My Asthma

(This page was last updated on 11-20-2015)

Vent 3

Yup, my real name is Stephen and my asthma is about as bad as it gets. The type of asthma I have is much different than what most people think of when they hear the word asthma.

I have what is generally referred to as Refractory Asthma. Or more specifically in the new age of severe asthma phenotyping, as “long duration Th2 low/mast cell high with remodeling” asthma.

What that basically means, is that I have a type of asthma which responds very poorly to treatment. Despite being on a maximum regimen of medications and treatments, Im short of breath almost all the time, and several times a year my symptoms escalate into life threatening exacerbations called “Status Asthmaticus” As a consequence, my quality of life is greatly impacted.

Over the span of 6 decades, I’ve endured countless days on ventilators, Bi-Pap machines, Heliox and continuous nebs. You name the treatment, Ive had it. On 11/01/2015 I racked up my 122nd hospital admission for asthma. Ive been intubated 23-24 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.
Fortunately, I’m privileged to have some of the best asthma doctors in the world involved in my care, including Dr. Sally Wenzel and John Fahy

Exacerbations( flare-ups, attacks, whatever you want to call them) that make me sick enough to require hospitalization, often come out of the blue with no obvious triggers. Most of the time it starts out as a nagging or mild increase in symptoms that might wax and wane in intensity for a couple of days, and then spirals totally out of control. Sometimes my flare-ups are precipitated by a chest cold or some type of flu, and those are the ones that usually make me the sickest. Other triggers such as indoor and outdoor allergies, changes in the weather, emotional stress, exercising too much or just plain getting run down can set me off.

Recovering from these severe exacerbations can often be a difficult and lengthy process, which only gets worse with each hospitalization.

Remodeling:Though I entered this world wheezing, I wasn’t officially diagnosed with asthma until the age of 6. At that time asthma was considered mostly a psychosomatic illness. Unless you were turning blue and not breathing at all, chances are no one would take you seriously.
Now, after a lifetime of persistent and frequent exacerbations, my airways have undergone what is known as remodeling. What began as garden variety allergic type childhood asthma, continued to get worse and morphed into a severe form of obstructive lung disease now known as Asthma- COPD OVERLAP SYNDROME. According to the latest research, it’s unclear as to whether the process of “remodeling” can be reversed. While there’s some evidence that it might be partially reversed in mild asthma, the opposite is probably true for those with severe disease.

As a result of this “remodeling” and scarring, my larger airways seem to have lost most of their hypersensitivity usually seen in people with touchy asthma, so I often don’t wheeze when Im tight. However, this same remodeling and scarring has caused the smaller airways in my lungs to become so stiff and narrowed, that just the slightest spasm and/or inflammation can cause them to completely close up, which is probably why I get so sick. I also have some bronchial malacia which causes my airways to close up or collapse more than they should when I exhale. Additionally there’s the problem of constant air-trapping( more about that later), that often makes me feel like I’m suffocating.

Asthma action Plan::The purpose of an asthma action plan, is to provide a set of agreed upon instructions on what to do when your asthma flares up. The primary goal being to keep you out of the hospital.
Because I respond poorly to treatment, and because my symptoms tend to escalate if not treated aggressively, my action plan calls for me to head to the Emergency Room sooner rather than later. The rationale being that the sooner I get to the hospital, the better the chance I’ll have of quelling the exacerbation before it turns into a big ordeal.
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;
* Spirometry (PFTs)

* Bronchoscopy ( SARP Studies in 2009,2014) Results: Lung scarring, and bronchial malacia.
* Lung biopsy(SARP Study 2009,2014)
* Genetic testing for the Beta Adrenergic Receptor Genotype. Results: Negative {hetereozygous Gly /Arg}
* Esophageal Ph monitoring test to rule out GERD . Results: negative
* 6 minute 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 Pulmonary Hypertension
* Sleep study: Results:Severe Obstructive Sleep Apnea (34 hyponea events per hour)
* Cardiac Catherization: Results: Negative ( as of 2003)
* Allergy Skin testing: Results: Severely reactive to tree pollens,dust mites and the usual asthma trigger suspects.
* Eosinophil levels. They were super high when I was young, now they’re super low.

