Wednesday, March 30, 2016

"I would appreciate more information on outcomes" in PF

Today, I received the following comment on my blog:

"While I appreciate your mantra, "No one is average. Demographics are not destiny. Doctors are not fortune tellers," as the positive message it is, but I would appreciate more information on outcomes. […] The lack of data about disease progression is frustrating. An analysis of PFT variables (e.g., FVC, DLCO) over time of the IPF population, for example, could be enlightening and allow reasonable planning for what's ahead. It would permit a person to see where one might fall on an outcome curve (because everyone is not average, but some people are above and some people are below, and we should have a right to understand that if we want)."
 – Anonymous

To provide some context: I have repeatedly stated in my blog (and in person to anyone who will listen) that "average" outcomes in this disease are not very helpful for an individual person living with PF. Instead, it is helpful is understanding the range of possible outcomes. You can read more about this on my blog here.

Anyway, my immediate response to this comment was: "Right on! I want that too." It's exactly what we all want and need in this disease. I think we can all agree that having information to help us "for what's ahead" is an important goal. So, I am going to give you the data, and then you can decide if it is of value.

In September 2014, a group of researchers led by Dr. Jeff Swigris (one of the leading minds in the field) examined what happens to pulmonary function tests over time in IPF, including mapping out the changes over 10 years in forced vital capacity, or FVC, which is the primary objective measurement we use to follow progression of the disease. Here is what things looked like:

In this figure, the x-axis along the bottom is "time" measured in years and the y-axis on the left is FVC measured in a percentage of normal (where "100" is 100%, which is normal for someone without lung disease). The solid blue line is the average FVC. For now, ignore the red lines and blue dots.

At first glance, this blue line seems to be helpful – it shows you what you might expect. But the truth is that the solid blue line only shows you the change in FVC over time averaged across everyone, and the blue line does not convey the range of possible changes of FVC over time. I suppose if the range of possible changes of FVC all looked very similar to the average, then the average would be of great value in helping each person understand what to expect over time. On the other hand, if the range of possible changes of FVC varies widely, then the blue line won't mean very much to any one person. (I think you know where I'm going here…)

Here is a picture showing the change in lung function (FVC) for each person in the study:

Each thin black line represents one person's change in FVC over time. There are a few things that are important to note about this picture:

1. The range of variability shown in this picture is way big. 

2. I see (maybe) one black line that looks like the "average" blue line in the first picture. Everyone else's black line looks nothing like the "average."

3. Most lines seem to trend down when looking from left to right (meaning that most people's lung function gets worse over time), but some lines look pretty flat, and some lines even go up (meaning that some people's lung function gets better over time).

4. There is no way that I could look at you in my office and tell you which line represents your future.

My point here is that knowing the average is only helpful if everyone's outcome looks pretty close to the average. In the case of PF (including IPF and other forms of PF), the average outcome doesn't seem to be helpful.

No one is average.

Big props to Jeff Swigris and his whole team for completing and publishing this important study. You can read the entire study here.

And here's the full citation:
Matthew J. Strand, PhD, David Sprunger, MD, Gregory P. Cosgrove, MD, FCCP, Evans R. Fernandez-Perez, MD, MPH, FCCP, Stephen K. Frankel, MD, FCCP, Tristan J. Huie, MD, FCCP, Amy L. Olson, MD, MSPH, Joshua Solomon, MD, FCCP, Kevin K. Brown, MD, FCCP, and Jeffrey J. Swigris, DO. Pulmonary Function and Survival in Idiopathic vs Secondary Usual Interstitial Pneumonia. Chest. 2014 Sep; 146(3): 775–785.


  1. Thank you, your post and this study do help. Interestingly, one might discern(though it's really hard to tell based on the plot) patterns from the data (e.g., that the lines extending out to the 6th, 7th and 8th years mark have similar slopes while those extending only to the 2nd, 3rd and 4th years have different similar slopes) -- the authors do pattern some analysis with the <100/>100 analysis points. I've recently read "When Breath Becomes Air," and I was sympathetic to the author's frustration about his doctor's reticence about not wanting to discuss outcomes too soon. That being said, I also appreciate your (and my own doctor's) emphasis on a positive message in the face of a difficult disease. Thank you for your blog and all you do for the PF community.

