P1 THE EFFECT OF MUSIC THERAPY FOR PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
Kawabata H1, Mihara Y1, Kurihara M1, Takahashi Y1, Nagashima T1, Tomita Y1, Takao M2, Mihara B1
1Institute of Brain and Blood Vessels, Mihara Memorial Hospital, Isesaki, Gumma, Japan, 2Department of Legal Medicine, Keio University, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: The definitive treatment for most intractable neurological diseases has not been established. The patients have to struggle with the disease for a long time. Since the psychological conditions of amyotrophic lateral sclerosis (ALS) are extremely distressing and stressful for patients, palliative care such as music therapy could be supportive. The aim of this study was to investigate the effects of music therapy for ALS patients who have low willingness and mental depression.
Method: Thirteen patients with ALS in a long‐term care ward were enrolled in this study. They were divided into two groups: the therapy group (11 cases, 63.91±10.9 years of age) and the non‐therapy group (8 cases, 66.0±11.2 years of age). Passive music therapy, appreciation of the patient's requested music, was provided for 30 to 45 minutes twice a week. We had eight sessions for each patient in the therapy group. We evaluated the Japan Stroke Scale‐Emotional Disturbance Scale‐ (JSS‐D, JSS‐E), and Vitality Index (VI). The serum level of an activity of NK cells was also measured before and after the music therapy.
Results: In the therapy group, the score of JSS‐D, JSS‐E was significantly decreased, and the score of VI increased significantly after the music therapy. The activity of NK cells also increased after the sessions. The changes of these scores and NK cell activity were not observed in the non‐therapy group.
Conclusion: Since the music therapy improved psychological conditions as well as immunocompetence, it may have beneficial effects as palliative care for individuals with ALS.
P2 COPING STRATEGIES IN LIFE WITH ALS/MND
Moeller J, Timm HU
Rehabilitation Centre for Neuromuscular Diseases, Aarhus, Denmark
E‐mail address for correspondence: [email protected]
Background: It is now possible to live with ALS for some years after respiratory failure has occurred by use of mechanical ventilation. To decide to live with permanent mechanical ventilation means that the patient and his or her family might have to live with the ongoing development of the disease and increasing disabilities for several years.
Knowledge about coping strategies for the ALS patient and the family to live with the ongoing losses of body functions and new problems from the disease, might be a resource for the professionals to give the right treatment and support at the right time.
Objectives: To acquire more knowledge about:
Which personal factors are important to ALS patients and their spouses to cope with the physical, psychological, economic and practical problems that are a consequence of having ALS
Which coping strategies can help people to have a good life despite ALS
Methods: Interviews were performed in ALS patients' homes from two counties in Denmark based on the following themes: 1) The impact of the disease on daily life; 2) Information obtained by the patient and the spouse about the disease and its consequences; 3) The former life of the patient and family; 4) Practical and personal help; 5) Expectations for the future, including choice of life‐prolonging treatment. At every interview the patient completed a Sense of Coherence (SOC) questionnaire, and at the first interview the patient and the spouse individually completed SF36. Analysis of the interviews was based on theoretical aspects of coping strategies.
Results: Twenty‐three patients (16 males and 7 females) were included from January 2004 to November 2005. Only one female lived alone. All participants were interviewed once and 17 were interviewed a second time. Five widows were interviewed within six months after their husbands had died. Ten participants have died and two live with permanent mechanical ventilation.
Conclusion: Analysis of the interviews is not finished but preliminary conclusions are:
Coping strategies among patients with ALS are similar to the way they coped with problems before the disease. Knowledge of the previous life of the patient is therefore a good resource for professionals supporting ALS patients.
Finding life meaningful and manageable for ALS patients seems to depend on what the individual patient considers to be important to preserve quality of life. Information from the professionals, degree of disability and dependence on physical and practical help are important, but not crucial for coping with the disease.
P3 PATIENTS' EXPERIENCES WHEN DIAGNOSED WITH ALS
Tomoko T, Yuasa T
1Tokyo International University, Tokyo, Japan, 2National Center of Neurology and Psychiatry, Ichikawa, Japan
E‐mail address for correspondence: [email protected]
Background: Disclosing the truth to patients with ALS is a daunting task for physicians. Today, many agree that the diagnosis should be announced directly to patients, preferably in the presence of patients' family member(s). Now the issue is when and how to tell the truth and there is no consensus of opinion. Probably there will be no single answer. In order to perform this difficult task in better way, we must learn what they feel and experience when they are confronted with the bad news.
Objectives: To understand the psychological state of patients when they are diagnosed with ALS. A better understanding of their emotional experiences will help us in providing holistic care and will enhance the QOL of patients.
Methods: Semi‐structured interviews were conducted with 18 patients (including seven ventilated). They were asked to report what they felt when diagnosed with ALS and any other comments on their experiences. Those who were not told the diagnosis reported their feelings when they knew the truth by other means. The purpose of the study was explained and they agreed to cooperate. Male: female ratio was 2:1 and mean age was 53 years (range 67–34 years).
Results: Fourteen patients were told the diagnosis and four were not. Various feelings were reported such as shock, disbelief, surprise, anger and anxiety. Five accepted the fact unperturbedly. Seven reported that they “felt somewhat relieved that the disease was identified”. Patients often expressed that “unidentified suspended state is harder than having an intractable disease”. Those who were indirectly told that they had ALS felt inadequate. Satisfied patients reported that their doctor was a good listener, sincere, and not too pessimistic but hope‐giving. Many emphasized the necessity of psychological care for themselves as well as for their family.
Discussion and conclusions: As all the patients could recall their emotional experiences clearly, it was an unforgettable life event for them. Identity issue has the greatest meaning. To live with ALS, they need truth, hope, psychological care and opportunity to express their feelings.
