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!

Five Feet Apart reaction

I have both a written response as well as a long YouTube reaction video. There’s some overlap between them, but they are not exactly the same. Both responses, however, are FULL of spoilers, so if you haven’t seen the movie and don’t want to be spoiled, steer clear of this post!

Well, I did it. I saw Five Feet Apart.

I went into it unsure to what degree I was going to be a nitpicky pest, and to what degree I was going to be completely traumatized. I was actually expecting to fall more on the traumatized side, since I was pretty traumatized just watching the girl huff and puff around with her oxygen tank in The Fault in Our Stars, and she didn’t even have CF. But I went anyway, because I really wanted to be able to have an informed opinion.

I figured it might be easier and/or more fun to do this with a crew, so Katherine and I met my mom and some friends at the theater and we had our own little watch party. I also knew the chances of encountering another CF patient in the theater was highly likely, thus raising our cross infection risks, which I thought was hilariously ironic considering that cross infection is the basis of the entire movie. So I committed to being a full on freak by not only wearing a mask and using hand sanitizer, but also literally spraying down my chair with Lysol. I then unintentionally upped the freak factor due to my lungs taking an unexpected vacation that day. I was struggling to maintain my oxygen level even while using the oxygen concentrator, so I got to slowly and painfully drag my way up the stairs and collapse gasping into my theater seat. If there is a single person in that theater who did not realize that I have CF, it absolutely is not my fault. I promise I did my very best to look the part.

There were definitely things that they really got right. The various treatments and procedures that we deal with and the intensity of the standard CF regimen was absolutely accurate. Moving into your hospital room and making it home, as well as the feeling of missing out on life experiences due to illness and hospitalizations, were absolutely things I could identify with. Some of the emotional struggles did hit home for me: the way your health struggles impact your loved ones, being afraid to expose the people you love to the misery that you go through, struggling to find a balance between living your life versus trying to stay alive… Those were the moments when it was very sobering to see some of my own thoughts and experiences playing out on screen. And of course, the struggle of being emotionally close with someone who you’re never allowed to be physically close to is a harsh reality of CF. I know the feeling of literally talking to a friend through glass because that was the closest we could get to each other. It’s extremely frustrating to be unable to hang out with the people who understand you the most because you could literally kill each other. I’m grateful that by the time they realized the risks of cross infection and started keeping CF patients away from each other, the internet was becoming a thing and I was able to make some connections that way. But still, nothing will ever be able to replace in-person contact.

Given all that, however, I ended up leaving the movie feeling pretty meh. Part of the problem is that there were a lot of inaccuracies throughout the movie, and they kept destroying my immersion and impeding my connection to the story. For example, one of my pet peeves is when characters in TV and movies run around on supplemental oxygen like it’s nothing. I wish I could get my hands on one of those magical movie oxygen concentrators that instantly turn you into a fully functional human being! But alas, using supplemental oxygen does not suddenly solve all your problems. Needing supplemental oxygen means that your lungs are really struggling to do basic lung tasks, so you’ll still be moving at least a little slower and at times will continue to be varying degrees of out of breath. Like I said, I gasped my way up the stairs and into my seat, and it took several minutes for me to stop feeling like I was suffocating, even WITH supplemental oxygen. It drives me crazy when characters run around with their oxygen without a care in the world, because it is SUCH an easy fix! Just slow down a little, breathe a little heavier, and recognize that your character will have more limited stamina and endurance. I was VERY surprised to see that even this movie fell into the magical oxygen concentrator trap, and it kept pulling me out of the story.

Additionally, for someone who was supposed to be on the verge of lung transplant, Stella did not appear anywhere near sick enough. I was pretty shocked when they got the new lungs call, because until then I wasn’t even sure she was on the transplant list, and if she was I assumed she was way further down. One of the factors for where you fall on the transplant list, in addition to factors like geography (organs can only travel so far) and blood type, is how sick you are. The sickest people go to the top of the list, because their need is more immediate and they are more likely to die without it. By the time you hit the top of the lung transplant list, you are in BAD shape. Whereas Stella actually seemed to be doing better than I am, and I’m not even close to being listed yet. It’s kind of funny to think that all the viewers who are unfamiliar with CF probably think Stella seemed horribly, desperately ill, while I’m sitting here like meh, I’ve seen worse. Welcome to Cystic Fibrosis!

Of course there were silly things like the incredibly luxurious hospital with shockingly lax regulations, but I could mostly overlook that. I wish there had been more trademark CF coughing – I definitely coughed more during the movie than either of the actors – but that’s also a minor nitpick. I spent a great deal of the movie being HIGHLY ANXIOUS about their lack of masks (PLEASE STOP YOU’RE GONNA KILL EACH OTHER MY HEART CAN’T TAKE THIS AAAAAH!), but I can definitely see people playing fast and loose when it comes to contact precautions even if they shouldn’t. Hell, I’ve had my own fair share of flagrantly breaking the rules when I was young and dumb(er). The truth is, I could mostly overlook all the inaccuracies I’ve mentioned so far, and I did for most of the movie. For about the first two thirds of the movie, my general impression was that they really got some things right, that they did ok with a bunch of other stuff, and that the movie wasn’t great but it was passable.

