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!!

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!

The hits keep coming

Can I return 2022? Mine is already broken.
 
Last week we were waiting for the results from susceptibility testing, desperately hoping to find an antibiotic with a less grueling infusion schedule. Instead, we found out that the previous 2 and a half weeks of sheer exhaustion were a complete waste, because MY INFECTION IS RESISTANT TO THE ANTIBIOTIC WE WERE USING. Not gonna lie, that was pretty crushing news, both because Katherine and I went through so much for no benefit whatsoever, but also because the only antibiotics left have bad side effects. Specifically, I have 2 antibiotics to choose from: tobramycin, which has already given me permanent (thankfully mild) tinnitus, and amikacin, which has already given me (also thankfully mild) hearing loss. The last time I tried tobramycin the tinnitus started getting louder in less than a week, so I decided to gamble on amikacin being slower to cause damage. Plus, hearing aids are a thing if necessary, whereas there is nothing you can do for tinnitus. What a garbage choice to have to make!
 
I have not yet started the new antibiotic schedule, because first they want to drain the abscesses so the antibiotics can penetrate more quickly. But they DON’T want to do full surgery, because my anti-rejection meds impede healing, so surgery could actually end up spreading the currently-contained infection without having a good arsenal of antibiotics available to treat it. Right now we’re waiting for the abscesses to get a bit bigger so they can go in and drain them with a needle, and THEN we’ll get me back on IV antibiotics.
 
We’re also exploring an experimental treatment called bacteriophage therapy. Bacteriophages are viruses that don’t cause infection in humans, but do infect and destroy bacteria. The catch is that each bacteriophage only targets a specific bacteria, so you need to cultivate exactly the right phage to treat an infection. There has been a fair bit of research on using bacteriophages to treat multi-resistant pseudomonas infections specifically, which is good news for me. So my pseudomonas sample was shipped off to Yale, and they were very impressed with how extremely nasty it is. Apparently it takes 4-6 weeks to get phage therapy approved and set up, so it’s a waiting game to see if I still need it by then or if the drainage and antibiotics take care of everything. At least I can say that I’ve gone to Yale and they were very impressed with me!
 
And because when it rains, it pours, we have even more on our plate. Unfortunately, after struggling for months, Katherine’s mother was sent home on hospice over the weekend. Katherine has been driving over an hour every day to be there, and will likely be visiting multiple times a week until she passes. To complicate things further, there was a potential Covid exposure in the home, so my doctor has recommended that I not go and that Katherine and I maintain social distance and wear masks to keep me safe. Hopefully we’ll be able to ease up on some of those restrictions soon, but for now the situation has been very difficult.
 
I really don’t have any good news to share right now unfortunately. I hope everyone else’s new year is going better than mine!

New year, new me!

I think I haven’t gotten a haircut for almost 2 years. I did go to a salon in September 2019 to get it bleached and dyed, but I’m pretty sure we didn’t cut it then, which means my last haircut was in February 2019 shortly before my big 40^th birthday party. After transplant I lost a LOT of hair, which actually isn’t unusual, but it was still pretty alarming to see it coming out by the handful. My doctor reassured me that it would stop within a few months (for those who know Dr. Courtwright, his exact words were “Don’t worry, you won’t end up looking like me”), and it did, but my hair was visibly thinner. Between that and the abuse I put it through with bleaching and dying, my hair has been looking pretty scraggly and gross, and I’ve been itching to get rid of it.
 
Meanwhile, Katherine has been cutting her own hair for decades, and also used to cut her friends’ hair. She’s been offering to cut my hair for AGES. I’ve been super restless lately and REALLY wanted to chop off my hair, but I also don’t feel comfortable allowing anyone near me right now, particularly since I’ve somehow managed to have 2 (low level) Covid exposures this month. So… Katherine finally got her wish! Introducing the new me!

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!

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!

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!!

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!

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!

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!

6-month bronchoscopy!

Today was 6 month bronchoscopy day - yes, it's been 6 months since my transplant! I'm currently in the post-bronchoscopy sore-throat worn-out still-groggy-from-anesthesia stage, so right now Katherine is dealing with me wandering around whimpering pathetically when I'm not laying in my recliner and pathetically whimpering from there. But she's the best girlfriend ever so she's cheerfully putting up with me and making me delicious mashed potatoes that won't be rough on my throat. And she got me yet another adorbs Bronchoscopy Animal for the collection, which always makes everything better.

