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.
One question we explored was whether or not there is a characteristic personality pattern of the ALS patient that is different from the normal population or other ill populations. To determine this, we evaluated each patient's psychological, social and spiritual status. This evaluation included tests measuring depression, hopelessness, perceived stress, anger expression, loneliness, health locus-of-control, life satisfaction and purpose in life. Surprisingly, patients' scores for each test scale covered a broad range, suggesting great variability in psychosocial-spiritual status. When the average scores for these tests were compared to those of a normal, healthy population, the only differences were that the ALS patients exhibited more depression (60% had some level of measurable depression compared to the 16 to 20% typical of the general population) and had a more external health locus-of-control (that is, they wanted others to make decisions for them and tell them what to do in regards to their health care and treatment options). Neither depression nor an external health locus-of-control is unusual in patients with chronic disease.
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
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
Mode of Onset of ALS
Severity of Disease
Length of Illness
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
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.
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.
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.
ALS is a disease that affects the entire family, and it can have profound physical and psychosocial-spiritual consequences for the spouse in particular. As a group, the psychosocial-spiritual profile of spouses was similar to the norm for the general adult population, except that they had higher depression scores (although much lower than patients had) and experienced a greater degree of loneliness. Many were clearly experiencing psychosocial-spiritual distress. Approximately 15% felt profoundly hopeless and were moderately to severely depressed, while 23% experienced a high degree of loneliness and 47% perceived a high degree of stress in their lives.
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.
Many factors affect the spouse-patient relationship during the course of ALS. These include changes in roles, differing experiences of the disease, the tendency for the patient and the spouse to experience emotional difficulties at different stages of the disease process, dissimilar perceptions of the patient's illness experience and presence or lack of family support.
CHANGE IN THE NATURE OF THE RELATIONSHIP
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
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
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
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.
The health-care professional can be an important part of an ALS patient's "wellness team." Even though they can do nothing to alter the progressive disability encountered in this disease except for providing symptomatic treatment, they can assist the patient and family in maintaining a high quality of life throughout the illness. This can happen in many ways. For example, depression is frequently a part of the disease process (nearly 60% of the patients in our study had some degree of depression), but it is not inevitable - many severely ill patients are happy or only mildly depressed. Depression can be assessed and, if appropriate, counseling and/or anti-depressant medication offered. Alleviating depression can greatly improve the patient's experience of her quality of life. In addition, it is becoming increasingly clear that depression negatively affects the body's immune system, thus contributing to the rate of decline in many diseases.
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.
It is possible for the ALS patient and family to move beyond merely coping with the disease to leading lives that have quality and dignity. For some, this may evolve naturally in the process of learning to cope. For others, it may be a step-by-step process of understanding and discovery and may require a lot of support from each other, friends and the health-care team. For still others, it may seem impossible to experience quality living in the face of ALS.
Keys to developing or sustaining a sense of well-being for the ALS patient and family include:
© 1992 Evy McDonald
|Evy McDonald, R.N., M.S., M.Div., former intensive and coronary care director, knows what dealing with a "terminal illness" is all about. In the 1980s she was diagnosed with ALS, which progressed until she was a "bowl of jello in a wheelchair." After her recovery from ALS, she conducted a medical research project on the mind-body-spirit connection in ALS. Today she has left the health arena and is in service as a Methodist minister, leaving behind a legacy of ALS articles and resources that offer science and inspiration to those dealing with serious health conditions.|