P4 PEOPLE WITH ALS/MND AS AUTHORS: WHO IS WRITING ABOUT LIFE WITH ALS/MND?
O'Brien MR1, Clark D2
1Edge Hill, Ormskirk, Lancashire, UK, 2Lancaster University, Lancaster, UK
E‐mail address for correspondence: [email protected]
Background: Health professionals are increasingly recognizing the value of illness narratives to inform them about the personal experiences of living with illness. Illness narratives are obtained directly from patients during interviews, or from their writing published in print medium or posted electronically on the internet. To date no published work has sought to investigate the use of illness narratives by people with ALS/MND.
Objectives: To identify published and unpublished illness narratives written by people with ALS/MND and compare the genres to establish the characteristics of the authors.
Method: A comprehensive search strategy was adopted to identify and locate electronic and print medium narratives written by people with ALS/MND. Following this scoping exercise, identified literature was assessed according to inclusion/exclusion criteria to obtain a comprehensive sample of 152 narratives (88 print narratives, 64 electronic narratives) to subject to analysis. Demographic details including sex, geographical location of author, occupation, age at diagnosis, decade of diagnosis and publication were recorded where available and comparisons made across the genres.
Results: Men wrote more than 60% of all narratives and within each genre produced more illness narratives than women. Although people from a number of countries worldwide have written about their illness experiences, over 60% of all narratives emanate from the USA. People from a wide variety of occupational backgrounds documented their illness experiences; 62% of authors specified their occupation. Twenty‐five per cent of print medium accounts were written by professional writers (academics/journalists); people in office‐based occupations (supervisor/clerical) wrote 21% and management/business people wrote 16% of printed narratives. Electronic narratives, in contrast, were dominated by writings from unskilled/manual workers and members of the emergency services/armed forces, who each produced 16% of the internet material.
The author's age at diagnosis was included in 55% of all narratives (range 20–76 years); those aged under 40 years of age at the time of their diagnosis produced more electronic (47%) than print medium (31%) narratives; while those aged over 41 years at diagnosis produced more print narratives (68%) than internet narratives (50%). The experiences reported within both genres spanned the decades from the 1960s; however, 75% of authors, regardless of the genre they adopted, received their diagnosis during the 1990s.
Discussion and conclusions: This study demonstrates the extent and availability of written narratives about the personal experience of living with ALS/MND. A substantial quantity of both published and unpublished personal illness narratives from the 1960s onwards has been identified. A broad cross‐section of people with ALS/MND, including those not normally associated with publication of personal stories, have documented their illness experiences within print and electronic mediums. The study has identified an unparalleled collection of personal illness narratives about life with ALS/MND that will help to illuminate what it means to be diagnosed with the illness.
P5 DISASTER PREPAREDNESS FOR THE FAMILIES OF PEOPLE WITH ALS
Kitamura Y, Kawamura H
National Rehabilitation Center, Tokorozawa, Saitama, Japan
E‐mail address for correspondence: [email protected]
Background and objectives: The purpose of this study was to learn about current evacuation plans for ALS patients with invasive ventilators at home in case of natural disasters. At least 30% of ALS patients wear invasive ventilators and 30% of them stay at home in Japan, where typhoons kill about 200 people a year and a large earthquake occurs every ten years. The Japanese government has been considering its role and encouraging local government to prepare shelters and plan for people with special health needs in case of natural disasters. However, the plans are currently mainly focused for hospital settings and shelters, not for ALS patients at home.
Methods: Semi‐structured interviews were conducted with five ALS patients with ventilators at home and seven family members. The conversations with the patients were helped by family members using transparent boards. The family members included two wives, one elder sister, one nephew and two daughters. The interviews were fully recorded with an IC recorder and transcribed to analyse the contents.
Results: 1) Every patients had at least one emergency bag for power cuts, and had the TV sets at lower places to avoid hitting the patients during earthquakes; 2) Every patient knew the place of evacuation that the local government had prepared for the residents, but none of them had ever tried to reach the place; 3) Everybody said that they would not move from the house until a rescuer came to help; this is because usually one family member, sometimes a young child, stays with the patient and there would be nobody to go and call for help. It also takes about two or three hours to prepare to go out; 4) Two patients had experienced no offer of any help by local government in the case of a minor natural disaster.
Discussion: The following preparations are suggested to be required for ALS patients with ventilators at home, in case of natural disasters; 1) emergency drills and a hotline to the emergency office to ask to move the patients to a safe place; 2) nursing assistants if the regular assistants are unable to come in case of a major disaster. 3) care for young children of patients because they are possibly forced to work as the carer in an emergency.
P6 ALS PATIENTS AND THE ROLE OF THE COORDINATOR FOR PATIENTS WITH INTRACTABLE NEUROLOGICAL DISEASES
Nakai M1, Fujita N2, Narita Y3, Kuzuhara S4
1The Liaison Council of the Networking for the Patients with Intractable Diseases, Tsu, Japan, 2Mie Prefectural Government, Department of Health and Welfare, Tsu, Japan, 3Mie University Hospital, Medical Care Networking Centre, Tsu, Japan, 4Mie University, Department of Neurology, Tsu, Japan
E‐mail address for correspondence: [email protected]
Background: More than a half of the 47 prefectures of Japan have posted one or more coordinators for patients with intractable neurological diseases. Their activities have so influenced health care professionals, patients and family that they have been convinced that coordinators can help patients' domiciliary care. However, there are many difficulties in finding suitable long‐term care facilities or hospitals to take responsibility for respite care. In particular it is very difficult for patients with amyotrophic lateral sclerosis (ALS) who have received tracheostomies and are on mechanical ventilation to find such a place. In our prefecture, a coordinator for patients with intractable neurological diseases has been posted at the university hospital for three years, as an adjunct to the staff at the Department of Health and Welfare of the prefectural government. The coordinator is a registered nurse with more than 15 years' experience and has accepted many consultations and negotiations. However, it is unclear whether or not all cases that need consultation with the coordinator are able to come to her.
