Transcript: Interview with a Pulmonologist

​Daniel Seifer:
One of the mentioned, they may come in on inhalers or not have had inhalers. That's an important piece of their history, but also have they been using it correctly? Who gave it to them? What were the expectations that they heard when they got the inhaler?

Liz Rohr:
Well, hey there, it's Liz Rohr from Real World NP and you are watching the Real World NP YouTube channel. We make weekly episodes to help save you time, frustration and help you take the best care of your patients.

In this week's episode, I'm sharing an interview I did with Dr. Daniel Seifer. He is a Pulmonology Specialist Physician and a good friend of mine and we took questions from the Real World NP community and had a nice discussion about a few different pulmonary pieces. He is such a deep and thoughtful person and we went really into some really core pieces about asthma, about referring patients, when to refer them, different levels of acuity and triage. We talked about testing before sending to pulmonology. We talked about different testing, ordering spirometry and pulmonary function tests, so many beautiful things. And we even touched on interstitial lung disease, which is a not super well known phenomenon, but is so, so, so important in the care of our patients. Things to watch out for, approach to diagnosis and assessment and sending them out, so many pearls of practice. There are so many other topics that we want to talk about in pulmonology, so I have a feeling we will be seeing Dr. Seifer again, but without further ado, here is my interview with Dr. Seifer.

Awesome. Well, thank you so much for being here. Will you introduce yourself?

Daniel Seifer:
Absolutely. Happy to. My name is Daniel Seifer. I am a Pulmonary Critical Care Provider here in Portland, Oregon, working at our sort of quaternary academic institution in town. I'm the director of our Interstitial Lung Disease Clinic from the pulmonary half, you'll hear a little bit more about the rheumatologic split of that as we go through what we're talking about today. I'm the director of our Pulmonary Function Lab and a physiologist that works in our Exercise Testing Group.

Liz Rohr:
You're also one of the smartest people I know, so I'm so honored.

Daniel Seifer:
No one told me this was going to be like... it was going to make my head get that big.

Liz Rohr:
Buttering up Daniel.

Daniel Seifer:
I'm not going to be able to fit through doorways.

Liz Rohr:
Well, we have so many questions for you, so thank you, again, just so much for being with us. Why don't we just jump in? We took some questions from the Real World NP community and I just want to really get into, like you and I talked about before we started recording, how do we bridge the gap between primary care and specialty care with pulmonology? Maybe let's start with talking about asthma. I think one of the couple common scenarios that we see in primary care is somebody who's coming to your clinic who has a kind of, quote, unquote, "Prior diagnosis of asthma," for their whole life. And they take inhalers here and there, but we don't really really have any documentation. They've never really had any testing. We had a lot of questions about how do you approach that kind of case and what are some of the things to be thinking about from your pulmonologist perspective of just thoughts about diagnosing asthma and making sure that it's not other things than that?

Daniel Seifer:
Good question. I think that this will become a theme as we move through some of this. I think that this is a problem throughout medicine, but of course I'll only speak to the pulmonary side of things in particular today. I think that the way that things get named can place a undo weight upon naming them and it kind of perpetuates itself and rolls forward. And what I mean about that is asthma is a very broad term actually. It's a heterogeneous condition. There are multiple subtypes of it, there are multiple things that can cause it, and there are multiple phenotypes, which means how patients will actually present, what they'll be feeling, what might trigger them or what might not.

Before thinking about what we call what they have, I think more about how bad is what they have? Anytime I'm intaking somebody for a triage and trying to help decide do they go to our severe asthma providers or can they go to our general pulmonary clinic, do they get taken directly to me or do they go someplace different? That's one of the questions that I'm asking because not only does that tell us something about what type of diagnostic and treatment approach we might want to pursue with those patients, it also tells us something about how quickly we have to pursue those things, which is oftentimes the most important piece.

And so that's why I'm focusing on that bit for the question, the question being when do you think about other things. It's like, well, it depends on how bad what they're experiencing it is. I admit to certainly having sort of the quaternary tilt of everything I see is terrible, but I do remember what it was like to see people that didn't have terrible things. The most common thing with asthma or something like it that had been called it at one point would be, "I get short of breath. Certain things seem to make it worse, certain things seem to make it better." It's really dyspnea. They have shortness of breath. And one of the mentioned, they may come in on inhalers or not have had inhalers. That's an important piece of their history, but also have they been using it correctly? Who gave it to them? What were the expectations that they heard when they got the inhaler?

Focused a little bit on what is asthma. It's a, broadly speaking, would be an inflammation of your airways that you hear a lot in training is reservable, which is not always the case, but broadly speaking, an inflammation of your airways from allergenic stimuli, which is also a fairly broad term, that when triggered will cause types of bronchoconstriction, which is a narrowing of the airways via muscular contraction. And over time, if that continues to recur, the airways will also narrow because of inflammatory reactions and eventually potentially even scarring or morphologic changes over time.

Lots of talk and talk and talk and talk, right? There's a lot of things, but what else kind of falls in there? Well, you've probably heard the term reactive airway disease, which is not a term. And one of the things that I wish that everybody knew is to delete that term.

Liz Rohr:
I was going to say, there's an ICD-10 code, it really should just be removed.

Daniel Seifer:
There is, there is. If there was actually a physical manifestation of ID ICD-10 codes, I would be in trouble for how many times I would've been lighting it on fire and burning and throwing it off a cliff because it perpetuates this. We have to put something, try to separate the billing. I'll put billing codes that get me what we need that are relatively defensible for the problems that the patient has. I don't see that as putting what they have, putting the diagnosis there, because half the time the things that we see don't actually have a well-thought-out name or an ICD-10 code.

Reactive airway disease, that doesn't make any sense. It should be chucked. Now, where did it come from? It came from the fact that primary care providers were really kind of set with the American Thoracic Society, and in Europe, the European Respiratory Society, because they felt like, "Well, you're telling us that asthma has X, Y, Z characteristics, but we're seeing a lot of patients that don't meet those characteristics, and yet they seem to get better when you put them on inhalers, so what are we going to call this? And we're going to call it something else."

Liz Rohr:
Yeah.

Daniel Seifer:
And that is due to a communications failure because there is something already to call it. These patients may have bronchial hyperresponsiveness. They may have elevated response to some type of stimuli, and it can differ between patients, that makes their bronchi constrict. And that is an even broader term than asthma. Bronchial hyperresponsiveness, or airway hyperresponsiveness, within that extremely large balloon, asthma, which is already a large balloon, is contained within that other one.

Other causes of airway hyperresponsiveness can be very similar to something like asthma and you'd actually end up treating them very similarly. Things like exercise-induced bronchospasm, which is distinct from asthma that is triggered by exercise. Those are two completely different things. And you can actually, talk a little bit about testing, you can test out the difference between the two of those if you have access to the correct resources.

Just eosinophilic cough would act very similarly. Laryngeal hyperspasm, laryngeal hyperfunction. There are definitely subtypes of paroxysmal vocal fold motion that will act this way.

Tracheobronchomalacia, which is collapse of your larger airways, can act this way. Although, that one, you wouldn't expect that to show any difference with an inhaler versus not.

Many of these conditions though would have airway hyperresponsive. You could have airway hyperresponsiveness to one trigger that may be mechanical, a certain type of dust. You could have it to one allergen. And if you only had it to that allergen, should you actually call it asthma? Probably not. There's a lot of distinction here.

