The journey

For many, many years, “breathing is overrated” was my favorite snarky catchphrase. But now I’m starting to think that I just might have been mistaken.

It’s so wild being able to breathe! So many simple tasks that were beyond me for quite a while are suddenly, wondrously easy. Things like being able to walk from one room to the next, or prepare food, or do dishes, or take a shower without being completely exhausted afterwards. It’s been months since I was able to do any of those things, and I’m so unbelievably grateful every time I’m able to get up and do something without having to calculate whether I have the oxygen and energy for it. It’s a whole new world, and I’m loving it!

Facebook tends to give a skewed impression of people’s lives, as we’re more likely to post the good instead of the bad. I think mine is a little more real, but at times I too will downplay things or try to make them look less dramatic. My previous health updates were definitely real, but I don’t think they fully encompassed how bad things got right before my transplant.

During the week and a half that I was home between admissions, things were pretty terrible. I just couldn’t get enough oxygen, and spent most of the time either sleeping or writhing and groaning in my recliner. The only time I got up was to use the bathroom, and that was a huge ordeal that sent my oxygen plunging and sapped my strength. I hope none of you ever have the experience of trying to decide whether it’s worth the effort to go to the bathroom, or if you’d rather just hold it a little longer because getting up and trying to walk is going to be so awful.

Things were even worse once I was hospitalized. At that point I couldn’t even manage walking the 5 feet to the bathroom anymore. My stubborn dignity refused to use a bedpan, so instead I had a bedside commode. And even on high flow oxygen, just getting out of bed and onto the commode was enough to drop my oxygen and utterly exhaust me. I pretty much spent most of my time looking like this:

During that time I started having “air hunger,” which meant I felt like I was suffocating even when my oxygen wasn’t too low. Thankfully they were able to treat this with oxycodone to make me more comfortable, but it was still a pretty miserable experience. I also came VERY close to needing to move to the ICU and be put on a ventilator, as my oxygen needs almost surpassed what could be provided on a regular unit via high flow. If the transplant had been even a few days later, I have a feeling I would have ended up in the ICU.

And throughout all of this, of course, I was coughing up huge quantities of thick, dark mucus. For almost 2 months straight I coughed up at least half a specimen cup every day, and some days came close to filling the cup. No matter how many treatments I did or how much I coughed out, there was always more. It was utterly exhausting.

Basically, things were really terrible. My discharge paperwork says that I came to the ER with “respiratory failure.” My lungs had just about given up, and I was – quite literally, and faster than anyone had anticipated – dying.

And then the transplant came through.

The before and after is absolutely night and day. Even while I was still in the ICU post-transplant, even with all the pain, I still felt SO much better! And as the days go by, as I continue recovering and getting stronger, things just keep getting better and better. I marvel at all that I’m now able to do, and can only imagine all the doors that will open for me as time goes on. It is truly miraculous.

Don’t get me wrong, coming out of a double lung transplant was no picnic. I woke up in the ICU with a ventilator down my throat, a nasogastric tube, 2 long, stapled clamshell incisions, 6 drain tubes coming out of my chest, a triple lumen catheter in my jugular, IVs in each arm, and a Foley catheter. I was also hooked up to multiple monitors and had wires going everywhere. Initially my hands were restrained to prevent me from pulling anything out. Once I was alert I wanted to communicate, but couldn’t because of the ventilator. We tried to use a letter board, but unfortunately didn’t realize that my vision was off. When I thought I was pointing at one letter I was actually pointing at something else, much to everyone’s confusion. Which is why that brief post I made post-transplant took 2 hours to write! Things went a little better when we got a pen and paper, and of course once the ventilator was removed I was eventually able to talk again.

Even once I got out of the ICU and onto the transplant unit, I was still very incapacitated. I was very weak and spent a lot of time sleeping. The painkillers often knocked me out or made me loopy, so I wasn’t exactly up for visitors. I had to relearn how to walk, how to swallow, how to be a basically functional person again. Breathing was absolutely wonderful, but it was still a very intense time.

I still have a long road ahead of me. It will take months for my incisions to heal, and some of the nerve damage may never heal fully. I will spend the rest of my life with the terrifying, unpredictable specter of rejection hanging over my head. My immune system will always be low in order to prevent rejection, so I must forever be extremely germ conscious and do everything possible to avoid getting sick. My days will be filled with pills and treatments to keep me going, including the whole new world of managing diabetes. And of course, though my lungs now have different genetic material and are completely CF-free, the rest of my body still has CF, so I still have non-respiratory CF symptoms that need to be managed.

But, hopefully, it WILL be a long road, with plenty of time for all kinds of adventures that I thought were beyond me. Hopefully I will have the chance to blaze through all kinds of open doors in joyous victory. And no matter what happens, however long I get, the breaths I’ve taken thus far make it all worthwhile.

Almost there

As part of the transplant process you get a Lung Allocation Score (LAS) from 1-100, which determines your placement on the list. A higher LAS puts you higher on the list and closer to transplant. When I was discharged, my LAS was 42, which was already decently high. When I went to the ER, it jumped up to 59.

Today, it's 86.

I'm pretty much at the top of the list now. My doctor says the transplant could happen any day, and he's even hopeful that maybe it will be this weekend. Which is great, because I've been having a really rough time. I've continued struggling with nausea and GI issues on top of struggling to breathe. I'm basically bed bound, because the slightest exertion drops my oxygen. I spent most of the last couple days in a haze of discomfort and misery.

