( This page was last updated on 06-20-2009)

Yup, my real name is Stephen and my asthma is about as bad as it gets. I’m labeled a “Severe persistent” or a ” Severe refractory asthmatic” by the medical establishment and always at risk for what they call “NFA” . What that basically means, is that despite being on a maximal therapeutic regime of meds and treatments, I still have symptoms pretty much 24/7 , which at times, escalate into pretty severe attacks. I’m basically ticking time bomb. I seem to make a lot of ER doctors nervous or, as one of my lung doctors jokingly says, ” I’m the reason why some doctors don’t want to become Pulmonologists”.

I was diagnosed with asthma at the age of two months, and after a lifetime of persistent and frequent severe exacerbations, my lungs have undergone extensive remodeling ,which now kinda resemble those of someone with emphysema. My baseline FEV1 is only about 35% , which equates to a “lung age” of 140 years! I also have a problem with persistent air-trapping (dynamic hyperinflation), which is very uncomfortable and complicates matters . Because of the permanent damage done to my lungs, I now fall into the asthma/COPD ( chronic obstructive pulmonary disease) category. However, when I have acute exacerbations that require emergency medical care, I’m treated primarily as an asthmatic.

Over the span of 50+ years, I’ve endured countless days and weeks on ventilators, Bi-Pap machines,Heliox and continuous nebs. In my lifetime Ive made over 100 trips to the ER , and on  6/01/2009 , I racked up my 94th hospital admission for asthma since birth . I’ve been intubated and mechanically ventilated 14 15 times for respiratory fatigue and/or for full blown respiratory failure, and one time at the age of 16, for a respiratory/cardiac arrest requiring CPR, which resulted in 2 fractured ribs, but which probably saved my life. I’ve come closer to receiving a hole in my neck (tracheotomy) , than I care to.

Exacerbations that land me in the hospital, usually come out of the blue, without warning and with no known triggers. Occasionally I’ll get an attack that’s precipitated by a simple viral infection (ie. a common cold), and these by far, are the one’s that make me the sickest.(Thankfully, I don’t get too many colds). Other triggers such as seasonal allergies, emotional stress, exercising too much or just plain getting run down, can set me off.

Wanna see how I really feel about this stinking disease ??

Poked and Prodded to rule out other causes:

Asthma action Plan: For most asthmatics the goal of an asthma care plan is to keep one step ahead of your asthma by following a pre-planned set of detailed instructions on how to control your asthma and stay out of the hospital. My care plan is a little different , in that the primary goal is simply to avoid intubation . As much as I hate the thought of it, going to the hospital is actually part of my preventative treatment plan. Let me re-phrase that….seeking immediate medical attention is part of my preventative care. In most cases, that means the ER. The problem is, I never know how bad things will get once an attack is underway. My lungs have bad habit of clamping down really tight without much warning. No air can get in, no air can get out. So, at the point where most asthmatics would just be instructed to increase their inhaler use and/or start on prednisone, my plan calls for me to go directly to the ER in case things escalate. If I do end up in the ER, after 6-12 hours , I’m almost always transferred to the intensive care unit ( what I call prison) for a couple days. Not necessarily because I’m critically ill , but because of my long history of crashing really really fast. If I’m lucky and get I to the hospital early enough, sometimes I can get by with just some continuous nebs, some IV steroids, and a regular hospital bed. In any case, once I pass through those Emergency Room doors, it’s pretty much a sure bet that I wont be walking out anytime soon ( which is another reason I’m hesitant to stick to my care plan).

My current care plan only has only 3 instructions that I’m supposed to follow religiously:

#1) I’m supposed to check my peak flows/FEV1 and assess my dyspnea level at least 4 times a day.
#2) If my peak flows drop into my yellow zone (which is PF reading of less than 290) for more than 8 hours, I’m supposed to bolus myself with 60 mg prednisone and then alert one of my lung docs.
#3) If after 24 hours my peaks are still in my yellow zone or if I dip into the red even briefly, I’m supposed to go immediately to the ER for treatment and monitoring . (Thats the part I’m not so compliant with, mainly because I don’t always feel as bad as the numbers indicate).

