It’s been almost a year since Ive updated my asthma status. Here’s the latest report card. For the year, I give myself a C +

In general my lung function has only declined a couple percent which is good. The problem of course, is that when you’re down in the lower ranges like I am, even the slightest change can have a huge impact on the way you feel. My baseline FEV1 now ranges from 33-42%, compared to 36-47% a year ago.

Probably the most noticeable change, and my biggest complaint by far, is that I get short of breath much faster now when engaging in even physical activity. Magnify that 3 or 4 fold when I do activities like racewalking or marathon walking.

My dependence on opiates and anxiolytics to quell my breathlessness keeps growing. Where I used to only take these drugs occasionally, I now take them on a daily basis.
The good news is that they do help and have probably kept me out of the hospital on more than on occasion.

My medical establishment labels are unchanged ( no big surprise there). Under the GINA guidelines, I’m still labeled a severe persistent asthmatic. Under the brand new SARP phenotypes for severe asthmatics, I’m labeled a Catagory/Cluster 5, Childhood onset asthmatic. ( Thank you Dr Wenzel)

Symptoms
• I’m pretty much short of breath to some degree all the time now (even when I’m in my green zone). Most of the time it doesn’t bother me because I’m used to it. But in addition to the low level chronic breathlessness, I’ve also been getting these short, but very intense bouts of dyspnea which seem to spring out of nowhere. The sensation is like that of sudden suffocation. It’s like someone put a bag over my head. It feels like my respiratory muscles are too weak to expand my lungs when I take a breath in. The sensation makes me anxious, which then perpetuates this viscous cycle of not being able to catch my breath. It’s awful. This video ,which Ive used before in my blog posts, does a great job of depicting what these intense mini attacks kinda feel like. The only difference is that I don’t struggle the way the actor does in the clip.
  Thankfully these bouts only last an hour or two and are usually relieved with meditative breathing, multiple back to back neb treatments and sometimes opiates. Unfortunately, these intense flares seem to be occurring more frequent now….almost daily. They’re probably a result of declining lung volumes and increased sensitivity to air-trapping.
• For the last 6 months I’ve also noticed a slight, but persistent audible insp wheeze when I breath. Hence, the “Inhaler voice” It’s more annoying than anything else, but I hate the way it makes my voice sound. ENT actually checked my vocal cords and they look fine ( no stenosis)
• It’s becoming increasingly more difficult for me to sleep laying flat. I now have to pretty much sleep with my back propped up, almost to a sitting position. Last year I switched from a conventional bed to a foam bed, which helped ease my lower back pain.
• A noticeable decreasing tolerance to exercise and physical exertion in general. I get winded much easier now when I do any type of exercise or walks.

Incarcerations

• 2 hospital admissions so far this year, totaling 12 days. 8 of those in the ICU, and 2 of those on a ventiltor.
• Ive had one intubation this year, bringing my lifetime total to 16 ( we’re talking Ripleys Believe it or not stuff)

Fitness and Diet
• Despite my worsening dyspnea, I still force myself to walk at least 4 days a week ( 4-6 miles per walk) and even farther when I’m training for a race.
• Earlier this month, I switched to an all organic diet. Additionally, Ive cut my sugar intake in half and eliminated HFCS almost completely. It’s way too soon to see how much of an impact this new way of eating will have on my health in general, but I suspect it will eventually be a positive one. How could it not.

Treatment Plans
• My Asthma action plan and medication list are pretty much unchanged, except that I take methadone (10mg) every evening now.

New Treatment Options
• Bronchial Thermoplasty is no longer an option for me. My asthma is too severe, my lungs are too scarred. (* Speaking of Bronchial Thermoplasty, this is one of better articles Ive read on the subject. It’s objective, factual and well written)
• I’m waiting for clinical trials to begin ( hopefully by this time next year) on some new Th2 (IL-4/IL-13) blocking drugs.

