¿La planificación de decisiones anticipadas mejora la calidad de vida de pacientes con enfermedad de la motoneurona? / Does advance care planning improve the quality of life of patients with motor neuron disease?

Fundamentos: La enfermedad de la motoneurona (EMN) es una enfermedad grave, caracterizada por una progresiva pérdida de la función motora, asociada a un deterioro cognitivo, y en ocasiones, acompañada de demencia frontotemploral. Aunque existen tecnologías que sostienen la vida de estos pacientes (por ejemplo, ventilación no invasiva y nutrición enteral), estas pueden comprometer su calidad de vida. Iniciar de manera oportuna la planificación de decisiones anticipadas (PDA) permite a los pacientes participar y elegir futuros cuidados; sin embargo, este enfoque esta poco explorado en pacientes con la EMN. Objetivo: Investigar las perspectivas de los cuidadores en la aceptación e impacto de la PDA, registrado en formato de carta, para pacientes con la EMN y sus cuidadores. Diseño: Estudio transversal cualitativo. Los datos fueron analizados desde un enfoque de síntesis narrativa. Participantes y emplazamiento: Se mantuvieron entrevistas estructuradas con personas que habían sido cuidadoras de pacientes con EMN. Diez de los cuales habían creado una directiva avanzada específica de la enfermedad, una «letter of future care» (LFC) los 8 restantes no. Resultados: Emergieron un total de 4 temas principales: preparación para la muerte, empoderamiento, relaciones y aclarar decisiones y elecciones. Algunos sintieron que la LFC era o podría ser beneficiosa, proporcionando autonomía y respeto para los pacientes, facilitar la toma de decisiones difíciles y mejor la comunicación dentro de las familias. Sin embargo, la «preparación» de los individuos para aceptar la invasión de la muerte podría influir en su realización. El momento oportuno para comenzar la planificación avanzada de los cuidados puede depender de las características personales y de las del caso clínico. Conclusión: La planificación de los cuidados avanzados puede ayudar a los pacientes a lograr un sentido de control y «la mente en paz» y facilitar importantes debates familiares. Sin embargo, la elección del momento oportuno y el estilo de su introducción necesitan ser enfocados con sensibilidad. Herramientas y estrategias que incrementen la eficacia de la PDA parar la EMN deberían ser evaluadas e implementadas (AU)

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The TiM system: developing a novel telehealth service to improve access to specialist care in motor neurone disease using user-centered design.

OBJECTIVES: Attendance at a specialist multidisciplinary motor neurone disease (MND) clinic is associated with improved survival and may also improve quality of life and reduce hospital admissions. However, patients struggle to travel to clinic and may experience difficulties between clinic visits that may not be addressed in a timely manner. We wanted to explore how we could improve access to specialist MND care. METHODS: We adopted an iterative, user-centered co-design approach, collaborating with those with experience of providing and receiving MND care including patients, carers, clinicians, and technology developers. We explored the unmet needs of those living with MND, how they might be met through service redesign and through the use of digital technologies. We developed a new digital solution and performed initial testing with potential users including clinicians, patients, and carers. RESULTS: We used these findings to develop a telehealth system (TiM) using an Android app into which patients and carers answer a series of questions about their condition on a weekly basis. The questions aim to capture all the physical, emotional, and social difficulties associated with MND. This information is immediately uploaded to the internet for review by the MND team. The data undergoes analysis in order to alert clinicians to any changes in a patient or carer's condition. CONCLUSIONS: We describe the benefits of developing a novel digitally enabled service underpinned by participatory design. Future trials must evaluate the feasibility and acceptability of the TiM system within a clinical environment.

The social and economic burden of frontotemporal degeneration.

OBJECTIVE: To quantify the socioeconomic burden of frontotemporal degeneration (FTD) compared to previously published data for Alzheimer disease (AD). METHODS: A 250-item internet survey was administered to primary caregivers of patients with behavioral-variant FTD (bvFTD), primary progressive aphasia, FTD with motor neuron disease, corticobasal syndrome, or progressive supranuclear palsy. The survey included validated scales for disease staging, behavior, activities of daily living, caregiver burden, and health economics, as well as investigator-designed questions to capture patient and caregiver experience with FTD. RESULTS: The entire survey was completed by 674 of 956 respondents (70.5%). Direct costs (2016 US dollars) equaled $47,916 and indirect costs $71,737, for a total annual per-patient cost of $119,654, nearly 2 times higher than reported costs for AD. Patients ≥65 years of age, with later stages of disease, and with bvFTD correlated with higher direct costs, while patients <65 years of age and men were associated with higher indirect costs. An FTD diagnosis produced a mean decrease in household income from $75,000 to $99,000 12 months before diagnosis to $50,000 to $59,999 12 months after diagnosis, resulting from lost days of work and early departure from the workforce. CONCLUSIONS: The economic burden of FTD is substantial. Counting productivity-related costs, per-patient costs for FTD appear to be greater than per-patient costs reported for AD. There is a need for biomarkers for accurate and timely diagnosis, effective treatments, and services to reduce this socioeconomic burden.

