CF Happens

Sometimes in life CF (Cystic Fibrosis) happens…pun intended

recent posts

  • Second Pulled Chest Muscle in 4 Weeks – Lung Functions Down Further – Another Round of IVs

    I redid my lung functions on my home spirometer last Friday (the 21st). They were down again from when I did them on Monday (the 17th) before I started prednisone. Monday (the 17th) they were 27%. Friday (the 21st) they were down even more to 25%. My normal lung function is usually between 30-32%, which what we call my “baseline.”

    I was hoping to be able to redo my lung functions again on Monday (the 24th) and send whichever numbers were better into my CF doctor, either Fridays numbers or Mondays numbers. The nurse practitioner asked me to redo my lung functions on either Friday or Monday and send them into the office so they could see how I’m progressing on the prednisone. Unfortunately, I was unable to do lung functions on Monday (the 24th) because I pulled another chest muscle on Saturday (the 22nd) from coughing so much. This is the second pulled chest muscle in 4 weeks.

    I sent a message to my CF clinic/CF doctor on Monday (the 24th) to fill them in that I did my lung functions on Friday and they were down to 25% and that I was not able to redo them on Monday because of this pulled muscle, the pain would not allow me to redo them. That message to them won me a “sick appointment” on Thursday (the 27th) because I’m not getting any better and going the wrong direction in the lung function department, along with 2 pulled chest muscles in 4 weeks. haha

    HERE’S A LITTLE RECAP (to make sure you’re up to date on all the craziness these asshole lungs have been up to): My body has been put through the wringer since my back surgery. My lungs always take a big hit each time I’m intubated and put under anesthesia. With my lung functions being as low as it is along with the amount of damage CF has already done to my lungs, it’s harder for me to recover from all of the stress all of that causes on my lungs and body all together. With that said, I recovered pretty well initially from being intubated and under anesthesia. I was able to finish my preventative IV antibiotics (5 days prior to surgery and 10 days following surgery) with no issues. Unfortunately, the freedom from IVs didn’t last. I ended up needing another round of IV antibiotics because I was dealing with an exacerbation a little over 3 weeks after finishing the preventative round of IVs.

    With only 3-4 days left of the second round of IV antibiotics I got hit with a brutal virus that tried to take me out along with a really bad pulled chest muscle that I pulled mid cough. The pulled chest muscles like to spasm randomly and takes my breath away and I really don’t have any extra breaths to give. haha. I made it through that pulled muscle and it started felling better after about 10 days or so. Since I can’t take a break from coughing and breathing, my chest muscles never get a break. About 2.5 weeks after I healed (ish) from that pulled chest muscle, my asthma went haywire and I was very tight, wheezy and short of breath. That’s when I asked to start a high dose taper of prednisone to hopefully help open up my lungs. Then I pulled this new chest muscle from coughing so much and so hard yet again about a week after starting the prednisone. That brings me to my “sick appointment” in CF clinic.

    Update from my “sick appointment”: First up was lung functions. I was able to push through the pain from the pulled muscle and do 2 tests. The machine decided it was done for the day and wouldn’t work for a 3rd try. I’m not mad. haha. My first try was 26% and my second try was FEV1 28%/0.70L. Yes, it’s still down but not down anymore than it was so I’ll take it.

    The treatment plan is the following: We are changing up the IV antibiotic that we’ve been using as my go which has been Cefepime. We are going with IV Zerbaxa (1.5g every 8 hours through the IV ball) for 14 days. I have taken Zerbaxa before and tolerated it well. It takes about an hour to infuse. Yes, it’s a longer infusion. Of course, it’s no fun that it’s every 8 hours and takes an hour but being home doing IVs is so much better than being in the hospital doing them. I rarely go inpatient if all I’m needing is IV antibiotics for the simple fact that being in the hospital opens me up to ALL of those germs that live in the hospital. I don’t need any extra things to add to my already complicated body. haha. She is also extending my prednisone taper. I’ll finish the 40mg that was already prescribed but I’ll go down to 30mg for 5 days then to 20mg for 5 days then 10mg for 5 days. I was set to go down to 20mg for 5 days after 40mg then done. I should get the Zerbaxa tomorrow. It’s an expensive medicine, so it’s not one that they keep in stock.

    Here’s a little about Zerbaxa since many haven’t heard of it: Zerbaxa is a combo antibiotic. It has both ceftolozane (in the cephalosporin family) and tazobactam (in the penicillin family and is a beta-lactamase inhibitor). It was first approved for use in the US by the FDA mid December of 2014, so rather new in the world of antibiotics. We need NEW antibiotics developed. I’m limited on what I can take because of my drug allergies and what my pseudomonas is sensitive to in comparison to what I can actually take.

    Thank you for joining my educational Ted Talk today. haha

    Until next time…

  • My Lungs Are Major A$$holes (As I lovingly Call Them)

    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…