The More Things Change...

. . . the more they stay just enough the same to drive you crazy.

December, 2009: Picture of dressing from port-a-cath #2.


July, 2011: Picture of dressing from port-a-cath #3 (in the exact same place!).


Happy belated 4th of July, beautiful people! As you can see, I've been a little busy, but I promise to be back soon.

xoxo,
Piper

And When It's Over, It's Over

Friends, family, beloved readers, today we gather to say a collective goodbye to the disaster we have all come to know as the "Great Port Debacle of 2009." True, it made for some amusing blog posts, but all in all I doubt that any of us will be too sad to see this one go -- I know that I for one am not shedding any tears. Thankfully, like all things good and bad, it is over when it's over. And this one is officially, 100%, fat-lady-has-sung-and-is-now-recovering-backstage style over. Feel free to applaud.

And, as often happens when one journey ends, another has just begun. A very exciting new journey, in this case. This very morning at approximately 8:30 am, I welcomed a new arrival into my life. Or, more specifically, I guess I should say I welcomed a new arrival into my chest. Ladies and gentlemen, I'm proud to introduce to you . . .

. . . my new chest port.

Seriously, isn't it lovely? It sits so high on my chest for a couple of reasons: 1) because it is threaded into my jugular vein in my neck, and 2) so that the transplant team won't have to remove it during surgery. It also sits off to the side somewhat, so that in actuality the port sits about three inches above my right breast and about an inch and half maybe from where arm meets my chest. This means that even accessed you can't see it wearing regular clothes, even a v-neck (obviously tank tops would be the exception to that rule). Right now it's accessed so I can finish up these IVs, but they also sorta loaded it up on gauze and whatnot, so normally when accessed it won't look quite so big. I'll be sure to post a pic once I remove the dressing and everything as well.

I cannot even begin to explain how happy I am to have a port again! Even right up until last night I was having some second (and third, fourth, fifth, and sixth) thoughts. I just really didn't want any more complications or chances for infection, and I wasn't entirely thrilled at the prospect of facing the OR again. (Sidenote: ports are often inserted by interventional radiology, just like a PICC -- which is how my original port was inserted 10 years ago. This time, due to all the complications with the removal of my last port, the fact that it took over 3 hours to place my last PICC, and the clot complications that they believe were caused by trauma due to difficulty with placement, everyone thought it would be safer to just have a vascular surgeon perform the procedure in the OR. Thank you, Columbia Presbyterian vascular surgery team for being so wise, helpful, and downright amazing!) But despite my misgivings, I can honestly say that waking up at 5 am to travel the 168+ blocks to my hospital was the hardest part of this whole ordeal. And as I sit here now, enjoying the flow of antibiotics into my system with absolutely no peripheral line in site and both hands free to type without pain, I am already SO grateful to my doctors for pushing me to try again on the port front. What a huge relief!

I am going to have to go on blood thinners again just to make sure there are no clot issues in the first couple of weeks. Small price to pay for sure. And as for the pain, so far there's not much. The lidocaine is just now wearing off though, so I've gone ahead and filled my vicodin prescription just in case I need it once the numbness fades. So who knows, it may be a very "merry" Christmas for me!

And finally, since I know you're all waiting on the trademark Piper ridiculousness that so inevitably accompanies any medical procedure, no matter how big or small, here you go:

Anesthesiologist: Okay, so you're getting a port?
Piper (still fully lucid): Yes.
A: So you'll need us to start an IV now for use during the procedure?
P: Oh no, I have this one that the homecare nurse put in for me.
A: We don't love to use lines not inserted by the hospital.
P: Please please please? I promise it's good!
A: Are you sure?
P: Yes, definitely. This one is good, I used it at midnight last night and it was fine. In fact, it's been working so well maybe we shouldn't even put in the port. (laughs)
A: (Also laughs) Okay. I'll just hook this up and cancel the procedure.
*A hooks up IV drip to peripheral IV*
*Silence*
Nurse: Um, doctor, I don't think that IV is dripping. Are you sure the line is good?
A: (cracking up) So maybe you'd like that port now, young lady?

Yeah, that's right, I blew a peripheral while in the OR to have a port placed, but obviously not before insisting up and down that it was a great line. Sigh. Oh well.

At least it's over.

Peripheral Vision

Okay, so if you've been following this blog at all for the past few weeks/months, you're probably familiar with the event that I've now termed "The Great Port Debacle of 2009" and its subsequent less-dramatic, but equally annoying, little sibling, "The Not-Quite-S0-Great PICC Clot Fiasco of 2009." These two events happened within a few weeks of each other (although the Port Debacle had, in fact, been going on for several months prior to its actual diagnosis) and made for a very exciting, if somewhat over the top, end of summer/beginning of fall.

Ah, memories.

Anyway, those days are long gone, and, like any good CFer, I've pretty much moved on to the next big thing at this point. And that thing just happens to be . . . (cue the drumroll please) . . .

peripheral IVs.

