THEME 1 MULTIDISCIPLINARY CARE AND QUALITY OF LIFE

P1 MULTIDISCIPLINARY ALLIED HEALTH PRACTICE GUIDELINES FOR PHYSICAL, SPEECH AND OCCUPATIONAL THERAPY IN ALS

Offeringa A

Broek Ten J

Oudenaarden J

Schaaf van der M

Academisch Medisch Centrum, Amsterdam, The Netherlands

Email address for correspondence: [email protected]

Keywords: multidisciplinary, guidelines, quality of life

Background: Evidence-based clinical practice guidelines improve quality, effectiveness and appropriateness of patient care. For ALS, such guidelines are not available for the allied health care professions.

Objectives: The aim of this project was to systematically develop guidelines for the multidisciplinary rehabilitation management of patients with ALS. The recommendations will support physical, speech and occupational therapists in clinical decision making with respect to the diagnosis, treatment and evaluation of relevant impairments and restrictions in functioning, activities and participation of patients with ALS.

Methods: A taskforce was formed and they started the project. The International Classification of Functioning, Disability and Health (1) formed the base. The guidelines were developed according to the model from the Dutch Institute for Healthcare Improvement (CBO) ‘Evidence based guideline development’ (2). This model contains the following steps: systematic literature search, quality assessment and summary of the evidence; formulation of concept recommendations; feedback from experts; rephrase of recommendations; final approval and authorisation by relevant stakeholders.

Results: The project resulted in practice guidelines with recommendations regarding diagnostics, intervention and evaluation of functioning within the relevant domains of the International Classification of Functioning, Disability and Health (1). The recommendations were developed for physical, speech and occupational therapists concerning all rehabilitation stages integrating evidence from research and clinical expertise of expert health care professionals, patients and their carer's preferences, national associations of physical therapy, speech therapy and occupational therapy and of the Netherlands ALS Center. In addition to the practice guidelines and the recommendations, a summary of the clinical reasoning process in algorithms is provided. The guidelines are e-published in Dutch and available at www.als-centrum.nl/als-richtlijn/ with free downloads.

Discussion and conclusion: The development of the multidisciplinary guidelines has allowed recommendations on diagnostic and therapeutic interventions for physical, speech and occupational therapists working with patients with ALS. Implementation of the guidelines will improve standardization and transparency of the diagnostic and therapeutic process. As the guidelines were developed simultaneously for these three allied health care professions, interdisciplinary aspects of treatment are covered as well which will improve care and quality of life for patients with ALS. Implementation of these guidelines is the next crucial step to take.

Acknowledgements

Project funding by Netherlands ALS Center and ALS Foundation Netherlands.

References

• World Health Organization. International Classification of Functioning, Disability and Health: 2001; ICF. Geneva, Switzerland.
   Google Scholar
• Everdingen JV, Burgers JS, Assendelft Van WJJ et al. 2004; Een leidraad voor de praktijk. Houten: Bohn Stafleu Van Loghum.
   Google Scholar

P2 DEVELOPING A REMOTE MULTIDISCIPLINARY CLINIC: INITIAL OBSERVATIONS AND LESSONS LEARNED

Kasarskis E¹

Vanderpool K¹

Goulson D²

^aUniversity of Kentucky, Lexington, KY, USA

^bOur Lady of Bellfonte Hospital, Ashland, KY, USA

Email address for correspondence: [email protected]

Keywords: remote clinics

Background: Bringing expert care to ALS patients residing in rural areas has been a frequent topic of discussion. A sustainable plan has not been enacted and tested for its cost and effectiveness. The situation is especially challenging in the Appalachian region of the US where poverty and geographic distance conspire to restrict access to tertiary care. Various approaches have been tried including personal travel by the ALS neurologist to a remote site or connecting via telemedicine links to directly evaluate the patient. Both approaches are expensive in terms of professional time and money, and therefore, lack sustainability. More importantly, the expertise of a team of regional therapists (PT, OT, RT, etc.) cannot be cultivated easily with these models.

Objectives: To establish a remote multidisciplinary clinic in eastern Kentucky (termed a Virtual ALS Center or VALSC), develop the expertise of regional therapists, communicate via telemedicine links, and measure the clinical effectiveness of this approach.

Methods: Our ALS Center at the University of Kentucky (UK) established a partnership with Our Lady of Bellefonte Hospital in Ashland, KY (approximately 125 miles, or 201 km distant) which agreed to host the multidisciplinary clinic staffed by their therapists. Regional ALS patients will be evaluated and treated in the VALSC in accordance with the AAN Practice Parameters.

We will identify a group of pre-intervention ALS patients who were residents of the target Appalachian region during 2005–12 and evaluate medical records to determine adherence to the AAN Practice Parameter. Their care will be compared to matched ALS patients attending our UK ALS center. The ongoing effectiveness of the VALSC will be determined by its adherence to the AAN Practice Parameter, the time and distance saved by patients attending the VALSC, and cash flow. Comparisons of the VALSC with other ALSA Certified Centers will be conducted.

Results: Development of infrastructure and training of the therapists for the VALSC has been accomplished. Approximately 40–60 historical ALS patients from the target region have been identified. The VALSC will begin operation in July 2013 and initial results will be presented.

Discussion and conclusion: The success of an enduring VALSC will depend on finding an institutional partner with shared values with the ALS Centers in the care of complex patients with nonsurgical illness. The partner needs to provide dedicated space and sufficient time commitment to develop the expertise of the local therapy team and neurologist. Telemedicine links to the tertiary ALS Center is critical for consultation and training. Issues of licensure, credentialing, privileging, and medical insurance are critical. Finally, the VALSC needs to be at least revenue-neutral for the host partner.

Acknowledgment

Study supported by a grant from the ALS Association.

P3 MULTIDISCIPLINARY TEAMS: EXCELLENT CARE FOR PATIENTS, BUT HOW DO WE CARE FOR OURSELVES?

Axline R

The Methodist Neurological Institute, Houston, TX, USA

Email address for correspondence: [email protected]

Keywords: bereavement, coping, grief response

Background: Caring for patients with life-threatening diseases can elicit grief symptoms in health care professionals. Limited descriptions exist of how individually and collectively, MND/ALS teams can safely express grief and model effective coping strategies.

Objectives: To explore mechanisms for managing patient loss and describe beneficial components of supportive models of intervention.

Methods: Individual interviews were conducted with healthcare team members to: identify challenges of work-related bereavement; current management of grief and loss; and ways of supporting interpersonal functioning and resiliency. Data collection involved a nine open-ended question instrument to elicit responses regarding emotions, experiences, perceptions, and grief resolution strategies.

Results: Over 28 months at The Methodist Neurological Institute, 176 new ALS patients were diagnosed and 124 deaths were reported. MDA/ALS Team Hope is comprised of 25 health carers in addition to staff caring for patients when hospitalized. Twenty-two individual interviews were completed. Responses were categorized into group and individual strategies. Group strategies included recognizing patient death by sending email to team; sending team signed card; attendance at funerals; discussing and reminiscing among team members; and staying in touch with family through ALS fundraisers. Individual ways of managing grief included sending private cards or emails; private tears; prayer; exercise; and journaling. Members indicate level of grief response often corresponded to: depth of their relationship with patient; similarity of patient to themselves in age or gender; and unexpected versus expected death. Grief response increased with frustration of late diagnosis, noncompliance with recommendations, and/or inappropriate treatment recommendations before patient arrived at the Institute. Members reported unique aspects of ALS-diagnosed population (positive outlook, giving spirit, and inspirational) as increasing grief response while simultaneously motivating professions to “do more” and “find a cure.”

Discussion: This descriptive exploration indicates the health care team is informally and formally providing a safe environment for grief responses following patient death. Having a team member conduct the interviews seemed to elicit deeper, more personal, and possibly cathartic responses. Majority indicated satisfaction with strategies as providing closure and validation of role and grief. Four individuals suggested adding “lessons learned” component to strategies. Grieving was recognized as a bonding experience; enhancing their ability to re-invest energy in patients. Dysfunction or burnout does not appear prevalent.

Conclusion: Further studies are needed and should include description of numerous MND/ALS health care teams to provide an outline of essential grief and coping components. With a grief and coping algorithm, teams can enhance their system for providing expression of normal grief responses. As health care professionals, developing some level of comfort with the issues of life and death and learning to live with our grief increases our ability to administer life-enhancing care, even to patients with a life-threatening disease. Learning self-care is an essential component in this process.

P4 PATIENTS’ AND PROFESSIONALS’ PERSPECTIVES ON CASE MANAGEMENT IN ALS CARE

Bakker M¹

Creemers H²

Schipper K¹

Beelen A²

Nollet F²

Abma T¹

^cVU University Medical Center, Amsterdam, The Netherlands

^dAcademic Medical Center, Amsterdam, The Netherlands

Email address for correspondence: [email protected]

Keywords: case management, care, patients’/caregivers’/ professionals’ perspective

Background: The concept of case management has been suggested as an innovative strategy to optimize complex multidisciplinary care in patients with ALS and their caregivers. In the absence of high quality evidence on the effectiveness of case management in ALS care, a cluster-randomized controlled trial (RCT) on case management in Dutch patients with ALS and their caregivers was conducted and showed no effect of case management on quality of life of ALS patients or on caregiver strain. More insight in the experiences of patients with ALS, their caregivers and health care professionals with case management may explain these negative results.

Objective: To explore the experiences of patients with ALS, their informal caregivers and health care professionals with case management in ALS care.

Methods: A qualitative study, nested in a large cluster-RCT on the effectiveness of case management. In-depth, narrative interviews with 10 ALS patients and their primary informal caregivers were held after 12 months case management. Also, 10 interviews were held with ALS health care professionals. Beside the interviews, a heterogeneous focus group was held with patients, informal caregivers and health care professionals. The data were audio taped, fully transcribed and analysed thematically by two independent researchers.

Results: Patients, informal caregivers and health care professionals noted that in certain circumstances case management may be of added value. Important factors were the adequacy of usual care. Also, a faster rate of progression of the disease was noted as an important factor, both by patients and professionals. Therefore, patients and health care professionals concluded that the need for case management additional to usual multidisciplinary care differs between patients.

The most important elements of the case management that were appreciated by patients and by health care professionals were the fact that the case manager did not work on a tight schedule and did not visit the patients and their caregivers at home.

Besides these elements, patients and caregivers pointed out that they valued the mental and practical support from the case manager, that is, to reflect on the situation with the case manager or to get help with applications for devices. However, the health care professionals pointed out that the need for additional practical support depends on the adequacy of usual care provided by the multidisciplinary team.

Conclusion: The study shows that a qualitative exploration of experiences with case management from different perspectives has made it possible to arrive at a greater understanding of the care needs of patients with ALS and their caregivers. Patients and health care professionals agree that in ALS care custom-made support is the key to care that best meets the needs of patients with ALS and their caregivers.

P5 COMPREHENSIVE CARE AND HOME TELEHEALTH FOR VETERANS WITH ALS

Kelsen L¹

McCoy S¹

Hoffman P¹

Patwa H^2,3

^eVeterans Health Administration Office of Rural Health- Veterans Rural Health Resource Center-Eastern Region, Gainesville, FL, USA

^fYale University, New Haven, CT, USA

^gVA Connecticut Healthcare System, West Haven, CT, USA

Email address for correspondence: [email protected]

Keywords: telehealth, caregiver burden, multidisciplinary care

Background: The Veterans Administration (VA) has been on the forefront of telemedicine for delivering care to veterans in rural communities. Veterans with ALS (VALS) often have limited access to specialty clinics because of impaired mobility, transportation of medical devices, and long travel times. Comprehensive multidisciplinary care for ALS has been effective for improving quality of life (QOL) and in some cases extending survival. VRHRC-ER in partnership with the North Florida/South Georgia Veterans Health System (NF/SG VHS) established a collaborative approach to comprehensive ALS care that includes home Clinical Video Telehealth (CVT) and provides close observation of VALS for decline in function and increased disability.

Objective: To demonstrate that home telehealth can be successfully implemented into the multidisciplinary management of VALS. In addition, we hope to determine if this data can be replicated in a larger study with additional patient and caregiver specific outcomes.

Methods: Home CVT was used to augment multidisciplinary care for VALS in NF/SGVHS. The comprehensive ALS care team included a neurologist, mid-level medical provider, physical therapist, occupational therapist, speech therapist, and social worker. After initial, and in some cases, follow-up face-to-face visits, VALS were given the option of using a home CVT unit immediately or when decline limited their ability to travel for follow-up visits scheduled at 3-month intervals. Outcome measures included weight loss, functional decline, detection of respiratory decline, reduction in hospitalizations, and reduction in caregiver burden.

Results: In the first 6 months, 17 VALS were enrolled at the NF/SGVHS ALS clinic. Eight were classified as rural or highly rural living greater than 2.5 hours from the ALS clinic. All 17 had an initial pulmonary function screening and PT assessment. To date, 109 Home CVT visits were conducted, saving 28,498 miles and nearly 525 hours of travel time by VALS and caregivers. Reimbursable mileage costs were reduced by $12,111. Data on functional decline, hospitalization, and caregiver burden will be presented.

