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Time Stamps

• 01:30 Deep Dive 1: Evaluating Incidental Findings
• 06:45 Deep Dive 2: Categorizing ILD
• 12:42 Deep Dive 3: Treatment for ILD
• 21:19 Deep Dive 4: Addressing Barriers to Care
• 28:10 Deep Dive 5: Ethical Considerations in ILD Treatment

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Show Notes

Deep Dive 1: Evaluating Incidental Findings

Why do incidental CT findings matter?  

• Subtle interstitial changes on a CT scan may indicate early fibrosis, atelectasis, aspiration, pulmonary edema, or infection. (Though not exhaustive list)
• What is  ILD protocol CT? 
  • High-resolution CT (HRCT) with inspiratory, expiratory, supine and prone images
  • Differentiate fibrosis from mimics
  • Involves:
    • Number of Acquisitions:
      • Supine Position: Inspiratory (volumetric) and expiratory (sequential or volumetric) acquisitions.
      • Prone Position (Optional): Only inspiratory scans, which can be sequential or volumetric.
      • Inspiratory scans should be obtained at full inspiration.

If fibrosis is suspected:

• Refer to pulmonology within 6 months 
• Order pulmonary function tests (PFTs) to expedite evaluation. 
  • PFTs provide data on disease progression and urgency of intervention.

Deep Dive 2: Categorizing ILD

What is a clinically relevant way to categorize the causes of ILD?  

• Broad groups to aid in diagnosis and treatment. Below is a streamlined overview:
  • Infectious ILDs  
    • Examples: Viral pneumonias, tuberculosis, fungal infections.  
  • Environmental or Medication-Induced ILDs  
    • Environmental exposures examples: tobacco/smoking, asbestos, silica, and other occupational exposures.  
    • Medication-Induced ILD: Common culprits include amiodarone and methotrexate.  
  • Autoimmune-Related ILDs  
    • Associated with systemic rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, and polymyositis.  
      • Subtype: Often referred to as connective tissue disease-associated ILDs (CTD-ILDs).  
  • Granulomatous ILDs  
    • Examples: Sarcoidosis, Langerhans cell histiocytosis.  
      • Sarcoidosis: 
        • Characterized by non-caseating granulomas.  
        • Requires exclusion of other granulomatous diseases.  
      • Langerhans Cell Histiocytosis:  
        • Often linked to smoking.  
    • Presents with cystic and nodular changes on HRCT.
  • Idiopathic ILDs  
    • Definition: Diagnosed after ruling out all other potential causes.  
    • Examples:  
      • Idiopathic Pulmonary Fibrosis (IPF): 
        • Most common form
        • Often managed with antifibrotics.  
      • Nonspecific Interstitial Pneumonia (NSIP): 
        • A more inflammatory subtype.  
      • Cryptogenic Organizing Pneumonia (COP): 
        • Frequently responds to corticosteroids.  

Deep Dive 3: Treatment for ILD

What are the primary treatment options?  

• Antifibrotics: Pirfenidone and Nintedanib slow the decline in FVC but do not reverse fibrosis.  
  • Indicated for all patients with progressive fibrosis, irrespective of subtype.  
  • Side effects include GI disturbances (e.g., diarrhea) and hepatotoxicity (requires liver enzyme monitoring).  
  • Key takeaways INBUILD trial
    • 1. Efficacy of Nintedanib:
      • Primary Outcome: Reduced annual FVC decline by 107 mL/year compared to placebo in the overall population (-80.8 mL vs. -187.8 mL, p<0.001).  
      • For patients with a UIP-like fibrotic pattern: Decline reduced by 128.2 mL/year (-82.9 mL vs. -211.1 mL, p<0.001).  
      • Patients with other fibrotic patterns showed a reduction of 75.3 mL/year.
    • Secondary Outcomes:
      • No statistically significant improvement in quality of life using the K-BILD questionnaire.  
      • Lower incidence of acute exacerbation or death: 7.8% in nintedanib vs. 9.7% in placebo.  
    • Safety Profile:
      • Most common adverse event: Diarrhea (66.9% in nintedanib vs. 23.9% in placebo).  
      • Liver enzyme elevations were more common in the nintedanib group (13.0% vs. 1.8%).  
      • Discontinuation due to adverse events: 19.6% in the nintedanib group vs. 10.3% in placebo.

• Immunosuppressants: 
  • Ex: Mycophenolate and azathioprine 
    • Used in non-IPF ILDs.  
  • Avoid in IPF, as shown by increased mortality in the PANTHER trial.  
    • PANTHER trial was a randomized, double-blind, placebo-controlled trial comparing a combination of prednisone, azathioprine, and N-acetylcysteine (NAC) (triple therapy) versus placebo.
      • Patients included had mild-to-moderate IPF with FVC ≥50% and DLCO ≥30% of predicted values.
    • Outcomes:
      • Increased Mortality:
        • Patients in the triple therapy group experienced an 8-fold higher mortality rate compared to placebo (10% vs. 1%, p = 0.01).
        • Deaths were primarily related to respiratory causes or treatment-related complications.
      • Hospitalizations:
        • Rates of all-cause hospitalization were significantly higher in the triple therapy group (30% vs. 9%, p < 0.001).
      • Serious Adverse Events (SAEs):
        • 31% of patients in the triple therapy group reported SAEs, compared to 10% in the placebo group (p = 0.001).
        • These included respiratory complications, infections, and general adverse drug reactions.
    • The PANTHER trial provided definitive evidence that immunosuppressants, particularly azathioprine and prednisone, should not be used in IPF due to their association with increased mortality, hospitalizations, and adverse events. Antifibrotic therapies remain the standard of care for this patient population.
  • What role do steroids play?  
    • Managing acute ILD exacerbations or flares
    • Rapidly progressive or exacerbating ILD, where they can help reduce inflammation and stabilize the patient’s condition.

