Wednesday, May 9, 2012

Where I've been...

To all those who are following my two previous posts with great interest, I am so has gotten a little bit complicated.

Ugh.  Where to start.  6 Weeks into my AMAZING opportunity with Kalydeco, I got a not so amazing letter from my insurance saying I would only be able to fill my Kalydeco for a few more months and then at that time there would be a "review"----in other words, "denial :)"  Wow.  So thankful for the chance, but kinda pissy the old insurance company ripped it away.

So, after I picked myself up off of the floor, I went into prevent/defense mode.  My doctor told me to stop taking it immediately :(  So sad... it is also so hard to know something is helping and have it go away.  I have been off of it now for three weeks and my PFTs have fallen back down :(  So, I was staying in the 1.55-1.58 range (on Kalydeco) and it promptly fell to 1.42 within two weeks...  I'm not testing it right now because I have NO DESIRE to get too down about it when I know there is something that can help.  Another lovely that is back is my SUPER SALTY SKIN.  Hubby pointed it out only one week off of Kalydeco...  All the moisture feeling that I had is moisture in the nose, mouth is dry again, less saliva.....:(  I have never produced a lot of mucus and my cough is not normally very productive, but I can definitely tell I am no longer on the drug because my lungs feel tighter and it is harder to get anything up.

Our plan as of right now is to get a sweat test in the next few weeks (four weeks is the magic number).  At that point, my doc and I will decide what I need to do.  He is CONVINCED that insurance is going to decline it for sure...  We are deciding between restarting it and getting as much data as we can for the next several months (sweat test, pfts etc.) or holding onto it for emergency.  Summer is normally my healthy season and he is wondering if we hold off until fall when I get an exacerbation...

I can't begin to describe how exciting, hopeful and completely defeating this whole exercise has been.  Since Kaly isn't made perfectly for my splice site mutation, it isn't my cure... just isn't.  It is, however, the closest thing to a control that my body has ever had and having it be ripped out of my life is pretty devastating....  The only thing I didn't experience was weight gain....everything else was pretty great.

I am praying that somewhere they will do a trial for those of us with these splice site mutations.  There are three of us (that I know of) who have splice site mutations and have had significant improvements with Kalydeco.... by significant I mean 10% to 15% increase in FEV1.... ugh.....why is it taking so long?????

So, no more blogging about my life with Kalydeco for atleast a few more weeks....  I will, however, blog about diet as a few people have emailed me about it...


  1. Thanks for the update, Mandy! Great post. We all have to stick together and get the word out about splice site mutations and Kalydeco. We are still getting a "no" from insurance as well. Looking forward to your diet post!

  2. Thanks so much for the update!! :)

  3. Oh my god so it did work on your mutations?
    Before getting excited off my tits about this because I have the same mutations, I have 2 questions to ask.
    Did you change the way you were doing your other therapies? Are you sure that you didn't get caught in a kind of rush of motivation to work better and harder to get better with the help of the drug?

    If I want to get Kalydeco, I will have to make a strong case to my pneumologist.

    Looking forward to your reply.

  4. As others have said, A HUGE THANK YOU for keeping us updated!! I also have the same mutations as you; tried to get my doctor to prescribe kalydeco but he was admamant it would not help me. Being able to point to some concrete measurements can only help me in the future try to change his mind. I am sooo glad to hear that it has helped you!!

    HOWEVER, I am really really unhappy that your insurance is interfering with this treatment though. It stinks (If I was not writing this in a public forum I'd use a much stronger word) that you feel so down. I'm hoping you and your doctor can make a case for continued approval.

    HUGS - Catchastar