Slight bump in the road

Of course things couldn’t just go smoothly, that would be BORING, and we all know that’s not how I roll.
 
To be fair, as complications go this one is more of an annoyance, and it’s not a completely unexpected one. In my last update I mentioned that they gave me a midline instead of a PICC line, because they initially didn’t realize I had an abscess and thought a 2 week course would be sufficient. In a not-completely-surprising turn of events, yesterday after exactly 2 weeks the midline decided to break. The unexpected part is HOW it broke. It flushes fine, but when I try to do an infusion it will only run if I hold my arm at a VERY specific angle. I had a dressing change the day it went wonky, so presumably something got jostled and now the end is blocked JUST enough to prevent a gravity drip. Holding my arm in one position wouldn’t be a problem if my infusion only lasted half an hour, but keeping your arm elevated and still for 3 hours straight, 3 times a day, is a bit much. Fortunately Interventional Radiology was able to get me in for an appointment tomorrow morning, so I’ll be ditching the midline and replacing it with a shiny new PICC, that hopefully will behave itself and NOT give me a blood clot this time around.
 
There has also been a slight change of plans regarding my treatment plan. Initially they wanted to drain the abscess, but Interventional Radiology said that it’s too small and in too difficult of a location, and that the slight potential benefit wouldn’t be worth it. The thoracic surgeon and my transplant doctor also consulted with my transplant surgeon, and they agreed that going in surgically wasn’t a great idea right now, as it would be a very painful and invasive procedure. And as my NP pointed out, surgeons like doing surgery, so if a surgeon tells you not to do surgery then you’re probably better off listening to them. So instead we’re just going ahead with 4 weeks of IV antibiotics, and then doing another MRI to see what kind of impact that had. Hopefully things will improve enough that I won’t need surgery, though I won’t be the least bit surprised if they end up extending the antibiotics beyond 4 weeks. I still have 2 weeks to go, so here's hoping the antibiotic is doing its job!
 
The last 2 weeks have been utterly exhausting, as we expected. Fetroja has a brutal infusion schedule, especially when you factor in the lack of a pump and the need to change the bag halfway through the 3 hour infusion. Adding a malfunctioning midline to the mix really seems pretty unnecessary! But despite everything, I somehow managed to cook a ridiculous amount of delicious food for Thanksgiving, AND I was able to slightly adjust my infusion schedule so that we could join my mom and Frank for Thanksgiving dinner. We’re trying our best to squeeze some good times around the current medical intensity. And as difficult as this whole situation is, we’re still so grateful that it’s NOT cancer, and that makes things slightly more bearable.
 
I did talk to my team about my concern that IV antibiotics alone won’t be enough, as we’ve already treated abscesses previously with IV antibiotics AND surgery only for them to return. It sounds like the long-term plan is bacteriophage therapy, but that takes time to set up, so we’re hoping the IV antibiotics will be enough to buy some time while we work on that. I’m 99.9% sure that this round of treatment will not be the end of Nodsferatu, but I can put up with a temporary solution as long as we’re working on something else for the long run. I fully expect to be battling these pseudomonas abscesses for quite some time, but I am hopeful that eventually we WILL find the right treatment combo to finally kick them to the curb!

I don't have cancer!!!

It’s official: I DON’T have cancer!!! My transplant doctor told me Friday afternoon that cancer is now off the table, and Nodsferatu is an abscess! Which is still problematic and needs to be dealt with, but is WAY better than cancer!!!
 
Right now they don’t want to go in and remove it surgically, because they’re afraid of popping it and spreading the infection. The current plan is to drain it and slam it with a long course of IV antibiotics. I assume that I may have a drain in place for some time after the procedure to make sure it empties completely, but that wasn’t actually discussed yet. The procedure also hasn’t been scheduled, but that’s to be expected since they only settled on this plan Friday afternoon. Hopefully I’ll hear from someone early in the week to schedule a date.
 
Right after talking to my transplant doctor I had an appointment with my infectious disease doctor, who helps my various teams figure out the best way to treat my complex, multi-resistant Pseudomonas monster. I talked to her about some of my concerns, such as the fact that we’ve treated my Pseudomonas abscesses with drainage, debridement, and antibiotics before, and they still came back. She agreed that this is a valid concern, and said she is going to explore bacteriophage treatment again in the hopes that it could provide a more permanent solution. She also agreed that it is likely that I have a leftover suture on the left side just like I did on the right side. While we won’t open me up just to look for a suture, if we do end up getting to the point of a surgical intervention, finding and removing that suture will definitely be a priority since it is likely the source of these recurrent infections. She also said that even after this is resolved, we will do maintenance imaging (probably CT scans) on a regular basis to monitor for potential problems and avoid getting caught by surprise again.
 
