Why I Want to Disagree When People Call Pulmonary Fibrosis ‘Rare’

Why I Want to Disagree When People Call Pulmonary Fibrosis ‘Rare’

A disease is considered rare in the United States when it affects fewer than 200,000 people (fewer than about 1 in 1,635 people), according to the Rare Diseases Act of 2002. The European Union considers a disease rare if it affects fewer than 1 in 2,000 people. Other countries have their own definitions of a rare disease, which usually revolve around the rate of incidence.

After my idiopathic pulmonary fibrosis (IPF) diagnosis, I began to learn more about rare diseases in general. I was surprised to learn that IPF is considered rare. Nearly 200,000 patients are affected in the U.S. and over 5 million patients worldwide. About 40,000 people die in the U.S. from IPF each year, which isn’t too different from the annual number of 42,260 people killed by breast cancer in the U.S. Yet, it seems everyone is aware of what the pink ribbon symbolizes, but not the IPF blue and red ribbon.

Despite the high mortality rate and incidence rate, many people have never heard of pulmonary fibrosis. That was the case with my friends and family when I shared the news of my diagnosis with them. In light of that, I can understand why it’s considered rare.

Sometimes, I want to loudly and proudly advocate for the needs of patients with IPF. Other times, I want to quietly educate others about my disease. Then there are the times when I don’t want to talk about it at all and try to mold a normal young adult lifestyle.

Much of my world now comprises people affected by this life-threatening lung disease. That is my choice. I feel closest to other IPF patients because they can relate to what I am going through and they intimately understand my struggles. That gives me peace and comfort. I feel less alone as a result.

Sometimes, however, I’ve encountered people outside that community who know about IPF, and suddenly, it no longer felt rare.

Awareness makes a difference

A few weeks ago, I had my car in for service. During the work, the front desk manager and I began chatting. I was wearing my supplemental oxygen, which is uncommon for someone my age. Unsurprisingly, my lung disease came up in the conversation.

This woman’s eyes welled with tears as she told me of her friend who had been diagnosed with familial PF in his late 20s. He had just received a double-lung transplant. Sadly, it didn’t go very well and he is really having a tough time. Since he is now in his early 30s, he is being considered for a second transplant. However, he doesn’t know if he wants to endure that surgery again.

I couldn’t believe my ears. Someone around my age in the same city I live in was also diagnosed with PF as a young adult. Not only did the front desk manager know what my lung disease was, but she was intimately living the complexities of it with her friend.

After that conversation, I reflected on other times I’ve encountered people who knew what IPF is. The number of experiences I could recall off the top of my head surprised me. In those moments, my disease suddenly didn’t feel as rare as the numbers make it out to be.

As a patient with IPF/PF, what can you do to help raise the profile of this life-threatening lung disease?

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Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.

10 comments

  1. Bill Mattila says:

    My dance with IPF has been a long one. It has touched many people in my life. Where as
    I had worked for a major car company and retired. The three people that got IPF worked in same areas that I did. Seems STRANGE that we should encounter a RARE malady.
    Well so Char we seem to have uncovered a strange fact that it not rare as they think it is.
    Two out of three I know where friends or related to my wife’s family. Strange isn’t it……
    It is not rare it is discovered in time so that I think they can jack up the prices of the drugs
    That fight the so called rare diseases. Just a statement of what I have witnessed, I would have posted in blog but had to put it down while it’s fresh in my mind. Keep Breathing….
    William Mattila

    • Charlene Marshall says:

      Hi Bill,

      Nice to hear from you, as always! Thanks so much for reading my column and getting in touch via the comments.
      Wow, it sure does seem strange that three people developed IPF in the same area of your workplace. I wonder if it is worth mentioning to the employer, that they should keep an eye on this or maybe have their workplace evaluated for any hazards/risks? I know that is asking a lot, but you’re very much right, it is odd! Strange how once diagnosed with IPF, suddenly our disease feels less rare when we’re thrust into a life of others who also have it, huh?

      Thanks for sharing your thoughts!
      Charlene.

  2. Peter Terry says:

    I’m in a quandary. A year ago I had a lung CT that showed lung scaring. I’ve been having PF tests every six months and had my second CT at the University of Wa. The Doctor there won’t call it Idiopathic until my tests are not stable. A couple of weeks ago I received a call from the Mayo Clinic offering me an opportunity to be included in a drug trial being conducted by Galapogos for IPF. I had to turn it down for lack of a confirmed diagnosis.

