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After almost 6 weeks in the hospital, I am FINALLY home!!

Donor Dash!

So this happened. Welcome to the 2019 Gift of Life Donor Dash, transplant unit style!

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!

Almost listed

So, I’m ALMOST on the transplant list.

The committee decided that I’m a good candidate for lung transplant, and they’re ready to list me. Buuut there’s one last hold up. For about 2 weeks now we’ve been trying to get authorization for Clofazamine, one of the antibiotics I’ll be taking to treat the mycobacterium. I’ll need to continue mycobacterium treatment post-transplant to prevent infection in the new lungs, and they need to determine whether I can tolerate Clofazamine or if they’ll have to find a different medication. So I won’t be listed until the stupid authorization comes through and I have a few days of Clofazamine in me. But once that happens, it’s on like Donkey Kong!

My health has definitely deteriorated during the last 3 weeks. They already gave me a Lung Allocation Score (LAS), which determines your placement on the transplant list, but they plan to redo some of my testing and the expected lower scores will bump me a little higher. If I continue deteriorating my LAS will keep going up, which means less time waiting for transplant. The expected wait time they initially told me of 3-4 months is now down to 2-3 months, and could get shorter. Basically, I should be sporting shiny new lungs for the summer. Talk about getting your beach body ready!

One of my nurses said if you’re getting a transplant anyway, it’s actually better to be relatively healthy and then suddenly get very sick very quickly. If you deteriorate gradually over time, you’re more likely to lose weight and muscle tone, both of which are important for recovery. Getting sick and getting transplanted quickly means your body is more likely to be stronger and more able to cope with the rigors of transplant. So apparently I’m doing this exactly right. Go me!

One big bummer is it looks like I won’t be discharged before Pesach. I started an experimental IV antibiotic on Wednesday (how cool am I?), and unfortunately it seems to be difficult to get, even more difficult to send home, and nearly impossible to ship out of state. They want me on it for 2 weeks, so unless they pull together a miracle I’ll probably be having my sedarim in the hospital. I’m really not thrilled about that, but it does fit with my current trend of hospitaling my way through the Jewish calendar. My rabbi pointed out that I should probably expect to have the transplant on Shavuos. He’s probably right!!

Aside from that it’s status quo around here. Please send red-tape cutting thoughts my way so I can finally get on the Clofazamine and get this show on the road!

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!

Hospital decor: Pole People!

The Pole People make their YouTube debut!

Why don’t you get a lung transplant?

“Why don’t you get a lung transplant?”

I have heard that question countless times over the decades, and more frequently as my health has declined in recent years. Each time I secretly gritted my teeth and reminded myself that most people are nowhere near as familiar with pulmonary disease as I am before trying to answer calmly. So I figured I should give a public answer to the question on so many people’s minds: Why doesn’t she get a lung transplant?

Short answer: Because it could kill me.

Long answer: All transplants are complicated, and lung transplants particularly so. CF patients always require double lung transplants, because both our lungs are so heavily impacted and full of infection that a single lung or partial lobe transplant is basically worthless. Statistically, close to 90% of lung transplant recipients survive the first year post-transplant, but that number drops to about 50-60% for surviving 5 years post-transplant. They aren’t going to consider a transplant until you’re already running out of time, because there’s a significant chance that the transplant itself could end up shortening your life.

Then you have to be lucky enough to actually get a transplant. You can’t exactly walk into Lungs-R-Us and grab a pair, and people tend to need their lungs while they’re still alive, which means we’re dependent on lungs from deceased donors. And that donor needs to match on several different criteria, the lungs need to be healthy and undamaged, and the donor needs to be within a certain geographic area as organs can only travel so far. Being listed doesn’t guarantee if or when you will receive lungs, and some people unfortunately die while waiting.

Once you get a transplant, the complications begin. The surgery itself is long, grueling, and dangerous, and is followed by a lengthy and complex recovery period. If all that goes well, then it’s time to adjust to your new life as a transplant recipient. That includes a lifetime of immunosuppressive drugs to prevent your immune system from rejecting the foreign organ in your body, which means massive lifestyle changes to avoid the infections that your weakened immune system can no longer fight off. This is particularly tricky with the lungs, the only transplant organ that is constantly exposed to the outside environment and all its contaminants with every breath you take. And even with all your best efforts, you will still likely experience and need to be treated for some form of rejection. There is an endless array of potential post-transplant complications, many of which can be fatal.

Is it worth it? If it goes well, yes. It’s basically trading one chronic illness for another, but at least in this one there’s a chance you might be able to breathe and possibly even function as a mostly-normal person. But it’s definitely not worth trying while you still have a fair amount of life and normal(ish)-person-ness left in you.

All that being said…

On Monday, my doctors shocked me by saying they wanted to start the process of getting me on the transplant list. I thought my lung function needed to be lower and that I had more time, but the fact that I’ve been having such frequent, severe, lengthy CF exacerbations that have been so minimally responsive to treatment makes me eligible. They’re also very concerned about the fact that we’re almost out of antibiotics that are at all effective against my pseudomonas infection. I’m running out of time more quickly than expected, and they want to pursue this option while I’m still healthy enough to have a greater chance of coming through it successfully.

This week has been a crazy whirlwind of testing and consults. Today the transplant team met and determined that, as long as the last few tests go well, I am a candidate for lung transplant. They have not yet decided whether or not to list me immediately, as they want to see how I respond to one last antibiotic. If it goes well, we may hold off a few more months. If, as I expect, it doesn’t have a significant impact, then they will likely list me immediately. And apparently HUP is a high volume transplant center, so the expected wait time once listed is only 3-4 months. Basically, by this time next year, it’s expected that I will be post-transplant.

It’s been a wild ride of a week. I’ve definitely been stunned and terrified, but at the same time I’m also relieved. The last 5 months have been pretty terrible, and the thought of slogging through a few more years like this was kind of horrifying. I was beginning to lose my will to fight, as I’ve been working so hard yet seeing almost no return. Things were starting to feel kind of hopeless and pointless, as I was spending so much time just surviving and very little time actually living. I honestly wasn’t sure how much longer I could keep going on like this. So as much as transplant is terrifying, it has also revitalized my will to fight, because at least now I have a goal and a chance of things being better. I can do grueling, I can keep hanging on by my fingernails, as long as I know that this fight won’t be forever and might actually accomplish something.

I’m very tired. And I’m definitely not looking forward to the trials coming my way. But I’m also not done quite yet. Bring it, CF! You still haven’t gotten the best of me!