Discussion and conclusion: Home telehealth in the care of VALS demonstrated improved patient satisfaction, reduced driving time, and reduced total cost and can be a valuable tool for patients who have limited access to specialty care. This demonstration project will be expanded to comprehensive ALS care clinics in New England (Providence, RI; West Haven, CT; and White River Junction, VT). The additive effect of home CVT on improving QOL, increasing survival, and decreasing caregiver stress will be determined. If successful, this model of ALS care may be available to all VALS in the USA.

P6 THE COST OF MANAGING ALS IN A TERTIARY REFERRAL CLINIC: A RETROSPECTIVE CHART REVIEW

Connolly S¹

Tobin K¹

Heslin C¹

Galvin M²

Hardiman O^1,3

^hTrinity College Dublin, Dublin, Ireland

ⁱDublin City University, Dublin, Ireland

^jBeaumont Hospital, Dublin, Ireland

Email address for correspondence: [email protected]

Keywords: retrospective, chart review, cost

Background: People with ALS are often intense users of medical services; however, relatively little is known about the cost of providing such services. Quantifying the cost of ALS services is important as it gives an indication of the potential savings arising from the development of treatments which slow down the progression of the condition.

Objectives: The aim of the study was to estimate the cost of managing ALS in a tertiary referral clinic in Ireland.

Methods: The analysis was based on a retrospective analysis of the medical charts of 119 representative patients who attended a tertiary referral clinic and who died between 2010 and 2013. Information on number of clinic visits, number of visits to allied health professionals, hospital admissions, procedures and aids, and home visits were recorded. Each resource was assigned a unit cost to provide the overall cost of providing care within the clinic. Costs were analysed separately by site of onset.

Results: 54% were male and mean age of onset was 64 years. 34% had bulbar onset disease. The mean length of time between the onset of symptoms and the first clinic visit was 13 months; while the mean survival time from first clinic visit was 21 months. The mean number of patient visits was 6 (minimum: 1 visit and maximum: 36 visits). 9% did not return to the clinic after the first visit, while 17% visited the clinic 10 or more times. The majority of patients were seen by allied health professionals during their clinic visit. For example, at their first clinic visit, 27% were referred to speech and language therapist, while 45% were referred to an occupational therapist. In addition, 51 patients received at least one home visit from the ALS clinical nurse specialist; while one patient received 10 such visits. Patients were in receipt of a wide range of interventions and aids including non-invasive ventilation and communicative and walking aids. A unit cost will be assigned to each of the resources which are used to provide an indication of the average cost of managing a patient within the clinic.

Discussion: Accurate estimates of the cost of care will permit modelling of the economic implications of symptomatic and disease modifying interventions.

Acknowledgements

The project was supported by a grant from Biogen Idec, and is funded by the Health Research Board (HRB) Dublin as part of the HRB Interdisciplinary Capacity Enhancement Awards.

P7 THE ROLE ANALYSIS OF THE COORDINATORS FOR PATIENTS WITH INTRACTABLE DISEASES IN JAPAN FROM THE POINT OF VIEW OF CONTINUITY OF CARE

Iwaki M¹

Nakai M²

Tateishi T³

Murai H⁴

Hayashi S⁴

Kira J⁴

^kThe Fukuoka Medical Committee for Intractable Diseases, Fukuoka Prefecture, Japan

^lThe Liaison Council of the Networking for the Patients with Intractable Diseases in Mie Prefecture, Mie Prefecture, Japan

^mDepartment of Neurology, Iizuka Hospital, Fukuoka Prefecture, Japan

ⁿDepartment of Neurology, Graduate School of Medical Sciences, Kyusyu University, Fukuoka Prefecture, Japan

Email address for correspondence: [email protected]

Keywords: coordinator, intractable neurological disease, continuity of care

Backgrounds: In the 1970s, the Ministry of Welfare, Japan, adopted a law to support those who had intractable diseases such as ALS. In 1998, prefectural medical health care networks were initiated to improve the care for patients with seriously intractable neurological diseases, especially those with ALS on artificial ventilation. Fifty-five medical care coordinators are currently introduced in 33 prefectural networks to support those patients. These coordinators need to have not only medical knowledge, but also information about the local welfare and healthcare system, and skills to cooperate closely with multidisciplinary teams. As they are employed on variable terms and conditions, most coordinators have few opportunities to have training about the expertise. Our previous study showed that most coordinators strongly felt the importance of training.

Objective: We attempted to define the roles of and the competencies for the coordinator and to make models for care coordination through case examination.

Methods: We collected the experiences for successful management from coordinators by e-mail. Successful management means that coordinators could adjust the care service taking patients’ QOL, and patients’ and their families’ needs into consideration. We classified these experiences into the following subjects: (1) management of the transfer to chronic care hospitals; (2) management of admission for respite care; (3) coordination of home care services; and (4) coordination of medical care system when patients move their residence to another prefecture. We used the Freemann's model of ‘continuity of care’ as the analytic frame for our study. The model was developed through people with long-term neurological conditions in the UK. The definitions comprise a number of elements such as (1) longitudinal continuity, (2) relational, personal and therapeutic continuity; (3) long-term neurological conditions; (4) cross-boundary continuity; (5) flexible continuity; (6) information continuity; (7) social context; and (8) personal agency. This concept is supposed to clarify the coordinator's roles and provided a way of examining the effects of integrated services.

Results: Twenty-one cases were collected from 18 coordinators. The response rate was 32%. According to qualitative case analyses, in almost all cases coordinators implemented the elements such as ‘longitudinal continuity’; ‘relational, personal, and therapeutic continuity’; ‘cross-boundary continuity’; and ‘information continuity’. In 50% of cases coordinators achieved ‘personal agency’ and ‘long-term neurological conditions’, in 30% cases ‘flexible continuity’, and in 25% cases ‘social context’.

Conclusions: Coordinators made a significant contribution to integrate services by medical and health professionals for patients with intractable diseases. The coordinate models are required to accomplish continuity of care for patients. Through case examination we can specifically share and improve approaching strategy to facilitate the continuity of care for patients. Using the concept of continuity of care, coordinators could recognize the expected role, evaluate their activity, and improve their faculty.

P8 THE TRACE TO THE FIGHT FOR THE IMPROVEMENT IN PALLIATIVE CARE OF THE PATIENTS WITH ALS IN JAPAN

Ogino M¹

Minami S⁴

Kanazawa N¹

Takahashi-Narita K³

Ogino Y^2,1

^oKitasato University Department of Neurology, Sagamihara/ Kanagawa, Japan

^pNational Hacone Hospital, Odawara/Kanagawa, Japan

^qKitasato University Department of Occupational Therapy, Sagamihara/Kanagawa, Japan

^rKitasato University Department of Molecular Diagnostics, Sagamihara/Kanagawa, Japan

Email address for correspondence: [email protected]

Keywords: palliative care, morphine, survey of ALS physicians

Background: Despite the fact that morphine prescription is a standard therapy for the end-of-life care of ALS in Western countries, it is not popular in Asian countries and other areas. Unlike cancer patients, the long-acting morphine and other opioids, and hospice care for ALS used to be outside of the insurance coverage in Japan. All we could do was to try to persuade the authority to make an exception or medical providers like us paid for the patients. In 2009, we conducted the survey on physicians treating ALS in Japan, and reported that only 21% of the respondents would prescribe morphine to their ALS patients under these circumstances.

Objective: The purpose of this paper is to share our experiences with other countries having difficulty in prescribing morphine for ALS patients.

Methods: For the past 3 years, we tried several projects to improve this situation to protect patients’ right. First we published a handbook on the palliative care for ALS, second we gave lectures on the palliative care for ALS many times throughout Japan, third we worked toward the Ministry of Health, Labor and Welfare to change the policy to make morphine for ALS covered by insurance.

Finally, in September 2011, hydrochloric acid morphine and morphine sulfate for ALS were covered by the national insurance. Therefore, we conducted the second survey on the physicians treating ALS to investigate any change occurred since 3 years ago. We sent out questionnaires to all of board-certified neurologists, same as the first survey.

Results: One thousand and fifty-three responded (response rate 22%) and their background was the same as the first survey. The percentage of answered respondents who prescribed morphine to their ALS patients was 32% (21% in first survey). The respondents who have prescribed morphine to more than five patients increased from 23% to 35.6%. 28.5% (38%) of respondents answered that the reason for not prescribing morphine is the absence of the national insurance coverage. 26.1% (38%) of respondents would prescribe if national insurance covers, and 63.6% (47%) of the respondents said they are determined to prescribe morphine whether or not the national insurance pays. Because our survey was conducted only 6 months after the policy change, many respondents may not have known the fact.

Conclusion: These results suggest that the recognition of morphine usage for ALS has changed, and it is very encouraging for us. We need an education system on the palliative care for ALS, because most of the respondents did not have enough experience of using these drugs for ALS. Therefore we have been organizing workshops on the palliative care for the ALS, twice so far. We hope all ALS patients in the world live peacefully until their end of life.

P9 PALLIATIVE APPROACH IN AMYOTROPHIC LATERAL SCLEROSIS: A POPULATION-BASED STUDY IN ITALY

Ilardi A¹

Cammarosano S¹

Manera U¹

Bertuzzo D¹

Pessia A¹

Moglia C¹

Calvo A¹

Veronese S²

Manazza A D³

Bersano G³

Chiò A¹

^sALS Center, ‘Rita Levi Montalcini’ Department of Neuroscience, University of Torino, Torino, Italy

^tFondazione FARO Onlus, Torino, Italy

^uSSD Cure Palliative, ASL TO4, Ivrea (Torino), Italy

Email address for correspondence: [email protected]

Keywords: ALS, palliative care, advance care planning

Background: Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder involving both upper and lower motor neurons; since the disease has a fatal course, patients’ optimal management requires a palliative approach with the aim of improving quality of life (QoL) through the evaluation and treatment of clinical symptoms and respect of patients’ decisional autonomy.

Aims: To evaluate the accessibility to Palliative Care Service (PCS) and the discussion rate of advanced directives in a population-based series of ALS patients’ resident in Piemonte, northern Italy.

Methods and Results: In our study, we enrolled 187 patients with definite or probable ALS according to the revisited El Escorial criteria, diagnosed over a period of 3 years, from 1 January 2008 through 31 December 2010, in the province of Torino, Piemonte, Italy. Ninty-nine patients were male (mean age at onset: 66.2 years (SD 9.9)), 88 were female (67.1 years (SD 10.8)). In 128 cases, the onset was spinal, while in the remaining 59 onset was bulbar; 172 patients had a sporadic ALS, 15 had a familial form of the disease. At the final date of the follow-up (1 April 2013), 139 patients were dead. In our ALS population, 41 patients (21.9% of all cases and 29.5% of deceased patients) were referred to PCS. The discussion of end-of-life issues was performed with 101 (54.0%) patients: 16 opted for tracheostomy, 81 refused tracheostomy, and 4 were uncertain. Patients’ advanced directives were followed in 90.6% of cases. We found no influence of gender, age at onset, ALS phenotype, marital status, and the use of NIPPV on patients’ choice about tracheostomy, while patients performing PEG were significantly more favorable to undergo tracheostomy (p = 0.0005). Most patients died at home, as previously observed, but patients referring to PCS received more frequent palliative sedation (p < 0.0001). Finally, in our ALS population, 12.6% of patients underwent tracheostomy, in keeping with a previous report.

Conclusion: According to our study, palliative interventions could improve patients’ QoL. In our opinion, it is necessary to make further efforts to increase the discussion rate of advance directives to comply with the personal autonomy of ALS patients.

P10 EXAMINING THE RELATIONSHIP OF BULBAR AND LIMB FUNCTION TO PATIENT REPORTED QUALITY OF LIFE: A MULTINATIONAL STUDY

Simmons Z¹

Stephens H¹

Felgoise S²

Abrahams S³

Czell D⁴

Genge A⁵

Gotkine M⁶

Jackson C⁷

Korngut L⁸

O’Connell C⁹

Weber M⁴

Zinman L¹⁰

^vPenn State Hershey Medical Center, Hershey, PA, USA

^wPhiladelphia College of Osteopathic Medicine, Philadelphia, PA, USA

^xUniversity of Edinburgh, Edinburgh, UK

^yNeuromuscular Disease Unit Kantonsspital St. Gallen, St. Gallen, Switzerland

^zMcGill University, Montreal, QC, Canada

^aaHadassah University Hospital, Jerusalem, Israel

^abUniversity of Texas Health Science Center, San Antonio, TX, USA

^acFoothills Medical Center, Calgary, AB, Canada

^adStan Cassidy Centre for Rehabilitation, Fredrickton, AB, Canada

^aeUniversity of Toronto, Toronto, ON, Canada

Email address for correspondence: [email protected]

Keywords: quality of life, functional rating, multinational research

Background: The ALS-Specific Quality of Life Instrument-Revised (ALSSQOL-R) was validated using an American sample. We previously reported country-specific differences in ALSSQOL-R scores despite similarities in overall function as measured by the ALS Functional Rating Scale Revised (ALSFRSR).