Deep Dive 4: Addressing Barriers to Care

How do you identify and address barriers to care?  

• Use Maslow’s hierarchy of needs to guide assessment:
  • A psychological theory that explains human motivation in five levels, starting from basic survival needs to personal growth.
  • You must meet lower-level needs before focusing on higher ones.
    • Basic Needs: Essentials like food, water, safety, and shelter.
      • Specifically:  Transportation, food security, and health literacy.
    • Social Needs: Relationships, love, and belonging.
    • Self-Worth: Feeling respected and accomplished.
    • Personal Growth: Achieving your full potential and pursuing your passions.
  • Collaborate with PCPs and social workers to address social determinants of health.  

Deep Dive 5: Ethical Considerations in ILD Treatment

How do you balance ethics in ILD care?  

• Utilitarian ethics focus on maximizing outcomes vs. 
• Egalitarian ethics emphasize equal care opportunities.  
  • Success in ILD management often hinges on equitable access to resources, rather than identical treatment outcomes for all patients.

What is the role of shared decision-making?  

• Frame treatment as a contract between physician and patient, ensuring both parties collaborate for effective care.

Key Takeaways

1. Evaluate incidental CT findings using ILD protocol CT with prone imaging, and follow up with PFTs.  
2. Categorize ILD broadly (e.g., infections, exposures, idiopathic) to guide therapy.  
3. Treat progressive fibrosis with antifibrotics; use immunosuppressants for non-IPF ILDs.  
4. Address social barriers to care using frameworks like Maslow’s hierarchy to improve patient adherence.  
5. Strive for equitable care opportunities through ethical and collaborative practices.  

Transcript

Dr. Alison Trainor: Welcome to Gray Matters, where we unpack how medical management is rarely black or white.

Dr. Jason Freed: And we go on deep dives along the way! I’m Jason Freed, a hematologist at Beth Israel Deaconess medical center.

Dr. Alison Trainor: and I’m Ali Trainor, a pulmonary and critical care physician at Mount Auburn Hospital and I have a case today that I’ve been ruminating on for a while that I would love to discuss.

Dr. Jason Freed: I’m ready!

Dr. Alison Trainor: So this is a patient who first presented to pulmonary clinic in May of 2020 so 4 years.

Dr. Jason Freed: Oh, wow, you have been ruminating for a while.

Dr. Alison Trainor: Haha yes, at that time she was 70 years old and carried a history of asthma and was seeing pulmonary for asthma and an incidental finding on a CT A/P which caught her lung bases and showed some mild interstitial changes concerning for early interstitial lung disease or ILD versus chronic microaspiration. 

Dr. Jason Freed: Ugh, bringing me back to the episode we did previously on incidental findings. They’re so hard

Dr. Alison Trainor: Yes, totally, especially when the read tells you it could be acid reflux or it could be interstitial lung disease. That’s a huge difference there.

Dr. Jason Freed: I feel like I see this in radiology reports all the time, I get a CT for some other reason, the patient has no pulmonary symptoms and then the radiology read says “fine subpleural reticulations at the lung bases which could be atelectasis, aspiration, or early ILD.”  So maybe we can do this for our first deep dive. What should we do when we have an incidental finding that could be ILD or could be nothing?

Deep Dive 1

Dr. Alison Trainor: I think a key question with incidental lung findings of course is, is this actually fibrosis? so if you’re really concerned, then getting a CT with ILD protocol can help to differentiate. I sat down with Dr. Rob Hallowell, a pulmonologist at MGH and a specialist in ILD to talk more about whats different about the ILD protocol.

Dr. Rob Hallowell: So the main differences when we talk about ILD protocol imaging, there’s two main features. It’s not necessarily just the higher resolution, but it’s one doing supine and prone imaging. So when patients are lying supine on their back, the weight of the body puts pressure on the posterior parts of the lungs. And so that can cause mild atelectasis, which can really mimic reticular changes and subtle interstitial lung abnormalities. And so early ILD can look just like mild atelectasis in the lung basis and the posterior lung fields can be very hard or almost impossible to distinguish the two. And so then the radiology technician will have the patient then flip over and lie in the prone position and repeat the imaging. And so if those changes go away, it’s not true interstitial lung disease.

Dr. Jason Freed: So does that mean that every patient who has subtle changes should be getting an ILD protocol CT scan? That seems like a lot of studies and I’m guessing with all the images they do with an ILD protocol that’s a lot more radiation. 

