Birthday musings

Today I am 41 years old. 

A year ago I had a huge blowout bash to celebrate my 40th birthday. The party was spectacular, but there was definitely a grim intent behind it. At 40 years old, I knew that I had far exceeded all expectations regarding my life expectancy. I was increasingly aware of this as my health deteriorated in recent years, and particularly when my lung function dropped sharply during the 3-4 months immediately before my birthday. I was keenly aware that I might not have that many years left, and I wanted to celebrate with as many of my family and friends as possible while we still had the chance. It was a very deliberate "come to my birthday, not my funeral" celebration. 

Even with all that, I had no idea just how quickly my fears would be realized. At the time I thought I had at least a few more years left, and I was just taking advantage of the 40th birthday milestone. Little did I know that just a few months later I would be in respiratory failure, on the verge of being ventilated, fighting for my life and waiting desperately for a lung transplant. Things got so bad during that time that I had multiple conversations with my loved ones about dying, trying to prepare them as I had been preparing myself. By the end I was so miserably uncomfortable that I actually wanted to die just so the suffering would be over. I was holding on by my fingernails, surviving one day at a time. I certainly wasn't thinking about my 41st birthday, and if I had it would have been to wonder whether I'd even see it.

Yet somehow, miraculously, here I am, celebrating yet another year of life. It definitely isn’t the life I would have expected a year ago, both for good and for bad. On the one hand, I can BREATHE, to an extent that I had forgotten was possible. I’ve mostly lost my trademark CF cough. I’m putting on weight without even trying, instead of struggling to hang on to every pound. My health is SO much better than I could ever have imagined! On the other hand, I’m still recovering from an incredibly difficult, complex, painful, and invasive surgery. I’m managing both short and long term complications, and may develop additional problems as time goes by. And I must always live with the knowledge that rejection could strike at any time, and that there’s no way to predict if or when that or any number of other complications could take me out.

It’s definitely a mixed bag – but, I’m alive! And as long as I’m alive, there’s always hope for better things and positive outcomes. Hope is a somewhat unfamiliar and, honestly, frightening emotion for me, and I’m still learning how to integrate it into my life. For some reason I have a much easier time anticipating and preparing for the worst. But I’m trying to learn how to infuse more positivity into my life, and to start anticipating the best for a change.

I’m 41 years old, but my lungs are only 20. Against all odds I’m still alive, yet for some reason my dear donor lived only half as long. I unfortunately don’t know anything about my donor, but it’s clear that their life ended before it even really began. In a way, every birthday that I celebrate extends their life just a little bit longer. They are an essential part of every experience I have, every single breath I take. I hope that I can use those breaths and live my life in a way that would make them and their family proud.

I spent my entire adult life expecting to be dead long before now. Suddenly, I’m trying to imagine what it might be like to continue living for another decade or two. That’s a lot of birthdays I never expected to see! However many I get, I hope I can make the most of them, and always remember how fragile and precious every moment of life truly is.

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!!