I Have Pulmonary Fibrosis… You Mean I Should Exercise?

Yes!

But, the first thing to say is that exercising regularly, even when you have completely normal lungs (and heart and not to mention knee and hip joints), is not easy—if it were, everyone would be doing it…and every other commercial on TV would be for something other than a “get fit quick” remedy.

We’ve all heard it from the time we were youngsters, “Get out there and exercise!” But really, why should we? Let’s cover the non-physical effects first; there are ample data out there to prove that exercise leads to:

• More energy
• Reduced stress
• Improved sleep
• A sense of achievement
• Improved mood
• Improved cognition

The biochemical effects of exercise include—among several other things—better blood sugar and lower cholesterol. The physical effects include stronger bones, improved body shape, reaching and maintaining a healthy weight, better balance and mobility in addition to effects on the heart and skeletal muscles.

Aerobic exercise also improves cardiac conditioning; that is, the heart can pump blood (and by extension, oxygen) to working muscles more efficiently—the heart muscle itself can do more work with less fuel…it is better conditioned or “in better shape.” Exercise also makes the skeletal muscles work more efficiently: they are stronger, more resistant to fatigue and function more ideally at the cellular and molecular levels—in English, that means they can also do more work with less fuel (read “oxygen”). That is, they are better conditioned or “in better shape.”

Exercise flow chart

But Doc, I have pulmonary fibrosis! Aren’t my lungs the stop gap here? Recall what I wrote in my last blog about the roles of the lungs, heart and muscles during exercise. Oxygen is the fuel; the lungs get it in the bloodstream; the heart pumps it to the muscles; and the muscles “pull” it out of the bloodstream and turn it into energy so they can do work. If the lungs are scarred (i.e., if pulmonary fibrosis is present) and not functioning normally—thus limiting the efficiency with which they do their job of getting oxygen into the bloodstream—a well-conditioned, efficiently working (read “in shape”) heart and skeletal muscle system is vital to “make up for” the under-performing lungs.

Remember, a conditioned—or “in shape”—heart and muscles are able to do more work with less fuel. Imagine if the lungs are under-performing AND the heart AND muscles were “out of shape!”…not a great situation.

Remember Judy from my last blog? Those big muscles that carried her to her mailbox and up that incline? Well, it turns out that, for IPF patients, fitness and strength of the large muscle groups of the lower extremities is really, really important. It shouldn’t be all that surprising to hear that if two IPF patients have the same forced vital capacity (FVC) and diffusing capacity (DLCO), the one with the stronger quadriceps muscles will have a better exercise capacity than the other. The interested reader can check out this article by clicking HERE by a very solid Japanese pulmonologist—Osamu Nishiyama—who also has an interest in exercise and quality of life in patients with pulmonary fibrosis.

So, patients with pulmonary fibrosis should exercise—not because exercise will make their lungs work more efficiently (although there are studies underway looking at respiratory muscle training for patients with lung disease)—but because it will allow the heart and muscles to do more with the oxygen that is available. For now, for patients with pulmonary fibrosis, we encourage aerobic exercise (the kind that gets your heart pumping fast and keeps it there for a while) and resistance training for the arms and legs (recall what Dr. Nishiyama’s work has shown). In my next blog I’ll cover the specifics.

  
33 Comments
  1. I have pulmonary fibrosis secondary to scleroderma. After taking Cellcept for five years my skin started to soften. Along with building the muscle strength I lost I also started some Yoga poses focusing on my lower back and pelvic region. I noticed an improvement with my breathing When i started to gain flexibility. Could this be part of the same benefits you gain from quadricep strengthing?

  2. Hi Ed:

    Thanks for the comment and the question. I love the idea of yoga. I think it’s probably under-utilized in patients with pulmonary fibrosis. There is a growing literature on yoga in COPD, and I’m happy to direct you to some studies if you’d like.

    I have several thoughts for you. Can you answer a couple questions about your case?

    1. Specifically what do you mean that your breathing got easier? Did you feel like you could take a deeper breath? Did your lung capacity improve? Did your oxygen saturation get better for a given activity?

    2. Do you have diffuse skin involvement (i.e., diffuse cutaneous systemic sclerosis)?…specifically, does the scleroderma involve your trunk?

