The plan of attack

We’re finally getting somewhere!
 
There was some confusion and communication mix-ups trying to coordinate between 2 surgeons, but in the end I’m scheduled for surgery with just my transplant surgeon tomorrow morning. He thinks that there’s a suture from transplant in my chest that has been harboring infection and allowing it to resurface, so he’s going to clean out the infected tissue and try to find and remove the suture. The last time I had these abscesses they also looked for a suture but couldn’t find it, so he plans to go deeper this time. Though I’ll be getting general anesthesia, he expects it to be a quick and easy surgery. And at least this time I’m only getting one side opened up, unlike last time when they had to open up both sides. Hopefully recovery will be a little easier this time.
 
One somewhat alarming thing is that on the MRI they saw a little bit of something starting on the other side too. They didn’t see enough to merit going in surgically, however. The current hope is that if an infection has also begun on the other side, the antibiotics I’ll be receiving will be enough to take care of it. The bad news is that susceptibility testing shows that my current infection is a multi-resistant monster like I had last time, so there aren’t many antibiotic options available. So far they have identified one antibiotic, which I started taking tonight. But they really want me on 2 antibiotics simultaneously, so they’re doing extended susceptibility testing in the hopes of finding other options. Right now the only other option available is Tobramycin, which I refuse to take. Tobramycin has already given me permanent (but thankfully mild) tinnitus, and the last time I attempted to take IV Tobramycin the ringing got louder within just a few days. It’s pretty clear that if I did a full 2 week course of IV Tobramycin, the ringing would become unbearable. Hopefully the extended testing will find at least one more antibiotic that I can use without terrible side effects.
 
We do still have another trick up our sleeve though. My transplant doctor has already been in touch with the bacteriophage (aka phage) therapy program at Yale. This is an experimental treatment that is not widely available in the United States. Fortunately for me, Yale has a phage therapy program specifically for CF patients with pseudomonas. Bacteriophages are a type of virus that attack bacteria without causing harm to humans. The catch is that each bacteriophage will only attack a specific bacteria, so they must be grown tailor made to the infection you’re trying to treat, which takes months. The current plan is to treat my infection immediately with surgery and antibiotics, submit a sample to Yale to develop phages for me, and then hit the infection again with phage therapy in a few months to hopefully eradicate it once and for all.
 
Right now my focus is on getting and recovering from surgery, and the impending course of antibiotics. It looks like I’ll be in the hospital through the weekend, and they’re tentatively talking about discharging me next week. It’s not fun being stuck in the hospital, and I’m definitely not looking forward to going through multiple rounds of treatment, but I’m grateful that I at least have some options available. Hopefully surgery will go smoothly and the recovery will be quick, and they’ll find one more great antibiotic for me soon!

The usual suspect

And to no one’s surprise, Lump is infected with… Pseudomonas.
 
That brings us one step closer to coming up with a treatment plan, but we’re still waiting for some important test results. Specifically, we’re waiting for susceptibility results, which will tell us which antibiotics can be used to treat it. Recently when I cultured Pseudomonas in my lungs, we were surprised to find it was less resistant than usual and was actually susceptible to multiple antibiotics. I have a hunch, however, that the current infection is leftover from the last time I had abscesses in my incisions, and that particular Pseudomonas was extremely resistant and had very limited treatment options. The susceptibility results are critical to determining what kind of treatment I’ll be getting, and we can’t really move forward without them. I’m hopeful that MAYBE they’ll come through tomorrow and we can get this show on the road.
 
Today’s adventure was getting an MRI to determine whether or not the infection has gotten into my bones. I had an MRI a few years ago the last time I had abscesses, but I had forgotten how unpleasant it was. I’m not usually claustrophobic, but being shoved into that little tube definitely had me a bit freaked out. It was also startlingly loud, and took way longer than I anticipated. All in all I spent around a half an hour in the machine, though I did request a brief break in the middle. And for some of the scans I had to repeatedly hold my breath for 20-30 seconds at a time, which was difficult. All in all it was NOT a fun experience! But thankfully it’s over, and hopefully I won’t have to get another for a long time, if ever!
 
On the IV front, unfortunately IV #3 didn’t even last a day. My team advocated very strongly for me to get a longer lasting type of IV called a PICC line, but apparently Interventional Radiology and the IV team are extremely stingy about them. Instead, the IV team put in an ultrasound guided IV, which THANKFULLY has actually lasted for over a day and is still going strong! And even more thankfully, tonight Interventional Radiology FINALLY told me that I could get a PICC line, so that should be happening sometime tomorrow. I’m really looking forward to NOT being a pincushion anymore!
 
On a lighter note, as is tradition, I have decorated my room with the latest batch of Pole People! A few staff members remembered them from previous hospitalizations, and a bunch of new-to-me staff have been pleasantly surprised by them. I hope you all enjoy them too!

Lump drama

Lump did not, in fact, keep it together.

Over the last week I've been both concerned about and frustrated by Lump. I had a few consecutive days where it was constantly itchy and/or painful, which was wearing me down. I still had so many weeks to go until my scheduled appointments, and I was not looking forward to being perpetually uncomfortable for at least month. Thankfully it eased off for a few days, but then it started acting up again on Friday. By Friday night it was swollen and red and looked particularly angry.

Late Friday night I woke up drenched, and immediately knew what had happened. Sure enough, Lump had burst and released a shocking amount of pus. I didn’t feel capable of dealing with anything at 3am, so I just cleaned up and went back to bed and decided it could be a tomorrow problem. In the morning it was still oozing an appalling amount of pus, so we reached out to my doctor, who fortunately had given me his cell phone for emergencies. Given the sudden escalation in symptoms and obvious signs of infection, he decided we couldn’t wait for my scheduled appointments, and it was time to be admitted to the hospital to fast track things and start IV antibiotics. So off to HUP we went!

