Idiopathic Pulmonary Fibrosis Podcast

How to Cut IPF Symptom Distress

Jeffrey J. Swigris, DO; Meena Kalluri, MD


June 07, 2023

This transcript has been edited for clarity. For more episodes, download the Medscape app or subscribe to the podcast on Apple Podcasts, Spotify, or your preferred podcast provider.

Jeffrey J. Swigris, DO: Welcome to Medscape InDiscussion on the topic of idiopathic pulmonary fibrosis, or IPF. I'm Dr Jeff Swigris, and today we'll be discussing palliative and supportive care for individuals with IPF. With me today is the founder and director of the Multidisciplinary Collaborative Interstitial Lung Disease Clinic at the University of Alberta, an award-winning instructor who supervises students, residents, and fellows. Her multidisciplinary clinical team emphasizes collaboration and patient-centric teamwork. Her research focuses on assessments and treatments of dyspnea and other symptoms of interstitial lung disease, developing advance care planning for patients and caregivers, and describing the impact of this care model on health outcomes and related economic analyses. Dr Meena Kalluri, welcome, and thank you for making time for this conversation.

Meena Kalluri, MD: Thank you, Dr Swigris. It's truly a pleasure to be here with you.

Swigris: So, as your introduction hinted, your clinic has been recognized both locally and nationally — even internationally — for its innovative approach to palliative and supportive care, end-of-life care, for patients with advanced lung disease. The multidisciplinary team you direct developed strategies to treat dyspnea, and you and I have had conversations about that. I'm really impressed with how carefully you thought about dyspnea, its measurement, and its treatment in these patients, and how it affects their quality of life. Tell us how you decided to break ground in that area. What has attracted you to that aspect of pulmonary medicine?

Kalluri: Like many clinicians, I was influenced by what I saw in the first few years of starting practice. As soon as I'd completed my fellowship and started the interstitial lung disease (ILD) clinic here at the university, I began to become really interested in patient narratives. I spent a lot of time exploring these narratives in clinic and allowing patients to talk to me about their symptoms and what these symptoms were doing to their routine life. So, for example, if the patients were breathless or were dealing with their cough spells, they would tell me how it was affecting their function, how it was affecting their work or their daily life at home. That's what began to instill in me the profound impact that this condition was having on these patients. And it was not just the patients; it's the family that suffers as well. When I began to process all of these things and to reflect on how many of these things we address in our traditional clinic — the biomedical model, where the emphasis is on making a diagnosis and prescribing pharmacotherapeutic agents — it became clear very quickly that that sort of approach was very reductionistic toward patients. To mitigate some of that and to address this huge spectrum of need, I figured out that I had to retrain myself in some of the ways of assessing patients. So, I found colleagues who were interested in rehab. I found colleagues who were interested in palliative medicine. I shadowed them. I left the comfort of my clinic and did lots and lots of home visits. I spent time with oxygen vendors. I spent time with physiotherapists and respiratory therapists, just to learn from their professional assessments what these patients were going through and what could be done to enhance assessment and care. I took all this knowledge, came back to clinic, and developed pathways and protocols. I had to train my own staff here at clinic, and then sort of create this network of individuals who would help me look after these patients in their community. So, that's how it all started.

What really hit home was this case of a 59-year-old veteran whom I saw in clinic. He had familial IPF and was waiting on the transplant list. When we saw him in clinic, he had become a shell of the person whom he really was. He had been very active, outgoing, volunteered for his community, avid golfer, camper, fisherman, who was now stuck indoors simply because he was breathless. His symptoms had not been managed. He was on high-flow oxygen, didn't know how to navigate life and deal with equipment and needs. So, using this comprehensive, multidisciplinary approach, we focused on his symptoms. We had a systematic way, which you alluded to, of assessing breathlessness. We used oxygen. We connected with the vendors, made sure he had equipment, and used opiates in the right doses — small doses — early on. A long story short, within 2 weeks of him being assessed in our clinic, he was able not just to negotiate his daily life at home, but to have a conversation, have a good night's rest, and, most important to him, he was able to attend all four quarters of a hockey game. So, while he didn't make it to receive his transplant, in the time that was left for him and his family, he was able to achieve some meaning, some joy, some purpose. That's what showed me that palliative care really has a transformative effect for the patients and for the families when it's done well. Even if the outcome is transplantation or death, the way you live your final days, weeks, months can be very powerfully impacted when you use palliative care well.

