P128 RESTLESS LEGS SYNDROME IN AMYOTROPHIC LATERAL SCLEROSIS
PRALINE J1,2, LIMOUSIN N1, ARNULF I3, BLASCO H4, CORCIA P1,2
1Centre SLA, Service de Neurologie et de Neurophysiologie Clinique, CHRU, Tours, France, 2INSERM UMR 930, Université François-Rabelais, Tours, France, 3Unité des Pathologies du Sommeil and UMR 975, Université Paris 6, Hôpital Pitié- Salpêtrière, Assistance Publique des Hôpitaux de Paris, Paris, France, 4Laboratoire de biochimie et biologie moléculaire, CHRU, Tours, France
E-mail address for correspondence: [email protected]
Keywords: restless legs syndrome, sleep disturbance
Background: The restless legs syndrome (RLS) is a sensory-motor disorder severely affecting 2–3% of adults in western populations. It seems to be more prevalent than in the general population in specific neurodegenerative disorders including Parkinson's disease, Huntington's disease, spinocerebellar atrophy, and hereditary spastic paraparesis. No such prevalence data exists for RLS in ALS patients. If present, RLS could alter the quality of life in patients with ALS, and could be treated. Furthermore, ALS leads to complete immobility of the legs, a condition that can possibly exacerbate a pre-existing mild RLS. Consequently, it seems to be important to search for RLS in ALS patients as this symptom could be improved by treatment.
Objectives: To determine the prevalence and determinants of the association of RLS and ALS.
Methods: Consecutive, unselected ALS patients were recruited in our ALS centre. Information on sex, age of inclusion, age of onset, body mass index, duration of the disease, site of onset, ALS Functional Rating Scale Revised, use of percutaneous endoscopic gastrostomy tube, non invasive ventilation and tracheotomy was obtained. Each patient underwent a sleep interview. RLS was diagnosed using the IRLSSG criteria. Investigations looking for symptomatic RLS including iron and ferritin plasma levels were performed.
Results: A total of 69 subjects (69.6±9.7 years) were included. RLS was found in thirteen patients (18.8%). Most patients (92%) were assigned to the group of moderate or severe. Only 4 patients had previously been diagnosed as having RLS and were on medication. When excluding causes of symptomatic RLS, including iron deficiency (n =1), sensory neuropathy (n =1) and drug adverse effects (n =3), 8 of 69 (11.6%) patients had a RLS without other causes. Patients with RLS had a worse score to the question “Has your ability to turn in bed and adjust the bed clothes changed?” than patients without it (P = 0.0004). No other significant difference about ALS features was found between these two groups.
Discussion: Our population survey was the first study about RLS prevalence in ALS patients and revealed a surprisingly high proportion of RLS. Progressive dysfunction of spinal axons is well known in ALS and could explain RLS. We discuss possible bias, especially the overlap between RLS and pain induced by immobilization.
Conclusion: RLS seems to be particularly frequent in ALS patients. An interview targeting RLS criteria should be performed in ALS patients with suggesting any sensory discomfort. The efficiency of dopaminergic agonists should be evaluated in ALS patients with coexisting RLS.
P129 ITALIAN VALIDATION OF AMYOTROPHIC LATERAL SCLEROSIS SPECIFIC QUALITY OF LIFE – REVISED (ALSSQOL-R): A COMPARISON BETWEEN U.S. AND ITALIAN SAMPLES
PAGNINI F1,2, SIMMONS Z4, FELGOISE S5, LUNETTA C3, CORBO M3
1Department of Psychology, Catholic University of Milan, Milan, Italy, 2Ph.D Program in Clinical Psychology, University o Bergamo, Bergamo, Italy, 3NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Ospedale Niguarda Cà Granda, Milan, Italy, 4Penn State University College of Medicine, Hershey, United States, 5Philadelphia College of Osteopathic Medicine, Philadelphia, United States
E-mail address for correspondence: [email protected]
Keywords: quality of life, psychological assessment, cross-cultural comparison
Background: There is no consensus on how to measure quality of life (QOL) in amyotrophic lateral sclerosis (ALS). Most QOL instruments are heavily weighted toward strength and physical function, and will decline as ALS progresses. The ALS Specific QOL-Revised (ALSSQOL-R) questionnaire has been validated on 389 English-speaking ALS patients, but has not been used outside of the US. It has 6 subscales: 1) Negative Emotion; 2) Interaction with People and the Environment; 3) Intimacy; 4) Religiosity; 5) Physical Symptoms; 6) Bulbar Function.
Objective: 1) To determine whether QOL scores on the ALSSQOL-R differ in an Italian sample from norms originated on English-speaking US residents; 2) To determine if the 6 ALSSQOL-R factors demonstrate internal consistency and predict global QOL in the Italian population, as they do in English-speaking US residents.
Methods: One hundred and fifty ALS patients were recruited at NEuroMuscular Omnicentre in Milan and completed the Italian version of the ALSSQOL-R. Scores were compared to those from a US sample. Independent T-tests were used to compare subscale scores of US and Italian cohorts. Principal components analysis and Cronbach's alpha were used to measure factorial structure and internal consistency.
Results: The two samples were comparable with respect to age (Italian mean: 61.04 years; US mean: 60.5 years), gender (Italian: 40% female, US: 39% female), ALSFRS-R (Italian mean: 33.4; US: 33.11), employment status, and educational level. US scores were significantly higher in ALSSQOL-R total score (P < 0.001) and in the subscales of Interaction (P <0.001), Intimacy (P < 0.001), Physical Symptoms (P < 0.01) and Bulbar Function (P< 0.05). There were no significant differences for Negative Emotion and Religiosity. The McGill QOL Single Item Score (MQOL-SIS) was significantly higher (P< 0.05) in the US sample. Principal components analysis with six factors explained 78.9% of the variance. All sub-scales demonstrated good internal consistency, with high Cronbach's Alpha: Negative Emotions (0.80), Interaction (0.81), Intimacy (0.70), Religiosity (0.84), Physical Symptoms (0.51) and Bulbar Function (0.84). ALSSQOL-R total score was positively correlated with MQOL-SIS (P<0.001).
