THEME 5 MULTIDISCIPLINARY CARE AND QUALITY OF LIFE

P93 DIAPHRAGM PACING REFERRAL PATHWAY AND OUTCOMES IN AN ACADEMIC MULTIDISCIPLINARY ALS CLINIC

Raheja D¹

Walsh S²

Stephens H¹

Simmons Z¹

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

^bALS Association Greater Philadelphia Chapter, Harrisburg, PA, USA

Email address for correspondence: [email protected]

Keywords: diaphragm pacing, respiratory management, screening protocol

Background: Diaphragm pacing (DP) is FDA approved for the treatment of hypoventilation in ALS. Our center received humanitarian use device (HUD) Institutional Review Board (IRB) approval for the implementation of DP devices in ALS. We developed a DP screen based on the recommended guidelines for DP use in ALS. If the patient met the criteria, DP was discussed and written information was provided. Criteria to initiate a discussion of DP as a treatment option included the following: negative inspiratory force (NIF) < -60 cm of H₂O, forced vital capacity (FVC) > 45% of predicted (less if bulbar symptoms present), and goals of treatment (as outlined in our Communication and Treatment Preference Assessment (CTPA) indicating “life extension at all cost” or “life extension with selected treatments”. Patients indicating a goal for treatment as “comfort only” and cognitive dysfunction were excluded. If the patient met the screening criteria and indicated an interest in DP, further tests of diaphragm function and respiratory status were undertaken: 1) sniff fluoroscopy of the chest or phrenic nerve stimulation to assess diaphragm movement; 2) arterial blood gas measurement. If the X-ray was normal or showed unilateral dysfunction, or if the blood gas showed hypercarbia (pCO² > 45), patients were referred to the surgeon for further discussion and evaluation.

Objectives: Examine the referral process and outcomes for DP in a multidisciplinary ALS Clinic.

Methods: All patients meeting definite, probable, probable laboratory supported or possible ALS between April-May 2014 were screened for DP. Evaluation sheets were designed to map the DP referral process of patients seen in the clinic. Descriptive statistics were utilized to examine the referral pathway for DP.

Results: 62 patients were evaluated for DP in the ALS clinic. Of those evaluated, 16 (25%) met criteria for DP to be offered as a treatment option and were referred for further testing. 5 (8%) were eventually referred to the surgeon for discussion of DP placement. Reasons for non-referral for DP included patients wishing comfort, not extension of life (39%), abnormal cognitive screen (20%), not meeting FVC criteria (24%) or NIF criteria (33%).

Discussion and conclusion: Utilizing the recommended guidelines for screening for DP and in addition the use of a CTPA has been a useful method for which to screen for and discuss DP as a treatment option for respiratory support for patients in the ALS clinic. Using both criteria has allowed the clinic team to present treatment options to patients that are in line with their goals for care in ALS.

P94 ASSESSMENT OF BULBAR FUNCTION IN ALS

Smith RA¹

Pattee G²

Pioro E³

Schoenfeld D⁴

^cCenter for Neurologic Study, La Jolla, CA, USA

^dNeurology Associates, Lincoln, Nebraska, USA

^eCleveland Clinic, Cleveland, Ohio, USA

^fMassachusetts General Hospital, Boston, Massachusettes, USA

Email address for correspondence: [email protected]

Keywords: bulbar function, clinical trials, self-report assessments

Objective: To determine the most useful methods for assessing bulbar function in ALS.

Background: The clinical assessment of bulbar function in ALS has not attained the standard of practice that is universally employed for the assessment of limb weakness. The ongoing bulbar function treatment trial offered an opportunity to compare self-report assessments of speech, swallowing, and salivation with ‘objective’ measures such as timed recording of speech and swallowing.

Methods: At screening and baseline visits, subjects were asked to rate bulbar function using two validated self-rating scales and subsequently, an evaluator measured the time in seconds to swallow 30cc of water, a teaspoon of cereal, and to read the “Rainbow Passage”.

Results: The three domains of bulbar function - speech, salivation, and swallowing for the self-report CNS-BFS and evaluator administered ALSFRS-R were highly correlated, as were the total scores for each test instrument. Both the self-report speech domains of the CNS-BFS and the evaluator administered ALSFRS-R compare favourably with the direct measurement of the time needed for subjects to read the Rainbow Passage. An even stronger correlation was noted when speech rate was compared with the total CNS-BFS and bulbar ALSFRS-R scores. In this population of subjects, there was little correlation between speech rate and non-bulbar symptoms. Similarly, the swallowing domains of both the CNS-BFS and ALSFRS-R were highly correlated with timed swallowing of liquids but the CNS-BFS was more informative in the instance of timed swallowing of solids. As might be expected, impaired swallowing was associated with increased difficulty handling secretions in this group of patients.

Discussion and conclusion: The Center for Neurologic Study Bulbar Function Scale (CNS-BFS), a self-report measure of bulbar function, demonstrates concordance with the bulbar domains of the ALSFRS-R that is administered by an evaluator. The speech domains of the CNS-BFS and the ALSFRS-R are highly correlated with timed speech. The swallowing domain of the CNS-BFS is highly correlated with timed swallowing of solids and liquids. The swallowing domain of the ALSFRS-R is highly correlated with timed swallowing of liquids but is poorly correlated with timed swallowing of solids. With a planned enrolment of 110 ALS subjects, it is anticipated that further refinements in the CNS-BFS will result in a self-report scale that will be clinically useful and a robust treatment trial endpoint.

Acknowledgments: Study supported by: The ALS Association.

P95 5% SCOPOLAMINE OINTMENT IS CLASSICAL BUT STILL USEFUL FOR ALS AND OTHER NEUROLOGICAL DISEASES

Nagashima K^1,2

Ogino M¹

Tominaga N¹

Uchino A¹

Ogino Y^1,3

Nishiyama K¹

^gKitasato University, Sagamihara, Kanagawa, Japan

^hGunma University, Gunma, Japan

ⁱHakone Hospital, Odawara, Kanagawa, Japan

Email address for correspondence: [email protected]

Keywords: scopolamine, sialorrhoea, quality of life

Background: Sialorrhea and the aspiration of saliva often become a problem in neuromuscular diseases. The pasting agent (scopoderm TTS^®) used in the USA and EU is not approved in many other countries. Furthermore, originally Scopoderm TTS^® is for motion sickness, and it is still unclear about the drug optimum dosage for the salivation.

Objectives: To determine the usefulness and side effects of 5% scopolamine ointment for sialorrhea.

Methods: From October, 2008 to March, 2014, we prescribed 5% scopolamine ointment for sialorrhea to 156 patients with neuromuscular diseases in our hospital. We applied it approximately 0.1 g to the posterior auricles in both sides.

For this study, we analyzed 133 cases in whom we evaluated the effectiveness of scopolamine ointment by VAS scale (divided A (0): not effective, B (1∼3): moderate effective, C (4∼5): obviously effective, D (6∼): extremity effective). These cases include 91 ALS; 27 Parkinson’s disease-related disease; 7 multiple system atrophy; 5 spinocerebellar degeneration; 4 cerebral palsy; 4 SBMA; 3 muscular disorder; 3 cerebral vascular disorder; 3 cortico-basal degeneration; and 9 other neurodegenerative disease.

Results: The effectiveness in 133 cases was A:not effective 30 cases; B:moderate effective 11 cases; C: obviously effective 46 cases; D: extremity effective 46 cases. In approximately 77% of cases, 5% scopolamine ointment was effective for sialorrhea. The side effects were observed in approximately 12% of patients. These were local rash (7); tensed sputum (4); dry mouth(4) and others(2). Although only one case complained of light headedness, otherwise there was no systemic side effect.

Discussion and conclusion: Our result shows that the scopolamine ointment was useful for improvement of sialorrhea without severe side effects. We reported that in healthy subjects, blood scopolamine concentration was lower in the 5% scopolamine ointment group than the scopoderm TTS^® group. One possible explanation is that 5% scopolamine ointment shows it effects through the local site circulation, thus it causes less systemic side effects. In this point of view, 5% scopolamine ointment might have some advantages over a pasting agent (scopoderm TTS^®).Our result shows that the scopolamine ointment was an effective, noninvasive, simple, reversible and easy method.

P96 AFFECTS OF DYSPHAGIA AND GASTROSTOMY FEEDING ON QUALITY OF LIFE FOR PEOPLE WITH MOTOR NEURONE DISEASE

Doyle L

McElligott K

Hardiman O

Beaumont Hospital, Dublin, Ireland

Email address for correspondence: [email protected]

Keywords: gastrostomy, quality of life, dysphagia

Background: Dysphagia and malnutrition are factors which can negatively affect prognosis and quality of life (QOL) for people with MND. This pilot study aimed to investigate to what extent dysphagia and gastrostomy feeding impacts on QOL for people with MND.

Methods: Over an eighteen month period, people with MND who were scheduled for gastrostomy insertion in one acute hospital completed a questionnaire at three stages. Prior to tube insertion (stage 1), participants (n = 14) completed the SWAL-QOL, which is a validated questionnaire regarding the effects of dysphagia on QOL. This was repeated at approximately six weeks (stage 2) and six months (stage 3) following insertion. At stages 2 and 3, participants also completed the RIG-QOL (a questionnaire devised by the researchers to examine changes in wellbeing relating to gastrostomy feeding based on the format of the SWAL-QOL).

Results: Each participant reported an array of swallowing difficulties including choking on food and liquid as well as problems chewing. At stage 1 (N = 14), all participants felt dysphagia had a moderate or significant impact on their QOL. At stage 2 (N = 11), all reported being at least ‘somewhat’ glad they had the feeding tube inserted while at stage 3 (N = 4) all participants were ‘very’ glad that they had a gastrostomy. Every respondent reported a better sense of wellbeing following gastrostomy insertion.

Conclusion: In this pilot study, people with MND reported that gastrostomy insertion had a positive impact on their QOL. This finding supports the need for further research in this area across a larger group of participants.

P97 ALS SYMPTOMS, DISABILITY AND QUALITY OF LIFE : LITERATURE REVIEW AND MODEL GENERATION

Young C^1,2

Aynsley G²

^jWalton Centre NHS Foundation Trust, Liverpool, UK

^kUniversity of Liverpool, Liverpool, UK

Email address for correspondence: [email protected]

Keywords: quality of life, disability, literature review

Background: The limited efficacy of disease modifiers for ALS/MND focuses management on symptom control and maintaining quality of life (QoL). As yet, there is no overview of how different symptoms may influence QoL in ALS/MND and how all the possible factors interplay.

We conducted a systematic literature review to determine how symptoms influence QoL for people with ALS/MND, in preparation for the Trajectories of Outcome in Neurological Conditions (TONiC) study, a British multicentre study of QoL in MND.

Objectives: This study reviews the published evidence on symptoms influencing QoL, identifying potential direct and indirect effects.

Methods: Literature searches were conducted in PubMed, Science Direct, Science Citation Index, EBSCOHOST, Scopus, CINAHL and PsycInfo to identify primary studies published from 1999 to March 2014 inclusive, assessing how disability or symptoms affect QoL in ALS/MND. The symptom list was derived from the American Academy of Neurology ALS Practice Parameters (1). Studies were excluded if their aim was to evaluate the effect of a therapy on QoL or if they provided no data on correlation between symptom and QoL. Direct effects were determined by correlation co-efficients, graded according to standard statistical criteria (2). Indirect effects were factors correlating with a direct effect.

Results: 111 potential studies were identified; of these, 82 met exclusion criteria. This left 29 studies describing overall disability severity and 7 symptoms as showing correlations with QoL. In descending order of strength of correlation co-efficients, these were overall disability severity, dyspnoea, fatigue, cognitive impairment, depression, emotional lability, anxiety and muscle weakness. Data on every factor for which more than one study was available was conflicting, in that there was at least one study showing no correlation as well as one or more showing some correlation. Factors indirectly contributing were pain (through low mood, anxiety, overall disability severity) and poor sleep (through dyspnoea and fatigue.) There were no studies providing information on correlations between cramps, sialorrhoea, spasticity, dysphagia or weight loss with QoL. A potential multifactorial model of symptoms influencing QoL directly and indirectly has been developed.

Discussion and conclusion: There is preliminary evidence that several physical and psychological symptoms do directly impact QoL for people with ALS/MND. Comparison of studies was challenging due to heterogeneity in study design and outcome measures. Inconsistencies between studies may reflect methodological variations. Some factors appear to have indirect effects by mediating other factors. Further work is needed to systematically identify potential factors and test these in large studies capable of examining direct and indirect effects.

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P98 THE RELATIONSHIP BETWEEN DYSARTHRIA AND QUALITY OF LIFE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Feroldi S¹

Ginocchio D^1,2

Lunetta C²

Sansone V²

Schindler A³

^lSalvatore Maugeri Foundation, IRCCS, Milan, Italy

^mNEuroMuscular Omnicentre, Ospedale Niguarda Cà Granda, Milan, Italy

ⁿDepartment of Biomedical and Clinical Sciences “L. Sacco”, Milan, Italy

Email address for correspondence: [email protected]

Keywords: dysarthria, quality of life, QOL-DyS

Background: Few studies have evaluated dysarthria-related quality of life (QoL) in patients with amyotrophic lateral sclerosis (ALS).

