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.

Being human

Sometimes, things are hard.

Of course, things aren’t nearly as hard as they were a few months ago. Which then makes me feel kind of guilty any time I feel down or cranky or anything less than grateful. I feel like I should be on a permanent high just from being alive, and (mostly) able to walk, and (also mostly) able to breathe. Life is SO much better and I am SO much more comfortable now, so why should I ever be anything less than happy?

But apparently, no matter how I may try to deny it, I am only human after all. And even aside from complications, post-transplant life involves some major mental and emotional re-adjustments. There are the emotions around knowing that my life was saved due to someone else’s death, and that while we are celebrating, somewhere out there another family is grieving. Additionally, for decades I didn’t expect to have much of a future as an adult, and at 40 I’ve actually survived far longer than anyone anticipated. In the months and weeks leading up to transplant I was so, SO sick that I basically prepared myself to die. The future post-lung transplant is far from certain, and I still could die – but, I also could live for 10 or 20 years or more. I now have to make the sudden mental adjustment to potentially having a life ahead of me, a life I never planned for or expected to see. You’d think that would be wonderful and exciting, but it’s also kind of terrifying! I was familiar with CF life and mostly knew what to expect and how to deal with it. Now I have to figure out what post-transplant life looks like, what I can and can’t do, how to take care of and protect myself while also doing things that make this all worthwhile. A tall order for someone who didn’t expect to be here!

And then there’s the recovery process and complications. I still have some pain in my incisions and a weird band of numbness across my chest. I have multiple appointments every week, including appointments with various specialists in Philadelphia a few times a month. I’m still figuring out how to manage my new diabetic life. The joint and muscle pain make everything difficult and wear me out. I haven’t been sleeping well, at least partially due to pain, and spend a lot of my time feeling fatigued. My airways keep narrowing and being obstructed with necrotic tissue, which means repeated bronchoscopies (not fun), plus I’m wheezing and rattling for at least a few weeks between bronchoscopies (also not fun). It feels like I have a lot on my plate right now, and it’s unclear when the load will lighten, which then makes me anxious. I’m very good at what if-ing: What if the pain doesn’t improve? What if my airways keep acting up? What if I’m not able to get back to a regular exercise routine? What if I don’t get my energy back? What if I do live a long time but everything just keeps being varying degrees of difficult forever? What if what if what if!

I have to remember that I’m still early in this process, and that it IS a process, and that it takes time. I want everything NOW, but that’s not how this works. Just because things are hard now doesn’t mean they’ll be hard forever, hopefully. Hopefully 3 months or 6 months or a year from now I’ll look back and laugh at my anxieties, because the complications will have settled and I’ll be comfortable with my new medical routine. It’s hard to be patient when I’m quite literally in pain, but I just need to get through one day, one week, one month after the next, and give things time to improve. Some things have already improved in the 4 months that have passed, and I need to remember that my body is still healing and recovering from a major trauma. I can’t know how much better things will get, but I need to be confident that things WILL get better.

And I need to remember that sometimes, I will have bad days, and that’s ok too. Emotional recovery is just as much a part of this process as the physical recovery. I’m only human, and that’s ok.

The journey

For many, many years, “breathing is overrated” was my favorite snarky catchphrase. But now I’m starting to think that I just might have been mistaken.

It’s so wild being able to breathe! So many simple tasks that were beyond me for quite a while are suddenly, wondrously easy. Things like being able to walk from one room to the next, or prepare food, or do dishes, or take a shower without being completely exhausted afterwards. It’s been months since I was able to do any of those things, and I’m so unbelievably grateful every time I’m able to get up and do something without having to calculate whether I have the oxygen and energy for it. It’s a whole new world, and I’m loving it!

Facebook tends to give a skewed impression of people’s lives, as we’re more likely to post the good instead of the bad. I think mine is a little more real, but at times I too will downplay things or try to make them look less dramatic. My previous health updates were definitely real, but I don’t think they fully encompassed how bad things got right before my transplant.