Objectives: To examine the relationship of bulbar and physical functioning to the bulbar QOL and physical QOL subscores of the ALSSQOL-R, as judged by patients with ALS, in a multinational sample.

Methods: Three Canadian, one Swiss, one Israeli, and one Scottish ALS Centers recruited individuals with definite, probable, probable laboratory-supported, or possible ALS. American data were obtained from a previous multi-center validation study. The ALSSQOL-R was administered in subject's native language. ALSSQOL-R bulbar and physical subscores as judged by patients, ranging from 0 (worst QOL) to 10 (best QOL), were calculated. Bulbar function was determined by adding the ALSFRSR speech, salivation and swallowing items. Physical function was calculated by summing the ALSFRSR handwriting, eating utensils, turning in bed, dressing and hygiene, walking, and climbing stairs items. Pearson correlations and one-way analysis of variance were calculated with P < 0.05 as significant. The study was approved by the IRB/ethics committee of each institution.

Results: For the study, 396 American, 75 Canadian, 35 Swiss, 56 Israeli and 10 Scottish subjects were recruited. The five groups were similar in age, sex distribution, and disease duration. Bulbar and physical function scores were similar among the American, Canadian, Swiss, and Scottish samples; the Israeli sample was significantly poorer, p < 0.05. Correlations between function and patient-reported QOL in these two domains differed among the groups. In the American sample, there was a positive correlation of bulbar function and bulbar QOL (r = 0.77, p < 0.05), and between physical function and physical QOL (r = 0.33, p < 0.05). A similar pattern was found for the Scottish sample, but only between bulbar function and bulbar QOL, (r = 0.73, p < 0.05). In the Canadian and Israeli samples, there were no significant relationships of bulbar and physical function with their related QOL constructs. In the Swiss sample, a significant negative correlation of bulbar function and bulbar QOL was found (r =− 0.69, p < 0.05).

Discussion and conclusion: The ALSSQOLR was developed and normalized on US residents. This study demonstrates possible country-specific or cultural differences in the relationship of function to QOL-related constructs. An alternative explanation is that differences in management of physical weakness (power lifts, power chairs, and hospital beds) and bulbar dysfunction (alternative communication devices and aggressive management of sialorrhea) may contribute to the impact of these factors on QOL. Caution is warranted when interpreting QOL measures in a population different from that in which the measures were validated. The factors which contribute to QOL in different countries, cultures, and disease-management systems merits further investigation.

P11 THE EFFECT OF A MULTIDISCIPLINARY CARE PROGRAM ON ALS PATIENTS SURVIVAL

Paipa A

Povedano M

Turon J

Hospital Universitari de Bellvitge, Barcelona, Spain

Email address for correspondence: [email protected]

Keywords: multidisciplinary care, survival, ALS

Background: Multidisciplinary care units have become the preferred delivery of care model for amyotrophic lateral sclerosis (ALS) patients over the last two decades; however, the impact of this approach on overall survival is still under debate.

Objectives: We sought to evaluate the impact of multidisciplinary care on mortality in our ALS population.

Methods: Retrospective analysis of survival of ALS patients treated in a referral tertiary care hospital under a general neurology clinic (GNC) program and after the implementation of a multidisciplinary care program (MCP).

Results: We evaluated 417 patients, 84 patients were followed on a general neurology clinic and 334 on a multidisciplinary care program. On baseline, the GNC patients were 4 years younger (59 vs 63 years) and with a male predominance (66% vs 53%). The MCP patients had a greater proportion of bulbar onset disease (30% vs 21%), though it did not reach statistical significance (p = 0.72). MCP patients were more likely to receive riluzole (88% vs 50% p < 0.01), NIPPV (48% vs 33% p 0.016), and PEG (32% vs 10%). Kaplan–Meier analysis showed that median survival was increased by 3 months (logrank 10.08, p = 0.001). Cox modeling showed that the multidisciplinary unit program was associated with a reduced risk of death (HR: 0.66 IC 95 0.49–0.85). On the subgroup analysis, we found a greater effect on the bulbar onset patients (HR: 0.43 IC 95: 0.23–0.83), though it was also found to be protective on spinal onset patients (HR: 0.69 IC 95 0.49–0.98).

Conclusions: The implementation of a MCP on the management of ALS patients has a significant impact on mortality. Bulbar onset patients are especially prone to benefit from interventions associated with such program.

References

• Miller RG et al. Neurology 2009;73:1227–1233.
   PubMed Web of Science ®Google Scholar
• Chio A, Bottacchi E, Buffa C et al. J Neurol Neurosurg Psychiatry 2006;77:948–950.
   PubMed Web of Science ®Google Scholar
• Traynor BJ, Alexander M, Corr B et al. J Neurol Neurosurg Psychiatry 2003;74:1258–1261.
   PubMed Web of Science ®Google Scholar

P12 EFFECTS OF COGNITIVE BEHAVIOURAL THERAPY (CBT) IN PATIENTS WITH ALS AND THEIR PARTNERS; PRELIMINARY RESULTS

van Groenestijn AC¹

Schröder CD¹

Visser-Meily JM¹

van den Berg LH²

^afRudolf Magnus Institute of Neuroscience and Center of Excellence for Rehabilitation Medicine, University Medical Center Utrecht and De Hoogstraat Rehabilitation, Utrecht, The Netherlands

^agDepartment of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Centre Utrecht, Utrecht, The Netherlands

Email address for correspondence: [email protected]

Keywords: QoL, CBT, partners

Background: The progressive and disabling nature of ALS produce an enormous impact on both patient's and partner's quality of life (QoL). CBT may be of benefit to patients and their partners.

Objective: To study the effects of CBT in addition to usual care compared with usual care alone, on QoL in patients with ALS and their partners.

Methods: A multicentre, single-blinded, randomized controlled trial (1) was conducted. A CBT intervention was developed targeting QoL in patients and partners. ALS patients who met the inclusion criteria (score ≥ 8 on the Hospital Anxiety and Depression Scale (HADS)) and their partners were randomised to CBT or usual care (UC). CBT consists of individual psychological support in 5–10 sessions over a 16-week period. QoL was assessed using the Short Form 36 (SF-36) pre-intervention, post–intervention, and at 3- and 6-months follow-up. For each group differences in mean scores of SF-36 mental health subscales at pre- intervention and 6-months follow-up were analysed using paired-sample t-test: Social Functioning (SF), Role- Emotional (RE), Vitality (VT), and Mental Health (MH).

Results: Only 47% of ALS patients who gave informed consent met the inclusion criterion of an elevated HADS scores. This resulted in an inclusion of 15 ALS patients within 3 years. Ten couples were randomised to the CBT and five couples to the UC. The median of received CBT sessions was 3 (range: 0–10), 50% of the couples needed less than the pre-planned minimum of five sessions. In both groups, T3-data were missing, because patients died (CBT N = 1; UC N = 1) or were overburdened due to disease progression (CBT N = 1). For both groups patients’ scores on SF, RE and MH subscales decreased, and VT increased. Partner's scores in the CBT increased on SF, RE and MH subscales, but decreased in VT, whereas partner's scores in the UC decreased in all subscales. However, all changes on the SF-36 subscales were not statistically significant.

Discussion and conclusion: The need for CBT in patients appeared to be low; on average three CBT sessions were sufficient to discuss their problems. Our preliminary results suggest that partners might benefit more than patients of a CBT-intervention targeting QoL. Future studies should focus more on needs of partners and other caregivers than on patients with ALS.

Acknowledgements

Funders: Prinses Beatrix Fonds and The Netherlands Organization for Health Research and Development.

Reference

• Van Groenestijn AC et al. BMC Neurology 2011;11:70.
   PubMed Web of Science ®Google Scholar

P13 BREAKING THE NEWS IN AMYOTROPHIC LATERAL SCLEROSIS. ALS PATIENTS’ REFLECTIONS ON THE TWO-TIERED APPROACH OF THE ALS CENTRE AMSTERDAM

Seeber A

Pols A

Hijdra A

Willems D

de Visser M

Academic Medical Centre Amsterdam, Amsterdam, The Netherlands

Email address for correspondence: [email protected]

Keywords: breaking bad news, tailor-made patient care, supportive care

Background: According to standards in oncology, breaking bad news should be fine-tuned to patients’ preferences, contain intelligible medical information, provide emotional support and offer a tailor-made treatment plan. To achieve these goals in ALS, neurologists of the tertiary ALS centre in Amsterdam see patients twice within 14 days to deliver the diagnosis and initiate the palliative care trajectory. However, this two-tiered approach to break the news has not yet been evaluated.

Objective: How do ALS patients reflect on the disclosure of the diagnosis and its consequences in a two-tiered appointment?

Methods: The first author performed non-participating observations of two-tiered appointments to break the diagnosis ALS, and subsequently interviewed patients in-depth about their experiences with this approach. Qualitative analysis consisted of open coding, followed by inductive analysis of all written material, observation reports and verbatim typed out interviews.

Results: Ten two-tiered appointments were observed and 21 ALS patients were interviewed in-depth about their views on this approach of breaking the news. All participants were native Dutchmen. They considered the sudden and full disclosure of suffering from an incurable and fatal disease as unavoidable and appropriate. The prospect of a short-term second appointment offered some structure for the period immediately following the devastating news. The time period between appointments left room for a first reorientation, allowing for the second appointment to be used to discuss various aspects of the diagnosis and its consequences in more detail. In addition, the second appointment was used to develop a plan for supportive management, adjusted to the needs and preferences of the patient.

Discussion: Our study shows that native Dutch ALS patients value full disclosure of the fatal diagnosis on the condition that professional follow-up is offered. The neurologist may play an important role in the transition of his patients to customized supportive and palliative care delivered by an ALS rehabilitation team. The approach of the ALS centre in Amsterdam may serve as a model for other – chronic – life-threatening diseases.

Conclusion: Dutch ALS patients value a two-tiered approach for breaking the bad news followed by a palliative care trajectory.

Acknowledgements

The authors would like to thank the ALS patients for their time and frankness in the interviews, and the Netherlands Organization for Health Research and Development (ZonMw) for funding.

P14 DIFFICULTIES OF HOME CARE NURSES SUPPORTING INDIVIDUALS WITH AMYOTROPHIC LATERAL SCLEROSIS UNTIL END-OF-LIFE

Ushikubo M¹

Iida M²

Okamoto K³

^ahGunma University, Maebashi-shi, Gunma, Japan

^aiGunma Prefectural University of Health Sciences, Maebashi-shi, Gunma, Japan

^ajHospital of Institute of Geriatrics, Maebashi-shi, Gunma, Japan

Email address for correspondence: [email protected]

Keywords: end of life, home care nursing, ALS

Objectives: Amyotrophic lateral sclerosis (ALS) is a life-limiting, devastating disease. No effective treatments are currently available. Respiratory failure is the leading cause of death and sudden death is not rare. The median time of ALS from onset of symptoms to death is 23–48 months. Thus, health care providers need to support ALS patients with the consideration that death may occur at any time, while getting a positive attitude towards life from their patients. The purpose of this study is to clarify the illness situations of individuals with ALS at home and difficulties of home care nurses supporting such patients from the initial stage of home care nursing to death.

Method: The study design was retrospective and qualitative. Home care nurses from 11 home care agencies in four prefectures participated in this study. Semi-structured interviews were conducted to each home care nurse about their patient's circumstances from the beginning of home care nursing to the end-of-life, details of the type of support provided, and difficulties they experienced. Qualitative-inductive analysis method was used to extract categories and sub-categories. This study was approved by the institutional review board of the author's university.

Results: Data from 14 ALS cases were collected, Six cases died at home and eight died in hospital. The final respiratory management of patients was as follows: none for three cases, oxygen administration for one, non-invasive positive pressure ventilation (NPPV) introduced, but not used for four, intermittent NPPV for one, and tracheostomy positive pressure ventilation (TPPV) for five. Difficulties experienced by home care nurse consisted of five categories and 14 sub-categories. The category ‘Support for decision-making of patients and families’ consisted of three sub-categories, ‘Difficulty to know the patient’s thoughts’, ‘Difficulty to communicate with patients’ and ‘Difficulty having patients use medical treatments and social services’. The category ‘Selection of how to deliver care’ consisted of three sub-categories including ‘Difficult palliation’. The category ‘Insufficient home care support system’ consisted of five sub-categories, ‘Difficulty to get a home doctor’, ‘Shortage of long-term facilities’, and three others. The category, ‘Support for caregivers’ consisted of the sub-category ‘Difficulty to deal with family caregiver’. The final category, ‘Disagreement about how to support’, consisted of two sub-categories, ‘Dilemma’ and ‘difficult assessment of approaching death’.

Conclusion: To increase the possibility of peaceful death at home, it is important for home care nurses to develop the skills for assessment of approaching death, methods to deliver palliative care, and support for decision-making of patients and families. Also, it is suggested that improving the possibility of home visits by home doctors or giving the right of death diagnosis to home care nurses will provide better care for their patients.