Objective: The aims of the study are: 1) to evaluate dysarthria and dysarthria-related QoL in patients with ALS; 2) to analyze its relationships with patients’ age, disease duration, motor functions, depression and general QoL.

Methods: 43 patients with ALS have been involved in the study (21 M, 22F; 33 spinal onset, 10 bulbar onset; age 58 ± 11; disease duration 30.2 months ± 20.1). Inclusion criteria were Mini Mental State Examination (MMSE) ≥ 24/30 and preserved reading function. Dysarthria severity and characteristics, ALS severity, depression, dysarthria-related and generic QoL were evaluated respectively by means of the Therapy Outcome Measure(1) (TOM) scale, the Robertson Profile(2), the revised Amyotrophic Lateral Sclerosis Functioning Rating Scale (ALSFRS-r), the Beck Depression Inventory(3) (BDI-II), the QoL of the dysarthric speaker(4) (QoL-DyS), Amyotrophic Lateral Sclerosis Assessment Questionnaire(5) (ALSAQ-40) and the Short Form-36 (SF-36) questionnaire(6).

Results: Dysarthria affected 31/43 patients. Dysarthria severity was mild-moderate in 13 patients, severe in 18 patients. Robertson Profile and QoL-DyS scores were significantly more compromised in dysarthric than in non-dysarthric patients (p < 0.001). QoL-DyS showed significant correlation with all the subscales of Robertson Profile (r = 0.812). A significant correlation was found between QoL-DyS and dysarthria severity measured in all TOM subscales: impairment (r = 0.797), disability (r = 0.705), handicap (r = 0.682).QOL-DyS showed a strong correlation also with ALSAQ-40 items referring to communication and eating (r = 0.857and r = 0.762); a significant difference between dysarthric and non-dysarthric patients (p < 0.001) was found. QoL-DyS showed no correlation with patients’ age, disease duration, mental health or depression. SF-36 scores between dysarthric and non-dysarthric patients were similar, with the exception of the physical activity (p = 0.021). ALSFRS-r total score (mean± SD 27.2 ± 7.8) between dysarthric and non-dysarthric patients showed no difference, while a significant correlation between QoL-Dys and ALSFRS-r bulbar (mean± SD 8.6 ± 3.0) (r = 0.616) and respiratory subscores (mean± SD 9.5 ± 2.3) (r = 0.421) was found.

Discussion and conclusion: Dysarthria severity affects QoL in ALS patients according to QoL-DyS scores. QoL-DyS should be used in clinical practice and research in patients with ALS.

Acknowledgements: The authors report no conflicts of interest. This study was only possible thanks to the participating patients and caregivers.

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P99 MIXED CONDUCTIVE-SENSORINEURAL HEARING LOSS IN PATIENTS WITH ADVANCED AMYOTROPHIC LATERAL SCLEROSIS

Ginocchio D^1,2

Barozzi S¹

Maestri E²

Corbo M^2,3

Sansone V²

Lunetta C²

^oAudiology Unit, Department of Clinical Sciences and Community Health, Università degli Studi di Milano; Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

^pNEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Niguarda Ca’ Granda Hospital, Milano, Italy

^qDepartment of Neurorehabilitation Sciences, Casa Cura Policlinico, Milano, Italy

Email address for correspondence: [email protected]

Keywords: hearing loss, hearing aids, communication

Background: Hearing Loss (HL) has not often been documented in individuals with amyotrophic lateral sclerosis (ALS) (1,2,3).

Objective: The aim of the study is to evaluate the characteristics of HL in ALS patients in advanced stages who complained of hearing difficulties, and to investigate the possibility of using a hearing amplification device.

Methods: Five individuals affected by ALS, who complained of hearing difficulties, were involved in the study (4 M, 1 F; median age 54 years; range 52–72 years). The mean disease duration was 49 months (range 23–227 months). All of them were in the advanced stage of the disease with invasive ventilation, anarthria, dysphagia in enteral feeding and tetraplegia. The patients did not present any co-morbidity factors linked with hearing loss. They all underwent an audiological assessment including otoscopic examination, pure-tone audiometry, and acoustic immittance tests. The degree of hearing loss was classified using standard Pure-Tone Average (PTA). All patients were fitted with monaural digital behind-the-ear hearing aids. A 5-item questionnaire, adapted from the Hearing Handicap Inventory for adults (HHIA) (4) (items: 15, 18, 20, 23, 25), was completed by the patients and caregivers before hearing aid application and 3 weeks after.

Results: All of the patients proved to be affected by bilateral mixed hearing loss. The degree of HL was moderate to severe (right ear: mean PTA: 60.6 dB HL, range: 43.3–73.3 dB HL; left ear: mean PTA: 66.3 dB HL; range: 53.3–73.3 dB HL). The tympanograms were all type B, indicating middle ear effusion. The higher scores obtained at HHIA when using amplification (unaided mean score: 18; aided mean score: 6) indicate a reduction of hearing difficulties with the use of amplification devices.

Discussion and conclusion: In individuals with ALS, HL may be an unrecognized condition because it can be masked by other disabling symptoms, and it may contribute to worsen individual quality of life. Our pilot study showed the presence of bilateral, mixed (conductive-sensorineural) HL in patients during the advanced stages of the disease. Moreover, the study emphasized the efficacy of hearing aids in reducing hearing difficulties. Our findings suggest that hearing sensitivity should be studied in ALS patients from the early stages of the disease in order to define hearing loss incidence, interaction with the different clinical phenotypes and timing of onset.

Acknowledgements: The authors report no conflicts of interest. This study was only possible thanks to the participating patients and caregivers.

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P100 AMYOTROPHIC LATERAL SCLEROSIS AND PAIN

Idrisoglu HA

Polat N

Idrisoglu M

Medical faculty, Istanbul, Turkey

Email address for correspondence: [email protected]

Keywords: pain, depression, quality of life

Background: ALS is a fatal neurodegenerative, progressive disease, with muscle weakness, respiratory failure and bulbar symptoms. Fasiculations and cramps are obvious during progression of disease. Secondary symptoms occur during progression. Pain is important in those secondary symptoms and problems. Pathomechanical properties of pain in ALS patients are missing.

Pain occurs in 20–70% of ALS patients during the case of the disease. Pain is very common as the disease progresses. Pain in ALS disease is described as an unpleasant sensory emotion. Pain becomes acute or chronic and pain in ALS is related to immobilisation in the coint and depressive status. The pain is seen in the arms, shoulders and knees. Aetiopathogenesis of this is not well understood.

Results: We have examined 300 patients over 5 years to determine whether pain occurs. We have established pain in 50 of 300 patients. We have seen that 20% of the patients have pain. There were 24 women and 26 men ALS patients with pain. The age interval was 23–82 mean age was 56.When we looked at the pain localization 20% of 50 patients had hip pain, 1% of 50 patients had neck pain, 15% of 50 patients had shoulder pain.

Discussion and conclusion: Our study suggests that pain is frequent in ALS patients and depressive symptoms with pain relate to poorer QoL. Clinicians should pay more attention to both pain and depressive symptoms in ALS patients considering their effect on QoL. İn this report pain in ALS is mentioned and this will be discussed with literature data.

References:

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P101 PAIN ASSESSMENTS VARY IN A MULTINATIONAL SAMPLE

Stephens H¹

Walsh S²

Simmons Z¹

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

^sALS Association Greater Philadelphia Chapter, Harrisburg, PA, USA

Email address for correspondence: [email protected]

Keywords: pain, quality of life, multinational research

Background: Recent research has demonstrated that pain is a significant symptom in ALS. Such studies are of patients within specific geographic regions or, most broadly, within one country. Perceptions of pain differ between cultures, health care systems vary, and pain management techniques are not uniform between countries. Such factors have not been explored in persons with ALS.

Objectives: To examine perceptions of pain severity and the relationships between pain, quality of life (QOL), disease duration, and function in a multinational convenience sample.

Methods: Three Canadian, 1 Swiss, 1 Israeli, 1 Scottish and 2 Italian ALS Centers contributed data in an Institutional Review Board approved multinational study of QOL. American data was obtained from a previous multi-center study. All patients completed the ALS Specific Quality of Life-Revised (ALSSQOLR) instrument that includes one item that rates pain by asking “how much of a problem pain has been in the last 7 days” using a scale of 0 (no problem) to 10 (tremendous problem). Data analyzed included the ALSSQOLR pain item, ALS Functional Rating Scale-R (ALSFRSR), and demographics. Analysis of variance (ANOVA) was used to assess the effect of country on pain ratings. Post hoc comparisons were made using the Tukey HSD test. Analysis of covariance (ANCOVA) determined the effects of ALSFRSR, disease duration, and negative emotion QOL on pain ratings. We established pain severity categories including: 0 = no problem; 1–3 = mild; 4–6 = moderate; 7–10 = severe. Chi square test examined country differences in distribution of pain severity.

Results: Data was obtained on 396 US, 76 Canadian, 52 Swiss, 56 Israeli, 22 Scottish and 150 Italian subjects. There was a significant effect of country on pain scores, (F (5, 752) = 23.79, p = 0.000). Mean US pain scores (2.32, SD = 2.77) were significantly lower than those from Israel (4.34, SD = 3.78), Canada (5.39, SD = 3.6), and Switzerland (6.06, SD = 3.78), but did not differ from those in Scotland (3.18, SD = 3.33) or Italy (3.00, SD = 3.33). The negative emotion QOL domain and disease duration were significant co-variates in the model (p < 0.01) whereas ALSFRSR was not. Greater emotional distress and longer disease duration relate to higher pain ratings within groups. The distribution of pain severity varied among countries, X² (15, N = 758) = 110.0, p < 0.001.

Discussion and conclusion: Ratings of pain severity by patients with ALS varied between the countries, and were related to the negative emotion domain of the ALSSQOLR (a measure of psychological morbidity) and to duration of disease, but not to physical function. Cultural factors, health care systems, and pain management techniques may contribute to these differences, and should be further explored as a step toward better symptom management in ALS.

P102 SELF-PERCEIVED EXPERIENCES OF PAIN IN ALS/MND

Åkerblom Y¹

Zetterberg L²

Jakobsson Larsson B³

Åsenlöf P²

^tUppsala University Hospital, Uppsala, Sweden

^uDepartment of neuroscience, physiotherapy, Uppsala University, Uppsala, Sweden

^vDepartment of neuroscience, neurology, Uppsala University Hospital, Uppsala, Sweden

Email address for correspondence: [email protected]

Keywords: pain, experience, interviews

Background: Although persons with ALS/MND commonly report pain symptoms (1,2), little is known about how pain experiences vary during the course of the disease and between persons.(3,4) This gap of knowledge hinders the possibilities for development of new pain management strategies.

Objectives: To explore variations in patients’ experiences of pain in ALS/MND.

Methods: Sixteen participants (male = 11) with various disease durations and a documented experience of pain were individually interviewed. Transcribed data were analyzed according to qualitative content analysis. Researcher triangulation was used to enhance the credibility of analysis (5).

Results: The most striking result was the great variety in patients’ experiences. Results were organized into four categories illustrating areas for this variation; description of pain, factors that contributed to the pain, management, impact on daily life. Pain was perceived to have different levels of seriousness; it was sometimes even the first symptom of the disease. Participants described multiple pain locations and seemed to occur more frequently in the morning, the evening, or at night compared to the wakening hours during the day. Drugs had impact on pain, sometimes enough to reach an acceptable level, however some reported unsatisfactory pain relief. Some participants even exclude drugs because of the side effects. Accepting the feeling of pain or repressing it were coping strategies described. Physical activity was another way of successful coping, but sometimes it worked in reverse order. Consequences of pain were immobility, energy drainage, and decreased well-being. Participants experienced that pain reduced their degree of autonomy. For some patients, it seriously affected their well-being by disturbed sleep and negative emotions.

Discussion and conclusion: The participants’ experience of pain varied greatly both within and between the patients. The results indicate a need for individually tailored treatment strategies including monitoring of pain and its consequences during the disease progression.

Acknowledgment: This study was financially supported by grants from the Medical Faculty; Caring Sciences Grant, Uppsala University and ALF-grant.

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P103 MORPHINE USAGE IN ALS PATIENTS ON NPPV DOES NOT MAKE LIFE PROGNOSIS WORSE

Tominaga N¹

Ogino M¹

Kaneko J¹

Nagashima K¹

Nagai M¹

Uchino A¹

Urano Y¹

Ogino Y^1,2

Nishiyama K¹

^wKitasato University, Sagamihara, Kanagawa, Japan

^xHakone hospital, Odawara, Kanagawa, Japan

Email address for correspondence: [email protected]

Keywords: end of life care, quality of life, morphine usage, prognosis

Background: About 50% of patients with ALS experience respiratory distress in late stage of illness. Thus many guidelines recommend usage of morphine for that situation. Morphine usage is well recognized in United States and Europa, but in other areas it is not popular yet. One possible explanation is there is fear that morphine could make life prognosis shorter. There are few reports that analyses the effects of morphine for the prognosis on ALS patients.

Objectives: To clarify whether morphine usage has effects on prognosis of ALS.

Methods: Two hundred eighty four ALS patients were admitted to our hospital from 2003/04/01 to 2012/04/01. 47 patients who 1)）died in our hospital; 2) had a detail medical history available; 3) were on NPPV longer than 22hrs a day; 4) were on tube feeding were enrolled. Patients were divided into morphine administered and non-morphine administered groups. To focus of effects of morphine usage, other factors which might effect for prognosis such as NPPV and tube feeding were equal between two groups. Duration between the day on which patients needed NPPV usage more than 22hrs a day and death was measured.

