ON BEHALF OF THE PATIENT

R. A. Smith

In earlier times, physicians were powerless to alter the course of serious illness. Many of their remedies were of no value, and some caused serious harm. In spite of this, the medical profession enjoyed a privileged place. Doctors could often offer a rational explanation to account for symptoms, which, at the minimum, dispelled superstitious notions of illness, and they could provide empathetic care. When every serious disease was hopeless, patients could expect to receive a similar level of care irrespective of their particular malady. In recent times, public awareness has focused on the physician as a master of technical innovation. The emphasis on curative rather than palliative treatment has had the effect of shifting attention toward the treatment of acute illness, i.e., illness which responds to surgical or pharmacologic intervention. Not only is there no cure for amyotrophic lateral sclerosis (ALS), but for patients with chronic diseases even access to care may be limited. Patients, in some cases, actually have to search for a physician who will take an interest in their care. The search may end with a general practitioner since some specialists make it known in one way or another that they are not interested in caring for the chronically ill. In despair, some patients give up entirely, and look to non-traditional care providers for advice and comfort.

Increasingly, neurologists have become sensitive to the needs of patients with chronic, presently incurable neurologic disease, who are eager to establish a bond with their caregivers. Their need in most instances, can easily be met with empathetic care and symptomatic treatment. Some of these therapies not only provided comfort, but they extend life. It turns out that many patients choose to live with considerable disability not because they are afraid to die, but because they have reasons to live. While physicians' attitudes have been everlastingly shaped by contact with the disabled, a number of social forces have been at work upon us, and patients alike. Patients' right to know and act upon matters of health has been given, in some instances, legal protection. Further, lay persons have been making claims to a place in the delivery process, substance abuse programs, etc. No longer are doctors the only source of knowledge or health services. Whereas once the physician's word was sacrosanct, little by little their authority came under challenge. Although some may prefer things as they were, change has given new meaning to the doctor-patient relationship. It is the intent of this discussion to focus on the elements of this relationship as it relates to the care of the ALS patient.

The diagnosis of ALS is usually made by a neurologist based on clinical findings and laboratory tests. In the absence of a family history, patients are generally unfamiliar with ALS and its consequences. Under these circumstances doctors are charged with a very delicate educational task. It is sad to report that patients give doctors low marks for their performance. Bearing bad news is not something that most of us like to do, but there is more to communication than transmitting a message. How one conveys something is as important as the thing itself. Anyone who wants to deal effectively with patients, especially at the juncture of life and death, needs to exhibit sensitivity and concern. From the patient's point of view, physicians could do a lot more to minimize the psychic trauma associated with diagnosis. What the physician says and does will greatly influence the patient's sense of well being. A poorly chosen word or gesture can dash hope and morale and understandably give rise to resentment.

To come to terms with ALS, the patient at some point must transcend the ALS experience if he or she is to achieve the wisdom necessary to find peace in a world which may seem arbitrary or without purpose. The path that the patient follows should be of more than passing interest to physicians, since the search for the inner meanings of life is an important human activity. Can it be that we who call ourselves "healers" have no taste or time for such weighty deliberations?

Once diagnostic procedures are out of the way and the diagnosis of ALS is certain, it is incumbent upon the physician to set apart time to discuss the diagnosis and its implications, and to outline a program of care. Since there is no specific diagnostic test for ALS, the manner in which the physician arrives at a diagnosis should be reviewed. After going through this, many patients and family members will not be convinced that the diagnosis is correct. Even if such apprehension is not expressed, patients with serious illness should be referred for a second opinion. Doctors have nothing to fear from a test of their opinions. Even when a physician is proven wrong, the patient appreciates the concern that led the physician to do what was obviously in the patient's interest. In remembering to do what is best for the patient, it is hard to err.

From the outset it is necessary to stress the seriousness of the disease, but it is also useful to make distinctions between ALS and other commonly feared diseases such as cancer. Patients are generally reassured to learn that ALS spares the mind and that it is usually painless. Further, doctors need to emphasize that the outcome is not predictable and that some patients enjoy a relatively benign course. Although sudden death may be viewed as a blessing in Western cultures, in some societies it is considered good fortune to die of a lingering illness that allows a person time to make one's peace in the world. Not only do ALS patients have an opportunity to come to terms with their illness, but in many cases they continue on with their lives, setting up new businesses or championing causes to which they are committed. It is as yet unexplained why some patients go on to live many years with the disease and others succumb rather rapidly. Long-term survivors hold that a number of non-specific factors - mental attitude, nutrition, exercise, massage, etc. - account for their relative resistance to their disease. Others feel that they are buoyed by faith, family and friends. Hearing from physicians that there is nothing to do for the disease, some patients have turned to common sense strategies for dealing with their needs. The lesson for us is clear. No one wants to hear that their circumstances are hopeless and that there is nothing to be done. In spite of the fact that there is no cure for ALS, there is treatment. Serious sequelae of ALS such as dysphagia can be effectively treated with gastrostomy and even respiratory failure can be alleviated with the aid of a ventilator. Less troublesome symptoms - cramps, salivary incontinence etc. - can also be treated symptomatically. In contrast to a number of serious illnesses, an ALS patient is able to choose to extend life. This is obviously a difficult decision to make, especially if family is involved and financial resources are limited. Acting as a guide, the physician has a unique opportunity to help the patient make the momentous decision of whether to go on living with ALS or let the disease take its course.

