Sunday, April 28, 2013

PacMan on Steroids...

. . . meet PacMan on Pulmozyme.

A few days ago, I got an email from a CF mom asking me if I'd tried "Muck Busters!" -- the new mucus-clearance video game from our friends and longtime CFF drug-development partners over at Genentech. (For more information on the game, please visit this post at CF Roundtable.) I hadn't, but I offered to download it (it's free) and give it a whirl so she could hear "a CF perspective" before passing the game along to her 9-year-old son. 45 mins, 5 levels, the start of possible carpal tunnel syndrome, and just the teeniest bit of competitive spirit later, I emailed her my thoughts and she suggested I post them on the blog as well. And here we are.

What I think is most striking about this app/game is this new notion that treatment time can and should be fun for kids, and that they should have some sense of what their treatments are actually doing for their body. I think this is an awesome idea, I just don't remember it being very popular with the pharma companies when I was young. Back then there was no "Bubbles the Fish" neb mask, no colored Vests with decorative stickers (those actually came out the year I got my transplant), and certainly no iPhone "hey, look, CF is cool!" apps. Quite honestly I wish there had been, and I'm thrilled that this disease has reached a point where children are encouraged to actually TAKE OWNERSHIP of CF, in the sense that they should understand the meds (at least a little), make the Vest their own, and yes, even have a little fun with it all. So right off the bat, kudos to Genentech for helping make CF a little easier.

That said, the game itself proved anything BUT easy, at least to my 31-year-old, half-over-the-hill fingers. There are 5 characters: Mr. P (who tunnels through mucus and breaks down DNA),  Andy Antibiotic (who sprays bacteria with his fire extinguisher), Broncho Bob (who opens airways), Shakey (who wears a vest and generates enough power to move things around on the screen), and Hydrator Hank (who uses a hose to clear the cut-up DNA off the screen). The object is to use Mr. P to tunnel through the mucus and clear it off the screen before time runs out. Bacteria get in his way and have to be removed (you have to switch characters to do that) and DNA strands have to be cut up before he can get around them. In other words, it's a little like playing PacMan  -- only in this case PacMan happens to be running around in a pair of CF lungs. Poor dude.

Right off the bat I started playing the game incorrectly, despite knowing the "rules." A CF adult to the bitter end, I was hell bent on killing as many bacteria as possible, even returning to areas already cleared of mucus to attack (bacteria will reproduce, so they're constantly popping up around the board). It got so ridiculous that my non-CF friend who was watching/playing with me had the following to say:

Her: Stop killing the bacteria! Why are you doing that?!
Me: What else should I do?
Her (excited, nearly grabbing the phone): Clear the mucus! Clear the mucus! (Yes, that happened.)
Me: But if you leave the bacteria it's going to get resistant!!
Her: (blank stare)

The point of this game is to clear mucus. Mr. P. is the star. Shakey the vest can't clear mucus -- he just moves bacteria out of the way. Broncho Bob opens airways that serve as shortcuts to the mucus -- he doesn't clear it either. Hydrator hank clear DNA remains, but he can't clear mucus, and neither can Andy Antibiotic, for obvious reasons. So for the purposes of the game you need only open airways if you plan to use the shortcuts, and you only need to hydrate your airways or clear infection if stuff is in your (Mr. P's) way. Oddly enough, you'll win more in the lower levels if you leave Hank and Bob alone altogether. Later on, they become helpful.

What's cool is that the star players make sense in CF land (Mr.P/pulmozyme, Andy Antibiotic/abx, Shakey/Vest, Hydrator Hank/HTS, Broncho Bob/bronchodialators) and the obstacles do too. The game has bacteria, "boss" bacteria (harder to kill), mucus, clogged airways, improper DNA, and -- most hilariously -- white blood cells that you can't kill at all but which will eventually run into bacteria, become inflamed, and explode obnoxious DNA all over the screen. The DNA makes the mucus harder to get through and means extra work for Mr. P. And that, my friends, is science.

All in all, I told my CF mom friend that the game is, in my opinion, totally appropriate for slightly older CFers with enough coordination to actually win a couple levels and enough understanding to know that, at the end of the day, CF is not a video game. The best thing about the game to me came with the understanding that different meds/therapies play different roles in our health, and if a game by the makers of Pulmozyme wants to place extra weight on mucus clearance above bacteria, well, can you really blame them?

