Sorry for the huge gap in between posts. With my trip oversees, the Christmas holidays and a couple stints in the pokey… well, you know how it is. Lets just say that things have been a little crazy, not all bad, just crazy.

Ive been out of the hospital for about month now and like so many other times during these past couple of years after a bad exacerbation the “T” word has sprung up again. We actually began this conversation 2 years ago during my initial SARP visit. What makes this time a little different though, is that Ive actually agreed now to take the next step and have the assessment done. NO, this doesn’t mean that Ive changed my mind about not wanting a transplant, I still believe that I would just be exchanging one set of problems for another. But at the same time I don’t want to burn ALL of my bridges.

The evaluation will happen at either Stanford or the University of Pittsburgh later this spring. While I would prefer to have it done in Pittsburgh (So Dr Wenzel can follow me) it’s not really practical, because if I ever got listed for transplant in that region, Id have to relocate there. Standford makes much more sense since I only live about 60 miles away. At this stage of the game however, it really makes no difference where the evaluation takes place, as I’m too healthy to be considered for transplant at the present time, and even I was deemed sick enough I wouldn’t want it done just yet.

The evaluation itself consists of lots of diagnostic tests and meetings with various specialists, including surgeons, pulmonologists, cardiologists, psychiatrist, social workers and of course financial people. (Lung transplants are incredibly expensive, in excess of a million dollars, and that’s not including the follow up care that you need for the rest of your life.)

So, if I don’t want a transplant, why am I being evaluated you ask? Well, basically because there’s nothing else that can be done for me and my Pulmonologists think that I have nothing to loose and everything to gain by at least being evaluated. As is the case for many people with advanced Cystic Fibrosis, being evaluated for transplant at an early stage is a good way to get a general idea of where you stand as far as severity of your disease and what the likelihood is that you will be eligible for listing at some point. With an FEV1 % predicted still in 30′s, I’m not as sick as most who go in for evaluation, but then again I don’t have CF or the other more common transplantable diseases, so the criteria is a little different. Which brings us to the next issue, only a handful of people have ever received lung transplants because of severe asthma. I think the reason for this, is that asthma is not considered an ultimately fatal disease, as are CF, COPD, Pulm Fibrosis, etc. While 4500 Americans still die of this disease every year, asthma for the most part is considered a controllable disease. Only a very small fraction of asthmatics actually progress to the point where their lungs are destroyed by the disease like mine are. Then there are questions as to whether asthma can reoccur in the new lungs. Ultimately, the determining factor for in deciding who is eligible for lung transplant, is prognosis and quality of life. Most centers will only list you if your expected survival is less than 2 years or if your quality of life is poor. The reason the bar is set so high, is that median survival time for lung transplant recipients in only 3.5 years. They want to make sure that transplant will actually extend your life. Finally for me, there’s the age issue,Im 57 years old and rapidly approaching the cut-off age so I can’t put this off for too long.

Bottom line, I plan to live with the lungs I was born with for as long as I possibly can, which will hopefully be until I die a natural death. I would only consider lung transplant if (or when) the quality of life becomes totally unbearable. I see these upcoming evaluations simply as a way to measure and monitor which direction I’m heading in, so that I can be better prepared for whatever the future holds.

I normally don’t post excerpts or links to other peoples posts, but this one I couldn’t resist. Like the many others who’ve reposted it, I just love it.

Piper, a recent double lung transplant recipient who had Cystic Fibrosis, lists her recommendations for that perfect holiday gift. I especially love the gift of prednisone.
Her thought provoking humor on this topic is the bitter sweet reality for a lot of lung transplant recipients. It made me think a lot about my own predicament and just how lucky I have it right now. I was going to post it last week when I first read it, but had an unexpected date with a hospital bed instead.

Every year the holidays roll around and hundreds of CF/post-transplant spouses and significant others are left struggling with what gift to buy their sensual, scarred sweetie. Tragically, many of these clueless Cassanovas will miss the mark entirely, presenting their beloved with a gift basket of grapefruit or a membership to the Raw Meat of the Month Club. But don’t be one of them! Let us help you stuff her stocking and trim her tree with helpful and exciting drugs from all walks of post-transplant life. Trust us, she (and her lungs) will thank you

read the entire post here

His human name is Steve Ferkau, and his story is truly amazing.

