Thanks For All The Fish

Fish. It started with fish.

I was diagnosed in 1998, went through a few years of mis-information and over-medication, then in 2003, Dr. Maron (in Boston) put me on a really good regimen that made me feel better than I had in a long while.

Whereas most HCM progresses gradually over a patient's life, mine moves in "stair-steps." I'll progress suddenly and rapidly, as I did with the onset of symptoms 1998, then suddenly "plateau" for long periods of time. The years between about 2000 and 2008 were a long plateau. Nothing changed on my echocardiogram, no increase in the thickness of my heart muscle, no change in my ejection fraction, or anything else to indicate that the HCM would get worse. Other than some stress-induced problems in 2005 (a story for another time), it looked like I was going to sail through life on a hefty dose of medications and no further complications.

I remember the exact "uh-oh" moment. It was the fall of 2008 and Christie had taken the kids somewhere for the weekend (Little Rock, Branson?) and left me at home. We lived in Bentonville, we were both in school and I had tons of papers and homework to catch up on. Plus I wanted to finish up a draft for one of my novels. I didn't want to waste time cooking, so I picked up a few of those TV dinners - not the microwave ones, the kind you put in the oven. I had been eating really healthy - homemade fruit juice, yogurt, and lots of raw veggies - and working out several times a week. So the TV dinners were a quick, nasty solution to my time crunch.

After eating the first sodium-laden fish dinner, I sat at the computer unable to work. Waves of nausea swept over me unlike anything I'd experienced before. I thought I had food poisoning, but as the night went on, I realized the pain was coming from my chest, not my stomach. It's tempting to think my body was reacting to the faux-food to which it was not accustomed, but it's clear to me now that my HCM simply chose that moment in time to progress again. Salt, stress, trans-fatty acids, exercise - HCM sneers at all of these things, then ignores them. As far as the research has discovered, HCM is on its own schedule and can't be hurried along or held back by anything external. This is hard to wrap our brains around because we've been bombarded with the idea that exercise and Cheerios keep your heart healthy. They probably do, for people with normal hearts. But you can't make HCM better with exercise or rest, and you can't make it worse with fried fish and beer. So really, the fish had nothing to do with it. That was just a clever title to make you read this and HERE YOU ARE.

Remember when I said that HCM is sneaky? It's unpredictable and inscrutable too. Most HCM literature mentions the risk of sudden death, but that's not as much of a danger now that we know a little more about the disease. The solution usually involves medications and, in the case of patients who are truly at risk for sudden death, implant devices such as defibrillators or pacemakers. Devices shock the heart back into a normal rhythm before anything really bad happens. In worst case scenarios, heart surgeons perform a myectomy or septal oblation in which they literally cut or burn away some of the stiffened heart tissue to prevent blockages in the heart and make its job easier. Any of these methods can typically "fix" the HCM symptoms enough that the HCMer can live a normal, if less than active life.

We've been told that HCM makes up 5% of all heart disease in the world's population. Of that 5%, I am part of the "less than 1%" for whom HCM progresses to the point where none of the above work. I have no electrical/disrhythmia problems, so implant devices do nothing for me. The only solution at this point is transplantation. It's so rare in fact that on both the HCM wiki article and in the HCM handbook written by my doctor's father (Dr. Barry Maron), it warrants one single sentence.

Aggressive HCM like mine presents considerable danger because on any given day, there can be progression, or loss of heart function - meaning that over time, the heart muscle becomes a little more stiff and inflexible, and is no longer doing its job. On top of that, because it is so rigid, it makes it that much harder for the remaining healthy muscle to do its job properly. A heart MRI last year shows that over the course of 2010, my HCM progressed to the point that I have very little functioning muscle left. Fortunately, I have "plateaued" again since that time - but who knows when the next big stair step will be? It's possible the rest of that muscle will lose its function very quickly, or that it could take years. That makes it really hard to plan much of anything, and I'm sure we seem like real flakes sometimes. But when your health changes so randomly, all you can really do is adapt and hang on.

Edema is the other serious risk. After the fish, I noticed that I suddenly felt very heavy. I never weighed on the scales because I've been skinny my whole life. I found that I had no energy to work out, food didn't taste good anymore, and I was developing a beer belly, despite never drinking beer. I started checking out the scales - I'd gained almost 20 lbs. in just a few days. My local cardiologist in Bentonville, AR told me I was just getting older and fatter. I started having searing pain in my lower back - so bad that Christie had to come home from school to help me get up from my desk chair one day. I suppose this was my liver and kidneys trying to deal with the massive amount of water weight I'd taken on. As the disease progresses, diuretics won't work much at all, and there is danger of flash-pulmonary edema - drowning in your own fluids. In the ER, there is the danger of being given too much fluid or too much diuretic, which can collapse the heart vessels causing immediate and lasting deadness.

The problem is that all of this looks very much like congestive heart failure to even an experienced cardiologist. CHF is so common in HCM patients that I've had doctors correct me when I tell them I'm in diastolic heart failure. They check for water on my ankles and ignore the tire around my middle. Even at an HCM clinic in St. Louis, Missouri, I was told that my cardiac output was fine and I would never need a transplant. It's exhausting to spend so many years knowing more about your disease than the doctors who are treating you.

This is all very House, if you know what I mean. Everyone thinks their medical problem is unique. So much so that doctors expect patients to act this way. I've had a lot of difficulty accepting just how rare my condition is. I don't even fit in with the HCM community, a community that identifies itself by the obscurity of its disease. This has driven us finally to realize that the cardiac team at Tufts (in Boston) are pretty much the only people who have seen anything like it before, or have the resources and knowledge to deal with it properly. Even then, I'm different from the other HCM transplant patients. Heather Cote' the transplant coordinator, took the time to go through the transplant handbook and point out all the things that didn't apply to me. It was a lot of stuff. If I hear the word "unique" one more time I might throw someone through a plate glass window.

So there's no community, no handbook, no one to really talk to about how this thing will go and what to expect. I'm sure there's someone out there, but it's very needle in a haystack.

I share all of this because, even though our families are probably sick of discussing it, many of our friends don't understand why Boston, why now, why so suddenly. Wasn't everything fine just a year ago?

The other bullet ricocheting around the room is the uncertainty of the next progression. We're headed for Boston to get on a transplant list, but what if the disease has decided to plateau for the next 8 years? I'll sit at the bottom of the list I guess.  All I know is that when I have the really bad days, I feel like I might be dying right then. I know that will happen more often and more intensely as my heart wears out, so I'm getting on a list now.

Still, getting a transplant is not the cure all it sounds like. I fully expect it to be an awesome new beginning, but the whole thing is very different from the way it looks on Grey's Anatomy. Actually, humans and life are very different from the way they look on Grey's Anatomy, but I digress...

Next post: Transplants, and why movies about them are stoopid.