Today I was really struggling with feeling like I was suffocating, even when my oxygen levels were ok. Turns out that's a known thing called "air hunger," and it's treated with... Oxycodone. So I'm feeling MUCH better now. I'm fine with just being high until transplant, that absolutely works for me. I feel like that would definitely improve my current quality of life!

Everyone keeps telling me I'm almost there. So I'm hanging on and hoping I get there SOON!

...but not for long

Well, I managed a week and a half before going back to the hospital.

Going home ended up being MUCH harder than anticipated. The in-home oxygen concentrators were strong enough to keep my oxygen up while I was at rest, but not during exertion. I had oxygen tanks that were stronger, but I couldn't rely on them because they'd get used up too fast and I needed to save them for going to appointments or for extra-exerting activities. Unfortunately, even just walking to the bathroom was enough to drop my oxygen levels. Not only was this uncomfortable and utterly exhausting, it also meant that I was way less functional than anticipated. I ended up spending most of my time either in bed or on the couch or recliner because I couldn't cope with doing anything else. A lot of the time just talking was difficult because I was struggling so much to get enough oxygen. I was still bringing up LOTS of thick mucus, which meant frequent coughing fits, which was another thing that all too often sent my oxygen plunging. I also woke up coughing every hour or two all night every night, so sleep wasn't going well. And on top of all that, I was dealing with nausea and almost no appetite. I basically spent most of my time curled up in a miserable ball, with the occasional hour or two of feeling somewhat better.

Katherine has been a trooper and a godsend. She's basically been waiting on me hand and foot for the last week and a half, while also having to cope with all the emotions involved in watching me be an utter train wreck. I truly don't know what I would do without her!

I was very torn, because on the one hand I wanted to be in the comfort of my own home, but on the other hand it was so very hard. We already knew within the first few days that I wouldn't be home for long, because it was clear that my oxygen needs would soon be too high to be maintained outside of the hospital. What we did not expect was for my oxygen to abruptly plummet yesterday. For reference, I was discharged on 7 liters of oxygen at rest, which kept my oxygen level around 94-96%. Anything below 90% is not good. Yesterday I used tanks to put myself on the highest oxygen level I could get at 15 liters, and I was still suffocating. After a few emails back and forth with my doctor, I was off to the ER.

Once we arrived I got to see what happens when you come to the ER with a REAL emergency. My oxygen on 15 liters was below 90%, so they immediately whisked me back to the ER's Resuscitation and Critical Care Unit, which was quite the intense experience! There were around 10 doctors and nurses waiting as they wheeled me into my bay, and I suddenly had a bunch of people bustling around and hooking me up to various things all at the same time. One of which was high flow oxygen, which helped IMMENSELY. Within half an hour I felt more awake and functional than I have since leaving the hospital. It was SUCH a relief!!

Unfortunately, high flow oxygen can't be done at home, which means I will now be inpatient until transplant. It also isn't exactly comfortable, though it's definitely more comfortable than constantly feeling like I'm suffocating. On the plus side, being on high flow bumps me way up the list, so hopefully I won't have to wait too long.

So here I am, back at HUP, my home away from home. This time I even get a special room with fancy monitors near the nurse's station so they can keep a close eye on me. Because we all know I like to bring the drama!

Listed!

As of this week, I am officially on the transplant list. FINALLY.

Now the waiting begins. The expected wait time is 2-3 months, but technically I could get the call at any moment if I happen to be the best match available when lungs come in. It’s kind of surreal, knowing that at any moment without warning I could be whisked off to massive life changing surgery. If I think about it too much it’s kind of scary, so I’m mostly not thinking about it. Same when it comes to the recovery process: I know it’s going to be grueling and painful, but I can’t do anything about it, so I’m just trying not to think about it. I’m more focused on how unpleasant my now is, and how ready I am for it to change.

I’m also very, very tired, so I don’t actually spend huge amounts of time thinking about much. For the most part I’m too exhausted for big thoughts and feelings. These days I kind of feel like I’m just… existing. And that alone is wearing me out. My existence right now is usually pretty uncomfortable, which is draining on multiple levels. I’m always, always struggling to breathe, some hours worse than others. Sometimes it really feels like I’m breathing through a straw, which is incredibly uncomfortable. I’m also still coughing up alarming quantities of thick, nasty mucus. I feel like I’m stuck in an infinite mucus loop: as soon as I cough stuff out, I fill right back up again. It’s exhausting.

Last week I had the added bonus of nausea and GI issues, probably due to the 7 different antibiotics I’ve been taking. So that just sucked all the life out of me, and I spent a lot of time curled up in a cranky ball of misery. Thankfully that seems to have eased off this week and I’m a little more functional. I’ve also been slightly more awake this week, so I guess the experimental antibiotic is doing something, even though it’s not as much as we would like. Due to this they’re extending it for up to 1 more week to see if we can squeeze out any more improvement. I’m all for improvement, but I’m also all for going home already, and am not thrilled that I’m stuck here at least until next week. But it does seem like I MIGHT be able to go home sometime next week, as long as I don’t do anything weird. They also have to make sure that the oxygen concentrator I have at home is strong enough for my new oxygen needs, and arrange for a replacement if necessary. I’m not feeling any better than when I was admitted – truth be told, I’m actually doing worse – but at this point we don’t really have any expectation of me doing better before transplant. If I’m going to feel gross anyway, I’d much rather do so in the comfort of my own home.

