Struggling

I just realized it’s been almost 3 months since I wrote a health update. I’ve *thought* about writing, and really felt like I *should* post something, but I just haven’t been able to muster up the mental energy to actually do it. Don’t let the social media break fool you into thinking that my life is quiet, however. It’s almost always a safe bet that I have some sort of stress and drama going on.
 
I once came across a little picture book called All My Friends Are Dead, which was a good fit for my often dark and morbid sense of humor. I’ve been thinking of that book a lot lately, because my life is starting to feel that way. This year has been pretty brutal for the CF server on Discord. In May we lost Jacob, less than 4 months after his double lung transplant, at just 25 years old. And then, a couple weeks ago, we lost Eve. Eve got her transplant in 2019 at only 13 years old. She struggled with rejection pretty quickly, and was waiting for a second transplant, but sadly she couldn’t hold on any longer. She was just 15 years old.
 
In the midst of all this death and tragedy, I also had my own health struggles. In June I started running a low grade fever, culminating in a sudden spike to 102.4. You don’t play around with any sign of infection when you’re immunosuppressed, particularly fevers, so a fever that high sent me straight to the ER. I followed almost exactly the same fever pattern last summer, and sure enough I once again had a pseudomonas infection brewing in my lungs. You might remember from last year that my pseudomonas has basically been supercharged from decades of antibiotics and is now resistant to almost everything. There’s now only 1 IV antibiotic that we can use without putting my hearing and/or kidneys at risk. Fortunately, that antibiotic can now be administered at home, so I only had to spend a few days in the hospital instead of the full 2 weeks. Unfortunately, I had to give myself a 3-hour infusion every 8 hours, which was divided into 2 separate 1.5 hour doses, so I didn’t get much in the way of sleep that whole time.
 
It’s very frustrating to keep battling these infections, especially since I know where they’re coming from yet can’t do much about it. I already had surgery last summer to try to clear out my sinuses and reduce the infections, but it doesn’t seem to be helping. I’m now back on a nebulized antibiotic every other month to try to keep things under control, and probably will be for life, since I ended up in the hospital when I tried stopping for a few months. My current medical routine is still WAY better than my pre-transplant life, but I can’t help wishing things would be just a little easier. Of course, then I feel bad about complaining, since I know many people struggle so much more than I do. Survivor’s guilt sucks.
 
And then of course there’s Covid. While it seems that the rest of the world is making their way back to business as usual, I remain mostly locked down. I remain at high risk for severe, life-threatening illness, as unfortunately my anti-rejection meds prevented me from producing Covid antibodies. This wasn’t surprising, but was still disappointing. My doctor does believe, however, that the vaccine produces other immune responses that don’t show up on testing, as vaccinated transplant recipients aren’t getting quite as severely ill if they catch Covid. There’s also evidence that some immunosuppressed people who didn’t respond to the first 2 shots DO respond to a 3^rd dose, so I got my 3^rd shot just over a week ago. Here’s hoping!!
 
Overall I’ve had a lot weighing me down over the last few months. I wasn’t exactly a paragon of emotional stability before that either, so I really didn’t need more stress and sadness in my life. With Covid continuing to hang over my head, and the annual danger of cold and flu season coming up, things aren’t feeling particularly hopefully right now. I am physically and mentally exhausted.
 
I don’t look at Facebook very much these days, so please don’t be offended if you share something important and I don’t react. Chances are I didn’t see it. I’ve also been even more terrible than usual at responding to texts and phone calls, as I tend to crawl into an antisocial cave when I’m stressed and unhappy, so please don’t take it personally. I really appreciate the efforts of those of you who continue reaching out to me despite this! Thank you for putting up with me!

Snow day sculpting!

 A solid storm with perfect sculpting snow means I got to make my first REAL snow sculpture since before transplant! 3 years ago Katherine and Frank had to pile up the snow for me because my lung function was too low to manage it. I still needed Katherine's help today, but I also was able to do more than half of the heavy lifting myself! Yay new lungs!! And so, in today's edition of snowmen are for amateurs, I'm proud to present: Kimba!

One foot in front of the other

I’m tired.

Since getting home from the hospital at the end of August, I’ve been very fatigued and kind of depressed. I wasn’t exactly full of joy and energy before then, but things seemed to get markedly worse. After about a month I realized that this coincided with starting Trikafta, so I reached out to my doctor. Turns out these can be side effects of Trikafta, so we halved my dose. I felt less depressed the following week, but have still been very fatigued. My doctor said this could be due to the CMV flare up over the summer, and that unfortunately it can take a while to recover.

Part of the problem is that it’s very difficult to tease out which issues are medical symptoms, and which are related to mental health. Transplant is a VERY intense experience, and depression and anxiety after transplant are not uncommon. I still have lingering trauma from what I went through before and during the transplant process. In addition, life after transplant is fragile and uncertain, a reality that is very stressful to live with. And as if that isn’t difficult enough, I also end up feeling guilty about being depressed and anxious! I feel like I should be perpetually full of joy and gratitude for this precious gift of life and relative health, and that I have a responsibility to live my best life for my donor as well as for myself. So I end up not only feeling depressed and anxious, but feeling bad about feeling depressed and anxious. It’s super fun.