    You probably know that pulmonary fibrosis makes the lungs stiff, and the stiffness restricts the lungs from filling…the lungs can’t expand as fully as they could if they were normal, so lung capacity is decreased. Things OUTSIDE the lungs can also restrict them from filling. For example, in people who are overweight, the extra weight in the abdomen and/or chest can restrict the lungs from filling…even if the lungs themselves are perfectly normal, lung capacity can be low.

    I have systemic sclerosis patients with scleroderma of the trunk, and the thick skin of the trunk restricts the lungs from filling to full capacity. If you have scleroderma that affects the skin of the trunk, and the Cellcept softened your skin, then the skin of your trunk would be less restricting, your lungs could expand more and your breathing would “get easier.”

    3. In some patients with systemic sclerosis (scleroderma), Cellcept actually improves interstitial lung disease. It turns out that the most common form of pulmonary fibrosis in scleroderma is a mixture of inflammation and fibrosis (under the microscope, we called the pattern fibrotic nonspecific interstitial pneumonia)…Cellcept could improve the inflammation and thus make your breathing easier. We’ve published a couple studies on this (here’s a link to one of them http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3676865/), but if you’d like me direct you to others, I’m happy to do so.

    4. Do you also practice yogic breathing? I think this controlled, conscientious breathing can go a long way toward making people breath easier…not sure if this has any role in your case.

    5. Depending on what yoga poses you’re doing, you could absolutely be strengthening your quads, glutes and hip flexors…thus making them more conditioned.

    Looking forward to your replies, so I can tighten up my answers for you.

    Jeff

    • I do have systemic scleroderma with skin involvement to chest and trunk. It was severe during the onset but now there is some skin softening.

      I had a lot of weight loss and that made it hard for me to breath with my skin tightening in the trunk, chest, and lower back. I had a tugging feeling in my diaphragm area and lower back. With the Cellcept, yoga and exercise I am able to strengthen my chest muscles and improve my (at least in my mind) capacity which has helped my 6 min walk. PFT’s are the same.

      It has helped with GERD and bloating by getting rid of the excess gas in my GI track. My Raynaud’s has benefited also by increased flexibility which has improved blood flow.

      I see a scleroderma specialist and they also agree that the changes are great considering my skin involvement.

      I started with an iPhone app, doing as many poses as I could tolerate.

  3. I also have systemic sclerosis. I have been walking 3 miles a day, six days a week since August. I am having tests done next month to see if that has helped my capacity. I seem to have more energy. I don’t sleep 14 hours a day anymore.

    I am interested in yoga, but I need the program for people who can’t get up and down.

    Just some random input.

  4. Hi Susan:

    Random input always appreciated. Great job with your walking program…keep it up; it’s really important, and you’re seeing the benefits…outstanding!

    I’m not exactly a yogi. I used to do yoga weekly when I was at Stanford, but that was many years ago, and I haven’t kept up with it at all.

    The cool thing about yoga is that it’s yours, right?…it’s your practice, and you can make of it what you want. Most poses have modifications for people who can’t bend themselves into a pretzel. There’s even “office” yoga or chair yoga that people can do at work…maybe try a Google search on those.

    Another idea if you’re really interested is to go to a yoga studio near your home and see if someone there can recommend either poses or an intro class that’s geared more for those of us who are less-limber. Those folks are usually really eager to help, and something like a beginning Vinyasa flow class may be right up your alley.

    Let me know what you find out.

    Jeff

  5. A well written article that mirrors my thoughts and results from exercise. When first diagnosed in 2011 I had a hard time walking around our local shopping mall without heavy duty pursed lips breathing or stopping to catch up. I started an intense program of walking, then race walking and eventually adding sprints to the mix along with swimming and weightlifting. I know run/sprint twice a week, swim twice and week and lift once or twice a week. Along with modifying my diet (Paleo) and stress reduction I’ve eliminated all prescription medicines (Wellbutrin, Nexium, Crestor) and my blood panel has improved, I’ve stabilized my IPF and my FVC and DLCO scores are exceptional to the point where my Pulmonologist (Dr. Christine Garcia at UT SW) has classified me as pre-clinical. I was diagnosed locally in Dallas as well as spending a week at National Jewish Health with Dr. Solomon and team. BTW I am now 75 and my wife are I are celebrating our 50th wedding anniversary this month. One of the ‘former’ Pulmonologist I had locally advised me shortly after diagnoses to take my 50th celebration in 2012 because I would not likely be able to in 2013…

    What’s bizarre to me is none of my local Pulmonologists at UT Southwestern are interested in exploring why my stats have improved. Could it be there is no money or expertise in researching something with little or no money attached to it?