So now it's your turn, adults and parents. My CF mom friend would appreciate some more feedback, and I'd love to hear your thoughts. Is this is a good thing for the CF community? Do you think it would have changed your CF experience to have games like this as a child? What are your thoughts on how to make treatments "easy" and keep kids educated about their (okay, OUR) disease? And for those of you who have played "Muck Busters!", feel free to share your experiences. Thanks!

Wednesday, April 17, 2013

Guest Blog: Friends for LIFE (Part 2)

When I set up this blog I wanted it to be about "Life, Lung Transplant, and Cystic Fibrosis. And Everything In Between." What I learned very quickly is that none of these -- not life, not CF, and certainly not transplant -- is a solitary journey. Because of that realization, A Matter of Life and Breath has been proud to have a number of wonderful "Guest Bloggers" ranging from my friends and family members to a couple of folks who are themselves CFers, organ recipients, or other fellow travelers. All of these contributions have been beyond awesome and so so meaningful. That said, I'm not sure any of the guest blogs to date have touched me personally as much as this one.

I first met Jess after her amazing gift, at an event to support the Cystic Fibrosis Foundation. She was vibrant, full of energy, and clearly excited to be giving so generously of her time and herself to support her friend. Jess ended up saying a few words that evening about why she was there, her gift to Allison, and the importance of supporting the search for a cure. As I listened to her words, I thought back to the statistic that one organ donor can save up to eight lives. Hearing her speak, it became crystal clear to me that while one single donor may directly save eight lives, the impact of that gift is far more widespread. When Jess offered Allison the gift of life, she offered me and many others who love Ali deeply (herself included) many more years with our daughter, sister, "cyster", and friend. She offered everyone who will ever come into contact with Ali the chance to be touched by the miracle of organ donation. She offered all of us with CF the gift of hope, and compounds her gift through the volunteer work she and her friends continue to do for a cure. In every single smile or laugh or good work Ali does for the rest of her long and healthy life, Jess will be a part of that. And she offered all of us the gift of inspiration with her truly HEROIC act. And that, beautiful people, is the gift I want to share with you through this blog. Jess -- our friend, our hero -- take it away:

The Gift

My I.V. was started by a young male intern, who to me, appeared to be approximately sixteen years old. As I inspected his credentials and braced myself for yet another needle poke…hmmm, probably for the twentieth time over the last few months of testing, I closed my eyes and thought of Allison. Across the holding area, behind her own curtain, she was also being pinched and stuck.
When the surgeon initialed my left side with a sharpie, I felt strong, calm, and at peace - with those initials on my side; at least we know they won’t take the wrong kidney. It wasn’t until I had to take a walk with the intern, who now looked like he was about fourteen, that it all seemed to hit me - I’m putting my life into their hands… I’m trusting the doctors and nurses to get me through this… More than that - I’m trusting God to get me through this.

When the doors opened to the operating room, all I could see was sharp, shiny metal…suction looking things…more metal. And a man covered head to toe in scrubs with a splash mask. As two nurses and the twelve-year-old in scrubs helped me up on to the table, I felt tears streaming down my face – when did I start crying? When I was asked to sign another consent form and state the purpose of my visit to the Columbia-Presbyterian O.R., oh hey I had nothing else to do today, I realized that reciting “laparoscopic nephrectomy of the left kidney for donation” while shaking and crying was harder than I thought it would be. I felt the fragility of my life all at once.

Then, it happened. The calm, still, quiet voice I had been listening to in the months prior when everyone looked at me like I had ten heads, washed over me like a warm wave. In my mind and body I was experiencing the greatest fear I had ever felt, but in my heart….down all the way to the bottom of my soul – I knew God was leading me forward and that I had to follow. I had to get to the other “side” of this.  And I did. I woke up four and a half hours later feeling like Rocky after he defeated the Russian. Beat up, but man was I a champ! I didn’t let anyone, or anything, not even a little fear, stop me from doing what I believed I was meant to do. If there was ever a moment where I thought, hey I’m not so bad, it was then.