Now take all that, and throw a life threatening and confining global pandemic on top of it. Just what I needed: another threat to add to my already long list of fears! How am I supposed to distract myself from those fears and live my best life when I can barely leave the house? I’m dealing with the same stresses and fears and struggles as everyone else, ON TOP of my already elevated baseline of stresses and fears and struggles. This makes it REALLY hard to figure out the cause of various symptoms. Is the fatigue a symptom of depression, or a medical issue? Have I been depressed because of medication, or because of the world at large? What is going on???

Despite all this, overall I’ve still been doing really well medically. I had a follow up appointment on Friday and once again my lung function was an amazing 92%! My chest CT scan looked good and showed that the pneumonia from August has cleared up completely. Tomorrow I have a bronchoscopy, so we’ll see how my airways look and get a sputum sample to check for infections. Here’s hoping Pseudomonas finally got the message and moved out!

I’m just trying to put one foot in front of the other and keep getting through this 2020 life. Hopefully it’ll get easier soon!

Looking back

Rosh Hashana was a week ago, so tis’ the season to take an accounting of the previous year! One year ago, my intense joy and gratitude for being alive after an intense life or death battle was beginning to be tempered by the difficulty of dealing with various post-transplant complications. Transplant complications unfortunately became a bit of a theme for the year, though thankfully nothing TOO severe. Nonetheless, life was already pretty stressful even before Covid hit. But I definitely didn’t expect to go into my second Rosh Hashana after transplant overwhelmed and exhausted from a far more subtle yet just as real months-long struggle for survival.

This has been my first year of post-transplant life. It has been marked by incredibly good progress, including a terrific level of lung function and no hint of rejection. But it definitely hasn’t been smooth sailing. I became diabetic due to my medications and had to learn how to manage my blood sugar with insulin. I spent last September through November almost incapacitated with terrible joint pain caused by one of my medications. I’ve been getting bronchoscopies every 1-2 months because my airways keep closing up. I’ve had repeated lung infections, which led to weeks of IV antibiotics, and ultimately resulted in pneumonia and a 2 and a half week hospitalization.

I also have CMV. CMV (Cytomegalovirus) is a common virus that many people carry asymptomatically. One of the reasons my transplant was considered high risk is because my donor had CMV, while I did not. Things were so dire before transplant that I didn’t consider turning down these lungs for even a second, but this means that we knowingly put a new virus into an immunocompromised person. Due to this I was on antiviral medication for the first year after transplant. After a year without any signs of CMV we tried stopping the antiviral, but a few months later CMV showed up in my bloodwork. I’m now back on the antiviral and likely will be for life. This isn’t a big deal, but a donor-recipient mismatch for CMV increases the risk for chronic rejection, as does the fact that I keep battling Pseudomonas infections.

We also don’t entirely know the current status of my Pseudomonas infection. I haven’t noticed any overt symptoms, but I also didn’t notice that I was developing pneumonia until I started running a fever. I’m scheduled for a follow up CT scan in 3 weeks to make sure the pneumonia is gone and that my lungs look ok. A week later I’ll have a bronchoscopy and will probably have my airways dilated once again. They’ll also take a sputum sample, which will show whether or not I still have Pseudomonas in my lungs. I kind of have an undercurrent of anxiety about Pseudomonas possibly lingering in my lungs, since it very much snuck up on me last time. I won’t really believe it’s gone until we get clear test results.

Of course, even if it is gone, it will never be GONE gone. There’s no way to clear out every nook and cranny of my sinuses, which are still full of infections and sticky CF mucus. Hopefully that’s improved now that I’m taking Trikafta, but when you have CF it’s almost impossible to fully eradicate infections like Pseudomonas. The question is whether we can improve my sinuses enough to keep them from repeatedly infecting my new lungs and putting them at risk. Only time will tell.

Pandemic life hasn’t exactly been kind to me. On the one hand, in some ways I had less to lose than a lot of people. My world was already pretty small, and I was already abiding by many of the restrictions that were new and surprising for everyone else. On the other hand, my life is very much in danger from this virus, which means I need to be even more careful than most people, and I’ve known from the start that I’ll need to do it for longer. On top of that, I’m keenly aware of the fact that I’m living on borrowed time and have no clue when the clock will run out. Most people anticipate many full years after the pandemic during which they can make up for the time and experiences they’re missing now. I, unfortunately, don’t have their confidence. It’s extremely hard to watch my precious post-transplant time being wasted by the pandemic. I have these wonderful new lungs, I have this precious gift of extra, unexpected life – but right now I can’t do anything with them, and I have no guarantees about how much time I’ll have left to enjoy them once the pandemic is over. It’s extremely frustrating.

I’m very, very good at anticipating and stressing about all the things that could possibly go wrong. This is particularly unfortunate after transplant, because it’s a LONG list! With Covid, I have fewer opportunities to distract myself with joyous life experiences, and more space to sit in my fears. It honestly hasn’t been great.

