The Night Before A Beatty Christmas

'Twas the night before Christmas
And all around here
Not a creature was stirring
Not even Sam Bear.

The meds were all taken
T-cells were suppressed
In SUNO pajamas
The whole fam was dressed.

The TOBI was over
The meal was done
And we were all dreaming
Of holiday fun

But before all the presents
And spreading of wealth
Let us say merry Christmas,
Peace to all, and good health!

With love, beautiful people.

Piper, Sampson, and the Beatty Family

On Swimming Through Fires

It's been kind of a rough week in CF land. And in the interest of full disclosure, please read this blog in its entirety before sharing with any young CFers or other children. It's not obscene or inappropriate, but it does deal with the harsher parts of cystic fibrosis.

One of the most painful realities of CF is that the more involved you are with the community -- the more you take the time to share with, learn from, and work alongside of other people who share your disease -- the more likely you are to come face to face with the super hard fact of how rotten this little monster really is. Or, to put it bluntly, the more likely you are to know people who will die. Of your disease. Of your complications. Of parts of your illness that you may or may not experience yourself, and may or may not survive if you do.

And let me tell you guys right now: it sucks. All of it.

Whether it's watching desperately from Facebook as a friend dies awaiting lung transplant and knowing that you were lucky enough to be given that gift, or watching from across the hallway as one of your friends struggles with severe complications FROM lung transplant and knowing you haven't necessarily escaped unscathed after all (and you certainly can't let your guard down NOW, if ever) -- well, it's all pretty much equally sucky, and it's all very much part of day-to-day reality of cystic fibrosis. And just because it's a side of CF that most of us don't like to talk about all that much, that doesn't make it any less true. For any of us.

I sometimes think about the lines from Mary Oliver's beautiful poem Dogfish:

And nobody gets out of it, having to
swim through the fires to stay in
this world.

And I know that there is truth in what she writes here, that nobody gets out of it.  I know that CFers aren't alone or unique in facing struggles -- even when it seems like we are, or maybe like we should be. I know that even a disease where surgeries become "no big deal" and the only hope for realistic, long-term survival is either to replace your vital organs or somehow find a way to modify your very genetic makeup, the things that make you what you are, is still not the be-all-end-all of human suffering. I know that when we start to think like that about CF it becomes isolating, paralyzing, and even self-desrtuctive. I know all that, and I know that every single one of us, everyone and everywhere, will have a moment when we are "swimming through the fires", and that mine are no more or less important than anyone else's. And nobody gets out of it, after all.

I know all that. Of course I do. But I just wish I didn't have to know it so damn well.

My heart goes out this holiday season to all of us who are swimming through fire, and especially to those who swim in my particular river. To my friend who celebrates the life of his sister taken far too soon, my beautiful girl who lies fighting on life support for her 1-year-old lungs so she can marry her fiancé next summer, my super smart friend whose heart is now too weak (barring a miracle) for new lungs to come, and all the rest of our wonderful underground who need help, love, positivity, or just a simple breath of air. You all are so much my current, my river, and my healing water that this fire, hot as it is, will never stand a chance.

And I will swim with you for as long as it takes.

Webinar: Providing Best Care for Individuals with Cystic Fibrosis

Today I had the pleasure of "attending" a webinar as part of the American Thoracic Society's Cycstic Fibrosis Week 2012, a joint product of the ATS and the Cystic Fibrosis Foundation. The topic of today's discussion was "Providing Best Care for Individuals with Cystic Fibrosis: A Partnership with Patients and Families." It featured an informative lecture by Dr. Michael Boyle of the CF Center at Johns Hopkins University and a Q&A period featuring some great contributions by CF patients, parents, and care partners. Special kudos for this event go to a woman I am proud to call a friend and fellow CF patient, JD, and Emory grad -- as well a phenomenal patient advocate and manager of the CF Legal Hotline -- Beth Sufian.

I encourage all of you to check out the taped version of the webinar yourself, which I'm told will be available at the ATS cystic fibrosis week website (link provided above). I also encourage you to get involved in CFF-related educational programs and webinars in the future, which are often provided through the CFF and through programs like the CFRI's CF Discovery Series. For now, though, here are some of my main takeaway points from this discussion. I should emphasize from the outset that these are MY observations and have not been discussed with Dr. Boyle, the CFF, or the ATS. Nothing below is meant to serve as anything other than my personal understandings and opinions.

