The information presented in this report is based on the results of the ALS Patient Profile Project and on the experience of one of the primary investigators, Evy McDonald, who over the last decade has met and talked with ALS patients, their families and their health-care providers. The ALS Patient Profile Project examined the psychosocial-spiritual, attitudinal and coping patterns in individuals with ALS and their primary caregivers and determined the effects of these factors on rate of disease progression and survival.

144 ALS patients and 123 spouses or primary caregivers in three U.S. metropolitan areas (Seattle, San Francisco and Philadelphia) participated in the study. Entry into the study was not limited by disease severity or length of time since diagnosis. Every three months over the course of a year and a half, participants completed ten standardized psychological tests and a data form containing questions about demographics, medical history, lifestyle and attitudes and beliefs about themselves and their lives. In addition, a physical assessment was done to evaluate physical function and ascertain the status of the disease. Patients in the study ranged in age from 25 to 82, two-thirds were male, 79% were married and 60% had attended some college. The average age when ALS was diagnosed was 55 years; patients had had ALS from one month to 30 years and ranged in severity of disease from barely perceptible functional impairment to extreme functional impairment and almost complete paralysis. Spouse participants ranged in age from 19 to 78, three-fourths were female and 60% had attended some college.

The wide variability and overall normality of these results suggest that there is no predictable psychosocial-spiritual profile for the ALS patient. Patients in the study fell anywhere on the psychosocial-spiritual spectrum from well-being to distress. A quarter of the individuals were, on average, mildly or not at all depressed, not lonely, had an internal health locus-of--control (that is, they wanted to be involved in decisions regarding their health care and treatment options), may have perceived stress but confronted it effectively, expressed anger appropriately, maintained a sense of hope and felt their lives had purpose. These qualities led to a sense of psycho-spiritual well-being and a high quality of life. On the other hand, another quarter of the participants were in distress, experiencing high levels of depression, hopelessness, loneliness and stress, low satisfaction with life, a lack of purpose, a more external health locus-of-control and little expression of anger.

As a group, those patients who experienced psychological well-being had higher-quality lives and longer survival time during the study than patients experiencing psychological distress. While such well-being is in part related to factors over which a patient doesn't have direct control (such as the severity of the disease, the rapidity of decline and their age, which we will discuss below), it is also related to factors the patient does have more control over - factors in the psychological, social, emotional and spiritual domains. The psychosocial-spiritual condition of the ALS patient profoundly influences his experience of the illness, his response to medical care and the overall quality of his life.

QUALITY OF LIFE
Quality of life cannot be measured simply by evaluating physical function. There were people in our study who lived rich and active lives despite being severely debilitated by ALS. High quality of life is also a function of an individual's perception and is reflected in a sense of psychosocial-spiritual well-being even in the face of severe physical handicaps. There are ALS patients who travel with their wheelchair and portable respirator, coach football, direct video productions, oil paint or explore the nature of the universe - despite profound physical limitations. The life of Stephen Hawking, a well-known British physicist whose physical function has been severely limited by ALS for many years, reminds all of us that physical disability in no way limits one's ability to contribute to humanity.

Perhaps quality of life is more accurately represented by the "spirit" of the patient. In our study, 37% of the patients found that some aspect of their life actually improved after they got ALS, in ways that ranged from closer relationships to an increased appreciation for life. This leads to one of the most relevant conclusions of this study - that any ALS patient and family has the potential to lead a life of quality, despite the profound physical limitations imposed by the disease. ALS may seem hopeless because there is no cure, but there is hope - because there are psychological, attitudinal and coping patterns that can lead to greater psychological well--being and a higher quality of life.

How can ALS patients and their families work toward or maintain a high quality of life? There is no simple answer, but there are resources available to the patient and family. There are areas where increased understanding can lead to greater well-being and quality of life, including the physical challenges associated with the progression of the disease, social factors, the patient's environment and family relationships, the patient's spirit and the patient's experiences with the health-care system. We will be examining these factors in the rest of this report.

RESPONSE TO PHYSICAL CHALLENGES
While the psychosocial-spiritual status of a patient is not solely determined by the physical aspects associated with progression of the disease, the two are related in a number of ways.