5780 has been an unexpectedly difficult and overwhelming year on multiple levels. Hopefully things will turn around in 5781!

Moving up in the world!

Current lung function: 92%! That’s my highest in 13 years! It’s overwhelming and emotional to think about how low I was just a year and a half ago, in contrast to how high I am now. It’s truly miraculous!

As my dear friend Mikey used to always say: Baruch Hashem yom yom! Day by glorious day!

Complications

Yesterday was LONG.

I went down for the bronchoscopy around 2:45pm, but didn’t go into the OR until after 4pm. When I woke up around 6:30pm they said a room was finally available and being cleaned, so I waited in recovery instead of going back to the ER. By the time the room was ready, however, it was 7pm shift change, and nothing happens easily at shift change. I eventually got upstairs shortly before 8pm, but I STILL couldn’t eat because my meds hadn’t arrived from pharmacy yet, and I can’t eat anything without digestive enzymes and insulin. I FINALLY got my first (and only) meal of the day at 10:30pm. Whew!

Unfortunately there wasn’t a room available on my usual unit, Silver 11, so I ended up on Founders 14 instead. Silver lining: Founders 14 has the BEST views!

Today we got an explanation for how on earth I ended up with pneumonia after a month of IV antibiotics. Despite the fact that testing in June showed that my Pseudomonas infection was susceptible to Zosyn, the new testing showed that it was RESISTANT. I don’t know when that happened, but clearly the latest round of IV antibiotics was ineffective. So far they’ve identified 2 effective antibiotics: Tobramycin, and Amikacin. Those names might ring a bell… Tobramycin gave me tinnitus, and Amikacin caused some hearing loss. We’d REALLY rather not use those antibiotics if at all possible, so they’re doing more testing to see if we have any other options.

I also learned a new word today: Immunoglobulin G. Since I’ve been struggling with stubborn infections lately, they did bloodwork to check my antibody levels. They’re supposed to be low since I’m immunosuppressed, but turns out they’re even lower than expected. Apparently this isn’t unusual after transplant, and has a pretty easy treatment. We obviously don’t want to lower my immunosuppression, so instead I’ll just use someone else’s antibodies! Immunoglobulin G is an antibody that can be taken out of donated blood and infused into someone who needs it. Hopefully it’ll be the boost my body needs to fight off this super stubborn Pseudomonas infection!

Unfortunately, these complications mean I’m not getting out of here too quickly. It’ll take time to complete testing and determine a course of antibiotics, and then they need to get me stabilized on the right dose before sending me home. So I’m here through the weekend, and then we’ll have to see how things go next week.

On the plus side, I did have a reassuring conversation with my doctor today. I was a bit alarmed by this whole situation, as it was far too similar to last year’s unsuccessful battle with treatment resistant Pseudomonas which resulted in my lung transplant. My doctor reassured me, however, that this time it will NOT cause permanent lung damage. It’s almost impossible to eradicate Pseudomonas from CF lungs. You can reduce and control the infection, but it’s always there, causing permanent damage which builds up over time. These lungs, however, are different. Once we find an effective antibiotic, we’ll be able to successfully treat and eradicate the infection before it causes permanent damage. Hearing that was a HUGE relief!!

So for the first time in over a year I’m settling in for a stay at my home away from home here at HUP. And we all know what that means… Pole People, of course!

Round... wait, what round am I up to?

Well that didn't exactly go as planned. 

During the last week I've struggled with off and on low grade fevers. I've been pretty wiped out with headaches, body ache, and fatigue, but despite lots of testing we didn't see a clear explanation. The fever subsided after a few days, and I was hopeful that it was just one of those weird random things that goes away on its own. After 5 fever free days, however, the fever suddenly returned with a vengeance. I started off at 99.4 Tuesday morning, and my temperature slowly climbed all day. Though I was in touch with my transplant team throughout the day, it was only at 5pm that my temperature finally hit 100.4. They previously told me to call if it got that high, so I called the after hours number, and after consulting with my doctor I was off to the ER to figure out what the heck was going on.

As usual the ER was a tedious waiting game. Once I got to the back we did All The Testing: bloodwork, Covid test, viral swab, chest and abdominal CT scans. And finally we found an explanation. Despite doing 10 days of IV antibiotics in June, and another 3 weeks in July, I somehow have pneumonia in both the upper and lower lobes on the right side. Which is super weird, because I haven't been coughing, or particularly congested, or short of breath. If it wasn't for the fever, I wouldn't have even known anything was wrong! My doctor thinks that I once again have narrowed airways, only this time they're preventing me from clearing out mucus,  which is causing pneumonia. Additionally, my sputum culture last week still showed Pseudomonas despite weeks of IV antibiotics.  Apparently Pseudomonas REALLY loves me! If narrowed airways are preventing me from clearing out secretions, that allows the Pseudomonas to just hang out in my lungs and keep making problems. And Pseudomonas is LONG overdue for an eviction!