Got it? Awesome. 

Takeaway #1: Yes, Compliance, Care, and Medication DO Matter!

Dr. Boyle spoke about a study on CF homozygous twins (meaning identical twins with the exact same genetic makeup) that showed a strong correlation betweens genetics and lung function in CF patients. But he also spoke about a surprising curve ball with this study, which was that identical twins with CF showed a far more significant disparity in lung function AFTER they separated and moved into separate homes. This indicates that non-genetic factors like compliance, care (wether self-care or care-center driven treatments), environment, and medications DO actually matter in determining outcomes for people with CF. And yeah, I know what you're thinking, which is probably a great big "duh, Piper, my mom told me that when I was, like, 5 years old!" kind of moment, but really: knowing and seeing actual evidence that the choices we make in life and in health make a difference to our overall well being even beyond the genetic card we happen to be dealt is always worth repeating.

Bottom line: when it comes to our bodies we are neither fully in the driver's seat nor fully passive, and the decisions we make are always going to have an impact. Most importantly, we can choose to be honest (yeah, really honest) with ourselves, our care partners, and our healthcare teams in addressing these issues.

Takeaway #2: Good Care Centers Are Active Care Centers

Dr. Boyle spoke about a care-center study that involved observation of CF care centers recognized for having some of the best therapeutic outcomes across the country. The results of this study were too numerous to really discuss each one in full here on the blog, but for me a couple of big findings were that 1) good care centers take time before the patient arrives to discuss each case and come to an agreement on what issues need to be addressed during the relatively short patient visit (optimized patient care) and 2) Dr. Boyle's statement that good care centers are empathetic, compassionate, and caring without necessarily being too nice. What he meant by this, apparently, is that good care centers are compassionate in their care but refuse to be "nice" when it comes to aggressively treating infections or drops in PFTs, nutritional issues, or the need for patients to be proactive about their own healthcare. Wow, what a cool distinction. 

What WE, as patients and families, can do in this issue is be involved (joining or forming a patient/parent advisory board at your clinic, for example, is a great first step), talk to our doctors about these sorts of findings and about ways to improve our personalized care, and basically lead by example. If we want our doctors to maximize the value of our time in the office, for instance, we can have our own "pre-visit meeting" with our families, our partners, or even just ourselves and a journal and consider questions we need answered, prescriptions we need filled, issues we need addressed, and concerns we want acknowledged. This is a great way to ensure we actually get what we pay for in terms of clinic visits, and bonus points if you can really impress your doc and make him/her take notice of your extra effort!

(Dr. Boyle also spoke at length about the fact that good care providers TALK TO PATIENTS. Duh moment number 2, I know. But what I took away from this is that your docs should be discussing your test results, x-rays, cultures, CT scans, etc WITH you, not just making decisions based on undisclosed results. If they don't do this, ASK. It's your body, for goodness sake, and you deserve to know what's inside of it as much as they do!)

Take Away #3: Transition is Tough, but Not Transitioning is Even Tougher

My personal favorite moment of the webinar came when Dr. Boyle showed a joke slide of two parents pushing a full-grown man in a stroller and joked that he thought maybe this family was on their way to CF clinic. Haha, right? But seriously, guys: HALF OF THE CF POPULATION IN 2012 ARE CF ADULTS. You, or your child, are/is going to GROW UP. It's up to families and patients alike to transition this care in a workable and meaningful way, and preferably BEFORE the patient his 18, 19, 20, or whatever and leaves the family compound.

The simple truth is that YES, you/your CFer can typically go to college, and YES, you/your CFer can typically live alone, and YES, you/your CFer had BETTER get used to talking directly to doctors about your/his/her body early, because that stuff matters. Dr. Boyle suggested that the most successful transitions start early (he noted that "transition begins at birth") but obviously parents and patients need to recognize their individuality in crafting specific timelines. Suffice it to say that if -- barring special circumstances, of course -- no 18-year-old CFer should be unable to list his/her medications, speak to a doctor, understand this disease, or prepare his/her own neb treatment. You wouldn't wait until someone was 16, hand her a driver's license, and expect her to be able to navigate a freeway -- and nor would you want her dependent on rides for the rest of her life -- so why would you do any less training with something as important as healthcare??

