“Don’t stop anything”

This past Wednesday I had CF clinic. It was my first appointment since starting Trikafta. I don’t know if this is the beginning of repeated check-ups like this one. If so, I may just be a sobbing-able-to-breathe-deeper-laughing-longer-without-coughing-no-longer-wheezy-singing mess for the rest of my life. All went..incredibly well. Typing now, I forever struggle to express the thankfulness in my heart. My only concerns were: a) follow-up labs one month after starting Trikafta to check liver enzymes; b) check sinuses to confirm if any issues as I’ve had more drainage / post nasal drip; c) I need new bras (added bonus? what.. how..?)

I gained 6 lbs in a month! I was shocked. Living with CF, being at a healthy weight is important. We need that reserve for when and if we become sick. Luckily, I’ve maintained a healthy weight for several years now. It’s not easy. As I’ve so wonderfully aged, I have noticed my metabolism has slowed down. Now that I’m taking Trikafta, my GI tract is likely going to be working more “normal” and therefore weight gain may happen easier. Which is good! And yet, I will be keeping an eye on it. I will monitor how the next several months go. My doc discussed with me a test down the road to see how pancreatic insufficient or if become sufficient. If enzyme dosage adjustments are necessary. That is wild…just mind-boggling!

A month ago, my FEV1 (the amount of air you can force from your lungs in one second) had revealed a decline again. On Wednesday, results revealed an increase! I’ll take any. It wasn’t the number I secretly hoped to see, but it is a few points shy from being back to my baseline. My Dr. is confident I will hit that number and possibly keep going. What encouragement and light to my heart and mind to hear him say that. There have been many times my FEV1 does not mirror how I feel. How I feel is what matters the most. The most wonderful part of my pulmonary function test (PFT) was how my lungs felt during. I could force the air out in that one second with more power. I didn’t sound like the penguin from Toy Story as the air was leaving my lungs. I didn’t feel winded at the end when normally I was certain I might pass out. I was able to take that quick deep inhale at the end comfortably. For me, this means everything. I thought to myself, “alright numbers, whatever you show it’s going to be just fine.” I felt relieved. Peaceful. This year, PFTs have given me elevated anxiety. Anxiety, if you allow it to, can eat you alive. I’ve dreaded doing them. I could tell my lungs were struggling with every attempt. It’s such a fearful mind thief. Hence hello uninvited anxiety. Perhaps now, hello peace of mind. Oh how I’ve missed you.

We discussed in detail all things regarding how I’ve been feeling since Trikafta. I shared with him that I hardly cough anymore. I purged within the first 24 hours of that first dose, a bit the second day. As each day has passed, now starting week 4, my cough has left me like a candle in the wind. This alone makes me smile and cry big, fat happy tears. When I do cough, it’s because I’m huffing to huff and coughing to cough to bring forth what I keep thinking will be more gunk. Just how is this? Over time, as Trikafta continues to filter all the tracts- lungs, sinuses, GI, kidneys, liver, etc…the tougher spots where mucus gets trapped (areas of scar tissue build up) should eventually clear out. My doc stated to not panic or be surprised if I have sudden bouts of more production as the medication is continuing to do it’s job. Yes please and thank you Trikafta.

A surreal encounter during my appt was my doctor’s reaction when listening to my lungs. I’ll never forget that moment. He chuckled and said “This is the clearest I’ve ever heard them. No crackles, wheezes, nothing.” Followed immediately with “Don’t stop anything. Keep doing all of your therapies, exercise, all.” The thought had crossed my mind, however I had not mentioned this to him. He informed me that during Trikafta clinical trials, those who participated were at maximal levels with therapies while taking the modulator. Therefore this reflected their outcomes. I want to have the best possible outcome. Nothing less. In addition, the years of damage to my lungs will not be reversed. Therefore, I plan to resume all therapies while taking Trikafta. I will do everything in my power to remain stable, to hopefully have less lung infections and less use of antibiotics. I have quite a resistance presently to many of them, which is frightening. I’m hopeful Trikafta will give my body a fighting chance without needing antibiotics as often.

Overall, I had a fantastic appointment. I am so grateful beyond words for a great clinic. My doc said come back in 3 months! I’ve been seeing him every 1-2 months since February this year. I wonder how things will be in a few months? Not only for myself but for all of us on this modulator. Time will tell. My spirit continues to feel renewed. I work hard for my lungs. Every day. I know my hard work all these years has paid off. Why do I feel that I don’t deserve this…change? I think this sometimes since that first dose. Those of us living with CF know that we must do all we can to stay alive. The progression of cystic fibrosis is inevitable. But now…Trikafta. It isn’t a cure. But oh how I want this for all of us. I won’t be stopping a thing. With each breath I take, I give my all in this and in everything. Life is so precious! If I am lucky to receive added years of living along with improved quality of life, that is the most wondrous gift I could ever be given.

One thought on ““Don’t stop anything”

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

%d bloggers like this: