Okay, people are rightly annoyed/concerned over my recent paucity of postings. Here’s what happened.
To bring newcomers quickly up to speed, I have Cystic Fibrosis and the last few years have been something of a roller coaster ride. At one point, I had even made it to the top of the waiting list for lung transplants, which is pretty serious business. Anyway, as I’ve written in the recent past, I’ve been doing much better over the last several months.
Roughly two weeks ago, I had a regularly scheduled check-up. For people with serious conditions, such appointments are moments of truth – you take some tests and get some news. You hope it will be good news.
Going into this appointment, I was confident. I know my body well, and am usually not swayed by even by my most ardent efforts at self-deception. In other words, I felt well and was not just hopeful but confident that the tests would bring good news.
As regular readers of this blog know, I spend more time being right than being wrong. So if you were expecting this introduction was a lead-up to a boy-was-I ever-wrong-and-got-a-crushing-blow moment, you should be kicking yourself. Not only did the tests turn out well, they turned out better than I could have possibly expected. It turns out that I’m in the best shape I’ve been in for five years. Not by a little, but by a lot.
WHEN YOU HAVE A CHRONIC AND PROGRESSIVE disease, you really don’t expect anything like this to happen. So, inquiring minds must be asking, how did this occur?
It’s actually a pretty good story. In the interests of space and keeping this remotely interesting, I’ll go easy on the scientific stuff.
Some time probably about 18 months ago, a CF doctor in Australia noticed that his patients who were surfers were far out-performing his patients who weren’t. Although the mechanisms that make CF such a destructive disease aren’t completely understood, it is known that the root cause of CF’s problems have something to do with the patient’s inability to process salt.
As a matter of fact, the test to see if someone had CF was, in the days before genetic screening, called a sweat test. A few electrodes were attached to the patient’s arm to generate a little swath of sweat which the guys in white coats then analyzed for its salt content. I may not have the number exactly right, but CF patients have roughly seven times the amount of salt in their sweat as normal people do.
Anyway, back to Australia. The CF doctor there had a theory that his surfer patients were thriving because of their prolonged exposure to salt water. This gave him an idea – he wanted his patients to inhale an aerosolized form of salt water a couple of times a day.
In this tiny Australian study (fewer than two dozen participants), the results were amazing. The patients who inhaled the hypertonic saline (the SAT-word way of saying “salt water”) showed markedly increased lung functions. More importantly, they showed a dramatically decreased rate in exacerbations.
Word of this study soon spread stateside. In North Carolina, a larger study was done that showed a similarly dramatic decrease in the rate of exacerbations but no statistically significant increase in lung function.
This is when the news of the hypertonic saline treatment went public. In mid-January, there was a story about the Australia and North Carolina trials in the New England Journal of Medicine. For those who don’t read the New England Journal of Medicine, the story even made it into WebMD, CNN, and a certain major New York newspaper whose name I refuse to type.
Naturally, the New England Journal of Medicine editorialized that we needed to know more about the treatment and that no one should run off half-cocked eager to suck down some salt water. (Obviously, I’m paraphrasing.) Reading about all of this in Southern Soxblog Manor, and after talking about it with my doctor, I made a few inferences.
First, I had known from previous studies where I had inhaled a placebo that even inhaling a placebo has a positive effect on a CF patient’s lungs. The name of the game in CF well-being is airway clearance. We have lungs filled with junk; if we get that junk up we feel okay. I knew by empirical first hand knowledge that any kind of inhalation treatment provokes airway clearance, which strikes the rest of you as us coughing our heads off.
Additionally, my doctor told me that sucking down this salt water was a vile treatment, one that even hard-core CF patients like myself had trouble complying with. This got me to making some conjectures as to why there were the differing results between North Carolina and Australia. In Australia, the study was smaller so patient non-compliance with the program would be easier to address. Additionally, perhaps because of their surfing experience, the Australians found the treatment more tolerable. Either that, or their flinty Down Under nature made them more likely to stick with it than their American counterparts.
