Progress and fun plans!

Yay steroids!

After a couple weeks on a lot of Prednisone, I’m feeling much better and my numbers are back to baseline. Since I’ve been on a higher dose for longer than usual, my doctor wants me to taper off very slowly to reduce the chance of reacting negatively. So I’ll still be on Prednisone for a few more weeks, and hopefully will continue maintaining my lung function once it’s stopped. Prednisone is notorious for miserable side effects, but it just seems to make me normal: Instead of being hyper, I’m just less fatigued. Instead of being ravenous, I just have an almost normal appetite for a change. I think Prednisone kinda works for me! Too bad long term steroid use isn’t actually good for you.

They finally removed the PICC line, which was a big relief! We discussed possibly getting a port placed, but first I need to have a consult with hematology to make sure they think it’s safe, given my clot history. I also still need to find a pulmonary rehab, which admittedly I haven’t put much effort into. Though I am GREATLY appreciating the fact that I can mostly walk and manage day to day activities again, I’m still slow and easily winded, and can probably be doing at least somewhat better. Hopefully pulmonary rehab can help me bump things up to the next level.

In the spirit of feeling better, I suddenly decided on Sunday that I want to see the full solar eclipse. Fortunately I come by my crazy honestly, and with minimal effort my mom was persuaded to join me. We somehow managed to score a hotel not too far from the zone of totality, so on Sunday my mom and I will hop in the car and drive to Tennessee for a hastily planned road trip. We can’t remember the last time we took a vacation together, so it should be a fun adventure! Wish us luck!

Fail

Well that was a fail.

Right after my last update, I started running a low grade fever. My cough also increased and I became more short of breath. By the time I saw the doctor last week, my pulmonary function numbers were basically back to pre-hospital levels. I wasn’t surprised, based on how I was feeling, but it was still disheartening. I also saw a physical therapist and tried a brief stair stepping exercise, which I couldn’t even finish and found completely exhausting. Apparently that was surprising and I shouldn’t be this short of breath, even with where my pulmonary function numbers are at. Clearly IV antibiotics didn’t accomplish much, so my doctor thinks it’s less about infection and more about inflammation and air trapping. Air trapping happens when inflammation and/or mucus starts blocking off small airways and air can’t be easily or completely exhaled. The leftover air keeps the lungs inflated and makes it harder to inhale, causing shortness of breath. Air trapping is also the kind of thing that can start to snowball once it starts and be difficult to get back under control.

The problem is there’s no quick and easy treatment for air trapping. Additionally, the last few times I’ve tried steroids for inflammation we didn’t see a significant response. This time we’re trying a stronger, longer dose of steroids, and looking into pulmonary rehab. Pulmonary rehab, like most forms of rehab, isn’t exactly a good time and requires a lot of effort. I’m not looking forward to it, but then again I don’t really enjoy not breathing, so I guess it’s the better option. I’m still trying to find a local (aka not Philly) pulmonary rehab program, so if anyone is familiar with any please let me know!

We also decided that the antifungal treatment wasn’t making any difference in my symptoms. It was definitely effective in terms of eradicating the aspergillus, but clearly that didn’t actually impact my lung functioning. So we gave up on that and stopped the antifungal medication.

I still have the PICC line, but it’s pretty much at my discretion at this point. I did ask to increase the IV fluids for a bit to help keep things loose in my chest, but I’m running out of patience and will probably have them pull the PICC this week. My skin never does well with tape, and I’m at the point where I don’t feel like dealing with rashes and blisters for such little return anymore. At least that will be one less annoyance in my life!

Getting there

As of last week my pulmonary function numbers were back to baseline, though not QUITE at their highest. I wasn’t feeling as good as I usually do after 3 weeks of IV antibiotics, but my doctor didn’t think it was worth doing an additional week, since I did improve and the standard for treatment is 10-21 days. We decided to keep the PICC line in for 2 more weeks though and continue IV hydration as needed. If I maintain lung function after 2 weeks, we’ll pull the PICC line; if I start to slide, we’ll look into putting in a port, which is a surgically implanted, permanent IV. The advantage of a port is we can easily access it whenever I need IV hydration or antibiotics without having to put in a PICC line every time. The disadvantage is that it has a risk of blood clots, just like a PICC line, and everyone is anxious after the blood clot drama in December. Even if I hold on for the next 2 weeks though, I’m pretty sure I’ll be getting a port sometime soon. It’s just not feasible to keep putting in a PICC line every few months, and the port will be far more convenient.