    • Bill,
      My husband was recently diagnosed with IPF. We live in Dallas and wonder if you hold any seminars for patients to learn more about your success. If the doctors aren’t interested, there are a lot of patients who need your help.

  6. Hi Bill:

    Thank you so much for your comments and for sharing your regimen. Keep up the outstanding work!…and 50 years of marriage…incredible! You’re an inspiration on many fronts.

    I must say you’re in great hands there with Christine…and clearly I’m biased, but I really like Dr. Solomon as well.

    If anyone were capable of exploring your genetics to try to learn more about your case, it would be Christine. Being a simpleton, I couldn’t ignore the work you’ve done with your exercise as being a huge contributor. The question of what diet may have beneficial effects in IPF is definitely worth exploring…it’s absolutely true that we are what we eat. For cells in the body that “turn over” (i.e., die and get replaced)–as alveolar epithelial cells do–I can’t believe that what we put in our body would have absolutely no effect…sorry about the double negative.

    Great to hear from you Bill. I’ll tell Dr. Solomon our e-paths have crossed.

    Jeff

  7. I was dx nsip 5 years ago, was on oxygen 24/7. Two months ago I realized I no longer need oxygen (had to walk new puppy without my tank). I tested and logged my exercise and sats, went to the lung doc, requested an overnight oxemitry, which I passed. They tell me my exercising, ie walking 2 to 3 miles a day, caused my good lung cells to get better. I like your explanation better. They should be picking up the concentrator in the next couple of days.

  8. Hi Dorothy:

    Fantastic.

    There’s a whole bunch of reasons why your sats could be better. One is that NSIP is usually a mixture of inflammation and fibrosis (in fact, there is a form of NSIP that is purely inflammatory…more on that in an upcoming blog), and the inflammation may improve. Why it would happen after five years is a mystery.

    Whatever the reason, it’s good news.

    Just to be safe, I’d encourage you to “check in” with your sats every now and then, and contact your doc if you consistently see numbers you “don’t like.”

    Keep up the great work with your walking, and enjoy your puppy.

    Jeff

  9. I was diagnosed five years ago with IPF. I am 74 years young and walk 4 miles
    in 52-55 minutes 5-7 days a week. I am not on oxygen and my 02 level is 98.
    I have a few issues but nothing a brisk walk won’t help!
    Thank you so much for your caring and sharing. you make my day!
    Jane

  10. Hi Jane:

    Thank you for sharing your comment and experience.

    Keep up the outstanding strong work!

    Jeff

  11. Dr Noah Greenspan of Pulmonary Wellness Centre (http://pulmonarywellness.com/) in New York City started a series of pulmonary wellness webinars tonight.

    Excellent overview of why, despite the traditional viewpoint that pulmonary rehab doesn’t change your PFTs, that a properly administered pulmonary rehab is what every patient with PF needs and should want.

    They are going to try to make the webinar available for latr viewing. The next one is February 26th on Breathe Deep! Your Absolute Best Breathing Techniques Ever!

    Dr Noah also has a blog on his website.

  12. Thanks for passing along the info Barbara.

  13. Hello, All: I like what I have read above. My feeling is that just about everyone will benefit from the right exercise program. With that in mind it is important to get clearance from your physician to make sure that there is not a legitimate reason why exercise could be dangerous to you. I LOVE treating patients with pulmonary fibrosis and one of the reasons is that I like clearing up misconceptions.

    One of the reasons why exercise can be “controversial” in PF is FEAR and I don’t just mean on the patient’s part. I mean on the clinician side as well. First of all, people with PF have some of the highest respiratory rates and lowest oxygen saturations of all patients we see. In fact, many people with PF look like they are in respiratory distress even at rest and so, naturally, one of the last things one might think to do would be exercise.

