Monday, August 3, 2009

Everything You Ever Wanted to Know about My Lungs (But Were Afraid to Ask)

So instead of random poetry and cryptic messages tonight, I thought I'd give you a general update on me and explain why my blogging etiquette may be a little wishy-washy for a little while.

If you've been following this blog, you'll know I had a lot of issues pre-Turkey, some of which were likely attributable to low potassium levels and some of which were really mysterious. My PFTs were down a bit from baseline and my cough was up, so we did a course of IVs, ordered just about every "just in case" script you could possibly need for a trip oversees (TamiFlu, Cipro, Levaquin . . . ) and even made sure I had the name of CF doctor in Istanbul. Then off I went.

The trip was phenomenal, despite the fact that I began running fevers and having severe soreness in my arms and legs about halfway through. Because I had started the cipro, though, I had managed to get a bit of sun rash even with SPF 75 (no joke), so I attributed the fevers/aches to a mild case of sun poisoning. I had pretty great energy aside from the pain though, and I spent the days sea kayaking, swimming, hiking, and generally tromping around the country, using O2 as needed. Basically I felt "better," despite the night fevers, sweats, and aches, than I had in a while.

Fast forward to coming home, where I more or less tanked. I was coughing up TONS of mucus despite being strictly compliant on my trip and was exhausted and really achey. Weirdly, though, my PFTs were up at 38%, which is very decent for me, but obviously a number isn't everything, so we started IVs, ran blood cultures, and tested for a virus called CMV, as well as flu and swine flu just to be safe.

Two days later I'm negative on all the viruses, I still have fevers of 101-102 daily, and I'm sleeping ALL day and sore as can be whenever I'm awake. At that point we noticed the low potassium levels so I got permission to just take advil round the clock and get the potassium back up to normal, in the hopes that this was a virus and would resolve as my body got healthy enough to fight it off.

No such luck. Two Sundays ago, in a last-ditch effort, I switched from Merrem to Imipenem, but by that Monday, after two weeks of fevers peaking at around 102.5ish, I went into the hospital, where I had every test under the sun and was found to have: 1) an enlarged spleen, 2) anemia, 3) low potassium (knew that), 4) elevated liver enzymes, 5) some residual pneumonia (remember, this is 2 weeks into IVs), 6) a collapsed upper left lobe (and okay, that last one is no big deal for me because it seems to happen intermittently and resolve itself), and 7) a fever of 102.6. Surprisingly though, after the initial day in the hospital, my fever never spiked above 99.5 again. My pain started to resolve and things were looking up. All blood cultures were negative and all virus/parasite tests came back clear. Infectious disease wanted to pull all my abx to get a blood culture without any drugs in the system (which might mask the problem), but my CF doc wasn't comfortable with that so I remained on tobra and imi. I also had an echocardiogram to rule out a heart valve infection. This was, of course, in addition to the two CT scans, abdominal ultrasound, and various other tests (mostly blood draws). They also tested my sputum for pretty much every weird bacteria under the sun, and so far no dice (except that I did learn from infectious disease that I have "dozens" of pseudo strains in addition to my staph and achromobacter -- lovely).

Okay, so no fevers in hospital = jailbreak, right? YAY! I got out last Thursday, went home, had some peanut butter, took a nap, and woke up with a fever of 100.5. No joke. Plus I had some weird rash all over my legs, so of course I called my doctor. The end result: benadryl for the rash, keep an eye on the fevers.

I ended up back in my clinic today after running daily fevers of 100+. Here's the deal, the fevers appear to be getting milder. The red splotches all over my skin aren't too concerning. My spleen, potassium, and liver are back to normal. We think this might be FINALLY resolving, EXCEPT: I still have fevers, I'm still sleeping all day, and I still ache like nobody's business. We ran more blood cultures, she wanted to admit me, I asked if it was really necessary since I have someone living with me right now (shout out to my awesome godmother, who flew in from CO to take care of me!), and she said it was fine to stay at home where I'm more comfy.

Here's the plan: IV abx through wednesday and I can take tylenol to break the fevers. Starting inhaled colistin. Discontinuing all oral abx including zithro. This way, IF the fevers persist past Wednesday and the stop of the abx, we can draw more blood cultures without the risk that the abx in my bloodstream are hiding the infection. And then possibly my port will need to come out.

