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Melanie Cole (Host):  Welcome to UF Health MedEd Cast with UF Health Shands Hospital. I’m Melanie Cole and today we’re discussing idiopathic pulmonary fibrosis. Joining me in this panel are Dr. Christopher Harden and Dr. Gabrielle Graves. Their both Pulmonologists at UF Health Shands Hospital and Assistant Professors of Medicine at the University of Florida College of Medicine. Doctors, thank you so much for joining us today. Dr. Graves, I’d like to start with you. What is pulmonary fibrosis? Tell us a little bit about the prevalence of this disease and what you’re seeing in the trends.

Gabrielle "Gaby" Graves, MD (Guest):  Yeah, so pulmonary fibrosis in particular idiopathic pulmonary fibrosis is a lung scarring disease that’s very rare. It occurs in less than one in a hundred thousand patients that end up having this disease. And the more we’re learning about it, I think the more we’re able to diagnose it more accurately and get patients on appropriate treatments.

Host:  Then Dr. Harden, she mentioned idiopathic pulmonary fibrosis. What do we know about it since idiopathic would suggest we don’t know the cause? How are you defining it?

Christopher "Chris" Harden, MD (Guest):  Right so, we’re defining it so while it’s idiopathic, it’s the importance of that is that there are multiple causes of potential pulmonary fibrosis in patients and the specific entity of idiopathic pulmonary fibrosis is one that we do not know the clear mechanism for why it happens. And so, as part of the diagnosis is a multifold approach. One of these is that we do a very detailed history as there are a lot of potential environmental and work exposures that could potentially cause people to have a progressive fibrosing process in their body. Patients who have those types of diseases, fall into a very different class than those that we call with idiopathic pulmonary fibrosis.

Additionally, in our clinic, we also do a very extensive rheumatological panel to look for potential underlying autoimmune causes of pulmonary fibrosis. Some things that are more common that listeners may be familiar with are things like rheumatoid arthritis, or lupus, things that we associate more typically with joint manifestations, skin manifestations, and those entities but they can actually have a very important role in the lung as well and can be a cause of potential progressive lung disease. Following that, to diagnose idiopathic pulmonary fibrosis, they tend to have a very specific pattern on their CAT scan and specifically on a certain type of CAT scan called a high resolution CT scan. This pattern carries a name called UIT which stands for usual interstitial pneumonia which tends to be characterized by lower lobe predominant subpleural fibrosis and it’s usually and can be further subdivided into a definite pattern which consists of clearly seeing honeycombing which are small rows, at least three in a row of cysts that are in the periphery base as well as typically seeing traction bronchiectasis which are widened airways going out to those as well. That is what we’ve define as a definite UIP pattern.

There are more subtleties in what we call a probable UIP pattern in which we definitely have a lower lobe predominant fibrotic process that’s in the basilar and dependent regions, however it doesn’t have that nice clear honeycombing picture, but may just have areas of what we call reticulation, and you may see some traction bronchiectasis. When we see that pattern, that is when we sometimes talk about doing more invasive testing and even to get things like a biopsy to actually see what the pathology of the lung looks like.

Host:  Thank you for that very comprehensive answer and Dr. Graves, if you would expand as he’s discussing pattern bronchiectasis, what other complications are you all looking at? What will happen to a person with idiopathic pulmonary fibrosis? And tell us a little bit about your program at UF Health Shands Hospital.

Dr. Graves:  Sure. So, the things we worry about with idiopathic pulmonary fibrosis is that we know it’s a progressive disease, that it carries bad prognosis in terms of mortality associated with it because as lung fibrosis and scarring worsens, the lungs’ function declines and people can end up requiring oxygen and having real limitations in terms of their functional abilities. The thing we worry about the most in all different types of pulmonary fibrosis and interstitial lung diseases including idiopathic pulmonary fibrosis is exacerbations or flare ups of the lung disease. And the things that we know cause them are viral infections can cause exacerbations of pulmonary fibrosis, surgeries and procedures are also known to carry a risk of causing exacerbations and sometimes we don’t know why people end up having these exacerbations. But what we do know is that there’s a big hit or insult to the lung and that there’s a rapid decline of lung function and it can be very severe to the point where it can cause people to be hospitalized, it can cause people to be on life support and can also cause people to unfortunately die. So, that’s one of the things we worry about the most.

