152. Optimizing the Multidisciplinary Management of Fibrosing Interstitial Lung Disease (CE)

On this accredited episode of NP Pulse: The Voice of the Nurse Practitioner®️, join expert nurse practitioners (NPs) Jessica Glennie and Corinne Young for an insightful discussion on fibrosing interstitial lung disease (ILD) — a complex and often underrecognized condition. Learn how to identify early signs, navigate the diagnostic pathway and support patients with emerging therapies and holistic care strategies. This essential program equips NPs with the tools to improve outcomes for patients living with ILD.

A participation code will be provided at the end of the podcast — make sure to write this code down. Once you have listened to the podcast and have the participation code, return to this activity in the AANP CE Center. Click on the "Next Steps" button of the activity and:

1. Enter the participation code that was provided.
2. Complete the posttest.
3. Complete the activity evaluation.

This will award your continuing education (CE) credit and certificate of completion.

1.2 CE, 0.4 RX, will be available through June 30, 2026.

This activity is supported by an independent medical educational grant from Boehringer Ingelheim Pharmaceuticals, Inc.

 

Episode Transcript

(00:14):
From the American Associationof Nurse Practitioners,
I'm the host of today'sspecial edition episode,
nurse practitioner and educationspecialist Patty Scalzo,
and this is NP Pulse,the voice of the nurse practitioner.
Welcometo NP Pulse, AANP's podcast,

(00:36):
bringing you unique nurse practitionervoices and expertise on issues that
matter to NPs and our patients. As always,
be sure to subscribe to thispodcast, share with your colleagues,
and check back often for newconversations with nurse practitioners and
healthcare leaders from across the nation.
NP Pulse podcast listeners may claimCE credit for this program through

(00:59):
June, 2026. After youlisten to the podcast,
visit AANP.org/cecenter.
Register for this activity.
Enter the participation codeprovided at the end of this podcast,
and then complete the post-test andevaluation. Nurse practitioners in the
primary care setting play a pivotalrole in the early recognition of

(01:22):
interstitial lung disease, ILD.
A group of conditions oftenunderdiagnosed in the early
stages. Maintaining ILD in thedifferential diagnosis for patients
presenting with persistent or unexplainedrespiratory symptoms is essential.
On average, there is a 2.1 year delayfrom symptom onset to diagnosis,

(01:44):
a critical window of time during whichdisease progression and irreversible
fibrosis can occur. These diagnosticdelays are associated with diminished
quality of life, emotional distress,accelerated disease progression,
and increased morbidity and mortality.
Enhancing awareness and clinical suspicionof ILD among primary care providers

(02:05):
is key to improving outcomesfor affected patients.
On today's podcast, we'rejoined by nurse practitioners,
Jessica Glennie and Corinne Young,
who will be discussing thediagnostic criteria, pathophysiology,
clinical guidelines, andtreatment of fibrosing ILD.
Jessica Glennie has served as a nursepractitioner in outpatient pulmonary

(02:26):
medicine at the Cleveland Clinicin Cleveland, Ohio since 2015.
She specializes in the management ofpatients within the Interstitial Lung
Disease Program,
providing comprehensive care acrossthe full spectrum of ILD conditions.
Her clinical interests focus particularlyon autoimmune related interstitial
lung disease. In addition toher clinical responsibilities,

(02:49):
Jessica leads a regional support groupfor individuals living with pulmonary
fibrosis in northeast Ohio, demonstratingher commitment to patient education,
empowerment,
and community engagement. CorinneYoung is a nationally recognized nurse
practitioner who has specializedin pulmonary medicine since 2002,
driven by a passion for advancing therole of advanced practice providers, APPs,

(03:13):
in pulmonary care.
She founded the Association ofPulmonary Advanced Medicine Providers,
APAPP,
a national organization dedicated tosupporting APPs in pulmonary critical
care and sleep medicine.
Corinne's influence extendsacross national platforms.
She has served on the AmericanBoard of Internal Medicine's.

(03:34):
Pulmonary Disease Board,
is a section editor for Chest PhysicianMagazine and contributes to multiple
chest networks. Most recently,
she has been appointed to the 2025Chest Conference Program Committee
further demonstrating her commitment toshaping the future of pulmonary care and
education. It is my pleasure towelcome our nurse practitioner experts,

(03:56):
Jessica and Corinne.
Thanks so much for that, Patty.It's great to be here with AANP.
My name is Jessica Glennie.
I'm a nurse practitioner at theCleveland Clinic in Cleveland, Ohio.
I work with the interstitial lung diseaseteam there and I'm joined here with
Corinne Young. I'll throw itover to Corinne to say hi.
Thank you Jessica Corinne Young,

(04:18):
nurse practitioner for ColoradoSprings Pulmonary Consultants.
I'm in general pulmonology and thepresident of the Association of Pulmonary
Advanced Practice Providers.
So today we'll be talking about optimizingthe multidisciplinary management of
fibrosing interstitial lung diseases.
We'll be talking aboutthe clinical context.
We'll be talking a bit about diagnosisand the guidelines surrounding how to

(04:38):
diagnose these diseases,
and then we'll be talking a little bitabout therapeutics and new therapeutics
on the horizon.
So first I'll be talking a bit aboutthe epidemiology, pathophysiology,
and patient burdensurrounding these diseases.
So interstitial lung diseases havebeen increasing in prevalence.
There are about 71 casesper 100,000 people.

(05:01):
It's also thought that the mortality ofthese diseases has been increasing and
we may see insignificantincrease in mortality by 2027 and
interstitial lung diseases,
about one third of all ILD caseswill have a progressive phenotype.
So it's really importantthat we are monitoring these

(05:22):
patients to see if they're going to havea progressive phenotype so that we can
get them on proper management.So these diseases
are characterized by two hallmark drivers,
fibrosis and or inflammation.
So for the most common causeof interstitial lung disease,
which is idiopathicpulmonary fibrosis IPF,

(05:45):
this is driven by fibrosis only.
We see no inflammationin that process. However,
for a lot of the other interstitiallung diseases caused by other things,
inflammation is a big driver ofthose in particular are autoimmune
disease related ILDs. Things likerheumatoid arthritis, scleroderma,
sjogren's, those can have a lot ofinflammation, especially at the outset.

(06:10):
Inflammation uncheckedover time can lead to
stimulation of that fibrotic processwithin the lung fibrotic process.
Fibrosis in the lung itselfcould be self-perpetuating.
We actually know that if we take afibrotic cell and put it next to a normal
cell in a Petri dish,
that fibrotic cell will tell thenormal cell to become a fibrotic cell.