The challenges of dysphagia in treating motor neurone disease.

Motor neurone disease (MND) is a relatively rare degenerative disorder. Its impacts are manifested in progressive loss of motor function and often accompanied by wider non-motor changes. Swallowing and speech abilities are frequently severely impaired. Effective management of dysphagia (swallowing difficulty) symptoms and nutritional care requires a holistic multidisciplinary approach. Care must be patient focused, facilitate patient decision making, and support planning towards end of life care. This article discusses the challenges of providing effective nutritional care to people living with motor neurone disease who have dysphagia.

Recommendations to support informal carers of people living with motor neurone disease.

Informal carers are increasingly providing specialist care at home for people living with motor neurone disease. The carers may experience significant deterioration in their quality of life as a result of the physical and psychological burden they undertake. This systematic review seeks to provide evidence-based recommendations to enable healthcare professionals to support carers appropriately to maintain their wellbeing and to continue providing care at home. Inclusion criteria included articles focusing on the experience of informal carers of people with motor neurone disease, particularly when reporting on their perspective of professional services. Twenty-three studies were included and a thematic analysis was undertaken. Four key recommendations were identified: providing support, early access to palliative care, information regarding availability of services, and offering carers training for using specialist equipment. These recommendations offer healthcare professionals practical, cost-effective suggestions to improve existing services.

Experiences of burden, needs, rewards and resilience in family caregivers of people living with Motor Neurone Disease/Amyotrophic Lateral Sclerosis: A secondary thematic analysis of qualitative interviews.

BACKGROUND: Family caregivers of people with Motor Neurone Disease/Amyotrophic Lateral Sclerosis, an incurable, mostly rapidly fatal neurodegenerative disease, face many challenges. Although there is considerable research on caregiver burden in Motor Neurone Disease/Amyotrophic Lateral Sclerosis, there is less knowledge of the positive aspects of caring. OBJECTIVE: To explore the experiences of family caregivers of people with Motor Neurone Disease/Amyotrophic Lateral Sclerosis, specifically the relationship between positive and negative experiences of caring, and to identify possible ways to better support these caregivers. METHODS: Secondary thematic analysis of 24 semi-structured qualitative interviews conducted longitudinally with 10 family caregivers. Interviews explored rewarding and unrewarding aspects of caring. RESULTS: Themes emerged around burden, needs, rewards and resilience. Resilience included getting active, retaining perspective and living for the moment. Burden was multifaceted, including social burden, responsibility, advocacy, ambivalence, guilt and struggling with acceptance. Rewards included being helped and 'ticking along'. Needs were multifaceted, including social, practical and psychological needs. The four main themes were interrelated. A model of coping was developed, integrating resilience (active/positive), burden (active/negative), needs (passive/negative) and reward (passive/positive). CONCLUSION: Burden, resilience, needs and rewards are interrelated. Caregivers' ability to cope with caring for a person with Motor Neurone Disease/Amyotrophic Lateral Sclerosis oscillates between positive and negative aspects of caring, being at times active, at times passive. CLINICAL IMPLICATIONS: Coping is a non-linear process, oscillating between different states of mind. The proposed model could enable clinicians to better understand the caregiver experience, help family caregivers foster resilience and identify rewards, and develop appropriate individualised caregiver support plans.

Fighting for MND specialists.

Awareness of motor neurone disease (MND) is rising, but many patients struggle to get the care they need. MND specialist nurses provide support and help people navigate the system. Gordon Aikman has campaigned successfully for funding to double the number of nurses in Scotland.

Conducting research interviews with bereaved family carers: when do we ask?

