non: [00:00:00] Welcome to the a TS Breathe Easy, the latest podcast. Here's your host, Patt.
Patti: Hello and welcome. We have an extraordinary guest today who will discuss idiopathic pulmonary fibrosis and her presentation at a TS 2025 International Conference. Dr. Mary Lu Puentes is not only a physician, but she's also a double lung transplant survivor. More from her in a bit, but first, here are some other latest headlines.
The measles outbreak continues. Experts warn that if you can't remember, if you're vaccinated. See your doctor and get the shot. And for more information on the outbreak, check out our February 24th Breathe Easy podcast with Dr. Paul Offit and see the [00:01:00] new patient fact sheet on measles@thoracic.org. Good news for those.
Who rely on telemedicine. The pandemic related telehealth flexibilities have been extended for several public health, Medicare and Medicaid authorities and programs, and the social media influencers are added. Again, sleep maxing is the latest wellness trend. And it's about getting the maximum benefit from sleep these days.
A good night's sleep about seven to nine hours, and that might seem as elusive. Social media isn't the answer for sleep tips based in science. Check out our show notes or links to the a TS patient sleep resources. Including questionnaires, we'll be right back.
non: The A TS 2025 [00:02:00] International Conference returns to San Francisco, May 16th to the 21st. Members get a discount on conference registration, so become an a TS member. Or renew your membership to take advantage of these savings at a TS 2025. You can network with colleagues. Find your next research collaborator.
Listen to patient stories and get inspired and be among the first to learn about breaking news in the pulmonary medicine. Register now to attend. Go to conference.thoracic.org today
Patti: and welcome back Dr. Mary Lewis. It's very nice to have you here. Uh, you look wonderful, by the way. And, uh, you were diagnosed, uh, with idiopathic pulmonary fibrosis and in your th forties, which is, uh, very young.
What is the difference between pulmonary fibrosis, idiopathic pulmonary fibro?
Dr Maryluz: Well, um, thank you Patty for having me here. I'm, I'm excited to be [00:03:00] here. And, um, uh, the pulmonary fibrosis is a broad term. The idiopathic pulmonary fibrosis is one, is the most common, uh, of the diseases, the lung diseases of the intertia lung disease.
Uh, there are about 123 plus, uh, diseases that are under this big umbrella. And, um, and idiopathic pulmonary fibrosis is one of them the most common.
Patti: Yes. What kind of symptoms were you having, uh, when you thought that you might have, uh, idiopathic pulmonary fibrosis? Were there some symptoms or were you misdiagnosed your physician?
Did you know what was going on?
Dr Maryluz: Well, I was 47. I was, uh, um, totally, um, otherwise healthy, uh, young female fully working in my late forties. And then, uh, I started having, uh, an insidious, uh, dry cough. Um, that didn't seem to go away. Never in my wildest dream. I thought that I was gonna have this kind of, uh, uh.[00:04:00]
Serious disease that I was diagnosed later on. Uh, the common symptoms of, uh, not so common because actually the overlap with another pulmonary diseases. Um, the common for the symptoms for um, pulmonary fibrosis or idiopathic pulmonary fibrosis are cough. I. Uh, it's a dry cough, uh, fatigue, shortness of breath, uh, weight loss.
And as you can see, those symptoms, um, can be, uh, found in another, um, lung diseases, for instance, as a physician, I. When I, um, had the cough, which was the symptom that I presented that was a dry cough, I thought in my mind that I had developed, uh, exercise, uh, induced asthma. I was convinced that that's what it was.
You know, when patients used to tell me, uh, patients that follow me every three months and have seen me told me, uh, dog, you need a dog. Uh, my, uh, my husband, uh, was the one that insisted that I see someone, so I went ahead and saw [00:05:00] a, um, an allergist because I was convinced that that's what I had developed.
In fact, I used to bike with a, a friend of mine and I used to sneak some bronchodilators out of the, the closet, the medicine closet in my practice and premedicate myself before the bike rides because I was con, I was convinced that that's what I. I had, um, but it didn't help. So after three months, I went to see a, um, an aist who gave me, uh, a spirometry and told me that I did not have, uh, exercise induced asthma, that I had chronic bronchitis, and he gave me 30 days of antibiotic at that time.
Um, as you can see, I mean, um, I continue having the cough and the shortness of breath and, uh, by late, um. Winter or early winter. The following, um, year, I was having very much short of breath and uh, I, at that time we had a 10-year-old daughter and a two story house, and I went to the second floor to talk her into bed and I have to sit on her bed [00:06:00] because I was panting.
