Sunday, April 19, 2009

Too Late or Too Early?

It's really, really late. Or else it's really, really early - just depends on your viewpoint. Either way, I probably shouldn't still be up, but my IV schedule coupled with a poorly pressurized intermate that took double the time to infuse, on top of an already somewhat late night spent at a fabulous restaurant in NYC enjoying some of my new-found (re-found?) health, all add up to a late night. A night that will, unfortunately, probably give way to an early morning b/c I still have to infuse the imipenem every 6 hours. But it's okay, I'm not complaining.

Life is worth it, right?

Being out with friends is worth it, going to see the band play last night was worth it, walking all the way from my TriBeCa apartment to the dogrun in Chelsea this afternoon just because it's cooler and I thought my puppy would have more fun was worth it. Not bothering to check my O2 levels during said walk because it was 70 degrees and sunny and I was out with my puppy along the river and just didn't want to think about CF for a couple of hours was with worth it too. Totally.

So I promised an update after Thursday's clinic appt, and I know it's a little late in coming. There was, to be honest, a lot to digest, and I'll probably be taking a little break from health blogging for a while as I just kind of sort through it all. But for right now I'll give you the bare facts as I know them:
  • FEV1 back up somewhat, at least to a level that didn't scare me completely
  • IVs until next Friday
  • Off work for a while -- have to recover and give my body a chance to get back where it needs to be and . . .
  • My doctors, both CF and transplant, feel it's time for me to go active on the transplant list
Yeah, that last one was a little bit of a shock to me too, especially that particular day. I just wasn't expecting it at all, which is a little strange considering a couple of months ago, in January, I was sort of questioning why exactly we weren't moving forward. But right now, I dunno . . . maybe it's because I'm on IVs right now and feeling pretty healthy, but it just kind of came out of nowhere for me.

No wait, scratch that. Out of nowhere would probably imply that I don't get my doctor's reasoning here, which isn't true. I get it. I get that I've been on prednisone at fairly high doses for several months without any sign of being able to successfully wean off it. I get that my BMI is way too low and I'm not really gaining weight. I get that my O2 levels are getting progressively lower, and that the last couple of infections have sent them plummeting into the mid-to-low 80s. I get that a week ago I was in the hospital sating 89 on 5 liters of oxygen. I get that in the past 6 months I've stopped responding to some of my "best" IVs. I get that I had 5 rounds of IVs in 2008. Most of all I get that this is already my third round of IVs in 2009, one of which lasted for 6 weeks, and it's only April. Believe me, dear God believe me . . . I GET IT! I am one sick little cystic, and nothing I do seems to be changing that fact.

But all those things are really only part of the story. The other part is that I still feel happy (most of the time) and I still feel like ME (almost all of the time). I still run, I still leave my house everyday and do pretty much whatever I need to get done, and I still go out and have fun with friends. I don't feel like I can't do those things, at least not when I'm healthy -- which is, admittedly, not nearly often enough, but as soon as I start the IVs I tend to bounce right back. My baseline FEV1 after IVs is higher than the level at which most CFers are even referred for evaluation. Most of all, I'm just stubborn. I don't want to be here, at this point. I want to do something magical and miraculous and suddenly have the infections stop and my FEV1 shoot up to 50% and have everyone stand around laughing and saying "wow, Pipe, you really had us worried there for a second!"

Anyone know how to make that happen? Please?

I don't want to wait so long that one of these infections becomes life threatening. I also don't want to give up on my lungs -- which, despite their CF and their crappy mucus-filled airways, are still after all MY LUNGS -- before I have to. I totally understand that I'm not a black-and-white case, that for some reason my silly brand of CF has taken textbook progression and decided to see it as a challenge to beat, but still . . . I'm only human, and no human particularly wants to have to face these facts, right?