Everything went well, and the surgeon said this time my airways looked great! He said they looked almost as good as after he finished my last bronchoscopy, they barely needed any dilation, and there was NO necrotic tissue! This means my lungs are finally healing up nicely and I shouldn't need another bronchoscopy for a few months. I probably won't make it the full 6 months until my regularly scheduled 1 year bronchoscopy, but anything more than a month between bronchs will be a welcome break!

The other good news this week is that I've been in significantly less pain. We stopped my anti-fungal medicine last week since I'm now 6 months post-transplant with no signs of fungal infection. My doctor said it was possible that medication was contributing to my joint pain, though he thought primary culprit was probably Prednisone withdrawal. A few days after stopping the anti-fungal, however, I suddenly had far less pain and much more range of motion! The pain isn't completely gone, but it's MUCH better and I feel much more functional. I did have a brief run of good days once before, so I'm a little anxious that it won't last, but I'm hopeful that maybe the anti-fungal was responsible for the bulk of my pain and that I'll do much better now that I'm off it. Fingers crossed!

So it's been a week of good news, despite bronchoscopies not exactly being a good time. Hopefully it'll be the start of an ongoing upwards trend!!

Being human

Sometimes, things are hard.

Of course, things aren’t nearly as hard as they were a few months ago. Which then makes me feel kind of guilty any time I feel down or cranky or anything less than grateful. I feel like I should be on a permanent high just from being alive, and (mostly) able to walk, and (also mostly) able to breathe. Life is SO much better and I am SO much more comfortable now, so why should I ever be anything less than happy?

But apparently, no matter how I may try to deny it, I am only human after all. And even aside from complications, post-transplant life involves some major mental and emotional re-adjustments. There are the emotions around knowing that my life was saved due to someone else’s death, and that while we are celebrating, somewhere out there another family is grieving. Additionally, for decades I didn’t expect to have much of a future as an adult, and at 40 I’ve actually survived far longer than anyone anticipated. In the months and weeks leading up to transplant I was so, SO sick that I basically prepared myself to die. The future post-lung transplant is far from certain, and I still could die – but, I also could live for 10 or 20 years or more. I now have to make the sudden mental adjustment to potentially having a life ahead of me, a life I never planned for or expected to see. You’d think that would be wonderful and exciting, but it’s also kind of terrifying! I was familiar with CF life and mostly knew what to expect and how to deal with it. Now I have to figure out what post-transplant life looks like, what I can and can’t do, how to take care of and protect myself while also doing things that make this all worthwhile. A tall order for someone who didn’t expect to be here!

And then there’s the recovery process and complications. I still have some pain in my incisions and a weird band of numbness across my chest. I have multiple appointments every week, including appointments with various specialists in Philadelphia a few times a month. I’m still figuring out how to manage my new diabetic life. The joint and muscle pain make everything difficult and wear me out. I haven’t been sleeping well, at least partially due to pain, and spend a lot of my time feeling fatigued. My airways keep narrowing and being obstructed with necrotic tissue, which means repeated bronchoscopies (not fun), plus I’m wheezing and rattling for at least a few weeks between bronchoscopies (also not fun). It feels like I have a lot on my plate right now, and it’s unclear when the load will lighten, which then makes me anxious. I’m very good at what if-ing: What if the pain doesn’t improve? What if my airways keep acting up? What if I’m not able to get back to a regular exercise routine? What if I don’t get my energy back? What if I do live a long time but everything just keeps being varying degrees of difficult forever? What if what if what if!

I have to remember that I’m still early in this process, and that it IS a process, and that it takes time. I want everything NOW, but that’s not how this works. Just because things are hard now doesn’t mean they’ll be hard forever, hopefully. Hopefully 3 months or 6 months or a year from now I’ll look back and laugh at my anxieties, because the complications will have settled and I’ll be comfortable with my new medical routine. It’s hard to be patient when I’m quite literally in pain, but I just need to get through one day, one week, one month after the next, and give things time to improve. Some things have already improved in the 4 months that have passed, and I need to remember that my body is still healing and recovering from a major trauma. I can’t know how much better things will get, but I need to be confident that things WILL get better.

