My Asthma

(This page was last updated on 12-30-2017)

Yup, my real name is Stephen and my asthma is about as bad as it gets. We’re talking off the charts..bad!

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 referred to as Severe refractory asthma with fixed obstruction. “Refractory” means that my asthma symptoms don’t improve much even after maximum treatment. “Fixed obstruction” means that both my larger and smaller airways are damaged in a way that makes them less responsive to the usual asthma medications. To complicate matters there’s been suggestion that I might also have a condition known as Constrictive Bronchiolitis Obliterons. The term Bronchiolitis Obliterons (BO for short), refers to non-specific inflammation and fibrosis of the tiniest airways, for which there is no effective treatment available.

In the simplest terms, I have a type of asthma that is not well understood and which responds poorly to current therapies. Despite doing everything possible to keep my symptoms in check, Im short of breath do some agree all the time. Quite often my symptoms escalate into full blown, life threatening, exacerbations called “status asthmaticus” which lands me in the hospital. Essentially, my life is a continuous cycle of exacerbations and recovery.

UCSF Airway Clinical Research Center 2018

It’s been difficult for researchers to identify a specific phenotype for my variety of asthma, as I don’t have the typical markers that indicate type TH2 inflammation ie, high blood or sputum eosinophils. However, I do have an extraordinary amount of mast cells in my airways, which leads them to believe I do have inflammatory driven asthma, but through a different immunologic pathway.

Over the span of 6 decades, I’ve endured countless days on ventilators, Bi-Pap machines, Heliox and continuous nebs. Excluding bronchial thermosplasty, which would probably kill me, Ive had just about every medical treatment available for asthma. On 12/21/2017 I racked up my 134th hospital admission for this disease. Ive been intubated and placed on a ventilator a ridiculous 38-39 times for respiratory failure, and one time for cardio-pulmonary arrest requiring CPR.

Dr Sally Wenzel and myself
Despite everything Ive been through, I consider myself luckier than some in that after 63 years Im still around to write about it. I’m also privileged to have some of the best asthma doctors in the world involved in my medical care, including Dr Sally Wenzel, Stephen Lazarus, John Fahy and Michael Peters. If you have difficult or severe asthma, you definitely want these doctors in your corner. They’re pretty awesome.

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. Sometimes my symptoms will gradually get worse over the course of several days and then spiral out of control. Other times my lungs can clamp up at the drop of hat without any warning at all. Sometimes my flare-ups are precipitated by a 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.

Surviving a life threatening asthma exacerbation is one thing, recovering from one is another story. It can take weeks and the drugs that keep flares at bay can reek havoc on your body. Amazingly, and despite the sure number attacks Ive endured throughout my life, I somehow manage to spring back much faster than most. So every time I get through one of these bouts, I get excited as hell on discharge day.

My discharge face

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 or nuisance disease. Unless you were turning blue or stopped breathing all together, chances are no one would take you seriously.
Now, after a lifetime of frequent and often severe exacerbations, my airways have undergone what is known as remodeling. What began as garden variety allergic type childhood asthma, continued to get worse, which gradually morphed into a form of obstructive lung disease now known as ASTHMA/COPD OVERLAP SYNDROME (ASOS). According to the latest research, it’s unclear as to whether the process of “remodeling” can be reversed. While there’s some evidence to suggest it might be partially reversed in mild asthma, the opposite is probably true for those with more severe disease.

As a result of this “remodeling” and scarring, my larger airways seem to have lost most of their twitchiness usually seen in people with brittle asthma, so I often don’t wheeze when Im tight. If that weren’t bad enough, my smaller airways have undergone similar changes, but in a different form referred to as “Bronchiolitis Obliterans”. “Bronchiolitis” means inflammation of the bronchioles ( bronchioles are the smaller airways). “Obliterans” means that the inflammation and /or fibrosis has caused irreversible airway obstruction in the affected airways. As a result they’ve become so stiff and narrowed, that just the slightest spasm and/or inflammation and/or mucus in those tiny air passages can cause them to completely close up, which is probably why I get so sick. Additionally there’s the problem of constant air-trapping( more about that later), that often makes me feel like I’m suffocating, even though my O2 sats might be normal.

