Iron is miraculous!

I finally have some GOOD news for a change: The iron infusions are working!
 
I got my first iron infusion on Monday November 30^th, and a few days later I already had more energy and motivation. Over the last couple weeks I’ve felt more like myself than I have in MONTHS. The biggest change I’ve noticed is that I’m cooking again! Since the summer, I’ve only cooked when I had extra motivation, such as Shabbos or holiday meals. The rest of the time I just scrounged up whatever was easiest because I couldn’t be bothered to do anything more. It got to the point where even boiling pasta was too much, and most of my weekday meals were pre-made garbage. The times that I forced myself to cook took a LOT of effort, and I was usually pretty cranky by the time I dragged myself off the couch and into the kitchen.
 
But then! A few days after my first iron infusion, on a random weekday, I made tuna casserole for the first time in months. A couple days later I baked cookies. Last week I decided to make latkes for the first time ever, and they were so good that I did it twice! And then I made more cookies! And then I got REALLY ambitious and tried my hand at homemade donuts!! These tasks that seemed impossible a few weeks ago are suddenly easy, and I’m not even grumpy about doing them! And whereas before I spent almost every day laying on the couch, over the last couple weeks I’ve become a bit more active, and a bit more social, and just overall happier and more energetic. It’s amazing what a little iron can do!!
 
The iron infusions haven’t exactly been a party, however. Fortunately my veins held up to repeated IVs, but it burned a bit going in and the IV site was pretty sore afterwards. The infusion itself took an hour and a half, and the whole process took almost 3 hours. And because of Covid, no one could come into the infusion center and sit with me. After each infusion I was exhausted, and also had abdominal cramps as a fun side effect. I got my 3^rd and final iron infusion this week, and I’m definitely glad they’re over! But I’m even more glad that they were actually worth it!!
 
I still have some medical shenanigans to go this week, in the form of a follow up appointment with my transplant team, and an infusion of Reclast to help promote bone growth since my bones have become thin due to steroid use. But HOPEFULLY after this I’ll FINALLY catch a bit of a break, and maybe even get a chance to actually enjoy feeling like myself again!

Let it snow!

IT'S SNOWING!!!

Hoping we get enough and it's the right consistency to sculpt with! I haven't snow sculpted since before transplant, it's time to see what these new lungs can do!!

A few hours later...

I did it!!! The snow was a bit crumbly and wasn't the easiest to work with so I kept it small this time, but I got my snow sculpt on! Meet Snowball the snow cat!

And the verdict is...

It’s official: Pseudomonas REALLY loves me.
 
A few weeks ago, we got the results back from the sample they took during my bronch. Unfortunately, even after all that treatment in August, they STILL found Pseudomonas in my lungs. It’s unclear whether we never fully eradicated it or if my sinuses just reinfected my lungs that quickly, but either way it was very upsetting. I literally screamed with frustration after hanging up the phone. Damn you Pseudomonas!!
 
The new plan is to put me back on a nebulized antibiotic, specifically Tobramycin. Tobramycin is the one that gave me tinnitus, but taking it via nebulizer is far less risky than IVs. I haven’t done Tobra nebs in years because over time my airways became increasingly reactive, and I started having significant asthmatic symptoms every time I took it. Hopefully my new lungs will tolerate it better, especially since my clinic’s protocol is to only give post-transplant patients a half dose. I’m also taking an albuterol nebulizer beforehand to help open my airways and counteract any negative reactions. The downside of this is that it’s been so long since I took albuterol that I’ve completely lost my tolerance for it. For years I did albuterol nebs at least twice a day, so they didn’t really affect me.  Yesterday I took my first albuterol neb in months, and boy did it mess with me. My heart was racing, my hands were shaking, and for hours afterwards I felt shaky and worn out and had a headache. I really hope my body gets reacclimated to albuterol soon!
 
I was pretty freaked out when I got the bronch results, but I’m feeling calmer after hearing from some other post-transplant CFers who have dealt with Pseudomonas without it impacting their lung function. My team also wasn’t alarmed and reassured me that this is not uncommon, and that I will likely just keep going on and off Tobra nebs as needed. I’m not excited about getting back into a twice a day neb routine after having a nice little break for the last year or so, but that’s definitely easier than IV antibiotics. I also feel a little spoiled complaining about a few nebs after spending most of my life doing multiple nebulizers 2-4 times a day on top of airway clearance. My current nebulizer routine is still SO much lighter than it was pre-transplant!
 
