Hiroshi Mitsumoto1 and Maura Del Bene2
1Director, Eleanor & Lou Gehrig MDA/ALS Center
2Clinical Administrator, Eleanor & Lou Gehrig MDA/ALS Center
The Neurological Institute
Columbia-Presbyterian Medical Center
New York, NY, USA
Correspondence:
Hiroshi Mitsumoto, MD, DmedSci
Department of Neurology
Columbia-Presbyterian Medical Center
710 West, 168th Street
New York, NY 10032, USA
Tel: (+1) 212 305 2940
Fax: (+1) 212 305 8398
Received 11 April 2000
Revised 12 October 2000
Accepted 25 October 2000
This paper was presented at the 10th International ALS/MND Symposium, Vancouver, BC, November 1999
In tandem with recent progress in basic and clinical research in amyotrophic lateral sclerosis (ALS), clinical care and management has improved substantially. Key processes and practices have been established and are specified in current ALS Practice Parameters; the international ALS databases identify the current status of care and management given by physicians. A number of prospective clinical management research studies are now well underway to establish the effective utility of enteral feeding, assistive ventilatory device, and exercise in patients with ALS. One of the main research objectives in the clinical management of ALS is to improve patients' quality of life. For patients with ALS, only comprehensive multidisciplinary care can effectively improve their overall quality of life from diagnosis to death. This paper will briefly review a number of factors that must be considered if we are to improve the quality of life for these patients, including comprehensive medical management, patient education, the importance of hope, the role of the health care professional, and the patients themselves. (ALS 2000; 1: 329-336)
Keywords: quality of life - ALS - psychosocial - management
Quality of life (QoL) is defined by Ruta1 as "the extent to which our hopes and ambitions are matched by experience". In amyotrophic lateral sclerosis (ALS), the rapid progression and severity of muscle weakness profoundly affects the patient's feelings of hope, self-worth and dignity, as well as their opportunities for personal activity, attainment of goals and continued employment. In terms of the patient's social function, the disease affects social role, participation in recreational and pastime activities, as well as sexual relationships. The stigma of disease-induced symptoms, changes in self-esteem, loss of independence, and psychosocial stress all play important roles in the patient's perception of health throughout the disease process.1-3
Our goal as healthcare professionals is to continually strive to improve QoL through comprehensive medical and psychosocial interventions, so as to close the gap between a patient's hopes and actual experiences. Integral to QoL is the perception of health, not only for the patient but also for their caregivers and healthcare professionals. This, along with other key issues for improving QoL for patients with ALS, will be discussed in this paper.
To improve QoL for people with ALS, it is essential to know their QoL status at the initial medical and psychosocial evaluation, and periodically thereafter. No clearly effective instrument is currently available to measure QoL in ALS specifically. The Sickness Impact Profile (SIP) is the most thoroughly validated generic QoL measurement tool and is widely used for general medical illness;4 however, it is quite lengthy and not disease-specific.5,3 A series of measurement tools, such as the SIP/ALS-19,5 SF=12 (Short Form),6 and SF=36,7 have been developed and used to assess QoL status in some clinical trials and in the ALS Clinical Assessment Research and Education (ALS CARE) database,8,9 but these instruments also are not specific to ALS and heavily emphasize the physical domain of the disease. Recently, Simmons and colleagues10 introduced the McGill QoL (MQOL) scale, which focuses more on the psychosocial than the physical domain. They suggest that the MQOL may be useful in measuring QoL in people with ALS. Another instrument is called Schedule for the Evaluation of Individual Quality of Life Scale (SEIQOL), which has been developed based on the philosophy that "quality of life is what the individual says it is."11 Recently, an instrument specific to ALS, the ALS assessment questionnaire-40 (ALSAQ-40), has been introduced.12 This QoL measurement tool appears to focus heavily on the physical domain (of a total of 40 questions, 30 concern physical aspects of ALS). While we welcome the introduction of an ALS-specific QoL instrument, the significance and full impact of this new scale requires further study.