Whatever the reason, the differences between the Australian and American results struck me as an obvious case of a patient compliance disparity. In sum, I felt the porotocol, if followed, had an excellent chance of being extremely helpful. What’s more, being just salt water, there was no way it could be harmful.
IN MARCH I FLEW UP from Florida to Boston and became Massachusetts General Hospital’s first patient to begin the hypertonic saline regimen. The stuff came as advertised – inhaling it was awful. I compared it to inhaling an aerosolized version of lox. Also, it took forever – almost half an hour, twice a day.
Now remember, I was already starting this additional treatment from a relative position of strength. I was doing as well as I had in a while and was feeling good. But shortly after starting the treatment, I began doing better – a lot better.
I hadn’t done any medical tests to support this feeling, but I felt more energetic than I had in memory. Lifting weights, I felt almost the same in the gym as I had when I was in my 20’s. People who hadn’t seen me in a while almost uniformly commented that I looked extremely healthy. While people always say that to sick people, even if we look like death warmed over, I could tell that in these instances they meant it.
Two weeks ago I went to the doctor’s office to get the numbers. At these appointments, you blow into a device that measure how well your lungs are functioning. While they always say when the numbers stink that you shouldn’t worry too much, when you’ve been around this stuff for long enough you know the numbers don’t lie. For the past five years, each appointment like this has given me the same I feeling I had on the morning of the SAT’s.
I wish I did as well on my SAT’s as I did on these lung function tests a couple of weeks ago. As I said up top, my lungs are now in better shape than when they began to significantly deteriorate five years ago. I’ve reclaimed a lot of lost ground.
From my perspective, this seems like if not a miracle, something damn close to it. For years good people, the world’s best, have been pouring their hearts, souls and money into finding effective treatments for CF and generally coming up with very little. And all of a sudden, a super-promising new treatment comes along. And it’s salt water!
There’s also the additional minor miracle that being just salt water, the treatment didn’t have to spend the better part of a decade navigating the FDA approval maze before reaching the general CF public that so desperately needs it. If a similarly effective treatment had been a medicine hatched in the labs of Genzyme, the FDA would have kept it out of the hands of the seriously ill people (who would eagerly roll the dice on an experimental treatment) until those seriously ill people had become seriously dead.
As an additional benefit, because it’s just salt water and thus cheap to make, no one will make much money off of it which means the CF community will be spared the ghastly sight of our nation’s Ted Kennedy types demonizing the pharmaceuticals who just pioneered a life-saving treatment for being profit oriented.
In other words, everyone wins!
SO WHY THE LIGHT BLOGGING? Fair question, and a not obvious answer. I’m almost 39. For a few years, I had considered myself fairly well along in my golden years. I had made peace with that fact, and had allowed myself to truly enjoy the good times I had left. I was also dealing with my regrets and disappointments, addressing those I could, letting lie those I couldn’t.
Two weeks ago I got the shocking news that I may not be so far along in my golden years as I figured. I may get a second crack at bat. The situation would be analogous to a 70 year old in declining health going to the doctor and finding out that not only might he make it to 80, his 70’s will be a lot better health-wise than his 60’s were.
This is incredibly thrilling news. It’s also daunting, and a bit disorienting. This news has turned my life as I knew it upside down. Given that my “right-side up life” represented serious illness followed by imminent death, there are a lot worse than things having such a life turned upside down.
I may be in the process of receiving a remarkable gift. Although it probably seems like an ingrate’s reactions, I have felt considerable self-induced pressure not to blow it and to use it wisely. I’ve been very promiscuous with rhetoric the last few years that I wish I had a fraction of the health and energy I did when I was young now that I have the knowledge of an old man. Woops! Looks like I’ve sort of backed myself into a corner.
But it’s a happy corner to be in, especially when the alternative was a pine box. While I’m not over my minor existential crisis, I am back to writing. That’s what I most want to do with this amazing second chance I have. I think it’s where I have the most chance to accomplish something meaningful and lasting. It’s also something I love.
In short, Soxblog is now re-opened for business.
Responses? Thoughts? Please email them to me at firstname.lastname@example.org