On the plus side, Pesach in the hospital wasn’t as bad as I anticipated. It turns out that a family a few floors down got a conference room and was making a seder, and they were thrilled to have a guest. So instead of a depressing seder-for-one on my tray table, I got to have a real seder with good company. We even had hot soup and food, which was so exciting! I don’t have any way to heat food up here on Shabbos and yuntiff since appliances aren’t really allowed, and I expected to eat cold food all yuntiff. But some family members were staying in a nearby apartment with a hot plate, so they brought thermoses and insulated bags, and we had warm food! It’s amazing how such a small thing can make such a big difference!

So that’s the story here as we head into the last days of Pesach. Here’s hoping that, just as the Jews left Egypt so unexpectedly fast that they couldn’t even bake their bread, my transplant comes through with such unexpected speed that it makes our heads spin!

Keeping on keeping on

Well, things haven’t exactly been going well around here. But then, I didn’t expect things to go well. At least I’m not disappointed.

After 3 weeks in the hospital, I’ve made no improvement. My lungs are still horribly congested, I’ve needed more supplemental oxygen (currently up to 5 liters at rest), my heart rate is sky high, and my endurance is terrible. Honestly, I feel like I may have even gotten slightly worse. If I did a pulmonary function test now, I have a sneaking suspicion that my numbers might actually be lower. Sigh.

My pseudomonas infection is being extremely stubborn and resisting all the antibiotics. The only ones left are ones that have risky side effects, such as hearing loss or kidney damage, or experimental treatments. I’ve already been on one antibiotic called Amikacin, which is related to Tobramycin and can cause hearing loss. Unlike Tobramycin, however, thus far Amikacin has not made my tinnitus worse, so we stuck with it – until this week, when a test showed that my hearing has deteriorated since the last time I was tested a year ago. Since the IV Amikacin wasn’t having a huge impact anyway, we switched to nebulized Amikacin to preserve my hearing. Most of my hearing is still at the low/borderline end of normal, but apparently I do really poorly with high frequencies. Whatever, who needs high frequencies? I’m not a dog!

I’m also on IV Colistin, which can cause kidney damage. We’re monitoring things very closely, and so far so good. Unfortunately, it doesn’t seem to be doing much in terms of actually making me feel any better. They’re now in the process of getting access to an experimental antibiotic which I may or may not be resistant to. We already determined that I’m resistant to the other experimental antibiotics – which actually impressed me, my super pseudomonas is resistant to drugs that AREN’T EVEN OUT YET – but for some reason this last one won’t let you test for resistance until you get approval to take it. So we’re going to start the drug and THEN test it, which seems a little backwards, but whatever. I’ve definitely seen weirder and more backwards things in the medical field.

They’re also getting things set up for me to start treatment for the NTM (non-tuberculosis mycobacterium). They don’t really think that’s the cause of my problems, but we’ve treated everything else so that’s the only thing left. Fortunately, they seem to have decided that nebulized Amikacin is adequate and I will not have to use IV Amikacin or Tobramycin. They’re still working on getting access to one of the other antibiotics I’ll need, but hopefully that should be settled this week.

On Monday I finished the last of my pre-transplant testing. Some of the tests are to determine how sick and in need of a transplant you are, but most of them are to make sure you’re healthy and strong enough to actually survive a transplant. Thus far I seem to have passed without a problem, so we’re all expecting the transplant committee to approve me when they present my case again on Thursday. It’s also likely that I’ll be listed immediately since I haven’t shown any improvement thus far.

I’ve been spending a lot of time time sleeping lately. Aside from normal hospital exhaustion, I also have pneumonia exhaustion, and just breathing takes a lot out of me these days. I’m also hacking up alarming quantities of mucus, which is very draining. I’ve spent a fair amount of time being extremely cranky, as Katherine and my mom can attest to. It’s hard to maintain a positive outlook when you feel so awful so much of the time. My nurses have definitely noticed that I’m not my usual chipper self, and that my health has deteriorated pretty dramatically just in the last few months. But they also say that I have a good attitude for going into transplant, and they’re hopeful that I’ll do great and feel so much better afterwards.

We’re hoping that I MIGHT be able to go home next week – not because I’ve recovered, but because there won’t be anything else they can do for me here. Once they get me set up with the last antibiotics to treat the pseudomonas and the mycobacterium, there isn’t really anything they can do here that I can’t do at home. It’s definitely better for me to be at home if possible, where I’ll be away from all the hospital germs, moving around more, and eating what I want when I want it. Activity and nutrition are VERY important pre-transplant, as the stronger you are going in, the more likely you are to recover well. I’m not thrilled that I came in with pneumonia and will likely be leaving with pneumonia, but if pneumonia is gonna be my way of life these days, I’d much rather have pneumonia at home!

Back to the hospital

I’m convinced my lungs looked at a calendar and are trying to make sure they hit every Jewish holiday.

Last week I did feel the tiniest bit better, even though it wasn’t anything to write home about. Sunday, however, I inexplicably started feeling worse. I hoped it was just a weird random bad day, but since then my oxygen has been dropping, I’ve been incredibly short of breath, and I’ve gotten significantly more congested. I was supposed to have a follow up appointment next week, but I emailed my doctor and they told me to come in today instead. They also mentioned that I might need to be hospitalized.

I felt pretty defeated yesterday. Hospital was NOT the word I wanted to hear, particularly since my last hospitalization was so long and brought so little improvement. It’s extremely frustrating to once again end up feeling WORSE while on IV antibiotics instead of getting better. It’s also scary, because we keep trying different treatments, and keep not seeing any significant improvement. It’s really hard to keep fighting when you never seem to get a win.