Determining QoL for patients with ALS is highly complex. An effective instrument to measure QoL in ALS should:
In ALS, establishing and presenting the diagnosis is the first step in providing comprehensive care. We have an ethical obligation to disclose the diagnosis to the patient in a sensitive and humane manner. This obligation is outlined in the practice parameter guidelines recently published by the American Academy of Neurology.14 Because ALS is such a devastating disease with no effective treatment, how the diagnosis is disclosed and discussed with each patient and family strongly influences both the perception of the disease and the effectiveness of the subsequent care.14
The diagnosing neurologist should take particular care to be honest, frank, and sensitive in presenting the diagnosis.15 For example, if the facts are presented without a plan for care and support, the patient may feel abandoned. It is imperative to maintain a positive and hopeful attitude and to strongly assure the patient that he or she will receive the highest quality of care. Patients need to be assured that they will be supported throughout the disease, that symptomatic treatment will be available, that specific goals will be set for treating the disease itself, and, furthermore, that ALS is not caused by specific activities or affiliations (careers, culture, religion, etc). During the appointment when the diagnosis is presented, the patient's spouse, companion, or key support person should be involved, if the patient allows. Involvement of such people at this time will reinforce the patient's available support system and provide opportunity for more information gathering. This is a long and often difficult process, which requires a significant amount of time, and in no way should it be replaced by a telephone conversation or occur during a busy clinic.
Although providing patient education and information is imperative during this appointment, too much information at this time may be overwhelming - often at the diagnostic visit, patients do not remember anything more than the diagnosis and the manner in which it was delivered. Discussing the general disease process, treatments, and what the patient and family can do may be the best approach at this time. At a second appointment, scheduled for very shortly after the diagnosis with the multidisciplinary team, more detailed information can be provided. In this way, patients will be better able to assimilate the information they receive initially and will have an opportunity to consider the diagnosis and their questions. When patients and family leave the neurologist's office, they must have a sense of hope, an outline of the future treatment plan including comprehensive care, and a follow-up appointment scheduled.16
Comprehensive care is critical for patients with ALS. Treatment of ALS is extraordinarily demanding for both patients and healthcare providers because it involves severe disability, a number of medical areas, and psychosocial issues. Medical issues are only one aspect of ALS care and management. Psychological, spiritual and financial concerns will arise that also must be addressed. The treating neurologist cannot manage such complex issues alone. Consequently, a multidisciplinary team approach within an ALS clinic is preferable16-18 but cannot always be accomplished. Therefore, the essential areas that need to be addressed within a multidisciplinary framework are the presentation of the diagnosis, symptomatic treatment, and the timing and content of the delivery of multidisciplinary care.16-20
Within 2 to 4 weeks after the patient receives the diagnosis, comprehensive care should be started. The timing of this first follow-up appointment is crucial because the patients and family members need time to consider the information they have been given, but rapid follow-up is needed too, not only so that patients can begin treatment but also so that they do not feel abandoned. At this second visit, the advanced practice nurse who will be coordinating the team for the patient's care can provide additional detailed patient education. The next challenge in starting comprehensive care relates to access to multidisciplinary clinics. Such ALS clinics are not available at many medical centers; thus, the ALS team must contact each patient's community healthcare professional, often the local neurologist, to begin coordination of care. Although the patient's local neurologist may be willing to make arrangements for local multidisciplinary treatment, he or she most likely will not have access to healthcare providers who have the specialist expertise of those who work in ALS centers. Consequently, effective collaboration is critical.
The quality of the ALS clinic must also be ensured so patients receive the most effective care. Establishing care pathways, consulting with other ALS centers to improve quality, offering educational programming to community providers, and practicing current treatment approaches are all part of quality care in ALS. Achieving these goals should be easier since the publication of the American Academy of Neurology practice parameters9 and the implementation of the ALS CARE database.14 Developing and participating in a database specific to each center, as well as nationally, is also recommended because it will ensure that the ALS teams perform identical evaluations as well as offer standardized interventions, thus minimizing unacceptable variability among therapists (Hiroshi Mitsumoto, MD, and Vanina Dal Bello-Haas, PT, MSEd, unpublished observation).