P15 FRAIL TERMINALITY: HEALTH PROFESSIONALS’ AND CARERS’ DYNAMIC AND DIVERGING PERCEPTIONS OF CHRONICITY AND TERMINALITY IN ALS/MND

Lerum S¹

Holmøy T^2,3

Solbrække K¹

Frich J¹

^akInstitute of Health and Society

^alAkershus University Hospital, Akershus, Norway

^amInstitute of Clinical Medicine, University of Oslo, Oslo, Norway

Email address for correspondence: [email protected]

Keywords: frail terminality, care work, coordination

Background: Several researchers have pointed out that ALS/MND resists a clear categorization as a chronic or terminal condition (1,2). In Norway, life sustaining technology is increasingly implemented in the hospitals, whereas a recent health reform (The 2012 Coordination Reform) reallocates further management to primary care and the home. The backdrop of this study is the unclear categorization of the ALS/MND diagnosis, and the need for good coordination in the complex division of labor in ALS/MND care.

Objectives: In this study we investigate how different actors in hospitals, primary care, and the home perceive and define ALS/MND as a chronic or terminal disease, and how this informs and motivates their work.

Methods: We have conducted 54 semi-structured interviews, lasting 1–2 hours, with carers at home, paid carers in primary care, and health professionals in hospitals and multidisciplinary teams, in Norway.

Results: The analysis demonstrates how the participants made continual assessments to whether a chronic or terminal framing should be used in perceiving day-to-day challenges. The actors involved in ALS/MND care emphasized chronic and terminal categorizations in subtly different ways, making for a potentially fragmented understanding of the challenge at hand. We suggest the concept frail terminality to describe such continual dynamics. ALS/MND is a fatal diagnosis. However, where the terminality is frail; it may be challenged and negotiated, as contingencies, choices, and interventions may make it less terminal. The indefinite time-scope of the prognosis may create an ongoing tension between enduring the situation in an unsustainable manner, appropriate for a terminal condition; simultaneously as the situation persists there is a need for more sustainable routines.

Discussion: The chronic-terminal unsettledness of ALS/MND complicates issues such as access to health services (hospice being one example), legal status (for instance regarding resuscitation), and attitudes among health professionals and carers. Awareness about how different stakeholders understand the challenges they encounter may facilitate a better coordination of care.

Conclusions: The concept of frail terminality may sensitise respective actors to articulate choices and dilemmas in ALS/MND care, allowing for more control in a potentially chaotic and overwhelming situation.

References

• Brown JB. Primary Health Care Research & Development 2003;3:207–217.
   Google Scholar
• Locock L, Ziebland S, Dumelow C. Sociology of Health & Illness 2009;7:1043–1058.
   Google Scholar

P16 PLANNING AHEAD FOR PATIENTS WITH MOTOR NEURONE DISEASE

Bates C¹

Greene M²

Rose G¹

Larrson E³

Radunovic A³

^anBarking, Havering & Redbridge University Hospital Trust, London, UK

^aoSt Francis Hospice, Essex, UK

^apBarts Health MND centre, London, UK

Email address for correspondence: [email protected]

Keywords: end of life, advance care planning

Objectives: To audit advance care planning (ACP) for MND patients under the care of a Regional MND Centre hospital multidisciplinary MND clinic, with reference to discussion about end-of-life care and documentation of the patient’s wishes, and to assess sharing of information between the hospital and hospice, and/or community setting.

Method: Retrospective Case note review of MND deaths during a 2-year period.

Results: Data were collected from 38 deaths. 58% of patients died within 12 months of diagnosis. Place of death was hospital (47%), home (29%), care home (8%) and hospice (13%). 42% had received Non-Invasive Ventilation (NIV). There was evidence of ACP in 28 (74%) patients. This took the form of an Advance Decision to Refuse Treatment (18%), an advance statement about preferred place of care (13%), a ‘Do not attempt resuscitation’ order (53%) and medical letters or notes describing the end-of-life care plan (63%). ACP focused on the preferred place of care (14/28), resuscitation (12/28), ventilation (11/28), artificial feeding (10/28) and antibiotics (3/28). There were significant gaps in sharing of information between the hospital and community setting; of 13 patients with DNAR forms, only two patients had the DNAR documented in both sets of notes. Information sharing was most successful via copies of clinic letters (shared in 12/14). 56% of patients died in their preferred place of care. Where preferred place of care (PPC) was not achieved, 71% of patients died in hospital. Overall, end-of-life care for 76% of patients was deemed to have completely or partially complied with the patient’s wishes.

Conclusion: A proactive approach to end-of-life planning by the multidisciplinary team led to evidence of planning ahead in 74% of patients. DNAR orders and compliance with refusal of ventilation and feeding was more successful than achieving PPC. Work is needed to strengthen ACP and coordination of care locally to reduce hospital deaths and improve patient care. Communication between care providers is the key to the delivery of high-quality end-of-life care for patients with MND.

P17 THE MEANING OF LOSS FOR PEOPLE WITH AMYOTROPHIC LATERAL SCLEROSIS: IMPACT ON DECISION-MAKING IN CARE

Foley G¹

Timonen V¹

Hardiman O^1,2

^aqTrinity College Dublin, Dublin, Ireland

^arBeaumont Hospital, Dublin, Ireland

Email address for correspondence: [email protected]

Keywords: loss, control, decision-making

Background: Few studies have investigated the meaning of loss for people with ALS, and no studies have explained how people with ALS engage with healthcare services as they adapt to loss. ‘Biographical disruption’ has been described in ALS, but little is known about how people with ALS construct loss and how their response to loss shapes how they engage with healthcare services.

Objectives: The aim of this study was to map key parameters of ALS service users’ experience of healthcare services. The objective of the study was to identify how and why people with ALS interact with services.

Methods: Grounded theory method (1) was used to identify psychosocial processes which underpin how people with ALS engage with health and social care services. A diverse group of people with ALS (n = 34) were theoretically sampled from the Irish ALS population-based register. In-depth qualitative interviews were conducted with each participant in their homes. Data were analysed using open, axial and selective coding procedures (1). Codes formed concepts which in turn formed categories (variables) and the relationships between categories formed substantive theory to explain how people with ALS engage with services.

Results: Participants were living with insurmountable loss and never regained what they had already lost. The meaning of loss expanded across multiple dimensions including: loss of control; loss of identity; loss of the future; loss of parenthood; loss of hope; loss of participation; and loss of independence.

Losing control and fighting to remain in control comprised two central components in how participants interacted with service providers. The perception of loss of control prompted service users to exert control as they engaged with services. Participants expressed a desire to follow through with their own preferences for care and they engaged with services in line with their own perceptions of disability as opposed to those of service providers. Assistance from providers was more likely to be accepted and tolerated when perceived to be on participants’ terms.

Discussion: We found that adaptation to loss in ALS is bidirectional: ALS service users navigate back and forth between resisting and accepting change. Living with ALS constitutes continuous loss for people with ALS, but they have the capacity to negotiate loss via complex appraisal strategies which include exerting control over and rendering control to service providers.

Conclusions: Service users’ need to feel in control of health and social care services arises from their experiences of loss. Service providers should pay close attention to how people with ALS adapt to loss as they support them in the decision-making process about care.

Acknowledgements

Funding: Health Research Board (HRB) of Ireland.

Reference

• Corbin J, Strauss A. Basics of qualitative research. Techniques and procedures for developing grounded theory 3rd ed. 2008; Thousand Oaks: Sage.
   Google Scholar

P18 WHERE AND HOW DO PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS) DIE?

Varela Cerdeira M

Sanz Peces E

Gainza Miranda D

Rodriguez Barrientos R

Alonso Babarro A

Rodriguez de Rivera F

Hospital Universitario La Paz, Madrid, Spain

Email address for correspondence: [email protected]

Keywords: home care, place of death, end of life symptoms

Introduction: The final phase in patients with ALS has been the subject of few studies. It was our main objective to discover where and how patients die who are under observation from a Palliative Home Care Team (PHCT). Other, secondary objectives were to analyse the care burden, the main symptoms and the administered drugs during the tracking period.

Methods: Descriptive and retrospective study of patients with ALS followed up by PHCT (2001–2012), composed of a Multidisciplinary Unit of ALS. Analysis of variables through SPSS 15.0 and survival with Kaplan–Meier curves was carried out.

Results: Thirty-eight patients were followed up by the PHCT during the course of the study: 29 had died at the time the study was finalized; Mean age was 64 years (SD 12.5); 58% were females; Barthel index average 25; 89% had received previous instructions (33); 39% (15) presented bulbar ALS; 58% (22) spinal ALS; and 3% (1) familial ALS. In 45% (17) of the cases a gastrostomy was placed for feeding; 68% (26) used non-invasive ventilation (NIV); and 11% (4) used invasive ventilation. 82% (31) of the patients was taking riluzona at the start of the observation by PHCT and four patients died while taking the drug. The average observation period was 80 days. PHCT made an average of 14 visits (SD 13). The average number of hospitalizations per patient during the observation was 0.7. The median survival of patients since they started NIV was 8 months (CI 95% 2.4–13.6).

At the beginning of the observation by PHCT, the principal symptoms were pain (27%); sialorrhea (24%), dyspnea (21%); dysphagia (18%); and insomnia (5%). For 68% (19) of the patients who died, a subcutaneous route was needed during the last 24 hours. The most commonly administered drugs were morphine (68%); anticholinergic (39%); and midazolan (39%). 28% (8) needed palliative sedation because of the following reasons: 50% (4) dyspnea; 37% (3) rejection of invasive and non-invasive mechanical ventilation; and 12% (1) delirium. The drug that was applied in most cases was midazolan with a mean dose of 40 mg/day.

The percentage of the patients who died at home was 66% (19); 10% (3) in a hospice; and 24% (7) at a general hospital. The cause of death was respiratory failure 52% (15); respiratory infection 20% (6); and 10% (3) other causes, while 18% (5) of the patients died while asleep.

Conclusions: For patients that were observed by a PHCT frequent visits were necessary due to their diverse symptomatology. Most of the patients could die at home. The most common cause of death was respiratory failure that needed treatment with morphine in most of the cases and palliative sedation in 29% of the patients.

P19 INTERACTION OF PHYSICAL FUNCTION, QUALITY OF LIFE AND DEPRESSION IN AMYOTROPHIC LATERAL SCLEROSIS: CHARACTERIZATION OF A LARGE PATIENT COHORT

Koerner S¹

Kollewe K¹

Abdulla S¹

Zapf A²

Dengler R¹

Petri S¹

^asMedical school Hannover, Hannover, Germany

^atUniversity Göttingen, Göttingen, Germany

Email address for correspondence: [email protected]

Keywords: depression, quality of life, physical impairment

Background: Due to lack of any curative therapy of amyotrophic lateral sclerosis (ALS), symptomatic treatment and maintenance of quality of life (QoL) is very important.

Objectives: We aimed to characterize the affected domains of QoL in ALS patients and to identify impact factors on QoL and depression.

Methods: One hundred and fifty-nine ALS patients answered three standardized questionnaires (Beck depression inventory – II, SF-36 Health Survey questionnaire, and revised ALS functional rating scale). Multiple regression analysis and t-tests for independent samples were used to identify correlations between clinical features of ALS patients and depression/QoL scores. In addition, QoL data from ALS patients were compared to age-matched reference values representing the German normal population.

Results: QoL of ALS patients was reduced in nearly all SF-36-categories. Progression of physical impairment was positively correlated with depression, but hardly with deterioration of QoL. However, QoL was considerably influenced by depression, independent from physical impairment. Regarding distinct patient characteristics, advanced bulbar symptoms and increasing age were correlated with significantly worse QoL results regarding social functioning. Spinal onset patients suffered significantly more often from pain and physical limitations.

Conclusions: Depressive symptoms had a strong influence on QoL, hence their detection and treatment is of particular importance. Different domains of QoL are differently affected in subgroups of ALS patients. The knowledge about these differences can be valuable for both ALS caregivers and physicians.

P20 BULBAR SYMPTOMS AS PHYSICAL DETERMINANTS OF QUALITY OF LIFE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS: A SYSTEMATIC REVIEW

Mohammad M²

Young C^1,2

^auWalton Centre NHS Trust, Liverpool, UK

^avUniversity of Liverpool, Liverpool, UK

Email address for correspondence: [email protected]

Keywords: quality of life, bulbar symptoms, TONiC study

Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease which can cause bulbar symptoms such as dysarthria, dysphagia and sialorrhoea. Previous work has found quality of life (QOL) in ALS to be weakly related to physical dysfunction and psychosocial factors appeared more influential. However, bulbar symptoms may be very distressing to people with ALS and so it is important to explore how these symptoms may affect QOL.

Objective: To systematically review existing literature concerning the effects of dysarthria, dysphagia or sialorrhoea on the QOL of patients with ALS, in preparation for the Trajectories of Outcome in Neurological Conditions (TONiC) study, a multicentre national study of QOL in ALS.

Methods: MEDLINE (1990–2013), ScienceDirect (1990–2013), Ovid SP (1990–2013) and Compendex (1990–2013) were searched. Inclusion criteria stated studies must examine one of the three symptoms and have QOL as an outcome measure. The bibliographies of other studies which examined physical and psychological determinants of QOL in ALS were reviewed for additional references.

