Symdeko Update

I have been on Symdeko for 10 weeks as of today!! 10 weeks!! I meant to write an update at 4 weeks, but time got away from me as it often does during the summer months.

The background

I feel like I should give some background information that might be important for someone looking for similar experiences. I should mention I was on Orkambi for a few years prior to making the switch. Starting Orkambi was brutal to say the least and it took about 3 months of Orkambi before I felt human again. After I adjusted I feel like I gained stability, but saw no improvement in my pfts. In fact, I felt like my baseline was a bit lower after starting, but because I was more stable I felt like it was worth the tradeoff. At the very end of my Orkambi experience I was hospitalized with my lowest lung function 29% (although I number I had seen before). 

Two weeks after ending IVs I started Symdeko. This made determining how well Symdeko was working tricky because I wasn't sure how much Symdeko was responsible for and how much was the result of being freshly off IVs. 

Starting Symdeko

The first week I noticed I had a bit of a headache, but nothing unmanageable. I also noticed my digestive system was off. I couldn't really pinpoint what was off except I went from being extremely regular and feeling fine as long as I always took my enzymes to having complete inconsistency with my bowel movements. This lasted maybe a week (and potentially could have been related to being off IVs recently. Although that usually makes me have loose stools which wasn't really the issue here).

The First Month

The first month I recorded my PFTs on a calendar (with a home meter) and I watched them steadily climb. 1.13, 1.19, 1.20, 1.25, 1.28. Part of me felt it was too good to be true and was a fluke with my meter and part of me was so hopeful that this was a sign that Symdeko was working. I did have some streaking of blood in my sputum around week 3 which is really rare for me and was somewhat concerning, but it resolved quickly.

First Clinic

My first clinic since Symdeko was around the 8 week mark. This was 10 weeks after finishing IVs so I was no longer "fresh off IVs" and so I felt at this point my pfts would be a pretty good indication of how well Symdeko was treating me vs a temporary high due to IVs. 

Guess what? My PFTs hit 40% for the first time in YEARS! 40% has been my goal for so many years now and I had 100% given up on every hitting the 40s again. But 2 weeks ago at clinic I totally hit 40%!!! It felt so surreal and was all the proof I needed that this drug was working!!

And that isn't even the best news. I was at the park the other day and for the first time in a very long time I had an overwhelming desire to run. RUN? Yes, run!!! Because when your lungs are functioning at 20 or 30% the idea of running or even speed walking feels so impossible that the thought never even crosses your mind. But when your lungs feel amazing and clear and so much more open things that you never even conceived possible actually seem doable and show up on your radar. And so at my suggestion my daughter and I ran across the field at the park and then we ran all the way back. And you should have seen the look on Kaylee's face. This was something we never experienced together before (or at least in her memory) and she was soaking up every minutes with the biggest smile plastered across her face. That night when I tucked her in bed she said, "mom, it was fun running with you today." And it shattered my heart that I spend 6 years unable to run with her in the way a young mother should with her child and yet it filled me with hope that now I am capable of running along side my 6 year old through the park. It gave me hope that I will be around to run with this precious girl for many more years to come. 

Now I feel even more impatient for the triple combination to come out so that all my fellow CFers can experience better numbers and a run through the park. Because we all deserve that at the very least!

One Week Of Symdeko

I received Symdeko 2 weeks ago, but when I received it I still had some Orkambi left. I was desperate to try Symdeko, but also felt guilty that I was going to waste Orkambi when so many people in the world still aren't able to get Orkambi (or any other meds to restore CFTR function). I also was having flashbacks to the time Acreedo messed up because they couldn't figure out how to bill my insurance (the same one they had been billing of rover a year) and I ended up missing several doses of Orkambi. I decided the responsible decision was to finish out my Orkambi which would allow me to have a few extra Symdeko tucked away in case I ran into a similar issue.


I am about to finish my first week of Symdeko and HOLY CRAP this drug is so much easier to take than Orkambi. You guys, I have continued to live my normal life for the past week! I can walk and jog and be a totally normal human being!! No gasping for air or aches or fevers, no elephant sitting on my chest! I am not terrified that I am digging myself into an early grave by taking this medicine the way I did with Orkambi. No MONTHS of torture before any reprieve. This is AMAZING!


I plan to give a one month update when I get there. I am noticing some things that make me believe that I am doing better on this medication than I was on Orkambi, but we all know how varied each day can be with CF and I want to have a better sense if these symptoms are flukes or actual signs of improvement before I write about them. Overall, I am very happy with Symdeko and I don't think I will miss Orkambi for even a second (although I am forever grateful I was given the opportunity to use orkambi for the past few years).

Symdeko

The last time I updated I was in the middle of a 2 week (turned into 3 week) course of Zosyn. Without too many boring details I went in after 10 days of meds and my lung function only climbed a bit. I went from 26% in clinic (dipped to 22% in the hospital, BUT I hadn't had my bronchodilator and I have horrible asthma and basically can't breathe without it so I totally don't think that one counts) to 28% in the ten days. A 2% increase is absolutely crushing because it isn't clinical significant. I felt totally defeated and super frustrated that the hospital and IVs were all a waste. We agreed to give it 14 days and I also added Bactrum since some of my bugs were sensitive to it. Around day 13 I actually started to feel better and called to extend my IVs to 21 days.

I just went into clinic today (28 days after starting IVs and a full week off of IVs) and my PFTs were 36% which is basically my baseline. Whew! I am so relieved to have improved so much. It just proves to me that my body really does need longer doses of IVs.

Getting back to baseline came at a perfect time because I am getting Symdeko tomorrow!! I wasn't overly optimistic I would have the opportunity to try Symdeko because it is more expensive that orkambi and I didn't have confidence my insurance would approve it. Somehow my insurance did approve it and I am starting soon! I am so curious to see if I feel any different between Orkambi and Symdeko.