The importance of patient education should not be undervalued. The better educated our patients are concerning the choices available and the outcomes of these choices, the better we will be able to improve their QoL. Continued efforts to offer community programming and to develop written materials on ALS, its treatment and care (on the internet as well as in traditional written format) can only improve the care of our patients, not only because they will have a better understanding of their disease but also because their professional caregivers will. Caution should be taken regarding the mass amount of information on the internet, as well as non-scientific therapeutic claims to treat the disease. Patients should be strongly encouraged to discuss new information with their clinical team.
At present, riluzole (Rilutek) is the only prescription drug available for the treatment of ALS. Studies of riluzole showed a modest benefit in prolonging survival21 in clinical trials, particularly when initiated early in treatment, suggesting that the drug may work better in the early stages of the disease.22,23 Treating neurologists should explain to their patients its availability, as well as how neurologists believe that it will be a cornerstone in future treatments that involve combination drug treatment. How it will affect the disease and its cost must also be discussed. Often patients get discouraged when they do not see an effect after taking riluzole for a period of time. Therefore, to help ensure compliance and to prevent loss of hope, it is essential to make sure that patients understand that riluzole may slow disease progression but will not produce outward improvement. Often insurance companies do not provide coverage for riluzole. This places a financial, thus emotional burden on the patient and family as the cost of riluzole is upwards of $700 per month. Is the benefit of riluzole enough to pay out of pocket or are these monies better spent on living each day or covering the cost of home care?
Probably the most important management issue for patients is effective symptomatic treatment. We need to remind ourselves that we can do so much for patients by aggressively pursuing treatment of symptoms such as muscle cramps and spasms, spasticity, pain, sialorrhea, dry mouth, depression, anxiety, sleep disturbances and constipation.22,24 What might otherwise be an unbearable loss of function may seem more manageable to the patient if painful or socially unacceptable symptoms can be alleviated. Choices regarding specific interventions and medical equipment for physical, speech and occupational therapy are beyond the scope of this paper and are well documented in other ALS sources.
Difficulty in speaking severely reduces the QoL for patients with ALS. To improve a patient's ability to communicate, use of an augmentative communication device is recommended by many ALS centers. However, a number of problems must be overcome before a patient can effectively use such devices. Patients may initially resist using them; whether this is because patients do not realize the degree to which their speech is unintelligible, or the degree to which their speech may become affected, or if we have failed in educating our patients when they decline these devices, is uncertain. Sometimes, language barriers present a problem because devices are not available even in many of the more commonly spoken languages. Further, although many options for augmentative communication devices exist, choosing the best option for the patient involves detailed evaluation of the individual, their social and work environments, insurance coverage and finances, as well as their cognitive function. Consequently, selecting the right device can be a complex process that requires extensive work on the part of the multidisciplinary team and it is usually the use of more than one device that works best for patients.
The goal of nutritional support is to maintain daily nutrition and hydration requirements so as to minimize muscle atrophy and weakness.14 For those patients whose condition continues to deteriorate, percutaneous gastrostomy (PEG) tube placement may be necessary to prolong survival.25 This possibility and its consequences must be discussed candidly with the patient. Whether PEG treatment actually improves QoL must be established through further studies.