  Th2 blocking drugs (if they turn out to work), block protein factors related to, but not the same thing as, allergy. These blockers are believed to work “higher up” on the immune inflammatory cascade (where things are getting started) so that they “could” block more things of relevance to asthma. I have a problem with mucus blocking my tiniest airways, which causes me to air trap. (air trapping is the number 1 cause of my suffering). We’re hoping that this new class of drugs might help with that. ( per Dr Sally Wenzel)

( read more)

Well, after all the training, all the planning, and all the hoping like crazy that my sore foot will somehow heal in time and that my lungs won’t act up too bad, for better or worse, to finish or not, the big daddy of race weekends is finally here!

Saturday night I catch the red eye out of San Francisco and should be in Boston bright and early Sunday morning. Mike was kind enough to pick up my Bib and Goodie-bag at the Expo today. This saves me from the hassle of having to go all the way into downtown on the subway after flying all night. The Boston marathon Expo is massive and a great place to buy race merchandise and get all psyched up about the race itself, but there’s nothing I really wanna buy this year and Ive already done the race once, so I’d just as soon skip the crowds and spend the day hanging around the hotel and resting up.The million dollar views of the Boston harbor and skyline from my hotel room are worth the trip alone.

My race top, courtesy of the University of Pittsburgh’s Asthma Institute and the best asthma doctor in the world, Sally Wenzel.
I figure that for every mile I walk, appx 10,000 people will see my shirt sleeves. My hope, is that my presence in this race will attract some much needed attention in the areas of severe asthma research, education and treatment.

Later on Sunday, Mike, myself and the crew are all gonna get together for an early dinner at a nice Italian restaurant. That’s probably where we’ll discuss the logistics of the race and who’s gonna to be doing what, when and where.The plan right now, is to arrive at the Athletes Village in Hopkinton around 7am. This will give us a couple hours to warm up and hopefully meet up with some of the other mobility impaired athletes, some of who’s stories are truly inspiring. The latest weather reports are forecasting intermittent scattered showers along the marathon course. Light, brief showers I can deal with, as long as it’s not too cold.

It’s weird, but I feel just as nervous and excited about this year’s trip, as I did last year. And even though I sorta know what to expect when I get there, it’s all still very much a big adventure for me. The only thing that will put a damper on the fun, is if I have to pull out of race because of my bad foot or my lungs. But no matter how the race turns out, I plan to have a kick-ass time in Boston!

So, that’s it. I’ll let you know how it all went next week after I return home.

To all the other runners out there who are doing Boston, good luck, have a blast, and keep an eye out for me!
To all my friends and readers, thanks so much for your continued support!

Oops…almost forgot, I know a lot of you who live in Canada and elsewhere might not be able to receive the Boston Marathon ATT athlete text alerts, so for those of you who are still interested on how we’re doing in the race, my friend Lis, has agreed to send out tweets throughout the day . Her twitter name is SFAUDIOGAL . I’m sure I’ll be updating on Facebook as well. Cheers!

This news came in after I posted……….

VIEW ALL NEWS HEADLINES

Just got my lung biopsy report back from Dr Wenzel. Seems I have high levels of a type of cell called a mast cell. A “normal” (not cancer) cell that has long been seen in asthma patients. But, mine are a slightly different version, which seems to be more common in people with really bad asthma. These new findings, along with all my other test results from the SARP study, are starting to paint a clearer picture of why my asthma is so severe.

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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(Arterial Line)

Thanks everyone for your support and especially to Kerri and Dr Wensel for staying in close contact during my 98th hospitalization. Thanks also to ALL the wonderful nurses who took care of me..You guys are awesome! Most of all, thank you to my dear Douglas who goes through hell every time I get sick. I’m so sorry I put him through this. Yes, living with a brittle asthmatic is definitely not for wimps.

