Annals of Internal Medicine
© 1996 American College of Physicians

My Not-So-Near-Death Experience
[On Being A Patient]
Hill, Lawrence N. MD

Manila, Philippines.

Ann Intern Med. 1996;125:855-857.

I am a hypochondriac. My medical degree offers me no immunity to this common ailment. My case is not a bad one, but I have it nonetheless. As a practicing oncologist for nearly 20 years before taking a generalist's job with the government, I empathized with my patients and became convinced regularly that I, too, had a malignant condition. Once I went so far as to walk into the reading room of my good friend, the local radiologist, and admit that I thought I might have male breast cancer, metastatic to bone, because I had discomfort in both the breast and the anterior knee. The knee films were negative, and the symptoms immediately resolved.

This time it was different. About a year ago, the fasciculations started. At first they were mainly in my calves and occurred after tennis or similar vigorous activity. At this stage, I passed them off as insignificant. But soon they began to spread, first up my legs, then into my torso and my arms, and finally to my face. Night and day, at rest and after exercise, they were there. Thirty seconds rarely elapsed without a twitch somewhere.

Oncologists don't often see amyotrophic lateral sclerosis (ALS), but the memories gleaned in medical school of twitching, bedridden patients with tracheostomies were still vivid. Incurable. Untreatable. Invariably fatal. I remembered those words and phrases, but I certainly did not recall reading of any recent positive developments. I became more than a bit frightened. Previously, my thoughts of impending death had never lasted for more than a few hours, but these persisted from days to weeks to months. I was able to function; I continued to see patients and dealt with them in a professional, competent, and caring manner. Between patients, however, my thoughts turned to my own body as well as to my family and friends, who I might soon be forced to leave. I thought a lot about the physicist Steven Hawkings and figured that if I were lucky, I, too, could live a long time in a wheelchair with at least the use of a single finger. I wondered whether I could afford the special computer that allows Hawking to translate his finger movements into written and audible words.

I was living in southern Asia when the fasciculations began. I had almost no access to the recent medical literature, and the country had no highly skilled neurologists. Even if it had, I was not about to go to a neurologist. As an oncologist, I always thought it best that pancreatic cancer be diagnosed after death. In my opinion, ALS is the neurological equivalent of pancreatic cancer. I was willing to sacrifice the distinct possibility of learning that I did not have this fatal illness to avoid the equally distinct possibility of learning that I did. The anxiety of not knowing was preferable to the despair of knowing the worst.

I did have an old copy of Brain's Neurology. As I recall, it said something like "most fasciculations are caused by motor neuron disease," but it also said that "rarely do the fasciculations precede weakness." I had not had any real weakness that I had noticed, but that made me decide to test myself. I hadn't done a push-up since my Army days in 1959. I did 15 the first day, and 1 more every day for about a month. I was no Olympian, but I did well enough. Using the rheumatologist's simple test of grip strength, I inflated the bag of the sphygmomanometer to 20 and squeezed it; I moved 300 mm Hg without trying too hard. Every day I repeated this exercise, sometimes a few times--the result was always 300. I was certainly not grossly weak. Wasting occurs with ALS, especially in the hands. I spent an inordinate amount of time examining my hands. I once saw a fold in the hypothenar eminence of the left hand that I hadn't remembered being there before. I returned to the squeeze bag: Still 300, but who knows, maybe, in my case, the wasting would precede my paralyzed slide into oblivion. I tested my reflexes, looking for the hyperreflexia that accompanies motor neuron disease. Perhaps that knee jerk was a bit brisk, I would think, and maybe those hand cramps that I get when I use chopsticks aren't meaningless after all. My anterior horn cells began to dominate my thought processes.

Two or 3 months later, my tour of duty in southern Asia was over; several weeks of home leave in California were to precede my resettlement to southeast Asia. Obviously, California has many neurologists, including some of my old friends. I had decided not to seek an appointment with any of them. Why, I asked myself, should I have an electromyography if my outcome will in no way be affected by the results? I had also had enough experience in my medium-sized community to remember the community's response when one of its physicians developed a life-threatening illness. I had seen medical confidentiality break down in this kind of situation as often as it was maintained. I did not want to be defined by my illness; I did not want to be known as Dr. Hill, Who Has ALS.

I did more push-ups and played lots of tennis; there was still no weakness 6 months later. I didn't have a pressure cuff, but I could still open the jelly jar when my wife couldn't. Even with these good signs, the twitching hadn't gotten any better; maybe it was a bit worse. I was pretty sure of the diagnosis. I became neither depressed nor angry. I became obsessed but, surprisingly, kept my obsession almost exclusively to myself.