Unfortunately, I’ve still been struggling with fatigue and low mood. We decided a couple weeks ago to discontinue the Trikafta entirely, since it’s unclear how much benefit I’m getting and it could be worsening those symptoms. I do think my mood has improved a bit, though I’m not sure about the fatigue. We recently discussed the fact that my iron levels have been a bit low, which could be contributing to my fatigue. Unfortunately, oral iron supplements can slow down the gut, which is already a problem for me due to CF digestive issues and gastroparesis (aka delayed gastric emptying). Due to this, I’ll have to go in for iron infusions once a week for 3 weeks. During that time I’m also scheduled to get an infusion of Reclast to improve my bone density, which has been damaged by years of steroid use. Apparently infusions are the theme for December! I’m a little worried about how my terrible veins will handle all these IVs and infusions, since I no longer have a port and I doubt they’ll give me a PICC line for just 4 infusions. Hopefully it won’t be too bad, and HOPEFULLY treating the anemia will be the fix I need to get my energy back!
 
I’m super not excited about heading into cold and flu plus Covid-Part-2 season. I guess it won’t really make much difference since I’m already isolating and being super careful, but it’s never fun knowing there are even more germs floating around trying to kill me. It’s also even less fun hunkering down and trying not to die when it’s all cold and dark and depressing outside. I remember how exhausting cold and flu season was last year, and how much I was looking forward to spring, only to get slammed with Covid instead. This year is worse since I’ve already been on varying degrees of lockdown for a full year now. Hopefully all the promising news about the Covid vaccine will come to fruition, and maybe I’ll FINALLY get released sometime this coming spring or summer!
 
That’s the latest in my life of medical shenanigans. Hopefully I’ll have a more uplifting update sometime soon!

New additions to the Bronchoscopy Collection!

I'm home! Bronch went well and the doctor said my airways looked significantly better this time. They still did some dilation, but hopefully I'm finally making some real progress after a year and a half of endless bronchs. Maybe some day I'll actually be able to get off this bronch train!
 
When I was hospitalized in August I had an unexpected bronch, and Katherine wasn't able to get me a bronchoscopy animal. So I got a bonus this time!

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!

Living on the edge

While discussing Katherine’s latest electric vehicle (in this case scooter) induced injury, and how the risk is part of the fun: 

Me: For me, just going outside is an extreme sport with risk of death. If I want to get really wild and crazy, I'll eat a rare steak!

(One of the many post-transplant rules is no undercooked food due to the risk of infection)

Freedom!

2 and a half weeks later: IV free and home at last!

Counting down!

The countdown is on!

Tomorrow I will finally finish a 2 week course of Cefiderocol and hopefully be rid of both pneumonia and Pseudomonas. Nausea and GI issues are now mostly under control, aside from IV antibiotic induced side effects which should hopefully clear up once I’m IV free. I also started Trikafta will no ill effects thus far. We’re discussing possibly starting a nebulized antibiotic so I can get onto a home treatment routine that will hopefully prevent Pseudomonas from causing more problems. We’ve just about gotten everything sorted out and squared away at this point, which means I can finally. Go. HOME!

Today I got to see more of HUP’s Covid discharge ritual, as a Covid patient was actually discharged from the floor I’m on. In addition to hearing Here Comes the Sun play over the intercom, I saw the staff line the hallway and clap and cheer as the patient left the unit. I feel like I’ve now had the full hospitalized-during-a-pandemic experience!

We had all kinds of hallway drama and excitement today. An angry and possibly confused patient roaming the halls at 1am yelling at someone on the phone, an accidental code call that sent everyone scrambling, said code call malfunctioning and ringing for an hour before they could turn it off, another patient who refused to stay in bed and triggered the bed alarm every five minutes… The last 20 hours have been the kind of day that nurses HATE. I, of course, am loving it. My room is right next to the nurse’s station, so I have a front row seat to watch all the chaos. I do wonder how my old neighbor is doing though, and if she’s still gifting Founders 14 with her taste in music – whether they want it or not!

The plan for tomorrow is to finish my last dose of Cefiderocol, pull my PICC line, and send me HOME! I’m honestly a little nervous to be IV free since the last time we pulled my PICC line I ended up in the ER less than 2 weeks later, but I’m hopeful that this time stubborn Pseudomonas has finally been evicted. I’ll find out for sure in a few weeks when we do follow up testing. But whatever the outcome, I’m VERY much looking forward to being home!!