My lung function numbers ended up being even worse than I expected. Somehow over the course of a week, while on IV antibiotics, I managed to drop from 48% to 42%. At that point I knew there was no way out of being hospitalized, but I did ask my doctor what they can accomplish in the hospital that we haven’t already tried at home. She pointed out that in the hospital they have full access to all kinds of testing and other diagnostic procedures to help figure out what’s going on, and that it’s much easier for them to make quick medication adjustments and try different treatments. They tried to admit me directly from clinic, but there were no beds available. The only other way to get admitted was through the ER, but thankfully they had mercy on me and told me to go home and enjoy Purim instead. They’ll try to direct admit me again tomorrow, and if they’re still unsuccessful I’ll start yet another ER adventure after I finish my Purim seuda.

They really don’t know why my health has been so poor and why I’ve been so unresponsive to treatment. It feels like we keep throwing darts hoping to hit a target, except I think we might also be throwing the darts while blindfolded. We tried an antifungal and got rid of the fungal infections, we tried an antibiotic and got rid of the MRSA, we tried IV antibiotics to treat the pseudomonas… and yet, I’m still getting worse. So now we’re trying to figure out what is it that we’re NOT treating that could be causing these problems. There are currently 2 top contenders. One possibility is a pulmonary embolism, a blood clot in the lungs. We actually tried to schedule a CT scan today to rule that out, but my lovely insurance is dragging their heels in approving it, so I guess they’ll just have to pay for it in the hospital instead. The other possibility is aspiration pneumonia. Even with medication I have terrible reflux, and they’re concerned that I might be inhaling reflux in my sleep since I use a feeding tube overnight for extra nutrition and hydration. We’ve explored surgery to combat the reflux, but my health hasn’t been stable enough to move forward with it. However, one thing we can do is change my G-tube to a G/J-tube. A G-tube pumps the supplement directly into my stomach, whereas a J-tube would bypass the stomach and pump it into my intestines. This could reduce overnight reflux issues, and apparently is a very easy change to make. So that will probably happen while I’m inpatient.

I didn’t have the energy or brain power to pull together a Purim costume, but fortunately I did have an unused super fun hat stashed away in my closet. And when I pulled it out I discovered that it also came with a tail, so that was an exciting bonus! I made it to shul for megillah reading tonight, and tomorrow I plan to go to shul again before joining Martelle and her family for an early seuda. At least I have the chance to get my Purim on before embarking on the next round of medical drama!

After megillah reading, Sarah Bienenfeld reminded me that Purim is the time of v’nahafoch hu, of things turning around, and of sudden unexpected salvation. Hopefully this hospitalization will be fueled by the power of Purim and I’ll see my own v’nahafoch hu soon!

Keeping on keeping on

Welp, I managed to regain a whole 1% in lung function. Go me.

I am feeling a little better. My oxygen has been a little more stable and my heart rate hasn’t been going quite as high with exertion, though I do still need some supplemental oxygen when I’m out and about. I’ve also been a little less congested. Overall though the gains haven’t been too significant, so much to my disappointment my doctor wants to try one more week of IV antibiotics. Thankfully, however, we’re stopping the Zosyn and switching me back to Zerbaxa, aka the Fancy Restricted Antibiotic. This is so that I can get a LITTLE more sleep, since Zosyn is run every 6 hours, whereas Zerbaxa is run every 8 hours. I’ve been an utterly sleep deprived zombie these last couple weeks, so at this point I’ll take whatever I can get! I feel like a cat these days: I don’t actually sleep anymore, I just take naps of varying lengths at various points throughout the day. I think cats enjoy this more than I do though!

On the plus side, my last sputum culture did NOT show MRSA or either of the two fungal infections that popped up previously. Unfortunately, that hasn’t actually translated into any real improvement in my health. If I don’t see any real improvement with another week of IV antibiotics, we’re going to move on to treating the other infection that has been cropping up lately in my sputum cultures: mycobacterium chelonae, a nontuberculosis mycobacterium (aka NTM). We haven’t treated it until now because it’s unclear whether the NTM is actually the source of any of my symptoms, and treating it requires taking multiple antibiotics for a very extended period of time. Apparently it can also be difficult to get approval for some of the medications involved. And, one of the meds used to treat it is Tobramycin, the IV antibiotic which gave me tinnitus and can cause hearing loss. We’re exploring whether I can take the Tobra via nebulizer, which has less risk of causing hearing issues, but unfortunately in the past the nebulizer formulation of Tobra (called TOBI) gave me asthma attacks. I’ve asked whether we can try giving me the IV version of Tobra to inhale, which is what I did for years without any problems before TOBI was developed. It’s unclear however whether we’ll be able to get approval for an off-brand use of injectable Tobra since a nebulizer version is available.

The other main goal now is getting me to be a little more active. I’ve been feeling sort of awful both physically and mentally, which has led to me spending a LOT of time moping on the couch. I’m trying to pull myself together and leave the house a little more, even if only briefly, and to do brief, mild exercise at home a few times a day. I’m obviously not running any marathons, but whatever exercise I can manage is good for both my physical and mental health. I just need to be careful about not pushing my heart rate too high, as unfortunately the “leaky pipes” have still been an issue. Thankfully I haven’t had a full bleed in two and a half weeks, but I’ve been having varying degrees of bloody mucus at least once a week all month. I’m trying really hard to avoid anything that could trigger a full bleed, as that would force me to hold various treatments and prevent me from clearing mucus out of my lungs. On the other hand, I do need to exercise, because not exercising is also detrimental to my lungs as well as the rest of my body. It’s a really fun catch-22.