UCSF therapy dog

Pulmonary Functions: currently my FEV1 runs 20-30 %, and that’s on a good day. MY FEV25-75 is in the teens and single digits. Up until late 2014 I was able to sometimes reverse all the way up to 50% after a neb treatment, but unfortunately those reversals only last a couple hours and then my FEV1 plummets again. My FVC runs 50-60%. My baseline peak flow usually hovers between 210-260 using an electronic PF meter.My expiratory reserve volume is about 200% with a normal TLC . Quantitative CT scan analysis shows marked peripheral air-trapping. My DLCO is in the normal range. And though I desaturate during severe exacerbations, my oxygen saturations usually return to normal within a few days of my recovery. I am NOT a CO2 retainer.

Poked and Prodded :
Other tests I’ve had done in recent years include;
* Pulmonary Function Tests
* Ventilation Perfusion Scan 2016
* Right and Left Heart Catherization 2016
* Bronchoscopy ( SARP Studies in 2009,2014) Results: Lung scarring.
* Lung biopsy(SARP Study 2009,2014)
* Esophageal Ph monitoring test to rule out GERD . Results: negative
* Genetic testing for the Beta Adrenergic Receptor Genotype. Results: Negative {hetereozygous Gly /Arg}
* 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.
* CT of Chest. Results:Negative. No Tracheal Malacia, No signs of Emphysema.
* Computerized Quantitative CT of the Chest. Results: Marked Air-trapping.
* CT of nasal Sinuses: Results:negative.
* Laryngoscopy to rule out vocal cord dysfunction VCD): 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)
* 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 only spike up once in a while.
* CRP level, high sensitivity (2016):Results:High

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. I can vividly remember an occasion when was I 10 years old crying because I couldnt breath and my mother telling me, “don’t be silly Stephen, nobody ever dies from asthma.” (Actually, 5,000 people DID die of asthma that year). As a teenager 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 can 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 theophylline pills or timed release theophilline either, as they made me constantly jittery and nauseated.

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, Dilaudid 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…. 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 wean 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 .

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 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! 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!
Today I continue to get my daily steroid fix through the inhaled variety along with a daily 5-10 mg maintenance dose of prednisone. During moderate to severe flare-ups 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 dying.

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. The fact is, I work exceedingly hard at projecting an outwardly healthy appearance. I hate to think of myself as a sick or disabled person. 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 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).

Long Term Outlook: 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 13 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 my body is still in pretty good shape. Many people say I look younger than my 63 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 : As long as you’re alive, there is always hope. But right now my treatment options are pretty limited. There are however a couple new biologic drugs on the not too distant horizon, which might offer some hope. In Nov of 2017 I began receiving an investigational biologic drug called “Dupilumab” on a compassionate use basis from the drug manufacturer, Sanofi. As of this writing it’s too soon to tell if the medication is helping, but Im cautiously optimistic.

As a last resort, my doctors suggested that I consider Lung transplant as an option. The discussion began during my participation in the SARP study at the University of Pittsburgh in 2010. It was recommended that I undergo evaluation for 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 not to proceed. 4 years later in 2016, because of a continuing decline in my lung function, quality of life and 14 more intubations, we once again initiated the evaluation process, this time at UCSF where my Pulmonologists could follow me closely. In early 2017 I completed the full evaluation, but because of my asthma diagnosis, a low allocation score and the potentially long wait, I withdrew from the program.

Asthma Action Plan:: An AAP is a written set of instructions that you and your doctor put together that lists what you should do, step by step, if your asthma starting flaring up. The primary goal being to keep your asthma in check and keep you out of the hospital. Everyone’s plan is a little different. Here’s mine:

Because I have very little reserve lung function, my symptoms tend to escalate rapidly if not treated aggressively right away. So my action plan only has 3 components.

Notifying my doctors
Tweaking my current therapy
Assess the situation and get your butt to the hospital if needed

It works like this…………
At the first hint that my breathing is getting worse, I initiate an email chain to all 5 of my pulmonologists to keep them in the loop of what’s going on. Emailing all 5 of them assures that at least one of them will get the alert and respond. Step #2 is to bolus or increase my steroid dose along with the frequency of neb treatments. The last component of my plan is assessment. If after 24 hours (or sometimes much sooner) Im not breathing better, or my PFs are super low, I head directly to the ER. No stopping, no passing GO, just straight to the hospital. If I don’t procrastinate for too long and head to the ER right away, I can sometimes avoid intubation.

(If you don’t already have one, here’s an asthma action plan template that you can download.)

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!