Happy Purim!

Despite the dumpster fire that my life has been lately, I was determined to celebrate Purim with my usual creative flair. And thanks to Amazon I pulled it off! After everything I've been through, my costume choice seemed obvious: the Operation game! I paired it with some medically themed mishloach manot. HUGE shout out to Katherine, who was involved in every part of making my holiday happen, from spending 40 minutes wrangling persnickety fabric tape to help me affix the pieces of my costume, to helping me put together all the parts of my mishloach manot, and finally to playing chauffeur and driving me around town for 2 hours while I made my deliveries on Purim day!
 
I didn't feel up to going to shul and was also concerned about being in a crowd with all the germs floating around, but thankfully my neighbor Eli Beck did both megilla readings for Rivka Sara and myself right in the comfort of our own building (and for the 2nd reading, in my own home!). And I wasn't able to join anyone for the seuda (festive meal), because my afternoon antibiotic dose runs from 4-7pm, right smack in the middle of seuda time. But I managed to get all my deliveries done and visited with some friends and just generally experienced the Purim festivities, and overall had a wonderful day!
 
I have tremendous gratitude to Hashem, who graced me with a good day with minimal pain, adequate energy, and feeling clear headed instead of foggy on EXACTLY the day I needed it! It's definitely been a rough few months, and fun outings have been few and far between, but Hashem granted me a lovely Purim despite everything!

Cutting labels for mishloach manot while running an infusion

Arizona RX Energy tea, "prescription" cookies, and a "med kit" candy bar

Getting into the Purim spirit!

It's been a long month

What a month!
 
It took a couple weeks, but thankfully Katherine and I have recovered from Covid. The Remdesivir infusions went well, and thanks to my friends I had plenty of food for Pesach (Passover). Unfortunately I kept struggling with nausea, and was wiped out from off and on low grade fevers, so my seders weren’t terribly festive. But I made it through Pesach, and my symptoms didn’t turn into anything serious, so I didn’t have to go to the hospital. Yay!
 
Getting through immediate illness without major complications is only the first hurdle though. Now I need to wait and see how my lungs do afterwards. Every infection has the potential to trigger rejection, which is part of the reason why infections are so dangerous for me. So far my lungs seem to be doing ok, but I need to keep an eye on them for the next few weeks and make sure nothing starts to deteriorate. I’m not sure exactly how long it will be until I’m in the clear, but my next transplant appointment is towards the end of May, so if my lung function looks good then I’ll assume everything is fine. Thank G-d, so far I’ve managed almost 6 years post-transplant without a single episode of rejection, which is remarkable! Hopefully I can continue that streak!!
 
Even though I was pretty much done with symptoms after 2 weeks, my transplant team said I was still presumed contagious for a full 21 days. Aside from making it difficult to fight infections, being immunosuppressed also makes it difficult to fully clear infections, so we tend to shed viruses for longer than most people. Which means I’ve been mostly quarantined for the last 3 weeks. The first 2 weeks didn’t really matter because I was too wiped out anyway, but I’ve really felt mostly fine for the last week. I was able to mask up and leave the house once for lab work and a vascular ultrasound (which thankfully showed that the blood clot in my arm has fully resolved! Woohoo!), but aside from that I’ve been stuck at home.
 
But today I was finally set free! I celebrated by… going to physical therapy. Ha. I’ve been dealing with off and on hip pain since transplant, but never quite got around to dealing with it. It’s typically only an issue if I walk for more than 15 minutes or so, and I have a pathetically sedentary lifestyle, so it doesn’t come up much. But a couple months ago I finally got a primary care doctor for the first time in years to look into some random shoulder pain, and I figured I may as well mention all my random aches and pains while I was at it. So she referred me to an orthopedist, who referred me for physical therapy, where I’m now working to strengthen my hips. I only went for 2 weeks though before catching Covid and being out of commission for the next 3 weeks. Hopefully now I can get back on track and get stronger, and maybe start doing something about making a change to that sedentary lifestyle!
 