Objectives: The aim of the present study is to investigate the ratio of cases which were brought to the coordinator compared to patients with intractable neurological diseases finding a suitable facility from the public health nurse, at all the nine public health institutes in the prefecture in the 2005 fiscal year (from April 2005 to March 2006) and to review the issues from the viewpoint for ALS.
Methods: The study design was cross‐sectional and the survey was conducted using a questionnaire that asked the public health nurses at the public health institutes who had responsibility for the care of the patients registered for the specified diseases by the Ministry of Health, Welfare and Labour of Japan. The survey was carried out in April 2006.
Results: The questionnaires were retrieved from all the nine public health institutes. Except for one, all the cases where the public health nurses recognized the necessity of coordination in the 2005 fiscal year were referred to the coordinator. There were 40 case records with the coordinator in this period. The records consisted of 24 ALS and 16 cases of other diseases. The ratio of ALS in the cases was disproportionately and significantly high for the prevalence rate in this period.
Discussion and conclusions: The present study has confirmed that most of ALS cases recognized by public health institutes were referred to the coordinator in the 2005 fiscal year. In addition, these findings suggest a possibility that there may be more ALS cases that were not recognized by the public health nurses or were not registered as an intractable neurological disease for any reason.
P7 THE ACTIVITIES OF MEDICAL HEALTH CARE NETWORKS FOR SERIOUSLY INTRACTABLE NEUROLOGICAL DISEASES IN JAPAN
Tateishi T1, Iwaki M2, Kikuchi H1, Nakai T2, Kira J1
1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka City, Japan, 2The Fukuoka Prefectual Medical Committee for Intractable Disease, Fukuoka Prefectural Government, Fukuoka City, Japan
E‐mail address for correspondence: [email protected]
Background: Ten years ago there was no public care network for seriously intractable neurological diseases in the Fukuoka region of Japan, making it extremely difficult to find chronic care hospitals for such patients. Thus, the Prefectural Medical Health Care Network for seriously intractable neurological diseases was initiated in 1998 to improve the care for seriously intractable neurological diseases, especially ALS.
Objectives: To clarify the problems and benefits of the Prefectural Medical Health Care Network for seriously intractable neurological diseases in the Fukuoka prefecture in Japan for the past eight years.
Methods: We investigated ALS patient transfers, care conditions, hospital medication, problematic cases, and analysed the system management.
Results: The network now consists of 15 basic cooperating hospitals, and 105 general cooperating hospitals. The total number of patients with seriously intractable neurological diseases who were referred to the network in the last eight years was 277, 163 of whom were ALS patients, and 75 were on mechanical ventilation. Of these, 253 were successfully transferred to chronic care hospitals or referred to regional visiting physicians through the network. Cooperating and non‐cooperating hospitals accepted 157 and 58 patients, respectively, while 38 were referred to visiting physicians. Two medical care coordinators managed the transfer of the network's patients based on information on the patient's condition and the capacity of cooperating hospitals, using information about the number of available beds in cooperating hospitals. Each year we investigated the hospitalization and home care environments of these ALS patients. When the network was started, 61% of the transfers were declined by hospitals, which reduced to 38% in 2005 as the network became accepted. The number of medical care consultations has increased year by year, and reached 17,652 in the eighth year. The main issues for these consultations were to make hospital arrangements for a patient. To resolve these issues, care conferences for patients and their family members were held with the attending physician. Additionally, a comprehensive care network for each patient that included hospital management and also home, psychological and urgent care, was constructed. For any emergency problem in the system, home visiting physicians were selected from the enrolled doctors and hospitals. Three times a year we had open meetings with the network's participants for training and information exchange about cases with intractable neurological disease. These workshops and hospital visits considerably encouraged co‐medicals and social workers.
Discussion and conclusions: Comprehensive long‐term care is necessary for Japanese ALS patients because one‐third are on mechanical ventilation. Despite this, it is still difficult to secure available beds for such patients who require long‐term care. A more efficient system needs to be constructed and more information provided to surrounding hospitals and home doctors.
P8 THE PRESENT STATUS AND PROBLEMS WITH UPPER EXTREMITY ORTHOSIS IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
Imazeki A1, Ogino M2, Ogino Y2, Kamide N3, Asai N3, Sakai F2
1Department of Rehabilitation, Kitasato University East Hospital, Sgaminara Kanagawa, Japan, 2Department of Neurology, School of Medicine, 3 School of Allied Health Sciences, Kitasato University, Sgaminara Kanagawa, Japan
E‐mail address for correspondence: [email protected]
Background: Upper extremity orthosis is able to help patients who have a muscle weakness of proximal upper extremity muscles in the activities of daily living (ADL). There are some studies on the use of orthosis in patients with duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA) and cervical spinal cord injury (SCI), but studies about their use in ALS patients are few. However, upper limb impairment is present in all of these conditions. Thus, it is suggested that orthosis is able to be adapted to ALS as well as DMD, SMA and SCI.
Objective: The purpose of this study was to examine the present status and problems about adaptation of upper extremity orthosis in patients with ALS who have a weakness of proximal muscle of upper limbs.
Methods: Six patients who were diagnosed with ALS were included in this study. Inclusion criteria were: 1) at least one muscle in the shoulder girdle or shoulder or elbow was below a grade of poor or better in manual muscle test (MMT); 2) passive range of motion (ROM) in shoulder flexion and an abduction was 0–90 degrees, internal rotation was 0–90 degrees, and elbow flexion was 0–110 degrees; 3) stability and tolerance for sitting in a wheelchair for more than 30 minutes.