Boredom

Two weeks in the hospital. I think my brain is melting... This was last night's entertainment.

Send help.

(Be sure to watch with sound to get the full effect!)

Let them eat... corn?

Seriously, dietary? On what planet is a pile of corn a reasonable meal? The best part is it isn't even kosher! The kosher meals come sealed, whereas this was clearly prepared and plated with non-kosher utensils. So they gave me a completely inadequate meal that I can't even eat!

Good thing I usually don't eat their meals anyway and have my own food. I'm even more grateful now to the Becks, Berdugos, Friedners, and Katherine for providing me with delicious food for Shabbos!!

A mixed bag

It’s been a bit of a mixed bag around here.

The good news is, we found an effective antibiotic that doesn’t have serious side effects. The bad news is, it can only be given in the hospital. Apparently this is a really new medication, and home infusion companies don’t want to touch it. It’s also very expensive, so even if we could find a home infusion company, insurance won’t pay for it. In the hospital, however, insurance pays a standard daily rate which covers all treatment and medications. This almost certainly costs more than just paying for the medication at home, but no one ever accused insurance companies of being sensible. I started the new medication on Friday, so I’ll be inpatient until August 27^th for a full 2 week course. I’m actually extremely lucky that I didn’t need IV antibiotics before now, since the only other antibiotics my Pseudomonas is susceptible to are either ototoxic (toxic to the ears) or nephrotoxic (toxic to the kidneys). I already have antibiotic-induced tinnitus and mild hearing loss, and my anti-rejection meds strain my kidneys, so I’m fortunate that this other antibiotic came out!

In other news, as so often happens, my stomach decided to jump on the bandwagon and start making problems as well. Despite the fact that weeks of IV antibiotics basically gave me an unwanted colon cleanse thanks to unpleasant side effects, my stubborn CF bowels have somehow managed to start working on a blockage anyway. Fortunately we caught it before things got too bad, but I’ve been very nauseous for the last couple days. We’ve been slamming me with laxatives since last night, so that’s always a joy, but I think I’m doing better now. Here’s hoping that tomorrow I won’t wake up nauseous for a change!

In other news, as of yesterday I started taking… Trikafta! That’s the fancy triple combo CF drug that came out last year, which has been life altering for many CF patients. That’s also the drug I waited years for, since the previous genetic modulators didn’t work on my CF mutations, only for my lungs to crash and burn JUST before it came out. Trikafta won’t work on my lungs anymore since they have different, CF-free genetic material, but CF is a full body experience and it can still help with other problems. We’re hoping it will improve my sinuses and cut down on sinus infections, thus reducing the risk of lung infections. It might also help my bowels, which would certainly be nice. The downside is it can have unpleasant side effects, sometimes to the point that people can’t continue taking it. Right now I’m on a half dose to see how it goes. It also interacts with my anti-rejection meds, which is why we haven’t tried it before now. So we’ll need to monitor my bloodwork very closely and adjust dosages as necessary to keep everything safe.

Starting Trikafta now definitely has a bit of a “too little too late” kind of feeling. If it had come out a year earlier, back in 2018 when my lung function was still at 60%, it might have done amazing things and my life now could be completely different. I’ve mostly resigned myself to the fact that for some reason my life was meant to follow a different path, but sometimes it still stings. But hopefully it can help me keep these lungs going for a long time, and maybe reduce some of my day to day CF symptoms in the bargain.

On the plus side, I’m very glad that they managed to transfer me to my usual floor, Silver 11. They know CF and lung transplant inside out around here, and my med schedule has gone much more smoothly. And they know ME, which is really nice! Disappointing as it is to be inpatient, it’s been nice to see everyone and tell them how GOOD I’ve been doing overall since my transplant. The last time I was hospitalized on this floor was just before transplant, when I could barely get out of bed and things were pretty dire. Meanwhile, yesterday I walked 17 laps around the nurse’s station at a pretty good pace! Everyone has been very happy to see how healthy I look now!

So that’s the story around here. I’m in it for the long haul, but hopefully Cefiderocol will finally kick this super stubborn Pseudomonas to the curb!

