They see me rollin'

I’m still dealing with what I’m now thinking of as Leaky Pipe Syndrome. Thankfully I haven’t had another full bleed, but I keep randomly streaking. Yesterday I finally managed 48 hours blood-free and resumed one of my treatments, only to streak at the end of the day and be forced to start all over again. Previously we wouldn’t react so strongly to just a little streaking, but my recent history of Big Dramatic Bleeds makes everyone anxious so we’re being very cautious. I have to keep reminding myself to Be Very Chill and not do anything to elevate my heart rate, which these days basically means not doing anything at all exerting. I am not enjoying being so limited and feeling this fragile.

Today I got my first experience using a motorized cart at Shoprite. I really wanted to actually go food shopping instead of trying to figure out what I needed online, but I also knew it definitely wasn’t safe to do that much walking. So I swallowed my pride and grabbed a motorized cart. I did feel awkward about looking perfectly healthy while puttering around on a scooter though, so I kept my oxygen on the whole time even though I didn’t really need it once I was sitting. I figured no one would question the girl with the nasal cannula. I managed to only run over Katherine’s foot once, and though I ALMOST knocked over a couple displays, nothing ACTUALLY ended up falling. Using the scooter ended up being a weird combination of fun, annoying, and occasionally slightly terrifying. Those things are harder to drive than you’d think!

Katherine has been amazing through all of this craziness. Since I’m mostly incapacitated, almost all the household tasks have fallen to her. I can put together a shopping list and zoom around on my scooter picking things out, but she’s the one shlepping it all home and putting everything away. Not to mention cleaning, and laundry, and just about all the things that keep a household going. I feel terrible about not being able to contribute much, but she does it all without complaint and reassures me that she’s happy to take care of things. I’m not sure how I got so lucky!

Of course, she did get entertainment value watching me almost take out an entire cereal display with my scooter, so maybe it’s a fair trade. Here’s the photographic evidence she took of my Shoprite experience. Maybe next time she’ll get video!

Not again...

I guess I should’ve gotten an update out yesterday before anything had a chance to go wrong…

I felt noticeably better over the course of the weekend. My heart rate didn’t shoot as high with mild exertion, I was able to maintain oxygen without using the concentrator, and I had a little more energy. At my appointment on Wednesday my pulmonary function was up to 52%, which made me very happy. Overall we were pleased with how things were going and I seemed to finally be on a stable, upwards trend.

But apparently this rollercoaster isn’t over QUITE yet. This morning, after 2 full weeks with not the slightest hint of blood in my mucus, I suddenly coughed up 15 mL of pure blood out of nowhere. I literally yelled “NO!!!” as soon as it happened, because I was so frustrated to once again take a step backwards right when things were looking up. Fortunately it was less than the bleed from 2 weeks ago and wasn’t close to ER level, but I still can’t help feeling that my lungs are like dilapidated, rusty pipes right now, just springing leaks all over the place without warning. So once again I’m holding various treatments until I’m completely blood free for 48 hours. Unfortunately this time I’m not on any home infusion, so I don’t have that extra support to help make up for the missing treatments. Hopefully the bleeding will stop soon and I won’t lose too much ground.

I also got to do a super fun barium swallow yesterday to see whether I’m aspirating things into my lungs when swallowing. Fortunately it turns out that I’m swallowing fine, but I’m definitely having significant reflux (which we already knew), and there’s concern that I may be messing up my lungs by aspirating that. I’m already on a bunch of reflux meds, which control the acid level but can’t stop the reflux from coming up. We started exploring surgical options a few months ago, but put that on the backburner while dealing with respiratory issues. Once we get things stabilized the plan is to continue testing and consultations to move towards surgery and see if that helps with some of the respiratory issues.

On the plus side, before things went south, Katherine and I took advantage of the good weather and my relatively decent health to go to the Mummers Parade! I’ve been watching this Philadelphia New Year’s Day tradition on TV since I was a kid, but never actually made it there in person. We shlepped the wheelchair on the train and joined the crowds at Broad Street and Sansom to watch the string bands perform, and had a great time! And one of the mummers even gave me his parasol, which was super cool!! Apparently looking pathetic in a wheelchair has its advantages!

I’m definitely frustrated about backsliding once again, but I’m glad it at least waited until after I squeezed in a great experience! Hopefully that was the last hurrah for this CF exacerbation, and I’ll get back on a steady upwards trend soon!

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!

I've been nerfed!

In the gaming world, “nerf” is a slang term for when game developers make changes to reduce the power or abilities of a (usually overpowered) character or item. Between my affinity for gaming and the many times I’ve imagined what I could accomplish in the world if I only had my full strength, this meme seemed made for me!