Im starting to sound like a broken record with these hospital reports, but no kidding, this flare-up was no picnic. It ranks up there as one of the worse on record for me. On a scale of 1-10, probably an 8 or 9. I was pretty sick there for a while and was starting to have doubts as to whether I was actually going to pull through it. But it seems I have more lives than all my kitty cats put together and will apparently live to do this all over again sometime the future ( I just hope it’s not the near future).
Not sure what the trigger was this time, but I’m guessing that the cold I had from the previous hospitalization never quite went away. ( walking in the rain last week probably didn’t help either)

The primary problem this time, and what usually happens when I get really sick like this, is something called CO2 retention. The act (or work) of breathing becomes so hard, that the level of CO2 in my blood climbs really high ( what they call respiratory failure). I was in failure for 4 days with PCO2s ranging from 38 to 70 (when breathing at a rate of 50, that’s not good) My lungs get so tight, that I can’t move any air…I don’t even wheeze! When you listen to my lungs ,you can’t hear any air moving.

Because of all the problems I experienced with lack of sedation while I was on the ventilator the last time, I chose not to be intubated this time ( probably a mistake,because it would have immediately lowered my Co2). Instead , we relied mostly on cont alb nebs, bipap, and steroids to reduce my CO2 indirectly. Thanks to a call put out to Dr Wenzel ( who btw, was in London England at the time), a huge increase in my steroids was ordered along with a few other strategies she recommended, seemed to set the stage for my eventual improvement. Finally on the 3rd day with the increased steroids on board, my lungs started opening up , my work of breathing decreased, and my CO2 started to fall. By day #4 my PCO2 was back to normal and I was starting to wheeze again ( a good thing) . Ironically, while all this was all going on, I found out that there was actually a world-wide shortage of propofol. So even if I chose to be intubated, they would have probably used an alternative sedative agent. Weird huh

On day # 5 I was stable enough to be transferred out of the ICU to the step down unit where I continued to improve. On day #7 I was bugging them to let me go home. On day #8 I was paroled.

This time around I thought Id do something different and post some of the good pictures instead of the bad ones. Of course, I still have to include my battle scars pics. I must have been stabbed 50 times just to gain access to 2 functional IVs and one arterial line. My arms look like black,blue and green swiss cheese and are really really sore.

Today, Im breathing a little better, though my lungs sound like a washing machine during the wash cycle. Course and wet sounding wheezes that you can hear from across the street. Again, this is a good thing and means that my lungs are opening up. The danger is not quite over, as there is a possibility that I could rebound during the next few days, but I think for the most part, I’m over the proverbial hump. In any event, I can already tell that recovering from this exacerbation is going to be slow and difficult process.

This may not come as a big shock to everyone, but these recent flares have taken such a toll on my body, that a Boston victory seems unlikely. There’s just not enough time to regain the training Ive lost. Having said that, I’m not going to let asthma stop me from doing what I love. Boston may, or may not happen. We’ll just have to wait and see.

Just one final thought…..Though I may look happy in the pictures above, it was all for the camera. I’m a ham, no doubt, but I’m also human. These recent exacerbations( 3 hospitalizations in 3 months) have left me incredibly frustrated and down on myself. Steroid induced or not, right now I’m in a pretty deep depression that I’m not sure how long will take to climb out of. I’m not really sure that I even wanna continue blogging about my disease. I hate to be selfish, but lately, just the word “asthma” or the thoughts it provokes, make me wanna run and hide. If I seem slow to respond or update, please bare with me. Thank you everyone for being such good friends. We have built a huge community around our disease and are getting the word out. You should all be very proud.

