This blog post was written by Mary Kitlowski, founder of Running On Air, who is living with Bronchiectasis.
After years of searching for answers to my health problems, my first real answer came from the diagnosis of bronchiectasis when I was 17 years old. Back in the 70’s and early 80’s the convention was to stay away from too many x-rays. My first chest x-ray revealed a piece to the puzzle that would eventually become my diagnosis of Primary Ciliary Dyskinesia (PCD).
I’ve always considered myself a PCD patient first. Bronchiectasis was just one of the symptoms of having PCD. It can be hard for me to separate out what is bronchiectasis and what is PCD. For example, I believe most bronchiectasis patients have a cough. PCD patients have a chronic cough too. Since our cilia doesn’t move properly, coughing is the only way we can clear mucous, bacterial and other irritants out of our lungs. I’ve been coughing almost from birth, most likely before bronchiectasis developed.
And again, it’s hard for me tell what is PCD related, bronchiectasis related or a combination of the two. All I can speak to is my own symptoms.
With 41% lung capacity (FEV1), almost every breath is a reminder for me that breathing is an effort. I describe it as varying degrees of breathing through a straw. I feel like I’m always breathing through some kind of straw. Sometimes it is one of those wide straws that you can slurp a thick shake through. Other times, it is one of those small straws that a grain of salt can get stuck in.
I never know which straw it will be from day to day. Or even throughout the day. I might wake up feeling great and then by the time I head out for the gym I feel like I’m wheezing loud enough for the person on the treadmill next to me to hear.
As I stated above, I’ve always had a cough. This, in fact, was one of the first things my allergist asked me about when I was diagnosed with bronchiectasis. He said, “I always ask you when you come in if your coughing any more than usual.” My response was, “I’m not. This how much I always cough.” To me coughing is just something I’ve always done. It has gotten worse over the years.
There are certain times of the year that I seem to cough more. I attribute some of this (rightly or not) to post nasal drip. And even though sinus issues are not bronchiectasis related, the additional mucous adding to already stressed and infected airways does not help.
The majority of the time my cough is productive. For people new to bronchiectasis or who may be more squeamish with medical issues, this might seem gross. Unfortunately though it is a fact of life for me. Believe me, I’d rather have healthy lungs that didn’t produce so much mucous. The reality though is that I cough up phlegm throughout the day. I try to be discreet, but since it is a factor as to how I feel and whether I get sick, I have long ago let go of being embarrassed.
I feel that society makes matters worse for those of us with chronic coughs by depicting coughers as annoyances in advertising. I get that you don’t want to sit through a movie next to someone coughing up a lung the entire time, and yet some of us don’t have a choice in being completely cough free for two or more hours.
There are some days when I can’t leave the house because of how much I’m coughing. I start having bronchospasms and I can go for thirty-plus minutes with non-stop coughing and barely being able to get a breath in. I’ve had to leave meetings because these attacks or spasms start up. I usually can’t tell when they will happen.
I always cough when I change head positions, as well as when I first wake up in the morning and when I go to bed at night. I also cough at first during my dental visits, when the chair is tilted back. I need a minute or two to adjust, and then I’m usually OK. When I first lie down at night, just a few coughs to clear out my lungs are enough. About 60 days out of the year though, I am coughing so much I need to leave my bed, so as not to keep my husband awake, and sleep elsewhere.
During these episodes though, the bronchospasms are such that it takes a little while to settle down, sometimes as much as an hour or two. The affect starts to become cumulative, creating a downward spiral. The coughing is preventing me from getting a good night’s sleep. Being sleep deprived can trigger more coughing. Being sleep deprived makes it difficult to get up the next morning and function at work. Finally, it can create the perfect storm for an infection.
While I take bronchodilators, antihistamines and expectorants (to name a few) in pill form, the bulk of my therapy time when healthy comes from using “high frequency chest wall oscillation technology” (think of wearing a big vest like blood pressure cup that fills up with air and starts vibrating) along with “oscillating positive expiratory pressure” (this is blowing hard into a little hand held device that offers some resistance, making the user cough) while inhaling bronchodilators, steroids and saline through a nebulizer. The process takes 30 minutes at a time and is done twice a day. Sometimes the bronchodilator is done more frequently.
Then, for 28 days, I spend about 5 minutes, three times a day, doing an inhaled antibiotic. There is currently only one antibiotic I can do inhaled as I’ve had a reaction to the other standard one used for treatment. Then I’m off the antibiotic for 28 days.
When I’m having flare-ups though, IV antibiotics are added to my treatments. The quantity of antibiotics and length of treatment will vary based on the severity of the infection and my response to the treatment. I am usually on IV at least once a year.
While supplemental oxygen is not a part of all bronchiectasis patients’ routines, it is becoming more so for me. I have used supplemental oxygen for exertion since March 2014, which for me is when I am exercising. I find though that my oxygen levels are fluctuating more and there are some days when I will wear it when I’m sitting at my desk working.
I also always wear it when I fly.
To me, exercise is one of the most important aspects for keeping my lungs healthy. This is another one of those downward spirals and challenges for those with degenerative lung disease though. Who feels like exercising when they don’t feel well? How do you handle that when you never feel well? We also measure our level of effort by how hard it is to breathe. For many of us with bronchiectasis just walking up a flight of stairs feels hard, let alone exerting effort on some kind of cardio torture device.
If we don’t do this though, our lungs will continue to get worse. Sometimes it’s not about maintaining what we have, but about slowing down the progression so we don’t lose everything we have so quickly. Part of what can encourage people to continue exercising is being able to see the difference or improvement. There is no guarantee that those of us with bronchiectasis will see any improvement; however, it is likely that without exercise there will be a steady decline, until it is too hard to even walk across a room.
With around 200,000 Americans living with non-CF bronchiectasis, there will be wide variations in symptoms and the speed of progression. I'm sure what most of us do have in common is a strong desire to get back to our "old selves", the ones who could breathe easily. Hopefully, one day soon, we will have improved treatments, and eventually, a cure. Until then, I encourage you to talk to your physician about what you can do to slow the progression in your case, and take each day one breath at a time.