THEME 10 MULTIDISCIPLINARY CARE AND QUALITY OF LIFE

P184 ALS OUTCOME MEASURES: INSENSITIVE TO EARLY CHANGE?

MENDOZA MC¹, MOORE DH¹, MILLER RG¹, FLORENCE J³, VERHEIJDE JL⁴, GORDON PH², AND THE MINOCYCLINE STUDY GROUP ²

¹California Pacific Medical Center, San Francisco, CA, United States, ²Columbia University, New York, NY, United States, ³Washington University, St. Louis, MO, United States, ⁴Mayo Clinic, Scottsdale, AZ, United States

E-mail address for correspondence: [email protected]

Keywords: FVC, MMT, ALSFRS-R

Background: Different quantitative measures have been used in clinical trials, but there is no consensus as to which tests most sensitively and reliably detect a modest drug effect in ALS. Most trials have included a pulmonary measure, and many have included the ALSFRS-R and a strength assessment. A better understanding of the strengths and limitations of outcome measures is important.

Objectives: To assess the relative sensitivity of FVC, MMT, and ALSFRS-R to early decline in ALS. To determine whether selecting patients who show early decline can increase study power.

Methods: We used data from 206 placebo patients enrolled in a clinical trial of minocycline. All had monthly measurements of FVC, MMT, and ALSFRS-R. We calculated the percentage of patients who had values that did not change from the baseline visit to months 1, 3, 6 and 12. We also investigated whether criteria, such as a maximum MMT score, could be used to define a subset of patients showing definite progression during the first 4 months following enrolment, and the effect of selecting only these patients on sample size estimates.

Results: 1) The ALSFRS-R had the highest percentage of patients with non-declining scores. At one month, 48% of patients had the same score or higher than at enrolment; the values were 33% at 3 months and 17% at 6 months.

2) For % FVC, 42% patients had <1% change at one month, 25% at 3 months, and 17% 6 months.

3) MMT showed the smallest percentage of non-declining patients. After one month, 41% failed to decline in strength, 27% at two months and 14% at 6 months had not declined.

4) Change in FVC correlated with the ALSFRS-R total score, but not with the respiratory subscale.

5) At 12 months the non-decliners (among those still being followed) were ALSFRS-R 4% (6/164), FVC 10% (12/118), MMT 12% (14/119)

Discussion and Conclusions: These data show the early insensitivity of 3 commonly used outcome measures. Although the 12 month sensitivity to change is higher than months 1 to 6 in all three measures, the lack of early change negatively impacts the best fit slope used to determine drug effect in clinical trials. Restricting trial inclusion to patient subsets showing early decline in the quantitative measures did not improve study power in our analysis. The need for additional strength and function assessments that are more sensitive to early change is apparent. Further study is needed to define how much insensitivity is due to the measurement tools, and how much is due to a subset of ALS patients who have only modest decline early in the disease.

P185 FEASIBILITY OF INTENSIVE CONTROLLED EXERCISE IN THE EARLY STAGES OF AMYOTROPHIC LATERAL SCLEROSIS (ALS-ICE), A PILOT STUDY

TEN BROEK-PASTOOR J¹, PIETERSE A¹, SCHELHAAS J¹, LINDEMAN E², MUNNEKE M¹

¹Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, ²University Medical Center Utrecht, Netherlands

E-mail address for correspondence: [email protected]

Keywords: Exercise, Intensive, Aerobic

Background:There is ongoing debate whether exercise will improve the independence of patients with ALS or whether exercise speeds up the deterioration. In addition, there is insufficient evidence to suggest which type and intensity of exercise, if any, would be best for patients with ALS.

Objective: To conduct a pilot study which assesses the feasibility of an intensity controlled exercise programme for patients with ALS.

Methods: Five patients conducted a moderate-intensive aerobic exercise programme of one hour of exercises, twice a week, during a period of twelve weeks. Outcome measures included aerobic capacity (VO₂max with Astrand-Rhyming test), functional health status (ALSFRS-R) and strength, Maximal Voluntary Isometric Contraction (MVIC). The MVIC was based on 7 bilateral muscles (4 arm and 3 leg). For each patient a mean z-score was calculated. Side-effects were measured by registering injury reports of the patients and by measuring fatigue on the Checklist Individual Strength, subscale fatigue severity (CIS-fatigue). Intensity of the exercise program was registered by monitoring heart rate and Rating of Perceived Exertion (RPE 0-11) in training-diaries. Measurements were planned before and after the 12 week training period.

Results: Four males and one female with ALS participated in the pilot study. Mean age was 47.6 years (range 32–56, SD 9.3). Mean ALSFRS at study onset was 50.6 (SD 2.2) All five patients had been diagnosed with probable or definite ALS according to the El Escorial criteria.

Intensity controlled exercise: Patients performed aerobic exercises between 55–70% of HRmax and RPE 3-5. Strength endurance exercises were set at 3 series of 12–15 repetitions (RPE 3-5).

Outcomes; All patients demonstrated a decrease in VO₂max (ranging from -8 to -67%). Functional Health Status did not change during the training period (change in ALSFRS-R ranged from +1 to – 3). Muscle strength deteriorated in all patients (range -0.05 to -0.88)

Side effects: Only one patient experienced more fatigue on the CIS scale after the period of the training. No other side effects were reported.

Patients’ experiences: All patients completed the training and tolerated the programme very well. They judged intensity as ‘intensive’ but not ‘too intensive’. All patients would recommended the training to other patients with ALS and all of them continued the training on completion of the study.

Conclusions: Intensity controlled exercise is feasible and appears to be safe in patients with ALS. A large multicenter trial will start this fall.

P186 INTERNATIONAL CLASSIFICATION OF FUNCTIONING, DISABILITY AND HEALTH (ICF) CORE SETS FOR AMYOTROPHIC LATERAL SCLEROSIS

BONGIOANNI P¹, AMATI M⁴, BROGI S³, CANTORE N¹, CIARDIELLO E⁴, D'AMICO I³, DAVITTI S², OCCHIPINTI P², PEPE G², RAFFAELLI M⁴, TRAMONTI F¹, TUCCIO MC⁴, UNGARETTI E², ROSSI B⁴

¹Neurorehabilitation Unit, Neuroscience Dpt, Azienda Ospedaliero-liero-Universitaria Pisana; ²NeuroCare-onlus, ³Respiratory Pathophysiology Unit; ⁴Neurorehabilitation Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy

E-mail address for correspondence: [email protected]

Keywords: ICF, functioning, disability

Background: Functioning is increasingly being taken into account for evaluating disease impact on the person living with amyotrophic lateral sclerosis (ALS) and drug treatment/rehabilitation effectiveness. With the International Classification of Functioning, Disability and Health (ICF), we can now rely on a globally-agreed-upon framework and system for classifying the typical spectrum of functioning problems of people with ALS, given the environmental context in which they live. ALS ICF core sets are lists of ICF categories selected to identify those aspects of functioning that are most likely to be affected by ALS.

Objective: To identify the preliminary comprehensive and brief core sets for ALS using the ICF approach.

Methods: Focus groups and a consensus process were used to identify ICF core sets for ALS, including: preliminary ICF studies; empirical patient data collection for 53 ALS participants and review of the evidence in ALS literature.

Results: One hundred and fifty-one second level ICF categories were selected. The comprehensive ALS ICF core set includes 38 categories from the component'body function’, 5 categories from'body structures’, 64 from'activities and participation’ and 44 from the component'environmental’ factors. Eleven categories in'personal factors’ in ALS were also suggested by the participants after intensive discussions. The brief set comprises 23 categories, 15% of categories in the comprehensive core set.

Discussion and conclusions: Consensus expert opinion can use ICF categories to identify the core set for ALS which reflects disease complexity and care burden for people living with ALS. Further research is needed to identify ICF categories of relevant personal factors to improve our understanding of the large social and cultural variance associated with them.

P187 PROBLEM-FOCUSED CHECKLIST FOR PATIENTS AT AN ALS MULTIDISCIPLINARY CLINIC

HARRINGTON-MORONE, G, MCELHINEY M, GORELIK R, BEDNARZ K, MITSUMOTO H, RABKIN JG

Columbia University, New York, United States

E-mail address for correspondence: [email protected]

Keywords: Patient care checklist

Background: Multidisciplinary clinics are the standard for the care of people with ALS. When patients come to an ALS multidisciplinary clinic for routine quarterly visits, they are seen by multiple clinicians. Patients usually present with different problems they want to discuss and there is often a need for some services over others. Too often, these are elicited late in the visit and the particular experts needed for these problems do not get enough time with the patient.

Objective: The aim was to develop a brief checklist to provide to patients in advance of their scheduled visit, asking them to check the problems and issues they wish to discuss. The goal was to provide more focused consultations in order to address each patient's concerns, and to improve the flow of the multidisciplinary team.

Method: The checklist has 2 parts. Part 1 lists 16 problems, both medical and psychological. Items include difficulty breathing, drooling, weight loss, fatigue, depression, and memory problems. Part 2 enumerates 9 services that the patient may seek, such as advice about advance directives, how to arrange for a home health aide, questions about equipment, PEG, and hospice.

Results: To date, 80 patients seen consecutively have completed the checklists. 57% were male, and mean time since diagnosis was 17 months. Mean ALSFRS score was 33 (7–46) and mean FVC was 67 (13–119). Median number of problems reported was 3 or 4. In Part 1, patients most often wanted help with limb weakness (71%), fatigue (50%), and difficulty speaking (43%). Depression, anxiety and memory problems were endorsed by only 18%, 14%, and 13%, respectively. In Part 2, the most commonly reported issues concerned assistive devices (34%), exercise (28%) arranging for a home health aide (23%) and advance directives (15%). Least endorsed were questions about relationship/sexuality issues (3%) and hospice (5%). Patients with more advanced disease (lower ALSFRS and FVC) checked more problems and issues, and the numbers of problems and issues endorsed were themselves correlated.

Conclusions: The checklist strategy was well received by patients, who appreciated being treated as partners in their care. The range of problems and issues identified indicates the need for the expertise of a broad range of specialists. In a multidisciplinary clinic where multiple specialists are available to see several patients on the same day, checklists appear helpful in allocating staff time to address specific needs, and high-need patients can be identified in advance. Data collection is ongoing, and if these patterns persist, clinic procedures can be revised accordingly.