(06:33):
So once there's fibrosis there,
it can become self-perpetuating.So we want to kind of look at both of
these processes,inflammation and fibrosis,
and make sure that we'retreating both as necessary.
There are a number of profibrotic andpro-inflammatory cascades in these
diseases. Some of them includeimmune-mediated inflammatory cytokines,

(06:56):
TGF-beta, PDE4B, phosphodiesterase 4B.
These are some of the different targetsthat we are utilizing or have utilized
in some of our therapeuticsfor these diseases.
So now I'm going to talk a little bitabout the burden of disease in these
patients.
These patients have a reduction in qualityof life and their daily functioning.

(07:21):
Fibrosing ILDs, in particularidiopathic pulmonary fibrosis,
are chronic, progressiveand ultimately fatal.
These patients can havea loss of about 150 to
200 mLs of their forced vitalcapacity over the course of a year.
Forced vital capacity is howmuch air those lungs can hold

(07:46):
and if there's more scar tissue, ifthere's more inflammation within the lung,
the lungs become more stiff. It's hardfor that patient to hold as much air in
the lungs.
So we can really use thatFVC to follow along our
patients and know if they'restable or know if they are
progressing. What are some ofthe signs and symptoms? Well,

(08:08):
some of the signs and symptomscan be very similar to some very
usual pulmonary diseases thatwe see or cardiac diseases.
Patients can havechronic cough in general,
this is a dry cough.
They can have exertional dyspnea andwhile sometimes exertional dyspnea can

(08:29):
be very acute in onset,
oftentimes the story that we getis a patient comes in and says,
I was starting to feel more and moreshort of breath, just slowly over time,
kind of an insidious onset of thisdyspnea. I didn't think much of it.
I'm getting older, maybe I'm out of shape.
But finally it reaches a point where it'sreally affecting their daily life and

(08:52):
maybe their ADLs. So they comein to see you. These patients,
especially in later stages oftheir interstitial lung disease,
may be hypoxemic,
so they may require supplementaloxygen and as referred to
above, they will havereduced lung function,
a decrease in their forced vital capacity.

(09:14):
In the case of patients with IPF,idiopathic pulmonary fibrosis,
the median survival withouttreatment is about two to five
years. And this can besimilar for those non IPF,
progressive fibrotic patientswho have this fibrosis
that's moving along and especially onesthat have a certain type of pattern on

(09:37):
their skin, they can havea similar poor survival.
So now I'm going to kindof throw things over to
Corinne. Corinne,
I know you said you work in generalpulmonary and we talked about how
the prevalence of these diseaseburden has been increasing.
Have you been seeingmore of these patients?

(09:59):
Have you been seeing increase in diseaseburden in your own practice recently?
I have and where I practicein Colorado Springs,
we have a lot of military basesand that then comes with a lot of
veterans and we do know with IPF,
although the underlying causeof the disease is idiopathic,
we do know that a lot of thoseIPF patients have some sort of

(10:23):
exposure in their lifetimesmoking or occupational exposure.
So we know a lot ofthese vets had exposure.
So I do feel like I see a lot morein my Colorado practice than I did in
my California practice previously. Also,
I think we're getting betterat identifying it over the years as we've been
practicing,
imaging slices have gottensmaller on CT scans, right?

(10:47):
I know when I started I think there werelike eight or 10 millimeters and now
they're down to 1.25 or smaller,
and so we're gettingfiner cuts of lung tissue,
which is identifying I think a lot ofearly reticulation and changes on CT
imaging that is maybe beingcaught in primary care,
maybe even being caught on otherscreening CT exams that are being

(11:09):
done of the chest or maybe even we'veeven had patients diagnosed because
they've had a scan of their abdomen andthe bottom portions lung windows are
captured in those images where theymight note some fibrosis or interstitial
changes that then get 'em to pulmonary.So I do feel like I'm seeing more
patients and the question always is,
is there more disease than there hasbeen or are we just getting better at

(11:31):
identifying it sooner? Right?
Yeah, I love that you mentioned just maybewe're getting better at finding these
diseases.
I always think maybesince we've started having
some treatments forthese starting in 2014,
the education surroundingthese has really grown a lot.

(11:53):
So I think there is something to that.
We're seeing some increase in incidentsjust based on the fact that we've got
better understanding of these,we're looking for these more,
we're finding these more often.
So I love that we talked a little bitabout the FVC and how important the
FVC is.
Are you getting spirometry on all thesepatients and do you find that that's a

(12:14):
helpful measure for you in yourpractice looking at the FVC
and monitoring that?
And are there other things that youlook at that help you to know if your
patient is stable or progressing?
Absolutely.
We are lucky enough to have apulmonary function lab in our
office with a full-time respiratorytherapist. And then of course,

(12:34):
just having the one PFT machine,we do get booked out a bit,
so we do also utilizethe hospital system PFTs.
These patients definitely getspirometry as soon as we meet them.
We try to get a full pulmonary functiontest where we can get FEC total lung
capacity or TLC and diffusioncapacity or DLCO so that we can really

(12:55):
get a good idea of how muchrestriction these patients might be
having. I tell them looking at theirCAT scan is like looking at a car.
It could have some dings and scratches,
but it doesn't necessarily tell us abouthow the engine's running or how much of
what we're seeing is impacting theirlung function and it can go either way.
I'm sure you've seen that too,
where their imaging may not lookterrible but their lung function is or

(13:18):
vice versa.The imaging looks pretty bad,
but you're kind of surprised byhow good their lung function is.
We do like to do this seriallyso that we can see trending for
decline. We know there is a predictedamount of FEC decline anywhere between
20 mls to 50 mls depending on the patient,
their aging process, thattype of thing. However,

(13:40):
we know with a lot ofprogressive fibrotic diseases,
they can lose a lot more in a year.
You mentioned earlier ahundred plus mls per year,
so we definitely want to make surewe're monitoring for that on their
FEC. Diffusion is important to me,
diffusion capacity only because weknow this is a disease out in the

(14:00):
interstitium and impactstheir diffusion of gas across
their air sacs. And so if I startseeing their diffusion decreasing,
if I haven't already,
I want to make sure that I'm doingwalk tests on these patients.
Are they desaturating when they're active?
They may come into the office and sitdown and they're not using very much gas
sitting there and theirsaturations may look very normal,

(14:22):
but you get 'em up and walk 'emaround and they're oxygen saturations
definitely drop and a good indicatorto start them on oxygen therapy at that
time. But sometimes your diffusionmaybe your first key to like, oh,
this is dropping.
This would be associated with theircomplaints of increased dyspnea and are we
at a point where we need tostart oxygen in those patients?

(14:42):
Yeah, I love that you mentionedespecially the diffusion capacity.
I think that's as importantas the FVC and we have to
remember that we have toput everything together,
not only the spirometryand the diffusion capacity,
but with the symptoms withtheir walk as you mentioned.
Are they requiring oxygen?