Results: For dysarthria, there were one non-blind experimental and one prospective study. In both studies, QOL was assessed in relation to communication support. Both concluded that there was a significant improvement in QOL with the introduction of communication support. Dysarthria was the only factor looked at in relation to QOL in the experimental study, but the prospective study also looked at non-invasive ventilation methods. For dysphagia, one survey was found on swallowing function. It concluded that apart from nutritional deficiencies created by dysphagia, there was also a reduction in QOL. For sialorrhoea, one double-blind randomized trial and five open-label prospective studies were found. The randomized trial showed no significant improvement in QOL after botulinum toxin treatment. From the five open-label studies, four looked at botulinum treatment and one looked at radiotherapy. All open-label studies concluded an effect on QOL.

Discussion and conclusion: The studies in this systematic review show variations in the methods used for measuring QOL, which impairs comparison between studies, and may have influenced findings. Each study has a small sample size which weakens the validity of their conclusions but as a group of nine studies, eight concluded that there was a relationship between QOL and bulbar symptoms. Physical, specifically bulbar, symptoms are associated with QOL in ALS. This highlights the possibility that psychosocial symptoms could be confounding physical symptoms when both are examined in combination. Further research is needed into the physical determinants of QOL, including looking at bulbar symptoms as they have been shown to have an impact on QOL in these initial studies.

P21 THE RELATIONSHIP BETWEEN BULBAR FUNCTION AND QUALITY OF LIFE IN PATIENTS WITH ALS

Davies J¹

Stephens H²

Simmons Z²

^awPenn State College of Medicine, Hershey, PA, USA

^axPenn State Hershey Medical Center, Hershey, PA, USA

Email address for correspondence: [email protected]

Keywords: quality of life, bulbar function, multidisciplinary care

Background: The ALS Specific Quality of Life-Revised (ALSSQOLR) questionnaire measures six domains of QOL: Negative Emotion, Interaction with People and the Environment, Intimacy, Religiosity, Physical Symptoms, and Bulbar Function. Overall QOL in patients with ALS is not related to overall physical function. However, bulbar dysfunction produces unique challenges with regard to speech, swallowing, and secretion/mucus management, and the relationship between QOL and bulbar function has not been studied.

Objectives: To compare QOL in ALS patients with significant bulbar dysfunction to that of patients whose bulbar function is normal or minimally impaired.

Methods: An IRB-approved retrospective review of data collected in a multidisciplinary ALS clinic during a recent 24-month period was performed. The earliest visit of each patient was reviewed, excluding patients with significant cognitive impairment. Bulbar patients were classified as those who reported cumulative ALSFRSR bulbar sub-scores of 9 or less on items of speech, salivation and swallowing, corresponding to items 1–3 of the ALSFRSR. QOL was assessed with the ALSSQOLR, in which scores range from 0 (worst QOL) to 10 (best QOL) for average total QOL as well as the six domain sub-scores. Demographic data was also collected. Analysis of variance was performed comparing bulbar to non-bulbar groups on the dependent variables of average total ALSSQOLR score and the domain sub-scores.

Results: There were 100 patients: 48 men, 52 women, mean age 61 years, and 59% had bulbar. Men comprised 39% of the non-bulbar group and 54% of the bulbar group (p = NS). There was no difference in mean age between the non-bulbar (59 years) and bulbar (62 years) groups. Average disease duration was 55.2 months (SD 55.0) in the non-bulbar group and 42.8 months (SD 37.4) in the bulbar group (p = NS). Mean ALSFRSR score was higher in the non-bulbar than the bulbar group: 31.4, SD 8.8 vs. 21.4, SD 9.8 (p < 0.05). Bulbar patients reported a significantly lower average total QOL score (mean: 6.34) compared to non-bulbar patients (mean: 6.96), p < 0.05. There were no differences between the two groups in the six domain sub-scores, except for mean Bulbar QOL which was lower in the bulbar than the non-bulbar group: 4.32 vs. 8.01 (p < 0.001).

Discussion and conclusions: QOL in 5 of 6 domains is equal in ALS patients without and with significant bulbar dysfunction, but the bulbar QOL domain is lower in the bulbar group, resulting in a lower overall QOL. This was true even in a multidisciplinary clinic in which great attention is paid to symptom management and optimization of QOL. Further work needs to be done to determine how the impact of bulbar symptoms on ALS patients’ QOL can best be addressed by health care teams.

P22 PSYCHOSOCIAL FACTORS AFFECTING QUALITY OF LIFE IN MOTOR NEURONE DISEASE: A SYSTEMATIC REVIEW OF THE LITERATURE

Nee L^1,2

Goldstein L³

Young C^1,2

^ayWalton Centre NHS Trust, Liverpool, UK

^azUniversity of Liverpool, Liverpool, UK

^baInstitute of Psychiatry, London, UK

Email address for correspondence: [email protected]

Keywords: quality of life, psychosocial factors, TONiC study

Background: As motor neurone disease (MND/ALS) is progressive and fatal, supporting quality of life (QOL) is a primary concern. Previous work has found QOL in MND/ALS to be weakly related to physical dysfunction, whereas some psychosocial factors appeared important.

Objective: To conduct a systematic literature review to determine psychosocial factors affecting QOL in MND/ALS, in preparation for the Trajectories of Outcome in Neurological Conditions (TONiC) study, a multicentre national study of QOL in MND/ALS.

Methods: Literature searches were conducted in Medline, Psychinfo, Cochrane Library, and the Amyotrophic Lateral Sclerosis and Frontotemporal Dementia journal to identify studies reporting relationships between psychosocial factors and QOL in people with MND/ALS.

Results: One hundred and three potential studies were retrieved after assessing the abstracts of publications identified through the searches. Of these, 75 were omitted on the basis of exclusion criteria such as reviews, case studies, diseases other than MND/ALS, outcomes other than QOL, psychosocial factors not included, and not published in English. This left 28 studies meeting the eligibility criteria. Five psychosocial factors were identified: These were social support; affective state (depression, anxiety, and hopelessness); religion/spirituality; coping strategies; and personality traits. Fifteen studies reported a positive relationship between social support and QOL. There were discrepancies among studies investigating affective state. Eight studies found that depression was negatively associated with QOL, whereas six found no significant relationship. Likewise, findings on anxiety were inconsistent. Four studies reported a negative association between anxiety and QOL while three found no relationship. Only two studies investigated hopelessness and in both it was negatively associated with QOL. While religion/spirituality was a positive factor in eight studies, one study disputed this. Coping strategies (two studies) and personality traits (two studies) received limited attention.

Discussion and conclusion: Social support has the strongest and most consistent evidence as a factor associated with QOL in MND/ALS. Discrepancies in the literature on affective state may be attributable to methodological considerations, such as differences in how QOL is conceptualised or measured. Fewer studies investigated anxiety than depression, despite several studies finding anxiety to be more strongly associated than depression with QOL. Anxiety may therefore represent an underestimated problem. Future research should establish its role. While the potential influence of religion/spirituality on QOL is strongly supported in the literature, one study found that patients infrequently identified it as a relevant factor. That study was conducted in a secularised country (Sweden). Future research should establish whether religion/spirituality is an important factor across different populations of MND/ALS patients. Coping strategies and personality traits warrant additional investigation. Of further note, all reviewed studies were observational. Interventional designs are required to investigate whether interventions targeting psychosocial factors positively affect QOL.

P23 FUNCTIONING, FATIGUE AND PSYCHOSOCIAL FEATURES OF MND/ALS: ASSOCIATIONS CHANGE OVER TIME AND IMPACT ON PATIENT QUALITY OF LIFE

Gibbons C²

Thornton E³

Ealing J⁴

Shaw PJ⁵

Talbot K⁷

Tennant A⁶

Young C^1,3

^bbWalton Centre NHS Trust, Liverpool, UK

^bcUniversity of Manchester, Manchester, UK

^bdUniversity of Liverpool, Liverpool, UK

^beSalford Royal NHS Foundation Trust, Manchester, UK

^bfUniversity of Sheffield, Sheffield, UK

^bgUniversity of Leeds, Leeds, UK

^bhDepartment of Clinical Neurology, Oxford, UK

Email address for correspondence: [email protected]

Keywords: quality of life, longitudinal study, TONiC study

Objective: We conducted a longitudinal study of important physical and psychosocial features of amyotrophic lateral sclerosis (ALS/MND) and investigated their associations, change over time and impact on patient quality of life. The work forms part of the development of the Trajectories of Outcome in Neurological Conditions (TONiC) study, a national multicentre study of quality of life (WHOQoL-BREF) in neurological conditions including ALS/MND.

Methods: Patients were recruited from five hospitals in England (Liverpool, Oxford, Preston, Salford, and Sheffield), and completed questionnaires at baseline and at 6-month follow up.

Study variables were measured with disease-validated questionnaires for: functional capacity (ALSFRS-R); fatigue (MND-NFI); depression and anxiety (HADS); social withdrawal (MND-SWS); coping (MND-Cope); and WHOQoL-BREF.

The suitability of questionnaires for this population was confirmed using Rasch analysis, so deriving latent estimates suitable for parametric analysis. Consequently, data were analysed using linear regression modelling and t-tests.

Results: One hundred and seven patients recruited to a longitudinal study completed questionnaire packs 6 months apart.

Mean scores for depression increased over the course of the study (p < 0.001). Fatigue was strongly related to depression, anxiety, coping and social withdrawal at baseline (p < 0.001). Fatigue was not related to functional ability at baseline (p = 0.33), but was associated with reduced functional capacity at follow-up (p = 0.003).

Fatigue, social withdrawal and functional impairment substantially increased over the course of the study (p <0.001). Mean scores for coping and anxiety remained stable throughout the study (p > 0.05).

WHOQoL-BREF at 6-month follow-up was associated with depression, anxiety fatigue, coping and social withdrawal at baseline, but not functional capacity. Fatigue and coping at baseline were the only significant predictors of WHOQoL-BREF at 6-month follow-up (p < 0.05).

Discussion and conclusion: Severity of depression appears to increase over the duration of the illness. Levels of depression were associated with fatigue, but did not predict WHOQoL-BREF at 6-month follow-up. Baseline fatigue and coping did predict QoL at follow-up.

This study provides longitudinal evidence demonstrating that high fatigue and poor ability to cope lead to reduced WHOQoL-BREF for patients with ALS/MND.

P24 PHYSICAL THERAPY AND EXERCISES TO PATIENTS WITH ALS

Versterre S

Buus L

Hilleroed Hospital, Hilleroed, Denmark

Email address for correspondence: [email protected]

Keywords: physical therapy, exercise therapy, chest physical therapy

Background: Danish physical therapists in the ALS teams are responsible for exercise guidance, evaluating needs for assistive devices, and chest physical therapy. Until now there has been no consensus among physical therapists, in Denmark, about which treatment to use and when to use it. Only very few articles, describe in detail the treatment given by physical therapist to patients with ALS.

Objectives: The aim of this study was to investigate which treatment is used, what is the evidence for the treatment, what treatment/instructions do the patient think is important, and based on that create a national clinical guideline in Denmark.

Methods: Fourteen hospitals in Denmark diagnose and treat patients with ALS. Thirteen have an ALS team, with a physical therapist. All 13 teams are included in the study.

The study is divided into three steps.

Step 1: Involvement of the physical therapists: (a) Answering a semistructured questionaire about their treatment strategies to patients with ALS; (b) Participation in a workshop, with focus on discussion of evidence, treatment strategies and creating consensus about the physiotherapeutic treatment to patients with ALS in the hospitals in Denmark; and (c) Qualifying the clinical guideline by testing it in clinical practice.

Step 2: Involvement of the literature: (a) A systematic search in the databases Pubmed, Cinahl, Cochrane and Pedro and (b) Evidence extracted.

Step 3: Involvement of the patients: (a) A focus group interview to assess what the patients think is important in the physical therapy treatment/instruction and (b) The clinical guideline is sent in consultation.

In the process there has been contact to The Danish Institute for Clinical Guidelines.

Results: Both the physical therapists, the patients and the literature agree that the physical therapist has an important role in guidance regarding respiration, exercise and assistive devices. Five papers included data about exercise and 12 about chest physical therapy. A national clinical guideline has been made and contains nine recommendations on exercise and 12 on chest physical therapy based on evidence level B–D.

Discussion and conclusion: A clinical guideline has been made and builds on best practice experience from the Danish ALS physical therapist, the patient view and evidence from the literature. The evidence level is low because there is lack of resource regarding exercise and chest physical therapy to patients with ALS, and there is a high dropout rate in the studies due to the nature of the disease. By standardizing physical therapy to ALS patients in Denmark, treatment quality is ensured and resources are made possible.

P25 EFFECTS OF ENDURANCE TRAINING ON QUALITY OF LIFE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

Prati C

Basilico M

Sartorelli L

Picardi M

Pain D

Marinou K

Mora G

I.R.C.C.S. Fondazione Salvatore Maugeri, Milan, Italy

Email address for correspondence: [email protected]

Keywords: endurance, quality of life, amyotrophic lateral sclerosis

Background: The control of fatigue can improve function and Quality of Life (QoL) in patient with progressive illness, reducing physical, psychological and spiritual distress. The role of exercise to reduce fatigue in people with ALS is controversial. However, recent studies promoting aerobic exercise in ALS patients as a source of well being on psychology of patients have been published (1,2).