View of my arm, circa 1989 (er, I mean yesterday)

So call me old fashioned. Call me old school. Heck, call me retro if you must, just please don't compare me to the Lower East Side Hipsters when you do it. The point is that my veins and I have been partying like it's 1989 for the past week and half or so, and weirdly enough, it actually hasn't been too bad. I mean, aside from the hair bands, the teased bangs, and the Saved by the Bell reruns, we've been managing just fine during our little trip down memory lane. In fact, to date I've had only 4 peripherals in 12 days, and considering that one lasted less than a full day and hardly counts, I'd say that's a pretty decent record. Especially when you add in the fact that most of the placements have been home runs, meaning the line is placed on the first try. Actually, scratch that -- it's not just "pretty decent", it's all out amazing.

I'm pretty sure that I owe this miracle of vein cooperativeness to my 9 1/2 years of portdom. Prior to getting my port, my veins had pretty much put out the "closed for new business" sign when it came to either peripherals or PICCs. Rolling, spasms, collapses, general refusal to give a blood draw -- you name the game, my veins have played it. I'm pretty sure I used to hear an audible groan when I walked into the IV lab for my placements, and nurses have been known to change shifts just to avoid my "veins of terror", but try telling that to my new awesome home health nurse, Janice, and she'd probably just laugh. Because as far as she's concerned, 9 years later, my veins (while perhaps a bit overused and overtired) are nothing more than petulant children waiting to be coaxed into line. What a difference a decade makes, I guess.

There's a lot of debate raging right now amidst my doctors about whether I should remain catheter-free or go for another port. Pre-clot we had agreed on a course of action, but now it all seems up in the air. The thing is, though, I'm suddenly a LOT less anxious about the prospect of going into transplant and beyond without a permanent IV line, now that I know my veins have managed to resurrect themselves to some degree. And it makes me, in retrospect, all the happier that I got the port placed when I did, because I'm thrilled that I managed to save at least some use of my veins for later -- after all, as permanent as a port might seem at the time, you never know when you're going to lose it.

For now, though, I'm happy just to be the old-young cystic with the outdated IV in her forearm. I haven't managed to score one of those nifty IV boards they used to give me at Children's Hospital yet, but it's still pretty darn cool to know that I don't have to get a PICC pulled at the end of all this. And yes, I used the word "nifty" without even a trace of irony, but that's totally allowed when you're going peripheral . . . or so I've been told.

Light(s) at the End of the Tunnel

Some of you may remember my rant about going to the dentist and learning that the dentist needed medical approval from the tx team in order to examine me/clean my teeth. The transplant team then turned around and demanded medical approval from the CF team b/c they're not my "primary doctors." So then my CF team finally gave clearance, which allowed the dentist to finally, finally, finally give clearance to the transplant team. Keeping in mind that in between getting all the medical clearance and actually returning to the dentist for the examination I ended up in the hospital three times in just over two weeks and you can probably begin to understand why I'm so excited right now. Because . . . (cue drumroll) . . .

As of today, I have officially completed every one of my dentist's pre-transplant "recommendations." Seriously, this is a HUGE milestone.

See, my dentist thought that my teeth were in fairly good shape overall. He saw two fillings that he thought were loose and wanted to fix them preferably pre-tx b/c loose fillings can 1) fall out, and 2) become a good place for new bacteria to grow in the space between the filling and the tooth. And since they don't recommend having any dental work done until 6 months after tx, we decided now was the time to take care of those things (although he said if got THE Call I could still accept, as he thought the fillings would likely survive 6 months anyway). He also wanted to do a root canal on one other tooth, which I was kind of nervous about having never done one of those suckers before. So I managed to get the fillings re-filled (capped, actually -- we went with the "better safe than sorry" option since they were two small fillings in the same tooth), and then I was left with the root canal. Definitely not something to look forward to.

Well, for anyone considering a root canal in the near future, I have a slight suggestion: have risky and complicated vascular surgery to remove an infected port without general anesthesia three days before your scheduled dental appointment. This fool-proof method of making a root canal seem easy has a number of perks, including: 1) your left arm will be sore because it will have three large incisions healing on it (no pain meds were offered, and I didn't request any, but it honestly wasn't too bad) -- the pain will distract you from the root canal unpleasantness, 2) your right arm will be slightly sore because of the PICC line and residual phlebitis from the blown peripheral IVs -- again, more distraction, 3) you will, I promise, have sudden flashbacks to your time in the OR prep area every time you start to get nervous over the root canal, at which point your brain will basically sigh with relief because the comparative risks of a root canal are so totally not worth stressing out about. Believe me, it works, and thanks to the miracles of novacaine and advancements in dentistry, I can honestly say the root canal was no big deal at all. I think I was in the chair all of maybe 45 minutes, although that may have been because my dentist has a periodontist on staff who does nothing but root canals, so he's super good and efficient. Lucky me.

So yeah, I got the root canal a little over a week ago, and today they finished it by capping the tooth to once again guard against any future infection. And now I'm totally and completely done with the dentist (aside from, of course, regular cleanings and check-ups). In other words, check another thing off the pre-tx to-do list, please!

