Hello, everybody, and welcome to the webinar on Bridging Specialties, Timely Diagnosis in ILD Patients. I'm Teja Kulkarni, an Associate Professor and Director of the Interstitial Lung Disease Program at the University of Alabama at Birmingham. And today we are joined by Dr. Mary Beth Sholand. Hi there, I'm Mary Beth Sholand, and I am Associate Professor and Director of the Interstitial Lung Disease Clinic at the University of Utah. And I'm happy to be with you. We, Teja and I, are representing a group of people who have done a great job putting together this presentation and this program. And these include Dr. Dilling, Jessica Glennie, Timothy Hernandez, Corey Kershaw, William Lago, Andrew Limper, and Dr. Nambiar, in addition to ourselves. And this program that we're presenting is sponsored by Three Lakes Foundation, which is a nonprofit that's sole purpose is really dedicated to serving as a catalyst for uniting research industries and philanthropy, all for the purpose of improving our understanding and treatment of pulmonary fibrosis. Three Lakes Foundation connects entrepreneurs, advocates, institutions into an ecosystem that can innovate and transform our approach, improving a time to diagnosis and accelerating new therapies for Three Lakes Foundation. And if you're interested in learning more, you can join their website at threelakesfoundation.org. So the purpose of this program and this idea of bridging specialties is really looking at gaps and needs in interstitial lung disease and focusing primarily on that diagnosis. So interstitial lung disease is out there, but it's a rare disease and it's not the first on the top of many of clinicians' lists of what might be going on, so it's often overlooked. This disease is difficult to diagnose and not because it's so weird or unusual in its presentation, actually quite the opposite. It presents with ubiquitous symptoms. Most commonly patients show up being short of breath or coughing, very common findings in any clinician's office, and that makes it very difficult to tease down and think about interstitial lung disease as the reason for this common presentation. In addition, comorbidities that have similar presentations are present in patients with interstitial lung disease and often are the first that are approached for treatment and diagnosis. So really the gaps are that we overlook this disease and the needs are that we need to get patients diagnosed and treated sooner so that we can improve their disease trajectory and their quality of life. So a lot of what I just talked about and a lot of our gaps and needs are illustrated by this clinical study that was published in 2019, where we looked at patients in an IPF registry and saw that we had a significant delay in the timing of the patient's presentation to the time they actually received health care, and interestingly the delays occur at many levels. So a patient presents, as you can see from this figure, with their symptoms and there's many reasons it takes us a while to get to what we need to do to get that diagnosis done. The patient can be the source, so as we said, those symptoms are ubiquitous, they're subtle, so patients being short of breath or coughing can attribute that to many different things in their own life, such as, you know, they're getting older, they're getting out of shape, they're gaining weight, etc. But ultimately they decide it's enough to show up at a primary care physician's office and similarly the primary care physician can ascribe this to many different etiologies and may be slow to actually refer them to a community hospital, which may be again slow to refer to an ILD center. ILD centers are notoriously overworked and so there's oftentimes a delay in getting that patient, you know, diagnosed, seen in the delay to the interstitial lung disease and then again delays in making that diagnosis. So at many levels a patient presenting may take a while to ultimately get to their diagnosis. And we can see here that a big part of this is really occurring earlier, earlier from the ILD center, really at that general, well the patient, but at that general practitioner in the community hospital. And so our focus is really to look at those two big delay sites, the general practitioner and community hospital, as illustrated here. So by doing this we are trying to create bridges, really how can the general practitioners and the general pulmonologists and the specialists all work together, each with our own view of the patient to get to an earlier diagnosis and identify interstitial lung disease and help patients move to that treatment sooner. So the program focusing at these gaps and needs, it begins with thinking about awareness of the problem, which we just illustrated that there is a certain amount of awareness, research into what could help us move to the next steps, education, and then trying to distribute and implement these ideas that we have into practices. So we started this program with some research and what we did was really dive into what's happening in the U.S. So we queried 306 practicing U.S. primary care physicians and 110 practicing United States general pulmonologists. And we wanted to find out how providers approach the evaluation of patients that might have interstitial