It has been a really, really difficult winter. Hopefully things will start looking up soon.

Phase 2

Two weeks ago my lung function was down to 45%. Last week I managed to pull it back up to… 47%. Yay.

So we moved on to Phase 2: IV antibiotics. Unfortunately, my most recent test results showed resistance to even more antibiotics, which partially explains the disaster in November. I’m pretty much down to Amikacin/Gentamicin/Tobramycin (all the same antibiotic class) and Zosyn when it comes to treating my chronic pseudomonas infection. The first 3 all have the potential to cause hearing problems, and we already know that Tobramycin gives me tinnitus (ringing in the ears), but the last time I took Amikacin it didn’t make the tinnitus worse so we’re trying that along with Zosyn. Unfortunately for me, Zosyn has to be infused every 6 hours, and it comes in bags that have to be hung from an IV pole instead of in the clever little self-infusing medi-balls that I usually get. So over the next couple weeks I won’t be getting much sleep and will be spending a lot of time chained to an IV pole. Sigh. Also, I’ve never taken Zosyn before, so here’s hoping it doesn’t turn out to be the next medication that I’m allergic to.

By the way, it only took a full week and doubling my dose of Prednisone, but the Vancomycin rash FINALLY faded. Everyone was quite impressed with its unusual stubbornness. Apparently my body REALLY doesn’t like Vancomycin.

In other news, after trying for the last 3 months, I finally managed to fail a walk test at clinic. This is actually a good thing! The last 2 times my oxygen dropped to juuuust above the insurance cutoff for in-home supplemental oxygen. I’ve been EXTREMELY grateful to the kind gentleman who gave me his old portable oxygen concentrator in December! There have been many times in recent months when I did need supplemental oxygen, but we just couldn’t get the required test results to justify it to the insurance company. Now I’ll be able to get an in-home concentrator that can provide a continuous flow of oxygen, as opposed to the portable concentrator that gives a puff of oxygen every time you breathe in. I tried to use the portable concentrator overnight once, but I couldn’t sleep with it because of the noise and the air puffing into my nose. A continuous flow concentrator will allow me to use oxygen overnight, hopefully giving my body a bit a of break and allowing me to get more restful sleep.

I also asked my team if there was any possibility of getting early access to Vertex’s “triple combo,” the new CF drug that’s on the verge of being released. They’re going to try, but told me not to get my hopes up, as they’ve not yet managed to get insurance approval for other patients in similar situations. And unfortunately, paying out of pocket really isn’t an option, because these drugs are OUTRAGEOUSLY expensive. The previous Vertex CF drug, Symdeko, costs $292,000 per year, and I expect the new drug to cost at least that much. So I’m not expecting anything, but who knows, maybe I’ll be the one in a million that actually gets insurance approval. Here’s hoping!

Ups and downs

The good news is, the “leaky pipe” issue FINALLY seems to be under control. During the last 3 weeks I’ve only had one brief incident of coughing up bloody mucus, and that was a week and a half ago. After on and off bleeding at various levels of severity for a month and a half, I’m almost afraid to talk about finally making progress, so here’s hoping I didn’t just jinx myself into a bloody disaster. Listen lungs, you don’t need to prove anything, just be chill!

I had a couple good weeks where my lung function was on an upward trend and seemed to be stabilizing. Despite the bleeding issues, my endurance was better and I barely needed to use supplemental oxygen. Unfortunately, that all came crashing down 2 weeks ago. After a day of increasing hoarseness, fatigue, and shortness of breath, I suddenly spiked a fever. I was convinced that I had caught a virus, but fortunately the fever only lasted one night and I didn’t develop any other viral symptoms. Despite that, since then I‘ve been extremely fatigued and even mild exertion sends my heart rate up and my oxygen level down. So I’ve been back on the supplemental oxygen train while we try to figure out what’s going on and what we want to do about it.

A few months ago if I was feeling like this they probably would’ve thrown me in the hospital. Now, nobody’s rushing to do anything drastic, since I just finished such a long course of IV antibiotics with so little return. I’m also reluctant to increase my steroid dosage, since I finally got the Prednisone down to 10mg and would REALLY like to get off it entirely due to all the side effects. Today we added in some IV fluids to see if extra hydration will help break up the chest congestion and improve my lung function. I have a follow up appointment on Friday, so we’ll see where things stand and figure out our next steps then.

Despite all this, I have been using most of my limited energy for very exciting party planning! Only half a week left until my big blowout 40^th birthday bash! I can’t believe how many people plan to come, and how many people have donated to the GoFundMe! I am so grateful and can’t wait to celebrate with everyone!!

They see me rollin'

I’m still dealing with what I’m now thinking of as Leaky Pipe Syndrome. Thankfully I haven’t had another full bleed, but I keep randomly streaking. Yesterday I finally managed 48 hours blood-free and resumed one of my treatments, only to streak at the end of the day and be forced to start all over again. Previously we wouldn’t react so strongly to just a little streaking, but my recent history of Big Dramatic Bleeds makes everyone anxious so we’re being very cautious. I have to keep reminding myself to Be Very Chill and not do anything to elevate my heart rate, which these days basically means not doing anything at all exerting. I am not enjoying being so limited and feeling this fragile.

Today I got my first experience using a motorized cart at Shoprite. I really wanted to actually go food shopping instead of trying to figure out what I needed online, but I also knew it definitely wasn’t safe to do that much walking. So I swallowed my pride and grabbed a motorized cart. I did feel awkward about looking perfectly healthy while puttering around on a scooter though, so I kept my oxygen on the whole time even though I didn’t really need it once I was sitting. I figured no one would question the girl with the nasal cannula. I managed to only run over Katherine’s foot once, and though I ALMOST knocked over a couple displays, nothing ACTUALLY ended up falling. Using the scooter ended up being a weird combination of fun, annoying, and occasionally slightly terrifying. Those things are harder to drive than you’d think!