As an outcome measure, the following three items were collected: 1) Acquired ability or changed ability by introduction of upper extremity orthosis; 2) the period over which patients could use the orthosis; 3) reasons that patients were not able to use the orthosis.
Results: 1) Acquired ability or changed ability: four patients could eat, and one patient could input a keyboard independently. One patient could not adapt, because she had dementia. 2) Time period: all patients could use only short term, between one to four months. 3) Reasons for withdrawal: two patients could not use due to decreased muscle strength (progression of disease), and the welfare system in Japan did not permit it in another three patients.
Discussion and conclusions: For patients with ALS, it was suggested that both ADL and QOL were improved by the use of upper extremity orthosis, but the period in which it can be used is limited to the short term due to the rapid progression of the disease. It may be effective for patients with ALS to introduce upper extremity orthosis to improve ADL and QOL, but it is necessary to introduce it as soon as possible. We have to clarify the standard of adaptation of orthosis and also discuss the welfare system for ALS.
P9 ALS PATIENTS AND PRESSURE SORES ON ADMISSION AT A UNIVERSITY HOSPITAL
Hayashi T1, Narita Y1, Okugawa N2, Kuzuhara S2
1Medical Care Networking Centre, Mie University Hospital, Japan, 2Department of Neurology, Mie University, Tsu, Mie, Japan
E‐mail address for correspondence: [email protected]
Background: Relatively low incidence rates of pressure sores in patients with amyotrophic lateral sclerosis (ALS) have been noticed and some specific skin changes, both morphological and biochemical, were revealed to account for the resistance of ALS patients' skin against pressure sores. However it is still unclear whether or not the incidence rate of pressure sores in patients with ALS is less than in other neurological conditions.
Objectives: To review the incidence rates and outcomes of pressure sores in patients with neurological diseases on admission to our hospital, and compare the rate of ALS with those of other diseases.
Methods: All the 595 patients who had been admitted to the neurology ward of our University Hospital for two years from 1 April 2004 to 31 March 2006 were included in this survey. One of the authors, TH, a wound‐ostomy‐continence specialist nurse (WOC) has evaluated and treated any skin lesions of all the patients on the neurology ward since 2004. Transverse analysis from the medical and nursing records was performed, particularly focusing on the differences among patients with ALS, Parkinsonism and other neurological disorders. JMP ver. 5.1 (SAS Institute Inc.) was used for statistical analysis.
Results: Seventeen patients (13 males and 4 females, age 33–87 years, average 70.3±15.5 years old) had pressure sores on admission: three of 41 ALS patients (7.3%), five of 126 Parkinsonism patients (4.0%) with Parkinsonism (66 Parkinson's disease, 15 progressive supranuclear palsy, 24 multisystem atrophy, eight dementia with Lewy bodies, five corticobasal degeneration, and eight other Parkinsonism). A proportional analysis by χ2 test failed to show a significant difference of incidence rates of pressure sores in ALS patients. The patients with ALS who had pressure sores had some complications other than motor symptoms such as cerebrovascular disorder and cognitive impairment together with some problems in home care, similar to the pressure sores in other neurological diseases. None of the patients developed a new pressure sore after admission. Most of 17 patients had light or moderate pressure sores and relatively good improvements during their hospital stay.
Discussion and conclusions: This preliminary survey failed to reveal a large number of cases with pressure sores. One of the reasons for relatively low incidence rates of pressure sores may have been due to earlier cases admitted to a teaching hospital, and good care at home or long‐term facilities for patients with neurological diseases prior to admission. However, we would like to stress that pressure sores can occur in ALS patients, particularly in conditions where complications other than motor neuron symptoms occur or the patients are put in poor hygiene circumstances.
P10 FATIGUE IN INDIVIDUALS WITH MND/ LATE EFFECTS OF POLIOMYELITIS: WITH OR WITHOUT POST‐POLIOMYELITIS SYNDROME (PPS)
Quadros AAJ, Oliveira ASB, Silva HCA, Silva TM, Pereira RDB, Conde MTRP, Maggi FA, Ramos PE, Silva PG, Marques BRRN, Viana CF, Vieira EAP, Carmona CP, Gabbai AA
Department of Neurology, Federal University of São Paulo (UNIFESP/EPM), São Paulo, Brazil
E‐mail address for correspondence: [email protected]
Introduction: Paralytic poliomyelitis is an infectious viral disease that mainly affects children and, in its acute forms, causes inflammation and death of motor neurons of the spinal cord and brainstem, leading to paralysis, muscular atrophy, and often deformity‐ poliomyelitis sequelae (PS). Post‐poliomyelitis syndrome (PPS) is a clinical syndrome of new weakness, fatigue and pain, which can occur several decades following recovery from paralytic poliomyelitis. Fatigue is defined as a state with reduced capacity for work following a period of mental or physical activity. One of the main symptoms of these patients is fatigue.
Objective: To study fatigue in polio survivors and to compare fatigue severity in patients with PS and patients with PPS.
Methods: We performed a retrospective analysis of medical archives of 303 outpatients with prior polio paralytic history, followed at the neuromuscular sector of UNIFESP/EPM that answered Fatigue Severity Scale (FSS) questionnaire in the first evaluation. We compared patients with PS and PPS using χ2 analysis with significance level (p ⩽ 0.05) and OR value with 95% confidence interval.
Results: The whole group was composed of 181 women and 122 men, mean age 46 (25−78±9.5) years. Eighty‐eight (29.0%) patients had PS and 215 (71.0%) had PPS. Non‐fatigue was found in 52 (53.1%) in PS and 46.9% in PPS group. Fatigue severity was worse in the post‐polio syndrome: mild fatigue was observed in 16 (34.8%) patients with PS and 30 (65.2%) with PPS. Moderate fatigue was present in 10 (18.9%) patients with PS and 43 (81.1%) with PPS. Severe fatigue was found in 10 (9.4%) patients with PS and 96 (90.6%) patients with PPS. There was a significant statistical difference between groups in the severity fatigue variables.