Acknowledgement: Eleanor & Lou Gehrig MDA/ALS Research Center team.

P188 DESCRIPTIVE AUDIT OF THE KEY CHARACTERISTICS OF THE CURRENT MND POPULATION IN A LARGE UK SPECIALIST MND CLINIC

O'BRIEN M¹, WHITEHEAD B², MITCHELL D², CALLAGHER P², JACK B¹

¹Edge Hill University, Ormskirk, United Kingdom, ²Lancashire Teaching Hospitals NHS Foundation Trust, Preston, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: audit, patient characteristics, specialist clinic

Background: A specialist service has been established at this centre for many years, providing on-going follow up for people with MND. As part of a larger study exploring the personal experience of living with MND, an audit of the patient population was undertaken to identify prevailing characteristics of patients currently followed up by the service.

Objectives: The aims of this study were to determine the personal demographic characteristics of the patient population; identify site/type of disease onset and current severity (using the ALS Health Severity Scale (ALSHSS) [1] and ALSFRS-R, [2]; assess level of uptake of PEG, NIV and aids/equipment; assess level and type of care required; determine the time from disease onset to diagnosis and identify the time from diagnosis to nurse specialist follow-up.

Methods: A comprehensive case note review was undertaken of patients (n = 98) currently receiving follow up at this centre. Statistical analysis was aided by SPSS version 14.0 to produce descriptive statistics indicating frequency of occurrence of key characteristics.

Results: The mean (standard deviation, SD) age at onset was 59.7 (12.6) years. 54.1% of the patients were male. Most patients (38.8%) had ALS with limb onset, with 29.6% having ALS with bulbar onset. The majority of patients were classed in the level 2 category of the ALSHSS, (58.2%) compared to 17.3% in level 1, 18.4% in level 3 and 6.1% in level 4. Four patients (4.1%) were on NIV and 16(16.3%) had a PEG inserted. The majority of patients relied on 1–3 items of equipment (46.9%) compared to 33% who used more than 3 items. 16.3% of patients did not currently use any aids or equipment. 28.6% of the patients lived independently with no uptake of care. 23.5% lived independently but with family assistance and 19.4% relied on family carers full time. 18.4% relied mainly on family but also accessed social services care. 5.1% of patients lived in residential accommodation. The mean time from symptom onset to diagnosis was 18.7 months (SD = 15.2) with 41.7% of patients receiving their diagnosis within 12 months of symptom onset. The majority of patients (78.5%) were commenced on nurse specialist follow up within 12 weeks of diagnosis with the mean length of time from diagnosis to commencement being 6.3 weeks (SD = 11.2).

Conclusions: This audit demonstrates the characteristic features of the current MND population followed up by this centre and for the first time provides detailed information regarding the level of disability and uptake of care services and equipment amongst this population. The length of time from symptom onset to diagnosis for most of the patients at this centre corresponds with evidence of a UK average of 11–22 month delay [3].

P189 CHARACTERIZATION OF PATIENTS SUFFERING FROM MOTOR NEURON DISEASE IN DENMARK

GREDAL O, THE DANISH ALS-REGISTER STUDY GROUP

Rehabilitation Centre for Neuromuscular Diseases, Copenhagen, Denmark

E-mail address for correspondence: [email protected]

Keywords: Clinical profile, diagnose

Background: Motor neuron disease (MND) consists of a group of neurodegenerative disorders including progressive muscle atrophy, amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis. In Denmark these disorders are mainly diagnosed by neurologists in the ALS-teams located at 14 different hospitals. The Danish ALS-register was established in 2003 and until now 8 ALS-teams have included all their MND-patients. The register consists of two parts 1) the diagnostic process and 2) essential data in the course of the disease such as FVC, BMI, ALS-FRS as well as data about aids, nurturance needs and the patients preference for PEG-tube and NIV/IV. All data were collected in the out-patient clinics and from home visits performed by the Rehabilitation Centre for Neuromuscular Diseases.

Objectives: To characterize the clinical profile of the ALS-patients in Denmark and to find out how the diagnosis was reached and which investigations were performed.

Methods: This prospective study included 252 patients with MND diagnosed according to the El Escorial revised criteria from 2003 until 2008. The data collection included information on age, sex, site of first MND symptoms, time from first symptom to diagnosis, the diagnostic process and the certainty of the diagnosis.

Results: 252 patients with sufficient information were analyzed. Of them 146 were men (57.9%) with a mean age at first symptom 59.2±12 and 106 were female (42.1%) with a mean age 61.2±11.7. 19 cases (7.5%) had a positive family history of MND and in one of these cases a mutation in the SOD1 gene was found. The frequency for the site of first MND symptom were lower limbs (36.9%), bulbar region (35.7%), upper limbs (34.1%), respiratory symptoms (1.9%) and cognitive problems (1.6%). At the time of diagnosis the frequency of MND symptom were upper limbs (77.7%), lower limbs (72.2%), bulbar region (65.9%), respiratory symptoms (34.9%) and cognitive problems (6.0%). The diagnostic foundation at inclusion were history, clinical signs, EMG (90.1%), MRI of cerebrum and medulla (48.0%), lumbar puncture (39.3%) and measurement of cerebral blood flow (2.0%). The certainty of the diagnose according to the revised El Escorial criteria were definite ALS 41.7%, probable ALS 18.7%, probable ALS laboratory supported 5.6%, possible ALS 6.0%, suspected ALS 17.9% (LMN signs only 15.1% and UMN signs only 2.8%) and unknown 10.3%. The mean delay between onset of symptoms and diagnosis for the total study group was16.4±13.2 months

A previous, unpublished, study with ALS patients from Copenhagen has shown that more than half of the patients chose PEG-tube when swallowing problems occurred, but only a few patients in this area wished for or chose NIV or respirators. From January 1998 until January 2008 only 8 out of more than 135 ALS patients in the central area of Copenhagen chose NIV and another two ALS patients were ventilated with a respirator.

P190 NON NEUROLOGICAL MULTIDISCIPLINARY CARE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS IN SPAIN

MORA JS¹, SALAS T¹, FAJARDO M², IVANEZ L²

¹ALS Unit, Hospital Carlos III, Madrid, Spain, ²Epidemiology Unit, Consejeria de Sanidad, Huelva y Sevilla, Spain

E-mail address for correspondence: [email protected]

Keywords: Multidisciplinary care, Non neurological care, Spain

Background: The health care of ALS patients depends on adequate knowledge of the disease and the resources of the health care system. Identification of care deficits, caused by failures of the system or the professionals, is the first step needed to resolve them.

Objectives: To determine the health care status of Spanish patients with ALS, specifically their non neurological multidisciplinary care in order to identify needs and to propose improvements.

Methods: We created a 156 item questionnaire that included demography, clinical status and administered health care. Of those, 73 questions related to non neurological multidisciplinary care. The questionnaire was hand delivered or mailed to up to 400 patients with ALS from the Spanish ALS Foundation nationwide. Response to date is 260. A univariant analysis with SPSS 10.0 for Windows was performed.

Results: The patients median profile is male/female (ratio 1:1); 54 years old (range 29–79); married (81%); spouse as primary caregiver (70.8%); and from Madrid (41.5%). Specific care habits and deficits were identified. Among those, 47.6% of patients did not receive any form of physiotherapy; and in the rest of patients the physiotherapy was limited and insufficient. Between diagnosis and prescription of physiotherapy there was a median delay of 9.7 months; the mean duration of therapy was 1.8 months. 25.6% of patients received it at private centers and 28.2% at their homes, all of them at their own cost. Specific technical aids needed and not provided were identified. Patients financed 73.1% of simple technical aids and 100% of advanced aids. 73.5% of patients with dysarthria did not receive speech therapy. Of those receiving it, 36.4% had it at a private center and 36.2% at their own home, all of them at their own cost. 72.4% of patients with dysphagia did not receive nutritional information or care. 56.4% of patients with respiratory insufficiency do not have any ventilatory assistance. Specific technical aids needed and not provided were identified. 75.0% of patients with emotional distress did not receive psychotherapy. 60.2% were taking psychotropic drugs. 43.2% of patients did not receive the home assistance needed. Detailed analysis in relation to clinical status will be presented.

Conclusions: There are very significant deficits in physicotherapy, technical aids, speech therapy, nutritional care, ventilatory assistance, psychotherapy, and home care. The solution of these deficits requires the creation and formation of true multidisciplinary and coordinated care teams in each large hospital. Public aid to finance technical aids for mobility and communication is required.

P191 THE REVISED ALS FUNCTIONAL RATING SCALE: VALIDATION OF AN ADAPTED VERSION TO THE SPANISH ALS POPULATION

SALAS T¹, CANO A¹, RODRIGUEZ F², ESTEBAN J³, MORA JS¹

¹ALS Unit, Hospital Carlos III, Madrid, ²School of Psychology, Universidad Autonoma, Madrid, ³ALS Unit, Hospital 12 de Octubre, Madrid, Spain

E-mail address for correspondence: [email protected]

Keywords: ALSFRSr, Validation, Spain

Background: The revised ALS Functional Rating Scale (ALSFRSr) is a widely validated instrument to measure disease progression in ALS. The ALSFRSr contains 12 items grouped into four domains (gross and fine tasks, bulbar and respiratory functions) which grade disabilities in activities of daily life (ADL). It has been used as a primary variable in several recent clinical trials. Clear understanding of each item scoring is necessary to avoid technical errors. Cultural differences make it necessary to validate any translation of the original English version into another language.

Objectives: To validate an adapted Spanish version of the ALSFRSr.

Methods: The ALSFRSr was translated into Spanish. Two raters carried out the evaluation of 60 patients with probable or definite diagnosis of ALS according to El Escorial Criteria. The items of the Spanish version of the ALSFRSr were correlated with the functional variables of the ALSAQ-40 quality of life questionnaire (physical mobility, ADL and independence, eating and drinking, and communication) and the respiratory variables of the SRI (Severe Respiratory Insufficiency) questionnaire (respiratory symptoms, physical function, and sleep). Internal consistency was examined by the Cronbach statistical alpha. Test-retest correlation analysis was made using the Spearman's rho. A factor analysis, with extraction of main components with Varimax rotation and Kaiser normalization, for comparison with the original scale was performed. Correlation between the ALSFRSr dimensions and the corresponding ones of the ALSAQ-40, and between the ALSFRSr respiratory dimension and the SRI questionnaire was obtained for validation.