(15:03):
We're kind of being sleuths and puttingthis all together to decide if things
are getting worse. I love your analogyabout the engine. That's great.
And then I get so manyquestions from a primary care
providers about the PFTs.
I don't know if you have any goodtidbits for them on how to read

(15:23):
PFTs.
I tell them if the PFT does tellyou what the lower limit of normal
is, kind of take a look at whatthe absolute number is on that FVC.
It isn't below the lower limit of whatis normal, then they're restricted.
But that's just my verysimple way of putting it.
Sometimes it takes a little bit of lookingat those to really understand where

(15:44):
they are.
It definitely does. I mean understandingpulmonary function testing,
you have to be a littlebit of a physiologist.
You have be a respiratorytherapist, you have to,
there's a lot to it and if any of youlistening have not done a pulmonary
function test,
I highly recommend you do so thatyou could see the level of effort
that we require from these patients.
You can really understand howhard these tests are for them,

(16:06):
gives you a little sympathy.They still need to have it done,
but you could empathize alittle bit more with them.
I think I almost simplify it a littlebit more by just letting them know 80%
of predicted is what we're shootingfor, so 80 is what we're looking for.
The FVC should be 80% or higher.The TLC should be 80% or higher.
The DLCO should be 80% or higher. So ifyou can't remember anything else, 80%.

(16:31):
And what can be a little bit trickyis that you could have patients with
terrible interstitial lung disease,
fibrotic lung disease that's just kindof starting and their FVC might be
94 and that's okay, we're trendingit. That's the most important thing.
So if they're 94 this time and in threemonths they're 90 and in another three
months they're 87,

(16:51):
this definitely tells you a story thatalthough their FVC is in a normal range,
they're progressing, they'refalling off the cliff.
We need to create a safety net, atreatment, a plan for these patients.
Yeah, absolutely. I lovethat you mentioned that.
I mean if somebody has FVC of80%, technically that's normal,
but maybe 10 years ago or only fiveyears ago or only one year ago,

(17:13):
they were at 105%, so that doesn'treally tell the whole story.
Absolutely. And I love the 80%that's much more simplified.
I'm going to utilize that. Okay,
so what have you seen with respect tothe burden that this puts on patients
maybe physically,emotionally in your practice?

(17:34):
It's a big burden. Unfortunately,
patients do go online andthey see that they have
anywhere between oneand three year survival.
They don't quite understand that there's220 different types of interstitial
lung diseases or more actually.And we are, like you said,
putting these puzzle piecestogether of what type do you have.

(17:56):
If they see fibrosis listed ontheir radiology report anywhere,
a lot of times they presume they haveIPF and there's a lot of old data out
there saying one to three years.
And we do know with treatment thatis lengthening dramatically for those
patients. And these therapies nowhave been on the market since 2015,
so we've got a decade of usage out ofthem plus patients who were in trials

(18:18):
prior patients are living 10 plus yearson therapy so far and we're going to
have more information as that goes on.But patients come in very
heartbroken already because they'vedone their own investigation or they've
heard from another provider who may notknow as much about interstitial lung
disease and have received very bad newsbecause this disease tends to be kind

(18:40):
of insidious in itssymptoms like you mentioned.
I just think I'm out of breathbecause I'm aging or gaining weight or
deconditioned. My clinic's atover 6,000 feet in elevation,
so everybody blames it on theelevation. That part's insidious.
But then once the disease reallyis fulminant and present and we
can see it on imaging and seeabnormal lung functioning,

(19:00):
it's pretty sudden andalarming for these patients.
Almost like a cancer diagnosis.They're like, oh my gosh,
this came out of nowhere and nowI'm dying of this lung disease.
And we do have to have some of those veryhard conversations with them about it
because it is irreversible.
Fibrosis is irreversible and havingthat conversation with them and
understanding the only way to get ridof this fibrosis is a lung transplant

(19:25):
or we take medications totry to slow it down or maybe
halt progression if we're lucky.It's a really tough diagnosis,
really scary information's out therefor them and prognosis is not great.
We have kind of limited treatmentoptions for them that are considered
aggressive.
Yeah, I think these patients,

(19:45):
especially the emotional burdenfrom beginning to end is really
difficult.
Trying to provide that goodeducation upfront sometimes is hard.
They may not understandat that first meeting.
I know sometimes for somebody that Ithink needs to go over things again,
I might do a virtual visit with them amonth out after starting a new treatment

(20:08):
or talking to them about their diagnosis.
I know there was a patient that I hadrecently this week who sent me a message
saying, I'm divorced. Myadult kids, they're near,
but I don't have anybody athome to talk to about this.
And the emotional burden of just goingthrough this alone was really hard.
I've had people in my support group say,

(20:31):
especially when it's earlier on,
this is almost an invisible disease.People look at me, I'm not wearing oxygen.
I'm not really huffing and puffing oranything yet while I'm walking and they
think I'm making it up that I'm notreally sick. And so I've heard that a lot
from patients too. And thenall the way to what you said,
once it becomes much more advanced,

(20:53):
they're really strugglingjust doing their daily living,
they're shopping,
all of those things that youdo at home have become really
difficult. So it's really anemotional journey for them.
I really try and get themhooked up with resources.
So I don't know what yougenerally use Corinne,

(21:14):
but I love the PulmonaryFibrosis Foundation. I tell my patients to go there.
They have now it's pretty new,
but they have kind of like a course fornew patients have been newly diagnosed
to go through and it teaches them aboutpulmonary fibrosis and the treatments.
They have pulmonaryrehab exercises on there.

(21:35):
They have support groups,they have journal clubs.
It's just an excellent resource for them.
And I would say for ourlisteners in primary care and even in general pulmonary,
they also have on therethe care center network,
meaning these are kind of vettedspecialized ILD centers where you can
find an ILD center that'sclosest to you or your patient

(21:59):
and get them sent for specialty care.
So Pulmonary Fibrosis Foundation I thinkis a really good one. I don't know.
Have you used anything else Corinne,
with your education or thingslike that with your patients?
Yeah, the Pulmonary FibrosisFoundation, the best,
and you can even send away for brochures,
which is great little things for them toread because some of them are not using
the internet to go to their website.

(22:21):
The other one that I like is theAmerican Thoracic Society has a patient
information sheet on ILD and Ithink one specifically on IPF.
So that's a great one to hand out tojust again for them to have something
visual to take home andunderstand a little bit better.
There's links and resources on thatpatient handout that they could access if
they do use the computer. Ithink those two are great.

