P262 USAGE OF CUSTOMIZED WHEELCHAIRS BY PATIENTS WITH ALS
SUKENICK R1, HARRINGTON-MORONE G1, DALTON K1, CALCAVECCHIA J2, ANDREWS J1, HABIB A1, MITSUMOTO H1
1Columbia University Medical Center, New York, NY, United States,2ATG Rehab, Newington, CT, United States
Email address for correspondence: [email protected]
Keywords: wheelchairs, cost, falls
Background: Wheelchair issues in ALS have rarely been studied (1). Customized wheelchairs are commonly prescribed for patients with ALS. In our clinic, 51% of patients who were seen for the first time during the year 2006 have used or are currently using customized wheelchairs. Wheelchairs maintain mobility once increased fall risk makes independent ambulation unsafe. They provide independence, comfort and pressure relief, allow increased participation in activities of daily living and reduce energy expenditure. Obtaining a customised wheelchair can take months and cost tens of thousands of dollars. Practice guidelines are not available to determine the optimal time to recommend a customized wheelchair. Our patients often wait an excessive amount of time, often past the point of need, for their customised wheelchairs. Some use the chairs for too short a time to justify the wait and cost.
Objectives: To understand the use of time and resources in obtaining and using customized wheelchairs in our patient population and to generate research questions for future study.
Method: Under a CUMC IRB approval, we reviewed the charts of patients with ALS who were evaluated for a customised wheelchair from April 2008–April 2009. This time period was chosen to coincide with the initiation of performing wheelchair evaluations within our multidisciplinary clinic. Wheelchairs were recommended to those who had a history of falls, demonstrated gait deficits and could no longer walk safely.
Results: A total of 38 patients’ charts were reviewed. 52% of patients were male and mean age was 66 years old. The mean ALSFRS and FVC (% normal) were 35 and 81% at diagnosis and 32 and 66% at time of wheelchair recommendation. The mean number of months from diagnosis to recommendation of a wheelchair was 30. Patients waited a median of 4 months (range 2–31 months). The average cost of each wheelchair was $25,648.97. Of the 38 patients, 24 fell and 13 were delayed in starting hospice while waiting for the delivery of their wheelchairs. Of the 38 patients, 8 have died. 4 of the 8 never received their wheelchairs prior to their death. The other 4 utilized their wheelchairs for a mean of 3 months prior to death (range 1–5 months).
Discussion and Conclusion: Streamlining and expediting the process of obtaining customized wheelchairs for ALS patients may allow maximum utilization of these devices improving the cost/benefit ratio. This may reduce falls and allow for quicker entry into hospice services. A prospective study investigating quality of life and medical economics is essential.
Acknowledgements: MDA and Wings Over Wall Street
Reference:
- Trail M, et al. Arch Phys Med Rehabil 2001;82:98–102
P263 POWER WHEELCHAIR PRESCRIPTION, UTILIZATION, SATISFACTION AND COST FOR PATIENTS WITH ALS/MND; PRELIMINARY DATA FOR EVIDENCE BASED GUIDELINES
WARD A, DUFFY K, WILLIAMS N, STENGER C, NICHOLS M, SANJAK M, BOCKENEK W, BRAVVER E, BROOKS BR
Carolinas Healthcare, Department of Neurology, Charlotte, NC, United States
Email address for correspondence: [email protected]
Keywords: assistive technology, neurorehabilitation, durable medical equipment
Background: Rapid disease progression in ALS/MND patients leads to unique needs for power wheelchair selection and continuous requirements for power chair updates to meet functional changes. There are no evidence-based guidelines available for employment of durable medical equipment specifically in the ALS/MND population. Current management of mobility equipment for ALS/MND patients is primarily based on the experience of clinicians and feedback from patients.
Objectives: To determine the features that ALS/MND patients require and choose on their power wheelchairs, how often the chairs/features are used and whether people like those choices over time.
Method: A questionnaire was sent to a convenience sample of 45 ALS/MND patients who are power wheelchair users and are currently seen at our multidisciplinary ALS/MDA clinic; 27 males and 18 females, with a mean age of 57.9 years (27–85). The average total ALSFRS-R score was 16 (0–38), and 17/45 of the patients used ventilators. The average time from first symptom to time of chair was 36.08 months (16–75), and from chair to questionnaire was 28.17 months (1–71). Thirty questionnaires (67%) were returned.
Results: Ninety-three percent (28/30) of respondents received their evaluations at a multidisciplinary ALS clinic, one via the Department of Veterans Affairs, and one was unknown. Seventy percent (21/30) of patients thought the chair evaluation was timed correctly, and 20% (6/30) wished they had started sooner. Forty-three percent (13/30) of people were able to walk a few steps, and 57% (17/30) were able to stand when their chairs arrived. We compared how many times a day they used tilt, recline, power legs, and seat elevate after receiving the chair and currently as well as overall time spent in their chairs for the same periods. When they first received the chair, 78% (22/28) were satisfied with the overall comfort of the chair, and 86% (23/27) were satisfied with the ease of use; currently, 66% (18/27) are satisfied with the overall comfort, and 66% (18/27) are satisfied with ease of use. The average cost for the power chairs was $26,404 ($19,376–$34,311), and the average cost per month is $937 ($242–$2,450). Overall, 88% (26/30) of respondents said they would get the same type of chair with the same features again and 81% (21/26) felt that the chair was good value for the cost.
Conclusions: We obtained first hand knowledge from 30 ALS/MND patients who are current power wheelchair users, on their utilization and satisfaction with their power wheelchairs from initial to current use. Information reported may assist wheelchair evaluators with what ALS/MND patients need and want, and will direct clients toward choices that will suit them for the long term. Based on this survey, ALS/MND patients are overall satisfied with their chairs, and further study of timing, adjustability, and customization of the equipment will help with the analysis of the cost/benefit to these patients.
P264 PILOT STUDY OF SUPPORTED TREADMILL AMBULATION TRAINING FOR AMYOTROPHIC LATERAL SCLEROSIS PATIENTS
SANJAK M1,2, DUFFY K1,2, RUSSO P1,2, BOCKENEK J1,2, HOLMES S1,2, BRAVVER E1,2, BROOKS BR1,2
1The Carolinas Neuromuscular/ALS-MDA Center., Charlotte, NC, United States,2Department Of Neurology, Charlotte, NC, United States
Email address for correspondence: [email protected]
Keywords: physical exercise, gait, active assisted exercise
Background: Repetitive rhythmic physical exercise (RRPE) has a positive health outcome in the general population. The effect of exercise in ALS is controversial. Exercise parameters such as type, mode, and dosing (duration, frequency and intensity) are lacking for ALS. Ambulatory patients with assistive devices (AD) can not engage in RRPE without assistance. STAT enables patients to ambulate on a treadmill while wearing a harness connected to an un-weighting system, so their weight can be partially reduced. STAT was shown to improve gait in a variety of neurologic deficits, but has not been used in ALS.
Objectives: To determine safety, tolerability, and efficacy of STAT in ambulatory ALS patients with AD.
Methods: ALS patients (n = 4) who qualified to enrol in this study: had definite ALS; were able to stand and ambulate independently with AD; were able to complete the 25 foot walk test (25FWT) in less than one minute; had a vital capacity (VC) of ≥65% predicted. The intervention consisted of 30 minutes of STAT (five minutes exercise and five minutes rest) with 40% of body weight supported. Patients build up their endurance to training as tolerated; walking speed was as tolerated by each patient. Exercise intensity was determined by each patient's rate of perceived exertion (RPE), but not to exceed level 12–13 (mild to moderate) on the modified Borg RPE scale. Six minute walk test on treadmill (T6MWT), ground (G6MWT), 25FWT, ALSFRSR, %VC, Manual muscle test (MMT) of lower extremities, fatigue severity scale (FSS), and RPE during T6MWT were evaluated at baseline (B), 4 and 8 wks post training.
Result: Three patients completed the 8 wks. One patient left after 7 visits due to shoulder pain and difficulty in swinging her leg. T6MWT distance increased 40% (±7%) and 45% (±3%), speed increased 31% (±10%) and 37% (±14%), RPE decreased 34% (±10%) and 46% (±9%), 25FWT time decreased 18% (±30%) and 26% (±30%) after 4 and 8 wks respectively. Patient number 3 25FWT time was 5.3 at B, a close to normal value for this test, and did not change. We suspected that G6MWT may be more sensitive than 25FWT for this Pt. G6MWT distances were 315, 427, 445 metres at B, 4 wks, and 8 wks respectively. ALSFRSR scores were 33 (±6), 37 (±6), and 36 (±7);%VC were 87 (±18), 85 (±20), and 85 (±20), MMT were 54 (±13), 57 (±14), 57 (±11), FSS were 37 (±6), 38 (±11), and 36 (±11) at B, 4 wks, and 8 wks respectively.
Discussion and Conclusion: STAT is tolerated with no adverse effects. Active assisted RRPE using STAT shows training effects and may be beneficial for ALS Patients by permitting exercise while protecting from overwork injuries and deconditioning leading to disuse atrophy. STAT needs to be further investigated in a larger clinical trial.
P265 CARDIOPULMONARY RESPONSES TO INCREMENTAL RAMP EXERCISE IN PATIENTS WITH MOTOR NEURON DISEASE
MEZZANI A1, PISANO F2, CAVALLI A2, TOMMASI MA2, COLOMBO S1, PSAROUDAKI M1, GIANNUZZI P1
1Cardiology Division,2Neurology Division, S. Maugeri Foundation, Veruno Scientific Institute, Veruno, Italy
Email address for correspondence: [email protected]
Keywords: aerobic exercise, exercise testing, oxygen consumption
Background: Scarce data are available about the physiological response to dynamic incremental exercise in patients with motor neuron disease (MND).