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 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 as a child came mostly through the social services system in the county where we lived. As a teenage I treated my almost daily chest tightness and wheezing with over- the -counter drugs like Primatine Mist, which my friends would 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.

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 60mg 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 12 months , I went from 60mg per day to ZERO per day. On 6-1-2007, I was restarted on a small maintenance dose of prednisone and continued with that for just over a year. In early 2010 I stopped the daily maintenance dose.

Today I continue to get my daily steroid fix through the inhaled variety. Ive been on most of them at one time or another. During moderate to severe flare-ups (which occur about every 10-12 weeks), I still have to load-up on Prednisone, 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.

Pulmonary Functions: On a good day my FEV1 runs 25-35 %. My are FEV25-75 is in the teens and single digits. Amazingly, I can sometimes reverse all the way up to 50% after maximum bronchodilators and prednisone, but unfortunately those reversals are very short lived.. a few hours tops.My FVC is 50-60%. My baseline peak flow usually hovers between 290-330 using an electronic PF meter. Volume studies show only mild hyperinflation and a normal TLC , but quantitative CT scan analysis shows marked peripheral air-trapping. My DLCO is in the normal range. And though I desaturate during exacerbations, my oxygen saturations usually return to normal within a few 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 a combination of mucus plugging and 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 effective minute ventilation, which causes more air -trapping and makes 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. At least in my opinion.

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 In my case, air trapping is thought to be caused my micro mucus plugs(common in severe asthmatics) that block the inside of the smaller airways, allowing air to enter, but not exit. Though not life threatening in and of itself, air trapping can be 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.

Other than standard breath control and stress reduction techniques to manage your dyspnea, there remains very few effective remedies for breathlessness in general. Drugs like ativan can help reduce the anxiety associated with breathlessness in general but they have side effects. Opiates drugs, like Morphine, Fentanyl and Vicodin seem to work well to quell severe chronic refractory dyspnea, but also carry with them negative side effects. As one of my Physicians put it…. these short acting opiates are clock watching drugs. You find yourself constantly checking the clock to see if it’s time for the next dose. Longer acting opiates like methadone are probably better, but are extremely physically addictive and difficult to ean off.

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 .

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 . 

Having said all that, the fact that Ive had 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” in my large airways, 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… It’s now been 11 years since that conversation took place ! So, if someone tells you that you have “end stage asthma or end stage COPD”, it doesn’t mean you’re gonna die soon. It just means that there’s not a lot that can be done to reverse the damage. The trick is to keep things from getting worse.
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 61 years. I’m probably luckier than most, because Ive learned to adapt to my physical limitations and to some degree, the discomfort of breathlessness.

Treatment Options/Lung Transplant?: As long as you’re alive, there is always hope. 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 or clinical trials. There are a couple of new promising drugs in the pipeline for severe asthma, but it’s doubtful that I would benefit from any of them.

Following my participation in the SARP study at the University of Pittsburgh in 2009, it was recommended that I undergo evaluation for lung transplant. I was eventually evaluated at Stanford in 2012, but because I had not suffered a near fatal exacerbation in the preceding 12 months, and because of limited experience in transplanting asthmatics, they felt it better to hold off. In late January 2014, I participated in SARP III where the topic of transplant came up again. The plan was to be re-evaluated, this time at the University of Pittsburgh where they do higher risk transplants. However, now that Ive reached the age of 60, Ive decided not to pursue this path further. Though I spend a lot of time in hospitals and suffer a lot from the effects of this disease, Ive managed to survive a long time and I still enjoy a relatively good quality of life. Better to save those precious donor lungs for people less fortunate.

While I’ve achieved some pretty amazing results by trying to stay physically active, every marathon or long distance 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 in the process and putting my life at risk.
Even though Im a Registered Respiratory Therapist, 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 taking on a new exercise program. Educate yourself and take an active roll in the treatment of your disease and never give up hope!


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