My home away from home

They managed to get me into a room on my usual floor, Silver 11! Everyone on Founders 14 was very nice, but Silver 11 is like Cheers: Where everybody knows your name!

Here Comes the Sun

 I just heard Hear Comes the Sun over the hospital intercom! That means a Covid patient was just discharged! First time during this hospitalization!

Who are the people in your neighborhood?

Some of you know that I’m VERY nosy when I’m in the hospital. I usually leave my door open during the day so I can hear hallways happenings and keep track of what’s going on around me. I LOVE some juicy hospital drama! When I first arrived to the unit, I was disappointed to see that I was all the way at the end of a hallway, thus limiting my opportunities for spying. Little did I know that across the hall is the most dramatic room on the floor!

I knew something was up as soon as I arrived and heard music BLASTING from my neighbor’s room, to the point that both the transport tech and I commented on it. I ended up making eye contact with my new neighbor, and tried to be friendly by saying “Sounds like a party! Can we join?” But she just… stared… and possibly looked a little angry? I felt bad and hoped she didn’t think I was judging her music.

A few hours later, however, I definitely WAS judging her music. The party was still going strong at midnight, and though I’m a night owl, I felt bad for my other neighbors who were presumably trying to sleep. Even with my door closed I could still hear the music pretty clearly. I asked a nurse why they didn’t close her door, and he implied that she’s been here a while and there have been some issues when things aren’t the way she wants them. He did feel bad though and offered earplugs. I joked about playing my own loud music and starting a competition, and he said someone else actually did that, and it resulted in a lot of yelling back and forth between the 2 rooms! So I guess I’ll save that plan for if I get REALLY bored.

The next day the music was off, but I discovered that my neighbor is a LOUD talker. I clearly heard every conversation that happened in that room all day long. She seemed friendly, and clearly has been here for a while, as she cheerfully greeted every nurse and tech by name when they passed her room. So I decided to make it my mission to become friends! When I went for a walk up and down the hallway, I made eye contact, smiled, waved, and said hello – and she reciprocated! Success! Later I got some non-kosher pudding on my lunch tray, and I asked a nurse to give it to my neighbor and let her know it was from me. Shortly afterwards I heard her very enthusiastic response loud and clear! When I went for another walk I asked how the pudding was, and she said it was delicious and happily thanked me. So I THINK I’m officially in her good graces!

During the day she mostly chatted (loudly) with a visitor and watched TV, but later at night the music was back. Conveniently I mostly agree with her taste in music, so I found myself in the bizarre position of listening and singing along to music coming from another room! I even tried an experiment and opened up Shazam to see if the music was loud enough for it to identify songs. And the result was… YUP!

Fortunately I’m pretty laid back and the goings on across the hall mostly amuse me. This isn’t ugly drama like screaming and moaning, or annoying drama like never ending bed alarms. I have a little clay left, so I might even make a Pole Person and send it over with a nurse. The nurses thought she would LOVE that –  and I know I’ll definitely hear her reaction!

You gotta make your own entertainment when you’re stuck in the hospital. This time it seems like I hit the jackpot!

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!

Post-op recovery

Baruch atah Hashem, Elokeynu melech ha’olam, borei minei b’samim

Blessed are you Hashem our G-d, King of the Universe, who creates different types of spices

This blessing shows appreciation for something that most of us take for granted: our ability to enjoy pleasant smells. We say it every Saturday night before smelling fragrant spices during the Havdala ceremony at the end of Shabbos. A few months ago, however, I had to stop saying that blessing. My sense of smell had disappeared completely a few months earlier, and I could no longer smell the spices. It’s very bizarre to stick your nose in something that you KNOW has a strong scent, and yet smell nothing. Life is just a little blander when you can’t smell anything. You don’t even realize how present scent is in life until it’s gone. I obviously could live without it, and would be ok if it never returned, but I definitely missed it.

Sinus surgery, as expected, was not fun. During surgery my doctor removed tissue and bone to widen my sinuses so they can drain more effectively in the future. My nose bled for a few days afterwards, so I had gauze tied to my face to keep from dripping everywhere. Initially I also had packing in my sinuses, which the nurse practitioner described as 2 tampons sutured into my nose. That stayed in for a week, during which my nose felt very stuffed up. Thankfully the packing was removed during the first follow up visit, which wasn’t exactly fun, but was definitely a relief to get out. I wasn’t allowed to blow my nose for the first 2 weeks after surgery, which is annoying when you can feel all kinds of gunk stuck in your head but can’t do anything about it. I was encouraged to do frequent sinus rinses however, during which I flush 8 ounces of saline through my nose, so that helped. And of course I’ve had varying degrees of headache throughout this whole process, which I expect to last for a few months.

