My Asthma

(This page was last updated on 2-24-2010)

Yup, my real name is Stephen and my asthma is about as bad as it gets. My disease is much different than what most people think of when they hear the word asthma. I’m labeled a “Severe refractory asthmatic” by the medical establishment, and am always at high 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 occasionally escalate into pretty nasty attacks. I’m basically a ticking asthma time bomb and tend to make a lot of ER doctors nervous when I show up at the front door. I am privileged to have a world renowned severe asthma specialist , Dr Sally Wenzel, following my care.

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 well over 120 trips to the Emergency room for flare-ups, and on 1/28/2010 , I racked up my 97th 98th hospital admission(since birth). Ive been intubated and mechanically ventilated 15 16 times for respiratory failure/fatigue, including two times for respiratory arrest requiring CPR. 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 or the flu), 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.

History: I was diagnosed with asthma at the age of two months, and after a lifetime of persistent and frequent exacerbations, my airways have undergone extensive remodeling, which now resemble those of someone with moderate to severe pulmonary fibrosis . What probably began as garden variety asthma in early childhood, somehow got out of control and morphed into severe irreversible lung disease that responds very poorly to treatment. On a good day my FEV1 is only about 40% of normal, which equates to a “lung age” of about 116 years!

Over the years as my lungs have become stiffer , Ive lost much of the twichyness and spacisity of my larger airways, so I’m less likely to wheeze or experience acute bronchospasm when my asthma flares up. But, because my smaller airways are so damaged and have become so narrowed, just the slightest spasm or inflammation can cause them to completely close up, which is what usually lands me into so much trouble. I also have a problem with air-trapping which tends to make my flare-ups more difficult to control. The tighter my lungs get , the more anxious I tend to get. The more anxious I get, the faster I breath. The faster I breath, the more I air-trap. The more more I air-trap, the worse my exacerbation gets. Until this vicious cycle starts feeding itself, landing me on a ventilator.

Asthma action Plan:: For most asthmatics the purpose of an asthma action plan, is to provide you with a set of detailed written instructions to follow, so that you’ll know what steps to take when your asthma flares up. The ultimate goal is to keep your asthma under control so that you can avoid a trip to the Emergency room. Because I don’t respond very well to the usual at-home asthma treatments or medications when an exacerbation is underway, the primary goal of my asthma action plan is simply to avoid a prolonged ICU stay and/or intubation, by seeking emergency medical care at the first sign of trouble. The rationale being, that the faster I go to the hospital for treatment, the better the chance I’ll have of quelling exacerbation before its gets out totally out of control
You can view my actual asthma action plan here.

Poked and Prodded :
Diagnostic studies I’ve had done in recent years include;

* Bronchoscopy ( SARP Study) Results: Severe scarring, which probably extends out to the smaller airways. View the inside of my airways.
* Lung biopsy(SARP Study)Results: View the actual slides and report from my biopsies. as interpreted by Dr Wenzel and SARP.
* Esophageal Ph monitoring test to rule out GERD . Results: negative
* 6 minutes walk test. Results:2400 feet ( more than anyone on record at UCSF with equivalent lung function)
* Exercise Stress test to differentiate exercise induced exercise bronchospasm vs dynamic air trapping. Results :inconclusive.

* Regular CT scans of the Chest. Results:Negative
* Computerized Quantitative CT scans of the Chest. Results: Marked Air-trapping.
* CT of the nasal Sinuses: Results:negative.
* Laryngoscopy to rule out vocal cord dysfunction: Results:negative.
* Alpha1 Anti trypsin levels: Results:normal range
* Echocardiogram to rule out PHT:Results: Mild to Moderate Pulmonary Hypertension
* Sleep study: Results:Normal
* Cardiac Catherization: Results: Negative
* Allergy Skin testing: Results: Severely reactive to tree pollens, grass and dust mites.
* Psychiatric eval : Results: Concluded that I do not have a general anxiety disorder. In fact , they found that I’m unusually stoic during potential respiratory crisis. They do think however, that I probably have some degree of steroid induced psychosis and/or anxiety, which can make my air hunger worse once an exacerbation is underway. They recommended certain medications while on high doses of prednisone . Except for the occasional use of Avitan and certain opiates to help quell the feeling of suffocation, I refuse to take long term mood altering drugs.