A matter of perception: Because of my life long breathlessness , I seem to have a higher tolerance for breathing discomfort than most people.For some reason, I don’t always accurately perceive how bad my breathing is getting until it’s too late. Add to that, my already very low baseline lung function, and it doesn’t take much to send me over the edge.
Being aware of even subtle changes in my breathing and using a peak flow meter to validate those changes, is the key to keeping me out of the hospital.

A victim of the 1960s asthma culture I attribute the severity of my disease on ignorance and the 1960-70s asthma mentality of over-treatment or under treatment, but never preventive treatment. ( Im currently writing a book on that subject). I came from a very poor insuranceless family. What little medical care I did receive, came mostly through the social services system in the county we lived in. As a child I survived mostly on toxic, over- the -counter drugs like Primatine Mist, which my friends would occasionally shoplift for me, as my family was too poor to afford asthma medications. As a young adult I was prescribed massive quantities of Theophyline ,which was a popular therapy at the time. I vividly recall my heart pounding fast and hard all the time . I also remember having constant tremors and nausea.
To make things worse, I started smoking cigarettes . Don’t ask me why…. but I think a lot of it had to do with my prednisone dependence ….and the Theophyline !! I even smoked in the hospital one time , right after being extubated!! Is that crazy or what???? Needless to say, the respiratory Therapist on duty wasn’t too pleased. In total , I smoked on and off for about 7 years . At the age of 36, through the hospital where I worked , I was offered the first nicotine patch available by prescription and went for it. Two frustrating years and a dozen quit attempts later, I was smoke free …. and remain so to this day!

Steroid Hell:I’ve taken some form of corticosteroids, continuously, my entire life. Good ol’e Prednisone & Solumedrol (aka “The Evil Candy” ) The double edged sword. You can’t live with it, you cant live without it. I try my best to live on very little. From the age of 16 to 38, I was prednisone dependent with daily doses sometimes exceeding 100mg per day. As a result , I developed severe Cushings syndrome , heart and adrenal problems as well as bouts of severe steroid psychosis ( maybe that’s why I’m so weird now.) You haven’t lived until you’ve experienced full blown Prednisone/steroid psychosis . When you’re on high doses of the drug, at best it can make you feel unreal, but more than likely , you will experience severe bouts of depression mixed with periods of euphoria. I can remember thoughts of suicide on more than one occasion while tapering off this poison. In my early 40’s , with the help of a skilled and caring Pulmonologist, some steroid sparing drugs like Methotrexate and the newer, more powerful steroid inhalers , I was able to gradually wean off the Prednisone. In a period of appx 18 months , I went from 80mg per day to ZERO per day. On 6-1-2007, I was restarted on a small maintence dose prednisone at 15mg a day, but discontinued that as well 6 months later. As of early 2008 , I have been prednisone free!

Today I continue to get my steroid fix with daily Advair , occasionally augmented with additional Flovent. During moderate to severe flare-ups (which occur about every 10-12 weeks), I still have to load-up on Prednisone to about 60mg , but I stay on it just long enough to get me over the hump and then I rapidly taper. I’m never on high doses for more than 3 weeks at a time now. Even when I’m hospitalized, I instruct the doctors only to administer enough steroids to save me from possible intubation or death.

As with most chronic steroid users, I’m suffering from the long term side effects of the drug, including osteoporosis , steroid induced diabetes, skin problems , muscle problems, vision problems and on and on and ..on .

Allergies: I’m atopic with severe reaction to dust mites , certain grass and tree pollens, but surprisingly… not to cat fur! At the age of 16, I had an anaphylatic reaction to penicillin. I seem to have a strong allergic component to my asthma as evidenced by extremely reactive skin tests to common allergens. Oddly enough though, my circulating IGE levels are usually low and this has my doctors perplexed. On December 20th 2006 I began a one year trial of Xolair injections. On 12-1-07, I stopped taking the Xolair as there was no evidence that it was helping me.

