaware #ClinicalTrials @antidote_me  

I'm Aware That I'm Rare: Laura Rivera (247)

Patient Laura Rivera has contended with Pulmonary Hypertension for 24 years. She hasn’t let the disease stop her from achieving her goals. Laura will be receiving her Bachelors Degree in nursing in a few weeks time and beginning her graduate studies in the fall. My name is Laura Rivera and I’m a pulmonary hypertension patient. We need to get the word out so that people understand this illness. I was diagnosed back in 1995 when it was rare. There was only one medication and no one really knew about it. I was diagnosed by Dr. Robyn Barst and she really helped me understand and she walked me through how to handle this illness when it was so brand new back then. The sooner you know what’s going on with you, the sooner you can get on medication and start feeling better. I was diagnosed by fluke. I was doing a medical assisting program, and the professor who was teaching us how to read EKGs was a cardiologist himself. When he read my EKG, he was like, “Oh no, there’s something wrong with you. You need to go see a doctor right away.” I do the telephone support line in Spanish. I also co-lead the Spanish support group in The Bronx, New York. I’m always, you know, trying to tell people who are interested in knowing, because I’m on IV Remodulin, so they see my pump, they see my line. Or if I’m walking around with my oxygen, they’re always asking me, “Well, what is it that you have?” So I’m always happy to educate on the street. Back in 1995, there was only one medication, which I refused to go on. I said to Dr. Barst, “Put me on all the research [studies] that you can. I’ll do it.” I did the study for a medication back then that, it didn’t get approved, but it’s good to do that because you can tell what works and what doesn’t work. It’s so much easier for them to just decipher, okay, this is not good for you, because now there’s so many types of medications that you can pick and choose what is going to work for the patient and not. It’s very scary in the beginning, but take a moment. Take a deep breath and keep striving for what you want. Don’t let this illness stop you. I just recently graduated with my Associate’s [degree]. I’m going back to do my nursing and it’s not going to stop you. As long as you do what the doctor tells you to do and take good care of yourself, you can go ahead and achieve your dreams. My name is Laura Rivera and I’m aware that I’m rare. Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: www.phaware.global/donate #phaware #ClinicalTrials @antidote_me  

I’m Aware That I’m Rare: Kathleen Richardson (223)

Pulmonary hypertension patient, Kathleen Richardson, discusses exercise, Team PHenomenal Hope and why research is critical. I’m Kathleen Richardson, and I am a pulmonary hypertension patient. It was after my son was born, my youngest son, he was about two years old, and I was feeling some shortness of breath. It seemed odd to me, but it wasn’t significant. I just figured it was after having three children and feeling a little bit short of breath. But then I went to SeaWorld with some friends, and we climbed all the way from the bottom to the top of this stadium, and I was so short of breath I didn’t think I was going to recover. But I did. It was short, but I looked around and no one else was breathing as heavy as I was. So I realized there really was something wrong, and I needed to get to the doctor. So I went to the doctor, and it took about six months going to test after test after test. They were coming back normal. The doctor asked me if I’d ever been diagnosed with panic attacks, because I think he was thinking maybe it was in my head. I don’t know. That’s the message I got anyway. I said, “No, it’s not panic attacks,” but I did start to question myself at that point and wondered whether I should keep looking because maybe there wasn’t anything wrong. It was just in my head. But I did keep pursuing, and they finally did a stress test. When they did that stress test, my blood oxygen saturation dropped significantly. It went down to 78. So then they said, “Yeah, there’s something wrong with you.” And I said, “Yeah, I kind of figured that.” So then I had a few more tests, and then finally had a heart catheterization. After the heart catheterization, the cardiologist said, “There’s nothing wrong with your heart. It’s got to be something with your lungs. We did notice a little elevation in pressure over here, but not too much.” Then a couple days later, he called me back and asked me to come to his office. It was there that he told me he consulted with another cardiologist and said he thought I had pulmonary hypertension. And he didn’t really tell me what it was, he just said, “You need to go to a cutting edge hospital. I don’t know anything about this disease, and it’s important for you to get to somewhere where they know about it.” Back then, there were three treatments for pulmonary hypertension. I don’t feel like there was a huge hopeful outlook. But over these last 17 years, they’ve developed 14 treatments for pulmonary hypertension, and it feels so much more hopeful. I feel like awareness has increased. I feel like there’s still a lot to be done, but there’s a lot more awareness, and I think there’s more organizations that reach out to pulmonary hypertension patients. I feel like it’s a lot more hopeful now. My life really did not change very much. I thought that it was going to, because when I learned what pulmonary hypertension was, at that time they were saying that the survival rate after diagnosis was like two to eight years average. There was a lot of emotional, psychological things to kind of work through. I had little kids. My youngest was two, my oldest was seven. So just kind of trying to deal with my own mortality really. But I responded very well to the medication that I was put on. I began to exercise. The doctor told me that I could get my heart rate up to 115, and that would still be kind of safe for me. Because above that, I would be doing more harm than good. So I started riding my bike, and I’ve gotten better in my pulmonary pressure. Dropped down to normal after a few years, and I’ve stayed real active and gotten involved in Team PHenomenal Hope. Because they started out as cyclists, and that was really exciting for me. So I’ve been involved with them for now the last four years. I’m on the team as an athlete, and I am a cyclist. But I’ve now kind of branched off into triathlons. So I’ve done a couple of sprint tris and I did an Olympic tri, and I’m hoping to do a half Ironman. I have a partner, a #LetMeBeYourLung partner. Haley York is my partner. Pulmonary hypertension awareness is important because we need to get diagnosed early. There are still so many people who go for a long time without getting diagnosed or they get misdiagnosed. So it’s really important for doctors to become more aware, medical professionals to become more aware. Because it’s still a rare disease, and people need to know about it so they get diagnosed early, get proper treatment early. I would say, really work hard to become knowledgeable about pulmonary hypertension. Get involved in a support group. Follow your doctor’s orders. Research and trials is critical, because there’s still so much that they don’t know about pulmonary hypertension, so much that still needs to be learned. We want a cure. We want to get rid of this whole disease. I’m Kathleen Richardson, and I’m aware that I’m rare. Learn more about pulmonary hypertension at Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: @antidote_me @accpchest #phaware @TeamPHHope