Experts in ALS care agree that the assessment of respiratory strength via forced vital capacity (FVC) should determine when the PEG tube is placed.14 However, in our experience, patients tend to defer making a decision regarding PEG as long as they can. Consequently, by the time the patient decides to have the tube placed, the FVC may be fairly low.26 For that patient whose FVC is so low that a PEG procedure may result in complications (e.g. an FVC below 50%), using non-invasive positive pressure ventilation, such as biphasic positive airway pressure (BiPAP), during the procedure may be helpful. However, whether such an approach does prevent complications is uncertain. Clearly, the best situation is to place the PEG tube before FVC becomes problematic. This decision is not easy, but we can help our patients by striving to better educate them concerning nutrition and PEG. They then may be able to more quickly make an informed decision with which they are satisfied.25,26
Providing respiratory support via BiPAP is a common practice in ALS treatment and provides symptomatic relief. However, many questions remain concerning its use. For example, when to initiate BiPAP and how to best deliver the respiratory support are uncertain. Furthermore, whether BiPAP is a purely symptomatic treatment or actually prolongs survival is unknown.1,27-30 Recently, the trend in ALS treatment has been to start BiPAP in the early stages of respiratory compromise, but at least one study does not support this approach.31 Abboussouan and colleagues32 also found that BiPAP did not improve respiratory function, and moreover, they found that FVC significantly declined after BiPAP was initiated. Until further prospective studies clarify the use and effectiveness of BiPAP in ALS, ALS medical providers continue to use it for symptomatic treatment of respiratory difficulty.29 Parallel analyses should be performed to determine whether BiPAP also improves QoL in ALS, whether initiated early or late in respiratory compromise. Well-designed prospective studies are urgently needed in this area.14
The transition from non-invasive to invasive mechanical ventilation requires thoughtful and timely discussion so that decisions can be made proactively. This step ties in closely with the need for advance directives to identify and respect the patient's desires for self-determination.33-35 In order that patients can make decisions regarding respiratory management in ALS, intense and consistent education is essential concerning disease progression and how to manage ultimate respiratory failure.33,34 Patients must understand that to not make a decision about management of respiratory failure in the future is in fact a decision to be mechanically ventilated and that the absence of a decision needs to be documented for their protection. This protection comes in the form of medical and financial planning. Additionally, this documentation assures the patient and family that their medical team knows their feelings regarding respiratory issues and will do their best to support the patient's decisions. Therefore, advance directive should be displayed in all patient records and shared amongst the healthcare team, and community providers. Documentation of the patient's wishes becomes especially important when a ventilated patient may become "locked in," as their QoL may change, thus changing their desire to maintain mechanical ventilation.
At least one retrospective study indicates that patients who are on invasive ventilatory support are generally happy with their choice and would choose it again if they were in the same situation.36 Prospective studies will provide more accurate information on QoL issues involved in invasive respiratory support and guidance on how to better approach this issue.20
Living with ALS is a tremendous financial challenge for patients and families.16 In the world of managed care, healthcare providers may spend more time justifying interventions to the insurer than with their patients. The justification and coverage of treatments such as riluzole and assistive devices to improve QoL remain a great challenge. Attempts to prove to insurance providers that the multidisciplinary care of patients with ALS is cost-effective are ongoing. Obtaining evidence from studies that analyze outcomes where recommended care pathways are employed is key to this goal. Financial constraints exist in most countries but in some, such as Japan, patients with ALS receive full payment for unlimited hospital stays from their national government, including in-hospital ventilatory care.
Where should care be provided when patients become progressively less mobile—in a hospital, a long-term care facility, an assisted living facility, or at home? Patients usually choose home for many reasons. In reality, a major reason why the home is almost always the primary care environment in the later stages of the disease is because insurers very rarely approve coverage for hospital or nursing home stays for ALS patients at this point. Although home care is often thought to be the best option, if done "right",17,37 many substantial hurdles must be overcome. Family members who are caregivers have only a limited amount of time each day to care for the patient, given the need to work outside the home to maintain household income and insurance coverage.38 As a result, home health aides and home care teams are required. Unfortunately, home care providers have limited knowledge as to how to care for patients with ALS, and the care provided is often inconsistent between the primary home care nurses and home health aides. Furthermore, insurance coverage is limited for home care. Also, typically the home is transformed into a mini-hospital, disrupting the family's living space and feelings of "home" considerably. All these factors lead to caregiver burden and stress, which can be high at times; patients are acutely aware of this and often feel strong guilt for the situation their loved ones are in, believing they have "caused" the problems.38
Caregiver issues are often the last to be attended to. Physical and mental exhaustion, loss of privacy, loss of social activity and the financial burden of caring for one with ALS are only a few of the issues caregivers face.38 Although we are summarizing the problems as if we know them well, providers of ALS care have much to learn. Presently, we approach home care on the premise that consistent, streamlined, supervised care in the home can potentially decrease caregiver burden and improve QoL for the patient and family. This goal can be achieved if the continuum of care includes a strong foundation of care at an ALS clinic. Further studies are critical to show that such an approach improves care and QoL.