Dr. Alison Trainor: Great point, there are some CT scans, for example CTAs are always done in the expiratory phase in which your lung parenchyma is going to be more squished together and so we often see these subtle changes in CTAs that are really just atelectasis and not all these patients need an ILD protocol. I would say if fibrosis is a real consideration then you should be getting an ILD protocol study and if you’re not certain if fibrosis is a real consideration the reading radiologist or any pulmonologist would be happy to take a look for you.

Dr. Jason Freed: Ok, so let’s say you get an ILD protocol CT and the changes persist, so we’re worried about fibrosis. What do we need to do? It was incidentally found so presumably the patient is asymptomatic and the CT changes are only mild.

Dr. Rob Hallowell: And I would argue that there’s no such thing as mild fibrotic changes in the sense that severe fibrosis once began as early discovered interstitial changes. Patients who have definitive interstitial changes probably warrant a pulmonary evaluation. Most of them don’t need an urgent evaluation if they’re not desaturating or have evidence of rapidly escalating symptoms. But I think it’s helpful that they all get to speak with a pulmonologist at some point.

Dr. Jason Freed: That’s helpful, but what are we talking about when we say “not urgent”. Does that mean within 3 months, 6 months, a year?

Dr. Alison Trainor: I think as long as you can get them in within 6 months and then counsel them to say “hey, if you develop symptoms you need to let me know so I can advocate for you to get in sooner”. I’ll also say that every pulmonologist at their initial visit is going to order PFTs, so you can also order the patient PFTs to help expedite their workup, and if the PFTs are normal, you know you’re ok waiting a bit and if they’re abnormal you can reach out to the pulmonary team for a sooner appointment.

Dr. Jason Freed: Ok, so just to make sure I’m following, to summarize incidental finding of interstitial abnormalities could be ILD atelectasis or aspiration. If it’s incidental finding with no pulmonary symptoms can call radiology or pulmonary to ask them how worried they are about ILD, and if worried or uncertain can go forward with ILD protocol CT, and if that shows these are real interstitial changes then that patient should see a pulmonologist. if they’re asymptomatic it’s less urgent and you can order them for PFts in advance to help speed up the workup.

Dr. Alison Trainor: That’s perfect.

Dr. Jason Freed: Ok great,  so back toyour patient. She was already plugged in with pulmonary, she had a follow up plan to get repeat pulmonary function tests in 6 months and to monitor her symptoms, so what happened with her next?

Dr. Alison Trainor: Well unfortunately around the time she was supposed to have her repeat PFTs and appointment there was another spike in the COVID pandemic so she canceled her appointment and then after that she was lost to follow up. She didn’t come back until about 2 years later. I got PFTs which showed a significant decline in her FEV1 and FVC so I got a repeat CT scan which showed progression of diffuse interstitial lung disease. So at this point I start sending a serologic workup trying to assess does she have CTD-ILD, UIP, NSIP…

Dr. Jason Freed: Ali… stop… the alphabet soup of pulmonary…I just can’t.

Dr. Alison Trainor: Ah thank you! I’m getting excited and ahead of myself. 

Dr. Jason Freed: Also, no offense, but does the alphabet soup even matter? Hematologist here, but I feel like every patient I see with ILD, regardless of which additional acronym you give them is on mycophenolate or azathioprine plus an antifibrotic and then when they come into the hospital you give them enormous doses of steroids, so at least to me they seem to be all treated the same regardless of which part of the alphabet soup they are.

Dr. Alison Trainor: Ok, some fair points there, and let’s dig into the ILD alphabet soup a bit more in deep dive 2. How important is it to differentiate the different types of ILD?

Deep Dive 2

Dr. Rob Hallowell: Nowadays, when I lecture on ILD and I teach the fellows about ILD. I don’t spend too much time on the alphabet soup. What’s more important are broad categories of approaching the patient and figuring out what general category are we dealing with. Because more often than not, that is what drives therapy and patient action and physician action, and that’s what’s clinically relevant.

Dr. Jason Freed: Ok fantastic so if I don’t need to know the alphabet soup then what are the broad categories here?

Dr. Alison Trainor: I’m going to let Dr. Hallowell flesh this out a bit more, but the broad categories are infections that mimic ILD, exposures which includes environmental and medications, autoimmune disease, syndromes which includes things like sarcoid and langerhans, and then the true idiopathic interstitial lung disease.

Dr. Rob Hallowell: So the main sort of buckets under the umbrella that we think about when we’re going through the ILD algorithm in general include infections. So there are infections that can mimic interstitial processes, and then we think about exposures. So environmental exposures both organic and inorganic. We think about medications. So there are medication exposures that are notorious for causing interstitial processes. We think about autoimmunity, so all the rheumatologic diseases, so almost every connective tissue disease causes interstitial lung disease with some very rare exceptions. And then you have your sort of syndromes and idiopathic things. I would lump sarcoid there, amyloidosis, Langerhans, as sort of your own little entities. And then all the idiopathic and interstitial lung diseases, your idiopathic NSIP, your cryptogenic organized pneumonia and your idiopathic pulmonary fibrosis, recognizing that you really have to do your diligence before you get to put the idiopathic before it in case you’ve missed something. 

Dr. Jason Freed: Ok, that makes a little bit more sense, but it seems like maybe Dr. Hallowell was starting to add in a little bit of the alphabet soup when he was getting to the idiopathic category?