After an entire day in recovery, I was moved to a bed on the seventh floor, to a unit reserved for transplant patients. It was then that my life changed.

My first roommate had had a kidney transplant from a deceased donor. She was sitting up on the telephone calling a car service to pick her up from the hospital. She walked over to say hello to me. I thought, wow she’s tough. My second roommate had a kidney and pancreas transplant from a deceased donor. She was in and out in a matter of one day. I listened to the nurse’s discharge instructions for my roommate and could not believe the amount of medication she would have to be on for the rest of her life. By the evening of the second day, I was able to get myself out of bed and walk. I had to walk. I had crippling pains and knew the only way to feel better was to get moving.

And I have to see Allison.

I cannot describe how great it felt to see my friend again. It seemed like she had been in recovery forever. Still experiencing complications from surgery, there she was – awake and alive. Sitting next to her in a chair and watching her smile at me, I could see who the real champ was then. It wasn’t me – it wasn’t me by a mile. Lying there in front of me was the strongest fighter I had ever met. I wandered the halls for hours, marveling at the fact that there were a lot of people on our unit, transplant patients like Allison, who had been waiting. In fact, each room was occupied, other people shuffling slowly in hospital gowns passed me in the hallway.

At around one a.m. on the final night of my stay, I received a text from Allison about her good friend Katy. Katy is considered one of Allison’s “cysters”, a term a group of girls all battling Cystic Fibrosis call each other. She was in the hospital, on our floor, waiting to hear if a pair of donor lungs would become hers. I didn’t sleep much that night, and at the first sign of day break, I found myself eager to go to Allison to find out what was happening.

Despite the early hour, the waiting room I passed was full of people…probably Katy’s family, I thought. In Allison’s room I was introduced to another cyster there to support Katy. When Lyndsey, also a double lung transplant recipient extended her hand and called me a hero – I found that I couldn’t speak. She asked if I’d like to come and meet Katy. I followed her to a room down the hall and looked in. There she was, so young, beautiful, with two blonde braids, and an I.V. sitting calmly. She called me a hero, just as Lyndsey had, and I can’t tell you how strange that word sounded coming from these amazing girls. It was the single most humbling experience of my life. I felt overwhelmed with emotion watching Katy sitting there, waiting to know if those lungs would be hers. If she would be able to take a deeper breath.

I muttered something; you’ll be in my prayers was it? And as I turned to walk away I felt the full weight and importance of that moment. In the face of all of that uncertainty and doubt, Katy looked at me and could see beyond her fear. She thanked me. Still awe struck, I had to remind myself of what for. I sat in silence in my room for the next hour and let the realization come over me that my friend Allison had once waited in just this same way. For lungs which finally made their way to her in 2004, and then for my kidney.

One day after being released from the hospital, there was Allison, sitting on the end of my couch eating a gigantic turkey hero as I shivered under a blanket. Is she made of steel or what? Things slowly went back to normal. My pain was moderate; the recovery itself has been more of a process than an overnight experience. I am tired often, but have the blood pressure and urine output of a normal person. I can jump, dance, go crazy with my kids, and keep up on a long walk. Allison and I laugh and hang together just like the old days.

 But I am different – forever changed. In giving, I have received the gift of becoming truly grateful. Of realizing how fragile and temporary life is. Those few days I spent on the seventh floor of Columbia Presbyterian gave me a glimpse into a world most people don’t even realize exists. A world of waiting, a place that exists where there are no simple answers or miracle cures. A world where each and every day people pray that their call will come – people with families who care, sick women, men and children from all walks of life. And what binds them is that they’re all hoping for the same thing - the chance that there is someone out there who will match, someone out there who has taken a few minutes to fill out a form. Someone who can bring life to the waiting.

Take a moment, be thankful for those heroes, and then register to DONATE LIFE!

Thursday, April 11, 2013

Guest Blog: Friends for LIFE

As anyone currently following me or this blog on FB probably knows, April is National Donate Life Month. As a transplant recipient, this is obviously a cause very near and dear to my heart (and my lungs!), and I thought it only appropriate that this blog take a time out to celebrate the miracle that is organ sharing and altruistic donation. 

With that in mind, I'd like to take you back in time to June, 2011, when I wrote this post about my dear friend and fellow CFer/lung transplantee, Allison, and her need for a life-saving kidney transplant. The response, even at the time, was pretty overwhelming. One woman wrote in from Texas asking if she could be tested, another reader indicated that she would be willing to travel from California if needed. More still wrote in expressing prayers, love, support, and their own stories of survival and hope. And in classic Cystic Underground style, the community rallied around Ali in a way that left no doubt that she was loved and supported -- we even had the spouse of another CF/transplantee test to see if he could be a match for her, though they'd never even met. 

Since then, I've received countless emails, comments, and FB messages asking for the ending. So without further ado, and in the spirit of true friendship, community, and thankfulness, I give you this guest blog by one of the strongest survivors I am honored to know. Ms. Allison, take it away:

Living Proof

Two years ago this month, my blood pressure was out of control, I looked swollen and just not right. My lung transplant team had a feeling my kidneys were on their way out after 6+ years of medications to keep rejection at bay, and referred me to a nephrologist. One visit, some scary preliminary bloodwork and a kidney biopsy later, that word was thrown my way again: transplant. This time it seemed more daunting than my lung transplant, because dropping kidney functions were not helping my lung functions stay stable, and also because the option of a living donor meant I could not just sit back and wait on a very long list. It was up to me to find someone willing to just be tested as a match. The months following my initial diagnosis of end stage renal disease were stressful, frustrating and incredibly tense. My siblings were great matches but they both ran into their own health issues and could not proceed with the donation process. I had pretty much (inwardly) given up the prospect of a living kidney donor when a good friend of mine, who had been one of the first to send in her organ donor evaluation forms and had told me with no hesitation that she was “the one,” again stepped up and chose to continue with the process.

Let me assure you, it takes a special person who is not even related to you to go through what she did. Countless trips to doctors, getting bloods drawn, assuring her family and friends who thought she was kind of nuts for even considering donating an organ. All while working full time and raising 2 small children. I told Jessica more than once that she could stop the process at any point if she didn’t want to follow through. It was totally fine with me if she backed out. But she insisted from the get-go that she always felt that she was supposed to do this; she had faith that this was meant to be. We had many long talks about it, and most of the time SHE was helping ME keep the faith, when I should have been reassuring HER that everything was going to work out and be okay. I think it is our friendship, however weird and funny it is, that got us through. Our sick senses of humor were and have been a key in keeping our sanity through a grueling time.

Since our surgery, our lives have continued as before. I say this because the day to day keeps going after such a life changing surgery. She has her children to tend to, a home that she takes care of, her life that she lives. My life goes on as well, with a few more doctor visits than before. We meet up for our Breakfast Club adventures, drink coffee, catch up on our lives and those of horrible reality TV. We go shopping together and make each other laugh until we can’t breathe. Our friendship feels like it did before, but on a more profound level now. She is, literally, a part of me!

Words can never express how grateful I am to Jessica. How can you thank someone enough for giving you the gift of extended life? For giving me more years to spend here on earth with the people that I love? To allow me to see more sunrises and sunsets that I probably wouldn’t have seen if it were not for her? Words can’t explain it. I know that she knows I am forever indebted to her. If she ever needs anything I would be there for her in a second.

With April being Organ Donation Registration Awareness month, I want to encourage everyone to register. It does not mean you have to be a living donor like Jessica. But when the time comes that you may not need those organs anymore, you could save someone’s life- maybe a few people’s lives. 

Believe me, I am living proof. 

To read more about Allison and her amazing journey with CF, new lungs, and "Sheen the Kidney Bean", please visit her blog at New Organs, New Life

Saturday, April 6, 2013

A Prayer In Celebration

In honor of National Donate Life Month and in recognition of the truly beautiful gift of organ donation, hundreds of donor families, living donors, recipients and their families, and healthcare workers will gather today at the amazing St. Patrick's Cathedral. Today, I offer this prayer in memory of my beloved Donor Bob -- the greatest guy I never got to meet -- and for all those who give of themselves to help save us all.

A Prayer in Celebration of a Life I Never Knew

I wasn't there to hold your hand
The day you slipped away.
I was going through the motions,
I was on my knees to pray.
Two hearts at once were beating,
Two strangers in the night;
Two families filled with grieving,
Two souls putting up a fight.
None of us were certain
What tomorrow's morn would bring
Lives were hanging in the balance
When we heard that telephone ring.

