Living with Eosinophilic Granulomatosis with Polyangiitis (EGPA): Podcast of a Patient-Physician Discussion

00:00:00: You are listening to an ADIS Journal podcast.

00:00:05: Hello, everyone.

00:00:06: I'm Dr.

00:00:06: Michael Wexler, Professor of Medicine and Director of the National Jewish Coen Family Asthma Institute in Denver, Colorado.

00:00:14: Today, I'm excited to participate in a podcast in which we will discuss eosinophilic granulovatosis with polyangiotis.

00:00:23: And I'm really excited because today we're going to be having a patient physician discussion.

00:00:29: And I am excited to have with me Autumn Smith, who will be discussing her experiences in terms of living with EGPA.

00:00:40: Autumn, do you want to introduce yourself?

00:00:42: My name is Autumn Smith.

00:00:44: I was diagnosed with eosinophilic granulomatosis with polyangitis in two thousand fourteen and have been managing that journey ever since.

00:00:54: Well, it's great to have you.

00:00:56: I think this is an important discussion.

00:00:58: I think before we get started with chatting with one another, we should talk a little bit about what is eGPA?

00:01:05: So eGPA is eosinophilic granulomatosis with polyangiitis.

00:01:10: It's a vasculitis that involves both the lungs as well as other organ systems.

00:01:17: It's associated with asthma, eosinophilia, pulmonary infiltrates, neuropathy, vasculitis in one or more end organ, sinus disease, and oftentimes it's associated with anti-neutrophil cytoplasmic antibodies.

00:01:35: It was first described by two pathologists in the nineteen fifties named Jacob Jerg and Lottie Strauss who were doing autopsy series on patients who died from asthma and noted that they had what they called vascular embarrassment on top of their asthma and eosinophilia.

00:01:53: And hence, for a number of years it was called Church Stress Syndrome.

00:01:57: Today we're going to refer to this just as EGPA.

00:02:00: And EGPA is really a disease that is manifest by high levels of blood eosinophils, a type of white blood cell that can cause some damage.

00:02:11: So now that we've gotten

00:02:12: that out of the

00:02:13: way, I want to ask Autumn a few questions.

00:02:15: First of all, Autumn, can you talk a little bit about your journey?

00:02:19: When were you diagnosed?

00:02:21: How were you diagnosed?

00:02:22: And what sort of symptoms did you have?

00:02:25: Absolutely.

00:02:26: I was diagnosed

00:02:27: in February of two thousand fourteen, but my symptoms all actually started in September or October of twenty thirteen.

00:02:36: It started with chest and shoulder pain and then I started seeing spots in my vision.

00:02:44: It progressed to include a lot of swelling in my stomach and ankles and then a ton of fatigue, a gait in my step and just all over exhaustion.

00:02:59: And when this all happened, I was a really fit person.

00:03:04: I worked out five or six days a week.

00:03:06: I ran all the time.

00:03:08: I was really strong despite only being four foot ten.

00:03:12: And by the time it ended, I was really just completely withered away.

00:03:20: Wow, that sounds really, really challenging.

00:03:23: So tell me a little bit more about what happened.

00:03:27: trip to the emergency room and how you were initially treated and a little bit more about your journey in the acute setting.

00:03:38: I started out, I just went to my PCP.

00:03:42: I had just met her once at my established care visit and then showed up again with all these disconnected symptoms.

00:03:51: And she was really concerned, but she didn't actually have any idea what was going on.

00:03:58: She said, if I saw spots again in my vision, just to go to the ER.

00:04:03: So I went to the ER.

00:04:06: I was there for a while, but they told me I had muscle pain and I left there with a prescription for pain.

00:04:17: And a few weeks later, I just progressively started getting worse, getting more fatigued.

00:04:24: It was getting really difficult to work.

00:04:27: I went back to the PCP and she did some more tests and it came back positive for H. pylori.

