Tuesday, September 20, 2016

One Year of Orkambi

This post was incredibly hard for me to write. Like all matters related to CF there was no black and white with Orkambi, for me at least. I wrote extensively on what the first four months were like, which were a rollercoaster to say the least. My purge was intense and utterly miserable if I am perfectly honest. Walking often induced shortness of breath that was so intense I wondered if I would ever walk again at times. In fact, sitting often brought on shortness of breath that left me gasping for air. At the four month mark my negative side effects disappeared and I continued on to have one of the healthiest winters in my recent history which I wrote about at the 24 week mark.

Was orkambi responsible for my winter health streak? I would like to say yes. If you asked me mid winter without a doubt I would have said, "Yes!!" Last winter, my first on Orkambi, was by far my healthiest winter ever. However, somewhere around June sickness struck again and I had three weeks of IVs, followed by weeks and weeks of oral and only now have I seemed to stabilize at a number that is still below my pre-Orkambi baseline. Would this last exacerbation be worse if I wasn't on Orkambi? Possibly. Could Orkambi be causing some inflammation that is keeping me from hitting my pre-Orkambi baseline? Potentially. So many factors come into play with CF that it can be hard to know exactly what is causing problems and what is helping. The progressive nature of the disease can complicate this issue even more. However, outside of several rough months this summer, I do feel that Orkambi has improved the consistency of my mucus and has made my lungs cleaner and clearer. I just can't quantify the way I feel and I have no real clinical data to back this feeling up.

Even though my lung function didn't improve like I had hoped and wished with all my might, my GI system has absolutely improved and that is unwavering to this day. Despite being sick for a couple months without reprieve my weight has remained stable. I no longer have days that I am so bloated my stomach feels stretched and painful. As long as I take my enzymes I really do not notice or think about my GI system at all. Of course, even Orkambi couldn't get rid of my CF belly, but as they say beggars can't be choosers and so I will take what I can get!


  1. Hey, even Kalydeco couldn't get rid of my CF belly! And didn't do anything for my GI issues - I think everything was just way too broken by the time I started it.

    I'm sorry Orkambi has been such a mixed bag for you. Crossing my fingers hard that something better comes along very soon!!!

    I've been thinking about you lately and how much I miss our chats about parenting and gardening and CF! <3 Do you e-mail? I'd love to e-mail back and forth with you!

    1. I miss our chats too. Since I gave up FB I actually check my e-mail now. I was so bad at that before! I will send you my e-mail address and would love to catch up!

      As for your GI system being to broken, that is how I think my lungs are. Sure, there is room for improvement, but I have never had good lung function (well, at some point before diagnosis, but who knows when) and through the years I have accumulated a lot of scar tissue so I am not expecting any miracles.

    2. Yay! I'll look forward to your e-mail. :) You were a teen diagnosis, right? I think that makes such a hard difference - everyone I know who was a late diagnosis but not, like, those super-mild cases who weren't diagnosed till adults, have had much decreased lung function because so much was lost before diagnosis. :-/ Ugh. I am glad that things are seeming more stable again.

    3. Yes, 14. On one hand I am so thankful I was able to survive 14 years with no CF care, no enzymes, etc. On the other hand it is frustrating that by the time I was able to care for myself I was already at a disadvantage! It is funny because the only other late diagnosis CFers I have met all have super mild cases so I don't actually know anyone in my same boat!