CF Happens

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

recent posts

  • Update from the outside (of lock up that is)

    (This may be information overload but I always try to explain things so they are easier to understand so bear with me)

    I had a Telehealth follow up with my CF nurse practitioner last Thursday. We decided that I was good to be done with IVs and to finish the prednisone taper. I did my lung function on my home spirometer Wednesday night in preparation for the appointment, unfortunately they were 27%/.75L. No improvement and a little loss. The nurse practitioner suggested I get seen by my transplant center considering my lung function is hanging out at 27% with no improvement. My CF nurse called me on Monday to let me know that they talked about me at the provider meeting in regards to the nurse practitioner suggesting that I see my transplant center. My CF doctor agreed with the nurse practitioner. I sent an email to my transplant coordinator with an update about this last admission and that my CF doctor wanted me to get seen.

    I sent the email later that day. My coordinator emailed me back the next day and said that she would call me today to get the details of getting me scheduled all situated. She spoke to the transplant doctor about my email and that I was wanting to seen. He wants me to do all the evaluation testing again since I did it last in 2016 and I haven’t been back to see them since 2019 because of the pandemic. So that means it’s 2-3 days (Monday through Wednesday) of testing and then meeting with dietary, social work for insurance things and financial talks on Thursday. I will be going for evaluation the week of August 20th.

    Evaluation testing consists of a bunch of lab work, 6 minute walk where they are monitoring my oxygen level the whole time to see if my sats drop below 90% at all which would indicate a need for supplemental oxygen, chest x-ray, chest CT, full PFTs (what I like to call the “gas chamber”), ABG (arterial blood gas aka needle stick in your wrist), VQ scan which will check to see which lung is oxygenating better and has better blood flow. The VQ scan helps in deciding which lung would be transplanted first during surgery. The lung with the poorest function is removed first then the new lung is all hooked up and gets working, then the next lung. The next test is a RVG nuclear scan which is to see how well the heart is pumping. I will then see the transplant doctor on Tuesday afternoon after all those test are completed to discuss all of those tests. If they want more testing now is the time that they will order those. A heart cath (both sides of the heart) will be scheduled for Wednesday as if they will want to proceed with testing. It can be canceled if they don’t want anymore testing at that time. I will also meet with dietary to address any dietary needs I may have and social work to talk about insurance and financial planning. If they don’t want anymore testing after I meet with the doctor on Tuesday I will be able to head home the next day (it’s a 7.5 hour drive for me). If they want to do more testing, to include the heart cath, I will be able to head home Friday. They will call me to fill me in on things after the whole team discusses my case.

    With all that said (I know it’s Information overload) I’ll try to answer some of the common questions I’ve gotten about this whole transplant evaluation/transplant process. Most common question is how I am feeling about it all. I am actually really good mentally with it all. I’m not freaked out or thinking the worst. Doing the testing/evaluation is a necessary evil in the process. It’s better for me to get all my testing up to date in the event that I have a random rapid decline and need to get listed quickly. You always want to be ahead of the game so you aren’t backpedaling trying to get all the required testing done yesterday. I always try to look at the positive side of things and not dwell on the negative side. Yes, it a scary process more so because I don’t know what to expect because I’ve not had a transplant before. No matter the number of friends I’ve been with on this transplant journey, it’s a whole different story when it’s me actually going through it all.

    Next common question, what is the magic number (lung function) that will get you listed? There isn’t a magic number. There are other factors that come in play like quality of life, running out of antibiotic options, not responding to treatment anymore, oxygen needs, not bouncing back after an exacerbation, needing IV antibiotics more frequently and needing to be inpatient more often for treatment to name a few. The goal has always been get everything I can out of these crappy lungs before getting a new set. Yes, it seems like just getting new lungs now would be easier because I’m more “healthy” so to speak but there’s a process for a reason. The lung transplant doctors are the experts on this so I will listen to them as far as how this all goes. This isn’t their first rodeo.

    I think that covers pretty much all of it. Feel free to comment or email me with any, and all, questions you may have. My passion has always been to help educate about CF and lung transplant. The more knowledge you have the better.

    Until next time…

  • Day 5 in lock up

    I’ll try to keep it short as possible but there’s a lot of details to shove in here to make sure you all are in the know. The weekend in lock up was boring as usual. Nothing gets done on the weekends unless it’s an emergency.

    I saw my regular CF doctor this morning, which is always nice because he knows me and what I need unlike the hospital pulmonary team who doesn’t see me regularly. He ordered an echocardiogram to make sure my heart is pumping like it should with the persistent shortness of breath. He also ordered a VQ scan. A VQ scan is a ventilation/perfusion lung scan to look at circulation of air and blood in the lungs. It checks for blood clots (pulmonary embolisms) which shortness of breath is a side effect of blood clots in the lungs. During the VQ scan you inhale a radioactive gas and they inject a radioactive substance and take different pictures of your lungs. The report for the VQ scan said it showed no blood clots. It did show air trapping in the upper lobes (not moving air out as well) which is consistent with my Bronchiectstasis (widening of bronchi/branches/airways) that I’ve had for awhile, along with volume loss in the upper lobes which was consistent on my recent chest CT as well. Bronchiectstasis is usually caused from repeat CF exacerbations where mucus and bacteria build up and kinda wreak havoc. I haven’t gotten a report on the echocardiogram yet.

    I did lung functions and they were the same as Friday, 29%/.80L. So no improvement as far as the numbers go but we also take into account how I’m feeling, not just the numbers. I’ll take no worse though. I am feeling less tight and wheezy and according to respiratory therapy and my doctors, I sound better as well. I am still getting short of breath when I’m up doing things but I’m recovering ok. I’m still on room air (no oxygen) and sating around 95%.

    The plan is for me to go home tomorrow and finish IV zosyn (infused every 6 hours over 30 minutes via IV ball) for a total of 14 days, to finish on the 20th. I will have a clinic appointment (with in person lung functions) or telehealth appointment (with home lung functions) on the 20th to discuss if I should continue another week for a total of 21 days or to be done at 14 days. They switched my steroid to 60mg oral prednisone starting today. The prednisone taper will look like this…60mg for 3 days, 50mg for 3 days, 40mg for 3 days, 30mg for 3 days, 20mg for 7 days and 10mg for 7 days. He said he would decide on how the 20mg and 10mg part looks later depending on how I’m doing.

    My home health company has already called so I could give them the “go ahead” on filling the antibiotics. They have to verify that I am ok with paying the out of pocket cost that my insurance doesn’t cover. They will get the final “ok” to fill the antibiotics tomorrow morning when they start my discharge orders. I will be released and home health will deliver my meds and supplies to my house. I think I got it all covered. I’ll update about the echo with my next update after I get home and settled when there’s more to update.

    I have had a few people ask what all this means as far as transplant goes. I’m still holding steady in the big picture of things. I have been hanging around 30-32% lung function for the past 6 or so months. Yes, my transplant center pays attention to how low my lung functions get and stay but they also take into account other things like not being able to bounce back as easily from CF exacerbations, running out of antibiotic options, limited antibiotic/treatment options, quality of life, oxygen needs, number of hospitalizations, etc. So we are still in the “wait it out” stage to see where things are in regards to transplant. We will be keeping a close eye on things and will contact my transplant center if needed. Thank you so much for always checking in on me, I appreciate all of your support very much!

    Until next time….