During the week and a half that I was home between admissions, things were pretty terrible. I just couldn’t get enough oxygen, and spent most of the time either sleeping or writhing and groaning in my recliner. The only time I got up was to use the bathroom, and that was a huge ordeal that sent my oxygen plunging and sapped my strength. I hope none of you ever have the experience of trying to decide whether it’s worth the effort to go to the bathroom, or if you’d rather just hold it a little longer because getting up and trying to walk is going to be so awful.

Things were even worse once I was hospitalized. At that point I couldn’t even manage walking the 5 feet to the bathroom anymore. My stubborn dignity refused to use a bedpan, so instead I had a bedside commode. And even on high flow oxygen, just getting out of bed and onto the commode was enough to drop my oxygen and utterly exhaust me. I pretty much spent most of my time looking like this:

During that time I started having “air hunger,” which meant I felt like I was suffocating even when my oxygen wasn’t too low. Thankfully they were able to treat this with oxycodone to make me more comfortable, but it was still a pretty miserable experience. I also came VERY close to needing to move to the ICU and be put on a ventilator, as my oxygen needs almost surpassed what could be provided on a regular unit via high flow. If the transplant had been even a few days later, I have a feeling I would have ended up in the ICU.

And throughout all of this, of course, I was coughing up huge quantities of thick, dark mucus. For almost 2 months straight I coughed up at least half a specimen cup every day, and some days came close to filling the cup. No matter how many treatments I did or how much I coughed out, there was always more. It was utterly exhausting.

Basically, things were really terrible. My discharge paperwork says that I came to the ER with “respiratory failure.” My lungs had just about given up, and I was – quite literally, and faster than anyone had anticipated – dying.

And then the transplant came through.

The before and after is absolutely night and day. Even while I was still in the ICU post-transplant, even with all the pain, I still felt SO much better! And as the days go by, as I continue recovering and getting stronger, things just keep getting better and better. I marvel at all that I’m now able to do, and can only imagine all the doors that will open for me as time goes on. It is truly miraculous.

Don’t get me wrong, coming out of a double lung transplant was no picnic. I woke up in the ICU with a ventilator down my throat, a nasogastric tube, 2 long, stapled clamshell incisions, 6 drain tubes coming out of my chest, a triple lumen catheter in my jugular, IVs in each arm, and a Foley catheter. I was also hooked up to multiple monitors and had wires going everywhere. Initially my hands were restrained to prevent me from pulling anything out. Once I was alert I wanted to communicate, but couldn’t because of the ventilator. We tried to use a letter board, but unfortunately didn’t realize that my vision was off. When I thought I was pointing at one letter I was actually pointing at something else, much to everyone’s confusion. Which is why that brief post I made post-transplant took 2 hours to write! Things went a little better when we got a pen and paper, and of course once the ventilator was removed I was eventually able to talk again.

Even once I got out of the ICU and onto the transplant unit, I was still very incapacitated. I was very weak and spent a lot of time sleeping. The painkillers often knocked me out or made me loopy, so I wasn’t exactly up for visitors. I had to relearn how to walk, how to swallow, how to be a basically functional person again. Breathing was absolutely wonderful, but it was still a very intense time.

I still have a long road ahead of me. It will take months for my incisions to heal, and some of the nerve damage may never heal fully. I will spend the rest of my life with the terrifying, unpredictable specter of rejection hanging over my head. My immune system will always be low in order to prevent rejection, so I must forever be extremely germ conscious and do everything possible to avoid getting sick. My days will be filled with pills and treatments to keep me going, including the whole new world of managing diabetes. And of course, though my lungs now have different genetic material and are completely CF-free, the rest of my body still has CF, so I still have non-respiratory CF symptoms that need to be managed.

But, hopefully, it WILL be a long road, with plenty of time for all kinds of adventures that I thought were beyond me. Hopefully I will have the chance to blaze through all kinds of open doors in joyous victory. And no matter what happens, however long I get, the breaths I’ve taken thus far make it all worthwhile.

Why don’t you get a lung transplant?

“Why don’t you get a lung transplant?”