Objectives: The aims of this study are to determine proper loads of therapeutic programs suited for each patient and to determine the effects of endurance training on QoL.

Methods: Twenty consecutive ALS patients (10 male and 10 female) have been recruited for this study and randomized in two groups: Control Group (CG) in which patients receive usual care and cicloergometer without increase in time training and load and Experimental Group (EG) in which patients received usual care and cicloergometer aerobic training in the stronger pair of limbs. The aerobic training was repeated thrice a week for 4 weeks. All patients underwent the following evaluations, at baseline and at the end of treatment: Cicloergometer incremental Test, ALS Funtional Rating Scale-revised (ALSFRS-R), Hospital Anxiety and Depression Scale (HADS), ALS Quality of Life-40 items (ALSAQ-40), Mini Mental State Examination (MMSE), and Fatigue Brief Inventory-Italian version (3). After every treatment patient underwent Numeric Pain Rating Scale (NPRS), Numeric Rating Fatigue Scale (NFRS), BORG Scale, and Fatigue Brief Inventory-Italian version.

Results: Mean age at evaluation was 60.7 (±12.4) years. Mean ALSFRS-R score was 33.38 (±5.78). Abnormal value of ALSAQ-40 were detected in 30% of patients (cut-off > 69), HADS was impaired in 55% of patients (cut-off > 8). A relationship between the mean score of Brief Fatigue Inventory and Anxiety (χ² = 28), Depression (χ² = 28), QoL (χ² = 42), and MMSE (χ² = 28) were found (p-value < 0.05). At baseline Brief Fatigue Inventory mean score was 26.7 (± 24.68), at T2 was 34.8 (± 24.72). Differences in the Brief Fatigue Inventory between the CG and the EG was detected (t = 2.58) (p-value < 0.05).

Discussion: Preliminary data showed positive effect of training on ALS patients fatigue and QoL. However, further analysis is needed to better clarify these observations.

References

• Dal Bello-Haas V et al. 2007.
   Google Scholar
• Van Groenestijn AC et al. 2011.
   Google Scholar
• Catania G et al. 2012.
   Google Scholar

P26 AN EVALUATION OF THE EFFECT OF CORTICOSTEROID INJECTION ON SHOULDER PAIN AND SLEEP QUALITY IN PATIENTS WITH MOTOR NEURON DISEASE

Campion A¹

Caldwell F¹

Gilsenan C¹

Murray D¹

Vance R¹

McGroarty D¹

Hardiman O²

^biPhysiotherapy Department, Beaumont Hospital, Beaumont Rd, Dublin 9, Ireland

^bjNeurology Department, Beaumont Hospital, Beaumont Rd, Dublin 9, Ireland

Email address for correspondence: [email protected]

Keywords: shoulder pain, steroid injection, sleep

Background: Shoulder pain and discomfort is a common feature of Motor Neuron Disease (MND) (1). Particular problems with shoulder pain arise for MND patients at night (2). Corticosteroid injection therapy (CIT) provides an effective treatment for shoulder pain (3), however, there is no evidence regarding its efficacy in MND patients specifically.

Objectives: To determine the effect of CIT on shoulder pain and sleep quality in patients with MND.

Methods: Patients with shoulder pain were identified at MND multidisciplinary clinic and suitability for treatment with CIT was determined. History of shoulder pain, intensity measured using numerical rating scales (NRS) and sleep quality using a Modified Pittsburgh Sleep Index were recorded. Patients were contacted by telephone 2 weeks and 2 months post CIT to reassess pain, sleep quality and global perceived change. Data were entered into Microsoft excel and exported to STATA 12 for analysis. Summary statistics were derived and change over the three time points was assessed using one-way ANOVA.

Results: Fourteen patients (F = 9) with shoulder pain, with average age of 63 ± 9.8 years, were treated with CIT. On reassessment at 2 weeks, 93% reported an improvement on the global perceived change and 88% reported improvement at 2 months. Two patients reported complete recovery of shoulder pain at 2 weeks and 2 months. Two patients were unable to complete 2-month follow-up. Average daily pain on NRS at baseline was 4.9 ± 2.6, at 2 weeks was 2.3 ± 1.7 and at 2 months was 2.1 ± 1.7 indicating a significant change post treatment (p = 0.003). Night pain also reduced, from 5.6 ± 3.3 to 2.5 ± 2.5 at 2 weeks and 1.9 ± 2.5 at 2 months (p = 0.006). The number of nights with sleep disturbance due to shoulder pain reduced significantly from 6.2 ± 1.5, to 4.8 ± 3.1 and to 3.8 ± 3.3 (p = 0.026).

Discussion and conclusion: CIT was found to significantly reduce shoulder pain, night pain and the number of nights with sleep disturbance due to shoulder pain in MND patients. This effect was seen both at 2 weeks post CIT and was maintained at 2 months post CIT. Therefore this finding provides strong evidence that this treatment should be offered to MND patients with shoulder pain and reduced sleep quality.

Acknowledgements

www.mnd.ie, Physiotherapy dept, MND MDT Beaumont Hospital.

References

• Newrick PG and Langton-Hewer R. J Neurol Neurosurg Psychiatry 1985;48:838–840.
   PubMed Web of Science ®Google Scholar
• Talbot K, Marsden R. “Motor Neuron Disease: The Facts”. Oxford University Press. 2008.
   Google Scholar
• Buchbinder R, Green S, Youd JM. Cochrane Database Syst Rev 2003; (Issue 1): Art No.CD004016.
   Google Scholar

P27 RELATIONSHIP BETWEEN QUALITY OF LIFE AND RESPIRATORY ASPECTS, DIAGNOSIS TIME AND FUNCTIONALITY IN AMYOTROPHIC LATERAL SCLEROSIS

de Carvalho EV^1,2

Soares Santos N^1,2

Gonçalvez Holsapfel S^1,2

Leico Oda A^1,2

Stanich P^1,2

Souza Bulle Oliveira A^1,2

^bkFederal University of Sao Paulo, Sao Paulo, Brazil

^blBrazilian Association of Amyotrophic Lateral Sclerosis, Sao Paulo, Brazil

Email address for correspondence: [email protected]

Keywords: quality of life, respiratory aspects, functionality

Introduction: Weakness and muscle atrophy, spasticity, fasciculations, dysarthria, dysphagia and respiratory failure are symptoms of ALS patients. The clinical picture of such individuals can influence the quality of life (QOL), defined according to the World Health Organization as how individuals understand their position in life, culture context, value systems and the relation established with the environment they live (1–3).

Objective: To correlate the QOL of ALS patients with respiratory aspects, diagnosis time, functionality.

Method: Cross-sectional study performed in the department of research on neuromuscular diseases, Federal University of São Paulo (UNIFES), Brazil. Based on the El Escorial criteria, able to answer questionnaires and conduct pulmonary function tests, we included subjects with confirmed diagnosis of ALS. There were 22 participants with average age of 54.59 ± 7.01: 11 (50%) men; 11 (50%) women. For the QOL assessment, participants answered the SF-36 and WHOQOL questionnaire. Tests applied are forced vital capacity (FVC); maximal inspiratory pressure (MIP); maximal expiratory pressure (MEP); and peak cough flow (PCF). From the medical records, we collected diagnosis time, onset of symptoms and use of non-invasive ventilation; moreover, using ALSFRS scale, we investigated the functionality. We also used the Pearson test, with significance level of p < 0.05.

Results: The WHOQOL physical domain correlates with MEP (r = 0.042/p = 0.049), PCF (r = 0.516/p = 0.014) and ALSFRS scale (r = 0.477/p = 0.025); WHOQOL social relationships domain, with ALSFRS scale (r = 0.452/p = 0.035). The SF-36 physical functioning domain positively correlated with MEP (r = 0.463/p = 0.030), PCF (r = 0.563/p = 0.006) and ALSFRS score (r = 0.593/p = 0.004). Moreover, the SF-36 physical limitations domain correlated with MEP (r = 0.482/p = 0.023) and PCF (r = 0.598/p = 0.003); SF-36 general health status domain, correlated with ALSFRS scale (r = 0.451/p = 0.035). The SF-36 mental health domain showed positive correlation with diagnosis time (r = 0.474/ p = 0.026).

Discussion: In ALS, FVC is an indication of respiratory impairment; however, ALS did not correlate with QOL, and only MEP and PCF values can affect on QOL. From the findings herein, functionality is enormously important for the QOL of ALS patients. The mental health domain correlated with diagnosis time, that is the longer the disease period the better the QOL in the mental health domain. This result may be related with the changes and acceptances imposed during the course of the disease.

Conclusion: The QOL, physical functioning-related domains and general health correlate with MEP, PCF and functionality; also, mental health domain correlates with diagnosis time.

References

• Wijesekera LC, Leigh PN. Orphanet J Rare Dis 2009;4:3.
   PubMed Web of Science ®Google Scholar
• Rowland LP, Shneider NA. N EnglJ Med 2001;344: 1688–1700.
   PubMed Web of Science ®Google Scholar
• Tramonti F, Bongioanni P, Di Bernardo C et al. Psychol Health Med 2012;17:621–8.
   PubMed Web of Science ®Google Scholar

P28 DISABILITY, ASSISTANCE AND MOBILITY AIDS IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

Ortiz-Corredor F^1,2

Mendoza-Pulido C²

Peña-Preciado M²

Mora M^1,2

^bmUniversidad Nacional de Colombia, Department of PM&R, Bogotá, Colombia

^bnInstituo de Ortopedia Infantil Roosevelt, Boogtá, Colombia

Email address for correspondence: [email protected]

Keywords: disability, ALSFRS-R, quality of life

Background: In patients with ALS, functioning may be described according to the assistance and mobility aids that are required. The goal of this study is to assess the validity of a disability classification system that is based on the ALSFRS-R and includes four levels of disability: mild, moderate, severe and extreme.

Methods: During an initial evaluation, 140 patients with ALS (mean age 57.3 years, SD 12.4; 59.2% male) were classified according to El Escorial criteria, the ALSFRS-R, the rate of progression of the disease and the number of muscles with severe weakness. The rate of progression of the disease was defined as the ratio of the actual ALSFRS-R/time from onset (months). For the neck flexion time, patients were asked to hold their heads flexed as long as possible (seconds). For the counting test, patients were asked to take a deep breath and count up until a new breath was needed (last number registered).

All patients were contacted 6 months later. Just 101 answered the ALSFRS-R and other questions related to assistance. Assistance was measured with an ordinal scale (0–8) based on the presence/absence (0/1) of nursery support, BPAP/CPAP, supplementary oxygen, wheelchair, dippers, gastrostomy, communication devices and need for other persons for transferences inside the house.

The levels of disability were established by taking one representative activity from the three domains of the ALSFRS-R that were defined by factor analysis (walking, cutting food and swallowing). Each of these was dichotomized as dependent (ALSFRS-R of 0, 1 or 2) or independent (3 or 4). Mild disability was defined as independence in all of the activities, moderate as dependence in one and independence in the other two, severe as dependence in two activities and independence in one. Extreme disability was defined as dependence in all three activities.

Results: A correlation was found between the disability scale and the rate of progression (r = 0.43, p < 0.001), the number of severely weak muscles (r = 0.33, p < 0.001), the neck flexion time (r = − 0.48, p = 0.004) and the counting test (r = − 0.48, p = 0.001). A correlation with the ordinal scale of assistance was found (r = 0.77, p < 0.001), especially with the number of persons needed for transferences (r = 0.6, p = 0.001).

Nursery care was needed in 0%; 0%; 23.1%; and 70.1% for the four levels of disability. The proportion of wheelchair-bound patients was 14.3%; 25%; 58.3%; and 94.1% (chi square p < 0.001). The need for other persons for transferences was 0%; 14%; 28%; and 57.1%. Non-significant correlation was found with BPAP/CPAP or supplementary oxygen.

Conclusion: This disability classification system is simple and easy to use in everyday practice. It is also useful as a guide for treatment and rehabilitation and as a reference for an easier communication between those involved in the care of patients.

P29 UROLOGICAL MANAGEMENT IN AMYOTROPHIC LATERAL SCLEROSIS/MOTOR NEURON DISEASE (ALS/MND): SUBRAPUBIC CATHETERS CAN IMPROVE QUALITY OF LIFE BUT ARE UNDERUTILIZED

Onders R^1,2

Elmo M¹

Ponsky L^1,2

Kaplan C¹

Katirji B^1,2

^boUniversity Hospitals Case Medical Center, Cleveland Ohio, USA

^bpCase Western Reserve University School of Medicine, Cleveland, Ohio, USA

Email address for correspondence: [email protected]

Keywords: urinary tract, suprapubic tract, diaphragm pacing

Background: Suprapubic catheters are standard therapy to treat neurogenic bladder in neuromuscular diseases such as spinal cord injury and multiple sclerosis. Urology literature also supports the use of indwelling catheters for immobility and end-of-life comfort. Review of the literature shows minimal information for use in ALS/MND. In other tetraplegics use of a suprapubic catheter in patients led to less urological complications and a better quality of life scores.