Mode of Onset of ALS
How the disease began - whether a patient's first symptoms of ALS were spinal (arms or legs) or bulbar (speech or respiration) - was not related to psychosocial-spiritual status. Many patients experienced fear and anger; for some it was over losing the ability to communicate clearly and for others it centered around the apparent or imminent loss of independence. Such a loss could be a major adjustment for the 66% of the patients in this study who described themselves as fiercely independent before ALS. Only 18% considered themselves fiercely independent at the time of the study. Regardless of the mode of onset, when unresolved anger and fear around the symptoms was present, there was often a reluctance to participate in family or social functions and a decrease in psychological well-being.

Age
The older population (those over 65) had higher levels of depression and hopelessness, indicating more psychosocial-spiritual distress. ALS may not be the cause of this depression or hopelessness but may exacerbate a state that existed prior to its onset, especially if the patient had already been facing end-of-life issues. In addition, many older patients voiced concern about being a burden on their spouses, particularly when the spouse had physical limitations or a chronic illness.

Severity of Disease
As one might expect, there is a relationship between psychosocial-spiritual status and severity of illness. Mildly ill patients had a higher level of well-being than moderately or severely ill patients; in particular, mildly ill patients were less depressed and perceived less stress. Interestingly enough, however, there was no difference in psychosocial--spiritual status between moderately and severely ill patients. Furthermore, psychosocial-spiritual well-being did not necessarily decline during the 18 months of participation in the study. While some individuals did show increased distress, an equal number experienced an increase in well-being, and the majority of patients remained at the same level of distress or well-being throughout the testing period.

Length of Illness
It is particularly interesting to observe the patient's psychosocial--spiritual status in relation to length of illness. The current assumption is that most ALS patients will die within 3 to 5 years after diagnosis and will have increasing feelings of depression, hopelessness and perceived stress and an ever decreasing purpose in life. Many patients have had experiences like the woman whose neurologist recently told her, "You have a horrible disease, and if you're planning a vacation take it now, for soon you won't be able to." However, studies report 5-year survival rates ranging from 18 to 42%, with many individuals surviving far longer than that. One study reports, "Other factors, such as the patient's will to live and the support of his family, play significant roles in extending his life."

Of the 144 ALS patients who entered our study, 31% had already survived longer than 5 years. Overall, these long-term survivors exhibited greater psychosocial-spiritual well-being than those who had had ALS a shorter period of time, even though one out of five were severely affected by the disease. Most of these patients saw a physician only rarely or had not seen one for years. "Why should I," many have asked, "when the doctor told me I'd be dead years ago and there was nothing more he could do for me?" These patients demonstrate that there is hope. Some ALS progresses very slowly or "burns out" and stabilizes; it may even reverse, although this is extremely rare. In addition, there are patients with a "normal" or even rapidly progressing disease who maintain a healthy psychosocial-spiritual status and have a quality of life far beyond what one might expect.

Rate of Decline
Rapidity of decline can influence an individual's psychosocial-spiritual well-being. In a patient whose disease is progressing rapidly, changes can occur so fast that there is little opportunity to integrate the loss of one function before another loss is confronted. The phase of healthy denial of the disease, which can give an individual time to integrate the diagnosis and call upon her inner resources to deal with it, is not available to individuals who every day experience themselves losing more functional abilities.

What about the individual whose disease is not progressing at an extraordinary rate? It is commonly assumed that these patients experience greater psychological well-being than those who are declining rapidly. In our study, an unexpected finding was that patients who were moderately affected by ALS and who had been diagnosed 1-1/2 to 5 years earlier were, as a group, in a state of psychosocial-spiritual distress that was exceeded only by severely ill patients who had had ALS less than 1-1/2 years. This distress was characterized by high levels of depression, hopelessness and perceived stress and by low purpose in life. Patients in such distress need support from their families and the medical team.