And we try very hard to try to prevent people from having this, so we always look to try to minimize any procedures, unless they are really necessary. We try to make sure people are up to date on all their immunizations and also talk about the social distancing and hand hygiene and wearing masks especially with COVID pandemic and with flu season now upon us. And then lastly, there have been two medications that have come on the market for idiopathic pulmonary fibrosis and these medications are nintedanib which is also called OFEV the name brand. And the other one is pirfenidone and the name brand for that is Esbriet. And these two medications have been FDA approved and have been shown to slow down disease progression based on pulmonary function tests.

So, we do now have two treatments for patients and then on top of that, there is a number of clinical studies that are going on right now at the University of Florida for patients with idiopathic pulmonary fibrosis to see if there is any other medications that we might be able to offer that might improve outcomes in these patients.

Host:  Dr. Harden, expand on treatment options if you would and what’s the role of managing comorbidities when you’re treating idiopathic pulmonary fibrosis?

Dr. Harden:  Sure absolutely. So, one very unique thing that I think we’re very fortunate here at the University of Florida that we have available to us is that we actually have a gastroenterologist or a GI doctor who specializes in esophageal motility disease and we work very closely with him and working on trying to eliminate potential causes of reflux and particularly reflux and poor esophageal motility which have all been potentially linked to making pulmonary fibrosis worse and possibly leading to exacerbations as well as progression of the disease.

This was actually initially started in our transplant program when they started working with some patients that they were actually able to even potentially transplant who were previously deemed by other facilities to not be transplant candidates. With regards to our research studies, we’re very excited because as Gabby mentioned, there are only two drugs currently that are FDA approved for treatment of this disease. And while in general, both of those medications are tolerated fairly well; in the event that there’s poor tolerance of those medicines, we don’t have a lot of other options at this time for treating IPF. We’re very excited because we offer in the studies that we offer, right now we can offer treatments that are both add-ons if you are already on those medications as well as potentially alternative treatment for those who want to try something differently or who may not want to take a pill and prefer something like an IV injection. So, we’re very excited right now in the pulmonary fibrosis world, that we have some new medicines potentially coming online that are in their stage II and stage III trials, so that hopefully we can provide our patients with a little bit more opportunities and more options moving down the road.

Host:  Dr. Graves, the program has a focus that’s engaging multidisciplinary teams to best treat these patients. What does this look like for your team? Tell us how you’re helping patients with those comorbidities, weightloss, smoking cessation, lifestyle, whatever that is and tell us all the different providers that are involved.

Dr. Graves:  So, as Dr. Harden mentioned, patients who have esophageal issues or dysmotility, we work very closely with our GI colleagues and especially Dr. Amaris who is one of his specialties is motility issues. Because we do know that chronic small micro-aspirations over time is thought to contribute to pulmonary fibrosis. So, we work very closely with our GI colleagues to try to optimize them from that standpoint. A lot of our patients have autoimmune diseases and so we work with our rheumatologists very closely as well and co-manage these patients. So, we actually have some days where out patients come in and they’ll see us, rheumatology and GI, all in the same day, back to back which is very nice especially if patients that are coming from out of town, that they only have to come in once and they can see all of us.

Another thing is, we work closely with our lung transplant because unfortunately, this disease can progress despite all the treatments that we have right now. And some of them do end up needing lung transplant and we have a wonderful lung transplant team here. Some will come in if we have a patient we’re concerned about, they are right down the hall so sometimes we’ll just grab one of them and say we really want you to meet this patient of ours. We like to get them plugged in with the lung transplant program as soon as possible. And so they’ll actually start that visit and introduce themselves and the lung transplant team right away and get them in a clinic very quickly.

Another group that we work with a lot is our pulmonary hypertension specialists. A lot of our patients do have pulmonary hypertension either as the cause of the fact that they have lung disease, so they have group three pulmonary hypertension and some of them because of their other disease processes have different types of pulmonary hypertension and so, we work with our pulmonary hypertension specialists a lot as well. And then again, as you mentioned, weightloss and our dieticians we meet with – we often refer our patients to them as well to help optimize their health as much as possible. And then of course, with the primary care doctors making sure that they are aware of everything we’re doing. If there’s anything that we noticed that we want to make sure the primary care doctor is aware of. I mean you name it; I feel like we work with a lot of different teams just making sure our patients are getting the best care they can.

