Diagnosis: Chronic Fibrosing ILD With Progressive Phenotype

June 26, 2020

A patient perspective of initial diagnosis of chronic fibrosing ILD with progressive phenotype and the subsequent impact on quality of life.

A patient perspective of initial diagnosis of chronic fibrosing ILD with progressive phenotype and the subsequent impact on quality of life.

Transcript:

Dawn Repola: At the time of my initial diagnosis, when the pulmonologist said there was fibrosis, there was also pneumonia, and it was clear that we needed to deal with the pneumonia first. I had discounted that diagnosis of some kind of pulmonary fibrosis. I did a little research on it, and I couldn’t find out much information. There just wasn’t much information available in 2009. I didn’t notice that it was really affecting my quality of life until I had pneumonia, had the second CT [computed tomography] scan, and heard that I had progressed.

When I got that diagnosis of interstitial lung disease, I perked up and said, “Hmm, I might need to pay attention to this.” I called a friend of mine who is a pulmonologist and told him what was going on. He helped me vet my new pulmonologist who I was being referred to, who is an expert in the area. He helped me think about all the diagnostic tests that were going to be run, and he helped me make that decision around whether I should go ahead and have a lung biopsy. When I got my initial diagnosis of ILD [interstitial lung disease], even though I took the medications and understood everything that the doctor said, I still did not take my diagnosis that seriously.

I wasn’t all that careful when he said, “You’re on immunosuppressants, and you need to reduce your exposure.” The holidays came around, and I wasn’t paying attention. I did all my normal stuff. Sure enough, I came down with the flu as well as with sinus infection, and I had multiple things going on. My pulmonologist says, “You’ve got to pay attention because immunosuppression is a big deal.” I began what is now known as social distancing at that point, during the cold and flu seasons.

A moment that really captured my attention occurred in 2018. I had a serious exacerbation. First, I ended up in the hospital, and then second, I lost 12% of my lung function. I went from not needing oxygen to needing oxygen all the time. I got better and had some improvement there, and now I need oxygen when I sleep and exercise and during times of high stress. You’ve just got to pay attention, and there is a portable unit that I carry around with me. I also have a stationary unit that stays in our bedroom. Those things, that exacerbation, is what really got my attention and had me make some significant life choices along with some lifestyle changes.

In terms of what manifestation is the most concerning to me, at first it was just the oxygen, because this is an external manifestation of something that’s going on that everybody else didn’t have any idea about. But all of a sudden you’re walking around, and you have a nasal cannula, you’ve got your tank, or you’ve got your portable oxygen concentrator. That’s something that’s external and something people can see. Coughing was a little bit of a deal, especially now. It’s difficult because if you have a coughing fit, people think you have COVID-19 [coronavirus disease 2019], and I always felt like I needed to add the explanation, “I’m not sick; it’s just my lungs.”

If I had my oxygen with me around people who understood, that was also a bit of an issue. The adverse effects from the immunosuppressant that I was on were also difficult to learn to live with. That made me have changes in my diet. There were some lifestyle changes that I had to make. Now that I’ve learned to manage those adverse effects and deal with them, I don’t really think about them. But initially they were bad enough that I stopped the immunosuppressants for a year.

That was probably what allowed the exacerbation to happen, so it’s very important to take your medications as your physician tells you to.