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 diminished lung function, and it doesn’t take much to send me over the edge.
Being aware of even subtle changes in my breathing and monitoring my peak flows and FEV1s to validate those changes, is the key to keeping me out of the hospital.

A victim of the 1960s asthma culture and just bad choices I attribute the severity of my disease, at least in part, on plain ole ignorance and the 1960-70s asthma mentality of over-treatment or under treatment, but never preventive treatment.   I came from a very poor , medically indigent family. What little medical care I did receive, came mostly through the social services system in the county where we lived. As a child I survived mostly on toxic, over- the -counter drugs like Primatine Mist, which my friends would occasionally shoplift for me, because my family was too poor to buy them. As a young adult I was prescribed massive quantities of Theophyline ,which was a popular therapy at the time. This guy was actually my doctor at that time.  I vividly recall my heart pounding hard and having tremors for several days after the injections. Needless to say, I wasn’t one of those patients who were supposedly “cured” by the aminophyline injections. I didn’t fair much better with theophyline pills or timed release aminophyline either, as they made me constantly jittery and nauseated.

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 5 years .
At the age of 32, through the hospital where I worked, I was offered the first nicotine patch available by prescription. 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 reaching or even exceeding 80mg 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.) Seriously! ….You haven’t lived, until you’ve experienced full blown steroid induced psychosis! High doses of the stuff can make you feel surreal . More than likely though, you’ll experience bouts of severe 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, and the newer, more powerful steroid inhalers , I was able to gradually wean off the Prednisone. In a period of appx 24 months , I went from 80mg per day to ZERO per day. On 6-1-2007, I was restarted on a small maintenance dose of prednisone.

Today I continue to get my steroid fix through that small maintenance dose of 10mgs prednisone plus 4 puffs of Qvar 80mcgs. ( On 10-1-2009 I began a trial of Ovar brand beclomethasone 80mg x 2 puffs BID .The rationale being, that because of it’s purported smaller particle size, Qvar might offer better distribution to my smaller airways . I will re-evaluate in 3 months.During moderate to severe flare-ups (which occur about every 10-12 weeks), I still load-up on Prednisone at 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 2 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 evidenced on bronchoscopy and 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 19%. 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 only mild hyperinflation and anormal TLC , but quantitative CT scan analysis shows marked peripheral air-trapping. My VC is 68% predicted and my DLCO is in the normal range. And though I desaturate significantly during exacerbations, my oxygen saturations usually return to normal within days of my recovery. I am not a CO2 retainer.

Air-trapping One of the most frustrating and debilitating symptoms associated with severe asthma and/or COPD is something called “air-trapping” It’s the main cause of my dyspnea . I experience some degree of air-trapping symptoms almost all the time and it can be quite uncomfortable. To make things worse, my air-trapping increases proportionally with exertion , which is not a good thing when you’re trying to stay healthy by exercising. 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 training for a marathon), incredibly difficult and sometimes even dangerous. The benefits of daily exercise in the long run however, greatly outweigh the risks.

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 hypersensitive 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 breathlessness associated with air trapping .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. Often made worse by strenuous or prolonged physical activity ( ie marathon walking), the feeling of breathlessness( dyspnea), can come out of nowhere, last from a few days to several weeks, and often , can make me anxious and extremely irritable. The increased anxiety level in turn, can make my breathing even worse , which then snowballs into a vicious cycle.

One of the research projects I completed a couple of years back (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. However, unlike most of the people in that study group who had emphysema, I also have to deal with airways that occasionally constrict and clamp up, sometimes without warning ….a double whammy if you will.

Other than the standard breath control and stress reduction techniques to manage your dyspnea, there remains very few effective remedies. Drugs like ativan can help reduce the anxiety associated with breathlessness in general . Opiates drugs, like Morphine, Fentanly and Methadone seem to work well to quell severe chronic dyspnea, but also carry with them, potential risks ,as they are also very potent respiratory depressants. More recently, a drug called Tiotropium (Spiriva) has been shown to help with breathlessness associated with COPD.
In general though, when I experience symptoms of intense air -trapping, I usually just have to tough it until my lungs decompress to a more normal state, which can sometimes take days.