BACKGROUND: Interviews with bereaved family carers to examine the end-of-life experience of the deceased are important tools for palliative care researchers, but the ethics of approaching the bereaved when they are grieving and vulnerable is often debated. OBJECTIVE: The aim of this study was to explore the insights of bereaved family carers about the most appropriate time to be involved in a research interview about the end of life and death of their family member. METHODS: This qualitative study used a social constructionist framework. Twenty-two bereaved family carers of people with motor neurone disease (MND) and cancer were interviewed in Western Australia. RESULTS: Most family carers (86%) feel comfortable being interviewed about the death of their family member within the first 5 months of bereavement, with 43% reporting they could be interviewed within weeks after death. Family carers reported that recall would be better earlier in bereavement and felt it may be helpful to them to talk about their experiences earlier. They said bereaved people should be allowed to decide for themselves when to be involved in an interview. CONCLUSIONS: These findings indicate that interviews with the bereaved may be most fruitful for researchers and beneficial to family carers when they are allowed to make the choice about timing for themselves, beginning weeks after the death of their family member.

The impact on the family carer of motor neurone disease and intervention with noninvasive ventilation.

BACKGROUND: The diagnosis of motor neurone disease (MND) has a profound effect on the functioning and well-being of both the patient and their family, with studies describing an increase in carer burden and depression as the disease progresses. AIM: This study aimed to assess whether patient use of noninvasive ventilation (NIV) impacted on their family carer, and to explore other sources of carer burden. DESIGN: The study used qualitative interviews and scaled measures of carer health and well-being completed at three monthly intervals until patient end of life. PARTICIPANTS: Sixteen family carers were followed up over a period ranging from one month to two years. RESULTS: NIV was perceived as having little impact on carer burden. The data however highlighted a range of sources of other burdens relating to the physical strain of caring. The Medical Outcomes Study Short Form (SF-36 Health Survey) Physical Component Summary (PCS) scores were considerably below that of the Mental Component Summary (MCS) score at baseline and at all following time points. Carers described the physical effort associated with patient care and role change; the challenge inherent in having time away; and problems relating to the timing of equipment and service delivery. CONCLUSIONS: NIV can be recommended to patients without concerns regarding increasing carer burden. The predominant source of burden described related to the physical impact of caring for a patient with MND. Services face challenges if this physical burden is to be reduced by providing equipment at an optimal time and successfully coordinating their input.

Integrated health care for patients with motor neurone disease.

This article presents the findings from a study trip to Kaiser Permanente (KP), a private healthcare provider in the USA. The aim of the trip was to understand how healthcare integration is managed in KP and how this might help patients in the UK with motor neurone disease (MND). This article makes reference to the American and British healthcare systems, identifying the simple differences between health economies, and their impact on health care, with specific reference to MND. The trip was undertaken as part of the author's ongoing work on how patients with MND rate services delivered by the multidisciplinary team (MDT) in the UK. The author's community matron role involves caring for patients with long-term conditions (LTCs) including long-term neurological conditions (LTNCs). In executing this role and in service delivery to patients with LTNCs, specifically MND, the author noticed a lack of robust integration, highlighting the need to consider and address the various contributory factors. This article presents a literature review and analyses the role of the MDT including specialist neurological professionals in executing duties and in delivering healthcare services to patients diagnosed with MND. The implications for practice are also presented along with areas for practice development.

A 10-year literature review of family caregiving for motor neurone disease: moving from caregiver burden studies to palliative care interventions.

BACKGROUND: There is growing awareness that different terminal diseases translate into different family caregiver experiences, and the palliative and supportive care needs of these families are both similar and unique. Family members caring for people with motor neurone disease may experience exceptional strain due to the usually rapid and progressive nature of this terminal illness. AIM: The purpose of this review is to synthesize contemporary research and provide a comprehensive summary of findings relevant to motor neurone disease family caregivers, as well as highlight some of the suggested interventions to alleviate burden and improve quality of life for this group. DESIGN: We conducted a comprehensive review of empirical research on family caregiving for people with motor neurone disease in peer-reviewed journals published in English, January 2000-April 2011. Fifty-nine studies met the inclusion criteria. RESULTS: This comprehensive literature review was consistent with previous research documenting the substantial burden and distress experienced by motor neurone disease family caregivers and revealed important points in the trajectory of care that have the potential for negative effects. The diagnosis experience, assisted ventilation, cognitive changes and end-of-life decision making create challenges within a short time. This review has also implicated the need for improvements in access to palliative care services and highlighted the absence of interventions to improve care. CONCLUSIONS: Caregiver burden and quality-of-life studies on motor neurone disease family caregivers have so far dominated the research landscape .The focus needs to be on developing interventions that provide direct practical and psychosocial supports for motor neurone disease family caregivers.

The need for support services for family carers of people with motor neurone disease (MND): views of current and former family caregivers a qualitative study.

