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.

Happy Chanukah!

For the first time since 2016, I was able to light the menorah on all 8 nights of Chanukah -- because for the first time since 2016 I was NOT in the hospital for ANY of Chanukah! It's also been almost 7 months since the last time I was on IV antibiotics and/or hospitalized, and I haven't managed THAT since at least 2015. May this be the first of many hospital-free Chanukahs!

Donor Dash!

Current goal: do the real Donor Dash this year instead of the inpatient version! Time to start training!!

Also, if anyone wants to see last year's inpatient donor dash, I actually recorded it for posterity: https://youtu.be/t7tZO8gqt0Y

Gift of Life Donor Dash 2020

6-month bronchoscopy!

Today was 6 month bronchoscopy day - yes, it's been 6 months since my transplant! I'm currently in the post-bronchoscopy sore-throat worn-out still-groggy-from-anesthesia stage, so right now Katherine is dealing with me wandering around whimpering pathetically when I'm not laying in my recliner and pathetically whimpering from there. But she's the best girlfriend ever so she's cheerfully putting up with me and making me delicious mashed potatoes that won't be rough on my throat. And she got me yet another adorbs Bronchoscopy Animal for the collection, which always makes everything better.

Everything went well, and the surgeon said this time my airways looked great! He said they looked almost as good as after he finished my last bronchoscopy, they barely needed any dilation, and there was NO necrotic tissue! This means my lungs are finally healing up nicely and I shouldn't need another bronchoscopy for a few months. I probably won't make it the full 6 months until my regularly scheduled 1 year bronchoscopy, but anything more than a month between bronchs will be a welcome break!

The other good news this week is that I've been in significantly less pain. We stopped my anti-fungal medicine last week since I'm now 6 months post-transplant with no signs of fungal infection. My doctor said it was possible that medication was contributing to my joint pain, though he thought primary culprit was probably Prednisone withdrawal. A few days after stopping the anti-fungal, however, I suddenly had far less pain and much more range of motion! The pain isn't completely gone, but it's MUCH better and I feel much more functional. I did have a brief run of good days once before, so I'm a little anxious that it won't last, but I'm hopeful that maybe the anti-fungal was responsible for the bulk of my pain and that I'll do much better now that I'm off it. Fingers crossed!

So it's been a week of good news, despite bronchoscopies not exactly being a good time. Hopefully it'll be the start of an ongoing upwards trend!!

Post-transplant video: Extubation and first breaths

If anyone's interested in seeing it without having to watch the whole long transplant story video, I put up the video of me being taken off the ventilator and taking my first independent breaths post-transplant. Be warned: being taken off the ventilator involves me being extubated, which is when they yank out the long tube that went down my throat and into my chest to breathe for me. Reactions have ranged from "Cool!!!" to "GROSS!!!" so you might want to skip it if you're squeamish!

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.