Excerpt From

How Will They Know If I'm Dead? Transcending Disability and Terminal Illness

FOREWORD

 I’ve known Robert Horn all his life, and I’ve learned a lot from him. From the time he was born, just about the time when my first son was Horn, I knew him as Robbie. My son and Robbie played together as toddlers, and then followed similar paths to academic careers, active sports, and family life. But then, with alarming suddenness, Robert’s path through life took a startling change. In 1988, he, like many others in many other circumstances, was forced to hear the chilling words of a ~rim diagnosis. A series of bothersome but apparently innocuous symptoms turned out to be ALS — amyotrophic lateral sclerosis, more commonly known as Lou Gerhig’s disease. ALS is a degenerative neuromuscular disease that quickly denies its victims voluntary muscle control, including the muscles needed to breathe. ALS is a terrible disease, and while it exacts its deadly toll from the body, it does not affect the mind. Its victims are completely aware of their continued and relentless physical degeneration. All too soon, ALS victims become unable to move, unable to speak above a strained whisper, unable to eat without choking, and barely able to breathe. ALS is a terminal disease, progressive and merciless. People with ALS don’t usually live very long, the average life expectancy after diagnosis is two to four years.

But Robert Horn has lived longer than that already. And Robert Horn has truly LIVED. Even while afflicted with this deadly crippling disease, Robert has done more real living than most of us ever do. Many in his condition would find it difficult to face life. Most people find it difficult to face death. Every day, Robert Horn faces life and death at the same time; he faces life and death with joy, grace, and triumph.

I’ve had my share of experiences with life and death issues throughout a medical career that has spanned over half a century. At about the time when I first met the newborn Robbie Horn, I and a few others were pioneers in the new field of pediatric surgery, attempting to give years of life to those newborns whose serious congenital defects then had a mortality rate of 90%. By the time I left my surgical career nearly 40 years later, we had converted that 90% mortality rate into a 90% survival rate. Modern medicine can do great things, but modern medicine cannot do everything, as the grim course of ALS reminds us. But all those tiny lives we saved as surgeons — as important as each one was — were fewer in number than the many lives I saw changed when I served as United States Surgeon General, lives saved, not by medical miracles, but by individual choices: choices not to smoke, choices to quit smoking, choices to practice healthful living.

Robert Horn as chosen life faced with the choice between death and a life all too many would say was not worth living. Robert chose life, and in doing so he has made so many of us realize anew how much life is worth living. He could easily have chosen another way; he was offered the option of ending his life painlessly and quickly. Instead he chose life, even life on a ventilator, “eating” through a tube inserted in his stomach, “talking” with his eyebrows, writing on a computer with his foot. Did he make the right choice? Robert’s words bear repeating: “You bet! I am convinced that what I have left is more valuable than what I have lost. I believe that the things I can do are more important than those I can’t. There is much more to life than physical ability. I am still a vibrant, healthy, and independent person mentally, emotionally, and spiritually. I can think, reason, and analyze, remember, read, write, learn and communicate. I can love, feel happiness and sadness, be enthusiastic, get angry, have highs and lows, feel joy. I can believe, hope, and have faith.”

By God’s grace, Robert lives a life of faith, hope, and love. And because he faces life and death with joy, grace, and triumph, I think you will, too, as you read his book.

 by C. Everett Koop, MD., Sc.D.

 I coached youth soccer for 15 years and I absolutely loved it. Eleven of those years I coached my daughter and the girls, from five- and six-year-old kids who were just beginning the sport all the way through city champion high schoolers and exceptionally talented under- 16 and under-19 club teams. I love the game the exercise, fun and, especially, the necessity of teamwork. Most of all, I loved the kids. I coached a lot of the girls for many years and got to know them and their families very well. They became almost daughters to me. “My girls” I proudly called them and they were and still are special young ladies.

I approached coaching as another form of teaching. Just as I was a professor in the classroom, I was a teacher on the soccer field. Further, I also was convinced that a person who takes on the responsibility of coaching young people has an obligation to try to teach them more than the mechanics of that particular sport. Being entrusted with these youth for even a few hours each week is a great privilege and the opportunity to pass along some “life lessons” should notbe missed. Like it or not, for better or worse, a coach is a role model and his/her attitude toward the game and toward life can have a significant impact on a youngster’s outlook. So, one’s influence had better be a positive one.