In the grand scheme of Creation,
Saving my life seems so small,
But I promise, lovely donor,
To my family: it means all. 

If I saw you on the street today 
I wouldn't know your face,
But I hope that you'd be proud of me
And look on me with Grace.
Because every breath I breathe this life
Is your breath breathing too,
Every song and whispered prayer
Made possible by you. 
The lessons you have taught to me,
I will not soon forget,
Like what a gift it is to save
A life we've never met.

So Donor Bob, this one's for you
(I hope that you like rhyme!)
You've given me amazing things
You've offered me more time.

And Lord, please hear this simple prayer,
Please look down from above,
Guide me to spread Bob's purest gift,
The gift of selfless love.


Wednesday, April 3, 2013

Blood is Thicker than Water

...and MY blood, apparently, is thicker than it should be.

So I visited my doctor yesterday for a quick clinic appointment, only instead of the normal chest x-ray/bloodwork/PFT routine this visit started with a CT scan, complete with IV contrast. And for those of you who need the cheat sheet version of why this additional test was necessary, let me quickly bring everyone up to speed:

In late January I visited clinic only to find that my PFTs had taken a rather severe plunge from where they'd been in December after all my photopheresis/rejection and port-placement "fun." The drop was concerning enough that my doctor ordered a bronch, which showed no acute rejection but did come back positive for a virus that can cause pneumonia. He also ordered a regular old CT scan (no contrast) and that showed some diffuse patches of what appeared to be infection. The end result of all this was IVs, rest, and waiting, with routine follow-ups scheduled to monitor my PFTs.

Since then, my PFTs have been falling. I'd say that it's been slow and steady, only it hasn't been slow; it's been terrifyingly fast. Since the start of the year I have lost well over 30% of my lung function. The weird thing, though, is that this hasn't presented like typical chronic rejection (or BOS). What I mean by that is the numbers are following a strange pattern, and the CT scans and other tests we've run haven't indicated that this is traditional BOS. And yet my lung function has been clearly falling, so much so that my doctor suggested we might want to consider an open-lung surgical biopsy -- and that I begin the process of updating my testing should I continue my free-fall right up to the point of retransplant.

Um, yeah. Scary.

So this past Tuesday I had my CT with contrast and then I headed up to the PFT lab, where I blew my little lungs out only to come up with numbers that were pretty similar to my last visit. I was trying to console myself with the fact that at least they hadn't gone down drastically, but to be honest, I was in a bad space. More to the point, I was super confused. I felt very strongly, in my heart of hearts, that this wasn't supposed to be the end of my journey with my Delightful Donor Bob. And, you know, I was scared. And sad. And, well, a little pissed off, to tell you the truth.

And then imagine my delight when my doctor called me into his exam room and told me that this latest CT had found -- drumroll please -- blood clots. In my lungs. And some dead lung tissue up behind the clotted area that is most likely what we were seeing on the original CT scans. And my heart, that little clotting trickster that it is, literally leapt for joy.

Only in CF-land could blood clots in the lungs be considered good news, right?

Blood clots are treatable, even if there may be some form of permanent damage from the clots. Blood clots are a known enemy -- which means we know what weapons we have to use against them and we know what strategies generally work to get things back on track. Blood clots do not require open-lung biopsies or testing, both of which were cancelled before I skipped out the door from clinic. Most importantly, though: blood clots are not chronic rejection. My lungs are not failing, nor is there any reason at this point to believe that they will.

Honestly, I need to take a second to let that one sink in.

I woke up this morning still clotted, but feeling infinitely more free. It's appropriate that just the other day I wrote about my gratitude for my donor, because this morning it was reaffirmed to me 1,000 fold. I have so much living left to do, and it looks like these lungs are going to have to stick around and come with me while I do it. For that, I know that I am beyond blessed.

So there you have it, beautiful people: all the (lung) news that's fit to print. And while I still have some tests to get through and some treatment to sort out before I can call this latest chapter "over", I can also delight in the knowledge that there are many more chapters to be written.

And I hope you'll stick around to share them.