00:04:36: So she just started treating me for that, but my symptoms just kept getting worse and worse.

00:04:43: And so I went to the ER again and they told me I had acid reflux and I left there with prescription for that and some tramadol.

00:04:55: So we went to face giving with my in-laws and I really just wasn't functioning at all.

00:05:04: Splinters appeared in my fingernails and I just slept basically the entire time.

00:05:10: I barely ate anything, but by the time I was trying to get ready to get on the plane to go home, I was so swollen that I couldn't actually butt in my pants.

00:05:22: My partner got me home somehow and the next day my PCP had set up a CT scan of my chest with contrast.

00:05:34: and so that's when they saw that my heart was enlarged.

00:05:39: and so I saw a cardiologist the next day and he admitted me to the hospital immediately.

00:05:46: and it was just kind of a round robin.

00:05:49: of doctors in and out of my room ordering various tests.

00:05:54: I think I was tested for everything from infectious disease to leukemia to various cardiothoracic tests and they managed to get me stable but I never ended up with a diagnosis.

00:06:13: They just were treating me for all the various symptoms with various IV medications and prednisone and diuretics.

00:06:22: Did they

00:06:23: recognize the high levels of blood eosinophils at the time?

00:06:28: Nobody actually said anything about that to me.

00:06:31: I'm sure that that was part of my chart.

00:06:35: What was interesting was that a retina specialist came in to look at my eyes And he ran in with all this equipment and he looked at me and he said, I think you have chirp straws.

00:06:48: Then he ran out of the room and we didn't know what to make of that because I didn't even know how to spell it.

00:06:55: So it wasn't until I think two months later, I flew to Cleveland Clinic and the doctor there gave me an official diagnosis.

00:07:06: Wow.

00:07:07: So this journey that you went through is really not very atypical.

00:07:12: This is a relatively rare disease, EGPA.

00:07:15: And it often goes under recognized and it presents with so many different symptoms.

00:07:22: It's hard for people, for physicians to put the pieces of the puzzle together.

00:07:27: And that's the big challenge.

00:07:29: And that can lead to delays in diagnosis.

00:07:32: It can lead to delays in treatment.

00:07:35: It can lead to worsening of the underlying disease.

00:07:38: So it's so important to understand.

00:07:41: all of the key features of the disease.

00:07:43: The presentation with eosinophilia is probably the key thing that people need to focus in on, but the eosinophils can affect any organ as they did with you, the GI tract, the blood vessels, and the splinter hemorrhages.

00:07:59: All of the revolving door of doctors, I think, is not atypical at all.

00:08:05: You need someone who has some experience who's heard of eGPA and who can put all the pieces of the puzzle together.

00:08:14: It's so important to think about all the different disease components and not just blow them off, so to speak, but to appreciate all the variegated symptoms that an individual can have and try to tie all those things together.

00:08:30: What sort of features, what disease components can be tied together?

00:08:36: So can you talk a little bit about usually EGPA associated with asthma?

00:08:40: Can you talk a little bit about your history of asthma?

00:08:44: Yeah, I had asthma as a kid.

00:08:46: that was actually quite acute.

00:08:48: I would go from fine to basically blue within a few minutes.

00:08:53: So I was constantly life-lighted from my local hospital to a bigger children's hospital in the city.

00:09:01: I think I was life-lighted fourteen different times and constantly in and out of the hospital, seeing different specialists.

00:09:10: but all of that stopped.

00:09:12: I had my last attack at twelve.

00:09:15: I don't know what changed but I never had another major asthma attack after that.

00:09:20: that required hospitalization and we were just managing it with steroids and various other medications.

00:09:29: For the most part as an adult my asthma was controlled.

00:09:34: So it's interesting because if one of the doctors didn't take that part of the history that may not have clued them in that you could have an eosinophilic disorder or an asthma related disorder like EGPA because EGPA is often associated with asthma.

00:09:48: You know, it's important to understand the whole typical patient journey and that usually involves presentation in one's forties and taking a good history.