I was very short of breath. And so I thought at that time that I had either, um, something wrong was with my heart or I was. Totally decondition, unconditional. I was, uh, totally outta shape. So I went and in my case, I mean, uh, I, of course I was familiar with, uh, the medical field. So I went and I scheduled an appointment, uh, a visit with a cardiologist who put me with, uh, um, uh, the battery of all these tests and told me that my heart was strong and I, uh, that I must see a pulmonologist and how that's how.
Um, like seven months later I went to see and, and the whole, since the, the symptoms presented, I went to see a pulmonologist who was the one, the first one who actually did a pulmonary function test and did a chest x-ray and told me that there were some infiltrates and he was, you know, looking at something else more serious, was going on.
I
Patti: out. Did you have a biopsy done?
Dr Maryluz: Yeah, as a matter of fact, since I was so young, I was 47. Um, and, and [00:07:00] in those days, um, when he gave me the, the pulmonary function test and the whole battery, I got a bronchoscopy and, uh, a lot of tests to ruling and rule out infectious diseases, connective tissue disorders, um, and then, uh, nothing was conclusive and.
They thought at that time that along the way I would develop a connective tissue disorder, but to these days, nothing had shown. So my diagnosis still was idiopathic for my fibrosis. I, I underwent, uh, a open lung biopsy because, um, it was inconclusive. I mean, they couldn't put me anywhere. And the pathology, uh, was read as a UIP, which is the, the, um.
The pathology for idiopathic ity fibrosis and, uh,
Patti: yes. You know, my, my mother too, around 2001. Uh, and she was 51. Um, and that's what, uh, was her diagnosis. And she went through [00:08:00] a bi biopsy, but she was also, uh, you know, people didn't know what, what she had. Um, so at, at, uh, at what point you were functioning well, fairly well.
Um. Were you taking oxygen, prednisone?
Dr Maryluz: Uh, well those days, and now we're talking about, uh, 2007, 2008, there were not any, any therapies for idiopathic pulmonary fibrosis. I was not put on, um, prednisone. Um, I took, uh, a drug called Imuran. Uh, on those days, um, we didn't have anything else. Uh, my disease.
Progress very slowly. Um, it took about 10 years, not until 2017 that I require, um, started feeling more shortness of breath and I'm becoming more desaturated and I needed, uh, oxygen. So I was started on a, a portable oxygen concentrator. Um, and I. [00:09:00] Use it. And, uh, it was mostly, uh, with exertion. So every time that I did some activity, I desaturated it became more short of breath.
Um, and, um, few years passed back, uh, and I continued to buy and I continued to work. Uh, it was far for me, but I accommodated. I I used to take the three flight of stairs to go to my office and I couldn't anymore. So I took the elevator. I hate it 'cause I saw, you know, so we brought it patients in using, uh, and so, but I, uh, but I in
Patti: at.
Were you in a denial?
Dr Maryluz: Not, not in a denial, but I, my pulmonologist always mention, uh, lung transplant, so, and that, those LT words was, so, I feared those two a lot. You know, it was just, uh, so I knew what I had and I, I, but I, but I continued to cough and there was before the stigma of the, the covid. And I remember that, um, I endure, um.
You know, people talking about my cough and commenting. I was just, you know, everywhere I work, [00:10:00] people diagnosed me. You have COPD, you got this, you got that, you know. Yeah. You know, and then I remember even, um, a patient, uh, going to, uh, HR and, uh, anonymous, anonymously saying. Um, how dare Dr. Fuentes, uh, coughing and seeing patients.
I wish she, uh, to this day that I knew who she was or something, so would've told you, Hey, what I have is not contagious. I have this rather, I wish I have something contagious that perhaps could be treated with antibiotics, but I have something more serious in it. No. And, and here I was with the chronic, um, uncurable disease, still taking care of my patients and doing my job.
Yeah.
Patti: So something happened during Covid that required you to get, not one but double lung transplant on a waiting list. All this time.
Dr Maryluz: What happened is, no, I was still, uh, functioning with my. My portable oxygen concentrated with my four liters of [00:11:00] oxygen. And then what happened? I, I contracted covid before the vaccines were a avail, were available.
So I, even though I did not die, um, later on, uh, within a month, I, uh. I noticed that my, um, stamina, my shortness of breath. Uh, um, it was, it was very, it was, it was getting harder for me to go on with my daily activities. Uh, coming from my bedroom to my kitchen was just a task doing my bed. I couldn't do it.
It was, I was totally short of breath. So I went back to my pulmonologist and I told him what had happened, the one who had treated me during Covid, and he, uh, did my pulmonary function test and a six minute walk. And, uh, realized that I, that my, um, the f uh, f um, FPC, which is the forced vital capacity, had declined more than 15% in my, um, oxygen diffusion.