And just to be extra celebratory, tomorrow I’m getting a tooth extracted! We found the first cavity of my entire life back in September, but it’s close to the nerve so they couldn’t drill it. It’s also in a wisdom tooth, so instead of going to the trouble of a root canal we’re just going to extract it. It took some time to get things squared away with my transplant team, and then to get an appointment with an oral surgeon, but eventually we scheduled an extraction appointment for December. Aaaand then Lump exploded, and the subsequent hospitalization, surgery, and other medical shenanigans promptly pushed all dental issues to the wayside. So now it’s finally time to address my tooth. After 46 years without a single significant dental issue, I am NOT excited for this. There’s also the fact that immunosuppression and steroids impede healing, so I have no clue how the recovery is going to go. Here’s hoping everything ends up going smoothly!
 
April has pretty much sucked, and the last few months in general have been A LOT. Hopefully this will be my last painful procedure for a long time!!

I'm so over this month

The last 2 weeks have been the ABSOLUTE WORST.
 
Last week, Cam died. And then, exactly one week later, we lost yet another member of the CF Discord server. Michael, aka scarz0ftime, was an actively involved long-time member. He got his double lung transplant about a year before I did, but after less than a year and a half he was already in chronic rejection. We knew that he was struggling and was working on getting listed for a second transplant, but he didn’t post publicly about how bad things had gotten. So it was a huge shock to find out that he died on Monday night. Scarz was a huge presence in the server, from welcoming newcomers, to offering advice and support, to joking around, to knitting people gifts and sending out random postcards to anyone who wanted one. He loved life, and fought long and hard and gave it his all, and he will truly be missed.
 
And then, because everything didn’t already suck enough: After avoiding it for 5 years, Katherine and I finally caught Covid. Which meant that the plans for my annual Pesach (Passover) trip to visit my long-time friend Anna and her family in Pittsburgh were abruptly canceled, and instead I had to scramble to make arrangements for spending the holiday at home. I am VERY fortunate to have amazing friends and an amazing Jewish community, so an army of people immediately sprang into action to get me food and make sure I have everything I need for the seders. I’m definitely not excited about being cooped up at home for the entirety of Pesach, but I am so grateful for all the support I’ve received!!
 
I also had to do all the Pesach cleaning while exhausted and short of breath. I couldn’t even pay someone to come do it for me, since I didn’t want to invite anyone in for a free Covid exposure. Fortunately thus far my symptoms haven’t been TOO terrible, but Katherine has been absolutely laid out by this miserable virus. Even though I seem to be handling it relatively well so far, my transplant team isn’t taking any chances and wants me to get treatment anyway. I can’t take Paxlovid since it interferes with my anti-rejection meds, so my only option is Remdesivir infusions. Insurance tried to deny it at least 3 times, but after 2 days of fighting they FINALLY approved it. A home infusion nurse is scheduled to come out today to put in an IV and run the first of 3 daily infusions. The hope is that they’ll get the IV in today and it’ll last through all 3 days, but my veins are in the habit of being pretty terrible, so I’m honestly very worried about how this will go. But there’s nothing I can do about it, so all I can do is pray and hope for the best.
 
So far the month of April has been just AWFUL. I’m sad, and sick, and utterly exhausted. I truly hope that the upcoming holiday of redemption will kick start a positive turn around.

Holiday season

I made it through the Jewish holidays! Having 4 major holidays in 4 weeks is intense, but it’s definitely much more enjoyable now that I’m able to get out and about for some of it. I went out for most of the meals, and even made it to shul a few times! My doctor didn’t think it was a good idea for me to be inside a crowded shul full of unmasked people, but I tried sitting outside on a porch and looking through a door that opened into the women’s section. That actually worked pretty well on the first day of Rosh Hashana, but I was foiled on the 2^nd day by jackhammering across the street which made it impossible to hear anything. It rained on Yom Kippur so I didn’t end up going to shul, but on Simchas Torah I was able to watch the dancing and hear the Torah reading from my porch seat. I even ran inside for a minute to dance VERY briefly before running back out to my anti-germ zone.
 