Swigris: The way that I talk to patients is, "We want to keep you living as well as possible for as long as possible" — whatever that means to the patient and their caregivers. As you alluded, it has such a profound impact not only on the patient but on the whole household as well. What do we say to patients with advanced lung disease, IPF, or other fibrosing ILDs who perhaps are early in the disease course? I'll sometimes say, "Let's think about palliative care." I think that scares patients. But what does it look like for them?

Kalluri: You're right when you talk about the fear that this word engenders — in patients, in the lay public, sometimes in the family members — because when they hear the words palliative care, they're really hearing that their clinician is no longer able to look after them or that "everything possible has been done." Now, we need to have a change in the philosophy of care. Perhaps they must go and visit a different team, see a different professional. It has a lot of different connotations, because from a patient perspective, what I've heard is that even though we're saying, "We want to do more and we want to be able to provide for your needs, but maybe I'm not the most qualified, so I will get another colleague to help me out," what the patient, and sometimes the family, hears is that you, as the clinician who knows them best, as the expert in ILD, are abandoning them. That's what they hear a lot of times when they hear us say we'll probably be sending them over to see somebody else who's an expert in palliative care.

I've also noticed that a lot of times, the way in which we phrase palliative care sets up these myths and wrong ideas in patients' heads. And I've been guilty of that plenty of times myself. Earlier on in my career, I've said things like, "You know, we've done everything we can. Now there's nothing more we can do, and therefore I'll send you to palliative care." So, sometimes we're equating "nothing more to do" with palliative care. If there's one thing I've learned after doing this sort of additive care in practice, it's that it's so far removed from doing nothing more. It means doing more than just disease-focused care. It means taking care of the patient's symptoms. It means taking care of their oxygen needs, their supportive needs at home, managing their family, etc. One of the first things I do is to let the patients and the family know we're not abandoning them in any way. We only want to do more for them, not less. I think that sets them at ease right off the bat. I also tell them that palliative care is quality-of-life medicine. So, we're not just going to focus on antifibrotics or immune-suppressing medication. We're going to start working solidly on what we can do to improve their quality of life. I think that's another assuring thing for patients to hear.

The needs of a patient will differ depending on where they are in their disease trajectory. What I found is that even when there are not a lot of physical symptoms, just the fact that you share with them that they have IPF now breeds a lot of uncertainty. There's uncertainty about progression. There's uncertainty about how or when the end of life may arrive, uncertainty about transplantation and whether they'll get it. It causes a lot of anxiety for patients because we're not very good at dealing with uncertainty. That requires a very sensitive, very informed conversation in which you allow the patient to process that information. You're allowing them to ask questions, and you're providing that time and space for this discussion. So, earlier on, even when they don't have a lot of physical needs, there is an immense need to address this information gap with the patient as well as with their family.

As the disease advances, patients start to experience the consequences of breathlessness, a cough spell, or feeling fatigued. That means now your focus shifts, and you start to talk about symptoms and how you assess them. How do you use nonpharmacologic strategies such as behavioral modification or activity modification? What I found is that this is sometimes the hardest thing to do, because patients have lived their lives a certain way. When they go out for a walk, they walk in a certain way. Now you're asking them to change everything about themselves — how they perform an activity, how they walk. That takes a lot of work, and you must work with physiotherapists, sometimes occupational therapists, sometimes patients' family members to remind them how to pace themselves well.

Then there's the oxygen. I find that that's one of the most neglected aspects of pulmonary medicine. We need to start thinking about oxygen much like we think about any of our medications. It needs to be titrated. Just starting them on an oxygen prescription and leaving them on it is not going to be sufficient; we need to be paying attention to that. Then, of course, opiates are important, but they're not the be-all and end-all of dyspnea management. You need to use them at appropriate times, at appropriate doses. You need to empower the caregivers. You need to share an action plan with them. Just like we have action plans for chronic obstructive pulmonary disease (COPD) and asthma, I think about action plans for symptoms for my patients. I provide them with these action plans in writing so that they feel confident and empowered at home. Then, the final piece is to find some sort of a community network — either a community nurse or a respiratory therapist — who can accompany the patients to their homes and provide them this ongoing education. I would say this is the span of the spectrum. Then, end-of-life care is its own different modality, in which you're talking about other issues, such as symptom crisis. You're talking about the need for palliative sedation, sometimes at home; the need to provide respite to caregivers. It's the same principles, but it's escalated to a completely different level. In a nutshell, palliative care to me is just that you look at symptoms, you assess, you manage it, and then you try to help the patients and the family prepare for a future that is fraught with uncertainty.