With all of these considerations in mind, and being careful not to intrude into the realm of parental authority, the coaches I worked with and I emphasized a few basic principles that apply not only to soccer but also to the broader concerns of life. For instance, we stressed sportsmanship, fair play, hard work, self-confidence, and respect for teammates and opponents. Simple precepts but fundamental, I think, to any endeavor and to that old-fashioned idea of “character.”

As the girls grew older, I became a bit more philosophical. Impressed with the concept of the game of soccer as a metaphor for life, I developed a little homily that became an integral part of my pre-game oratory. In its full version, it went something like this: “Soccer is like life. It doesn’t matter how often you get knocked down or if you lose the ball or make a mistake. Those things happen to each of us. What does matter is whether and how quickly you recover, learn from the experience, and get back in the game. That’s the test of character.”

Somehow, when you are delivering such inspirational pronouncements, you are not thinking of yourself but, rather, in terms of your audience. At least, that was true in my case. In my life, after all, I had had to overcome setbacks, disappointments, and my own mistakes but, thankfully, none of them had been major or life-threatening. So it was that I was unprepared to apply my little bit of homespun philosophy to my own life in a big-time way.

All that changed on one sunny Friday afternoon in June 1988: I was given a death sentence.

On that horrific day, I was told that I had Lou Gehrig’s disease, known medically as Amyotrophic Lateral Sclerosis or ALS. The diagnosis came out of the blue. I went into the neurologist’s office with a minor twitch in my left arm and came out with a fatal motor neuron disease. My world was suddenly turned upside-down. ALS is a particularly nasty disease that kills you gradually, progressively, by blocking the messages from the brain to the muscles. Within just a few years, often less than two, the muscles atrophy to the point of total paralysis. This paralysis includes the muscles that control swallowing and, most essential to life, breathing. Basically, death is by suffocation. ALS leaves the mind intact so it can observe this steady physical deterioration.

The statistics regarding ALS are as grisly and despair-inducing as the disease itself No one survives it. It is terminal in all cases. A small percentage of its victims live for 10 years and an even smaller number live for 20 years (British scientist Stephen Hawking is probably the best­known example) but ALS always wins. The overriding, and overwhelming, statistic is that the average life expectancy for an ALS

victim is only three to five years from the time of the diagnosis. Some sources put it at two to four years.

I was not a quantitative political scientist when I was teaching at the university and I am no whiz at statistics, but it seems to me that, judging from the above numbers, I should be dead. In fact, by the “law” of statistical probability, I should have died several years ago!

Perhaps that’s why I distrust statistics. Except in sports, especially baseball. In baseball, statistics tell all — actually, they don’t in “real” baseball but they do in “fantasy” baseball. I have had a team in a fantasy league for as long as I have had ALS and every morning for the last seven seasons I have poured over box scores to see how various members of my team — Da Slugs — performed in five batting and five pitching categories. Their aggregate statistics determine everything.

In sum, from my perspective, baseball statistics are almost sacred, fit to be carved in stone. Statistics about ALS, on the other hand, are best ignored. To the extent that such negative statistics can become self-fulfilling prophecies, they are to be doubly ignored.

Moreover, the fact of the matter is that I am just too busy living to pay much attention to ALS mortality statistics.

Most of the books I have read about ALS and other terminal illnesses are understandably focused mostly on dying and death. This book is not mainly about either. Nor, as you might guess, does it deal with statistics. There are hardly any numbers in it at all. It is not about the medical aspects of ALS or other crippling and terminal diseases. Nor is it a compilation of the experiences of various people dealing with ALS or anything else. Finally, it is not by a celebrity author about his recent illness and recovery.

What this book is about, then, is living. It is about life. First and foremost, it is about the value and rewards of living life to the fullest extent possible in spite of being under the shadow of a terminal disease and having severe disabilities. It is about coping, about perseverance and determination, and, ultimately, about hope. It is a story of one person’s struggle. It is a personal celebration of the triumph of life over disease and death.