Sukkos was a little more difficult. On Sukkos we’re supposed to eat all our meals in a sukka, which is a temporary outdoor hut. One of the rules of the sukka is that the roof has to be made of plant matter, which unfortunately is typically prone to growing mold. My doctor was very concerned about the possibility of picking up a mold or fungal infection, so I was not able to sit inside the sukka. I compromised by going to the Adlers for a couple meals, because their sukka is open on one side against a glass wall looking into their dining room. So I sat in the dining room and could at least SEE the sukka through the wall and kind of feel like I was part of the festivities.
 
I guess sitting outside and looking in was kind of a theme across all of my holidays. Whether sitting outside shul or outside the sukka, unfortunately I’m often unable to fully participate in various activities and instead have to come up with compromises to protect my health. Being forced to separate myself definitely isn’t fun, but it’s still a vast improvement over the height of Covid when I couldn’t do ANYTHING. Unfortunately I’ll always have to live with some degree of separation regardless of Covid. Mold in the sukka will always be an issue, as will crowds and all their potential germs, since even a cold or flu could be deadly for me. It’s a difficult balancing act figuring out an acceptable risk level that protects my health while also allowing me to be part of my community and enjoy various activities and outings. Sometimes I get sick of all the restrictions and push the boundaries a little, but thankfully so far I’ve made it through those moments without catching anything. Figuring out how to navigate between being safe and living life is definitely still a work in progress.
 
As the weather cools off and there are fewer outdoor activities, and as cold and flu season ramps up, it will definitely be more difficult for me to safely get out and about. Winter definitely is not my best season (except for when it snows enough for me to snow sculpt of course!). Hopefully I’ll be able to find some relatively safe activities and social opportunities to keep me busy until spring!

The medically low-key life

Things have been about as medically quiet as they get for me. That doesn’t mean no complications, but it does mean nothing particularly dire. One frustrating thing is that I’ve had continuous lung infections since finishing IV antibiotics. In March I cultured staph, but fortunately it was an easily treatable one that was resolved with oral antibiotics. Then in April I cultured my old nemesis pseudomonas. I haven’t had much in the way of symptoms however, so we haven’t jumped into any drastic treatments yet. I already do a nebulized antibiotic on a 1 month on, 1 month off schedule, and April was my off month, so as long as I remain mostly symptom free we’ll just see if it clears up by the end of May. I’m also having an appointment with my ENT to see if there’s anything else we can do with my miserable sinuses to keep the infections from dripping down into my lungs.
 
The main symptom I’ve had is fatigue, but it’s hard to tell how much of that is from the infection and how much is just my life. I’ve struggled with poor sleep for decades, but I definitely feel like it got worse after transplant, so that obviously contributes to fatigue. I think I’ve heard that insomnia and fatigue aren’t uncommon after transplant, so it could just be transplant life. And I’m sure battling a pseudomonas infection doesn’t help. All I know is that I’ve been VERY tired lately, which makes it really difficult to get anything done and kinda sucks the life out of me. Here’s hoping that improves along with the pseudomonas infection.
 
I also had my first post-transplant dermatology appointment. You’re supposed to have one every year after transplant, but due to Covid we pushed off most non-essential appointments. Penn now has a special transplant dermatology program though, and they’re good about making sure we all get in to see them. I knew that transplant recipients are at higher risk for all kinds of cancers, but I didn’t realize that the risk of skin cancers in particular is ridiculously high. Thankfully my skin looked good this time, but the doctor said that 3-5 years after transplant is when they typically start seeing skin cancers. And guess whose 3 year lungiversary is this week?? So that’s another worry to add to my long list of fears. I’ll definitely be more cautious about covering up and using sunscreen!
 