One more week

As I predicted, we’re keeping me on IV antibiotics for another week. I was surprised, however, to hear that my doctor originally expected to send me back into the hospital at yesterday’s appointment. Fortunately, I looked better than she anticipated, and my chest didn’t sound any worse (though it also didn’t sound any better). She would have stopped the IV antibiotics yesterday, but I could use the extra support while recovering from the recent bleed, particularly since I’m still holding chest PT and half my nebulizer treatments. If I can just stay blood free for 48 hours I can gradually resume those treatments and get back on track with clearing the congestion out of my chest.

The good news is that there is FINALLY an end in sight. As long as nothing stupid happens within the next week, we will finally stop the IV antibiotics next Friday. Depending how I’m feeling we might continue the IV hydration, but we might also just give me a break from everything, since by then I will have been on IVs for more than 8 weeks. The nice thing about the port is that giving me a break is a viable option since it’s so easy to just re-access it and resume IVs if necessary. Though I wish I had seen a stronger response to treatment, I am very much looking forward to moving on from endless IVs and trying to put my life back together.

Yesterday, for the first time, I went into a store with my new oxygen concentrator. It was an interesting and conflicted experience. I definitely appreciated the fact that the oxygen made it easier for me to move around without huffing and puffing. But I also had conflicting thoughts and feelings about making my until now mostly invisible illness suddenly and inescapably visible. On the one hand I appreciated and on some level even enjoyed the validation of having something external to match the internal struggle. There have been many, many times over the years when various every day tasks were extremely difficult for me, and yet you would never know it from the outside. It can be very frustrating to push through your day feeling like a train wreck while looking like the picture of health. On the other hand, it felt very odd to walk through the store with a blatant sign of severe illness strapped to my face, knowing that everyone who saw me instantly knew that something was very, very wrong. I found myself wondering what people saw when they looked at me, this baby-faced girl afflicted with some terrible mysterious illness, a tragic image to tug at your heartstrings. I wasn’t entirely comfortable with strangers being able to immediately categorize me as the sick girl without knowing anything else about me. But that won’t stop me from living my life and using the medical supports I need to do so. It was an interesting experience, and I’m sure I’ll get used to it with time.

Hopefully my lungs will behave themselves and stop. freaking. bleeding. so I can get back on track with treatments and exercise and continue clawing my way back to health!

Frustration

Things seem determined not to go smoothly this time around.

During the last few weeks I’ve been sporadically coughing up small amounts of blood. This week the bleeds were a bit more frequent and slightly more severe, which is concerning as previously that pattern led up to a big bleed that landed me in the hospital. The normal response to bleeding is to hold some of the treatments that can be irritating to my airways, but we’ve been hesitant to do that as I’m still very congested and really need those treatments to help pull me out of this exacerbation. It’s a catch-22: the treatments could provoke a bleed, which is bad for my lungs, but holding treatments increases congestion, which is also bad for my lungs. Increased congestion can also lead to increased infection, which can provoke bleeding. There’s no good answer here, and I’ve pretty much been walking a tightrope, hoping not to fall off.

Last night things finally gave way, and I coughed up 25 mL of pure blood. Thankfully that’s not ER level, but it’s significant, and left me no choice but to hold treatments. Currently I’m under orders to hold treatments until my mucus has been COMPLETELY blood free for 48 hours. The 48 hour countdown hasn’t even begun yet, as my mucus remains blood streaked, though thankfully far less so now than this morning. So I’m already 1 day into holding treatments, and have at least 2 more days to go, if not more. Each day without these treatments means I’m less able to clear the congestion out of my lungs, and makes it more likely that I’ll lose the little ground I’ve managed to gain over the last several weeks of IV antibiotics. I also can’t exercise or do anything to elevate my heart rate, as the extra pressure on my weakened blood vessels could trigger a bleed, so any efforts to regain my strength and get back to normal life have also been put on hold.

Honestly, I am extremely frustrated right now. I have now been on IV antibiotics for 7 weeks straight, and will probably be going into week 8. I have a follow up appointment tomorrow, but we can’t even do pulmonary function testing because the effort involved could provoke a bleed. I’m pretty sure they’re going to keep me on antibiotics for at least another week while we get the bleeding under control, so that will be 2 full months of treatment. It was already upsetting that with all this treatment I’ve made such limited progress. To now be forced to stop the treatments and exercises that could help me move forward and watch at least some of that progress be unraveled is just infuriating. On the other hand, I’m also terrified of having another massive bleed like I did in February and July, and I REALLY don’t want to go back in the hospital. I’m caught between a rock and a hard place with no way to win here.

I’m tired. Hopefully things will get better soon.

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.