The end of an era

It’s the end of an era.

A week ago I had my last scheduled appointment with Dr. Courtwright, who has been my transplant doctor for the last 5 and a half years. I first met him a few years before my transplant during one of my many hospital admissions. The outpatient lung transplant doctors are also part of the inpatient Advanced Lung Disease team, so they all take turns being the attending doctor on the inpatient unit. I was always happy when Dr. Courtwright was my attending. Not only is he extremely skilled and knowledgeable, but he is also friendly, supportive, and has a good sense of humor. When I was being worked up for transplant, it was actually Dr. Courtwright who told me that I could choose anyone from the team to be my outpatient doctor after transplant. As soon as he asked, I immediately chose him!

Dr. Courtwright happened to be working on the inpatient unit the week of my transplant. A couple days beforehand he told me that I was at the top of the list and that he thought I might even be transplanted that weekend. And he was right! We actually had a funny moment with him after I went down to pre-op. I was brought down first, and Katherine and my mom came down shortly afterwards. It was a Sunday, however, which meant most of the operating area was closed. When they tried to take an elevator to the pre-op area, the elevator wouldn’t open! They had to go back up to the floor and find a nurse with an access card to let them in. Then when they finally got to the pre-op area, they couldn’t find me! Everything was closed and dark since it was the weekend. They were wandering around and couldn’t find anyone to direct them, when somehow they ran into Dr. Courtwright, who was also looking for me! The 3 of them video chatted with me asking where I was, and I showed them the area I was in, which gave Dr. Courtwright enough information to lead them to the one open pre-op area where I had been taken. It was a chaotic but funny interlude during a stressful time!

Over the last 5 and a half years I have had countless appointments with Dr. Courtwright. He has guided me through various complications both big and small. He has always been available for all my questions and concerns, and never once made me feel like anything other than a top priority. He is a very special doctor, and I’ve been so blessed to have him! Sadly for me and the entire transplant team, he and his family are relocating to Salt Lake City. I trust the lung transplant team at Penn and am sure that my new doctor will be great, but nobody will be able to replace Dr. Courtwright. Having a good relationship with your doctor is HUGE when you have a chronic illness, and I’ve been so lucky to have someone as phenomenal as Dr. Courtwright in charge of my care for so long. I will truly miss him, and I wish him the best of luck in everything wherever he goes!

Medically busy

A couple weeks ago was the most medically busy I’ve been in a while!
 
Over the last several months I’ve been getting out of breath more easily, and have found myself getting short of breath even walking short distances. My doctor thought that my airways might have narrowed again, possibly triggered by the 2 respiratory infections I had earlier this year. During the first couple years post-transplant my airways narrowed frequently and I needed regular bronchoscopies to dilate them. Thankfully this settled down over time, and it had been over 2 years since my last bronchoscopy. But a couple weeks ago it was finally bronchoscopy day once again! Fortunately it went very well and I recovered relatively easily. They dilated some of my airways and took sputum samples to check for infections. To no one’s surprise they found pseudomonas in my lungs, which has been a recurrent issue. Right now I’m not showing any significant symptoms so we’re not jumping to treat it, and will just continue monitoring the situation. On the plus side, once I recovered from the procedure I definitely noticed that I was breathing better and was able to walk more easily. Hopefully that’ll take care of the issue and I won’t need another bronch for a long time!
 
That wasn’t my only medical procedure though! Back in February my right ear got clogged up while I was recovering from a cold. It was uncomfortable and also impacted my hearing. At first we expected it to clear up on its own, but after a couple months it hadn’t made any progress. I already do regular medicated sinus rinses, so we tried adding a nose spray to open things up. When that didn’t work we tried raising my prednisone for a couple weeks (aka a prednisone burst), which also didn’t help. Finally I made an appointment with a specialist to get my ear checked out. I originally thought I could see my regular ENT, but apparently he only does sinuses, so I had to go see a completely different ENT. A hearing test confirmed that my hearing was compromised in that ear, but it also showed that the hearing loss was due to a blockage. So they put a tube in my ear! They poked a tiny hole in my eardrum to let the fluid drain, and stuck a little plastic tube into the hole to keep it open long enough to clear everything out. They said since it had been so many months the fluid is probably thick and will take time to come out, so the tube will keep the hole open for 6-12 months before falling out. Getting it in was uncomfortable, but I felt an IMMEDIATE difference both in terms of less pressure in my ear and being able to hear MUCH better. During the day my ear feels fine and I don’t even notice the tube. Overnight, however, the fluid drains out when I lay on my side. It’s a little uncomfortable and a little gross, but it’s better than not being able to hear!
 
After not having much going on medically for a while, 2 procedures in one week felt like a lot! Thankfully they were both pretty routine, and both produced immediate results. Hopefully they’ll just be an unexpected bump in the road and I won’t need any more procedures anytime soon!