00:10:00: One finds that there can be a history of.

00:10:03: asthma, sinus disease, and then when one sees the eosinophilia, then one can go in and start probing about other organ systems that are involved.

00:10:15: It's challenging because every patient journey is unique and there are gonna be some patients who are gonna be diagnosed quickly, but then there are gonna be others like you in whom it can be very challenging and it can take some time to get a diagnosis.

00:10:29: That's why it's so important.

00:10:32: for physicians, clinicians to be aware of this disease, the common symptoms, the unusual symptoms, and the different patient journeys so we can help patients avoid the long and complicated diagnostic experience that you experienced.

00:10:48: Tell us a little bit about how EGPA has affected your life.

00:10:53: What symptoms do you have on a chronic basis?

00:10:56: And what are some of the emotional challenges you've had in terms of living with EGPA on a daily basis?

00:11:03: And how has that affected your work life and your social life?

00:11:07: Absolutely.

00:11:08: When I was first out of the hospital, I was so exhausted and withered away on so many medications.

00:11:16: The hospital basically just handed me a box of medications and discharged me because they said I was stable.

00:11:23: I didn't know if I was going to die because in the hospital, they handed me a folder about heart failure and I didn't understand exactly what that meant.

00:11:35: There was a lot of fear when I first left the hospital for both me and my partner.

00:11:43: We didn't know what was going to happen.

00:11:46: We didn't have someone that we could ask questions to.

00:11:51: My friends didn't understand.

00:11:52: They didn't know fully what happened to me.

00:11:56: And I didn't really want to tell anybody.

00:11:58: So I felt really alone because I didn't have any answers.

00:12:04: I just didn't know how to communicate that to my friends and my family.

00:12:10: It was really difficult just trying to maintain a day-to-day rhythm, continue to work with all of the different side effects and the exhaustion.

00:12:21: Luckily had a very flexible boss when this happened.

00:12:26: And so he was able to really work with me to make sure that I could fit in what I needed to do, but then still be able to rest.

00:12:35: I just had so little energy all of the time that I would do a little bit of work, go back to bed, and then I'd get up.

00:12:46: My friends were wondering what happened and my partner had actually said, I need help.

00:12:53: So we started telling more people and then everybody really rallied around to bring us meals.

00:13:00: We also found out a year later that I was going to need open heart surgery to repair all the damage that happened during that experience.

00:13:11: We really had a good group of people that came together to help keep us together.

00:13:16: And in terms of treatment, I was on prednisone, quite high doses for a long time.

00:13:23: When I finally went to Cleveland Clinic, the doctor there had mentioned that they probably would have put me on cyclophosphamide, but I had really bounced back so much that they decided to use azothiopram instead.

00:13:41: And so I was on doses of that for quite a while, probably almost five years.

00:13:48: Wow.

00:13:48: Well, you described so many sentiments that my patients describe.

00:13:54: It can be very, very scary and you need the social support system that sounds like you had.

00:14:01: It's so important to have supportive friends.

00:14:05: have supportive family and also have a supportive work environment where they recognize that you're going through a difficult time and you need some of that time in order to seek out the medical care that you need.

00:14:19: The other challenges that a lot of patients talk about are just the challenges in terms of understanding their physicians, getting appointments with physicians, getting appointments with appropriate physicians, and then there are all the side effects of medication.

00:14:35: patients, the steroids that can have a huge, huge impact.

00:14:39: I would say that's the number one thing that my patients talk about is, how can I get off oral corticosteroids?

00:14:46: They cause so much irritability, they cause weight gain, they impact my quality of life and make it difficult to sleep.

00:14:54: And so those are some of the big, big challenges.

00:14:58: And we've seen time and again, how EGPA affects quality of life.

00:15:03: other outcomes that are important to patients, people talk about having little energy, feeling

00:15:11: blah.

