CF Happens

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

recent posts

  • 3 Month CF Clinic Check Up (and a little extra CF education)

    I had CF clinic today. It was the first time I’ve been seen since I was in the hospital in July with really bad asthma issues. My lung functions were up today. My FEV1 was 34%/.93L. They were 29% in July. My baseline is around 32%, so I’ll take 34%!! My weight was down a few more pounds which is not a bad thing considering I gained close to 40 pounds after starting Trikafta. The weight gain was strictly from Trikafta because I counted calories to try and see if I could adjust my intake to help lose the extra weight. I was barely taking in the number of calories that was the allotted amount for my weightless goal. I wasn’t willing to stop Trikafta to lose the weight. Thankfully my body figured it out that Trikafta was staying for the long haul and it needed to get its sh*t together and adjust my weight. haha. Sometimes you have to decide if the side effects of a medicine that’s helping you are worth it. Risk vs Reward. No, I didn’t gain any lung function with Trikafta like others have but if it means I’m able to put off lung transplant for longer then it’s worth to me.

    My lungs sounded good, no wheezes or rattles. That’s thanks to my asthma behaving right now. My doctor got all the notes and labs from Barnes from August when I was last there getting all my transplant testing up to date. Of course he wants a few more labs (vitamin levels being one) that they didn’t draw in the 12 tubes they took. haha. Everyone said I looked great!! They all know I just got back from a trip to Hawaii and that it’s my happy place. I took the phonebook I made of our trip and everyone loved it. They all said that Hawaii obviously did you good. I said of course! There’s no humidity which my lungs are not a fan of, I get to breathe in the salty air and I get to be in the sunshine which are all great things if you ask me. I go back in 3 months for a regular check up. The hope is always that I make it the 3 months without needing to call them to seen sooner because I’m sick. Fingers crossed as always! I also have appointments at Barnes in March for transplant clinic check up.

    Ok, I’m going to go off on a tangent for a second (hear me out, it’s going to teach you something)…

    I’m going to cover the things that are the regulars of CF world. Not all clinics are the same, so some don’t do everything the same as my clinic do. Although, there are things that CF clinics need to do to make sure they stay an accredited CF center and to get funding. This is NOT the gold standard of CF care but it may look similar to this. At every appointment they do your height, weight, blood pressure, oxygen saturation. Your height and weight are monitored closely because they need to make sure you are maintaining a good BMI. If you drop weight it usually indicates that you may have an active infection going on that your body is using your weight reserve to fight an infection which takes calories to do as does breathing in general when you have crappy lungs. You should see your CF clinic at least 4 times (quarterly) a year. In that you should do at least 2 pulmonary function tests to measure lung function. I do this at every appointment and more frequently when I’m actively sick. You should also get a chest x-ray/chest CT every 1-2 years, sometimes clinics like to do both to have all the bases covered. You get them much more often than that if have an active exacerbations (CF sick). A sputum culture should be done quarterly as well. I do this every appointment and when in the hospital to make sure we are using the correct antibiotics. A bone density scan is recommended every 2 years because with the crappy pancreas you don’t get all the vitamin D and calcium that you need to keep your bones healthy and strong. Also, yearly labs that include vitamin levels (once again thanks to a crappy pancreas) and liver enzymes because a lot of meds are filtered through the liver so it has to work overtime. Plus CF related liver disease is very common in CF. You should do a yearly GTT (glucose tolerance test) to check for CFRD, CF related diabetes. This is one of my least favorite tests because I have bad veins and you have to be stuck at least 3 times and sometimes the glucose drink gives me a headache. Once again thanks to having a crappy pancreas it needs to be checked often. If you have been diagnosed with CFRD you don’t have to do the GTT anymore because you already know you have CFRD. You should have colonoscopies every 5 years after 40 years old unless you have family history of polyps, colon cancer or other colon issues. If you have any of the things I just mentioned, you should do colonoscopies sooner and more frequently. Next up, yearly abdominal ultrasound to check the liver, gallbladder (if you have one, haha), spleen and kidneys…basically anything and everything you have in your gut because CF doesn’t play nice and can affect many different areas in the body. The abdominal ultrasound was just added as a recommended maintenance test a few years ago at the national CFF conference.

    I saved an important one for the last one…yearly depression and anxiety screening with the PHQ-9 survey and the GAD-7 (social worker gibberish) survey. Living with CF isn’t for the faint of heart and isn’t not easy. It takes a toll on your mental health. As you know, I’m an open book about my depression and anxiety struggles. I preach to anyone who will listen to me to GET THE HELP you need! Whether that be antidepressants, anti anxiety meds or talk therapy with a therapist/counselor/social worker…or a combo of all three. Having a great support system helps immensely! With them you know that even though you are the one living the day to day physical side of CF, your support system has your back and they are there to help anyway they can…just ask! Something as little as doing carpool for you if you are a parent, cleaning your house for you (even one room), doing the dishes, making dinner, joining you at appointments so you have company because clinic days are long, hanging out on your couch while you talk with the TV on for background noise or even picking up your prescriptions for you (among many other ways to support the CFer) means much more than you know or more than the CFer will let you know.

    I cherish my family and friends that are a part of my support system. They help me more than they realize. I am a stubborn patient/person at times (my husband would probably say ALL the time) and don’t like to ask for help. Over the years I’ve learned that asking for help is NOT a sign of weakness. If anything it helps me to be stronger. I still struggle asking at times though. I’m working on it every day. Ok, I think I have “talked” your ear off enough. I hope you stuck it out and read this post ALL the way to the end. As always, I’ll update if/when there’s something to update about.

    Until next time…

  • 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…