C49 PALLIATIVE CARE IN ALS: CURRENT INTERNATIONAL GUIDELINES AND INITIATIVES
HARDIMAN O1,2, BEDE P1,2, OLIVER D3, O BRANNAGÁIN D4
1Trinity College, Dublin, Ireland, 2Beaumont Hospital, Dublin, Ireland, 3Wisdom Hospice, Rochester, UK, 4Our Lady of Lourdes Hospital, Drogheda, Ireland
Email address for correspondence: [email protected]
Keywords: palliative care, guidelines
The neurodegenerative diseases are relentlessly progressive and in the absence of effective disease modifying therapies, the emphasis remains on symptom management and maintenance of quality of life for the patient and carer. Optimal management requires a palliative approach from diagnosis. Palliative care enhances quality of life of patients and their carers by managing medical symptoms while also addressing individual psychological social and spiritual needs. Emphasis is placed on patient autonomy and dignity.
Despite an international consensus that management of neurodegenerative disease should adopt a multidisciplinary approach, integration of palliative care into disease management varies considerably across health care systems. However, common themes and principles of engagement can be identified across different jurisdictions, and measurement systems have been established that can assess the impact of palliative care intervention. International consensus guidelines would assist in the development of a framework for active palliative care engagement in ALS and other neurodegenerative diseases.
C50 THE EFFECTIVENESS OF PALLIATIVE CARE IN THE LATER STAGES OF MND/ALS
OLIVER D1,2, VERONESE S2
1Wisdom Hospice, Rochester, UK, 2University of Kent, Chatham, UK
Email address for correspondence: [email protected]
Keywords: palliative care, effectivenss, quality of life
Background: Although palliative care has been involved widely in the care of people with ALS / MND there has been little research showing the effectiveness of the involvement. Previous studies on multiple sclerosis have shown limited effect.
Objectives: Using a mixed methods approach to both assess the perceived needs of people with progressive neurological disease and ascertain if specialist palliative care would reduce these needs and improve the quality of life of patients.
Methods: A qualitative needs assessment of 22 people with progressive neurological disease - ALS/MND, multiple sclerosis and Parkinson's disease - was undertaken with patients, their carers and professionals. A quantitative explorative randomised control trial of specialist palliative care was undertaken with 50 patients, comparing the immediate provision of the service with standard care.
Results: The qualitative study showed that there were many problems experienced by people with ALS/ MND, in all areas of care - physical, psychosocial, and spiritual. These needs had been noted by the professionals and they were positive about a new service to improve the care offered.
The randomised trial showed that there was statistically significant improvement in quality of life, pain, breathlessness, sleep disturbance and intestinal symptoms for the larger group of people with progressive neurological disease.
Conclusions: People with ALS/MND have a high burden of symptoms and other issues - psychological, social and spiritual. Specialist palliative care appears to be helpful in addressing these areas and in improving overall quality of life.
C51 OPINIONS AND BEHAVIORS OF JAPANESE AND AMERICAN NEUROLOGISTS REGARDING TRACHEOSTOMY WITH INVASIVE VENTILATION (TIV)
DIRIENZO A1, OGINO M2, RABKIN J1, GOETZ R1, PASMANTIER M1, MCELHINEY M1, MITSUMOTO H1, TIV STUDY GROUP2
1Columbia University, New York, NY, USA, 2Kitasato University, Tokyo, Japan
Email address for correspondence: [email protected]
Keywords: tracheostomy, invasive ventilation, questionnaire
Background: Studies in the United States, Europe and Japan have found widely disparate rates of Tracheostomy with Invasive Ventilation (TIV) use between countries, between areas within countries, and even in the same hospital. Many contributory factors have been suggested, including the treating neurologist's opinions.
Objectives: To obtain neurologists’ approach to TIV in ALS patients.
Methods: We conducted national surveys of American and Japanese neurologists who specialize in ALS, selected because American patients have among the lowest, and Japanese the highest rates of TIV. We are asking patients and caregivers parallel questions. Here we present selected findings from the neurologist surveys.
E-mail address lists of American neurologists were obtained from the Muscular Dystrophy Association and the ALS Association, and cover letters were sent with surveys as attachments. In Japan, surveys were mailed to neurologists at major institutions caring for ALS patients.
Results: 100 American neurologists, living in 44 states, and 80 Japanese neurologists have responded to date. Although over half of each sample has practiced neurology for more than 20 years, Americans were far more specialized: 77% currently treat > 20 patients/year, vs. 11% of Japanese neurologists.
While no Americans said their role was to make treatment decisions for their patients and inform them what will be done, 21% of Japanese neurologists do so. When asked what proportion of their own patients got TIV, 5% of American and 36% of Japanese said more than one-quarter of their patients did so. When we asked whether they generally suggest and encourage use of TIV, 79% of Americans and 38% of Japanese said “never” or “almost never.”
Finally, of the 90 American neurologists who responded, 70% said they had been asked by patients or family members to discontinue TIV, compared to 59% of the 51 Japanese who responded. This difference is not statistically significant. Americans all complied, usually after several explanatory discussions, sometimes with an Ethics Committee consultation. In Japan, withdrawal of permanent ventilation is subject to prosecution.
Despite wide divergence in the likelihood of recommending TIV, and having patients get TIV, there is a striking similarity among American and Japanese neurologists when asked whether, if they themselves got ALS, would they accept TIV. Only 7% of Americans and 14% of Japanese said “yes” or “probably yes,” compared to 76% of Americans and 71% of Japanese who said “no” or “probably no.”
