CF Happens

DISCLAIMER: This ended up being a longer post than I anticipated. I elaborated on quite a bit of things, threw in some educational stuff and answered some questions that I’m commonly asked.

Welp, I caved and asked my CF doctor for prednisone. You know my asthma is really bothering me if I’m asking for prednisone. Ugh. Me taking prednisone isn’t ideal in the big picture.

One of the nurse practitioners (NP) called in the prednisone. My doctor may be off for spring break with his kids. It never fails that he’s gone when I need him..

Unfortunately, prednisone doesn’t work the wonders it once did and it takes much higher doses and a much longer taper than it used it, if it works at all.

I’ve tried to push through it but I can’t do it anymore. My lungs are very tight and breathing treatments are not giving me any relief. I’m unable to do simple things like walking across the house from one side to the other without getting out of breath. Therefore, doing any house chores, or much of anything really, is ridiculous.

I also did my home spirometry and my FEV1 is down to 27% which I very much expected.

Unfortunately, on top of CF I have really bad asthma AND bronchiectasis. The “cystic” part of CF refers to the formation of cysts in the lungs (as well as the pancreas). The “fibrosis” part of CF refers to the scarring and thickening of tissue in the lungs (as well as the pancreas). Asthma causes airway inflammation and narrowing, while bronchiectasis involves permanent damage and abnormal widening (dilation) of the airways. Bronchiectasis is irreversible because the damage to the airways is permanent. I always say that it seems very counterproductive having all of that going on in these little ol’ lungs of mine. Below I added a graphic showing the difference in symptoms for asthma and bronchiectasis.

I basically have bronchiectasis because of CF from repeat lung infections. Bronchiectasis makes it really hard to clear the thick mucus that my CF body produces constantly. Since I’m not able to clear the thick mucus it sits in my lungs and breeds infection. Thanks to CF, I grow pseudomonas ariginosa in my lungs all the time. There’s no way to ever clear my lungs of all the mucus therefore I’m unable to rid my lungs of pseudomonas.

Many people ask me if the doctors can just go in and suck it out (a bronchoscope). I sure wish! Unfortunately, that’s not an option. My body is constantly producing more mucus so it would be a never ending process. The times I have had a bronchoscope, it really pissed off my lungs causing major bronchospasms which is when the muscles that line the lungs’ airways tighten and make breathing difficult. I get bronchospasms with some of the allergic/adverse reactions to different medicines I’ve taken as well.

When I had my first bronch back in 2008, I needed to be intubated at the end of it and be on the ventilator for about 12 hours. Putting me on the vent was to try and get my lungs to calm down enough from the bronch that really pissed off my lungs.

Yes, I was asleep during the bronch so I didn’t know I was being put on the vent at the time. I became slightly aware I was on the vent while I was still intubated in my ICU room because they had lightened my sedation a bit and I started to thrash around, “like a bucking bronco” according to my mom I tried to pull the vent tube out that was in my throat. Oopsie. I was then put in restraints around my wrists and they upped my sedation a bit to calm me down. They gave me a pen and paper so I could answer my mom and my husband’s questions. I told them to take lots of pictures (of me on the vent) because obviously I wouldn’t remember it. So me! haha

Not knowing that being put on the vent was a possibility after the bronch (if my lungs weren’t behaving) made coming off the vent traumatic in a sense. When it was time to take me off the vent they stopped my sedation to wake me up and that’s when I really understood that I was on the vent. Even though I was being my silly self asking for pictures to be taken while I was sedated and on the vent, being truly aware of what was going on was scary and freaked me out, just a tad. I’m the type of person that wants to be told what all the possible outcomes are and what to expect…the good, the bad and the ugly. My doctor failed to let me in on his little secret that me being put on the vent was a possibility. Not cool doc, not cool at all. Had I known being on the vent was a possibility, I could have mentally prepared myself. My husband failed to let me know that he thought it would be a possibility as well. Thanks for the heads up. Rude!

Time came for the tube to be pulled (extubation)…the RT (respiratory therapist) took his sweet time getting set up to pull my tube and suctioning out any secretions (aka mucus) in my vent tube, which he didn’t do a great job because the amount of mucus that came out with my tube was a lot. My husband, who was a trauma medic in the Air Force at the time, later told me he was about to shove the RT aside and pull my tube himself because he could see how scared and uncomfortable I was and the RT was being so slow.

With that said, for obvious reasons, I’m not a huge fan of bronchoscopies and only want them on a “must do” basis. I’ve had 3 or 4 more bronchs since my first one but did not need to be put on the vent for any other bronchs.

I’m hoping I can get some relief because I’m kinda miserable with the whole not breathing able to breathe well.

Until next time…