    This is exactly the predicament that my mentor, Dr. Horacio Pineda and I found ourselves in in 1995 at the Rusk Institue in NYC. We were starting to see a lot of patients with IPF, patients whose saturations would often be in the 80’s at rest and even worse with even the slightest activity ON OXYGEN. Most people were afraid to touch them from the rehabilitation perspective.

    Then we made a decision. If there truly was nothing that could be done to improve the condition (or make it worse) we had nothing to lose. We began exercising people on higher levels of oxygen and on masks instead of nasal cannulas. This allowed us to provide up to 15 lpm which on a mask is nearly 100 percent oxygen, this compared to 40% maximum that we could provide using a cannula.

    Guess what? It worked. People were able to remain saturated during exercise and over time, we began to see their sats at rest improve as well even on lower levels of oxygen even if there was no significant change in lung function. But guess what? In many cases there was.

    In my experience, there is really only one way to improve lung function and that is through VIGOROUS exercise. The key is figuring out a way to get you there.

    For those of you that missed my first lecture/webinar, Ultimate Pulmonary Wellness: Your Best 2014 and Beyond! I will be reviewing some of these principles in a town hall style webinar on Feb, 5 at 7:30 PM Eastern Standard Time.

    You can register at: https://attendee.gotowebinar.com/register/8376808912560412929

    Hope to see you online!

    noah

  14. My grandmother was recently diagnosed with Pulmonary Fibrosis. We have been told there is nothing we can do. I was wondering if there were support groups or anything. This is all very new to us. I hope to find some support here.

    • Hi Jessica,

      Thank you for your post. I’m sorry to hear about your grandma’s recent diagnosis. A new diagnosis of pulmonary fibrosis (PF) can certainly be overwhelming – there is so much to learn.

      Seeking support is absolutely beneficial, and luckily with the Internet, you can find support even if you don’t have a local PF support group that meets in person. The Pulmonary Fibrosis Foundation (PFF) has a list of most if not all support groups that meet locally. The list is broken down by state and includes meeting times, location and contact info. The PFF also has online support groups (Inspire) for both people living with PF and their caregivers. You can find information about local and the Inspire online support groups here: http://pulmonaryfibrosis.org/patient.

      There’s also a site called PatientsLikeMe that is worth looking into. And if you’re on Facebook, I’d suggest checking out the different groups devoted to providing information and support for those affected by PF. And if/while you’re looking for information on PF, you can also check out Dr. David Lederer’s blog (just google him and you’ll find it).

      Finally, you can always come here (the blog or forum) to ask any questions you might have or even to share your and your grandma’s experiences learning to live with PF.

      You’ll find there’s a strong community of people affected by PF and if you have questions or concerns along the way, there will be someone there to help. You aren’t alone.

      Sorry for such a lengthy response, I just wanted to make sure to provide you and everyone else with as many options for support as possible. If anyone knows of any other resources, please do share.

      -Amanda

  15. Hi, I’ve just found your blog today and found it informative and read-able – thanks!

    I’m not sure where to ask this but my respirologist tells me I have fibrosis, due to an assumed diagnosis of sarcoidosis. I have a periodic crackle/wheeze in my lungs which had my doctor do a chest x-ray. That showed bilateral hilar adenopathy. I’ve since had CT scans which he says show granulomas and fibros. I go for breathing tests every 6 months and follow up with him. So far, no progression and no treatment, although since I saw him 6 months ago, I think the breathing has been worse.

    My symptoms are cough, which is sometimes productive, chest pain upon exertion (snow shoveling or using my elliptical trainer for example). Exercise also increases the coughing – it feels like my lungs are being stretched… kind of. Ventilin improves my lung function about 10%.

    Questions:
    Is my elliptical trainer a good type of exercise? If so, how do I approach this? Exercise until my lungs hurt?

    Should I push for a definitive diagnosis? The respirologist is balking at doing a biopsy / bronchoscopy since it’s invasive and he is quite confident in the sarcoidosis diagnosis.