And I need to remember that sometimes, I will have bad days, and that’s ok too. Emotional recovery is just as much a part of this process as the physical recovery. I’m only human, and that’s ok.

Listed!

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

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

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

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

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

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

Keeping on keeping on

Well, things haven’t exactly been going well around here. But then, I didn’t expect things to go well. At least I’m not disappointed.

After 3 weeks in the hospital, I’ve made no improvement. My lungs are still horribly congested, I’ve needed more supplemental oxygen (currently up to 5 liters at rest), my heart rate is sky high, and my endurance is terrible. Honestly, I feel like I may have even gotten slightly worse. If I did a pulmonary function test now, I have a sneaking suspicion that my numbers might actually be lower. Sigh.

My pseudomonas infection is being extremely stubborn and resisting all the antibiotics. The only ones left are ones that have risky side effects, such as hearing loss or kidney damage, or experimental treatments. I’ve already been on one antibiotic called Amikacin, which is related to Tobramycin and can cause hearing loss. Unlike Tobramycin, however, thus far Amikacin has not made my tinnitus worse, so we stuck with it – until this week, when a test showed that my hearing has deteriorated since the last time I was tested a year ago. Since the IV Amikacin wasn’t having a huge impact anyway, we switched to nebulized Amikacin to preserve my hearing. Most of my hearing is still at the low/borderline end of normal, but apparently I do really poorly with high frequencies. Whatever, who needs high frequencies? I’m not a dog!

I’m also on IV Colistin, which can cause kidney damage. We’re monitoring things very closely, and so far so good. Unfortunately, it doesn’t seem to be doing much in terms of actually making me feel any better. They’re now in the process of getting access to an experimental antibiotic which I may or may not be resistant to. We already determined that I’m resistant to the other experimental antibiotics – which actually impressed me, my super pseudomonas is resistant to drugs that AREN’T EVEN OUT YET – but for some reason this last one won’t let you test for resistance until you get approval to take it. So we’re going to start the drug and THEN test it, which seems a little backwards, but whatever. I’ve definitely seen weirder and more backwards things in the medical field.

They’re also getting things set up for me to start treatment for the NTM (non-tuberculosis mycobacterium). They don’t really think that’s the cause of my problems, but we’ve treated everything else so that’s the only thing left. Fortunately, they seem to have decided that nebulized Amikacin is adequate and I will not have to use IV Amikacin or Tobramycin. They’re still working on getting access to one of the other antibiotics I’ll need, but hopefully that should be settled this week.

On Monday I finished the last of my pre-transplant testing. Some of the tests are to determine how sick and in need of a transplant you are, but most of them are to make sure you’re healthy and strong enough to actually survive a transplant. Thus far I seem to have passed without a problem, so we’re all expecting the transplant committee to approve me when they present my case again on Thursday. It’s also likely that I’ll be listed immediately since I haven’t shown any improvement thus far.

I’ve been spending a lot of time time sleeping lately. Aside from normal hospital exhaustion, I also have pneumonia exhaustion, and just breathing takes a lot out of me these days. I’m also hacking up alarming quantities of mucus, which is very draining. I’ve spent a fair amount of time being extremely cranky, as Katherine and my mom can attest to. It’s hard to maintain a positive outlook when you feel so awful so much of the time. My nurses have definitely noticed that I’m not my usual chipper self, and that my health has deteriorated pretty dramatically just in the last few months. But they also say that I have a good attitude for going into transplant, and they’re hopeful that I’ll do great and feel so much better afterwards.

We’re hoping that I MIGHT be able to go home next week – not because I’ve recovered, but because there won’t be anything else they can do for me here. Once they get me set up with the last antibiotics to treat the pseudomonas and the mycobacterium, there isn’t really anything they can do here that I can’t do at home. It’s definitely better for me to be at home if possible, where I’ll be away from all the hospital germs, moving around more, and eating what I want when I want it. Activity and nutrition are VERY important pre-transplant, as the stronger you are going in, the more likely you are to recover well. I’m not thrilled that I came in with pneumonia and will likely be leaving with pneumonia, but if pneumonia is gonna be my way of life these days, I’d much rather have pneumonia at home!

Throwing darts and hoping for a hit

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

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

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

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

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

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

Keeping on keeping on

Welp, I managed to regain a whole 1% in lung function. Go me.