I also asked about something on the MRI report which seemed to indicate that the infection has created a tract through one of my ribs. During our brief phone call my transplant doctor said that the thoracic surgeon did not see any bone infection, which seemed to be at odds with the MRI report. My infectious disease doctor was also confused about this, and plans to follow up with the radiologist as well as the other doctors involved to confirm whether or not there is bone involvement. Bone infections are even harder to treat than abscesses, and require an even longer course of IV antibiotics. Right now the plan is 4 weeks of IV antibiotics, but if there is bone involvement that will be extended to 6 weeks. Either way I’m in for a long haul, but I already knew that.
 
One other minor issue is that this course of IV antibiotics was planned before we knew that Nodsferatu is an abscess, and was only meant to last 2 weeks to treat the infection that showed up during the biopsy. Due to this they gave me a midline instead of a PICC line. The problem with that is midlines are only meant for short term use, not the 4-6 weeks required for an abscess or bone infection. Right now the plan is to try to use it for as long as it lasts, but I might have to get it replaced with a PICC line later on.
 
It's only day 5 of this round of IV antibiotics, and Katherine and I are already utterly exhausted. Katherine is beyond wonderful and is helping me by running my morning dose so I can get some sleep after staying up past 3am with the late night dose. So I’m staying up late, she’s waking up early, and we’re both napping randomly throughout the day. We pretty much hate this antibiotic because of its grueling schedule, but the only other option is Tobramycin. When the choice is between severe tinnitus and exhaustion, we’ll take exhaustion every time. And at the end of the day we are SO relieved and grateful that I don’t have cancer that it ALMOST makes this process look easy!

Maybe not cancer??

This week brings yet another unexpected twist, but this time hopefully it’s a good one!
 
The thoracic surgeon looked through all my imaging going back YEARS, and discovered that Nodsferatu has actually been there since at least 2021! Back then it was WAY too small to notice, but once you know what to look for you can see a tiny bump on the scans. But the really exciting part is that he looked at the chest MRI taken when I had an abscess back in December, and he thought that Nodsferatu looked like a fluid collection and NOT a tumor! If that’s true, that would mean that Nodsferatu is likely just yet another Pseudomonas abscess, and NOT cancer! In which case instead of putting me through a rigorous surgery and opening my chest to remove it, he MIGHT be able to just drain it and treat the infection with IV antibiotics, which would be MUCH less invasive!
 
This was a very exciting development, but it definitely required more imaging before coming to any conclusions. He ordered a chest MRI, and remarkably I was able to get it done that very same day. And if I’m reading this MRI report correctly, Nodsferatu looks like an abscess!!!
 
Meanwhile my transplant team was already working on getting me setup with home infusion to treat the Pseudomonas infection that showed up in the biopsy. Yesterday I had a midline placed, and today I started IV antibiotics. Unfortunately the antibiotic of choice is Fetroja (aka cefiderocol), which requires 3 hour infusions 3 times a day. To make matters worse, each dose is split into 2 bags, so it has to be changed halfway through. It’s far from our first rodeo with this particular antibiotic, so Katherine and I already know we won’t be getting much sleep for the next few weeks. And abscess treatment usually isn’t a quick process, since there isn’t any blood flow inside an abscess and it takes a while for the antibiotic to penetrate it. We’re not exactly looking forward to getting back on the no-sleep train.
 
HOWEVER, I think I mentioned in a previous update that our bar has been lowered DRAMATICALLY since this whole nodule saga began. A month ago if you had told me that I have YET ANOTHER Pseudomonas abscess that needs to be treated, I probably would have lost my damn mind. But NOW, I will be THRILLED to be told that I only have an abscess and not freaking cancer! It’s amazing how quickly your perspective can change!
 
Even though it’s the much better option by far, Pseudomonas is still one stubborn bastard and is ABSOLUTELY my arch nemesis. So to recognize this, Katherine somehow managed to find the absolute best plushy in existence! Apparently the site that provided the cancer plushy also had another incredibly topical item in their shop. I was SO surprised and laughed SO HARD when she gave it to me! And now whenever Pseudomonas ticks me off, I can just punch it in the face!

Biopsy fail

Well, the biopsy… happened. And that’s pretty much all there is to say about that.
 
The biopsy samples came back showing… nothing. And according to the PET scan there is definitely Something, so we think they missed the target when collecting the samples. Which is incredibly unhelpful.
 