(22:45):
And then the American Lung Associationhas a Better Breathers Club and a lot
of towns have a local chapter and theydon't necessarily have to have COPD.
It could be any underlying lung diseasewhere they could just get together with
other patients or have respiratory issues.So that's always nice to have that
group, pulmonary rehab, which I'msure we're going to talk about later,
is a great place to send them also becausethey get the education and they get

(23:08):
to meet other patientsin that environment.
So I think that there's a lot tobe said for those interactions with
others suffering as well.
So Jessica and I were talking previouslyabout the burden incidents prevalence
of this disease,
and I'm going to walk you through alittle bit more talking about when to be

(23:28):
suspicious for these patients,
talking about guidelinedirected diagnostic pathways and also about the need for
early and accurate diagnosis of thesepatients with fibrosing interstitial lung
disease.
So early diagnosis is very important ifwe're going to really try to push this
home to you mainly because lungthat is fibrosed is lung that is
lost.
We can't unscramble an eggis what sometimes I tell patients when they want to

(23:51):
know, is this reversible?Is it curable? Not yet is my answer.
Technology is always changing.Medicine is constantly moving forward.
We're having more and more informationabout fibrosing lung disease in regards
to genetics that might beplaying a role as well.
And all sorts of genetic mutations andmodifications now that we're even looking
at in other lung diseasestates using CRISPR technology.

(24:13):
So who knows what's tocome in interstitial lung disease, but at the moment,
unfortunately it is irreversible,
especially specifically whenthere is fibrosis present.
So we want to make sure that we arediagnosing these patients early for that
reason. However,
most patients take about 2.1years or more to be diagnosed

(24:35):
and part of that reason isbecause their symptoms are
very ambiguous. They havecoughing that Jessica mentioned.
They've got some shortness of breath,they have some activity, intolerance,
fatigue. This could be coronary arterydisease. This could be heart failure,
this could be COPD or asthma.This could be fill in the blank.

(24:57):
I mean there's so many other conditionsthat we may think about and unlike
COPD, if your patient has a historyof smoking, you might think, oh,
I'm going to screen you for COPD.
There's really not a screening forinterstitial lung disease unfortunately,
until the patient becomes symptomatic.
Oftentimes we might get lucky and someinterstitial processes may be found very

(25:17):
early when the patient is havinglung cancer screening scans or I
mentioned before, a scan of their abdomen,
but it catches the bases of their lungswhere a lot of fibrosing lung diseases
like to be,
and that might be an early cue tofind something before the patient's
symptomatic.
But that ambiguity of symptomsis definitely something that

(25:37):
prohibits those patients who arebeing identified early. And again,
we don't have great screeningprotocols for these patients either,
so that also does not allow us tograb them earlier. And so far there's
not a test either a screening bloodtest or serum test biomarker or
genetics that we have as of yet to tellus that your patient is more likely to

(25:58):
develop a fibrotic lungdisease in the future.
So we unfortunately have towait until they're symptomatic.
Some other things that can beassociated with diagnostic delays is
their quality of life starts to suffer,
which then of course affectstheir emotional wellbeing.
These patients were avidgolfers or playing tennis or they were doing something

(26:18):
very active that now theycannot participate in because of their symptoms and
maybe with some pulmonary rehab,
maybe with some medications wemight be able to get them back,
but they're definitely not going to getback to their baseline that they were at
previously. They also, again havethat increased disease progression,
specifically that fibrotic change in thelung tissue and then we're increasing

(26:39):
their morbidity and mortality. Thelonger we let this disease go unchecked,
the higher their morbidityand mortality is.
And we kind of think of interstitial lungdisease almost like a disease that is
like a bomn with a timer,once this disease starts,
they are on a clock and how long we canget that clock to stretch out is what
we're trying to do with treatment inthese patients. But unfortunately,

(27:02):
if we let a lot of that time run outbefore we actually see them diagnose them
and start treating them,
they're already on a shortenedamount of time for treatment.
So really affects theirmorbidity and mortality.
As far as clinicalsuspicion for your patients,
who would you suspect in primarycare patients who are having
coughing, breathlessness?Again, that could be anything.

(27:25):
Do your due diligence.
This patient has a really high suspiciousindex for coronary artery disease
based on their family history andcalcium scoring and all of those things.
Absolutely, you're going to wantto chase that down, but I'm biased.
Coming from pulmonary medicine,
everybody should have an x-ray at leastsome sort of chest image if they're
breathless. And I think we're going totalk a little bit more about imaging,

(27:48):
but x-rays unfortunately may not catchfibrosing interstitial lung diseases
until they are pretty progressed. However,
CAT scans can catch thempretty early. So imaging,
again being very importantin these patients.
Other symptoms that might make yoususpicious that they have at interstitial
lung disease is inspiratorycrackles or rails.

(28:09):
They tend to be in the bases.They tend to be on inspiration.
You have to listen very well on the skinbecause if you've got a patient with a
hairy back, you're goingto hear lots of crackles,
especially if you're puttingit on top of their shirt.
A lot of materials can crackle and youmay not be able to hear it or rub against
your stethoscope. So highly recommendlistening on the skin for inspiratory
rails. That's always ahallmark sign in patients,

(28:31):
again who have already laid downfibrosis in their lungs early on
before fibrotic disease is there. You maynot hear any crackles or rails at all,
however you are hearing them. Again,
that's a suspicion for interstitiallung disease specifically with fibrosis.
Other things that can cause crackles.
We think about heart failures andpneumonia and things along those lines.

(28:51):
So again,
you're going to go with your highestlevel of suspicion if other comorbid
conditions are happening at the same time.
But your patient who doesn't have anyother comorbid condition with inspiratory
rails, breathlessness and coughing,
definitely that should raise somered flags that this patient is likely
possibly has a fibrosing lung disease.
They also tend to haveunexplained patterns of bilateral pulmonary fibrosis on

(29:15):
their x-ray. It does show upon an x-ray or their CT scan,
so it doesn't tend to be just the rightlower lobe with no disease in the left
lung at all. That could be all sortsof other things that are causing some
fibrosis or damage or injury.
Insult infection can causesome damage unilaterally,
but generally with progressivefibrosing diseases,
it's going to impact both lungs. Again,

(29:37):
those basilar inspiratory crackles onexam are a big cue for you and definitely
want to have a high level of suspicionin patients who are 60 years old or
older. So this is not a diseasewe're going to see in our 20,
30-year-old patients. Generallyit's going to be 60 plus. However,
there are some patients whomay have a familial history and
we sometimes will see earlierdisease presence around 40 to late

(30:02):
fifties in those patients,
but definitely not something you'regoing to be thinking about in your
20-year-old patients. Definitely 60 plus.
There are some guidelines outthere to help us with a diagnostic
algorithm for these patients.In 2022, ARS,
ERS, JRS and ALAT all got together and

(30:22):
created a expert consensusdocument talking about how
to look at diagnosticcriteria for these patients.
Also,
multiple disciplinary team-baseddiscussions are also a great option.
I know I'm in community practice inprivate practice and we do not have an
MDD or an MDC forinterstitial lung disease.

(30:45):
So this is really where Jessica comesinto play at Cleveland Clinic in an ILD
center, a tertiary care centerthat is just seeing ILD patients.
I'm sure she's going totalk a little bit more,
or at least we'll ask her somequestions about her MDD or MDC
involvement in interstitial lung diseasepatients. But it's great because again,
as I mentioned previously,
there are so many different typesof interstitial lung disease.

