Friday, June 21, 2013

My Name is Piper...

Okay, guys, confession time:

I'm kind of a druggie.

Actually, I think the term my doctor prefers to use is "compliant patient," but come on -- let's call a spade a spade. On a typical day I take pills, shots, and a few drugs designed to be snorted directly up the nose. Supplement that with the occasional (nebulizer) pipe and at least a few binges of hardcore IV drugs a year, and it's tough to make the argument that I'm not completely dependent on the stuff. I'm more than a little grateful for the chance to have all these little treats in my arsenal against rejection, infection, and their old pal cystic fibrosis. And I think it's fair to say that it's come to a point where getting my daily fix is more than a habit -- it's literally a matter of life and, well, breath.

And I'm also pretty lucky, because I can tolerate almost any new drug my doctors decide to throw at me  in terms of allergies or side effects. Longtime readers may remember that right before my transplant I was reacting to literally everything under the sun, but in terms of relative severity to many of my fellow journeyers down the CF road, my "no deal" meds list is pretty small, and is entirely limited to antibiotics. Even drugs like the rabbit juice (um, make that "rATG") don't seem to impact me much, and beyond a slight sense of discomfort or irritation I'm generally good to go.

And then I met Campath.

Yesterday wasn't terrible, honestly. They premedicated me with a LOT of drugs, including IV solu-medrol (steroids), a small dose of oral pred, IV zofran (anti-nausea), tylenol, and benadryl. They also ran the drug pretty slowly, with what I understand was 30mgs infusing over a period of 2 hours after a dose of prophylactic IV antifungals. And the whole time I'm sitting there kind of giggling at the process, because I think there is NO WAY I'm going to need all these drugs because, really, I'm pretty darn stoic when it comes to medical stuff. So we start the drug and I upload an episode of Project Runway on my computer (let s/he who is without reality TV show sin cast the first stone), and there we go. Easy as pie.

Easy as rancid, gross, totally disgusting pie.

About 1 hour, or halfway, into the infusion I suddenly felt extremely tired and nauseas. Approximately 30 seconds later I had my head in a hospital basin and things were not looking pretty. My long-suffering mom ran to grab the chemo nurse while I contemplated the truly gross reality that is hospital food the second time around. Trust me when I say that I will never again claim that hospital food "looks like vomit." Been there, done that, ain't going back.

After a few minutes of that awesome activity, my body decided it was done trying to rid itself of IV poison via traditional methods, and decided instead to go into hibernation mode. They checked my vitals as I stared stupidly at the machine wondering what all those pretty numbers meant, and I emerged from complete burnout only long enough to insist I wanted to continue the infusion. Then I conked out for the entire rest of the treatment and then some, which I highly recommend to anyone thinking of receiving this drug. This has reaffirmed my long-held belief that the best way to combat problems you absolutely cannot solve is to go to sleep. Because when you wake up you'll probably see things differently, be refreshed, feel ready to tackle the rest of the day, and um . . . have some really cool looking hives?

Okay, maybe not so much that last one.

The hives didn't show up for a couple of hours after finishing the treatment, which apparently isn't that uncommon with this particular drug. It started with a distracting itch on my leg, and when I went to examine it I found a couple of small red bumps. I wasn't concerned, checked my stomach and scar area only to discover more hives, and chirpily called my nurse for some additional benadryl, which she promptly brought within 5-10 minutes or so of the request. By which time I was a raging ball of hives madness. And yes, I'm serious. It was like 0 to 6 million hives in 5 minutes flat.

I'm still pretty lucky, by the way. In the grand scheme of things, vomiting, aches, fatigue, and hives aren't much to complain about, especially as it seems I got off pretty easy compared to some people. There was no closing of the throat, no scary "oh my God I can't BREATHE!" style moments, and nothing particularly remarkable about the entire event -- unless you count my lifting my shirt right up to my scar line in the hallway to show a member of the medical team (some of) my spots. Classy. Thankfully that too was a relatively minor blip, as the very old man with the walker seemed completely oblivious to my very red, very bumpy midriff at that moment. Can't say that I blame him on that score, honestly.

The truly great news is that I woke up this morning with far fewer spots and hopefully at least a some fewer t-cells. I asked the other day how long it takes Campath to "work" in the body (i.e., how long before I have the desired effect of dramatically lowered t-cell counts) and was told that the drug works actually remains in your system and continues to kill t-cells (and b-cells, by the way) for a long time after you receive it. This is why it takes so long for t-cells to regenerate after this treatment. As for how fast my own counts are going down, that appears to vary some from patient to patient. In other words, I may be taking some of these suckers home with me after all, but they're not off the hook by any stretch.

So there you have it: the good, the bad, and the totally gross. Suffice it to say that I'm pretty sure this drug would have a very low street value if offered to the general public. It is NOT a fun ride, but I trust my team wholeheartedly and truly believe they are giving me the best chance at keeping Donor Bob around for a long, long time to come.

And that, beautiful people, is a habit I'd prefer never to break.

Thursday, June 20, 2013

On the War(Cam)path

So in case you haven't heard, I'm back in the hospital.