The good news? We've eliminated literally everything they know of that might be super serious. If it is a blood infection (please pray it's not), then it hasn't spread to my heart valves, which is fantastic. It's entirely possible this is just a virus they don't really know much about, similar to mono, and it may even BE mono except that I had it years ago so there's no way to definitively test me for that now.

The bad news: if the fevers aren't gone by Friday, viral or not, I need to go back into the hospital and wait this out. And if they truly can't find any cause they may remove my port just to be safe, which is annoying as hell. Then again, seeing as I've had the thing for 9 years there's actually a good chance it's part of the problem -- they rarely last that long without some sort of issues. I'm really hoping if they remove it they'll allow me to get it back in my arm; my doctor promised to work on that.

The awesome news: I'm still at home with my puppy and typically only having one fever per day. Once it breaks, which it does with rest, gatorade, and tylenol, I've been cleared to do some light walking to keep my strength up and regain some of my lost workout time. Nothing serious here, obviously, but honestly I never knew walking a shorkie down a crowded lower-manhattan street could be so much fun. And tomorrow I'm going to meet my friend at the best pizza bar on Wall St. for lunch, assuming I'm up for it, so life is slowly, slowly regaining SOME sense of normalcy. AND, despite the collapsed lung that hurts like the devil everytime I breathe in (you get used to it, believe me) I suddenly have O2 sats that actually reached 97 on room air! They're hovering around 94 now all the time, which is unbelievable, amazing, wonderful, and beyond all words. I can't wait to see what my PFTs are when this is all over, considering they started at 38%.

Alright, sorry for the symptom dump. I just thought I'd fill everyone in just in case I go AWOL again, or on the off chance any of you were torn to pieces by my sudden lack of fun updates. I'm pretty sure there's more fun to be had though, even if it does have to wait it's turn through all the madness.


  1. Hang in there Cyster! 97% pulse ox! Sweet!

  2. Man you have a lot going on! I would kill for 97% on room air, so keep up whatever you're doing :) That stinks about a possible port removal....could your body be attacking? Is it possible to reject a port? I know literally nothing about them.

    I'll be thinking of you.


  3. Wow Piper! I wish you were feeling better and rid of the fevers. It sounds like your doctor is totally awesome and 250% on top of things... as are you of course!!! I hope you can avoid lockup and if it is the port that's causing the issues that you'll bounce back lickedy split!

    And hey, no need to apologize for your blog tone or lack of posting or anything. Don't go adding any sense of obligation on yourself or anything silly like that. We luv you just the way you are! It's the real PIPER package! :D {{Hugs}} ~Juliet

  4. Hi -- de-lurking to (and, oh, I really hope this isn't too annoying or creepy) say, first, I'm so sorry you're feeling crappy, it sounds like it has be a hell of a year health wise (to put it lightly) and, well, I hope you start to feel better soon.

    And, second, have they tested for EBV? I'm sure they have; your docs sound great. The reason I ask is that Will, my partner, who also has CF and is post tx had a number of the same types of symptoms post tx (fevers that wouldn't go away, body aches all over, enlarged spleen) and they turned out to be a run away EBV infection. It was the enlarged spleen that really got me thinking about it, since that is common with EBV. (We watch EBV really closly since those infections have a direct correlation with Will's two bouts of PTLD and one bout of Hodgkin's lymphoma, but I think the cancer/EBV connection is strictly a transplant-related thing owing to the new lungs being EBV positive for EBV antibodies when Will didn't have any EBV antibodies pre tx.) So, sorry if this has offended in any way and really don't mean to meddle, just to offer this experience in case it helps in any way. Best,

  5. I wish I had something more constructive to say. I don't. But I wanted to leave a comment anyway, because I wanted you to know I'm thinkin' of ya and always look forward to your blog posts, be they happy, sad, frustrated, poetic...; I like 'em any which way you deal 'em!

  6. Can we be anymore alike??? My PFTs were up when I went in the hospital and I was having fevers of 102+. We knew it wasn't because of a PICC or Port because I didn't have one. I am on my last few doses of IV antibiotics and guess what...fevers again. I hate this cycle. My thoughts are with you.

  7. Might sound like a shitty way to go but I hope its the port and you can get it removed, clear the fevers, feel normal and then get another one in the other arm :)

    Sending vibes up to NYC from Philly :)

  8. I answered your question under the comments Piper! Some other might find the info useful, otherwise I would have just emailed you.