November 30, 2012

Clinging to the cliff

I have often said to friends over the last decade that I strongly suspect the course of my CF would not be a long, slow glidepath to a predictable end, but rather that I'd last a long time in my 40s (FEV1, not age) and then experience a sudden drop, as if my lungs had stepped off a cliff.  This has proven prescient on my part.

While the drop in my lung function over the last six to eight months can certainly be resemble a stock market crash when graphed, it has been more like losing one's footing on a steep and scree-covered slope.  You're going along navigating things pretty well one moment and then - zing! - you're falling down the hill at a great rate of speed, getting pretty cut up along the way, and desperately looking for something, anything, that will arrest your fall - especially since the bottom of the slope is a dropoff into death.

But there are branches to catch onto, if you know where they are and can grab at them just right.  There are IV antibiotics at regular intervals, there are oral and inhaled steroids, there are bronchodilators.  There's exercise and nutrition.  These can all slow your fall.  And to pad it - to keep from getting too banged up to survive the landing - are other devices: supplemental oxygen and ultimately that great portal to a different slope, the transplant.

I am now on oxygen.  And though that needs a whole post in itself, I will say only what has been rattling through my head this afternoon about that: oxygen makes me feel strong again.  I'm not as actually strong as I was this time last year, but at least I can get up out of the subway without gasping for breath or having to rest at the top. (Or worse: midway up.)

Right at this moment, I have temporarily arrested my slide.  Maybe I have found myself on a narrow ledge treacherous in itself, but less deadly than the slope I've been coming down or the cliff that comes next.  You see, when I went to my siblings' town for Thanksgiving, I was so wrapped up in dealing with the portable oxygen concentrator and packing IV meds (yes, I was on IVs the whole time!) that I forgot my inhaled medications.

Let me repeat that:  for three days, I had NO pulmozyme, hypertonic saline, pulmocort, or aztreonam.  I only happened to have albuterol in my suitcase already because as it doesn't need refrigeration, I had some extra in there.  And by the time I returned to New York, my lungs felt great.  Could it be the cleaner air in Greenville, SC?  Could it be that giving my lungs a break from these harsh medications is what they needed?  I ask because I've been back less than a week and already the tightness and the wheezing has returned.  I'm tempted to DC the inhaled meds for a three day block again just to see what happens.

And what, clinically, DID happen? PFTs are up!  I had a series of tests and appointments on Wednesday and in stark contrast to my recent decline, my numbers had improved substantially.  My lung volume up 17 percentage points.  My FEV1 up 8 percentage points.  I had not thought this possible!  My weight, however, is down.  By a lot.  I must make a very concerted effort at better nutrition, gaining weight, putting on muscle mass, etc.  I am even considering making another run at going on disability, though I still think that has more against it than for it right now.  But I cannot let work get in the way of taking care of myself.

Lastly, I'll note that I'm staring into a void I've long known about, but am now in a very real position to have to deal with.  My team at NY Presbyterian is going to have their monthly review meeting about their patients and actually bring my name up to be put on the list this time.  That may or may not happen.  Had my PFTs not improved, I'd say it was a certainty.  Now, maybe 65/45.  Even once listed, I'll have a very low Lung Allocation Score, since I am not on oxygen 24/7, but only with exertion and while sleeping.

Even standing here on this narrow ledge, I realize I have much to do before going over the side, with or without a successful lung transplant.  And the time I have left to do it in is getting so short.  And if I come out the other side in good shape, I have so much MORE to do then, too!  

November 15, 2012

I Get By With A Little Help From My Friends

A long, long time ago, in a state clear across the country, there used to be sleep-away camps for kids with cystic fibrosis.  (This was before the doctors figured out we were literally killing each other with cross-infection.)  Anyhow, the one I went to was called Camp Pilfeather and my memories of it remain clear and sharp even after all this time.  One of my favorite moments was at night, when the entire camp would gather around the bonfire and sing songs to guitar.  Lots of 60's music of course: Yellow Submarine, This Land is Your Land, etc.

A memento of that time which I kept for YEARS after was my camp shirt, which had rainbow-colored printing (yeah, hippies, I know) that spelled Camp Pilfeather on the front and on the back the motto: I Get By With A Little Help From My Friends.

I didn't fully appreciate the motto at that time.  In fact, haven't for most of my life, as I am generally an independent spirit and thrive on being able to accomplish things on my own that others would have to work together to do.  (Others might couch this in terms like "loner"; I prefer to say that I am a "future hermit".)

But times change, my health has changed, and my perception of and relationship to the very concept of relationship has changed.  This is not to say I'm becoming wholly dependent on anybody, but I am definitely enjoying the fruits of hard-won experience that my friends have and by whose example I profit.  My own path is illuminated all the better for the lights they've strung up for themselves.  And I am immensely grateful!