That DLC has also plummeted. So he [00:12:00] immediately changed my portable oxygen concentrator to a, um, littles of, um, um, tank, tank of oxygens and send it to my home, and also upgraded my um, um. My concentrator that I had a home from, that went from fat little that, uh, to 10 little, and I immediately sent me for an, uh, lung transplant evaluation at a, at an excellent center.
So how. Yes, and I, uh, they did all the battery of exams. It took about, about a, about a week, and I was a perfect candidate. Um, and then I was, uh, later on, after everything is done and you get all, all the, uh, the list and all the exams and everything that you need, you were put into a list and I was put on a list in a waiting in a and a, in a waiting list.
And, uh, it took about, uh, 10 weeks until I was called. I had the call with, uh, that I, nothing set of loans waiting for me.
Patti: Very short waiting list. 'cause some people wait several years.
Dr Maryluz: I know. [00:13:00] Yes, I was, I'm very fortunate.
Patti: And so tell me what happened after that.
Dr Maryluz: Well, I, uh,
Patti: quality of life. How did you, you know, function after that?
Dr Maryluz: Yeah, I had worked that day and I had finished my shift and I, my husband told me that, uh, let's go out for, uh, dinner. So we went out to dinner and then the phone ran and I didn't answer because it was a, um, it was not a disclosed, uh, number, it was a private number. So I disregarded and then. They called my husband and then he finally got it on.
And then we were so nervous. And, and then, so we came home and I remember that I took a shower. I had already packed because they tell you to get packed. And then we called my daughter and my sisters and uh, the rest of the family. And we actually, we have four hours, uh, to get to the center. 'cause it was about four hours, six hours we had, it was about four hours by car.
So my husband and my daughter were in the front of the car and I was in the. Back finish, you know, in my, the [00:14:00] charts and the things that I have done today and sending my information about the, you know, with the, to the organization that I worked to tell them that I was, I was going to. So, um, it was a, the procedure is a monumental procedure like I always said.
Um, and it's not like you get the, the, the, the transplant and everything finishing there. No, this is a compromise for life. Um, my quality of life have changed. I can do my normal life without oxygen. However, I have challenges. Patients do have challenges of the anti-rejection medication that, um, take a toll.
And, uh, you are in a stage of, uh, immunosuppression, more prone to infections, to, um. And, uh, uh, and of course, you, you take, um, prednisone for the rest of your life that, uh, put you at risk to develop diabetes as well. Um, some other, the, uh, the anti-rejection [00:15:00] medication make you prone to cancers to, uh, renal failure.
So, but, uh. But I am very grateful. I am, I feel that I am very grateful to have had this procedure in, um, my, my, it was not, I don't have, I didn't have any other options. So patients do not have any other options today. There are not, um, any treatments. There are treatments that slow the progression of the disease, but nothing that cured the disease.
Well,
Patti: you look fantastic.
Dr Maryluz: Thank
Patti: you. With my, with my mom, there was, um, only prednisone and they had her on such a high dose of prednisone. Mm-hmm. That, uh, that, you know, she fell and broke her hip and in the hospital she acquired pneumonia and staph infection and died at the age of 56, but she only was on like two liters of oxygen.
She was very active, and then three weeks later she's dead. Um, it happened that fast. Um, and, [00:16:00] uh, it was, uh, so what they say, two to six years, you generally live, it's sort of like being choked to death. That's such a devastating, horrible way, uh, to, to die. Right? Because your, your lung capacity is changing.
Yes. And my, my mother didn't have any support groups and I, I hear that you started a support group of people.
Dr Maryluz: Yes. Uh, two years after my diagnosis, I was on the, you know, I had this aha moment that I said I am a physician, but I also a patient and I need to find out if there are other patients who had had this condition.
So I, um, I remember period with my pulmonologist and then. Talking about creating and funding a, uh, uh, support group in the city that I used to live in. And, uh, I also ran an article in the, uh, in the local newspaper back in those days. And also, [00:17:00] and then, uh, and then, uh, also, uh, wrote an article in a, uh, publication that the hospital had that reach about 40,000 people.
So with that, I was trying to raise awareness. So when the first, uh, um. Support group. I had three people and some of them came and, you know, I, my husband had this, and, and somebody had this. And then, then they found that they, they saw somebody that also liked them, had something in common. So the group grew, uh, and, uh, I was very satisfied until I left, uh, the city where I used to live.
And, um, the, the, I'm proud to say that the group still is. Running by another patient. Uh, and, uh, continue, we'll continue to raise awareness and educating, uh, patients and caregivers.
Patti: Well, you're helping a lot of people. Um, so do they know what the cause is? Some people say hereditary, others say it's chemicals.
Used to be that they called it an old white man [00:18:00] disease. And, and is it common among, uh, amongst Latin or Hispanic community? I know that. We are South Asian and my mother was young and, and, and you were young when, when you were diagnosed and there's, they still don't know what's, what causes it, correct?