The big advantage of being in the hospital is that I don't have to sit around waiting for appointments. They sent me for an updated CT scan, and took cultures of the disgustingness that was draining out of me so they can finally identify the infection and determine the best course of treatment. They also got me started on both an IV and oral antibiotic that they hope will be effective against whatever I have. They’re currently debating whether or not I need an MRI, and working on scheduling surgery to clean out the infection and finally evict Lump. They MIGHT manage to get me in by Friday, but that's unclear because they’re trying to have both my transplant surgeon and a plastic surgeon be involved in the procedure, so that’s 2 busy schedules to coordinate. In any event, I will definitely be getting things taken care of MUCH faster than originally planned!

Unfortunately, my veins have been particularly terrible thus far. My first IV went bad in less than 2 hours, before they even had a chance to run anything though it. My second IV did better, but still didn’t even last a day. And both of those IVs took 2 tries each to get in. Thankfully, IV #3 went in on the first try. Here’s hoping it has more staying power than the first 2 did!

So right now I'm in the wait and see part of this hospital stay. Waiting to get the results back from my cultures and determine a treatment plan, and waiting to see when they can schedule surgery. But at least now the wait will be MUCH shorter than it would’ve been as an outpatient!

Medically busy again!

It’s been a medically busy month!
 
I started things off with a CT scan. Almost 2 months ago I noticed a lump on one of my transplant incisions near where I had an abscess a few years ago. It wasn’t visible and was barely noticeable, but I was obviously concerned due to my history. The CT scan fortunately didn’t show any signs of abscess or infection, and my doctor reassured me that it was just a suture granuloma, which happens when the immune system reacts to a foreign object (in this case, a leftover suture from my transplant) by trying to form a barrier around it and wall it off from the rest of the body. My doctor gave me the option to meet with a plastic surgeon and have it surgically removed, but I decided not to pursue that unless it became more of a bother. Well, barely a week later it started bothering. Not only had it grown, but it had become slightly red and sore, which are both bad signs. I’m now scheduled for an MRI in mid-December, and will be having a plastic surgery consult afterwards. Let’s just hope Lump can keep it together until then and I don’t end up rushing to the ER with more significant symptoms the way I did the last time I dealt with something like this.
 
A week after my CT scan I got to have a colonoscopy. Not only have I reached colonoscopy age, but both CF and transplant put me at higher risk for colorectal cancer. Unfortunately for me, the last time I had a colonoscopy a few years ago the prep didn’t fully clear me thanks to my stupid stubborn CF gut. So this time I got to do the extended prep, which meant 2 days straight of clear liquid diet plus a ridiculous amount of laxatives. And guess what? After all that, I wasn’t clear AGAIN! What they were able to see looked fine, but instead of waiting 5 years for my next one, I have to do it again in 2 years. And who knows what kind of godawful prep I’ll have to go through next time! I’m already dreading it!
 
The week after that I had my last scheduled appointment with Dr. Courtwright, my transplant doctor, before he moves across the country. He’s not actually leaving until December 13^th though, so if anything goes wrong before then he’ll still be my doctor. And with the current Lump situation, I wouldn’t be surprised if I see him again before he leaves!
 
Last week I had my annual appointment with my ENT. Though a recent CT scan showed that my sinuses are blocked, he actually didn’t think that I would get much benefit from sinus surgery. One the one hand I’m glad to not have to go through an unpleasant surgery, but I’m also disappointed that there’s basically no chance of ever getting my sense of smell back. It disappeared completely again several months ago, and though there was no guarantee that I would get it back even with surgery, I had some hope because I unexpectedly got it back after my last sinus surgery. But no surgery unfortunately means no chance of regaining my sense of smell. I can definitely live without it, but I do miss it sometimes. My ENT also looked at the ear that has been giving me problems and saw a lot of drainage from the ear tube. He gave me antibiotic and steroid ear drops, and sent off a sample to see what infection I have brewing in there. And OF COURSE, as always, it’s my nemesis pseudomonas! My ENT is currently consulting with both my CF team and with colleagues who specialize in ears to come up with a treatment plan. I actually was pleasantly surprised that he dealt with my ear at all, as previously he referred me to another ENT since he specializes in sinuses and not ears. Hopefully he’ll be able to come up with a plan that finally deals with this ear issue once and for all!
 
And that brings us to today, and my consult with an oral surgeon. A couple months ago I went to the dentist for the first time in years. Between transplant and Covid, dental appointments kind of fell by the wayside, but fortunately I have good teeth so I could mostly get away with it. For years I said that G-d balanced things out for me, and gave me good eyes and good teeth to make up for all my internal organs being trash. I never needed glasses, or braces, or any other significant dental work. In fact, I never even had so much as a cavity! Well apparently getting new lungs voided the contract, because earlier this year I finally got glasses, and a couple months ago they found the first cavity of my entire life. Unfortunately, it’s close to the nerve, so they have to either extract the tooth or do a root canal. On the plus side, it’s in a wisdom tooth which is fully in, so extracting it isn’t a big deal. My dentist wasn’t comfortable with the angle of the tooth, however, so she referred me to an oral surgeon. The oral surgeon said it should be easy to extract with just local anesthetic, so I have an extraction appointment scheduled in 2 weeks. Here’s hoping it goes smoothly!
 
Overall my medical needs have been keeping me pretty busy lately, with still more to come! Hopefully I can get these various issues taken care of quickly and things will settle down soon!

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!!