Results: Cronbach's alpha values were between 0.75 and 0.95 for all dimensions to prove internal consistency. Spearman's rho values were between 0.80 and 0.95 for the test-retest analysis. The factor analysis showed a contribution of nearly 55% of the principal component; the load of each item to its correspondent factor was between 0.50–0.90 except for the “salivation” item. The structure of the scale was found, through the Varimax rotation, to be divided into 3 factors: neuromuscular, respiratory and bulbar functions. The construct validity revealed strong correlations (r_s>0.60) with the ALSAQ-40 between Gross Motor task and Mobility, between Gross and Fine Motor tasks and ADL, between Bulbar Function and Communication and between Bulbar and Respiratory and Eating and Drinking. Similar index correlation was obtained between the ALSFRSr respiratory dimension and its corresponding ones of the SRI.

Conclusions: According to these results, the Spanish version of the ALSFRSr is a highly reliable and valid instrument for the functional assessment of Spanish patients with ALS.

P192 INTERDISCIPLINARY CARE IN AMYOTROPHIC LATERAL SCLEROSIS (ALS)

CLEIDE SANTOS SALVIONI C, STANICH P, CELIS LEME TORINO V, R. BARROS D, ELVA MAYRINK C, LEAL MAGALHÃES CHIAPPETTA AL, FIORATO JÚNIOR J, JUVINIANO QUARDROS AA, HOLSAPFEL S, A. GARCIA C, BERTO MC, SOUZA BULLE OLIVEIRA A

University Federal of São Paulo, São Paulo, Brazil

E-mail address for correspondence: [email protected]

Keywords: multidisciplinary care, nutrition, dysphagia

Background: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease, causing progressive weakness and loss of the muscular strength of superior and inferior members subsequently compromising, the respiratory system. Dysphagia, weight loss, dyspnea and depression, besides limitations of the activities of daily living (ADL), are difficulties presented with the progression of the disease. The integration of the informations is the benefit of the interdisciplinary care, providing better quality of life to patients and caregivers.

Objective: To study the effectiveness of the interdisciplinary care in patients with MND/ALS.

Method: This prospective non-randomized study was carried between June 2007 and February 2008 in 33 patients with ALS, who were regularly receiving interdisciplinary care and were following the therapeutic protocols. Specialties included were neurology, respiratory physiotherapy, speech and language therapey and nutrition. The period of evaluation was quarterly and patients were assessed for medical treatment, nutritional status, dysphagia, forced vital capacity (FVC) and use of BiPAP. For statistical analysis test of Wilcoxon was used.

Results: The average age was 55,0 years, ranging from 33 and 82 years; 20 (60.6%) was male and 13 (39.4%) female; 84,8% were using Riluzole. Tamoxifen was associated in 36.7% and 39.4% were taking E and C vitamins. Complex B was used in 6.0%. In spite of the nutritional orientations, 12.0% (4) presented with malnutrition. Twelve (36.3%) had PEG and 21 (63.3%) had oral feeding. The majority (54.4%) did not change their food consistency, 42.4% were ingesting a creamy diet and 6.0% were receiving nutrition exclusively by PEG. During the study, only 21.1% of the patients presented exchange of food consistency, while 33.3% experienced increasing dysphagia; 33.0% did not use BiPAP despite necessity (FVC reduction nocturnal and respiratory compromise and/or during the day). The values of FVC varied between 17,0% and 84,0%. Despite necessity, respiratory physiotherapy is not even assessed in the same proportion of those adhering to the medicine and to the changes in the diet. We didn't observe differences in the nutritional status, feeding route, dysphagia intensity, use of BiPAP and measure of FVC. We obtained statistically significant difference (p = 0,11) only for food consistency.

Conclusion: The interdisciplinary care brings adherence to the treatment of the MND/ALS. However, the application of the given orientations depends of the availability of the patient and caregivers.

P193 FIRST IMPRESSIONS COUNT-SATISFACTION AT THE TIME OF DIAGNOSIS PREDICTS PERCEIVED LEVEL OF SUPPORT RECEIVED AT PRESENT IN MND PATIENTS

GOULD L¹, CALABRESI M², GRUNDY N², YOUNG C²

¹University of Liverpool, ²The Walton Centre for Neurology and Neurosurgery, Liverpool, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: Diagnosis, satisfaction, survey

Background: The Motor Neurone Disease (MND) service is based in the Walton Centre for Neurology and Neurosurgery in Liverpool. The service covers the North West of England, Wales and the Isle of Man with a combined population of 3 million people.

Objectives: The aims of the present study are twofold 1) to evaluate which elements of diagnosis are most strongly predictive of a patient's level of support with the service provided at diagnosis, and 2) to measure whether the patients’ support level at diagnosis is predictive of their satisfaction with their present support.

Methods: The MND database at the Walton Centre was used to identify potential participants. Patients too ill to participate, who had opted not to be contacted by the service for research or had a known history of dementia were excluded from this postal questionnaire survey. 142 out of 170 patients on the database were contacted. Perceived support at the time of diagnosis was measured as excellent, good, satisfactory or poor. Access to information and health professionals at the time of diagnosis, and satisfaction with services at the time of participation in the survey were used as predictors of perceived support.

Results: The response rate was 54 out of 142 (38%).

With regard to patients’ view on the service at the time of diagnosis, 13% rated it as poor, 20% as satisfactory and 66% as good or excellent. Elements of diagnosis that were assessed included meeting an MND specialist nurse around the time of diagnosis and being given information on the MND Association.

Two elements of diagnosis were predictive of a patient's level of satisfaction with the service. Patients informed about the MND Association rated the support received at diagnosis as excellent or good 78% of the time, versus 11% of those who were not informed. Patients meeting the MND specialist nurse rated the support received at diagnosis as excellent or good 89% of the time; in contrast only 26% of those who did not meet the nurse rated support as good or excellent.

A short scale measuring current level of support was constructed with a maximum score of 9 (absolute satisfaction) and a minimum of -9. Patients who rated their support at diagnosis as ‘excellent’ were the most satisfied and those who rated their level of support as ‘poor’ the least.

Discussion: Explaining about the MND Association leads to greater levels of perceived support at diagnosis. The importance of early contact with an MND specialist nurse is also highlighted. We suggest that people who feel supported and engaged with current services report better disease-specific input at diagnosis.

P194 IDENTIFICATION OF COPING STRATEGIES USED BY CARERS OF FAMILY MEMBERS WITH A DIAGNOSIS OF MOTOR NEURONE DISEASE – A QUALITATIVE STUDY

GENT C

Queens Medical Centre, University Hospital, Nottingham, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: Motor Neurone Disease, Carers, Coping strategies

Background: Caring for a person living with a progressive illness, such as Motor Neurone Disease is acknowledged as both challenging and demanding [1]. The demand of caring for a person living with Motor Neurone Disease can become a source of stress for informal carers.

Despite literature identifying coping strategies used by carers of individuals with conditions such as stroke or cancer, it was difficult to identify coping strategies used when caring for a family member with Motor Neurone Disease. Therefore the Author felt that this study, as part of an MSc in Rehabilitation, would provide fruitful data by exploration of the thoughts and experiences of these individuals.

Objectives: The key objective of the study was to identify coping strategies used when caring for a family member with Motor Neurone Disease.

Methods: Ethical approval was obtained. A qualitative method was chosen for the study. Purposeful and convenience sampling was used. Six participants agreed to participate in the study, (three female and three male). Semi-structured, face to face interviews were carried out using an interview guide. Data analysis was carried out by using a thematic content analysis method [2].

Results: The following themes were identified:

Category one – Support for carers.

Category two – Emotional Coping Strategies

Category three – Protected Time for Carers

Category four – Carer's Role

Discussion and Conclusions: The findings were comparable to that of existing evidence, which used different patient groups within the study population such as individuals effected by cancer. The semi-structured interviews within this study allowed the generation of data providing an insight into the carer's personal experiences.

Implications for Future Practice: The following actions could be taken i) Development of information leaflet highlighting coping strategies. ii) Gauge how useful carers would find a MND specific carer support group, with view to setting such groups locally

A number of areas for future research were identified: i) Longitudinal study identifying coping strategies used from diagnosis. ii) Comparison study of support services involved in the management of MND in Lincolnshire and Cambridgeshire and iii) Compare the coping strategies used by family carers of individuals with a diagnosis of MS and MND

P195 DIFFERENCES IN BURDEN AMONG CAREGIVERS OF PEOPLE LIVING WITH AMYOTROPHIC LATERAL SCLEROSIS

TRAMONTI F¹, BONGIOANNI P¹, DAVITTI S³, TUCCIO MC², PEPE G³, ROSSI B²

¹Neurorehabilitation Unit, ²Neurorehabilitation Unit, Neuroscience Dpt, Azienda Ospedaliero-Universitaria Pisana, Pisa, ³NeuroCare-onlus, Pisa, Italy,

E-mail address for correspondence: [email protected]

Keywords: CBI, quality of life, support

Background: Caregivers of patients with Amyotrophic Lateral Sclerosis (ALS) have to cope with several practical difficulties together with the suffering of people who usually are very close and intimate to them. Therefore, we need to assess the amount of psychological distress caregivers commonly have to face, taking into account how such a distress can be devastating for their health and quality of life. Moreover, in terms of intervention, working for caregivers’ wellbeing can exert a positive effect on patients’ quality of life too.

Objective: We aimed to evaluate caregivers’ burden, in relation to variables such as sex differences among caregivers and ALS patients’ physical impairment due to disease progression.

Methods: We used the Caregiver Burden Inventory (CBI), a 24-item questionnaire based on 5 subscales measuring different aspects of caregivers’ burden. We administered CBI to 31 caregivers of ALS patients and we divided the sample in three age groups (Age 1: 26–45; Age 2: 46–59; Age 3: 60–70) and in three classes identified by the patients’ ALS Functional Rating Scale scores (Class 1: 36–48; Class 2: 25–35, Class 3: 0–24). We correlated different CBI dimensions with caregivers’ age, sex and disease severity.