Objectives: Analyze the outcomes of ALS/MND patients who received suprapubic catheters.

Methods: Subgroup retrospective analysis of a prospective database at a single site of all patients who had suprapubic catheter placement was carried out.

Results: Six patients had suprapubic catheters placed with three patients having simultaneous diaphragm pacing (DP), suprapubic catheters and feeding tubes all placed at the same surgical encounter. Three are males. Average age is 56 with range of 35–69 years old. All were wheelchair bound requiring full lifts to transfer. There were no surgical complications. There has been no cross-contamination or infections between the surgical sites. There have been no significant long-term complications. One patient reports having had three urinary tract infections in 5 months. All patients report benefits of catheter outweigh the negatives. Benefits reported are increase in freedom and eases care. Patient’s primary caregivers also report same benefits of suprapubic. No patients have returned to their pre-suprapubic catheter regimen and all state they would have done this sooner.

Discussion: Indwelling suprapubic catheters have been used in other neuromuscular diseases as standard therapy. ALS/MND patients may not experience neurogenic bladders or as have as much spasticity, but they do have the same immobility issues. For patients who require lifts to transfer from the wheelchair to a commode makes leaving the house difficult. Physiologically, immobile women require significant more care. We have had patients who decrease their oral intake and allow themselves to be slightly dehydrated to avoid having to urinate. The use of suprapubic would obviate these difficulties. Having a surgical team which includes an urologist available to work together can help simplify bladder issues for patients by having several procedures done simultaneously. The major risk of a suprapubic catheter is infection and the catheter requires routine maintenance with monthly changes.

Conclusion: Suprapubic catheters are low-risk surgical procedures that can be done outpatient. For patients undergoing DP, the placement of a suprapubic is a short extension of surgical time. Benefits outweigh the risks and should be discussed in the appropriate clinical conditions.

Reference

• Bothing R, Hirschfeld S, Thietje R. Spinal Cord 2012;50:247–251.
   Google Scholar

P30 HOW MUCH IS THE OPTIMAL INITIAL DOSE OF MORPHINE FOR ALS PATIENTS?

Ogino Y^1,2

Miyakawa S²

Urano Y²

Kitamura E²

Uchino A²

Kaneko J²

Tominaga N²

Takahashi-Narita K³

Nagashima K²

Nagai M²

Ogino M²

^bqNational hokone hospital, Odawara/Kanagawa, Japan

^brKitasato university Department of Neurology, Sagamihara/Kanagawa, Japan

^bsKitasato university Department of Occupational Therapy, Sagamihara/Kanagawa, Japan

Email address for correspondence: [email protected]

Keywords: palliative care, morphine, initial dosage

Background: We started to use morphine to palliate dyspnea in ALS patients in 2005. At that time, morphine use was not approved by national health insurance in Japan. In September 2011, at last national health insurance gave permission to use morphine on ALS patients. Since then, more and more neurologists started to use morphine. However, administration protocol, optimal dosage, effectiveness and side effects in Japanese ALS patients are not yet clarified. Since morphine has been used on ALS more than 40 years ago, it is very difficult to find out good evidence for the optimal initial dose of morphine for ALS.

Objectives: To clarify administration protocol, optimal dosage, effectiveness and side effects in Japanese ALS patients.

Methods: Thirty-three ALS patients whom morphine was used to palliate dyspnea were analyzed. We used 2.5 mg (1.25 mg in case PaCO2 > 60 Torr) of morphine chloride PO or IV as an initial dose. Dosage was increased until relief of dyspnea was achieved (maximum dose 10 mg), and administration was repeated based on patients needs.

Results: Morphine was used in 33 ALS patients between December 2007 and April 2012 (age: 43–93 years, average disease duration 35.6 month). Although initial dose was 2.5 mg in 47%, 1.25 mg in 27%, and 5mg in 10% of patients, initial effective dose was 2.5 mg in 40%, 5 mg in 37%, and 1.25 mg in 13% of patients. Maintenance dose was 30–60 mg. Final dosage at the time of death was 5–230 mg (average 56.1 mg). Morphine was effective in palliation of dyspnea in all patients. PCO2 was not increased after morphine administration in 28 patients (85%) except for the patients whose remaining days were less than several days. Side effects were constipation (31 cases), sleepiness (2 cases), and drowsiness (2 cases). Respiratory distress was observed in only one case.

Discussion and conclusions: Morphine is very effective and can be used safely in Japanese ALS patients. An initial dose should be 1.25–2.5mg depend on PaCO2. Maintenance dose was much lower compared to cancer patients. Respiratory distress was observed in only one case. We are afraid that even now some Japanese neurologists are reluctant to use morphine. A survey conducted on physician treating ALS in 2012 showed that only 30% of Japanese neurologists use morphine on ALS. We assume the reasons are (1) fear to cause respiratory distress; (2) prejudice that morphine is only used for pain; and (3) not familiar with opioids use. Dyspnea is one of the most severe sufferings in ALS patients and at the moment morphine is the best choice. All ALS patients must be provided with proper palliative care. We must keep trying to make more Japanese neurologists accept the importance and usefulness of morphine on ALS patients and make a chance to learn about palliative care for ALS.

P31 VOICE BANKING AND VOICE RECONSTRUCTION FOR MND PATIENTS

Veaux C¹

Yamagishi J¹

King S¹

Colville S²

^btCSTR, Edinburgh, UK

^buAnne Rowling Clinic, Edinburgh, UK

^bvEuan MacDonald Centre, Edinburgh, UK

Email address for correspondence: [email protected]

Keywords: voice output communication aids, voice reconstruction, speech synthesis

When the speech of an individual becomes unintelligible due to a degenerative disease such as motor neurone disease (MND), a voice output communication aid (VOCA) can be used. To fully replace all functions of speech communication – communication of information, maintenance of social relationships and displaying identity – the voice must be intelligible, natural-sounding and retain the vocal identity of the speaker. Attempts have been made to capture the voice before it is lost, using a process known as voice banking. But, for patients with MND, the speech deterioration frequently coincides or quickly follows diagnosis.

Using model-based speech synthesis, it is now possible to retain the vocal identity of the patient with minimal data recordings and even deteriorating speech. The power of this approach is that it takes advantage of the speech already collected from several voice donors so that only the essential parts of one individual’s voice must be recorded.

Ideally, we record the patient’s voice early on in the disease and create a synthetic version of his voice for use if required at a later date when speech may become affected. However, if the patient’s voice has begun to deteriorate at the time of the recording, a voice repair technique can be applied in order to compensate for the disordered characteristics found in the patient’s speech.

We present here an ongoing project for voice banking and voice reconstruction based on this technology.

P32 FINDING IN THE INTELLIGIBILITY SCALE OF SPEECH RELATED TO MYOELASTIC-AERODYNAMIC MECHANISMS OF PATIENTS WITH MOTOR NEURONE DISEASES

Oda AL

Sierra HNM

Alves PCL

Vecina ALC

Brunoro ACV

Silva R

Stanich P

Oliveira ASB

Federal University Of SÃo Paulo, SÃo Paulo/SP, Brazil

Email address for correspondence: [email protected]

Keywords: speech intelligibility, fatigue, speech

Background: The loss of oral communication has been broadly argued as a strong indicator of a decrease in the quality of life in patients with MND. One of the factors that cause the change in the intelligibility of speech is the denervation of the tongue muscle and the limitation of expiratory flow.

Objective: To analyze the relation among the intelligibility of speech scale, the peak cough flow and tongue fatigability.

Methods: The assessment was carried with 37 patients, 24 (64.8%) of them had amyotrophic lateral sclerosis (ALS) and 13 (35.2%) had progressive bulbar paralysis (PBP). The speech therapist evaluation included tongue fatigability in lateral movements, tip elevation and back elevation after verbal commands, intelligibility scale of speech, and peak cough flow (PCF).

Results: The first (1) degree on the intelligibility scale refers to vocal communication and the tenth degree (10) to normal speech. Patients with ALS who showed 1 degree did not have any voluntary tongue movement and the average PCF was 93.3 l/minute (l/min); 2 degree ALS: average PCF of 115 l/min and tongue fatigue indication up to three isotonic movements; 5 degree ALS: average PCF of 145 l/min and fatigue after five movements; 7 degree (ALS): average PCF of 289 l/min and fatigue after 5–12 lateral movements, 3–8 movements of tip elevation, 5–18 movements of back elevation; 8–10 degree ALS: average PCF of 322 l/min fatigue after 4–20 lateral movements, 7–10 tip elevation movements and no fatigue after 20 back tongue elevation. Patients with PBP with 1 degree did not have any voluntary movement of the tongue and the average PCF was 50 l/min; 2 degree PBP: average PCF of 217 l/min and tongue fatigue that varied between absence of contraction and fatigue after three movements; 5 degree PBP: average PCF of 325 l/min and tongue fatigue after 3–8 lateral movements, four tip elevation movement and eight back elevation movements; 7 degree PBP: average PCF of 225 l/min and tongue fatigue after 5–20 lateral movements, 3–10 tip elevation movements and 15–16 back elevation movements.

Discussion: There is a relationship between aerodynamics and myoelastics mechanisms speech. To know and to respect the limits of muscle fatigability is one of the main challenges of rehabilitation work. The speech therapist should include therapeutics strategies of breathing and myofunctional therapy, with caution in prescribing the type and amount of exercises in patients with MND.

Conclusion: It was observed that the lower the speech intelligibility, the lower is the peak cough flow and the symptoms of fatigue will be evident sooner, showed by the reduction in the range of motion, the worsening of the quality of movement or respiratory distress associated with the task requested.

P33 A SURVEY OF POWER WHEELCHAIR USERS WITH ALS/MND; CHANGING NEEDS OVER TIME

Ward A

Carolinas Medical Center-Carolinas Healthcare System, Dept. of Neurology, Neuroscience and Spine Institute, Charlotte, NC, USA, Cabarrus College of Health Sciences, Concord, NC, USA

Email address for correspondence: amber.ward@carolinas healthcare.org

Keywords: power wheelchair, quality of life, survey

Background: Recent publication by Ward et al. in 2010 began the process toward developing “must haves” on a power wheelchair for persons with ALS/MND to meet their long-term needs. One other article exists which also looks at powered wheelchair use in ALS/MND from 2001. Rapid progression of the disease means the power wheelchair must be flexible for comfort and function.

Objectives: To survey persons with ALS/MND at 1 month and 6 months after receiving power wheelchairs to determine what is required for long-term effective use, comfort and function.

Methods: Thirty-three-question survey and Psychosocial Impact of Assistive Devices Scale (PIADS) was sent 1 month after getting a new power wheelchair, follow-up survey sent at 6 months. The survey addressed satisfaction, feature use, comfort and function with the power wheelchairs.

Results: Based on satisfaction and feature use survey results, at 1 month, 56% of users are performing tilt, recline and elevating legs at least five times a day, and at 6 months 50% are performing these tasks. For users at 1 month, 72% are still pleased with their choice of cushion, headrest and power features, and this percentage drops only slightly at 6 months. Their quality of life increased and pain decreased at 1 month and 6 months. According to the PIADS, the power wheelchair gave users increased ability to participate and sense of competence.

Discussion: Power wheelchair use is a stage most people with ALS go through during the disease process, and it is important that Physicians, Nurses and Allied Health Clinicians are aware of the need for long-term flexibility in the power wheelchairs, as well as what users of these chairs say about their function and comfort requirements.

Conclusion: This survey has important results for the ALS/MND population, and is the first of its kind to assess power wheelchair users at 1 month and 6 months after getting their chairs. Our results demonstrate the usage, psychosocial issues, function and quality of life impact that the power wheelchair has on the life of someone with ALS/MND.

References

• Trail M, Nelson N, Van JN et al. Arch Phys Med Rehabil 2001;82:98–102.
   PubMed Web of Science ®Google Scholar
• Ward AL, Sanjak M, Duffy K et al. Arch Phys Med Rehab 2010;91:268–272.
   PubMed Web of Science ®Google Scholar
• Bromburg MD, Brownell AA, Forshew DA et al. A L S 2010;11(1–2):110–115.
   Google Scholar

P34 EYE-TRACKING COMPUTER SYSTEM UTILIZATION BY PATIENTS WITH ADVANCED AMYOTROPHIC LATERAL SCLEROSIS

Spataro R¹

Manno C¹

Ciriacono M²

La Bella V¹

^bwALS Clinical Research Center, Palermo, Italy

^bxDept Phys Med Rehabil, Local Health Unit, Palermo, Italy

Email address for correspondence: [email protected]

Keywords: eye-tracking system, locked-in, alternative augmentative communication

Background: Most ALS patients in the advanced stages of the disease become locked-in (LIS). This is a severe clinical condition characterized by quadriplegia and anarthria, with preservation of consciousness and eye movements. Taking advantage of preserved eye blinking and movements, LIS patients can now use an eye-tracking computer system (ETCS) as an interface for augmentative/alternative communication (AAC). ETCS allows an easy access to communication, internet navigation, webmail, social networks and even domotics. A few studies have suggested that the device has a high rate of acceptance in ALS, with a positive impact on quality-of-life.