Postscript 2-7-2010; I had no idea I would strike such a chord with my friends over my comment about not wanting to blog about my asthma anymore.
Don’t worry, I will definitely continue to blog and post on asthma support sites. Maybe not as often , and maybe not focusing as much on my own asthma, but I will definitely keep everyone up to date on what’s happening in my crazy world. I’m just really burnt out right now and I need to focus my attention on things other than my own breathing. Ive been blogging about my asthma and my declining health for 5 continuous years now. I don’t want to be one of those people who chronicles their own death(as noble as that may be). I need to start paying more homage to my idol, the King of the ballad , Mr Manilow. (I haven’t even seen his new show at the Paris Vegas yet.)
So NO worries…. I’m not going away, my life is way too fascinating to keep all to myself

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 after each bad asthma exacerbation or flare-up, I feel compelled to write something about the recovery phase that follows. You would think that after going through this process more than a hundred times, that I would get use to it. You learn to deal with it a little better, but you never get used to it.
Better in some ways, and worse in others, the anatomy of this recovery is pretty typical. So far, this is how it’s been going……

Dyspnea : As with most of my post hospital recovery periods, days 5 and 6 have been the hardest to deal with in terms of being short of breath. I was breathing well for the first couple days after being discharged from the hospital, but then my dyspnea levels gradually crept back up again. Yesterday it was unbearable. A mixture of bronchospasm ,air-trapping, stomach bloating and humid weather, I felt like I was suffocating . I’m not sure what today will bring, but I hope things turn around soon as I’m starting to get to really tired of this.

Steroid Withdrawals : This time around they’ve been fairly mild. No major psychosis, just some mild muscle cramps, acne and mood swings. The main reason Ive been spared this time, is because my maximum dose in the hospital was only 60 mg per day. In previous hospitalizations, Ive been on as high as 300-500 mg per day, which can lead to weeks of intense withdrawals and even the potential to re-exacerbate. I have to thank Dr Wenzel for this one. She’s been pretty much able to prove, that high doses of steroids don’t do much for someone with my type of asthma. Currently on my taper, I’m down to 30mg.

Opiate Withdrawals: Next to the bouts of severe breathlessness, the worst problem Ive had to deal with this time, are opiate withdrawal symptoms. While I was in the hospital, I received a lot of IV narcotics for my dyspnea. And because I was on a ventilator this time, I received even more than I normally do. I was getting them almost every hour for the first 4 days and then about every 2-3 hours for the last 3 days. That figures out to more than 100 doses of intravenous Dilaudid and/or Fentanyl. That’s a lot of opiates to put in your body in just a 7 day period. And since I don’t take any of these more potent morphine-like drugs outside of the hospital, stopping them abruptly ( ie cold turkey), always causes me some pretty nasty withdrawal symptoms. Insomnia, nonstop chills/rigors, restless leg syndrome and muscle cramps, just to name a few. The symptoms gradually fade, but the first week can really be a bear.

Body Trauma and weakness: Laying in a hospital bed for a week, working really hard to get a breath, getting jabbed with needles and pumped with drugs and having a tube shoved in my wind pipe (and one in another place), has been pretty traumatic to this old body. I’m starting to feel all the aches from all the IV bruises and Im still weak as hell. You should see the welts left on my belly left from the Lovenox injections.

Looking forward to better days: No matter how bleak the situation seems during the first week of a rough recovery, I always try to focus on better days ahead. Consider this if you will; Yesterday, I could barely walk from my bedroom to the living room without getting totally winded, a distance of less than 10 meters. In just 13 weeks from now, I will attempt to walk 42 THOUSAND meters, at the Boston marathon. That means that between now and April, my endurance will have to increase 4000 fold! The way I feel right now, it seems an impossibility. Give me another week, and my outlook will probably be totally different.

Doing something special for myself: Finally, and maybe this is the selfish part of me, but if survive this exacerbation ,I plan to treat myself to a few goodies. This time, a haircut, a new pair of racing comps (shoes), and a ticket to Barry’s new show over at the Paris Las Vegas. Ok, so I’m a little spoiled.

Battle scars: 4 failed arterial line attempts and 12 failed IV attempts. I have no veins left and my radial arteries are so scarred up from previous insertions, that it’s virtually impossible to get access unless they put a central line in. Very frustrating for the doctors and very painful for me.