    In your opinion, how much is open to interpretation with chest x-rays and CT scans? I guess I’m asking how possible it is that I have some issue other than sarcoidosis.

    Thanks so much for this blog. I have felt very in the dark about these things for a year and a half now.

  16. hello..!
    I too have this… ILD with diffuse systemic scleroderma,rynauds and
    its been so difficult for me to even walk 20 steps without getting breathless.
    currently i am taking mycophenolate sodium and Methylprednisolone.
    my physical activity is Zero.

  17. Hi Supriya:

    Thank you for your post.

    I am sorry to hear about your systemic sclerosis.

    Do you desaturate when you exert, and if so, do you use supplemental oxygen?

  18. This is so encouraging. I have been diagnosed with PF for several years without any worsening per say. I have wondered about yoga as well as breathing exercises. I have a video purchased while in HI that incorporate Yoga and breathing. I just remembered it this week and did the 30 minute workout and felt so much better that day. My biggest problem is mowing the yard uphill.. I get super winded however sometimes I feel like after words my breathing is better? Does this make sense. Sad thing I have been seeing a specialist since 2010 I had fungal pneumonia and it seems that that is when this condition was found and I did not find out my diagnosis till this year!! He never told me my condition! I am so freaked out! I have a reason to live with a large family and want to see if anything will effect my life expectancy!

  19. Hi Antoinette:

    Thanks for the post.

    I think the idea of yoga and yogic breathing is outstanding.

    Feeling “super winded” while mowing your yard uphill is what I would expect…first of all, you’re pushing a mower; secondly, you’re going up hill. What that means is that your “pushing” muscles and your “hill climbing” muscles are gobbling up oxygen like crazy (and asking for more) as you continue the activity. The hill climbing muscles are some of the biggest in the body; that means they use and require the most oxygen of the skeletal muscles in the body.

    One big question is what is your oxygen saturation when you are mowing uphill? If it’s less than 90%, you should have a conversation with your doc.

    Please keep us posted with how you’re doing.

    Jeff

  20. Great Question currently I do not have a monitor for my oxygen… I guess next time I go I could inquire at the office about getting one. My level for my check ups are still great at about 90-94. Thank you for this advice… I do walk every day with my dogs.. I think it is a great motivator. Some days I feel like longer walks than others.. the weather is a huge factor and humidity levels.. I check the pollen levels too to see if this could be a contributor… again.. thankful for this positive blog.. I have been to others I had to leave they were very depressing and just don’t want to dwell on that time until I have to and hoping for that to be in the far future…

    • Hi Antoinette:

      Thank you for your response.

      You can purchase a small, finger pulse oximeter on your own…not sure where you live, but you can get them in any number of online or “real” stores.

  21. I’m not sure if this page is still monitored or not, but I was recently diagnosed with Pulmonary Fibrosis back in January of this year. I am 37 and I have always been very active, working out 3 times a week, playing basketball occasionally, and then flag football on the weekend despite having asthma since I was about 5 years old.

    I guess I can say my PF symptoms started sometime in 2012, Initially I didn’t notice significant issues with shortness of breath, but I did notice I would have to take more breaks during football, but had no problem after resting for a minute or so. Another thing I noticed is that my hands would turn white in cold, then turn purple, and red as they warmed back up (Rynaud’s). Again I thought nothing of it since I have always been somewhat susceptible to cold. I also noticed a painful burning rash on my chest that wouldn’t go away but again, I just attributed it to an allergic reaction of some sort. The rash eventually went away, but the scars are still there.

    I didn’t start noticing significant problems with my breathing until early 2013, I would be completely winded after running one route during football. Again nothing too alarming because I was still able to finish a game, however it got to a point where I finally went to a Pulmonologists. Even this doctor didn’t think it was serious but after I didn’t improve with steroids and antibiotics he finally ordered an XRay which in when he saw pulmonary infiltrates and a “ground glass appearance”. He bumped my steroids up and ordered blood tests but found nothing out of the ordinary. When the steroids didn’t improve my condition, he eventually did a bronch on me, but found nothing, and even after listening to my breathing on many occasions, he ruled out pulmonary fibrosis because he didn’t hear crackling. He sent me to an infectious disease doctor as my bronchial lavage showed a possible mycobacterium infection. So three more antibiotics, but still no improvement in my Xrays. and my in office PFTs were abnormally low.