Discussion and conclusions: The US sample demonstrated higher overall QOL scores and higher subscores in 4 of 6 categories than the Italian sample, despite comparable demographics and functional measures. Factor analysis and measures of internal consistency indicate that the Italian version of the ALSSQOL-R has good psychometric properties, and appears to be a valid self-assessment screening tool in ALS care. The differences in normative values for some scales between the Italian ALS population and those in English-speaking US residents emphasises the need to independently test this instrument in ALS patients from different cultural backgrounds.
P130 CASE MANAGEMENT IN ALS: A NEXT STEP TOWARDS EXCELLENT CARE FOR PEOPLE WITH ALS?
CREEMERS H1, VELDINK JH2, GRUPSTRA H1, NOLLET F1, VAN DEN BERG LH2, BEELEN A1
1ALS Center The Netherlands/AMC, Amsterdam, Netherlands, 2ALS Center The Netherlands/UMC Utrecht, Utrecht, Netherlands
E-mail address for correspondence: [email protected]
Keywords: case management, quality of life, needs assessment
Background: Studies have shown that the multidisciplinary ALS care is not always optimal, resulting in perceived problems by ALS patients, family and professional caregivers. The concept of case management has been suggested as an innovative strategy to optimize complex palliative care. We therefore wanted to investigate the potential optimizing effect of a case manager on quality of life of patients with ALS and the caregiver's burden by performing a cluster randomized trial. The case manager assesses the changing complex needs and preferences of the ALS-patients and their caregivers and arranges, in consultation and collaboration with the ALS care team, additional care and services.
Objectives: To report on the issues faced by ALS patients and their informal caregivers for which they consulted the case manager, and the related services and support provided by the case manager.
Methods: The randomized controlled trial included 132 ALS patients and their caregiver who received treatment from ALS care teams at 31 different institutions from all over the country. 67 patients received case management in addition to usual care (intervention group).
The case manager visited the participants at the start of the study and every three months at home. During these home visits participants were invited to share experiences, ask questions or raise issues that were problematic for them or they wished to be informed about.
The starting point was the questions of the participants. Between the home visits support from the case manager was provided via e-mail and telephone.
Results: Participants differed in their need for information, support, treatment, resources and care. Assessment and review of problems faced by ALS-patients and their caregivers regarding symptoms of ALS rarely resulted in actions of the case manager as treatment options provided by the ALS team were adequately considered and participants were on average satisfied with the approach of the ALS team.
Actions of the case manager were specifically in the area of emotional well-being by providing a listening ear for participants, discussing the impact of having ALS and the impact of symptoms on daily functioning. Other ALS-related issues were also raised frequently, eg the provision of assistive products and technology, advance directives, supporting children, respite care, power of attorney.
The number of services and support provided by case management is partly determined by the personal situation of the ALS patient, the stage of the disease, the progression of weakness, the support from the environment and the satisfaction with the offered care and treatment.
Conclusions: Comprehensive case management in ALS may optimize the complex care by providing emotional and practical support to people with ALS and their caregivers. Case management is highly intensive individualized care and starts with identifying cases in need of intensified management.
P131 PATIENT CARE COORDINATION AT CAROLINAS NEUROMUSCULAR/ALS – MDA CENTER: ALS CLINIC STAFF WEEKLY MEETING ACTION DOMAINS
BLACK KJ, WILLIAMS NM
Carolinas Medical Center – Department of Neurology, Charlotte, NC, United States
E-mail address for correspondence: [email protected]
Keywords: ALS team communication, quality improvement, audit
Background: What is important in care coordination within multidisciplinary teams has not been fully studied. Delivery of services requires organization of patient care together with sufficient and timely information. Patients require access and navigation through the healthcare system and there has to be a needs assessment with communication of needs to key personnel (1,2).
Objective: Identify ALS clinic staff weekly meeting action domains discussed during patient care coordination of a large ALS Clinic in the Department of Neurology situated in the third largest public healthcare system in the United States – Carolinas Healthcare System.
Methods: Quarterly audits of ALS Clinic Staff Weekly meetings were performed. Action domains were based on patient needs and problem solving.
Results: New patients were reviewed weekly and 10% of the patients referred for the diagnosis of ALS had other diagnoses. Patient care reviews included 4–13 weekly with additional 0–2 hospitalized or nursing home patient discussions weekly together with 0–3 hospice referral patient discussions weekly. Home visits (0–13 weekly) were reviewed. Safety measures were reviewed weekly including in-clinic falls which occurred in 4 patients annually. All ALS deaths were reviewed (8–18 annually). ALS Care Fund dispersals were reviewed quarterly. Chemotherapy, gastrostomy tube, ventilator and wound (decubitus, tracheostomy, etc) status were discussed at initiation and then at change of status in each patient. Advanced directives together with altered mental status were discussed at each post-multidisciplinary clinic conference monthly. Research study and clinical trial patients (9–17 weekly) were discussed for 5 clinical studies and 6 clinical trials.
Conclusions: Action domains: newly diagnosed (8.5%), mis-diagnosed (0.7%), follow-up non-hospitalized (42.4%), hospitalized (5.2%), hospice (5.5%), home visit (34.6%) and deceased (3.0%) patients, were systematically reviewed weekly by the ALS Clinic Staff to identify new problems and outline implementation measures. ALS Clinic Staff weekly meetings provide the venue for improving quality of care (3) and improving communication with innovative techniques (4).