lung disease, assess what the provider's knowledge about this is, you know, are they aware of this, are they thinking about this, and also their attitudes about making that diagnosis, is it worth it making, how onerous is it, those sort of concepts. And then identify through this research what might be motivators to get this diagnosis obtained and what might be barriers in getting that done. So the survey results point to the fact that there are gaps in knowledge and there are gaps in clinical behavior, no surprise here, that contribute to these delays that we talked about, delays in diagnosis, and also some attitudes about whether the treatment options really have effects of the limitation of the treatment options and how we can understand quality of life measures. And reviewing a sample case is what we did in this survey. So the sample case was a woman who was age 55, presented with very nonspecific symptoms we just talked about, shortness of breath, that only occurs with exertion, by the way, so easily forgettable when the patient's resting, cough, exhaustion, and difficulty sleeping. Primary care physicians say this is typically what they would do. That there would be delays in this diagnosis. So we would like to then move on to how we might approach these delays and these gaps that we've seen by a toolkit that we've developed. And with that, I'm going to let Dr. Kulkarni walk you through some of the ideas that we've come up with. Thank you, Dr. Sholand. So as Dr. Sholand mentioned, there is a gap that was identified. So we researched by sending out the survey to understand how we may address these gaps. And from all that information put together, this toolkit has been formed essentially to look at how can we educate? What are the practicalities of applying these in practice? Changing the focus on how the patient is approached. What are the evidence behind all these changes? And then how do we engage the primary care physician community, the community pulmonologist, and the patient to then connect them to the ILD center and do this in a timely fashion? So for the first item was an updated patient questionnaire. So when we looked at the data from the survey results, it showed that most likely when they were taking the patient's history at the primary care physician's clinic, they don't really include questions that could clue into early symptoms of IPF or other interstitial lung diseases. For example, is there a presence of prescription drug-induced interstitial lung disease? Could there be various household exposures, environmental exposures, workplace exposures, a complete review of autoimmune conditions, and the presence of joint disease? But we also have to understand that from the primary care perspective, time is short when they're seeing new patients or follow-up patients. And being able to ask this extensive list of questions may not be easily feasible or practical. And so with that in mind, we created the updated patient questionnaire, which essentially has questions on the cough, the shortness of breath. What is, how often is the cough, or how long has it lasted for? And then going to questions about smoking history, family history, medications, hobbies, occupational and environmental exposures. And so this questionnaire can be given to a patient while they're in the waiting room. And this will just be a quick review on the physician's part as they're talking to the patient and understanding where these symptoms may be coming from. The other component that we have formed from the committee standpoint is that about half of the primary care physicians evaluate or refer patients for cardiac issues as a leading differential diagnosis for a patient, such as what we had as an example, who presents with shortness of breath. And only a minority of the respondents said that they would do spirometry or full PFTs and diffusion capacity was a part of the initial workup only in about 11% of them. And then when we talked about HRCT scans, high resolution CT scans, which are essential for ILD diagnosis, only 62% would order that if the chest X-ray was, did not show any changes. But we have to understand that in early dystitial lung diseases, we may not be able to see those changes on a chest X-ray alone. And only 50% would order if the examination revealed crackling upon inspiration. So with this information, the ILD e-learning module and the decision-making tools were created. Another piece of information that added is that only again, 39% said they would, you know, proceed with modification of the current medications over taking next steps and actually evaluating the symptoms of this patient to see if, you know, there's an undiagnosed condition that is not being treated by using inhaled therapy. And then half of the PCP respondents would refer to pulmonary consult at this stage. And, but 54% said that they would retain the patient for some more initial testing or attempted changes in treatment. So again, all of this contributed to the e-learning module and the decision-making tool. So what does this module do? It's, it's called the early detection for ILD module. The goal of that course is really to identify the classic symptoms that are associated with ILD, to be able to describe what the patient's history