Aside from all that, I also did 3 weeks of home IV antibiotics after surgery to help reduce the infections in my sinuses. That meant 3 more weeks of lugging around an IV pump 24/7, and 3 more weeks of really fun side effects. My body does NOT like Zosyn. I once again had significant GI issues, various nutrient and medication levels dropped because I wasn’t absorbing things properly, and I was utterly exhausted. Between the 10 days of Zosyn in June, surgery, and then 3 more weeks of Zosyn, I haven’t exactly been living my best life this summer.

But! A few days after the packing was removed, I suddenly realized that I could smell things again! After months of nothing it was SO exciting to run around sniffing different things and realize that I could smell all of them! My doctor was surprised and said that most of his CF patients don’t regain their sense of smell, as by the time they have surgery the receptors have already been permanently damaged. I’m very grateful that I still had some sense of smell left to be saved! It’s a small pleasure, but I’m extremely appreciative to have it back.

As of last week I finished the Zosyn, and my PICC line was removed after 6 straight weeks. I am VERY glad to finally be IV free! Hopefully I’ll manage to squeeze a little bit of summer fun into the limited space that’s left around Covid and medical shenanigans!

Freedom!

After 6 straight weeks I am FINALLY IV-free! Goodbye Zosyn, you won't be missed!

Smelling the roses!

I CAN SMELL STUFF!!!! I've literally had NO sense of smell whatsoever for MONTHS, but as of tonight I can smell EVERYTHING!!! I'm currently running around the apartment sniffing random things and grinning like a maniac! SURGERY WORKED!!!

Home sweet home!

I'm home! My nose is still bleeding and I feel disgusting and I'm utterly exhausted, but I'm home. And on the plus side, my pain is already MUCH better. Now it's time to sleep alllll Shabbos!

Small victories

Here's a sweet tidbit: Every time HUP discharges a Covid patient, they play a snippet of Here Comes the Sun over the intercom as they're walked out. So far I've heard it twice!

Post-op update

Surgery went well, of course I'm in pain, but I've definitely been through worse. Now for the 24 hour nose bleed and not being allowed to blow my nose, fun stuff. I'm settled into my hospital room, and hopefully should be discharged tomorrow. And I just got some Percocet so hopefully life will be better soon.

Slogging towards surgery

Well, I haven’t exactly been living my best life lately.

10 days of IV antibiotics was not fun. I started off with some (thankfully minor) PICC line issues, and ended up getting 3 dressing changes during the first 5 days, so that was great for my sensitive skin. Then the area around the suture holding the PICC in place became red and painful, so the suture had to be removed. I was mad that they put in a suture anyway though, so that was actually a relief. And then there were the side effects. The antibiotic absolutely trashed my digestive system, which wasn’t fun. I was also super exhausted, and had some weird unexpected reactions like random heart palpitations. Nothing too terrible as potential side effects go, but it wasn’t comfortable or enjoyable.

I was also hooked up to a pump 24/7. Due to the fact that we were fighting multiple infections, including a strain of pseudomonas that is resistant to most antibiotics, they could only find one antibiotic that would be effective. That antibiotic, Zosyn, has to be given every 6 hours. I had 2 options: I could run the infusion myself every 6 hours using a little self-infusing device called an Eclipse ball, which would mean never getting more than a few hours of continuous sleep for 10 days straight. Or I could get a pump that would run the infusions automatically and only needed to be set up once a day, but I could never take it off. I’ve used the Eclipse balls for 6-hour dosing before, and I about lost my mind from sleep deprivation, so I tried the pump this time. It weighed about 2 pounds and came in a little carry bag. It was definitely annoying to cart around all day, but that was way better than never sleeping, so I think I’ll stick with it for future infusions.

Fortunately, it worked. After 10 days I was less congested and my white blood cell count came down. It’s funny, after all the congestion I had for decades pre-transplant, I actually barely even noticed the little bit of congestion I had this time around until it cleared up. Congestion is so normal for me that I forgot these lungs aren’t supposed to have any!