Results: Thirty patients with morphine use lived 112.1 days in average, 17 patients without morphine use lived 93.5 days. There is no statistical significant differences.

Discussion and conclusion: Morphine use does not shorten life prognosis in ALS patients. Unfortunately some doctors still have prejudice for morphine use in non-cancer patients. We have to let them know that morphine is safe and effective in palliative care of ALS patients.

P104 PREVALENCE OF BOWEL AND BLADDER SYMPTOMS ATTRIBUTABLE TO ALS

Jerant P

Gibson SB

Hansen A

Bromberg MB

Biltaji E

Deka R

University of Utah, Salt Lake City, Ut, USA

Email address for correspondence: [email protected]

Keywords: bowel, bladder, survey

Background: Symptoms of bowel and bladder disturbances are common in both the general population and among ALS patients. There is overlap in the range of symptoms representing true disturbances or normal variations. Prevalence values vary depending upon the range of symptoms queried and whether responses are based on formal survey instruments or by self-report.

Objectives: We undertook a survey to determine prevalence rates of bowel and bladder symptoms and whether the symptoms could be attributed to ALS.

Methods: Two surveys were administered at a single clinic visit: a bowel function survey based on Rome III (1), and a bladder function survey based on Primary Overactive Bladder Symptom Questionnaire (POSQ) (2). Both were supplemented with questions concerning the temporal relation to onset of ALS symptoms or diagnosis.

Results: Most patients were in their 6^th decade and were male, and the average time from symptom onset to the survey was 2 years.

Amongst bowel symptoms, 19% answered yes to the general question “do you feel you have constipation now”, while 57% met Rome III diagnostic criteria for constipation. 62% noted straining during bowel movements as a new symptom since diagnosis, 38% noted hard or lumpy stools, and 19% acknowledged bowel urgency. Bowel movement frequency did not change with the onset of ALS. A variety of treatments were used and polyethylene glycol was the most effective.

Amongst urinary symptoms, 29% answered yes to the general question “do you feel you have urinary problems now”. However, to specific questions, 71% were bothered by urgency, 38% were bothered by frequent daytime urination, 48% were bothered by frequent night-time urination, and 33% were bothered by urge incontinence. While virtually all patients were offered treatment, 60% were satisfied with treatment.

Discussion and conclusion: Bowel and bladder symptoms are a common complaint amongst ALS patients, but a formal survey shows a lesser percentage than informal surveys. Among symptoms self-reported by ALS patients, nearly 50% acknowledge bowel issues and nearly 70% bladder issues (3). Symptoms were also under-reported when patients were asked general questions versus asking about specific constipation and urinary symptoms. From our formal survey there appears to be a range of symptoms, treatment options are only partially successful, and providers need to ask specific questions about symptoms to best treat the patient.

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P105 THE PATIENT EXPERIENCE OF SPASTICITY IN MOTOR NEURONE DISEASE

Milinis K^1,2

Young C^1,2

on behalf of the Tonic study group

^yThe Walton Centre NHS Foundation Trust, Liverpool, UK

^zUniversity of Liverpool, Liverpool, UK

Email address for correspondence: [email protected]

Keywords: spasticity, measurement, qualitative

Background: Spasticity is a common impairment in motor neurone disease (MND). Studies in multiple sclerosis and spinal cord injury have found that spasticity affects patients in diverse and complex ways (1, 2). To date no studies have investigated the patient experience of spasticity in MND.

Objective: To explore the lived experience of spasticity in patients with MND.

Methods: Semi-structured interviews were conducted with a random convenience sample of volunteers who attended an MND clinic and who were experiencing any spasticity.

Results: 11 subjects (7 male) aged 40–83 years (median 51) took part, 7 had a limb onset MND, 3 bulbar and 1 respiratory. Median duration of disease was 36 months (range 1–98). The sample was representative of a range of disabilities (Amyotrophic Lateral Sclerosis Functioning Rating Scale range 25–48). Participants described their spasticity using words such as ‘muscle stiffness’, ‘rigidity’, ‘tightness’ ‘cramps’ and ‘spasms’. Three main themes emerged: (i) physical symptoms, (ii) modifying factors and (iii) negative impact.

(i)	Symptomatology of spasticity was classed into three domains: (1a) muscle stiffness, (1b) spasms and (1c) sensory consequences (pain and discomfort).
(ii)	Factors that worsened or triggered spasticity were sudden movements, writing, stretching, cold, fatigue and exercise. Heat, massage, relaxation and antispasticity medication were found to relieve the symptoms. Bulbar spasms occurred unprovoked or were triggered by yawning, laughing, speaking and eating.
(iii)	Negative impact of spasticity varied across the sample. While some participants reported that spasticity was barely noticeable, for others spasticity significantly affected mobility, activities of daily living, social relationships, sleep and mood. Particularly distressing were bulbar spasms, characterized by sudden laryngeal closure and in the most extreme cases causing interference with breathing. In contrast to previous studies on spasticity in other neurological conditions which found that spasticity may have desirable properties, none of the patients with MND reported positive effects of spasticity on mobility or transfers (1,2).

Discussion and conclusion: Spasticity can have far reaching negative effects on people with MND. The findings also suggest that MND-related spasticity can manifest in unique ways, such as bulbar spasms, which have rarely been described in other conditions. Understanding the symptomatology of spasticity and its consequences is an important step forward in developing comprehensive measures of spasticity in MND in the future.

Acknowledgements: This work was supported by Neurological Disability Fund, Walton Centre NHS Foundation Trust and the Motor Neurone Disease Association (UK).

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P106 SELF-ASSESSMENT OF PHYSICAL THERAPY IN ALS

Meyer R¹

Maier A¹

Kettemann D¹

Funke A¹

Münch C^1,3

Walter B¹

Gajewski N²

Karnapp C²

Hildebrandt B³

Meyer T^1,3

^aaCharité - University Hospital, Department of Neurology, ALS/MND outpatient department, Berlin, Germany

^abCharité - University Hospital, Department of Neurology, Project team AmbulanzPartner, Berlin, Germany

^acAmbulanzPartner SozioTechnologie GmbH, Berlin, Germany

Email address for correspondence: [email protected]

Keywords: physical therapy, self-assessment, multidisciplinary care

Background: Although physical therapy (PT) is widely administered in ALS there are but a few systematic investigations on the type, duration and frequency of PT. The subjective experience of PT has remained largely unknown.

Objectives: To evaluate the patients’ subjective experience of physical therapy and their likelihood of recommending it.

Methods: 45 ALS patients (64.4 % male; age: 58.6 years; duration of disease: 37.5 months) were included. We employed the Measure Yourself Medical Outcome Profile (MYMOP2) and Net Promoter Score (NPS) for evaluation. With MYMOP2, the patient defined the subjectively most bothersome symptom and a resulting restriction of activity in relation to their self-assessment of general well-being. These are evaluated on a 6-point scale (6 = lowest). NPS is an evaluation instrument elucidating therapy satisfaction by stating the patient's likelihood of recommending their therapy (1–10, 10 = extremely likely). Over a period of 24 weeks, 27 patients were required to fill in an online score every fortnight which was provided on the web portal AmbulanzPartner.de.

Results: The median time spent weekly on PT was 248 minutes (80–615 min). Motor symptoms in the following regions were perceived as being the most bothersome: arms (33 %), legs (60 %) and the trunk (7 %). At the beginning of the observation period, the symptom scored 3.22 (SD 1.08) and activity restriction 3.18 (SD 1.13) points on the MYMOP2-score. Over time, the severity of symptoms and restriction of function increased significantly. Despite functional deterioration patient satisfaction with PT increased in 48% of patients (n = 13). In 22 % (n = 6), satisfaction decreased and in 30 % (n = 8) it remained the same. Median satisfaction increased from 7.7 to 9.0 NPS points. Patients that became increasingly less satisfied had a higher ALS progression rate (p = 0.05) and frequency of treatment and a longer overall term of therapy.

Discussion and conclusion: Despite progression of symptoms and loss of motor function, most of the patients described high or increasing satisfaction with PT. High ALS progression rate was a risk factor for low subjective satisfaction. The dissociation of decreasing motor function and increasing subjective satisfaction supports the concept of palliative PT over the course of the disease.

Acknowledgements: This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported by the BMBF (Federal Ministry of Education and Research) under the aegis of JPND. Further funding comes from the BMBF Joint Project “MND-NET” as well as the Foundation Georgsmarienhütte and the ALS Initiative “Aid for People with ALS”.

P107 HOW STORIES COMMUNICATE DAILY LIVING WITH ALS

Jeppesen J^1,2

Rahbek J¹

Gredal O¹

Ploug Hansen H²

^adThe National Rehabilitation Centre for Neuromuscular Diseases, Aarhus, Denmark

^aeUniversity of Southern Denmark, Odense, Denmark

Email address for correspondence: [email protected]

Keywords: communication, illness experience, patients’ perspective

Background: To complement the clinical and therapeutic knowledge about symptoms, prognosis, and social implications of ALS, health research and care need to develop methods that capture and communicate the unique individual impact on daily living with the disease (1, 2, 3).

Objectives: To explore how stories can communicate experiences of daily living with ALS and compensate the progressive loss of ability to speak.

Methods: Twenty-four interviews at home with six people diagnosed with ALS were transformed into narrative journalistic stories (4). A formal readership was selected by the participant among his or her most significant health professionals. Topics of stories were categorized and selected themes analysed and interpreted.

Results: The stories communicated daily living with ALS as a continuous process of creating a new normality of everyday life. The stories also revealed conflicting views between patient and professionals about information about disease and prognosis (5, 6).

Discussion and conclusion: The narrative journalistic story enhances communication about daily living with ALS by offering a mode of sharing experiences that compensate the progressive loss of communicative abilities. The story can sustain meaning in living with ALS and support patients to appreciate a day-to-day life which is not just a waiting for death. By showing that living with ALS is a constant struggle of leading a life as normal as possible, and how important this hard work is for the patient, narrative journalistic storytelling may educate health professionals that a medical prognosis should be complemented by understanding the individual’s unique experience of vulnerability.

Acknowledgements: This study was funded by the Danish Ministry of Science, Innovation and Higher Education (grant reference number 09-053456), the National Rehabilitation Centre for Neuromuscular Diseases (RehabiliteringsCenter for Muskelsvind), and the Neuromuscular Patient Association (Muskelsvindfonden).

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P108 PATIENT COMMUNICATION AND TREATMENT PREFERENCES IN AN ALS CLINIC

Walsh S¹

Stephens H²

Simmons Z²

^afALS Association, Greater Philadelphia Chapter, Harrisburg, PA, USA

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

Email address for correspondence: [email protected]

Keywords: goals for care, end of life, advance care planning

Background: The Penn State Hershey Communication and Treatment Preference Assessment (CTPA) was developed in an effort to document elements that would guide the ALS health care team in treatment and end of life discussions.

Objectives: Describe communication and treatment preferences of patients in an ALS Clinic as captured by the CTPA.

Methods: Completed CTPAs forms from patients who had given consent to an IRB approved ALS data repository were reviewed for descriptive analysis.

Results: 37 CTPA patient forms were reviewed comprising the sample (65% men, mean ALS Functional Rating Scale-R score = 28.76 (SD = 10.22), average forced vital capacity 61%, and mean ALS-Specific Quality of Life total score of 6.92/10. These statistics match previously reported norms for our ALS clinic. Over 90% of the sample had in place a living will and financial power of attorney (POA) while 75% had in place a healthcare POA and 67% an advance directive. Most patients (81%) preferred to make health care decisions together with family and the medical staff. Few wished to make decisions solely with medical staff (14%) or delegate decision-making to others (5%). Treatment goals of the sample were comfort, not life extension (49%), life extension with selected treatments (40%), and less frequently to extend life at all costs (11%). Half of the sample (50%) preferred information presented in a basic way, while the other half preferred detailed information. 97% of the sample wished to know when their condition has worsened and life expectancy would be short. Most frequent treatments that patients wanted to discuss during the clinic visit included experimental research (50%), feeding tube (50%), respiratory support (43%), diaphragm pacer (36%), and ventilator support (29%). After reviewing the completed forms, advance directives were discussed with 25% of the patients and living wills were discussed with 19%. Financial and Health care POAs were less frequently discussed (< 14%). One third of the patients made a treatment decision during the visit (feeding tube (40%), diaphragm pacer and ventilator support (30%) and experimental research (10%)).

Discussion and conclusion: The CTPA provides structure to the ALS clinic visit and prioritizes discussion of treatments based on patient request and need. The form provides an opportunity to present information in a framework that is most useful to patient's style preferences. The CTPA is a vehicle for assessing the presence of legal documents and providing information to encourage completion of these documents. Most importantly, the CTPA has guided our team to present treatment options that are consistent with the patient's explicit goals for care.