Anyway, now that that's all over, I also have a CF appt tomorrow, and then on Thursday I have a surgical consult to check out my wounds from port-fest 2009 and, um, to check out my other arm for the placement of -- you guessed it -- Piper's Arm Port II. I got word from my tx team that they are willing to leave arm ports in for CFers because you often end up needing IVs within the first year after tx anyway, plus everyone is on IV drugs for at least a little while immediately post surgery. It is clear that I'll need to get it taken out once it seems like my need for IVs is less (probably within or right after the first year), but since there's no guarantee of how soon I'll be transplanted and I don't want a PICC in my arm all year, not to mention the fact that I don't want to have to have a PICC placed every time I need IVs after tx as well, I've decided to go ahead and let my favorite vascular surgeon put another port in place. And yeah, because of my history it has to be done through surgery (again, no general anesthesia obviously) instead of IR. But the same amazing vascular surgeon who got me through the amazing port debacle has told me he'll be glad to handle my next placement. So I know I'm in great hands, and I'm actually pretty stoked to get another arm port, truth be told.

I also got my LTD from work approved, so I am officially no longer part of my Firm. Bittersweet, to be sure, but the right decision for now and I know I'm lucky in this economy to have this sort of option. I'll be applying for SSDI as well in the upcoming weeks in order to ensure I can get Medicare by the time my COBRA benefits run out. Definitely NOT the time to be caught without healthcare.

So that's the news. Next tx appt is on the 14th and I have to repeat PFTs and the 6 min walk. I'm almost positive my distance on the walk test will be lower than it was in May (I went 2200 feet at that time, which is apparently pretty good for someone on the tx list). The good news is that a slower walk would bump up my score, but I'll be bummed if it's too low anyway. And I know I'll have to wear O2 for it this time as well, so that's kind of a nasty milestone. This whole "24/7 O2 because my sats drop below 90 basically anytime I move more than 5 feet" has been kind of a rough pill to swallow. Since I'll be doing PFTs tomorrow at CF clinic I should have a pretty good sense ahead of time of what my score will be on that front. Hopefully I won't have dropped from my 33% a couple of weeks ago -- here's hoping I can even pull it up a notch or two! Again, it's kind of a double-edged sword with the LAS (lung allocation score, for the non-tx people), but I'd still rather be up than down.

The best part about all of this is that I've been feeling amazing lately. My aunt and uncle were in town this weekend from Texas and we took them all over, plus I've been enjoying dinners and lunches with friends, walks outside, and generally trying to make the most of the mild fall weather. I seriously love this time of year, so I'm so so grateful to be able to enjoy it. Just thinking back to where I was at this time last month -- honestly wondering if I would get out of the hospital at all pre-tx -- is enough to remind me to be grateful. Well, most of the time anyway.

Here is the picture of the week: gorgeous colored glass lights hanging from one of the stalls of the Grand Bazaar in Istanbul. I was honestly transfixed by all the color and light there and the picture doesn't do it justice -- it was like staring through the stained glass windows at the Saint Chapelle in Paris. I brought home a small one for my bedroom, but it's just not the same!

Live Strong

Okay, so I know I've been terrible about updating the blog lately. I really have no excuses for my bad blogiquette, except for the fact that I'm finally feeling good (actually, the right word might be "wonderful") and have been seriously enjoying the process of coming "back to life" after so many revolving door trips to the hospital and so much health drama. I guess actually living leaves less time for my blog than sitting in a hospital room does -- go figure -- but I think it's a sacrifice I can live with.

In other words, it's nice to finally be normal again (even if the definition of "normal" in this case includes jumping about 5 feet every time my phone rings b/c I think it might be "THE Call"). I'm still on IVs, but just Merrem, which I guess is the one antibiotic that seems to hit both my lungs bugs and the silly little critters that decided to infect my now absent port. My doctor and I had talked about stopping the drug on the 1st, which would be tomorrow, but seeing as my homecare company just delivered WAY more than one day's worth of new eclipse balls, I'm thinking I'm in this for the long haul. It's not particularly surprising -- there was some debate about whether I would need IVs for 4 weeks or 6 weeks to totally clear up this blood infection, and my guess is everyone's thinking better safe than sorry at this point. Hard to argue with that logic, believe me. Although considering I started the original lung IVs a week before we even discovered and starting treating the port snafu, I'm definitely feeling ready to be off of IVs sometime soon!