I am feeling a little better. My oxygen has been a little more stable and my heart rate hasn’t been going quite as high with exertion, though I do still need some supplemental oxygen when I’m out and about. I’ve also been a little less congested. Overall though the gains haven’t been too significant, so much to my disappointment my doctor wants to try one more week of IV antibiotics. Thankfully, however, we’re stopping the Zosyn and switching me back to Zerbaxa, aka the Fancy Restricted Antibiotic. This is so that I can get a LITTLE more sleep, since Zosyn is run every 6 hours, whereas Zerbaxa is run every 8 hours. I’ve been an utterly sleep deprived zombie these last couple weeks, so at this point I’ll take whatever I can get! I feel like a cat these days: I don’t actually sleep anymore, I just take naps of varying lengths at various points throughout the day. I think cats enjoy this more than I do though!

On the plus side, my last sputum culture did NOT show MRSA or either of the two fungal infections that popped up previously. Unfortunately, that hasn’t actually translated into any real improvement in my health. If I don’t see any real improvement with another week of IV antibiotics, we’re going to move on to treating the other infection that has been cropping up lately in my sputum cultures: mycobacterium chelonae, a nontuberculosis mycobacterium (aka NTM). We haven’t treated it until now because it’s unclear whether the NTM is actually the source of any of my symptoms, and treating it requires taking multiple antibiotics for a very extended period of time. Apparently it can also be difficult to get approval for some of the medications involved. And, one of the meds used to treat it is Tobramycin, the IV antibiotic which gave me tinnitus and can cause hearing loss. We’re exploring whether I can take the Tobra via nebulizer, which has less risk of causing hearing issues, but unfortunately in the past the nebulizer formulation of Tobra (called TOBI) gave me asthma attacks. I’ve asked whether we can try giving me the IV version of Tobra to inhale, which is what I did for years without any problems before TOBI was developed. It’s unclear however whether we’ll be able to get approval for an off-brand use of injectable Tobra since a nebulizer version is available.

The other main goal now is getting me to be a little more active. I’ve been feeling sort of awful both physically and mentally, which has led to me spending a LOT of time moping on the couch. I’m trying to pull myself together and leave the house a little more, even if only briefly, and to do brief, mild exercise at home a few times a day. I’m obviously not running any marathons, but whatever exercise I can manage is good for both my physical and mental health. I just need to be careful about not pushing my heart rate too high, as unfortunately the “leaky pipes” have still been an issue. Thankfully I haven’t had a full bleed in two and a half weeks, but I’ve been having varying degrees of bloody mucus at least once a week all month. I’m trying really hard to avoid anything that could trigger a full bleed, as that would force me to hold various treatments and prevent me from clearing mucus out of my lungs. On the other hand, I do need to exercise, because not exercising is also detrimental to my lungs as well as the rest of my body. It’s a really fun catch-22.

It has been a really, really difficult winter. Hopefully things will start looking up soon.

Phase 2

Two weeks ago my lung function was down to 45%. Last week I managed to pull it back up to… 47%. Yay.

So we moved on to Phase 2: IV antibiotics. Unfortunately, my most recent test results showed resistance to even more antibiotics, which partially explains the disaster in November. I’m pretty much down to Amikacin/Gentamicin/Tobramycin (all the same antibiotic class) and Zosyn when it comes to treating my chronic pseudomonas infection. The first 3 all have the potential to cause hearing problems, and we already know that Tobramycin gives me tinnitus (ringing in the ears), but the last time I took Amikacin it didn’t make the tinnitus worse so we’re trying that along with Zosyn. Unfortunately for me, Zosyn has to be infused every 6 hours, and it comes in bags that have to be hung from an IV pole instead of in the clever little self-infusing medi-balls that I usually get. So over the next couple weeks I won’t be getting much sleep and will be spending a lot of time chained to an IV pole. Sigh. Also, I’ve never taken Zosyn before, so here’s hoping it doesn’t turn out to be the next medication that I’m allergic to.

By the way, it only took a full week and doubling my dose of Prednisone, but the Vancomycin rash FINALLY faded. Everyone was quite impressed with its unusual stubbornness. Apparently my body REALLY doesn’t like Vancomycin.