Respirator Usage
As the disease progresses, the patient, spouse, family and health-care provider have to face the question of whether or not to use a ventilator as a treatment to prolong life. Many health-care providers have strong feelings (both pro and con) about placing patients on respiratory support. They may forcefully encourage it or they may refrain from discussing it, depending on their personal stance. Often this stance is based on their experience with one or two patients or on their projection of what they themselves would do in that situation. Of the ALS patients we evaluated, only 24% said they would be willing to use a respirator. Little information has been available about how individuals on ventilators fare psychologically; however the prevalent assumption is that life on a ventilator must be psychologically distressing.

Eighteen patients (12%) were on respirators when they entered our study. Contrary to expectation, these patients were not, as a group, more psychologically distressed than other ALS patients. They had similar levels of depression, loneliness, hopelessness and perceived stress, and they considered their lives to have purpose and meaning. In addition, they had a more internal health locus-of-control than ALS patients who did not need ventilation. While one might assume that only those individuals with a strong family support system would choose ventilatory support, we observed that patients electing to be placed on respirators included those dependent on hired caregivers as well as those with tight-knit families.

Being on a ventilator does not condemn a patient to a miserable life, devoid of purpose and isolated from friends and family. In fact, experientially quite the opposite was true for many of our 18 patients. Working with their families and health-care practitioners, many of these individuals found life more satisfying than before they went on the respirator - one reason being that ventilatory support allowed them to leave the house and participate more fully in life.

NONPHYSICAL CHALLENGES
In addition to the physical challenges of ALS, the disease places numerous other stresses on the patient and family. Many of these ultimately affect the patient's quality of life and sense of well-being. Individuals with ALS often experience ever-increasing isolation from family, friends, events of the world and nature. Many have fewer visitors, just at the time when they are less able to get out of the house. This was true for 40% of the patients in our study. Living in a culture that values physical perfection, the ALS patient may feel embarrassed or ashamed by the condition of his body and therefore be reluctant to appear in public. For many, the main social event is a visit to the clinic or doctor's office. Some patients stop going to church (16% in our study), and only a small percentage (19% in our study) participate in support groups.

Over time, patients may no longer be able to function in roles that once defined their lives and their sense of purpose. The "breadwinner" can no longer work; the one accustomed to cooking can only watch someone else do it. The patient may begin to question her competency as a spouse, parent, friend or lover, and family relationships may become a source of stress rather than support. Worries about money often increase dramatically. Being a burden on others can be the most difficult aspect of ALS; this was true for 12% of the patients in the study.

Most participants in our study also felt that their lives were devoid of leisure, despite the fact that they watched an average of four hours of television every day. Evidently there was no part of the day that was experienced as leisurely - that is, devoted to having fun and to participating in activities that would truly distract them from their disease.

Many changes take place over time in the spouse's daily life. When the patient's traditional roles in the family alter with the decrease in functional ability, these roles must be taken on by the spouse or other family members. Careers may need to be suspended. Wives may need to undertake everything from managing household finances to mowing the lawn for the first time in their lives; husbands may find themselves chief cook, dishwasher and child-rearer. Everyone in the family system will probably experience changes in the roles they play.

Approximately 70% of the spouses in the study were working prior to their mate's diagnosis of ALS. By the time of the initial interview, nearly a quarter of them had stopped working in order to care for the patient. The resulting reduction in income, along with soaring medical costs, greatly increased the occurrence of financial difficulties. Prior to ALS, only 3% of the spouses described themselves as "very worried" about money; after its onset, 18% were "very worried." Some spouses (9% in the study) took on a job in order to meet their family's financial obligations, often at a time when they were increasingly needed at home.

Many spouses curtailed their volunteer work and church or community activities in order to stay home and care for the patient. The number of spouses doing volunteer work decreased from almost half before diagnosis to a third at the time the study began. The percentage not attending church almost doubled. Such changes can lead to a sense of isolation and loneliness, loss of sense of self and resentment toward the ill spouse. Fortunately, most spouses reported that they had an outlet for their emotions and were able to have some amount of time alone.

CHANGE IN THE NATURE OF THE RELATIONSHIP
The changes in roles necessitated by the decreased functional abilities of the ALS patient can lead to an unrecognized and unacknowledged shift in the relationship between spouse and patient - from that of husband-wife to that of caregiver-patient. For the psychosocial-spiritual well-being of both patient and spouse, the husband-wife relationship needs to be nurtured and maintained. The couple can accomplish this by having other people help with physical care and by continuing previously shared activities, such as seeing movies, playing cards, watching sunsets, having sex or going to the park.