In the meantime, I think I have a little bit of port envy. I'm sure Freud would have a field day with that statement, but it's true -- I have rediscovered that I really, really, REALLY don't like PICC lines. Mostly this is just because I feel like I have to be more careful about my arm when a PICC is in than I ever did with my port (and for good reason: they clot easier and are more prone to infection). The big thing for me is my secret fear that this thing is going to actually rip out of my arm at some point, although that seems unlikely since it's actually stitched on there. (By the way, is that normal? I don't remember having stitches with my old ports -- 9 years ago, remember -- but this one definitely has two stitches connecting it to my arm right where the catheter leaves my body. It's annoying because they hurt under the dressing by pressing into my skin.) So yeah, in the upcoming weeks/months I have to decide if I want to 1) leave this PICC in for several months regardless of whether I'm on IVs the whole time and hope it lasts me to transplant (downside: annoying, plus have to deal with above-mentioned secret fear even while not on IVs, which seems unfair), 2) get this PICC pulled and get a new one if/when I need new IVs (downside: my veins are pretty much shot -- it took IR 3 hours to place this one), or 3) get a port until transplant (downside: I'm still a little gun shy on the port thing, just because of recent experience, plus it might have to come out with the tx surgery anyway, making it pointless?). Any suggestions out there from people who have been through this choice -- or something vaguely similar? All advice is welcome.

My other main focus right now (besides, you know, getting back together with friends and enjoying this great fall weather we've been having) has been on regaining some of my strength that I lost during this whole ordeal. It's fair to say lying around in a hospital bed, while sometimes good for your overall well being, is NOT good for your muscles. Seriously, for the first week I was out of there I would get tired after a 10 minute walk to the store -- and when I say "tired" I mean like 2-hour-nap-style exhaustion. Obviously part of that was just my body's need for continued rest since I wasn't totally healed yet, but a lot of it was just plain weakness, which I happen to think is both understandable and excusable after 4 hospital stays in as many weeks. Understandable, yes. Fun, no. So lately I've been trying to face the problem head-on and have gotten back in touch with my old friend the treadmill, as well as much outside walking as I can handle and my new favorite resistance training/core building exercise: gyrotonics. Love this. There is a great place close to my house where I can get private instruction a couple of times a week, and I'm totally addicted. Right now I'm going easy on the arms (see previous ramblings re: PICC issues, not to mention the surgery I had on the other arm a couple of weeks ago), but my legs and core feel amazing! You do work out with weights as part of this technique, but it's also very much about flexibility and working your muscles together as a complete system. I think of it as yoga meets pilates meets Sven the bodybuilder. Fun stuff.

The main difference in my exercise mentality over the past couple of weeks has been that the focus right now is on getting ME as strong as I can possibly be. I know that sounds really simple, but to be honest, for a while there after I heard the word "transplant" I wasn't really very focused on my personal well-being when I hit the gym. Weird, huh? Let me explain what I mean: I had heard so many stories (inspiring, to be sure) about people raising their lung function and getting off the list through exercise that THAT became my only goal. I was constantly waiting for some sort of "treadmill miracle" to take place that would somehow shoot my numbers up out of transplant range, banish all infection, and let me get back to a place where CF didn't always have to be such a huge obstacle for me each morning. But the fact of the matter is, the only thing I gained out of that whole thing was a sense of failure and a lot of panic attacks. (As an aside here, I'm sure it doesn't help that I'm a total "type A" personality and tend to be VERY hard on myself, and I'm also sometimes a rather unrelenting optimist, which is normally a great thing but here maybe led me to set my sights on something that wasn't entirely realistic for me.) The point is: I wasn't working out for me so much as I was working out for my NUMBERS -- and as anyone who's gone crazy trying to work out just to lose weight will tell you, it's a lot harder to really focus on getting healthy when you're too focused on a print out from some silly machine.

So now here's the deal: I would love to get off the transplant list, but I really feel that my best bet for doing so is to actually get transplanted. Don't get me wrong, I'd love to make these lungs last a few more years, and I do believe in miracles, but I also have faith that transplant will be a blessing and an opportunity sent from God, my donor, and a great team of doctors -- it will not be a failure on my part. Having realized this much is true (and, sheesh, it took me long enough), I'm going into this new stretch of exercise and life with the goal of becoming as strong as I can, whether that strength ultimately helps me to avoid transplant or to come through it that much easier when THE Call actually comes. And if the numbers on the machine never change for me again (or even if they go down), well, I'd rather be a strong and active 30% than a defeated and panicky one who wonders why she can't be a 35%. And right now I'm feeling strangely proud of all my cysters and fibros who push themselves to do what they can, because I'm more sure than ever that this game is about so much more than just the numbers.

As a personal sidebar, I completely LOVE using the word "fibros." Cystic slang at its absolute finest (and most ridiculous).

Stay well everyone.

Third Time's the Charm

Good news: the catheter is OUT!

Yep, afer 2 1/2 months of weird fevers, blood cultures every few days, over 6 CT scans of various parts of my body, countless x-rays, 4 hospital stays, and continuous use of one IV or another (aside from a brief 2 week break in there), the probable culprit is out of my body for good! I cannot even tell you how excited I am.

Of course, in true Piper style, I didn't make it easy on anyone. They were scared to put me under general, so the whole thing was under conscious sedation (apparently I told the nurses all about my sister's awesome fashion line -- how great of a little sister am I?). They cut open my arm in multiple places, clamped off a vein, removed the scar tissue, and then ended up having to literally "lasso" the catheter as it started slipping away into my heart. Wow. They thought the surgery would take about 20 mins to an hour -- it took over 2! But I have an awesome team, they got the job done, they did it safely, and I'm scheduled to finally go home tomorrow. Hopefully minus one very pesky blood infection.