We're not in Never-Never Land anymore.

Finally, I just have to mention Dr. Boyle's awesome point that the goal is not perfection: the goal is LIFE.  This echoes my personal best practices advice for anyone dealing with this little monster: don't beat yourself up for little mistakes or bumps along the road, and also never, EVER doubt have the power inside of you to make this work. You just do. Sorry, cystic fibrosis -- you'll never be as strong as we are.

So what do you guys think? Because no matter how many webinars or webcasts or whatever we attend (or don't) the real takeaway message here is that we've got to join the conversation. Our bodies, our disease, our little monster. Have at it, guys.

Thanks to Beth, the ATS, Dr. Boyle, the CFF who made this webinar a truly great educational experience.

Catching Up

Oh, hello again, beautiful people.

First of all, I want to thank everyone for the super sweet messages asking where I've been since my last post. I also want to apologize to anyone who might have been worried. When I started this blog, way back in 2008, I decided I wanted to write about "Life, Lung Transplant, and Cystic Fibrosis. And Everything In Between." At the time I was 26, working as an indentured servant -- um, I mean as an "associate" -- in a New York City law firm, and had just been evaluated for a double-lung transplant, for which I was at that point inactively listed. I was on a never-ending cycle of IVs, was using O2 to get around most of the time, and was pretty constantly in and out of the hospital. What I didn't realize at the time was that my life back then WAS more or less about lung transplant and cystic fibrosis. What was in between was a whole lot of work -- working to keep myself "healthy" enough to stay out of the hospital and inactive on the list, working to stay ahead of the curve at a job I loved even though I was barely able to keep myself breathing, working to fit in all my treatments and exercise and appointments and IVs and whatever else I had going on at the time. Working, more or less, to keep my head above water. You know, where the real air is.

In the past two and a half years, I've had what some people would consider a pretty wild ride post-transplant. There have been some bumps in the road. I spent most of the first year after my surgery STILL on IVs, for example, and I've been in and out of the hospital a few times even just in the last couple of months. I've had port infections and cath infections and lung infections and "normal people" infections and even some just really strange infections, although I'm told that nothing is really all that strange in the twilight zone that is lung transplant living. But the simple fact is that even with all this stuff, I have still experienced way more of LIFE in the past 2.5 years than I had for a long time prior to receiving my new lungs. And sure, Donor Bob and I have now been through a couple of ports and a couple of rough patches and a couple of rounds of photopheresis (with more to come on that score, lucky us!), but we've also been through a couple of birthdays I wouldn't otherwise have seen, had a couple of trips back to my gorgeous home state of Colorado (which I couldn't even visit anymore with my CF lungs), and shared more than a couple truly wonderful experiences and opportunities that I could never have imagined without the gift he gave me. And better than just having all those things together, we've LIVED them. Which is to say that I've been able to live again because of his gift, and I hope a little piece of his spirit lives on through me somehow. I know I carry his memory with me always, as well as his lungs.

So here's my latest update, lovely friends, which is that Donor Bob and I have been busy catching up since our last post.

We've been catching up with lung transplant with our continued determination against this silly rejection and the placement of our brand new Vortex port for photopheresis (scheduled for hopefully next week -- yay!).

We've been catching up with cystic fibrosis through the incredible opportunity to participate on a new project with the fabulous folks at the Cystic Fibrosis Foundation and through more and more chances to meet and be inspired by so many of you all -- our indomitable "cystic underground", for which we are forever grateful.

Best of all, though, we've been catching up on life by turning 31 together. Take that, CF! And, of course, through a thousand little miracles each and every day and the countless breaths that allow us to share in them together.

But don't worry, beautiful breathheads, we'll still be here to catch up with you all via this blog as often as we can. In the meantime though, we wish you all a very beautiful holiday season, the happiest of Hanukkahs, the merriest of Christmases, and the most joyous of new years full of hope, health, and as much of this wonderful life as y'all can possibly handle.

And Everything In Between. 

Piper, Donor Bob, and Sampson