There are many types of pulmonary fibrosis, including Idiopathic Pulmonary Fibrosis (IPF). However, the journey towards a diagnosis of IPF is not always straight forward and it can also be difficult to understand the diagnostic process. In this episode of “Spotlight of Pulmonary Fibrosis”, we hear from Patient A.M. an IPF patient from Bulgaria, Dr. Kathrin Hostettler, a pulmonologist from Switzerland, and Prof. Jens Bremerich radiologist from Switzerland
Welcome to Spotlight on Pulmonary Fibrosis, a podcast provided by Boehringer Ingelheim. This podcast series aims to help people navigate their way through the complex pulmonary fibrosis journey, leading doctors and patient organization representatives from across Europe share their insights on the topics of diagnosis, treatment, and management of pulmonary fibrosis. Welcome to this episode of spotlight on pulmonary fibrosis. In this episode, we are going to be talking about understanding treatment and here with us today are Gali Rottner, and IPF patient from Israel. Hello Gali.
Gali Rottner (00:41):
Hello. Hello, Julie.
Thanks for joining us today. And we also have Professor Yochai Adir, a pulmonologist from Israel and chair of the Israel society of pulmonology. Thank you for joining us.
Prof. Adir (00:52):
Hi and thank you very much for inviting me. I think it's a very important session and I am happy to be here together with Gali.
We're very happy that both of you are here today. So we're gonna start with you Gali. Could you please tell us your story as a patient and patient organization representative, why did you choose to become a patient advocate and why is the topic so important to you?
Gali Rottner (01:15):
I'm very happy to take part in this important podcast. If I start my story, so I'm 47 years old, I'm married and I'm a mother of two daughters age 18 and 11 years old. I was first diagnosed six and a half years ago. At that time, I was playing catch ball in a nonprofessional team at my hometown for about three years. In Israel, we call it Mama net, and I started to feel a bit of shortness of breath. Despite my young age, I was 40 years old, I decided to get a medical checkup because of my family history. Unfortunately, my mother died from IPF six years before I was diagnosed. And already at my first visit to pulmonologist, my lung capacity was 70%. At first, it didn't seem like IPF. My doctor recommended on having a cryobiopsy, which didn't give me any significant information. And after two years of many medical tests and continuous decline in my lung capacity, a senior pulmonary professor determined that I have IPF. He recommended to start immediately pulmonary rehabilitation along with taking an antifibrotic medication for IPF, despite the fact that I was familiar with this disease because of my mother’s illness and that I were aware of the poor prognosis, I was hoping that the new antifibrotic medication will be a game changer. I also hoped that during the eight years between my mother's death and the time I was diagnosed, new research would have brought along new discoveries about IPF. The same professor who diagnosed me with IPF suggested me to contact the IPF Israeli Patient Association. I got from our dedicated team, all the support and information I needed for the last four and a half years. I'm on antifibrotic medication and I'm working very hard on rehabilitation. And I hope that I will have some more few years before I will need a lung transplant.
I certainly hope so too.
Gali Rottner (03:58):
I just want to say that I see in this podcast a great opportunity to raise awareness of this rare disease, because I really believe that early detection is very important and crucial for individuals that might be at a risk group. So, if even one person hear this podcast and recognize the symptoms and go to check himself, I think it'll be a great deal for me.
Gali Rottner (04:26):
And I think I could share from my experience and from my knowledge as a patient over the past six years.
Yes. Most definitely. Well, thank you for sharing that moving story, as in the beginning of your journey. We're going to turn to you professor Adir, as a specialist. Could you tell us more about your role as a pulmonologist and how it all began for you and why you wanted to specialize in pulmonology, and perhaps a little bit about what it's like to work closely with patients with this disease?
Prof. Adir (04:55):
So actually, since I was a teenager, my grandfather was becoming very sick and the medicine didn't have a lot of things to offer to him. And then I decided that I would like to go to study medicine and maybe I can help other patient, and maybe we'll find new solution for diseases that we didn't have solution at that time. And that's why I went to medical school, and I decided then to specialize in pulmonary medicine. And as you know, there is many diseases, some of them are rare diseases that we don't have good answer for these diseases. And I am very glad that, you know, now with all the new discoveries and the progression that we have in medicine, we find and continue to try find new drugs and new solution for those patient and especially patients with IPF.