P109 A QUALITATIVE STUDY TO CONSTRUCT A PREDICTIVE SCALE ON INTERNAL CONFLICT FOR PEOPLE WITH AMYOTROPHIC LATERAL SCLEROSIS

Nakai M¹

Narita Y²

^ahSuzuka University of Medical Science, Suzuka-city, Mie prefecture, Japan

^aiMie University, Tsu-City, Mie prefecture, Japan

Email address for correspondence: [email protected]

Keywords: internal conflict, predict scale, specialists’ perception

Background: People (patients and their families) with amyotrophic lateral sclerosis (ALS) frequently confront difficult decision-makings for symptom management or their quality of life. A number of patients and their family members experienced internal conflict (IC) which was defined as disagreements and opposition on various issues regarding patients care. IC was regarded as one of the factors which have negative impacts on their well-being. To predict IC at early stage will bring some benefit to support both patients and family members.

Objectives: The aim of this study was gathering specialists’ opinion to construct a predictive scale on an internal conflict for people with ALS.

Methods: In the first step we generated a draft scale of 54 items to predict IC for ALS patients and their family which was based on a data derived from pre-existing studies. Second step, pre-test and semi-structured interview were conducted by ten specialists (male:5, neurologist = 6, domiciliary nurse = 3, clinical psychotherapist = 1) between July 2013 and October 2013. The audio recorded interviews were transcribed, coded and analyzed according to a qualitative study.

Results: Four points were derived from the interviews. 1) Comments about the problem of inadequacy on item wording and response format, redundancy, compound sentences, which valid the scale contents and lead to more comprehensive scale items. 2) Factors which were recognized as mainly correlated of IC in ALS by health professionals: family communication, decision making style, role changes, values and understanding of the disease. 3) Consideration for impact of interview to patients and family: Three participants mentioned about burden associate with the interviews, 1 of them detected potential risk of burden. Domiciliary nurses suggested that the interview should open up communication within patients, family members and health professionals. 4) Actual condition of IC which experienced by health professionals were detailed.

Discussion and conclusion: Through the pilot test to the specialists and the interviews, we had many suggestions to revise the draft scale and to improve the procedure. All participants had experienced IC in some ALS cases. Factors which were recognized to be associated with IC in ALS from specialist’ perspectives were extracted. Interview by the IC scale may be a beneficial support tool to identify IC and trigger to discuss among stakeholders. Due to awareness of IC may have seemed to depend on the background of each specialist, existence of IC should be determined by all stakeholders’ perception.

P110 ANALYSIS OF DEPRESSION AND ANXIETY IN ALS PATIENTS

Tejado DA

Pérez RS

Moreno MJ

Turón SJ

Povedano PM

Paipa MA

Lazo La Torre C

Melón BA

Hospital Bellvitge, Barcelona, Spain

Email address for correspondence: [email protected]

Keywords: anxiety, depression

Background: Chronic diseases are often accompanied by anxiety and depression. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that leads the patients to a high grade of functional dependence, consequently a state of depression and anxiety.

Objectives: Analyse the grade of anxiety and depression using the Goldberg scale in patients with ALS disease attended in by ALS Multidisciplinary Unit in a tertiary center of Barcelona.

Methods: Goldberg Scale (Goldberg and al, 1198, Spanish version GZEMPP, 1993) was collected during the period of May 2013 to March 2014 in adult patients with sporadic ALS disease in different stages of disease evolution. It excluded patients with antecedents of psychiatric disease or cognitive involvement.

Results: There were 89 patients, 43 were men and 46 women and 60 patients had a spinal onset and 29 a bulbar onset.

Mixed syndrome is the most frequent in the overall cohort (88.2%). According by sex, the depressive syndrome was more frequent in women (36.9%) and mixed syndromes (AS + DS) predominated in men (53%). According by phenotype, the mixed syndrome is slightly increased in patients with bulbar involvement (45% and 43%) without statistical significance.

In order to homogenize the sample we classified the cohort into two subgroups of age, with cut-off line of 45 years, we then obtained 15 patients younger than 45 years and 74 older. Mixed syndromes predominated in both subgroups without difference in age; however depressive syndrome is clearly more frequent in younger patients (40%).

To correlate the time of evolution of disease, we classified the cohort into three subgroups: patients in process of diagnosis and/or very recent diagnostic (11); with disease duration of less than three years (35) and with more than 3 years of duration of disease (43). The mixed syndrome was more frequent in the first subgroups (72%). Patients with more than 3 years of disease have more frequency depressive syndrome, but in the overall cohort the frequency of all the syndromes tend to decrease over the course of disease.

Discussion and conclusion: Mixed syndrome predominated in all patients, quite more frequent in bulbar patients and clearly more frequent in earlier stages of disease. Depressive syndrome is more frequent in younger patients and in later stage of disease. The overall disorders tend to be less frequent in later stages of disease.

Our results show that people in process of diagnosis are more susceptible to anxiety-depressive disorders, this would be related with a period in which the disease is not yet accepted, and younger patients are more vulnerable. Also the longer disease duration the less prevalent they are, would be correlated with a better acceptation of disease.

P111 ILLNESS REPRESENTATIONS AND COPING STRATEGIES AS DETERMINANTS OF QUALITY OF LIFE IN AMYOTROPHIC LATERAL SCLEROSIS: FINDINGS FROM A GREEK SAMPLE

Ferentinos P¹

Rentzos M²

Christidi F²

Zouvelou V²

Alexakis T²

Athanassouli P²

Paparrigopoulos T¹

Evdokimidis I²

^aj1st Department of Psychiatry

^ak1st Department of Neurology,University of Athens, Eginition Hospital, Athens, Greece

Email address for correspondence: [email protected]

Keywords: quality of life, illness representations, coping strategies

Background: Leventhal’s Common Sense Model (CSM) of illness representations hypothesizes that individuals create on the basis of a priori information cognitive and emotional representations of their illness, which help shape coping strategies and behaviours to manage their problem. Both illness representations and coping strategies are important determinants of disease state, and physical and emotional adjustment. The inter correlations between illness representations, coping strategies and physical and emotional outcomes are understudied in patients with amyotrophic lateral sclerosis (ALS).

Objectives: The present study examines the effect of illness representations and coping strategies on physical and psychological adjustment (as measured by physical and mental components of health-related quality of life) in a sample of Greek patients with ALS.

Methods: We included 56 consecutive patients diagnosed with ALS on the basis of El Escorial revised criteria. Patients with severe respiratory insufficiency or dementia were excluded due to difficulty in going through psychometric evaluations. Demographic characteristics and disease-related parameters (disease duration, time since diagnosis, body area affected, bulbar onset, ALSFRS scores) were recorded. Patients’ illness representations were extracted with the revised Illness Perception Questionnaire (IPQ-R), which consists of identity, timeline (acute/chronic and cyclical), consequences, personal control, treatment control, illness coherence and emotional representations dimensions. Coping strategies were assessed using the revised Ways of Coping Checklist (WOC), which consists of five broad factors: positive approach (positive reappraisal and problem-solving efforts), seeking social support, wishful thinking, avoidance/distancing, and confrontive coping. Quality of life (QoL) was evaluated using the SF-36 Health Survey (SF-36), measuring eight generic concepts: physical functioning, physical role, bodily pain, general health perceptions, vitality, social functioning, emotional role and mental health. All measures have been previously validated in the Greek population. A two-step clustering method was employed to classify ALS patients into clusters based on SF-36 scores. A hierarchical logistic regression approach was then used to identify predictors of SF-36 cluster membership belonging to each of four domains: demographic characteristics, disease-related parameters, IPQ-R dimensions and WOC factors.

Results: Clusters 1 and 2 were obtained, with 32 and 24 patients, respectively. Cluster 1 patients scored significantly lower on all SF-36 subscales. In the final logistic regression model, education (years), legs affected, consequences IPQ-R dimension, problem-solving efforts and wishful thinking WOC strategies were tested as predictors of SF-36 cluster membership. Legs affected and consequences IPQ-R dimension were significantly associated with lower QoL scores (p = 0.002 and 0.013, respectively) while problem-solving efforts were associated with higher QoL (p = 0.014); Nagelkerke R^{2 =} 0.695.

Discussion and conclusion: In a Greek sample of patients with ALS, lower limb involvement and a negative perception of the impact of the disease on overall QoL and functionality were associated with worse physical and psychological adjustment; a problem-focused coping approach was linked to better QoL.

P112 COGNITIVE CHANGE AND QUALITY OF LIFE IN AMYOTROPHIC LATERAL SCLEROSIS

Niven E^1,2

Newton J^1,3

Bak T^1,3

Stephens H⁴

Simmons Z⁴

Abrahams S^1,3

^alThe University of Edinburgh, Edinburgh, UK

^amEuan MacDonald Centre for Motor Neurone Disease Research, Edinburgh, UK

^anAnne Rowling Regenerate Neurology Clinic, Edinburgh, UK

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

Email address for correspondence: [email protected]

Keywords: cognitive, assessment, quality of life

Background: Cognitive change is known to occur in up to 50% of patients with ALS, affecting abilities including thinking, language and/or behaviour. However, the relationship between cognitive change and patients’ self-reported quality of life (QOL) has not been a focus of research.

Objectives: To investigate the relationship between cognitive profile and self-reported quality of life (QOL) in patients with ALS.

Methods: 30 non-demented patients with ALS (age 64.50 + 9.23 years) completed both The Edinburgh Cognitive and Behaviour ALS Screen (ECAS)(1) - developed for ALS patients with physical disability - and the ALS Specific Quality of Life-Revised instrument (ALSSQOL-R)(2). The ECAS’s multi-domain screen was designed to detect the specific profile of cognition and behaviour changes characteristically found in ALS, providing ALS-Specific (executive functions, fluency, and language), ALS Non-specific (memory and visuospatial) and total scores. The ALSSQOL-R is designed to reach beyond physically dominated perspectives of QOL and provides a single item score and an average total ALSSQOL-R score as well as six domain scores (negative emotion, interaction with people and the environment, intimacy, religiosity, physical symptoms, and bulbar function) representing answers to 50 wide-ranging and disease-specific items.

Results: Data showed that patients who presented as impaired via ECAS screening (displaying impairment on ALS-Specific or ECAS total scores) did not provide significantly lower QOL single item scores (M = 6.27, SE = 0.75) than those who showed no impairment (M = 6.42, SE = 0.45; t(28) = -0.82, p > 0.05). Nor did patients with impairment (M = 6.33, SE = 0.44) provide average total ALSSQOL-R scores - derived from responses to all answered questions - than patients not presenting with impairment (M = 6.34, SE = 0.32; t(28) = - 0.03, p > 0.05). However, the inclusion of subdomain scores allows a more detailed representation of QOL assessment in these two patient sub-groups.

Discussion and conclusion: According to our results, patients with ALS who present with impaired performance at cognitive screening may provide similar overall and similar averaged totals of self-reported QOL assessment. However, it is important to give consideration both to patients’ cognitive status and to the nuanced, disease-specific subscale information available through the ALSSQOL-R instrument. The use of these two short screens together provides a means to allow a fuller understanding of the obtained profiles.

Acknowledgements: We are grateful to the Motor Neurone Disease Association (UK) and the Euan MacDonald Centre for Motor Neurone Disease Research.

References:

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P113 WHAT IS UNIQUE ABOUT QUALITY OF LIFE IN MOTOR NEURONE DISEASE?: A QUALITATIVE QUERY

Young C^1,2

Ando H^3,1

Cousins R³

^apWalton Centre NHS Foundation Trust, Liverpool, UK

^aqUniversity of Liverpool, Liverpool, UK

^arHope University, Liverpool, UK

Email address for correspondence: [email protected]

Keywords: quality of life, end of life, qualitative study

Background: Quality of Life (QoL) is an important parameter in neurological clinical care. The debate about generic and disease specific measures of quality of life reflects uncertainty about whether there are unique aspects to understanding QoL in different diseases. If there are congruities to different disabling neurological diseases, it may be credible to extrapolate from more common and better-studied conditions, like multiple sclerosis (MS) to rarer diseases like motor neurone disease (MND). In contrast, divergence across neurological conditions would suggest that strategies to maximize QoL must be disease specific.

Objectives: In this analysis our aim was to explore psychosocial factors affecting QoL in MND in comparison with MS.

Methods: The study employed both semi-structured interviews and focus groups. Each focus group consisted of either MND or MS. All interviews and group discussions were audio recorded and transcribed verbatim for thematic analysis. Themes were inductively developed within a condition before they were compared across the two conditions for cross-sectional exploration.

Results: Data was collected from 40 people with MND: 26 interviews (male = 14; mean age = 64yrs; mean illness duration = 1.6yrs); 14 in focus groups (male = 9; mean age = 62yrs; mean illness duration = 3.5yrs); and from 61 people with MS: 43 interviews (male = 16; mean age = 52yrs; mean illness duration = 15yrs); 18 in focus groups (male = 6; mean age = 47yrs; mean illness duration = 9yrs). A codebook for each condition was developed for the analysis and saturation was achieved in both MND and MS.

Whilst the analysis identified the same psychosocial domains to be important for QoL irrespective of disease, there was an aspect unique to MND of it being a terminal condition. Both MS and MND patients valued their lives, but MND patients recognized their shorter life expectancy and differed from MS patients because thoughts of imminent death adversely impacted QoL. Participants’ concerns about death were not restricted to their own death, but extended to its impact on significant others. The differences between MND and MS were further observed with regard to factors that were beneficial to QoL. Although the influence of environmental factors and spirituality were found in both conditions, additional implications of these factors were found amongst the MND group: it was found that the importance of environment included the place of death, and spirituality was described to provide hope even beyond death.