Supported by: Carolinas ALS Endowment, Pinstripes Foundation, Carolinas Healthcare Foundation, Muscular Dystrophy Association/ALS Division.
References:
- Walsh J, Young JM, Harrison JD, et al. Eur J Cancer Care (Engl) 2010 (Epub ahead of print)
- Walsh JM, Harrison JD, Young JM, et al. BMC Health Serv Res. 2010;10(1):132.
- Kraynack NC, McBride JT. Semin Respir Crit Care Med. 2009;30(5):547–58.
- Albisser AM, Alejandro R, Sperlich M, Ricordi C. J. Diabetes Sci Technol. 2009;3(3):619–23.
P132 PATIENT CARE COORDINATION AT CAROLINAS NEUROMUSCULAR/ALS – MDA CENTER: IN- HOME SERVICES – HOME CARE COORDINATION
NICHOLS MS1, WILLIAMS NM1, BLACK KJ1, WRIGHT KA1, LANGFORD V1, DUFFY KS1, SANJAK MS1, WARD A1, O'NEIL M1, WALGREN K1, OPLINGER H1, BOCKENEK WL1,3, BRAVVER E1,2, BROOKS BR1,2
1Carolinas Medical Center – Department of Neurology, Charlotte, North Carolina, United States, 2University of North Carolina-Chapel Hill School of Medicine Department of Neurology, Chapel Hill, North Carolina, United States, 3Carolinas Rehabilitation, Charlotte, North Carolina, United States
E-mail address for correspondence: [email protected]
Keywords: home care, in-home services, altered mental status
Background: In-Home Services provide an extension of ALS care to specific patients who require additional attention between ALS multidisciplinary clinic visits. Some visits when supported by patients’ insurance are provided through private home health services. When other visits are needed, help may be provided by patient service organizations such as the ALS Association and Muscular Dystrophy Association and local sources such as the Joe Martin Foundation. Only a limited number of ALS Clinics have a dedicated division providing in-home services.
Objective: Identify baseline activities provided by the in-home services division of a large ALS Clinic in the Department of Neurology situated in the third largest public healthcare system in the United States – Carolinas Healthcare System.
Methods: Home visits were audited quarterly and new problems identified by home visits categorized. Summary data was analyzed with descriptive statistics.
Results: Total annual contacts for one RN were 296 (152 males; 144 females). Home visits (97.3%) were the major contact while nursing home visits (2.7%) were less frequent. New problems (61.1%) were identified in home visits. The most common new problems (16.8%) were speech-augmented communication placement (8.5%) and swallowing-attention to gastrostomy tube (8.3%). The second most common problems (15.1%) were physical therapy and occupational therapy problems including falls (3.0%), new durable medical equipment (9.7%) and decubitus ulcers (4.4%). Next were respiratory problems (13.1%) comprised of drooling-hypersiallorhea (4.4%), ventilator issues (4.4%) and pneumonia-bronchitis (4.3%). Administrative problems (8.4%) included patients who were new to or missed recent clinics (5.6%) and benefit or hospice issues (2.8%). Altered mental status (5.7%) required nurse visits to identify the next course of action.
Conclusions: Three of five in-home service encounters by a dedicated in-home services division of an ALS Clinic identified new problems providing information that led to hospitalization (17.7%) with (5.2%) and without mortality. This audit identified that in-home service encounters comprise approximately 40% of the total combined intake clinic, multidisciplinary clinic, ventilator clinic and home visit encounters. This audit provides benchmark criteria for expanding in-home services when appropriate resources are available.
Supported by: Carolinas ALS Endowment, Pinstripes Foundation, Carolinas Healthcare Foundation, Muscular Dystrophy Association/ALS Division.
References:
- Hoffman JJ. Home Healthcare Nurse. 2008;26(6):337–42.
- Bottino-Bravo P, Thomson J. Home Healthcare Nurse. 2008;26(4):244–50.
- Cline D. Home Healthcare Nurse. 2006;24(1):54–7.
- Krivickas LS, Shockley L, Mitsumoto H. Journal of the Neurological Sciences 1997;152 Suppl 1:S82–S89.
P133 THE ‘PREFERRED PRIORITIES FOR CARE’ DOCUMENT IN AMYOTROPHIC LATERAL SCLEROSIS: VIEWS OF BEREAVED RELATIVES AND CARERS
PRESTON H1, COHEN-FINEBURG I2, CALLAGHER P1, MITCHELL JD1
1Preston MND Care and Research Centre, Royal Preston Hospital, Preston, Lancashire, United Kingdom, 2International Observatory on End of Life Care, University of Lancaster, Lancaster, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: Preferred Priorities for Care, end-of-life, advanced care planning
Background: Increasing emphasis is being placed on the need for advanced care planning (ACP) at the end-of-life. The Preferred Priorities for Care document (PPC), formerly titled the Preferred Place of Care document is a patient-held record, promoted by the UK Department of Health's End of Life Strategy introduced in 2007. As an ACP tool, it aims to promote discussion and communication of wishes and preferences amongst patients, family and health care providers at the end-of-life. Because the majority of patients with Amyotrophic Lateral Sclerosis (ALS) lose verbal communication yet retain the capacity to make decisions, early discussion of wishes, a central aspect of ACP, is particularly important. The congruence between actual and preferred place of death is becoming increasingly recognised as an outcome in evaluating the quality of end-of-life care and PPC is anticipated to help patients achieve their preferred place of death. However there has been little research evaluating PPC's effectiveness, or exploring user views, particularly in non-malignant disease.
Objectives: This study aimed to look qualitatively at ALS patients’ bereaved relatives’ experiences of using the PPC document and their perceptions about its impact on end-of-life care.
