Tag Archives: trikafta

Trikafta…4ish Years Later

Posted in Blog Life by

I have many friends that have benefited so much from Trikafta with an increase in lung function and a better quality of life. I’m so happy for them! Many have even been able to stop doing breathing treatments. Many call Trikafta a “miracle drug” because of the changes in quality of life. I know for many it is a miracle just in the way they feel. With all the good things that have come with Trikafta there are also not so great side effects in regard to the mental health side effects of things. Many say they will deal with the mental health side effects of it because they have a better quality of life in the physical aspect.

As you know, I haven’t gained any lung function since starting Trikafta in November of 2020, I think that’s when I started it. haha. I didn’t have high expectations when I started it because I knew that I have a lot of scarring and damage in my lungs. I had/have between 30-35% lung function which isn’t great. If taking Trikafta helped me maintain 30-35% lung function for longer which would hopefully put off lung transplant for longer then it was going to be worth it. Also, my really bad asthma doesn’t help the state of my lungs as it is. No, I’m not upset that I didn’t gain any lung function. It is what it is. Just like everything in life, things go differently for everyone. You can’t compare other people’s lives or experiences to your life. It will always be your own journey no matter what. Even if you emulate others, it won’t be the same outcome.

I have CF friends that were able to stop breathing treatments because of Trikafta. Unfortunately, some were hit with viruses that went to their lungs and they needed to restart breathing treatments to get relief to get over the virus. Obviously, they are not very happy about needing to do breathing treatments again. I told my husband the other night that I feel like it’s a blessing in disguise that I’ve had to continue all my breathing treatments because I don’t know what’s it’s like to not do breathing treatments (except for when I was little, which of course I don’t remember) so I can’t get mad at having to restart them. It’s just a part of my life. I can’t miss the extra hours gained in a day not doing treatments if I never gained those hours. It would be weird to me to not do breathing treatments honestly. I know with my bad asthma, not doing breathing treatments is out of the question anyway. Plus I feel like even if I’m having a “decent” breathing day (can’t remember the last time that was the case though) they are a part of my regular maintenance, so to speak. Of course, after transplant I hope I will be able to stop breathing treatments or at least decrease the frequency of treatments at some point which will be fantastic!

I also have many CF friends that were transplanted around the time Trikafta was approved. They have a whole different set of emotions regarding Trikafta. They will always wonder if Trikafta would have helped them gain lung function or if it would have helped stabilize them for a longer period of time putting transplant off. Many are a little bitter that Trikafta was approved and they never had a chance to take it. Things happened the way they did and you can’t change the past. There’s no reason to dwell on things you have no control over. I absolutely know that that is easier said than done but you lose precious time worrying about things you have no control over. You need to use the time you’re given wisely and don’t miss out on time with those close to you. You can’t live in the “what if” mind set.

I believe things happen for a reason, even if it’s because of your bad decisions. Life happens. It has ups and downs. Make the best of it.

I decided to share my point of view because I’ve had conversations with multiple CF friends, pre and post transplant, regarding Trikafta recently. We are all in this life together no matter where we are on our CF journey. You got this y’all!!

Until next time…

Day 2 in lock up

Posted in Blog Life by

I didn’t get much sleep last night per the usual with it being the first night and not getting here until shift change (7pm). Getting all the orders in and going takes awhile. Everything is staggered when things get started at random times throughout the night. I did get started on 60mg of solumedrol last night as well as both IV antibiotics, amikacin (every 12 hours) and zosyn (every 6 hours, infused over 4 hours each time).

I had a chest CT this morning and I just asked my nurse if there was any report in the computer from radiology. It didn’t show any new pneumonia or much change from my CT when I was inpatient in December. It did show bronchiectasis, which one had for awhile. Bronciectasis is when the airways (bronchi) are thickened and damaged making it harder to move air easily. It also showed narrowing of the upper lobes which isn’t new either. The issue going on now is a lot of asthma with a side of CF. It’s aggravating the CF bugs in my lungs and all the other things as far as the scar tissue and irreversible lung damage that’s already there.

They drew a amukacin peak and trough, before and after a dose to check the level in my system to make there’s not too much. Since I’m on amikacin they did a hearing test because it’s in the same family as tobramycin which is an aminoglycoside which hearing loss is a side effect. My hearing test showed that I’m not hearing high pitched sounds that great but all the other levels are good. They will continue to monitor my hearing while I’m on amikacin. I also did lung functions. My FEV1 was 29%/.80L. They were 27% on my home spirometer. So I would call it steady with no loss. Different PFT machines show different values but as long as they are steady and consistent that’s what matters.

They did start me on another inhaled medicine, revefenacin (once a day) which is used to control wheezing, shortness of breath, coughing, and chest tightness. It can be used in place of duoneb (atrovent/albuterol combo) which I take. I chose to do both the revefenacin and duoneb in hopes that I can put as much in my lungs to hopefully get me open. That’s basically the update for today.

This is going to be a long boring weekend in lock up as usual. Nothing gets done on the weekend as far as any testing or new orders unless it’s necessary. I’m hoping to do lung functions again on Monday and fingers crossed there is some improvement and that I actually feel more open and not as short of breath. With that said, if there is improvement I’m hoping to be home Tuesday or Wednesday but of course I’m not holding my breath (literally and figuratively). I’ll update tomorrow if there’s anything to update about which means don’t freak out if I don’t post until Monday evening after lung functions and seeing the pulmonologist during rounds on Monday.