Dr. Alison Trainor: Yes, so in that bucket he mentioned organizing pneumonia, idiopathic non-specific interstitial pneumonia or NSIP, and then idiopathic pulmonary fibrosis or IPF. We’ll get to why it’s relevant to distinguish that bucket when we get to treatment.

Dr. Jason Freed: Ok, anything else we should know about differentiating buckets of ILD?

Dr. Alison Trainor: I think I would just like to emphasize what Dr. Hallowell said about the amount of work required to really do your due diligence before labeling someone as having an idiopathic process. This requires social history taking, physical exam, serologic workup, and potentially bronchoscopy and/or a biopsy. And this is where the expertise of the pulmonologist really comes in. So we’re simplifying it a lot here which I think is necessary and helpful, but there’s a lot to do before labeling someone idiopathic.

Dr. Jason Freed: I actually love the social history taking part of pulmonary.

Dr. Alison Trainor:  Same! Dr. Hallowell probably wouldn’t remember this, but I remember as a resident on pulmonary consults presenting a patient to him who had new ILD and on history, I found out he was part of an underground pigeon racing league that met up in an old factory that had asbestos in it. 

Dr. Jason Freed: That’s crazy! But going back to the buckets of interstitial changes and within that the idiopathic lung disease that have all these acronyms. It must  be difficult to explain this in a way patients understand, because I’m only just beginning to understand this

Dr. Rob Hallowell: I think what happens nine times out of 10 is patients come into clinic, certainly into my clinic, they all come to me saying, I know I have idiopathic pulmonary fibrosis and I only have three years to live. And the majority of the patients actually don’t have idiopathic pulmonary fibrosis. They’ve seen the word fibrosis used somewhere on the report or somebody has mentioned it to them and either a provider or Dr Google has told them this is what you have and this is the life expectancy and all of that is incredibly inaccurate and that can lead to a lot of anxiety, lifestyle changes that are inappropriate, et cetera.

Dr. Jason Freed: Yeah this happens in my specialty as well, people hear cancer and immediately think the worst.

Dr. Rob Hallowell: I think the language you should be using is that there is an interstitial process here. There are many different flavors. There are many different breeds of dog. They all bark, they all need to be walked, but a chihuahua is not a pit bull. And the distinction is critical. And right now we just hear something barking, we don’t know what we’re dealing with yet, and the details matter. And I think managing expectations and being careful with the verbiage we use is really important because by the time they come into our specialty clinics, our niche clinics, our ILD clinics, it’s really hard to walk some of this back and can impact how we treat patients, what patients are willing to try and reframing.

Dr. Jason Freed: That dog analogy is a great way to frame it.

Dr. Alison Trainor: Definitely, I’m going to steal that haha, but anyway getting back to your point about it seeming like regardless of which ILD diagnosis a patient is given, they all seem to have the same treatment which honestly is a pretty fair assessment. But I will say though that determining the subcategory is still important because if there’s an exposure, we eliminate the exposure, if there’s an underlying rheumatic disease we treat that, but when it gets down to treating the ILD itself, we really do only have two classes of treatments – immunosuppression and antifibrotics. 

Dr. Jason Freed: That makes sense. Ok so we really have two categories of treatment for interstitial lung diseases.

Dr. Alison Trainor: Exactly, so for deep dive 3 let’s get into the treatments.

Deep Dive 3

Dr. Jason Freed: Should we start with antifibrotics?

Dr. Rob Hallowell: So the antifibrotics, there’s two on the market right now. The brand names are Esbriet and Ofev or Pirfenidone and Nintedanib.  Prior to that, when I was in training, when I was a med student resident, et cetera, we had nothing. So if you had progressive fibrotic lung disease, it was a death sentence. We had all these trials looking at things like immunosuppression, et cetera. They all failed.

Dr. Jason Freed: How well do the antifibrotics work?

Dr. Rob Hallowell: None of them reverse fibrosis. So both of those agents, they’re considered to be equal in efficacy. They both have been shown in many, many trials to slow the decline in FVC. So the cohorts of patients, they all continue to progress over 52 weeks, their FVCs all go down, but the decline was roughly cut in half for the cohort as a whole. Now that means some people stabilize, some people continue to get worse, but on average the FVC decline was cut in half.

Dr. Jason Freed: Ok, so unfortunately, can’t reverse fibrosis but can add quality years with having better lung capacity, especially if started early.

Dr. Alison Trainor: Exactly.

Dr. Jason Freed: Obviously would be great if we had something to reverse disease, but seems like we’re at least doing much better than before antifibrotics. So this seems great.

Dr. Alison Trainor: Yes, definitely, it’s made a huge impact for our patients with fibrosis, the one thing I’ll add though that’s important to know is that they have a lot of GI side effects – mainly diarrhea but some patients also get nausea. We have some ways to address this including anti-diarrheals and dose titration, but overall something patients and people taking care of them should be aware of. These patients also need to be monitored with labs for hepatotoxicity.

Dr. Jason Freed: Ah, no free lunch. Ok, so who gets antifibrotics?

Dr. Alison Trainor: The original study looking at nintedanib only assessed its effect in patients with IPF so that’s where we started, but in 2019 there was a trial published called “INBUILD” that showed nintedanib was effective in patients with progressive pulmonary fibrosis of any etiology. 