Results: Female caregivers scored significantly higher in the subscales related to time for assistance, physical burden and sensation of not having the same opportunities of people of the same generation. As far as the “age” variable is concerned, we only found a significant difference in physical burden, that-as one could expect-was higher in the eldest caregiver (Age 3 Group), in comparison to that of the youngest (Age 1 Group). Another finding concerns the significantly higher time caregivers of the most physically impaired patients have to spend giving assistance: statistically significant differences on this CBI dimension were observed between Class 3 and Class 1 and also between Class 3 and Class 2.

Discussion and Conclusions: Our data can foster further investigation on caregivers’ burden. We found some interesting differences among caregivers which could be of great relevance in setting of suitable person-centred psychological intervention: caregivers should be regarded as people who need support and at the same time, subjects playing a key role in patients’ support.

P196 RELIGIOSITY DOES NOT INFLUENCE QUALITY OF LIFE AND DEPRESSION IN ALS CAREGIVERS

CHIÒ A, CALVO A, GHIGLIONE P, GIACONE S, PESSIA A, MOGLIA C, GUZZO A, VIGNOLA A

Department of Neuroscience, Torino, Italy

E-mail address for correspondence: [email protected]

Keywords: Caregivers, religiosity, depression

Background: It has been demonstrated that religiosity has a profound effect on ALS patients outcome [1] and quality of life (QoL) [2]. However, there are no studies on the influence of religiosity on caregivers of ALS patients.

Aim: To assess the effects and correlates of religiosity on ALS caregivers.

Methods: A total of 75 consecutive informal caregivers of ALS patients were interviewed. Religiosity was evaluated using the Idler Index of Religiosity (IIR), a four-item measure consisting of two items assessing public religiosity and two items assessing private religiousness. Total scores range from 4 (least religious) to 17 (most religious). QoL was evaluated with McGill Quality of Life (MQOL) questionnaire, and Satisfaction with Life Questionnaire (SWLQ). Depression was evaluated with Zung Depression Scale (ZDS) and anxiety with the Spielberger's State and Trait Anxiety Inventory (STAI). Correlations between religiosity and the other variables was performed with Pearson's correlation; multivariable analysis was performed with linear regression.

Results: The 75 caregivers (26 men and 49 women) had a mean age of 55.8 years (SD 12.0). They included 47 spouses, 16 children, 5 parents, 3 siblings, 2 nephews, 2 brother/sister-in-law. Caregivers’ mean IIR score was 10.7 (SD 3.0) (private 5.9 (SD 2.1), public 4.8 (1.3)). Their mean ZDS score was 39.3 (SD 8.1); 6 (8%) caregivers had a score above the depression cut-off:. Caregivers’ mean MQoL score was 7.4 (SD 1.3), their mean STAIs score was 48.1 (SD 11.7); state anxiety level was low in 19 (25.7%) caregivers, medium in 44 (59.4%) and high in 12 (16.2%). Caregivers’ mean SWLS score was 25.0 (SD 6.9). In multivariable analysis caregivers’ MQoL was related to SWLS score (p = 0.001) and ZDS score (p = 0.02) but not to total IID, private IID or public IID. Private IIR was related to caregivers’ age (p = 0.006) and education (p = 0.047), while public IIR was related only to caregivers’ education (p = 0.021).

Conclusions: Differently from patients, religiosity had no relevant influence on caregivers’ QoL, which was conversely related to Satisfaction with Life and depression. The reasons for these discrepancies should be investigated further.

P197 HOW AWARE ARE CAREGIVERS OF THE CONSEQUENCES AND MANAGEMENT OF BULBAR INVOLVEMENT IN ALS?

CHAULET S, BAILLOT J, CAMU W

CHU de Montpellier ALS center, Montpellier, France

E-mail address for correspondence: [email protected]

Keywords: caregivers, bulbar involvement, speech therapy

Background: The important role of caregivers in ALS is increasingly recognized. The bulbar involvement needs the speech therapist to work on several aspects of this deficit: dysphonia, dysarthria, dysphagia, tongue and cheeks paresis and emotional lability. His role is not restricted to the patient, as informations must be given to the caregiver. These caregivers have to be informed of several aspects concerning dysphagia or dysphonia and their consequences. They also must learn how to behave in case or emergency.

Objectives: We tried to determine how much and how correctly the caregivers in bulbar ALS cases are informed.

Material and Methods: During a 30 minute interviews, caregivers were asked to answer a questionnaire about the current speech therapy at home, dysphagia, food intake, communication and also about the information they had received simce the beginning of ALS. Each interview took place in our center during the quarterly multidisciplinary consultation.

Results: There were 45 caregivers, 69% were spouses of the patients and mean age was 59.2yrs. 91% of the corresponding patients had begun speech therapy and for 98% of them more than once a week. For 57% speech therapy was done at home. For only 45% of the patients, the caregiver was present during the speech therapy session.

Dysphagia: 40% of the caregivers did not know the appropriate posture for swallowing, only 29% of them would be able to perform a Heimlich Maneuver and 36% of them had never heard of it. Food intake: Only 44% of the caregivers were aware of the usefulness of mixed food, 14% were informed about water jelly while 76% recognized that the patient had feeding troubles. Communication: 37% had frequent difficulties in understanding the patient's talk and 18% never understood. Almost 16% of the caregivers never used written communication. Information: 69% of the caregivers considered their own information about communication tools as insufficient. 64% felt the same about gastrostomy. For 60% of the caregivers, better information and better knowledge would help them to be more efficient in providing patient care.

Discussion: This work underlines huge gaps in the knowledge of caregivers about ALS and the specific deficits of the patient they care for. For bulbar involvement, the speech therapist may help improve such knowledge. However, it seems important to draw the attention of the caregiver on the specific deficits induced by ALS during specific sessions at the expert center. This work may help physicians and expert teams pay particular attention to those points and to create training and information sessions devoted to caregivers as early as possible in the disease course.

P198 THE LONG TERM EFFECTS OF SALIVARY GLAND RADIATION THERAPY ON SWALLOWING AND MEALTIME RELATED QUALITY OF LIFE: A CASE STUDY

CLEARY S, JHA N, JOHNSTON W, KALRA S

University of Alberta, Edmonton, Alberta, Canada

E-mail address for correspondence: [email protected]

Keywords: Sialorrhea, swallowing, quality of life

Sialorrhea is a common bulbar symptom in patients with ALS that results in significant functional, social and psychological burden on patients, families, and caregivers. Neuromuscular induced weakness and wasting of the lips, tongue, pharynx and larynx results in open mouth postures, poor oral containment of thin salivary secretions and ineffective swallowing. Most research on sialorrhea has focused on the anterior spillage of saliva out of the mouth (drooling); limited data exist on the impact of posterior spillage of saliva into pharynx, which is particularly problematic in bulbar cases of ALS. As secretions spill into the pharynx, they place constant demands on airway protective mechanisms. Frequent coughing, gagging, and discomfort at mealtimes can have a profound impact on quality of life. If patients are unresponsive to pharmacological interventions or these are contraindicated, other approaches such as salivary gland radiation therapy (XRT) may be used. Positive results have been reported in the few treatment outcome studies that exist on XRT however most studies to date have focused on quantifying saliva production following treatment (e.g., drooling collection units, tissue counts) and limited data are available on patient perspectives on the impact of XRT on swallowing function and mealtime related quality of life.

Objectives: The purpose of this case study was to describe the long-term effects of salivary gland radiation therapy on swallowing and mealtime related quality of life for one patient with ALS.

Methods: A 29 year- old male with a bulbar onset of ALS received an increasing dosage of amitryptiline over 12 months and presented with moderate oropharyngeal dysphagia and severe sialorrhea including skin irritations around nose and upper face, clothing changes 2–3 times daily, with excessive salivary issues noted at mealtimes and while sleeping. He received two courses of radiation therapy over 5 weeks (i.e., five bilateral parotid gland treatments and five submandibular treatments). The Salivation Scale; Degree of Drooling Scale, The Saliva Control Assessment, the Post-Saliva-Surgery Assessment Form [1] the ALS-FRS and the SWAL-QUAL swallowing related quality of life instrument [2] were administered prior to XRT and then one month and thirteen months after treatment.

Results: Prior to XRT he stated that the effect of sialorrhea on his quality of life was severe and after XRT all saliva management issues were eliminated and the patient had a sufficient quantity of saliva to maintain more normal swallowing. He also reported improved psychosocial function at mealtimes. After XRT, he reported a decreased in overt symptoms of swallowing difficulty (episodes of coughing, choking, gagging, etc.). Additionally, his perceived level of psychological distress at mealtimes improved by 70%. His perception of these functions remained at this elevated level a year later across multiple measures.

Discussion and Conclusions: Saliva management, swallowing and airway protection are integrally related functions that affect quality of life.Results from this case study will form the basis for larger studies of the effects of XRT and other sialorrhea interventions on the swallowing and mealtime related quality of life of individuals with ALS.

1. Scott A & Johnson H, A Practical Approach to the Management of Saliva, 2004, Pro-Ed, Austin, Texas

2. McHorney C, Bricker D, Kramer A et al Dysphagia 2000;15:115–121

P199 A PROSPECTIVE, RANDOMIZED CONTROLLED STUDY COMPARING RADIOTHERAPY VERSUS BOTULINUM TOXIN A AS A TREATMENT FOR DROOLING IN ALS

WEIKAMP J, SCHINAGL D, VERSTAPPEN C, DE SWART B, SCHELHAAS J, ZWARTS M

Radboud University Medical Centre, Nijmegen, Netherlands

E-mail address for correspondence: [email protected]

Keywords: drooling, treatment, comparison

Background: Drooling is a common and invalidating symptom in patients with Amyotrophic Lateral Sclerosis (ALS). Radiotherapy and botulinum toxin injections are now well accepted as alternative treatment options to the more tradional and often disappointing drug management of this symptom. Still, a well-designed prospective and controlled trial to compare these two methods has never been performed.

Objective: To answer the question which treatment has preference when taking into account effectiveness and side effects.

Methods: In a prospective, randomized, controlled trial we will compare efficacy and tolerability of ultrasound-guided BTX-A injections in parotids and submandibular glands with radiotherapy performed by a single dosage of 7.0Gy bilateral to the parotid glands and the posterior parts of the submandibular glands. To evaluate the effect of treatment measurements will be performed at baseline and after 4, 12 and 36 weeks by the number of used paper handkerchiefs, a drool rating scale, a VAS-scale, ALSAQ-40 and ALS-FRS-revised. Side effects will also be strictly monitored by evaluation of bulbar function.