Objectives: To assess the cardiopulmonary response to cycle ergometer incremental exercise in a group of MND patients, using ramp protocols routinely performed in the clinical setting for patients’ functional evaluation.
Methods: We studied 14 patients affected by MND (12 amyotrophic lateral sclerosis, 1 primary lateral sclerosis and 1 progressive muscle atrophy, age 57±11 years, 43% males, 10±9 months from diagnosis) and without associated diseases affecting the cardiorespiratory response to exercise. All patients underwent a ramp incremental cardiopulmonary exercise testing (CPET), with power increments of 5 W/min, 7 W/min, or 10 W/min according to the referred level of patients' habitual activities. Respiratory gas exchange measurements were obtained breath-by-breath using a computerized metabolic cart (Vmax29; Sensormedics; Yorba Linda, CA). Peak VO2 was the mean VO2 value observed during the last 30 s of the exercise period. Ventilatory anaerobic threshold was estimated by the V-slope and/or respiratory equivalents methods. Functional Rating Scale (FRS) and resting forced vital capacity (FVC) were also evaluated in the whole study population.
Results: No adverse event was observed during exercise tests. Peak VO2 averaged 15.4±5.7 ml/kg/min, corresponding to 61±18% of predicted maximum, i.e. to a slightly reduced aerobic power; however, the mean VO2/W relationship slope was 10.2±1.4 ml/W/min, testifying to a globally preserved efficiency of the O2 transport and utilization system. Peak respiratory exchange ratio averaged 1.03±0.05, attesting near-maximal effort attainment. Ventilatory anaerobic threshold was identified in 50% of patients, with VO2 values averaging 10.7±3 ml/kg/min, i.e. 64±11% of peak VO2. Mean peak ventilation was 36±12 1/min, with a VE/VCO2 relationship slope mean value of 30±6, indicating a preserved ventilatory efficiency. FRS score averaged 37±6, and FVC mean value was 2.6±0.9l, equal to 86±24% of predicted. None of the evaluated parameters was related to time from diagnosis.
Discussion and Conclusions: Patients with MND are usually prevented from aerobic training due to concern about possible negative effects on disease course (muscle fatigue worsening). Our data show that patients with a mean time from diagnosis around 1 year and quite preserved FRS score and FVC can safely perform a near-maximal ramp incremental CPET. The lack of correlation between the evaluated parameters and time from diagnosis suggests an early stage pathophysiology in this study group. In such a clinical setting, the observed slight reduction of peak VO2 with respect to predicted values in the presence of normal VO2/W and VE/VCO2 slope values supports a peripheral, i.e. skeletal muscle, rather than central, i.e. heart and lung, limitation to exercise tolerance. As no data are currently available regarding the effects of aerobic exercise training on exercise tolerance, quality of life, and prognosis of patients with MND, our findings may be used as a rational basis for future studies aimed at addressing these important issues.
P266 EFFECTS OF A HYDROTHERAPY PROGRAM ON FUNCTION AND MUSCLE STRENGTH IN PATIENTS WITH SPORADIC AMYOTROPHIC LATERAL SCLEROSIS
SILVA T, CHAVES AC, CONCEICAO E, CUNHA M, QUADROS A, OLIVEIRA A
UNIFESP/EPM, Sao Paulo, Brazil
Email address for correspondence: [email protected]
Keywords: hydrotherapy, function
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of the neuromuscular system resulting in paralysis and ultimately death. Currently, no effective therapy is prescribed for patients; however, several therapeutic strategies are showing promise. Kaspar et al indicate that a drug treatment in combination with appropriate exercise may provide the most promising therapy for ALS. While both upper and lower motor neuron dysfunction may contribute to impaired muscle function in ALS, the precise mechanisms of muscle fatigue have not been clarified in this disease. Patients with ALS had less intramuscular phosphocreatine depletion and less fatigue of stimulated tetanic force during exercise compared to controls. Thus, due to the central failure, there was decreased muscle activation resulting in a smaller metabolic demand and less fatigue within the muscle itself. These data demonstrate a major contribution of central factors to muscle fatigue in ALS.
The role of physical activity for patients with ALS is controversial. A regular moderate physical exercise program has a short-lived positive effect on disability in ALS patients and should be recommended. Endurance exercise training is known to increase antioxidant capacity in skeletal muscle. In light of a recent report describing increased survival following low-intensity endurance training, it appears that training intensity is an important determinant of survival in the G93A mouse.
People with chronic or long-term physical disabilities, in addition to having basic needs for optimal sensory-motor function and ADL independence, are becoming increasingly interested in physical fitness. The swimming pool program offers a wide range of opportunities, medical and recreational, functional and social, to the consumer with physical dysfunctions. Our objective was to evaluate the effects of a swimming pool exercise program on function and muscle strength of lower limbs in patients with ALS.
This study was performed at the outpatients’ Amyotrophic Lateral Sclerosis clinic of UNIFESP/EPM by a physical therapy team from May to December 2005. Twenty-two ALS patients were randomized to receive a moderate exercise program (n = 11) or not to perform any physical activity beyond their usual daily requirements (n = 11). At baseline and after 6 months, patients were assessed by manual muscle strength testing, and ALS functional rating scale (FRS).
At 6 months, there was no significant difference between groups, although a trend towards less deterioration in the treated group on both scales was observed.
P267 INTRATHECAL BACLOFEN FOR SPASTICITY IN MOTOR NEURON DISEASE: CRITERIA FOR ITB PUMP PLACEMENT
SCHWEIKERT K1,2, KÄTTERER C2, WILMES S2, WASNER M3, WEBER M4
1Neuromuscular Center University Hospital Basel, Basel, Switzerland,2REHAB, Basel, Switzerland,3Department of Neurosurgery University Hospital Basel, Basel, Switzerland,4Kantonsspital St Gallen, St. Gallen, Switzerland
Email address for correspondence: [email protected]
Keywords: spasticity, intrathecal baclofen, baclofen pump
Background: Management of severe spasticity in Motor Neuron Disease (MND) is often unsatisfactory because patients do not tolerate or are refractory to oral medications. In Primary Lateral Sclerosis (PLS) and upper motor neuron predominant Amyotrophic Lateral Sclerosis (ALS) intrathecal baclofen (ITB) might be an option. Mobility, activities of daily living (ADL), and quality of life can improve, but deterioration is also possible due to unwanted loss of muscle tone. Furthermore ITB therapy is invasive and expensive.
Objectives: To evaluate the usefulness of ITB for intractable spasticity in MND and to define criteria when a permanent ITB pump is indicated.
Methods: Patients were referred by experts of ALS clinics for ITB therapy, hospitalised, and examined by a neurologist, an occupational, a physical, and a speech therapist pre-operatively. ALS Functional Rating Scale (ALSFRS-R) and Functional Independence Measure (FIM) scores were taken at baseline, another FIM during ITB therapy. A probatory external baclofen pump, connected with a subcutaneous intrathecal catheter, was placed with local anaesthesia about 40–60 cm above L3/L4 puncture level. ITB was started at a continuous rate of 24 µg/d. The dosage was increased according to clinical signs, oral antispastic medication tapered and stopped. Spasticity was evaluated daily by the modified Ashworth scale; selected ADL, timed 10 m walk, speech and swallowing examined by members of a multiprofessional rehabilitation team. Videographic documentation was made before and under ITB treatment.
Results: From 2007 to 2009 six consecutive patients (4 men, 2 women), mean age 48.5 years, were treated with ITB via probatory external pump. Three were diagnosed with PLS, 3 with upper motor neuron predominant ALS. Mean disease duration preoperatively was 55 months, mean ALSFRS-R at baseline 29.2. Mean time of probatory ITB external pump was 9.8 days, mean baclofen dosage 44.5 ug. In all patients spasticity was reduced; no side effects were reported. Mean FIM changed from 82.5 to 84.1. One patient did not go on a permanent baclofen pump because swallowing and gait deteriorated under ITB, another patient because ADL and transfers did not improve.
Discussion and Conclusions: The pattern of muscle tone and strength varies substantially and individually in MND patients. Therefore definition of therapeutic targets with the patient and carers is crucial before starting ITB, considering especially swallowing, speech, gait, ADL, head and trunk stability, pain, positioning, transfers. A reliable evaluation requires determination of reproducible items, consistency of investigators and setting, and adequate time period.
ITB is effective but not always beneficial in MND. We recommend analysis of the usefulness of ITB by means of a temporary external pump in an experienced interdisciplinary in-house rehabilitation team. This setting provides an adequate time frame and information level for patients, carers, and professionals to define if placement of a permanent ITB pump is indicated.
P268 TEMPOROMANDIBULAR JOINT DYSFUNCTION IN ALS: A TREATABLE CONDITION
CHAULET S, RAOUL M, NICOLAS P, CAMU W
ALS center, CHU and UMI, Montpellier, France
Email address for correspondence: [email protected]
Keywords: temporomandibular joint, treatment, prevention
Objectives: To describe temporomandibular joint dysfunction (TMJD) in ALS patients and its efficient treatment.
Background: ALS onset is bulbar in approximately 25 to 30% of cases. Except for the oculomotor nuclei, motor impairment is due to the degeneration of motor neurons from 6 cranial nerves at different degrees. To date, except speech therapy, physiotherapy and treatments for excess saliva, few conditions in bulbar patients have been described as being improved by therapy.