In other news, after trying for the last 3 months, I finally managed to fail a walk test at clinic. This is actually a good thing! The last 2 times my oxygen dropped to juuuust above the insurance cutoff for in-home supplemental oxygen. I’ve been EXTREMELY grateful to the kind gentleman who gave me his old portable oxygen concentrator in December! There have been many times in recent months when I did need supplemental oxygen, but we just couldn’t get the required test results to justify it to the insurance company. Now I’ll be able to get an in-home concentrator that can provide a continuous flow of oxygen, as opposed to the portable concentrator that gives a puff of oxygen every time you breathe in. I tried to use the portable concentrator overnight once, but I couldn’t sleep with it because of the noise and the air puffing into my nose. A continuous flow concentrator will allow me to use oxygen overnight, hopefully giving my body a bit a of break and allowing me to get more restful sleep.

I also asked my team if there was any possibility of getting early access to Vertex’s “triple combo,” the new CF drug that’s on the verge of being released. They’re going to try, but told me not to get my hopes up, as they’ve not yet managed to get insurance approval for other patients in similar situations. And unfortunately, paying out of pocket really isn’t an option, because these drugs are OUTRAGEOUSLY expensive. The previous Vertex CF drug, Symdeko, costs $292,000 per year, and I expect the new drug to cost at least that much. So I’m not expecting anything, but who knows, maybe I’ll be the one in a million that actually gets insurance approval. Here’s hoping!

Hanging in there

Lately it seems like every time we do a sputum culture we find a new infection. I think my lungs put up a flyer at the hottest bacteria hangout. “Vacancies available! Low prices! A comfortable place to raise your spawn!” This month’s pest: MRSA. I’m kind of feeling like a leper these days, since I know MRSA is supposed to be highly contagious, but I’m trying to stay calm since healthy people aren’t supposed to be susceptible to it. It’s unclear whether MRSA is the cause of my recent health struggles, but we aren’t taking any chances, so I started IV Vancomycin on Friday to try to eradicate it.

I’ve never taken Vancomycin before, so they also sent out an “anaphylaxis kit,” which wasn’t alarming at all. Fortunately the first dose went well and the anaphylaxis kit remains unused. Unfortunately, after a couple days I developed a rash all over my torso. It took 40 years, but it seems we finally found a medication that I’m allergic to! Due to this we stopped the Vancomycin and are switching me over to an oral antibiotic. In the meantime, the rash still hasn’t gone away and actually seems to be spreading, despite the fact that my last dose was Sunday night and I’ve been taking Benadryl multiple times a day. So that’s been fun. Of course, I also just realized that my Benadryl expired in September of 2017. Maybe some non-expired Benadryl will work better.

Aside from all this, my lungs decided to bring the drama and bleed Friday night. I thought the leaky pipe issue was under control, but apparently not. Thankfully it was “only” 15 mL, which for me isn’t too bad. I did make an appropriately dramatic scene though by spraying blood all over the white bathroom sink. If it wasn’t Shabbos I totally would have taken a picture of the crime scene for posterity. On a related note: squeamish people should never, ever randomly scroll through the pictures on my phone. You never know what random horrifying medical disasters you might find in there.

I’m also still very hoarse and scratchy as well as super congested. I haven’t been sleeping well because I wake up rattling and coughing multiple times overnight. But I’m a little less tired with the higher dose of Prednisone, and I think my oxygen is doing a little better this week.

I know none of this sounds great, and honestly I’ve been having a pretty cranky week. But despite all this, I did have one really good day, because 2 friends who couldn’t make it to the party made a special trip in from out of state to visit me! Sarah and Anna joined forces to drive in from Cleveland and Pittsburgh for an awesome roommate reunion. And unlike the actual party, we got to spend hours chatting and hanging out together, which was wonderful! I’m so lucky to have such terrific friends!

Also, Sarah wrote a thing about me, so I’m basically famous now. Get in line for autographs everyone!

www.ou.org/life/inspiration/the-40th-birthday-bash-appreciating-each-day

Almost free

B”H I finally stopped bleeding and was able to gradually resume all my treatments without incident. I was scheduled to finish my IV antibiotics on Friday, but we ended up stopping a day early because the ringing in my ears got slightly worse. The tinnitus originally started back in January following a long course of Tobramycin, and gradually improved over several months, but never faded completely. We knew doing another round of Tobramycin was risky, but we also really needed an antibiotic that might make a difference. Fortunately the ringing is still mild and mostly unobtrusive, and hopefully will improve again now that I have stopped the Tobramycin.