00:15:12: And that's something very common that I hear from my patients with asthma and EGPA in particular and anybody who's on oral corticosteroids.

00:15:23: These can be challenging to manage, challenging for the patients, challenging for the physicians in terms of dealing with all the side effects of the medications.

00:15:34: And so it's important for there to be good.

00:15:37: resources for patients from the physicians to the patient's family to work environment and having good friends.

00:15:46: All of these different domains, the physical domains, psychological domains, the social domains and the financial domains can really help one get through this and enjoy a reasonable quality of life.

00:15:58: But you need to have this good support system to get through this psychologically because so many patients experience a level of anxiety due to having this underlying disease.

00:16:09: So your experience aligns very much with many of my patients' experiences.

00:16:14: Can you talk a little bit about your initial treatments and medications that you got after diagnosis and what your experience was with some of those treatments?

00:16:25: Talk about the prednisone and how it affected you and the other medications that you were on.

00:16:31: Prednisone is... such a bittersweet drug, right?

00:16:35: It solves so many problems, but it also causes so many.

00:16:40: I definitely experienced the weight gain, the puffiness, horrible, horrible mood swings.

00:16:47: Just so many times I felt like I was having an out-of-body experience where I would yell at my partner for no reason, but I couldn't stop it, and then I would just start to cry.

00:17:00: and it impacts everything.

00:17:03: I'm so grateful that he is such a patient person, but it really can make life very difficult.

00:17:10: And on top of the prednisone, I was on azathiopran.

00:17:14: I didn't experience any side effects from that.

00:17:17: I know that's not always a typical drug that's used in the treatment of EGPA, but it did manage to do the job for me.

00:17:27: I was also at that time on blood thinners, a diuretic, beta blockers, all these things that I was put on in the hospital and it took a long time to determine if I could come off them.

00:17:41: So that was a long process as well.

00:17:45: I think it took me two years to fully come off the prednisone.

00:17:50: I actually did have one relapse.

00:17:53: a few years later and that's when we switched me over to the mepalizum ab.

00:18:01: Things have evolved certainly in the management of eGPA since you were first diagnosed and a lot of times many of the medications that people used were medications that were approved for other vascularities for GPA or for MPA.

00:18:18: People would use drugs like azethioprine including other immunosuppressants such as cyclophosphamide or Rituximab, and they had modest efficacy, but it really wasn't until the anti-IL-Vs came about that we began to see greater efficacy and fewer side effects.

00:18:40: Pretend to zone them itself is associated with significant side effects.

00:18:44: The longer you use steroids, the more weight gain you have, the greater the risk of osteoporosis, cataracts, glaucoma, increased risk of infections.

00:18:55: One of the major goals is to get people off steroids.

00:18:59: And you're fortunate to get off within a couple of years because some patients can't get off ever.

00:19:05: And that can be very, very challenging in terms of managing all of the side effects.

00:19:10: So maybe we can discuss a little bit about your experience with anti IL-V therapies, including mepalizumab.

00:19:20: And tell us a little bit how you felt any side effects, how it's worked.

00:19:26: Talk a little bit about your experience with the more recent medications.

00:19:32: So far, mepalizumab has been really working well for me.

00:19:37: It doesn't control my asthma quite as well.

00:19:41: I do have moments where I wake up wheezing, but in terms of EGPA, my eosinophils are still zero and everything has been pretty stable for the last several years.

00:19:56: I took it every four weeks.

00:19:59: for four years and I have just recently switched to starting to extend it to every six weeks and that has still been working pretty well.

00:20:11: What about the cost of medication?

00:20:13: Is that a challenge for you?

00:20:16: Do you have any insurance related issues?

00:20:19: In America there are always insurance related issues.

00:20:23: It is definitely a lot of work.

00:20:26: I joke sometimes with friends that when you get diagnosed with a disease, you should also get an assistant to help you manage it because it's a lot of phone calls, a lot of fighting people, a lot of filling out of paperwork.