Currently I'm still in the ER waiting for a bed at my old stomping grounds, Silver(stein) 11. I'm also on the list to get a bronch today to open up my airways and clear things out. It probably won't happen until later in the afternoon since I'm an add on, which is unfortunate as I'm not allowed to eat or drink anything until after the procedure. Aside from that, the plan is to set me up with another round of antibiotics, get a new long term IV, and send me home within a few days to finish up on home infusion.

My summer is REALLY sucking. I'm grateful that I don't deal with these issues anywhere near as often as I did pre-transplant, but it's still been a very draining couple months. Here's hoping that Pseudomonas finally gets the message goes away for good!

Birthday musings

Today I am 41 years old. 

A year ago I had a huge blowout bash to celebrate my 40th birthday. The party was spectacular, but there was definitely a grim intent behind it. At 40 years old, I knew that I had far exceeded all expectations regarding my life expectancy. I was increasingly aware of this as my health deteriorated in recent years, and particularly when my lung function dropped sharply during the 3-4 months immediately before my birthday. I was keenly aware that I might not have that many years left, and I wanted to celebrate with as many of my family and friends as possible while we still had the chance. It was a very deliberate "come to my birthday, not my funeral" celebration. 

Even with all that, I had no idea just how quickly my fears would be realized. At the time I thought I had at least a few more years left, and I was just taking advantage of the 40th birthday milestone. Little did I know that just a few months later I would be in respiratory failure, on the verge of being ventilated, fighting for my life and waiting desperately for a lung transplant. Things got so bad during that time that I had multiple conversations with my loved ones about dying, trying to prepare them as I had been preparing myself. By the end I was so miserably uncomfortable that I actually wanted to die just so the suffering would be over. I was holding on by my fingernails, surviving one day at a time. I certainly wasn't thinking about my 41st birthday, and if I had it would have been to wonder whether I'd even see it.

Yet somehow, miraculously, here I am, celebrating yet another year of life. It definitely isn’t the life I would have expected a year ago, both for good and for bad. On the one hand, I can BREATHE, to an extent that I had forgotten was possible. I’ve mostly lost my trademark CF cough. I’m putting on weight without even trying, instead of struggling to hang on to every pound. My health is SO much better than I could ever have imagined! On the other hand, I’m still recovering from an incredibly difficult, complex, painful, and invasive surgery. I’m managing both short and long term complications, and may develop additional problems as time goes by. And I must always live with the knowledge that rejection could strike at any time, and that there’s no way to predict if or when that or any number of other complications could take me out.

It’s definitely a mixed bag – but, I’m alive! And as long as I’m alive, there’s always hope for better things and positive outcomes. Hope is a somewhat unfamiliar and, honestly, frightening emotion for me, and I’m still learning how to integrate it into my life. For some reason I have a much easier time anticipating and preparing for the worst. But I’m trying to learn how to infuse more positivity into my life, and to start anticipating the best for a change.

I’m 41 years old, but my lungs are only 20. Against all odds I’m still alive, yet for some reason my dear donor lived only half as long. I unfortunately don’t know anything about my donor, but it’s clear that their life ended before it even really began. In a way, every birthday that I celebrate extends their life just a little bit longer. They are an essential part of every experience I have, every single breath I take. I hope that I can use those breaths and live my life in a way that would make them and their family proud.

I spent my entire adult life expecting to be dead long before now. Suddenly, I’m trying to imagine what it might be like to continue living for another decade or two. That’s a lot of birthdays I never expected to see! However many I get, I hope I can make the most of them, and always remember how fragile and precious every moment of life truly is.

Up and down but mostly up

What a week!

Last weekend I decided it was time to do something new with my hair, since most of the purple had washed out. One box of hair dye and a few hours later, I had successfully turned my hair, my skin, and my bathtub a stunning shade of Sonic the Hedgehog blue. Apparently allowing a bottle of hair dye to fall into the hands of a novice was a disaster waiting to happen. Anyone considering using Splat’s Midnight Indigo, be warned: that stuff is basically a nuclear bomb of color! I loved what it did to my hair, but was less than thrilled about becoming half Smurf in the process, and about the Jackson Pollack paint spatters all over the bathroom. Fortunately most of the extraneous color came out with a lot of scrubbing, though I keep turning the bathtub blue all over again every time I take a shower. After all this I may never touch blue hair dye again, but at least it’s fun for now!

On Monday I had my monthly bronchoscopy. Usually it just leaves me groggy and with a moderately sore throat for about a day, but for some reason I had a much rougher recovery this time around. When I woke up afterwards my throat was KILLING me, I was nauseous and had a headache, and I was very groggy and wobbly. It took me days to feel human again, including one night where I didn’t sleep at all due to persistent coughing because my throat was so scratched. So that was loads of fun. On the plus side, my doctor said my airways definitely looked better than last time. Nothing was clogged with debris, and though the airways were narrowed, they weren’t AS narrow as the previous time. He said it’s still too early to see real results from the Sirolimus, but hopefully it will have an impact by the time I have my next bronchoscopy. And HOPEFULLY soon we’ll be able wait more than 4-6 weeks between bronchoscopies.