What I think that the big takeaway is, don't worry about whether it's asthma or not, worry about the following two things. How bad is it and is it getting worse? And if it's either not that bad but it is getting worse over time, or if it's really bad already, those mean you've got to figure out something more. You don't have as much time to just go, "Oh yeah, this is probably asthma. It's going to be good, it's going to be bad some days. Here's the things I'm going to do." It means you might need to get a little bit more aggressive. And that's in terms of both your diagnostics, but also in terms of your referrals, because there's a difference between, and you all know this better than I do, there's a difference between clicking on a button and saying, "I want to refer this patient to this clinic and it's standard," "I want to refer this patient and it's urgent."

And honestly, most of those go into the same box, although different institutions have different ways they deal with urgent versus I'm not going to get yelled at by some billing department, but that's different than when you identify a patient in your panel and you say, "Wow, this person's really sick and I'm worried about them and I'm not just going to send this referral over. I'm going to call my local office, even if it's an hour and a half away, I'm going to say, 'Hey, team that I've sent people to before, I've got this patient that I'm actually really worried about. And this isn't a trigger that we pull lightly. This is someone I'm worried about. This person's been to the emergency room twice in the last blankety-blank hours. They have ended up on oral corticosteroids like prednisone. I'm not even sure if it's making a difference, but things seem to be spiraling out of control and nothing we've tried has worked.'"

And when you get a call like that where there's personal experience and there's something said about it, okay, the tertiary centers or the secondary centers or the quaternary centers, depending on what you're closest to, they act more quickly.

Liz Rohr:
Yeah.

Daniel Seifer:
Because they go, "Okay, this person has provided a filter already and I'm going to bring this person in sooner."

Liz Rohr:
Totally.

Daniel Seifer:
When it comes to those patients that are really severe or spiraling, while there are some pieces of workup that you can send, you all have limited time, you have limited resources to devote to patients. You have less time with patients than I do, and none of us have a lot, so it's probably most effective to spend that time on the ones that you... And then hopefully this ends up only being less than 5% of the people that you identify. I'm talking about the ones that trigger that worry reflex. Your time is probably best spent in ensuring their safety and pushing them towards a center as opposed to going, "Oh yeah, what did I hear Dan Seifer say on that podcast that one time about running this one breathing test?" Because that's not going to matter.

Liz Rohr:
Yeah.

Daniel Seifer:
When you send them into us, that's not what we're going to do first either. We're going to use the fact that we have resources and we're going to be super aggressive, things that you couldn't do even if you wanted to in a primary care setting.

Liz Rohr:
Yeah, totally.

Daniel Seifer:
What kind of things do you do in the light or the medium cases? And most of those, you kind of handle the same way. It's just a question of how much you order and what you don't. I think the first thing that's worth talking about is spirometry or pulmonary function testing.

Liz Rohr:
Oh my gosh, yes. So many questions about how to order it and interpretation.

Daniel Seifer:
Yeah, great.

Liz Rohr:
Yes. And when to order it.

Daniel Seifer:
Right.

Liz Rohr:
Because I think that's a huge question people have is they're the light-medium category. They're not super short of breath, they're not going to the hospital. What tests do we do first and how to order them correctly is 100%, they're a 1,000 times on the list. Go for it. Go for it.

Daniel Seifer:
Pulmonary function testing is really a broad term and it refers to all of the different types of maneuvers that we can run on a patient that that then allows us to compare them to their peers. When we talk about spirometry, within that, there are separate maneuvers. And I know the order sets will say something like, "I'm ordering spirometry," or, "I'm ordering PFTs," which is kind of nonsensical honestly, in my opinion. What you're ordering are these maneuvers. People might call them tests, but they're not. We're trying to ask the patient to do something for us such that we can measure them, what they're doing, and then compare it to their peers. It's like if you're asking someone, "I need you to run a 100-meter dash." That's a maneuver.

Liz Rohr:
Yeah.

Daniel Seifer:
And there are rules to that maneuver because if you ask someone to run a 100-meter dash and instead you just say, "I need you to get from this point to that point as fast as you can. One, two, three, go," and the person turns around, gets on their hands and knees and does the crab walk backwards? Yes, they tried very hard, they might be tired, but that's not going to be comparable to somebody that just took off and started running like bi-ped. It's not going to do it. Or someone that slowly built up speed.

There's going to be a lot of rules about what you're asking them to do, and the more rules that you ask them to follow, the harder the maneuver gets, but also the more comparable the maneuver gets because now you're going to be able to say, "Okay, this person stood upright, started on both feet, they didn't have a catapult that launched them out, they were wearing shoes." I'm just adding to this joke, this metaphor, but if everyone meets those criteria while running this race, okay, maybe at the end of that you can say that this person was faster than this person. You'll be able to identify both exceptionality, which is the top end of high things, and pathology, which is the low end. That's the idea behind asking people to do a maneuver.

When you actually get a report back from sending someone for spirometry, if you just click the button spirometry, most of times what will come back to you is one maneuver actually, just one. And there are a lot of numbers on that sheet, but it's all coming from one maneuver. You've probably heard FVC, forced vital capacity, and that's a maneuver name. That's actually a maneuver name. The vital capacity is a physiologic number, something you can measure, but a forced vital capacity, you're asking them to show you their vital capacity in a forced manner. They take a deep breath in and they blast it out as hard as they can. And that covers a certain portion of their lung function. And then we divide it up into all these little slices. FEV1 is just the air that was expelled during that maneuver in the first second. All the other numbers on there are just percentages, divisions, whatever, of that one maneuver if you just click spirometry.

Liz Rohr:
Totally.

Daniel Seifer:
Okay, so what am I even talking about or why am I going into this? It's because what do you order and what don't you order? That's the question that you were sort of asking. And the answer is it depends on what you want to know.

Liz Rohr:
Yeah.

Daniel Seifer:
If you want to know do they have asthma, there are certain types of breathing tests that you can order, but none of them comes back with, "The asthma level in their air that they breathed out was 2.5%, now they have asthma." That's not how it works, unfortunately, I wish it worked like that, like a blood test where you can order a blood test, you'd be like, "That sodium is 112. That is low." It doesn't quite work that way.

When you order these tests, what you're asking for is compared to their peers or to their past, if they've had results in the past, has there either been change, if you're comparing results to the past, or compared to their peers, are they exceptional or is there a pathology here? And if there's a pathology, what is the list of things that might explain this pathology?

When you order spirometry, there's two types of things that you'll often see in the results when people comment upon it. They will say something like obstruction or restriction, though that's also a little bit of inexact terminology. The correct terminology would be obstructive impairment or restrictive impairment. And spirometry is how you diagnose an obstructive impairment, period. If you do spirometry and you don't have an obstructive impairment, then you don't have an obstructive impairment. "Oh, so that's good." I'm not sure how much stats gets talked on the channel, but a pretty good positive and negative predictive value, in essence, 100% in some ways. If it's there, then you have one, and if it's not there, then you don't. Just plain spirometry can not say that about a restrictive impairment.

There's another maneuver you would need to send for. It's a mouthful. Plethysmography is one way of doing it, but also nitrogen dilution. What usually gets clicked on the order box though is lung volumes. If you were sending someone for lung volumes, there's really only one question you're really trying to answer there, and that is do they have a restrictive impairment or not? That's either there or it's not.

I know I harp on the semantics of this, but that's because there's a difference between diagnosing an impairment versus having a disease because it's actually possible, believe it or not, it's possible that on spirometry you might not have an obstructive lung impairment. You may not have an obstructive impairment, but you may actually have a disease that causes obstruction.

How is that possible? Well, it may be that the time that we've tested you at, it wasn't happening to you.

Liz Rohr:
Yeah.

Daniel Seifer:
And that's asthma. That's what makes asthma. You can't send someone for spirometry that then comes back and then tells you do they have asthma or not, because maybe their muscles aren't spasming right then, so you might not see it.