Cases: We describe 3 types of patients with TMJD. In the first type, involvement of trigeminal motor neurons is predominant and patients have asymmetrical amyotrophy of the temporalis, leading to a major disequilibrium of the mandibula when feeding, sometimes with dislocation of the joint. In the second type, patients present with significant facial pain due to contractures of the masseters and temporalis, leading to misdiagnosis. In the third type, ALS patients complain of increased dysphagia associated with pain. In those cases a contracture of internal and external pterigoidian muscles have been found. In all three types, the patients were female and the consequence of the TMJD was weight loss, one important prognostic factor in ALS. For all the patients, the complaints completely disappeared with specific physiotherapy. In some cases, after several months, the same TMJD recurred and was successfully treated again.
Discussion: TMJD is treatable condition in bulbar ALS patients that may present in different ways but is always responsible for impaired quality of life and weight loss. For those reasons TMJD should be recognized and treated as soon as possible. While some presentations could be misleading, such as intense facial pain, physicians should be aware that facial pain should not a priori lead them to exclude ALS.
P269 NON-SURGICAL TREATMENTS FOR DROOLING IN MND/ALS: A SYSTEMATIC REVIEW
SQUIRES N1, ARTHUR A2, WILLS A1
1Queen's Medical Centre, Nottingham, United Kingdom,2The University of Nottingham, Nottingham, United Kingdom
Email address for correspondence: [email protected]
Keywords: saliva, systematic review
Background: Bulbar weakness is the main presenting feature in approximately 25% of patients with MND/ALS. Drooling of saliva is a distressing symptom associated with bulbar dysfunction. This can have important physical, psychological and social effects on the individual and their family. Patients can experience thick mucoid secretions pooling in the oropharynx and/or thin watery saliva which leads to drooling. It is important to identify the type of difficulty experienced to guide intervention. Treatment approaches vary, therefore to improve patient care, disease-specific evidence-based guidelines are needed.
Objectives: To assess the evidence to support current non-surgical treatments to reduce drooling in MND/ALS.
Methods: A systematic review of the treatment of drooling was carried out. Inclusion criteria were:1) evaluations of non-surgical interventions to reduce thin saliva in MND/ALS; 2) studies of more than two subjects; and 3) published in English language. The search strategy was divided into four steps 1) electronic search of four clinically relevant databases; 2) hand searches of all 19 International ALS/MND symposium journals; 3) an email request for unpublished studies sent to the 16 MND care centres in the UK and Ireland; and 4) hand searching of reference lists.
Results: Of the 64 published reports identified on the basis of title and abstract, only 15 met the inclusion criteria. The methodological quality of these studies varied, only one was a randomised controlled trial. Of the 15 studies, 4 reported the use of radiation, 8 studies the use of botulinum toxin injections, 1 reported a combination of radiation and botulinum, 1 a comparison of botulinum and amitriptyline and 1 the sublingual administration of atropine. Sample sizes were small (n = 4 to n = 20). Outcomes included the weight of cotton wool, number of tissues used and patient rating scales. The average length of follow up varied between 2 and 80 weeks. Although all studies reported the intervention tested to be effective only 5 studies showed statistically significant changes.
Discussion and Conclusions: Although many authors in the studies reviewed claimed that botulinum toxin injections and radiation are effective in reducing saliva in patients with MND/ALS, it has not been possible hitherto to demonstrate this with a definitive randomised controlled trial. While lack of consensus on the best treatment available for salivation in MND suggests that a trial could be justified on the basis of equipoise, recruiting sufficient patient numbers to detect important differences might be problematic. We argue that to achieve this goal, a consensus needs to be built on how to standardise treatments, and identify the most patient-centred and robust outcome. This will then allow for such treatments to be tested in an ethical manner in multiple centres to allow meaningful statistical interpretation.
P270 TREATMENT WITH CLONIDINE THROUGH INHALATION TO REDUCE THE SIALORRHEA IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
LUNETTA C, BANFI P, ROMA E, CELLOTTO N, GORNI K, CORBO M
NEuroMuscular Omnicentre (Nemo), Fondazione Serena Onlus, Milano, Lombardia, Italy
Email address for correspondence: [email protected]
Keywords: sialorrhea, clonidine, aerosol
Background: Fifty percent of patients with amyotrophic lateral sclerosis (ALS) experience problems handling serous saliva and 20% fail to achieve adequate control of sialorrhea with anticholinergic medications, or experience intolerable adverse effects from these drugs.
Objectives: This Open-label prospective study of clonidine administered through inhalation for the treatment of sialorrhea in patients with ALS was designed to determine safety as well as efficacy.
Methods: ALS patients with sialorrhea refractory to medical therapy were enrolled in this study to be treated once a day with a clonidine aerosol (0.07%). Primary outcome was rate of responders (improvement >50% on visual analogue scales (VAS) of severity and disability of sialorrhea) after 1 month of treatment. Other outcomes included subjective (drooling and quality of life questionnaires) and objective (number of paper handkerchiefs used) evaluations. Safety evaluations included daily blood pressure measurement.
Results: Fourteen ALS patients were included. At 1 month the rate of responders was 80% with a mean reduction of 70% in severity and disabling VASs. Thirteen patients (92%) reported some benefit with drooling reduction. In objective measurements there was a reduction over 60% in the number of handkerchiefs used. Onset of effect occurred few minutes after the inhalation and the duration of the effect was about 24 h. Most patients reported better quality of living. The most frequent side-effects were viscous saliva. There were no changes in blood pressure or cardiac rate. At 3 months, there was still a positive effect in all outcomes. All patients except one manifested their willingness to continue the treatment.
Discussion and Conclusions: Clonidine aerosol seems to be effective and safe to treat sialorrhea in ALS. It is suggested that the salivary reduction induced by clonidine may be mediated by a stimulation of alpha 2-adrenoceptors, which exerts an inhibitory control of the parasympathetic activity on the salivary glands.
P271 DIAGNOSIS AND TREATMENT OF DYSPHAGIA IN AMYOTROPHIC LATERAL SCLEROSIS
RIECKER A1, LINDNER-PFLEGHAR B1, SPERFELD A-D2, LUDOLPH AC1
1University of Ulm, Department of Neurology, Ulm, Germany,2HELIOS Klinikum Bad Saarow, Berlin, Germany
Email address for correspondence: [email protected]
Keywords: dysphagia, videoendoscopic evaluation of swallowing (VEES)
Background and objectives: ALS is the most common neurodegenerative disease of the motor system. Dysphagia is a frequent symptom of ALS and results in reduction of life expectancy/quality. At the beginning of the disease 20–30% of the patients demonstrate dysphagia whereas in later stages nearly 100% are affected. We started to use standardized testing for dysphagia in ALS to determine if compensatory/restitutional methods, dietary modifications, augmented feeding techniques, pharmacological interventions or surgical options are necessary (1).
Methods: The assessment comprises a clinical examination and VEES which is useful for educating patients and carers, as they clearly reveal the process of swallowing and demonstrate the risks involved in swallowing. It enables direct observation of pre-deglutitive and post-deglutitive actions within the pharynx and larynx. On the basis of a clinical examination and VEES observations, a treatment plan for swallowing therapy can be established or a decision can be made concerning further alimentation.
Results: Dysphagia in ALS can either result within a pseudobulbar paralysis or from muscular weakness innervated by trigeminal (V), facial (VII), hypoglossal (XII), glossopharyngeal (IX) or vagal (X) nerves. Swallowing is generally conceptualized as occurring in several distinct phases. In the following we describe the typical disorders during these phases: 1) In the pre-oral phase muscular weakness leads to failure of bolus breakdown/manipulation, preparation of solids and decreased bolus control resulting in premature spillage, pre-swallow pooling and post-swallow residuals in the oral cavity and/or the pharynx; 2) During the oral phase weakness of the tongue leads to increased bolus transition time, incomplete oral bolus transport and to oral/sublingual post-swallow residuals, whereas a delayed swallow reflex results in premature spillage, penetration and aspiration; 3) In the pharyngeal phase decreased base of tongue to the posterior pharyngeal wall approximation as well as incomplete larynx elevation results in post-swallow residuals; reduced pharyngeal motility and supraglottic compression lead to intra-swallow penetration/aspiration and post-swallow residuals; impairment in opening the upper oesophageal sphincter results in post-swallow residuals and aspiration; vagal deficits results in decreased effectiveness of cough on aspiration (motor) or silent aspiration (sensory); 4) The esophageal phase is not affected in ALS.
Discussion and Conclusion: Optimal care of patients with ALS requires attention to laryngeal and pharyngeal symptoms. With the assistance of a speech and language therapist the patient can overcome mild to moderate degrees of dysphagia. The adoption of special techniques can aid swallowing. In advanced stages augmented feeding strategies should be considered (1).
Reference:
- Kühnlein et al. Nature Clinical Practice Neurology 2008; 4: 366–374
P272 QUANTITATIVE VOICE ANALYSIS IN AMYOTROPHIC LATERAL SCLEROSIS PATIENTS WITH BULBAR ONSET
TOMIK J1, TOMIK B2, STREK P1, WIATR M1, GOLENIA A2, ZAWISLAK D2, WYROZUMSKA-ZUR K2, OSTROWSKA M2, GAJEC S1, SKLADZIEN J1, SZCZUDLIK A2
1Department of Otolaryngology, Medical College Jagiellonian University, Krakow, Poland,2Department of Neurology, Medical College Jagiellonian University, Krakow, Poland
Email address for correspondence: [email protected]
Background: Patients with early symptoms of bulbar ALS are usually referred to the otolaryngologist without a diagnosis. One of the earliest symptoms of bulbar involvement in ALS is voice deterioration. Voice assessment in ALS patients has been done frequently mainly by perceptual analysis. The objective parameters, including acoustic measures and videostroboscopy analysis, have been measured only in a few, small series of patients.
