"Madame, bear in mind That princes govern all things--save the wind." -Victor Hugo, The Infanta's Rose

Thursday, January 04, 2007

Not great news

I've held off writing about this for the last couple of weeks, mainly because I wasn't sure exactly what I felt or how to express it. It's also some fairly serious stuff, and I didn't feel like throwing a wet blanket on anyone's holiday spirit. But now that we're into the new year, it's time for me to face the results of my doctor visit just before Christmas, and the first step in dealing with it is to get it out in the open.

In a nutshell, my lung functions have gotten worse since I was last tested in June, and my doctor is concerned that I could be on the verge of a decline that will necessitate my transplant surgery sooner rather than later. To understand this a little better, take a look at this chart which plots lung capacity (technically referred to as "FVC" or "forced vital capacity", measured by a standard pulmonary function test) over time:

First of all, note the dotted diagonal line. This is the "typical" rate at which a patient diagnosed with Idiopathic Pulmonary Fibrosis might be expected to lose lung capacity. An often-quoted statistic is that someone with IPF will, on average, die within two to five years following diagnosis if they don't receive a lung transplant. Of course, in reality no one tracks this line exactly. Some people may last for 8-10 years, others much less. Much depends on age and other contributing health factors. Actual results over time will bounce around and hardly ever be a nice, straight line as shown on the graph. Still, this can be considered an average for statistical purposes.

My actual, measured results are the solid line, beginning when I first started noticing shortness of breath in mid-2001 and had my first PFT. At that time they could find nothing wrong; my capacity of 78% was considered "within normal limits" and it was suggested that I lose weight and exercise to improve my physical condition. A year later, when I continued to be breathless despite doing this, I was tested again and found to have a 55% capacity. More testing and a lung biopsy followed, and I was diagnosed with IPF at the end of 2002.

Things get somewhat interesting after this. After being prescribed Prednisone (a corticosteroid), my rate of decline slowed but still continued a steady drop. Based on these tests, my remaining life expectancy was estimated at less than two years and I began the lengthy qualification process for a lung transplant at Houston's Methodist Hospital. However, at around the same time I was also approved to participate in a clinical trial, and shortly afterwards began taking an experimental medication called Pirfenidone. Note how this is at the point where my numbers bottom out, and following this begin to show improvement. In the last year and a half since, this improvement has been nothing short of dramatic, and when I was tested last June just prior to leaving on the big 2006 Toasted Tour Road Trip, my PFT numbers were higher than they had been since 2002. This is virtually unheard of in IPF patients. Test results can fluctuate, bouncing up and down by small amounts depending on a host of variable factors, but a sustained upward trend like I had been showing since starting the new med has been cause for celebration and optimism in 2006. I began to believe that maybe, just maybe, the drug was working and that I might not need the lung transplant after all. Could I dare to hope that I would be the first patient in history to be "cured" of IPF?

However, my doctor is one of the best transplant pulmonologists in the nation; as a result of having seen hundreds of IPF patients over the years, he knows the clinical history of this disease all too well and is naturally skeptical of any miracles. While reasoned optimism is not a bad thing, he has wisely cautioned me not to set my hopes too high, lest I fall into depression from "burst bubble syndrome" if my rally doesn't last. And sure enough, you will note that this last series of tests has put me on the other side of the bump. The dashed blue "trend" line is a mathematical weighted-average of my PFT scores which shows, overall, that I am still losing ground over time -- although at not nearly as steep a rate as our hypothetical "typical" patient.

What mainly concerns my doctor right now is the possibility that I could be on the verge of dropping into a sudden decline. He has seen IPF patients go from relatively well-off to death in a matter of a few months. This is frequently the result of something like the flu, which can quickly turn into pneumonia in someone with a compromised respiratory system, but it can also happen without precipitation; that's just the nature of this fucking disease. As I said at the outset, the numbers don't necessarily follow that predictable linear dotted line -- all that's known for sure is that they'll wind up at the bottom of the chart eventually.

The most important thing we need to ask now is where the next data point on this chart will be, so I'm supposed to go back in another six weeks to be tested again. This most recent decline could be an anomaly; I could remain stable, or even continue to improve, in which case we'll continue our wait-and-see approach. However, if I appear to be continuing down the slope, I've been advised to prepare myself mentally and emotionally for the transplant surgery to take place within a relatively short period of time, perhaps weeks. This has been a bit hard for me to grasp, as I'm still encouraged by my apparent favorable response to the pirfenidone, and not feeling all that bad physically as long as I don't exert too much and get myself out of breath. However, I do understand that the goal of transplantation is to find the perfect window of opportunity where the person is, obviously, sick enough to need it to survive long-term, yet healthy enough to withstand the extreme stress of the surgery and recovery. They want me to have every possible advantage going in to this, and my chances for survival will be better if they err on the healthier side of that window as opposed to waiting until I'm in poor shape.