The book, like Caesar’s Gaul, is divided into three parts. Part One, “Living Life,” sets the stage for my confrontation with ALS. First, it presents some personal background which is intended to give the reader an idea of how much I enjoyed my “previous life” and where, literally, I am coming from. Second, it discusses my attitude toward, well. ..attitude. “Choosing Life,” Part Two, focuses on my struggle with the disease, the extraordinary support I received in those difficult times, my decision in favor of life and the means of coping with my new situation. Part Three, “Affirming Life,” discusses the fragility of life when you are dependent on others for survival and also reflects on what I gratefully consider to be my new lease on life. The “nonconclusion” attempts to tie the remaining loose ends together.

I have been coping, battling if you prefer, with ALS for just over seven years now. I have had virtually no functioning muscles for five years. I can’t eat, speak, or move. And I have been hooked to a ventilator for the past four-and-a-half years. That machine breathes for me. Everything I do takes a great deal of time. Communicating is especially slow. I “speak” with my eyebrows and spelling out every word letter by letter is a tedious and lengthy process. About five hours of each of my days are consumed by necessary medical attention, getting bathed and dressed, taking a nap and, finally, being transported out to the living room where my computer waits with considerably more patience than its eager user. On my good days, I am usually able to work for five to six hours before I get too tired. I type with the only part of my body that I can move, my right foot, and this is another slow process. It takes me, on average, almost two days to type a page. Everything is a project.

What, then, is my message? It is simply this: I have found, along with many others, that despite the difficult conditions of disability and terminal illness, life can be meaningful, productive, fulfilling, rewarding, and valuable.

     Where I am confused, however, is with another part of her definition: she writes that acceptance also includes an acknowledgement that the struggle is over. Maybe I have misinterpreted her but my struggle is definitely not over. To paraphrase one of my favorite characters from, embarrassing though it may be to admit, one of my favorite movies, Bluto (played by the incomparable John Belushi) in Animal House, it ain’t over until I say it’s over. I refuse just to sit around waiting to die.

    For me, every day is a struggle. I have always believed that being alive meant reaching, extending myself— not in a frantic manner but in a way that promotes continued growth, learning, expansion of my horizons, and the enrichment of my life and, I would be so presumptuous to hope, the lives of some others. This attitude is reinforced by the topic of Kubler-­Ross’s next chapter after the one on acceptance: hope. She doesn’t list hope as a separate stage but an emotion accompanying all the stages. This is so important that it deserves a fuller treatment and I will return to it later.

     In sum, I feel that I have achieved the stage of acceptance and the state of peace. But it is certainly not an acceptance or peace of resignation to my “fate.” Rather, it is an acceptance and peace combined with a fighting spirit, a determination to make the most of what I have for as long as I have, and hope.

Searching 

     Not only did I run the gamut of emotions in the months following the diagnosis, I also went through my share of doctors, clinics, potential treatments, and experiments. With each, I unwisely got my hopes up. I was seen by neurologists and checked out in ALS clinics from Los Angeles to Detroit to Edmonton, Alberta, to San Diego. I participated in clinical trials and double-blind studies. Among other things, I injected myself with large doses of vitamin B, took increased quantities of other vitamins orally, and tried amino acids for quite a while (which also necessitated a substantial change in my diet).

     Perhaps the possible “cure” that I placed my greatest hopes in was Chinese herbs. Western medicine certainly didn’t have any answers. (On that topic, I can well imagine the frustration physicians must feel when confronted with a case of ALS. There is no known cause nor treatment nor, most importantly, cure.) Moreover, Chinese medicine has worked for centuries. In any event, twice a day Judy and I brewed an awful-looking concoction of a dozen or so dried “things” we had gotten at a pharmacy in Chinatown. But the looks of the brew didn’t hold a candle to the horrible smell. And, unfortunately for me, the gross smell paled in comparison to the taste. Talk about foul! I dutifully drank two mugs of this broth every day for several months.

     These and other hoped-for remedies — including Chinese acupressure, which at least felt good — were to no avail. I am glad I tried them, however. I would have regretted not exhausting every seeming possibility.

