In 2024, 33,989 people with cystic fibrosis resided in the US. Moreover, of those 33,989 the CF Patient Registry states:
“Notably, 10.0 percent of people in the Registry are now between the ages of 40 and 49, and 9.0 percent are aged 50 or older. This trend reflects increased longevity among people with CF, highlighting progress in treatment and care over the past several decades.”
This statistic is phenomenal. It’s absolutely wonderful and yet, there is a notable loss.
Let’s break this down:
If I was to travel back in time to grade school, and I am in a class of 30 CF children, 10% of those children would reach my age. That’s 3 children.
I have lived and been acutely aware of this absence all my life. For years, I never had words for it, just a vacancy in and around me. A hush, looks, disappearance children and families.
After many years of reading and researching and trying to figure this awareness or feeling of loss or vacancy. My logical mind today says – I found it.
On the flip side, the stats used to be so much worse.
Building onto this, I am unbelievably blessed, thankful, mixed with a straight up feeling of disbelief.
Why am I even bringing this up?
I went to my CF doctor asking what people with CF are doing today regarding their treatments. If possible, statistically, data proven, there is a way that I could cut down on my daily therapies.
I am not looking to cut back on my daily nebulizer vest or treadmill therapies. I think it would never happen in my lifetime.
I am asking to see if I can somehow cut down on my antibiotic nebulizer therapies that treat my very stubborn pseudomonas aeruginosa.
Her answer in summary:
“Patients are doing all sorts of things. Some are tossing their vest jackets to the side completely. Some are doing it when they feel they need it. Some have cut back slightly but not all. We do not encourage patients to stop altogether. We have found that scarring or reduced lung capacity damage shows up later in years. An area that may not have affected you for 20 years and it suddenly rushes back to be an issue today. The truth is we don’t have the data. You don’t have the data to support where you will be in ten years. What you are doing today is not harmful and in fact could definitely help you, as it has been done for years. You do have slight sensitives to Tobi but just slight.[1]
What does this mean?
While a large sector of the CF population is throwing caution to the wind. I can see that if I was twenty years old and perhaps didn’t have a lot of scaring. Am I doing a ridiculous number of therapies? I feel good and can operate at a high level. But could I feel just as good and operate at the same level if I reduced some of my antibiotic treatments?
What are my choices?
- Keep doing what I am doing. My regular nebs followed by Tobi inhalation or Cayston inhalation. Tobi is 28 days, Cayston 28 days. I have been flip flopping these two meds back and forth for 12 months a year since 2016. I barely take a day off, and it discouraged to take time off to keep consistent treatment of pseudomonas aeruginosa. I have had these bacteria probably in some number, on a cellular level, since I was at least three years old. Sometimes I do not culture it, but it is presumed it is lurking.
- Rollback to what I did in my younger years. Stop altogether of the back-and-forth continuous use. Only treat with antibiotic inhalation when I actually feel unwell or my culture demonstrates I should. Then chose what med to do, Tobi or Cayston.
- Do one med every other month and choose Tobi OR Cayston.
Tobi: twice a day and take a minimum of twenty minutes each time. Must be refrigerated and best to do a different neb cup, not to mix. Tobi is more of an irritant to my lungs. Meaning I cough more and it is more annoying.
Cayston: 3 times a day and takes 3 minutes each time. Goal 4 hours a part and must do the 3rd because the medication efficacy is not there with 2 doses. A whole new med cup, neb machine, and batteries are needed. The cleaning is more extensive requires a two-step process. Cayston is smoother and I cough less.
- Do both Tobi and Cayston but at two-week intervals vs. four-week. The time frame for antibiotics was somewhat arbitrary. They had to create a time interval to give the body a break from the antibiotic use and 28 days seemed logical given the structure of months.
- Leave antibiotics at home when traveling, “if you feel okay.” This would reduce the labor. In a future post, I will detail my new travel gear and tips.
Sidebar:
Because Tobi is more of an irritant for me, maybe that would be nice going back and forth every-two weeks or may make me crazy. Neb cups coming out of my ears and keeping track of my rotating supply.
Tobi has been around since 1999 or 2000 and is a gold-standard medication. It was a breakthrough med that had a targeted bacterial treatment vs. playing Wack-a-mole with Ceftin, amoxicillin, alike. All of which I cannot take anymore.
Cayston has been since 2010 and has been recognized and considered very effective medicine to treat pseudomonas aeruginosa.
I also culture another bacterium fairly regularly that has no good treatment option for me. However, I culture it lower grades and more infrequent as pseudomonas aeruginosa. My treating pseudomonas aeruginosa just overall helps the lungs fight off that particular bacterium. A little bit twofer but not exactly.
So, which way to roll the dice?
To be determined. I have a hunch but unsure at this moment.
As I have said before, cystic fibrosis asks so much from a person. I have been doing all of this all my life really. When I started the on and off antibiotic use in 2016, I thought, this may put me over. We do as we do, and it didn’t. But it has compressed my freedom in the short-term, but has expanded my life in the long-term. Although I don’t have data on that for sure lol.
I am the data, I am living it in real-time data and there are no clear-cut answers. No predictors.[2]
The best part of this is that I have choices unlike prior to Tobi 1999/2000 mark, or Trikafta.
So, there you are friends. It’s up to me but it’s also up to you.
What do you want in your own life?
How do you want to feel, live in ten, twenty years? What does that look like in real-time? What do you have to do today to be that person? What do you want to do as the years click on?
I am constantly exercising my will and strength and knowing this is the key to life. My stamina and endurance are dependent on my will and strength. The stronger I am, I hope on the cellular level it aids or eases that challenge.
They say living well is the best revenge.
I’d also say, living well is the best gift to yourself. A sweet taste of honey that lingers long after.
[1] Tobi is an inhalation antibiotic and does not pass through the liver. The tendency to build resistance to it and much, much lower than oral or IV.
[2] All my data, every bit of it, goes to Bethesda, Maryland to the headquarters of the cystic fibrosis foundation for research.