(31:06):
These MDD really help usunderstand what type it may be,
which may help us understand better waysto treat them and also prognosis for
those patients. When we look atthe primary care recognition,
we want to make sure that it is beingrecognized early in primary care,
even if it's just a suspicion you're notquite sure if there is really something
there or not. We want you torefer them as soon as you can.

(31:28):
It's never too early. I'msure Jessica will echo that.
It's never too early to see them.
We'd rather see them and it be nothingthan see them very late in the game.
I think another hurdle for primarycare is sometimes radiology will
list chronic interstitial changesin the lungs and it may give a
false sense of security that this, oh,this is chronic, it's not anything new,

(31:51):
and that does not necessarily mean thatthey don't need to be seen or that this
patient's not in danger. Sodon't let that fool you there.
We definitely want to collaboratewith our primary care providers,
our pulmonologists. Sometimeseven we think about aerodigestive,
we get GI involved.Jessica mentioned earlier,
rheumatology often gets involved withour patients who have scleroderma or they

(32:12):
have other rheumatological conditionsthat can impact their lungs.
And so that is one of the nicethings about that MDD is that it's a
multidisciplinary approachto those patients. Now,
the most important testing, wetalked about lung function earlier,
is a high resolution CT image.
There are lots of articles out there andYouTube videos and things that you can

(32:36):
watch looking at what interstitial lungdisease looks like on a plain CT film
versus a high resolution CT.
And oftentimes we can pick up a lotmore disease that can be missed on a
regular CT, on a highresolution CT. It's finer cuts,
thinner slices of lung tissue thatwe can see and definitely is the

(32:56):
go-to for diagnosis of these patients.And
again, we want to make sure we aretrying to figure out exactly what type of
interstitial lung disease it is.
And so that's where these MDDsdefinitely come into play.
Now when we're talking aboutthe difference between IPF,
which is idiopathic pulmonaryfibrosis and progressive pulmonary

(33:19):
fibrosis, PPF, they soundso close, don't they?
But they really arelooking at two different
buckets of fibrotic lung diseases.
IPF that I being idiopathicis a disease where we
have ruled out all other causes,
sources of what could possibly becausing fibrosis in this patient's lungs.

(33:42):
IPF is generally progressive as well,
but it is its own disease that we do havea lot more information than we've ever
had before on it. Still more weprobably don't know about it than we do,
but is definitely its own diseasestate, progressive pulmonary fibrosis.
That family of lung diseases canencompass a lot of everything else that we

(34:03):
can identify an underlying cause,
whether it be a hypersensitivity,fibrotic hypersensitivity,
pneumonitis that we may see with patientsowning birds or a patient who's had
a drug reaction that is triggeringa progressive fibrotic process.
Patients who have autoimmune disordersthat are what we used to call connective

(34:24):
tissue disease related interstitial lungdiseases that are driving that fibrotic
marker in the lung tissue.
We can identify basically theunderlying cause in the PPF patients.
We do define two of the following eventsoccurring in at least 12 months with
these PPF patients that they haveworsening respiratory symptoms and

(34:44):
or physiological evidence of progressionthat's measured in the decline of their
FEC that we talked about alittle bit earlier of 5% or more.
That would be a sign that this isprogressing and or radiological
evidence of progression. So wehad maybe subpleural disease,
now we're getting lobar disease or it'sprogressing into other lobes where it

(35:05):
may not have been before.
Two out of those three or all three woulddefinitely be an indicator that this
is a progressive fibrotic disease.
Other longitudinal monitoring thatwe might do in these patients,
again as a role for primary careis that we definitely want you
alerting pulmonary services if thesepatients are having increased symptoms,

(35:25):
if they've been hospitalizedfor a respiratory worsening of
symptoms because oftentimes this couldbe a flare or an exacerbation of the
underlying interstitial lung diseasethat could be misinterpreted as a
pneumonia, it could bemisinterpreted as bronchitis,
it could be misinterpreted as a lot ofother overlapping respiratory conditions,

(35:48):
but really could be a flare.So pulmonary practices, Jessica's clinic,
the ILD clinics,
we definitely want to know if you knowyour patient has been admitted or is
having worsening symptoms.
So definitely something wewant to collaborate with primary care and the purpose
behind that is it definitely helpsus survey their disease progression,
helps us identify thoseacute exacerbations that might be mislabeled by those

(36:10):
not working with interstitiallung disease patients. And again,
evaluate them early, to talk abouttreatment and those treatments,
safety and efficacy andif they are on treatments,
monitor for side effects and howthey're doing with therapies.
So modalities of how we do thatare centered around, again,
watching their signs and symptoms,
looking at serial pulmonary functiontesting and six minute walk tests or

(36:33):
exertional testing tocheck their oxygen levels.
If you're not able to do a formal sixminute walk test in your office. And
again, we do repeat CT imaging anddepending on who you speak with,
sometimes it is a serial follow-up andsometimes it is a reactive follow-up of
CT imaging based on patient's symptomsor changes in symptoms or changes in

(36:54):
lung function that may triggerthen a new high res CT image.
So now that we've gonethrough all of that,
I'm going to pull Jessica backinto the conversation. So Jessica,
when you are seeing your patientswho are developing a fibrosing ILD,
what kind of practical tips do youhave or pearls for the audience on
clinical suspicion? What makes youthe most suspicious of those patients?

(37:17):
Yeah, thanks Corinne. So I thinkyou hit on a lot of those things,
but thinking about somebody whomight be in the primary care
area, seeing a patient,
I certainly think whenyou talked about those
basilar inspiratory crackles kindof sounds like Velcro or I've heard
people describe it as like Rice Krispies,

(37:39):
just that crackling down atthe bases on inspiration,
that should automatically kindof put your suspicion up. Yes,
you can hear that with other things,
but that is really a hallmarkof fibrosis within the lung.
Another thing is that a chestx-ray is not a diagnostic modality
really for these,

(38:00):
but it can raise suspicion ifsomebody is having shortness
of breath. You get a chest and yousee the radiologist says increased
interstitial markingswithin the lungs that may be
pulmonary fibrosis. Andthen if you have a patient,
you talked a little bit Corinne aboutthese diseases really can act like other

(38:24):
more common diseases. Well,
if you have a patient thatyou've kind of worked up well,
you've ruled out cardiaccause of their symptoms and
maybe you've tried treating themwith an inhaler, for example,
for COPD or asthma and nothing's working,
maybe thinking about goingonto the next step and

(38:45):
trying to get some PFTsor maybe even a CT.
But I think those crackles andmaybe even that chest x-ray
might help guide that primarycare provider towards that
diagnosis.
I'm glad you mentioned that increasedinterstitial markings on CT imaging
could be fibrosis because I think somepeople have it in their mind that there

(39:09):
has to be honeycomb for it to be fibrosis.
And I think that's a great distinctionthat you just brought out is that it
doesn't have to be honeycombingthat's kind of late stage,
so it doesn't have to behoneycombing to be fibrosis.
Yeah, that's absolutely right.
Really it starts as just muchmore faint kind of markings,

(39:29):
things we call reticulationswithin the lungs.
And I think that sometimes getsignored. Reticulation is noted.
It could sound very passive by theradiologist who might be interpreting it.
I encourage everyone to lookat all of their imaging.
I love looking at CAT scansand I know I've got problems,
but I really like looking at CAT scans.