Katherine has been amazing through all of this craziness. Since I’m mostly incapacitated, almost all the household tasks have fallen to her. I can put together a shopping list and zoom around on my scooter picking things out, but she’s the one shlepping it all home and putting everything away. Not to mention cleaning, and laundry, and just about all the things that keep a household going. I feel terrible about not being able to contribute much, but she does it all without complaint and reassures me that she’s happy to take care of things. I’m not sure how I got so lucky!

Of course, she did get entertainment value watching me almost take out an entire cereal display with my scooter, so maybe it’s a fair trade. Here’s the photographic evidence she took of my Shoprite experience. Maybe next time she’ll get video!

Not again...

I guess I should’ve gotten an update out yesterday before anything had a chance to go wrong…

I felt noticeably better over the course of the weekend. My heart rate didn’t shoot as high with mild exertion, I was able to maintain oxygen without using the concentrator, and I had a little more energy. At my appointment on Wednesday my pulmonary function was up to 52%, which made me very happy. Overall we were pleased with how things were going and I seemed to finally be on a stable, upwards trend.

But apparently this rollercoaster isn’t over QUITE yet. This morning, after 2 full weeks with not the slightest hint of blood in my mucus, I suddenly coughed up 15 mL of pure blood out of nowhere. I literally yelled “NO!!!” as soon as it happened, because I was so frustrated to once again take a step backwards right when things were looking up. Fortunately it was less than the bleed from 2 weeks ago and wasn’t close to ER level, but I still can’t help feeling that my lungs are like dilapidated, rusty pipes right now, just springing leaks all over the place without warning. So once again I’m holding various treatments until I’m completely blood free for 48 hours. Unfortunately this time I’m not on any home infusion, so I don’t have that extra support to help make up for the missing treatments. Hopefully the bleeding will stop soon and I won’t lose too much ground.

I also got to do a super fun barium swallow yesterday to see whether I’m aspirating things into my lungs when swallowing. Fortunately it turns out that I’m swallowing fine, but I’m definitely having significant reflux (which we already knew), and there’s concern that I may be messing up my lungs by aspirating that. I’m already on a bunch of reflux meds, which control the acid level but can’t stop the reflux from coming up. We started exploring surgical options a few months ago, but put that on the backburner while dealing with respiratory issues. Once we get things stabilized the plan is to continue testing and consultations to move towards surgery and see if that helps with some of the respiratory issues.

On the plus side, before things went south, Katherine and I took advantage of the good weather and my relatively decent health to go to the Mummers Parade! I’ve been watching this Philadelphia New Year’s Day tradition on TV since I was a kid, but never actually made it there in person. We shlepped the wheelchair on the train and joined the crowds at Broad Street and Sansom to watch the string bands perform, and had a great time! And one of the mummers even gave me his parasol, which was super cool!! Apparently looking pathetic in a wheelchair has its advantages!

I’m definitely frustrated about backsliding once again, but I’m glad it at least waited until after I squeezed in a great experience! Hopefully that was the last hurrah for this CF exacerbation, and I’ll get back on a steady upwards trend soon!

One more week

As I predicted, we’re keeping me on IV antibiotics for another week. I was surprised, however, to hear that my doctor originally expected to send me back into the hospital at yesterday’s appointment. Fortunately, I looked better than she anticipated, and my chest didn’t sound any worse (though it also didn’t sound any better). She would have stopped the IV antibiotics yesterday, but I could use the extra support while recovering from the recent bleed, particularly since I’m still holding chest PT and half my nebulizer treatments. If I can just stay blood free for 48 hours I can gradually resume those treatments and get back on track with clearing the congestion out of my chest.

The good news is that there is FINALLY an end in sight. As long as nothing stupid happens within the next week, we will finally stop the IV antibiotics next Friday. Depending how I’m feeling we might continue the IV hydration, but we might also just give me a break from everything, since by then I will have been on IVs for more than 8 weeks. The nice thing about the port is that giving me a break is a viable option since it’s so easy to just re-access it and resume IVs if necessary. Though I wish I had seen a stronger response to treatment, I am very much looking forward to moving on from endless IVs and trying to put my life back together.

Yesterday, for the first time, I went into a store with my new oxygen concentrator. It was an interesting and conflicted experience. I definitely appreciated the fact that the oxygen made it easier for me to move around without huffing and puffing. But I also had conflicting thoughts and feelings about making my until now mostly invisible illness suddenly and inescapably visible. On the one hand I appreciated and on some level even enjoyed the validation of having something external to match the internal struggle. There have been many, many times over the years when various every day tasks were extremely difficult for me, and yet you would never know it from the outside. It can be very frustrating to push through your day feeling like a train wreck while looking like the picture of health. On the other hand, it felt very odd to walk through the store with a blatant sign of severe illness strapped to my face, knowing that everyone who saw me instantly knew that something was very, very wrong. I found myself wondering what people saw when they looked at me, this baby-faced girl afflicted with some terrible mysterious illness, a tragic image to tug at your heartstrings. I wasn’t entirely comfortable with strangers being able to immediately categorize me as the sick girl without knowing anything else about me. But that won’t stop me from living my life and using the medical supports I need to do so. It was an interesting experience, and I’m sure I’ll get used to it with time.

