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Chronic Fibrosing ILD With Progressive Phenotype: Clinical Burden


Shared insight on the clinical burden of chronic fibrosing interstitial lung disease with progressive phenotype, including first-hand patient experience.


Neil B. Minkoff, MD: There is an economic burden that payers are looking at, and therefore the people who pay the payers, which are the employers or the government. But there is the big clinical burden. This is what we’re relying on your expertise for today is to help us understand the clinical burden of lung function, the delays in diagnosis with the downstream effects of that are, and then the limited treatment options. Dr Danoff can you share your experience with the clinical burden?

Sonye Danoff, MD, PhD: This goes back to a couple of the themes we’ve talked about before which is, identification of patients who have interstitial lung disease and understanding what the etiology. Once the etiology has been identified, there is a therapeutic option that is likely to benefit the individual. And treat the individual with appropriate therapy. We also worry about the use of medications and therapies that are not effective. We see lots of patients with ILD who are put on inhalers and various and sundry medications, which don’t have any efficacy. This is a cost to the patient because there’s the cost of the medication, cost of having to remember to take it, and the adverse effects. Ideally what we want is to get the diagnosis as quickly as possible. What is the best therapy available? Then follow the patient to determine if that therapy is actually working. That is if it’s stabilizing and improving their lung disease. And if it’s not, what are the alternative options. For a lot of patients as Dawn pointed out, there were many sentinel points. One of them is when people need to use oxygen. And all of a sudden their disease becomes very public because people who might not have recognized that there was a problem, now see them wearing oxygen, they know that there’s problem. We see plenty of employers who are uncomfortable with having their workers come back in wearing oxygen. It can be a burden in their employment history. As the disease progresses, people are less able to do the things that matter to them. Depending on your phase of life, they might be taking care of your children. This also affects people who are quite young. And so it can have a vast impact on a very wide age range. And ultimately it can be life limiting. At that point then we’re also determining the costs of having to consider lung transplant in our patients, which is a rare and very expensive treatment. And is unfortunately not available to everybody. It has an enormous clinical burden and an enormous burden in terms of quality of life and length of life of our patients.

Neil B. Minkoff, MD: Dr Hummers, in your interaction between trying to deal with the clinical burden while there are those around you the payers, particularly dealing with the economic burden, are they aware of what you’re doing and what your patients are going through? Do you find that they are interested in outcomes and on productivity for their patient populations?

Laura Hummers, MD: I don’t interact that often with payers other than trying to get our medications approved. But it is a challenge. And it’s a particular challenge in rheumatology where until nintedanib came along, there has not been one FDA approved drug to treat systemic sclerosis, for example. Trying to convey that the patients are sick, they’re having progression, and we have limited data for some of our medications. There are reasons why we’re choosing one drug over another drug depending on what manifestations of the disease they have. It doesn’t come across well and ICD 10 data and claims data. I write a lot of letters, right. We have no FDA approved drugs for the most part and everything I give somebody is off label, until nintedanib came along for scleroderma patients. And I had to write a lot of letters explaining what their burden is and explain this person had to stop working and they’re having trouble doing their activities of daily living. You need that level of detail to convey the whole picture.

Neil B. Minkoff, MD: Ms. Repola, you’re going through this process as a patient you’re involved in seeing the clinicians, being monitored. How is that being conducted? And what’s your experience been like as a patient going through that level of the monitoring?

Dawn Repola: He had the pulmonary function tests about every 3 months, and I have a 6-minute walk test, about every 6 months, and then once a year I have a high-risk CAT scan. Other than that I just sort of monitor at home. I use my pulse oximeter and I’m now at the point where I need oxygen when I exercise, sleep, and during high stress times. I have a portable oxygen concentrator and I sort of just monitor on my own. That’s what my monitoring looks like.

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