Methods: The research was conducted in consultation with the International Observatory on End of Life Care at Lancaster University. Participants were bereaved relatives or primary carers of patients with ALS known to the Preston MND Care and Research Centre in the North West of England, as identified by its database. Semi-structured face-to-face interviews were conducted at the participant's home and thematic analysis was used to analyse the written interview data.
Results: Eleven participants were interviewed and were mostly over 65 years, male, British White and living with the patient. Four main themes specific to the PPC document were identified: I) completion, II) document availability to others, III) importance and influence on end-of-life experience and IV) limitations.
Discussion: Completing the PPC was generally viewed as a positive experience for both participant and patient, affording peace of mind and a sense of control. It appeared less important as a communication aid between patients and participants, as a result of discussions held prior to its completion, than in raising awareness of patient preferences amongst Health Care Professionals (HCP's). Lack of awareness of the PPC amongst HCP's was identified by participants as the most important area for improving effectiveness of the document.
Conclusions: Participants felt the PPC to be an important document although its influence on the end-of-life experience was less clear. Importantly a perceived lack of awareness of the PPC document amongst HCP's has been highlighted. The implications for practice include looking at levels of and ways to improve awareness, particularly in light of the increasing pressure to achieve patient preferences at the end of life.
P134 THE MAPPA PROJECT: A LONGITUDINAL STUDY ON PSYCHOLOGICAL WELL-BEING OF ALS PATIENTS AND THEIR CAREGIVERS
PAGNINI F1,2, LUNETTA C3, ROSSI G3, BANFI P3, GORNI K3, CELLOTTO N3, MARCONI A3, FOSSATI F3, CASTELNUOVO G1, CORBO M3, MOLINARI E1
1Department of Psychology, Catholic University of Milan, Milan, Italy, 2Ph.D Program in Clinical Psychology, University of Bergamo, Bergamo, Italy, 3NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Ospedale Niguarda Cà Granda, Milan, Italy
E-mail address for correspondence: [email protected]
Keywords: quality of life, longitudinal study, caregivers
Background: Quality of Life (QOL) and psychological well-being in ALS are an important clinical issue, for both patients and caregivers. These psychological variables are widely considered in cross sectional studies, but few works have investigated their evolution in longitudinal research.
Objective: We aim to evaluate how QOL, depression and anxiety change, during a period of one year, in ALS patients and their caregivers.
Methods: We conducted a repeated-measures study. Forty ALS patients, together with their caregivers, were assessed every 4 months (baseline, T1, T2 and T3). All subjects completed Beck Depression Inventory (BDI), McGill QOL Single-Item Scale (MG-SIS), State-Trait Anxiety Inventory (STAI) and caregivers also filled out the Zarit Burden Inventory (ZBI).
Results: We found a tendency of worsening in all analyzed constructs. Patient's MG-SIS gradually decreased over time, while BDI and STAI scores increased, with a statistical difference between every step's value, compared to previous assessment (T-test for related samples, P from <0.05 to <0.001). Caregivers also indicated a worsening of all psychological variables with a decrease of MGSIS and an increase of BDI, STAI and ZBI. Changes between times were higher for caregivers that for patients in all considered constructs.
Discussion: Psychological well-being of ALS patients and their caregivers gets worst over time. Considering the progressive nature of the disease, it seems that the advancement of physical impairment has a negative effect on QOL and promotes anxiety and depression in patients and caregivers, with an increase of burden for carers. Caregivers, in particular, get psychologically worse with disease progression. It is therefore important to offer psychological (sometimes psychiatric) support to both patients and carers, in order to reduce or contain the illness’ effects on their psychological well-being.
Conclusion: The longitudinal design of our study indicates a worsening of psychological well-being indexes during time. This is true for ALS patients and even more for their caregivers that presented a greater worsening of psychological disease and a lowering of their QOL, compared to patients.
P135 SUPPORT SERVICES FOR LAY CARERS: VIEWS OF CURRENT AND PAST FAMILY CAREGIVERS OF PEOPLE WITH MND
O'BRIEN M1, WHITEHEAD B1,2, MITCHELL D1, JACK B1
1Edge Hill University, Ormskirk, United Kingdom, 2Lancashire Teaching Hospitals NHS Foundation Trust, Preston, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: family carers, support, qualitative research
Background: Family caregivers play an important role in caring for people with MND. Little is known about the experiences of such family caregivers and the levels of support available to them or accessed by them. By developing an understanding of the concepts related to caring under these circumstances it should better enable us to meet the support needs of carers.
Objectives: To explore, from a qualitative perspective, the views of current and past carers of people with MND regarding their need for and use of support services.
Methods: Narrative interviews were carried out with a purposively selected sample of current (n=18) and past (n = 10) carers of people with MND. Thematic analysis was aided by NVIVO 7.
Results: Consensus amongst participants was apparent in the themes identified within the data. Being a carer was described as extremely draining, both physically and emotionally. Despite the intense pressure and strain on carers, and the belief that respite care would make a difference to them and the person with MND, participants reported that such support was often not readily available.
Carers who had successfully accessed respite care regarded it as beneficial. However in some cases, where respite care was available, it was not accessed as carers felt guilty about leaving their partner in the care of someone else. Carers reported being unable to talk to friends and family about the impact of the disease on them and some expressed a wish to receive counselling. Those who had been able to access counselling services had found them beneficial. However, counselling was often not available or difficult to organise and some carers did not know how to access this service.
Some carers had accessed support from carers’ groups, although not all found this met their expectations. Those who did not have access to a local support group felt this would be beneficial.
Support failed to address educational needs regarding care and equipment.
