(This page was last updated on 3-02-2012)
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 have what they call Severe refractory asthma, which puts me at constant risk for “NFA”
What all those fancy words basically mean, is that despite being on a maximum regime of medications and treatments, I still have symptoms pretty much 24/7 , which occasionally escalate into life threatening exacerbations (known as “status asthmaticus”) . I’m a ticking asthma time bomb and tend to make Emergency room physicians a little nervous when I show up at their front door.
Over the span of 50+ years, I’ve endured countless days and weeks on ventilators, Bi-Pap machines,Heliox and continuous nebs. You name the treatment, Ive had it. In my lifetime Ive made well over a hundred trips to the Emergency room for flare-ups, and on 1/25/2012 I racked up my 104th hospital admission for asthma (since birth). Ive been intubated 18 19 times for respiratory failure , and one time for respiratory arrest requiring CPR. I’ve come closer to receiving a hole in my neck (tracheotomy) than I care to. (Here’s a clip of me weaning off the ventilator) Fortunately, I have one of the worlds best severe asthma specialists and researchers, Dr. Sally Wenzel , following my case, and also a wonderful local pulmonologist overseeing my day to day care.
Exacerbations that land me in the hospital, often come out of the blue with no obvious trigger. Most of the time it starts out as a mild flare-up that kinda waxes and wanes for couple of days and then all of a sudden spirals out of control. Sometimes my flare-ups are precipitated by a cold or the flu and those are the ones that usually make me the sickest. (Thankfully, I don’t get too many colds). Other triggers such as seasonal allergies, changes in the weather, emotional stress, exercising too much or just plain getting run down can set me off.
History:Though I ended this world wheezing ,I wasn’t officially diagnosed until the age of 5. Now, after a lifetime of persistent and frequent exacerbations, my airways have undergone extensive remodeling, which now resemble those of someone with severe COPD and/or fibrosis . What probably began as garden variety asthma when I was a child, somehow got out of control and morphed into severe irreversible lung disease that responds very poorly to treatment. My baseline FEV1 average about 35% , which equates to a “lung age” of about 120 years!
Over the years as my lungs have become stiffer, Ive lost much of the twichyness (bronchospasm)normally seen in asthmatics. But because my airways are so damaged and have become so narrowed, just the slightest spasm and/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 is present all the time and that often makes me feel like I’m suffocating. * Have you seen the TV commercial where the elephant is sitting on the persons chest? That’s what it feels like). The air-trapping also makes my exacerbations more difficult to control.
In 2009 following my participation in the SARP study at the University of Pittsburgh, it was recommended that I consider lung transplant surgery. As of 3/2012 I have decided not to pursue that option, but have agreed to at least be evaluated.
Asthma action Plan:: For most asthmatics the purpose of an asthma action plan, is to provide them with a set of detailed written instructions, so that they know what steps to take when their asthma flares up. The main goal is to keep one’s asthma under control so that you can avoid a trip to the Emergency room.
Because I respond very poorly to at-home treatments once an exacerbation is underway, and because my symptoms tend to escalate very rapidly, my asthma action plan directs me to go to the Emergency Room without procrastinating or trying to tough it by myself. The rationale being, that the sooner I get to the hospital for treatment, the better the chance I’ll have of quelling the exacerbation before it turns into a life threatening situation.
If you don’t already have one, here’s an asthma action plan template that you can download. 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.
* Genetic testing for the Beta Adrenergic Receptor Genotype. Results: Negative {hetereozygous Gly /Arg}
* 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 : The Psychiatrists 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 2 puffs of Advair 550/50 and 2 puffs QVAR ( Qvar has a purported smaller particle size, which might offer better distribution to my smaller airways). 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 39 % , during exacerbations it can drop as low as 12%. 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 a normal 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” …aka lung hyperinflation. It’s believed that my air trapping is caused by the loss of elastic recoil of my lungs tissue, as well as from a lack of alveolar attachments ( fibers that pull the alveoli open from the outside, preventing them from collapsing ). Air-trapping is the main cause of my chronic 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 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 and air-trapping.
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 fact that Ive had severe life long breathing problems may actually be one of the reasons that I actually CAN walk marathons. Dr Wenzel, believes that because I have lost most of the “twitchiness” 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 less in someone like me. In other words, I’m 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, I was told I had less than a 50/50 chance of surviving another 2 years… that conversation took place almost 7 years ago! So much for guessing how long a severe asthmatic will live.
There’s little or no data yet on the mortality rate of chronic severe asthmatics, so Im not sure what they based that earlier prognosis on. 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 my longevity is such a mystery . 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 obstructive lung disease in general, 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 DNA. 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 57 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 researchers 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, I’m considered too high risk for even the most experimental of treatments.There are a couple of new promising drugs in the pipeline for severe asthma, but it’s doubtful that I would benefit from any new “pill”.
Lung transplant is another option, but I’m not totally convinced that it would make my life any better in the short term, so for the time being I’m not interested. Though there are exceptions, the 3 survival rate is still only about %60 and the complications and life style changes are numerous. To my knowledge there have only been a handful of asthmatics ever transplanted and most of them survived less than 2 years. I’ll cross that bridge, if and when I get there.
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 ??)
