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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