Conclusions: Exploration of carers’ experiences in this manner has made it possible to arrive at a greater understanding of the challenges faced when caring for a family member with MND. It is apparent that there needs to be a review of support services provided for such carers as current availability is not only haphazard but also difficult to access at times. It should be noted that where support services are available individual circumstances may still preclude their uptake. It is evident from this study that one size does not fit all and what might be appropriate to one carer may be unacceptable to another. It is recommended that a range of support services be made available, with ease of access, from which carers can select those most appropriate for their individual needs.
P136 SOCIAL CARE AND OUT OF POCKET DISEASE COST IN PATIENTS AND FAMILIES WITH AMYOTROPHIC LATERAL SCLEROSIS IN SPAIN
MORA JS1, SALAS T1, MARÍN S1, MORÁN Y1, FAJARDO ML2
1ALS Unit Hospital Carlos III, Madrid, Spain, 2Epidemiology Unit Regional Health Authority, Huelva, Andalucía, Spain
E-mail address for correspondence: [email protected]
Keywords: social-care, financial cost, Spain
Background: Suffering an adult onset progressively disabling disease such as ALS may produce a significant decrease in family income caused by changes in the provider roles of patients and caregivers, as well as a significant increase in expenses for health and social care not appropriately provided by the public system. There is not yet data on this subject in ALS care in Spain, where there is a public health care system.
Objectives: To know the status of social care and economic burden on Spanish ALS patients and families in order to specify needs and to propose improvements.
Methods: We created a 156 item questionnaire that included demography, clinical status and administered health care. An additional 61 questions covered labour status, social aids and out of pocket economic cost of the disease. The questionnaire was hand delivered or mailed to up to 400 patients with ALS from the Spanish ALS Foundation nationwide. Response to date is 260. A univariant analysis with SPSS 10.0 for Windows was performed.
Results: The patients’ median profile is male/female (ratio 1:1); 54 years old (range 29–79); married (81%); spouse as primary caregiver (70.8%); and from Madrid (41.5%). Specific situations related to social care and disease cost were identified. Among them, 6.7% of patients have a full-time job versus 51.7% prior to the disease. 45.9% of caregivers were reduced to a part-time job. Median delay in the official recognition of disability was 11.4 months; 5.2 additional months to receive financial aid. These delays negatively influence the recognized degree of disability and the aid obtained. 38.7% of patients depend on this aid, and 33.9% on spousal income. During the last 12 months the aid received by 67.7% of patients was less than 3,000 Euros; and over the next 12 months 69.6% of families expect to receive less than 6,000 Euros. Family cost of the disease varies from 10,000 to 60,000 Euros per year depending on clinical status, knowledge of needs and available aids, and family economic resources. Detailed data will be presented.
Conclusions: Social aid is delayed and insufficient. Family expenses and required needs are often higher than the aid received and the family total income. A more agile processing of the disability recognition and an increase in financial aid are necessary. The recent approval of a Dependency Law to finance caregivers should help to lessen the economic burden of the disease, although the current financial crisis delays the practical application of the law.
P137 ALS PATIENTS ON A UNIVERSITY PALLIATIVE CARE UNIT – DEMOGRAPHIC AND CLINICAL DATA
LORENZL S, HENSLER M
Department of Neurology and Palliative Care Unit, LMU, Munich, Germany
E-mail address for correspondence: [email protected]
Keywords: university palliative care unit, symptom control
Purpose: We have evaluated the demographic data of admissions of ALS patients to our palliative care unit at the University of Munich, Campus Grosshadern from January 2007 until December 2009.
Methods: Retrospective analysis of patients’ charts.
Results: In 2007 there were 12 patients (4% of all patients) with ALS (m:w = 6:6). The mean duration of hospitalization was 11±6 days. Four patients had a second stay (12±4 days). Five patients died during their time on our palliative care unit. In 2008 there were 14 patients (5 %) with ALS (m:w =7:7). The mean duration of hospitalization was 9±7 days. One patient had a second stay (7 days). Seven patients died during their time on our palliative care unit. In 2009 there were 10 patients (3 %) with ALS (m:w = 5:5). The mean duration of hospitalization was 11±6 days. None of the patients had a second stay. Three patients died during their time on our palliative care unit.
The main reasons for admission have been pain, respiratory insufficiency/depression and swallowing problems as well as increasing weakness. There has been no request for hastened death during the observation period. Two patients have been admitted to a hospice, but the majority have been able to get home again.
Conclusions: Although the number of patients with ALS admitted to our palliative care unit is relatively low it has been almost stable over three years. However, the duration of the hospitalization tends to decrease with admission of patients who are severely ill and die at the unit. Admission to a palliative care unit has not been associated with the wish to hasten death during this period.
P138 DEVELOPMENT OF AN INTERACTIVE SOFTWARE FOR EVIDENCE BASED SYMPTOMATIC MANAGEMENT OF MOTOR NEURON DISEASE WITH SPECIFIC TRIGGER POINTS TO SPECIALIST PALLIATIVE CARE INTERVENTION
BEDE P1,2, OLIVER D3, O BRANNAGAIN D4, HARDIMAN O1,2
1Trinity College, Dublin, Ireland, 2Beaumont Hospital, Dublin, Ireland, 3University of Kent, Rochester, United Kingdom, 4Dochas Centre, Drogheda, Ireland
E-mail address for correspondence: [email protected]
Keywords: palliative care, symptomatic management, software
Background: The optimal management of Motor Neuron Disease (MND) requires a palliative approach from diagnosis; maintaining quality of life for patients and carers with effective symptom control and respecting patient autonomy. While the management of specific symptoms is well described in the literature, the timing and triggers to specialist palliative care intervention are poorly defined. Frequently specialist palliative care referral only happens in the terminal phase of the disease. We propose a flexible model of episodic, consultation based palliative framework from the diagnostic stage of the disease.