(39:50):
I don't want to say youcan't trust your radiologist,
but sometimes you can't trust yourradiologist and you really need to look at
those images and I do encourage youto look at them if you're not already
looking at them. Tell me alittle bit about your MDD or MDC.
I have an MDC for lung nodules,
but I don't have one forinterstitial lung disease.
So tell me how that works for you.
So I can tell you how itworks at the Cleveland Clinic.

(40:11):
I know from talking to otherinstitutions with these multidisciplinary
conferences that they can runa little bit differently in our
facility. We get togetherwith all the pulmonologists,
our thoracic radiologists, one ofthem, one of our thoracic radiologists,
one of our thoracic pathologists, andthen sometimes rheumatology, nursing,

(40:33):
respiratory therapy.
We all come together at this conferenceto talk about difficult cases.
Now at some places they talkabout every single patient,
they come together and theycome to a consensus diagnosis.
We just have too many patients,we can't talk about that many.
So we'll come together withour difficult patients.
Patients were having troublewith diagnosing patients that were having trouble

(40:56):
with deciding on treatment or maybethey're not responding to treatment,
we're bringing them back to the MDDand we'll talk about those cases. The
clinician will kind of go over the case,
the symptoms when thingsstarted, their autoimmune,
serologies, blood work, things like that.
The radiologist will then go over allof the imaging and then the pathologist

(41:17):
goes over the pathology if we have it.
And then we all come back togetherand we try to come to a consensus.
I would say more than half the time wecome up with undetermined because coming
there with the most difficult cases,
but it really is a great
intellectual kind of meetingof the minds to really help

(41:39):
manage these patients.
And so I hope that we'regoing to be able to roll
these out more so for places that don'thave them, maybe like your clinic,
Corinne,
it would be wonderful if we could makethese available to other people in the
community.
I think that'd be great.And I know some MDCs,

(42:00):
if you're credentialed at that facility,you could go because HIPAA, right?
They're sharing a lotof patient information.
But if you're credentialedat that facility,
sometimes you might be able toattend. So I would say, look,
if you're part of a hospitalsystem, university system,
you might want to see if there is a MDDor MDC that you could attend. I agree.
I think the educational content is great.
You get to learn from other disciplineshow they're looking at the disease.

(42:22):
It's a whole nother perspective tolook at. So if you have a chance,
I think everybody should go.
So we talked a little bit aboutthe 2022 consensus diagnostic
algorithm that came out. Do youfeel that's being practiced?
Is that being utilized where you are?
Do you think it's being practicedout in community? Why and why not?
Yeah,

(42:42):
so the new guidelines specifically forprogressive pulmonary fibrosis for non
IPF patients, we might be alittle bit more loose about that.
We definitely use it as a goodguideline if two of those three,
either clinical signs and symptoms,
their pulmonary physiologywith their FEC and or their

(43:03):
radiology. So it shows progression.
We definitely utilize that to guide uson whether we're going to start treatment
for their fibrosis.
But we always have to take into contextthe patient sitting in front of us.
Sometimes it's justnot a good fit for that
patient. Sometimes it's apatient-centered discussion,

(43:24):
they don't want to start treatment.So there's a lot that goes into that.
It's not just guidelineson a piece of paper.
There's always patient discussionand patient input into that.
We definitely are followingfor IPF guidelines where we're
utilizing a thoracicradiologist to read our scans.

(43:45):
We are getting autoimmune serologiesto rule out autoimmune causes.
We're doing a really detailed historywith the patient to make sure that there's
no other causes, drugs oroccupational causes, things like that.
And if needed,
taking to our multidisciplinarydiscussion and if needed getting a
biopsy. But generally, yes, generallywe follow those guidelines pretty well.

(44:10):
What about you, Corinne? Howdoes that work in your office?
I know it's not a specialized ILD office,
so I'd be interested in howthat works in your office.
I would say it depends on theprovider you're seeing in general
pulmonology and we've gota pretty large practice,
you have to be a little schizophrenicand practice all the physicians that

(44:31):
you're practicing with.
I think it's definitelyharder in general pulmonology,
it's the wild west and who's doing what.
And that's been my experience with talkingto other general pulmonary practices,
what guidelines they're following to aT, what they're kind of modifying for,
how they practice and where they practice.
Which definitely brings me to the pointof there are patients with interstitial

(44:52):
lung disease, we all want them to go toCleveland Clinic and see you. However,
a lot of patients can't. There'snot an ILD center in their area.
They have transportation issues,
all sorts of reasons why patients can'tfollow with a specific ILD center and
even some that can't even getto pulmonary, just general pulmonary like myself.
And so primary care is definitelysaddled a lot of times,

(45:14):
especially in very ruralareas managing these patients.
So what advice would you give themabout long-term monitoring of these
patients with interstitial lung disease?How often should they evaluate them?
Tools, things along those lines,what advice would you give them?
Yeah,
that's a tough space to be in and Iknow that we've got so many of our

(45:36):
amazing nurse practitionersout there in primary care,
especially in the center of the countrythat don't have the access maybe to good
thoracic radiology readingsto good ILD centers or
MDD. So it is, it's really hard.
I would say that I wouldkeep a close eye on these

(45:57):
patients,
especially at the beginning of theirdisease until how their disease is
acting. So I would probablyevaluate them every three months.
I would have them come in andat the very least talk to you
about symptoms, talk to them alot about their level of dyspnea,

(46:18):
what type of activities they'rehaving, shortness of breath with,
trying to really determine from that ifthings seem to be getting worse or not.
If you have access to PFTs, that is very,
very helpful. Very helpful tokind of follow those along,
follow the FVC and the DLCO.
So if you're seeing thatFVC and DLCO are dropping,

(46:40):
maybe the FVC more than 10%or the DLCO more than 15%,
that's a reason. Maybe we needto, if they're not on therapy,
get them on therapy or maybe think againabout getting them to an ILD center.
So at the beginning there I woulddefinitely follow them quite frequently.
As for CAT scans, maybeonce a year. But again,

(47:03):
sometimes we don't need them quite thatoften if things are stable or we get
reactive CTs, as you said, Corinne wheresomething is changing and we get CTs.
So CTs are a bit more variable.
But in general I love tohave PFTs each time. So yeah,
I guess is a rule maybe everythree months at the beginning,
if you find that they're pretty stable,
you could maybe do every sixmonths unless something changes.