On the non-medical front, we unfortunately have some sad news. After having a bad fall last summer and then struggling for months with one medical issue after the next, Katherine’s mother passed away last month. It was a strange mix of unexpected and expected: unexpected, because before the fall she was doing fine, and expected, because she declined for months before finally being sent home on hospice. Things were pretty awful at the end, and it was a mercy when she finally passed. It’s been difficult, but Katherine is holding up ok, and we all hope her mother is finally at peace.
 
On a brighter note, I’ve really been appreciating being able to get out and socialize a bit instead of being on full Covid lockdown. I went out for half of the holiday meals on Pesach, which was AMAZING and made the holiday SO much more enjoyable. I’ve also been going out for some Shabbos meals. And in an effort to get some exercise, I started taking lessons with my friend Abrielle Fuerst, who teaches martial arts professionally. So that’s guaranteed once a week that I get out of the house and see a friend, and I get to learn how to be a ninja on top of it! I am playing with NUNCHUCKS, people! Watch out!

Happy Purim!

Until last week I had no plans for Purim. I was so exhausted and miserable that I just didn’t have it in me to come up with a theme and a costume. Once my doctor told me that I could rejoin the world, however, I was revitalized, and I started putting together my theme that very night! Flowers seemed like a fun and appropriate way to represent the feeling that I’m beginning to blossom and come back to life. With a little online searching I stumbled upon the fact that you can bake a cake in a terracotta flower pot, and thus my mishloach manot were born!
 
Additionally, I decided to join forces with the Becks and make an Our Doctors Said No Communal Seuda seuda. And then Baby Berdugo arrived so we got to invite the Berdugos to join us! I have a hunch that when my doctor said I could start carefully socializing again, he didn’t intend for me to host a literal party, but I feel pretty safe with both the Becks and the Berdugos and couldn’t pass up the opportunity. Aside from the fact that we haven’t had anyone over for a meal in over 2 years, Katherine and I have actually never hosted more than a couple guests at a time in either apartment! Everyone brought wonderful food and even better company, and we had a blast!
 
After 2 years of isolation, running around delivering mishloach manot and then having a lovely seuda with a bunch of good friends was absolutely AMAZING! May this holiday be only the beginning of bigger and better things and many more good times shared with family and friends!!

Happy Purim!

Initially I wasn't going to make a costume or bother with a theme for my shalach manos, but I decided that would just make Purim even MORE depressing. So instead, my costume pretty much sums up how life feels right now! Despite everything, I got to go outside a bit and see a few people and even have a delicious meal from Izzy's Smokehouse. And then I got a rambling blessing in Yiddish from an outrageously drunk chasid, so overall I'd say my Purim was a success!

Hopefully next year we'll finally be able to celebrate together in person!!!

Pandemic bronchoscopy and Pesach madness

You know your life is intense when your immediate reaction to the thought of potentially catching COVID-19 and suffocating to death is, “Not AGAIN!!”

I’ve been feeling oddly emotionally detached from the chaos and trauma that are currently engulfing the world. Part of it is because my life hasn’t actually changed that much, since I’ve been hunkering down and desperately trying to avoid germs for months now. I also have lots of experience with pondering all the terrible ways I could die and the fact that it could happen at any time, both before and after transplant. So I guess my whole life, and particularly the last year, have been excellent preparation for a world altering pandemic.