Conclusion: Fatigue is presenting in both groups; however, fatigue is high more frequently and most severe in the post‐poliomyelitis syndrome than in the poliomyelitis sequelae group.
Acknowledgement: The study was supported by CNPq.
P11 COMMUNICATION METHODS FOR ALS PATIENTS USING A TELEVISED MOBILE PHONE SYSTEM
Imai T1, Tsubai F1, Shizawa S1, Kurihara K2, Osumi E3, Matsuo M4
1Miyagi National Hospital, Watari, Miyagi, Japan, 2Miyagi Council for Intractable Disorder, Sendai, Miyagi, Japan, 3Nishitaga National Hospital, Sendai, Miyagi, Japan, 4Funcom Co. Ltd., Moriguchi, Osaka, Japan
E‐mail address for correspondence: [email protected]
Background: ALS is a progressive neurodegenerative disorder, and dysarthria worsens gradually. The patients in advanced stages of this disease are not able to join in social activities or enjoy self‐supporting life.
Objective: To determine the utility of a televised mobile phone system as a means of communication support for ALS patients.
Methods: A 48‐year‐old male ALS patient living in Chiba City cooperated in the study after informed consent. He had been living with the disease for six years and wearing a mechanical ventilator for four years.
Stage 1. Preliminary experiments were performed on telecommunications using a televised mobile phone system between the Study Group Secretariat in Miyagi National Hospital and the patient's room in Chiba City with technical aids provided by a visiting technician.
Stage 2. In an educational session for patients with intractable disease, sponsored by the Miyagi Council for Intractable Disease Care, the patient provided a remote lecture on peer support from his room to the venue via the televised mobile phone system.
Results: 1) Preliminary experiments: the telecommunications between the Study Group Secretariat and the patient's room were successful with good quality in both sound and images. 2) A total of 90 people participated in the session, including patients' families and medical care personnel. The lecture with the theme ‘Peer Support – Empower Our Fellows through Creation’ took place using the televised mobile phone system, featuring the patient's images from his room in Chiba City and voices generated by a computerized communicator into which he input sentences by himself. The participants at the venue in Sendai City were able to listen to the lecture while watching the lecturer being projected on a screen. However, the voices were much less audible, owing to sound distortion, compared to the preliminary experiments when the volume on the speaker system was maximized. This was possibly because of the spatial situation at the venue where the system was used and the system's acoustic performance (i.e. personal computer, mobile phones, and speakers).
The session was highly rated by the participants. Their opinions included “I was deeply impressed by the patient's first‐hand talk and I highly appreciated the use of the TV phone system”, and “The use of the latest equipment by patients will extend the range of their daily activities and lead to their self‐fulfilment”. These opinions suggest the possibly increased feasibility of care support, including peer support, provided by a patient for other patients, using the televised mobile phone system.
Conclusion: In recent years there have been increasing opportunities for ALS patients wearing a mechanical ventilator to go outside their home, which imposes significant financial burdens associated with caregiver allowances, transportation fees and other expenses. In the present study, the use of the televised mobile phone system enabled the patient to make the remote lecture without the aid of caregivers. This system will enable patients with intractable disease to join social activities and may help them enjoy self‐supporting life.
P12 PERSONAL COMPUTER COMMUNICATION RESTORATION SOLUTIONS FOR THOSE WITH ALS/MND
Gallagher K
Taiwan MND Association, Taipei, Taiwan
E‐mail address for correspondence: [email protected]
Background: Taiwan is estimated to have between 800 and 1000 people with ALS/MND, but the true figure remains unknown. Of the around 250 registered with the Taiwan MND Association, over half have been identified as requiring AAC support. However, only a minimal number have actually received a communication aid, something the Association urgently seeks to redress. As standard AAC strategies will take time to reach these shores, and with limited government funding, other solutions are needed. This report should be read from a perspective of a country with developing rehabilitation social service structures.
Objectives: To find a low cost but effective way to resolve the communication difficulties of those with ALS/MND in the Taiwan regional area.
Methods: Taiwan is a computer literate society and has the benefits of low cost computer hardware. The concept was that, as many homes possess a personal computer, if this was used for communication restoration, the problems of cost and lack of AAC services could be largely overcome. However, to achieve this, alternative computer control devices have to be used, as few MND non‐verbal users have the necessary motor control for mouse operation. With a suitable alternative device, users would be free to use standard computer packages, such as Word, Power Point etc. for communication purposes. The user would also be provided with full access to other computer applications, such as multimedia, web access, etc. The device assessment outcome trade‐off was always between speed and reliable operability.
Results: Apart from TLS cases, successful devices could be found for all evaluated cases. Usage varied, but communication was restored to all. Although limited to only seven clients, the often dramatic increase in client quality of life has validated this method for communication restoration.
Discussion and Conclusions: All available alternative mouse devices were offered, but due to the progressive nature of the disease, in the future, assessment strategies will focus more on reliable operability and will see more use of single switch solutions (1). Restoring full PC control rather than focusing on only AAC devices has also provided client access to the world beyond the immediate environment. For those with only eye movement, studies into an eyeball switch/single switch mouse combination are underway.
References
- Hatakeyama T, Todoroki T. Human Interface 1996;12:139–44.
