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I'm Aware That I'm Rare: Raymond Benza, MD (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 clinicaltrials.gov/ct2/show/NCT04456998. 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 clinicaltrials.gov/ct2/show/NCT04456998 or the clinicaltrials.gov/ct2/show/NCT04456998. 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 www.phaware.global/clinicaltrials. Never miss an episode with the www.phaware.global/phaware-podcast-app Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: www.phaware.global/donate #phaware #ClinicalTrials

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

I'm Aware That I'm Rare: Gwendolyn R. Brown (225)

40 year long-term pulmonary hypertension survivor Gwendolyn R. Brown discusses the importance of clinical trials, building a great PH care team, and how THE LION KING is a perfect metaphor for PH. My name is Gwendolyn R. Brown, and I am a 40 year survivor with pulmonary hypertension. When I was diagnosed at that time, there was nothing out there. For three years prior, I was getting sick, I thought it was a cold. And I kept going to the doctor, and then they diagnosed me with asthma. The asthma medication wasn’t working, and it started getting worse and worse. I started passing out. And my last time I passed out, I remember waking up in the ICU and there was this one doctor there. And he was like, “I think you have this disease. It’s a rare disease, but I need to find out.” Back then, they didn’t do right heart catheterization, they did lung biopsies. So, I had to have a lung biopsy done, and when he came back, he said, “You have pulmonary hypertension.” He said, “It’s a very rare lung disease.” He said, “We don’t even know nothing about it right now.” He said, “All I can tell you is go home and get your life in order.” He said, “I don’t expect you to live two years with this.” And I did, I went home and got my life in order. I did research, and I just took care of myself. I ate healthy, I lost the weight, and walked every day. I did everything my doctor told me to do. And that lasted until 1998, when Flolan came out, and I was picked as one of the trials to go try it out at Duke University. And that’s where I met the great Dr. Victor Tapson. He introduced me to Flolan, and from then on it was amazing. I think [clinical trials] are important for patients, because even though the trial might not help me, it can help someone else. With the disease being as rare as it was, I put my name down for every trial. I am so amazed when I see all the medicine, all the treatments that are available now for people. It’s just astounded me. I end up crying because it’s just beautiful to see it all. But like I said, when I was coming up, there was nothing. And when they would start new trials, I was like, “I’m here. Can you help me?” And with the Flolan, at that time I was on oxygen all day. I was in a wheelchair. I was on the heart and lung transplant, because back in those days, they were only going to both lung and heart transplant. And I was like, “No, I don’t want that.” But then they came out with Flolan and I went and had the trial. And immediately [when I went on Flolan] I was off oxygen. So, I think trials are great for everyone to try out, and research, I don’t think they should stop research. More money should be invested into it, to see if we can’t cure it, at least put it in recession. Get it to stop growing. Being on 40 years of surviving and watching people, I feel amazed, I feel gifted to see it. I really do. Just to be here, to see people that I can help, that I can tell them, “You can do this, you can get through this. It don’t define who you are.” We all have to live through our tests and trials. I tell people, “I have PH, PH don’t have me.” I’m going to live my life. And I like when they say, “You lived 40 years, who do you do it?” I say, “One step at a time, with my faith. And you have to have a positive attitude, because if you sit depressed, you’re going to be depressed. If you be happy, you can make it through it.” And to see other people doing what they’re doing, living what they’re doing. I tell them do what your body says you can do. I rode a bike, I was crying on that bike ride and people thought I was going crazy. But every step. Do what you can do, what your body says you can do. But always take care of it. I think when you’re first diagnosed, make sure you have a great team. And that’s including your doctor, your specialist, your nurse, your caregiver. I love the movie The Lion King, because it tells you about the circle of life. And all these events go with this circle. And you end up right back where you started from at the end, and that’s how I look at it with PH. It’s a circle of life that we have to start with our doctors. But then eventually it comes down to us, that we take over and start taking care of us. But we keep that circle going, and I think that with a new person, listen to your doctor. Listen to your body. Hang around positive people, people that encourage you, not people to put you down. And I think that will help you be able to handle this disease and live with it. My name is Gwendolyn R. Brown, and I’m aware that I am 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: Judith Moatti (224)