Swigris: One of the things you alluded to in that wonderful summary was our communication — physicians or practitioners — about palliative care. We could do a better job of managing expectations up front. Maybe it's just about the way we talk to our patients: "Things may change, but no matter how bad it gets, no matter what this condition looks like, we are going to be managing your symptoms — or attempting to manage your symptoms — so that we maintain your quality of life." We're not necessarily predicting, because we can't really predict disease course with any certainty, although most fibrosis, and certainly most cases of IPF, progress. But saying something like, "If this gets worse, our focus will continue to be on your symptoms and your quality of life. How we manage those may change, but we're still going to be involved." Setting patients up for that early on seems like an appropriate thing to do. Another thing that you mentioned was getting patients to modify activities or change activities. You and I both know that can be really challenging for these patients who, as the disease progresses and they become more short of breath, may need oxygen and can't do the things that they once did. There really is a grieving process. They grieve that former life in which they were much more carefree and active. I think it's important to, as you said, bring in more folks who have the same message: "Here's how we need to think about things now for you to get along in your daily life and accomplish the things that you want to do or that you need to do, with fewer symptoms and better quality of life."

I want to spend a little time talking about narcotics now. Many patients and their caregivers have real concerns and trepidation over using narcotics, despite how often we say, "You know, I think this is going to really help you." Can you share with us your approach to that? How do you start talking to patients about using narcotics to manage dyspnea?

Kalluri: There's a lot of trepidation around narcotics and understandably so, given the ravages of the opioid epidemic that we've all seen. This is coming up in the clinical conversations more and more, especially in the past 4 or 5 years that I've observed. To give you an example, just a few weeks ago, we had a patient who was recently discharged from the hospital for worsening of his IPF. He had hypoxemia, respiratory failure, and ended up being prescribed opiates and was sent home. He went home, and because of his history of substance abuse, he didn't use the prescription. When we saw him in follow-up in clinic, he was very clear that he did not relish the idea of starting the opiates again after having struggled with the addiction and working very hard to overcome it. So, it's important to acknowledge that and to validate a patient's concerns, but then I always link it back to what the patient's goals are. What is the current situation? In this case, we went through this patient's detailed symptom inventory and figured out that he was starting to experience a crisis. I like the idea of not just measuring in terms of its disability, or using the modified MRC (Medical Research Council) scale or just the MRC scale, but to really talk about symptom intensity. It became very clear through assessment that the patient's symptom intensity was high. His intensity was almost 8, 9, 10 out of 10. When we asked him what his goals were, he basically said that at this point, given his advanced disease, he really wanted to be able to walk independently to the dining room, sit down, have a meal without being breathless or coughing too much, and have a conversation with friends. Using oxygen, pacing — all the nonpharmacologic strategies we had optimized — he was not able to do that. The patient became aware of it because of the way we assessed his symptoms and then pointed out the crisis.

That's when I start to have the conversation about opiates, because I am very clear with my patients about how we approach this. We always tell them that we're using the lowest possible doses. We're always using it in a very thoughtful, systematic fashion, only after optimizing everything else. We think of opiates as a last resort. When the patients have clarity and we're able to link how the current strategies are not effective in ameliorating their distress, they become more open, because they are suffering through this crisis every day and most patients want relief. So, in this patient's case, we offered him a prescription. He was willing to purchase the drug, and then we also arranged a Zoom appointment. We had him take the first dose under observation. Unlike using opiates around the clock, sometimes when the prescription is predominantly for treating exertional dyspnea, I like preemptive dosing — meaning, take a small dose just before the activity so that it blunts that distress and you're able to perform without causing too much discomfort. In this man's case, we gave him a supervised dose on Zoom. He takes the dose, can walk back and forth to his dining room, and can have a conversation. His stress was blunted, and his numbers were about 4 out of 10 instead of being 8 or 9 out of 10. So, clearly he was able to accomplish his own goal. He was able to have a conversation, and he saw that the power, the control, was in his hands, and he didn't suffer any consequences from it. He became empowered through that experience. I think it takes time, a personalized approach, and to be very open about communication — when you're going to use it and how you're going to use it. Shared decision-making is the key. Once you provide them with that education, I feel like that's the most empowering part of self-management because it is in their own hands, and they understand when to use it and how to use it.

Swigris: You mentioned low doses. Talk to us about your dosing strategy — that is, pre-exertion or pre-exercise, pre-activity dosing, as well as rescue dosing. How do you approach that?