The plan now is to finish the last of my IV fluids on Friday and see how I do at my follow up appointment on Wednesday. I had the option of continuing just the IV fluids, but after 2 continuous months of IV infusions I needed a break. Aside from Prednisone interfering with my sleep schedule, I never sleep well while on IV antibiotics since I have to run infusions every 8 hours. So 2 full months of IV antibiotics also means 2 full months of poor sleep, even after my symptoms improved and I got out of the hospital. I am very much looking forward to NOT waking up to any alarms!

I’ve been extremely fatigued this week, but I don’t know if it’s from lack of sleep or if it’s because we’re slowly decreasing the Prednisone and I’m not quite as hopped up on steroids. My appetite has also slipped without the Prednisone munchies, but unfortunately I’m still stuck with uncomfortable Prednisone bloat. If only Prednisone would let me choose my side effects! We’re currently in the process of getting me started on a monthly injection for severe asthma, which we hope will control my asthma-like symptoms enough to allow me to get off the steroids completely for the first time in 2 years. Hopefully soon I can finally say goodbye to Prednisone side effects!

The big question is what will my pulmonary function numbers look like on Wednesday after a few days without IV infusions. Here’s hoping that I managed to regain a few more percentage points, and that a few days of sleep will do me a world of good!

In it for the long haul

Well, that didn’t exactly go as I had hoped.

At the end of October my lung function was at 57%. Just 2 weeks later, despite IV antibiotics, it had unexpectedly plummeted to 45%. After three weeks in the hospital and an additional week of home IV antibiotics, my lung function now is up to… 48%.

I knew I was nowhere near my baseline of 60%, but I thought I had at least made it back into the 50s. It was definitely disappointing to see so little progress after so much treatment. I’m certainly doing much better now than I was 6 weeks ago, but no matter what we try I just can’t seem to get very far. My lack of progress doesn’t make sense, because various tests have shown that my infections SHOULD be susceptible to the 4 IV antibiotics we’ve tried so far. But for some reason, during each of the last 2 rounds I only made a little progress before stalling. We’re going to try 1 more IV antibiotic and see if that finally makes a difference. Unfortunately, that antibiotic is Tobramycin, which caused tinnitus (ringing in the ears) back in January that never fully faded. Hopefully a brief course won’t have auditory side effects, but I will be monitoring things carefully, and we will stop at the first sign of any problems.

If I still don’t have any progress after a week of Tobra, we’re going to take a break from IV antibiotics and give my body some time to heal on its own. We’re also going to do some testing to see if it’s possible that my terrible reflux is contributing to the problem, and if maybe some of this is being caused by aspirating things into my lungs. Unfortunately, the steroid which is helping control the inflammation can also exacerbate reflux, so it might actually be making things worse. I’m in the process of starting a monthly injection used for severe asthma in the hopes that it will enable me to get off of or at least lower the steroid.

On a positive note, I do have a terrific hashgacha pratis (divine providence) story! While hospitalized I met one of my neighbors as we both walked up and down the halls getting our exercise. He doesn’t have CF, but he did have a lung transplant back in March. It turns out that he had multiple oxygen concentrators that he no longer needed, and he and his wife had been looking for a person or agency to donate them to in order to pay it forward. This week they dropped one off at the outpatient clinic, and I am now the proud owner of my very own portable oxygen concentrator! This is GREAT, as my oxygen continues to drop with exertion, but not low enough to qualify for supplemental oxygen to be covered by insurance. These machines cost thousands of dollars and I never would have gotten one on my own. Having supplemental oxygen available will make it easier for me to be active and get back on track with exercise so I can build my strength back up. So, I HAPPENED to be next door to this gentleman, who HAPPENED to have a spare oxygen concentrator, EXACTLY when I could benefit from in-home supplemental oxygen for the first time in my life but had no way to get it. Thank you Hashem!!

This has definitely been a long haul. It’s still unclear how much longer of a haul I can expect, or what the eventual outcome will be. Which is, honestly, frightening, frustrating, and discouraging. But I am still hopeful that things can get better than they are now, whether through treatment or simply with time. And even if they don’t, I am also confident that I will learn to adjust to my new normal and figure out how to live life within it, just like I have in the past. One way or another, I will roll with the punches and come out on top, just like I always do.