Last week I had a bronchoscopy. We debated whether it was worth the risk, but decided it was better to do it now before the world becomes even more chaotic and dangerous. Apparently I made it in just under the wire, as shortly afterwards almost all procedures were suspended. The whole bronch-during-a-pandemic experience was pretty surreal.  It started with a bizarre phone call over an hour before my appointment letting me know that they had an earlier opening available and I could come in whenever I wanted. That NEVER happens!! We then zipped through the trafficless city streets in record time. Valet parking was closed, so we used the lot across the street and walked to the hospital, which meant we got to see the usually jam-packed Perelman Center looking like a ghost town. On arriving at the hospital we had to use hand sanitizer and get checked for fever, and were asked about any potential COVID exposure. The pre-op waiting room was absolutely empty, which is absolutely unheard of. And then there were all the new regulations and procedures to protect both staff and patients: everyone in masks at all times, new extended cleaning routines, being placed in an isolation room post-op. I definitely wasn’t thrilled about heading to the hospital and potentially exposing myself to all kinds of germs, but I did feel pretty safe in the end.

Thankfully this bronch looked better than the last one, and we think I’ll be able to wait at least 2 months before doing it again. Who knows what the world will look like then, but hopefully, HOPEFULLY, it’ll look better than it does now. And 2 months from now will also be after my one year “lungiversary,” which is pretty mind blowing! It’s so bizarre that I somehow managed to survive respiratory failure and a double lung transplant only to end up trying to survive a global pandemic less than a year later. I’m not sure what I imagined my post-transplant life would look like, but it certainly wasn’t this!

Right now, however, I have a more immediate concern: Pesach (Passover)!! The last couple weeks have been a whirlwind of planning and lists and enlisting family and friends to go shopping for me and finding online retailers that still ship in a reasonable time frame and cleaning and cooking and tears and panic attacks. And I know I’m not the only one who has been losing my mind over suddenly needing to make Pesach for the first time. I know this has been an extremely difficult time for the entire Jewish community, and that so many of us are now forced to be alone during a holiday that typically involves multiple huge celebratory meals filled with family and friends. Honestly, I really wasn’t sure I could manage everything! Yet somehow here I am, several hours before Pesach, with almost everything prepared and ready to go. I hope everyone else also found unexpected reserves of strength to get through this, and that we all manage to make the holiday as festive and meaningful as possible.

Last year I was in the hospital for Pesach. I definitely had much higher hopes for this Pesach! But, at least I’m home, and mostly healthy. The mere fact that I’m alive to see this Pesach is a miraculous blessing! Yes, preparing was stressful, and exhausting, and the next few days will definitely be long. But, I did it! Last year I couldn’t have managed even a fraction of the work I put in these last few days. This year, I can BREATHE! So instead of focusing on everything and everyone that’s missing, I’ll try to focus on that. I’ll try to appreciate all that I accomplished, and the very fact that I was able to accomplish it. I’ll try to have the best Pesach I can under these conditions, and know that next year WILL be better. Next year we will be together again, and we will appreciate it all the more for having missed each other this year.

Chag kasher v’sameach (Have a happy and kosher Passover)!!

From Purim to Pandemic to Pesach

The good news is I haven’t had any symptoms since Purim. The bad news is the world has gone to pieces since then!

It’s kind of funny because while everyone else is losing their minds, for me these restrictions and anxieties are just a mild escalation. Welcome to post-transplant life everyone! I’ve been scrubbing my hands and avoiding crowds and running away from anyone showing any signs of illness for almost a year now. And even before transplant I was cautious about germs, as any respiratory infection had the potential to be disastrous. Since I’ve already been on edge all cold and flu season, I initially felt relatively calm about the whole COVID-19 situation.

Then the shuls closed down. And the schools. Large gatherings were prohibited, then smaller gatherings. The list of closed businesses grew by the day, and an 8pm curfew was established. Places that previously were safe for me became off limits, as stores were swamped with crowds of panicked shoppers at all hours of the day. Then there were the regular emails from Penn Medicine with new restrictions: all non-urgent appointments and procedures postponed indefinitely, routine followups to be conducted virtually, no visitors allowed. I started feeling more anxious as my already small world kept getting smaller.