Since the start of the study eleven patients have been included. Five patients were treated with radiotherapy (RT), five patients with botulinum toxin (BT) injection and one patient with both.

Results: A positive effect to initial treatment was 80% after RT versus 50% after BT. Retreatment of RT raised positive response to 100% and retreatment of BT raised response to 66%. The mean effect size was 48 days after RT versus 45 days after BT (range between one week and 4 months). As a result of both treatments xerostomia and changed viscosity of saliva were mentioned as side effects. No decrease of bulbar functions was observed.

Conclusions: After treatment 10 patients were satisfied for at least a short period of time. No serious side effects were found. The effect of the treatments did not significantly differ in this small group of patients.

P200 CHALLENGE OF HEAD-NECK SUPPORT ORTHOSIS IN AMYOTROPIC LATERAL SCLEROSIS PATIENTS FOR IMPROVING ACTIVITIES OF DAILY LIVING

MASAGAKI A¹, YORIMOTO K², OHKUBO H², TAMADA Y², OHNO M¹

¹Kawamura-gishi. Co., Matsudo, Japan, ²International Medical Center of Japan KOHNODAI Hospital, Ichikawa, Japan

E-mail address for correspondence: [email protected]

Keywords: cervical orthosis, ADL, head-neck support

Background: ALS patients often have problems supporting their own head due to the loss of neck muscle power. With this problem, activities of daily living (ADL) such as transfer, gait, and feeding seem to be prevented. Also, some patients complain about dyspnea and neck pain. From 2002 to 2008, we have tried several types of cervical orthosis (CO). In this study, we would like to report three cases which had a good results in improving ADL after using CO. Also, retrospective research was conducted.

Objectives: Case 1: 64 yrs old, male with bulbar palsy (BP). Though he has a typical orthopedic mold-type CO, he has not used it due to the difficulty in attaching it and neck discomfort.

Case 2: 55 yrs old, female with upper extremity palsy (UEP). Though she was recommended a typical orthopedic soft-type CO, she has not used it because head support is not enough, due to the pressure of her neck and discomfort in the humidity and heat.

Case 3: 72 yrs old, male with bulbar palsy (BP). He is tracheotomy ventilated and bed ridden. He sits in a wheel chair every day for a physical therapy programme.

Methods and Results: Case 1: We measured SpO₂ and heart rate on sitting and after 10 minutes distance. After using CO, better influence was seen for respiration.

Case 2: We compared head position before and after using CO. With CO, she could stand by herself in the short term.

Case 3: We measured SpO₂, heart rate and discomfort before and after using the CO.

Conclusions: Using CO in these above mentioned cases and including the results from our retrospective research, ADL such as sitting, standing and gait of ALS patients are improved. More possibilities of orthoses for motor neuron diseases are should be studied.

P201 TO IMPROVE LIVING WITH ALS/MND: THE PROVISION OF ASSISTIVE DEVICES AND ENVIRONMENTAL ADAPTATIONS

CREEMERS HWJ, BEELEN AJ, NOLLET F, VAN DEN BERG LH, GRUPSTRA HF, GROSZE NIPPER LHM

ALS Center the Netherlands/Academic Medical Center, Amsterdam, Netherlands

E-mail address for correspondence: [email protected]

Keywords: Need, devices, provision

Background: ALS/MND patients need assistive devices and environmental adaptations to remain independent for as long as possible and to limit restrictions in autonomy and participation. In the Netherlands health insurance companies and municipalities provide aids and adaptations needed by ALS/MND patients.

Objectives: The aim of this explorative study was to describe the need for assistive devices and environmental adaptations among ALS/MND patients in The Netherlands and to investigate problems encountered by ALS/MND patients in the process of providing assistive devices and environmental adaptations.

Methods: A questionnaire designed for the purpose of the study was sent to 306 of the estimated 1000–1200 patients with ALS/MND of whom 214 (179 with ALS and 35 with PMA) completed the questionnaire (response rate 68%).

Results: The need for assistive devices and environmental adaptations: 82% of the respondents needed one or more devices. 66% ALS/MND patients needed home adaptations in the bathroom, 21% applied for a stairlift and 65% applied for an attendant-propelled wheelchair. 49% ALS/MND patients needed a riser chair, 42% an electric nursing bed and 41% a rollator.

The experienced problems: Major problems identified by ALS/MND patients are: i) The long duration of the process of application and provision (39%). ii) Authorities’ lack of knowledge on disease characteristics of ALS/MND and insufficient sympathy for the situation of the individual patient (22%). iii) The bureaucracy and paperwork (15%). iv) Lack of clarity of the legal proceedings (15%).

Five out of 10 ALS/MND patients who needed one or more aids or adaptations experienced problems in the process of providing assistive devices and environmental adaptations.

The two major problems indicated by ALS/MND patients which they considered as being most urgent to be solved are: 1) time delay in the delivery of devices and adaptations and 2) the authorities’ lack of knowledge on the disease.

Discussion and Conclusions: The results of this study indicate major problems perceived by ALS/MND patients in the process of providing assistive devices and environmental adaptations.

The bureaucracy, the authorities’ lack of knowledge on disease characteristics and the long time delays are the main incumberances to the ALS/MND patient that need to be addressed to reduce patient distress.

The most appropriate measures to be taken are: i) to support ALS/MND patients where needed and to meet their information needs during the process of provision. ii) To increase authorities’ knowledge on disease characteristics of ALS/MND.

P202 DISCUSSIONS BETWEEN ALS PATIENTS AND THEIR FAMILIES ABOUT INVASIVE MECHANICAL VENTILATOR USE

TAKAHASHI N¹, YAMAUCHI R², NONAKA M²

¹Department of Nursing, ²Department of Neurology, Sapporo Medical University, Sapporo/Hokkaido, Japan

E-mail address for correspondence: [email protected]

Keywords: decision making, quality of life, qualitative reseach

Background: About 7300 patients were being treated for ALS in Japan in 2007, with a relatively high rate of invasive mechanical ventilator use compared to other countries. The burden of ventilator use on family caregivers is problematic, so making a unilateral decision to use a ventilator is difficult for patients. Understanding what patients and their families think about this decision is thus important.

Objectives: To determine factors related to decision making about ventilator use, ALS patients not using ventilators were interviewed about their thoughts from diagnosis to the present. This study attempted to assess discussions between ALS patients and their families specifically about invasive mechanical ventilator use.

Methods: This qualitative, descriptive study was performed between May and September 2005. Subjects comprised 5 ALS patients satisfying four criteria and 5 family members acting as primary caregivers. Criteria for patients were: 1) knowledge of diagnosis; 2) understanding of the possible need for ventilator use; 3) no current ventilator use; and 4) capacity to tell others about their thoughts.

The 3 main questions examined what patients thought when diagnosed with ALS, changes in daily life as symptoms progressed and thoughts about ventilator use. Data were collated from semi-structured interviews.

Interviews were transcribed, then sentences related to study objectives were extracted to: analyze decision-making processes regarding ventilator use between patients and family caregivers; evaluate family discussions; and ascertain characteristics of each patient/caregiver pair.

Results: Subjects were interviewed once or twice. Mean interview durations for patients and caregivers were 150 and 105 min, respectively. Regarding patient thoughts on ventilator use, one patient was willing, two patients were unwilling and two patients were undecided. In addition, three caregivers wanted to respect patient decisions, one caregiver was undecided and one caregiver did not want a ventilator. Four pairs had not discussed ventilator use, for reasons such as: 1) both parties were undecided; 2) the caregiver was unenthusiastic about ventilator use; 3) the patient had decided against ventilator use; or 4) the caregiver did not want their opinion to affect the decision-making process of the patient.

Discussion and Conclusions: Only one of the 5 pairs had discussed ventilator use. The other 4 pairs had not held such discussions. The pair that had talked about the issue agreed on not using a ventilator. Individual differences were seen in the amount of information and data about ALS, with some subjects worried about making decisions regarding ventilator use, since they could not anticipate disease progression. As decisions regarding ventilator use are life-or-death decisions, some subjects stated how difficult this issue was to talk about and expressed a desire for professional intervention.

In order to make a decision about using invasive ventilation that satisfies both patients and their families, it is important for them, with the support of specialists, to fully discuss the decision on ventilator use.

P203 AT-HOME ARTIFICIAL VENTILATION CARE AND AUTONOMY OF ALS PATIENTS

TSUBAI F, IMAI T, KAWAUCHI Y, KODAIRA S, ETSUKO O, KIMURA I

National Hospital Organization Miyagi Hospital ALS Care Center, Watari-gun, Miyagi, Japan

E-mail address for correspondence: [email protected]

Keywords: Home Artificial Ventilation Care, Autonomy, QOL

Objective: To determine what conditions are needed for ALS patients who have undergone medical procedures to continue to live alone, we examined the difference in process of support between a patient who failed to continue at-home artificial ventilation care and a patient who received training at the ALS Care Center of Miyagi National Hospital and has been receiving at-home care.

Patients: Patient 1 is a 53-year-old female who developed ALS in 1995 and was placed under artificial ventilation in 2003, with a total duration of disease of 12 years.

Patient 2 is a 60-year-old female who developed ALS in 2003 and was placed on artificial ventilation in 2006, with a total duration of disease of 5 years.

Methods of support: Patient 1: Based on the patient's wish to receive at-home care, a care manager recruited caretakers, who were trained in aspiration techniques at the hospital, and at-home care was then started. To support the patient's life under at-home care, 8 to 10 caretakers sent from a care agency were always by the patient, working for 24 hours and 365 days per year, on a four-shift per day basis. Shortage of care expenses was covered by the care agency.

Patient 2: Support was provided to encourage the patient to make a care plan for at-home care and make a request to her primary care physician for herself. In addition, she was required to go in and out of the hospital repeatedly so that she was able to gradually develop an environment for at-home care herself while solving problems associated with at-home care.

Results: Patient 2 posted a call for volunteer caretakers in newspapers herself. This activity expanded the network of support and thereby dramatically reduced the burden of care on her family member (daughter), who had been totally responsible for her care. This resulted in consolidation of the basis of the at-home care system, and an environment suitable for at-home artificial ventilation care with 24-hour caretakers has been established.