or presentation is, ILD specific questionnaires, and what is the additional workup that may impact early detection in adults with suspected ILD. And then finally, recognize how this early suspicion of ILD and detection can benefit the patient in terms of receiving treatment options earlier, through the use of the receiving treatment options earlier through partnerships with either community pulmonologists or referral to the ILD center. I mean, the goal is not really to teach primary care physicians everything there is about ILD. I mean, they are seeing patients with so many different conditions and it is not easy for them or practical to be able to go through all these questionnaires or be able to work through multiple differential diagnosis. But the goal really is to put ILD on the radar, say, you know, a lot of the patients present with similar symptoms that can be related to cardiac conditions like congestive heart failure or COPD, which is much more prevalent than interstitial lung disease, but really to say, okay, I need to think about ILD. Why is that important? Because time lost is lung lost. When it comes to idiopathic pulmonary fibrosis, it is ubiquitously progressive in all the patients. The progression patterns are variable and unpredictable. We do have two antifibrotic therapies, but they slow down the disease progression and do not reverse the fibrosis that has already ensued. And that is really the longer we take to the time of diagnosis and initiating treatment is part of the lung lost. So, what causes these delays? So, this module teaches us about what are the causes of these delays. Misdiagnosis is very common. Nonspecific signs and symptoms are characteristics of many diseases, like I mentioned. And how do we differentiate these conditions from ILD? There's some discussion about that. Again, looking back at the classic signs and symptoms, how does the patient with idiopathic pulmonary fibrosis or other interstitial lung disease present to clinic? There's not one specific clinical factor that can say, oh, this patient has IPF. It's a conglomerate of all the symptoms and physical exam and workup that's essential to come to this diagnosis. There's also information about when to suspect ILD in a patient and more information as you go through each of the classic symptoms, which is chronic cough and shortness of breath, physical exam findings, crackles on auscultation, clubbing in fingers, oxygen desaturation with ambulation or hypoxemia at rest. With workup, now physical examination is focused on pulmonary cardiac arrest and fibrosis. So, we're talking about cardiac and musculoskeletal systems. Can you think about an alternative diagnosis? Is that clear after this initial workup or is there a need for additional workup? And then if additional workup is necessary, what is the next step? Again, when you think about if you have a high suspicion for ILD in a patient, we're talking about a detailed medical history, a family history, social history, and this is illustrated in this module. What is important is being proactive. A timely diagnosis really depends on the awareness of the at-risk population for ILD and including understanding the classic symptoms and overall presentation. With that, I will hand it back over to Dr. Sholand to talk about the decision-making tool. Great. Thank you. So many interesting ideas as we think about the gaps and what we can do to address them. The next tool that we did is we talked a little bit about how we're going to identify it, but actually taking participants in this toolkit through a decision-making process, how it really works, how do you think about these people? We came up with three different patients within this decision-making tool, all of whom are going to have some type of interstitial lung disease, but who present a little bit differently, right? So how we would recognize the many faces of ILD. So every patient has a little bit of a different sign and symptom, and the participants in the toolkit can visit the patients in any order, but ultimately we'll go through all three so they can compare and contrast. And then, you know, make decisions as you go. And then these decisions that are made by the participant can be then guided along the way about how that look, you know, how that could be modified or how that's getting to where we need to be getting to. So we can talk about these patients on the next slide, I think. We have Harry, we have Michael and Layla, and the participant can select a patient and walk through these scenarios. And Harry is a 66-year-old, maybe a typical IPF patient we might see who's short of breath with physical therapy, has the, you know, the smoking history that we anticipate. Then we have Michael, who's a little bit younger, who actually isn't really presenting with symptoms, but presents for routine lung cancer screening. And then we have Layla, who's even younger, 35-year-old with reflux and coughing, who comes to you as a new patient. And then we walk through each of these patients through a variety of multiple visits, taking their history, deciding on what testing to obtain, deciding on treatment and seeing how that treatment works, and then deciding when or if a referral is appropriate. And then we have yet more information