If there’s any good news in all this, it’s that there’s a new drug in the pipeline that could possibly help people like me who have this cellular abnormality.

Dr Wenzel to me;

( 2-24-2010)

Steve, At long last, some MICROSCOPIC pictures of your airway! These are “orange colored STAIN” to identify cells called mast cells. The first AE2typrt identifies mast cells which make tryptase (an enzyme…. we don’t really know what it does, but it identifies this type of mast cell) the 2nd AE2chym is a different enzyme (chymase) also made by mast cells, but a different “type” of mast cells. As you can VISUALLY tell from the 1st slide, you have “orange” everywhere. LOTS and LOTS of mast cells (even though almost all of the OTHER cells we associate with asthma (eosinophils, lymphocytes) are gone) And, interestingly, about 50% or more of these mast cells make the 2nd enzyme chymase, (2nd slide) which we ONLY see in severe asthma. WE think understanding what causes this increase in these cells is REALLY critical to understanding severe asthma. We are WORKING On that… but you clearly fit the picture that we have seen in other severe asthmatics, maybe even to a greater degree than many.

Finally, you ALSO have an epithelial layer (the top of the slide with the oval-like holes in it) and some cilia on top) which is MOSTLY mucus producing cells. LOTS AND LOTS of them too. We think the mast cells and the mucus cells are somehow related and we are working on that. And, there MAY be some drugs that block PGD2 (a prostaglandin molecule also made by mast cells) which MIGHT help your asthma… but probably wont be able to try those for another year or 2 as part of a study, and if they work, 5+ years as a marketed drug. But, there MIGHT be hope!

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I’m not totally out of the woods yet and I don’t want to jinx myself, but for the first time in 2 weeks, I actually feel like a human being again. I can breath!
To celebrate, I went ahead and got the first of those 3 goodies that I promised myself…..namely, a haircut! (Yikes…I look a little too str8 now). The new shoes and the Manilow Paris Las Vegas show tickets are in the mail.

You know with all the suffering that’s been going on in the world lately, I feel extremely fortunate in so many ways. I may have really bad asthma, but I also have access to some of the best medical care on the planet. I’m surrounded by caring people, and blessed with the support of others who know what’s it like to live with a chronic disease. I would say I’m pretty lucky and have very little to complain about.

And in case you haven’t heard, SARP is now on Facebook. And thanks to Kerri’s efforts, there’s also a Fan page.

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.

Well, not only did she take the time to answer all of my questions, but the answers she gave ….made total sense.
I’m posting some of that discussion here, because I think it might help others out there who are in a similar situation. At the very least, it makes a great refresher course for the RT’s out there.

[Steve ] Because my larger airways are so scarred and stiff, does that mean that they ‘re incapable of clamping up or spasming or narrowing? [Dr. Wenzel]  No, not completely, but certainly will be harder to spasm.

[Steve] If that’s the case, and my larger airways are not clamping down, when I have an exacerbation, is it the smaller airways that are reacting? [Dr.Wenzel] Yes, they most certainly are likely to be and because it is also likely that they are narrower to begin with, just a little spasm COULD effect it a lot.

[Steve] I thought there was no smooth muscle in the smaller airways? [Dr.Wenzel] There IS smooth muscle in your small airways. But, you probably ALSO have “Scarring”/fibrosis in those airways which probably leaves them normally much narrower than normal small airways. Just a little mucus or spasm and they will close.

[Steve] You mentioned earlier that I might not actually have chronic inflammation of my airways.  If there’s no inflammatory process going on, or no smooth muscle spasm going on, what causes the obstruction or air flow limitation when I flare-up? [Dr.Wenzel]  See answers above. In addition, you likely have “loss of alveolar-airway attachments”. The alveolar septae attach to the outside of the small airways and actually help to “tether” the airway open. When those get destroyed, as we think they do in SEVERE asthma, that tendency to PULL the airway open from the outside is lost.

If my airways are non twitchy or non-reactive because they are so stiff,  how is it that I can have severe asthma exacerbations that land me in the hospital and sometimes even on a ventilator? [Dr. Wenzel] Your lungs (I think) are very stiff and when you have an attack your work of breathing becomes VERY BIG. That is why your CO2 increases. In addition, you did have a couple of attacks where your lactate levels did increase… that also goes along with your breathing VERY HARD and causing your muscles to start breaking down.

[Steve] If my airways are so damaged, why do I respond, and even partially reverse, with certain bronchodilators and steroids.[Dr. Wenzel] Although you likely don’t have much inflammation, the little that is there (plus some edema) reverses with the steroids. The bronchodilators likely just relax your smooth muscle enough to have an effect, albeit not a big one!