On Tuesday I went to Penn’s COVID-19 testing center for a drive-through test, even though I haven’t had any symptoms aside from a few hours of fever and feeling a little off for one day last week. We don’t actually think I have COVID-19, as we’d expect an immunosuppressed transplant recipient to have a far more significant response. But nothing else showed up on my viral swab to explain my symptoms, and now I’m due for another bronchoscopy. During a bronchoscopy the infection could become aerosolized and contaminate the room, making it impossible to adequately clean between patients. Due to this I need to be cleared of COVID-19 before I can schedule a bronchoscopy. We also need to make sure I don’t catch it post-test and pre-bronch, so I’m basically semi-quarantined now. And even after the bronch, my transplant team recommends that I pretty much stay home and avoid most in-person human contact now that cases are spreading more rapidly and becoming more prevalent.

Honestly, even that wouldn’t be the biggest deal except for one thing: Pesach. I’ve been feeling increasingly overwhelmed and anxious every time I think about it. I usually go out for yuntiff and Shabbos meals on Pesach, if I’m even home at all. The extent of my Pesach cooking before now has pretty much been limited to hard boiled eggs and matza pizza. I also have never been in this apartment for Pesach and don’t have anything prepared here, since last year I was in the hospital. But now for the first time I can’t rely on going out for ANY Pesach meals, and I don’t know if I’ll even be able to safely accept food prepared by others by then. Suddenly I need to determine and purchase all the utensils and cookware and kitchen accessories I’ll need for Pesach, plus get all the food to make both yuntiff and weekday meals, including a seder. And somehow I’m supposed to do all of this while unable to leave home! I don’t even know what I need or what’s available, how exactly am I supposed to make a shopping list for someone else? Just thinking about it is overwhelming!

Ironically, I spent all of last Pesach stuck in the hospital wishing desperately to be home. And now this Pesach I’ll be locked up inside my home wishing desperately that I could get out. G-d certainly has an interesting sense of humor!

The best laid plans...

Well, after making it through the Jewish calendar without incident over the last several months, I guess I was overdue for having a holiday get messed up.

A year ago, I was extremely ill and was admitted to the hospital on Purim. A couple weeks later I unexpectedly found myself being worked up for a lung transplant. I've been a little freaked out thinking about that, and eagerly anticipated having a MUCH better Purim this year.

My Purim plans were already small. Between cold and flu season and the Corona virus, my doctor strongly encouraged me to avoid crowds, particularly crowds in small enclosed spaces. So no shul or parties or big communal seuda for me. But I still made plans, and I was really looking forward to them! I spent hours making lung lollipops and cookies for my fancy lung-themed shalach manos, and was excited to show off my costume while delivering them. I've had my costume planned for a year, ever since they started my transplant workup. I OBVIOUSLY needed to be Frankenstein's monster now that I'm cobbled together with parts from multiple people! For seuda I was going to join an immunocompromised friend whose journey to bone marrow transplant last year closely paralleled my own journey to lung transplant. Since neither of us could go to any large seudas, we decided to make our own. My Purim celebrations would be slightly curtailed, but I would still have a nice holiday, and I was very excited for it.

Aaaand then I woke up feverish today.

Thankfully no other symptoms except a headache, fatigue, and a slight increase in coughing, but my doctor still wanted to see me and get testing done. So instead of delivering shalach manos and having a seuda, I got to rush to Philly for an appointment. My chest x-ray and bloodwork came back normal, but it'll take at least a day to get the results from the viral swab. If we identify a virus but my symptoms remain mild, I'll be able to treat it at home. If the viral panel comes back negative but I'm still running a fever, I'll have to go to the ER for a chest CT scan and a COVID-19 test so we can figure out what's causing my symptoms. And if my symptoms get significantly worse, I'll have to go inpatient for treatment.

On the plus side, I haven't had a fever for the last several hours, so hopefully it'll stay gone and this will have been a lot of fuss over nothing. But that's the reality of post-transplant life: any sign of infection is pounced on and treated aggressively, lest it turn into something worse. Here's hoping I can weather this one at home!

