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!

The path not taken

I had my monthly bronchoscopy last week. Though there was once again narrowing in some of my airways, there was less necrotic tissue and overall things looked better than last time. Hopefully that means things are moving in the right direction and settling down. I haven’t started wheezing yet, so that’s also progress, as previously it only took about a week for the wheezing to start up again. They still want me to come back next month for another bronchoscopy, but I should start needing them less, and eventually not at all, hopefully soon! The doctor said that this happens in 10-15% of lung transplants, because of course I would have an atypical complication. I’m officially statistically special. We all know I like to keep them on their toes!

During Simchas Torah I lived on the edge and braved the crowds at shul. I of course wore a mask and was careful about washing my hands, and spent a good amount of time outside where there was more airflow and therefore less risk of germs. Probably if I wanted to be 100% safe I should’ve stayed home, but what’s the point of getting a life saving transplant if you don’t actually participate in life? I’m basically still making the same calculations that I did pre-transplant to balance protecting my health with living a fulfilling life. Hopefully I calculate correctly, but at the end of the day I’d rather have a short life filled with good memories than a long life of empty boredom.

I’m still struggling with significant joint pain, which has now spread from my hips, shoulders, and a few fingers to almost all of my fingers, my knees, elbows, and one wrist. Pretty much any movement hurts, and I often hurt when I’m sitting still too. Going to sleep is really fun, as laying in bed without distraction is the perfect time to really focus on all my aches and pains. I have an appointment with an orthopedist tomorrow, so hopefully he’ll be able to offer some relief.

In the CF world, the BIG news this week is that the FDA approved Trikafta, a new genetic modulator that had huge results in clinical trials and treats the vast majority of CF mutation combinations. This is the triple combo drug I tried unsuccessfully to get early access to when my lungs were failing. Not gonna lie, I’m definitely feeling some kinda way about the whole thing. I waited YEARS for them to come out with a genetic modulator that I was eligible for. One breakthrough after the next I was disappointed, as my mutation combination was not eligible for any of the new drugs. Then, FINALLY, the one that I could take was being developed – and my lungs promptly went to pieces. From November through March I kept being overcome with rage as my lung function deteriorated right when a promising new drug was on the horizon. By the time we started talking transplant, I moved from enraged to resigned. And now, here I am, approaching 6 months post-transplant, seeing that long awaited drug finally hit the market. Yeah, I’m a little bitter about the whole thing.

But, I’m trying not to waste time on bitterness. For whatever reason, that wasn’t the path I was meant to take. I need to focus on the path that I’m on and getting through this transplant life. The truth is, I’m probably breathing better than I ever would have even if Trikafta worked miracles for me. If we could just get my pain under control I feel like I could conquer the world! Maybe I’m just supposed to enjoy breathing with these lungs for however long I get.

It’s hard not to imagine the what-ifs and could’ve-beens, especially while struggling with physical pain. But I’ll try instead to look at what is and what will be, to keep putting one foot in front if the other, and to get the most out of the life I have.

Just out of reach

So close, yet so far…

https://www.cff.org/News/News-Archive/2019/New-Positive-Results-Announced-for-Triple-Combination-Therapy

We’ve already started the process to try to access the triple combo before it gets FDA approval. The first step is for a doctor to reach out to the drug manufacturer, Vertex, and find out whether they are willing to provide the drug. If they are, the doctor then submits an application to the FDA for Individual Patient Expanded Access, which allows patients with an immediately life threatening or serious condition with no other alternative therapies to access investigational treatments. If the FDA approves it, presumably we then try to convince my insurance company to pay for it.

My doctor has already contacted Vertex. However, there’s a hitch: Vertex is not offering expanded access for the triple combo yet. They are currently studying 2 different triple combo treatments, and will only move forward with one of them based on the study results. They won’t provide expanded access until they choose which drug they’re submitting for FDA approval, which could take a few more months.

So now it’s a waiting game. Here’s hoping they finish up the studies and make a decision 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!

Quicksand

WARNING: This is not a happy-go-lucky Eliana post. Proceed at your own risk.

Sometimes it gets hard to keep fighting this fight. All too often battling CF feels like struggling to climb out of quicksand: a lot of effort, but little progress. I’ve been feeling very quicksanded lately. At this point I’ve been varying degrees of very sick for almost 4 months straight. It’s exhausting and demoralizing.

Aside from soul-sucking fatigue, I think I hate the bleeding the most. It’s probably my most visually dramatic symptom, and it’s always been the thing that really made me feel the weight and severity of CF. But it’s also physically uncomfortable on multiple levels. The only way to get the blood out of my airways is by coughing, which can be very draining. Additionally, blood isn’t exactly a substance that’s supposed to go down your throat, so I’m usually hoarse and scratchy afterwards. And most bleeds don’t stop immediately, so I’ll be coughing up thick bloody mucus for hours or days afterwards, which is physically and mentally exhausting.

I bled again last night, by the way. I know it’s not over yet because I can still taste blood in the back of my throat. Here’s hoping it settles down sooner than later.

The most frustrating part is that we are soooo close to the release of a new drug that has potential to be extremely beneficial to me. Several years ago they started coming out with drugs that targeted specific CF mutations. It’s not a cure, but for the first time they were able to treat the underlying cause instead of just the symptoms. Unfortunately, none of those drugs treated my specific mutation combination. CF is recessive, which means I hit the genetic lottery and got 2 copies of the CF gene. One of my mutations is the most common one, but my second mutation is rare, which means it won’t be getting much money and attention when it comes to researching new drugs. However, they’ve finally developed a treatment for people who only have one copy of the most common mutation, and people have shown tremendous improvement in the trials. It’s supposed to go for FDA approval by the summer and hit the market by next year.

This should be good news, right? It definitely is! Unfortunately, while the drug can stop or significantly slow the damage CF causes, it can’t undo preexisting permanent lung damage. People have regained significant percentages of lung function, but they can only regain so much. So the lower I fall now, the lower I’ll be even if I get the drug and regain some lung function. Over the summer my lung function was 60%. If I had regained 10% of my lung function then, I’d be back up to 70%. I was still working full time at 70%! But lately, I’ve been struggling to even hit 50%. The more ground I lose now, the less benefit I’ll see – if the drug even works for me at all, of course, which is not guaranteed. It’s extremely frustrating that things have been snowballing out of control now when hope for a real treatment is so close.

So, I’m tired. I’m frustrated. And, honestly, I’m scared. I don’t know what’s going on with my lungs right now, or what’s going to happen over the next year. I’m just trying to push through from one day to the next, one handful of quicksand at a time.