In contrast, in the case of patient 1, shortage of caretakers occurred because of withdrawal of many of the caretakers due to psychological burden of the difficulty of their work, resulting in shrinkage of the network of support and failure of at-home care.

Discussion: There were marked differences between patients 1 and 2 in methods of support before the start of at-home care. Specifically, patient 1 was not involved in the process of developing an environment for at-home care, and preparation of the care environment was mainly performed by a care manager. In the case of patient 2, support was provided to facilitate the development of autonomy, leading to a willingness on behalf of the patient to improve her own life herself and encouraging her to work positively to develop a care environment herself. This resulted in a difference in quality of the at-home care system between the two patients.

Conclusion: Providing patients with support before the start of at-home care facilitates the development of autonomy, a willingness to improve his/her own life, leading to expansion of the network of support, and consolidation of the basis of the at-home care system.

P204 OUTCOMES OF PATIENTS WITH ALS WHO HAD SEIQOL-DW ASSESSMENTS BEFORE MAKING ADVANCE DIRECTIVES

NARITA Y¹, NAKAI M², KUZUHARA S³

¹Mie University Hospital, Medical Care Networking Centre, Tsu, ²The Liaison Council of the Network for Patients with Intractable Neurological Diseases, Tsu, Mie, Japan, ³National Center of Neurology and Psychiatry Musashi Hospital, Kodaira, Tokyo, Japan

E-mail address for correspondence: [email protected]

Keywords: SEIQoL-DW, ALS, advance directive

Background: We reported previously that some Japanese patients with amyotrophic lateral sclerosis (ALS) wished to control their treatment and management after respiratory or swallowing function deteriorated, especially in regards to the decision to use mechanical ventilation via tracheostomy. Although there is no legislation mandating advance directives in Japan, discussion about patients’ involvement in decision making has increased since a presentation of the Guideline for the Process of Decision Making on End-of-Life Care by the Ministry of Health, Labour and Welfare of Japan, in May 2007. The newly presented Guideline is stimulating discussion among doctors who treat patients with serious conditions, but it may also accelerate excessive use of an advance directive documents without assessing patients’ thoughts and efforts to improve their own situations. The Schedule for the Evaluation of Individual QOL-Direct Weighting (SEIQoL-DW) is an acceptable tool to evaluate patients’ thoughts. While it is still very rare for Japanese patients to have an advance directive, we had some patients who completed a SEIQoL-DW assessment before writing a directive.

Objectives: Our goal was to survey patient outcomes and the index of SEIQoL-DW regarding individuals’ needs to improve their care at the time of making advance directives.

Methods: From case records of ALS patients who had follow-ups and were visited more than once at their homes, six domiciliary patients who had advance directives from August 2004 to March 2006 were found. Four of them had a SEIQoL-DW assessment before making an advance directive, and had follow-ups until they died or revoked their directives through March 2008.

Results: There were three male and one female patients, between 38–68 years of age (mean age = 54.3±15.0 years) with a disease duration of 3–6 years (mean duration 4.3±1.3 years). Two patients had died, and two were alive at home. The deceased patients’ SEIQoL-DW indices were 60.9 and 68. The two living patients had scores of 11 and 30. One patient was followed up with repeated SEIQoL-DW assessments and showed a remarkable increase of the index from 30 initially to 77.5 on the most recent assessment. This increase was attributed to support provided for improving communication, through which the patient learned computer skills and was able to access the Internet and communicate with friends, family, and healthcare professionals. She revoked her advance directive, and accepted 24 hour mechanical ventilation at home.

Discussion and Conclusions: As the number of subjects was too small to evaluate statistically, the effectiveness of the assessment requires further investigation; but SEIQoL-DW assessments could reflect the patients’ need for their situation to be improved before making advance directives.

P205 STOPPING LIFE PROLONGING RESPIRATORY SUPPORT. REFLECTION ON OUR PRACTICE

FAULL C

LOROS the Leicestershire and Rutland Hospice, Leicester, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: withdrawing treatment, respiratory support, reflection

Background: Some patients who use respiratory support decide that they no longer wish for this to continue to prolong there lives. This concerns their perception of their dignity, quality of life and fear of the future. Removing such respiratory support can be physically and emotionally very distressing for the patient as they develop hypoxia, hypercapnia and acute breathlessness. This raises considerable practical, emotional and ethical issues for the health care team. Reflection is an intentional and skilled activity requiring an ability to analyse practice actions and make judgements regarding their effectiveness’ [1]. At the core of reflective practice is a purposeful contemplation of an experience or event which leads to new ways of thinking or behaving in practice.

Objectives: 1) To develop the safety, efficacy and quality of the care for future patients. 2) To minimise risk for the health care team. 3) To further understanding of this are of clinical practice.

Methods: The specialist palliative care team reflected on two patients who had requested support in stopping their nasal intermittent positive pressure ventilation. The reflection took place both individually and as a group using the Driscoll ‘What’ model [2]. The role of the various health care professionals in working through this decision with the patient, family and carers and the care that was provided as the respiratory support was removed were specifically considered.

Results: We have found that there is no literature to inform our practice. Our reflection identified a breadth of concerns amongst professionals. These include; anxiety about competence in alleviating symptoms of distress within ethical boundaries; a profound challenge to individual practitioners’ values which causes stress; a vital need to support each other in the distress related to proximity of administration of sedative medication to relieve distress and the occurrence of death.

Conclusions: The needs of the team who provide the care at the time of withdrawal support must be considered and planned for in advance. Introducing them to the patients care at an earlier stage is of importance.

1. Andrews M, Gidman J, Humphreys A (1998) Reflection: does it enhance professional nursing practice? British Journal of Nursing 7(7):413–417

2. Driscoll, J (1994) Reflective practice for practise – a framework of structured reflection for clinical areas. Senior Nurse 14(1):47–50

P206 ANALYSIS OF THE IMAGE AND CORPOREAL SCHEME AND THE QUALITY OF LIFE OF PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

BERNARDO DO NASCIMENTO DINIZ L¹, CAMARGO POLZATTO H¹, SOARES RODRIGUES PEREIRA S¹, MEIRE FAVERO F¹, VELOSO FONTES S¹, AUGUSTO JUVINIANO QUADROS A², SOUZA BULLE OLIVEIRA A¹

¹Universidade Federal de São Paulo, ²Associação Brasileira de Esclerose Lateral Amiotrófica, São Paulo, São Paulo, Brazil

E-mail address for correspondence: [email protected]

Key Words: Amyotrophic Lateral Sclerosis, Image and Corporeal Scheme, Quality of Life.

Background: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease creates changes in the self-perception of the patient. Body Image (BI) is a mental representation formed by the body itself, perception, movement and the emotional/affective and libidinal reactions are also part of its development. Analysis of the BI identifies alterations that influence the Quality of Life (QoL), it can be analysed by Drawing the Human Figure (DHF) which reflects the knowledge and the experience of the body itself.

Objectives: 1) To evaluate the image and corporeal scheme of patients diagnosed with ALS to verify possible alterations or distortions, 2) to evaluate the quality of life and the possible correlations between the many dimensions of the 40 item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) and the DHF.

Methods: Seventeen patients with ALS participated in this research answering the questionnaire ALSAQ-40 and completing the DHF, which was evaluated quantitatively and qualitatively. The Spearman correlation coefficient was used to verify the relationship between the alterations in self-perception and the dimensions of ALSAQ-40.

Results: The corporeal image and all the dimensions of the ALSAQ-40 was altered in all seventeen patients with ALS, that being the case, many Mobility problems became evident in relation to Emotional function. There is a correlation between the DHF and the Eating and Drinking dimensions (ALSAQ-40) besides the many direct correlations between the dimensions themselves.

Discussion: The results correlated with the literature, showing that the Emotional functioning dimension (ALSAQ-40) did not present itself as the most altered. Although, during the evaluation of the DHF, it was possible to observe projections of feelings like preoccupation, fear, anxiety, sadness, escaping from reality and restlessness in the spheres of sexuality and family contradicting what was presented in the questionnaire. While the ALSAQ-40 is a specific and self-applicable instrument to evaluate the QOL of the patients with ALS, the execution of the DHF allows the patients to project themselves subconsciously.

Conclusion: The individual results obtained through the use of both instruments show the need for more attention to be given to the self-perception of patients, as QoL is directly related to self-image and self-image has a strong emotional component. The use of DHF presents the most vulnerable points of the patient, giving the therapist conditions to assist the patient with ALS in a more systematic and humanized way.

P207 HEALTH STATE AND EMOTIONAL IMPACT OF THE DISEASE IN SPANISH PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS)

SALAS T¹, MORA JS¹, FAJARDO ML², IVAÑEZ L², ESTEBAN J³, RODRÍGUEZ F⁴

¹ALS Unit, Hospital Carlos III, Madrid, ²Consejeria de Salud de Andalucia, Huelva and Seville, ³Hospital 12 de Octubre, Madrid, ⁴School of Psychology, Universidad Autonoma, Madrid, Spain

E-mail address for correspondence: [email protected]

Keywords: ALSAQ-40, Quality of Life, Emotional Impact

Background: ALS has a high emotional impact on the patients, an impact that probably affects their quality of life, in different ways during the disease. The ALS Assessment Questionnaire 40 (ALSAQ-40) is used to evaluate the patient's health state in five scales: Physical Mobility, Activities of Daily Life (ADL)/Independence, Eating and Drinking, Communication, and Emotional Functioning. The Spanish version has been validated by us previously.

Objectives: To analyze the emotional impact of the disease in the established different areas of the ALSA-40 along the course of the disease. To determine which disabilities and when produce higher emotional distress.

Methods: A descriptive and longitudinal study was conducted over 4 years on perceived health status in 231 ALS patients. The ALSAQ-40 was used at three different stages: after diagnosis, at a time when their disability had progressed, and at the terminal stage. All patients were diagnosed with definite or probable ALS by the El Escorial Diagnostic Criteria.

Results: In the Spanish patients there was not a direct correlation between grade of severity of the disease and emotional impact. The deepest feeling of depression occurred more frequently around the time of diagnosis. At the first evaluation, 20% of patients felt “frequently” depressed and 17% were “never” depressed versus 12% and 30% respectively at the third evaluation. Only 31% of the variations were in the Emotional Functioning scale are correlated significantly with the variable physical state. The progressive reduction in ADL/Independence is the single factor most related to depression. There were not significant feelings of loneliness, boredom, shame or hopelessness. Hopelessness about the future decreased with the course of the disease while increases concerned the future consequences of the disease.