Liz Rohr:
Yeah.

Daniel Seifer:
If you sent someone for spirometry and it came back and it said they had a obstructive impairment, asthma is one possible answer.

Liz Rohr:
Yeah.

Daniel Seifer:
There are others, COPD, maybe the maneuver didn't go well and the pulmonologist didn't read it well, maybe they have a tracheal stenosis, maybe they're having tracheobronchomalacia. There's other things on the list.

What can separate asthma is that it's reversible, generally speaking, if you give them a med. If you sent someone for spirometry with bronchodilator testing, albuterol testing, what they do is we do an FVC maneuver and then we give them, under watchful eye, if the pulmonary function lab is good, we give them under watchful eye a short-acting bronchodilator. And then we see, do they get better? Do we redo the FVC maneuver afterwards?

And so if you have a patient who you send for spirometry with bronchodilator who has an obstructive impairment, and then they do the albuterol puffs and then they repeat the FVC maneuver, which is part of this all testing that you'd get and it's gone, that's a really strong suggestion that they have asthma. Okay, good. That would be very useful. Does it 100% diagnose them with asthma? No, but that's a pretty good certainty. That sort of set of answers, that's a pretty good positive predictive value that now you've got asthma, whatever else you may have that's hiding else under there, you've at least got asthma.

What if you send someone and you do that though, and they have an obstructive impairment, they do the bronchodilator and it either doesn't get better or it only gets a little better, it doesn't go back to the normal range? Well, now what do they have? Well, it actually still could be asthma. It could be asthma and they're being triggered so hard that the albuterol isn't working. It could be that they have COPD and they have asthma. The literal definition of COPD is having an obstructive impairment on spirometry that does not go away with bronchodilator testing.

Liz Rohr:
Yeah.

Daniel Seifer:
These things get confusing.

Liz Rohr:
Totally.

Daniel Seifer:
But what I think is important to remember about all of it is when you send somebody for the thing, recognize that the test is giving you a piece of information, but it can't really give you the diagnosis or not, especially with asthma, and that's what's relevant.

Liz Rohr:
Yeah, that's super helpful. I really appreciate that. I think that it just brings up so many questions and I wonder if you want to get into... I'll give you the choice. The first thing that's coming up in my mind is what are some examples of the high, medium, low in terms of acuity of patients? What are the examples that you see? What would be examples of which one? And then I think the other piece that's coming up for me, so you get to pick which one, but when it comes to ordering lung function testing, it's basically spirometry or PFTs in the order set for most of the clinics that I've worked at, or it's only PFTs and then it's like, "Okay, I'm just going to order this thing," and then I get this big long list back and it's like, "Well, I really only wanted spirometry, but I got this big long thing." Where do you want to go next with that? Do you want to talk more about PFTs? Do you want to talk about the different types of patients?

Daniel Seifer:
Great. Let's do both. I think the PFT one, I can answer quickly. While I'm curious at those clinics, usually just from a building perspective, you can't just click PFTs and then get multiple maneuvers back. When you click PFTs and you get a whole bunch of numbers, most often what will come back to you is just a bunch of numbers from the spirometry maneuver. A lot of places can't even do a lung volume. It's beyond their capabilities. They may have the ability to do a diffusion capacity, which is yet another maneuver, but they may not have the ability to do a lung volume test. You don't lose anything by having all this extra information.

If these numbers are coming back to you without any interpretation, that's a huge problem because that's like kind of getting an echocardiogram back and the person that did it saying, "Here are the pictures." It's like, "Well, that's interesting, I guess, but I need you to tell me what's up."

Liz Rohr:
Totally, totally.

Daniel Seifer:
It really actually shouldn't be legal to bill, based on my understanding, unless a pulmonologist has read those results.

Liz Rohr:
I see, I see.

Daniel Seifer:
And if the reading pulmonologist is paying a lot of attention, they will answer many of those questions that I was just sort of dancing around there. Although I confess that those answers vary pretty widely depending on the lab and who's reading them.

If you are the only one who has to interpret this, which can happen because there are spirometers in offices, there are things like that, following the latest up-to-date ATS guidelines is the best way to go.

Liz Rohr:
Yeah.

Daniel Seifer:
And that won't tell you what to do next, but answering the other question that you just asked will tell you what to do next. What are the examples of the severe medium and low? And that's sort of why I kind of started on that to begin with.

What triggers me to think severe? Not just about what was really happening to the patient in the past, which does matter, but what's been happening recently? If in the last three months before you've seen the patient, they've been on multiple courses of prednisone or they're still on prednisone or some other oral glucocorticoid and they've not been able to come off because the disease severity has been too high, that's bad.

You may not see the end results of this very frequently, but staying on prednisone will kill you eventually. Prednisone is not one of those things where there are potential side effects from a med, and sometimes you get them and sometimes you don't. That happens with prednisone, not everybody gets really agitated. You certainly don't get suicidal or psychotic, everybody, but some people do. But there are certain effects of prednisone that if you're on it long enough, it's not a side effect, it's a primary effect. You're giving your body a stress hormone for a long enough time, you will get diabetic, your bones will demineralize, your muscles will waste, your nervous system will start going bonkers. Your intraocular pressure will eventually start to raise. It will tear you apart. And those things, we're like, "Well, they're just on it for a month." Trust me, a month can cause more damage to someone whose health was already not great than you think.

And so if someone is on steroids or receiving them frequently and doesn't seem to be able to get off of them, that is automatically a, "Hey, I'm concerned," situation. And that's different than someone who's like, "I've had asthma my whole life, once or twice a year, I get a five-day course of steroids. I always get off of them. When I'm not on steroids, I feel like I'm okay. I can do my work." That's a very different story. That would be more of a medium.

Liz Rohr:
Yeah.

Daniel Seifer:
Versus a mild would be, "I never take oral steroids. I have some trouble breathing sometimes. Once upon a time a doctor gave me an inhaler, I kind of have it. Sometimes I spray myself in the forehead with it. I don't know if it helps or not." And so that's how I stratify. And hopefully when I was sort of giving those examples, the first one sounded like, "I should do something about that." And the second two sounded like, "Okay, this is more a diagnostic question that I can maybe dig into a little bit without having to be like, 'Action first, thinking second.'"

The second two... Inhaler technique is a huge thing.

Liz Rohr:
I have some big feels about inhaler technique. Yes.

Daniel Seifer:
Yeah. Yeah. It's a problem. I wonder, actually, I've got one of them up here. I don't have the spacer sitting here. I think that's sitting downstairs somewhere. But just some better understanding of what the inhaler technique is. What are the different types of inhalers? How does that type difference change the technique? Makes a big difference for patients. If you're not using the correct technique for the inhaler that you've got, your drug delivery can actually be zero. You can be getting zero of the medicine down to where it needs to go. 0%.

Liz Rohr:
Yeah.

Daniel Seifer:
On the other hand, if you're doing the right thing, you can be getting a lot of the medicine down to the right place. And in fact, it can be more effective than nebulizers. You'll hear this frequently that patients are like, "The nebulizers work a lot better than my inhaler." Nebulizers are useful because when someone is having an acute exacerbation, they actually can't perform the correct inhaler technique. If they could, it would actually be better for them to use the inhaler because more drug can be delivered that way and better types of drugs can be delivered that way.

Liz Rohr:
Interesting.