And now I’m gearing up for the next round: sinus surgery. Tomorrow I get to have Covid test #4, and Thursday morning I go in for surgery. I’ve been out of sorts and sleeping really poorly recently, and I think I’m stressing about surgery. On the one hand, I’ve done it before, so I know it’s not a huge deal. On the other hand, I’ve done it before, and I know that recovery isn’t pleasant. I also haven’t done it in 11 years, so I don’t entirely know what to expect. And of course I have no clue how it will go and how much improvement I will see. It’s funny, I’m actually stressing more over this comparatively minor surgery than I did for my lung transplant! Before transplant I was SO sick that I didn’t even have the physical or mental energy to stress about it. I just wanted anything that would get me out of the hell I was living through. It also happened so quickly that I barely had time to think about it, since I was only actively on the list for 3 weeks. So this is the first time in years that I have both the time and the mental capacity to anticipate and be stressed about upcoming surgery. I’m not enjoying it.

I’m also not enjoying the fact that I’ll be back on Zosyn for 3 full weeks after surgery. 3 more weeks of this annoying PICC line, 3 more weeks of side effects, 3 more weeks of being attached to a pump 24/7. And of course this is all on top of a global pandemic!

Overall, it’s shaping up to be an incredibly annoying summer. I REALLY hope this all ends up being worth it!

Hello IV antibiotics my old friend

Alright sinuses, that is ENOUGH out of you!

Last Thursday I had a bronchoscopy, which thankfully went well. Once again the airways were narrowed and had to be dilated, so it looks like I’ll still be getting regular bronchoscopies for the time being. Thankfully the recovery wasn’t too bad this time. With that done, I thought my next big medical adventure would be sinus surgery in July.

I thought wrong.

During bronchoscopies they always collect a sputum sample and check for infection, and this time pseudomonas and staph showed up. That’s not great, but it also isn’t the first time I’ve had lung infections post-transplant. These infections are most likely coming from my terrible sinuses, and we’re hopeful that sinus surgery will help significantly. Initially my doctor didn’t think I needed immediate treatment, and planned just wait for the IV antibiotics I will receive after sinus surgery. However, since the bronch I’ve noticed a slight increase in congestion, and this week my bloodwork showed an increase in white blood cells, which indicates that my body is fighting an infection. That’s a problem, since my immune system is suppressed and far less able to overcome infections. Additionally, once my immune system is activated it could easily notice that these lungs aren’t original parts and reject them. That means it’s time to help my body out and zap me with antibiotics to get rid of the infections and send my immune system back to sleep.

This morning I got to have YET ANOTHER Covid test (that’s #3!) so I can go in to get a PICC line placed tomorrow morning. Once that’s in I’ll get started on 10 days of home IV antibiotics to wipe out the nasties in my lungs. After that the PICC line will stay in until my sinus surgery, and I’ll do another round of IV antibiotics afterwards to help clear out my sinuses and protect me during recovery.

I’m definitely not thrilled about having a PICC line for over a month. They’re uncomfortable, and I can’t take a normal shower the whole time as I have to be extremely careful not to get the dressings wet. I’ve been feeling pretty cranky about the whole situation. On the other hand, compared to what I was dealing with before transplant, this is nothing! I’m trying to remember that I know from experience that things could be SO much worse, and that this is a relatively minor bump in the road. Even with the procedures and issues I’ve had to deal with, my post-transplant recovery has really been going remarkably well, and I am SO grateful for that.

Despite everything, Katherine and I got out of the house and went on a nice outing today. Our 3 year anniversary is this Saturday, but we decided to celebrate today before the medical shenanigans get underway. We picked up takeout in Philly and took it to Penn’s Landing for an outdoor dinner on the waterfront. It was great to get out for a bit and do something halfway normal for a change! It’s so important to remember all the things that make this fight worthwhile.

So here I go, back into the fray, once again battling my nemesis pseudomonas and its friends. I hope those little unwelcome invaders are ready, because they’re about to be evicted!!

More procedures

It’s probably not a good sign when a medical professional looks at your CT scan and immediately says, “Oh you poor thing!”

I saw the ENT on Monday to check on my terrible sinuses. They’re always bad, but over the last few months I’ve been coughing a LOT more, which may be due to post-nasal drip. I’ve also completely lost my limited remaining sense of smell. Additionally, I’ve had recurrent infections in my lungs, which may be coming from my sinuses. The CT scan showed that my sinuses are all blocked up and full of polyps, which is kinda standard for CF. Between the ages of 19 and 30 I had sinus surgery 4 times to clear things out, and I only stopped due to feeling that the last couple surgeries didn’t give me enough relief to be worth the difficulty. I mostly just got used to having miserable sinuses, and at this point I haven’t had surgery since 2009.