There are numerous ways the physician can facilitate the decision- making process. One of them is to introduce the patient to others who have made the choice to live with ALS in spite of their handicaps. Try as we may, it is difficult for us to completely divorce ourselves from the physician's point of view. Patients and families speak from a knowing, first-hand perspective. Whatever the decision - to proceed with a tracheostomy or gastrostomy, etc. - patients who have made choices have definite ideas as to the merits of their decisions.

Our manner of bringing patients and their families together is to sponsor monthly meetings, a support group led and an educational program. The support group is chaired by a leader who facilitates discussion and insures that no one person dominates the agenda. The leader need not be a person with special medical skills. Support groups can introduce patients to a peer network which can serve important social needs over the course of a prolonged illness. The monthly educational program addresses other needs of the patient. Topics of current interest are discussed by a wide spectrum of invited speakers, including scientists physicians, and other allied health professionals. In some instances, patients may take an active role, either preparing the program or setting the agenda. For many persons this is their first opportunity to meet a scientist. Interestingly, researchers will also admit to having never met patients whom their laboratory research is presumably intended to benefit. It is likely that these activities have changed the way that we see our patients, and the way that they in turn see us. Meeting patients outside the confines of a scheduled office visit invariably creates bonds which go beyond the normal doctor-patient relationship. There are some very practical consequences as well. Patients can be seen informally on a regular basis. A few words passing back and forth can reassure a patient about unexpected symptoms, and in some cases prompt the recognition of symptoms which may need more immediate attention.

In addition to hearing about research's advances, patients are highly motivated to participate in investigational studies. This can take the form of participation in basic research or in experimental treatment. Although it is to be expected that the patient's morale would be enhanced by participation in research, it is not generally appreciated that physician morale is affected as well. It is likely that the ability to offer hope to patients adds a considerable dimension to the physician's ability to shoulder the care of patients with chronic disease. The interest and enthusiasm of the research physician is transparent to patient and family alike. This attitude must, however, not engender false hope or undermine the patient's psychological adaptation. Further, care must be taken to ensure that the patient's access to empathetic care is not conditioned upon their participation in research programs. The physician's interest in the patient must be linked to the life of the patient rather than the life of research grants or drug trials. It goes without saying that patient participation in research must be voluntary. Informed consent is essential on both moral and legal grounds.

As in the case of other decision-making processes, the physician can employ a number of creative educational techniques to ensure that the patient fully understands the planned experiments. It is our habit to encourage patients to review our drug protocols, even though these employ technical terms. Further group meetings can be used to explain research studies and to introduce prospective treatment candidates to patients who have already participated in research programs. In the case of experimental treatment trials, it is obvious to the patient that they stand the most to gain if the work is a success. In the case of basic research, there is little chance that the patient would reap personal benefit. In spite of this, patients enthusiastically participate if they feel that the research is worthwhile and likely to make a contribution to future generations. It is particularly in this case that the risks must be balanced against the benefits. Painful, disfiguring or potentially hazardous procedures should not be undertaken unless an important scientific contribution is anticipated. Human research committees bear responsibility for weighing these issues, but ultimately it is the patient who must make a choice to participate or not. In general, physicians would do well to see patients as active collaborators rather than as passive objects for study.

Amyotrophic lateral sclerosis, like life itself, is marked by a number of landmarks. At the outset, there is the matter of diagnosis, and its implications. As the disease proceeds, patients face a number of decisions. Ultimately, the patient faces death itself. In the course of ALS, physicians interact in critical ways with the patient and their families. At each point the physician has an opportunity to greatly influence the patient's welfare. Charged with this responsibility, physicians are challenged to manifest the highest ideals of the healing arts. Whatever their level of interaction with the patient, physicians will distinguish themselves if they act honorably on the patient's behalf.

Acknowledgements

This work is dedicated to my wife and children who share many of my concerns for patients and their families. Benefactors of CNS, especially the Thagard Foundation have made it possible for me to devote much of my professional life to the cause of ALS. Professor Andrew Feenberg and Dr. Kubler-Ross and patients John Stockton, James Waldal, Charles Bordner, Art Waaga, Arthur Levien, Lois Maiwald and Frank Parodi have greatly influenced my thinking about patient care. Dr. Forbes and Dee Norris, who have collaborated with me over the years, have kindly encouraged me to put my thoughts about patient care into writing.

Home | News/Events | Clinical Trials | Papers/Essays | About CNS | Helping CNS | Links

The Center for Neurologic Study
© 2002 All rights reserved.
Comments about the site? Let us know.