Side note, the Bronchoscopy Collection is growing nicely. Right now they all live on the coffee table, but a few more bronchoscopies and they may need to find a new home!

On Friday I had an appointment with my transplant team, and did pulmonary function testing. Since I was several days post-bronch, my airways were pretty much at their best. Even so, I was surprised to find out that my lung function was up to 90%! That’s the highest I’ve managed post-transplant! It was very exciting to break into the 90s, so of course Katherine and I had to celebrate with Classic Cake!

It was an up and down sort of week, but fortunately I did eventually recover from the bronch, and definitely ended on a positive note. Hopefully everything will continue on an upwards trend from here!

Back on the bronch train

In November my airways looked so good during my bronchoscopy that we thought things were finally healing, and that I’d be able to go longer than a month before needing another one. At the time that was very exciting news, but unfortunately it was a bit overly optimistic. A few weeks later I was already wheezing and my lung function started dropping. I stubbornly tried to push through it, but finally gave in after drastically huffing and puffing my way up a flight of stairs and watching my numbers continue dropping rapidly at home on my hand-held spirometer. I didn’t expect to be able to get in for a bronchoscopy until after the new year, so I was very surprised when they had me on the schedule less than a week later on December 26^th. I guess not many people want to go in for procedures the day after Christmas!

They found a lot of obstructed airways during the bronchoscopy, far worse than the one in November. We don’t know why my airways were so good in November and then so bad a month later, but I’m once again on a monthly bronchoscopy schedule to keep on top of things. On the right side they once again found airways narrowed due to scar tissue, but on the left side an airway was fully obstructed with “debris” such as dried out mucus. Due to this I need to get back on track doing nebulizers at least twice a day to help clear out secretions, after seriously slacking and hardly nebbing at all during the last couple months. I’ve still been slacking, but I have been consistently nebbing once a day, and am working on getting back up to twice a day. My doctor also wants me to start a medication (Sirolimus) to help reduce scar tissue, as he expected my airways to stop narrowing by now. I couldn’t take Sirolimus before now as it impedes the healing process, which is pretty much the opposite of what you want immediately after a major surgery. Sirolimus is also an immunosuppressant, so we’ll have to keep an eye on my bloodwork and adjust all my other anti-rejection meds accordingly. The whole thing is pretty disappointing, and it kinda feels like I’ll be doing bronchoscopies every month for the rest of my life, but hopefully my lungs will get their act together and chill out already sometime soon.

I’ve been feeling kinda blah the last few months. I became increasingly unhappy and demotivated while dealing with months of debilitating joint pain, but even now that the pain is gone I’m having difficulty getting my mojo back. I’m very tired a lot of the time, which apparently is common post-transplant, but it’s hard to be social or productive when all you want to do is sleep. It’s also a difficult time of year for getting out and about, since right now a lot of activities involve indoor crowds, which is really risky for me during cold and flu season. I keep thinking of fun things to do, like going to the Convention Center portion of the Mummer’s Parade, and then realizing I can’t. I’m also definitely still adjusting emotionally to post-transplant life, and probably will be for a long time. I randomly get overwhelmed by fear and anxiety when I think about what I went through and all the risks and uncertainties that my future holds. A lot of the time I just want to curl up on the couch and hide from everything. But I’m trying to push back against that and figure out how to make a life that makes me happy while also protecting my health.

I fully recognize how extremely well my transplant is going and how very lucky I am. I’ve seen people go through far worse, and am painfully aware of how bad things could be. At the same time, no matter how smoothly things go, transplant is a difficult journey on multiple levels. I’m navigating my way through both physical and emotional challenges, and sometimes I get stuck. But I’ll keep pulling myself free and moving forward, and putting the pieces together one by one, as I continue figuring out my post-transplant life.

Breathing easy

A month ago, before my most recent bronchoscopy, my lung function was down to 71%. I knew things were going better this time around, based on my home spirometry numbers as well as how I feel. With previous bronchoscopies it only took a week for the wheezing to start up again. It’s now three weeks since my last bronchoscopy, and the wheezing is still minimal. But even so, I was shocked when I did pulmonary function testing today, and the respiratory therapist said my lung function was up to… 89%!!! I thought I heard him wrong, or that the machine was broken! That’s the highest I’ve managed not only post-transplant, but in 14 years! And that was with some wheezing on the right side, which means with completely open airways I could go even higher!

Clearly my lungs are doing great! Unfortunately, the rest of my body is still catching up. Joint pain is an ongoing issue, and it’s pretty rough to deal with since it’s so pervasive and nearly constant. The slightest every day activities can trigger pain so sharp it makes me yelp. A week and a half ago the orthopedist reviewed x-rays as well as blood tests, and said nothing indicated structural problems or arthritis. Like my transplant team, he thinks the pain is most likely my body withdrawing from the steroids. He referred me for aquatic therapy so that I can get exercise and maintain strength and muscle tone without straining my joints. And he said my best bet for controlling my pain while waiting for my body to finish adjusting is… medical marijuana! I thought medical marijuana wasn’t an option due to an interaction with one of my anti-rejection medications, but apparently I CAN take it (in edible form only!) as long as I take it on a set schedule every day and we monitor my levels closely. Unfortunately the orthopedist wasn’t able to set it up for me, as he’s licensed in PA and I live in NJ. I wasn’t thrilled to have to continue waiting in pain, but fortunately I was able to get an appointment with an NJ provider relatively soon. I’ll keep you posted about my new life as a pothead!