Yes, indeed, thank you for sharing your story. And on previous episodes, we learned about the journey to diagnosis and how to find the right specialist for your condition. And while getting a diagnosis is a key step for patients with pulmonary fibrosis, patients and their families may find it difficult to understand what it means for them. Today, we're going to be discussing what happens after diagnosis with the focus on the prognosis and treatments that are available. Gali, I believe that you have some questions for professor Adir.
Gali Rottner (06:17):
Yeah. Can you please explain to us what is pulmonary fibrosis? What causes pulmonary fibrosis, are there different types of pulmonary fibrosis and what are the common signs and symptoms people would notice?
Prof. Adir (06:34):
Okay, there are very good and valid questions. So when we look on pulmonary fibrosis, which is what we call interstitial lung disease, which is like scars that are forming in the lung tissue, there are many reasons or diseases that can cause lung injury with interstitial lung disease that lead to pulmonary fibrosis. And usually what is accepted today, that there is groups of diseases that form specific form of interstitial lung disease. For example, one group is secondary to environmental exposure. Like for example, patients who were working with silica, patients who were working with exposure to asbestos, there is what we call Granulomatous interstitial lung disease that include patient with sarcoidosis or patient with another disease, that's called hypersensitivity pneumonitis, which is like an kind of inflammatory allergic response, for example, to birds or farmers that was working in silos and et cetera. And the largest group is what we call idiopathic, that we don't know exactly what is the reason for interstitial lung disease, it’s probably combination of genetics, environmental exposure and other, but there is no specific reason that we know that caused the disease. IPF is part of the interstitial idiopathic lung disease. We saw that antifibrotic drugs that are a help to slow the disease and patient with IPF, there was other study that showed that the same drug do the same effect on patient with pulmonary fibrosis due to other lung disease, for example, secondary to connective tissue disease or secondary to granulomatous disease, et cetera. And there is more movement today to say that the patient had fibrotic or non-fibrotic lung disease, which is progressive or not. And this is very important, considering the indication for treatment with antifibrotic. We don't know exactly how the disease will behave in the future, especially in IPF, we know that they usually the symptoms in the beginning are dry cough and exceptional dyspnea.
Prof. Adir (08:36):
These are the most common symptoms, but they are very nonspecific. And that's why, as you mentioned, Gali, sometimes it takes a long time for the family physician to diagnose that disease, and when they are doing the CT scan, and the other test, we see that the patient has idiopathic pulmonary fibrosis after excluding other reasons. So, it's recommended that any patient with dry cough will do at least a chest x-ray, to see if the patient has interstitial changes in the lung, and then we refer to CT scan. Heavy smoking is considering a risk factor for IPF, and if the patient has family member with IPF, of course, we need to think about it. One of the problem regarding the behavior of IPF is that we cannot accept what will be the course of the disease. Some patients have relatively steady disease with very slow decline over the year.
Prof. Adir (09:29):
Some patients have a very severe progressive disease that in several months to a year, then if the patient is not getting lung for transplantation, the patient will die quickly. And there are some patients that have a disease that is progressing and suddenly they can have what we call the acute exacerbation of the disease with acute deterioration, which carry out a high rate of mortality. But if we can stabilize the patient, the patient is in a lower level than it was before the exacerbation. And one exacerbation, usually the risk for another exacerbation is higher.
That's why we usually send every patient with diagnosis of IPF for evaluation, for lung transplantation, because we cannot say what will be in the future. If the patient will have a slowly progressing disease or will have a faster progression of the disease, and we want that if the patient is progressing, that we will have everything ready, if we need to do a lung transplantation.
Thank you for those answers professor Adir. Can you tell us also, what are the long-term effects of pulmonary fibrosis on the lungs themselves?
Prof. Adir (10:37):
If the disease continue to progress, there is a problem with gas exchange in the lung and the patient become hypoxemic, meaning the level of oxygen in the blood is getting down, because there is a disturbance in the blood get barrier in the lung. The patient become Hypoxemic, in the beginning during exercise, and when the disease progress, the patient will need oxygen all day long. Secondly, because of the disease, another complication that can develop is pulmonary hypertension, because the fibrosis is also cause the torture of the lung vessels with an increased pulmonary artery pressures, the have effect on the right side of the heart, with right ventricular failure, and also the extent of fibrosis can by itself, without pulmonary hypertension, disturb the action of the right ventricle with the peripheral edema in signs of right ventricular failure. That's why we would like to prevent this pulmonary fibrosis.