Objectives: To determine the vocal parameter characteristics for bulbar ALS patients.
Material and Methods: ALS patients were diagnosed according to El Escorial criteria (1998) in the Department of Neurology, Jagiellonian University Medical College in Krakow, between 2007–2008. The voices of 23 bulbar ALS were studied in the Department of Otolaryngology using a quantitative voice analysis system (with acoustic parameters) and laryngovideostroboscopy examination (LVSS). Measurements were compared with those obtained in 20 normal subjects (controls).
Results: Ninety-six per cent of ALS patients studied complained of dysarthria, 67% of dysphagia, and 56% of dysphonia. Hoarseness (37%), hypernasality (32%), asthenicity voice (31%) were the most common voice perception scores, in addition to being dysarthric. In 69% of all ALS patients timing of phonation was characterized by frequent complete voicing interruptions during running speech and vowel prolongation. The most common findings on LVSS were slowness of one/both vocal fold (VF)-87%, incomplete closure of VF-63%, bowing VF-30%, decreased VF abduction-27%, hyperadduction of VF-23% as well as a mucous pooling-19%. The shorter phonation time (PT) was detected (98%) as well as abnormal frequency of the voice (range: 60–140 Hz) (86%). The analysis of the amplitude and frequency perturbation of sound (Jitter and Shimmer parameters) have presented statistical significance difference (p < 0.01) as compared to the control group. Phonatory instability was also presented.
Discussion and Conclusion: We have previously stressed that hyper-/hyponasality, articulation defects, voice harshness and breathiness as well as swallowing difficulties should all be viewed as possible early signs of ALS and may allow the otolaryngologist to be the primary diagnostician. This study shows that on LVSS, slowness of one/both vocal folds, was the most frequent observation in bulbar ALS cases. These patients’ voices were characterized with abnormal Jitter and Shimmer and very short phonation time.
We suggest that in bulbar ALS patients careful examination of the speech quality, a videostroboscopy examination as well as an acoustic analysis should be performed to measure voice disturbances due to disease.
P273 NEW URINARY MANAGEMENT SYSTEM FOR MALE PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
NONAKA M1, YAMAUCHI R2, CHIBA S1, INOUE K1, WARABI T1
1Sapporo Yamanoue Hospital, Sapporo, Hokkaido, Japan,2Sapporo Medical University, Sapporo, Hokkaido, Japan
Email address for correspondence: [email protected]
Keywords: urinary management, QOL, burden
Background: A distinctive characteristic of Amyotrophic Lateral Sclerosis (ALS) is that it does not affect urinary function, but systemic muscle weakness impairs the movements necessary for maintaining normal urinary habits. In a survey of the actual situation of ALS patients and the level of burden on the caregiver, the top 3 burdens identified by caregivers were suction of mucous and saliva (44%), defecation (36%), and urination (33%). In particular, a heavy burden associated with multiple episodes of night time urination was identified. Urination is a very private matter, and in providing care or assistance, it is important that we allow the patient to retain his dignity, especially in patients with ALS, because their intellectual function and personality are preserved.
Objectives: We report our experience of using a new system for urinary management in male ALS patients with urinary care problems.
Methods: A new urinary management system for males (afex®) was originally developed to manage incontinence associated with complications of prostate cancer surgery. The system comprises several components which can be combined in different ways to match the needs of ambulant users, wheelchair users, bedridden users, and users of a handheld urine receptacle. The key component of the system is the “receptacle”, consisting of an outer layer of high-density polyethylene and an inner layer of soft PVC which has multiple perforations allowing urine flow. It is designed to prevent backflow to some degree. The receptacle has a diameter of 45 mm and does not put pressure on the penis.
Results: In the present study, we tested the system as adapted for wheelchair and bedridden users, and in both situations it resolved the problems the patients had faced prior to using the system, and improved their QOL. Patients have also used the device successfully when going out of the house and no longer need to worry about how to handle urination when away from home. There were no adverse events preventing continued use of the system and the patients were using them comfortably. Sometimes while at work, the patient was bothered by an ammoniac smell, but was able to solve this problem by placing a ceramic, non-woven sheet with deodorant capability over the receptacle. There was much less skin trouble than with a condom catheter and it was much more comfortable to wear. Use of this system incurs an additional annual cost to the patient, but both patients were very satisfied and intend to continue using the system. For the patient who had used diapers, a cost saving was also achieved.
Conclusion: The new system for managing male urinary continence is very useful for ALS patients at all stages of the disease, and in the diverse situations of their daily lives.
P274 MULTIDISCIPLINARY CARE FOR MOTOR NEURONE DISEASE
NG L1, KHAN F1,2, MATHERS S3
1The Royal Melbourne Hospital, Parkville, Victoria, Australia,2University of Melbourne, Carlton, Victoria, Australia,3Bethlehem Hospital, Caulfield, Victoria, Australia
Email address for correspondence: [email protected]
Keywords: multidisciplinary care, quality of life, rehabilitation
Background: Multidisciplinary care (MDC) is an important means of symptomatic and supportive management for motor neurone disease (MND) but the evidence base for its effectiveness is unclear.
Objectives: To assess the effectiveness of MDC in adults with MND, especially in the types of approaches that are effective (settings, intensity) and the outcomes that are affected.
Methods:Search strategy: We searched the Cochrane Neuromuscular Disease Group Trials Register, Cochrane Central Register of Controlled Trials (CENTRAL 1966-present), MEDLINE (1966-present), EMBASE (1980-present), CINAHL (1982-present), AMED (1985-present) and LILACS (1982-present). Selection Criteria: Randomised and controlled clinical trials (RCTs, CCTs) that compared MDC in MND/ALS with either routinely available local services or lower levels of intervention; or studies that compared MDC in different settings or at different levels of intensity. Trials of ‘other designs’ (ODs) (such as observational studies) were also included with the understanding that such trials could only be of limited contribution to the best evidence synthesis. Data collection and analysis: A “best evidence” synthesis based on methodological quality was performed. Studies were grouped in terms of setting and intensity (high or low) of therapy.
Results: No RCTs /CCTs were identified. Five ODs, three of ‘low quality’ and two of ‘very low quality’ based on methodological quality assessment were analysed. There was ‘very low level quality’ evidence for MDC in low intensity outpatient settings for improvement in the mental health domain of quality of life (QoL). There was ‘low level quality’ evidence for hospitalisation in terms of reduced hospital length of stay (LOS) and fewer re-admissions; and conflicting evidence regarding survival outcomes (two studies demonstrated improved survival and one did not). As for high intensity MDC, there was ‘very low level’ evidence for improvement in impairment and disability.
Discussion and Conclusions: In the absence of RCTs/CCTs, the “best” evidence to date is based on ‘low’ to ‘very low quality’ ODs. These suggest an advantage for mental health domains (only) of QoL, reduced hospitalisation, and improved disability for people with MND. The evidence for survival is conflicting. These conclusions are tentative, and need further research into appropriate study designs; outcome measurement; carer needs; and the evaluation of optimal settings, type, intensity/frequency and cost effectiveness of MDC in this population. Future research should focus on observational designs (Practice Clinical Trials), to assess care and outcomes in “real-life” settings. The interface between neurology, rehabilitation and palliative care should be explored to provide long-term support for MND.
P275 INDEPENDENT LIVING FOR SPINAL MUSCULAR ATROPHY PATIENTS: MENTAL CARE FOR PATIENTS LEAVING LONG-TERM HOSPITALIZATION
ITO K1, KAWAGUCHI Y1, KAWAHARA H1
1Ritsumeikan University, Kyoto, Japan,2Japanese ALS Society, Tokyo, Japan,3National Hospital Organization, Yakumo Hospital, Hokkaido, Japan
Email address for correspondence: [email protected]
Keywords: SMA, long-term hospitalization, independent living
Background: In Japan, patients with degenerative motor neuron diseases such as Amyotrophic Lateral Sclerosis (ALS) or Spinal Muscle Atrophy (SMA) have tended to be regarded as people with a critical illness. As a result, such patients have sometimes had to live in facilities or hospitals for years or even decades if their families could not take care of them. Moreover, some patients have had to live in the hospital from childhood in order to attend school there. In a recent change, however, such patients are beginning to be considered as people with a severe disability, and they are being given the opportunity to live independently outside of hospitals or institutions. Nevertheless, no manual or method has yet been established for providing the necessary mental support to such patients, both before and after leaving the hospital, to enable them to live independent lives.
Objectives: We attempt to outline the mental support necessary for enabling long-term hospitalized patients to leave hospitals and live independently. These patients should receive counselling and training to learn how to communicate in society, go shopping, do the washing, cook their meals and perform other aspects of independent living.
Method: To investigate the life of SMA patients after long-term hospitalization, we conducted interviews with 5 SMA patients living independently in society. We also conducted a questionnaire survey of disabled people who live independently in society after leaving institutions or hospitals. In addition, to make a model case, we conducted research about a long-term hospitalized patient's actual departure from hospital.
