My Two Favorite Dorks

My Two Favorite Dorks

Sunday, June 29, 2014

Hearing the Right Thing at the Right Time


The Big Appointment

I wanted to talk a bit more about Thursday's "big appointment" with one of the head pulmonologists at Duke - big because this was the appointment which could have (and did) give me the final green light that I was ready for transplant. (Note: this is not the same thing as officially being listed yet.)

There were two really key things that happened for me there.

First of all, this nagging feeling I've had (and my mom even more so) that I'm not quite ready to be listed yet...how much I can do, how fit I am...was finally (I hope) abolished. The doctor said first off how it was strange to be arguing this side - usually people are anxious to be listed and Duke is telling them all the reasons to hold off!

He said that it was obvious by looking at my how proactive I've been over the years and what good care I've taken of myself (Aww, shucks!) A lot of people with CF get to transplant stage and they are in nowhere near as good of shape as I am. But don't be fooled, he told my mother, she's sicker than she looks.


He went on to explain. Everyone with CF will reach a tipping point - the point where you end up hospitalized, in ICU, your carbon dioxide retention goes way up, you need bi-pap or to be intubated... While getting lungs at that time is still possible, it becomes a crisis situation/race against the clock and recovery from surgery in your weakened state becomes much more difficult. I am personally hoping to avoid all of that if possible.

For me, the tipping point could be in three months or in three years, there's no way to tell for sure. But because of all I have going on, it has become too risky to wait around for that tipping point to come: I have started to retain a low level of carbon dioxide; my PFTs, while actually up 10%! have been on a consistent downward trend; I have increased oxygen needs; and I am on antibiotics a lot - now with the additional complication of needing to be desensitized to any drug I will ever need in the beta lactam family.

It suddenly all made perfect sense to me. And to my mom as well, I hope.

There's Just This One Other Thing...

There was just one more thing eating away at us. This week, the CF Foundation announced success from phase three studies of a gene therapy for my mutation. They hope to fast-track the medication through FDA approval and have it on the market by early 2015.

It's hard to hear there is something that could help you - or could have helped you - when you are getting ready to be listed for a lung transplant. It's even harder for my mother who has waited literally 32 years for something like this to come along.


I had to see what the doctor had to say about this. Could this help me? Could it mean putting off transplant a while longer?

The fact is, there are way too many unknowns still. We don't know when it would be approved for sure, we don't know how well it would work on people with severe lung disease (it's only studied in the more healthy population) or even how safe it would be. Upon closer examination, the trials did not even significantly improve FEV1 in the participants. With all that in consideration, the doctor said, I'm just too far along for this to help me.

Again, I knew that, but I just had to hear him say it. Before I put all of my eggs into this one crazy basket. I felt so much better.

And I really have continued to feel good about things since then. I am much more relieved, optimistic, even starting to look forward to what my new life could be like.

Look for more blogging very soon!


p.s. I will continue to keep my What's the Latest thread updated with any daily developments in the coming weeks.

1 comment:

  1. I found the CF Gene Mutations illustration the best explanation I've ever seen. Most interesting.

    ReplyDelete