Before anyone freaks out, please know that I am not "sick," at least not in the traditional, infectious sense. I don't have a virus or a bacterial infection brewing in my lungs, no fever or strange symptoms, I'm not in pain or dealing with chills or nausea . . . at least not yet, anyway.

What I have right now is what most people want: an immune system that refuses to quit. Unfortunately, in a transplant patient, too much of an immune system is definitely too much of a good thing. And in my case in particular, it appears that we may be dealing with one (or both) of the following: recurrent acute rejection (recurring inflammation in the lung tissue and airways indicating an immune reaction, nonresponsive to traditional therapies like steroids) and chronic rejection (bronchiolitis obliterans syndrome, which is basically just persistent scarring and obstruction of the airways). Either way, the one thing that's clear is that my immune system and I have reached a standoff. It's been crampin' my style for awhile now, and there just ain't room in this body for the both of us.

So I'm officially evicting my t-cells from the premises, starting today.

T-cells are a subset of white blood cells that are part of the lymphocyte family. They fight infection by attacking intruders to the body, such as viruses, bacteria, or (in some cases) foreign organs. They are the border patrol of the metaphorical country of the human body. And mine have been misbehaving to an alarming degree, which we already knew. We've tried several methods to rein these guys in, but they just can't seem to help themselves. And just in case you think I'm kidding, here's a brief recap of the therapies we've tried thus far:

Increased steroids
Added immunosuppression drugs (aka, azithromyacin)
Antithymoglobulin Rabbit, or rATG (See this post: On Rabbits and Rejection)
Photophersis (See this post: This Little Light of Mine)

And now, please welcome to the stage (drumroll please) . . . Campath.

Campath is a brand name for the generic drug alemtuzumab, which is as crazy as it is unpronounceable. In very basic medical terms, it's an antibody infusion that targets t-cells and flags them for destruction by the body, leaving the person receiving the drug with little to no mechanism infection control. The good news is this can help stabilize and sometimes even improve declining lung function due to rejection. The bad news is, well, you lose your immune system. Like, entirely. For months or even years. Small price to pay for lungs that work, right?

To bring it back to the border patrol metaphor, using photopheresis for rejection is a little like starting a civil war to distract the patrol from attacking the friendly immigrants (like, say, a transplanted organ). Photopheresis changes some of your t-cells so that your t-cells don't recognize each other and therefore begin fighting amongst themselves. Kind of a dirty trick, but hey: all's fair in love and transplant.

Campath is more like a nuclear bomb -- instead of distracting the patrol, it just annihilates them and hopes that something else (aka, IV and oral antibiotics, antifungals, and antivirals) can pick up the slack to stop the bad immigrants like bacteria and viruses. It's generally considered a riskier option because -- like most bombs -- it can come with some added casualties and damage that no one really wants to deal with. In addition to leaving me ridiculously immunocompromised, for example, this drug can also increase the chance of things like skin cancer. Thankfully, regular visits to other docs like the dermatologist for preventative screening can help out with those additional risks.

Overall, I feel good about the plan, although I haven't actually received the drug yet. I agree with my doctor that right now we need to be aggressive in wiping out these pesky little patrolmen gone rogue, and as always I'm somewhat fascinated by the complexity of modern-day transplant care. I'm grateful that we have this option and so so hopeful that it will help my awesome Donor Bob feel a little more at home in his new(ish) surroundings. And trust me, between the option of keeping Bob around and keeping those rebellious t-cells alive and hopping, well, that's just a no brainer. I've enlisted some of the best doctors in the world to serve as generals in this crazy revolution, and I have every confidence that they will bring me through it with as little collateral damage as possible.

And that makes this a fight worth picking.

Wednesday, June 12, 2013


Today marks 3 years from the day my beloved Donor Bob gave me the gift of life in the form of his precious lungs. It marks 3 years of all sorts of wonderful, from turning 30 to watching my sister come into her own as a fabulous designer; from meeting the children of some of my best friends to meeting someone absolutely amazing in every way. It marks 3 years of breathing, celebrating, and LIVING (with all the awesome ups and downs that word entails) and it marks 3 years of continued involvement in the quest to find a CURE for this little monster -- for all of us. The past 3 years have been hectic, amazing, scary, irresistible, indescribable, and -- above all -- beautiful. Every breath is a reminder of the gift I've been given, of the power we all have as humans to make a positive difference, and of the fact that too many out there still struggle for air. It is a lesson I learn and relearn daily, all because of an unknown stranger who had the grace to teach it me as his last act on earth.

And I am grateful.

Today also marks about 6 months since my PFTs began declining. So far, despite multiple treatments, IVs, bronchs, CT scans, and other fun procedures, we have yet to stabilize the numbers to my doctor's liking. So today now marks 2 days since I met with the doctors at a different, affiliated hospital to (re)start photopheresis, a blood treatment that may help stabilize my numbers by confusing the heck out of my immune system, for lack of a better description. (For those of you who like details, check out this post on the ins and outs of photo -- with pictures! -- for more information.) In addition, we're going to add a fourth immunsuppressant drug to my daily routine (this in addition to the prograf, cellcept, and prednisone I'm currently taking), and run some additional tests for other causes of lung damage such as reflux. Pending results on those treatment options, we'll make some tough decisions, but the end goal is get Donor Bob to stick around for as long as possible. What can I say but that after 3 years, I've become pretty attached to him.