I’d been having vague flu like symptoms, such as stomach bloating and chills for about a week before my lungs started to act up. Less than 3 weeks earlier I had received the H1H1 vaccine as well as the regular annual flu shot, so the last thing on my mind is that I might actually be coming down with the flu.

By New Years Eve afternoon my peak flows started to trend downward, and by that evening they had dropped from 340 to 200. My sats fell from 97 to 89%. I was requiring neb treatments almost every hour and had considered going to the ER right then and there, but had decided against it because of it being New Years eve and all. I figured the hospitals would be jammed with New Years revelers , so I decided I would just try to tough it out till morning and then re-assess. Maybe by then I would feel better. After a horribly breathless and sleepless night spent next to the nebulizer machine, I figured I had had enough, bit the bullet and went to the ER.

As is usual for me, rather than being carted in or delivered by ambulance, I strolled into ER on foot. I told the triage Nurse what the problem was. She took down my name and asked if I was in serious distress at the moment ( since I never really look sick) , I said I was OK at the moment . She had me take a seat in the waiting room ( which by the way, was pretty much emptied at 11am). 5 minutes had past, then 10 minutes, and 30 , and still they had not called me in. By now it had been almost an hour since I had a breathing treatment and I was really starting to close up. I hate making a scene , but I thought I was going to pass out, so I stood up and walked back over the triage window. The Nurse took one look at me and said ” OH MY GOD .. You haven’t been seen yet??? I said no, and I think I’m going to pass out. Within 30 seconds I was on a gurney on my way to the resuscitation room . I didn’t actually pass out, but I sure prompted a lot of people into action. Before I knew it, I was on a continuous albuterol neb with people whirling around me trying to get IVs in. They asked the usual questions.. Have you ever been intubated? Before I could answer, one of the ER doctors recognized me and said “He’s a bad one….he’s been intubated a dozen times”
Within a record 30 minutes, the ICU team had completed their evaluation and I was on my way to the unit.

In the ICU they put me on the usual Bipap setting of 12/5 with 15mg/hr of Albuterol piped in. Because of my flu symptoms, they swabbed my nose with the rapid flu test, put me on droplet precautions and placed me in isolation, which means anyone coming in contact with me had to wear a mask and eye shields. Im sure the staff weren’t too please about that ( I know from personal experience what a pain it can be to work with a patient who is in isolation, especially a ventilator patient.)

For the first few hours, the bipap seemed like it was helping, my Sats had increased to 98% with an FIO2 of just 40%. Although I was saturating well, I was starting to feel that all familiar ache that I feel when my CO2 starts to climb. After several unsuccessful attempts at placing an Arterial-line ( which hurt like hell), they finally gave up and resorted to doing individual ABG draws.

(My Hannibal Lecter look before Intubation)

I had now been on bipap for about 4 hours and my PCo2 was starting to climb. The first PCO2 came back at 43, but I was on bipap and my resp rate was in 50s ( this is not a good sign). An hour later my PCO2 was 60. A half hour after that, it was 66 and my Ph 7.29 . It was time for the intubation talk. Rather than himming and hawing about how long to wait, this time I told them straight up, just intubate when you think Ive had enough. They agreed, and 30 minutes later it was lights out for me. 14 hours later I woke up with a tube down my windpipe.

Here’s a clip my camera-happy partner took. They were in the process of weaning me off the ventilator and just about to take the ET tube out.

About 1 hour after that video was taken, I was extubated and talking up a storm …or should I say cuzzing up a storm. Why was I not kept down longer? Why didn’t they use propofol to sedate me. Why were the RTs being so rude to me this time?

The anesthesiologist had promised me that they were going to use propofol and keep me asleep for at least 48 hours, but it turns out that I had the same weird reaction to propofol as I did during the previous intubation. Something called “infusion syndrome“,so they had to stop giving it to me. They thought it was strange, because propofol infusion syndrome usually occurs in people who are sedated for several days.
For that reason they had to use Versed and Fentanyl instead of propofol to keep me sedated, making it much harder to keep me asleep. At one point I actually remember walking up “paralyzed” and unable to move or communicate. Apparently they had not sedated me enough and I woke up before the paralytic agent (a drug they use when they insert the tube) wore off. Talk about a horrible experience. Nothing like being a zombie. In total, I was on the ventilator for less than 2 days.