I'll still be on lung IVs for a while, but hopefully not too long. This is week 3 and I normally do 3-4, so here's hoping I get to call it all quits after that. We're going to leave in my PICC for now, in part because it seems silly to get a new port when they'll have to remove it during transplant anyway. The other reason is that it took them 3 hours to place this PICC in interventional radiology (no joke) because my veins simply "do not do PICCs" anymore, as the radiologist said to me afterward. So it might be wise to leave this one in just in case I end up back on IVs in the next month or so. The other option is a new port, and while I'm not opposed, I am a little gun-shy right now. I think it's justified.

By the way, this is totally not to say CFers should avoid ports. Totally not. I LOVED mine. And it was great, for 9 whole years. It saved me countless PICCs (off the top of my head I can say that I've had at least 25 rounds of IVs in that 9 years, which is ridiculous -- most of them in the last 3), and a lot of pain. What happened to me with the stuck catheter was totally weird and very rare, so please don't take it as anti-port propaganda.

Okay, so other news.

1) I am now "officially" on O2 24/7. I say "officially" because I am actually still stating a little above 90 most of the time, but I desat with minimal activity (like walking around the apt), so my doctor placed me on 2 liters with instructions to use my judgment and be careful. It's a new development to have the script for the 24/7 O2, but it doesn't change much, since I was being pretty careful to use it with any real exertion anyway.

2) I have a Frequencer! This is a new airway clearance device that I asked my doc to look into and she, being amazing, got me a demo and then a free trial. I love it! It works like chest CPT only using sound waves that go through your body. You don't feel squeezing or beating, but then when you speak your voice vibrates just like the vest! You hold the little speaker over each part of the lung for 2 mins, just like CPT (front only -- it travels through muscle and gets both sides of the lobe), and it works wonders. I cough up tons every time I use it. Highly recommended to try out, especially for people who want a basically silent, tiny, and super effective alternative to the vest. I promise pics and a more in-depth post later.

3) Just an interesting tidbit: I just found out that my center is one of just a couple in the country currently experimenting with transplanting CFers directly off a type of artificial lung called ECMO -- basically a machine that takes blood out of your body, oxygenates it, and returns it. The idea is that the vent is problematic for CFers because you can't cough. So my center is experimenting with taking CFers who go into respiratory distress and weaning them off the vent and onto ECMO, and then actually taking them out from under sedation so that even while hooked up to ECMO they can cough and do some airway clearance. The idea is that the CFer will then be in better shape going into transplant and hopefully have better outcomes than off the vent transplant (though many of those turn out fine, it has to be said). I don't entirely get it, but it was offered to me as an option should things get that far (knock on wood). ECMO is sometimes used after transplant to help lungs that for some reason aren't functioning well in the first couple of days, but this is apparently a much more novel and experimental use. There has already been at least one success story here using the method. I haven't decided yet if I would go for this option or not, but I'm discussing it with my family now just in case. I just think it's crazy incredible what new things are developing in the world of CF lung transplants!

That's it. Thanks everyone for your thoughts and prayers -- they definitely came in handy to make this try a success!

Surgery Fun Tomorrow

Well, I've been home for exactly a week today, and I officially go back into the hospital tomorrow. Not for a tune up though (still on at-home daptomyacin for the blood infection and merrem for the lungs). This time it's for surgery.

Yep, fingers crossed, we're finally going to get rid of the rest of that blasted port.

Basically, I've been running low to mid-grade fevers since I got home, and while we're pretty sure part of that was from the lungs (which had to be off all anti-pseudomonals for a few days due to bad kidney functions after the polymyxin), we still think part of it is from that lingering port/blood issue. But having gone in twice prior and failed to remove the catheter -- once in his office with lidocaine and once in the OR with lido and "twilight" sedation -- because the darn thing is fused to my vein in the armpit/shoulder area, the surgeon is going to take a more aggressive approach this time.

The plan is to go in through my armpit (sidenote: not sure if this means through the actual "pit" itself, or if it really means a bit higher -- better shave just in case!) and clamp off the offending vein on either side of the scar tissue. Then he'll be able to cut in there with a tiny incision, dislodge the catheter, and then hopefully pull it out no problem (the port itself is already gone) and sew up the vein.

Yeah, you read right: sew up the vein. Vascular surgery is amazing.

Anyway, because I'm thin enough for the surgery to be rather close to the surface (thanks, CF!) and because I have crappy lungs (um, thanks again, CF?), we're going to do this whole thing under "twilight" sedation once again. So I won't be asleep, just very, very loopy. And normally that means that I would be able to just go home when it was all over, but my CF doc wants me in the hospital for one night just in case. I'm not making any protests there.

One night to (hopefully) set this whole thing in order and get rid of the mystery fevers of 2 months and counting once and for all sounds pretty damn good to me, thanks very much.