(47:27):
I like that plan. That'sexactly what we do.
The only other call out I would say to low
threshold for a new CT scan outsideof symptom changes, if they're stable,
lung function, stable, the patient isstable. If they have a smoking history,
I tend to do their lung cancer screeningjust because, correct me if I'm wrong,
don't IPF patients have a littlehigher risk of lung cancer?

(47:50):
They absolutely do.
So if they have a smoking history andan IPF history that they have a higher
risk of lung cancer. So those patientsdefinitely need to be screened.
And unfortunately with the lungcancer screening guidelines,
if they stopped smokingmore than 15 years ago,
you could always rescreenthem because of their ILD.
So you could just use the ILD diagnosisfor a repeat scan if they don't qualify

(48:13):
for lung cancer screening.
And since we're talking about the scan,
I'll just put in a plug forwhat you said earlier, the HRCT,
the high resolution CT,
if you have the ability toget a high resolution CT,
which is no more radiologythan a regular CT,
they're not exposed moreradiation if you will.
It's just reconstituted differently.
They give more slices ofthe lungs, smaller slices,

(48:37):
one to two millimeter slices.
That's so important to really figuringout what the pattern of disease
is to help you then knowwhat's causing the disease.
So that's a super important piece.
And it's right, so your patients withkidney disease and non-contrast, yep,
you don't have to worry about them innon-contrast CT patients who have iodine,
they're telling you, I can't havea scan because I'm allergic to dye.

(48:59):
You don't have to worryabout that. Alright,
well I believe you're going to betalking to us next about the future.
Yep, absolutely.
So I'm going to talk a little bit aboutboth the current therapeutics that we
have for pulmonary fibrosisas well as some potential
future therapeutics that wehave. But before I go into that,
I just want to chat a little bitabout what we should be doing for

(49:24):
all of our pulmonary fibrosispatients, all of our ILD patients,
we should really be thinking aboutpulmonary rehabilitation for our patients,
sending them there.
It does such wonders forimproving their level of
dyspnea, improving theircardiovascular health,
improving their core and musclestrength. They get taught nutrition,

(49:47):
they get taught breathing exercises, theymeet other people. It's so important.
So pulmonary rehab is a must for allof these patients if they need oxygen,
getting them hooked upwith some oxygen therapy,
palliative medicine to kind of goover advanced care planning and also
management of symptoms and also just toget them hooked in with something. If
things continue to getworse with their disease,

(50:10):
they can easily get hooked in withhospice and then sometimes lung
transplantation or clinicaltrials for these patients.
So those are things to thinkabout besides the therapeutics,
but what therapeutics do we have?
So if you remember way backto the beginning in the podcast we talked about the
inflammatory cascade and wetalked about the fibrotic cascade.

(50:33):
So today we're not going to talk at allabout the inflammatory cascade and what
we utilize therapeutic wise for that,
but we use anti-inflammatories and weuse immunosuppression medications for
that.
We're going to talk todayabout the antifibrotic arm of
therapeutics.
So we currently have twoapproved medications for

(50:56):
pulmonary fibrosis,
nintedanib or OFEV andpirfenidone or Esbriet.
Both of these came out on the same day in
2014.
There were the phase threetrial for OFEV or nintedanib was
INPULSIS and for pirfenidone was ASCEND.And both of these found that

(51:16):
these drugs were just aseffective as each other.
So we don't say take thisor that medication based on
one being more effective than the other.
The primary endpoint in bothof these trials was reduction
in the decline of FVC over52 weeks over one year.
And both of these met that endpoint andboth of them found that in the treatment

(51:40):
arms there was about 150 ml reduction in
FVC decline as compared to the placebo.
So pretty good reduction inthat FVC decline for both of
them. Now these medications,
they slow progression of fibrosis,
but what they don't do isthey do not halt disease and

(52:05):
they do not reverse disease.
I think this is something that's veryimportant not only for clinicians to know,
but for our patients to know.
They need to know that thesearen't absolute home runs. We are hoping for a holy
grail of something that canreduce or reverse the fibrosis,
but for right now we just havethese that slow the progression.
So both of these medicationscan have some toxicities

(52:30):
associated with them aswell as some side effects.
So the toxicities arewith regard to the liver.
So we do have to check liver enzymes withthese generally once monthly for three
months when they start and then everythree months it's pretty rare that they
have elevations of their liver enzymes,
but we do have to check that andthen we warn them about side effects.

(52:51):
So there is a bit of discontinuationof these medications due to side
effects. So for nintedanibdiarrhea is the most common side
effect.
I would say two thirds or so ormore of patients will have diarrhea
for pirfenidone or Esbriet.It's more stomach upset,
upper GI like reflux, heartburn,

(53:13):
maybe some nausea.They can also have some sun sensitivity.
And then both of these can affectappetite and decrease weight.
So you really want to keep an eye onthese patients with this chronic lung
disease, make sure that they're notlosing weight on these medications.
That would be a reasonfor discontinuation.
I will say that in the trials theyfound about a 20% discontinuation rate

(53:37):
In more recent trials,
retrospective trials thatare more real world trials,
they found even up to 50%discontinuation rates.
So depending on the trials,discontinuation rates can be quite high.
These are medications kindof better than nothing.
We finally have something to offerour patients to help slow disease,

(53:59):
but it doesn't halt or reverse diseaseand they do have some toxicities and some
side effects to them.
So what do we have on thehorizon for therapeutics?
So the one therapeuticthat is probably closest
to being approved is anew medication called

(54:19):
nerandomilast.Nerandomilast is a PDE4B
inhibitor.
So this is in the same classas roflumilast that we use for
COPD patients.
So nerandomilast was lookedat in two side-by-side
studies FIBRONEER-IPF and

(54:40):
FIBRONEER-ILD.
FIBRONEER-IPFstudied
this drug in the IPFpopulation FIBRONEER-ILD
studied this in patients withILD that was not IPF but had a
progressive phenotype and for both the
FIBRONEER-IPF and FIBRONEER-ILD trials,

(55:00):
this drug met its primaryendpoint of reducing that decline
in FVC over 52 weeks.
So it was positive for both of thosetrials, FIBRONEER-IPF and FIBRONEER-ILD.
So now we'll move on to anotherdrug inhaled treprostinil,
which is showing potentiallysome promise for pulmonary

(55:22):
fibrosis. Some of you maybe familiar with this drug.
Inhaled treprostinil is a medicationthat is currently approved for
PAH as well as for PHILD.It was
recently approved in about 2022 Ithink trial called the iINCREASE trial,
which looked at this medication usedin ILD patients that had pulmonary

(55:44):
hypertension and looked at theirsix minute walk and some other
factors over 16 weeks. Now they found,
interestingly when theylooked over all of the data,
they had looked at the FVC of thesepatients as well and they found that these
patients had stabilization oftheir FVC in that 16 week trial.