Conclusions: Psychological support around the time of diagnosis is advisable to overcome depression. Psychotherapy along the course of the disease must take into consideration the emotional impact of the progressive loss of autonomy and the fear of future consequences of the disease.

P208 END OF LIFE CARE OF PEOPLE WITH MND/ALS – A SURVEY OF THE MEDICATION USED IN SPECIALIST PALLIATIVE CARE

OLIVER D¹, CAMPBELL C², SLOAN R³, SYKES N⁴, TALLON C⁵, O'BRIEN T⁶

¹Wisdom Hospice, Rochester, United Kingdom, ²St Catherine's Hospice, Scarborough, United Kingdom, ³Weldmar Hospicecare Trust, Dorchester, United Kingdom, ⁴St Christopher's Hospice, London, United Kingdom, ⁵Cynthia Spencer Hospice, Northampton, United Kingdom, ⁶Marymount Hospice, Cork, Ireland

E-mail address for correspondence: [email protected]

Keywords: palliative care, opioids, end of life

Background: Palliative care is increasingly provided for people with MND/ALS. This often starts soon after diagnosis as there is no curative treatment for the disease. The final stages of the progression of the disease are often difficult with increasing symptoms and issues for patient, family and professional carers [1]. Professional carers often fear the use of medication, in particular opioids [2].

Objectives: This study aimed to show the medication used within specialist palliative care in the end of life care for people with MND/ALS.

Methods: A questionnaire was developed to record medication given to patients with MND/ALS in the last 72 hours of life, together with basic information about the patient and disease progression. Six hospices in the UK and Ireland agreed to complete the details of the last 10 patients who had died under their care in the hospice.

Results: 60 patients were analysed and the results show that medication is usually necessary in the control of symptoms at the end of life. These are commonly morphine as analgesic, midazolam as sedative and muscle relaxant and glycopyrronium bromide to reduce lung secretions. The doses of the medication are not greater than the doses used in the palliative care of other patient groups, such as those with cancer, or advancing respiratory or cardiac disease.

Discussion: Many patients with MND/ALS, their families and carers and often the professional carers fear the final stages of the progression of MND/ALS – fearing in particular pain, dyspnoea and choking ([1],[3]). These results show that medication can be used effectively in the management of the symptoms experienced at the end of life and the doses are not different to those used in the care of other patient groups. Health professionals can be reassured that after appropriate symptom assessment the careful use of opioids, and other medication, is helpful and appropriate at the end of life.

P209 SYMPTOMS AND UNBEARABLE SUFFERING IN PATIENTS REQUESTING EUTHANASIA OR PHYSICIAN-ASSISTED SUICIDE: ARE THERE DIFFERENCES BETWEEN ALS, HEART FAILURE AND CANCER?

MAESSEN M¹, ONWUTEAKA-PHILIPSEN B², VELDINK J¹, VAN DEN BERG L¹

¹UMC Utrecht, Utrecht, Netherlands, ²EMGO, VU University, Amsterdam, Netherlands

E-mail address for correspondence: [email protected]

Keywords: euthanasia, cancer, heart failure

Background The percentage (20%) of patients with amyotrophic lateral sclerose (ALS) that die after euthanasia or physician-assisted suicide (EAS) is higher compared to most other terminal diseases in the Netherlands including cancer (5%) and heart failure patients (0.5%). One of the mandatory conditions in the Dutch law for performing EAS is that the patient requesting EAS suffers unbearably. Palliative care should prevent this unbearable suffering if possible. It is unclear why the rate of euthanasia in ALS is higher compared to other terminal diseases, therefore more insight in the disease specific unbearable suffering, which causes patients to prefer death over life is desirable.

Objectives: In this research we studied whether ALS patients have different reasons for requesting EAS compared to cancer and heart failure patients.

Methods: Dutch physicians of patients with a request for EAS can consult an independent EAS specialized physician (SCEN physician). This SCEN physician visits the patient and reports the patients’ reasons for unbearable suffering that are the motives for requesting EAS. For this study, the treating physicians who consulted the SCEN physicians, mostly general practitioners, were asked to fill out a questionnaire on sex, age, diagnosis and physical and depressive symptoms of the patients requesting EAS.

We collected all (n = 3891) SCEN reports in the Netherlands between April 2000 and the end of 2002. Next, we selected all (n = 51) SCEN reports of ALS patients. To compare their reasons of unbearable suffering we also selected all (n = 61) heart failure patients and made a random selection of the cancer patients (n = 73). We collected and compared questionnaires from the treating physician on the patients’ physical and mental symptoms during their request for EAS.

Results: The top three most reported reasons for unbearable suffering in ALS patients were fear of suffocation (45%), feeling dependent (29%) and loss of dignity (20%). The top three of cancer patients was pain (46%), fatigue (28%) and loss of dignity (24%). The top three of heart failure patients was dyspnoea (52%), dependency on others (37%) and knowing that the suffering will only get worse (30%). According to the treating physician ALS patients had less frequent fatigue (OR 0.169, 95% CI 0.043–0.658), pain (OR 0.265, 95% CI 0.119–0.591), nausea (OR 0.106, 95% CI 0.042–0.270) and vomiting (OR 0.093, 95% CI 0.028–0.307) than cancer patients at the time of the EAS request and poor appetite less frequently (OR 0.183, 95% CI 0.051–0.653), dyspnoea (OR 0.242, 95% CI 0.081–0.723) or nausea (OR 0.338, 95% CI 0.115–0.994) compared to heart failure patients.

Cancer and heart failure patients reported significantly more frequent somatic complaints as a reason for EAS than ALS patients (P < 0.001).

Conclusions: ALS patients most often mention psychosocial reasons for requesting EAS. Compared to cancer and heart failure patients, somatic complaints are significantly less mentioned.

P210 INTERACTING WITH OTHER PEOPLE LIVING WITH ALS/MND: PATIENT AND CARER ATTITUDES TO SUPPORT GROUPS

LOCOCK L, BROWN J

¹University of Oxford, United Kingdom, ²University of Southampton, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: Support groups, personal experiences, qualitative research

Background: As ALS/MND is rare, general understanding of its impact on people's lives remains limited. Support groups, both face-to-face and online, offer contact with others who understand at first-hand what living with MND is like. These interactions can provide practical advice and emotional support. However, it is unknown why some people choose not to get involved.

Objectives: To explore the range of individual needs and attitudes to support groups amongst people living with ALS/MND and carers; to understand what motivates people to get involved or not; to develop recommendations to help professionals and voluntary groups in organising support groups.

Methods: This paper draws on narrative interview data collected for two research projects in the UK. The collective sample includes 48 patients living with ALS/MND and 22 carers, interviewed between 2005 and 2007. Data about attitudes to and needs for support groups will be subjected to a secondary analysis, using a coding framework based on both anticipated and emergent themes, and refined using constant comparison and deviant case analysis. This will be conducted by the two researchers responsible for the original research, making this an innovative approach to data sharing and re-analysis. Ethical approval is already in place.

Results: Preliminary observation of the data suggests several issues to be explored. Positive advantages of face-to-face and online support groups include practical advice (e.g. on welfare benefits and equipment). Many people draw comfort from talking to others who understand the range of emotions they experience.

Seeing others managing with more severe disability can provide an encouraging example, but can also create anxiety. Equally, some people with slow progression worry that their presence at support groups could upset others with more rapid progression. Deaths of fellow group members are saddening and depressing. Some people avoid support groups because they do not want their identity and social life to be defined by the condition.

Practical difficulties (e.g. timing and transport) affect some people's attendance of face-to-face groups, whilst lack of IT skills or computer adaptations may affect involvement in online groups.

Some people living with ALS/MND feel face-to-face groups are inappropriately dominated by carers and volunteers, whereas some carers feel unable to talk about issues that concern them when people living with the condition are present. Online support groups can offer a more indirect way of interacting with others, but some users are still upset by photographic images posted with messages.

Discussion: There has been little research into attitudes to support groups for ALS/MND. Greater understanding of the range of reactions that people have to support groups, their hopes for what they may gain, and their anxieties about what they may encounter can help those involved in organising groups to improve their appropriateness and accessibility.

P211 TRANSFORMATION OF RELATIONSHIPS FOLLOWING A DIAGNOSIS WITH ALS/MND

O'BRIEN M¹, CLARK D²

¹Edge Hill University, Ormskirk, United Kingdom, ²Lancaster University, Lancaster, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: relationships, narratives, changes

Background: Health professionals are increasingly attempting to understand illness from the patient's perspective. Making use of illness narratives is one way of becoming more informed about the personal experience of living with illness.

Objectives: To explore the personal experience of living with ALS/MND as documented in personal illness narratives, written by people diagnosed with the illness.

Methods: A detailed search strategy loosely based on systematic review methodology was adopted to identify illness narratives in print and internet form written by people diagnosed with ALS/MND. The 161 narratives identified were subject to content and thematic analysis. Data management was aided by Nvivo 7 software.

Results: A number of key themes emerged. This paper focuses on the transformation of relationships at a number of levels following a diagnosis of ALS/MND. Authors comment that they endure changes in their relationships within the family and an altered dynamic within the domestic setting. This is particularly apparent with considerations of gender, where individuals are no longer able to perform ‘usual’ gender specific activities. There is evidence of inversion of relationships as changes occur within generational roles as individuals develop dependent relationships on others. Numerous narratives, regardless of genre, document authors’ changing relationships with their own bodies. There is a strong impression of accelerated aging and the use of powerful imagery in an attempt to capture the impact of the disease at a personal level. For some, defined by their occupation, there is loss of identity when employment ends. Changes within the immediate environment result in the loss of individuality and private space.

Many authors convey their sorrow at the loss of their ability to initiate physical contact, particularly with their spouse/partner. Relationships are also affected by a loss of intimacy between husband and wife. The disabilities caused by ALS/MND engender a sense of vulnerability in some people at having to rely on others for their needs.

Discussion and Conclusion: What we have here is authors writing about the various changes forced on them as a result of their illness. What emerges is a sense of attempts to overcome adversity and re-evaluate life in light of current circumstances. These illness narratives provide an insight into the effects of the disease on the individual diagnosed with ALS/MND. They also provide a glimpse into the world of other people from the perspective of the ill person. As such, the findings have implications for the training of health care professionals involved in the care of people with ALS/MND.

