Goals of Treating Chronic Fibrosing ILD With Progressive Phenotype


Neil B. Minkoff, MD: Dr Danoff, when you’re choosing therapies or you’re looking to treat patients, how do you establish your goals for the patient? And what are you looking to do besides treating symptoms?

Sonye Danoff, MD, PhD: Knowledge is power here. A big part of what we do in the clinic is spending time educating people about, how do you monitor your disease? What is this pulse oximeter that you’re using? What does that mean? What are the numbers you’re looking for? How do you read your pulmonary function tests? We pull up CT scans so people can look at honeycombing, what does that mean? We try to spend a lot of time teaching the patients what we’re looking at. Even if they can’t be an expert, they can be knowledgeable about what it is that we’re measuring. We are trying to do several things. We have ways of measuring lung function, like pulmonary function testing. But I’ve never had a patient who walked in the room and said, “Doctor, I feel so much better because my FVC [forced vital capacity] is better.” It’s, “I feel better because I can climb the stairs to watch my kid play baseball. Or, I was able to go to my house that’s in Aspen and I wasn’t gasping for air,” and things like that. What we want to do is deal with the holistic needs of our patients. We want to fix the physiology that allows them to live the best life for the longest period possible. But we also have to be very mindful of how multidimensional this disease is. And it can be things like, “I can breathe fine, but I cough. And so I can’t go to church anymore because I’m afraid I’m going to disrupt services. Or, I can’t go to a movie anymore because I don’t want to cough at a movie.” We really have to deal with it at many levels. We have to fix the physiology. But we also have to treat the symptoms. We want people to have the greatest opportunity for the best quality of life.

Neil B. Minkoff, MD: How does the patient severity upon presentation influence the treatments you offer?

Sonye Danoff, MD, PhD: That’s what we’ve talked about, earlier is better. We have more options. It was not uncommon 15, 20 years ago that we typically would see patients for the first time when they needed oxygen. They would have progressed so far that the very first time we met them, it would be us prescribing oxygen. And now we’re fortunate that there is more awareness. The CT scanning is better, the knowledge in the community is better about looking for interstitial lung disease [ILD]. And now we’re seeing patients who come very early in disease when they may be almost pre-symptomatic before they’ve developed shortness of breath, or cough, or other symptoms. The earlier they come in, the more options we have, the more likely we are to be able to slow or stop or reverse the process that they have, depending on what their initial diagnosis is. And obviously the earlier they come, the more options will be available for them. Over the course of time I’ve been doing this, we’ve gone from zero drugs to 2 drugs. And I’m sure there will be more. Honestly, I think that this is an exciting time because there’s such a groundswell of interest in identifying novel approaches to treating interstitial lung disease. I think that those will be therapies that come to fruition for people like Dawn and other people who are going through this pathway now. It matters when they show up because it matters how many options we’re going to have for them.

Neil B. Minkoff, MD: Does anybody else want to weigh in on that?

Justin Michael Oldham, MD, MS: One thing I think is worth mentioning too is even though we do know that nintedanib now is effective for progressive ILD, it’s still important to get the upstream diagnosis correct. Because if you have an inflammatory ILD it would be doing the patient a disservice to forego immunosuppression that could potentially help them and go right to an antifibrotic [medication]. It’s still important to do that work upstream to try to best characterize the patient. It may turn out that immunosuppression and antifibrotic together are the most effective. We just don’t know that yet. There is no one-size-fits-all for ILD at this point. There’s still a lot of work to do.

Neil B. Minkoff, MD: Could you all weigh in on the nonpharmacologic lifestyle changes and things that can be done to try to help manage the disease? Dr Hummers?

Laura Hummers, MD: Nonpharmacologic management is important. Sonye and everybody mentioned education. Educating the patients on what to expect, how things might change, what limitations would be. And importantly, a lot of my patients who have lung disease are nervous about, “What can I do? Can I exercise? Can I travel? Can I get on a plane? Do I need oxygen on a plane?” Those fundamental life questions should be addressed in advance, if possible because those are the things people are thinking about. I’m also a huge advocate for my patients with lung disease to consider pulmonary rehabilitation. This plays an important part. I think from the exercise standpoint it helps us address that anxiety patients might have. “I have lung disease, am I going to make myself worse, risk heart damage, things like that, if I’m going to exercise and I don’t have enough oxygen?”

Pulmonary rehab is a great way to get patients into a program. It gives them a great sense of relief to say, “OK, now I’m exercising in a monitored setting. I know how much I can do before I need oxygen. Or how much I can do, how much I can push myself.” Whereas before they were trying to guess that, or maybe not doing anything at all because they were too nervous about doing it. And then in my patients I’ve mentioned before about reflux, which is a very common cause of cough. But also for some of my patients, it might be a contributing factor for their lung disease.

We’re not sure about that, but people speculate that the reflux and possible aspiration, at least in scleroderma patients, might be playing a role in the progression of their lung disease. We make sure we’re managing that aspect of their disease. For that there are behavior modification things that patients can do that are effective, and pharmacologic treatments for that, not to forget about that component. But I think these pieces are really important for our patients.

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