Dr. Jason Freed: So anyone with progressive pulmonary fibrosis, regardless of where they fit in the alphabet soup, is a candidate for an antifibrotic.

Dr. Alison Trainor: Exactly.

Dr. Jason Freed: Ok, that seems fairly straight forward. What about the other category, immunosuppression? What do we typically use and who gets immunosuppression?

Dr. Alison Trainor: The most common agents used are mycophenolate and azathioprine, both have their downsides and need to be monitored but in general they are better than being on long term steroids.

Dr. Jason Freed: I feel like I have seen patients with ILD on steroids though?

Dr. Alison Trainor: Yes, so if someone has a flare we will burst them with steroids and also if someone is a new ILD presentation with severe ILD, we’ll start steroids up front to try to calm down inflammation while we’re waiting for the steroid sparing immunosuppressive agent to take effect.

Dr. Jason Freed: So do all patients with ILD get immunosuppression?

Dr. Alison Trainor: Not quite – most patients with ILD will get immunosuppression based on CT findings or underlying etiology, but the one really important distinction to keep in mind, and this is where the alphabet soup comes in, is that patients with IPF should NOT get immunosuppression.

Dr. Rob Hallowell: Idiopathic pulmonary fibrosis. Currently the only treatments we are using are antifibrotics. All the other categories, depending on how the patient is progressing and with what pattern may get immunosuppression, may get antifibrotics or a combination of the two. 

Dr. Jason Freed: I’m guessing there’s some large pulmonary trial that taught us not to use immunosuppression in IPF?

Dr. Alison Trainor: Exactly. There was a study called the PANTHER trial which compared placebo to what was considered the standard treatment at that time – a combination of azathioprine, prednisone, and N-acetylcysteine. This showed that there were significantly more adverse events and significantly more deaths in the treatment group, 8 deaths for every 1 death in the placebo group. And so it has been extrapolated from this that we do not give any immunosuppressants to people with IPF and our only treatment is antifibrotics. 

Dr. Jason Freed: Alright I’m going to summarize to make sure I have this correct. More important than the alphabet soup are the broad categories of ILD which include infections, exposures, autoimmune disease, specific syndromes like sarcoid, and then the idiopathic category. And then for treatment we have immunosuppression and antifibrotics. All patients with progressive pulmonary fibrosis are a candidate for an antifibrotic and patients will also likely get immunosuppression if they do not have IPF.

Dr. Alison Trainor: Perfect, you’re a pulmonologist now.

Dr. Jason Freed: So I stopped you when you were getting into the alphabet soup possibilities for your patient. Did you ever end up nailing down a diagnosis for her?

Dr. Alison Trainor: We did our due diligence with history, exam, serologic workup and there was nothing, so she ended up in the idiopathic bucket, and based on her CT scan findings, we ended up diagnosing her with idiopathic NSIP or non-specific interstitial pneumonia.

Dr. Jason Freed: Wow, idiopathic non-specific, such a clear diagnosis.

Dr. Alison Trainor: LOL.

Dr. Jason Freed: Alright I’m going to flex my new pulmonary knowledge here. Sounds like she might warrant some immunosuppression since its nonspecific interstitial pneumonia and not IPF and if I remember correctly, she had had a significant decline in her PFTs she should get an antifibrotic as well?

Dr. Alison Trainor: Nice! Yeah so for her she had a lot of ground glass opacities on her CT scan which suggested to us a lot of active inflammation in addition to the fibrosis so our priority was getting her on an immunosuppressive agent and decided to start her on mycophenolate mofetil (MMF).

Dr. Jason Freed: One of my favorite meds as well.

Dr. Alison Trainor: I spent a lot of time during our visit counseling her on what the indication for the medication was, the possibility of GI side effects to be aware of, and the need for lab monitoring. 

Dr. Jason Freed: And for any listeners who aren’t prescribing mycophenolate as much, for most autoimmune disease we’re checking a CBC with differential 1-2 weeks after we start and then about every 6-8 weeks after that to look for bone marrow suppression and mainly neutropenia. 

Dr. Alison Trainor: And so, she leaves the office that day and is supposed to get a same day baseline CBC but leaves without getting labs checked. I try calling her several times but she doesn’t answer and she doesn’t have her voice mail box setup. I’ve also already sent over the prescription for the mycophenolate, the MMF and she gets her medications in blister packs so she’s going to be taking it and I’m guessing if she left without getting baseline labs she’s probably not going to be coming in for monitoring labs.

Dr. Jason Freed: Yeah, that’s concerning.

Dr. Alison Trainor: And so I tried calling her several times but was going to voicemail. She didn’t call back so I tried again but no answer so I called her daughter who said that the patient is bad with her phone and she doesn’t answer when her daughter calls either. So I called her assisted living and she’s a private resident there but fortunately I got a kind nurse who walked down to the patient’s home and called me back from the nurse’s cell phone and so when I finally get a hold of her she said that she doesn’t know how to answer her phone and she doesn’t know how to check her voicemails and traveling into boston to get labs done is too difficult.