Of course. Is it possible actually to come up with an overall prognosis of pulmonary fibrosis or is it absolutely individual between the various different patients and patients with different underlying diseases?
Prof. Adir (11:43):
Well, it's depends if the patient, for example, have the worst prognosis with IPF, if patient has another interstitial lung disease, he has a better prognosis and also patient with connective tissue disease related interstitial lung disease, usually we treat them differently and they have a better prognosis. The diagnosis of IPF is depend on symptoms, on the look on CT scan and after excluding other causes and environmental exposure, occupational exposure or autoimmune disease or connective tissue disease that also can have the same characteristic on the lung CT scan and the same symptoms, It can be confusing and we need to exclude all other reason, because there is, to IPF, the only treatment today is antifibrotic, that try to prevent the derivation and prolong the time until the patient will have exacerbation of the disease.
You spoke there about the importance of early diagnosis, but what can be done to help patients manage their condition throughout the different stages of the disease early, for example, or advanced stage of the lung disease and why, and when can there be a need for a referral for a lung transplantation?
Prof. Adir (12:53):
So, as I explained before, because we don't know how the disease will behave, what we usually do is maybe except for patients with very, very mild disease, we send all patient to all the tests and all the evaluation that they will be ready for lung transplantation, because today it can be relatively in good condition, and after three months you can do acute exacerbation and deter it quite quickly.
So, we like to that we will be ready before. So usually patient with diagnosis of IPF, we do all the tests relatively early, we don't know to say which patient will deteriorate and which patient will not. We’ll have one in slow progression, and we need to see what's going on in the, during time. However, when we have patients, we need not only to look on his lung disease, we also need to look on other comorbidities that can affect the prognosis of patient with lung disease.
Prof. Adir (13:44):
For example, pulmonary hypertension in patient with IPF have worse diagnosis than another patient with the same lung disease, but with no pulmonary hypertension. And also, these patient with IPF have relatively high prevalence of obstructive sleep apnea. And we know that patient with obstructive sleep apnea and IPF has worse prognosis than patient with no obstructive sleep apnea. So therefore, we need to diagnose it too, and to treat the obstructive sleep apnea with what we call continuous positive airway pressure treatment, the CPAP machine, we need to send all patients for pulmonary rehabilitation, as Gali did, because it's very important to try to be in, in a good shape as possible. It's also important for the patient will need in the future lung transplantation. We need to look on the nutritional side and to see that the patient received good nutrition and also social and psychological support, which are very, very important. Some patient are depressed and it's very important to support them and to look on the disease as a whole, not only to treat the lung part of the disease,
Of course, and the progression of the disease is very individual. And so it makes it more complicated of course, to diagnose and treat. Gali, I believe you have another question from a patient's point of view for professor Adir?
Gali Rottner (14:59):
Yeah. What advice do you have to, uh, fellow physicians on how to deliver the information on the prognosis to the patients and their families?
Prof. Adir (15:10):
Well, first of all, I think that we need to do more regarding this because we don't have a structured psychological and social support for the patient and the family. And we need to do like a patient group support and to ask the patient to have some meetings with the social workers and psychologists, because as you said, it's affect actually everyday activities and the way you live with your family in the house. And so what I usually advise young fellows that first they have to know a lot of the disease, because they need to explain the patient, the family, what is the expected course of the disease and what the patient should expect during his coming years. And also, about the drugs, what are the drugs that are available? What are they doing? What are the side effect? How can they manage the side effect? And they need to be very supportive to the patients and the family.
Thank you for that. Although pulmonary fibrosis is a chronic progressive disease, there are treatments available that can help manage the condition and also slow down the progression of the disease. However, as a patient and also for family members, it can sometimes be difficult to understand the prescribed treatment, for example, how does it work? Why is it needed and what effect it has. Gali, from a patient's perspective, why is it important for patients with pulmonary fibrosis to understand their treatment properly? And why can that be challenging?
Gali Rottner (16:36):
Well, dealing with IPF diagnosis can be mentally and physically very difficult and frustrating as the doctor said, my treatment plan combines longer rehabilitation program at the hospital, along with an antifibrotic medication. And it's very important for me to understand every aspect of the treatment, so I can keep up the treatment, and so I know what is best for me, what to avoid. I can show that those two kinds of treatment can be very challenging. Rehabilitation is physically very exhausting to live with. The IPF is very exhausting. The short of breathness, weakness, and regarding the antifibrotic medication, I need to deal with side effects, mostly diarrhea, loss of weight, loss of appetite, headaches. And also I need to check my liver function every month.