Cal Ripken was about to surpass the record of Lou Gehrig. I was on a plane between Los Angeles and Washington, D.C., and the man next to me had a copy of USA Today. I saw a headline about Gehrig; the subheadline alluded to a new drug for the disease that he had made famous. The New England Journal of Medicine or Annals of Internal Medicine it wasn't, but I was prepared to read anything that might give me hope. Eventually, I mustered the courage to ask my neighbor if I could just borrow the sports section for a minute. The article disappointed me; it contained nothing of clinical significance.

My usually imperturbable wife, who is well aware of my hypochondriasis, had ignored my initial undramatic lamentations, but when she saw enough of the twitching she encouraged me, both for my benefit and her own, to talk to her brother-in-law, a neurosurgeon. We did so over a beer in his television room. There was no examination. I didn't even show him my muscles. "Probably benign," he said. I wanted to feel reassured. I did, but not very.

A week later, just before my departure for southeast Asia, my wife and I were on a golfing trip in San Diego. The game wasn't too bad; my drives, always short, weren't any shorter. At the end of a round, I was told that I had an urgent telephone call from the neurosurgeon. Thinking someone in the family had died, I nervously returned the call. He said, "I talked to a neurologist friend and he is concerned. He thinks you should come in for an exam and an EMG. And by the way, does your tongue fasciculate?" He informed me that tongue fasciculations tend to be a more specific indicator of motor neuron disease than fasciculations of the trunk or extremities.

"No thanks," I said to the examination. My reasoning had not changed. The results would in no way benefit me unless they were negative, and the consequences of a positive test result were more than I felt I could handle. I hadn't given any thought to my tongue in decades, but suddenly it was the central focus of my existence. I couldn't see any obvious fasciculations in the mirror, but maybe there was an occasional flicker. Once I saw an unmistakable twitch. I felt doomed.

Why go to Asia, I asked myself, when they will only have to send me back in a few months for custodial care? Then again, maybe I'll last longer than that. Plus, I had always wanted to see Manila, Hanoi, Phnom Penh, and the other great sights of southeast Asia. And maybe, just maybe, I didn't have ALS after all. That fervent hope of a benign conclusion to the entire matter was always central to my thinking. After all, all of those other illnesses I imagined had disappeared. I went to Asia. In my work during the previous 5 years, I had departed from the United States several times. The goodbyes this time were very different. I felt strongly that many of them would be ultimate farewells, but I said that to no one.

We have a better library here in Manila than we did at my last venue. It contains the 1989 edition of Adams and Victor's Principles of Neurology. Continuing my amateurish attempts at denial, I waited a couple of weeks before opening it. With all due respect to those fine authors, their index could be better. I read the section on ALS, which reinforced the idea that it is most unusual for the fasciculations to antedate the weakness. But unusual does not mean never. I returned to the push-up and squeeze bag ritual. I was no weaker. It wasn't until another fortnight went by that I opened the text again and looked under "fasciculations." There I found the statement, "A simple clinical rule is that fasciculations in relaxed muscle are never (my italics) indicative of motor system disease unless there is an associated weakness, atrophy or reflex change." My response to that simple sentence was like that of a wrongly convicted man when he hears that the governor has just reexamined the evidence and spared him the electric chair.

Since then I have learned that there is a not-uncommon syndrome of widespread, continuous fasciculations that may last for months or even years. It has been described in large part in health professionals, presumably because they know about the malignant potential of muscle twitching and seek medical care accordingly. The rest of the affected population, in its ignorant bliss, thinks that they just have some twitches, not enough of a problem to warrant paying for an office call. The last line in Adams and Victor's description of the syndrome is, "eventual recovery can be expected."

I have replayed the scenario of the past year over and over in my mind. Had I sought medical attention and had I had electromyography early on, I would have spared myself months of emotional distress. I might well have come across a sage neurologist who was fully aware of the syndrome and who could have reassured me even without the invasive test. But that would have been a gamble. On the losing side of that gamble would have been the knowledge that I had a disease that would inexorably take from me my means of movement, then my means of communication with those whom I loved, and then my life. I did not like the odds. I would probably make the same choice again today.

I'm still twitching. I don't squeeze the cuff anymore, and I've ceased doing the push-ups. I don't have Lou Gehrig disease. I am awaiting the next disorder that may cause my premature death.

Requests for Reprints: Lawrence N. Hill, MD, c/o American Embassy, Manila APO AP 96440, Philippines.

Accession Number: 00000605-199611150-00012

Reprinted with permission.