CF Happens

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

recent posts

  • Day 1 of Lung Transplant Evaluation Testing…Take 2 (7 Years Later)

    I was going to do an update after all the appointments this week but I decided that would be way too long of an update. I wanted to give more detail on the tests so non CF people/non medical people will better understand what I’ve been up to this week. Haha. Hang in there reading this, there’s going to be a lot of information but it’s good information.

    First stop this morning was the transplant office bright and early at 7am. I received my lung transplant handbook with lots of information regarding pre and post transplant life, as well as transplant surgery information. I also received my schedule for this week and signed a few things. Next up was labs (10 tubes!), EKG and chest X-ray.

    Next stop was meeting with the social worker to discuss the mental aspect of transplant. She asked lots of questions regarding my life with CF, my mental health, how I handled/handle certain situations in my life, family life, who my support system is, who my caregiver will be post transplant (my husband), who the backup caregiver will be during the first 3 months after transplant if my husband needs to be away for any reason and lots of other questions. She has to make sure I will be able to handle the mental side of transplant as well as the mental health of my caregivers. Being the primary caregiver post transplant is a lot of work physically and mentally. My caregiver will have to be with me 24/7 for the first 3 months. I won’t be able to drive for at least 6 weeks after transplant. I will have appointments at the hospital Monday through Friday after I’m out of the hospital. Pulmonary rehab every day for an hour, labs twice a week, see the doctor once every 2 weeks, bronchoscopes (to clear secretions and do biopsies to check for rejection) sprinkled in there as well. Labs, seeing the doctor and bronchoscopes can vary due to how I’m doing. If I’m having a lot of bumps in the road after transplant and need to be monitored closer then those appointments will increase.

    Next up…6 minute walk. Yes, this is a 6 minute walk. Haha. During the walk I wear a oxygen saturation sensor (O2 sensor) to watch my sats to see if my oxygen level drops when walking. If it were to drop to 88% or below there would put oxygen on me via a nasal cannula and continue the walk. During the walk I walk as fast or as slow as I need to. I can take breaks as well but the timer keeps going. The goal is for me to walk at least 1,000 feet (if I remember correctly). I ended up walking 1,203 feet with no breaks and I didn’t need oxygen. My sats stayed at 94-95%.

    Now it was time for PFTs (lung functions). This includes ALL the tests. The first one is spirometry which is what I do at all my CF clinic appointments or at home for telehealth appointments. There are 2 other tests that show how much oxygen I am taking in and how much goes back out (how much “gets stuck). My FEV1 was 32% before a bronchodilator (albuterol inhaler) and after 2 puffs of ventolin inhaler they went up to 33%. The predicted values of a healthy female my age differ in each machine so my numbers differ in each machine but they are relatively similar. They also do a blood gas (ABG) where they stick a small needle in the artery in the underside of your wrist and the small syringe fills on its own because the blood pumps it in there.

    Next up was a VQ (ventilation perfusion) scan. The scan shows the air and blood flow in my lungs. This shows which lung is weaker vs stronger. The purpose of this scan is that during transplant surgery they start with taking out the weaker lung first and then move on to the stronger lung. Now to describe this test/scan. First up, I inhale a radioactive gas (with oxygen mixed in) through a mask that covers my nose and mouth with a seal so no gas leaks out while taking pictures. Not very fun when it feels like you aren’t really getting real/good air in but this part takes 8-10 minutes then the mask gets taken off. I’m not a fan of feeling like I can’t breathe, especially with 30% lung function. For the next part of this scan I had to get an IV (in my hand because my ACs (the bends in the arms) are too scared to use). They inject a radioactive medicine in the IV, flush it with saline and then take the IV out. I had to be stuck twice, once in each hand, to get an IV in because I’m a hard stick with little rolling veins. So the IV was in for like a minute and 45 seconds. Haha. Anyway, after the radioactive med was in and the IV was removed I had to get more pictures/scans of my lungs. Both sets of pictures/scans are taken while sitting on a stool in front of a machine that looks just like what you stand in front of when you get a chest X-ray. They take pictures straight on (back to the machine) and 1 picture of each side at about 45° (in between side and center) with both the radioactive gas and radioactive injection med.

    Last stop was chest CT without contrast. This is a quick one. You lay on the table and they send you through the machine while you hold your breath for like 5 seconds while you move through 3 different times. If you are given contrast for the CT they usually take more pictures while you hold your breath for like 5-7 second increments while they take the pictures in sections more so than your whole chest on one pass. It’s more detailed basically.

    I did look at my results for my labs and chest X-ray in my patient portal. Everything is good except for some lab values pertaining to my iron levels being a little low. My chest X-ray showed “extensive bronciectasis with associated volume loss” (normal for me). My upper lobes looked a bit clearer compared to my last X-ray from 2019 which was the last X-ray they have on record. Obviously the doctor will go through all my results from both days when I see him tomorrow and we will discuss them all and any things I need to do. There is no report on my chest CT in my patient portal yet.

    Tomorrow I have a RVG scan (Radionuclide ventriculography) which shows how well your heart is pumping. It’s another neuc med test (like the VQ scan) so I will be getting another IV (or 2) because there is 2 different radioactive meds they will be injecting. After the RVG scan I will meet with the dietitian which is an appointment everyone has to do. I am pancreatic insufficient because of my CF. My pancreas doesn’t make all the enzymes it needs to digest my food so I take enzymes when I eat to help digest my food. My weight is great and I have no other dietary needs so that should be a quick appointment. Last stop for tomorrow will be seeing the doctor. At that appointment he will go over all my test results, any questions we have about transplant and he will order any extra tests he wants done to include the heart cath that is already scheduled on Wednesday. If he doesn’t want me to do the heart cath at this time it will be canceled. We will discuss if he thinks I need to move forward with getting listed soon (which will include any extra testing to include the heart cath) or if he feels like I can hold off for a little while longer and continue every 6-12 month follow ups with them. Thursday the financial coordinator will call me to discuss insurance coverage and different fundraising options to cover all non covered expenses associated with transplant (pre and post). The transplant team will discuss my case Thursday afternoon with input from the doctor who saw me to determine what the next step is, move forward with getting listed soon or continue regular follow ups for right now.

    I know that a lot of information but I wanted you to be able to get a glimpse of what transplant evaluation testing looks like. You know I like to educate you. Haha. I’ll update more later.

    Until next time…

  • 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…