    He finally referred me to the local teaching hospital, and after a 2 month wait I was finally able to be seen. The doctor there immediately thought I had scarring in my lungs, and when he listened to my chest he said he heard crackling so that was the diagnosis and my life felt like it was over. He said it didn’t seem like it was IPF, but instead the scarring seems like it was a result of some sort of autoimmune disorder, but I’m not testing positive for any of them..yet. The doctor says it could take a year or so to have a positive test for an autoimmune disorder, which adds to my apprehension.

    I went through the whole 3 day gauntlet of pre lung transplant tests even though I’m not on oxygen. In addition these tests I’ve also had catherization on my both my right and left heart. The constant poking, prodding, and endless tests were emotionally draining to the point where I don’t even like going there for regular follow up appointments. I have another appointment later this week with the RA doctor and I already know what’s going to happen: my blood test will be negative for the autoimmune disorders they test for.

    Anyway the purpose of this post is to say that I’ve started Pulmonary Rehabilitation about 3 weeks ago in hopes it will increase my excessive tolerance where I don’t have to completely give up what I love. I have been playing sports for over 30 years and the thought of having to give it up so soon is extremely depressing. This in addition to the possibility of me being dead or near death in two to five years certainly doesn’t help either. I have basically been depressed since January, some days are better than others emotionally, but for the most part I’m always down and only feel good when I’m sleeping. I also really slacked off from working out, always finding an excuse not to go because I really didn’t feel like doing anything other than sleep. Sometimes I feel like if I am going to die or need a lung transplant, it needs to go ahead and happen now because sitting around waiting for it to happen is almost worse than the disease itself. When I’m not working, working, out or sleeping, I use much of my spare time looking at caskets and funeral planning websites. Also some of my day consists of a few hours of blaming myself for getting this disease and going through a serious of “what ifs”. I’m still also upset that the first doctor wasted so much time while at the same time missing the diagnosis that could have possibly been caught earlier.

    It may be too early to tell if Pulmonary Rehab will help me, but I have started getting very serious in doing everything I can to help my body function better with my diseased lungs. In addition to getting back to working out 3 times a week (weights M, W, F), The other two days are used for Pulmonary Rehab, so I now exercise 5 times a week in addition to football on the weekends. I still end up very depressed after playing football, but I must say immediately after working out, and my pulmonary rehab sessions I feel better both physically and emotionally and I feel like I can play a full game of basketball or football and am eager to get back to the gym so I can workout some more. This all eventually wears off and I go back to my normal depressed mode, but I guess its progress. Up until last week, I was strictly on machine weight machines, but I finally got up enough nerve, confidence, and more importantly energy to ease back to free weights. I don’t know if my pulmonary rehab sessions have something to do with this, but I’ll take any positive I can get.

  22. Hi Chris:

    Thank you very much for your post and for having such courage to open up about your experience on this forum.

    You are not alone here.

    I know it’s little consolation, but many (too many) other people are experiencing exactly what you are…the frustration of not being able to exert as long or as hard as you once did, the fear that this disease will progress, wondering when the “other shoe will drop,” etc.

    I like the fact that you are remaining active; I think that’s a very good thing. I also like the fact that you went through transplant evaluation; hopefully, you’ll never need it, but it’s smart to be plugged in to the system just in case.

    Have you been tried on any therapy yet? If so, which one(s). If you’d rather share that information with me via another route, you are welcome to email me directly at swigrisj@njc.org.

    How is your support system? Have you told your friends/family what’s up?

    I am very, very concerned about your low mood.

    I urge you to share this with your doctor this week.

    You have done so well focusing on your physical health, let’s see what can be done for your emotional well-being…after all, it’s the biggest part of who you are.

    Wishing you well,
    Jeff

  23. Try to keep a positive attitude… you are young and that is a plus.. I find it hard just getting helpful information on the subject. I think I have had lung issues for years.. I was diagnosed in 2011 and honestly it has not worsened. I have some bad days but not disabling Thank God! Keep your chin up this site is more positive…

    • Thanks Antoinette, I didn’t realize you responded as well until I sent my reply to Jeff. It is good to hear your condition hasn’t progressed since 2011. Did your doctor give you a clue as what could have caused it for you?