P212 ‘ON THE INTERNET YOU DON'T NEED FEET’: USING TECHNOLOGY TO OVERCOME CHANGES IN RELATIONSHIPS WITH SOCIETY FOLLOWING A DIAGNOSIS OF ALS/MND.

O'BRIEN M¹, CLARK D²

¹Edge Hill University, Ormskirk, United Kingdom, ²Lancaster University, Lancaster, United Kingdom

E-mail address for correspondence: [email protected]

Keywords: narratives, internet, society

Background: Recording personal experiences of ill health within an illness narrative enables individuals to document how their condition has impacted on their lives and helps to illuminate the steps they have taken to overcome adversity.

Objectives: To gain an understanding of the role of the internet in the personal experience of living with ALS/MND.

Methods: 161 print and internet narratives identified as a result of a detailed systematic search strategy were subject to content and thematic analysis. Data management was aided by Nvivo 7 software.

Results: A number of key themes emerged. This paper focuses on the use of modern technology by people diagnosed with ALS/MND in their attempts to cope with the disease. Being isolated pervades many narratives, as authors retreat from their everyday activities. The emergence of the internet and the availability of chat rooms and discussion groups specifically for people with ALS/MND have opened up numerous opportunities for interaction with the outside world. This has helped to ease the sense of isolation experienced by many authors as their condition deteriorates. New virtual friendships develop as traditional friendships fall away. Those authors experiencing dysarthria comment on how it removes them from spontaneous interaction with others. Communication becomes a matter of need rather than a social exchange. An inability to convert one's thoughts into the spoken word results in a sense of increasing isolation from the world. Accessing the internet allows authors to overcome their disabilities, even if only momentarily.

Discussion and Conclusion: Illness narratives about life with ALS/MND allow individuals to document how they have adjusted to the effects of the disease. These findings indicate that, despite their disabilities, those diagnosed with the illness can continue to involve themselves within society on a number of levels through accessing the internet.

P213 COMPARING OUTCOMES OF PATIENTS WITH ALS IN THE COMMUNITY VS LARGE ALS CENTERS, INSIGHTS FROM THE WEB-BASED ALS PATIENT C.A.R.E DATABASE

CHAMPION S¹, SPITALNY GM¹, KUSHNER G¹, FORSHEW D¹, MOORE DH¹, HUANG WEI T², ANDERSON F², MILLER RG¹

¹California Pacific Medical Center, San Francisco, CA, United States, ²University of Massachusetts Medical Center, Boston, MA, United States

E-mail address for correspondence: [email protected]

Keywords: Outcomes, registry, diagnosis

Background: Data on outcomes for patients cared for in large ALS centers comes from the ALS C.A.R.E database.

Objective: To compare outcomes of patients treated in the community compared to large specialized ALS clinics.

Method: Treatment and outcomes were compared from the ALS C.A.R.E database with data entered in the web-based, patient-driven database, ALS Connection, launched in January 2006. This analysis compares 82 patients enrolled in ALS Connection cared for in community practices (SOLO) with 4971 patients enrolled in the ALS C.A.R.E database (C.A.R.E), cared for in large multidisciplinary ALS clinics.

Results: SOLO patients were generally more advanced than C.A.R.E patients (limited in most activities 77% SOLO, vs 20% C.A.R.E).

More SOLO patients (56%) than C.A.R.E patients (21%) were unsatisfied with the way they were told about their diagnosis. More SOLO patients felt that their diagnosis was told to them in an insensitive manner (16% SOLO vs 3% C.A.R.E), that they were given too little information about the disease (20% vs 5%), and that they were given no sense of hope (22% vs 5%). 69% of C.A.R.E patients were given information about ALS agencies such as MDA and ALSA, compared to only 37% of SOLO patients.

Regarding therapeutics, fewer SOLO patients utilized therapists: speech therapist (29% SOLO vs 63% C.A.R.E); nutritionist (29% SOLO vs 66% C.A.R.E), social worker (22% vs 65%), home nursing (22% vs 65%)or psychologist (16% vs 66%).

Although a similar percentage of SOLO & C.A.R.E (60% vs 54%) patients have seen a physical therapist, 22% of SOLO patients who have tried physical therapy found it unhelpful, whereas only 2% of C.A.R.E patients felt the same way. The same is true for occupational therapy. 60% of C.A.R.E patients have seen an occupational therapist, and only 2% of those found it unhelpful. 45% of SOLO patients have tried OT, and 19% of those who tried thought it was unhelpful.

More Web patients got most information regarding ALS from the internet (53% SOLO vs 9% C.A.R.E). More patients were satisfied with their care in the ALS C.A.R.E database (70% SOLO vs 91%C.A.R.E).

Conclusion: Patients with ALS who enrolled in a web-based registry were less satisfied with care, had a less satisfactory experience in learning about the diagnosis, less access to symptomatic therapy, and less satisfactory experiences with therapy, than those in ALS C.A.R.E. Further research is needed to identify the reasons for these differences and how stable these differences are.

P214 STIMULATING INNOVATION TO IMPROVE THE QUALITY OF LIFE IN ALS VIA A DESIGN CHALLENGE: EXPERIENCES AND DIRECTIONS

EISEN A, MCEWEN J, HORVITZ E

¹University of British Columbia, Vancouver, BC, Canada, ²Microsoft Research, Redmond, Washington, United States

E-mail address for correspondence: [email protected]

Keywords: assistive technology, innovation, students

Background: Technical advances to assist patients with ALS have great potential for enhancing their quality of life. Unfortunately, technological innovations have been slow in development and have not kept pace with other advances in the care and understanding of ALS. We believe that innovation can be accelerated significantly by increasing the familiarity of biomedical engineers, human factors experts and computer scientists with the challenges faced by people with ALS and their care team.

Objectives: To encourage the development of innovative technologies to improve quality of life for patients with ALS through collaboration with young engineers, computer scientists and technologists in academic settings and highlight the compelling opportunities for creative exploration and prototyping

Clinical Description: Through the ALS Society of British Columbia, Canada, a prize was offered to students at undergraduate and postgraduate levels of engineering, including the fields of biomedical engineering, electrical and computer engineering, mechanical engineering and engineering science, to design innovative technologies that would assist and improve the quality of life of ALS patients. Prioritized areas of need were first identified by the ALS Society through surveys and interviews involving ALS patients and caregivers, as well as members of professional ALS care teams. These were grouped into four general areas: mobility, communications, breathing, and feeding. Students were encouraged to initially meet directly with patients, with appropriate mentoring and supervision, to learn more about the identified needs and to develop potential design solutions through adaptation of existing devices or development of new technologies. The resulting design projects were completed by six teams at three universities and a college over the course of an academic year. The results of four projects were suitable for live demonstration involving ALS patients, caregivers and a multidisciplinary evaluation panel: 1) A dynamic, versatile neck brace that whilst supporting the head and neck allows for ready movement of the neck in all directions, and stabilizes the head and neck for vehicular travel; 2) An eye gaze tracking system to enhance communication in patients with severe dysarthria or unintelligible speech; and 3) Integration of breathing and voice technology for improved communications in BiPAP dependent patients during the later stages of ALS.

Recommendations: A modest prize for innovative technology has stimulated potentially novel methods to improve quality of life for ALS patients, develop collaborations between young engineers and technology developers, physicians and the ALS Society of BC. It has also introduced ALS to those seeking rewarding career opportunities that involve the development and adaptation of innovative technologies. The initially developed technologies themselves have raised important questions in and of themselves which will result in further improvements.

P215 AN EEG IMPEDANCE CORRELATION STUDY FOR BRAIN COMPUTER INTERFACE

KING J¹, FELDMAN S¹, HEIMAN-PATTERSON T¹, DEBOO A¹, VAUGHAN T², SELLERS E², WOLPAW J²

¹Drexel University College of Medicine, Philadelphia, PA, 19107, United States, ²Wadsworth Center, New York State Department of Health, Albany, New York, 12201, United States

E-mail address for correspondence: [email protected]

Keywords: brain-computer interface (BCI), electroencephalography (EEG), communication

Background: Amyotrophic lateral sclerosis (ALS) is characterized by progressive weakness resulting in a state of profound disability including the loss of functional speech. The rise of new technologies allows people living with ALS and other individuals with severe motor disabilities to communicate as well as access the computer using alternate methods. One alternative access and communication method is an Electroencephalographic (EEG)-based brain-computer interface (BCI). The EEG-based BCI allows individuals with severe disabilities to communicate using a computer even when other conventional devices have failed, as the system is independent of neuromuscular activity. Impedance is a critical factor in the optimum performance of electrical systems and current literature correlates lower impedance with superior EEG recordings. However, no systematic study examines the relationship between BCI performance and impedance. The need to obtain low impedance may require significant additional time and abrading the skin may result in patient discomfort. Therefore, striking a reasonable balance between impedance and BCI performance is critical to the successful implementation of the BCI.

Objectives: The objective of this study was to determine the correlation between BCI performance and EEG impedance to determine the upper limit of impedance which allows optimal performance with BCI in a practical and clinically relevant paradigm.

Methods: The subjects have an electrode cap placed on their head to record their EEG. A small amount of gel is applied to each electrode. Impedance measurements are taken. Each subject is requested to attempt to spell a sequence of words using the P300-based BCI speller while the EEG is being recorded. This spelling sequence is then repeated 5–10 times after reducing the impedance. The impedance values, number of sequences, time, and performance percentages are recorded. Overall, the subjects attend 2 sessions during which the EEG and impedance measurements are recorded.

Results: In the three subjects (two ALS and one control) studied to date, data indicates a correlation between impedance and BCI performance. High impedance values result in poor spelling performance. In our subjects, adequate performance was obtained with up to an average value of 20 kohm impedance. Further testing will expand these results and fully determine the upper limit of acceptable impedance that still allows functional use of BCI technology in order to make the technology acceptable and practical for patients and caregivers.

Discussion and Conclusions: Current P300-based BCI protocols suggest that each electrode must have impedance values below 10 kohm for optimum performance. However, in subjects studied to date, we have found that BCI protocols may be amended to use impedance values as high as 20 kohm, resulting in a time savings and easier application for the caregiver. This study will help to translate BCI to practical use for ALS patients with severe motor and speech limitations.