Until next time…

In lock up I go

Posted in Blog Life by

I had a telehealth appointment with my CF doctor this afternoon because I’m not feeling any better on the oral Levaquin and prednisone taper. I did do home spirometry (lung functions) before my appointment so we knew where I stand as far as my numbers go. They are down to 27%. I made the decision to be admitted and get started on higher dose IV solumedrol (steroid) and IV antibiotics (possibly zosyn and amikacin). I had a few options to choose from as far as going inpatient or trying IV zosyn at home and continuing the prednisone taper. If I went the route of trying IVs at home and was no better come the beginning of the week I would end up going inpatient to do IV solumedrol and possibly change or add IV antibiotics. So I decided to get it over with now verses waiting another 5 or so days to most likely be admitted in the end.

I just got to my room. It’s shift change so it will be a little bit before my real orders for solumedrol, breathing treatments and IV antibiotics are in the system and get started. They will access my port once antibiotic orders are in the system. I did a breathing treatment before I came knowing it’s a slow process getting things going in the hospital.

It is very hard for me to throw in the towel and willingly be admitted. I’m not a fan of being in lock up. I’m very consistent in my treatments, IV times and taking my oral meds on time. I’m a little bit of a control freak (regimented) when it comes to my medical care and wanting things to be right. When I’m inpatient things get off with IV times because obviously I’m not the only patient they are taking care of. It’s hard for me to hand over that control. Being inpatient also takes a toll on my mental health, especially in the summer, my favorite season. I want to be outside in the sunshine doing things and not stuck in the bed. Heck, just being free to go eat lunch at a restaurant or window shop is a luxury that isn’t lost on me.

Taking care of myself is my priority. With that said, my mental health is also a priority. I know what I need to do but that doesn’t mean there won’t be a little kicking and screaming involved. I’m stubborn in a lot of things but I ultimately do what I need to do to get better. I will update tomorrow with more details on what things get started and how my busy night is as usual. Little sleep will be had I’m sure.

Until next time…

3 Month Check Up/Check Up after IVs

Posted in Blog Life by

Today I had my regular 3 month check up which happened to fall after I finished IVs.My nurse called me to ask if I wanted to change my 8am appointment to a Telehealth appointment instead of having to go in. I, of course, said sign me up!! At my clinic we go to the pulmonary lab 1 hour before our clinic appointment to do PFTs. That means that I have to check in to admitting before the pulmonary lab appointment. That would mean that I would have to get up around 5:45am to do a treatment, get ready and then drive 27 minutes to the hospital to check in. So basically that would suck. So yes, please sign me up for the Telehealth appointment! With that said, if I were feeling bad I would have gone in so he could listen to me and I could do the more accurate lung functions in the pulmonary lab. When I do a Telehealth appointment I use my home spirometer the day before my appointment and email my results in so my doctor can look them over before he sees me. Ok now onto the actual appointment update…

My lung functions were basically steady. My FEV1 was .85L/31%. When I finished IVs I did lung functions on my home spirometer and they were .89L/32%, so no real change. We did discuss what antibiotics would be my next choice for the next time I need IVs. Meaning which ones my sputum is sensitive to. Unfortunately, I don’t have many options with my drug allergies and with the few that I’m actually sensitive to. That’s not to say that I’m out of options because that’s not the case but we may have to be strategic with what we use and how we use them. Antibiotics just don’t work like they used to for me.

My cough is more of an asthma cough which means it tighter and has a high pitched wheeze. I’m not loosening up anything with my coughs. I’ve never been one that coughs much up anyway and that changed even more after I started Trikafta. My cough right now is more of an irritated cough, like my airways and lungs are irritated and inflamed. I’m not coughing a ton but when I do it isn’t productive and the cough doesn’t relieve any tightness and wheezing. My duoneb (albuterol & atrovent combo) treatments don’t always help to open me up that great. That’s asthma and CF combo crappy lungs for ya. My allergy asthma doctor that prescribed the Dupixent injections (every 2 weeks) for my asthma wants me to stay on it for a total of 6 months to make I give it a chance. I told her that I have seen no improvement at all. So at the end of July if I am still not seeing any improvements, I will stop taking it. There’s no reason to stay on it if it’s not doing anything. I take enough medicine so if I can eliminate one because it’s not working then I’m all for it.

That’s pretty much the update. My doctor is great about putting our brainstorming in his notes as far as what antibiotics to go for next time so that if he’s not available to see me the nurse practitioner will know what we want to do. He looks a few steps ahead not just in the right now which I appreciate. Everything else is steady. I’ll have another appointment in 3 months for another regular check up like usual.

Until next time…

Happy birthday to me…I’m 43!!

Posted in Blog Life by

My 43rd birthday was yesterday. February 1st should be a national holiday by now! Haha

I absolutely love my birthday. I know most women don’t like getting older but I’m not most women. Getting older is a blessing a lot of people take for granted.

Life expectancy numbers have never really worried me. It’s just a number. When I was born the life expectancy was around 12. Now for CF patients that were born between 2017-2021 the life expectancy is 53!!! There are now many more treatments and medications for CF including the revolutionary modulators like Trikafta that works on the cellular level. Research for CF is so important to extend the lives of those with CF. Finding a cure for CF is the ultimate goal. The CF Foundation has more than 70 chapters in the United States. Their slogan is “make CF stand for Cure Found.” You can go to http://www.CFF.org to find a local chapter or to donate to the cause. You can also find a Great Strides walk close to you. You can start a team or be an individual walker. Great Strides is the biggest fundraiser for the CFF.

Thank you for always checking in on me and for all the prayers and positive thoughts. Just remember that growing old is a blessing that isn’t offered to everyone.

Until next time…