P13 THE COMMUNICATION METHOD FOR ALS PATIENTS WITH TPPV USING A LED SENSOR TO DETECT EYE MOVEMENT FROM EARLY PHASES OF THE DISEASE
Honma m, Nagumo H, Yamanaka Y, Takashita M, Yamaguchi H, Chikaura M, Hino H, Nagao M, Kawata A, Hayashi H
Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: For ALS patients with relentlessly aggravating motor impairments, support for their difficulties in pushing a switch for call is indispensable. In our hospital, when the patient's finger movement was not able to grasp and push the usual nurse call, we systematically changed the call devices with various types of sensors and means for switching such as grasping, pushing and touching, depending on the residual voluntary movements of the patients. ALS patients, however, always feel anxiety that they will not be able to use the current switch in the near future. On the other hand, ocular movement is the least affected voluntary movement, but it was generally used for a switch only after any other movement switches became unusable. This is partly due to the fact that the current call devices detecting the electrical activities of the extra‐ocular muscles by surface electrodes cannot necessarily pick up the patient's signs correctly.
Objectives and methods: To use the relatively well‐preserved ocular movement of ALS patients as a measure of switching from the early stage of the disease, we developed a switching device using a LED sensor, which can detect the difference in the light absorbance of white and black (or brown) colour of the eyeballs. To solve the problems of adjusting the subtle position and angle of LED sensor just before the eyeball, we developed a system using goggle glasses. To evaluate the usefulness of our device, we applied it to four ALS patients and nine healthy controls with informed consent.
Results: We attached a small transparent rubber sucker to the peripheral outer surface of the goggle glasses so as not to obstruct the patient's visual field. A LED and fiber sensor with leads to a battery was installed on a piece of flexible short wire fixed in the middle of the sucker. In this way, we could change the position of the sucker with LED sensor freely on the surface of the goggle glasses, and also change the direction of the leads to any direction. The light of LED could be projected onto the desired position of the eyeball easily. We defined that if a subject looked at the LED light it meant “Yes” and if not, it meant “No”.
The signals from 13 subjects using our device were stable and we could judge their signs correctly. Both ALS patients and healthy controls could look at the LED light transiently (within 1 second) or for several seconds. The latter ‘long switch on state’ may be applied to mean a third sign other than the Yes or No sign. The comments of ALS patients and controls were as follows: 1) This device is safe for their eyes; 2) It makes it possible to detect patient's intentions accurately; 3) It may be applied not only to simple Yes or No signs but also to signals for other more complicated communication devices.
Discussion and conclusions: Our device using LED sensor can sensitively detect the difference in the light absorbance of colours of the eyeballs and exchange the signals to Yes or No signs correctly. Compared to previously reported LED devices, our method is safe for eyes, easy to set and has possibilities of various uses from the early stage of the disease.
P14 THE CHINESE ALS HEALTH PROFILE STUDY: PRELIMINARY REPORT OF ALS CLINIC STUDY GROUP IN CHINA
Li X1, Cui L1, Fan D2, Jiang Y3, Zhang C4, Zheng H5, Li Y6, Liu M1, Zhang H2, Fan H3, Huo X5, Yao X4, Li X1
1Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 2Peking University Third Hospital, Beijing, 3Fudan University Huashan Hospital, Shanghai, 4Zhongshan Univesity First Hospital, Guangzhou, 5Second Military Medical University Changhai Hospital, Shanghai, 6Second Military Medical University Changzheng Hospital, Shanghai, China
E‐mail address for correspondence: [email protected]
Background: ALS starts with progressive muscle weakness, wasting and fasciculations, in conjunction with spasticity, hyperreflexia and pathological corticospinal tract findings characterized neuropathologically by degeneration of motor neurons in the brainstem, spinal cord and cerebral cortex. Clinical syndrome evolves to death in 3.5 years. Substantial variations exist in managing ALS in China, but there has been no database to measure ALS patients' health profile.
Objectives: The Chinese ALS Health Profile Study (HPS) was designed as a longitudinal survey of patients diagnosed with ALS. It was created to improve the quality of care for patients with ALS by 1) obtaining the profile of the patients diagnosed in China, 2) obtaining data on the physicians‐perceived health status and well‐being for patients with ALS, and 3) surveying the difficulties of the patients in complying with the treatment on a long‐term basis.
Methods: This observational database is open to six ALS clinics in three cities (Beijing, Shanghai, Guanzhou) of China. Data were collected between January and June 2002 by using standard data collection instruments. Forms were submitted to a central data‐coordinating centre. The Chinese ALS‐HPS has a First Visiting Form and Follow‐up Form which include general information, physician assessments and therapeutic interventions or aids currently in use. General information comprises name, sex, birth date, height, weight, hospital of diagnosis, month and year of symptom onset and diagnosis. Physician assessments comprise region affected, ALS type (familiar/sporadic), diagnostic classification, diagnostic test performed (e.g. EMG, motor NCV, conduction block test, routine blood test, thyroid function, serum anti‐GM1 antibody, brain/spine MRI, muscle biopsy) atypical features, forced vital capacity, ALS Functional Rating Scale, and SF‐36. Current clinical problems and medications were also recorded.
Results: Beginning in January 2002 through to 30 June 2002, 139 patients were enrolled at six clinical sites in three cities of China. On enrolment, patients had a mean age of 49.17 years, 68% were men and 88% were sporadic. The mean time from first symptom onset to confirmation of diagnosis was about 18 months. EMG was performed in 94% of patients. Brain and spine MRI, blood and CSF screening were widely used. Seventeen per cent of patients experienced depression. Riluzole was prescribed for 19% of patients overall. Vitamins or antioxidants and Chinese traditional medicine was prescribed for 45% and 25%, respectively. Physical therapy was the most common non‐pharmacological intervention (13.67%).
Conclusions: The Chinese ALS HPS appears to provide valuable data on physician practices and patient health profile in ALS. The information generated will help to identify problematic areas of management, which can be used to help to issue practice guidelines for ALS management in China, and early diagnosis should be emphasized.