Methods: Based on a literature review of current international guidelines on palliative care intervention in MND we have developed a computerised interactive guide to help physicians and general practitioners to consider evidence based interventions at various stages of the disease. The programming language Object Pascal has been used in the development of the software.
Results: A user-friendly software using a series of simple dropdown menus has been developed giving evidence based recommendations depending on the severity of the following complaints: Dyspnoea, Dysphagia, Dysarthria, Sialorrhea, Pseudobulbar affect, Fatigue, Cognitive and behavioural deficits, Musculoskeletal pain, Sleep disturbances, Muscle weakness, Functional decline, Psychological distress and End of life decisions. The proposed software suggests referral to specialist palliative services based on symptom severity. A number of rating scale calculators and assessment algorithms are also included.
Conclusion: The proposed program is intended as an interactive aid to consider management options and specialist palliative care referral at the various stages of Motor Neuron Disease.
P139 UNILATERAL PAROTID ELECTRON RADIOTHERAPY AS A PALLIATIVE TREATMENT FOR SIALORRHEA IN AMYOTROPHIC LATERAL SCLEROSIS
HODSKINS J, ST. CLAIR W, KASARSKIS E
University of Kentucky College of Medicine, Lexington, KY, United States
E-mail address for correspondence: [email protected]
Keywords: sialorrhea, radiation, parotid
When ALS patients experience some degree of bulbar involvement, sialorrhea can become a considerable challenge. Drooling has a profound negative impact in ALS patients’ quality of life with embarrassing social implications. Several therapeutic modalities, including anticholinergic drugs, botulinum toxin injection, and radiotherapy have emerged as treatments for drooling in ALS. This retrospective case-series study examined the effect of palliative radiotherapy in controlling problematic oral secretions in 5 ALS patients refractory to medical management. In this series, external beam radiation with 500 cGy electrons was targeted to the parotid gland unilaterally at a depth determined by 3D CT scanning. Two patients received additional doses to the contralateral parotid due to persistent secretions. All patients reported some degree of benefit, resulting in a reduction in intensity and amount of drooling. There were no major side effects of treatment. We conclude that low-dose, conservative electron radiotherapy directed to the parotid glands provides satisfactory relief from sialorrhea in ALS patients and should be considered as a therapeutic option for patients refractory to medical management.
P140 SPEECH THERAPY AND COMMUNICATION DEVICES: IMPACT ON QUALITY OF LIFE IN PATIENTS WITH ALS
SCHMALBACH S, SIENIAWSKI M, KOLLEWE K, DENGLER R, KRAMPFL K, PETRI S
Department of Neurology, Medical School Hannover, Hannover, Germany
E-mail address for correspondence: [email protected]
Keywords: speech therapy, communication device, quality of life
Background: Bulbar weakness is the first and main presenting feature in approximately 25 to 30% of patients with motor neuron disease (MND). The degeneration of brainstem motor nuclei results in dysarthria and dysphagia. The loss of speech as a result of dysarthria is one of the most profound changes that a patient with MND will experience. It is very distressing not only due to the resulting inability to communicate but also due to the loss of expression of personal characteristics such as humour and emotions.
Objectives: The aim of the present study was to investigate the impact of speech therapy and communication devices on the quality of life in patients with Amyotrophic Lateral Sclerosis (ALS).
Methods: ALS patients (n=30) with dys- or anarthria, who underwent speech therapy and/or used communication devices participated in the survey. They filled in three standardized questionnaires (Beck depression inventory (BDI), SF-36 questionnaire, revised ALS functional rating scale (ALS-FRS-R)) and were further interviewed about their experience with and benefit of speech therapy and communication devices.
Results: Results confirmed that loss of speech is a very high burden for patients. The effect of speech therapy on quality of life was only rated as moderate by most patients. In contradiction the prescription of a communication device mostly generated a considerable retrieval of quality of life, although initial troubles with the handling were often reported. We could further verify that information for patients about adjuvant methods is often poor if they do not attend specialized MND centres.
Discussion and conclusions: The inevitable decline in speech intelligibility in patients suffering from ALS with bulbar involvement can only marginally be delayed by speech therapy which might explain the only moderate impact on quality of life. When patients suffer from advanced dysarthria/ anarthria, the use of a communication device can restore the ability to communicate, which was in general rated as having a great effect on quality of life.
In conclusion, informing patients about adjuvant methods like speech therapy and communication devices is very important, which highlights the significance of treatment in specialized MND centres. For maximum benefit, patients must be professionally instructed in the use of the particular communication device.
P141 POWER WHEELCHAIR MOBILITY OVER TIME IN ALS/MND: PSYCHOSOCIAL IMPACT, FUNCTIONAL USAGE, FALLS, PAIN RELIEF, AND COST
WARD A1, GORELIK R2, BROOKS BR1, MITSUMOTO H2
1Carolinas Medical Center, Charlotte, NC, United States, 2Columbia University Medical Center, New York, NY, United States
E-mail address for correspondence: [email protected]
Keywords: adaptive equipment, assistive technology, quality of life
Background: Preliminary retrospective studies have been completed, and this survey furthers the information gathered to include expanded surveys and a psychosocial scale for more information on this population.
Objectives: To determine how the wheelchairs/features are used over time, the psychosocial impact of using a power wheelchair, information about pain and falls and cost/value information.
Methods: A questionnaire and the Psychosocial Impact of Assistive Devices Scale (PIADS) were sent to a consecutive sample of ALS patients who are power wheelchair users and are currently seen at one of two multidisciplinary ALS clinics; 35 questionnaires were returned. The surveys were sent out at one month and 6 months to yield information over time.