Discussion and Conclusions: It seems that, before leaving the hospital, patients worry about their future caretakers and economic condition, and, after leaving the hospital, they worry about their relationships with their caretakers. When patients with a neurological disorder are in a bad condition, they absolutely require proper care from caretakers. In such situations, they seem to worry whether caretakers can understand how to take care of their special neurological disorder, because few caretakers have experience with severely disabled patients. Taking care of patients with a neurological disorder is extremely confusing, because it is difficult to understand their social life and how to talk with them in some situations. However, they can be independent with the support of others. Many patients want to live in their own home, not in a hospital or an institution. Mental support is necessary so that all patients who want to live independently in society can do so.
P276 ‘WHEN SOMEONE CLOSE HAS MND’: NEW RESOURCE FOR CHILDREN
SCHILLERSTROM S, GALE L
MND Association, Northampton, United Kingdom
Email address for correspondence: [email protected]
Keywords: interactive, workbook, children
Background: MND is a distressing disease for children to grasp. They may feel inquisitive, but fearful of asking questions that might upset anyone. When reviewing MND Association literature for children, we wanted to make sure new literature reflected the needs of the child.
Objectives: To create a resource to support children from 4–10 years affected by MND in their family. Acknowledging that ‘one size doesn't fit all’, we wanted a resource that could be adapted and personalised to meet individual needs, and would create opportunities for children to ask questions and take part in discussion.
Methods: A collaborative project between the MND Association and Trinity Hospice, London. Extensive evidence based research (1–3) established how children learn, develop thinking skills, reflect upon information presented to them, and mechanisms children use to help them cope.
Results: We developed the interactive work book ‘When someone close has MND' as a way of encouraging children to identify and develop their own coping strategies. The introductory section of the work book includes information for adults about how they can best support a child when an adult is seriously ill (4–6). As it wasn't appropriate to involve children between 4–10 years to participate in the development of this resource, we approached people aged 15–23 years who had previously lost someone close to MND.
This brightly illustrated resource is presented in loose leaf format so adults can introduce information as and when it becomes timely, relevant and appropriate, allowing pacing of information, which is best practice when supporting a child when someone close to them is seriously ill. This format allows it to be personalised for individual needs.
This new resource will enable people affected by MND to talk about the things that really matter, one step at a time, to meet the needs of the child. We will measure the success of this resource by seeking feedback from adults and children who request it.
References:
- Chowns G, End of Life Care 2009;3(1): 41–47
- Dunning S, Clinical Social Work Journal 2006;34(4): 499–513
- Neilson S and Clifford F, editors. Motor Neurone Disease. London: Class Publishing 2003
- Oliver, D, editor. Motor Neurone Disease: A family affair. London: Sheldon Press 1995
- Rauch K and Muriel A, editors. Raising an emotionally healthy child when a parent is sick. London: McGraw-Hill 2006
- Stokes, J. and Stubbs, D, editors. As big as it gets: supporting a child when a parent is seriously ill. Gloucester: Winston’s Wish 2007
P277 THE EXPERIENCES OF HOSPICE NURSES WHO CARE FOR PEOPLE WITH MOTOR NEURONE DISEASE
CUTLER G
Keele University, Staffordshire, United Kingdom
Email address for correspondence: [email protected]
Keywords: caring, nursing, counselling
Background: The study has been designed to examine the relationship which develops between the person with MND and their nurses. The patients reported that the relationship they had with their nurses sustained them and fostered a sense of hope despite the ongoing nature of their difficulties. This study examines the experiences of Registered General Nurses (RGN) who work in a hospice and have cared for people who have Motor Neurone Disease (MND).
Method: A qualitative approach was used to capture the experiences of a purposeful sample of RGNs who had the required experience. Six nurses were interviewed, using a semi structured interview. The interviews were recorded, and then transcribed. The data was analysed using Interpretive Phenomenological Analysis (IPA).
Results: Six themes emerged from the data; they were: 1) the nature of the disease progression for the patient with MND; 2) the nature of suffering in the patient with MND; 3) the impact that communication impairment had on the nurse-patient relationship; 4) the nursing approach used and the skills required to care for the patient with MND; 5) conflicts in care which arose for the nurses when caring for a patient in a hospice environment; 6) the challenges and the rewards which arose for the nurse when caring for the patient with MND. On deeper analysis a master theme emerged linking all the other themes. This was the nurse-patient relationship.
Discussion and Conclusion: Findings demonstrate that the nurses in the study found the experience of caring for the patient with MND challenging and very satisfying. The intensity and complexity of the experience, combined with the difficulties of working with people who have severe communication impairment meant that they had to call on all of their nursing skills to meet the challenges. The nurses found that working in supportive nursing and multidisciplinary teams encouraged them to use skills of emotional intelligence and intuition. This experience has meant that they are able to establish deeper, more satisfying relationships with other patients.
Recommendations from the study include the recognition that nurses are able to establish and maintain therapeutic relationships with their patients. This leads to positive outcomes in patient care, including support during times of distress and emotional anguish. Nurses would benefit from using a model such as person-centred counselling to understand the importance of the relationship, in supporting adaptation to illness, disability and dying. Nurse educators need to focus on fostering broader nursing expertise than competencies and skills with an acknowledgement that depth of relationship improves patient outcomes.
P278 CARING FOR THE CAREGIVER PART 2: EVALUATION OF AN ALS CAREGIVER ASSESSMENT FORM FOR A MULTIDISCIPLINARY ALS CLINIC
STEPHENS HE1, WALSH S2, BREMER B3, SIMMONS Z1
1Penn State Hershey Medical Center, Hershey, Pennsylvania, United States,2ALS Association, Greater Philadelphia Chapter, Harrisburg, Pennsylvania, United States,3Penn State University, Harrisburg, Pennsylvania, United States
Email address for correspondence: [email protected]
Keywords: caregiving, assessment, quality of life
Background: The family caregiver is heavily involved in direct and indirect caregiving activities of the patient with ALS. These activities include assisting with activities of daily living, involvement in medical care planning and decision making, use of medical devices, and providing emotional support. Caregivers are often burdened by the volume and intensity of the ALS experience and require professional assessment and intervention.
Objectives: We will report on the evaluation of a Caregiver Assessment Form in a multidisciplinary ALS Clinic.
Methods: A team of ALS clinicians and researchers used the evidence based practice (EBP) method to develop a Caregiver Assessment Form containing questions about demographics, concerns with caregiving tasks, health and well-being, and supportive activities. The form was mailed to caregivers prior to the ALS Clinic visit, and the completed questionnaire was made available to clinicians during clinic. Caregiver exit interviews were conducted to evaluate the content of the instrument and its perceived ability to meet caregiver needs. Interviews with clinicians were completed to evaluate the clinical utility of the instrument for ALS Clinic.
Results: The assessment form was completed by 39 (90%) of the caregivers who were asked to do so. 84% of caregivers found the tool easy to use. 80% believed that content in the form was appropriate. Completing the assessment form helped 46% identify caregiving concerns about which they were unaware. 92% felt that their concerns were addressed satisfactorily. 92% thought that the addition of a caregiver assessment form would improve the support received from the clinical team. The evaluation of the form by the clinical team members showed that 79% felt that the form helped them to assess the needs of the caregiver. Clinicians indicated that they would use the tool to get the “big picture” of what the caregivers are dealing with, and to help focus the visit on high concern areas related to patient management. A time constraint was identified, with only 50% of clinicians reporting that they were able to review the tool prior to visiting the patient and caregiver.
Discussion and Conclusions: The caregiver assessment form was positively received by caregivers and the clinical team. The addition of a caregiver assessment form to a multidisciplinary ALS clinic appears to have enhanced the caregivers’ and the clinicians’ ability to identify caregiver needs as they relate to the care of the patient with ALS. A caregiver assessment form is an intervention that can be used to support caregivers of patients with ALS.
P279 THE PERSONAL EXPERIENCE OF CARERS OF INDIVIDUALS WITH MOTOR NEURONE DISEASE (MND) AND THEIR EXPERIENCES OF SERVICES
Smith H
University of Birmingham, Birmingham, United Kingdom
Email address for correspondence: [email protected]
Keywords: caregiving, couples, interpretive phenomenology
Background: Carers play an essential role in the lives of people suffering from chronic health problems. Understanding the concepts related to caregiving experiences and the relationships among them can enable us to better address the needs of caregivers. MND raises many of the issues relevant to caring for people with chronic disabling conditions, and yet there is a lack of research exploring the experiences of those assisting someone with MND. The findings to date are diverse and sometimes contradictory.
Objectives: To find out what it is like to experience having a partner with MND, how services are experienced, and the meanings people give to these experiences. To understand, interpret and contextualise the detailed accounts of eight individuals. To use the knowledge gained from this study to improve the provision of services provided to individuals with MND and their carers, and enhance the general body of research on carers and their needs.
Methods: Eight semi-structured interviews were carried out and transcripts were analysed from an Interpretative Phenomenological perspective.
Results: The main themes are: 1) impact on life, which is broken down into: needing to be strong for partner, concern for partner's safety, impact on relationship, anger and frustration, physical tiredness, and uncertainty around the future; 2) adjusting to the situation, which includes: experience of services, a problem-solving approach to practical difficulties, living day-to-day, adapting to lifestyle changes, and ability to remain positive.
Discussion and Conclusions: From the results, the need to be strong for partners was evident, as well as worries for partners’ safety. Physical tiredness as a result of caring for their partners and uncertainty around the future were also themes in all accounts. Individual differences were evident around the impact on their relationships; some experienced a loss of intimacy more than others and the role of caring for their partner appeared to change the dynamics of the relationship for some and not others. Anger and frustration were also clear in some of the descriptions, but not all. Experiences of services were varied, although most people described negative experiences around receiving the diagnosis and felt well supported by the professionals at the specialist MND clinic. A problem-solving approach to practical difficulties appeared to help in coping with the emotional impact around their partner's deterioration, as well as living from day-to-day. Participants varied in their ability to adapt to lifestyle changes, and in their ability to remain positive.