Daniel Seifer:
There was a recent... Well, maybe recent is dating myself a little bit, but finally the ATS and American recommendations have caught up to what the ERS has been doing, basically like the SMART trial with inhalers, that the old model of having someone on an inhaled corticosteroid and then having a short-acting bronchodilator like albuterol that they can use once in a while is substandard compared to having someone on a ICS long-acting bronchodilator combination inhaler, something like, you'll have to forgive me, trade names, I intentionally forget them. It's weaponizing my own attention issues. But something like budesonide-formoterol, which I believe is Symbicort. That can be dosed and used in a way that is much more effective for both treating the disease from asthma control scores, but also for preventing patients from needing to go to the ED and preventing them from needing oral corticosteroids to begin with, which is sort of the game. I'll hush myself for a second, and you said you had questions about inhaler technique though, so please...

Liz Rohr:
Well, no, I just was going to say that I have some big feels about it because it's just literally every single time I have somebody with asthma, we've had multiple conversations. We talk about inhaler technique, I watch them. It's a struggle. And I think there are people also, and I think a lot of people would concur, that people say that, "Oh, well, I don't need the spacer. I do it fine." Well, isn't delivery only 30% or something without the spacer anyway?

Daniel Seifer:
Right. Let me show you. It depends on the thing. Where to start with this. Okay, let's go with this way. Make a distinction between there are different types of inhalers in terms of which kind of medicines they deliver, but there's also different types in terms of their delivery systems.

Liz Rohr:
Totally.

Daniel Seifer:
This one that I'm holding in my hand is the classic, and this is an HFA. You may have seen that.

Liz Rohr:
Totally.

Daniel Seifer:
Hydrofluoroalkane. That doesn't refer to the medicine, it refers to the propellant that shoots it out. If somebody's got one of these HFA inhalers, I'm going to show you, and I think it'll be captured here. This is just me holding it out and I fire it. That was even further than I thought. I'd have to move my hand over here.

Liz Rohr:
I didn't see it all the way to the side, but yeah.

Daniel Seifer:
Visually and physically, I can feel it hitting my hand all the way back over here. And my hand is not breathing in, I don't have some sort of vacuum cleaner on my hand, that is just the propellant from this. Unless you're giving this to a 10-story tall giant of a creature, if you put this in your mouth and you trigger it, every single bit of that is going into the back of your throat, almost all of it. And so I find that the actual physical demonstration and being like, "Hey, is your throat that long?" No, it's not. Even if you happen to suck in really hard right when you trigger and you get a little bit of the medicine down, you're missing most of it, at least 70%, and most likely more.

The spacers are important, but so too is the breathing technique. The spacers don't help you in and of themselves. Where you're trying to get, in actual asthma, where the bronchoconstriction, the muscles are the problem, that's not happening around the trachea, that's not happening around your main stem bronchi. That's happening in the smaller order airways. Branches and branches down.

Your task is how do you get this medicine, which is in effect topical, it has to touch the problem. How do you get it down there? And so there's a couple of really important steps. And I won't do that thing where I'm like, "Tell me what you know about inhaler technique." I'll just show you, but I'll be curious afterward if there's anything that I did that you've maybe never seen before or you've been like, "Oh, I didn't even know that was important." And in frank, maybe contrary to what is in the instructions for the companies.

Liz Rohr:
Oh, I'm excited. Go for it. Let's do it.

Daniel Seifer:
Well, imagine I have a spacer, which is a plastic cylinder, which I don't have right now. I'm shaking it up. With an HFA, you really have to shake it up because the medicine will separate from the propellant, and if you just fire it, the propellant will be at the bottom and so it will trigger out more. And you end up in a situation where you're not getting dosed as highly later in the month as you would be earlier in the dose schedules. And it can't just be shaky, shaky, shaky, you've really got to give it a go, especially if it's been sitting around for a while. You give it a big old shake.

Liz Rohr:
The amount of information in your brain, I just can't, I just can't.

Daniel Seifer:
The number of times I've repeated it perhaps. Repetition makes it easier to store things. You've got the thing, you've shaken it up, and now you see most people stick it in their mouth. No, you don't stick in your mouth yet because there's something in the way. There's something in the way. And what is in the way is the air that is already in your lungs. Your lungs aren't collapsed right now. There's air in there. You've got to get most of it out of the way, especially the parts that are way down in there. Otherwise, you're never going to be able to put something new in its place.

Before you stick it in your mouth, the first thing is you've got to breathe out. And most people will go, "I breathed out." No, because we need you to get below... Well, that's getting into some deep physiology there. We need you to get more air out than that. You can't actually collapse your lungs by breathing out, but we need you to empty your lungs. When you do spirometry, it's going to look weird, when you send someone for spirometry, you do the forced vital capacity maneuver that I was mentioning earlier. I don't know if you've ever seen anybody do this or seen someone do spirometry. There's a mouthpiece, there's nasal plugs. And we say, "Okay, when you're ready, breathe tidally, in, out, in, out, and then take a deep breath in." I'm going to show you what the maneuver actually looks like. Imagine if I'm on a mouthpiece, you're not just in air, but this is what it looks like. In, out, in, out.

Liz Rohr:
Oh my god.

Daniel Seifer:
You probably heard a lot of noise at the beginning.

Liz Rohr:
Yes.

Daniel Seifer:
Because that's a lot of air was coming out. That's why the FEV1, that's why more air comes out in the first second. You can hear it. But why was I sitting there forcing it? That's what's actually required because there's still air in there. There's still air down there. And believe it or not, if I had a flow sensor hooked up to my mouth, it would've shown right up until about the end, there was still air in there.

Liz Rohr:
Wow.

Daniel Seifer:
Now, do I need patients to do that every time before they hit the inhaler? No. But I do need for them to get fairly close to that same lung point. I need them to get close to that amount of air out, but I don't need them to force it. There's another maneuver called a slow vital capacity maneuver, and that looks like this.

Liz Rohr:
That's a lot longer than I thought.

Daniel Seifer:
I got pretty close to the same amount of air out, but I didn't have to try as hard because I was going easy. I show them this in the office. I say, "We're going to do it together," and I keep moving the hand like this, or if I'm on a video visit, I move my hands, they can make sure they're seeing, or I snap if we're just on a phone visit. And I say, "The whole time I'm snapping, I'm exhaling." Gently, gently, gently. You get it all out.

Now, once you've got it all out, then we've got to stick this thing in our mouth and we're going to imagine that I have a spacer here. There's another thing here. I keep reminding everybody. There's another piece here. I always jest with my patients, I'm like, "I know you don't know this part," or rather, "I know you know this part, but I'm going to say it out loud anyway." You don't want it in front of your teeth, that doesn't help anybody. Actually, you don't want it like this either where your teeth are resting on top of it. What you need is your lips sealed, but your jaw open. Lips sealed. Am I good there?

I show them, I go... You drive down your jaw, which gets your tongue out of the way, which will help prevent this from just nailing your tongue and either causing glossitis or making it more likely that you're going to get thrush or something like that. Then go down like this, you don't do this, you don't do this. You want a neutral neck. You've shaken this up, you've done a really long, slow breath out. You've stuck this in your mouth the correct way. And with a spacer, you'd then trigger this and then you'd breathe in like I'm about to show you.
All this is ready, all this is done, trigger.

Liz Rohr:
That's awesome.

Daniel Seifer:
What you've done there is you've long, slow breath in and then you hold it for eight seconds. You've gotten the medicine down where it needs to go. And then you've allowed it time, that eight seconds, to deposit. Once it's stuck down there, then you breathe out. And one of the reasons that it's so important to go so slow, the spacers have a whistle on them and if you go too fast, they whistle, but that whistle is too high actually. You should go slower if you're using one of these HFAs, because what you're trying to do if you breathe in quickly and go... The fastest way that the air will go down into your throat is turbulent flow. The air spins or jostles. It's kind of like if you have a jug of milk and you're trying to pour it through a tiny hole. If you wanted to just get the most milk out of the jug as fast as you can, you would turn it upside down and shake it all would pour out, but not a lot of it would go through the hole.

