Couch Talk: Bill and Bev’s IPF Story
VO: It is important to remember that everyone’s experience with IPF is unique and you should talk with your own doctor about your specific diagnosis and clinical status.
Bev: We were at a loss for words you know, feeling like we'd just been blindsided when the pulmonologist told us that Bill had IPF.
Bill: You know, we sat there listening to what the doctor described, what IPF was doing in my lungs, but you know, we weren’t ready to hear any of that.
Jorge: IPF can certainly be a confusing and overwhelming disease. So, let’s break it down. Idiopathic means unknown cause; pulmonary means impacting the lungs; and fibrosis means scarring. So, in short, it’s a disease where excess scar tissue builds up in the lungs from unknown causes, but known causes of lung fibrosis include smoking, certain medications, radiation, genetics, acid reflux disease, and certain environmental exposures.
Dr. Bemiss: IPF affects up to 132 thousand people in the U.S. As a matter of fact, IPF falls into an even larger group of diseases called the interstitial lung diseases, also known as ILD, which includes all diseases that have inflammation and/or scarring in the lungs. Some interstitial lung diseases don't include scar tissue. When an interstitial lung disease does include scar tissue in the lung, we call it pulmonary fibrosis. As the scar tissue worsens, it causes destruction of the lung tissue and may make parts of the lungs look like honeycombs when viewed on high-resolution CT scan.
Jorge: Over time, the scar tissue makes it harder for oxygen to enter your body, which results in impaired lung function, and it becomes hard to breathe. Bill, what was your experience that started you off on your ILD journey?
Bill: Well, we discovered my IPF quite by accident. One spring I noticed that I had a growth on my sternum right here, about the size of my thumbnail, and obviously I was concerned about that and so I asked my general practitioner to take a look at it at my next visit. He assured me it was nothing to be concerned with, but he ordered me an HRCT scan anyway.
Bev: After reviewing the CT scan, the doctor said, "We have good news, and we have bad news."
Bill: Well, the good news was the growth in my sternum was indeed benign and truly nothing to worry about. The bad news was they found something they weren't quite looking for. There was a shadowing in the lower lobes of my lungs, and my doctor suggested that I see a pulmonologist, which I did. And after reviewing the HRCT scan and hearing the crackling in my lungs, my pulmonologist diagnosed me with idiopathic pulmonary fibrosis, also known as IPF.
Bev: Boy, one of my first thoughts then was maybe the pulmonologist was wrong, you know. Bill didn't have any symptoms. He was so healthy; you know he just had this little growth on his sternum, and then we found this?
Jorge: Of course, Bev, a diagnosis of IPF can be surprising. Everyone's experience with IPF is unique, but the typical symptoms of an ILD with scarring include shortness of breath, dry, hacking cough and fatigue. Also while your pulmonologist listens to your lungs, they may hear what’s been described as a Velcro-like tearing sound. All of these symptoms are often misdiagnosed as asthma, COPD, and congestive heart failure.
Dr. Bemiss: Because it can be hard to diagnose, your healthcare team should consider your medical history and your physical examination in addition to other test results including a high-res CT scan like in Bill's case or a lung biopsy. But your health care team may conduct a number of other tests to assess your overall lung health.
Bev: You know, it was easy to forget about Bill's IPF, even after his diagnosis. He still wasn't showing any symptoms and he continued doing everything he liked doing like golfing and fishing, spending time with family. We even still went on vacations and mission trips. But as the years passed, Bill's lung function tests showed that his disease was progressing. I remember the doctor saying, when Bill was first diagnosed, that some people go along for a long time and then suddenly they drop off a cliff.
Jorge: And that can certainly be tough to hear. The course of IPF is unpredictable. In some patients like Bill it can progress at a slower pace; but in others, it can progress rapidly. In any case, however, it's a serious, progressive and irreversible disease. Specifically, people with IPF may experience sudden and severe worsening of symptoms known as an acute exacerbation. The symptoms of an acute exacerbation are associated with accelerated disease progression. Now the cause of an acute exacerbation in IPF is still unknown and is being investigated.
Dr. Bemiss: Therefore, an early diagnosis is important. The sooner you receive a diagnosis the sooner you can work with your healthcare team to assess your treatment options. Although there's not a cure for IPF, there are FDA-approved medications available that may help slow the progression. Common approaches to managing IPF symptoms include pulmonary rehabilitation and oxygen therapy. It's important to discuss treatment options with your healthcare team.
Jorge: It's definitely important to make the most of your time with your healthcare provider. If you are confused or unsure about anything, don't be afraid to ask questions. And be sure to track your signs and symptoms no matter how insignificant or unrelated they may seem.
Bill: I'm fortunate to have great relationships with all my doctors. I can talk to them openly, and if they tell me something I don't understand or don't agree with, we sit down and we talk about it until we're on the same page. My first pulmonologist told us about treatment options, but they seemed way beyond our capabilities. Later on, my new pulmonologist told me there were programs out there that will offset the cost, and I was able to get on a treatment program.
Bev: You know, we have also learned not to run ahead of ourselves, not to worry about what's going to happen tomorrow because tomorrow's going to take care of itself, not to let IPF define who we are and how we live our lives. We do plan and we do prepare, but we have to be grateful for today, just today.
Bill: Yeah, you know, I'm still able to do the things I love to do–going for walks, golfing, fishing. I simply do them at a slower pace. My faith is at the top of that list for me. Believing that God is in control and he is trustworthy to his promises has kept Bev and I in a positive frame of mind, and helped us to remove signs of depression or fear or self-pity from our thought process.
Bev: You know, we've had many exciting chapters in our 57 years together, but our story's not finished yet, and we look forward to many more years to come.
VO: Don’t watch and wait! It could be IPF, and if left undiagnosed and unmanaged, progression leads to worsening symptoms and deteriorating health.
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Hear Bill, living with idiopathic pulmonary fibrosis (IPF), and his caregiver Bev, discuss their experience with IPF, a common type of interstitial lung disease.