Results: One hundred percent of respondents felt their quality of life and overall mobility had improved since getting the power wheelchair. Twenty-five percent reported falls even after getting their chair, and no patients reported falling out of their chairs. All patients reported improvements in amounts of pain and lower extremity edema since getting the chair. All respondents reported sleeping for 1–2 hours in their chairs. We were able to compare usage of power features at 1 month and 6 months, and all respondents demonstrated increased usage of their chairs at 6 months. On the PIADS, all respondents reported the power chair had increased their competence, happiness, independence, quality of life, sense of control and ability to participate. Respondents noted an increase as well with their ability to adapt to activities of daily living, skillfulness and capability. Respondents also noted that having the power wheelchair decreased their embarrassment, confusion and frustration. Overall reported positive outcomes from having the chair, included items like freedom, safety, ability to go outside and longer distances, feeling less tired overall, feeling less dependent and having more ability to do daily tasks. Overall reported negative outcomes included being unable to visit inaccessible places, and that the power wheelchair was too big. No respondents had to delay Hospice to get their chair. The average time from onset of symptoms to wheelchair delivery was 48.6 months, and the time from diagnosis to wheelchair delivery was 38.4 months. The average cost of the power wheelchairs was $26,949, the average amount of wait time from evaluation to chair was 2.6 months, and from recommendation of the chair to delivery was 6.2 months.
Conclusions: We obtained first hand knowledge from 35 ALS patients who are current power wheelchair users, on their functional usage, falls, pain and edema management, psychosocial adjustment, cost and satisfaction with their power wheelchairs over time.
P142 POSSESSION OF ASSISTIVE DEVICES AMONG PERSONS WITH AMYOTROPHIC LATERAL SCLEROSIS
OTERA A, TAKAHASHI K, OGINO M, FUKUDA M
Kitasato University East Hospital, Sagamihara Kanagawa, Japan
E-mail address for correspondence: [email protected]
Keywords: occupational therapy, assistive devices, activities of daily living
Background: Amyotrophic lateral sclerosis (ALS), the progressive neurologic degenerative disease, has been shown to affect the person's ability to continue performing activities of daily living (ADL). Occupational therapists play an important role in prescribing appropriate assistive devices at appropriate timing to maintain their independence in ADL. There are some studies on the use of wheelchairs in persons with ALS. However there are only few studies looking at other assistive devices. Without any guidelines, the processes of selecting the assistive devices tend to depend on the experience of therapists or the advice from the mentor.
Objective: The purpose of this study was to describe the type and the timing of prescription of assistive devices such as orthoses, aids for eating, drinking and communication in persons with ALS.
Methods: The participants in our study were 97 persons with ALS/MND who were receiving occupational therapy at our hospital from April 2003 to March 2009 (57 men and 40 women; age range, 30–82 y). ALS onset types were either of upper type (46%), lower type (27%), or bulbar type (24%).
We retrospectively looked up the clinical recording for the past six years, and researched the type of assistive devices and the timing of the prescription.
The data was analyzed according to the following four points: 1) Assistive devices were grouped into five categories according to the criteria determined by the association of technical aids in Japan. We then accounted the rate of possession for each category; 2) In terms of ALS onset type; 3) In terms of the period of onset; 4) In terms of the score on ALSFRS-R.
Results: The report showed that 65% of the participants in our study (n = 63) were prescribed at least one assistive device. The average number of devices in those 63 participants was 2.8 (median = 3.0). 1) Rate of possession for each category of assistive devices were: cervical collar (13%), wrist-hand orthoses (32%), upper limb orthoses (non-body worn) (15 %), aids for personal care (5%), aids for eating and drinking (27%), computer (12%), sound transmission system (5%), face to face communication aids (31%); 2) Assistive devices were prescribed mostly in persons with upper type ALS. Cervical collars were prescribed in bulbar type. There was no peculiarity in other types of ALS; 3) There was no relationship between the type of the assistive devices and the period of onset; 4) When persons had a high score on ALSFRS-R, they took possession of orthoses and aids for personal care (eating and drinking). When they had a low score on ALSFRS-R, they took possession of computers and communication aids.
Discussion and conclusions: This study demonstrated the possession of the assistive devices among persons with ALS quantitatively.
P143 HYDROTHERAPY PROGRAM FOR PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS WHO HAVE LOST THE ABILITY TO WALK
SILVA T1,2, CONCEIÇÃO E1, CHAVES CA1, CUNHA M1,2, DURAN M1,2, OLIVEIRA A1
1UNIFESP–EPM, São Paulo, Brazil, 2ABRELA, São Paulo, Brazil
E-mail address for correspondence: [email protected]
Keywords: hydrotherapy, walk
Background: The ability to walk in patients with amyotrophic lateral sclerosis tends to undergo changes due to the beginning of muscle atrophy; also later, foot equinus, as well as changes in balance. Although treatments carried out in swimming pools are widely used due to the benefits such treatment provides, there are very few studies which focus on the effects produced by body immersion. The heated water of a swimming pool facilitates initial gait training and balance.
Objective: Analyzing the starting time for gait loss in patients with sporadic and bulbar ALS.
Method: Fifty four patients with a diagnosis of ALS (42 with sporadic ALS and 12 with bulbar ALS) were accompanied for 3 years by a physiotherapy team from the Neuromuscular Disease sector of UNIFESP-EPM. Group 1 (SALS): 28 men and 14 women, mean age 55.5 years (28–83 years); group 2 (BALS): 2 men and 10 women, mean age 64 years (53–75 years). None of the 54 patients exhibited changes in their ability to walk during their initial assessment using item ‘H’ of the ALS-FRS Scale (Amyotrophic Lateral Sclerosis – Functional Rating). All 54 ALS patients were performing hydrotherapy once a week at a clinic-school. We analyzed the time when the first adaptations for walking were prescribed (use of anti-footdrop ankle orthosis, canes, or walkers), and when these patients lost their ability to walk and initiated use of a wheelchair. Patients were evaluated every 3 months.