It is certainly very challenging. Yes. Thank you for sharing that with us. And as a patient, you have some related questions for professor Adir about the treatment.
Gali Rottner (17:45):
Yeah. Professor, what do you recommended should be communicated to the patient and their families during the creation of the patient individual treatment plan?
Prof. Adir (17:56):
I think that many patients think that when they will get the antifibrotic, they will feel much better. There will be no dyspnea, no cough. And this is not the aim of the treatment. The aim of the treatment is to slow disease progression and to delay as much as we can, the exacerbation of the disease. And as you said, Gali, you still have dyspnea when you are doing exercise. The antifibrotic does not have any effect, usually, on exercise dyspnea. There is some studies that they show, they have some effect on cough, with improvement of the cough. It's difficult for the patient to understand if he will look one year after starting the treatment on himself and the patient that refused to get the treatment, he will see that he may be in a better place, meaning that he, his lung function didn't deteriorate so much. And his CT scan is quite stable, as opposed to a patient who didn't take it.
Prof. Adir (18:51):
And I think that this is very important to communicate to the patient, that they will need to understand that the aim of the treatment is to slow disease progression, because this is a progressive disease. And as we know today, there is no cure for the disease. And the only final result is lung transplant. And it's very important to explain the patient this point, because many patients come back to me and say to me, it didn't improve my dyspnea, I still have difficulty to do some exercise or to climb the stairs or daily activities. And this is not the end of the treatment. The second thing which is very important is to tell the patient exactly what are the common side effect, and how we can deal with the side effects, because the patient should be ready, that he can have gastrointestinal symptoms, like Gali said, diarrhea, vomiting, abdominal pain, he can lose appetite, he can lose weight, he can have disturbance in his liver function. It's very important to tell the patient in advance, how can he reduce the intensity of the side effect that he can live a regular daily life.
Gali in your experience as a patient and a patient advocate, what are the most common questions that patients have about their treatment plan for the doctors?
Gali Rottner (20:02):
I think that the most common question that patients have about the treatment are at what stage of the disease the patient should start taking the antifibrotic medication? How can I manage my adverse events better? How does the treatment works?
Mm-hmm, professor Adir, could you confirm that these are the topics that patients ask you frequently in your role as their physician? Or is there anything else that you would like to add to that? And what would your usual answer be to these questions mentioned by Gali?
Prof. Adir (20:33):
Yes. I agree with Gali. These are the most common questions, especially in patients with early diseases, that we recommend to start antifibrotic when the patient is quite well. And he ask me, why do I need this kind of medication, because I'm feeling more or less fine? And as many times I even show him some example of CT scan of a patient like him that was not treated with antifibrotics, and what happened to the lung after several years with no treatment, and try to convince the patient, and sometimes it works, sometimes it doesn't work. I had a patient that refused any treatment until he had very advanced disease, when he was on oxygen, and it was in too late stage to stop or to slow disease progression. And also the patients like to know about the side effect, many patient going to the internet. And they're reading about the drug, reading about the disease. And then, they're coming back and ask you about the drug and you need to answer them and to explain why we give this drug or another drug. And it's very important as I see it to be open as much as you can to the patient and not just to give them short answer and to answer the question, because it's very, very important to, that, the attitude that you give to the patient in order that the patient will have a good compliance and we take the drug as we would like him to do.
So, would you say that it's easier for you as a physician to support patients when they're well prepared and knowledgeable about their condition and treatment plans?
Prof. Adir (22:02):
Yes, certainly. Yes. We usually, I like to explain to the patient about the disease, about the treatment plan, what are the side effects, what the patient should expect in the future, and when they have this knowledge, it's much easier for me to tell them, okay, now you have a diarrhea. So do 1, 2, 3, 4. Why is that? Is it due to the drug or not? And so, if the patient has the knowledge, it's much more helpful for me to support the patient.
It can also happen that the patients might find it difficult, however, to adhere to the treatment plan prescribed by their physicians. Based on your experience, what are the most common factors for poor treatment adherence among patients with pulmonary fibrosis?
Prof. Adir (22:41):
I think that the most, the most common side effect is gastrointestinal symptoms. And this is, I think, the side effect that bothering patients, especially loss of appetite, loss of weight, nausea, and a lot of diarrhea, and sometimes even if we tell them to do certain measures to lower the intensity of the symptoms, some of them cannot tolerate the drug. And unfortunately, they stop the drug. They can switch from one drug to another drug. Sometimes it works sometimes no, but side effects are the main reason for poor treatment advance, because if they can tolerate the drug, usually the rate of non adamance is low.