Discussion and conclusion: The current study reveals complexity in ascertaining QoL across neurological diseases. Despite distinct illness trajectories, the same psychosocial factors were identified to be important for QoL in MND and MS. Nevertheless, MND as a terminal condition was found to further challenge patient's QoL. The findings confirm the importance of addressing this aspect of the condition in addition to commonly investigated psychosocial factors.

Acknowledgements: Motor Neurone Disease Association (UK) and Neurological Disability Fund, Walton Centre Foundation Trust.

P114 THE DEVELOPMENT OF A CONSENSUS PAPER ON PALLIATIVE CARE IN NEUROLOGY – THE IMPLICATIONS FOR ALS CARE

Oliver D¹

Borasio GD²

Voltz R³

Caraceni A⁴

De Visser M⁵

Lorenzl S⁶

Veronese S⁷

Grisold W⁸

^asUniversity of Kent, Chatham, UK

^atCenter Hospitalier Universitaire Vaudois, Lausanne, Switzerland

^auUniversity Hospital, Cologne, Germany

^avFonazione IRCCS, Milan, Italy

^awAMC, Amsterdam, The Netherlands

^axUniversity Hospital, Munich, Germany

^ayFARO, Turin, Italy

^azKaiser Franz Josef Hospital, Vienna, Austria

Email address for correspondence: [email protected]

Keywords: guidelines, care planning, training

Background: A joint working group of the European Association for Palliative Care and the European Federation of Neurological Societies has produced a Consensus paper on the available evidence for the palliative care in progressive neurological disease. This has been a generic paper looking at all progressive neurological disease – including ALS/MND, multiple sclerosis, Parkinson's disease, stroke and primary brain tumours. The principles need to be considered separately for all disease groups as well – including ALS/ MND.

Objectives: To show the important areas for consideration in the palliative care for people with ALS/MND.

Methods: A literature search was undertaken including the main electronic databases and looking at the main areas of palliative care and neurology. Two investigators then looked at this literature, determined seven main areas and developed a draft list of papers. These were then commented on by a small group and then more widely until a consensus was developed.

Results: The seven main areas all apply to the care of people with ALS/MND, and it could be argued that with the short prognosis of ALS/MND and the variable rate of progression this approach should be from the time of diagnosis.

The areas of recommendation are: Palliative care should be considered early in the disease trajectory; the assessment and care should be provided by a multidisciplinary team approach, with access to specialist palliative care; communication should be open with patients and families and advance care planning is recommended. This should be as soon as possible in view of the likelihood of difficulties in communication and the development of cognitive change in ALS/MND; symptoms – physical and psychosocial – should be managed actively and appropriately; care needs should be assessed and carers supported before and after death. Professional carers should receive education, support and supervision to reduce the risks of emotional exhaustion; there should be repeated and continued discussion about end of life issues and discussion of patients’ wishes and aims. The recognition of the deterioration and dying phase will allow appropriate management and intervention; palliative care principles should be included with the training and continuing medical education of neurologists and palliative care professionals should understand the issues for neurological patients.

Discussion and conclusion: The recommendations within the Consensus document can be seen to apply to the care of people with ALS/MND and there is a challenge to ensure that these principles are extended as widely as possible to support people with ALS/MND and their carers.

P115 END OF LIFE IN PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS): A REVIEW

Connolly S¹

Galvin M²

Hardiman O^1,3

^baTrinity College Dublin, Dublin, Ireland

^bbDublin City University, Dublin, Ireland

^bcBeaumont Hospital, Dublin, Ireland

Email address for correspondence: [email protected]

Keywords: end of life, dying, decision-making

Background: ALS is an incurable condition with approximately 70% of those affected dying within three years from symptom onset. However, there has been relatively little discussion about the end-of-life phase; perhaps because the thrust of medical education is generally towards curative therapies and end-of-life discussions require a different perspective, including coming to terms with the limitations of medical interventions as life promoting initiatives.

Objectives: The aim of this review is to identify and review the most important themes in the context of management of end of life in ALS.

Methods: Relevant end-of-life themes were identified by a literature search and through discussion with health professionals in Neurology and Palliative Care in the Irish National Centre for ALS. Relevant publications were identified through a search of Medline and Pubmed using the following Keywords: motor neuron disease (MND), amyotrophic lateral sclerosis (ALS), end of life, dying, death, decision-making, advance care directive and euthanasia.

Results: Through discussion with health professionals and literature search five major themes were identified - (1) importance of end-of-life discussions and decisions; (2) use of life prolonging interventions and technologies; (3) life limiting interventions (4) the experience of dying and (5) best practice at end of life.

Discussion and conclusion: Early and honest discussion of end-of-life issues allows time for reflection and planning, can obviate the introduction of unwanted interventions or procedures, and help alleviate fear around dying. Advance care directives can provide patients with options to exercize autonomy regarding preferred end-of-life management strategies, although their legal validity and use varies from country to country. Preferences around end-of-life interventions and use of technologies vary, and it is important that health care professionals respect patients autonomy. Formal care at the end of life should aim to maximize quality of life of both the patient and caregiver and where possible incorporate appropriate palliation of distressing physical, psychosocial and existential distress. Training of health care professionals should include the development of skills that help to sensitively manage the inevitability of death in terminal illnesses such as ALS.

While the importance of end-of-life care is increasingly being recognized, the particular needs of ALS patients and their families require more attention. Successful management of patients through the end-of-life period can be challenging for the healthcare professional, trained in promoting life and preventing death.

P116 ETHICAL ASPECTS TO CONSIDER IN COUNSELLING FOR END-OF-LIFE DECISION MAKING

Lulé D

Badura-Lotter G

Böhm S

Ludolph AC

Fangerau H

University of Ulm, Ulm, Germany

Email address for correspondence: [email protected]

Keywords: end of life, decision-making, palliative care, ethics

Background: Optimized patient care is the primary goal of modern medicine. However, the definition of the optimum is often difficult. ALS is a challenging diagnosis with many different aspects to consider in clinical counselling: decisions in the course of the disease regarding acceptance or denial of life prolonging (invasive and non-invasive ventilation, percutaneous endoscopic gastrostomy) and life shortening treatments (Euthanasia and physician assisted suicide) have to be considered. Cultural and ethical factors might have a strong impact on palliative care decisions and might explain the variety of decisions between countries. In our project, we analyse decision making processes in ALS with special focus on cultural and ethical factors (eg, the dominance of different ethical principles like beneficence, autonomy, non-maleficence and justice).

Methods: Based on two different longitudinal studies on decision making in ALS, an ethical analysis of decision factors was performed. In a first approach, n = 93 ALS patients were interviewed three times in the course of one year and in a second approach n = 100 ALS-patients were interviewed twice in the course of one year with questionnaires concerning their decisions for life prolonging or life shortening treatments. Possible cultural and ethical determinants were acquired such as subjective feeling of being a burden, subjective feeling of autonomy and family bonding. Furthermore, determinants such as subjective quality of life, depression, strategies in coping, religiousness and varies factors of cognition were measured.

Results: Key ethical factors of decision making were feeling of being a burden, family ties and personal thrive for autonomy. Furthermore, many patients were initially unable to decide with regards to life-prolonging treatments, as they were unable to anticipate the therapeutic and clinical relevance of the decision. Furthermore, cultural aspects such as religion have to be considered in clinical counselling. It could be shown that autonomy plays at least a double role in counselling and medical treatment: respect for autonomy as a core ethical principle for health care providers, and autonomy as independence, where it can be seen as a coping strategy and therefore belonging to their quality of life.

Discussion and conclusion: Ethical principles have to be considered in clinical counselling: beneficence (timely provision of appropriate therapeutic treatments to sustain good quality of life), autonomy (patients try to harmonize antagonistic wishes of their need for autonomy and their need for participation of family members in decisions), non-maleficence (no life prolongation if it is considered as suffocation) and justice (every patient has the same rights to prolong or shorten life). Dynamic counselling to balance therapeutic relevance, patients’ personal needs (autonomy and family bonding) and cultural determinants (eg, religion) should be the primary goal of modern medicine to match actual needs of patients.

P117 ASSISTIVE DEVICES IN ALS – ANALYSIS OF 3 YEARS OF MANAGED CARE

Funke A¹

Münch C^1,2

Walter B¹

Gajewski N³

Karnapp C³

Hildebrandt B³

Kettemann D¹

Meyer R¹

Maier A¹

Meyer T^1,2

^bdCharité – Universitätsmedizin Berlin, Neurologische Hochschulambulanz, Ambulanz für ALS und andere Motoneuronenerkrankungen (AAM), Berlin, Germany

^beCharité – Universitätsmedizin Berlin, Neurologische Hochschulambulanz, Projektgruppe AmbulanzPartner, Berlin, Germany

^bfAmbulanzPartner SozioTechnologie GmbH, Berlin, Germany

Email address for correspondence: [email protected]

Keywords: assistive device, managed care, health politics

Background: ALS is a disease prototypical for the medical need of assistive devices such as mobility and transfer aids or advanced communication systems. Despite the high demand and widespread use, there are few systematic investigations into of assistive devices in ALS.

Methods: Since 4–2011, the provision of assistive devices has been coordinated following a managed care concept which comprises case management and an internet-based management portal (“outpatient partner”; OPP). The digitalisation of care processes allows for the electronic capturing of “real life” data for the provision of assistive devices in ALS. Subject of analysis were the type and use of devices typically applied in ALS and the rejection rate by the health insurance companies in Germany.

Results: Between 3–2011 and 3–2014, 1,254 ALS patients at the Charity were coordinated on the internet platform OPP. In the process, 7,394 assistive devices were prescribed. The overall rejection rate by health insurance companies was 22.3%. The distribution based on the number of assistive devices (AD) per patient was as follows: 1–3 AD 46.9%; 4–10 AD 39.3% as well as > 10 AD 13.8%. The frequency distribution of medical indication (prescription rate) and rejection rate for ALS typical AD was the following: orthotics n = 863 (9% rejected); advanced electronic communication aids n = 561 (26% rejected); mobility exercise equipment n = 104 (43% rejected); multifunction wheelchairs n = 203 (40% rejected), electric-powered wheelchairs n = 489 (33% rejected). As for the rejection rate for electric-powered wheelchairs there were distinct regional differences; ie, Berlin (39%), and the federal states of Brandenburg (28%), Mecklenburg-Western Pomerania (40%), Saxony-Anhalt (32%) and Saxony (34%), respectively.

Discussion and conclusion: The predominant assistive devices in ALS are orthotics, communication aids and electric-powered wheelchairs. The acceptance rate of AD prescribed by ALS neurologists is highly variable among health insurance companies (rejection rate of 9 to 43%). The relative risk of rejection for an electric-powered wheelchair for patients in Berlin was 1.41 as compared to patients from the neighbouring state Brandenburg. High rejection rates for individual groups of assistive devices as well as regional differences in their provision point to a pronounced need for research with high relevance to health policies. There is also further need for research into managed care with regard to the utilisation rate and satisfaction relating to the provision of assistive devices.

Acknowledgement: This is an EU Joint Programme - Neurodegenerative Disease Research (JPND) project. The project is supported by the BMBF (Federal Ministry of Education and Research) under the aegis of JPND. Further funding comes from the BMBF Joint Project “MND-NET” as well as the Foundation Georgsmarienhütte and the ALS Inititative “Aid for People with ALS”.Thomas Meyer and Christoph Münch are founding partners of the internet portal AmbulanzPartner (OPP) and shareholders of APST Ltd.

P118 EQUIPMENT UTILIZATION IN THE ALS/MND POPULATION – TRENDS AND TIMING

Allred P¹

Feldman S²

^bgCedars Sinai Medical Center, Los Angeles, California, USA

^bhDrexel University College of Medicine, Philadelphia, Pennsylvania, USA

Email address for correspondence: [email protected]

Keywords: equipment utilization, quality of life, proactive care

Background: Equipment use by those with ALS/MND has long been established as a mechanism for enhancing safe function and improving quality of life. Just as ALS/MND has highly variant presentations and progression rates, the types and timing of equipment utilized by those with ALS/MND also varies. Multidisciplinary teams and coordination of care have greatly enhanced proactive equipment recommendation, but consistency in the timing of recommendations based on ALS/MND disease progression parameters has not been established. Equipment for those with ALS/MND in the US must often be deemed medically necessary before being paid for by insurance companies. Establishing trends and timelines for equipment utilization based on functional measures may assist in procuring equipment earlier. These timelines may also assist patients, families, and outside care teams to increase awareness of equipment needs over the disease duration.

Objective: The objective of our work is to investigate what type of equipment is being utilized by individuals with ALS/MND and at what time point in the disease duration to potentially develop predictive algorithms for equipment recommendations.

Methods: Retrospective chart review was performed at two large ALS centers in the US. Information on the types of equipment recommended, prescribed, and/or utilized by ALS/MND patients throughout their disease duration was collected. Disease parameters such as ALS Functional Rating Scale –Revised scores, vital capacity measures, strength, and disease duration were correlated with the time of equipment recommendation and use.

Results: Proactive recommendation of equipment was evident in the documentation, but notes regarding the utilization of recommended equipment were highly variable. Specific timelines for equipment recommendation and correlations with disease progression parameters will be provided in the presentation.