PHA Canada ambassador Judith Moatti discusses the importance of early diagnosis and awareness. Judith was first diagnosed with Pulmonary Arterial Hypertension (PAH) in 2009, at the young age of 25. After a long road to stability and coping with her new reality, she has become a strong advocate and spokesperson for those affected by PAH. Locally, she became a patient representative for Foundation HTAPQ (a Québec-based patient support organization) in 2012. And she also works as a support group leader for patients living or affected by PH in her region. Hi, my name is Judith Moatti, and I’m a pulmonary hypertension patient. When I first got diagnosed, I got my diagnosis by fluke. My gallbladder got swelled up, and I went to [the ER], because it was hurting a lot and I could barely walk anymore. When I got to emergency, they thought I had stones, but they couldn’t find anything. They wanted to remove my gallbladder. In the process, my mom refused to let them take me for surgery because she thought it wasn’t reasonable to take out my gallbladder if we didn’t know why it was swelled up. I had to go through a bunch of autoimmune disease tests, and [my doctor] realized that I didn’t have any autoimmune disease. They were going to send me home. So, I’m waiting for the doctor with the release form. As I was waiting in her office, I saw a poster on the wall that described pulmonary hypertension and the symptoms. My friend who was with me told her to test me for that because I had been short of breath when I was walking. We started with the chest x-ray, and she said my lung arteries looked a little small, but that it might not mean anything. Then we went for a heart ultrasound. That’s when my life changed for the worst in a way. I started with one technician in radiology. I ended up with 15 doctors and residents in the room because I was in heart failure. That’s when they announced to me that I had pulmonary hypertension. They transferred me to the cardiologist specialist at the Montreal Jewish Hospital and as soon as I met with him, he explained to me that I was really close to death, and that I had to quit everything I was doing. I had to quit my job. I couldn’t have children anymore. We started on IV therapy right away because there was nothing else that was working in bringing my pressures down. It was a difficult therapy, of course. After a while, I had to be switched therapies because the site pain never left in my case. After a few years of switching therapies and going on research protocols, I was put on all oral therapies. I tried to go back to work. My pressure was going back up, so my doctor suggested that I completely stop working. If not, I was going to die young. When he stopped me from working, I was wondering what can I do to help other people? So I started to host support groups. Then, there’s also a foundation in Quebec, HTAPQ, and we help patients in our province. They asked me to be part of the board, and so I became a board member. Through all these activities, I met PHA Canada, because as soon as we are diagnosed and we become a member of the other foundation, we become a member with PHA Canada. I’ve always wanted to be involved with them, but I had to take it slow because I didn’t know everything, and I didn’t know how I was going to help people. Eventually, I started going to their conferences, which are really good conferences. I met a lot of different people. I realized I wasn’t alone and that I could help others like me. Not long ago, I saw that there was a post on Facebook where there was an opening for an ambassador role, and right away I applied, went through an interview, and was chosen to actually be a part of that. I’m really glad I am because I can help more people through PHA Canada because it’s on a national level. I want to be an ambassador because I want to help other people know about pulmonary hypertension. I think not a lot of people know what it is and how it affects us on a daily basis. I think it’s important to help others as well. When I got my diagnosis, I felt very alone. When I started following PHA Canada, I realized that there was a lot of other people like me in Canada that were having pulmonary hypertension, that had the same issues as me. I definitely wanted to help them because I went through a hard, hard time, and I know how I got out of it. So I know how to help others and share my story with other so that they can understand that they’re not alone either. We’re trying to make people aware and doctors especially, like emergency doctors, general practitioners, we’re trying to teach them about pulmonary hypertension because oftentimes, people go to their family doctor and they’re misdiagnosed. In my case, it was the same thing. I went to my family doctor who told me it was normal that I would faint once in a while. Going through this experience, I knew that I wanted to help others not go through the same, that it’s really important to get an early diagnosis because people can potentially keep working and have somewhat of a normal life even with pulmonary hypertension. So it’s really important for me to try and reach those people because they need to understand that someone [who presents with] shortness of breath, it doesn’t necessarily mean they have asthma or that they’re not training enough. It’s really important to teach them as well that there’s something bigger that you might want to check out before you send someone home and tell them that they’re normal and everything is fine. There’s so much that changed throughout the last years. I’ve had my diagnosis for eight years now, and in the little bit of eight years that I’ve been sick, I’ve seen a lot of changes. There’s been a lot of new therapies, a lot more support groups, a lot more people are sharing their stories now, which I find is wonderful because this is how we’re going to get the word across. My name is Judith Moatti, and I’m aware that I am 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 #LifeInPurple #PHUnited #ClinicalTrials @antidote_me @PHACanada www.sometimesitsph.ca