(56:05):
And in fact when you looked atthe IPF patients in particular,
they actually had a signalthat maybe their FVC improved.
And so this was quite exciting,
although PH trials are much shorterthan pulmonary fibrosis trials. So
the proof is in the pudding hereif that lasts over 52 weeks.
But they have the TETON trialnow that is looking at whether

(56:29):
this inhaled treprostinil alsohas antifibrotic properties
and can decrease fibrosisor halt fibrosis. So this is
still in the works.
The European trials will befinishing soon-ish in the next year.
The US trials just finished enrolling,
but hopefully by 2026 weshould have some data on this.

(56:53):
So now I'm going to goover to the role of the
NP and we talked a lot about therole of that primary care NP.
You are the eyes and the ears.
You are the ones out there seeingthese patients and detecting these
patients. And so I'm goingto throw it over to Corinne.
She works within general pulmonary.

(57:15):
She can kind of go over all ofthe primary care and pulmonary
multidisciplinary managementof these patients.
Thank you Jessica. Yeah,
I mentioned earlier it's never toosoon to send them to pulmonary,
either general pulmonary in yourcommunity or to a tertiary care
center like Jessica's.

(57:35):
We would again rather see them andit be nothing than it be something.
And even if you're not comfortablecalling this an interstitial lung disease,
you could use the diagnosis code ofabnormal CT imaging of the chest if you're
just not quite sure. It doesn't lookright to you. Patient's symptomatic,
trust your gut, send 'em to us.
We're happy to see them and discussthat process in that patient and what's

(57:56):
going on. Interfacing betweenprimary care and pulmonary medicine,
again is so crucial.
And I know oftentimes there's a littlebit of a bias that pulmonary medicine is
a little too busy. It takes so long toget in to see us oftentimes. But again,
most of the time I think most practicesdo triage their referrals and somebody
who has a chronic cough versussomeone who has an abnormal CT scan,

(58:19):
the abnormal CT scan,
patient's going to be seen sooner andtriaged a little sooner to that. So we
definitely want to see them and we wantto work with you because you're often
seeing those patients morefrequently than we are.
And there is this TripleA model which is access,
anticipate and act.
The idea behind it is to anticipateby monitoring the disease with serial

(58:40):
testing, identifying patient andcaregiver needs, because again,
there's a lot of support these patientsneed emotionally and their caregiver is
going to need advanced care planning,
and talking about preventative measureslike immunizations that they may not
have been agreeable to before,
but now that they know they have alung disease that is worthy of a new
conversation with them. Stopping smoking,

(59:02):
jobs that may be caustic forthem to have inhaled exposures to
again,
kind of anticipating things for thosepatients as far as acting or thinking
about disease modifying treatments.
So is this patient a candidate forantifibrotic therapy? Is this a patient a
candidate for lung transplant?
What can we act on to improve theirsymptom relief and end of life

(59:24):
discussions?
Definitely need to be had these patientsas soon as they're diagnosed at the
very least, need apalliative care consultation.
They may not utilize it to itsfullest in the initial stages,
but they're going to at some point anddefinitely want to talk about that and
act on it with them. As far asaccess, we want to make sure,
do they have access tospecialized centers? Do they have transportation access,

(59:46):
financial access insurance,access to those things,
electronic health technologies, I knowa lot of centers are doing telehealth,
reach out to rural areas.Is that an option for them?
Making sure they have accessto information in their language in a way that they
understand it.
Are they a visual person? Are they someonewho would benefit from videos versus
handheld materials toread disease awareness,

(01:00:09):
and also access to clinical trials,
which are usually happeningat those ILD centers,
but could be happening even sometimesin general pulmonary practices.
So are they interested in a trial andwhere can we get them access to that?
So those are the three A's. Alright.
So as far as the importanceof multidisciplinary collaboration and MDC in the

(01:00:29):
near term and long termacross continuum care,
how much weight do you put into that andhow hard would you push primary care to
try to get their patientsinvolved in that?
I think collaborating with otherplayers is paramount for these patients.
I mean the radiologist justitself is an important one.

(01:00:50):
We may not have in allparts of the country,
thoracic radiologists who really knowtheir interstitial lung diseases.
But having a radiologistthat you trust that being
able to go over thosecases with a radiologist,
that's really what makes these tertiarycare centers so really great for these

(01:01:11):
patients. I think that reallyhelps the clinician a lot.
Having pathologists,
having experts in different areas thatyou can put your heads together with.
And then also things likeexercise physiologists,
those respiratory therapists,
people that can help improve symptoms foryour patients with pulmonary rehab can
help nurses to call your patients andmake sure that things are going well with

(01:01:35):
our medications, able to stay onmedications, better, things like that.
So important I think wereally have to make sure that
we're pulling all of our colleaguesthat we have available to us to help us
with these patients.
Well I think this hasbeen a great discussion.
I think we've covered alot of points. Jessica,
would you like to summarize it for us?What the big takeaways should have been?

(01:01:59):
I think if you have a suspicionof pulmonary fibrosis in your
patients, there are a lotof resources out there.
So go to your resources that we'vediscussed here. Listen for your crackles,
get a CAT scan if you have highsuspicion for your patients and try and
get them into general pulmonologistor if you have something close by

(01:02:22):
an ILD center.
It's been so great talking about thisand this is an area that I'm really
passionate about.
So thanks Corinne for being my partnerin this and thank you so much to
AANP as well.
We're really looking forward to thefuture of ILD and future therapeutics.
Thank you.
Well, thank you so
much, Jessica and Corinne.

(01:02:42):
It's been an absolute pleasure listeningto you and gaining your perspective and
insights on this extremelyimportant topic to our listeners.
I hope you found this episode educationaland can apply some of what was
discussed to your practice.
Join your national professional association and add your voice to over
120,000 of your NP colleagues nationwide.

(01:03:03):
I urge you to become an AANP member today.
Membership gives youaccess to so many benefits,
including tools andresources for your practice,
and the AANP CE Center,
which offers a comprehensive libraryof CE activities for NPs of all
specialties and experiencelevels, exclusive discounts,
and many free activities are yours asan AANP member to help you complete

(01:03:27):
state licensure requirements and earnthe credits needed for recertification.
Remember that you may claim CEcredit for this program through
June, 2026 by logginginto the CE center at
AANP.org/cecenter.
Register for this program.Enter the participation code

(01:03:47):
FILD2526. Again,
that's FILD2526,
and then complete thepost-test and evaluation.
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share with your colleagues andcheck back often for new episodes.
Thank you for listening.