Chapter 1
The Diagnosis
"This is a difficult diagnosis. You have cardiac sarcoidosis. We do not know what causes it, there is no known cure, and it is usually fatal."
My wife, Sharry, and I were sitting in the office of Dr. Robert Matthews in Annandale, Virginia. We were stunned. We had been prepared for bad news, but neither of us had any indication that a potentially deadly situation would be confronting us.
I was the first to break the heavy silence. "How long does some one with this disease have to live?"
Dr. Matthews folded his hands and spoke softly, "Typically two, maybe three, years." Neither his face nor his somber demeanor changed. "There have been instances of over ten years. On the other hand, you could experience sudden death in the very near term. It is impossible to predict, Ed."
I suddenly realized that I had been given the medical equivalent of the death penalty.
Sharry, who is a registered nurse, asked Matthews how he could be so sure. Her voice was remarkably steady, but I could tell from her eyes that she was obviously shocked. It was as if she had been punched in the stomach.
Dr. Matthews, still in the same calm voice, replied, "During the heart catheterization, we took five biopsies from your heart tissue. All of your pressures and flow rates were essentially normal, and the tissue samples were almost an afterthought while we were in there. One of the biopsies showed an abnormal cell structure, commonly caused by a disease called sarcoidosis. The hospital lab spent considerable time analyzing the sample. I have looked at the sample myself. It appears that the cell structure is consistent with a formation unique to cardiac sarcoidosis."
By now Sharry and I were totally stunned. We both now knew what Matthews had meant when he used the term "a difficult diagnosis." The difficult part had not been the biopsy analysis, but determining how to tell a family that one member has a fatal disease. The date was May 8, 1991.
As Sharry and I instinctively reached for the other's hand, our eyes met in mutual disbelief. It was obvious that both of our minds were racing to find further questions to ask Matthews. Sharry was the first to speak: "Tell us more about this disease. How does it affect the heart? Why is it dangerous?"
Dr. Matthews was sitting in his office in an expensive-looking chair with a very large desk separating us from him. Although we were at least eight feet from the doctor, his eyes seemed much closer and penetrating. He paused before giving us a rather detailed description of the mechanism of the disease and how it harmfully affects heart tissue.
Most of his words were rather meaningless to me, but Sharry, of course, understood much of what he was saying. Her face became ashen. She understood the gravity of our situation. Due to my engineering background I was able to form a layman's view of the effects of the disease on the heart, but it was only a general understanding of my condition. In the months ahead I was to become very knowledgeable about sarcoidosis (the term "sarcoid" has been used interchangeably by my doctors).
"Your situation, Ed, is extremely rare. I haven't been able to find any other case like it," Matthews explained. He was speaking slowly while rubbing his hands together slowly. He was definitely having difficulty with this conversation.
"Having sarcoid only in the heart and no where else....we just haven't seen this very often. Usually it affects several areas within the body - the kidneys, lungs, eyes, even your skin. Most of the time, the lungs are involved. That's a long term thing, and it generally doesn't kill you by itself. If the sarcoid is in the eyes, a person can lose sight. In general, the disease is not a killer. But when it gets in the heart....that's a problem."
I found my interest decreasing as he spoke of the typical effects on other body organs. At that moment I did not care what might be happening to people with sarcoid elsewhere in their body. I was still trying to handle the implications of my own situation. "How can this be happening to me?" I wondered. "I have been in perfect health, I have no noticeable symptoms, and I have never had any indication of a problem with any part of my body - especially my heart." Basically I felt cheated.
Sharry now asked what I also was thinking. "Dr. Matthews, is there anything that can be done?"
Her voice already had a touch of desperation to it. I was glad that she had asked, because I did not have the courage to ask a question for which the answer may be "No."
Matthews paused a moment, but it was obvious what his answer would be. He was simply searching for an appropriate way to tell us more bad news. Eventually he responded that there are no known treatments which would guarantee recovery, that sarcoidosis has been known to the medical community for nearly a century, and that the best method to show any significant effect was steroid treatment. He cautioned, however, that sarcoid remained a mysterious disease and that, even if it were to go dormant, damage to the heart would undoubtedly be significant due to scarring of the affected tissue.