I did manage to catch a megilla reading at the Adlers (while wearing a mask, staying on the outskirts, and trying not to touch or breathe on anyone or anything) before rushing to Philly. I also got to see some people and enjoy the in-person reactions to my costume and shalach manos, and some of my wonderful friends delivered the rest of my shalach manos for me. And just for fun I wore my costume to my doctor's appointment, and enjoyed watching each member of my medical team do a double take as they walked in the door. I had already given them lung lollipops and cookies last week at a follow up appointment - which they greatly enjoyed!! - so they basically got the full Purim experience!

So that was my Purim. Not exactly what I had planned! But at least I managed to stay out of the hospital this year, and hopefully I'll continue to do so for the foreseeable future!

(If you want to see more of my shalach manos, I made a video of the whole cookie and lollipop making process: https://www.youtube.com/watch?v=29ARL9xdQ-4)

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.

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!

Listed!

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

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

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

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

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

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

Almost listed

So, I’m ALMOST on the transplant list.

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

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

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

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

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

Throwing darts and hoping for a hit

Much to my surprise, I got a call Thursday morning saying that they DID have a room for me and could do a direct admission! Even the nurse was shocked, as it has been almost impossible for them to direct admit anyone recently. I was SO relieved not to have to spend hours waiting in the ER, and I fully attribute this blatant miracle to the power of Purim! Even better, they didn’t call me to come in until about 4pm, so I got to enjoy a full Purim seuda without interruption. Celebrating Purim before going inpatient definitely helped put me in a better frame of mind. I also realized that getting mishloach manot immediately before being hospitalized was absolutely perfect. Instead of going shopping for hospital snacks, I just waited at home and the snacks came to me! Winning!

We did a CT scan, and fortunately I DON’T have a pulmonary embolism. Unfortunately, I once again managed to develop pneumonia while on IV antibiotics, because I am super talented. This time I decided to take it to the next level and get pneumonia in BOTH lungs, because I’m an overachiever. If you’re gonna do something, may as well do it right!

There’s no way to tell whether the pneumonia is due to infection or due to aspirating reflux in my sleep, so we’re tackling both at the same time. On Monday I’m hopefully having my G-tube changed to a G/J-tube, which is less likely to cause reflux overnight. In terms of infection, the original plan was to continue IV antibiotics, as well as a whole lot of nebulizers and chest PT, and see if I had any improvement by Monday. If I didn’t improve, the plan was to start treatment for the NTM (non-tuberculosis mycobacterium) that’s been showing up in my sputum in recent months. The problem is that requires 3-6 months on multiple antibiotics, one of which is IV Tobramycin, the antibiotic that gave me tinnitus and can cause hearing loss. I REALLY wasn’t thrilled about having to choose whether I wanted to breathe or whether I wanted to hear. HOWEVER, on Sunday they got some new test results back, and discovered that my pseudomonas infection is now resistant to 2 of the 3 antibiotics I’ve been taking. Surprise! They also found one more antibiotic that it should be susceptible to, so the new plan is to try that for a week and see if I feel any better. I’m all for anything that pushes off going back on Tobramycin!

It was a pretty rough weekend. Between coughing, fevers, being poked and prodded at all hours, and just generally feeling gross and uncomfortable, sleep hasn’t been happening on any sort of regular basis. Additionally, I have been coughing up A LOT of mucus. Getting it out is definitely a good thing, but it isn’t coming out easily, and the whole process has been exhausting. My throat is ragged from all the coughing, and I’m having trouble sleeping because I keep waking up choking on mucus. I’m not exactly living my best life right now, but hopefully the new antibiotic will help and things will pick up soon.

I’ve also been doing vlogs of my hospital adventures if anyone is interested in a slightly different version of these health updates. If you subscribe to my YouTube channel and click the notification bell, you’ll get alerts when a new post goes up. Let me know what you think!

https://www.youtube.com/channel/UC0yVedzFwXoGoqVI_Sp9NhA