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!

Adulting

The joy of trying to catch up on 4 months of bills (mostly medical, and I haven't even gotten the transplant bill yet) and mail. Apparently the world doesn't stop just because you almost die. It's kind of amusing watching the billing letters get more strident as the months go by. Gee, so sorry my near death experience was inconvenient for your bottom line!

#adulting 

Back to the hospital

I’m convinced my lungs looked at a calendar and are trying to make sure they hit every Jewish holiday.

Last week I did feel the tiniest bit better, even though it wasn’t anything to write home about. Sunday, however, I inexplicably started feeling worse. I hoped it was just a weird random bad day, but since then my oxygen has been dropping, I’ve been incredibly short of breath, and I’ve gotten significantly more congested. I was supposed to have a follow up appointment next week, but I emailed my doctor and they told me to come in today instead. They also mentioned that I might need to be hospitalized.

I felt pretty defeated yesterday. Hospital was NOT the word I wanted to hear, particularly since my last hospitalization was so long and brought so little improvement. It’s extremely frustrating to once again end up feeling WORSE while on IV antibiotics instead of getting better. It’s also scary, because we keep trying different treatments, and keep not seeing any significant improvement. It’s really hard to keep fighting when you never seem to get a win.

My lung function numbers ended up being even worse than I expected. Somehow over the course of a week, while on IV antibiotics, I managed to drop from 48% to 42%. At that point I knew there was no way out of being hospitalized, but I did ask my doctor what they can accomplish in the hospital that we haven’t already tried at home. She pointed out that in the hospital they have full access to all kinds of testing and other diagnostic procedures to help figure out what’s going on, and that it’s much easier for them to make quick medication adjustments and try different treatments. They tried to admit me directly from clinic, but there were no beds available. The only other way to get admitted was through the ER, but thankfully they had mercy on me and told me to go home and enjoy Purim instead. They’ll try to direct admit me again tomorrow, and if they’re still unsuccessful I’ll start yet another ER adventure after I finish my Purim seuda.

They really don’t know why my health has been so poor and why I’ve been so unresponsive to treatment. It feels like we keep throwing darts hoping to hit a target, except I think we might also be throwing the darts while blindfolded. We tried an antifungal and got rid of the fungal infections, we tried an antibiotic and got rid of the MRSA, we tried IV antibiotics to treat the pseudomonas… and yet, I’m still getting worse. So now we’re trying to figure out what is it that we’re NOT treating that could be causing these problems. There are currently 2 top contenders. One possibility is a pulmonary embolism, a blood clot in the lungs. We actually tried to schedule a CT scan today to rule that out, but my lovely insurance is dragging their heels in approving it, so I guess they’ll just have to pay for it in the hospital instead. The other possibility is aspiration pneumonia. Even with medication I have terrible reflux, and they’re concerned that I might be inhaling reflux in my sleep since I use a feeding tube overnight for extra nutrition and hydration. We’ve explored surgery to combat the reflux, but my health hasn’t been stable enough to move forward with it. However, one thing we can do is change my G-tube to a G/J-tube. A G-tube pumps the supplement directly into my stomach, whereas a J-tube would bypass the stomach and pump it into my intestines. This could reduce overnight reflux issues, and apparently is a very easy change to make. So that will probably happen while I’m inpatient.

I didn’t have the energy or brain power to pull together a Purim costume, but fortunately I did have an unused super fun hat stashed away in my closet. And when I pulled it out I discovered that it also came with a tail, so that was an exciting bonus! I made it to shul for megillah reading tonight, and tomorrow I plan to go to shul again before joining Martelle and her family for an early seuda. At least I have the chance to get my Purim on before embarking on the next round of medical drama!

After megillah reading, Sarah Bienenfeld reminded me that Purim is the time of v’nahafoch hu, of things turning around, and of sudden unexpected salvation. Hopefully this hospitalization will be fueled by the power of Purim and I’ll see my own v’nahafoch hu soon!

Drama free and moving on

You know how some places have those signs, “This workplace has been incident free for x days?” Katherine and I decided that I should have one for bleeding. Current count: 17 days!

Since I’ve managed to stop bleeding and my lungs finally sound clearer, I’m finishing up one last day of IV antibiotics and fluids and then calling it quits for this round. After being on IVs for a full month, I am very much looking forward to freedom and a full night’s sleep! Next task: Getting back to pulmonary rehab and rebuilding my stamina. I’ve been trying to keep somewhat active since the bleeding settled, so hopefully I haven’t deconditioned too much. I’m also hopeful that maybe I’ll manage another few months of relatively good health like I did after the last round of IV antibiotics. Fingers crossed!

One less exciting bit of news is that I’m starting to show side effects from long-term steroid use. A scan showed that my bones are thinning, not quite to the point of osteoporosis, but definitely on the borderline. It’s not a huge deal, but it’s something to be aware of. That should become less of an issue as we slowly taper the Prednisone down, and hopefully I’ll be able to get down to a maintenance dose that’s low enough to have minimal long-term impact. In the meantime, I’ll be starting medication to help rebuild bone density and hopefully prevent osteoporosis.

On the other hand, I also got some unexpectedly good news! Apparently by the end of May I reached the out-of-pocket maximum on my medical benefit, which means medical is now covered at 100% and I no longer have any medical copays (though I still have prescription copays, as the pharmacy benefit has no out of pocket maximum). I was surprised, as I have spent less than half of the $6,700 I was anticipating. It turns out that some of my more expensive specialty medications are covered under the medical benefit instead of the pharmacy benefit, so those copays made a significant dent in my out-of-pocket maximum. Even better, I have a grant that covered those copays, so I didn’t even have to pay them myself! Having my out-of-pocket maximum essentially halved was a HUGE relief and will make the rest of the year MUCH easier. Additionally, I got two other prescription copay assistance programs set up, and my pharmacy expenses finally hit the catastrophic coverage phase, which means that my prescription copays have dropped significantly. Now that I have everything set up, next year should be much smoother financially!

It’s been a difficult summer, but B”H things are finally looking up. Looking forward to getting back on track and hopefully squeezing in a few good months before the next round!