that we're reviewing from our research results that really focus more on the pulmonologist. And while we saw that our general pulmonologist could tell us, oh, we understand IPF, we learned that in fellowship, right? And they have a strong knowledge score, there seems to be a little bit more diversity in how antifibrotics are used. So 65% say they would routinely prescribe an antifibrotic only after a patient shows progression. And so this research informs some thinking on whether we want to do some ongoing chest education and ILD. And the next slide sort of shows us the, you know, our new, our components of this up and coming toolkit that we've talked about. So an ILD checklist. So thinking about what, how you would go through making this. Tejas showed us the questionnaire, the e-learning modules we've talked about, but ongoing things include resources and, like, such as radiology modules, so we can become a little more conversant in what the radiology looks like, infographics, and some gaming, some educational games are on their way to add to the toolkit that we've already presented to you. In addition to this, we're really interested in seeing how this is implemented. Is this really helpful? Is it making a difference in practices? So we have some grant opportunities that are going to be coming up for pulmonologists and primary care doctors to work with their partners in, or pulmonologists to work with their partners in primary care. So together with ILD centers to implement the toolkit. Is it helpful? Did it change what you learned? Is it useful to you? Measuring outcomes and innovating by looking at the grant outcomes, new ways to improve our time to diagnosis to ILD. So the whole goal of this is extending what we know. So looking at the research that we have, looking at the, and then moving our education to more tools that are really useful and pragmatic for people, and providing resources that are easily accessible and actually functional, you know, as Tejas said, in these fast-paced PCP clinics where we can give them functional resources to expedite their ability to evaluate a patient with ILD. So we're looking ahead to implement these interventions on practice and seeing what we can do, seeing what that long-term impact would be on how clinicians behave, how systems change, and ultimately what we can do for patients, patient outcomes. So we're excited. I mean, this is an ambitious program and we're hoping that we have some outcomes. And this is where Dr. Kulkarni and I would love to chat a little bit about this. But outcomes that we'd like to see, I think, are, you know, improving our collaboration. What happens when we get this earlier diagnosis? Can we improve patients' disease course? Can we improve their quality of life? You know, I think sometimes we wonder what we need to do before we get someone to an ILD center, and then how we get people more involved in research. I will say one of the things that I was struck by is the data you presented where 39% still, it's a minority, but a substantial minority of primary care doctors want to keep tinkering with medications before they want to refer on. I thought that was a really interesting outcome that really, or research result that extended what we saw from the study from 2019. What are your thoughts on that? Yeah, I think, I agree. I think that was very interesting to see. One of the main things it stems from is, you know, the patient presentation. I mean, a lot of our ILD patients present similar to the COPD asthma patients, the COPD patients, especially. Their older patients have a history of smoking and aneurysm exposures through work. But, and the general approach is, you know, this patient likely has COPD. I mean, you saw the number of, the small number of patients, physicians who actually get full PFT on these patients. And so the general approach really, and I've seen this in practice is where, oh, this is COPD. This is a former smoker, current smoker patient. Let's start inhalers and see what happens. I mean, and then the patient doesn't improve. So there's addition of inhalers or switch around until we get to the point where really this is not working. The patient is continuing to progress. We need to do something, right? So I think that's really an approach that we would like to influence and change that to where ILD is on the forefront when patients present with these symptoms. Yeah. And I wonder if it isn't part of what we're trying to do when we say building bridges. I mean, the whole name of the program is, you know, our ILD center is too daunting or are we, you know, not accessible? Are we not, you know, helping our partners and community pulmonologists or PCPs, you know, make that diagnosis or be available to them? And I, that minimally necessary workup prior to the ILD center, you know, I mean, you know, I don't know how you feel about it, but I'm happy to take anybody, right? I mean, if, if it's, if the suspicion is there, I don't feel strongly that, you know, they have to nail a diagnosis before I see them. Right. I don't know, but I wonder about that perception. No, I agree. It's probably the perception. I think it's, it's multifactorial. I think there's you know, community physician factors as well as patient related factors. But