Liz Rohr:
Yeah.

Daniel Seifer:
It would splash around. If you wanted to get the most through the hole, you'd have to pour it nice and slowly. And you'll think about the milk, it's like, "Okay." It's a smooth pour, but there's a word for that, laminar. You're creating laminar flow. And laminar airflow means the air, and in this case, the air that is containing the medicine, stays in the middle of your airway and it's not interacting with the sides of your airway as much. And because of that, less medicine gets wasted as it's going on down and it can get to where it needs to go.

And so that whole thing that I just showed you was one puff. You don't go two puffs and breathe it in. That was one puff. And so if an inhaler says two puffs in the morning, you do that once and then you can immediately just do that again. But if you think about this, that's not an easy thing to do, that whole maneuver I just showed you. If someone's short of breath and they're tachypneic, they're never going to be able to do this. And so now, by the time they're not feeling well, it's hard for them to use the inhalers.

Liz Rohr:
Yeah.

Daniel Seifer:
Inhalers function much better as armor than they do as quick relief. They work as quick relief, but the sicker someone gets, the less the inhaler is going to help them in the short term. And that's why nebulizers exist or other people do different things in the hospital.
If we're talking about the light mediums, what would be the guideline driven approach to them? If you think of asthma or something similar to it that would respond to the same medicines, I don't consider them to have failed inhaler therapy until I have told them that in person, I have given them one of the combination ICS LABA inhalers. You don't necessarily need to start with a high dose version of that. Speaking of budesonide-formoterol AKA Symbicort, there's an 80-4.5 microgram variant and there's 160-4.5 microgram variant.

You don't have to start with the 160, you can, but you could start with the 80, especially in the moderates. Two puffs in the morning, two at night. And I say, "Listen, try this technique." It's possible and actually very frequent that within two weeks they're like, "Wow, that's working great. Why'd this never work before?" But it can take up to six weeks to know because the inhaled corticosteroid component doesn't work quick. It takes time. We're talking 80 micrograms. That's 1/1000th of a gram of something like prednisone. It's tiny, and that's not even talking about potency, but tiny, tiny doses. It takes a while for that part to have an effect.

The part that could work sooner would be the long-acting beta-agonist. There are differences amongst the long-acting beta-agonists. Salmeterol was the first one on market. It is the least good of them. The higher quality ones are formoterol, which is what's in the budesonide-formoterol. Also, vilanterol. Fluticasone-vilanterol is the other type of inhaler structure, which is a DPI, which stands for dry powder inhaler. The vilanterol is also one of the higher quality, more potent, quicker acting, long-acting beta-agonists. And they work so quickly, actually, they work almost as quickly as albuterol. Many times now we just give patients that inhaler. We don't even give them this one. And we say, "When you're having an acute attack, you should just hit your Symbicort or your budesonide-formoterol."

Liz Rohr:
Oh, that's so fascinating because I've actually had a lot of people say stuff like that. They're like, "Oh, well, whenever I feel sick, I just take..." I don't like using brand names either.

Daniel Seifer:
Yeah.

Liz Rohr:
But I will say Symbicort, I'll just use that. In textbook, it seems like it's like, "Oh, that should take a really long time," but that makes sense that it's the beta-agonist that's helping.

Daniel Seifer:
Yeah, quickly, very quickly.

Liz Rohr:
Yeah.

Daniel Seifer:
Formoterol is actually pretty quick acting and it's way more potent than albuterol too as a molecule. If you're able to get formoterol down there, and that's the actual problem, it'll have a really beneficial effect pretty quickly. For your listeners, if they want to learn more about this, the current ATS and ERS guidelines have caught up to each other a little bit.

Liz Rohr:
Awesome.

Daniel Seifer:
But ERS does sort of still lead the way. And they refer to this as flex dosing, F-L-E-X dosing of a ICS LABA. And the trials, the first one that sort of started to show this really robust effect in a good way was the SMART trial over in Europe.

Liz Rohr:
That's awesome.

Daniel Seifer:
S-M-A-R-T.

Liz Rohr:
I love that.

Daniel Seifer:
That's worth digging into if people have more questions.

Liz Rohr:
Totally.

I have one follow-up question.

Daniel Seifer:
Yeah, please.

Liz Rohr:
About the kind of mild to spicy level of patient referral. Well, first of all, I want to just thank you and acknowledge you for saying picking up the phone and talking about a case with a clinic.

Daniel Seifer:
Yeah.

Liz Rohr:
So many newer clinicians are like, "I don't want to bother anybody." Cold calling is the way to go and literally every specialist that I bring on this channel verifies that, "Just call us. We'll just talk about it. It's great."

Daniel Seifer:
Yeah.

Liz Rohr:
For those more urgent patients, if you have any pearls of practice about the light-medium patients, they probably should have an appointment because they're sort of doing okay, sort of not, maybe with their asthma or maybe we're not sure about a COPD diagnosis. This comes up with a lot of specialists where it's are there patients who are sent to you that are not appropriate or that you're like, "Wow, I really wish they had done something first," Because I know you're not in kind of, quote, unquote, regular pulmonology at this point.

Daniel Seifer:
Yeah.

Liz Rohr:
But primary care providers really do want to know that. Is this an appropriate referral? What should I have done more? Did I send a late referral? What's something that I missed? Any thoughts about that?

Daniel Seifer:
Great question. And yes, I confess that the answer in your local area may be very different than me. I'm going to try and answer more of the general pulmonologist than the quaternary severe asthma ILD sort of perspective here. Although I'll start with the part of the answer that I think is the same for both. And this is focused on asthma here for a moment.

If you're giving repeated courses of corticosteroids and that goes on for three months, that is too long. It's the severe cases that need to come through quickly. The other ones, they might need an appointment, they might not, but the chances of if asthma is what is on the list, and remember one of the things I was using to class severity was you're noticing that it's getting worse and worse, but if someone's not getting worse and worse over a six-month period, it's probably not one of those things. You're not going to run into a case there where it's like, "Ooh, these specialists wish I'd referred them years ago and something should have been done immediately."

Liz Rohr:
Yeah.

Daniel Seifer:
Making that distinction between medium and spicy, so to speak, is the most important for how quick I should get them and make the call. Are there things that should not be sent in? That's a good question.

Liz Rohr:
Ooh, give it to me. Get on your soapbox.

Daniel Seifer:
Again, maybe some community physicians are going to come running after me with a stick here, but I don't believe that independent people should be responsible for the failures of the American health system. The right time to call a specialist or refer to a specialist is when you're uncomfortable and you have a question. And if training was such, or your environment is such, or the local setup is such that you don't have that option or that you're being punished for trying to utilize that option, that's ridiculous. That's absurd.

We need to be helping people to understand and increasing the comfort level with which patients you keep with you. But if you're starting to feel uncomfortable, that's when it's reasonable to call. I think that the very basics, if asthma's on the differential and patients aren't meeting the severe, is I do think that a trial of something like budesonide-formoterol or one of the other high quality ones gone through in the way we just described with the patient getting the right expectations about six weeks of time and that kind of thing, potentially a basic chest x-ray to look for other mimic pathologies and maybe potentially baseline spirometry, those are reasonable things to do before you're like, "Oh yeah, I'm going to send people on." Because just saying someone has uncontrolled asthma and clicking the box in doesn't help.

Liz Rohr:
Yeah.

