Things seem to be changing at a lightening pace now and it’s making my head explode. Too much for an over-thinker like me to think about. Gotta slow down and literally catch my breath.
So my 3rd hospitalization and 5th intubation in just the first 4 months of this year, seems to have caught the attention of more and more of the medical center’s Pulmonologists. They’re perplexed over the rise in the number of exacerbations Ive been having recently are trying to find ways to fix it.
Because I don’t always display typical asthma symptoms when I flare, some of my lung doctors even think that my disease might be more akin to a type of obstructive bronchiolitis, in which the small airways become scarred an narrowed, than that of simple asthma. There’s no question that I have asthma, but it seems to have morphed into something much more complicated, making it difficult to identify and treat.
In any case, a lot opinions are starting to come forth on how best to treat certain aspects of this apparent hybrid disease of mine. Some of which we discussed as recently as last week when I was still in the hospital. Below are a few of the problems we identified and the potential treatment options to address those problems.
Problem #1 Intubation) Let me preface this by saying, that I believe the extraordinary number of intubations Ive had is at least partly due to an over abundance of caution by some doctors. In the age of instant medical record access, my reputation as an intubation prone asthmatic precedes me, so that when I show up in the ER in distress, people sometimes jump the gun and I end up tubed maybe a little sooner that I should have. In any case, because Im intubated so often and because Im extremely hard to sedate, I sometimes suffer from the mental trauma of being totally awake during the intubation induction process. It’s an absolutely terrifying and often painful experience, because your muscles are paralyzed, but you’re wide awake in every other respect. Ive written about this topic extensively, so I’ll just leave it there.
Treatment Option: They’re recommending I get an elective tracheostomy to prevent the need for endotracheal intubations. If I need to be on bipap or hooked up to a ventilator they could attach it directly to the trach tube without needing to paralyze me in order to put a tube down my throat. The down side to having a trach, is that there’s a lot of maintenance required to keep the area free of infection. It’s hard to cough up secretions through a trach tube, so you usually need to be suctioned. It’s also easy to aspirate stuff into your lungs when you’re eating. My experience as an RT and working with trach patients over the years, is probably contributing to my reluctance of having something so drastic done, even if it eliminated the need for future intubations.
Problem #2 Air trapping) Because my lungs are hyper inflated all the time, I tend to breath very shallow at maximal expiratory flow rates. If my airways start obstructing, it forces me to breath faster to compensate. This kind of breathing can perpetuate a vicious cycle leading to the inability to move any air at all. The increasing effort required to continue breathing seems to fuel my exacerbations, which then causes my CO2 to rise and my O2 to drop. If it gets bad enough I can’t break the cycle and I end up in the hospital on bipap or a ventilator until things calm down.
Treatment option: They want me to consider going on an anti depressive/anxiety drug which will slow down my breathing chemically and force my body to adapt to higher CO2 levels. In theory this should reduce the air trapping cycle, but in reality I would probably become an abtunded zombie unable to react to my own breathlessness and probably not be able to exercise.
Problem #3 Steroids) As much as I hate the side effect of steroids, particularly the psychological side effects, I definitely breath better when I’m on them. The breakthrough number for me seems to be around 20 mg per day. If I drop below that threshold, within a week or so, my symptoms tend to ramp back up again.
Treatment option: This is one’s a no brainer. They want me to consider staying on a maintenance dose of 20 mg permanently!
Problem #4 Anti-IL drugs) Is kinda related to #3. Long term steroid use is fraught with serious side effects, so while they make breathing easier in the short term, they are potent immunosuppressants and cause damage to other organ systems. One of the side potential sideeffect of long term steroid use…is death. Can’t have a more serious side effect than that.
Treatment Option:There’s a slight chance that one of the new IL blocking drugs could take the place of steroids and improve my PFT numbers, or at least reduce the number of exacerbations I experience. There are several new ones coming out on the market this year, but they were designed to treat a different type of asthma than the one I have. They think my best bet is a drug in the early development phase which blocks IL 33. It has something to do with targeting mast cells, which I seem to have a lot of in my airways. Unfortunately the drug wont be available for at least 3 more years.
Problem #5 Damaged lungs) The bottom line is that my lung function is dropping critically now. I can’t just “think” my way out of it…It’s not a mind over matter thing anymore. No matter how well I take care of myself, how diligent I am about exercising and preventing exacerbations, the damage to my lungs is extensive, irreversible and more than likely will only get worse.
Treatment Option: Because my quality of life is not so great anymore, most of my pulmonologists(I say pulmonologist(s) because I have a whole team of them working with me), believe that lung transplant is the best option for me in the long run. It may only give me a few extra years, but they believe that they would be good quality years. I honestly cannot remember more than a handful of days in my 60 years, where my breathing was normal for a full 24 hour period. Now imagine awaking from a surgery after which you have no more asthma, no more shortness of breath, no more difficulty breathing. It’s absolutely mind blowing.
Never say never, but option #1 and 2 are non-starters, they’re just too radical for me.
So, out of all these treatment options, 3, 4 and 5 (in that order) are the ones Im following through on.
The steroid taper that Im currently on will only drop to 10mg this time instead of zero. I will stay on that dose for a few months to see if it makes a difference. As far as the new biologics like dupilumab, Im already on the compassionate use radar. The drug company involved is trying to expedite FDA approval to get me the drug early, but of course there are always snags.
Regarding lung transplant, my sentiments haven’t changed much on that topic. It’s a treatment of last resort. I don’t want it unless Im reasonably certain that Im gonna die soon without it. But if transplant becomes the only path left to extend my life, I will embrace it wholeheartedly.
As far as short term changes, there are some of those too. Last week we came up with a better system to keep my doctors informed about sudden changes in my health or flare ups. In the past this has been a challenge because the Pulmonoogists who follow me are also researchers who are extremely busy and travel a lot. My updated asthma asthma action plan now includes calling or emailing not just one, but all three of my primary Pulmonologists , so that at least one of them will get back to me. The hope is that by making contact with one of my doctors early on, that we might prevent an exacerbation from turning into a huge ordeal. They also wanted me to know that emailing them, even with a low priority question, was not an annoyance to them. I thought that was nice of them to say that.
Hey, they’re pretty cool people. They have to be, to be able to put up with a patient like me.