This post is mostly about Hypertrophic Cardiomyopathy (HCM), a congenital heart disease that I was born with. HCM is actually fairly common—at least 1 in 500 people have it, which is more than many well-known diseases such as Multiple Sclerosis, Lou Gehrig’s disease (ALS), or Muscular Dystrophy. When you hear of a young athlete suddenly dying on the playing field, the cause is almost always HCM.
A cardiomyopathy is disease of the heart muscle, distinct from coronary disease, which is disease of the blood vessels that supply the heart, typically caused by buildup of cholesterol that clogs the vessels. When a coronary artery is blocked, the result is often a cardiomyopathy caused by dying blood-starved heart muscle, a typical heart attack. HCM often causes symptoms that resemble a mild heart attack.
HCM is different. In HCM, heart muscle fibers grow thick and stiff. In my case, my heart grew in such a way that blood flow through my left ventricle was impeded. Eventually, I went to the Mayo Clinic where Dr. Werner Schaff stopped my heart for half an hour while he reshaped the interior of my ventricle by removing heart tissue. After the surgery, he told me the tissue he removed was the size of my thumb. That was in 2008.
When I returned to Whatcom County from Rochester Minnesota, I was weak, but I felt better. All my life, I felt palpitations, sessions after heavy physical labor, or for no apparent reason, my heart pounded in my chest to the point that I could think of nothing else. I assumed everyone experienced the same thing. I have not felt those pounding palpitations since the surgery.
In retrospect, I had a close call in the fourth or fifth grade. Custer, Central, and North Bellingham Elementary competed in a softball league. I was on the about the fifth string of the North Bellingham team playing Central, the elementary for the town of Ferndale. When there was no possibility of putting anyone else up to bat, I was at the plate. The Central pitcher threw a slow one, smack down the middle, no doubt because the chance that I would hit it was near zero. I saw it coming and swung. Miracle of miracles, I drove it into the outfield.
I started running. I made it to first and rounded toward second, the only time in my entire life that I have made that turn. About midway to second, my feet seemed to disappear from under me and I was engulfed in a reddish-brown fog. The next thing I knew, a crowd of kids were looking down at me and the umpire called me out. They pulled me up and I stumbled back to the bench, blaming myself for being so clumsy. So much for my baseball career.
Knowing what I know now about HCM, I believe I was lucky. Other young athletes have never left that brown fog. The most common first symptom of HCM is sudden cardiac death.
I made it until my early fifties. I’ve always liked bicycles. At that point in my life, I was riding a bike to work every day. We had bought a second home in Redmond, about six miles from the office. Most of my ride was on the Sammamish River Trail segment of the Burke-Gillman Trail, the bike super highway of the Seattle area. The trail is almost level, but I had a climb from the trail to the office on 128th in Kirkland.
I had a regular checkup with my doctor in Redmond. I mentioned that my chest ached on the uphill from the river trail. He frowned, asked me a few questions I’ve forgotten, ran an EKG, and shoved me across the hall in a wheel chair to a cardiologist. The cardiologist ordered an echocardiogram and pushed me into his echo lab. The technician performed an echocardiogram, the first of many. At one point, she called in the cardiologist to look at the screen. After a while, she gave me a cloth to wipe off the jelly she had smeared on my chest and told me to get dressed. She escorted me into the cardiologist’s office. A routine checkup had turned interesting.
The cardiologist told me that my echo-cardiogram was abnormal. He had not seen my condition often, but he thought it was idiopathic hypertrophic subaortic stenosis (IHSS), the old name for HCM.
I went through a series of medications, none of which seemed to do any good. The last straw was a tread mill echo-cardiogram that the cardiologist in charge refused to complete. I had a referral to the Mayo Clinic.
That was ten years ago last November. My heart has improved steadily after Dr. Schaff’s knife work.
Last Sunday, my friend, Bill Merrow, and I rode the historic Cascade Bicycle SR99 Tunnel Ride. I was, frankly, scared, thinking about sudden cardiac death and my aborted softball career, but I also felt like I could do it. Riding through the tunnel before it was opened to car traffic was a once in a lifetime event.
So, I tried. And succeeded. Bill was forbearing, tolerating my slow speed, being passed by 10-year-olds on rocket bikes and families pulling trailer-loads of infants. I stopped once, on the final ascent of the return trip through the tunnel to let my heart slow down and a few muscles relax, but I made it.
Folks with HCM, once they have made it through young adulthood, typically live as long as anyone else. That’s my plan.