I’m Aware That I’m Rare: Diane Dauwalder (228)

In this episode, pulmonary hypertension patient Diane Dauwalder discusses how she became symptomatic in 2002, preparing for the end of her life, and how thanks to PH research and new therapies how she adjusted to a new normal. Hi, my name’s Diane Dauwalder. I’m a pulmonary hypertension patient, and I’m from Fort Worth, Texas. I’m the Fort Worth support group leader. I was confirmed diagnosed in February of 2005, but I started to become symptomatic around 2002 when I moved from Arizona to Colorado. Everybody had thought it was just the altitude and that I needed to get used to it, but all of a sudden I couldn’t take stairs two at a time. I used to be a courier for Federal Express, so I was used to a fast-paced lifestyle. All of a sudden, I was getting short of breath. I knew something was wrong. So we did move from Colorado to Fort Worth Texas and I was still having problems. I’m no longer at mile high and was still having difficulty breathing. Nobody knew what was wrong with me until I ended up in the ER. Severe sleep apnea was the first diagnosis. Then finally I was able to get to UT Southwestern, who did the right heart catheterization and confirmed my pulmonary hypertension. It was good to know what I was dealing with, because I knew that something was wrong with me but I had no idea what. At that time, and we’re talking over 10 years ago, if you went online you had three to five years to live. It was very scary. There was only two medications out. I was getting prepared for the end of my life. At that time, I had a long talk with myself. I wasn’t working obviously. At first, I was in denial, which I think we all go through. I thought they were going to give me a pill and make me better. Well, after the first six months of being on a pill and knowing I wasn’t getting better, I was like, okay, time to come to terms with the Lord and make our peace here, because I’ve had a good life. I was ready. Then fortunately, through research, through fundraising, more drugs came out and it kept me going. I was getting better. So I wasn’t going to be in a scooter and on oxygen 24/7 anymore. I started to get better. Not much better, but I got adjusted to my new norm. I heard about support group meetings. I didn’t know anything about pulmonary hypertension. I did go to a support group meeting, and I went to a couple of them and then I walked out because I got too depressed. People who I saw walk in, the next meeting they were in wheelchairs. I just couldn’t deal with it. I guess I was still in a little bit of denial. Again, there was a third drug added to my regimen, and I was very faithful because I was on inhaled prostacyclin, which I had to do nine treatments a day every two hours, and I did it because I did not want IV therapy. From there, it was just all uphill for me. Then I ended up becoming the support group leader. It was good. Every time that phone rings, I’m thankful that I’m doing what I’m doing, because that other person on the phone is so alone and so scared, and it’s not that bad. You can have a normal life. Life can be good. You can enjoy it. When they came out with the (Sometimes It’s PH) zebra campaign to raise awareness, which I thought was great, because it gave us an icon to use, a zebra. We are medical zebras. I got us on Good Morning Texas for our Zebra Fest, because I don’t think everybody’s heard of Zebra Fest that we have in North Texas. I think the zebra was a good way to raise awareness, because it made people look and listen. My primary care physician for instance, every time I went to him he would write COPD down. I kept telling him I don’t have COPD. It’s PH. Even though the symptoms are the same. So finally, I started bringing him in different materials to hang on his wall, saying sometimes it’s the other blood pressure. It’s been good, because I think we’re finally getting patients diagnosed sooner than later, which is really good because I think more people are aware of the illness. I think there’s a lot more people who have pulmonary hypertension that were diagnosed incorrectly or were never really diagnosed, because nobody knew what it was. If I met a newly diagnosed patient, I would give them a hug first of all, tell them that they’re not alone, that it’s a journey, but it’s a good journey, and just to get used to your new normal and life goes on. It can be good. I’m Diane Dauwalder and I am aware that 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