Daniel Seifer:
Because did anyone attempt to control it? Do they even have asthma? Now, sometimes those questions may be nobody knows because it's really been a difficult case and the patient may have tried stuff, but we're not sure if it's the inhaler that's not working or if the patient's not using it correctly. But for whatever reason, they keep ending up on all this stuff and the spirometry is abnormal, but it's not reversing with the... Okay, that's referring, but that's very different than a patient coming into your office and them going, "I have asthma and I feel like it's bad," and you asking them questions and you're like, "I don't know if I feel like that's bad. I feel like you cough a couple times." And then putting in an urgent referral to pulmonary for uncontrolled asthma.

Liz Rohr:
Yeah.

Daniel Seifer:
Because then, by the time we're triaging a referral like that, it's like, "Well, I'm going to send that to general practice at our institution, not even pulmonary."

Liz Rohr:
Yeah. Yeah. Thank you so much for saying that. And I really do stand behind that same feeling of there are a lot of challenges in our healthcare system. And I think there's a both/and there, especially for newer clinicians, and speaking from the nurse practitioner perspective is what are the resources we can look at? What is the best we can do in terms of our workup and understanding and collaborating and asking questions and then referring when it's appropriate. That's a felt sense over time, but how can we do our best due diligence first and then kind of tag people in? But I just appreciate you acknowledging that there's a healthcare system that's not really well set up for us.

I guess I'm wondering about where we should go from here. There were a lot of questions about long COVID, COPD, and I know you and I talked about interstitial lung disease and alpha-1 antitrypsin. Don't know if I'm saying that right. It's been a while.

Daniel Seifer:
Yeah, you're saying it correctly. Yeah.

Liz Rohr:
Thank you.

Daniel Seifer:
It's a very different thing than interstitial lung disease, that particular condition.

Liz Rohr:
Totally, totally. Did we talk about that? We wanted to talk about that. I know there are questions from the people about it, but I don't know.

Daniel Seifer:
Interesting, about alpha-1.

Liz Rohr:
Where do you want to go?

Daniel Seifer:
Okay, well, if I get to pick where we drive a little bit, I think I'll drive it to the part that I think that I'm perhaps most enmeshed in day to day, which would be interstitial lung disease slash interstitial lung damage.

Liz Rohr:
Beautiful.

Daniel Seifer:
Because there is one takeaway, and I'll say it now and I'll probably repeat it again as we're talking about this, the only way to know if somebody has interstitial lung damage or not is a high resolution CT scan of the chest. That is the only way to know.

Liz Rohr:
I'm glad you brought up CT scans.

Daniel Seifer:
Period.

Liz Rohr:
Because I really want to talk about that. Go ahead. Go ahead.

Daniel Seifer:
Period. Chest x-ray doesn't do anything. It may have a decent positive predictive value. If you see it on a chest x-ray, okay, great, it's there, but most of the time with an interstitial lung disease or a source of interstitial lung damage, you're trying to answer the other question in a general practice. You're trying to answer, "Do they have it? It seems unlikely, but we should check."

Liz Rohr:
Yeah.

Daniel Seifer:
And the only way then to have a negative predictive value, what's the statistical usefulness of a negative test? That is a high resolution CT scan of the chest. Period. Without that, you don't know if they have it or not. I suppose if they die, pathology, but hopefully not. A high resolution CT scan of the chest is the only way to know. Are they perfect? No. Do they definitely rely on the reading team? Yes. Every single scan that comes through our collaborative interstitial lung disease group, we have actual thoracic radiologists that read these. These are radiologists that have especially devoted their career to imaging of the thorax.

Liz Rohr:
Wow.

Daniel Seifer:
That's what it takes. And so the decision about what type of damage they have and what we might want to do about it, that's actually not just beyond primary care, that's beyond community centers, in my opinion, my strong opinion.

Liz Rohr:
Yeah.

Daniel Seifer:
And it's beyond a pulmonologist most of the time, in my opinion, frankly, too. When we started this collaborative clinic here at our center in 2018, when I say we, my partner in this is Dr. Julianna Desmarais, and she is a rheumatologist. And we both had expressed frustration to one another that the way that physicians or whoever the privileged old white dudes did 100 years ago divided up the body, we divided it up on gross anatomy, pathology. It's like, "Okay, well we cut this big old lump out, that's a lung. This is the heart," but that's nonsensical when we're dealing with conditions that actually affect the entire body.

And then you end up with this incredible problem of care fragmentation where these patients with these conditions that are not only life-threatening, they're probably going to take their life at some point, get bounced around between providers that aren't talking to each other with the wrong things being done and the right things being missed and everything being delayed before they finally get spat out to a quaternary care center. And then maybe the right thing happens.

And we were really frustrated about that and we're like, "Well, we can't control everything. We're not going to just overthrow the government and say that the only thing that matters in America is the correct diagnosis and treatment of interstitial lung damage, but what can we change from our perspective? We said, "Nobody should own this anymore. Pulmonary shouldn't own this. Rheumatology shouldn't own this." The correct owners is a clinic specifically devoted to this in which there's a shared interest of providers from different subspecialties and different disciplines that are committed to seeing these patients and making these diagnoses because one person can't do it all to the best degree that it can be done.

I know we were talking before we went live here, that doesn't mean a pulmonologist can't try. Now I'm talking to general practice pulmonologists, it doesn't mean don't try, but it does mean that for these conditions in particular, there are things that might actually help them if a certain diagnosis is made versus another that you do not have the resources to both diagnose or deliver quickly in a community setting. You just don't have them. You definitely probably have not written for rituximab. And if you have, you haven't done it quickly. You don't have anyone who knows how to watch or manage the side effect of those medications. Prednisone is not a substitute for other types of immunomodulation. And I could just go on and on and on.

And we started with Dr. Desmarais and myself as the two providers that were sort of going to co-see these patients. The patient would come in, we'd both see them, we'd both make a decision. We'd look at the imaging. We'd work in concert with our thoracic radiology team, which at the time was one person that was working with us, and then move on. And now we are... Well, we're bigger. We're a lot bigger. We've got now I think four dedicated pulmonologists, soon to be five, three dedicated rheumatologists, three thoracic radiologists that work with us. And then what has grown off of that from a sarcoid perspective, which is one of these conditions that can cause issues like this, sarcoid is even more notorious for other types of organ involvement. And so for there, we have dedicated provider members from electrophysiology, heart failure, general cardiology, dermatology, ophthalmology, neurology. You just stack, stack, stack, stack, stack.

Liz Rohr:
Oh, cool.

Daniel Seifer:
And that's something we get to do.

Liz Rohr:
Yeah.

Daniel Seifer:
That's the privilege that we have at this institution is having all these experts at easy fingertips. But we can't go out into your community and get these people. We can't just go grab them. And really bad things can happen if these people aren't referred quickly. What is the referral versus not referral about this?

Liz Rohr:
Yeah.

Daniel Seifer:
The high-resolution CT scan. If you get a high resolution CT scan and it really doesn't show there's anything weird going on in the lung, okay, everybody can take a breather for a second.

Liz Rohr:
Yeah, yeah.

Daniel Seifer:
There are differences of course for sarcoid because sarcoid can kill you in other ways through heart involvement or through brain involvement, but if we're just speaking about lung for a second, if you have a clear normal read or close to it high resolution CT scan, you can feel a lot easier. And you can take your time, maybe go to a community center.

If on the other hand someone comes in and says, "I'm feeling short of breath," and you get a high resolution CTT scan of the chest and it either shows interstitial lung damage or it shows pulmonary fibrosis, which I'll talk a little bit more about in a second, that needs to go to a multidisciplinary care center or tertiary referral center and not six months from now. It needs to go. That's where they need to get seen.

And maybe it's like, "Oh, this is minor, there's nothing to do about it." Okay, fine. And maybe this is going to be we're going to be suggesting life altering immunomodulation therapy. We're going to be doing all this. That decision can't be made until the workup gets done and that workup can't get done where the patient is.