And then things went off the rails.

First there was Poe. I’m not a heartless monster, I cried, it was super sad! But, it was also unrealistic, which once again took me out of the story. It is not typical for CF patients to suddenly drop dead out of nowhere, particularly not from a pneumothorax. Pneumothorax means collapsed lung, and that actually is something that can happen to CF patients out of nowhere. However, it’s typically either one lung or a part of one lung, and it’s not an instant death sentence. People can actually walk around for days not realizing that’s the reason for all their pain and misery before getting treatment. As far as I know, a spontaneous full double lung collapse is EXTREMELY unlikely. But in addition to that, in general Cystic Fibrosis is not a quick killer. Dying from CF is a long, slow, agonizing process for everyone involved. You won’t know exactly when the person is going to die, you may not expect it that very day, but you will very much know that death is a possibility and that it’s coming sooner than later. The more I thought about Poe’s death, the angrier I got, because it took the very real pain and fear of watching our friends die and turned it into cheap Hollywood drama to propel the story while wringing more tears out of the audience. So many of us have watched friends die. I’ve watched friends die. It’s a unique sort of excruciating to watch someone you care about die from the thing that will probably eventually kill you too. It’s impossible to describe the pain and fear of seeing your own future played out before your eyes while losing a friend in the process. But all that agony was overlooked for one cheap moment of sudden, unexpected drama. I’m not okay with that. That’s the one part of the movie that actually ended up offending me.

And then, of course, the ice scene. That was the point at which the drama was just too unnecessarily over the top and pretty much ruined the movie for me. First of all, again, no way patients at that stage of lung disease are walking two miles, up a hill, on a cold snowy night. But from that point on the drama was just non-stop: She’s getting new lungs! She’s ignoring the call, even though up until this moment she was all about new lungs! She’s falling off the bridge! Oh wait, she’s fine – OH WAIT, she’s not, the ice broke and she’s drowning! He can’t get her out! He CAN get her out, but she’s not breathing, and he has to break all the cross infection protocols to give her mouth to mouth! It didn’t work – wait, it DID work! But now HE’S dead! Oh wait, no he isn’t. Quick, rush everyone back and get that lung transplant!!

Too. Damn. Much. Even the teenage girls sitting behind me were groaning. When even teenage girls think something is over the top, you’ve DEFINITELY gone too far.

So that was my breaking point. I could have overlooked and forgiven a lot until then, but once they went full on off the rails rapid fire gratuitous Hollywood drama, I was done. And sadly, that then colored my impression of the rest of the movie, and only made all the previous inaccuracies stand out even more.

For me, the part that makes it all so frustrating is that they were so close. They did get so many things right, and they really did try. It would only have taken a few tweaks to save things. I can see the possibility in there for a great story that just didn’t quite manifest, and I find that even more upsetting than if it had been awful from beginning to end. I’m disappointed precisely BECAUSE they tried so hard and came so close. From what I've heard, everyone involved in the movie did genuinely have a great deal of respect for the CF community and wanted to do us justice. I have no ill will towards any of them! I just wish they had hit the mark more.

I know there are many other CFers who did connect to this movie and feel represented by it, and I’m genuinely happy for them! I don’t begrudge anyone their opinion, and I actually would love to talk to CFers who enjoyed the movie and hear why it worked for them and what they gained from it. It’s also entirely possible that I’m just a cranky curmudgeon who’s too old and cynical for teenage romance dramas, and that the movie was perfect for its target demographic. I hope it does raise the awareness that some people are expecting and brings CF more into mainstream consciousness. If it accomplishes that, I can forgive all its flaws and drama and disappointment.

But I probably won’t be watching it again.

YouTube here I come!

Well, after months of pondering and friendly nagging, I’m finally making my YouTube debut. I have no idea where I’m going with this or how often I’ll post anything, but the first video is officially up. Feel free to share and comment on the video and give me those sweet sweet likes and views!

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.

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!!

Five Feet Apart

For those who don't know, there's a Fault in Our Stars style teen romance drama coming out next Wednesday about CF patients. I plan to go see it and nitpick, or be traumatized, or possibly both, and thought it might be fun to do so with a group. I'll probably go to the AMC Loews in Cherry Hill on a Sunday (since unlike me most people have, like, jobs and responsibilities and stuff). I also plan to wear a mask and spray Lysol on my seat, because ironically for a movie that is based on the risks of cross infection, the chances of multiple CF patients being in the same room and cross infecting each other is pretty darn high. Anyways, anyone wanna join me?

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!