Cue the Scary Music

Well, I got the news yesterday. That's right, THE news. The news I've been trying to pretend would never come, only yesterday it did. And apparently that means it's time for the final transplant evaluation test, the one I've been putting off for as long as as I possibly can...the dreaded right heart catheterization.

Yuck.

Seriously, I'm really NOT looking forward to this. At all. I don't entirely know why, except that it sounds like kind of an invasive and scary test to me. Oh yeah, and there's also one other tiny little detail that I may have so far failed to mention on this blog...

See, in addition to CF, I also have another handy little genetic "mutation" of sorts that also happens to have a convenient little acronym: SVT. SVT stands for supra-ventricular tachycardia, and it basically means that I have an "extra" current pathway in my heart that, when triggered, causes my heart to beat really, really fast. Actually, race would probably be a better word, and even then it's more of a sprint. We're talking 260+ BPM here. Yeah, fast.

I'm really lucky SVT-wise, though. I have about 2-3 episodes per year, normally brought on by some sort of very rapid, exerted effort on my part. Actually, in one of the great ironic jokes of the universe, the most common trigger for me is pulmonary function tests. The second is definitely coughing fits. Awesome. But, like I said, I'm lucky, because while I have a couple of episodes each year, they never last more than 30 minutes or so and I've never needed medication to stop them (knock on wood!). Instead, I just have to let it go for a few minutes while relaxing my body, and then bear down hard and put a little bit of pressure on my heart. I do this by biting my thumb and contracting my muscles as though I were "blowing out" without actually letting any air escape. Once I do that a couple of times, it typically resolves.

Actually, on an interesting sidenote, the first time I ever had an attack I was in the hospital for my CF and, you guessed it, had to blow PFTs. When I did so, I told the PFT tech that my heart felt funny. She put a pulseox on my finger, freaked out, and called cardiology to come ASAP to the PFT room of all places. They had me sitting there, half naked, hooked up to the portable EKG and a heart monitor which both showed my heart just racing out of control. I was, of course, totally freaked out. Then in strides this suave looking cadiologist (head of the dept, of course), and he glances at me and says "Piper, I want you to take a deep breath and then push down like you're trying to have a baby." Weird instructions to give a 21 year-old, for sure, but I did the best I could given the circumstances. And then, all of the sudden, my HR literally plummeted in two seconds from 250 to about 130, and I could see thw whole thing on the monitor. It was totally surreal and totally amazing to "watch" - freaky as the whole thing was. And that was my introduction to SVT.

Anyway, back to the right heart cath. I've definitely told my tx team that I have SVT, obviously, and they've assured me that it should be fine and that people with SVT normally have no problems with the heart cath. And I've also been lucky enough with it that I'm not too scared even if the heart cath does carry with it the risk of an episode, because logic tells me I should be able to stop it as I've always done before. And even if that doesn't work, well, at least I'll be in a hospital, right?

But I'm still really nervous. I don't want anyone messing with my heart, I don't want doctors to go in there and make it angry, and I don't want to have to worry anymore about the weird way that CF and SVT interact. I know that's a little babyish, because CF is constantly interacting with the rest of the body and some people have it much worse than I do in that respect, but it's still a valid complaint. It both amuses me (in a very tongue-in-cheek kind of way) and pisses me off that all my SVT issues seem directly tied to CF (e.g., PFTs, coughing fits, stupid right heart caths). I know CF didn't cause this extra current pathway, but it sure seems to irritate it.

My appointment isn't until May, so I have some time to prepare myself. I think I'm going to email my transplant doctor and ask some very specific questions about SVT and heart cath. I'd certainly rather know the risks going into this and be aware of everything than not. And I want to remind him of what he's dealing with, because I don't want him confusing me with some boring, run of the mill CFer!

Just kidding guys ;)