My transplant doctor has been talking about sinus surgery to clean me out and reduce the risk of lung infections. The problem is that there’s no guarantee as to how much surgery will actually help me. It’s not the worst surgery in the world, but it’s definitely not fun, and it can take months for everything to heal up and see any results. A big deciding factor for me was my sense of smell. The ENT said the damage might already be permanent and surgery might not help, but not having surgery guarantees that the damage will become irreversible. He also said that based on my CT scan, I’ll definitely feel SOME improvement from having surgery, it’s just unclear how much. The only thing we can do is try and see what happens, so I’m currently scheduled for sinus surgery in July.

Even though it was my choice, I’m having all kinds of conflicting emotions about it. For one thing, any surgery is a big deal when you’re immunosuppressed, and it’s typical to get prophylactic antibiotics. So I’ll be hospitalized for (hopefully only) one night afterwards for observation, and then sent home with a PICC line for a few weeks of IV antibiotics. At this point it’s been over a year since I was on IV antibiotics, which is wild considering I used to get them 3 times a year for a month or 2 at a time! So on the one hand I’m super grateful for how much healthier I am, but having to do IVs again is stirring up old memories and fears, and part of me is bitter about having to revisit that part of my life. It’s also hard to force myself to go through what I know is an unpleasant procedure and recovery process without knowing how much benefit I’m actually going to see. It’ll be extremely upsetting if I go through all this and it doesn’t end up improving my quality of life. But there’s no way to know what the outcome will be, so I just have to try and hope for the best.

It also feels like my life these days consists only of sitting at home or going out for medical appointments and procedures. Covid life is definitely getting to me, particularly because no matter what the governor says, I’ll still be on my own personal lockdown due to being in a high risk category. As everything opens up I’ll need to be even MORE cautious, as there will be more spread in the community. It’s frustrating to go through all that transplant entails only to have life look dismayingly similar to how it did back when I was sick. It’s wonderful to be able to breathe, but it would be nice to experience more from life than just managing medical issues.

Speaking of which, tomorrow is bronch day. Which means I had to go get a Covid test today, because you can only go into the OR if you’re cleared. So THAT was fun. Apparently I’m currently on the monthly Covid test plan: this month for the bronch, next month for sinus surgery, and the month after that for my Pulmonary Function Test (PFT). Boy do I know how to party!!

And so my life of lockdown and medical shenanigans continues. Can’t wait for the day that I get to leave at least some of this drama behind and finally get back to LIVING!

As the world burns

My country is burning, and I don’t know what to do.

I mostly avoid posting about current events or politics – partially due to mental and physical exhaustion, partially because it feels like an exercise in futility. But the current situation is so profound that it feels wrong to be silent. Silence is complicity, and I refuse to be complicit.

It’s so easy for those of us who are insulated from or unaffected by racial discrimination to point fingers and decry the violence that has erupted. But did we also decry the violence that brought things to this boiling point? Did we also take a stand against lifetimes of inequality and oppression, and one senseless death after the next? Why is it only NOW that so many people suddenly feel the need to speak up and condemn? I certainly don’t agree with violent riots, but part of me definitely understands them. It’s very nice to talk about peaceful protests, but people have been protesting peacefully for decades, and little has changed. When peaceful protests are ignored, violent protests follow. Violence is tragic, but it certainly forces an immediate response.

At the same time, it’s extremely frustrating to watch the narrative get hijacked. I watched the news tonight, and most of the reporting focused on looting and vandalism, while the actual protests and the cause they champion seemed to fade into the background. It’s infuriating that activists who are actually seeking justice are being ignored in favor of individuals who are simply using the cause as an excuse to sow chaos for their own personal gain.

I look at the world, and I feel a terrible hopeless cynicism. The problems are so big, so deeply entrenched, that they feel insurmountable. I despair when I see the chasms dividing our country, how quick people are to villainize and discredit everyone they disagree with, how little effort there is to open real dialogue and attempt to build bridges. Even as the country burns, I still can’t see change happening. I just see everyone backing further into their corners and doubling down on everything they already believe. I watch and I wonder how long we can go on like this, how much more we can withstand before society collapses into irreparable lawless chaos.