Pulmonary Functions: I have significant airway remodeling as evidenced by decades of worsening pulmonary function tests. At present, my baseline post bronchodilator spirometry numbers are all in 5-38% of predicted range. On a good day my FEV1 runs about 40 % , during exacerbations it can drop as low as 15%. My FEV1/FCV ratio is less than 50% . My baseline peak flow can be as high as 400 , but fluctuates constantly, and can fall below 200 within minutes. Volume studies show some hyperinflation, but My TLC is within normal limits. My VC is 78% predicted. My DLCO is in the normal range, and though I desaturate during severe flare-ups, my oxygen saturations usually return to normal with in days of my recovery. I am not a CO2 retainer.

Air-trapping I also have a problem with air-trapping that increases proportionally with exertion. Because I already breath at maximal expiratory flow rates, its difficult for me to increase my minute ventilation , making strenuous or prolonged physical activity ( ie marathon walking), incredibly difficult and sometimes dangerous. I would also like to add , that while I agree that I have a strong air-trapping component to my disease , which is exacerbated during prolonged physical exertion, I believe that a lot of this air-trapping is caused by chronic inflammation of my airways, as opposed to structural changes that you would see in a person who has emphysema. I believe this, because there is always a delay in the onset of symptoms after exposure to prolonged exercise. I believe it’s during this”delay” period, that some kind of physiological process or response is taking place in my lungs and is responsible for the symptoms that develop later on.

Medications: Albuterol MDI 2-4 puffs Q2 PRN along with nebulized Albuterol solution Q2-4 hrs PRN , Spiriva QD, Advaire500/50mg BID with additional Flovent 220mg up to 6 puff BID ( when I’m weaning off prednisone) off the pills , Flonase, Zyrtec and Singulair QD , Zileuton ER tabs BID, Septra QD ( to prevent pneumocystis pneumonia Discontinued on 6/2008) ,Prednisone 60 mg for flare-ups only (with rapid taper), Xolair 150mg injection Q monthly ( discontinued 2-2008)
During the last half century, I’ve probably inhaled more Beta agonists (ie. Albuterol), than any other living person . On 8-1-2006, I underwent genetic testing for the “Beta Adrenergic Receptor Genotype” to see if some of these drugs might actually make my breathing worse……. The results came back as {hetereozygous Gly /Arg} , which would predict a normal response to Beta Agonists ie, Albuterol .

Breathlessness: I’ve pretty much been short of breath to some degree, my entire life. Unless it gets really bad, it’s no big deal for me–I’m used to it… I don’t know any better. Sure, I have my share of really good breathing days , but I don’t think Ive ever experienced a FULL 24 hour period when I was completely symptom free. Often I have a distorted perception of how I sense my own breathing. Sometimes I have a hypersensative awareness to my breathing, and something as subtle as a small change in barometric pressure or an increase in relative humidity can make me feel like I’m suffocating to death. Other times, I can be on the brink of full blown respiratory failure and not even realize it.
As mentioned earlier, I experience frequent bouts of air trapping ,which attributes to most of dyspnea. Though not life threatening in and of itself, these breathless bouts are extremely uncomfortable and make you feel absolutely MISERABLE! It feels like someone sitting on your chest, just enough to where it’s hard to breath. Though usually attributable to over-exerting ones self, these bouts can also come out of nowhere, can last from a few days to several weeks, and often , can make me anxious and extremely irritable. This increased anxiety level in turn, can make my breathing even worse , which then snowballs into a vicious cycle. I find that Bronchodialators don’t offer much relief from this type of breathlessness, but sometimes anti- anxiety and/or opiates/ and /or flutter valve therapy helps. Recently , Ive noticed that Anticholenergics (ie. Atrovent ) in combination with Beta Agonists ( Albuterol), seems to help take the edge off..at least temporarily . But usually , I just have to tough it out until the condition resolves itself — (usually when the lungs decompress).

One of the research projects I recently completed (conducted by UCSF and the Washington State Univ), dealt with dyspnea and the importance of regular exercise for patients with chronic lung disease.  In that program I learned a lot about breath control, relaxation, and imagery techniques to help control the breathlessness that I get from air trapping and obstruction. But, unlike most of the people in that study group who had mostly pure emphysema, I also have to deal with very twitchy bronchial tubes that can constrict without warning ( bronchospasm)and can leave me gasping for air at the drop of a hat …… a double whammy if you will.