Couples who reported having high-quality lives despite ALS said that living, not ALS, was the center of their lives. We found that couples who were successful in living fully and maintaining their relationship saw the disease as a shared experience and were able to communicate well enough so that resentment, guilt or other negative emotions didn't build up over time.

EXPERIENCES OF THE DISEASE
The stressful aspects of life with ALS may be quite different for the patient than they are for the spouse. 83% of the spouses listed health issues as their major stressor as opposed to only 55% of the patients. Many patients (12%) were most concerned about financial matters while only a few spouses (2%) listed this as their major stressor. It's easy to see how communication difficulties can arise when the spouse wants to talk about the patient's health at a time when his attention is focused on financial concerns, or when the spouse is preoccupied with coping with the disease while the patient is wanting to focus on living, not on his illness. In fact, relationships themselves were a major stressor for many patients (18%) and spouses (12%). As a group, the spouses in the study were more lonely than the patients. The spouses, having given up such previous activities as pursuing a career, luncheons with friends or competing on a bowling team, often felt house-bound. At the same time they spoke of a reluctance to leave the house because the patient could not. This sense of loneliness and isolation sometimes escalates to resentment and guilt. It is important for the spouse to maintain activities with friends in order to help lessen the experience of loneliness. The patient who encourages his spouse to take time away often finds that the spouse has more to give to the patient, not less.

Spouses may want to be more involved in the health-care plan than the patient does. It sometimes happens that a spouse tries to talk with the patient about his research into ALS and can't understand why his ill partner doesn't want to know more about the disease. Or the spouse may want an in--depth explanation from the physician of all the phases and complications of ALS that her ill partner will probably encounter, along with a discussion of the various forms of supportive treatment available, while the patient has no interest in participating in the process. The opposite may also be true: the spouse may simply want to be told what to do to help his partner without taking part in any decision making. Such different approaches to illness are simply part of human nature; respecting these differences is a key to maintaining a loving husband-wife relationship.

TIMING OF PSYCHOSOCIAL-SPIRITUAL DISTRESS
One of the most important findings in our study was that patient and spouse often experienced psychosocial-spiritual distress at very different stages of the illness. The couple may expect to experience the stress of ALS in the same manner at the same time - i.e., if the patient is depressed and lonely, he may assume that his spouse is having similar feelings.

Sometimes this is true, but there are many times when the patient may be experiencing distress while the spouse is not. Or the patient may be feeling a sense of well-being and be unaware that the spouse is feeling hopeless and lonely. Such differences can lead to friction or misunderstanding between the patient and spouse. It is essential for patients to acknowledge to their spouse and health-care professional how they are actually feeling and equally important for the spouse to refrain from assuming how the patient is feeling and "talking" for him to the health-care professional. It is also important for the patient to be sensitive to how the spouse is feeling. If a couple having very different emotional experiences has not already developed skills in listening to and understanding each other, then friction and feelings of isolation can develop. Learning the art of really listening to each other can increase understanding, tolerance and love. The help of a qualified counselor can be invaluable in a situation like this.

DIFFERING PERCEPTIONS OF THE PATIENT'S EXPERIENCE
The spouse often perceives the patient's experience to be quite different from the experience reported by the patient, and this can be a complicating factor in the whole realm of mutual understanding and communication. Spouses in the study tended to rate their ill partner's level of life satisfaction lower than the ill partner rated it - a very interesting finding. Spouses' ratings of the patient's self-acceptance and acceptance of help were very often much lower than the patient's own evaluation. Spouses also thought that patients were more worried about money than patients themselves reported. Furthermore, the spouses felt the patients were not as honest in communicating how they felt as the patients reported they were.

Many dynamics can play a role in these differences. For example, the patient may be in denial, not ready to acknowledge or accept the current reality of her life. In addition, both patient and spouse may perceive their partners through the filter of their own emotional state - which, as reported earlier, may be quite different from that of their mate. Here, it is important to remember that rarely can one individual accurately know the psycho-spiritual state of another. Too often, we project how we think we would feel if it were us. It's easy to project feelings of hopelessness, depression, anger and guilt onto others, but it's important to remember that these feelings do not have to be the baseline from which to live each day.