Being sick for long periods like this, often has me thinking about lung transplant surgery and how wonderful it would be, at least in my mind, to not have to struggle to breath all the time. This month also marks the one year anniversary of my initial SARP work up, and the infamous meeting of the minds over at UCSF. At that time we had all decided that my disease wasn’t severe enough to be considered for transplant, and even if I did meet the criteria, I had pretty much made up my mind at that time, that transplant surgery was something that I wasn’t interested in. Well that was then, this is now.

Here I am a year later, getting more breathless everyday and starting to seriously re-think my earlier decision. But wait… even if I were to change my mind and actively pursue lung transplant surgery as a treatment option, my current baseline FEV1 of 35% is still too high to qualify. And if I’m suffering this much in the mid 30′s, I can’t even imagine how bad it must feel to be even lower.

The fact is, most people on the transplant waiting list have FEV1′s in the 20′s and sometimes in the teens. The majority are also on supplemental oxygen 24/7, and some of the sickest people are even on ventilators while they wait for donor lungs. I only require oxygen when I’m actively flaring and my FEV1 rarely drops into the 20′s unless I’m really really sick and Ive always managed to bounce back. [This whole oxygen criteria thing shouldn't even apply to an asthmatic waiting for transplant. The pathology of asthma is much different that Cystic Fibrosis or COPD. Asthma is a disease of the airways, not the alveoli. Most asthmatics don't require supplemental O2 unless they're actively flaring].

I suppose I should thank my lucky stars that my FEV1 readings are still in the mid 30′s, and occasionally even into the 40′s, but as good as I have it in comparison to those waiting for new lungs, life in the the 30′s is not exactly a picnic. And the fact that my lung function has dropped almost 30% in 6 years, tells me that my numbers will only continue on that downward path. It’s not a matter of if they will drop into the 20′s, but when .

Living in the 30 percent range is like living in a kind of breathless purgatory. Your days are filled with this low-level dyspnea that doesn’t get too severe unless you’re flaring, but is always there in the background effecting everything you do and making you miserable. In the 30′s percent range your lung disease becomes so advanced that most conventional therapies or medications no longer work, yet you’re not quite sick enough to qualify for transplant. Now, if I had emphysema and had lots of air-trapping, I would be a perfect candidate for LVRS or airway stents. But, I don’t have emphysema, nor do I have the type of hyperinflation or air-trapping that could be helped with airway stents. I have severe asthma with fixed obstruction and there’s very little that science can do for people like me.

There’s another criteria for lung transplant surgery that creeps me out a little and even makes my doctor a little uneasy, and that is, the longevity issue. How long can I be expected to live if I do nothing? Ive had doctors give me predictions of as much 5 years all the way down to one year. In order to be a transplant candidate you have to have a life expectancy of 2 years or less. Most transplant centers base that prediction on a BODE score of 5 or more.Currently, my BODE score is a 4.

I know I should be grateful for all Ive achieved through fitness and pure stubbornness, but sometimes I feel like I’m being penalized for doing the right thing and staying in shape. Had I not done all these healthy things, my FEV1 would have surely declined faster, putting me in a position where Id be eligible for transplant by now. Let’s face it, not too many people with an FEV1 of 35% have completed 7 marathons. What’s really ironic though, is that transplant candidates are scored on their level of physical fitness. Transplant centers want people who will be healthy enough to survive the wait time, ( up to 2 years in some cases), the surgery and the recovery. The stronger you are going in, the better the results. How that’s possible with an FEV1 in the 20′s or teens is be beyond me, but that’s the way it is. I have the required physical and mental stamina now, but will I still have them when my other numbers finally catch up. And even if I do meet all the criteria, will it happen in time? Most institutions set the maximum age for lung transplants at 60, I’m 56.