  2. There are the "famous" 3-possible outcome paths for IPF (a figure of which David could post here or perhaps a link). After 5-years of charting my own data, I would say that I am on the slow progressing path rather than the rapid or stable. My path has had dips from exacerbations that with treatment and proactive effort from me and my medical team have taken me out of the dips so that my trend line resembles the slow progression.

    However, what I did not know 5 years ago is which path my disease progression would follow and neither did my doctors. No one handed me a card and said go stand in Line A, B, or C.

    The FVC and DlCO are only indicators or milestones -- an action or event marking a significant change or stage in development -- BUT THEY DON'T TELL THE WHOLE STORY. I view them more as points at which certain discussions or options should be explored. My FVC at the beginning of March was 42.7% of predicted. I keep my oxygen flow at home on 10 lpm so that I can climb a flight of stairs and move around and do things. I exercise at 12 lpm. I still drive. I still do all the shopping and cooking and most of the housework. I totally look after myself and while I may do things slower, less frequently, and for a shorter duration than I used do, I still pretty much can do anything I did 5 years ago. I walked 1713 feet in my 6MWT last December 2015 compared to 1742 in January 2011. What I can't do now that I could 5 years ago and do all those things without supplemental oxygen.

    I hear many patients frustrated and hurt because when they asked their doctor "am I going to die" the doctor responds "yes we're all going to die". We don't have an expiry date stamped anywhere on our bodies. At the time of our birth we were not given a death certificate with the date filled in. Why when given a terminal diagnosis does that change or our expectations are that if not the exact time at least at better estimate of how long we have left? We just have to live that "dash" between birth and death. My diagnosis has meant that I hear my heart ticking faster and louder than it was before my diagnosis. I've had to come to the realization that I'm unlikely to live into my 90s like my father but I've lived almost 10 years longer than my mother did. I realize that lung failure is most likely to be my cause of death but that isn't a 100% certainty.

    After a bad exacerbation early 2013 I was assessed for transplant. By that time I was recovering from my exacerbation and although deemed a suitable candidate I was considered too earlier. Since then my respirologist and I keep having the discussion. I've jokingly said that going on the transplant list would interfere with my qualify of life or I read on the internet that I have 5 years to live and my 5 years isn't up yet.

    1. I have travelled 2 paths over the last 5 years -- managing my IPF and my end of life. As I managed my IPF, decisions took into consideration how it might affect my other path, in particular transplant. My journey along the transplant path helped to support and often solidify, with knowledge, what I needed to do to manage my IPF. I knew these paths were not truly parallel, that they were converging -- and that convergence is very close for me now.

      What I realized I have been doing if treating my IPF as a chronic disease, one that will and did progress continuing to challenge me to either fight back and work on not giving up on what I could do and my quality of life while adjusting to the new me when I had to.

      It is LIFE that I consider terminal and my transplant path is my plan of action for how I might be able to push death out farther into my future -- to foil the plans of the Grim Reaper but I know that even if I should be so lucky to be gifted with a 2nd chance due to the generosity of another, there is still no guarantee.

      The average IPF patient dies within 3-5 years after diagnosis. As I've learned from my medical mentors such as David Lederer and Jeff Swigris and from the PF/IPF Community is that means I am dealing with a serious disease, that I am acutely aware if I wasn't before that life is not infinite for me, but I've also learned there is also hope and lots that I can do with my medical team and for myself and I just have to keep on living until I'm not.

    2. Barbara, thank you for sharing this with all of us and for painting the 'living' side of prognosis. I hope many people find comfort in your words.

  3. What disappoints me is the lack of crosstabs on this "statistical analysis" What is the correlation between age and loss of lung function due to IPF? What different prognosis difference is there between someone diagnosed in their 40s, 50s 60s or 70s? What effect do medications such as esbriet and ofev have on loss of lung function at the different ages of diagnosis? What about the effects of diet and exercise outside of anecdotes? There are billions spend every year on lung disease research. over 40,000 people in the US die from IPF every year. It's extremely disappointing that those in the lung associations have done so little publication on the relation of these factors to IPF. what's the problem in the pulmonology field here?