To help the patient make a successful transition from traditional home care to hospice care requires a keen understanding of the disease. A smooth transition is often not achieved because most home care professionals have limited knowledge of ALS and its transition to the active terminal stage.38 Moreover, most hospice programs focus on the needs of cancer patients specifically and are unaware of how to provide effective palliative care to patients with ALS. The ongoing challenge of how to define the terminal stages of ALS further compounds the problems of providing adequate hospice care.39
The term 'hospice care' is often deceiving because it is defined as the delivery of palliative care to a person with a disease that does not respond to curative treatment. ALS has no cure and therefore qualifies for hospice care from the time of diagnosis. Critical to the provision of palliative care within the clinic or home is the development of care pathways to treat symptoms. Basic questions concerning what medications are best, how they are to be administered and when they should be started still need to be answered.39 Once we improve symptomatic care, we will ultimately be able to improve how patients live and die with ALS.40
Care, whether it is provided in a hospice facility or in the home, should ideally be structured so that the palliative care team is an extension of the ALS multidisciplinary team.17 Under the guidance and supervision of the ALS team, palliative care should offer: 1. respite for both the patient and caregiver; 2. assistance to the patient and family to ensure that they maintain control, independence, and function; 3. respect for the patient's dignity; 4. state-of-the-art pathways to promote comfort and dignity throughout the disease process.
A guiding idea in delivering palliative care should be that we not only are striving to improve quality of living but also the quality of dying.
Patients with ALS who are less distressed and less depressed live longer, suggesting that psychological well-being is an important factor in the prognosis of ALS.41 In fact, recent studies indicate that QoL correlates poorly with functional impairment but fairly well with psychosocial and spiritual or existential factors, particularly when the disease progresses.10 Thus, we need to pay more attention to psychosocial and spiritual issues when patients enter the more advanced stages of the disease. To address these needs, mental health professionals should be part of the ALS care team or available as consultants to the multidisciplinary ALS clinics.
Rabkin and colleagues recently pointed out that clinical depression or significant depressive symptomatology is not an inevitable or common outcome in life-threatening diseases like ALS, even in the presence of major disability.42 In contrast to this finding, neurologists tend to aggressively treat what is perceived to be depression. Typically, tricyclic antidepressants are prescribed as opposed to psychotherapy, but given that clinical depression may not be common, we need to consider whether this is the right approach. Again, further studies appear to be needed.
A number of other, perhaps less tangible, factors carry much weight in a patient's QoL. They include hope, the influence of healthcare providers, and the patient's own spiritual or existential strengths before the illness.
We believe that hope is a driving force that enables patients and families to persevere and that it is one of the most important factors in maintaining QoL in ALS.43 Although neither the cause of ALS nor a cure is in immediate sight, vigorous research and clinical trials provide the strongest hope for everyone involved in the ALS community. Government funding, voluntary disease-centered associations, and philanthropic organizations are vital in supporting key research in ALS.
Clinical trials are a crossroad for patients who want to try potentially beneficial drugs, for investigators who want to treat the disease, and pharmaceutical companies who want to develop a new drug for ALS. It is likely that we will continue to see vigorous clinical trials in ALS in the future. The World Federation of Neurology (WFN) has developed standard guidelines and recommendations for conducting clinical trials in ALS.44 Our challenge is to recognize and emphasize the importance of hope for patients and families. Hope may vary (for example, its importance, what brings hope for the patient) from patient to patient and also from stage to stage throughout the disease continuum. Health- care providers must be keenly aware of how hope manifests itself, so they can recognize it in their patients and families and work to develop and maintain that hope.
It is important to remember that neurologists and allied healthcare professionals can greatly influence patients and families. Even if we do not express our opinions specifically, our attitudes, beliefs, and opinions are conveyed to patients and families in many ways. Thus, if we believe in the progress in ALS research and the comprehensive approach to care, these beliefs will be communicated to our patients and hopefully they, too, will feel encouraged. Our patients must perceive our positive outlook and hope. We must also approach each patient and family as individuals. Accordingly, we need to be compassionate, warm, empathetic, and sensitive to each patient's unique needs.