Discussion and conclusion: Proactive management of symptom progression is a primary goal in ALS care. Establishing timeframes for equipment utilization based on commonly collected disease parameters may allow practitioners to prepare people with ALS/MND for equipment use prior to emergent need. Standardized flow sheets of equipment recommendations and actual utilization are suggested as a mechanism for consistent and accurate documentation across all medical records.

P119 DEVELOPMENT OF A POWERED ‘NEURO’ WHEELCHAIR PRESCRIPTION

Rolfe J^1,2

^biMND Association, Northampton, UK

^bjUniversity of Oxford, Oxford, UK

Email address for correspondence: [email protected]

Keywords: wheelchairs, quality of life, anticipatory equipment provision

Background: Timely provision of wheelchairs for people with MND has a major impact on maintaining quality of life and social participation, but providers are often unable to keep up with the changing needs of people living with MND, resulting in inappropriate equipment and limited usage (1,2). Wheelchair manufacturers currently offer a range of electrically powered indoor-outdoor chairs (EPIOC). In the UK NHS system the particular needs of people living with MND mean that wheelchairs have to be ordered ‘off prescription’, increasing the overall cost and delayed delivery timescales.

Objectives: To develop an easy to use and accessible prescription form for a range of EPIOC's for people living with progressive neurological illness, to reduce under prescribing, utilize anticipatory prescribing and be cost effective for public health providers.

Methods: In the first phase of the project, three major wheelchair manufactures (Invacare, Sunrise and Ottobock) were invited to discuss the concept of a Powered Neuro Wheelchair prescription form for their existing range EPIOC's suitable for people living with MND. Steering and focus groups, stakeholder days and user questionnaires were used to inform the development of clinical, functional, and operational requirements of the chair. In the second phase of the project, data is being collected on the uptake, usage and success of the Powered Neuro wheelchair, with preliminary data available by November 2014 (reported by manufacturers sales figures and user and provider satisfaction surveys). On the launch of the wheelchair prescriptions, information was provided to wheelchair providers and users by means of presentations at study days and conferences, manufacturer press and promotional materials and a jointly funded information DVD and product comparison document.

Results: Three manufacturers have developed specific Powered Neuro Wheelchair prescriptions for a range of their EPIOCs for use, at an accessible price within a public healthcare system. These prevent under prescribing as they have a minimum specification meeting the ongoing needs of people living with MND and other progressive neurological diseases.

Discussion and conclusion: The Powered Neuro Wheelchair provides the opportunity to select a clinically appropriate wheelchair for people with progressive neurological conditions. The availability of this chair is likely to result in a reduction in waiting time for provision, and facilitates changes to wheelchair accessories as the disease progresses.

Acknowledgements: Motor Neurone Disease Association (UK), The Department of Health, Oxford MND Care and Research Centre, University of Oxford, Invacare, Sunrise, Ottobock

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P120 EVALUATION OF CURRENTLY AVAILABLE NECK COLLARS FOR COMFORT AND IMPACT ON ACTIVITIES OF DAILY LIVING

Langley J^1,3

Reed H¹

Stanton A¹

Heron N³

Clarke Z⁴

Judge S^4,3

Shaw PJ²

Quinn A⁵

McDermott CJ^2,3

^bkLab4Living, Sheffield Hallam University, Sheffield, S Yorks, UK

^blSheffield Institute for Translational Neuroscience, The University of Sheffield, Sheffield, S Yorks, UK

^bmDevices for Dignity, Healthcare Technology Co-operative, Royal Hallamshire Hospital, Sheffield, S Yorks, UK

^bnBarnsley NHS, Barnsley, S Yorks, UK

^boMotor Neurone Disease Association, South Yorkshire, UK

Email address for correspondence: [email protected]

Keywords: design, neck collar, comfort

Background: People with Motor Neurone Disease (MND) often develop weak neck muscles, leading to pain, restricted movement and problems with swallowing, breathing and communication. Ideally, a neck collar would help alleviate these. However, neck collars currently available are of limited use for people with MND and often rejected by patients. The Head-Up project is a 2 year study funded by the National Institute for Health Research (NIHR) Invention for Innovation programme with a budget of £400k. The principal aim is to develop a novel neck orthosis for neck weakness that supports the head, whilst allowing freedom to move, without negatively impacting quality of life. Further papers have been submitted outlining the co-design process and clinical evaluation comparing the new design with a list of user needs established in a workshop before the project with people living with MND.

Objectives: To start with the co-design team wished to understand more about the currently available collars with respect to user comfort and impact on Activities of Daily Living (ADLs).

Methods: A comfort assessment study was conducted using healthy volunteers. Four different neck collars were identified based on common usage and distinct design differences (Philadelphia, Headmaster, Apsen and Stro II). Participants wore each collar for a four hour period on different days, separated by at least a week. During each test period the participants recorded locations of pain/discomfort and associated perceptions of relative severity of pain/discomfort using an instrument based on a McGill pain questionnaire and a Likert Scale. They recorded data relating to emotional reactions caused by the collars, impact on specific ADL’s and aesthetic considerations using a Likert Scale.

Results: Thirty-five healthy participants completed the comfort assessment protocol for each of the four collars. There was a clear differentiation between the four. It was observed that the more support a collar gives, the less freedom of movement it gives and the less comfortable it is. Three of the collars resulted in frustration overall whilst the fourth was neutral. All collars had some degree of design compromise such that none met all the needs of the users.

Discussion and conclusion: From a design perspective this study gave the design team a clear insight into existing products and their impact on an individual’s day-to-day life. Further the study indicated that existing solutions fall short of meeting user needs whether they are functional, clinical or related to quality of life and wellbeing. This reinforced the necessity of this programme and co-design approach, intended to be more sensitive to and inclusive of user needs and priorities.

Acknowledgements: We would like to thank the study participants and the funders. This work was funded by the Motor Neurone Disease Association (UK), Devices for Dignity (D4D) and the NIHR.

P121 HEAD UP: CO-DESIGNING A NOVEL CERVICAL ORTHOSIS FOR MND PATIENTS

Reed H¹

Stanton A¹

Langley J¹

Shaw PJ²

Heron N³

Quinn A⁴

McDermott CJ²

^bpSheffield Hallam University, Sheffield, UK

^bqSheffield Institute of Translational Neurology, Sheffield, UK

^brNational Institute of Health Research Devices for Dignity Healthcare Technology Co-operative, Sheffield, UK

^bsMotor Neurone Disease Association, South Yorkshire, UK

Email address for correspondence: [email protected]

Keywords: co-design, product innovation, assistive products

Background: People diagnosed with Motor Neurone Disease (MND) can develop neck muscle weakness and experience ‘head drop’ leading to pain, restricted movement, problems with swallowing, breathing and importantly face to face communication. To help alleviate these quality of life problems patients are regularly prescribed either a ‘rigid’ neck orthosis or soft collar. However, many currently prescribed neck collar and orthotics products offer either limited degrees of head and neck support or limit head movement through full immobilization being primarily designed for a variety of conditions other than MND, from whiplash to more severe cervical head and neck trauma. Neck collars currently available can be of limited use for people with MND and are often rejected by patients.

Objectives: The aim of the Head Up design project was to develop and design an orthosis for neck weakness that supported the head whilst allowing increased degrees of freedom of head movement and that minimise negative impacts on quality of life. In these respects the Head Up project addresses a currently unmet need and this report describes the design steps undertaken, working with patients, health and clinical practitioners, that have brought about a new concept in head and neck support for people living with MND.

Methods: A multidisciplinary research collaboration involving patients / users, designers, engineers, carers, clinical and healthcare professionals, has taken place using co-research and co-design process methodologies that take into account specific needs and desires. In the main these have been delivered in workshop and focus group events in two principal phases of new product development. Firstly, iterative co-creation through user engagement, a process in which early ideas move through stages of investigative prototyping and feedback, and how discoveries have subsequently manifested in product features, and secondly, how pre- production prototypes were built, are how they have been appraised by users in a formal evaluation phase.

Results: Funded by the National Institute for Health Research (NIHR) Invention for Innovation (i4i) program, and delivered by teams from the University of Sheffield (UoS), Sheffield Hallam University (SHU), the Sheffield Institute for Translational Neuroscience (SITraN) and Devices for Dignity (D4D). The research has resulted in a commercially viable new product with distinct USPs over and above current product offerings that include provision of support with movement, asymmetric and task specific support capabilities and scope for individually tailored support both in terms of level of disease progression and anthropometrically.

Discussion and conclusion: This report is design practice led and as such it discusses the value of design involvement in highly specific subject areas, engaging design more deeply, empathetically and early on in the development process. It illustrates how design can build on clinically focused approaches to patient need by asking ‘what’ as well as ‘how’ we should be designing, and discusses the role of co-design in helping to shape and direct research itself ’...moving design from a problem solving activity to a question asking activity’.

P122 NEW DISEASE MANAGEMENT TOOLS – CERNER ELECTRONIC MEDICAL RECORD DEPLOYMENT OF AMYOTROPHIC LATERAL SCLEROSIS FUNCTIONAL RATING SCALE-REVISED (ALS FRS-R) VALIDATED WITH MOBILE SMARTPHONE (IPHONE/ANDROID) APPLICATION (ALSFRSR-LITE)

Bockenek JA^1,7

Brooks BR^1,6

Sanjak MS^1,2

Lucas NM¹

Smith NP¹

Nichols MS¹

Belcher SL¹

Lary C¹

Nemeth J¹

Russo PC¹

Langford VL¹

Wright KA¹

Holsten SE¹

Ward AL^1,5

Fischer MP¹

Oplinger H¹

Bravver EK^1,6

Desai UG^1,6

Story SJ^1,6

Bockenek WL^3,6

^btCarolinas Neuromuscular/ALS-MDA Center Carolinas Medical Center Department of Neurology Carolinas Healthcare System Neurosciences Institute, Charlotte North Carolina, USA

^buDepartment of Kinesiology, University of North Carolina Charlotte, Charlotte North Carolina, USA

^bvDepartment of Physical Medicine and Rehabilitation - Carolinas Rehabilitation, Charlotte North Carolina, USA

^bwDepartment of Internal Medicine Carolinas Medical Center, Charlotte North Carolina, USA

^bxCabbarus College of Health Sciences Occupational Therapy, Concord North Carolina, USA

^byUniversity of North Carolina School of Medicine Charlotte Campus, Charlotte North Carolina, USA

^bzNorth Carolina State University, Raleigh North Carolina, USA

Email address for correspondence: [email protected]

Keywords: ALSFRSR-Lite, electronic medical record, quality improvement

Background: ALS FRS-R assessed at diagnosis and throughout the course of ALS provides information for developing prognosis (1), survival and service needs in ALS patients. ALSFRS-R assessment has been validated via telephony and via web portal. Bland-Altman analysis demonstrate good reliability of face-to-face and test-retest assessments. Internet and smartphone utilization of patient-provided clinical information has improved cost effectiveness of ALS patient management. Deployment of ALSFRS-R to electronic medical records has been slow despite numerous benefits regarding monitoring and improving care for ALS patients.

Objectives: To deploy Cerner EMR version of ALSFRS-R and validate same in ALS multidisciplinary Clinic with Mobile Smartphone (iPhone/Android) Application (ALSFRSR-Lite).

Methods: Standardized version of ALSFRS-R was codified in Cerner EMR and put online beginning April 2014. ALSFRSR was performed by RNs and entered in Cerner EMR online. ALSFRS-R was performed in parallel by coordinator using ALSFRSR-Lite. Correlation analysis by MedCalc Software Belgium.

Results: 82 Cerner EMR ALSFRS-R and ALSFRSR-Lite ALSFRS-R pairs were analyzed. Compared with paper assessment of ALSFRS-R and scanning ALSFRS-R forms into Cerner EMR, online entry demonstrated increased number of completed ALSFRS-R forms compared with paper forms (98 ± 2 vs 92 ± 5), fewer missing item scores (99 ± 1 vs 88 ± 4), fewer total score summations (0 vs 96 ± 2). Patients misidentified gastrostomy status in ALSFRSR-Lite and Cerner EMR compared with paper ALSFRSR-Lite (36 ± 14 vs 2 ± 2 vs 2 ± 2). Patients scored themselves consistently higher on ALSFRSR-Lite than did RN raters (0.8 ± 0.4).

Discussion and conclusion: Cerner EMR online ALSFRSR assessment showed improved numbers of completed forms, fewer missing item scores, fewer total score summation errors. Patients using ALSFRSR-Lite consistently rated themselves higher than did in-person RNs. Use of online tools permits improved ascertainment of ALS patient ALSFRS-R

Acknowledgements: ALS and Neuromuscular Garden-Carolinas Garden of Hope Funds, Carolinas ALS Research Fund, Pinstripes ALS Foundation, Edwin C Holt Communications Laboratory Fund, Mike Rucker ALS Care Fund, Carolinas HealthCare Foundation, North Carolina Jim “Catfish” Hunter Chapter - ALS Association, Muscular Dystrophy Association - ALS Division

Reference:

• Cedarbaum JM. et al. J Neurol Sci. 1999;169(1–2): 13–21.
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P123 UNDERSTANDING PSYCHO-SOCIAL PROCESSES THAT UNDERPIN HOW PEOPLE WITH ALS MAKE DECISIONS ABOUT CARE

Foley G¹

Timonen V¹

Hardiman O^1,2

^caTrinity College Dublin, Dublin, Ireland

^cbBeaumont Hospital, Dublin, Ireland

Email address for correspondence: [email protected]

Keywords: healthcare, experiences, decision making

Background: Researchers have explored ALS service users’ experiences of healthcare services and their perceptions of care. However, few studies had identified key psycho-social processes that underpin service user engagement with healthcare services in ALS.

