Drs Fernando J. Martinez and Steven D. Nathan review the goals of therapy and the importance of non-pharmacological therapies for the management of IPF.
Fernando J. Martinez, MD, MS: The goals of therapy are driven by the patient and their caregiver. One thing we’ve learned is the importance of understanding what the patient and their caregivers want, what their expectations are. This idea of shared decision-making at each step of the process, from diagnosis to therapy to end-of-life care, needs to include what the patient wants and what their caregivers want. It’s appropriate to set up those expectations up front.
There have been a series of qualitative studies, very well-known studies interviewing patients and their caregivers. It’s very clear that several themes are consistent. Those include frustration with the diagnostic process and being able to provide accurate information at each step of the process, a key thing. Understanding what the patient wants in terms of quality of life and symptom management. Cough, for example, is something that frequently comes up. It’s very disruptive to the patient’s life. Then having a sense of what the expectations are with regard to their disease course and when they should start considering advanced care planning. That’s usually done earlier rather than later, but explicitly discussing that with the patient and the caregiver is crucial up front. I’m biased with regard to the advantage that specialist centers provide where there’s a lot of expertise, not only among clinicians but also among the nursing staff, respiratory therapists, and rehab programs.
The qualitative studies have suggested that patients really do feel a more comprehensive view of their picture if they’re discussed in that specialty setting. A lot of this is done in conjunction with our primary care colleagues because we can do a lot of things virtually, which is 1 of the few good things that has come out of the COVID-19 pandemic. But the key thing is to understand what the patient and their caregivers want at each step, from diagnosis to therapy to the very end. That process has to be as explicit and as patient focused as possible.
Supportive care and patient education are crucial in the qualitative work and in my own ILD [interstitial lung disease] clinic. It’s very clear that there’s a lot of misinformation, and it can be very confusing in an area that’s already confusing for patients and their caregivers. We have to understand how to provide accurate, appropriate information for the patient and the caregiver at each step and how to guide them regarding what’s good and what’s not good when they’re looking virtually on the Internet. There’s a lot of misinformation. We published a paper in the “blue journal” [American Journal of Respiratory and Critical Care Medicine] a few years ago from a group that analyzed the accuracy of the online information.
You would look at it and go, “My God, a lot of this stuff doesn’t make any sense.” Being able to ensure that you’re providing explicit information that the patient and caretaker can understand at each step is absolutely crucial. That relates in large part to supportive care, whether it’s at the primary care physician’s office, done by support groups within our ILD programs, or done through pulmonary rehab programs. There are national and international organizations that have developed very rigorous, well-thought-out processes for support. The Pulmonary Fibrosis Foundation I’ve been involved with since its inception, so I’m biased in terms of that group. But they’ve done a wonderful job in providing explicit patient-level information that’s accurate. It’s easy to understand. It’s presented in a very appropriate, often very positive fashion.
I always recommend to my patients that they consider participating and being actively engaged in this case with PFF as a supportive group.
Steven D. Nathan, MD, FCCP: The management of IPF can be divided into the pharmacologic and the nonpharmacologic, and the 2 are not mutually exclusive. It’s not 1 or the other. They both can happen in parallel. When we talk about nonpharmacologic, there are things like pulmonary rehab, which is of benefit to many of these patients at some point in their disease course. Oxygen therapy would be another nonpharmacologic, although arguably oxygen is a pharmacologic intervention that’s generally regarded at nonpharmacologic.
The implementation of supplemental oxygen is usually first with activity, and then many patients progress to needing it 24-7. I would put lung transplant evaluation under nonpharmacological management, but that’s only for a certain subset of patients. Looking for and managing comorbidities would be other nonpharmacological interventions. If they have sleep apnea, we put them on appropriate CPAP [continuous positive airway pressure] or other therapies. I would also put palliative care under nonpharmacological management. Sometimes we get palliative care involved early on to help with intractable cough or shortness of breath, and then it becomes somewhat of an easier transition if or when the patient needs to go on hospice care.
In terms of pharmacologic management, we have 2 approved antifibrotic drugs: pirfenidone and nintedanib. Both were approved at the same time in October 2014, so we have had 7-plus years to use these medications and have accrued a large body of evidence about the utility of both drugs. As I mentioned, implementing pharmacological measures can happen at the same time as nonpharmacological measures.
Transcript Edited for Clarity