This study has highlighted some important aspects of the participants' perspectives in relation to having a partner with MND, and it is clear that services around people with MND need to review the support offered to carers.
P280 WHY DON'T PEOPLE WITH ALS/MND ACCESS SOCIAL SERVICES CARE AND WHAT IMPACT DOES THIS HAVE ON CARERS?
O'BRIEN M1, WHITEHEAD B1,2, MITCHELL D2, CALLAGHER P2, JACK B1
1Edge Hill University, Lancashire, United Kingdom, 2Lancashire Teaching Hospitals NHS Foundation Trust, Preston, United Kingdom
Email address for correspondence: [email protected]
Keywords: social services care, carer burden, support
Background: A descriptive audit of the key characteristics of the MND population in a large UK specialist MND clinic was undertaken in 2008 as part of a larger study exploring the personal experience of living with MND (1). The audit revealed that a significant number of patients and carers were not accessing external caring support provided by local social services.
Objectives: The aims of this part of the study were to identify the reasons for limited care input from social services and to explore carers’ experiences of care services.
Methods: Narrative interview data were collected from people with MND (n = 24) and their spouses or partners (n = 18). Thematic analysis has been aided by NVIVO.
Results: A number of themes relating to experiences of caring have been highlighted in the interview data and a variety of reasons for limited external care provision have been reported. These include carers’ and patients’ lack of information or understanding of the roles of services; issues around the allocation of social workers; carers’ and patients’ desire to maintain a sense of normality; reluctance to surrender privacy or control of home lives. Carers report feelings of anxiety or guilt when considering accessing care outside of the home. A number of individuals have reported lack of consistency and organization of care agencies, poor standards of care and limited understanding and training in MND. Carers report experiencing significant levels of emotional and physical exhaustion and a lack of social and psychological support, training and information available to them.
Conclusions: This study has provided detailed information regarding the uptake of care services by the current MND population followed up by a specialist UK MND clinic. It explains to some extent why patients and carers are not accessing services and highlights a number of the issues faced by carers of people with MND.
Reference:
- O’Brien M, Whitehead B, Mitchell D et al. ALS 2008; 9 (Supp 1): 147
P281 SELF-PERCEIVED BURDEN AND A SPIRITUALITY OF INTERDEPENDENCE
STANG V
The Ottawa Hospital Rehabilitation Centre, Ottawa, Ontario, Canada
Email address for correspondence: [email protected]
Keywords: burden, spirituality, interdependence
Background: People living with ALS/MND can sometimes perceive themselves as burdensome to their families and society. Their sense of self is diminished by the many losses that come with the diagnosis and disease. This can lead to spiritual distress. They may feel unworthy of the care of others or do not want to trouble others with their problems. They may also be afraid of suffering. A spirituality of interdependence can offer a new direction by helping people with ALS/MND to understand their experience in the context of relationships rather than individualism.
Objectives: This presentation will explore the phenomenon of feeling like a burden from a spiritual perspective. Reasons for feeling burdensome to others will be explored and a framework of spiritual care interventions based on a theology of interdependence will be offered.
Methods: A review of the psychological literature on self-perceived burden will be presented. Case studies highlighting the experience of feeling burdensome to others will be shared. Some of the possible outcomes for the person with ALS/MND will be identified. Feeling like a burden can lead to varying degrees of spiritual distress including the desire for altruistic suicide.
Results: Spiritual care interventions can address the feeling of being a burden to others. Spiritual discussions can include exploring a person's sense of self-Who am I now with this ALS diagnosis?- as well as a person's sense of relationship with others. A theology of interdependence will explore what it means to “bear one another's burdens” (Galatians 6:2). This passage from Scripture means not merely to “tolerate” or “put up with” but rather, to “uphold lovingly.” To uphold lovingly one who is struggling with feeling a burden can be done by significant others as well as a community. Cultivating community requires that people with ALS and their families are open to community and build their relationships.
Discussion and Conclusion: It is difficult to come to a place of acceptance of one's own vulnerability and interdependence with others, especially in the North American culture which values individual autonomy so highly. Being in relationships with others emphasizes a theology of interdependence: we give and receive in our human interactions. The blessing of community whether it is a health care, ALS or faith community will be discussed. Other theological themes will be presented including some insights from Jewish theologian Emmanuel Levinas and philosopher Paul Ricoeur.
Feeling a burden is a reality for some people living with ALS/MND. Spiritual resources can be drawn upon to help people with ALS/MND address this struggle, support their sense of hope and improve their quality of life.
P282 SATISFACTION OF LIFE AMONG PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS, WITH AND WITHOUT LONG-TERM MECHANICAL VENTILATION: A PILOT STUDY
LORIO R1, PALMIERI A2, VIANELLO A3, SORARÚ G4, VOLPATO C1, LAZZARINI L1, MENEGHELLO F1, TONIN P1, PICCIONE F1
1IRCCS San Camillo Hospital, Venice, Italy;2Department of Neurosciences,3Department of Respiratory Physiopathology4Depatment of Neuropathology; Padua University, Padua, Italy
Email address for correspondence: [email protected]
Keywords: depression, life satisfaction
Background: Previous data suggest that life satisfaction may be poor for individuals with ALS who are administered tracheostomy and long-term mechanical ventilation (LTMV) at the time of a respiratory crisis. In such a situation, this aggressive ventilatory approach may lead to an uncertain and sometimes unwanted prolongation of life. Moreover, the dependence on ventilatory support may determine a dramatic physical, psychological and economic burden on family and caregivers causing a sense of guilt in patients’ psychological status.
Objectives: To further investigate the impact of tracheostomy on patients’ mood tone, satisfaction of life, and religious beliefs on this condition.
Methods: We administered the Beck Depression Inventory (BDI), Life-Satisfaction Questionnaire (LSI-11) and Royal Free Interview (RFI) to 13 ALS patients with LTMV compared to 32 ALS patients without LTMV, of comparable for demographic variables. All patients (45) were non-demented.
Results: Despite major physical limitations, no significant differences (p < 0.05) were found between ALS patients with LTMV and ALS patients without LTMV in terms of BDI, LSI-11 and RFI scores. BDI data show only 15% of our LTMV patients and 9% of patients who did not receive LTMV admitted severe depressive symptoms. Satisfaction of life appeared highly comparable with the normal population for both ALS groups. The two ALS groups were similar for religious beliefs.
Discussion: Although depression can occur in ALS patients, good satisfaction with life is observed both in ALS groups, with and without LTMV. Religious beliefs and probably social support are positive factors for both groups of patients. Our findings might help physicians to face the challenging ethical dilemma associated with the management of ALS patients who acutely require LTMV or who require detailed information about LTMV treatment. Questions can arise about the psychological impact of unplanned tracheostomy.
P283 THE ROLE OF BURDEN, DEPRESSION, ANXIETY AND SOCIAL SUPPORT IN ALS CARE
PAGNINI F1,2, ROSSI G3, LUNETTA C3, GRANATIERO O1,2, GORNI K3, CASTELNUOVO G1, CORBO M3, MOLINARI E1
1Department of Psychology, Catholic University of Milan, Milan, Italy,2PhD Program in Clinical Psychology, University of Bergamo, Bergamo, Italy,3NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Ospedale Niguarda Cà Granda, Milan, Italy
Email address for correspondence: [email protected]
Keywords: care burden, caregivers, social support
Background: The natural history of ALS patients is associated with an increasing burden experienced by their caregivers. There are still few studies that have investigated the emotional impact of ALS care.
Objectives: We aim to evaluate the relationships between burden and psychological aspects in ALS caregivers and the impact of social support.
Methods: We conducted a cross-sectional study among 30 ALS caregivers, using Zarit Burden Inventory to assess burden of care, Beck Depression Inventory-II for depression, State-Trait Anxiety Inventory for anxiety, Social Support sub-scale from the McGill Quality of Life Questionnaire and ALSFRS-r for patients’ severity of disease.
Results: Caregivers’ burden, depression and anxiety levels were positively related to each other, with a negative correlation with perception of social support. Patients’ illness severity was correlated with caregivers’ burden, anxiety and somatic expression of depression. Caregivers expressed worries only for their own health conditions.
Discussion and Conclusion: In our study we showed that burden is directly related with psychological difficulties in ALS caregivers, increasing together with the patient's disability. Furthermore, social support provides a positive effect to limit the worsening of burden, depression and anxiety experienced by the caregiver.
P284 EYE-TRACKING COMMUNICATION SYSTEM: IMPACT ON QUALITY OF LIFE AND MOOD IN PATIENTS WITH ALS IN LOCKED-IN SYNDROME
CALVO A1, PASIAN V1,2, MOGLIA C1, BALMA M1, MONTUSCHI A1,2, CORNO F3, CHIÒ A1
1ALS Center, Department of Neurology, University of Torino, Torino, Italy,2APASLA, Torino, Italy,3Department of Automatic and Informatic, Politechnic University, Torino, Italy
Email address for correspondence: [email protected]
Keywords: eye-tracking system, communication, quality of life
Background: During the course of ALS the ability to communicate is progressively impaired, up to the complete loss of useful communication. There are no studies on quality of life (QoL) and psychological aspects related to the eye-gaze system of communication in ALS patients in locked-in syndrome.
Objectives: To assess the impact on QoL and mood of an eye-tracking system (ETS) for communication in a series of ALS patients with locked-in syndrome.