I have heard that question countless times over the decades, and more frequently as my health has declined in recent years. Each time I secretly gritted my teeth and reminded myself that most people are nowhere near as familiar with pulmonary disease as I am before trying to answer calmly. So I figured I should give a public answer to the question on so many people’s minds: Why doesn’t she get a lung transplant?

Short answer: Because it could kill me.

Long answer: All transplants are complicated, and lung transplants particularly so. CF patients always require double lung transplants, because both our lungs are so heavily impacted and full of infection that a single lung or partial lobe transplant is basically worthless. Statistically, close to 90% of lung transplant recipients survive the first year post-transplant, but that number drops to about 50-60% for surviving 5 years post-transplant. They aren’t going to consider a transplant until you’re already running out of time, because there’s a significant chance that the transplant itself could end up shortening your life.

Then you have to be lucky enough to actually get a transplant. You can’t exactly walk into Lungs-R-Us and grab a pair, and people tend to need their lungs while they’re still alive, which means we’re dependent on lungs from deceased donors. And that donor needs to match on several different criteria, the lungs need to be healthy and undamaged, and the donor needs to be within a certain geographic area as organs can only travel so far. Being listed doesn’t guarantee if or when you will receive lungs, and some people unfortunately die while waiting.

Once you get a transplant, the complications begin. The surgery itself is long, grueling, and dangerous, and is followed by a lengthy and complex recovery period. If all that goes well, then it’s time to adjust to your new life as a transplant recipient. That includes a lifetime of immunosuppressive drugs to prevent your immune system from rejecting the foreign organ in your body, which means massive lifestyle changes to avoid the infections that your weakened immune system can no longer fight off. This is particularly tricky with the lungs, the only transplant organ that is constantly exposed to the outside environment and all its contaminants with every breath you take. And even with all your best efforts, you will still likely experience and need to be treated for some form of rejection. There is an endless array of potential post-transplant complications, many of which can be fatal.

Is it worth it? If it goes well, yes. It’s basically trading one chronic illness for another, but at least in this one there’s a chance you might be able to breathe and possibly even function as a mostly-normal person. But it’s definitely not worth trying while you still have a fair amount of life and normal(ish)-person-ness left in you.

All that being said…

On Monday, my doctors shocked me by saying they wanted to start the process of getting me on the transplant list. I thought my lung function needed to be lower and that I had more time, but the fact that I’ve been having such frequent, severe, lengthy CF exacerbations that have been so minimally responsive to treatment makes me eligible. They’re also very concerned about the fact that we’re almost out of antibiotics that are at all effective against my pseudomonas infection. I’m running out of time more quickly than expected, and they want to pursue this option while I’m still healthy enough to have a greater chance of coming through it successfully.

This week has been a crazy whirlwind of testing and consults. Today the transplant team met and determined that, as long as the last few tests go well, I am a candidate for lung transplant. They have not yet decided whether or not to list me immediately, as they want to see how I respond to one last antibiotic. If it goes well, we may hold off a few more months. If, as I expect, it doesn’t have a significant impact, then they will likely list me immediately. And apparently HUP is a high volume transplant center, so the expected wait time once listed is only 3-4 months. Basically, by this time next year, it’s expected that I will be post-transplant.

It’s been a wild ride of a week. I’ve definitely been stunned and terrified, but at the same time I’m also relieved. The last 5 months have been pretty terrible, and the thought of slogging through a few more years like this was kind of horrifying. I was beginning to lose my will to fight, as I’ve been working so hard yet seeing almost no return. Things were starting to feel kind of hopeless and pointless, as I was spending so much time just surviving and very little time actually living. I honestly wasn’t sure how much longer I could keep going on like this. So as much as transplant is terrifying, it has also revitalized my will to fight, because at least now I have a goal and a chance of things being better. I can do grueling, I can keep hanging on by my fingernails, as long as I know that this fight won’t be forever and might actually accomplish something.

I’m very tired. And I’m definitely not looking forward to the trials coming my way. But I’m also not done quite yet. Bring it, CF! You still haven’t gotten the best of me!

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!!