Well,
Dr Maryluz: the, uh, the idiopathic pulmonary fibrosis is an on unknown cause there are some contributing factors such as, uh, smoking age. You know, the, the women men, uh, are more prone to develop the pulmonary fibrosis than women. Women are more likely to develop pulmonary fibrosis. Secondary to a connective tissue disorder.
Um, there is, um, and there's also familial pulmonary fibrosis that runs within families as well. Um, yes.
Patti: So you also help the, uh, Hispanic community.
Dr Maryluz: I do, I, um, I'm bilingual, if you can guess for my, um, Hispanic accent. Uh, [00:19:00] and um, uh, with, uh, there is an organization called PF Warriors, uh, who was founded by a patient is the motto, is helping patient, helping patients.
Um, and at that time also we talked about that it was a, uh, a great need among the. Spanish speaking patients in Central America and South America and Spain, uh, where, uh, to make sure that we, um, talk about the, the myth and the misconception and the education in, in their native tongue. So I decided along with Nancy, so Suarez Lee, um, we both run, which is called the Guerrero, uh, which is a, um, uh, geared to um, Spanish speaking countries.
And we. Meet every, um, the, the, the fourth Tuesday every month. And we've been, we bring, uh, speakers, uh, that educate, uh, uh, the patients and the caregivers. So it's, uh, we're very proud that we're doing very well and, and hoping and, and, uh, [00:20:00] helping people in the this countries. You mentioned
Patti: that there hasn't been a whole lot of progress.
When my, my mother was diagnosed Ner Stone was the only thing that was around 2001. And you were diagnosed about five or six years later. Um. Given the current situation right now, are you afraid that, um, that research funding is gonna be cut? Uh,
Dr Maryluz: I am. Afraid, and I think it's very important, but I'm hopeful that, um, that we will continue pushing, uh, and loving for the importance of getting, uh, more research.
Um, there has been, uh, a treatment that has not been an approved, uh. FDA approved treatment within the past a, it's been a decade. Uh, there have been, we, um, have, um, drugs and, and phase, um, uh, clinical trial phase three, which is, um, um, which are [00:21:00] very excited about, um, and a lot of research ongoing on right now.
So yes, it's very important that we focus on that research and continue to lobby for, um. There is a lot of unmet needs in among the, the community of pulmonary fibrosis.
Patti: Yeah. It's very important for clinicians and researchers to hear your story. What are you going to be telling people at the conference in May in San Francisco, the 2025 a TS conference?
Dr Maryluz: Well, what I will be telling, um, clinicians and patients and the a TA, uh, a TS conference is that I, I had, had the opportunity to be in the, in the physicians, in the care provider, um, bench, as well as in the patient's bench. And um, so I know, I feel what as a patient is to have this chronic progressive.
Debilitated incurable disease. [00:22:00] So we need to have a representation, a voice of patients, uh, to continue pushing for some more. Um, studies would not only focus on the, uh, uh, uh, forced vital capacity, but also in the quality of their life and their feelings on, and, and, um, that patients and caregiver do have.
Patti: Is there a stigma against, uh, IPF? Do people think that, uh, that people brought it upon themselves or something? They don't know anything about the disease?
Dr Maryluz: Um, I think there is still stigma and mostly because of the, uh, the symptoms that they don't know what PE people or patients that aren't familiar.
They think it's a contagious disease. It's not a contagious disease, and the cough make it like, almost like you had cooties. Um, so, but, um, you mentioned somebody, your patients, uh, thought that, uh, you, you had covid and you were coughing on them. Exactly. It's, [00:23:00] uh, I mean, and I remember, I mean even that was before Covid and after Covid.
I remember one time sitting on a restaurant when they opened up, um, and there was a man about a feet, about three feet, uh, in front of us, whispering under himself that I could hear. Why did you stay here? What did, why didn't you stay home? Why did you come over here, blah, blah. Things like that, you know? So, um, it is, it is sad, but, uh, yeah, but hopefully, I mean, we continue to work hard, uh, within the, the clinicians, the researchers, the patients, the caregivers, the, the media just to be, um, to raise more awareness, to educate more people.
Patti: I look forward to your talk. I will be there as well, and thank you so much for joining me on Breathe Easy, the latest. And, uh, hope to see you in May.
Dr Maryluz: Thank you, Patty. Appreciate that. Thank you very much for having me here.[00:24:00]
non: Thank you for joining us today. To learn more, visit our website@thoracic.org. Find more ats, breathe Easy podcasts on transistor, YouTube, apple podcasts, and Spotify. Don't forget to like, comment, and subscribe, so you never miss a show.