P15 INDIVIDUALIZED QUALITY‐OF‐LIFE MEASUREMENT FOR PATIENTS WITH NEUROLOGICAL ‘INTRACTABLE DISEASE’ IN JAPAN
Ohbu S1, Nakajima T2, Akiyama M3, Narita Y4, Kuzuhara S4
1Department of Sociology, Rikkyo University, Tokyo, Japan, 2Niigata National Hospital, National Hosopital Organization, Kashiwazaki, Japan, 3Department of Psychiatric Nursing, School of Nursing,Tokyo Healthcare University, Tokyo, Japan, 4Department of Neurology, Mie University School of Medicine, Tsu, Japan
E‐mail address for correspondence: [email protected]
Background: Conventional quality of life (QoL) scales often have questions that are not relevant to many patients. The direct‐weighting version of the schedule for evaluation of individual quality of life (SEIQoL‐DW) can give more appropriate indicators of QoL.
Objectives: To develop a version of the SEIQoL‐DW for use in Japan, and to promote its use by health professionals caring for patients with neurological ‘Nanbyo’ (‘intractable diseases’).
Methods: In 2000 we used a preliminary Japanese version of the SEIQoL‐DW in the introductory part of a qualitative study of ALS patients and caregivers. In 2003 we had a special training program on the SEIQoL‐DW by two major government‐supported groups carrying out research on neurological intractable diseases. In that program we collected comments by participants. Considering their feedback, we improved the Japanese version and a back‐translation was reviewed by one of the original authors in 2005. We surveyed the state of utilization of the SEIQoL‐DW by sending e‐mail questionnaires to 49 registered users.
Results:Statistics. Responses (one per institution), 20; Number of cases of administration (per researcher), 0–50; Diseases – ALS, 8 researchers, stroke, 3, respiratory disease, 1; Average time spent for one exam, 20–40 minutes.
Reasons for using the SEIQoL‐DW. Closer to real QoL; can follow QoL even when patients' hopes and needs change; useful for evaluation of palliative care by health professionals; can reflect subjective QoL and is useful therapeutically.
Problems in practice. Can be invasive and harmful if examiner is not well trained; patients sometimes have trouble understanding concepts of ‘levels’ and ‘weighting’; time‐consuming; number of cues is sometimes less than 5; difficult to use with elderly patients; direct‐weighting instrument is difficult to handle (the instrument has five interlocking disks, which form a type of pie chart and can be rotated to indicate the relative importance that the patient assigns to each of five different areas of life).
Discussion and conclusions: The SEIQoL‐DW is an excellent tool to facilitate communication between patients and health professionals, and is a unique indicator of QoL for clinical research. We will continue promoting its use. We will also evaluate the psychometric properties of the Japanese version of the SEIQoL‐DW, and we will consider easier methods of administration (computer‐based).
P16 IDENTIFYING CARE ELEMENTS FOR PALS PROVIDED BY PERSONAL ASSISTANTS
Kawaguchi YK1, Kawamura SK2, Konagaya MK3
1Ritsumeikan University Graduate School of Core Ethics and Frontier Science, Suginami‐ku, Tokyo, Japan, 2Aomori University of Health and Welfare, Department of Nursing, Aomori City, Japan, 3Tokyo Women's Medical University Bulletin of School of Nursing, Shinjuku‐ku,Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: PALS in Japan have been provided with government funded home care services since 2000. Non‐professional personal assistants without any medical qualification are therefore urgently needed to provide home care services, which include tracheotomy suctioning at a patient's home. With that, there arises a need of identifying a standard of care among these unskilled caregivers.
Objective: We aimed to identify the standard daily care for PALS provided by personal assistants.
Method: We observed four PALS who had been receiving HMV for more than one year at their homes in Tokyo. A researcher recorded the frequency and situations of each care service or needs of the PALS on a minute basis throughout a day. Care service included respiratory management. When there were more than two assistants caring for a PALS at the same time, the total amount of care and time was calculated. The observations were recorded on a standard form. We then sorted the data into professional work and non‐professional work.
Results: The total time of respiratory management for one male PALS was 55 minutes a day. Three female PALS needed tracheotomy suctioning once or twice an hour, excluding sleeping hours; the total time ranged from 177 to 200 minutes a day. In addition, ‘to keep a close watch’ was the most needed care, which required a total time of 171 to 527 minutes a day. ‘To keep a close watch’ was to closely observe or monitor signs and symptoms of pain or uncomfortable feelings, general conditions, communication need, and airway management (tracheotomy suctioning).
Although personal assistants can provide some medical care including tracheotomy suctioning, because of a lack of proper training they still need support, advice or consultation from medical professionals at the care receiver's home at any time.
Conclusion: Low cost long‐term home care can be successfully achieved by utilizing care services of personal assistants who have received proper training from medical professionals.
P17 THE ALS‐SPECIFIC ADVANCE DIRECTIVES
Uchiyama T, Hashizume A, Yamaguchi M, Suzuki Y, Suzuki M, Itou M, Shimizu T, Atsumi T, Oohashi T
Seirei Hamamatsu General Hospital, Hamamatsu, Japan
E‐mail address for correspondence: [email protected]
Background: Patients with ALS encounter severe problems with nutritional and respiratory support when the disease progresses. The predictability of the natural history allows them to face the process of planning for their future medical care. Thus, advance directives might be a useful tool for improvement of their quality of life.
Objectives: The purpose of this study was to develop the ALS‐specific advance directives.
Methods: Patients with ALS at Seirei Hamamatsu General Hospital were recruited for the study. Retrospective data about advance directives were collected from hospital records between 1995 and 2004. According to the obtained findings, a list of terms and definitions that affected clear decision‐making was explored to develop the ALS‐specific advance directives. Finally, the developed format for advance directive was applied to patients newly diagnosed with ALS since 2004.
