Patient Management of Chronic Fibrosing Interstitial Lung Disease With Progressive Phenotype - Episode 17
The importance of comprehensive multidisciplinary care in chronic fibrosing interstitial lung disease with progressive phenotype.
Neil B. Minkoff, MD: We’ve talked about the multidisciplinary, multifactorial nature of care for chronic fibrosing ILD [interstitial lung disease] with the progressive phenotype, but let’s revisit. If we could understand the role of the rheumatologists, the pulmonologist, and other specialists who may be looking into these diseases or trying to prescribe nintedanib, what do you think the appropriate roles are as other clinicians are tuning into this?
Laura Hummers, MD: From a rheumatologist standpoint, when there is an established diagnosis of ILD but the underlying etiology is not clear, the rheumatologist plays a role in trying to help figuring out where they fit in from a phenotype standpoint. We’re screening our patients who have established rheumatic disease for lung disease so we can figure out who is going to be in that progressive phenotype. We’re not great at this yet. We certainly need to do a better job to identify the patients earlier who are going to progress and get those progressive patients on therapy. And that can be a shared process with our pulmonary colleagues. It’s highly variable. We are in a specialized center, and we treat scleroderma patients. We end up managing a fair amount of the ILD ourselves. Oftentimes we do it in collaboration with our pulmonary colleagues, particularly if things are more complex or not quite so clear. Having the conversation is the most important thing about who is going to take charge from a management standpoint. And then having these conversations about the other manifestations of the disease that might also need therapy. Because that may have an impact on the medications we choose to treat their lung disease.
Neil B. Minkoff, MD: And from our pulmonary colleagues?
Sonye Danoff, MD, PhD: There is a huge amount of shared decision-making here. As Laura very rightly points out, you know, somebody has to be the point person for any of the therapies that we’re giving but particularly for antifibrotics that have a lot of adverse effects. Because we’re not managing just physiology, we’re managing quality of life. There are times where adverse effects become a very limiting feature in terms of treatment, and that’s true with many of the medications we give.
We have to be very sensitive to cost of medication. We have to be sensitive to the potential adverse effects of medication. We work with patients to see what we can do to mitigate those adverse effects. And then if we can’t mitigate those adverse effects, we need to consider what the alternatives are. I know both in my work in the IPF [idiopathic pulmonary fibrosis] patients but also with patients who have been prescribed nintedanib for progressive fibrosing lung disease, that is sometimes a critical issue. Being able to control adverse effects to allow it to be a satisfactory use of the medication. I don’t know if you have a different experience with that, Justin. What you would do?
Justin Michael Oldham, MD, MS: You hit the nail on the head. I will put in a plug for the multidisciplinary teams outside rheumatology and pulmonology. Having a good chest radiologist is critical. Because while we all get better looking at our own CT [computed tomography] scans, there are some subtleties we probably miss that a radiologist is very helpful with. We don’t just come up with a multidisciplinary diagnosis. We have a multidisciplinary follow-up meeting to make sure the patient is doing well, having the radiologists look for any subtle sort of changes on the CT scans is quite helpful.
Sonye Danoff, MD, PhD: I would be remiss not to put in a plug for our nursing staff, the frontline folks who manage adverse effects and often the first people our patients reached out to. We could not manage these medications without having their assistance and their engagement with the patients.
Justin Michael Oldham, MD, MS: I agree.
Neil B. Minkoff, MD: A lot of payers are restricted nintedanib prescribing to pulmonologists, pulmonologists, or rheumatologists because of some of the things that you described. Do you think that’s appropriate?
Sonye Danoff, MD, PhD: I have to say that these drugs require a lot of monitoring, and I’m just not sure every office is capable of doing it. Laura can certainly speak to this. But when you come from a background as giving patients immunosuppressants where you’re often following white [blood cell] counts, LFTs [liver function tests], and renal function, you typically have a system in place that allows you to monitor safety labs.
Having that capacity to monitor safety labs and having the commitment and the people to do it, it is incredibly important with the antifibriotics. The last thing we want to do is harm our patient with a medication that we’re using. I’m not sure whose offices are. I’m not sure if the pulmonologist’s office is capable of monitoring these drugs adequately. It has to be a commitment on the part of the individual physician to say, “Yes, I am willing to make sure that the patient is getting their therapeutic monitoring labs and that’s somebody’s feeding it back to them if there is an abnormality.”
Justin Michael Oldham, MD, MS: I totally agree, and as Sonye hit on we’re specialists in ILD. We’re both her Pulmonary Fibrosis Foundation care centers, centers of excellence and we see this day in and day out. And being managed in the community is very much possible so long as you have the resources to do so. But you know the American Thoracic Society recommends this for IPF, and that should be true of most patients with ILD: If possible they should be seen at a care center just to get the ball rolling on the right treatment and ensuring that the appropriate follow-up and that holistic approach that Sonye described is being employed.
Laura Hummers, MD: Yes, having that threshold of experience with these certain medications is so helpful in terms of mitigating adverse effects up front and having nursing staff who can help the patients continue to manage adverse effects and their therapeutic monitoring. If this is not something you are doing as a routine in your practice, no matter what kind of practice you’re in, this is something that is going to be difficult to be done, in practice that is primed and ready to do it. Not to mention the fact of the coverage issues and prior authorizations and things like that. If you don’t have the infrastructure in place for that, it is going to be hard for you to manage these medicines.