Living with IGCM (Idiopathic Giant Cell Myocarditis)

I count myself as lucky--most people with this diagnosis would have a heart transplant by now. My cardiologist attributes my better condition to a variety of things: 1)it was caught "early" in the progression of the disease; 2)I got onto the immunosuppressants and steroids quickly; 3)the disease seems to have focused mostly on my right ventricle and although it is probably present in the left ventricle, has not caused too much damage to the heart's pumping function (making me a unique case among rare cases), and 4)the progression of the disease has not been rapid and extensive as most other cases seen by my cardiologist.

I am doing what I can to keep my current heart. This means keeping stressors on my heart low: low sodium, low extra fluid, low sugar intake (the high steroid doses are screwing with my blood sugar levels), mild exercise, regular medication regimen, and reduced anxiety/stress levels. Lots to monitor and regulate.

I go back in this Friday, April 21, for another cardiac biopsy of my right ventricle tissue. Dr. Smith hopes to get some samples that will indicate the current condition of the IGCM and whether things have improved...or stayed the same...or whatever. Since the last biopsy landed me in the hospital, I intend to take stuff with us in the car so that if I get admitted to the hospital again Janie won't have to travel back and forth with everything.

I am beginning to read a book I picked up from Amazon by Naparstek on Guided Visualization and Imagery for Self-Healing. Because of my background in biofeedback and self-regulation I know that it's possible to make connections between mind and body. I'm trying to learn more about how you "connect" with an immune system and help "re-regulate" it. I don't want my immune system to shut off, and I don't want it to get stronger and continue to attack, I want it to re-direct itself so it doesn't attack my normal, healthy heart tissue. Most MDs don't see autoimmune disorders as something that "turns off" over time, and Dr. Smith envisions I'll be on immunosuppressants and steroids into the far future. I'd rather see what I can do about making that an option rather than a requirement.

My ICD can be shocking!

On Thursday, March 30, I was trying to get a few boxes moved and organized in my basement. I wanted to locate some important materials and belongings that were still hidden away in boxes from our move. I didn't feel overworked or taxed--although I was expending more energy than usual--and I was feeling OK. While I was simply standing and looking over some materials I had on a bookshelf I got a strong shock from my ICD unit. It felt like someone had taken a fist and slammed it into my chest about mid-sternum, only it felt more like it was coming from the inside out, rather than from the outside in. It was scary, disorienting, and unexpected.

The Arrythmia Center had told me to call them if this happened, so I did. They said if it were to happen again I was to come into their center right away. I took the rest of the day off of work and kept myself relaxed and calm. Luckily I have not had another shock since that day (cross my fingers). On my next echocardiogram on April 3, the pacemaker technician interrogated the chip in my ICD and discovered that I did get a 5 Joule shock that day, and that my atria (not my ventricles) were in a very fast rate, making my ventricles speed up to the point that the ICD tried to pace the ventricles to a slower rate, could not, and then administered a shock. The good news is that it was not the ventricles that generated the need for the shock, but the mediocre news was that the atria could suddenly get into a fast pace and push the ventricles into a higher pace. Dr. Smith felt the ICD was fine as is, and still is playing a protective role regarding my ventricles and their rhythm. Good information from my April 3rd echocardiogram: my echo "looks good," and my heart's pumping function is still good (ejection fraction is still at 55%).

In the last couple weeks I have also noticed I am more prone to water retention, especially if I get additional salt in my diet. My weight can fluctuate by a few pounds of water a day if I am not careful with my salt and fluid consumption. I have had an episode of pitting edema in my legs one weekend when I sat for too long and fluid accumulated in my lower legs. It took sodium and fluid restriction over the weekend to get my weight back down. Pitting edema is when enough fluid accumulated in my legs that if I press into the skin and then remove my thumb there's a small depression (pit) remaining where the fluid was pushed out. The pit gradually refills as fluid comes back into the tissue from surrounding tissue. (This is why I am following a "heart failure" diet with restricted fluids, low sodium, etc. My dad and I have more in common now with our diet than I thought would occur...

In a Medical Limbo... and my next Cardiac Biopsy

Since my diagnosis of IGCM (Idiopathic giant cell myocarditis) on February 14th, I have been back to my cardiologist for echocardiograms on Feb 21 and Feb 28. The good news was that my heart function was still strong and that I had not progressed into a worse condition. It appears that the drugs and medical regimen I was following were beneficial. The more negative side was that I was having some side effects associated with all the steroids and other drugs.

On March 13th I went into Emory Clinic for a cardiac biopsy. Dr. Smith did a local anesthetic on my neck, inserted a snake-like device into my jugular vein that he moved into the right ventricle, and then gathered small samples of heart tissue from my heart. While I was resting afterwards, my heart decided after about an hour to start having spontaneous tachycardia events (up to 155 bpm) that would occur for about 45 seconds and then resolve on their own. This would happen every few minutes. They decided this was not good, and so I was again admitted to the hospital--this time Emory University Hospital--for observation. They wired me up with EKG telemetry and began monitoring my heart rhythm. Over the next few days they found runs of Premature Ventricular Contractions (PVCs) up to 21 in length, started me on a beta-blocker (Coreg), and began upping the dose to regulate my heart and the possible PVCs. PVCs can be dangerous because if they don't resolve I can go into ventricular fibrillation (this is where blood is no longer being pumped by the heart), and go into cardiac arrest. However, after a few days of observation, a new prescription of Coreg, and knowledge that I do have an ICD device now monitoring my heart, they discharged me from the hospital on March 16. The ironic part is that the cardiac biopsy results came back inconclusive--all 3 samples had essentially scar tissue only and no clear indication whether the IGCM was better, worse, the same, or whatever.