[Steve] Do I have any elements of COPD or Emphysema? [Dr.Wenzel] NO you don’t have COPD OR EMPHYSEMA!!!!

[Steve]Isn’t that generally the course that asthma takes over the long term? [Sally] NO!!! ASTHMA ALMOST NEVER BECOMES EMPHYSEMA…unless you smoke!

[Steve] Regarding my FEV1s, I think the reason I got a 50% reading that one time, was because I exhaled less forcefully during that particular manover ( I was still sleepy from the bronchoscopy). For some reason, when I blast out as hard and as fast as I can, I get slightly lower numbers. ( I think because my airways are narrowing too fast?) [Sally] YES, that is most certainly the case. There is a FORCED vital capacity and a SLOW vital capacity (meaning just that, that you exhale SLOWLY from Total lung capacity to residual volume) It IS likely that your SMALL AIRWAYS do collapse when you exhale fast due to the external force on the airway being greater than the force holding them open during expiration (when you have negative pressures in the airways themselves) .

And finally, there was this….
[Steve] BTW…..The wager we had regarding me being able to ever blow a 50% FEV1 as a result of using Qvar , was that you would get a new set of golf clubs. [Sally]  hee-heee… I expect only the finest clubs when I get you there!

(I just noticed what it says on the pillow)

First , the good news…..

I finally got to meet Dr. Sally Wenzel (up close and personal you might say, considering she analyzed my DNA and probed around inside my lungs). It’s difficult for me to find just the right adjectives to describe how I feel about her, so I’ll just say … I love this women! She’s like my pulmonary physician soul mate. I admire her, not only because she’s an awesome Pulmonologist and research scientist, but mainly because of her fiery personality and the passion she has in trying to understand asthmatics and what makes us tick. And get this….she’s a good listener too! She actually cares enough about what you have to say, that she takes the time to listen. Imagine that.

I think Sally (Dr Wenzel) probably knows more about asthma pathology and the personalities of asthmatics in general, than any non-asthmatic person Ive ever met. It’s absolutely uncanny the things she knows about our disease and how we behave and live. She understands things about asthmatics that they don’t teach you in medical school. Things that you would only know by interviewing thousands of us and immersing yourself in the severe asthma culture. I could spend literally days just talking with her about this subject. We seem to teach each other. I am so lucky that I was given the opportunity to hook up with her. I think we will be friends for a long time.

The accolades don’t stop with Dr Wenzel, there were dozens of other wonderful people involved in this study as well. Here are just a few that Id like to mention by name and position.

Erin———–SARP research coordinator extraordinaire.
Cathrine RN—-Dr Wenzel’s assistant and other right hand
Mat tech——Very cool PFT tech.
Celo tech—–Just one of many super cool people on the bronch team
Trish RN——-A special Nurse. She actually called in on her day off to see how I was doing.
Lindsey RN—–Probably the sweetest Nurse Ive had the pleasure of meeting
Chuck RN——They don’t get better than this “young man”.
Rose RN——-Kind, Kind, Kind!
Michelle RN—-A Super Nurse.

To the above people , Id just like to say Thank You!
In all the years that Ive been in and out of various hospitals and clinics, Ive never come a cross a better team of compassionate and caring medical professionals like those associated with the SARP study, especially the people who took care of me in the Translational Research Care Unit at UMPC after my bronchoscopy. Kudo’s to you guys!

As for the SARP study itself, well, it’s probably the best coordinated and well thought out research experience Ive ever been a part of. With so many ancillary departments involved, like Nuclear Medicine, Pulmonary Functions and Diagnostics , it’s amazing how smooth the whole system runs. Everything is coordinated and timed down to the minute. In the bronchoscopy room, they actually had a group of lab personnel standing by in their running shoes, so that they could literally run the tissue samples over to the lab the moment they they were removed from my body. This method ensures that the samples will be ultra fresh. (I think).

Btw, if you’re not familiar with this part of the Country, the University of Pittsburgh and it’s affiliated medical schools ,hospitals and clinics, is massive in size. More like a small city, I counted at least 4 city blocks of medical buildings 2 and 3 deep. Some the size of mini skyscrapers, all connected through a series of enclosed pedestrian bridges. Pretty neat since it snows here in the winter.

Here’s an abbreviated version of my actual testing itinerary:

Wednesday Afternoon: Completed a ton of questionnaires,did a verbal interview, and did pre&post bronchodilator spirometry, sputum induction attempt.
Thursday: Allergy skin testing (scratch test, not sub q), Full PFT ( lung volumes) pre&post bronchodilator.
Friday: Bronchoscopy prep, ( ie pre-medications and neb treatments), then nasal brushing, then the actual bronchoscopy with brushings and biopsy, but no lavage because of the potential side effects, more pfts
Friday Evening-Saturday,:Monitored in the hospital overnight.