I asked my doctor today what’s the longest he’s seen someone go through joint pain from Prednisone withdrawal, and he said a year. This was NOT the answer I wanted to hear, but at least I know that I’m “normal,” and that improvement IS still possible. He was also encouraged when I told him I randomly had three good days in a row last week, and said that a lot of people don’t have any good days before they recover. I don’t know why I suddenly felt better or why it went away, but hopefully it’ll happen again. And hopefully I WON’T be someone who takes a year to recover, and I’ll be able to get the most out of these shiny new lungs soon!

The path not taken

I had my monthly bronchoscopy last week. Though there was once again narrowing in some of my airways, there was less necrotic tissue and overall things looked better than last time. Hopefully that means things are moving in the right direction and settling down. I haven’t started wheezing yet, so that’s also progress, as previously it only took about a week for the wheezing to start up again. They still want me to come back next month for another bronchoscopy, but I should start needing them less, and eventually not at all, hopefully soon! The doctor said that this happens in 10-15% of lung transplants, because of course I would have an atypical complication. I’m officially statistically special. We all know I like to keep them on their toes!

During Simchas Torah I lived on the edge and braved the crowds at shul. I of course wore a mask and was careful about washing my hands, and spent a good amount of time outside where there was more airflow and therefore less risk of germs. Probably if I wanted to be 100% safe I should’ve stayed home, but what’s the point of getting a life saving transplant if you don’t actually participate in life? I’m basically still making the same calculations that I did pre-transplant to balance protecting my health with living a fulfilling life. Hopefully I calculate correctly, but at the end of the day I’d rather have a short life filled with good memories than a long life of empty boredom.

I’m still struggling with significant joint pain, which has now spread from my hips, shoulders, and a few fingers to almost all of my fingers, my knees, elbows, and one wrist. Pretty much any movement hurts, and I often hurt when I’m sitting still too. Going to sleep is really fun, as laying in bed without distraction is the perfect time to really focus on all my aches and pains. I have an appointment with an orthopedist tomorrow, so hopefully he’ll be able to offer some relief.

In the CF world, the BIG news this week is that the FDA approved Trikafta, a new genetic modulator that had huge results in clinical trials and treats the vast majority of CF mutation combinations. This is the triple combo drug I tried unsuccessfully to get early access to when my lungs were failing. Not gonna lie, I’m definitely feeling some kinda way about the whole thing. I waited YEARS for them to come out with a genetic modulator that I was eligible for. One breakthrough after the next I was disappointed, as my mutation combination was not eligible for any of the new drugs. Then, FINALLY, the one that I could take was being developed – and my lungs promptly went to pieces. From November through March I kept being overcome with rage as my lung function deteriorated right when a promising new drug was on the horizon. By the time we started talking transplant, I moved from enraged to resigned. And now, here I am, approaching 6 months post-transplant, seeing that long awaited drug finally hit the market. Yeah, I’m a little bitter about the whole thing.

But, I’m trying not to waste time on bitterness. For whatever reason, that wasn’t the path I was meant to take. I need to focus on the path that I’m on and getting through this transplant life. The truth is, I’m probably breathing better than I ever would have even if Trikafta worked miracles for me. If we could just get my pain under control I feel like I could conquer the world! Maybe I’m just supposed to enjoy breathing with these lungs for however long I get.

It’s hard not to imagine the what-ifs and could’ve-beens, especially while struggling with physical pain. But I’ll try instead to look at what is and what will be, to keep putting one foot in front if the other, and to get the most out of the life I have.

Being human

Sometimes, things are hard.

Of course, things aren’t nearly as hard as they were a few months ago. Which then makes me feel kind of guilty any time I feel down or cranky or anything less than grateful. I feel like I should be on a permanent high just from being alive, and (mostly) able to walk, and (also mostly) able to breathe. Life is SO much better and I am SO much more comfortable now, so why should I ever be anything less than happy?

But apparently, no matter how I may try to deny it, I am only human after all. And even aside from complications, post-transplant life involves some major mental and emotional re-adjustments. There are the emotions around knowing that my life was saved due to someone else’s death, and that while we are celebrating, somewhere out there another family is grieving. Additionally, for decades I didn’t expect to have much of a future as an adult, and at 40 I’ve actually survived far longer than anyone anticipated. In the months and weeks leading up to transplant I was so, SO sick that I basically prepared myself to die. The future post-lung transplant is far from certain, and I still could die – but, I also could live for 10 or 20 years or more. I now have to make the sudden mental adjustment to potentially having a life ahead of me, a life I never planned for or expected to see. You’d think that would be wonderful and exciting, but it’s also kind of terrifying! I was familiar with CF life and mostly knew what to expect and how to deal with it. Now I have to figure out what post-transplant life looks like, what I can and can’t do, how to take care of and protect myself while also doing things that make this all worthwhile. A tall order for someone who didn’t expect to be here!