Dr. Jason Freed: Yikes

Dr. Alison Trainor: There’s more haha. So I called her PCP to see if he would be willing to get the labs because she goes there more frequently and it’s easier to get to. He said he’d be happy to but that she often misses her appointments and he also has trouble getting in touch with her. So this is the point i felt really torn – this immunosuppressive medication could really help her, but if not monitored appropriately it could really harm her. 

Dr. Jason Freed: oh man, I’ll resist my urge to talk about dice rolls because I really don’t know how to do the markov analysis here. Like what is the probability of benefit and what is the probability of harm and what is the marginal utility of each and how does that compare to untreated NSIP? My brain is going to explode trying to decide the right thing to do. 

Dr. Alison Trainor: I think one of the things I was struggling with most here is that I felt like there must be some way to get this patient the treatment that she needed, but I just couldn’t do it and was banging my head against the wall. So for deep Dive 4 I wanted to go a bit more into what can we do when our patients are having trouble getting the care that they need I reached out to Dr. Kelly Graham, a primary care physician at Beth Israel Deaconess in Boston to help me out.

Deep Dive 4

Dr. Kelly Graham: So I always So I always tell people, you know, you spend all of your training learning the what, like what do, what is the illness? What is the treatment, what is the evidence? And then there’s this whole other stage of your career where you have to learn the how, how do you get the inhaler into the patient’s house, into their mouth, pumped correctly, and then paid for and reme, you know, how do you do that in the context of their family community, um, orientation. And we don’t spend a lot of time learning that in residency or in fellowship. And so we’re left kind of when, when a patient like this comes in, these are the hardest patients I found early in my career to take care of because I felt like I had, you know, I was bringing a, a screwdriver to a, um, to a, I don’t know, a, a cooking class , um, I when I needed a spatula. So, um, and that was act that’s actually a really frustrating place to be in as a physician. 

Dr. Jason Freed: Oh man, I relate so much to bringing a screwdriver to a cooking class. Like even the most knowledgeable evidence based screwdriver doesn’t help you cook. 

Dr. Alison Trainor: A really helpful piece of advice Kelly gave is to think of the patients in terms of where they are on Maslow’s hierarchy of needs.

Dr. Jason Freed: Oh, I love that, but before we jump into that can we just go over what those are for anyone who might not have heard of this before.

Dr. Alison Trainor: Yeah so it’s this pyramid of human needs, with the most basic survival needs on the bottom, and then at the top are the more creative or intellectual needs.

Dr. Jason Freed: Ok, so the bottom of the pyramid is basic needs like food, shelter, a job, health and then the top of the pyramid is things like social connections, sense of purpose, self actualization.

Dr. Kelly Graham: This patient I would argue is probably at one of the bottom two levels this is a patient who’s thinking about, um, basic needs and resources. She’s thinking about transportation. Her health literacy is quite low. Um, and, um, you know, she’s, she’s not able to get up there with you where you need her to engage in pretty risky treatment for a, a really difficult to understand illness. Um, and so what I would be doing is kind of going down and back up with her, try to figure out where she is at that, at that level and addressing those needs. Actually first in parallel with prescribing medications. It’s not that you’re gonna delay treatment, but if you’re not sort of doing that, you’re, it’s like you’re trying to drive over a chasm without a bridge, right? Like you need to build that bridge so the patient can, can meet you there and sometimes you can never get quite up high enough for patients to, to be engaged in their treatment with you. But I would be trying to address her basic needs so that I could ever hope to be at the level of getting to the point where I can give her advanced complex treatment for a, for an illness.

Dr. Alison Trainor: This was so helpful because I’m thinking about mycophenolate and lab monitoring and she’s thinking about her next meal and how she’s going to get to the store.

Dr. Jason Freed: Yeah, I think this comes up a lot in the hospital too where we’re getting ready to discharge a patient and I’m asking them if they have any medical questions and they’re asking about taxi vouchers and if they’ll get home in time to feed their dog.

Dr. Alison Trainor: Totally, And when Kelly gave me this framing I felt really inspired, but also initially a little paralyzed. I did not feel equipped to help her out with the things she really needed help with in order for her to be able to take this medication.

Dr. Kelly Graham: There’s a wonderful pie chart that you can pull up on Google and genetics and clinical care account for like something like 15% of the disease burden that our patients are dealing with. And the rest is all social determinants of health. And it doesn’t matter if your cardiologist or an endocrinologist or whatever, right? It’s, it’s, it’s a massive part of what we do that we have, we turn a blind eye to. And as long as we’re doing that, you know, we’re not, we’re not really meeting our potential as physicians.

Dr. Jason Freed: This is why I need a spatula at cooking class!

Dr. Kelly Graham: I think that the truth of the matter is that, um, we have drawn sort of some false designations over who does what in internal medicine, the inpatient doctor versus the outpatient doctor. The specialist versus the generalist…but the vast majority of us are gonna be like you and I sitting in a clinic taking care of patients over a long-term period. And as long as we continue to say social medicine belongs with primary care, um, and, uh, we don’t embrace this as part of fellowship training as part of an ambulatory skillset that fellows need in addition to a residency skill that primary care doctors need, right? As long as we have that viewpoint, we’re gonna continue to run into this situation.