Methods: Nine tracheostomized ALS patients (7 males; mean age 43.8, range 29–61), without clinical cognitive impairment, who use an ETS daily, underwent a questionnaire specifically created by our ALS Centre, focused on the qualitative-organizing aspects from the patients’ perspective; they were also administered the McGill Quality of life questionnaire (MQoL), the Zung Depression Scale (ZDS), and the Satisfaction with Life Scale (SWLS). The questionnaires were sent to the patients and returned by email. MQoL, ZDS and SWLS had already been administered to the patients 2 to 5 months before tracheostomy.
Results: All patients were able to fill in the questionnaires using the ETS without the help of their caregivers. All patients stated that the use of an ETS improved their life conditions and also their caregiver's activity; moreover patients felt that after the availability of the ETS they could be in contact with the surrounding world again. The major problems reported by patients were tiredness related to the prolonged use of the ETS (7 patients) and early difficulties in using the ETS related to their poor previous experience with computer technologies (4 patients). In 7 patients QoL, as well as level of depression, improved after the use of the eye-tracking system; only two patients were still depressed and reported a low level of QoL, although after the use of ETS both ratings improved compared to the evaluation performed before tracheostomy. SWLS score showed a high variability.
Conclusion: Eye-tracking systems appear to be effective in improving QoL and mood in locked-in ALS patients and their caregivers. Moreover, they effectively increase the communication between patients and caregivers and give to patients the feeling of being able to better control their lives.
P285 A COMPARISON OF EYE-GAZE COMMUNICATION SYSTEMS AND A PROTOCOL FOR ASSESSMENT
WRIGHT A1, CLARKE C2
1Carolinas Neuromuscular/ ALS/MDA Center, Charlotte, NC, United States,2Assistive Technology Works Inc, Staunton, VA, United States
Email address for correspondence: [email protected]
Keywords: eye-gaze, communication, technology
Background: Speech pathologists who work with patients diagnosed with ALS/MND frequently recommend Augmentative/Alternative Communication equipment. These recommendations should identify the most appropriate system based on trials with several different speech generating devices. With regard to eye-gaze technology, new systems have recently been introduced and few professionals have had hands-on experience with all of them. Each of these systems employ complex eye-tracking technology which can be impacted by a number of variables. In addition, eye-gaze system set-up and troubleshooting can be more involved than that with other systems or access methods. When eye-gaze is determined to be the most reliable access method for a patient who requires a speech generating device, one must take many different factors into consideration to ensure user success.
Objectives: To describe the differences between different types of video-based eye-tracking technology, introduce a protocol for eye-gaze evaluations and discuss patient/caregiver feedback.
Methods: To clearly define the different eye-gaze technologies available, a review of literature on eye tracking methodology will be completed. A protocol for conducting eye-gaze evaluations with ALS/MND patients will be utilized to assess patient interaction with 6 different eye-gaze communication systems. Each system will be rated according to a number of variables including the following: 1) simplicity of calibration, 2) speed and accuracy of communication at word, phrase and sentence level (communication software features & functionality), 3) functionality and ease of use for internet access, email and other applications, 4) length of time needed from initial introduction to functional use of the system.
Results: How differences in eye-tracking methodology affect the patient evaluation process and patient satisfaction will be discussed. Patient feedback and anecdotal evidence will be reported. Expected outcomes include varied patient success and preference based on ease of use, comfort/experience with technology, educational and financial status, expectations and communication, environmental control and computer access needs. Results of a patient/caregiver questionnaire will be presented to summarize concerns specific to each individual.
Discussion and Conclusions: Based on our experience, eye-gaze systems can be an ideal communication solution for patients with ALS provided a thorough assessment is completed and the most appropriate system is obtained. It is recommended that speech pathologists working with this patient population become familiar with the different types of eye-gaze technology and perform evaluative trials whenever possible.
P286 COMMUNICATION: HOW DO PEOPLE REALLY FEEL ABOUT THE LOSS?
WHITE M
Chatsworth Rehabilitation Centre, Mansfield, Nottinghamshire, United Kingdom
Email address for correspondence: [email protected]
Keywords: communication, feelings, therapy
Background: The author's experience with MND and other neurological progressive disorders (PND) led to the conclusion that what is shown to others by those who are communication impaired is only the tip of a deep pain and despair which the client is unable to fully articulate. Communication is not just a means of satisfying wants and physical needs, but is also necessary to have some control over our lives and what happens to us.
Objectives: This study aimed to research the lived experience of communication impairment and to attempt to uncover the true feelings of clients and carers about the communication problems in MND and other PNDs. A deeper understanding of the problems should lead to more effective Speech & Language Therapy management and treatment services, which would be based on the expressed needs and views of the people using them.
Methods: A qualitative approach which used in-depth interviews with 4 people with MND and 2 others who had progressive neurological conditions, and their main informal carer. These interviews were taped and transcribed and content analysis used to identify themes which were further allocated to overt and covert themes. Because many of the clients had extremely limited communication, ‘vignettes' were written from the interview with each participant to attempt to describe their feelings. This was verified as correct by one patient and carer as a control to ensure the extracted meanings were correct.
Results: Both overt and covert themes were uncovered. Overt themes involve those which became apparent from the conversations with clients and carers. Covert themes are those which were not actually discussed openly, but were meanings embedded in discussion, and uncovered by continuous analysis of the transcripts.
Discussion and Conclusions: Overt themes included the overall difficulties of loss of communication and the clients’ consequent feelings of guilt and self-blame; covert themes related to the reactions to this loss and the adjustment and coping strategies utilised by both client and carer. Functional and emotional communication were major issues in this study, but also an important quality of life issue was uncovered which relates to social communication, and how the loss of this leads the person to a depleted quality of life and on a pathway to isolation. This led to curtailment and eventual inability of the person not only to control their lives and express their emotions but also to play a part in society. This study has given a greater understanding of the true feelings of both client and carer with regards to communication and has also altered the therapeutic approach offered, by the introduction of a simple ‘life map' method of managing communication deterioration.
P287 SPEECH INTELLIGIBILITY AND MARITAL COMMUNICATION IN MND
JOUBERT K1, BORNMAN J2
1University of the Witwatersrand, Johannesburg, South Africa,2University of Pretoria, Pretoria, South Africa
Email address for correspondence: [email protected]
Keywords: speech intelligibility, marital communication, augmentative and alternative communication
Background: The onset of a progressive, terminal illness such as Motor Neuron Disease (MND) inevitably results in physical and communication disabilities that impinge on the individual's ability to remain functionally independent. The loss of speech as a result of dysarthria, a motor speech disorder, is one of the most profound changes that the person with MND will experience. The decline in the individual's speech intelligibility, which negatively influences communication effectiveness, implies that in 80% of cases alternative and augmentative communication (AAC) strategies are required to support the daily communication needs of individuals with MND.
The dyadic nature of chronic illness implies that multiple aspects of one of the most important adult relationships, marriage, will be affected. Roles and responsibilities performed by each member of the couple will continually change as the disease progresses. The emotional trauma of adjusting to the unavoidable alteration in their relationship elicits strong emotions such as guilt, anger and frustration. Communication is the one of the most constructive ways of dealing with these emotions. The ability of spouses to convey their innermost thoughts, feelings and intimacy through communicative interaction is vitally important in marital communication.
Objectives: The aim of this study was to determine the association between the deteriorating speech of people with MND and the couple's perception of marital communication.
Methods: Fourteen couples (n = 28) divided into two participant groups, people with MND and spouses, participated in this non-experimental correlational research study. Data were collected during three visits at six-monthly intervals over a 12 month period. At each of these visits both participant groups completed a variety of objective and subjective measures, of which 20% were inter-rated by independent raters.
Results: Results confirmed the inevitable decline in speech intelligibility of people with MND across the disease progression. The people with MND did not report a change in their perception of marital communication although their spouses indicated a statistically significant decrease between the first and last visits. Interestingly, there was not a statistically significant relationship between the deteriorating speech of people with MND and the couples’ perception of marital communication.
Conclusions: It is evident from this study that marital communication is not influenced by the deteriorating speech of people with MND. Even in the face of loss of speech, communication as a means of facilitating and maintaining the marital relationship is particularly pertinent to people with MND and their spouses. Health professionals should therefore take a holistic approach to provide timely and appropriate communication interventions that not only address the communication needs of the individual with MND but also consider the needs of their spouse to ensure successful intimate communication for the couple.
P288 ATTITUDES TOWARD LIFE SUSTAINING TREATMENT IN ALS-PATIENTS
NONNEMACHER S1, SORG S2, LULÉ D2, LUDOLPH AC2, HAUTZINGER M1, KÜBLER A1,3
1Institute of Medical Psychology and Behavioral Neurobiology, Tübingen, Germany,2Department of Neurology, University of Ulm, Ulm, Germany,3Biological Psychology, Clinical Psychology, and Psychotherapy, Würzburg, Germany
Email address for correspondence: [email protected]
Keywords: life sustaining treatment, education, decision process
Background: In Germany there is no information about the frequency of invasive ventilatory treatment for ALS patients. Neurological centers mention percentages between 5%–7%. It is known that despite the physical impairment, the patients adapt to the progressing paralysis and experience a high quality of life. However, there is no information about how attitudes toward life sustaining treatment (LST) change in relation to the process of adaptation.
Objectives: The goal of the present explorative study was to measure patients’ attitudes toward life sustaining treatment and toward end-of-life (EOL) issues.
