CF Happens

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

recent posts

  • Day 2 of Transplant Evaluation Testing…Take 2 (7 Years later)

    (Bear with me, this is going to be another long post in order to give all the information i want to give. Once again you will probably learn something new while reading the update)

    The first test today was the RVG (radionuclide ventriculogram) scan. It took 2 tries to get 1 IV so they used the same IV for both meds they injected. They like to have 2 IVs to use (1 for each med) but it is ok to do with 1 IV. They flushed it with 3 saline syringes to make sure it was good and clear. The first med is not radioactive. They injected the first med and then 15 minutes later they injected the radioactive med. The meds basically combine in the blood stream to make it able to see how the blood is flowing and how the heart is pumping. I had to lay down on the bed and lay still while the machine was at an angle taking pictures of my heart but 10 minutes. The machine was then moved to another angle for pictures for another 10 minutes. Then it was moved again to another angle for pictures for another 10 minutes. The images were checked by the doctor to make sure they were ok. They were ok’d then my IV was removed.

    Next appointment was with the dietitian. Every CFer that goes through the lung transplant evaluation has to meet with the dietitian. This appointment is to talk about any nutritional needs that I may have. There are quite a few CFers that have feeding tubes especially when they are close to needing a transplant to get enough calories in to maintain their weight. I obviously don’t have a feeding tube or any need for supplemental calories because I am able to maintain my weight.

    Final appointment for today was with the transplant doctor. It went as I expected it would go. I am still early in the transplant process and getting all my testing up to date was the main objective of this weeks appointments. Having all my testing up to date will make it much easier for me to get listed quickly in the event of a rapid decline in my health. We don’t want to have to backtrack and do any of the necessary testing in an emergency that would result in my needing to be listed quickly. The heart cath will happen when I’m closer to being listed for transplant.

    The goal is for me to use my natural lungs for as long as possible. A lot of people ask why I can’t just get lungs now since I obviously have trouble breathing already, my lung function is at 30% and I’m “healthy.” Unfortunately that’s not how it works. You need to use up your natural lungs for all that they have to offer before getting a new set of lungs to extend your life even further. So yes, I will get worse before needing/getting new lungs. It’s just how it goes. Trust me, if it were an easy surgery, easy recovery and if it meant I would live until I was 100 with no complications and I was allowed to get the surgery I would be all over getting a new set of lungs. That’s just not how it works. I just have to keep doing what I’m doing and living life to the fullest while I can. We will cross the transplant bridge at some point. I’m doing everything I can to be as prepared as possible for when the time comes. Most everyone has asked how I’m doing mentally with all this. I’m actually great with it all. I know this is just the next step in the process for transplant. Not everyone chooses to be evaluated for transplant for one reason or another which is just fine. I’ve always known this was the path I wanted to take when it was time and my lungs crapped out. My depression is under control, as is my anxiety. Yes, I take an antidepressant to help and I see a counselor who I love! I have all the things in place to help with my mental health which is very important to me. That’s not even mentioning my great support system. I’m thankful for all of you.

    The doctor said that (of course) I need to start exercising to help prolong the lung function I do have. Exercise is a great airway clearance just like the vest I wear during my breathing treatments. The deep breathing during exercise helps keep my lungs open and working like they should while conditioning them. The bad part is I absolutely hate exercising, especially organized exercise like walking on the treadmill. It’s boring and I’m not a fan of being short of breath. It really feels counterproductive. Haha. I know it’s what I need to do but exercising has never been my thing no matter the amount of lung function I have. I’m going to try my best to get in a routine of walking on the treadmill to work my way up to walking 30 minutes a day, at least 5 days a week. Just know that I’m not happy about it. Haha

    I still have the appointment with the financial coordinator on Thursday to discuss what insurance covers in regards to transplant and fundraising options. I do have a COTA (Childrens Organ Transplant Association) fundraising page. COTA is great because they offer their services for CF patients of all ages not just 18 and under. All donations made to my COTA page are tax deductible which is great. So unlike gofundme who takes a percentage of the money donated, all of the money I raise through my COTA page goes to me and my transplant associates financial needs. I am able to submit receipts for things I pay out of pocket for and then I’m reimbursed from the funds I raised. Some of the things that I’m able to use my COTA fund for are medication copay’s, hospital bills, doctor bills, hotels for appointments because I’m not local to my transplant hospital, food (groceries or eating out) while at appointments for pre and post transplant care, living expenses associated with post transplant like an apartment, gas to and from appointments, all bills to maintain our home in TN post transplant while I’m in St. Louis recovering for at least 3 months, to name a few.

    You can go to my COTA page to read up but I do all updates here or you can go donate if you would like to. Here is the link…. https://cota.org/campaigns/COTAforShannonM

    I will follow up with the transplant clinic in 6 months. I will do labs, full PFTs, 6 minute walk and see the doctor during my 6 month check up. Thank you all so much for all of your support over the years. It is much appreciated!

    Until next time…

  • 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…