Objectives: We performed a telephone interview to the ALS LIS patients, living in Sicily, which had received an ECTS device between January 2011 and June 2012. We explored the clinical and demographic variables that are likely to affect its utilization, the level of acceptance and the impact of this device on the patient's ability to be in contact with the outer world.

Methods: Twenty-seven consecutive non-demented ALS patients at LIS stage were enrolled. Mean age at interview was 55.1 + 11.6 years, with a median education of 13 years (8–13). Among ALS patients, onset was spinal in 23 and bulbar in four, with an M/F ratio of two. All patients were previously trained for ETCS use. A telephone interview was made to each patient. Answers were provided in a subsequent phone call with the help of the caregiver. The interview included items about demographic and clinical variables, and factors affecting the daily ETCS utilization.

Results: At the time of interview, patients were with the ETCS device for a mean period of 15.8 + 11 months, with a daily utilization of 359 + 266 minutes. ETCS users were young ALS patients and with high education when compared to the whole population of ALS. Patients mostly used ETCS to communicate with caregiver/relatives and to navigate social networks/internet. 33.6% of LIS patients (all were ALS) had a rather low daily utilization of the ETCS device (ie less than two hours); most frequent reported causes were early eye gaze, fatigue and oculomotor dysfunction.

Discussion and conclusion: ETCS is a useful AAC device and most LIS patients can use it with a good performance for a wide range of purposes, including internet navigation. However, our study suggests that the device is mostly requested, and fruitfully used, by relatively young patients with a rather high education. The development of oculomotor impairment may limit the ETCS correct use.

References

• Ball LJ et al. J Med Speech Language Pathol 2010; 18:11–23.
   Web of Science ®Google Scholar
• Sarma R et al. Arch Neurol 2011;68:857–861.
   Google Scholar

P35 SATISFACTION OF ALS CLIENTS AND CAREGIVERS WITH TRAINING ON USE OF AN EYE-GAZE SYSTEM IN A FACILITY SETTING

Cham E

Poirier B

Vancouver Coastal Health: GF Strong Rehabilitation Centre - ALS Centre, Vancouver, Canada

Email address for correspondence: [email protected]

Keywords: eye tracking system, communication, satisfaction

Background: Due to the progressive nature of ALS, some clients eventually reach a locked-in state making communication a major challenge. This is particularly true for clients who choose invasive ventilation as an intervention. Eye-gaze is often the only available access method to high-tech AAC for this population. The Vancouver Coastal Health ALS Centre OT and SLP have been offering support and training to clients, caregivers and families on how to use eye-gaze systems.

Objective: To evaluate satisfaction levels of clients, families and healthcare providers with training and use of an eye-gaze system in a facility setting.

Method: ERICA eye-gaze systems were allocated to three ventilator dependent ALS clients living in one care facility over a 4 year period. Training sessions were provided to clients, facility staff and caregivers. Limited technical support was offered over the phone and by email. A satisfaction questionnaire, including 7–8 items on a 5-point Likert Scale and five open-ended questions, was distributed to clients and caregivers after receiving the device. Five semi-directed interviews were conducted, and data were obtained from three clients, 15 healthcare providers and two family members. Results from the questionnaires were collated and descriptively analyzed. Interviews were recorded, transcribed and qualitatively analyzed.

Results: ALS clients, their families, and the healthcare providers generally expressed a high level of satisfaction with the use of the eye-gaze system; higher in those with more family support and reported higher motivation to use the internet. Clients with limited family support and limited computer skills were more dependent on facility staff for set up and opportunities to use the device. ALS clients expressed a higher satisfaction with the eye-gaze training provided compared to the caregivers.

Discussion: While the sample size was small, ALS clients and their caregivers identified the need for more caregiver training to increase proficiency with the eye-gaze system. The greater satisfaction reported by clients with more family support, may be due to increased opportunities for practice. Barriers to successful implementation of eye-gaze use in a facility setting may include staff accessibility and turnover, limited family participation, and eye-gaze software difficulties and set up concerns.

Conclusion: Caregiver comfort with technology and sufficient training on device set-up are keys to successful eye-gaze use for ALS clients. These factors appear to be essential in maintaining an enhanced quality of life for the users. Future plans could include comparing competitor eye-gaze system use, and comparing caregiver satisfaction ratings between facility, home and acute care settings.

Acknowledgements

We thank the following for their support: ALS Clients, ALS Society of British Columbia, Chris Speropoulos and Doug Gayton: ATSS at G.F. Strong Rehab Centre and the Facility staff at Carelife Fleetwood - Maple Unit.

P36 VIDEOENDOSCOPIC EVALUATION OF SWALLOWING (VEES) IN PATIENTS WITH MOTOR NEURON DISEASE/AMYOTROPHIC LATERAL SCLEROSIS

Schweikert K^1,2

Wilmes S¹

Dunkel N³

Weber M⁴

Schlaegel W¹

^byREHAB Basel, Basel, Switzerland

^bzNeuromuscular Center University Hospital Basel, Basel, Switzerland

^caDepartment of Speech and Language Pathology REHAB BAsel, Basel, Switzerland

^cbMuscular Disease Unit/ALS Clinic, St. Gallen, Switzerland

Email address for correspondence: [email protected]

Keywords: videoendoscopic evaluation of swallowing, dysphagia, deglutition

Background: Dysphagia is a common, serious symptom in motor neuron disease (MND). In primary lateral sclerosis (PLS), degeneration of upper motor neurons can result in pseudo-bulbar paralysis. In amyotrophic lateral sclerosis (ALS) also lower motor neurons in the brainstem are affected: at disease onset in about 30%, in later stages in nearly all patients. Monitoring deglutition is crucial to avoid malnutrition, weight loss, aspiration, and to timely implement diet changes, adaptive strategies, or tube feeding. Videoendoscopic examination of swallowing (VFES) noninvasively visualizes pharyngeal dysphagia and effectiveness of compensatory maneuvers (1).

Objectives: To retrospectively report on VFES and therapeutic consequences in consecutive MND patients.

Methods: Prior to VEES, a speech and language therapist examined each patient clinically and documented the current diet by the functional oral intake scale (FOIS) (2). VEES was indicated to evaluate whether deglutition was safe, swallowing strategies and diet appropriate. For assessment of phyaryngeal swallowing, we used the Penetration Aspiration Scale (PAS) (3) and noted if residues were present.

Results: Between April 2007 and March 2013, 45 consecutive MND patients underwent VEES. There were 31 men and 14 women (mean age: 66 and 64 years, range: 31–85 years); three men and one women were diagnosed PLS. ALS was characterized by bulbar-, upper-, and lower-limb-onset in 10, 13, and 18 patients, respectively. Mean disease duration at first VEES was 4 years (range: 1–17 years). Nine patients had a gastric tube (PEG). Eleven patients needed multiple VEES evaluations because of disease progression. In total 64 VEES were performed; only one was stopped due to excessive choking. Pharyngeal deglutition was preserved in most assessments, but in 23 of those 49 VESS with PAS 1, relevant residues were detected. After VEES, we recommended modification of diet and insertion of PEG in 19 and 9 patients, respectively. According to 39 VEES examinations, adequate feeding and swallowing strategies were demonstrated and patients as well as their carers could be reassured.

Discussion and conclusion: VEES is a non-invasive, well-tolerated method to monitor deglutition and security of nutrition in all stages of MND. As it provides relevant information for the management of dysphagia, nutritional surveillance, dietary counselling, timing of enteral nutrition, and can improve insight of patients and carers in swallowing problems, it should become an integral part of diagnosis in multiprofessional ALS centers.

References

• Leder SB, Novella S, Patwa H. Dysphagia. 2004;19(3): 177–81.
   Google Scholar
• Crary MA, Mann GD, Groher ME. Arch Phys Med Rehabil. 2005;86(8):1516–20.
   Google Scholar
• Rosenbek JC, Robbins JA, Roecker EB et al. Dysphagia. 1996;11(2):93–8.
   Google Scholar

P37 PALATAL PLATE IMPLANTATION FOR THE TREATMENT OF DYSARTHRIA AND SEVERE HYPERNASALITY IN ALS PATIENTS

Pasian V¹

Piumetto E¹

Ceruti P²

Moglia C¹

Calvo A¹

Gassino G²

Chiò A¹

^ccALS Center, Department of Neuroscience, Torino, Italy

^cdDental School, Torino, Italy

Email address for correspondence: [email protected]

Keywords: ALS, palatal plate, dysarthria

Background: Dysarthria in ALS is typically characterized by muscle weakness and hyposthenia of buccofacial districts, causing inaccurate production of phonemes sometimes associated to dysphonia and hypernasality.

Objectives: To evaluate if a palatal plate, stiffing the soft palate, can improve dysarthria and severe hypernasality of a patient with bulbar ALS, increasing the intelligibility of speech.

Case report: A 60-year old woman with a one-year history of bulbar onset ALS was referred for a phoniatrics assessment. She had ALS-FRS-R score of 41, with a speech score of 2. Clinical and instrumental examination showed signs of mild dysarthria, severe hypomobility of velum palatinum, reduced strength and precision of exercises of tongue, mouth and face, normotrophic tongue, absence of dysphagia. She underwent rhinomanometry and acoustic analysis of voice. The patient was adapted to a palatal plate on measure after dental impression; this device has a retainer for removable prostheses located in the dorso-palatal area of the mouth. The acrylic plates elevate the soft palate and reduce palatopharyngeal incompetency. The patient was advised to use the plate during all hours of the day except during meal times. After a period of adjustment to the palatal plate of about 15 days the patient underwent a clinical and instrumental re-evaluation.

Results: Qualitative analysis by the patient and by the specialists has found an improvement of intelligibility of speech and a reduction of hypernasality. These findings were supported by objective evaluations carried out with rhinomanometry and acoustic analysis of the voice. In particular, the rhinomanometry showed a marked reduction of nasal airflow and increased nasal resistance. Acoustic analysis of the voice-identified reduction of the parameters relating to voice turbulence index; the spectrographic analysis showed a reduction of background noise and the more obvious differences between minimal pairs.

Discussion and conclusion: We have shown that in selected cases of ALS patients with mild dysarthria associated with hypernasality, the speech intelligibility can be improved by the use of specific palatal prosthesis, greatly raising the quality of life of the patient.

P38 ONE-HANDED FEEDING TUBE ASSIST DEVICE FOR ALS CLIENTS WITH DECREASED HAND FUNCTION

Beggs K

Cham E

Vancouver Coastal Health; G.F. Strong Rehabilitation Centre-ALS Centre, Vancouver, British Columbia, Canada

Email address for correspondence: [email protected]

Keywords: tube feeding, nutrition, hand function

Background: Many ALS clients have feeding tubes placed to supplement their nutrition and hydration. In 2012–2013, Vancouver Coastal Health ALS Centre saw 87 clients with feeding tubes representing 22% of the provincial caseload. Prior to tube placement, the ALS Centre Occupational Therapist (OT) assesses clients’ hand function to use a syringe and open a feeding tube end. The ALS Centre Dietician (RD) provides tube feeding education and supplies to support clients at home. A client must be able to flush the tube with water to maintain a minimum standard of tube care. Independence is compromised when clients have hand weakness. Not all clients in the province of British Columbia have access to home support workers who are trained to assist with tube feeding due to risk management concerns, and a reluctance for home support agencies to institute a delegation of task (DOT) for tube feeding assistance. An alternative solution was required to support ALS clients with limited hand function to be independent in basic tube feeding activity.

Objective: To develop a device to assist clients with decreased hand function to independently manage their tube feeds at home.

Method: In 2012, Orthotists at G. F. Strong Rehabilitation Centre developed a tube feeding stand to assist a stroke client who only had the use of one hand. The device allowed this client to be independent and he was discharged home rather than requiring facility placement. The device consisted of a board which holds a bowl of water to allow for flushing the tube, a custom bracket to help draw water with the syringe, a stand to hold the feeding tube end, and a mount to stabilize the syringe and plunge water into the feeding tube.

Results: Four ALS clients have since trialled the device with varying degrees of success. Three clients/caregivers had insufficient hand dexterity to operate the system. One client is presently using the device. In May 2013, the ALS Society of British Columbia provided funding to have an ambidextrous device made. The device can now be loaned out to clients for the time period they have adequate hand function to independently administer their tube feedings.

Conclusion: This unique device allows ALS clients to independently optimize their nutrition via their feeding tubes. Future considerations may include developing specific criteria to predict which ALS clients could benefit from using the device, and modifying the system so it can be fixed to a table for seated or standing use.

Acknowledgements

We thank the following for their support: ALS Clients, ALS society of British Columbia, Patrick O’Brien and Jeff MacDonald-Bain, Orthotists at G.F. Strong Rehab Centre, Acquired Brain Injury Team at G.F. Strong Rehab Centre.