00:20:40: I'm lucky in that I have had insurance through this.

00:20:44: I also spoke with everyone and filled out all the necessary information to get relevant copay cards for each of these medications.

00:20:54: that I've been on throughout this journey.

00:20:56: So that has made it accessible for me.

00:21:00: I do have to call every month and get my refill and have it shipped here in a little cooler.

00:21:08: And so that always takes a little bit of collaboration and planning so that you make sure you're here.

00:21:16: You can get the medication, put it right in the fridge.

00:21:20: Thus far, I have been very lucky with all of that.

00:21:24: Every country has its own issues and some places it's harder to access these types of therapies and other places.

00:21:31: You have to go through some hurdles, but our goal is physicians try to get the right drugs, the right patients and hope that the health care systems will provide.

00:21:41: One other quick question about your experience with mepalizumab, any major side effects, do you tolerate it well?

00:21:48: I have been tolerating it quite well.

00:21:50: I don't have any side effects that I have been specifically able to attribute to that melissa map.

00:21:57: So I'm lucky in that regard that it's been managing everything well.

00:22:04: A lot of patients with eGPA suffer from relapses or flares where their asthma gets worse or their sinuses get worse or they get pulmonary infiltrates or they get a new rash.

00:22:19: Have you experienced any of those while you've been on mepalizumab?

00:22:23: I have always had sinus and ear issues.

00:22:27: that has been consistent my whole life.

00:22:30: I had multiple surgeries on my ears and nose when I was a child as well.

00:22:36: And so my allergies do tend to ebb and flow depending on the season and they will get bad.

00:22:45: And whether it's EGPA or plain allergies, I'm never quite sure, but it hasn't been bad enough at any point to go back on prednisone or any other medications.

00:23:01: It's so important to give the right drug to the right patients and you've been fortunate that you got the anti-IL-V therapy.

00:23:09: Some patients do require other immunosuppressive therapies because they can't get the disease fully under control.

00:23:17: Some patients require drugs like azothioprine or tuximab, particularly if they have anti-neutrophilicidoplasmic or ANCA antibodies.

00:23:27: And sometimes both the antial five and the other immunosuppressants can help facilitate steroid withdrawal, but the real key is to try to avoid some of the side.

00:23:38: effects.

00:23:39: I think that's where the antial fives come in because they don't have the same risk of infection and the same depletion of other cells that can cause other issues.

00:23:52: One of the other things that you mentioned is your response to mepalizumab.

00:23:58: It's important to recognize how these anti-ial fives therapy is both meplizumab and benrolizumab, which is also now approved for eGPA, have revolutionized the management of this disease.

00:24:12: The way these drugs work is they bind up interleukin-V, or they bind the receptor for interleukin-V.

00:24:19: Interleukin-V is a key cytokine that's involved in the maturation, proliferation, and activation of eosinophils.

00:24:27: Since eosinophils are involved in causing eGPA, when you block interleukin-V, you block eosinophils, and that can have a significant impact on eGPA.

00:24:38: Both of the drugs that I mentioned, meplizumab and benrolizumab, are very effective in terms of reducing or depleting eosinophils, and that can help facilitate corticosteroid tapering and even get many patients into remission.

00:24:53: And when we talk about remission eGPA, we're talking about having no significant symptoms and being on four milligrams or less of prednisone, our real goal is to get people off prednisone completely.

00:25:05: And we know from the two key pivotal studies, the Merit trial that compared to placebo and the Mandar trial in which MEPLISMAP and BENDLISMAP were compared to one another, we can achieve great effects.

00:25:20: Almost eight out of ten or nine out of ten patients achieve some degree of benefit in terms of management of their eGPA, whether it's steroid reduction, whether it's cutting the number of relapses in half or getting patients into that broader definition of remission that we talked about.

00:25:41: And that's why these therapies are included in the more recent EGPA guidelines that have been set forth from the American College of Rheumatology and the European League Against Rheumatism and the Vasculitis Foundation.