Results: Eighty‐nine patients were recruited. Prognosis and treatment options of ALS were openly discussed with 77 patients (87%), of whom 7 patients (9%) chose respiratory support, 24% nutritional support without ventilation, and 31% neither support. These decisions were described without the uniform format, and hospital records provided insufficient information about the reason for their choices. Moreover, the remaining patients' decisions could not be detected in hospital records. These findings generated the uniform format for advance directives, which consisted of understanding of the informed consent and definitions of future medical care. Additionally, a feature of this format was that the patient could reject the immediate decision, while he/she should describe the reasons of his/her decision regardless of the choice. Upon adequate explanation of ALS (1), the novel format was performed without any trouble in eight patients.
Conclusions: The developed format for advance directives in the study is anticipated to be ALS disease‐specific and to be useful in approaching conversations with patients about the end of life.
References
- Yamaguchi M, et al. Medical Journal of Seirei Hamamatsu General Hospital 2004; 4: 88–90 (in Japanese.)
P18 END OF LIFE DECISION MAKING: A CROSS CULTURAL STUDY
OLIVER D1, CAMPBELL C2, SLOAN R3, SYKES N4, TALLON C5, WEBB S6, ALBERT S7, NAKAJIMA T8 AND THE JAPANESE RESEARCH GROUP ON QUALITY OF LIFE
1Wisdom Hospice, Rochester, United Kingdom, 2St Catherine's Hospice, Scarborough, UK, 3Joseph Weld Hospice, Dorchester, UK, 4St Christopher's Hospice, London, UK, 5Cynthia Spencer Hospice, Northampton, UK, 6St Wilfrid's Hospice, Chichester, UK, 7University of Pittsburgh, Pittsburgh, USA, 8Niigata National Hospital, Kashiwazki, Japan
E‐mail address for correspondence: [email protected]
Background: The care of the person with MND/ALS is often complex and, as the disease progresses, there are many decisions that need to be taken. These are even more prominent as the later stages are reached, as decisions about the care at the end of life are necessary in anticipation of the deterioration, which may be only over a few days.
Objectives: The aim of this study is to look at the different approaches to end of life care in three countries – the UK, USA and Japan.
Methods: A questionnaire has been developed to allow the retrospective audit of the notes of patients who have died under the services.
Results: In the UK 47 sets of notes have been analysed and have shown that there are discussions with patients on some issues – in particular gastrostomy insertion – in 77% of cases, with a mean time before death of 9.6 months. Discussion on advance directives occurred in only 30% of cases, with a mean time before death of nine months, and for DNAR (Do Not Attempt Resuscitation) in 36% with a mean time before death of 6.1 months.
The analysis of 10 notes from the USA showed that advance directives were discussed with 80% of patients; DNAR was discussed with 70% and both were discussed with a mean of 10 months before death.
Fifty‐five notes from eight units in Japan were analysed; advance directives and DNAR had been discussed with 60% of the patients, although the details of decisions regarding end of life were unclear. However, the decisions made were different as 36% of patients received non‐invasive ventilation for a mean time of five months (UK 15% for a mean of 11 months and USA 20% for a mean of five months) and 11% received invasive ventilation for a mean of 6.7 years (UK two patients for a mean time of eight months, USA none).
Discussion: These results show that there are differences in the discussion and consideration of end of life issues within different units and countries. Decision making appears to vary and in particular the use of ventilatory support is used more often in Japan than in either the UK or USA.
As the care of patients develops, and the use of international guidelines continues, these areas will need to be considered further. The discussion regarding end of life will become increasingly important, particularly as there is now awareness of cognitive impairment, and possible reduction in decision making capability. If professionals are to be able to undertake the wishes of their patients, and families, these discussions are necessary earlier in the disease progression, while decision making is possible.
P19 PREFERENCE‐BASED QUALITY‐OF‐LIFE MEASURES IN AMYOTROPHIC LATERAL SCLEROSIS
Ogata K1, Ogawa M2, Kawai M1
1NHO Higashisaitama Hospital, Hasuda, Saitama, Japan, 2National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan
E‐mail address for correspondence: [email protected]
Background: Preference‐based measures are a type of health‐related quality‐of‐life (QOL) scale designed to measure the relative utility value, which can be used to calculate quality‐adjusted life‐years for cost effectiveness analysis. Significant differences were previously reported among three preference‐based scales in a sample of patients with Parkinson's disease (1). It is uncertain that preference‐based scales are appropriate for patients with neurodegenerative/neuromuscular diseases.
Objectives: To assess the validity of using preference‐based scales in patients with amyotrophic lateral sclerosis (ALS).
Methods: Twelve patients with ALS were rated using two preference‐based scales, EQ‐5D (2) and HUI (3). At the same time, we measured ALSAQ‐40 (4,5) and scales of clinical severity and activities of daily living (ADL), ALSFRS‐R (6), Norris scale, Appel scale (7), Barthel index and motor function items of Functional Independence Measure (FIM‐M).
Results: All the clinical and ADL measures were highly correlated with one another. Scores from the EQ‐5D and HUI correlated well with the ALSAQ‐40 and all the clinical and ADL measures. The value for the EQ‐5D was highly correlated with the HUI (p = 0.003 for Spearman's rank‐order correlation). The mean scores were different; EQ‐5D was 0.411±0.566, the HUI was 0.13±0.58.
Discussion and conclusions: In the patients with ALS, the EQ‐5D and the HUI correlated well with measures of clinical severity and ADL, and each other. However, they gave strikingly different values. This difference may result in the discrepancies in cost‐effectiveness analysis with ALS. Progression of the disease might affect the relative utility value measured with preference‐based QOL scales. These results suggest that care should be taken when applying preference‐based QOL measures to ALS.
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