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.

  
17 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?

  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.

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