2 year update

It’s been a LONG time since I wrote a health update. That’s partially been because, thank G-d, things have been going pretty smoothly! That doesn’t mean NO complications, just nothing major. Honestly, just maintaining a healthy baseline involves a bunch of medical stuff. I have to get bloodwork at least once a month, and sometimes more often if something is out of whack and needs to be monitored a little more closely. That something is usually either my Tacrolimus (anti-rejection med) levels being off and requiring a dosage adjustment, or my kidney levels being worse than usual. My kidneys have been struggling a bit, which is expected thanks to the Tacrolimus, but thankfully are still chugging along at a reasonable rate. We recently discovered that they still respond well to extra hydration, so if I drink A TON of water (which is really hard for me!) my levels are almost normal. Aside from bloodwork, I have a bunch of appointments that I need to have on a regular basis. Transplant clinic at least every 3 months, endocrinologist every 6 months, dermatologist at least annually, ENT at least annually. Then there are the minor procedures, such as getting my feeding tube changed every 6-12 months. I no longer require supplemental nutrition, but I DO rely on the feeding tube for additional hydration overnight to maintain my kidney function. Sometimes I’m on top of changing it and it’s just a routine appointment, and sometimes it gets slightly dramatic, such as a few months ago when I was overdue for a change and then accidentally yanked it half out of my stomach, resulting in it painfully sticking out of me overnight until I could get in for an emergency appointment the next day. I also have a Reclast infusion that I get once a year to try to offset the damage that long-term steroid use has done to my bones. And of course aside from all this, I take a ton of medication every day to stave off rejection and keep my body functioning. So even when things are medically calm, there’s still a lot going on!
 
I think the biggest complication that I’ve had was at a dermatologist visit last year. The risk for all cancers goes up significantly after transplant due to the anti-rejection meds, but the risk of skin cancer is PARTICULARLY high. At my last appointment they found that 3 small bumps on my face were pre-cancerous and needed to be removed. It wasn’t a big deal at all, the doctor froze them off during the appointment and I just had to deal with some unpleasant blistering afterwards, but even pre-cancerous was still a scary thing to hear. I have to be very careful about sun exposure and check my skin regularly for anything unusual, and of course keep up with regular dermatology appointments to keep an eye on things.
 
There’s another reason I fell out of writing health updates though, and that was thanks to Trikafta. Trikafta is the groundbreaking CF drug that came out shortly after my transplant. My doctors wanted me to take it to improve my miserable CF sinuses and reduce the risk of infections dripping down into my lungs. The first time I tried taking it about a year after transplant, I got REALLY depressed and fatigued, so we discontinued it. We decided to try again at a lower dose in July 2022. I seemed to do better at the lower dose, but when we tried to increase it I immediately had a bad reaction, so we dropped back down to the initial dose. I still ended up struggling with depression and extreme fatigue, but wanted to stick it out and see if the side effects faded with time. In January of 2023 we tried adding an anti-depressant to see if that would help with the side effects and allow me to stay on Trikafta. By June of 2023, however, it was clear that the depression and extreme fatigue were not going away. I decided it wasn’t worth being miserable and dysfunctional just to maybe improve my sinuses, so we discontinued the Trikafta and have no plans of trying it again. It took a WHILE for things to improve even once I stopped taking it, but thankfully I’m doing much better now. I still struggle with fatigue, but it’s back to the level I was dealing with before Trikafta, and my mood is MUCH better. It’s a shame that I waited so many years for a drug that targeted my genotype, only to be unable to tolerate it. At least it makes me feel a little better though that I didn’t end up missing out on anything by getting a transplant before it came out.
 
My only other “complication” is that after not catching any infections for over 4 years post-transplant, I managed to catch 2 colds this year, one in January and one in April. My symptoms were relatively mild, but with any infection there’s always the risk that it could trigger rejection. Thankfully I did NOT have any rejection after either illness – and in fact have not had any rejection since my transplant! Hopefully I can continue that streak, and also keep myself infection free for at least the rest of this year!
 
That’s pretty much what’s been going on the last couple years. Now that I’m finally caught up, I’ll try to be on top of writing health updates more regularly – but let’s hope I won’t have much to talk about!!

Happy 5th Lungiversary to me!

5 years!!!

Sunday May 12th was my 5th lungiversary! 5 years is statistically a Big Deal, as around half of lung transplant recipients don't survive 5 years after transplant. To not only survive but be doing really well and have had NO rejection during those 5 years is amazing!

We knew we had to celebrate this incredible milestone, but we thought it would be rude to host a party on Mother's Day (even though I actually got my transplant on Mother's Day!). We pushed the party off for a week, and Sunday was the day! We started off with a bagel brunch in my mom's backyard, before going to Philly for a sightseeing trolley tour! I had a great time spending the day celebrating with family and friends, and really appreciate all the people who came out to recognize this special occasion!

I also was surprised on Shabbos by several friends from my synagogue who sponsored kiddush in honor of my lungiversay! They even got me a cake for the occasion! Between kiddush on Saturday and the party on Sunday, it was a very celebratory weekend!

I am so grateful not only for being here to celebrate 5 years post-transplant, but for having so many people who love me and share in my joy! Here's to many more lungiversary celebrations!