Objectives: The objectives of this study were two-fold. First, we sought to pinpoint key contexts that shape how people with ALS make decisions about care. Second, we aimed to identify psycho-social processes that underpin their decision making in care.

Methods: Using qualitative methods (1), 34 people were sampled from the Irish ALS population-based register between September 2011 and August 2012. Variation in participants’ experiences was sought to capture the impact of different contexts on their decision making in care. An in-depth qualitative interview was conducted with each participant in their home focused on their healthcare experiences since the onset of ALS. All interviews were audio-recorded and transcribed. Data were analysed using open, axial, and selective coding procedures to identify key psycho-social processes in the data (1).

Results: Participants exerted control in healthcare services because they perceived that they had lost control (or were losing control) over their lives. Exerting control in healthcare included the freedom to accept and decline services. The majority of participants needed time to process the life-altering impact of ALS before they readily engaged with healthcare services. Participants’ anxiety about end-of-life care was alleviated when they engaged with healthcare professionals who they trusted and who reassured them about their care. Participants trusted and felt reassured by healthcare professionals who enabled them to be in control of their care. Family, parenthood and life stage were primary contexts to how participants engaged with services. Those with dependent children struggled most to come to terms with loss and they actively engaged with supportive and life-sustaining interventions to continue parenting. Participants in later life were somewhat more accepting of death than young and middle-aged participants because they perceived their children to be self-sufficient. Older adults were less inclined to seek life-sustaining interventions. Those without a spouse/partner and/or children suggested they had more freedom than those with family in making decisions about care.

Discussion and conclusion: Service providers should be attuned to the role of parenthood at different life stages for people with ALS and how parenting impacts on the decision-making process in care. Life-course trajectories shape how people with ALS engage with services. Service providers should afford choice to people with ALS about their care and engage with them when they are ready to engage. People with ALS negotiate loss by engaging with services on their own terms.

Acknowledgements: Funding: Health Research Board (Ireland)

Reference:

• Corbin J, Strauss A. Thousand Oaks, CA: Sage, 2008.
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P124 DISCRETE CHOICE EXPERIMENT FOR PREFERENCES OF CARE IN MOTOR NEURONE PATIENTS AND THEIR CARERS

Maguire S

Tobin K

Normand C

Hardiman O

Trinity College Dublin, Dublin, Ireland

Email address for correspondence: [email protected]

Keywords: preferences, discrete choice experiment, health services

Background: Multidisciplinary care improves survival and quality of life in ALS. However, patient preference has not been fully assessed. Stated preference methods in particular Discrete Choice Experiments (DCE) have been frequently used in healthcare in recent years to study the preferences of patients (1).

Objectives: To design and analyse a DCE to elicit patient and carer preferences around healthcare services and palliative care intervention, in the absence of revealed preference data.

Methods: Patients/carers were asked to state their preference over alternative clinical scenarios/services. Responses are used to determine whether preferences are significantly influenced by the attributes and also their relative importance.

Attributes for the choice experiments were generated through a number of formal discussions with healthcare professionals. These included provision of care, price of services, distance to clinic and waiting times, place of care, patient autonomy, timing of palliative care intervention, personal care arrangements, use of communication technology, availability of phone advice, emotional support and dependence on healthcare professionals. The experiment was piloted with 10 patients and carers to ensure validity and relevance. A fractional factorial design was constructed using R software. A consecutive sample of 29 patients and 22 carers who attended the National ALS Clinic participated in the study. The results were analysed using random effects probit model.

Results: Patients preferred to receive all the information about ALS at the time of diagnosis, rather than when they think they need it (P = 0.047) or not at all (P = 0.021). Patients preferred that personal care in the home be provided by someone who is not a relative or friend (P = 0.012). Referral to palliative care services later in rather than early in the course of the illness (P = 0.010). Carers showed a preference toward their loved one receiving regular visits from the community multidisciplinary team and public health nurse rather than having to arrange appointments themselves (significant at the level P = 0.068). Carers also preferred to have no emotional support rather than see a counsellor (P = 0.056) or go to group sessions with other MND carers (P = 0.065).

Conclusion: Using a small convenience sample, the opinions of patients/carers differed regarding service provision. The preferences of patients to maximize the availability of information and delay services that are likely to be of benefit may reflect their preference to retain control throughout the course of their illness, even if at the cost of accessing care and services that are potentially beneficial.(2)

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P125 SOUTH WALES MOTOR NEURONE DISEASE CARE NETWORK – AN AUDIT OF CARE PROVISION IN SOUTH WALES

Glew R

Hancock K

Baker I

Dawson K

Hadjikoutis S

South Wales MND Care Network, South Wales, UK

Email address for correspondence: [email protected]

Keywords: care network, audit, service development

Background: The South Wales Motor Neurone Disease Care Network was established in autumn 2012. It provides an innovative approach to care for people with MND (pwMND) locally through planning, delivery, implementation and evaluation of multidisciplinary teams and clinics within each local health board in South Wales, thus promoting seamless co-ordinated local care. To inform the network development an audit was carried out in January 2013 to discover the issues around care pwMND received.

Objectives: To provide a baseline on which to measure the success of the network approach against MND Association standards of care: to engage pwMND in the new network approach; to identify the needs of pwMND in South Wales and to obtain feedback regarding current services; to ascertain demographic information relating to pwMND.

Methods: A questionnaire consisting of 65 multi- choice and free text questions was sent to the 172 pwMND known to the MND Association and former Cardiff Care Centre. Questions explored all aspects of the patient journey.

Results: Of the 172 questionnaires sent out, 76 questionnaires were returned giving a 44% response rate. 89% of respondents had limb weakness, 56% had bulbar symptoms and 34% had respiratory involvement. 65% were cared for by a spouse or partner but 8% had no carer. PwMND had access to a huge range of equipment but experienced delays in obtaining equipment. 50% of respondents had received MND Association information and 27% had contact with an Association Visitor. 75% had attended a hospital clinic. 85% felt they had had no opportunity to discuss advance care planning. 29% had had an emergency admission to hospital since diagnosis. The attitude of professionals had a big impact on respondents’ perceptions of care. Respondents commented on delays in diagnosis and access to care and equipment, lack of communication and change in staff particularly unhelpful. They suggested that improved communication and information, and regular contact with specialist professionals would improve care provision.

Discussion and conclusion: These audit findings have provided an overview of service provision exposing areas of good and not so good practice. The findings have already been used to inform the planning and implementation of the network model and to identify areas requiring service development. The audit will be repeated in January 2015 and subsequent years to close the audit loop and continue to evaluate the impact of the work of the network.

This audit provides a baseline on which to measure improvements in service provision now that the network model is being established and will provide very useful information into the impact of this innovative network approach to care.

Acknowledgements: Abertawe Bro-Morgannwg University Health Board Patient Experience Unit. PwMND who took time and effort to complete the forms.

P126 VISION FOR THE FUTURE - THE NETWORK APPROACH: A NEW MODEL FOR CARE PROVISION FOR PEOPLE WITH MOTOR NEURONE DISEASE (MND) - SOUTH WALES MND CARE NETWORK

Glew R¹

Hancock K¹

James J²

Baker I¹

Dawson K¹

Hajikoutis S¹

^ccSouth Wales Motor Neurone Disease Care Network, South Wales, UK

^cdMotor Neurone Disease Association, Northampton, UK

Email address for correspondence: [email protected]

Keywords: care network, care, multidisciplinary team

Background: Historically the model for specialist MND patient care provision in the UK has been largely through specialist regional care centres. In South Wales this effectively reached half of the MND population. The new network approach seeks to deliver better more equitable care for people with MND (pwMND) and their families/carers. The model encompasses holistic multi-disciplinary team working with early palliative medicine input at a local level.

Objectives: (i)To highlight the need for a new approach; to inform delegates of our new approach to care provision; (ii) to support professionals within the MND community to provide equity of access to care and care which meets the MND Association Standards of Care.

Aims of network: (i) Development of multi-disciplinary teams (MDT’s) and clinics within each local health board to include regular 3 monthly assessment at clinic from Consultant Neurologist, Consultant in Palliative Medicine, respiratory services, care co-ordinator, occupational therapist, physiotherapist, dietician, speech and language therapist, social worker and support from MND Association Visitor. MDT outreach to community for pwMND unable to attend clinic. (ii) Promotion of effective integrated working between health, social service and voluntary sectors. (iii) Improved support and co-ordination of services including training and education.

Results: We now have 4 multi-disciplinary MND clinics established in South Wales, 3 others being established currently and the original Cardiff clinic with associated MDT meetings. Early indications from both pwMND and health and social service staff involved in caring for this patient group show that the network approach is an effective and efficient way of addressing the needs of people with MND and their families.

Patients report feeling more supported and reassured by the presence of a team. They are able to develop a relationship with Palliative Medicine professionals supporting end of life decision making. There are fewer hospital appointments and less disruption to other activities. Team members report better communication between them and report feeling more supported in providing care.

Discussion and conclusion: The poster presentation will outline the South Wales MND Care Network progress to date and is accompanied by a sister poster outlining the processes involved in setting up a network and associated challenges and rewards. By offering 3 monthly assessments within a clinic setting by all professionals involved, problems can be identified early and interventions discussed fully with pwMND and implemented immediately ensuring timely interventions. Providing assessment and care in this way has led to a more co-ordinated approach.

This model of care has benefits for both pwMND and professionals involved in their care. We are presently developing an outcome framework to further evaluate the impact of this approach on health care and patient outcomes.

Acknowledgments: Motor Neurone Disease Association (UK).

P127 VALIDATION OF A NURSING CONTINUING EDUCATION PROGRAM FOR THE CARE OF PATIENTS WITH INTRACTABLE NEUROLOGICAL DISEASE, WITH AN EMPHASIS ON ACTIVE LISTENING

Hara M¹

Konagaya M²

Okada M³

Takeuchi C⁴

Miyamae R⁵

Shimodaira Y⁶

^ceTokyo Women’s Medical University School of Nursing, Shinjuku-ku Tokyo metropolitan, Japan

^cfSchool of Nursing and Rehabilitation Sciences Showa University, Yokohama-city, Japan

^cgTokyo Women’s Medical University School of Medicine, Shinjuku-ku Tokyo metropolitan, Japan

^chTokyo Women’s Medical University yachio Medical Center, Yachiyo-ctiy Chiba prefecture, Japan

^ciNTT Medical Center Tokyo, Shinagawa-ku Tokyo prefecture, Japan

^cjJapanese Red Cross Akita College of Nursing, Akita prefecture, Japan

Email address for correspondence: [email protected]

Keywords: nursing, education, PBL

Background: Our previous study revealed that nurses experience difficulties in active listening when faced with the ongoing and unforeseen distress and anxiety felt by patients with intractable neurological disease(1).

Objectives: The aim of this study was to validate the efficacy of a nursing continuing education program that would enable nurses to engage in better active listening as a part of the psychological care of patients with intractable neurological disease. Toward this end, the program employed the problem-based learning (PBL) approach (2). Here, we reveal the features of nurses’ learning using the PBL approach.

Methods: An educational program comprising three monthly seminars was held for nurses who had cared for patients with intractable neurological disease, and the PBL approach was used in the second and third seminars. Common cases of communication difficulties with patients were recreated and presented. A questionnaire survey was conducted at the end of the program in order to reveal features of the PBL approach. This study was reviewed and approved by the ethics committee of our university.

Results: Analysis of questionnaire results showed that participants derived learning themes, such as “how to encourage patients to engage in conversation”, “the meaning of silence”, and “preparation for listening to patients who shut others out”, through teamwork, thereby experiencing the “successful identification of matters of interest”. Nurses gained learning experience, such as “objectively observing how they were listening to patients”, “thinking where problems exist and deepening their thoughts”, and “perceiving various viewpoints by exchanging opinions”, through role-playing and the PBL, thereby identifying clues for addressing communication problems.

Discussion and conclusion: The PBL approach was effective in resolving problems associated with nurses’ communication with patients with intractable neurological disease. In particular, role-playing provided nurses with clues to perceive their communication patterns with patients.

Acknowledgements: This study was supported by Grants-in-Aid for Scientific Research (Scientific Research B) from the Japanese Ministry of Education, Culture, Sports, Science and Technology.

References:

• Mikiko H. et al. Journal of the Japan Intractable Illness Nursing Society, 17(2): 137–148. 2012. (in Japanese).
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• Undergraduate Nursing Education, McMaster University, Program Handbook 2010–2011.
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P128 THE SCOTTISH MOTOR NEURON DISEASE AUDIT, RESEARCH AND TRIALS (SMART) STUDY: AN AUDIT OF THE HEALTH CARE OF PEOPLE WITH MND/ALS IN SCOTLAND

Swingler R^1,2

Gorrie G³

Davenport R¹

Salman A-S¹

Malik A³

Hall G⁴

Fernandes P¹

Livingstone S³

Pal S¹

Colville S¹

Stephenson L¹

Chandran S¹

^ckUniversity Of Edinburgh, Edinburgh, UK

^clNHS Tayside, Dundee, UK

^cmSouthern General Hospital, Glasgow, UK

^cnAberdeen Royal Infirmary, Aberdeen, UK

Email address for correspondence: [email protected]

Keywords: epidemiology, care, standards

Background: In 2005 the Scottish government introduced a programme to improve the care of people with MND/ALS by developing standards for access to services, diagnosis and ongoing care (1,2)

Objectives: We designed the Scottish MND Audit, Research and Trials (SMART) study to provide an independent evaluation of the care of people with MND in Scotland against these standards.