As with any long distance trip , I didn’t eat or sleep very well and I’m totally worn out, but the effort of getting here to Pittsburgh to do this study was totally worth it. Definitively a positive experience and I would gladly return again if asked.

(Matthew, myself and Erin in the PFT lab)

(Chuck and Trish, two of the best nurses in the world)

Look at all the blood they took from me

(And check out this reaction I had to Grass allergen compared to tree allergen,which is the little dot to the left.)

Now for the not so good news….

Because this was the first time Ive ever had a bronchoscopy, it’s really the first time anyone has ever gotten a close-up look at the inside of my lungs. And what Dr Wenzel saw down there, wasn’t good. What she saw was lung anatomy that had undergone extensive remodeling. My airways appeared very scarred, stiff and fibrotic in nature…. seems my disease is much more advanced than previously thought. She actually found it quite amazing that I was still able to function as well as I do, let alone still alive. What was baffling to me, is that she couldn’t find any physical evidence of air-trapping or any of the other tell tale signs of COPD or emphysema that I often thought I had. It will be a few more weeks before they’ll have a final report, as they sent bits and pieces of my lung tissue and blood samples to various university labs for analysis, but suffice it to say, my long term survival isn’t looking too rosy right now.

They recommended that I seriously consider lung transplant surgery somewhere down the road. We all agreed that I’m not even close to that point right now, but that when the time comes, I shouldn’t procrastinate, as the waiting time for new lungs can take up to 3 years (asthmatics are usually put at the bottom of the list.) My one saving grace so far, is that my blood oxygenation is still relatively good(probably from all the aerobic exercise I do), and I’m still able to exercise, though that probably won’t be the case for very much longer.

Who knows, maybe someday I’ll be able to RUN the Boston marathon … instead of walking it!

Update as of 12-15-2009: Results from the computerized quantitative lung scans, did indeed show significant air trapping.

The field of Asthma Research desperately needs human test subjects for a variety of invasive and non-invasive clinical studies. If you’re an asthmatic, or if asthma effects your life in someway, you should really consider getting involved. We need to put and end to the suffering that this disease causes.

So anyway, later today I’m off to Pittsburgh, Pennsylvania for the SARP study. While I’m there, I’ll finally get to meet the famous Dr. Sally Wenzel . She’s done so much to advance the knowledge of the severe forms of this disease. I admire her and feel like I already know her.

My plane leaves at 11 pm tonight, and with a connection in Chicago, doesn’t arrive in Pittsburgh until 9 am tomorrow morning. I hate overnight flights, but that’s cool this time, because it’s one less night I would have had to shell out for a hotel room. In that regard, I was able to find a hotel just 2 blocks from the University of Pittsburgh’s Asthma Institute, where they’re doing most of the research procedures.

I’m scheduled for 3 days of testing, beginning with spirometry , CT scans and sputum collection on Wednesday afternoon. Thursday will be a day of pulmonary function tests and blood work, and then on Friday morning, Dr Wenzel is going to take look inside my lungs (bronchoscopy) and take some lung tissue samples for analysis. As a precautionary measure, they want to monitor me overnight in the hospital following the bronchoscopy procedure. If there are no complications(knock on wood), I’ll complete the study late Saturday morning, just in time to catch my afternoon flight back to San Francisco.

Not sure if I’ll have much time in between appointments to check out the sights, but I’d definitely like to try some of the local food joints. Pittsburgh is supposed to be home to some of the worlds finest fast food eateries. Primanti Bros and Original Hot Dogs, just to name a few. I saw both of these places on the travel channel a few years ago. The food looks awesome. Not exactly healthy cuisine, but it sure looks really tasty.

Ive decided not to haul my computer along on this trip, so I unless I have access to one while Im there and feel like blogging, I probably won’t be updating again until sometime next week.

Behave yourselves and try not to miss me too much.

The more I hear about this amazing women and all of her contributions to the study of this disease, the more excited I am to meet her and become one of her research subjects.

As Dr Wenzel jokingly told me the other day;
“A little MORE closing the loop still needs to happen. That is why I am so excited that I will get to meet you and have you personally participate in closing the loop.”

Wouldn’t that be something, if my DNA, lung tissue or some other bio marker did actually help close the loop?

Dr Sorkness does a great job of explaining the basics of asthma as well as the research the SARP is doing, in trying to find out what makes the severe form of the disease different from the milder forms. This is the same SARP study that I will be participating in later this year. Listen here