Not particularly excited about the whole process, obviously, but I feel blessed to have such a competent team, and I'm super happy about not having to risk the breathing tube at this point in my CF life. Avoiding the vent is key right now and I've been promised that if my O2 drops below expected levels they'll do a type of blowback O2 (where they literally blow it into your mouth with a machine) and try basically everything else before they do a tube. But I've had conscious sedation plenty of times and never needed more than my nasal cannula, so I'm just going to stay focused on that. It also apparently says all over my surgical instructions "no general!" and "no intubation!" -- or so the nurse told me on the phone.

Hopefully I'll be able to update tomorrow from Columbia and let you all know that things went smoothly and that the little bugger is finally OUT! Everyone seems confidant, and I trust this surgeon a lot, so I'm anticipating a good outcome for sure.

Thoughts, prayers, intentions, and good vibes are always welcome in the meantime, though!

All About Me

So things are moving right along towards transplant over here in Columbia-land. My score has increased quite a bit lately because of increased O2 needs, lower PFTs, poor weight gain, and a decrease in my 6 minute walk score. They actually think they may have lungs for me here in the next couple of months -- some people were even talking as if I might get transplanted during this hospital stay. Yowza. I'm definitely on board, and I have a huge amount of faith all of the sudden that this is right, things are falling into place to give me a second chance at life for which I will be forever grateful, and that God and the Universe and Columbia Presbyterian will bring me these lungs at the exact right moment for me. I'm definitely ready to breathe and be back to my old, energetic, happy, excited, optimistic, hyper, "normal" (haha) self. I'm just so grateful to even have the chance at that sort of opportunity.

In the meantime though, I'd like to go home. I mean, not to sound snippy or anything, but this whole hospital and failed surgery thing is getting old. Luckily my sats and energy levels are up, and my fever is down, so my doctor is on board with letting me go as soon as they place the PICC line. Unfortunately (there is always an "unfortunately" in the hospital -- have you noticed that?), the PICC and IR teams are out until Tuesday, so that's the earliest I'm gonna get it placed it looks like. Long weekends are so much fun for the outside world, but in the hospital it tends to just mean 3 days of not seeing your doctor and not being able to move forward, which is frustrating. My poor arm has blown through 8 peripheral IVs in three days and is swollen up like a basketball. Hopefully tonight they'll at least be able to get permission to use my left arm, which so far has just been recovering from surgery and resting. I think it's about time it started pulling its weight around here, although it points out that for the past 9 years it's handled all the IV work. What a complainer!

Not much other news. My blood infection is clearing up and I've had a couple of negative cultures. They're still worried that it will come back as soon as they stop the dapomyacin, but if that's the case then I can either: 1) have vascular surgery in my shoulder under "twilight" sedation to remove the rest of the catheter, or 2) go on oral abx to control the blood infection until I get the transplant, at which point the catheter will be removed anyway. I think we're probably looking at option 2, but I'm pretty fine with either at this point. It kind of creeps me out to have that catheter still in me without the port, but whatever. I guess it's not as though I have to see it, right? And it just adds to my awesome "sickgirl" powers, I'm sure.

Speaking of power, I'm really really trying to keep my strength up and regain what I lost. When this blood infection hit, it made me more or less catatonic for a couple of days, and when you're already on prednisone that will wipe out muscle mass pretty quick. I felt like I was dreaming when my doctor suggested that I spend "a little time every day" sitting up in a chair to keep up my strength. Sitting?! I asked if I could walk the hall and she seemed a little concerned but of course said yes, and even offered me a walker! Well, I'm proud to report that after several days of walking laps up and down the ward, today I used my pass and walked OUTSIDE, down the block, around the corner, and down through another building to get to the hospital courtyard. Granted, once there I promptly fell asleep and proceeded to nap sitting up for the next half hour or so, but then I got up and walked back, with a short detour to the vending machines. No, it wasn't the workout of my dreams, but it was something, and I got fresh air, and to be honest it was all I had in me for now. I've also been spending time sitting int he lounge, and I plan to walk some more laps this afternoon. It's not great, but it should hold me until I can get back to my treadmill on Tuesday!

The one great thing about the hospital on weekends is fewer people come into your room and but you for random reasons. I've had my CPT for the day, done most of my IVs, and seen allt eh residents covering for my doctors, so for the first time since I got here I think I can actually shut my door, get out my book, and be as antisocial as I please.

Which is exactly what I plan to do.

To My Darling Sampson

Hey Sammybear, it's your mama writing. I'm pretty sure you haven't learned how to read or use a computer in my absence (although if you have, MAJOR props go to grandma for her puppy-training skills), but I still just couldn't resist the chance to send you a message.

Now before you even say it, I know I'm in the doghouse (yes, pun intended) for my recent extended absence. I know that being gone for 5 days and then coming home for one ridiculous night before leaving AGAIN for another week is hardly good puppy-mom etiquette. And while I know you're enjoying having both your grandma and grandad in town for a while plus extended visits with Aunt Erin, I also realize that it just plain sucks when your best friend and playmate goes totally AWOL. You're such a sweet puppy, Sam (random rocket-science experiments aside), and I'm so sorry that I keep having to leave you.

