Saturday, March 5, 2016

24 Weeks of Orkambi

Two years ago I tried to get in the Orkambi trials, but after going through all the pre-trial testing I was denied. When I finally received the medication Sept 2015 I was curious as to how my life may have differed today if I had been the trial two years ago (and had the same dose medication that was approved by FDA which would not have necessarily been the case). Would I have been healthier with better lung function? Would my two years of decline and instability have been less "eventful"? There is no way of knowing how things would have been different had I started the trial 2 years ago, but I was still curious as to what my experience on the trial would have been like. So after starting my Orkambi I marked my calendar for 24 weeks (the duration of the Vertex trials) and waited.

I blogged about the first couple of months here if you want more detail as to the rough start I had with Orkambi. The biggest changes from the last 24 weeks of Orkambi:

1. Plugs!
I coughed out my first plug about 2 months into Orkambi and I had no idea at the time, but they would continue to come out at an alarming rate peaking at 4 months. There were days I would cough out up to four or five plugs in a single day. Around 4 months I started to lose sinus plugs at an equally alarming rate. There were weeks that I got either a lung or sinus plug and often multiple times a day! 
**For reference, prior to Orkambi the only time I would cough out a plug was during a round of IVs and these plugs were usually "newer" plugs that were more gelatinous and lighter in color. Orkambi plugs were dark and were so foul I often gagged as they came out sometimes verging on throwing up. They were clearly bacteria ridden and extraordinarily old. Good riddance!**

2. Sickness
I have had a rough couple of years. There was an entire year that every single time I started to feel better I would instantly catch a cold and be incredibly sick again. It seemed that once I felt well for a few days I would wake up the next day feeling a little off only to be sick with high fevers or completely bed ridden by afternoon. Sickness came quickly and often. I hardly left the house and bathed in hand sanitizer and was still constantly sick. I also ended up with reoccurring infections because my lungs couldn't handle the stress and mucus overload.

Since starting Orkambi I have had the first winter in a very long time that I was NOT SICK AT ALL! (Yes, I shouted that). What is even more amazing is that Kaylee and I have not restricted ourselves due to risk of getting sick like we have in the past. We continued storytime at the library, had regular playdates, and generally acted like normal human beings. And somehow I did not get sick! The most insane part is that I never even touched a bottle of hand sanitizer this winter. I ran out and never replaced it. Clearly, my body working more properly has allowed my immune system to function again (years ago I had a pretty amazing immune system that was so taxed the past few years it seemed to completely stop working).

There were 3 times over winter that I thought I was getting sick (the achy tired feeling is always a red flag that sickness is approaching). All three times I mentally prepared for a hospital stay because the last few years sickness always ended with IVs because my lungs just couldn't handle the overload. But unlike pre-Orkambi somehow I got over whatever was making me feel off. As in, I never actually got sick. This was such a sharp contrast from the past few years that the first sign of ache always left me feverishly shaking in bed within 24 hours.

3. FEV
So unfortunately, I have not seen an increase in FEV1. In fact, my last appointment I was down a little. However we are in the throws of allergy season at the moment which is my hardest season. My allergies present themselves as asthma and my lungs often feel like they completely shut down during allergy season. At this point I do not believe I will be one of those people with CF that see a positive change on FEV1. I won't lie I would absolutely love extra lung function and really wish I would have seen some improvement in that regard.

I am a skeptic when it comes to... well most things. I did not assume Orkambi would be a miracle and I did not assume every positive (or negative) change in my body would be from Orkambi. Our bodies are complicated and CF can be so unpredictable that it makes distinguishing correlation vs causation hard to separate. However, the hundreds of plugs I have coughed out in the past 6 months and the fact that my completely unstable health (especially in winter that usually left me a hermit for months on end) seemed to stabilize (for now) is enough to make this skeptical person confident that Orkambi has greatly improved my quality of life and I am so thankful it came into my life when it did.


  1. Hope this isn't too gross...but how do you know it's a plug that comes out? Does it look different than normal sputum?

    1. No question about CF is too gross to me :)

      When I refer to a plug I am referring to old mucus that has been in the airway so long that it is somewhat dehydrated and molded to the shape of the airway. They are usually the consistency of the inside of a jelly bean or even harder. For me, the more dense they are (and often darker) the older the plug is. Be glad you if you have never coughed one out before!