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!

No news is (mostly) good news!

It’s been quite a while since I shared a health update. This time around, no news is mostly good news!
 
Things have actually been relatively calm in recent months. I had a bronch and a sweat test in July, but aside from those and bloodwork every few weeks I’ve actually managed to avoid any other procedures. I haven’t even had any doctor’s appointments! Katherine and I have been taking advantage of the calm to go on outings and little day trips. We went out to a free concert and fireworks for the 4^th of July, visited the beautiful Chinese Lantern Festival, drove to Edison for a cat expo, road a scenic railroad in Delaware, went to Wildwood to check out a tattoo convention, and spent an afternoon at the art museum. We’ve had a pretty busy summer, and still have more outings planned! Definitely a nice change from medical drama!!
 
There is one big thing going on though: Trikafta. I started taking it over a month ago, and it hasn’t gone entirely smoothly. When I first tried it a couple years ago I ended up stopping because it seemed to be making me fatigued and depressed. This time we tried increasing the dose slowly to give me time to adjust, and while I did ok with the first dosage, as soon as I moved up to the next level I started struggling with fatigue and depression again. Right now I’m back to the lowest dose and planning to stay here for a while to really give my body time to adjust before trying to go up again. I don’t want to waste the rest of the good weather and spend the Jewish holiday season being exhausted and depressed, so I won’t be changing the dose until the end of October at the earliest. I do think I’m still having some side effects, such as insomnia and fatigue, which is difficult but definitely much more manageable than what I experienced at the higher dose. It’s really frustrating that there’s this amazing medicine that could nearly “cure” my remaining CF symptoms, but I may not be able to benefit from it due to side effects. I have heard though that for some people the side effects fade after a month or 2, so I’m hoping that eventually I’ll be able to sloooowly work my way up to a full dose. Here’s hoping!
 
This month will be a little more medical, as I have appointments coming up with my transplant team and my endocrinologist. I also have a dexa scan scheduled to check my bone density, since my bones have been compromised by years of steroids. But aside from that I plan to keep getting out and about and enjoying myself as much as I can!

Free again!

Hooray for (relative) freedom!

A few weeks ago I got Evusheld, an antibody injection for people who either can’t take or are unlikely to respond to the Covid vaccine. We already know that I didn’t produce antibodies in response to my vaccines and boosters, so Evusheld is an important tool to help keep me safe from Covid. My doctor has seen really good results so far, both in that transplant patients who get Evusheld are less likely to catch Covid, and that those who do catch it have much less severe illness. Between finally getting Evusheld, and the local Covid numbers going down, he actually felt comfortable loosening some of my Covid restrictions! I can once again be unmasked indoors with a few people at a time as long as they are vaccinated and will warn me if they have any symptoms of illness. He does still want me to avoid indoor crowds entirely and to wear a mask if I’m in a crowded space outdoors, but that’s pretty manageable. Regardless of Covid I need to avoid people with any signs of illness, since I’m highly susceptible to infections and even a simple cold could be devastating, so that and avoiding crowds are just standard post-transplant precautions. It’s so nice to mostly be back to my regular levels of paranoia now that I finally have some antibodies in my system!

On a less exciting note, my lungs continue to be a petri dish. After a month of nebulized antibiotics the pseudomonas was thankfully gone, but it was replaced with staph. I’m still not having major symptoms so we aren’t jumping to treat it yet, but it’s frustrating to keep having one infection after another. We know it’s all coming from my sinuses, but my ENT didn’t think another surgery would be particularly helpful. So instead we’re going to try Trikafta! Trikafta is the ground-breaking CF treatment that came out shortly after my transplant. I actually tried it briefly a couple years ago, but stopped because I was possibly having side effects and it wasn’t clear whether it would even help my sinuses. But now there is more research showing that it DOES help the sinuses, so we’re going to try again and see if I can tolerate it, possibly on a partial dose.

Before starting though, I’m getting a sweat test. One of the weird things that happens when you have CF is that your sweat is extra salty, and a sweat test has been standard to diagnose CF for decades. So why do I need one now? Trikafta addresses CF on a cellular level, and even corrects the salt levels in the sweat. Since I might not be on a full dose, we need a way to check if the partial dose is working. If I get a baseline sweat test now, we can then compare my salt levels on Trikafta to see how much it changes. This won’t be necessary if I end up tolerating the full dose, but could be helpful if I need to take a modified dose. The funny thing is that I have to go to the children’s hospital to get it done, since it’s not typical for adults to get this kind of diagnostic testing. That should be interesting!

One other issue is that I’ve been having some on and off chest congestion over the last couple months and have been having trouble coughing it out. My doctor heard some stuff rattling around in my lungs during my last appointment, and thinks it might be time for another bronchoscopy to open up my airways. So that’s Monday’s adventure. Thankfully it’s been nearly 10 months since my last bronch, which is a record for me! Hopefully it’ll go smoothly and I’ll feel better afterwards.

That’s the latest medical shenanigans around here. Thankfully nothing too dramatic this time!