The problem this time, just so you know, was the port. You know that thing in my arm that means the nurses have to come once a month and you get to root around in their bags for fun things to chew on? Yeah, well while you're off snacking on rubber gloves and stethoscopes, the nurse is busy taking care of my port-a-cath, a permanent IV line running from my arm to my chest which has now become infected. So on Tuesday when they sent me home because my lung infection seemed under control, they had no idea that I was going to crash very suddenly overnight because of a full-on systemic infection from the line. Neither did I, and of course, neither did you. You were just happy, like any good dog would be, to have your rightful human home and back where she belongs.

And speaking of back, Sammy, I really am. As in, back in the EXACT SAME ROOM. I wasn't even gone 24 hours, so they managed to put me right back where I came from, which is nice because it kind of feels a little more homey. Only not really homey because, of course, you're not here. (Note to self: speak to hospital about establishing designated "shorkie visiting hours.") But yeah, anyway, back to why I won't be home sooner . . .

See, once they decided my port was infected yesterday, they sent me to have it taken out. So I went down to the procedure room and got all numbed up with lidocaine and they opened up my arm and started to remove the port. Sounds pretty simple right? Pop out port, pull out catheter, sew up patient. Granted, it's not quite as easy as it sounds, I'm sure, but still, none of us were anticipating any problems.

But then again, it is your mama we're talking about, Sammy, so maybe we should have been better prepared for total ridiculousness.

Step 1 went fine - the port popped out no problem. Step 2, on the other hand, not so much. The surgeon tried to pull out the catheter and I told him I felt a pain in my back, so he stopped. When he stopped, the catheter literally sprang back into my vein, sort of like a vacuum cord when you hit the "automatic retract" button. Weird. Okay then, so he tried again. Same thing. Seriously, the thing was stuck. Solution? Tuck the port back into the arm and proceed directly to step 3 (sew up patient), and then schedule port removal in the actual OR for the following day. And I wasn't too upset about it. After all, if I'd been happily nesting in some place for 9 years, I probably wouldn't want to leave either.

So today I had surgery in the OR to get my port removed, sort of like when you had surgery to get your . . . well, you remember your surgery, Sampson. Anyway, unlike you, I was able to remain awake for my surgery, but I was given some fun sedative drugs that made me nice and talkative to the surgeons. This time the plan was a bit more complex: open up arm even farther, remove port, try again to remove catheter, if that fails, slide larger catheter up around current catheter in attempt to gently dislodge catheter from vein, sew up patient.

Well, they tried Sammybear, but it still didn't happen. Turns out this baby is stuck to my vein with some serious scar tissue way up in my shoulder, and the only way to remove it would be through full on surgery (sorta like what you had) only no one wants to do that to me because of the risks associated with putting a CFer on the vent. So I'm kinda stuck for now with an infected catheter in my system (they did, interestingly, remove the actual port -- just snipped it off and put something on the catheter to keep it from sliding into the vein). The good news is that the infection is strep, not staph or pseudo, so it's very treatable and I'm already responding to the additional antibiotic they've started me on to attack the bug. So the infection as it stands now isn't so much dangerous. The bad news is that they can only use this antibiotic for 4-6 weeks, and after that they'll take more blood cultures. Since the catheter is still in there, though, it's likely that the infection will reoccur, in which case we're either going to have to decide how to treat it (we could alternate antibiotics, maybe) or go ahead with the removal. If I can somehow deal with this until transplant then they should be able to just remove the catheter once they're already inside there. Fun, fun, huh Sam?

Anyway, in the meantime I'm getting meds through a lame-old peripheral IV line in my hand. I've already blown one, and my left arm is out of commission until it heals from surgery in a couple of days, so let's hope they can find enough veins in my right arm! You'd have fun with me, Sammy, I have tubes everywhere! On Tuesday, once they're sure the infection is under control for now, they're going to give me an old-fashioned PICC line so that I can keep doing these IVs without continuing to blow veins left and right. And the good news is, once the PICC is placed then I can FINALLY get home to you! I know you can barely contain your little puppy self with all the excitement!

As for the future of the port (and the fun nurses with their magic bags of chew toys), well, I may or may not get a new one. They don't want to put a new one in if the old one is going to be a continued source of infection because it would just infect the new port (sort of like why they do double-lung transplants in CFers). So if in 6 weeks or so after I'm done with all the IVs the blood cultures come back clear and they think the old catheter is clear of infection, I can get a new line placed pre-tx. Otherwise, no dice. And they also seem to think I'll be transplanted sooner rather than later at this point, so it may not even be a major issue, since all ports would have to come out during transplant anyway.

So that's the story, Sammybear. I miss you madly, but hopefully now that we finally have this a little more figured out I'll be able to come home to you a much happier, healthier, and spunkier Piper.