Right, yes, it makes sense. Gali, you have a couple more questions about the treatment for the professor?
Gali Rottner (23:23):
Yeah. What advice can you give to patients who find it difficult to manage the disease symptoms and the potential treatment related side effects? What can you tell us about it?
Prof. Adir (23:35):
So, if patient come to us with side effect, for example, if the patient, have a lot diarrhea, we try to give him drugs that can slow bowel movements, and so he will have less diarrhea. We can offer him nutritional support. Pulmonary rehabilitation is very important, and we try to deal with this side effect. We also have a lot of studies recently on new treatment for the disease itself in order to try to change the course of the disease. And we offer this patient to join to these studies. It's not specifically on the side effect, but it's on new drugs for IPF. And there is still a lot of research on how we can manage not only to slow disease progression, we offer to maybe do some reversing of the fibrosis.
Gali Rottner (24:23):
Thank you. Gali, you have some more questions.
Gali Rottner (24:26):
Why is it important that patient, their treatment plan?
Prof. Adir (24:30):
It's important because as I said, the drugs slow the disease and delay the event of acute exacerbation of the disease, which had a very poor prognosis. So, it's important to adhere to the treatment planning, to slow disease progression, and actually to improve the survival of this patient because without any treatment, the median survival without any transplantation is two to three years. With antifibrotic, we know that the median survival is far higher, it's seven, eight, even more years. So, it's very, very important to adhere to the treatment plan.
Gali Rottner (25:02):
And what are the long-term benefits of treatment for the patient?
Prof. Adir (25:07):
As I just mentioned, it's prolong the survival, it's delay the time that they need to be referred for lung transplantation, they have less acute exacerbation, so it's very effective drugs in this point.
As we mentioned earlier on many patients and their families want to learn more about the various aspects of the disease journey and they turn to the internet to inform themselves, but it can be difficult to find trusted sources, of course, in a patient friendly language. Gali, you have a question about self-education for the professor.
Gali Rottner (25:38):
Yeah. Can you give a patient any recommendations on how they can educate themself about the disease journey, as well as on the benefits and risks associated with the different treatment alternatives, for example, where can patients look to find trusted sources?
Prof. Adir (25:58):
I think that the best way is to ask their treating physician, to give them, uh, links or some other form of media that they can go and read about the disease. Because as I say earlier, many of the patients go to the internet when they read articles that are difficult for them to understand. And the conclusions that they get from this reading is, is not the right conclusion. I think that we don't do enough, to be honest. And I think that we need to give them more support. We need to ask the pharmaceutical company, I know that there's many times they're doing a small book explaining the disease, how the disease is progressing, what are the treatment that we have today, how they can deal with it. I think if we can give the patient like a summary of the disease with links to some websites that are reliable, I think it can be very helpful for the patient.
And speaking of support, you are a patient advocate yourself, Gali. How can patient organizations help patients to educate themselves about their disease and associated treatment plans?
Gali Rottner (26:58):
Well, the patient organization are a center of support and knowledge for the, the patient, they doing their best to bring the patient all the new information and research from the best medical centers and also to pass the wisdom of all the experience gained and collect from the patient. Another important aspect is the mental support of a group that's dealing with the same, uh, rare disease, providing all the information and support on all aspects helps you educate. In Israel, once a year, our IPF association organizes a conference with the participation of senior pulmonologist, physiotherapist and the nutrition specialist, and therefore all the patients get up to date on news about research and information, how to manage the illness better. And I think it's very important mentally and informative.
Well, I think that's a good place to wrap it up at this point. I would like to thank you both for your interesting stories and your expert information. Gali, all the best to you. Thank you for joining us.
Gali Rottner (28:12):
Thank you. Thank you very much.
And professor Yochai Adir, thank you so much for your expertise and for spending time to tell us about it today.
Prof. Adir (28:21):
Thank you very much. And I think, as I said before, it's very important for the patient and for us, the physician to, in order that we can do better for our patient.
Absolutely. Thank you so much. Thank you for listening to this episode of the Spotlight on Pulmonary Fibrosis Podcast. For more podcasts in this series, you can subscribe for free on whatever platform you use. The information on this podcast is for informational purposes only and is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with any questions you may have regarding a medical condition or treatment.