yes, our center too. And I think most ILD centers are more than willing to see patients. Yes. The wait times do tend to be a little bit longer than ideal, but at least, you know, everybody is more than willing to evaluate the patients and come up with a good treatment plan after discussions in multidisciplinary discussion and most of these centers. I think there's also some of the barriers, the patient related factors. I remember in one of our previous discussions where Dr. Lago, who's a primary care physician was on the discussion as well. And I had mentioned about, you know, the primary care physicians and the patients have a very strong relationship over years. And then when they're asked to see another new physician, there's some hesitation from the patient's part. And then you bring in rurality of the patients. If they live several hours away from an ILD center, they don't have the ability or not are not able to travel. And those may be some of the factors that the physicians are maybe physicians are also thinking about. But the main question is how do we break down that barrier and build that bridge to bring us all together for all the patients, irrespective of, you know, where they live or socioeconomic status or what they're able to, how much the patient is able to do from there. Yeah, I completely agree that bridge is a multifactorial issue. And so there's much to be done on all for all of us, really. Yeah. What do you think? Do you think what about this idea of better access to clinical trials? I mean, do you think that's enticing for primary care doctors, general pulmonologist patients, or do you think we tend to overrate that as specialists? No, I think it's, I think it's very important. The main thing that I think we need to focus on is getting that information to the community about the trials that are ongoing, what phase of the trials these are, and what does that mean for the patient, how to make that better, more accessible. I think it's equally important, especially when it comes to idiopathic pulmonary fibrosis. I mean, yes, we have medications that slow down the disease progression, but we are, it's not enough. I think we need to do more for our patients. And it's, that again, is a collaborative effort between the patients and the ILD center. I mean, let's go back to this diagnosis by LD, you know, how do you think this really impacts the disease course in our patients and what can we do about it? Yeah, no, I think that's a really important outcome to consider here as we go through this is, you know, if we're, if we're successful in this, building these bridges and we're getting these diagnosis, you know, what do we think is going to happen? And I think you, you know, you've, you've highlighted a lot of, you know, the concepts of our current therapy is that we have therapy, it's less than perfect, but we have therapy and it does slow progression. And I think, you know, this idea of beginning therapy early in their course, the disease, so we can slow progression really extends the improved quality of life, right? So when patients start losing lung function, they become more short of breath, they start using oxygen, you know, sort of in, in, in series. And, you know, the longer we can put off these important changes in their lives so they can stay active and they can do their activities of daily living, and they don't suffer from the lung disease that's underlying, the better their quality of life can be. So I think we, I do believe that, you know, knowing that these antifibrotic therapies slow progression by 50%, that we will impact their disease course by diagnosing early and getting on medications that slow the progression and we will improve their quality of life. And so I think, you know, we just talked about clinical trials and yes, we absolutely, you know, would love to have better therapies and more effective therapies and can reverse fibrosis and magically make it disappear. But even in the current status, you know, making that diagnosis early has positive impacts for patients. Ultimately, these are the outcomes we need to think about. You know, the time lost is long lost. So focusing on earlier diagnosis and improving collaborations between the primary care physicians and community pulmonologists. And from there, from community pulmonologist to the ILD pulmonologist, I think is a bridge that we need to work on building together. And that really will get access to clinical trials, to multidisciplinary discussions for patient treatment plans, and ultimately improve outcomes in our patients with interstitial lung disease. Dr. Sholand, do you have other? No, I agree. And I think that this toolkit is very exciting and innovative. And we're really excited to see how people, how it works, how people play with it and see what they learn and see if they enjoy it. And these grants that look at outcomes and how it's really being implemented so we can continue to improve this process and build these bridges. It's really just the beginning of a very exciting journey. So thanks again for your attention. And we're happy to answer any questions anytime. Thank you.

Bridging Specialties

Timely Diagnosis

Interstitial Lung Disease

Gaps in Diagnosis

Toolkit for Diagnosis

Improving Collaboration