Dr. Jason Freed: Our job as specialists is at a minimum to identify these barriers to care, and then do what we can to partner with primary care to see where there might be resources to help these patients. I know primary care offices are often stretched so thin but sometimes they can help with engaging in resources that specialty clinics but might not be as connected with.

Dr. Alison Trainor: Yeah, a key point Kelly emphasized to me is that it’s everyones job to try to identify these barriers, and if you or your clinic are ill equipped to help you can always reach out to the PCP to partner with them on these issues. So ultimately, I did end up deciding that with difficulty getting in touch with her and obtaining labs it just wasn’t safe to prescribe her this medication and so I called up her pharmacy and took the mycophenolate out of her blister pack and just left her ILD untreated.

Dr. Jason Freed: Oof yeah, that’s hard. It sounds like you did everything possible to get her the medication safely, but at the end of the day it feels really risky to just leave her on a medication that she could be harmed from if not coming in for monitoring.

Dr. Alison Trainor: Yes, At the time I was pretty conflicted about this decision, but after talking to Kelly I felt much better because I understand she needed to meet her basic needs on the bottom two levels of Maslow’s pyramid and she just wasn’t there and now I have the tools to think about patients with this framing and have tools to get them the necessary care, BUT at the end of the day I was still unable to get her what she needed in order to make sure she was able to take the medication and take it safely.

Dr. Jason Freed: Yeah, I think maybe the one aspect of the conversation we’re missing here is that this isn’t that she’s never going to get the medication. You tried to give it with where she’s at currently, and it wasn’t possible, so the hope is that this is just a pause so that you can address her barriers and try again.

Dr. Alison Trainor: That’s a very important point, but I also just couldn’t help but think what do you do when you have two patients, with the exact same disease, one with resources and health literacy who can take the medication and one without resources and health literacy who can’t. It just feels wrong and I don’t know how to reconcile that. So for Deep Dive 5 I want to dig a little bit more into how we might think about these situations. 

Deep Dive 5

Dr. Kelly Graham: What you’re essentially saying is the conflict between utilitarian and and egalitarian ethics system, right? Yeah. So, um, you want, do you want to just get MMF to as many patients as you can or do you want to focus on giving as many patients the opportunity to MMF as possible? And that is a subtle difference, but it’s a really important one that I would like to spend a little bit of time on because I think you’re having moral distress. This is where these things can help. As a physician, what I try to do is focus on opportunity. So if, if I have that who has resources and family and income and health literacy, there’s not much I need to do for them except write the prescription, have them return for labs to monitor and maybe give them my number to set up a follow up appointment, which they will do on their own for the patient, like the case to build opportunity, I actually have to spend more time on them. Instead of thinking about your time as being equally distributed through all to all of your patients, you think about it in terms of, I distribute it based on how much they need to have the same opportunity for help. Now the patient might not get there and I have to forgive myself of that because I, there, there’s, I, I am limited in what I can do, right? But I have provided them with everything that I can to, to enhance their opportunity for health. And so my metric of success isn’t whether they got there necessarily. It’s whether I’ve done all the things I can do in the system that I work in, which is limited to get them to the same place and I can go to sleep at night feeling good at that. And if they come back the next day and they still haven’t taken their medication, I just kind of back down, go back up. What, what happened? Where was the barrier? What, why weren’t you able to do this? And you just keep at it, keep at it at it, keep at it, you address what you can. And at the end of the day, that’s how you measure your success as a physician.

Dr. Jason Freed: This is such an important point and circles back to what we were talking about before where in medical school we learn the what – what is the diagnosis, what is the treatment, but in reality the harder part is often the how – how do I explain this to the patient, how do I get them the medication.

Dr. Alison Trainor: Completely agree, and what I took away from my conversation with Kelly was that although getting the patients the resources they need to get the care they need might not always be the purview of the physician, it is our responsibility to help recognize these barriers so we can get patients what they need in order to increase their opportunity to be able to get the treatment they need.

Dr. Jason Freed: Well said.

Dr. Alison Trainor: But the hard part for me here was also that I felt like I was being a little bit paternalistic when I took the medication out of her blister pack. It wasn’t safe for her to take it, BUT I was also unilaterally making the decision to remove it because it was so hard for me to communicate with her, so I stopped treating her ILD and she didn’t even know

Dr. Kelly Graham: I don’t think that when you removed the, the medication from her blister pack that what you did was paternalistic. Um, I think that, um, as physicians, we, we also have to have boundaries for how we engage in care. And this comes up all the time with trainees. But essentially, um, if you’re going to get into this, um, exchange or engagement with a patient with a high risk medication or a high risk procedure, the paternalistic thing to do is to assume that you are all knowing and all powerful, and that you have full control over whatever happens to their, their bodies when really what you, the, the agreement that you implicitly enter with a patient when you’re gonna use a medication like M M F that needs a lot of monitoring is we’re in this together, I’m gonna prescribe this medication, but in order to do it safely, you need to come in for follow-up visits and for lab monitoring. And we can only do this holding hands moving forward together. Um, that’s the opposite of paternalism.

Dr. Jason Freed: This is such a great point. Ali, you and I talk a lot with each other about paternalism and its various merits and downfalls, but framing it this way is so helpful. You’re entering into a contract with the patient and they have to hold up their end of the bargain.