And then there’s the recovery process and complications. I still have some pain in my incisions and a weird band of numbness across my chest. I have multiple appointments every week, including appointments with various specialists in Philadelphia a few times a month. I’m still figuring out how to manage my new diabetic life. The joint and muscle pain make everything difficult and wear me out. I haven’t been sleeping well, at least partially due to pain, and spend a lot of my time feeling fatigued. My airways keep narrowing and being obstructed with necrotic tissue, which means repeated bronchoscopies (not fun), plus I’m wheezing and rattling for at least a few weeks between bronchoscopies (also not fun). It feels like I have a lot on my plate right now, and it’s unclear when the load will lighten, which then makes me anxious. I’m very good at what if-ing: What if the pain doesn’t improve? What if my airways keep acting up? What if I’m not able to get back to a regular exercise routine? What if I don’t get my energy back? What if I do live a long time but everything just keeps being varying degrees of difficult forever? What if what if what if!

I have to remember that I’m still early in this process, and that it IS a process, and that it takes time. I want everything NOW, but that’s not how this works. Just because things are hard now doesn’t mean they’ll be hard forever, hopefully. Hopefully 3 months or 6 months or a year from now I’ll look back and laugh at my anxieties, because the complications will have settled and I’ll be comfortable with my new medical routine. It’s hard to be patient when I’m quite literally in pain, but I just need to get through one day, one week, one month after the next, and give things time to improve. Some things have already improved in the 4 months that have passed, and I need to remember that my body is still healing and recovering from a major trauma. I can’t know how much better things will get, but I need to be confident that things WILL get better.

And I need to remember that sometimes, I will have bad days, and that’s ok too. Emotional recovery is just as much a part of this process as the physical recovery. I’m only human, and that’s ok.

Bumps in the road

A couple weeks ago I noticed that I was wheezing again, similar to how I was before the bronchoscopy. I had a hunch my airways were already narrowing and that I would need another bronchoscopy sooner than later. On Wednesday my pulmonary function numbers were down 4% and my doctor confirmed that the wheezing and constriction is all on the right side, the same side they had to clean out the last 2 times. He wasn’t concerned and said that this will settle down eventually as my lungs finish healing, and that things already looked better during my last bronchoscopy. Nevertheless, we need to keep on top of it, so that means another bronchoscopy in a couple weeks to open up the airways and clean out any dead tissue. On the plus side, that means another stuffed animal for the Bronchoscopy Collection!

My hips, meanwhile, continue to misbehave. I’ve only seen slight improvement with physical therapy, so we’re trying to get me an appointment with an orthopedic doctor to figure out the next steps. I’ve also been having pain in other joints, including my fingers, shoulders, and knees. I kinda feel like a creaky old lady! My doctor said it’s possible this could be due to one of my medications, but it’s more likely from my body adjusting to being on a lower dose of Prednisone after being on such high doses of steroids for so long. Unfortunately it could take months for my body to finish adjusting, so it looks like pain is just going to be part of my life for now. What’s also unfortunate is that post-transplant I can’t take any drugs that include ibuprofen due to drug interactions, so I’m stuck with Tylenol instead. And before someone suggests medical marijuana, that’s out too, as that also interacts with one of my anti-rejection meds. I never tried marijuana before, and apparently now I never will. Alas!

Once again I have to remind myself that transplant recovery is a long process, and allow my body to heal at its own pace. Which is hard when I feel so much better and want to do all the things! It also isn’t exactly fun being in varying degrees of pain most of the time. But I’ll keep working on being patient, and hopefully things will settle down soon!

Moving up in the world

Had my first post-bronch pulmonary function test (PFT) yesterday, and my lung function is up to... 79%! That's an improvement of almost 10% from where I was 10 days earlier! I *told* you I'd get back to 80% and beyond! 😊

3 month bronchoscopy!

Check out https://youtu.be/W1pQaEHWdDk if you’d like to see a video recap of bronchoscopy day!

Thursday was my big bronchoscopy day! Post-transplant it’s standard to have bronchoscopies after 6 weeks, 3 months, 6 months, and 1 year, and then annually after that. During a bronchoscopy they put a scope down my throat and into my lungs to look around and make sure everything is ok, and take sputum samples and biopsies to check for infection and rejection. In my case, we already knew things weren’t 100% perfect and that they’d have to open up and clear out some airways. Thankfully, I’m under general anesthesia for this whole process. General anesthesia used to be a big scary deal with my old lungs, but seems to be a lot easier and more standard with my shiny new ones. So on Thursday I went to HUP, changed into my very fashionable hospital gown, and went to sleep for a few hours while they fixed me up.