    1. I feel your frustration. Some of these data are available in research publications (particularly about differences in prognosis by age ), but the data is from the 1990s. I can tell you that the Pulmonary Fibrosis Foundation has created a Patient Registry at 40 centers around the U.S. to answer exactly the questions you raise. The Registry launched just a few weeks ago. You can learn more at

  4. I was diagnosed with lung cancer 24 months ago. Through chemotherapy I have been cancer free for 18 months. However, I went through 37 radiation treatments finishing in October, 2014. I'm told that due to all the radiation, plus previous illnesses, I have been diagnosed with IPF. In January of this year, I started O2 therapy, as well as Pulmonary Rehab, and Prednisone. I started with -2- litres of O2 and 30mg of prednisone. As of today, April 19, I am up to -8- litres and 30mg. My latest CTSCAN, from 04-07-16, showed more fibrosis than the previous one, which, unfortunately for me is normal. It has obviously gotten worse rapidly for me. I am not eligible for a transplant, for a variety of reasons. I know this disease will eventually kill me. And I know no Doctor wants to, or is able to, "play God". But, I just wish someone would be able to look me in the eye and tell me "get your stuff together. It won't be long". Is that asking for too much?

    1. Thank you for sharing your story. As you expressed, PF is a serious and progressive disease. Each person with PF experiences their own pattern of progression over time -- Heather's story below about her father illustrates one person's story of living with PF for over 10 years. Of course, other people with PF have a rapid rate of progression, and others fall somewhere in between. Since I am not your doctor, I can't give you a prognosis. But, as my patients would tell you, I don't give anyone a prognosis. I just share a range of possible outcomes, and I share my concerns about how likely a "worst case scenario" might be. I think that is an honest approach.

  5. Hi Anonymous, I am so sorry you've been through so much. To deal with lung cancer and IPF - just is unimaginable. Stay strong.

    My Dad has IPF - he will be 81 years old on May 5th. My Mom tells me he was diagnosed with the disease in 2003, but they didn't tell us kids until 2012. So actually, I'm a bit uncertain of the facts, but in any case it was a huge shock.
    My Dad delayed O2 treatment, because he wanted to live life as normal as possible. He's a very strong man, he was still working right up to December 2015! It gave him purpose, something to do.
    But it became clear mid-December that he would need to resign from work and start oxygen - in January he went from 2 to 6 liters. In February he caught pneumonia, and Feb 26 got admitted to ICU.
    In ICU his respirology team decided to do a 3-day agressive treatment with Prednisone and antibiotics for the pneumonia. He was in ICU for 2 weeks, and they moved him to a rehabilitation floor for another 2 weeks. The doctors told us he has weeks left, to prepare everything (funeral, anything else). In the 2 weeks time, my Dad forced himself to eat to gain strength. And day by day, he got stronger!
    On March 25th (Good Friday this year) we took him home, and set up all the home palliative care he would need. Roughly 4 weeks on, he is holding on, he is trying to be as independent as he was.
    It is baffling for his medical team.

    His respirologist tells us that what is happening on the inside, he is not showing it on the outside and any day now it could flip. Any day now. I have no idea what this means ... Obviously, I understand what his doctor is saying, but it is very hard to not know. To wake up every day with anxiety, over what could possibly happen today.

    It is very hard. As I said, I can't even imagine what you must be going through. Sending much positivity and wishes for you.

    1. Hi Heather. Thank you for sharing your father's experience. I hope many find it helpful.

  6. Heather, your story reads like an exact synopsis of my husband's, although he's 20 years younger than your dad. He's had IPF for 16 years now and still presents a strong front. He only wears his oxygen when he needs to use it in uncompromising situations. He, too, has baffled his medical team. We, too, never know the next situation or when he may be told he needs a transplant. Our mantra: Live life fully everyday; time waits for no one.

    1. Hi Pat. Thanks for sharing this. I like your mantra very much. I may steal it to share with my patients.

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