In a weird turn of events, I still don’t have answers, but I DO have the beginnings of a plan. I spoke to my transplant doctor yesterday, and she said instead of trying another biopsy, she’d rather just take the whole thing out and figure out what it is afterwards. I definitely prefer this option, because the biopsy ended up being more painful than I expected. The procedure itself was fine because I was high as a kite on Versed and fentanyl, which was the one enjoyable part of this whole experience. Once the anesthesia wore off, however, I was in quite a bit of pain. I was also anxious, because there is a significant risk for pneumothorax (aka a collapsed lung) after a lung biopsy, and I wasn’t sure how I was supposed to tell the difference between normal chest pain and pneumothorax chest pain. Fortunately I didn’t have any symptoms other than pain, and that has been getting better as the days go on, though some is still lingering. They also put the biopsy needle in through my back by my left shoulder blade, which happens to be almost the exact spot where I’ve been having pain that I’ve been going to PT for over the last couple months, so that was less than fun. If I’m probably going to need surgery anyway, I’d rather cut (ha) to the chase and just get it over with instead of throwing in additional painful procedures. I have a consult with the thoracic surgeon Monday morning, so hopefully they can get me in for surgery just as quickly as they got me an appointment!
 
The one thing that DID show up in the biopsy results was... Pseudomonas. I was simultaneously INFURIATED and relieved at the thought that this whole thing might just be a severe Pseudomonas infection. I believe my initial reaction was something along the lines of “PSEUDO-F@#%ING-MONAS!!!” That damned bacteria is DETERMINED to kill me one of these days! But I also realize that a Pseudomonas infection would be FAR better than cancer. Unfortunately, my transplant doctor thinks it only showed up because my lungs are colonized with Pseudomonas and that it’s not actually the cause of the nodule. We might still treat it with a round of IV antibiotics anyway though.
 
Speaking of The Nodule, Katherine and I decided that it needs a name. I’ve been thinking of it as an alien monster in my chest, which of course brings to mind the chestbursters from the Alien movies, but they don’t really have names and Xenomorph just didn’t feel right. I tried to think of other horror movie monsters, and with the help of the CF Discord server came up with Nodsferatu (a play on Nosferatu), aka The Nodsferatule, or Nods for short. Katherine also decided that I needed a new critter after my biopsy, but she couldn’t just get a regular bronchoscopy animal since this wasn’t a bronchoscopy. So she somehow managed to find a cancer plushy! And even better, you can flip it inside out to turn it into a healthy cell! It was so surprising and definitely made me laugh!
 
So once again I’m waiting. Hopefully not for long, and HOPEFULLY the next procedure will be the LAST one for this particular problem!

One step closer to answers

The biopsy has officially been scheduled for the morning of November 10^th. Unfortunately that was the first appointment they had available, but now I’m on the waiting list, so hopefully something will open up sooner. Waiting 2 full weeks to confirm whether or not you have freaking CANCER and find out what kind it is doesn’t exactly sound like a great time, but there’s not much I can do about it. On the other hand, maybe it’s reassuring that this isn’t a 5-alarm, all-hands-on-deck, get-her-in-NOW situation.
 
In the end Interventional Radiology will be doing the biopsy, which means it will not be a bronchoscopy. They said I will receive both sedation and local anesthesia, and that I shouldn’t be in the procedure room for more than an hour and a half, but afterwards I will have to wait in recovery for 2 hours and get 2 chest x-rays to make sure everything is ok. I also have to arrive an hour early, so the whole thing will take about 4 hours. I don’t know how long it will take them to get results afterwards, but hopefully it will be quick!
 
So now I get to twiddle my thumbs for the next 2 weeks and try not to think cancer thoughts or something. We’ll see how that goes!

The big C

Well, the good news is that there’s only 1 nodule. The bad news is that it’s probably cancer.
 
The way a PET scan works is that they inject you with a radioactive tracer, and then wait for your body to absorb it. Then they scan you to see how much of the tracer different tissues absorbed. Different types of tissue absorb different amounts of the tracer. Unfortunately, the nodule absorbed the tracer in way that strongly suggests that it’s malignant.
 
Katherine says that as soon as they told me it would very unusual for it to be cancer, she should’ve known right then and there that OF COURSE it was cancer, because that’s just how I roll.
 
The next step is to get a biopsy and find out exactly what we’re dealing with. The assumption is that we’re dealing with PTLD, but we won’t know for sure until it’s biopsied. My transplant team is currently coordinating with multiple other teams to determine the best team and the best method to do the biopsy. One of the questions is, will they do a bronchoscopy and get a sample from the inside, or will they come in from the outside instead. It seems that the tumor is right on the outer edge of my lung, which makes it a bit less clear cut. So now we’re waiting for the other teams to do their assessments and follow up with transplant. We had hoped to get an answer before the weekend, but it’s hard to coordinate between so many different people, and unfortunately things just haven’t come together yet. So now everything is on hold for the weekend and will resume next week.
 