The world is sick, and I feel the need to do something to help heal it. But I also feel small and helpless, immobilized by my limitations. I can barely leave my apartment, much less go to protests or join in community work. So I watch, and despair, and pray that someone else will do the work for me. And soon even that will become too much, so I’ll close my eyes again, and turn away from the pain and tragedies that I am currently powerless change.

I will retreat back into silence, but it’s not because I don’t care. It’s because I care too much.

Happy Lungiversary to me!

It’s officially been 1 year since my successful double lung transplant! Happy lungiversary to me!!

The last few months have been emotionally intense. Obviously the whole world is in crisis right now, and everyone is struggling and suffering in various ways. I have the added bonus of being in a high risk category and getting to worry about yet another threat on the long list of things that could kill me. But in addition to that, I’ve spent a lot of time remembering what life was like last year during the months leading up to transplant. Thankfully my memories of what it was like to be slowly suffocating to death are faded, and most of that whole time period is a bit of a blur. But I do still remember that it was horrible to the point that I wished for death just so the suffering would end. During these last few months I kept thinking about where I was a year earlier, and being a little traumatized by the memories. It’s been worse during the last couple weeks, which is when I struggled the most and went into respiratory failure last year. Getting a taste of a slow and painful death takes more than a year to get over.

But on May 12^th the memories were different. I remembered getting the news, and calling Katherine and my mom to tell them. I remembered waiting to be brought down to surgery, and then going down to pre-op. My memories of that day are also hazy, but at some point in the ICU afterwards I asked how long the surgery took, and wrote down all the times. 2:19 PM – into the OR. 4 PM – anesthesia. 6 PM – first cut. 12:17 AM – out of the OR. And 6:15 PM on May 13^th, less than 24 hours after surgery – taken off the ventilator and extubated.

I didn’t have any sort of major celebration right after the transplant for multiple reasons. The immediate aftermath of a lung transplant is a crazy whirlwind of pulmonary rehab and testing and doctor’s appointments. It’s exhausting and stressful and painful, and putting together a party was not on my priority list. Due to heavy immunosuppression I also couldn’t be in crowded spaces, which makes it a little difficult to host anything. But most importantly, I was well aware that the first year after transplant is a critical and tenuous time, and I didn’t feel comfortable celebrating until I saw how things went.

As we got closer to the year mark with relatively smooth sailing, I started considering whether I should have a party on the actual lungiversary, or wait a month or 2 for my immunosuppression to be lowered so I could be around more people. Never did I imagine that all in-person gatherings would be cancelled anyway by a global pandemic! Initially I was too upset to plan any sort of celebration. Having life basically be cancelled and being cooped up in my apartment week after week with no end in sight didn’t exactly have me in a festive frame of mind. But as the date came closer, I started to wonder… Maybe we could do a drive-by celebration, as has become popular these days? Maybe a Zoom party? Maybe both?? So I flung it out to Facebook, and the enthusiastic response convinced me to go for it. And I’m so glad I did!! It was so wonderful to see everyone who turned out, both in-person (at a social distance of course!) and online. The car parade in particular was wonderful fun and really got me into a celebratory frame of mind! I got balloons and streamers, and printed posters for my balcony, and baked my specialty lung-shaped cookies for the occasion. Seeing everyone drive by cheering and honking, waving posters, streamers and balloons blowing in the wind, was absolutely amazing! And then I ended up on the news to boot! What an amazing way to celebrate the incredible gift of life!!

At the same time, I’m also keenly aware that while we’re celebrating, somewhere out there another family is mourning. Somewhere out there another family is coping with death instead of celebrating life, and remembering the tragic loss of their loved one. This is the duality of transplant: my life could only be saved if someone else died. I am so endlessly grateful to my donor and their family, and I will never forget their pain and loss. I will always wish there had been some other way.

One year. It’s amazing how much can change in just one year! I’m still recovering, and COVID-19 has thrown unexpected complications into that process. And of course life after lung transplant is never guaranteed, and I will always live with a certain amount of fear and caution and what-ifs hanging over my head. But, against all odds, somehow at 41 years old I’m still here, still laughing, loving, and celebrating. And hopefully I’ll have the opportunity to celebrate many more lungiversaries for many years to come!