SUPPORT SYSTEM
The more people involved in a support system, the greater the support is thought to be. However, many participants in the study expressed satisfaction with their support system even though it involved just a few people. The number of people involved doesn't seem to matter as much as whether the patient and/or spouse perceive that support is being offered. The family and friends of an ALS patient can be supportive in many ways. As functional abilities decrease, some patients lose their sense of purpose in life. Those patients who continue to participate in activities with their family, friends and co-workers relate that their lives still have purpose and meaning. The family of the patient can find ways to involve the patient more in their lives, no matter how severely affected he may be by ALS. This may range from making the extra effort to take the patient on family outings, to seeking his input on family decisions, keeping him apprised of happenings in each family member's life and discovering how his many gifts can still be given. The experience of ALS can draw family and friends closer. While many friends may drift away, others may want to deepen their friendship. Some friends can even become an integral part of the family life, lending their skills to design and implement assistive devices, being there to listen and relieving the spouse from the demands of physical care so that she can have some time for herself.

The better the patient and spouse can communicate their desires, the better assistance the health-care team can provide. For example, the patient and spouse can express how active a role each wants in planning the patient's care. Do they want to be told what to do, or would they like to be an active part of the decision-making process? How much information does each want on ALS, drug trials and support groups?

The patient and spouse may want different levels of participation. If the patient has a high external health locus-of-control, she may not want to be given multiple options for symptomatic support or treatment. She may want the health-care team to be directive, telling her the course of action to be taken and demonstrating how she can most accurately follow instructions. On the other hand, if the patient has a highly developed internal health locus--of-control, she will often study the disease and learn about options for experimental treatments or alternative therapies. Telling her what to do without giving her information and options may lead to conflict between the health-care professional and the patient. She wants the health-care professional to be a team member who facilitates decision-making but recognizes that the patient will have the final say. The level of involvement is not the point; what matters is that there is clear communication and mutual respect.

For those wanting an active role in their health care, there are many things they can do. The patient and spouse will benefit by preparing for their visit to their primary physician or clinic beforehand. They might list their concerns and questions and, if they are attending a clinic, make a separate list for each team member (the dietician, the speech therapist, etc). The informed patient will be able to grapple more effectively with the difficult questions about life support and other treatment options. They can also make their needs known to the health-care team and offer suggestions. For example, the patient might want a medication to dry up secretions, or the patient and spouse or whole family might want a referral to a counselor to learn better techniques for coping and communicating.

If the patient and spouse do not feel a rapport with their primary physician or are not being accepted as part of the team, they can change physicians. Dr. Forbes Norris, a leading ALS neurologist, reminds us to "never forget that the patient is in charge throughout the illness."

The spouse can also ask the health-care team for assistance for herself. There are times when the spouse may need counseling, psychotherapy or treatment for depression. The health-care team can also explain available home-care options, such as hospice care, respite care, home health aides and how other family members or even friends can be trained to care for the patient; this can help the spouse avoid feeling trapped or abandoned. The spouse can also ask where to seek financial assistance, apply for disability and locate needed assistive devices.

Keys to developing or sustaining a sense of well-being for the ALS patient and family include:

1. Maintaining a sense of hope. Hope not necessarily in the sense of believing that "They'll find a cure," but in knowing what research is happening, in participating in clinical trials when possible, but most of all in recognizing that they can still lead meaningful and high-quality lives.
2. Drawing on one's own inner strength and maintaining a sense of purpose and meaning.
3. Sustaining or developing a nurturing support system.
4. Shifting from a focus on the effects of ALS to a focus on sharing life together and maintaining the highest quality of life possible.
5. Developing an understanding and respect for each other and communicating what each other is thinking and feeling.
6. Including the patient in family activities and encouraging the patient to participate.
7. Enlisting the support of the health-care team.
8. Having a willingness to seek treatment or counseling if needed for patient, spouse or family.

© 1992 Evy McDonald