Liz Rohr:
Totally.

Daniel Seifer:
The high-resolution CT scan was a big one. I did see that one of the questions related to pulmonary fibrosis. And I knew that we were going to be touching back on some of these things. I'm glad we started with them. ICD-10 codes can burn. They're ridiculous. Pulmonary fibrosis is not a diagnosis. Period. It's not a diagnosis. Pulmonary fibrosis literally means lung scarring. That's what it means. That's like if you said skin scarring. Okay.

Liz Rohr:
There's a lot of ICD-10 codes that will...

Daniel Seifer:
There's a lot of things that can cause skin scarring.

Liz Rohr:
Yeah.

Daniel Seifer:
It's a huge swath. And some of them, like this scar here, was when I slipped with a razor blade being a silly, silly person when I was much younger and I almost cut my finger off slash did cut the fingertip off. There's a scar. Is that a concerning scar? Maybe for my intelligence as a 18-year-old, but it is not a concerning scar now because it's not spreading, nothing is happening with it. It is stagnant and stable. More so than the scarring, it's why? What happened?

Liz Rohr:
Totally.

Daniel Seifer:
If you have a patient, for instance, just being ridiculous, but actually I've seen this, who got stabbed in the chest by a piece of metal during a car accident and six years later they have a minor cough and you get a high resolution CT scan of the chest, HRCT, and you look and there's a band of scar right where all the rib damage had been done. That's a very different pulmonary fibrosis than someone who's like, "Yeah, for the last six months I've been getting a little short of breath, maybe a little dry cough," and you get a scan and you see, someone says, "Well, there's scarring. It's worse at the edges or maybe there's some ground glass opacification. This is making me worried for interstitial lung disease or interstitial lung damage."

You may see these acronyms that people misuse all the time. NSIP or UIP. Those are not diagnoses either. Those are patterns of damage that used to be diagnoses because people were misunderstanding the problem. NSIP standing for non-specific interstitial pneumonitis or pneumonia, UIP, usual interstitial pneumonitis or pneumonia. If you hear any of those things on the scan, it is time, it's time to go. That's something that was worth, I think, just going over and saying, because it kind of answers the question.

The prognosis then of pulmonary fibrosis, it depends entirely on why the scar is there. And you can't tell them because you don't know.

Liz Rohr:
Yeah.

Daniel Seifer:
Someone else is going to have to figure that out.

Liz Rohr:
Totally.

Daniel Seifer:
And really bad things can happen if the wrong things are done to figure this out. Even 10 years ago, people were really advocating that you had to make, maybe 12 years ago, you really had to do a surgical lung biopsy to make a diagnosis like this. You do not, not even close. And in fact, sometimes you go through all of that problem, actually all that trouble, and actually oftentimes it gives you no new information and it has put the patient at significant risk, not just because you've now cut through their chest wall for a video-assisted hysteroscopic surgery. You've done that. You can end up with nerve damage, you can end up with pain. No, because actually anytime you cut something and the body has to heal, you're activating both the scar system and the immune system. And if their problems have to do with overactive fibroblasts or with their immune system, they will flare and get worse because of your diagnostic tests that you did that you didn't even need to do and they might die.

These aren't like, "Oh yeah, mild, medium, spicy for the asthma." Even in spicy asthma, a basic ED could intubate them, put them on a ventilator and they wouldn't die. There are things about interstitial lung disease slash interstitial lung damage that there are only certain places that the evaluations and care can take place. I think it's something that people should know.

Liz Rohr:
Totally.

Daniel Seifer:
But on the flip side, if you've got a high resolution CT scan of the chest and it's not looking concerning, you don't have to be biting your nails being like, "Oh my gosh, did I miss something?" No, it's not there. And if it is somehow there and the CT scan missed it, you've got some long time, you got years before this is going to be an issue. It's really stark, unlike the asthma where it's kind of like a gradient.

Liz Rohr:
Yeah.

Daniel Seifer:
There is a easy identifiable place where you're like, "Got to get this person in, got to make that call," like we were talking about to these bigger centers, versus, "I don't know what this is, why they're dyspneic. Well, they have a little nodule in their lung and maybe there's a little bit of irritation in this upper lobe." That's not interstitial lung damage. That's not scarring. You've got more time to figure that out.

Liz Rohr:
Totally. I have a question. If we work backwards to in the wild, in general practice, let's just bring it all the way back. If somebody sees you in the clinic, what are you looking at when a patient's coming in? What has you suspecting interstitial lung disease? Are they dyspneic, they have a cough, they have a chronic cough, you're doing a chest x-ray that's showing some weird stuff? And then it's like, "Oh, recommended you do a CT scan," and you're like, "Okay, I'm going to do that." Is that the process? What does it usually look like?

Daniel Seifer:
Okay, sure. By the time it's on x-ray, things have progressed. If an x-ray says this could be interstitial damage, the CT should be ordered that day, check it out.

Liz Rohr:
Sweet.

Daniel Seifer:
Because that's the only way you're going to get your answer. The x-ray's just too nonspecific.

I'm going to say something scary here is that this type of damage oftentimes in the beginning is asymptomatic. They won't have symptoms. It can be caught incidentally. It can be they had a CT scan of their abdomen and the bottoms of the lungs showed some interstitial, but the radiologist barely even commented on it. It wasn't even in their impressions because they got sent for a CT scan and whether they had a kidney stone or not and just said, "Oh, there was maybe some lung scarring down there." Well, okay, now a dedicated high resolution CT scan of the chest is warranted to actually follow that up.

Liz Rohr:
I see.

Daniel Seifer:
What types of things though, when it is symptomatic, would you hear? Most of the time it's shortness of breath. Cough is possible too, depending on the etiology of what was causing it. Sometimes cough comes early, sometimes cough comes later. Some types of interstitial lung damage sound oftentimes very allergic. I was exposed to this dust or I was exposed to this fire smoke out here in the West and I've just been short of breath and short of breath. That's when you look. You look with a HRCT and you see what is actually going on in this person's lung tissue.

There are other conditions for whom you should have a higher baseline level of suspicion.

Liz Rohr:
Yeah.

Daniel Seifer:
And I say this because these are the patients that not only have a higher incidence of developing this type of damage, but they're also the ones that we can also do something about to halt it or slow it down. And that's not the case for every source of interstitial lung damage. It's not. What you may have heard of as IPF or idiopathic pulmonary fibrosis, I know I was railing about the ICD-10, not only is that not an ICD-10 code, it's not really a diagnosis like other diagnoses or diagnosis. It's a diagnosis of exclusion.

Liz Rohr:
Yeah.

Daniel Seifer:
And anytime you're making a diagnosis of exclusion, which this is worth talking about because this comes up more than just in pulmonary, and oftentimes general practitioners will be trying to make diagnoses of exclusion however you're doing it. A diagnosis of exclusion is saying, "Well, I see this thing in front of me. It's a fruit. It is not a grape, an apple or an orange. It absolutely is a persimmon." And you're like, "Wait a minute, what do you mean it's absolutely? You said three other things." It's like, "Nope, it's a persimmon." And it's like, "Well, but there are other fruits." And you're like, "I don't know. No one's ever proven it. It's just a persimmon." That's ridiculous. Because you've named and characterized three things, doesn't mean that everything that is not those three things is now the same thing. That's absurd. But doctors don't like saying that we don't know. And the truth is, even though we put it in the name of this condition, idiopathic means who knows? We still talk about it like it's a disease.

Liz Rohr:
Yeah.

