Hello All,
I know you've been anxiously awaiting an update on my Trikafta journey, so here we are!
If you don't really know what Trikafta is/does...I've gotta get a little scientific. The genetic mutation with CF causes the Cystic fibrosis transmembrane conductance regulator (CFTR) Proteins to either not reach the surface of the cell or if they do reach the surface of the cell, then they do not open properly, which in turn causes a disruption to the sodium and water balance. Therefore causing thicker and stickier mucus in all your bodies passage ways. So CF affects the lungs, digestive system, etc. The thicker and stickier mucus in the lungs makes it's easier for bacteria to adhere and more difficult to get it out, so infections (colds, flu, etc) hang around longer because it's harder to get rid of the bacteria/virus that causes them...and it makes it more difficult to breathe by causing inflammation. The mucus causes issues in the digestive system by making it harder to absorb nutrients from food (that's why many people with CF have difficulty gaining weight). The enzymes that help break down the food in the digestive system, have trouble getting out of the pancreas because of the thick and sticky mucus that clogs the ducts to the stomach and intestines...this is the same for the insulin from the pancreas. So many people with CF end up with Cystic Fibrosis related Diabetes. Trikafta is a triple combination. It's made of 3 different medications. It helps the CFTR Proteins to reach the cell surface and to open properly and stay open longer, therefore helping the mucus to thin out, which makes it easier to cough up.
Trikafta is NOT a cure. It is the first medication/treatment that we have that actually treats at the cellular level. But it is not a cure!
I'm going to start by telling you my hope for my Trikafta journey.
My hope is to at least maintain my lung function, and decrease the decline of my health, and to see a decrease in my symptoms of CF. If that's all that happens I will be happy.
So...how am I doing?
A lot of people I'm friends with on FB or who have posted in FB groups I'm in have posted that they no longer feel like they have CF. Some have had 20-30 percent increases in their lung function. Some have no longer had coughing fits. Some have no trouble breathing or coughing when exercising. Some people have had what they're calling "the purge", where they are coughing up lots and lots of thick mucus.
I don't feel hugely different...
I haven't noticed a whole lot of changes...
On days 2 and 3, I'd say I had my "purge". And by purge I mean I coughed up maybe 3-4 tablespoons worth of mucus total. I'll save you all from posting the picture I took of it. But I will tell you this, I RARELY ever cough anything up. It's a chore, usually, to produce something when they want a specimen at the doctors office. Unless I'm sick, I hardly ever have a productive cough. So, to EASILY cough up a tablespoon or so at a time without much effort, was HUGE for me.
My sister lives 2 doors down, and on day 3 or 4 I ran from the back of her garage, to my garage (maybe 50 yards total) and I was not Short of breath, upon arrival, nor did I have a coughing fit afterwards.
I'm not sure what my weight was before starting Trikafta, but at my most in my life, I was around 136. I haven't been there for a few years, but I weighed myself on Thanksgiving (albeit after I ate, at my parents) and I was back to 136. Heather, we may not need the cure to make me fat after all...HAHA!
I usually sleep through the night and really well. The first week I woke up every night with a dry cough that wouldn't subside until I did albuterol and a round of my vest. I think that's starting to pass, not sure though if it's related to the Trikafta, or that I was not at my 100% when I started the medication to begin with and Fall is my difficult time. But this is the only real "negative" I've seen since starting the medication.
AND that's about all I've noticed so far.
I don't have a follow up for lung functions for another couple months. We will see if I maintain or if it does give me an increase. Again, my hope is to have a decrease in symptoms and to maintain my current lung function. At this point, less shortness of breath with increased activity and being able to produce mucus easier to expel have hit those marks.
I want you to check in. I want you to ask, but please understand, it is hard for me to answer how I'm doing, what I'm seeing and if I'm "better"...
While I am excited about Trikafta, hopeful, happy, I am also nervous and cautiously optimistic. Again, Trikafta is not a cure. Some people may not see a change in their CF, it may not help them. Even on this medication we will still have people with decline, we will still lose precious lives too soon.
Trikafta is only approved for 90% of the CF population. Which means there is 10% of my Cysters and Fibros that have no CFTR modulator or can't take one yet. They are sitting in the wings waiting for treatment. They have nothing and would give anything to be at my stage.
I also have some survivors guilt.
When I think of friends who have passed, that never got to get to this point. Some were adults, some have been children. Why do I get to experience this and they didn't? I'm grateful but sad all at the same time.
So it's hard for me to think only of the "better" it will bring to me and to those that are able to take it.
With all that said...please keep my CF family in your thoughts and prayers. Pray for those who are on these CFTR modulators, that we would see the results we hope for, and maybe a few nice surprises on the side. Pray for those who have no modulator, that they would thrive despite not having these treatments at their disposal...and that the scientists would be swift in developing modulators and new treatments, and Ultimately a cure for us all!
I love you all and am so blessed to have so many loving friends and family to share this journey with. I am a Salty Girl and my Team and Tribe of supporters are the BEST around.
As D-Dub says: #LoveEternal
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