Medications:
See my “Drug List”
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 .

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 by 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 how “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. Ive also been accused by some pulmonologists of having a dare-devil attitude towards my disease and not taking it seriously.

Exercise: You wouldn’t believe the criticism I get from some people who think I’m actually making myself sicker by exercising.The fact is, if it weren’t for all the exercise and walking I do, I would have died from this disease years ago. As counter intuitive as it may sound , daily exercise is essential for people with all stages of lung disease…including severe asthma! Breathlessness should never be an excuse for not exercising. Is it difficult to exercise when you can’t breath? Of course it is. But with persistence, proper breathing technique and learning to pace yourself, it will eventually become easier. Daily exercise can actually reduces ones breathlessness by re-conditioning the respiratory muscles and makes them function better. In fact, if you don’t exercise, your lung function will decline at an even faster rate.

Still, and despite the fact that I have slowly conditioned myself to walk marathon distances, my exercise tolerance varies sharply from day to day. That’s just the nature of this disease. 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 some of the elasticity of my small airways, so I tend to air-trap (air can get in, but not out.)Exercise seems to make this air trapping worse …a catch 22….. but a necessary one!

Having said all that, the severity of my asthma MAY be one of the reasons that I actually CAN walk marathons. My Pulmonologist, Sally Wenzel, believes that because I have lost most of the “twitchyiness” and now have stiffer airways due to scarring, that the wild up and down swings usually seen in people with highly reactive airways, is probably a little less in someone like me. I am basically “Bad” ALL of the time! …which is probably easier for my body to adapt to (or something like that).

Prognosis: In October of 2004 after surviving a near fatal exacerbation , my partner was told I had less than a 50/50 chance of surviving another 2 years… that was 5 years ago!
There’s not much data on people who , despite having very severe asthma, are able to function as well as I do. The good news, is that even though I have a low baseline FEV1, my BODE index rating is actually quite good ( 2-3 on the scale). I’m also somewhat of a medical oddity, in that despite my limited lung function , I have an enormous capacity for physical exertion. In previous research studies, I was able to walk 5 times farther during a 6 minute walk test, than other people with equivalent lung function.

Personally I don’t think it’s such a big mystery as to why Ive lived so long. I’m pretty sure it’s because of all walking and exercise I do. If you look at all the survival predictors for people with severe lung disease, it’s the people who are still able to exercise, are able to keep their weight stable, and who have a relatively good quality of life, that seem to live the longest. That….plus maybe good genetics. My lungs might be trashed, but the rest of me is still in really good shape.Many people say I look much younger than my 55 years. I’m probably luckier than most, because Ive learned to adapt to my physical limitations. I’m also very privileged to have one of the best severe asthma doctors in the world ( Sally Wenzel) studying my case.

Treatment Options: As long as you’re alive, there is always hope I guess. But right now my treatment options are far and few between. Because my asthma is so severe, Im considered too high risk for even experimental treatments.
On 11-20-2009 after completing the SARP study, it was recommended that I seriously consider lung transplant surgery as a treatment option. As of this writing, I’m actually still too healthy to be accepted as a transplant candidate at most transplant centers. If and when the time comes that I do meet all the criteria, I don’t think this is an option I will pursue.
There are however, a couple new promising drugs in the pipeline that might help people like me who have the non-eosinophilic form of severe asthma. I hope to be a research guinea pig for these news drugs ( in about 2 years) if they make it to the clinical trials phase.

Finally, THE DISCLAIMER: So that I can have a clean conscience.
While I’ve achieved some pretty amazing results by trying to stay physically active, every marathon or half marathon walk that I do, 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 putting my life at risk.
Any advice I provide on my site, is meant to complement and not replace any advice or information given to you by your personal doctor. Daily exercise is crucial for people with any form of lung disease! Just be careful. Don’t over do It, and ALWAYS consult with your Physician before doing crazy stuff like I do. Take an active roll in the treatment of your disease and never give up hope.

BREATH EASY!

(Curious to see how I really feel about this wonderful disease ??)