So as I’m getting ready to finish the closing paragraph of this post, the “But” part of the title hits me in the head like a ton of bricks. Life sucks with an FEV1′s in the 30′s, but I’m not to the point yet where I dread waking up everyday. I’m not to the point where everything I do is a total struggle. I still have the occasional “good breathing day” (or “not so bad breathing day”, whichever way you want to look at it). I’m not ready yet to trade my lungs in for a pair that may, or may not give me more of those precious “good breathing days”. But most importantly, and regardless of all the other criteria, I know I’m not ready for a lung transplant, because I’m still questioning whether I can live without one. I’ll know Ive hit my rock bottom when I’m no longer able to walk and life is no longer worth living…it’s as simple as that! I can see now why they set the transplant bar so high. It’s meant as a last resort, not a treatment option. So on that note, I’ll stop crying poor me and get on with life.

And as far as predicting how long I’ll be able to continuing walking, who knows, but it sure would be cool to rack up 3 Bostons in a row, before throwing in the towel.

Before I begin, let me preface this post by saying that I haven’t made up my mind as to whether I would even want lung transplant surgery, should it ever get to that point. I wanted however, to see what some of my Pulmonologists views were on this subject, and to get a feel for what kind of support I would have in whatever treatment option I pursued.

So last week I got together with 3 of my UCSF Pulmonologists to discuss my SARP evaluation and Dr Wenzel’s recommendation that I consider lung transplant surgery in the future. I began the conversation by bringing up all the wonderful things that the SARP study was accomplishing, which seemed to put a smile on a everyone’s face. But, the minute I mentioned the “T” word, you could sense the tension in the air. You might even say, that tempers were starting to flare…including mine.

No Way! … are you even close to being a candidate for lung transplant surgery, was their general response. After all, you can walk marathons! ( yeah…like I didn’t see that one coming )
Even if my FEV1s were to totally bottom out and I was at the point where I was needing supplemental oxygen 24 hours a day, if I were still able to walk ….even just a 10K, I would not be eligible for new lungs. It just wouldn’t look right.

They went on to reassure me however, that even though I have extremely severe asthma, that my PFTs have been relatively stable over the past 4 years and that there’s no scientific evidence to suggest that people with severe disease plateau at a certain level and then suddenly experience a rapid deterioration . (I’m not an MD or a scientist, but I don’t totally agree with that last statement.)

Beyond whether or not I would meet the actual criteria for acceptance into a transplant program, I think their main concern, was that I somehow didn’t understand all the implications of having this life-altering , can’t- undo-it , type of procedure done . In their view , I would just be exchanging one set of problems for another. Though they agree that I would probably be breathing a lot easier after transplantation, there’s also a good chance that my life would be cut short. This is why they prefer you to be on deaths door before considering such radical treatment. Tissue rejection is still a huge problem in lung transplant recipients, as they haven’t quite figured a way to administer just the right amount of medication to prevent rejection, without killing the patient in the process.

They claim, that while there are a few miracle stories out there of people thriving and living long lives after receiving new lungs, the fact is, the vast majority of lung transplant recipients are not so lucky. The survival rate is still very dismal and pretty much a crap shoot. Only 2 out of 5 people will survive 5 years after transplant, and many of the people who do survive, are plagued with frequent life threatening infections due to weakened immune systems. You have to be on potent anti-rejection drugs for the rest of your life and will need constant medical monitoring. I’m not sure I like the idea of having to be around hospitals, anymore often than I am now.

OK, so I see their point about this being an option of last resort, and I appreciate all the concern . I don’t agree with all of it, but I understand it. I think the real reason I got the response I got, is because I have asthma as a diagnosis. There have only been a few asthmatics that have ever been transplanted. The fact that I don’t require supplemental oxygen ( yet) is a huge factor too. In every other way, my lungs are trashed, but Im able to oxygenate.

I guess I should be relieved that I’m not considered sick enough to warrant transplant surgery, but at the same time, when one of the most respected asthma doctors in the world, tells you that you should seriously consider it, I think it’s worth learning as much as you can about it , so that you’re better prepared if the time should come. And while my current feeling is that I wouldn’t want the surgery done, who knows how I’ll feel about it a year from now, or if I get to the point where I’m struggling for every breath.

That bit about “being able to walk marathons” really got me to thinking about just how misunderstood I am and how misinformed people can be. It felt as those they were using my incredible physical fitness achievements, against me. For some reason there’s this crazy notion, even among lung doctors, that people with severe lung disease are supposed to fit a certain mold.
My response to that would be… why would I even want to.

They agreed that we should revisit this issue again in 6 months. Maybe things will be different then.