If we are to convey this attitude, we must remember that even how we obtain objective evaluations of the patient's condition can affect their outlook. For example, measuring muscle strength in nearly or completely paralyzed muscles or asking the patient "how disabled or depressed are you?" focuses on the degree of disability and can be counterproductive. This does not necessarily require a new evaluation tool but rather a different approach to performing and discussing the evaluation with patients and their families. All healthcare providers in ALS must work toward framing the patient's condition from a more positive perspective, emphasizing the physical and mental strengths that the patients possess, despite the physical deterioration. The potential for the healthcare professional to experience "burn out" or feel that the terminal nature of the disease is "too depressing" may be present if their psychological needs are not met. Just as patients and families need mental health evaluation and support, so do healthcare professionals. Mental health support offers an opportunity for the multidisciplinary team to process their feelings and experiences related to their patient care and to ultimately maintain psychological well-being which will be beneficial to their patients in the end.
Also, healthcare professionals tend to make assumptions about the factors and issues that affect the patient's QoL. This interpretation is then translated into specific interventions based on these assumptions. However, we must remember that QoL is highly subjective and that the level of QoL cannot be assumed based on the presence of functional limitations.45 Bach45 warns us that healthcare professionals universally rate a patient's QoL much lower than the patients rate their own QoL. Such discrepancy presents another important reason to strive for a more effective QoL assessment tool in ALS.
Finally, we must ask ourselves, "How are we doing?" Analyses of data in the ALS CARE database and the equivalent European database will provide clear goals for improving the current and future care and management in ALS.9,46 We await these results with much enthusiasm. Yet it is incumbent upon us as individuals to constantly review the care and management options we offer to our patients and families living with this devastating disease.
Why do some patients and families experience little or no burden or stress in the presence of ALS? Is there something more to the "equation of care" than what we offer as medical professionals that makes this so? Some people may have positive attitudes toward life and existence—perhaps they are more proactive in their spiritual or existential beliefs, are more goal-oriented, or have strong life objectives. Are these traits inherent or can we foster this type of approach in our patients? Most often, these characteristics were part of the patient's personality and life before they became ill. Yet, our task for improving QoL is perhaps to guide patients and families to adopt this approach. Introducing patients to religious, spiritual, or existential experiences (e.g., meditation) may be one way; guiding them in maintaining or forming new goals or objectives in life may be another way. Our experience tells us there is something more than medicine that produces well-being. We need to understand psychological and spiritual strength better as they relate to ALS.
We have discussed a number of areas in which the QoL may be improved for patients with ALS. Comprehensive multidisciplinary care is the key to successfully improving overall QoL throughout ALS, from diagnosis to death. At the same time, hope, the attitudes and beliefs of the healthcare professional, and the patients and families themselves are important factors that strongly influence patient QoL. What we have considered in this paper is by no means new or unique to anyone who is engaged in caring for patients with ALS. Our intention is simply to reiterate the principles of general patient care and the ways to improve care and thus QoL, which all of us think about. However, the majority of our recommendations are not evidence-based. Although clinical management has become an important research objective, much remains to be done. Until we have better treatments, we must work toward one goal in ALS care—improving the QoL for our patients.
We would like to express our gratitude to our patients and their families for their willingness to openly discuss issues arising during their illness—they have taught us an extraordinary amount about ALS. Hiroshi Mitsumoto MD, in particular, offers thanks to Professor PN Leigh, Department of Clinical Neuroscience, Institute of Psychiatry, King's College, London, and the International ALS/MND Symposium Program Committee for giving him the opportunity to speak on this subject.
We also gratefully acknowledge the members of the Eleanor & Lou Gehrig MDA/ALS Center (Marion Brandis MA RN; Sheila Hayes MS, PT; Regan Schwam BA; Steven Albert PhD, MS; and Judith Rabkin PhD) and the founder of the center and our mentor, Lewis P Rowland MD. We are particularly grateful to the Muscular Dystrophy Association for their longstanding support to our ALS Center.
We also thank The Cleveland Clinic Foundation ALS Clinic members (Doreen Andrews-Hinders RN; Jennifer Bocian RN; Vanina Dal Bello-Haas PT, MSEd; and Terese Wheeler), Mary Lyon RN, at the ALS Association, Dee Holden Norris RN, at the Forbes H Norris ALS and Neuromuscular Foundation.
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