00:25:56: All of these guidelines have included mepalizumab as first line treatment for non-severe.

00:26:02: EGPA.

00:26:04: We don't have Benrolizumab yet because the guidelines haven't been updated yet, but I'm sure the next iteration of guidelines will include that as well.

00:26:13: The only issue is that we don't have good trial data in terms of the acute management of EGPA and for patients with severe disease because those patients were not included in any of the trials that we've done to date.

00:26:28: It'll be interesting to see whether we can postulate based on the efficacy of these drugs in terms of more chronic management of EGPA, whether they can be utilized in more severe disease and whether they'll be included in future guidelines in that regard.

00:26:44: Generally, these therapies are very well tolerated, which is important.

00:26:49: Generally, they allow steroid tapering.

00:26:52: We want a drug that's both effective and safe.

00:26:56: And while most patients don't discontinue these drugs based on toxicity, you can see some common adverse reactions, including injection site reactions, localized pain, occasionally a rash can occur.

00:27:12: And that's what's been reported in the medical literature.

00:27:15: Well, this has been a fascinating discussion.

00:27:18: Autumn, I want to thank you for really participating and sharing with us and the listeners how your experience with eGPA has been, all the challenges that you've had in terms of living with eGPA.

00:27:33: And I just want to ask you, do you have any advice or words of encouragement that you can share for anybody that's been diagnosed with eGPA?

00:27:44: Absolutely.

00:27:45: First off, hang in there and just try to get through each day and don't think about the long-term effects.

00:27:53: Health is a journey.

00:27:55: It's never going to have an end.

00:27:57: It's always going to be a process and you have to keep working at it a little bit every day.

00:28:04: And also for people that are in the United States, I would encourage you to watch videos about how insurance works.

00:28:12: or do some research because you certainly will get charged for things that you shouldn't and you always have the ability to appeal.

00:28:21: The other thing is, like we talked about earlier, really leaning into your community.

00:28:28: Once I learned how to accept help, I realized how pivotal that was in my recovery and just having someone to talk to and to be open with.

00:28:40: And just to remind yourself that there is always beauty around.

00:28:44: You can look around and see a bird or a flower and try to bring yourself back into this present moment and really focus on what's happening right now and not all the could happens that can send you down a negative spiral really quickly.

00:29:06: So just Stay in the moment and keep moving forward.

00:29:10: Thank you, Autumn, for sharing your story and your experiences and some of those important life lessons.

00:29:17: The only one all that is.

00:29:19: you

00:29:20: have to seek out care and try to continue to find physicians who have experienced with rare diseases like this.

00:29:29: And it's so important to be a self-advocate as well.

00:29:32: but thank you so much for participating and I just want to summarize some of the key points.

00:29:39: It's so important to recognize eGPA to put the pieces of the puzzle together and really the goals of management in eGPA.

00:29:47: First of all, to recognize the disease, to get the disease under control when it first presents, to try to prevent the complications of the disease, but also to prevent complications of potential therapies, particularly the steroids that have so many adverse events that can affect an individual with EGPA and anybody who takes systemic corticosteroids.

00:30:11: And then our major goals as physicians are to prevent relapses and to get patients down to a lowest dose of steroids as possible and even get off of steroids as possible.

00:30:24: You have to have a good community.

00:30:26: You have to have good support from physicians, from friends, from family, from your work, and you need to seek out optimal care.

00:30:37: So I want to thank you, the listeners, for listening and taking in Autumn's story.

00:30:43: It's an important one.

00:30:44: I think it can inform us about not just eGPA, but also many other rare diseases where we really need to work together.

00:30:54: patient, provider, and a whole host of providers in different specialties need to come together to help put the pieces of the puzzle together.

00:31:04: So thank you so much for joining us today and thank you, Autumn, for sharing your story.

00:31:09: Thank

00:31:10: you for having me.

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