Physical Decline

     Meanwhile, my physical condition began to deteriorate more rapidly. For the first year and a couple of months my struggle was almost entirely psychological. The only physical symptoms I had were a slight weakness in my left arm and a slight limp in my left leg due to the onset of “foot drop.” (This is where the muscles in the front of the leg atrophy faster than those in the back causing the front of the foot to be pulled downward. It is not painful — not until, that is, it becomes so pronounced that it causes you to trip and fall.) In fact, some neurologists thought I might have a mild case. In just over a year, from autumn 1989, through the end of 1990, however, I went from those symptoms to almost total paralysis coupled with great difficulty eating, speaking, and even breathing.

     It began with my legs. I started stumbling, tripping, and losing my balance. I had some harrowing falls, including one into, and through, a shower door. I progressed through a number of assistive devices for mobility: a brace to try to improve the foot drop, a regular cane to a multi-pronged to a walker, an ordinary wheelchair to a fancy one, and two terrific battery-powered vehicles, a scooter which was perfect for school and soccer fields and an upholstered chair, called a Foxy, which was absolutely wonderful for use at home.

     My other means of transportation were changing, too. I soon had to stop commuting to the university on my bike. That was a sad day because I had been enjoying that for almost 20 years. I also had to give up hiking up the eight floors to my office in favor of the elevator, a major concession.  Mv withering left arm forced me to trade in my stick shift car for an automatic. That didn’t last very long before I thought myself a menace n the road and had to depend on others to drive me to school and elsewhere.

     The other difficulties I was experiencing were not so easily compensated for. My increasingly labored breathing weakened my voice making talking and especially lecturing quite a chore. Finally, due to my growing inability to swallow, I was having a hard time eating. In less than a year, I went from 170 pounds, give or take a pepperoni and extra cheese pizza, to 112. Foods I loved were steadily eliminated from my diet. Despite soft and mushy food I started choking more often leading to frequent calls to the paramedics and brief sojourns in hospital emergency rooms.

     Eventually, by the end of February 1991, all of the above conditions, particularly swallowing and breathing, had gotten so bad that I was admitted to the hospital. There I was confronted with the biggest decision of my life: whether or not to go on a ventilator. A life or death decision.

With a Lot of Help from My Friends

     I believe that God works through people. He is not some force that intervenes occasionally, in the style of “deus ex machina,” in the affairs of humankind. God’s presence in our world is a constant, living and ongoing one. It is there, obvious to anyone to see or hear, in the manifold expressions of love made by ordinary people (and the scarce extraordinary) every day.

    In the words of a beautiful song from the outstanding musical, Les Miserables, “To love another person is to see the face of God.” Indeed, gift of love, to love and be loved, is perhaps God’s greatest gift. Mine not a very complicated theology.

    I did not announce my illness to anyone beyond the family and our store at first. By the fall of 1989, however, that something was wrong _s becoming increasingly difficult to conceal from those who knew me Ii. (I had shrugged off questions with casual remarks that I had hurt leg playing basketball or some such.) I remember finally telling a long-time friend, the father of one of the girls on the soccer team. He got choked up, gave me a big hug, and said, “Bob, don’t ever give up.”

     That extremely emotional moment, simple and brief though it was, encapsulated both the great difficulty I had in telling close friends — I had trouble handling their reactions — and the immense rewards of support from doing so. Once I surmounted this very high hurdle, it seemed that everywhere my family and I looked all we could see were reflections “the face of God.” The outpouring of love and support, in words and deeds, truly astonished all of us. This blessing of love was the foundation e relied on to cope with my downhill progression.

     Naturally, my most crucial source of support came from my wife, Judy, and our three children. Without their love, understanding, patience, help, humor, and enthusiasm for life, I don’t know if I could have made Apart from specific things they did for me — including, wheelchair­bound, a marvelous visit to New York City with Judy, Laura, and our special Australian friends, Wendy and Joe O’Hara (our best buddies while n Malaysia) — they also managed to strike just the right psychological balance of sorrow and encouraging me to get on with life. That was a critical support for me.

     This essential support of family is, unfortunately, not always so natural. I have heard sad stories of cases where the unafflicted spouse responded to discussion of assistive devices with a dismissive “Would that really be worth it?” It seems to me that that decision should be made by the patient. Family members who will be caregivers can have a say but the patient has the deciding vote.