Was there any good, or even semi-good, news available? I know that this was the thought in my own mind, and I suspected the same re action was hitting Sharry. The look on her face and the increased pres sure with which we were squeezing each other's hand told me that she was desperately searching for a silver lining.
Dr. Matthews broke the silence by turning around in his chair and picking up a stack of papers. "Both of you are intelligent. I think that it would be best for you to know as much as possible about this disease. I have made copies of several journal articles which have been written about sarcoidosis. I would like you to read them at your convenience. I also encourage you to feel free to use the library facilities at Fairfax Hospital to gather as much information as possible about your disease."
For some reason I became further upset by his use of the words "your disease." This nightmare at four in the afternoon seemed to be going from bad to worse. I chose not to press him on the life expectancy issue which he had stated earlier. At the moment I did not want to hear about probabilities or receive false hope.
After another long period of silence, Dr. Matthews told us that it would be very helpful to obtain a second, or even third, opinion concerning the diagnosis. Although we had known Matthews for only a month, he was obviously very concerned about our emotional state.
Sharry responded, "Whom do you recommend? Is there some one around this area? How soon should we do this?"
Dr. Matthews was well prepared for sharry's questions. He told us of two cardiologists in particular whom he recommended. Both had considerable experience in sarcoid diagnosis and treatment. "I have known both of them for years. Although we do not get many opportunities to see each other, I talk to them fairly often. In terms of cardiac sarcoid, they are tops in the field."
Somehow this discussion of specialists was reassuring to both Sharry and me. Perhaps there was an escape route away from the terribly bad news which we had been receiving from Matthews.
As I silently stared at the doctor, my mind was searching desperately for any avenue of hope, "Maybe his diagnosis is incorrect. Both of the specialists, one at Johns Hopkins University Hospital in Baltimore and the other at Duke University Medical Center in North Carolina, are sufficiently close for us to reach in a day. We'll go there. Maybe some-one having greater familiarity with sarcoidosis can give us a different diagnosis, perhaps something which is treatable. Or maybe they will know of some recently developed cure for sarcoidosis. Or maybe..." I was definitely grasping.
Dr. Matthews continued this somewhat optimistic hope for us by stating that he would telephone each of his two friends to brief them on my case and, more importantly, to ensure that I could receive an appointment in the near term.
Matthews then rose and requested that I accompany him to an examining room. Sharry remained in his office. She looked exhausted, but managed a smile as I left the room.
The examination was thorough, but unremarkable, in that nothin seemed to be different from previous ones by Matthews. In less than ten minutes we returned to Sharry. She again smiled at me as if to say, "Don't worry, we are in this together." I was too shocked to return her smile.
Following a brief period of rather uncomfortable small talk, we shook hands with the doctor, and left the building. Sharry and I had come to the doctor's office from our work places in separate cars, but we both walked directly to my pickup truck. Although we had not spoken, we were obviously very much in communication. Twenty years of marriage together will do that. As we sat silently on the bench seat in the front of the pickup, we stared intensely into each other's eyes. Both of us were obviously struggling to digest what we had just heard from Dr. Matthews. No words came out as we fought to hold back tears. Suddenly we lunged to hug one another.
My eyes were squeezing together intensely, as I felt both of us trembling. Sharry was the first to break the silence. "Don't worry, sweetheart. Somehow or other we will make it through this."
I was not as optimistic, but I did not voice my fear. I was 47 years old, in what I had thought was perfect health. I had recently run a marathon, and I was working daily as a high school teacher and coach. No serious problems had surfaced during physical exams during my 20 years in submarines in Navy nor in the six years since my retirement from the service. What could be happening? It all seemed so impossible.
We held each other for about 30 seconds, and then Sharry kissed me as she left to return to her car. She said that she would follow me home. I did not make an immediate move to place the key into the ignition. I just sat there stunned.
I did not realize at the time that I had just been placed on "Life Row." Instead of lawyers and judges maneuvering the legal system around a prisoner sentenced to death row, my family, friends, work colleagues, doctors, nurses and hundreds of others in the medical community worked with me in a process to save my life. This is the story, this is "Life Row."