Kalluri: Most of the literature — if you were to quickly scan through the randomized controlled trials, which have been focused on advanced lung disease, COPD-related trials — tends to use doses upwards of 5 or 10 mg extended-release, sometimes using them twice a day. I know of an IPF study that used about 5 mg of an opiate four times a day. In my own experience, I've found that that's a high dose to use. If you start to think about preemptive dosing and pre-exertional dosing, you really don't need that kind of high dosing and you certainly don't need to give this around the clock. Right off the bat, that shaves the amount of opiate that you're using, therefore reducing the side effects, the potential of abuse, and the potential of overdose that sometimes worries patients a lot. When I say low dose, I'm talking about starting off with something like 0.5 mg of morphine or 0.1 to 0.2 mg of hydromorphone. That's the dose we start off with, especially when the patients are frail, elderly, and opioid-naive. Then we use the dyspnea numeric rating scale (NRS) to titrate and to understand when to increase the dosing. It's also guided by the number of crisis episodes they might experience, and by the optimal use of oxygen and all other strategies. I find that that really is the sweet spot where you've optimized everything else, and you recognize very clearly that the symptom intensity is still there despite optimization. That's the point at which we introduce opiates and titrate very, very carefully and very, very systematically.

The other part that's really important is not just to prescribe a dose and say "Follow up in 1 month, 2 months, 3 months" but to have a case management approach, in which either the nurse from your clinic or nurses in the community are available and there's an ongoing follow-up. Typically, when we start opiates, we're following up with these patients within 2 or 3 days via Zoom or via telephone calls, rather than waiting for an in-person or clinic follow-up that happens in weeks. Close follow-up is the key.

Swigris: You mentioned the NRS. It sounds as if you shoot for a certain target for dyspnea. Maybe cut the score in half or aim to get it below a 3? Is there a target or is it more individualized?

Kalluri: I think it's a combination of both. We tend to use the cutoffs that are available in the literature on symptoms of pain and dyspnea. Generally, anything above 7 is considered quite severe; 4 to 7 is considered moderate. So, I'm aiming for something below a 4. But then these are also very subjective things. In certain situations, I would ask the patient if they are finding significant relief. Because the goal is to make sure that the patient is feeling the benefit, that the patient is seeing an improvement and can at least function at a reasonable level without being distressed.

Swigris: I feel like we could talk for another 2 hours, but we're running low on time. So, given all that we've covered in this short time, for the average practitioner who's treating patients with fibrosing ILD, IPF, and other similar conditions, what should we all be considering differently in terms of our approach to symptom management, palliative care, or quality-of-life care? What would you emphasize for those of us taking care of these folks?

Kalluri: One of the most important things is to recognize that palliative care is now being thought of as active holistic care. I know that all clinicians pride themselves on delivering patient-centered care. We all want to do the best that we can for our patients. The recognition and understanding that palliative care is really a natural extension of routine clinical care is so important, to fully accept that we as practitioners, as providers, can now go forward and say, "Yes, this is my job. This is part of what I do. This is something that I will deliver. It's my ethical and moral responsibility toward our patients because our patients deserve it." That is the new reality.

The next step is to reflect on your own practice, to see where there are gaps and what you can improve. There's no harm and no shame in admitting that there are some things we don't know. We should feel free to acknowledge that and reach out for help — whether it's informal training or formal training — so that we all build competency. It's like anything else in medicine, like doing an arterial blood gas, putting in a chest tube, or doing a central line — it takes practice. So, whether it's to become comfortable understanding how to prescribe opiates; how to titrate opiates; or to have a sensitive, informed, well-meaning advance care planning conversation, it's a skill just like any other skill in medicine.

For low-hanging fruit, I would say, start to incorporate some sort of an NRS scale for measuring symptom intensity. It's relatively quick. It takes no more than a minute or so if you have three or four symptoms to evaluate. Once you recognize that, you will begin to appreciate the burden that these patients face. I have no doubt that clinicians will rise to that occasion when they're faced with that reality and they know that it's their responsibility. I have no doubt at all.

Swigris: Very well said. Meena, thank you so much for being here today and for sharing your expertise. It's been wonderful.

Kalluri: Thank you so much. It's been a pleasure chatting with you.

Swigris: And to the audience, thank you for listening to this conversation on IPF with our guest, Meena Kalluri. There's much more ahead in the upcoming episodes, so be sure to check out the Medscape app, and share, save, and subscribe if you enjoyed this episode. I'm Dr Jeff Swigris for Medscape InDiscussion.


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