And still I am grateful.

I'm grateful because today I got to take a jog/walk down to my old apartment, the one I lived in right after I left the Upper West Side. This was the apartment I lived in when I was lawyer, when I was first told I needed a transplant, when I got evaluated, when I started this blog, and when I first met Mr. Sampson Bear. It was also an apartment I lived in when I was sick. Like really sick. I stood out front of it today, in my running clothes, and I breathed. I breathed because I still have breath to do it. I breathed because Donor Bob, however down, is not yet out. I breathed because together we are still standing, we are still moving forward. And I breathed because it never hurts to take a moment to look back on where you came from -- even when you're not sure where you're going.

And then I turned around and went home. Round trip, it was exactly 3 miles.


Monday, June 3, 2013

Community Faces: Julie Wagoner

One of the exciting things about the Community Faces Project is the number of people without CF who have written posts. Parents, siblings, spouses/partners, family members, and friends of people with CF (crazy fools) bring a totally unique perspective to the table -- and it's a viewpoint that I, for one, never tire of hearing (or of learning from).

This is one of those stories. Written by an amazing CF mom, it captures that moment of recognition so many of us remember when we first understood the reality of cystic fibrosis. Perhaps not surprisingly, I relate to this story on an extremely personal level. I remember wandering into my grandmother's living room as a child, plonking myself down on the floor next to an end table that doubled as a bookshelf, and skimming the titles until my eye landed on the words "cystic fibrosis." The book was Frank Deford's memoir of his daughter's short but beautiful life, Alex: the Life of a Child. I snuck it back to my room and spent the next week or so turning its pages and shedding tears. Years later I would win the award from the National CFF that bears her name (that's Mr. Deford and myself on the right, holding the amazing painting I received for the award). She remains to this day one of the many CF spirit guides I carry with me on this journey.

CF was already a huge part of my life when I read about Alex's heartbreaking and inspiring life. But for many people who read the best-seller or watched the movie made of her life, that part of the picture would only unfold later. And this is Julie's wonderful story.

Community Faces: Sounds from the Underground

Name: Julie Wagoner
Relationship to CF: Mom to an almost 15 year-old CF superhero named Gage. DF508/N1303K, diagnosed at 23 months, upper right lobectomy at 7, two port placements, lots of bronchs and IVs, kidney stones, one sinus surgery, and a partridge in a pear tree.
CF Perspective: CF has been a struggle, but not one without rewards. The current reward is facing the dreaded teen years, which are absolutely kicking my butt! But, quite honestly, I wouldn't have it any other way.  

The year was 1986, the year I turned 14. I was a scrawny girl, burdened with prepubescent gangly limbs, stringy long hair, sporting sunburn and freckles, skinned knees and mosquito-bitten ankles scratched raw. It was summer. We were visiting family at the beach and I sat huddled against a door frame, my ear to the wall, peeking out from behind my cousin's bedroom door in order to see the television in the family room. My tears were hidden there, my throat sore from holding back sobs, embarrassed at the thought of being caught and teased, but unable to look away from the story unfolding on the grainy screen.
It was about a little girl, younger than myself, and she was dying. I'd seen plenty of movies before, about disease, about war, about monsters. I confess to being tender-hearted, though I hid it from most. But something about this story hurt in a way that was different, frightening. I grieved. I was grieving for a girl I had never, nor would ever, meet.
Nothing could be done for her? There was nothing? Surely that couldn't be right. Medicine could fix so much. How was it that this tiny young girl had no life ahead of her? I couldn't understand.
I watched to the inevitable end and stayed in my cousin's room that night, unsettled, sad, and confused. And while my vacation went on, as did my summer, she lingered in my thoughts. I found the book that the movie was based on at the library once we'd returned home, but I could not bring myself to actually check it out. It was too harsh, too cruel. Instead, I returned each week to find it, still on the shelf where I'd hidden it, and sat alone in a darkened corner, quietly reading the entire story, written in her father's hand, once more hiding my tears. No other piece of writing ever quite affected me the way that book did.
Had I known? Looking back I wonder if something deep inside me knew, even then, that the disease that stole this child from her parents lurked inside of me, hidden, silent, waiting. If some base instinct in my body foretold of what I would pass on to my son so many years later. If some part of myself had grieved, even as a child, not only for her, but for myself.
The movie was 'Alex: The Life of a Child', based on the book, by Frank Deford. It was about a girl named Alex, who died in 1980 at the age of 8 from cystic fibrosis.
My son, Gage, was born in 1998 and diagnosed with the same disease in July of 2000, 14 years to the month since I sat in that bedroom and watched the story of Alex. And because of her and her story, our lives are improved. Saved.  
I will always carry a piece of her in my heart.
Awareness. Pass it on.