Daniel Seifer:
Like it's a problem. You could nicely call it a problem. I call it a spectrum. Remind yourself with the word that you're calling it that this is we couldn't identify the particular other cause, so now you're in this grab bag. And the reality is there's probably eight or nine different problems in that grab bag. And in the long run, we'll find treatments that help some of them and not others. Similar to how with cystic fibrosis, the early CFTR modulators only helped people with certain mutations because now we got down to the very granular, "Why did you have the problem?"

Most of the other treatments people tried for cystic fibrosis and before the modulators came onto the market, they would help in some ways, but they were treating downstream, they were treating everybody as if they had the same problem, which they don't. On a cellular level, they have a different problem. The protein is misfolding in different ways or it's not getting translocated at the surface or the channel isn't developing correctly. And that's what eventually we're going to find with this grab bag that is IPF or idiopathic pulmonary fibrosis spectrum disease as I prefer to sort of say it.

The prognosis varies wildly because most people that have IPF spectrum disease, there's really nothing we can do. There are two medicines on the market, neither one of which has ever been shown to improve mortality, which are pirfenidone and nintedanib. Ooh man, would the companies like to tell you otherwise, but it is, at best, at best, it maybe slows the disease down very, very, very slightly. It's about as far from a home run as you could possibly hit. There are lots of things in trials and in pipelines, but those are accessible through tertiary centers, not through community centers.

Liz Rohr:
Yeah, totally.

Daniel Seifer:
Contrast that though with patients who have autoimmune sources of this damage or allergic sources of this damage. Both things related to the immune system, it's just whether the immune system is targeting something outside you in an allergen, or in the case of autoimmunity, targeting yourself, those things can be stopped. They can be treated. You can do something about it if they're diagnosed and the right kind of team is in place and that team has the facility and the options to use those medicines because prednisone is not enough and it's not sufficient and eventually it will kill you just as certainly as the actual problem might. Or maybe not just as certainly, but it's not good. You can't stay on it for long.

The patients that you may see that have rheumatoid arthritis or have... You probably don't see very many of scleroderma, dermatomyositis, by the time someone has something like this, they're already at a tertiary center most of the time. But there are still definitely community people walking around with rheumatoid arthritis with things like that.

If you start to see someone who has a lot of other complaints, "My joints get big, they get big and they hurt. My skin is getting tighter. I've got dry mouth. I'm always sipping water, 24/7 sipping water. My eyes are so dry. Look at this rash on my skin. My fingertips are kind of cracking at the edges here. I've got these new papules on the back of my hands." I'm not saying that you immediately start worrying about lung disease in those patients, however, if a patient that is saying those things also says to you, "Over the last six months I've been getting a little more short of breath," it's CT scan time and it's referral time because the chances that this patient has a systemic condition that may be affecting these multiple different systems is starting to get high enough that you need to do something about it.

Liz Rohr:
Yeah.

Daniel Seifer:
Prognosis of pulmonary fibrosis, there we go, in the largest nutshell that has ever existed.

Liz Rohr:
Well, I could talk to you for 24 straight hours of asking questions and hearing you. Unfortunately, we do have to wrap up, but hopefully, I know you're a super busy person, but hopefully at some point we can reconnect. I would love to ask you more questions, but what are, I don't know, any sort of parting pearls or practice for our people?

Daniel Seifer:
I'd say I'd love to continue the discussion again as well. I think that SARS-CoV-2 and the symptoms and the damage that can be done from SARS-CoV-2 is a relevant issue that the GPs of the world are taking the brunt of it.

Liz Rohr:
Right, totally.

Daniel Seifer:
And it's worth talking about the fact that there's a lot of misunderstanding about that other too, and we could devote an entire hour just to talking about that for sure.

Liz Rohr:
Totally.

Daniel Seifer:
But pearls of stuff related to what we've spoken about today or in general, I think I'll go back to what you and I spoke briefly about before we went live, which is what I'm about to say is a challenge for everybody, I think it's a challenge for quaternary academic physicians, for the most rural general care practitioners, is knowing what you don't know being one piece of it, but also knowing where you stand about reality of what you can offer and what you can't and what the patient can access and what they can't.

There are going to be times when you are the best available option for three months, for six months, for whatever, because America's health system has failed its populace and there is no center. There is nothing to get them in. It's not your job in that situation. Don't put it on yourself, "I have to be the pulmonologist." You don't. You don't have to be the pulmonologist. You can't. I can't be a surgeon, as we were sort of joking about earlier. I can't do a lung transplant. That just results in a lot of arrests. But we can focus on what we can do.

And in those situations where you are the best option, coming to terms with the fact that you may be trying to deliver care that in retrospect someone else might find out that they could have done it better, that doesn't mean you did the wrong thing. It means you did the best that you could in the situation that you could. But if that's the case, you have to be sure that that really is the case, not just one of those things where it's like, "Well, I clicked a little checkbox and said it'll be six months and that was it." It's like, "No, I tried to call and this patient can't even drive, so where the heck am I going to send them?" Then it's like, "Okay, I'm going to get on DynaMed or UpToDate and I'm going to say, "Could I send some of these other labs first? Could I call a virtual consult line? What can I do? I'm going to try to extend myself if I have the time."

On the flip side, just as important, if you're like, "Oh, this is an interesting case. I don't see many of those. I'm going to spend some time on UpToDate and DynaMed and all these things," and you never think about referral for something that you've never encountered before when the severity may be higher or starting to get worse," that's bad news bears.

And I think what makes these two things so challenging, both for academics and for people, is that we get comfortable dealing with what we see a lot of, and humans like to act in situations where we know the rules and boundaries, but the reality is that that changes patient to patient. The way you may have to act with someone who could drive six hours to a tertiary care center versus someone who can't, that might be completely different and you have no control over that. And it's frustrating. It's really frustrating to be like, "This person, I would like to do this, but they literally can't come to my academic center." We are talking on the phone, they are in Northern California.

Liz Rohr:
Yeah.

Daniel Seifer:
What do you do? It's going to have to be different. And as long as... I'm just speaking from my perspective, in those situations, I very clearly document this. I say, "All the things that I'd like to try, I can't." I've made that clear verbally to the patient. "This health system, America's health system, is a mess. Here's what we're going to try instead as the best available option."

On the flip side, if someone's here and I'm like, "Oh, that's an interesting finding on echocardiogram. Why did they get really bradycardic when we ran this test?" Should I sit there and just page through my phone and be like, "Oh, no, no. What's going on?" Or maybe I got busy, I'll call them back in three weeks. I'm right here. I can throw a rock and hit a cardiologist that has got national qualifications, so I throw the rock. Why would I not throw the rock?

I want to just tell everyone that that's not something that you're alone in. Every practitioner, no matter where they're practicing at, should do that and get good at being uncomfortable and dealing with each situation as it comes. That will serve you more than saying, "Wow, I really need to burnish my knowledge in this one area so I can make up for the fact that there's no cardiologist in town." I could sit here and read books on cardiology for six months. It's not going to make me a cardiologist. Tough.

Liz Rohr:
Thank you so much for saying that so eloquently because it's so impactful, I think, because I feel like I try to tell that to people and it's so impactful hearing from you who's such a thoughtful, bright person. And you really care. I so appreciate that. Thank you so much for giving us your time, and I really would love to talk to you all the time. Thank you.

Daniel Seifer:
I'd be happy to make a return. Thank you for creating this platform. I think it's something that's desperately needed and clearly you're doing such an excellent job with it that there's actual listeners. No one ever listens when I talk, so hey, look, you are the correct tool for this. Thank you for even reaching out to me. I'm honored that you even thought that it was worthwhile hearing from me, and I'd be happy to chat again if we can find the time.

Liz Rohr:
Thank you.