05.21.07
Hypertrophic Cardiomyopathy
This is a long and possibly boring story of my (John’s) journey with this disease called HCM. It’s not really cathartic for me, since the disease is more of an annoyance than anything else. But, I wanted to get it all down for the record and also to potentially help anyone else who may have experienced similar symptoms.
I made an appointment to see my family doctor in August of 2004. I was having trouble sleeping, because whenever I found myself in a prone position, my heart would start beating in a strange way. It was not beating too fast. Rather, it was beating too hard, or over compressing. It seemed to maintain a good rhythm and speed; it just produced these crushing contractions. I had been sensing this for some time, and finally by providence I decided to have it diagnosed.
I described all this to the doctor, and he told me that I was most likely feeling PVCs (pre-ventricular contractions). He hooked me up to an EKG and showed me a few PVC occurrences and reassured me that this was pretty normal for someone my age (39). I felt like he was a bit dismissive, but he suggested a treadmill test. I agreed and took that test in September.
I didn’t get very far on that treadmill, being quite out of shape. However, the readout did not reveal anything abnormal. My doctor said I need not worry, but that if I wanted he would order a nuclear treadmill. I was tempted to drop the whole thing then and there. I mean, who likes these tests? But, something told me – probably the Spirit of God himself – that I should go and get this next test. I was being prompted that this whole ordeal was not quite over. I communicated all this to my doctor and he said, Well, I hate to do a 180 on ya. But, I’ll justify and order the test.
Now, a treadmill test is around $250 dollars or so. A nuclear treadmill test is over 10 times that money. Once on this road, it’s hard to get off. The money pit looms pretty large and insurance or not, it creates stress. It probably aggravated my already stressed septum. Anyway, I digress.
I had the nuclear treadmill early in October and the results showed some abnormality. From that point on, my family doctor handed the case over to a cardiologist. This guy was fairly young, but he had an inkling of what was really going on in my thoracic cavity from the first few minutes of reviewing the treadmill data. He said, “Follow me, and take off your shoes.” We wandered into the room with the fancy ultrasound machine, and he did a quick impromptu test. He said, “I think you may have hypertrophic cardiomyopathy.€” He was wanding around my chest with the probe for just a few seconds. Then he says, “And…yes! You have it!”
He explained to me a bit about the disease, and had me return later that day for a full echocardiogram. I went back to work, and began to surf the net for information on HCM. I received a modicum of education, but not enough to fill the gaps regarding the seriousness of the condition.
After the full echo, the whole place was a bit panicked. My septum was 45mm which was quite exaggerated against the normal 10 mm measurement. I visited the EP (electrophysiologist) and he said I had to get in for a full EP study soon. The cardiologist wanted to do an angiogram as well. I was honestly starting to get a little freaked out, but remained unafraid.
I was scheduled for the whole ordeal late in November. The EP explained that if I did not get good results from the EP study, that they would install an ICD right then and there. I was quite adverse to this idea. I felt like if I had this machine in me that it was a mark of weakness and that it would somehow make me less of a man. Plus, I was still pretty stressed out about how much all this was going to cost. In the meantime, I visited their office twice more for a Holter monitor test. The cardiologist put me on a 50mg dose of blood pressure medication. Although my blood pressure was and is normal, this drug allows the heart to relax a bit and eject a greater volume of blood from the left ventricle. I feel pretty normal when taking this particular medication, and I don’t have the PVCs or “crushing beats” as much.
On the day of the surgery, the angiogram was first, and that test went very smoothly. I had just turned 40, and my arteries were totally clear. (This was good news, since my triglycerides are very high, and plugged arteries seem to run in the family.) The worst part of the experience was having the nurse put her full weight on my thigh, pressing with her palms, as she did her best to prevent a hematoma. She did this twice, 15 minutes each time. That was not comfortable to say the least.
The EP study was in the same room and a bit more intense. I was splayed out naked on a cold table and restrained. This makes any person nervous, but I was trying to remain calm. They tell you to not eat the whole day since a nervous stomach makes for potential episodes of vomiting, and nobody wants to deal with that in an OR. They applied defibrillator pads which are adhesive to the chest and about a dozen transducers for monitoring. They numbed my upper left thigh and inserted 3 separate catheters. They started an IV and gave me a very small amount of sedative to keep me relaxed. It’s important to stay very still during an EP study.
Sedated, but very much awake, they began the test. I did not feel much of anything. There was a nurse right by my side watching my overall condition. I could see the same heart monitor that she was viewing. The doctor at this time is in another room with a bank of computers that perform the stimulus and monitor the response.
After 30 or 45 minutes, I noticed the monitor change from the normal rhythmic blips into a VT pattern. I recognized this since I had been researching the whole condition now for over a month. I said to the nurse, “Oh, there’s VT, right?.” She said, “Yeah, OK, now start to count backward from 10.” I got to about 7, and passed out. Then, they hit me with the 200+ Joules on the defib pads, and I felt it. I don’t think they let me pass out enough, since I was pretty sure that a large TV monitor mounted above the table had come off the stand and landed on my chest. I groaned and lurched hard. The nurse slammed me back to the table. I was conscious enough to figure out what happened but before I could get angry, they upped the sedative and I was out for the duration. Apparently they want you awake for the first VT/VF, then, thankfully, you get to sleep through the rest of them.
It’s funny how insistent they were that I did not feel the wicked discharge from the defib pads. I have a very lucid memory of it. It’s like being hit with a 2×4 across the chest. It’s more shock than pain. It’s somewhat hard to describe, but I did feel it, no matter what they say. The EP said he put me into VF about seven times in total. It was pretty bad, so they put in the ICD. They had trouble getting the wire situated, so they had to pull it and do it again. I finally got out of there about 6 hours later. My family was getting kind of stressed out since it was taking way longer than it was supposed to. But, everything was OK and I felt fine save the soreness of the incision. Later, the next day they decided the wire was still not quite right. So, they opened me up again and adjusted the wire for a third time. I was not that happy, but at that point I was worn out and pretty submissive.
They let me go home the third day. I had a large arm brace to prevent me from raising my left arm and upsetting the wire lodged in the bottom of my heart muscle. I could not drive for 6 weeks and I took a couple of weeks off of work. All in all, the impact was minimal. By January, I was installing a garage door opener for my mother and felt fine. I am continuing on the blood pressure medication to keep the heart “quieter” as it were, and most days and nights I feel normal.
The thing about the ICD is that is does nothing about the condition itself. The condition presents two main problems, an electrical problem and a mechanical problem. I feel the mechanical problem, but it does not impede me in any way. I still can do virtually anything, except get a pilot’s license or play contact sports. If the mechanical problem does actually begin to surface (in the form of dizziness, fainting, fatigue) they can do a procedure to limit the size of the septum. It’s a spooky procedure, where they ablate the septum with alcohol so that part of it dies, and atrophies. This allows more blood volume in the ventricle. They do it at the Mayo Clinic, and only a few other places, so it’s not a trivial procedure.
The electrical problem if manifested usually results in instant death. The signal propagation through the heart is upset, and the heart will not beat. It will just quiver, and move no blood, so death is pretty quick. There is nothing anyone can do to permanently fix the electrical problem or the potential thereof. Yet, a small computer can analyze the heart actively and determine if the rhythm is normal or not. If the rhythm is abnormal, the computer can deliver a shock. This is the wonder of the ICD. It has a small computer and a bunch of capacitors and can send up to 30 Joules (a Joule is one watt delivered for one second, or one volt, with one amp, delivered for one second) right into my heart. It’s also smart enough to know if the shock (known as the “therapy”) worked and if it should try again or not. That to me is pretty remarkable.