Thursday, January 8, 2015


I have learned to expect the unexpected during our CF clinic visits. I suppose that is why I brace myself for days in advance of these appointments, slowly becoming more anxious and more agitated. Something surprising almost always happens, and the surprise is usually not a good one. It's kind of like cranking the handle on a Jack-in-the-Box, waiting for the creepy looking clown to pop out. You know it's coming, and you cringe in anticipation of the moment you will inevitably flinch. It never fails.

Today was no different in the surprise department. Today's surprise, however, was not bad. It was very, very good. Perhaps even too good to be true. I'm still not sure how I feel about the news we received today. I guess it will take some time to process.

As many of you know, Kalydeco was recently approved by the FDA for use with CFers having at least one copy of R117h. Based on previous conversations with doctors at the CF Clinic, I was under the impression Caleb would not be considered for this drug because he is doing so well and the long-term effects of Kalydeco are still somewhat unknown.

I was wrong.

When Caleb turns six, he will begin taking Kalydeco. The benefits to him far outweigh the potential risks, and he will start using Kalydeco as soon as he is old enough (right now, it is only approved for ages six and up). There is a possibility he'll start sooner; a safety study is underway right now on younger kids.

Kalydeco, along with regular daily treatments, is significantly slowing the progression of CF in people for whom it has been approved. It makes total sense for Caleb to begin using it as soon as possible, before the disease has an opportunity to create permanent damage. Even though Caleb appears to be completely healthy on the outside, his most recent chest x-rays show some evidence of disease progression. It seems to be in his best interest to begin as soon as possible and delay any further damage. There are still so many unknowns, so many unanswered questions, but so many, many, many benefits. It boggles my mind.

Any words I could use to describe how thrilled I am with this news would be woefully inadequate. I am also scared about long-term side-effects we may know nothing about. I can't believe this is happening. It is a very real possibility this will have a life-changing positive impact on Caleb's health. For some reason, I'm unable to breathe the sigh of relief I thought would come in this moment. 

Thank you for all of your love, support, prayers, words of encouragement, and hugs. I have needed every one. I will continue to need them as we travel down this new "leg" of our CF journey. Please continue to pray for Caleb, for the wisdom of the people making important decisions regarding his health, and for the rest of our family as we navigate these uncharted waters. It's scary. It's exhilarating. It's unbelievable.

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