Dr. Alison Trainor: Exactly, and the other aspect that was so hard for me was that when she wasn’t holding up her end of the bargain I felt like it was my fault and that I had failed her in some way.

Dr. Kelly Graham: I think your measure of success with her is, did I, did I activate all the systems I possibly could so this could be safe with her? … So there’s ways that you worked around the systems that your patients live in, but then sometimes you can’t. Right. And then this, in this case, there wasn’t much you could do other than just push the pause button and say, this isn’t safe and we need to work on making it safe for you.

Dr. Jason Freed: So Ali, after all these conversations, How are you feeling about this case now?

Dr. Alison Trainor: Honestly, a lot better because I recognize that I really did do my best for her and it’s helpful framing things in terms of Maslow’s hierarchy of needs, but it still does make me sad that we know social determinants of health are a huge driver of health outcomes, yet our ability to address them is so limited.

Dr. Jason Freed: Yeah, I think the way I might summarize my takeaway from all that Dr Graham said was that, yes, social determinants are huge, and yes we are in some ways limited to address them; however, this doesn’t give us a hall pass to just ignore them and not address them. There is a lot more that every physician, generalists or specialists, could be doing to identify these barriers and help patients out. 

Dr. Alison Trainor: Alright, I think that’s a good spot to end on. Should we recap a few learning points?

Dr. Jason Freed: In deep dive 1 we learned that if you see an incidental finding of interstitial changes on a CT scan you can do an ILD protocol CT with prone imaging to help determine if it’s really fibrosis. If it is really fibrosis and not aspiration or atelectasis it makes sense to send them to pulmonary for an evaluation. 

Dr. Alison Trainor: In deep dive 2 we discussed that it’s more important to differentiate the big buckets of ILD rather than to get too far into the alphabet soup. The big buckets are infections, exposures, autoimmune disease, clinical syndromes like sarcoid, and finally idiopathic.

Dr. Jason Freed: In deep dive 3 we discussed treatment. The two main categories are antifibrotics and immunosuppression. Most patients with progressive fibrosis should be considered for an antifibrotic. Many will also be considered for immunosuppression unless they have idiopathic pulmonary fibrosis.

Dr. Alison Trainor: In deep dive 4 we learned that if your patient is having difficulty engaging with the health care system in any way, it can be helpful to think about where they are on Maslow’s pyramid and engage them at whatever level they are at.

Dr. Jason Freed: In deep dive 5 we talked about how it can be challenging practicing in a healthcare system where patients have such different resources and abilities and one way to reconcile this is to think about caring for your patients in a utilitarian vs egalitarian way in which not all patients will be able to get the same care but you need to try to give patients the same opportunity to care.

Dr. Alison Trainor: That is all for today, thank you so much for listening.

Dr. Jason Freed: If you found this episode helpful, please share with your team and colleagues and give it a rating on Apple podcasts or whatever podcast app you use! It really does help people find us! 

Dr. Alison Trainor: If you have a case you’d like to bring on air, please email us at hello@coreimpodcast.com. Thank you to Daksh Bhatia for the audio editing. Opinions expressed are our own and do not represent the opinions of any affiliated institutions.

References

• Expert Panel on Thoracic Imaging:, Hobbs SB, Chung JH, et al. ACR Appropriateness Criteria® Diffuse Lung Disease. J Am Coll Radiol. 2021;18(11S):S320-S329.
• Pritchard D, Adegunsoye A, Lafond E, et al. Diagnostic test interpretation and referral delay in patients with interstitial lung disease. Respir Res. 2019;20(1):253. Published 2019 Nov 12.
• Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47.
• Ryu JH, Daniels CE, Hartman TE, Yi ES. Diagnosis of interstitial lung diseases. Mayo Clin Proc. 2007;82(8):976-986. 
• Wijsenbeek M, Suzuki A, Maher TM. Interstitial lung diseases. Lancet. 2022;400(10354):769-786.
• Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3):814-824.
• Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733-748.
• Adegunsoye A, Ryerson CJ. Diagnostic Classification of Interstitial Lung Disease in Clinical Practice. Clin Chest Med. 2021;42(2):251-261.
• Wells AU, Flaherty KR, Brown KK, et al. Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med. 2020;8(5):453-460.
• Idiopathic Pulmonary Fibrosis Clinical Research Network, Martinez FJ, de Andrade JA, Anstrom KJ, King TE Jr, Raghu G. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2093-2101.
• McLean AEB, Webster SE, Fry M, et al. Priorities and expectations of patients attending a multidisciplinary interstitial lung disease clinic. Respirology.
• Fritz Z, Cox CL. Integrating philosophy, policy and practice to create a just and fair health service. J Med Ethics. 2020;46(12):797-802.
• Graney BA, He C, Marll M, et al. Essential Components of an Interstitial Lung Disease Clinic: Results From a Delphi Survey and Patient Focus Group Analysis. Chest. 2021;159(4):1517-1530.
• Maher TM. Interstitial Lung Disease: A Review. JAMA. 2024;331(19):1655-1665.
• Goobie GC. Neighborhood Disparities in Interstitial Lung Disease: How Do We Capture the Most Vulnerable?. Ann Am Thorac Soc. 2024;21(3):377-380.