Methods: The newly developed Life Sustaining Treatment Questionnaire (LSTQ) was applied. It comprised 115 descriptive items, distributed across attitudes toward four topics: (1) artificial nutrition, (2) respiratory mask, (3) tracheal ventilation (tracheostomy), and (4) EOL issues like death and euthanasia. The first three topics also included questions about the stability of attitudes; what kind of, and whether, decisions have been made; the reasons and certainty of these decisions; the information received; the persuasion experienced, and feeling of being a burden. Response options were either dichotomic, or on a Likert-scale (4 grades), multiple choice or open.
Results: Fifty-four ALS-patients were interviewed in their homes. Interviews lasted anything between one and two hours dependent on the patient's physical condition. Forty-four percent received sufficient information on artificial nutrition, 6% were persuaded to agree to this treatment, 24% made a decision for the treatment, 56% have not yet made a decision and 95% who had made a decision felt certain about it. Sixty percent of the patients received sufficient information about non-invasive artificial ventilation, 6% were persuaded to agree to this treatment, 57% decided for the treatment, 39% had not made a decision yet and 93% who had made a decision felt certain about it. Twenty-five percent received sufficient information about invasive ventilation, 5% were persuaded to agree to this treatment, 8% decided for the treatment, 60% had not made a decision yet and 84% who had made a decision felt certain about it.
Discussion and Conclusion: The acceptance of the LSTQ was high. The patients felt that questions were accurately verbalized and encouraged them to reflect about the disease. 8% refused the EOL questions because of emotional stress. In conclusion, ALS-patients received insufficient information about LST particularly with regards to invasive ventilation. Better information may reduce uncertainty and support coping. Further, mostly the decisions were not influenced by others. The majority of those who had made a decision felt certain about it which underlines the notion that the decision for LST is a good predictor for coping with a life dependent on sustaining treatment.
P289 ATTITUDES OF ALS PATIENTS WITH REGARDS TO END-OF-LIFE ISSUES
KICK E1, NONNENMACHER S1, HAUTZINGER M1, LUDOLF AC2, KÜBLER A3,1
1Institute of Medical Psychology and Behavioural Neurobiology, University Tübingen, Tübingen, Germany,2Department of Neurology, University of Ulm, Ulm, Germany,3Biological Psychology, Clinical Psychology, and Psychotherapy, University Würzburg, Würzburg, Germany
Email address for correspondence: [email protected]
Keywords: end-of-life, advance directive, hastened death
Background: ALS-patients are confronted with end-of life (EOL) issues because of the progress of the disease. However, to date there has not been any specific information about how these people experience this process emotionally and cognitively.
Objectives: The goal of the current study was to measure attitudes of ALS patients with regard to EOL issues. Furthermore, the study intended to find those aspects which are the basis of these attitudes.
Methods: Thirty-one ALS-patients participated in the study. The following measures were administered: socio-demographical and disease related data, wish to hasten death (WTHD) by the “Schedule towards Hastened Death” (SAHD), quality of life by the “Schedule for the Evaluation of Individual Quality of Life” (SEIQoL-DW) and EOL issues by the newly developed “Life Sustaining Treatment Questionnaire” (LSTQ; Häcker, 2008, unpublished).
Results: Average SEIQoL scores were in the range of non-terminal chronic diseases (M = 67.47±12.26). The sample was split into two groups by using the criterion WTHD existent (WTHD = SAHD cumulative value of the patient > mean of SAHD + 1 standard deviation) or non-existent. There were no differences between these two groups with regards to their socio-demographical or disease related data as well as their quality of life. However, patients with WTHD mentioned “Autonomy”, measured by the SEIQoL-DW, significantly more often as important for their QoL than those without WTHD (Chi2=6.62, p = 0.03). Fifty-one per cent stated that they experienced fear of death. Those who were afraid of dying showed a higher fear of suffocating (Chi2=44.05; p = 0.00) than the rest of the patients. Twenty-nine per cent of the patients had advance directives (AD). Sixty-four per cent of those patients without AD explained that they trust in significant others' abilities to represent their will. Fifty-five per cent stated that they were unable to make at this point in time the right decision with regards to the future. The approval of active euthanasia was correlated to the frequency (r = 0.37; p < 0.05) and intensity (r = 0.31; p = 0.07) of pain.
Discussion and Conclusions: Elucidating what determines patients’ decisions toward the end life will help to provide optimally adapted palliative care. Fear of pain was prevalent among ALS patients and may have been a reason for the positive attitude toward legalizing assisted suicide. Likewise, ALS patients were afraid of suffocation. This demonstrated an alarming lack of information about available treatments. Patients have to be provided with all possible information and treatment to keep anxiety as low as possible, because anxiety seems to lead patients to wish for a hastened death. We argue that instead of legalizing assisted suicide we have to improve information transfer, palliative and terminal care.
P290 END-OF-LIFE DECISIONS AND ADVANCE CARE DIRECTIVES IN MOTOR NEURONE DISEASE: EVIDENCE BASED GUIDELINES FOR BEST PRACTICE
CORR B1,2, HARDIMAN O1, COWMAN S2
1Beaumont Hospital, Dublin, Ireland,2R.C.S.I., Dublin, Ireland
Email address for correspondence: [email protected]
Keywords: advance care directives, end-of-life decisions, autonomy
Background: The clinical management of ALS/MND is palliative from the time of diagnosis, and is focused on symptom control and adjustment to the progressive loss of neurological function with the certainty of early death. Formal guidelines regarding advance care planning, end-of-life decisions and advance care directives in neurodegenerative disease are currently unavailable in Ireland.
Objectives: 1) To identify the attitudes, understandings and experiences of patients with ALS/MND, their carers and their health-care providers to end-of-life decisions and advance care directives. 2) To determine if the views of patients, carers and their health-care providers concur. 3) To explore the relationship and impact of self reported quality of life, on patients with ALS/MND concerning end-of-life decisions and advance care directives. 4) To make recommendations for the formulation of advance care directives and end-of-life decisions in the ALS/MND population in Ireland.
Methods: Hermeneutic Phenomenology was chosen as an appropriate methodology for this study as it illuminates the meaning and understanding of the lived experience of contemplating end-of-life decisions and advance care directives for patients with ALS/MND, their carers and their healthcare professionals. Purposive sampling was used to identify participants for this study. Participants included patients, their carers, consultant neurologists, palliative care consultants and nurses. All participants had experience pertaining to the phenomena under investigation.
Results: This project has provided valuable insight into the attitudes, understandings and experiences of Irish patients with ALS/MND, their carers and their health-care professionals towards end-of life decisions and advance care directives. It has identified areas where attitudes of patients, carers and health professionals are not concordant. The project has identified a number of evidence-based guidelines that are pertinent to the formulation of advance care directives and advance care planning, within the confines of the Irish medical and legal system, and may be applicable to patients with other terminal conditions.
Conclusions: There are many ethical dilemmas to be considered in caring for patients with ALS/MND, especially regarding nutrition and respiration. It is essential that these issues are discussed in a timely and appropriate manner. Increasing our understanding of the attitudes, experiences and difficulties for patients, their carers and their health-care professionals in discussing end-of-life issues will ultimately improve practice. It is imperative that we avoid a “tick box” scenario to end-of-life management; individualised advance care plans need to be devised. As healthcare providers we have an obligation to create an appropriate atmosphere that encourages patients and their carers to discuss their legitimate issues and fears regarding end-of-life decisions and advance care directives.
P291 PERSONAL ORIENTATIONS TO LIVING WITH ALS/MND: FINDINGS FROM THE STUDY OF UNSOLICITED WRITTEN ILLNESS NARRATIVES
O'BRIEN M1, CLARK D2
1Edge Hill University, Lancashire, United Kingdom,2Lancaster University, Lancaster, United Kingdom
Email address for correspondence: [email protected]
Keywords: coping strategy, illness narratives, personal experiences
Background: Health professionals are increasingly attempting to understand illness from the patient's perspective. Making use of illness narratives is one way of becoming more informed about the personal experience of living with illness.
Objectives: To explore the personal experience of living with ALS/MND as documented in personal illness narratives, written by people diagnosed with the illness.
Methods: We previously reported the processes used for locating published and unpublished personal illness narratives about life with ALS/MND (1). The 161 narratives identified were subject to content and thematic analysis. Data management was aided by Nvivo 7 software. A number of themes were identified from the analysis.
Results: We focus here on the theme ‘personal orientation to illness’ which is concerned with the varying approaches adopted by people with ALS/MND to manage both the physical and psychological impact of their condition. Authors write of modification as they adapt to the effects of their disability. There is tolerance in the acceptance of illness, often emerging from a period of intense turmoil. A positive attitude is emphasised in reflections on what can be done, rather than dwelling on what has been lost. The search for a cure pervades the narratives, whether through conventional, experimental or alternative treatments. Remarkably, many authors identify benefits to their diagnosis.
Conclusions: Here we have accounts that demonstrate how individuals with ALS/MND adapt to their illness; their personal strategy for coping. A variety of approaches are advocated as people undergo the transition from health to ill health. What emerges for many is a sense of bringing priorities into clearer focus and putting irrelevancies to one side. Written illness narratives provide understanding of the experience of illness; by familiarising themselves with them, those caring for the sick and dying can ‘learn how medicine looks from the patient's point of view’ (2). Stories of living with ALS/MND are regarded as excellent teaching tools, providing insight not obtainable from classic textbook descriptions (3).
References:
- O’Brien M & Clark D. ALS 2006; 7: 72
- Bregman L and Thierman L. First person mortal. Personal narratives of illness, dying and grief. New York: Paragon House, 1995
- Newrick P, Langton-Hewer R. BMJ 1984; 289 (6444): 539–542