After being extubated the RT gave me the choice of using the continuous neb or the bipap…. of course I chose the neb. I did Ok for about an hour , but then once again ,my PCO2 started to climb. Not happy with my recent ABGs, the RT comes into the room accusing me of causing this spike in my CO2, because of not following her instructions of using the bipap. WHAT THE F!
Since when is the patient suppose to decide what type of therapy he gets? Even an RT/ patient deserves someone else to call the shots when he’s sick. I didn’t like her attitude and I told her. The next thing I know, she sends in her supervisor who starts patronizing me. He says to me…” your Ph is 7.30 and your CO2 is climbing again. If you want , I can help you correct it. If you’re not willing to help,then there’s nothing I can do for you. WHAT THE F AGAIN? What’s with the all the attitude. Eveyone was nice to me before I got exubated. What did I do?

I was so pissed off I wanted to scream, but I figured that they would treat me better if I did what they wanted. So I just sucked it up and did what they asked. The male RT set me up on what they call “non-Invasive” ventilation. It’s basically where they hook you up to a ventilator with a mask instead of an endotracheal tube. Its just a fancy way to give Bipap. He also encouraged me to play with the ventilator settings , so that I could adjust it the way I wanted. ( Remember, I’m an RT. I know how to operate ventilators). At this point I couldn’t figure out if he was making fun of me, or if he appreciated the fact that I was trying to play ball with him and cooperate. In any case, his act of showing a little kindness paid off, and within a short time my ABGs were stabilized and everyone was happy, including myself.

12 hours later I was strong enough to breath on my own and they were able to discontinue the breathing machine entirely. So, what was up with all the attitude they were directing my way? ( more about that later)

I have now been in the hospital for 3 days, been on and off the ventilator, and was starting to breath a little better. The Rapid flu antigen test came back negative, but because the test is only 50% accurate, they decided to do the more sensitive test for swine flu. That test takes 6 days and had to be send the to state for processing. What this basically meant, is that even if the test was negative, I would be spending the rest of my hospital stay in isolation. I really didn’t mind because it assured me a private room my entire hospital stay.

On Day 4 I was deemed stable enough to be transferred to the step down unit, where I stayed until my discharge 3 days later. While there , I was placed back on a continuous albuterol neb, but this time the dose was decreased to 5 mg per hour.I did fine on that , but began coughing much more than I usually do. My biggest complaint was the congested nose and the unrelenting chills from whatever virus I had. Eventually , my chest became congested as well. I was able to cough up some gunk , which they analyzed in the lab. Seems on top of this virus from hell, I also had a bacterial infection brewing and now had bronchitis. The next morning they started me on Doxycycline and decided to keep me in the hospital an extra day. On Friday I still felt like crap, but was home sick and convinced them that I felt well enough to go home.

So, what was all this rudeness and attitude I was detecting from some of the RTs and some of the doctors? Well , it turns out that some of the ICU doctors were concerned about my tolerance for opiates. The fact that I was requesting Fentanyl and Dilaudid to ease my breathlessness was apparently a red-flag to them. Despite the fact that my personal pulmonlogist’s condone the use of opiates to treat severe breathlessness, it’s still not a widely accepted practice in the ICU. For many critical care doctors and respiratory therapists, if you’re taking opiates, you either have an addiction to them, or you have psychosomatic illness. In either case, you are definitely treated differently. You’re basically treated like a psych patient instead of a medical patient. In my case , neither of their assumptions were true. The fact is, I only take opiates ( or request them) when the work of breathing becomes overwhelming to me. The breathlessness that I experience is caused by my narrowing airways during an attack. It’s this breathlessness that causes my anxiety, not the other other way around. And that’s the part that people have a hard time accepting.