Methods: We ascertained patients added to the SMND Register in 2011 and 2012 (3), and designed a web-based tool to audit medical records retrospectively (4).

• Access: We recorded the time from referral to assessment by a neurologist, MND Specialist nurse and a defined MND service.

• Diagnosis: We recorded the time from initial consultation to investigation with EMG and MRI, and the time to review with the results.

• Ongoing care: We determined whether the health boards of Scotland had an auditable care pathway in place, and reviewed several aspects of care.

Results: In 2013 an interim analysis of 805 patients showed that 97% of patients had access to a neurologist, compared to 80% in 1989-98, and 91% were seen in a defined MND service, but only 75% of patients attended a service providing a full range of care. 51% of patients had access to MRI and diagnostic EMG within 20 working days of initial assessment, and 70% of patients were reviewed within 15 working days of completion of these investigations. Only three out of fourteen health boards (21%) had care pathways in place.

Discussion and conclusion: Qualitative studies indicate that people with MND in Scotland think that access to health services is poor, that the diagnostic process is slow, and that there are deficiencies in long-term care (5). Our quantitative data indicate that access to a neurologist has improved, and most patients are seen in a defined MND service, but the diagnostic process is slow, and few health boards have auditable care pathways.

This is the first national audit of the care of people with MND in Scotland. It shows that access to care has improved, but the diagnostic phase is slow, and we are at present developing tools to assess long-term care more effectively.

Acknowledgements: MND Scotland, The Euan MacDonald Centre for MND Research, participating medical staff

References:

• Quality Improvement Scotland, Clinical Standards for Neurological Health Services, NHS Scotland Edinburgh 2009.
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P129 CAREGIVING EXPERIENCES OF CHILDREN AND ADOLESCENTS OF A FAMILY MEMBER WITH ALS

Kavanaugh MS¹

Banker-Horner L²

Barkhaus PE³

^coUniversity of Wisconsin - Milwaukee, Milwaukee, WI, USA

^cpALS Association, Wisconsin Chapter, Milwaukee, WI, USA

^cqMedical College of Wisconsin, Milwaukee, WI, USA

Email address for correspondence: [email protected]

Keywords: young carers, quality of life, social support

Background: Caregiving is a critical component of how ALS patients (PALS) survive with a quality of life (QOL). Understanding this caregiving experience is critical for the well-being of both caregivers and PALS. Currently, this is limited to adult caregivers. There are an estimated 1.4 million Young carers (YCs) (< 19 years of age) in the U.S. who provide care to chronically ill family members.(1) To our knowledge, there is no extant data on YCs in ALS. Given the complexity of ALS symptomology and the relatively short duration of disease in many patients, appreciation of the YC’s experience may have significant impact on a PALS and their family’s QOL.

Objectives: This exploratory study identifies and describes the caregiving experience in a previously unknown population of YCs who have a family member with ALS.

Methods: Semi-structured interviews were conducted with an initial sample of 13 YCs who participate in the care of a family member with ALS. Study participants were accessed through the Wisconsin Chapter of the U.S. ALS Association. Qualitative data was analyzed using thematic content analysis, with descriptive statistics used for quantitative data.

Results: ALS YCs ranged from 8 to 18 years of age. They provide care for both parents (N = 10) and grandparents (N = 3), and spend an average of 10 hours/ week performing tasks including feeding, dressing, and transferring. Sixty-nine percent (N = 9) of YCs stated they had a lot of responsibility. Of those, 5 scored high (13), on the depression inventory. Almost all (N = 12) participants reported they did not have enough information, or support, in dealing with ALS. Qualitative analysis elicited several themes including: (i) caregiving is stressful; (ii) being a caregiver is socially isolating; (iii) caregiving as an opportunity to spend time with their family member with ALS.

Discussion and conclusion: Our findings suggest these YCs for PALS have complex, negative and positive, caregiving experiences. Clearly, they are intricately involved in caregiving, yet receive little acknowledgement or support in that role. Given that social support is vital for the well-being of caregivers, this lack of acknowledgement of the role of YCs in the home can be a source of additional problems for a family struggling with ALS. Results provide clear implications for health care professionals in designing best care practices for PALS, thus lessening the impact on YCs. As more data on YCs becomes available, the results will be used to implement programs, services, and interventions.

Acknowledgements: The study is funded through Helen Bader School of Social Welfare, University of Wisconsin - Milwaukee.

Reference:

• Hunt G, Levine C, Naiditch L. Bethesda, MD: National Alliance on Family Caregiving. 2005. (in collaboration with the United Hospital Fund).
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P130 BEREAVEMENT SUPPORT NEEDS OF FAMILY CARERS OF PEOPLE WITH MOTOR NEURONE DISEASE (MND)

O’Brien M¹

Kirkcaldy A¹

Jack B¹

Bell S²

Knighting K¹

Roe B¹

^crEdge Hill University, Ormskirk, Lancashire, UK

^csMotor Neurone Disease Association, Northampton, UK

Email address for correspondence: [email protected]

Keywords: caregivers, bereavement needs, assessment

Background: The importance of assessing and meeting carers’ needs during end-of-life care and into bereavement is well documented (1). The bereaved have worse health outcomes than comparable groups of non-bereaved individuals and bereavement is associated with mental health issues, increased risk of suicide and increased healthcare costs (2; 3). Around 10% of bereaved people will develop ‘Prolonged Grief Disorder’ (PGD) (4) - an unusually intense reaction to the death of a loved one extending beyond the time typically considered normal in bereavement. Little attention has been given to this condition in carers of people with MND.

Objectives: To seek the views of carers and health/social care professionals on bereavement support for carers.

Methods: Two focus groups were conducted with carers (n = 16) and an online survey administered to health/social care professionals in England (MND Care Centre staff, District Nurses and MND Association Regional Care Development Advisors (RCDAs) (n = 59)).

Results: Our focus group data indicate the strain and burden experienced by carers and the need for appropriate support both pre and post-bereavement to be identified and delivered in a timely manner. The survey revealed that health/social care professionals were unsure of the best course of action to take to support carers experiencing bereavement. Forty (68%) respondents suggested that bereavement-related support could be improved; 24 (42%) respondents had encountered family carers of people with MND they considered were suffering from PGD; 38 (68%) respondents did not feel able to accurately predict future cases of PGD and 40 (71%) respondents felt that the implementation of an alert tool to assist in predicting family carers at risk of PGD would be worthwhile.

Discussion and conclusion: Based on our findings bereavement-related support for carers needs to be improved, especially as PGD may be experienced at a higher rate (42%) in this population and we cannot as yet reliably identify the risk of developing PGD amongst carers of people living with MND (plwMND). There is therefore a need to develop an alert system to enable those working with carers of plwMND to identify those carers at risk and subsequently refer them to appropriate support in a timely manner. This is the focus of future work by the team.

References:

• Arthur A. et al. Final Report of review commissioned by DH to support implementation of the End of Life Care Strategy, University of Nottingham, Department of Health, Nottingham. 2011.
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P131 WHY/HOW ARE FAMILIES IN JAPAN ABLE TO CONTINUE PROVIDING LONG-TERM CARE IN SPITE OF THE ASSOCIATED BURDENS?

Kawaguchi Y¹

Ishijima K²

Konagaya ME⁴

Nakayama Y³

^ctNPO ALS/MND Support Center SAKURA, Tokyo, Japan

^cuThe University of Tokyo, Tokyo, Japan

^cvLaboratory of Nursing Research for Intractable Disease, Tokyo, Japan

^cwSchool of Nursing and Rehabilitation Sciences, Showa University, Tokyo, Japan

Email address for correspondence: [email protected]

Keywords: burden on family, TPPV, long-term Care

Background: In many countries, most patients do not use Tracheostomy Positive Pressure Ventilation (TPPV), because they do not want to burden their families with long-term care. However, the proportion of Japanese patients who use TPPV is relatively high. Although some researchers have indicated that this ratio is affected by the recommendations of neurologists (1), there has been less empirical research investigating the effect of family members.

Objectives: Our objective is to reveal why/how family members make long-term care possible in Japan despite the associated burdens. This issue is important because our findings can help patients and families in other countries who want to use TPPV but are hesitant to do so.

Methods: Using snowball sampling, we selected 18 children who were providing care to a parent. We focused on children, because they are expected to take care of a patient if his or her spouse continues to work.

We designed a mixed methods research study. First, we quantitatively measured the children’s burdens using the Cumulative Fatigue Symptoms Index (CFSI) (2). Then we conducted focus group interviews with them and obtained qualitative data about their long-term care. We analyzed these qualitative data with reference to the CFSI results.

Results: A summary of the CFSI data showed that the children clearly felt burdened. Interview data indicated that this is not only because they have to care for the patients all day but also because the patients sometimes behave abnormally. However, we also found that the children’s lives were not completely taken up with long-term care. Some had hobbies such as painting, cooking or photography, for instance, or had obtained paid employment utilizing the skills they had gained through providing long-term care. We also found that when the children were not crushed by the burden of care the patients did not regret using TPPV.

Discussion and conclusion: Of course children do not want their parents to become ill and to have to provide long-term care, but we show that their burdens do not necessarily make long-term care impossible. Even though these children bear a burden, they are able to handle it and allow the patients to live longer. Our findings therefore suggest that patients do not have to give up their desire to live on the grounds of not wanting to burden their families.

Acknowledgements: We would like to express our appreciation to the Welfare And Medical Service Agency, which gave us a subsidy to conduct our research.

References:

• Rabkin J. et al. Amyotroph Lateral Sclero Frontotemporal Degener. 2013; 14: 116–123.
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P132 AN AMBULATORY MODEL OF MEDICAL AND SOCIAL CARE FOR MND PATIENTS IN RUSSIA

Lysogorskaia E¹

Chervyakov A¹

Fominykh V¹

Ivanova M¹

Vorobyeva A¹

Zakharova M¹

Brylev L²

Byalik M³

Dikhter E³

Fominykh M³

Orlova O⁴

Shtabnitskiy V³

Sonkina A⁵

^cxResearch Center of Neurology Russian Academy of Medical Sciences, Moscow, Russia

^cyMoscow Clinical Hospital 12, Moscow, Russia

^cz“Miloserdie” Medical Center, Moscow, Russia

^daPsychological center on Pyatnitskaya street, Moscow, Russia

^dbRussian National Research Medical University, Moscow, Russia

Email address for correspondence: [email protected]

Keywords: multidisciplinary care, epidemiology, quality of life

Background: According to official statistics there are 3585 cases of MND registered in Russian annually.

Methods: We present the experience of an outpatient program for patients with MND in Russia based on the work of the Martha-Mary Medical Center Miloserdie during 2011–2013 years - the result of collaboration of 11 doctors, 1 psychologist, 2 social workers, 3 nurses, 3 coordinators and 20 volunteers. Our organization provides multidisciplinary care at home, hospitals and with on-line service consultations and work with our patients in the end-of-life decision making process. Our team holds a monthly out-patient clinic. We provide patients with NIV and IV machines and consult on their installation and use. We also organize hospitalizations in local hospitals to perform gastrostomy and tracheostomy. We provide information support through mndfund.ru website.

Results: 250 patients applied to our organization from 20 regions of Russia. 82 of patients died during the studies period. Average patient’s age is 58 ± 13 years. 28.4% of patients or their family members applied to out patient clinics. Only 15% of patients take Riluzole because this drug is not registered in the Russian Federation. The first neurologist consultation was provided after 9 months of symptom’s onset in average. ALS diagnosis was confirmed in 14 months after disease manifestation.

Only 30% patients have been informed about their diagnosis when they apply for support and treatment to our service. 34% patients had bulbar form of ALS, 66% had spinal form. Mean ALSFRS-R score was 33 ± 6. The large majority of our patients (78%) apply to our service when they have the 4th stage of MND, in particular 31% patients are on 4A stage and 47% - on stage 4B.30% patients use PEG or tube feeding and get enteral nutrition. From the beginning of organization’s functioning, 73 patients received NIV machines and 6 received IV. Currently we observe over 37 NIV patients and 7 IV patients. 66% had dyspnea during exertion, daytime sleepiness or morning headaches. 38% patients had pain complaints.

Discussion and conclusion: Our patients had a long time from symptom onset until neurologist consultation, and a long time from consultation to diagnosis establishment. This is not because of slow disease progression but because of the lack of awareness of ALS disease in Russia. Common feature of Russia ALS patients is low level of disease knowledge and education. A main problem is the inability to perform and high cost of NIV and IV machines in Russia, patients do not have full access to pain and anxiety medication, because of strict contraindications in Russia, although we reported high level of pain and dyspnea among our patients. All data concludes that the care for ALS patients in Russia should be reorganized. That was the reason to create our service, and we need to continue our activities.