Hopefully my lungs will behave themselves and stop. freaking. bleeding. so I can get back on track with treatments and exercise and continue clawing my way back to health!

In it for the long haul

Well, that didn’t exactly go as I had hoped.

At the end of October my lung function was at 57%. Just 2 weeks later, despite IV antibiotics, it had unexpectedly plummeted to 45%. After three weeks in the hospital and an additional week of home IV antibiotics, my lung function now is up to… 48%.

I knew I was nowhere near my baseline of 60%, but I thought I had at least made it back into the 50s. It was definitely disappointing to see so little progress after so much treatment. I’m certainly doing much better now than I was 6 weeks ago, but no matter what we try I just can’t seem to get very far. My lack of progress doesn’t make sense, because various tests have shown that my infections SHOULD be susceptible to the 4 IV antibiotics we’ve tried so far. But for some reason, during each of the last 2 rounds I only made a little progress before stalling. We’re going to try 1 more IV antibiotic and see if that finally makes a difference. Unfortunately, that antibiotic is Tobramycin, which caused tinnitus (ringing in the ears) back in January that never fully faded. Hopefully a brief course won’t have auditory side effects, but I will be monitoring things carefully, and we will stop at the first sign of any problems.

If I still don’t have any progress after a week of Tobra, we’re going to take a break from IV antibiotics and give my body some time to heal on its own. We’re also going to do some testing to see if it’s possible that my terrible reflux is contributing to the problem, and if maybe some of this is being caused by aspirating things into my lungs. Unfortunately, the steroid which is helping control the inflammation can also exacerbate reflux, so it might actually be making things worse. I’m in the process of starting a monthly injection used for severe asthma in the hopes that it will enable me to get off of or at least lower the steroid.

On a positive note, I do have a terrific hashgacha pratis (divine providence) story! While hospitalized I met one of my neighbors as we both walked up and down the halls getting our exercise. He doesn’t have CF, but he did have a lung transplant back in March. It turns out that he had multiple oxygen concentrators that he no longer needed, and he and his wife had been looking for a person or agency to donate them to in order to pay it forward. This week they dropped one off at the outpatient clinic, and I am now the proud owner of my very own portable oxygen concentrator! This is GREAT, as my oxygen continues to drop with exertion, but not low enough to qualify for supplemental oxygen to be covered by insurance. These machines cost thousands of dollars and I never would have gotten one on my own. Having supplemental oxygen available will make it easier for me to be active and get back on track with exercise so I can build my strength back up. So, I HAPPENED to be next door to this gentleman, who HAPPENED to have a spare oxygen concentrator, EXACTLY when I could benefit from in-home supplemental oxygen for the first time in my life but had no way to get it. Thank you Hashem!!

This has definitely been a long haul. It’s still unclear how much longer of a haul I can expect, or what the eventual outcome will be. Which is, honestly, frightening, frustrating, and discouraging. But I am still hopeful that things can get better than they are now, whether through treatment or simply with time. And even if they don’t, I am also confident that I will learn to adjust to my new normal and figure out how to live life within it, just like I have in the past. One way or another, I will roll with the punches and come out on top, just like I always do.

Holding steady

Well it was definitely a FRA-lichen Chanukah (thank you Shira Rubin for the wonderful wordplay)!

It is sooo good to be home! I’m still not getting excessive amounts of sleep due to coughing and needing to run an IV every 8 hours, but I am far more rested than I was in the hospital. And of course the food situation is greatly improved! Week #2 of the Fancy Restricted Antibiotic, however, hasn’t been quite as dramatic as I would’ve liked, and medically I’m basically at the same place as I was when they discharged me. I’m still dealing with a fair amount of congestion and tightness. My oxygen levels mostly hold steady when I’m sitting still, but slide with the slightest exertion. Unfortunately for me, they only slide to juuuust over the cutoff point for supplemental oxygen. Which means it’s difficult for me to manage basic daily living tasks, but I also don’t qualify for the in-home supplemental oxygen that would make things easier. It’s a bit of a catch-22: I know I’m deconditioned and need to exercise, but I don’t have the supplemental oxygen that would make exercise easier. I do have a possible lead on that issue though, and it may be resolved within the next couple days… Stay tuned for further developments!

Another catch-22 is that for the last week I’ve been having sporadic episodes of coughing up small amounts of blood. The usual response to that is to hold some of my more irritating treatments to allow the wound to heal – but those treatments are also clearing the congestion out of my chest. Clearing congestion helps reduce infection, which helps reduce the risk of bleeding. So we’re walking a delicate balance between not provoking a bigger bleed – because G-d knows I don’t need any more drama in my life! – and not undoing the progress we’ve made in clearing things out. Oh, and all my bleeds in the last week have followed more significant exertion, since an elevated heart rate puts more pressure on the blood vessels and makes it more likely that any weak spots could give way. Apparently I need to just be very chill for the next however long until things finish healing… which doesn’t exactly help with the whole trying to exercise and not become even more deconditioned thing.

Of course I can’t just be boring and straightforward about things, everything has to be complicated. I’m so high maintenance.

My follow-up appointment is on Wednesday, and I fully expect at least another week of treatment, possibly with yet another antibiotic. My lungs are being so very stubborn this time around (which I guess shouldn’t be a surprise, they are MY lungs after all)! I’m super curious to see what my pulmonary function testing looks like. I know I’ve improved from that dismal 45% a month ago, but I also know I’m not back to my usual 60%. The question is how much progress have I managed, and how much more might I be able to accomplish.

