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.

Hanging in there

Lately it seems like every time we do a sputum culture we find a new infection. I think my lungs put up a flyer at the hottest bacteria hangout. “Vacancies available! Low prices! A comfortable place to raise your spawn!” This month’s pest: MRSA. I’m kind of feeling like a leper these days, since I know MRSA is supposed to be highly contagious, but I’m trying to stay calm since healthy people aren’t supposed to be susceptible to it. It’s unclear whether MRSA is the cause of my recent health struggles, but we aren’t taking any chances, so I started IV Vancomycin on Friday to try to eradicate it.

I’ve never taken Vancomycin before, so they also sent out an “anaphylaxis kit,” which wasn’t alarming at all. Fortunately the first dose went well and the anaphylaxis kit remains unused. Unfortunately, after a couple days I developed a rash all over my torso. It took 40 years, but it seems we finally found a medication that I’m allergic to! Due to this we stopped the Vancomycin and are switching me over to an oral antibiotic. In the meantime, the rash still hasn’t gone away and actually seems to be spreading, despite the fact that my last dose was Sunday night and I’ve been taking Benadryl multiple times a day. So that’s been fun. Of course, I also just realized that my Benadryl expired in September of 2017. Maybe some non-expired Benadryl will work better.

Aside from all this, my lungs decided to bring the drama and bleed Friday night. I thought the leaky pipe issue was under control, but apparently not. Thankfully it was “only” 15 mL, which for me isn’t too bad. I did make an appropriately dramatic scene though by spraying blood all over the white bathroom sink. If it wasn’t Shabbos I totally would have taken a picture of the crime scene for posterity. On a related note: squeamish people should never, ever randomly scroll through the pictures on my phone. You never know what random horrifying medical disasters you might find in there.

I’m also still very hoarse and scratchy as well as super congested. I haven’t been sleeping well because I wake up rattling and coughing multiple times overnight. But I’m a little less tired with the higher dose of Prednisone, and I think my oxygen is doing a little better this week.

I know none of this sounds great, and honestly I’ve been having a pretty cranky week. But despite all this, I did have one really good day, because 2 friends who couldn’t make it to the party made a special trip in from out of state to visit me! Sarah and Anna joined forces to drive in from Cleveland and Pittsburgh for an awesome roommate reunion. And unlike the actual party, we got to spend hours chatting and hanging out together, which was wonderful! I’m so lucky to have such terrific friends!

Also, Sarah wrote a thing about me, so I’m basically famous now. Get in line for autographs everyone!

www.ou.org/life/inspiration/the-40th-birthday-bash-appreciating-each-day

Happy birthday to me!

Turning 40 is a big milestone for anyone, but when you have Cystic Fibrosis it’s nothing short of a miracle. When I was a kid they didn’t think I’d live to graduate high school, much less see my 40^th birthday! I don’t know how I’ve merited to still be standing when so many others have fallen. I am so very grateful for every single year, and will always proudly proclaim my age and celebrate every victorious birthday!

On Sunday I had a massive blowout bash to celebrate this amazing milestone. I didn’t have a specific guest list, I just kind of threw the invitation to the winds for anyone who wanted to share in my joy. And before I knew it, almost 90 people had RSVPed! I was absolutely blown away by the turnout and with how far people traveled just to celebrate with me for a few hours. It was a completely amazing day and I loved every second of it! I only wish I had more time to catch up with everyone there!

There’s a gallery of beautiful professional photos available at www.artofamoment.com/p340748368. I also created a collaborative photo gallery at lifebox.mobi/S3fZncaTX9f1NyI1I6LkQccFKBaGX2ZVirGO30NKvVuDV0OSGKt5W2EfWftHBKzx. If you were at the party, please upload your photos so I can collect as many scenes and memories from that wonderful day as possible!

For anyone who missed it or wants to hear it again, here’s the video of my little pre-cake cutting birthday speech. Apparently people seem to think I’m inspiring or something, so I figured I should maintain my reputation with some appropriate words of wisdom. I think it pretty much sums up the way I try to live my life, and I hope others can gain something from my perspective.