I am not an anxious person, unless I’m suffocating to death. I can see now though, that my requests for painkillers to make my breathing easier, would probably account for why some of the doctors were acting weird around me. On more than one occasion, I has some of the doctors trying to convince me that I should try some long acting anti depressants and/or anti -anxiety meds, instead of opiates to manage my dyspnea. They also gave me a list of referral to various specialists who supposedly deal with dyspnea management ( all of whom, Id seen in the past without success). I reminded her, that I was taking opiates on the recommendation of one of UCSFs own highly acclaimed palliative care doctors.
In the end, I basically told her that we had been down this road many times before, and I that I refuse to discuss the matter any further. It’s my life, and if I choose to take opiates during bouts of severe breathlessness, that’s my choice, and it should in no way, change how I am treated during an active severe asthma exacerbation. Case closed!

Dont get me wrong, I’m grateful, and for the most part , satisfied with the medical care I received during this hospital stay. There were a lot of good things that happened as well. For example, for the first time ever, the doctors actually granted my request to not exceed 60 mg per day of Solumedrol or prednisone, which really helped. I’m am however, not too happy with the way I was spoken to by some of the other staff, especially the RTs . There seems to be this weird vibe I get from other Respiratory Therapists when they find out that I’m a Therapist myself. Either they like me right off the bat or they are intimidated by me… I’m not sure what’s up with that. I was also made to feel by some of the physicians, that I somehow brought this exacerbation on myself by over -reacting to my own dyspnea. That simply wasn’t the case. I’m pretty sure it was the flu that triggered this event, not anxiety. Besides, I think it would be pretty hard to go into full blown clinical respiratory failure, just from anxiety alone.

Bottom line…Much like credit report, incorrect statements or diagnosis that end up in your chart , can have a negative effect on the type of medical care you receive for years to come. I think this is especially true when it comes to medically complex patients (such as severe asthmatics.) A lot of this misinformation could be avoided if physicians were allowed to follow their patients in the hospital. Of course, that’s not going to happen. Evidence based medicine is here to stay.

On asthma severity scale of 1-10, this probably only ranks a 6 or 7, though having the flu at the same made it feel like a 10. Number 97 is behind me now, but I still have a long recovery ahead. It will take me weeks to regain the strength I lost in just one week of illness. As Ive said before, the aftermath of a severe asthma exacerbation is often more difficult to deal with than the actual attack itself.
It often takes several days after the hospitalization before you start feeling the trauma of what your body has been through. Being jabbed dozens of time with needles and catheters. Lack of sleep, sensory overload, having a hose shoved down your windpipe, being place on artificial life support. And then of course , there’s all those potent drugs. All these catch up with you. Yes, the recovery is often the worse than the treatment.

If there are any bright spots to this other wise crappy week, it would be the wonderful Nurses I encountered in both the ICU and Step down units who have taken care of me many times. They are angels.

And finally, a special thank you and hug for Kerri and Dr Wenzel who took the time to check up on me everyday while I was in the hospital via telephone. Dr Wenzel recommended that they do a PFT on me for discharging me,..and they actually did it! Now, that’s what I call clout!

PS…In all fairness, the RTs in question were new to me . I had never worked with them before. Maybe they weren’t aware of my little quirks, but it still doesn’t give them the right to treat me like a 2 year old. As far as some of the physicians concerns, I suppose they were justified, I just wish they would talk to me before rushing to conclusions.

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.

Last week after finding out just how damaged my lungs really are, the only thing going through my head was..WHY? All of the assumptions I had made about my asthma throughout the years, suddenly didn’t seem to make sense anymore. I was beginning to wonder if I really ever had asthma at all? Maybe I just thought it was asthma, when it was actually something totally different? These are the crazy things that were going through my head. I had a ton of questions I desperately needed answered , and since she knows my lungs inside out ( literally) , who better to ask than Dr Wenzel.

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.