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.

Living the high life

That moment when you take a painkiller and realize that it doesn't actually reduce your pain, it just makes you care less about being in pain. Which I guess is an improvement?

Also, on Facebook I wanted to put "Feeling high," but they didn't give that as an option so I had to settle for "Feeling chill" instead. Don't judge my life choices, Facebook!

Happy New Year

I’ve been feeling kinda blah medically the last couple weeks. I had my bronchoscopy, which did help me to breathe more easily once I finished healing from the procedure, but just a week later I already felt the wheezing starting up again. I’m pretty much on a monthly bronch schedule now until my lungs finish sorting themselves out. When they went in this time the narrowed airway on the left side looked better, and there was less necrotic tissue around the incision, but the narrowed airways on the right side looked about the same. So it looks like nebulizers, wheezing, and monthly bronchoscopies will continue to be in my future for a while.

I also had an evaluation at physical therapy, and confirmed that I haven’t made any progress with my hips after almost 2 months. Due to this we decided to hold off on further PT sessions until I see an orthopedist in a few weeks. I do appreciate having a few less appointments filling my schedule, but it was pretty disheartening to go through all that effort and not get anywhere. It’s also exhausting trying to live around near constant pain. I really hope we can find a solution soon.

Erev Rosh Hashana in Cherry Hill was basically total chaos. Saturday night we found out that, sadly, Rabbi Bienenfeld’s father had passed away. The funeral and shiva were on Sunday in New York. In the midst of the community making arrangements for people to make the trip, we were suddenly hit by a massive thunderstorm that caused major damage. The strength of the winds was basically equivalent to a tornado! Trees and power lines were down, roads flooded, transformers exploded, and there were even a couple fires. It was terrifying! Lots of people lost power, which is NOT what you want to happen the day before a holiday when you’re trying to cook and clean and get everything ready. What’s even stranger is that though the whole area got rain, the severe damage was localized to the area around the west side of Cherry Hill. The whole thing felt bizarrely targeted.

I felt pretty unsettled and anxious about our judgement going into Rosh Hashana. Outside was a disaster, with trees and power lines down and roads closed everywhere. People were scrambling trying to figure out how to prepare for the holiday. And not only would Young Israel be without our rabbi, but we would be without our shul as well, as downed trees and power lines made it unsafe to enter the building. All in all it was not feeling like a particularly auspicious time!

But with some help from friends, I was able to focus instead on the beauty that came out of all this. Despite the distance and difficult circumstances, a group of people got up early and drove  to NY to be with Rabbi Bienenfeld for the funeral and shiva. In Cherry Hill, the people who did have power gladly opened their homes to those who didn’t. Someone coordinated with Chaverim and picked up a bunch of generators and fuel to help supply some homes with power. Foxman Torah Institute provided space for Young Israel to daven on Rosh Hashana. All day long on Sunday I saw messages flying back and forth asking for and offering help, and rejoicing together as the pieces fell into place. And somehow, despite everything, we managed to have a beautiful holiday!

I’m still not quite sure what to make of everything. I was already unsettled going into Rosh Hashana, as I’ve had quite the topsy turvy year, and I don’t know what exactly Hashem is trying to tell me. Apparently last Rosh Hashana for some reason Hashem decreed that I should have a year of rollercoaster highs and lows. The chaos erev Rosh Hashana definitely didn’t do anything to ease my mind about what He has in store for me in the coming year! But maybe I should focus instead on how my community somehow pulled everything together in the end. Sometimes we take a hit, and sometimes the hit is hard, but we can still pick ourselves up and keep moving forward. It may be difficult, but even in an unexpected disaster it’s still possible to put all the pieces together and make things work. Even when times are dark and stormy, Hashem is still there, quietly guiding us through it.

May we all have a year with minimal storms and distress, and maximum peace and joy!

My lung transplant story, now on YouTube

I finally put together a video about my transplant! I drafted my mom and Katherine to help tell the tale, and included pictures and videos of the whole process, including video of me being extubated in the ICU and taking my first breaths less than 24 hours after transplant. Sorry for the length, it's kinda hard to condense such a major life experience! I noted times and different topics we discussed in the video description to make things easier to find.

Bronchoscopy day

Another day, another bronchoscopy! Does this gown go with my hair?

The procedure went well, so while I was in recovery it was time for our post-bronch traditions: a new Bronchoscopy Animal and Rita's water ice!

New lungs, new hair!

So this happened! Apparently I'm living the stereotype: survive a traumatic experience, do something crazy with your hair. Shout out to Megan at AVM Salon for making the first time I ever dyed my hair a great experience!

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.

Celebration time

Fancy dinner with my mom and Katherine at Citron and Rose Tavern and Market to celebrate my double lung transplant, courtesy of my mom's wonderful coworkers!

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!

Transplant humor

I Survived a Lung Transplant and All I Got Were These Stupid T-shirts  😂
#TransplantHumor

Graduation!

Officially graduated from pulmonary rehab! But the hard work continues as now I need to keep up with a home exercise program to maintain my progress. For now though, we're celebrating with cake!

On the road again!

Finally got clearance to drive! Between being sick pre-transplant and recovering afterwards, it's my first time behind the wheel in almost 4 months!

Happy 4th of July!

Such a fun 4th of July! Thank you Avigayil for shlepping all the way out here from NYC just to visit me for a couple hours! Somehow we managed to pull off a delicious barbeque and still got her to the bus back just before it left! Then later Katherine and I headed off to Cooper River Park with our $10 box of fireworks from Target to fulfill our patriotic duty of blowing stuff up. Apparently Cooper River Park is the place to be at 9:30pm on the 4th of July! There were a bunch of other people setting off fireworks in the parking lot, including a group who were adorned with multicolor glow stick necklaces. We (aka I, because Katherine is "sensible") set off our fireworks without losing any fingers, and shared our sparklers with some small children and made them very happy. All in all it was a great day!

A trunk full of summer: beach bag, BBQ supplies, grill, rockhounding bag, and kites - all fitting so nicely because there's NO WHEELCHAIR 😀

The aftermath (yes we cleaned it all up and threw it away)

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!