Dr. Harden:  Sure and if you don’t mind, I’ll jump in and add one more thing there. Which is that we also try to do at least every other month, we are working on getting it to every month but with the COVID times it has unfortunately bounced to about every other month. We are doing group support meetings for patients who have pulmonary fibrosis of all different kinds. And those provide great resources for the patients as they get to mix and mingle with other patients who have similar diseases, hear their stories, hear what has worked for them. I know at least in one particular case; I had a patient who had made a recommendation regarding a certain type of way that he was using his oxygen and it made a huge difference in another one of my patients’ life after hearing that. Because I had made that recommendation to another patient after I heard his problems.

At these meetings, we usually try to have one structured lecture at each of them whether it be on different medicines – that the pharmacists coming to talk about different medicines that they may be used or may not be used in their various disease processes. We’ve had our friends from gastroenterology come and speak about the role that reflux and as Gabby mentioned, these chronic micro-aspirations play and then I’ve also had our colleagues from lung transplant as well as nutritionists and physical therapists all come and talk about different ways that they can potentially help. And that’s really helped as a whole kind of get the word out to the entire group about different things that we may be able to do and I think as a whole that our patients have in general, really enjoyed these meetings that we try to do.

Dr. Graves:  Also one other thing that I forgot to mention before too is that we have just to make sure that we are getting the diagnosis correct, we have a multidisciplinary meeting where we’ll review everything with our pathologists and with our radiologists and they’ll go through patients to make sure that we have a cohesive diagnosis and so that’s another thing that we offer here at UF for our patients.

Host:  Well it certainly is a multimodal and multidisciplinary approach to pulmonary fibrosis. So, Dr. Graves, first last word to you. What would you like referring physicians to know about the program at UF Health Shands Hospital and when you feel it’s important that they refer.

Dr. Graves:  So, I always feel like the earlier we can see the patients, the better. Mainly so that way, we can make a diagnosis and start them on treatments to try to preserve lung function and quality of life for as long as possible. And we’re always happy to see patients so, never feel bad or guilty about sending patients to us. We are more than happy to weigh in and give a second opinion and then we also are happy to see patients who – you know we have snowbirds here in Florida, so co-managing is something that we’re very familiar with and happy to do. So, we are more than happy to see people and help patients as much as we can.

Host:  And Dr. Harden, last word here. Tell us about any promising new therapies, looking forward to the next ten years in the field, since sometimes this does have such a poor prognosis. Tell us what you see that’s exciting, hopeful, something that’s going to happen in the future or that you’re looking to happen.

Dr. Harden:  Right so, as I said, I think everybody in the pulmonary fibrosis world right now is pretty excited about some potential treatments that are coming down the line at this time. ITP in particular, is an unfortunately progressive disease that up until this point, we still give approximate 50% mortality rate at three to five years, which is pretty significant and obviously can be life altering news when delivered to a patient. And we only currently have those two oral medications right now that are being prescribed and that are FDA approved. Unfortunately, sometimes those medications have potential side effects, in particular with the antifibrotic agent that were mentioned before. They tend to have sometimes some pretty significant stomach side effects with patients experiencing some pretty severe diarrhea or potentially nausea. And so, unfortunately, up until now, we didn’t really have anything else to offer them.

And while I don’t want to mention any of the particular studies by name, to try to bias anybody into one over the other; I will say that what we’re really excited to offer right now is that we have multiple studies ongoing in IPF patients that are national wide studies all in phase III trials which means that they are ready, this is the final stage before potentially coming out to treatment. And what’s really exciting about them is that each of the trials that we have offered can range from being another potential pill that they can take to an IV injection that they get every three weeks to even actually a new inhaler therapy that we’re excited to potentially offer in hopefully the coming months that may be coming to our program. So, and definitely now and in the very near future, we are hopeful to be able to offer our patients potential other treatment options if they’re not happy with the current antifibrotics that are on the market.

Host:  Thank you doctors, so much for joining us today and sharing your expertise for other providers. And that concludes today’s episode of UF Health MedEd Cast with UF Health Shands Hospital. To learn more about this and other healthcare topics at UF Health Shands Hospital, please visit www.ufhealth.org/medmatters to get connected with one of our providers. Please also remember to subscribe, rate and review hit podcast and all the other UF Health Shands Hospital podcasts. I’m Melanie Cole.