      You are right, its hard getting helpful information on this disease. This site is one of the few that actually is helpful and provides hope. All the other sites as you may know are very negative like, “poor prognosis”, “terminal”, “no cure”, or my personal favorite, “patients die off” in x number of years.

      I will do my best to stay positive and now that there is a helpful positive website in the midst of all the negative ones, I will be frequently visiting here.

  24. Hi Jeff,

    I’m on an imumnosuppressant and a low dose of steroids which seems to be standard protocol for PF patients. I’m also on Flonase to try to combat that annoying throat irritation I now have. I have voluntarily stopped taking my meds on occasion because I am very concerned about the long term side effects (cancer, osteoporosis, liver and kidney damage etc) and sometimes wonder if they are causing more harm than good. But for now I am back on them.

    My family knows, as well as my boss, a couple of co workers, and a couple of my friends. They are being supportive as they can, but without going through it themselves, I don’t think they fully understand what PF patients go through especially at a relatively young age; just the usual “snap out of it” suggestions, but it is very hard especially with everything being so sudden. If I were older I may have been able to handle it a little better, but having it occur during my active years is devastating. Almost constant throat irritation and unexpected coughing spells and no longer being able to normally do things I’ve always taken for granted like yawning, sneezing, and even talking and laughing, are a daily frustration.

    My hospital assigned me a mentor who was diagnosed with IPF at age 37 and eventually had a bilateral transplant about five years later. He is currently two years post transplant and is doing great and other than the lifetime meds and some adjustments in his life (no alcohol, requiring sunscreen to go outside, avoiding reflux causing foods), he is back to his pre lung diseased state. Even though he was recently assigned to me, and we only talked once so far, he has been very helpful in easing my concerns about a transplant if I end up needing it. Like me he was very active and based on what he told me I think he was probably diagnosed at an earlier stage than I was, so he was able to handle it better at first until he started to decline rapidly to the point he needed oxygen 24/7 and was forbidden from flying. The decline in health is what scares me a lot and like I mentioned if I ended up needing a transplant, I would prefer it to be now it were now so I could get it over with and move on and get it over with. I understand transplants are done based on need as they should be, but the thought of slowly suffocating before I’m even put on the list is terrifying. I considered declining a transplant if I needed one but after talking with my mentor it seems worth it to try to get a 2nd chance at life. Right now its like everything is in limbo until there is some sort of resolution whether it ends up being a lung transplant or I flat don’t make it; of course now I would choose a transplant as my number one choice, but the second choice of not making it doesn’t seem like a bad number two option, both are an end to this disease which means an end to the waiting, the constant frustration, “what ifs”, and depression.

    Thanks so much for responding Jeff. I am glad I found this site; I actually found it when searching Pulmonary rehabilitation and the information here does give me some sort of hope in the midst my depression.

    • Thanks Chris.

      It has been my experience (and seems only natural to me) that my younger patients with PF are more likely to be depressed and angry with this disease.

      I don’t think “snap out of it” is the right advice.

      To the extent possible, try to maintain focus on the positives: what are the things you are still able to do despite having this disease? Don’t linger on the things that you aren’t able to do.

      Lean on your doctor (with whom you should discuss your low mood), your mentor, your friends, your family and the people on the P3F forum for support.

  25. Jeff,
    I know it is not IPF thank God but it is an allergen that is causing the scar tissue. We have done several cat scans and that seem to read the same. I am not sure really… in my younger years I did cleaning for households and think this really could have been a key trigger. I had fungal pneumonia in 2011 this is how it was discovered. I am so thankful I still have energy to get most things done. I take frequent naps I do seem to tire easier. I have noticed that when I am on the coast I breathe fantastic. I think it is because there is no vegetation and the salt air is healing. I have considered moving there but it is so hard to transition at my age…. just some food for thought!!

    • Hi Antoinette,
      Which coast Antoinette, where exactly? Thanks.
      Bonnie

  26. Thanks for sharing Antoinette.

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