My transplant team has reassured me that they are taking this extremely seriously and will get me the best care possible. At the same time, they also acknowledged that after almost 6 and a half years on anti-rejection meds, this is not an unexpected complication. There is a long list of things that can go wrong after transplant, and for years I’ve been keenly aware that the top 2 on the list are rejection and cancer. So even though this is definitely scary, it also isn’t a huge surprise. There’s a reason I get so much testing done on a very regular basis, and it’s precisely so that we can catch and treat things like this before they do too much damage.
 
Katherine and I have been handling it surprisingly well so far. Not only have we had several days to process and prepare for this possibility, but our bar has been dramatically lowered over the course of the last week. Instead of being like “oh no, cancer!” we were more like “yay, only 1 cancer!” We were both very worried that the PET scan would show multiple areas of concern, and are immensely relieved that only 1 nodule showed up. Now we’re moving on from wondering and worrying mode, and transitioning into go mode. Unfortunately we have a lot of practice dealing with dramatic life-or-death medical issues, and it’s honestly easier to have a problem to tackle than to be sitting around wondering what if.
 
There are still a lot of questions to be answered, and right now I have no idea what this journey is going to look like. I am so grateful to have so much support, not just from Katherine and my mom, but also from my amazing friends and extended family and community. So I'm squaring my shoulders, putting on my fight song playlist, and getting ready for the next battle. CF still hasn’t managed to take me out after 46 years, and it’s for damned sure that I'm not giving up now!

I'm radioactive, radioactive!

Well that was interesting! Check out the shielded needle they used to inject the radioactive tracer for my PET scan! I just love when they're all, here's this dangerous substance that we need to make sure nobody touches - let's put it inside you! 😂

Also this is my current theme song:

https://youtu.be/ktvTqknDobU?si=PhY7nBAVO7wxKkV0

I'm radioactive, radioactive!

And the other shoe drops

Remember how in my last post I said I get anxious when I go too long between updates because I’m always waiting for the other shoe to drop? Well, it may have just dropped.
 
The part I left out of the last update is that I was already waiting for test results when I wrote it. We weren’t actually expecting anything dramatic, but I wanted to get the big catch up post out of the way just in case things went sideways. It’s still unclear exactly how dramatic things are about to get, but we’ve definitely moved from “it’s probably nothing” to “it’s definitely something, but we don’t know what yet.” So I guess it’s time for another update.
 
A few weeks ago I had a regularly scheduled transplant clinic appointment, during which I got a chest x-ray as I always do. They saw something a little off on the chest x-ray, and reassured me that it was probably nothing, but ordered a CT scan just to double check. They actually downplayed it so much that I completely forgot about it until I looked at my calendar the night before and was like “CT scan? What CT scan? Why am I getting a CT scan again??” So I looked up the chest x-ray report in my patient portal, which was a mistake, because it mentioned assessing for post-transplant lymphoproliferative disorder (aka PTLD), a type of cancer specific to transplant patients thanks to the anti-rejection meds. Katherine and I mostly managed to convince ourselves that it was probably nothing and stayed calm over the weekend, but that ended abruptly around 4pm on Monday when the CT scan results hit my patient portal and showed a nodule on my lung. In a weirdly fortuitous twist, having the abscess last winter ended up being helpful, because we had multiple CT scans of my chest to compare back to.  Unfortunately, those showed that the nodule has been there for at least a year and has approximately doubled in size during that time. Since it was late in the day my transplant team didn’t get a chance to look at the results right away, so we had to sit for almost 24 hours looking up words like “left pleural-based nodule of uncertain etiology” and “could reflect a solitary fibrous tumor or an unusual manifestation of PTLD.” It wasn’t exactly a good time.
 
I finally spoke to my team on Tuesday afternoon, and they were quick to reassure me that it does NOT look like cancer to them. But I do have a bit of a history of medical oddities, so it would be very on brand for me to end up with something atypical. It could also be a fibrous tumor, which is just a benign growth, or some sort of infection. We won’t know for sure until we get a biopsy. The first step is getting a PET scan to get a better look at whatever-it-is and also check if there are any other mystery growths we should be checking out. After that they’re probably going to want a bronchoscopy to get a general look at my lungs and check for infections, and they might even be able to do the biopsy during the bronch. If they can’t, we’ll have to schedule a separate procedure for that. So it’s going to be a bit until we can get some definitive answers.
 
On the plus side, Penn Cherry Hill amazingly had an opening tomorrow morning for the PET scan. I don’t know how long it takes to get results, but I’m REALLY hoping they come through in time for my team to review them before the weekend so we can get to work on scheduling the next procedure(s). But I also know I might have to wait until next week for that.
 
I had debated whether to say something now or wait until I had more answers, but Katherine was adamant that I needed to post right away and get the prayer chain activated. I will gratefully accept any and all prayers and good vibes for a good outcome. My full Hebrew name is Keren Eliana bas Sarah.
 
Here’s hoping this all turns out to be a big nothingburger and a whole lot of unnecessary fussing!!