To Wheeze or not wheeze , YES … almost every asthmatic wheezes, but not all the time and certainly not always during a severe attack .In fact, red zone or green zone, I rarely wheeze at all ! When I suffer a severe attack , what they call(” Status Asthmaticus” ), I often get so tight , I can barely move any air, not even enough to produce a wheeze. If my peak flows drop below 150 , it usually means I’m gonna be buying a tube shortly ( hospital lingo …for “intubation” ) After a few days of machines and drugs, and as my lungs gradually begin to open up, I’ll usually start wheezing, but not always. It’s usually when the exacerbation is triggered a chest cold, that I wheeze up a storm. So loud in fact, that the noise of my own breathing will keep me awake for days on end. Unlike many asthmatics I rarely cough or produce any phlegm.

Self Image : Because of I’ve had breathing problems my entire life, to this day, I am still embarrassed by it. Don’t ask me why, but I’ve learned to disguise most of my symptoms, and most people, when they meet me for the first time, will comment on how “healthy” and ” Normal I look. Call it “denial” if you want , but I will always downplay how sick I feel , especially if I think it will keep me out of the hospital. Of course this strategy usually backfires and I end up getting sicker than I would have been, had I come in for treatment at the earliest signs of trouble.

Exercise: Despite the fact that I have slowly conditioned myself to walk marathon distances, my exercise tolerance STILL varies sharply from day to day. There are some days I’m too short of breath to take the garbage out, while on other days I can walk for hours on end without a problem .  Much like a person with emphysema, I’ve lost most of the elasticity of my small airways, so I tend to air-trap ( dynamically hyper-inflate.) This lung hyperinflation occurs proportionally , and over time, with any physical exertion. This means that the longer, or harder, or the faster I exercise or walk, the more hyper inflated my lungs become. Sometimes this air trapping make it very uncomfortable for me to continue exerting myself, other times it doesn’t bother me at all.  The key of course to any fitness program, is moderation. It is essential that all persons with asthma or COPD , exercise EVERYDAY…just don’t over do it!…… like I do

Documentation If you’ve read down this far, feel free to take a peak at some of my actual medical records, if you’re so inclined , by clicking the link on the sidebar. The password is “lungs”.

Prognosis: How Ive managed to survive for this long..no one really knows. There’s not much data on people who , despite having severe refractory disease, are able to function as well as I do. I’m somewhat of a medical oddity, in that despite the severity of my disease and my constant breathlessness, not only have I survived into my 50’s, but I can also walk marathons!
I’m probably luckier than most too, because Ive learned to live fairly well, despite my disease . Having decent health insurance and a team of superb doctors, who for the most part, support the crazy things I do, probably doesn’t hurt either.

Pinning down a firm prognosis for someone like me is difficult , because I have elements of both asthma and COPD ( Fixed asthma). My ratio of good days to bad days is still about 50/50 (though recently it’s begun to trend the wrong direction )
If you go by the GOLD COPD FEV1 severity staging index , or the B.O.D.E survival prediction formula , or by my history of intubations ,hospitalizations and NFA’s, I should have kicked the bucke years ago. No doubt I’m living on borrowed time, but aren’t we all? Seriously though, I don’t think my longevity is such a big mystery. Im pretty sure that I’m alive today, because of all walking and exercise I do. If you look at all the survival predictors out there for severe lung disease ( which includes severe asthma), it’s the people who are still able to exercise, more specifically people who can walk, that live the longest.(That….plus maybe good genetics.) My lungs might be trashed, but the rest of me is in pretty decent shape. Maybe I’ll live to be 100! I might live longer….or I might linger longer…..who knows.

Finally, THE DISCLAIMER: So that I can have a clean conscience.
While I’ve achieved some pretty amazing results with my fitness walking program, every marathon or half marathon walk that I attempt, takes a lot out of me! I have somewhat of a “dare-devil attitude toward my disease and sometimes I foolishly try to exceed my physical limitations, making myself sick and occasionally putting my life at risk.
Daily exercise is crucial for people with any form of lung disease!  We all know that.    Just be careful. Don’t over do It, and ALWAYS consult with your Physician before doing crazy stuff like I do.

BREATH EASY!