I’m still very tired, though my endurance seems to be slowly improving. My new thing is I’ve been getting a lot of headaches recently, which isn’t fun. I don’t know if it’s because I haven’t gotten allergy shots while sick and seasonal allergies are creeping back up on me, or if my sinuses are acting up again and it’s time for another round of sinus surgery. I’ll be resuming allergy shots soon, and if that doesn’t help I’ll follow up with my ENT.

Despite all this, I’ve been fortunate to have a bunch of visitors, and have been able to get out occasionally for some low-key fun. Hopefully I will continue feeling better and will manage to be a semi-normal person for at least part of the summer. My recovery has been slower than usual, but I AM recovering, and hopefully I’ll be able to stay away from respiratory infections and hold it together for more than a few weeks this time around. Fingers crossed!

Homeward bound!

I'M GOING HOME!!!!!

Ouch

Last week they somehow managed to mistake me for a pincushion. At one point I actually had 3 separate IVs, which I’m pretty sure is my all-time high, and honestly seemed a bit excessive. A friend looked at my arms and commented that I was rocking some hardcore body modifications. Forget about piercing the skin, I go straight for the veins! Maybe I’ll start a new trend!

As usual, things are moving slowly. But, they ARE moving. My chest is a little less tight and my airways are a little clearer. Lately it’s been taking 3 weeks of IV antibiotics to bring me back to baseline, so that’s what I’m expecting this time around as well. However, I will NOT be hospitalized that entire time. My goal is to get out and set up on home infusion before Shabbos. Fingers crossed!!

The Pole People return!

The latest batch of Pole People!

One foot in front of the other

At a doctor’s appointment last month, we agreed that I was kind of hovering on the edge. Not terrible, not great… I could go back on IVs, or continue with the prednisone and oral antibiotics and come back in a month. I chose the latter, and by the end of May I was actually feeling significantly better. I was breathing and walking more easily, and even got out to enjoy a couple day trips without the wheelchair. I stopped the prednisone last month, and the oral antibiotics a week into June, thinking I had actually managed to avoid an exacerbation this time.

Last week, the other shoe dropped. After being off antibiotics for a week, my endurance started slipping again. I quickly resumed taking them, but a couple days later I suddenly started running a fever. By the end of the weekend the fever had settled, but my endurance was shot and I was dealing with miserable coughing fits and loads of upper respiratory irritation. Of course, I can’t just follow standard illness models like a NORMAL person. If I was a normal CF patient, I’d be horribly congested – but I’m not. If I was a normal asthmatic, I’d be wheezing – but I’m not. I’m just in this bizarre no-man’s-land of shortness of breath and upper respiratory irritation and dropping pulmonary function numbers. I wouldn’t want my doctors to get BORED or anything! The oral antibiotics and prednisone clearly aren’t cutting it, so we’re busting out the big guns, and I’m heading back to the hospital for IV antibiotics and hydration.

Honestly, I was not my usual Zen, happy-go-lucky self last night. Since December I’ve been going in the hospital and on IVs every 3 months, and not actually feeling that stellar during a lot of the time between admissions. It’s draining and, frankly, frightening. I’ve known since I was 15 that CF is a progressive illness, and the fact that I’ve made it this far is truly miraculous. I am so thankful for all I’ve managed to accomplish in 38 years despite – or possibly because of? – this disease. I am so very grateful that even though I’ve become disabled and had my life turned upside down, I’m somehow coping with it more calmly and gracefully than I ever thought was possible. But still, putting one foot in front of the other, day after day, while feeling awful is exhausting, and watching this snowball pick up speed is scary.

The one person I desperately wanted to talk to last night was… Mikey. How did you do it, I wanted to ask him? How did you get through this for so many years with a laugh and a smile and a Day By Glorious Day? What is your secret?? Tragically, CF took Mikey away from us a long time ago, and I can only learn from his example without hearing his advice. But, I guess, maybe, I’m already doing it. I keep putting one foot in front of the other and not giving up. I keep squeezing as much life as I can get out of the healthy moments, and even some of the not-so-healthy moments. It may feel like my world keeps getting smaller, but I’m still managing to live within it. And, most of the time, I’m still laughing.

Sometimes, it doesn’t feel like enough. But, hopefully, it will be.