    • Charlene Marshall says:

      Hi Peter,

      Thanks so much for reading my columns and getting in touch via the comments. Sorry to hear that you’re investigating whether or not you’re dealing with this cruel IPF. I know the process of obtaining a diagnosis, or something else, can be daunting in general. Did you explain why you were turning down this opportunity to Mayo Clinic, due to not having a confirmed diagnosis? Maybe they could connect with your physician to expedite the diagnosis process as I know being left in limbo is so confusing. Sorry to hear this is your experience!
      Take care and feel free to write any time.
      Warm regards,
      Charlene.

  3. Carolyn says:

    Dear Charlene,
    It’s funny you are having this discussion, because I have been thinking the same thing! Right before my husband was diagnosed with probable IPF, a friend’s husband passed away from it after a two year fight. He was however, close to 80, so I didn’t think much about it. Once my husband was diagnosed, and I started to read about it, I began to suspect that perhaps his father had it. HIs family always told me he had copd, but he had worn O2 since I had met him and he had those shallow frequent breaths. I didn’t meet his dad’s brothers, but they also had “copd” when they passed. This past week, a priest that had done my mother, brother’s and close friend’s memorial services passed from it.
    Today, my husband was interviewed by a new pulmonologist, who said that it is very probable that all of Gil’s family members with “copd” truly had IPF. He said that CT scans weren’t done like they are now and so many people were misdiagnosed. Also, the meds have only been in use in North America, since 2015-2016 ish, so even if they knew more about IPF, there was very little that could have been done. So, I would agree that although it might have been rare, it is probably going to be less rare very soon. Testing has greatly improved with CT and now even higher resolution scans are being done to therefore diagnose the fibrotic honeycomb like lungs more easily. Perhaps more and more studies will be done in the future as more are diagnosed with IPF. My husband agreed today to participate in a study at UVA that looks at causes of Interstitial Lung Disease (ILD). The purpose is to “obtain information on people with and without an ILD in order to better understand the natural course of different types of ILD.” The other goal is to “collect blood samples to study whether biologic markers can predict or track disease activity.” This study may not help my husband necessarily but it may help his two children. Wouldn’t it be great if studies like this can help you, since you’ve been diagnosed with this disease at such a young age? I will pray so.

    • Charlene Marshall says:

      Hi Carolyn,

      So nice to hear from you – thank you for reading my columns and sharing your comments. Not that I ever want others to endure this disease, but somehow it feels a little less isolating when you know others also going through this journey. Sorry to hear of your husband’s family possibly having IPF as opposed to their suspected cases of COPD. In addition to the terrible side effects, how sneaky this disease is to be diagnosed feels additionally cruel. Thank you (and Gil!) for your willingness to participate in these studies to help all of us living with this disease in future. I am so grateful, as I know many others are as well 🙂

      Warm regards,
      Charlene.

  4. Linda Smith says:

    I was diagnosed for years as being a victim of copd and/or asthma. I had never heard of IPF until I finally found the right doctor.

    I suspect the disease is undiagnosed quite frequently, which makes it an ‘orphan’ disease.

    • Charlene Marshall says:

      Hi Linda,

      Thanks so much for reading my columns and getting in touch via the comments. Sadly, I think you’re correct regarding how often this disease goes undiagnosed. Hopefully the foundations, researchers and medical community out there is looking for ways to expedite IPF diagnosis’ in order to start treatment sooner. Better yet, that they’re getting close to a cure! Thanks for sharing your thoughts 🙂

      Charlene.

  5. Ted Figlock says:

    As a veteran of the Vietnam War (Senior MACV medical advisor Tm 95, attatched to Vietnamese 73rd Med Grp, III CTZ)I took my shots and chloraquine/primaquine malaria prophylaxis. I got a snootful of agent orange, being housed in a former plywood factory compound near the Bien Hoa Airbase, where planes which sprayed AO and insecticide emptied their tanks while airborne before landing. I had my heart attack, coronary rotor-rooter (angioplasty) and stent in 1997. Pulm. Fib. was Dx 3yrs ago and I’m doing well on Esbriet. I’ve lost 40 lbs and now getting dizzy on standing up. Bad enough for a recent 911 call. Dx DEHYDRATION. Any other vets with similar story?

    • Charlene Marshall says:

      Hi Ted,

      Thanks for reading my columns and getting in touch via the comments! I certainly can’t relate to your experience as a veteran, but am thankful for your dedication. Agent orange has been a discussion topic on the PF forums, you can read comments relating to it here if you’re interested: https://pulmonaryfibrosisnews.com/forums/forums/topic/correlation-between-dioxin-agent-orange-and-ipf/

      Sadly, I can however relate to the dizziness and dehydration. I have experienced this several times too, and my dizziness (and fatigue!) have been really bad the last few weeks, despite drinking tons of water, eating right, etc. I see the doctor on Friday for this. Hang in there, I know dehydration can be a big problem for us.

      Warm regards,
      Charlene.

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