Results: Fifteen months after diagnosis we verified that rehabilitation for SALS patients can increase the time needed for starting to use gait adaptations, and also after 24 months to increase the mean time for losing gait and starting the first adaptations. For BALS patients, 3 (12) lost gait after 19 months and 9 (12) developed changes for walking and started using a device to improve gait 20 months after diagnosis.
Conclusion: The findings suggest that rehabilitation can increase walking time and the ability to walk for SALS and BALS patients.
P144 FALLS IN MND: A REHABILITATION EXPERIENCE
TAYLOR E1, RAYKAR V2, DEWHURST E-K1
1St Joseph's Hospital, Auburn, NSW, Australia, 2Concord Hospital, Sydney South West Area Health Service, Concord, NSW, Australia
E-mail address for correspondence: [email protected]
Keywords: falls, injury, multidisciplinary care
Background: Falls are very common in older people and often under reported. It is estimated 1 in 3 Australians over 65 fall at least once per year and the incidence increases with increasing age (1). Significant injuries from falls, including fractures is reported to be approximately 2–6% (2). A fall is defined as ‘an unexpected event in which the participant comes to rest on the ground, floor or lower level’ (3).
People with MND have multiple risk factors for falls.
The MND service at St Joseph's Hospital services the western suburbs of Sydney, Australia. Newly diagnosed patients are admitted for a 2–3 day stay for purposes of assessment and early education. Patients are then seen in the multidisciplinary clinic every 3–4 months and are reviewed in the interim by any staff members as required.
Objectives: We reviewed the incidence of falls in people with MND, the injuries sustained and the consequences of those injuries.
Methods: We completed a retrospective review of the incidence of falls in people who attended our MND multidisciplinary service from 2007–2009 to review the incidence of falls, the incidence of injurious falls and the impact of those falls on function.
Discussion: Approximately 50 patients were seen by the MND service between 2007–2009. Of those patients, approximately 90% of patients had experienced at least 1 fall, with at least 50% of patients experiencing multiple falls.
We looked at the rate of injurious falls. Our definition of an injurious fall was a fracture – we did not record injuries such as superficial cuts and bruises. Approximately 10% of patients seen over this time had an injurious fall. 5 people had fractures that involved either the humerus, pelvis, fibula, patella or big toe. One person had 2 injurious falls, both resulting in lower limb fractures.
The consequences of these falls have been very significant. Not only did the injurious falls all require medical input, but 3 people required inpatient hospitalisation following the fall. The injurious falls had a significant impact on ADL performance and independence. The fractures following falls impacted mobility, activities of daily living, pain and problems with self-care and feeding and bed mobility.
This can also lead to an increase in carer burden.
We have recently implemented regular screening, falls prevention education and ensured the prescription of assistive aids.
Conclusions: Falls are very common in people with MND. Injury from falls has a significant impact on ADL's and carer burden in people with MND. Appropriate screening, education and use of prescriptive aids can prevent a proportion of these falls. Prevention of falls is a significant issue that needs to be addressed in people with MND.
References:
- Lord SR, Ward JA, Williams P, Anstey K. J. Australian Journal of Public Health, 1993;17:240–5.
- Lord SR, Sherrington C, Menz HB, Close JCT. Falls in Older People Cambridge University Press 2nd ed. 2007.
- Prevention of Falls Europe Network Consensus.
P145 MANAGING MEDICAL CRISES: THE EXPERIENCES OF PEOPLE AFFECTED BY MOTOR NEURONE DISEASE AND THEIR CARERS
HILL J
University of Nottingham, Nottingham, United Kingdom
E-mail address for correspondence: [email protected]
Keywords: out of hours, emergency services, medical emergency
Background: MND is a non-curative neurodegenerative disorder that has a rapidly progressive course. Due to the direct and indirect symptoms of the disease, people living with MND can suffer from a range of acute medical problems/emergencies throughout the course of their illness requiring a rapid response and proactive planning by professionals. The majority of people are supported and cared for at home and during an acute incident need to access primary care services and/or crisis intervention services ie out-of-hours doctors/nurses or ambulance services. There is limited knowledge and literature on the experiences of patients and carers of out-of-hours and ambulance services.
Objective: To explore the experiences of people affected by MND using crisis intervention services during a medical emergency.
Method: The research aim was met through a qualitative approach using in-depth, semi-structured interviews. Seven people with MND were interviewed, five primary unpaid carers of someone with MND and two bereaved carers.
Results: Three central themes were identified from the analysis of the data for participants with MND – independence, control and the quality of the crisis intervention services. The additional theme, guilt, was identified for carers.
From the themes discussed, three important aspects arose from this study: 1) Provision of information: Participants required information on how to manage practical aspects of the condition and to know who to ring in an emergency. They needed information on the systems, procedures and response times of out-of-hours and ambulance services; 2) Proactive management: In the study all participants with MND had experienced a fall, the results therefore highlighted the need for the provision of a key worker to provide proactive management of cases, support and information on services and advice on symptom management for people with MND and their carers; 3) Communication: The study identified a need for a system that enables the details of people with MND who have frequent falls to be ‘flagged up’ on the ambulance service computer system. The ambulance service lacked information on the crisis they were attending.
Conclusion: The study findings have highlighted a number of key areas for future research; in particular they suggest that the need to provide clear information to users of the ambulance service is important so that they understand and have confidence in the service. The study prompts a recommendation that work is carried out on the design and evaluation of an information leaflet on aspects of the ambulance service relevant to this patient group. An information leaflet will help reduce the anxiety felt by carers of people with MND and improve the quality of the care provided by the ambulance service.