And I'm sure you're already plotting some creative ways to keep me busy.

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.

Welcome to the Hotel Presbyterian

Such a lovely place, although I'm hoping I can both check out AND leave -- preferably by the end of the week.

Yeah, I landed back on my ass in 9 Hudson South (actually the best ward going over here, with a menu for ordering dinner, private rooms/bath, and guest internet). The twist is that we don't actually think this one's all about the lung infection, or at least 2 1/2 weeks of solid 101 degree fevers daily says this isn't your average pneumonia. We're thinking viral, but they're not ruling out an infection that we're just not hitting yet . . . including the ever-dreaded port infection. Fingers crossed it's not that, although other contenders include such fun little visitors as Lyme Disease or a relapse of Mononucleosis.

I'm just hoping that whatever it is, it starts to resolve itself FAST.

They've already switch my abx from Merrem to Imipenem, which they did over the weekend at home and may already be making somewhat a difference. Also my potassium levels are climbing, which is wonderful. So basically I'm in here, according to my doctor, "for a couple of days" (famous last words!) to get some tests run and hopefully figure this thing out for good. It's been bothering me for waaaay too long.

I didn't even put up my usual crazy stubborn (um, I mean, polite and well-articulated) fight to stay out of the joint this time. Sometimes you just know you're headed for those plastic sheets, and the best that you can do is just grit your teeth and bring your own comforter. As my blogger cyster Cystic Gal might quote, "you got to know when to hold 'em, know when to fold 'em". . . and hope to God that your doctors know when to let you walk away ;)

Self, Shout-Outs, and Sammy (Of course!)

Hi guys!

So first off, a couple of people have asked me lately about the arm port and how much it "shows" when it's not accessed. I thought I'd share a couple of pictures since right now seems to be the first time in FOREVER that I can actually showcase the darn thing without a needle sticking out of it. Fun, fun. Anyway, here is is, for your viewing pleasure. (And for those of you who might want to see it accessed, well, I have photos of that in my archives from 2008.)

1) Close up of the port site (keep in mind that it looks angry right now because my last IVs just ended a week or so ago):

2) View of my arm normally (notice that it's more or less invisible, unless you're looking REALLY hard for the tiny bump):

3) Me, just so everyone trusts that this really is my arm:

4) Sammybear, because really, why not?


Bonus points if you can spot the O2 tanks in that last shot, btw ;)

In other news, I'm changing up my routine a bit. Mainly I've started separating Dnase and HTS, which isn't thrilling since it makes the third treatment a requirement instead of optional. Oh well, I guess I knew I should be doing three anyways. And I've also started a trial run on the PharmaNAC fizzy tabs. I ordered a month's supply (assuming I do 1 tab, twice a day, which is what my doc recommended). I doubt I'll see much change in a month, so I'll probably need to extend my testing time. I've know some people who swear by this stuff and then others who claim they saw no change even after a year. But at this point I figure I need to keep trying everything I can. I'm not looking for a miracle cure (although if anyone's offering one, I'll take it!) but I need all the help I can get just to break this infection cycle. Blah.

And my other big news...(drumroll)...I gained 2 pounds! This might not sound like much, but considering my doctor has been encouraging me to exercise LESS just so I can preserve more calories, 2 pounds is a breakthrough! And I'm still hitting the cardio - so I managed to gain AND stay on top of my game exercise wise. Very proud of that.

I just realized this is a very self-congratulatory post. Which frankly I'm okay with, because we need those every so often. But before I go too far overboard with the me me me thing, can I just say how much some of my fellow CFers have been blowing me away lately? Seriously, I know one who has been super sick and continues to reach outside of herself to everyone else and leave encouraging comments on my blog (and others) even though I know she's going through a rough time with lots of changes, one who has been going through a hellish road with sinus surgery and still sounds less whiney than I ever do, one who lost a dear friend and decided to change it into a beautiful project to help other cystics, one who is currently on IVs and has some other serious health complications but never seems to let it get her down, one who brought her FEV1 up from the start of transplant range all the way up to over 50%, one who selflessly allowed his amazing wife to share his transplant story start to finish for the benefit of others, one who is participating in a study of an new inhaled version of an antibiotic to help all of us, one who posts amazing articles and helpful info to her blog daily, one who was brave enough to post a great glimpse into the "raw" side of CF on her blog last week, one who powers on in the face of huge obstacles from his health and from outside sources, one who I recently corresponded with who is currently putting us all to shame with his amazing exercise routine (and putting me personally to shame with his awesome attitude), and several -- both pre- and post-transplant -- who blow me away with their ability to be amazing mamas and awesome people. This is in addition to the countless other cystics I know who are just plain unbelievable. Seriously, I can't imagine a more selfless, inspiring group.

I don't mean to sound sappy, but honestly I really am in awe of these people. Especially because they're not afraid to admit, collectively, that CF sucks, that it's hard work, and that acknowledging that does not in any way make you less of a fighter. And I'm really proud to count myself among their ranks.