So that's where we stand -- waiting for the next dot on the chart. I'm trying to feel upbeat about this, and intend to work at getting as healthy as possible in the weeks ahead. I'm going to go on a diet and try to lose even more weight, and step up my pulmonary rehabilitation program to develop greater exercise tolerance. I won't give up fighting!!

Sorry for this depressing post, but I want to blog about everything that goes on in my world, and this is certainly a large part of it. Thanks for reading, and for your support, y'all. It means a lot to me.



  • At 1/04/2007 07:02:00 PM, Blogger Baketown said…

    Hang in there! You can do it.

  • At 1/04/2007 09:01:00 PM, Blogger Sphincter said…

    How scary. Mr. Toast, we are pulling for you! I hope that you and Mrs. Toast are getting the support you need from family, friends and community. Thank you for sharing your thoughts and experiences with all of us. It's very generous of you, considering all that you have on your plate right now.

  • At 1/04/2007 09:47:00 PM, Anonymous Anonymous said…

    Toaster, thanks for sharing this info. It helps a lot when I see this to understand what my own Papa is going through. He is starting some experimental Japanese crap that is supposed to last for 60 weeks which we are very hopeful for. As it is now, not only can he not hop on a plane, he can't even be in Denver at altitude. Agh, what a stupid disease.

    I'm wishing and hoping and sending all of my thoughts for a better chart in six weeks.

  • At 1/04/2007 11:26:00 PM, Anonymous Anonymous said…

    All I can say right now is, WOW. I really hope that you see improvements. I definately do not want anything terrible to happen to you. You and Mrs. Toast are in my thoughts and prayers. On Sundays, after mass, I will light an extra candle for your family. Hang in there, Toast.

  • At 1/05/2007 03:54:00 PM, Anonymous Anonymous said…

    thanks,mr. toast. my son was recently diagnosed at the age of 19 and at this point in time they are saying he has only 50% lung capacity. I don't really understand all of this, because to look at him or to see him out , you would never know anything was wrong. He is a patient at Duke Medical Hospital in NC where they say this is the best treatment around for him. But, they are currently saying that they will watch him (he is on NO medication for this at all) as he progresses. They say he is doing fine as is. But what exactly does 50% mean? Please help!!

  • At 1/05/2007 04:53:00 PM, Blogger Mr. Toast said…

    Thanks for the encouragement, you guys -- I appreciate it very much. Anonymous, I have your email address and will write you privately about your son.

  • At 1/06/2007 10:21:00 AM, Anonymous Anonymous said…

    Like ronnagail says, "hang in there!" Stay on top of it and keep fighting. Meantime, we are all with you looking for the next dot in the chart to be up.

  • At 1/08/2007 02:07:00 PM, Blogger Chandira said…

    Ah! Mr Toast.. Wow. A temporary anomaly, on your way up again. Let's hold that thought.

    Thinking of you!!!

  • At 1/08/2007 03:25:00 PM, Anonymous Anonymous said…

    i love your attitude. despite having your very serious health issues you still are out here helping others. i truly do admire and respect you. you are gonna make it as you are a fighter. i believe this to be true.

  • At 1/10/2007 10:44:00 AM, Blogger Mr. Toast said…

    I started to write "you guys are the best virtual friends in the world" and then realized there's nothing "virtual" about it. Just because I've never met you face-to-face doesn't make you any less "real" ... in fact y'all are better friends then some of the people I have met in person. Anyone facing a serious illness or other crisis will tell you that their support network is one of THE MOST important resources they have for dealing with it.

    So let me just simply say, "thanks". I love you guys. [snif]

  • At 1/10/2007 01:30:00 PM, Blogger April said…

    Dido on all of the above. We will all be here to support you through whatever you have to do. You're a brave guy, Stay positive, you're aware of how mood can affect health, and you've prooven that time and time again. WE LOVE YOU TOO MR. TOAST!

  • At 1/12/2007 03:29:00 PM, Blogger bossann said…

    It has (obviously) been a while since last I visited your blog. I found myself breaking a company rule to come here today (whilst at work!) since you were so much on my mind. I begin to understand your silence lately...and I firmly believe that this latest number is indeed an anomaly…this will be proven in a matter of weeks. You are in my thoughts and prayers daily…even when I don’t visit here as often as I should (or would like).


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