Thank you everyone for making my birthday amazing! I am so grateful that my life is filled with so much love and so many wonderful people!!

Downs and ups

Well, my numbers were terrible. That wasn’t really a surprise though, because I’ve been feeling pretty terrible. My pulmonary function is back down to 45%, though fortunately I don’t feel QUITE as bad as when I hit 45% in November. We’re not entirely sure what’s going on. It’s possible I had a virus a couple weeks ago that my immune system knocked out right away, but which left lingering inflammation that hasn’t faded. It’s possible that we tapered the Prednisone too quickly and didn’t give the new injectable asthma medication enough time to kick in. It’s possible that the various infections in my lungs just aren’t very responsive to antibiotics anymore. Or it could be any combination of these factors.

It’s also possible that there’s a different, untreated infection acting up and causing me problems. Recent sputum cultures have shown 2 new pests hanging out in my lungs: a second fungal infection called fusarium to keep my long-standing off-and-on aspergillus infection company, and another bacterial infection called MAC (myobacterium chelonae specifically, for the medically inclined). This is in addition to my usual pseudomonas and staph infections, of course. I feel like a freaking petri dish these days. I already started an antifungal in November, so hopefully that should address both the fusarium and the aspergillus. Treating MAC, however, is more complicated. Apparently it involves taking multiple antibiotics simultaneously for a long period of time, and they have a lot of potential medication interactions as well as side effects.

For now, we’re upping the Prednisone again to see if that helps bring things back under control. If that doesn’t work, we’ll probably try IV antibiotics. And if that doesn’t work, we’ll try treating the MAC and hope that finally makes a difference.

On a more celebratory note, I have the best CF team in the world! They surprised me by coming in to sing happy birthday and give me a present at my appointment! Unfortunately they won’t be able to come to the big birthday bash, but it was so super sweet of them to celebrate with me at clinic!

I’m not thrilled to increase my steroid dosage again, but I guess it’s better than feeling terrible. I can definitely use the extra energy for my big exciting party tomorrow!!

Ups and downs

The good news is, the “leaky pipe” issue FINALLY seems to be under control. During the last 3 weeks I’ve only had one brief incident of coughing up bloody mucus, and that was a week and a half ago. After on and off bleeding at various levels of severity for a month and a half, I’m almost afraid to talk about finally making progress, so here’s hoping I didn’t just jinx myself into a bloody disaster. Listen lungs, you don’t need to prove anything, just be chill!

I had a couple good weeks where my lung function was on an upward trend and seemed to be stabilizing. Despite the bleeding issues, my endurance was better and I barely needed to use supplemental oxygen. Unfortunately, that all came crashing down 2 weeks ago. After a day of increasing hoarseness, fatigue, and shortness of breath, I suddenly spiked a fever. I was convinced that I had caught a virus, but fortunately the fever only lasted one night and I didn’t develop any other viral symptoms. Despite that, since then I‘ve been extremely fatigued and even mild exertion sends my heart rate up and my oxygen level down. So I’ve been back on the supplemental oxygen train while we try to figure out what’s going on and what we want to do about it.

A few months ago if I was feeling like this they probably would’ve thrown me in the hospital. Now, nobody’s rushing to do anything drastic, since I just finished such a long course of IV antibiotics with so little return. I’m also reluctant to increase my steroid dosage, since I finally got the Prednisone down to 10mg and would REALLY like to get off it entirely due to all the side effects. Today we added in some IV fluids to see if extra hydration will help break up the chest congestion and improve my lung function. I have a follow up appointment on Friday, so we’ll see where things stand and figure out our next steps then.

Despite all this, I have been using most of my limited energy for very exciting party planning! Only half a week left until my big blowout 40^th birthday bash! I can’t believe how many people plan to come, and how many people have donated to the GoFundMe! I am so grateful and can’t wait to celebrate with everyone!!