Discussion and conclusions: Compared to their Japanese colleagues, American neurologists do not encourage TIV, and have few patients who choose it. However, the large majority of both American and Japanese neurologists would decline TIV if they themselves were diagnosed with ALS. This surprising convergence of personal preferences suggests that other factors play a significant role in neurologists’ behavior and recommendations regarding TIV.
MDA funded
C52 COMPUTER ACCESSIBILITY: RECOMMENDING HEALTHCARE PROFESSIONALS AS A RESOURCE FOR INDIVIDUALS WITH ALS
FELDMAN S, HEIMAN-PATTERSON T
Drexel University College of Medicine, Philadelphia, PA, USA
Email address for correspondence: [email protected]
Keywords: computer access, assistive technology, healthcare professional
Background: New technologies are enabling individuals with motor disabilities to have alternative access to their computers. Though information regarding these technologies is available from a variety of sources, individuals with amyotrophic lateral sclerosis (ALS) present to the center with limited knowledge about these features.
Objectives: This study will collect information to evaluate the level of computer accessibility, familiarity and expectations of resources to determine if there is a need to provide education in this area.
Methods: A questionnaire was administered to individuals with ALS to establish their requirements and determine difficulties accessing their computers. They were asked to rate any difficulty they were having with their computer keyboard and/or mouse; their knowledge of the accessibility features currently available on the computer; and their knowledge of related software or hardware. Information was collected on how they have gained their existing knowledge and what they think the proper venue to obtain future education would be. The ALSFRS-R was recorded. In addition, the individuals rated their quality of life on a single item scale. The data was analyzed using the t-test (p = 0.05).
Results: A total of 40 subjects completed the questionnaire: 26 used the computer for pleasure only, none for work only, 13 for both, and one was unable to use the computer.
Only 7% of the subjects reported they never had difficulty with either the keyboard or the mouse. Over 60% of subjects indicated that their computer use was limited by accessibility. In reference to the accessibility features, 30% of subjects indicated ‘Good’ to ‘Excellent’ knowledge while 70% related that they had ‘Fair’ to ‘No’ knowledge.
Fifteen subjects reported no knowledge of accessibility features and 25 reported at least some knowledge. Of these 25 subjects, 60% indicated that they had gained their knowledge on their own. Only 15% of people reported a healthcare professional was involved as one of their resources, yet 72% of people indicated that they would expect a healthcare professional to be a resource. In addition, individuals with reduced hand function as measured by the ALSFRS-R score were more likely to indicate that a healthcare professional should be the source for information (p = 0.02).
Discussion and conclusions: Subjects reported a decrease in computer use as difficulty with accessibility increased. There was a high percentage of people with little or no knowledge of accessibility features (70%) and there was no consistent reliable resource. The majority of subjects indicated they would look to a healthcare professional as a resource. This is an unfulfilled need in this population and an assistive technology assessment would logically fit into the physical/occupational therapy evaluation in the clinical setting. It is recommended that healthcare professionals take the steps necessary to become a computer access resource for individuals with ALS.
C53 WWW.ALSHOME.DE – SELF-ASSESSED ONLINE SYMPTOM MONITORING IN ALS
MAIER A, HOLM T, LINKE P, MEYER R, MÜNCH C, STEINFURTH L, JANSSEN C, MEYER T
Charité University Hospital, Berlin, Germany
Email address for correspondence: [email protected]
Keywords: electronic self-assessment, online management, ALSFRSr
Background: Self-assessment of symptom progression and quality of life in chronic diseases is of increasing importance in clinical research and specialised outpatient healthcare. Against this background, we developed the Internet portal www.ALShome.de which provides online access to the ALS Functional Rating Scale, revised (ALSFRSr) and other established self-assessment questionnaires dealing with e.g. dyspnoea and appetite. In home care, an Internet-based self-assessment is a possible perspective for the monitoring of ALS-associated symptoms.
Objectives: To survey web-based self-assessment of ALS symptoms using the ALSFRSr and other established self-assessment questionnaires.
Methods: www.ALShome.de was created as a secure and closed Internet portal for patients. The application was developed in c-sharp (c#) with the persistent data storage being realised via an MS-SQL database throughout. Data are captured by generic questionnaires, are visualised on the website and administered via a content management system (CMS). Patients are assigned a discretionary number of online visits in defined intervals. In a prospective, controlled and stratified study, patients conduct a self-assessment of ALS-associated symptoms besides routine outpatient visits and home care.
Result: After one year of inclusion 162 patients (50 female, 112 male) gave informed consent to this study. Correlation between baseline and first online ALSFRSr (mean interval: 8.8 days) was excellent with a coefficient of 0.96 (p < 0.001) and the agreement of both capturing methods (online vs. onsite) was very good with more than 95% of all pairs of measurements within limits of agreement. 75% of the patients who attended a follow up onsite visit after 3.5 months in average performed online self-assessments all along. Patients were interviewed about the time burden and the emotional and physical strain of web visits. More than 95% felt that they are not at all or only slightly affected by these aspects.
Discussion and conclusion: The web-based self-assessment of ALS symptoms in a home care environment complements the well-established application of the ALSFRSr in outpatient departments. The results of our study indicate the good feasibility of the ALSFRSr as an Internet-based patient reported outcome measurement (PRO) and hence other PROs for the vertical and longitudinal measurement of outcomes. The study supports the hypothesis that innovative elements of self-management perspectively gain significant relevance in outpatient care.