My doctor called me "stoic" about CF -- she said it was easy to get a false sense of security from looking at and talking to me, that you would never guess. She apparently hasn't read this blog ;)

I have some questions. I need some answers. And then I'll make a decision with the help of my amazing medical team (who I trust COMPLETELY) and that decision will be right for me right now. I don't care if that sounds sappy and trite, I'm still stubborn enough to believe that it's true.

Did I mention it's late? Or maybe it's early.


  1. Sending peaceful vibes Piper.

  2. You will figure it our Piper. Talk through it, write it out, do what you need to do to decide if you want to be listed right now.

    I'm excited for you to get new lungs and be able to breathe and through all this BS behind you that you have endured these last months/years...but I'm also nervous for you since it is a major surgery...and hell I'm JUST a cyster! I can't imagine what it is like for you!!!

    We love ya Piper and I'm sending hugs and love to NYC for you!!!!


  3. I had the same problem for a long time. On paper I seemed really sick but to look at me you would never know and I think that was part of the reason it took me SO long to be ok with tx. Last year I had a lot of hospitalizations too and have already been in the hospital once this year and the way my track record has been I won't be surprised if I am back in by the end of may! I am just SO ready to feel good and get back to my old self that I finally stopped being scared of tx and I am actually looking forward to it being over and done with! Being sick all the time has never been me so I don't like it very much and I know you don't either! :) You and your doctors will decide what is best when the time is right and you will know when that is. I was just you sleep with O2 or use it at all?

  4. Hey Piper:

    I don't have any magical advice, but believe me I wish I did. You seem like such a cool girl with a great life. It must be shocking to have your doctors tell you that.

    Can I ask what your FEV1 is and how long it's been there? The only reason I ask is because I met a tx cystic recently that was in the FEV1 of the 30's for 10 years before her transplant.

    I think it all depends on the quailty of life aspect. Do you want to live with your lungs at the quality of life you have now and work on getting better? Or do you want a "guaranteed" increase in quality of life but take on different battles and possibly a shortened life? I know it's tough and I'm sorry that you're in this position.

    I'll be praying for you.


  5. Piper,
    I enjoy reading your blog- you have a great sense of humor, a cool writing style, and a really interesting life! I was really surprised to read you might be “activated”. Even though you have had a rough year, you were working up to a couple of weeks ago, you are not on oxygen full time, and you still are living a life. Transplant is for when you have nothing to lose. Although your doctors are great people, I would question how much experience they have really had with advanced CF patients. It sounds like they are frustrated and have run out of ideas. Think about getting a second opinion, trying different drugs next time you are sick, maybe go on a study drug, change your environment, and maybe take a vacation. Once you are on the active list, you could get a call anytime, and it doesn’t sound like you are at that place yet.
    Good luck!

  6. Piper-
    I have been reading your blog for quite some time. Your personality reminds me of my Cory.

    You asked if it was worth is. It is all worth it. Life is aall about creating memories and that is so much more important for people with CF. They have to make those memories while they can, before the next hospital visit of just the "feeling like crap"
    I will you the best and I am saying lots of prayers that you get a call for a new set of lungs very quickly

  7. Being listed is some serious scary shit. I hope you're able to find the peace that I eventually did with the tx decision. It was the best thing I've ever done, but I know it isn't for everyone. If at all else during this time, never give up doing things that you love. Be you, walk your dog, go out with friends... you know, enjoy life :)

    Just know that you have a ton of people pulling for you!

  8. You sound a lot like me prior to transplant. I was still doing normal everyday things until I got sick. I tended to bounce back, but it started to take longer and longer...and when I got sick, I was sicker.

    How are your sinuses looking? Have you had issues in the past? My first sinus surgery was a requirement of the lung transplant...I was 15. I did very well for 4 years after that first surgery and then had a second at 20 I was able to be be "put on hold" for the transplant because I was doing so well. Just food for thought.

    I think i've said this before...forgive me if I have...but my docs always said the healthier you are going in, the quicker/faster/easier the recovery. But, you don't want to do it too early either. It's just a big catch 22!! Hang in there and we're all here for ya'!