My Valentine's Day Diagnosis

I went home from the hospital and pacemaker installation on January 28, not knowing why my heart was in the condition it was. My meeting with my cardiologist, Merlino, on February 3rd, didn't provide any further explanation on why this had occurred. None of the tests he'd done came back positive for any problem he'd checked. One cardiologist thought I might have ARVD (Arrythmogenic Right Ventiricular Dysfunction), a genetic disorder.

On Thursday, Feb 9, I felt bad. My heartbeat felt fast and irregular. I called up and scheduled an early meeting at the Arrythmia Center at Crawford-Long Hospital. I guessed something was wrong when the pacemaker technologist electronically interrogated my pacemaker and then the next thing I knew there were 2 more nurses and a couple doctors that quickly filled the room. The lead doctor informed me my heart was in ventricular tachycardia at 160 bpm and that was not good. However, using the pacing abilities of the pacemaker, they got it back into a normal rate. They admitted me to the hospital.

In the next few days I went through a right ventricular catheterization, a cardiac biopsy, and they changed out my pacemaker for an ICD (implantable cardioverter defibrillator) that could not only pace my heart but also deliver a strong shock to get it out of an arrythmia if need be. (They wanted to make certain if my ventricular tachycardia progressed into ventricular fibrillation that I would be protected/prepared.)

On February 14, Valentine's Day, my team of doctors visited me and gave me the results of the cardiac biopsy. I did not have ARVD, but Idiopathic Giant Cell Myocarditic (IGCM). Basically, my own immune system was attacting my own heart muscle. This disease is characterized by the fusion of white blood cells into giant cells (multiple nuclei within the giant cell), with these giant cells attacking the heart muscle myosin fibers. I would need to go on immunosuppressants to reduce my own system's response, high doses of steroids to reduce the inflammation of my heart, and various drugs to reduce heart stress and the side effects of the other drugs. I started reading about IGCM and didn't like what I read. A 1997 study indicated that about 60% of people diagnosed with IGCM had to have a heart transplant (or died) within 6 months of diagnosis. About 20% of patients were able to stay on the drugs and keep their original heart out to 5 years (the length of the study). My new cardiologist, Dr. Andrew Smith, the leading authority in Atlanta and Georgia, began meeting with me. As the leading authority, he did say it's fairly rare: he has seen 5 cases in the last 14 years. A colleague of his in Alabama has seen about 8 to 10 cases in the last 20 years. So there's not a lot of cases to build upon. He would be closely monitoring the status of my heart using echocardiogram and cardiac biopsy into the future, and we would prepare for a possible heart transplant. He was optimistic, though, because my heart's pumping function was still strong and I was not presenting as a fulminant case (full blown, rapidly deteriorating heart disease). I was released from the hospital on February 16.

My Trip to the Emergency Room

The next day, Friday, January 27, I did take 1 atenolol as prescribed in the AM. I did have some shortness of breath when walking up a hill to go from one building at work to another so I took 1 nitroglycerine tablet as recommended. These medications, in combination with others is was taking, may have precipitated my trip to the emergency room at Crawford Long Hospital. By noon I had difficulty standing without getting dizzy. I walked out of a lunch meeting to answer my cell phone and couldn't stand up from a chair to get back into the meeting. I managed to take the elevator to the lobby so that if I fainted I would be somewhere where others would see me. I opened my cell phone and punched in 911, contemplating the scene I was about to create in the main lobby of Northrop Grumman. One of my colleagues, Alexis, who in a previous career was a nurse, came into the lobby and asked me how I was doing. I told her the truth and she sat down and talked to me. She agreed it was time to activate 911 and had the lobby guard call for me. When the ambulance staff arrived, they checked me over and found my pulse was 35 and BP was 80/40. Not good for my body size. They put me on a gurney and took me to the ambulance. While on the way to the hospital (no sirens) I was getting major dizziness spells and slipping in and out of consciousness. I remember being wheeled into the ER, and then I remember waking up in what looked more like an operating room with about 5 or 6 people around me. I tried staying conscious and answering questions as best I could, but I remember coming back to consciousness a few times during that time.
I next remember what can be described as torture. I was shocked awake by what I discovered were external cardiac pacing pads. They were shocking my heart through my chest wall into contracting on a regular basis as they were wheeling me to an operating room where they were going to install an internal pacemaker. With the electrodes going off (what seemed like) every second or two, I felt myself bracing for the shock and muscles also tightening when the shock did come. It was such relief when the internal pacemaker was actually in place.
It turns out I had gone into complete heart block and my atria (upper chambers of the heart) were not working in conjunction with the ventricles (lower chambers of the heart). The pacemeaker did help regulate my heartbeat, but they had no idea why this had happened. I was actually discharged the next day from the hospital with a new pacemaker and newfound feelings of mortality.