So, not exactly the glowing update I would have liked to share, but also not terrible. Holding steady is definitely better than going down! And being home is definitely, DEFINITELY better than being in the hospital!!

Almost there!

B”H I think I have FINALLY turned the corner!

The last couple days I’ve been noticeably less dependent on supplemental oxygen. I’ve mostly been off it while at rest, and haven’t needed as much while walking around. Overall I’m breathing and moving better and have more energy. It seems that the Fancy Restricted Antibiotic is doing the trick!

Today my doctor gave me the best Chanukah present ever by finally talking about DISCHARGE! I still need more treatment, but thankfully I’ve gotten to the point where I don’t need to be in the hospital for it. The plan is to get me back on home IV antibiotics and fluids so I can finish up the Fancy Restricted Antibiotic in the comfort of my own home. I’ll also still need to make sure to do LOTS of extra nebulizers and chest PT to keep working on clearing out the remaining chest congestion. But it’s so much easier to do all that at home instead of in a hospital bed!

The question now is how I end up feeling tomorrow and how long it takes to set up all the home care services. If all goes well, I should hopefully be going home within the next couple days!

I am SO grateful that I will be able to have at least part of Chanukah at home and finally get to light my menorah! Turns out the Fancy Restricted Antibiotic really was my Chanukah miracle!

Slow going

If I had to pick just one word to describe this hospitalization, it would probably be “slow.”
I’ve definitely improved from when I got here. My airways are more open and I’m more awake, and I’m using less supplemental oxygen. But any progress has been incremental and very, very slow. At this point I’ve basically been stalled in the same place for nearly a week now. My airways are better, but still tight. I’m still congested. I’m still dependent on supplemental oxygen both at rest and with exertion. I’m also still on a high dose of steroids. I got my nurse to pull up some of my test results today, and not only has my white blood cell count remained at the same elevated level (which means I’m still battling infection), but today’s chest x-ray looked about the same as the one we took when I got here. I just don’t seem to be gaining ground.
Today I got to have a special consult with the Infectious Disease team, which I thought sounded wonderfully dramatic. It was particularly dramatic when they stood outside my door, still in the hallway, and loudly announced “We’re here from INFECTIOUS DISEASE.” So I’m pretty sure the whole unit now thinks I have the plague or something. Making friends and going places!
The purpose of the consult was to determine whether or not I could get approval for a “restricted” antibiotic. Due to the ever-increasing risk of treatment-resistant bacteria, the hospital holds a few antibiotics in reserve to only be used if nothing else works. By using these antibiotics sparingly it is hoped that they will remain effective for longer. Since I’ve been less than responsive to treatment for a few years now, with this exacerbation being particularly bad, I got the green light to get what I am officially calling Fancy Restricted Antibiotics. I got the first dose tonight, and they’re giving me up to a week to see results. If it helps, I’ll get a full 2 week course. If it doesn’t, they’ll (I imagine) whisk it back to a secret vault somewhere and lock it up tight until they can find some bacteria who actually appreciate it, and will put me back on regular antibiotics.
The big question that no one can answer is how much, if any, permanent lung damage I’ve taken this time around. It’s possible that this is my new baseline and I can’t get any better. It’s also possible that I *will* get better and am just doing so very, very slowly. Unfortunately there’s no way to predict the outcome and we just have to wait and see, possibly over the course of weeks or months. I’m trying not to be an impatient patient here, but it’s not easy.
On the plus side, now that I’m more awake, I’m more able to handle visitors. Please plan it with me though so I don’t have too many people here at once – I’m ready for a few visitors, not a party. Also, if you have ANY cold/flu/respiratory symptoms whatsoever, or have been around people who do, PLEASE keep those germs far, far away from me. If you’ve been exposed to a respiratory infection, it’s entirely possible for you to be contagious before you even start showing symptoms. I don’t even want to imagine how miserable I’d be if I caught a virus on top of the current mess.
Here’s hoping the Fancy Restricted Antibiotic ends up being my Chanukah miracle!

Struggling

So things have been less than stellar around here…

Even with supplemental oxygen I was having trouble breathing. Things finally stabilized once we bumped me up to 4 liters of supplemental oxygen, but even with that my oxygen saturation was still a bit low. I was also REALLY struggling with “tightness,” which is how I describe the feeling in my chest when airways are inflamed and irritated. The tightness was so bad that it prevented me from taking deep breaths and clearing out the congestion, instead leaving me with a dry hacking cough. I was also struggling with significant stomach issues, and all the coughing didn’t exactly help with nausea. All in all I was not living my best life.

After a few days with no progress, the doctors decided to try adding another antibiotic called Amikacin. Amikacin is in the same family as Tobramycin, and also has a risk of hearing loss, but less of a risk than Tobramycin. They also decided to start waking me for nebulizers every 4 hours to prevent me from losing ground overnight. So for the last few days I’ve been even more sleep deprived than usual in addition to all the stomach and respiratory symptoms. I’ve pretty much been sleeping most of the day and feeling pretty gross during my few waking hours.

Yesterday we finally saw a small amount of progress. Various medications finally kicked in and started getting the stomach issues under control. I was also able to lower the supplemental oxygen for the first time, and maintained decent oxygen saturation even with only 2 liters of supplemental oxygen. Unfortunately, my chest is still really tight and I’m still not moving air well. They’re going to try increasing the dose of Amikacin, but if there isn’t real improvement within the next few days we’ll have to move on to Tobramycin instead.

This has been my worst CF exacerbation in a very long time. Hopefully things will start to turn around soon.