My biggest problem post-bronchoscopy is usually that my throat is very sore and it’s difficult to eat. This time we came prepared with a quart of Rita’s water ice in a cooler so I’d have something cold and delicious to soothe my throat. The nurses and other patients in the recovery room were quite impressed with our foresight and planning! Katherine also continued our new tradition of getting a cute little stuffed animal from the gift shop for me to wake up to post-bronchoscopy. So far there are two, and I’m calling them the Bronchoscopy Collection. At least now I have something fun to look forward to each time!

Shortly after the procedure they let us know that a few airways had been narrowed on the right side and one was narrow on the left side, so they went in and inflated a balloon to open and widen them. There was also necrotic tissue blocking some airways on the right side, which sounds scary but apparently is common. They saw the same thing during my previous bronchoscopy, and both times they were able to remove the dead tissue and open up the airways. I’m wheezing a lot less now, though I’m still a bit congested as I clear out the mucus that was trapped during the last several weeks. Hopefully things will heal up and clear out by the time I see the doctor again in 2 weeks, and my pulmonary function numbers will go way up!

Today I got my test results from the sputum samples and biopsies, and the verdict is… no infection and no rejection! We didn’t get cake this time, but it’s very exciting and encouraging that things continue to go so well! Now I just need to get the hip pain under control so I can get back on track with my exercise routine and rebuild my strength and endurance. I’m also still having some pain in my incisions, though it’s gotten MUCH better. Katherine keeps reminding me that it’s only been 3 months, and that I need to relax and not push myself too hard. Which is hard when I’m feeling SO much better and more capable. I want to conquer the world NOW!

Fiiiine, I guess I’ll try to cut myself some slack and allow this process unfold at its own pace – but I WILL get these lungs back up to 80% and beyond! Just you wait and see!!

         

Another speedbump

First, the good news: it’s NOT rejection!

I’ve been kinda quiet about my health status the last couple weeks, because I didn’t want to say anything until I knew what was going on. Now I know what’s going on: but first, we need to go back to something that I think I might not have mentioned. Immediately post-transplant my sputum culture showed some of the infections that I had pre-transplant. My treatment team didn’t seem too concerned, however, since my new lungs don’t produce the sticky CF mucus that would allow infections to settle in and make big problems. With antibiotics those infections eventually disappeared, and I had infection-free sputum cultures for the first time in decades. That lasted for a few weeks, but 2 weeks ago the infections showed up again. This isn’t surprising, as I still have my same old terrible CF sinuses, which have the same infections and will probably keep re-infecting my lungs. Again, my treatment team wasn’t concerned, and we just put me back on an inhaled antibiotic to treat the infections.

A week later, not only was I still slightly more congested, but I was also wheezing a little. Worse, my PFT (Pulmonary Function Test) numbers had dropped, from 80% (!!!!) to 75%. We added an oral antibiotic and continued monitoring. This week the wheezing was more pronounced and I started having a dry, scratchy cough early in the morning. Today my PFTs had dropped even more and were down to 69%. I was trying not to panic, as I was convinced this meant that I was experiencing rejection. However, to quote my doctor, “rejection doesn’t wheeze.” Apparently it also doesn’t cough, or choose sides, as my right side sounded junky and wheezy while the left side was clear. My doctor said it’s just scabs and scar tissue narrowing the airways on the right side, which they had already seen during my last bronchoscopy. During my next bronchoscopy they’ll go in and open things up again, which should solve the problem. Originally I wasn’t supposed to have a bronchoscopy until the end of August, but they’re going to move it up since I’m having issues. I hope it happens soon so I can stop wheezing and rattling!

Despite the drop in numbers, I’ve still been feeling pretty good! I’m still keeping active and my endurance hasn’t dropped significantly. I think things were SO bad before transplant that even dropping down to 69% still feels AMAZING! My biggest problem, aside from wheezing and rattling being mildly uncomfortable, has actually been… my hips! During my last couple weeks of pulmonary rehab I started having significant hip pain. We think the rigors of rehab after being mostly bedridden for so long might have been too much for my body. The pain level goes up and down day by day, but there are some days where I’m almost hobbling from the pain. My lungs are ready to go, but my body not so much! I’ll be starting physical therapy this week to address that as well as back pain. My back has been a mess for years from all the coughing, and being bedridden and going through major surgery didn’t do it any favors. Hopefully I’ll actually be able to do something about it now that I hardly cough anymore!

So that’s the story around here. Speedbumps are expected during this process, and as speedbumps go it sounds like this isn’t a bad one. Hopefully I’ll get those numbers back to 80% and beyond soon!

Celebration time!

Celebrating the results from my bronchoscopy which showed no infection and no rejection!!!!!

Enjoying life!

My first major outing post double lung transplant! I decided to be bold and NOT bring the wheelchair. I managed to walk around (with some breaks) for almost 3 hours without a problem. As usual the Chinese Lantern Festival was absolutely gorgeous. The weather was perfect and we all had a great time. It's so wonderful to be able to get out and do things again!

We found a giant kosher cookie!

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.