PURPOSE: Family carers provide the majority of home-based care for people with motor neurone disease (MND). Carers' need for, and use of, support services are not fully understood; this study aimed to explore, from a qualitative perspective, the views of current and former family carers of people with MND. METHODS: A qualitative study was undertaken in Northwest England, using narrative interviews with current (18) and former (10) carers of a family member with MND. An optional longitudinal element involving diary completion was offered to the current carers. Data were analyzed using a thematic framework approach. RESULTS: Carer's needs vary, but encompass the provision of information and training, availability of respite care, counselling, and access to trained paid-for carers. CONCLUSIONS: There is need for a range of support services to be made available from which carers can select those most appropriate for them. Some support services are not always available for carers of this client group. There is a need for carers to access greater manual handling and training for physical care. Without sufficient support, carer burden can be overwhelming which may impact on the place of care of the patient and ultimately has implications for health and social care services.

Motor Neurone Disease family carers' experiences of caring, palliative care and bereavement: an exploratory qualitative study.

BACKGROUND: Motor Neurone Disease (MND) is a neurodegenerative disease with a sudden onset, a rapid progression, a profile of complex disabilities and fatal consequences. Caring for a person with MND is an unremitting commitment, yet little research has examined the experiences and needs of carers for palliative care and bereavement care. AIM: This study explored the experiences of MND family carers, both during their time as carers and following bereavement. Particular attention was paid to the carers' prolonged grief status and to the implications for service delivery, including palliative care. DESIGN: A qualitative approach consisted of interviews with 16 bereaved family carers. The Prolonged Grief tool (PG-13) measured the carers' prolonged grief. SETTING/PARTICIPANTS: sixteen family carers participated in the study, between one and four years after the death of their spouse from MND in Western Australia. RESULTS: The thematic analysis of the interview transcripts revealed five themes - the work of family carers, the change in relationship from spouse to family carer, family caring as a series of losses, coping mechanisms of family carers and supportive and palliative care experiences of family carers. The six participants who met the criteria for prolonged grief disorder accessed palliative care at a later stage in the disease trajectory. CONCLUSIONS: The study provided a basis for more research into the role palliative care services has in supporting MND carers before and after the death of their spouse and in particular the provision of more tailored respite and bereavement support.

Clinical measurement of limb spasticity in adults: state of the science.

Spasticity is a neuromuscular dysfunction characterized by tight or stiff muscles. Spasticity occurs across the spectrum of upper motor neuron disease and complicates the course and quality of life of those affected. Accurate and precise assessment of spasticity is the first step in providing safe and effective treatments to patients for management of spasticity. Examiner evaluations (Ashworth Scale, Modified Ashworth, and Visual Analog Scale) and patient self-reports (Visual Analog Scale and Numeric Rating Scale) are used to assess spasticity in clinical practice. We reviewed the biology of spasticity and summarized research that assessed properties of scores obtained from clinical scales when used in a variety of upper motor neuron diseases. The definition of spasticity was inconsistent. Rater reliability or agreement on clinical scales varied widely. Correspondence with electromyogram results was mixed. There was dissimilarity in patient reports and examiner assessments. Scores from clinical scales are responsive (decrease after initiation of treatment with known effectiveness), but the utility of scores for indexing individual change associated with the natural history of upper motor neuron disease is unknown. Future research incorporating patient reports and examiner findings over time will help to clarify the definition and capture the essence of spasticity.

Importance of nutrition in palliative care of patients with chronic disease.

Good nutrition is important in caring for patients with life-limiting illness, not only for meeting the body's physical requirements but also because of its associated social, cultural and psychological benefits. A decision to provide nutritional care and support requires an understanding of and respect for patients' wishes, as well as an appreciation of their expectations and the benefits and burdens of such support. The patient, his or her family and healthcare professionals should openly discuss and agree the goals of nutritional care and support at an early stage, rather than waiting until patients are unable to discuss their preferences with family and carers.

The one-stop shop.

Motor neurone disease is a devastating condition which needs expert interventions. Now some patients have access to a fast-track service.

How people with motor neurone disease talk about living with their illness: a narrative study.

AIM: This paper is a report of a study which explores patients' experiences and how they talk about living and coping with motor neurone disease. BACKGROUND: Living with motor neurone disease is challenging, frightening and disabling. It leads to progressive physical decline, normally with a prognosis of 3-5 years. Patients have to deal with many problems, including loss of mobility and the inability to communicate. There is little evidence about how people manage these problems or live with this illness. METHOD: Narrative case studies were used, the unit of analysis being a patient living in their own home or a care home. Thirteen adults were recruited through purposeful sampling. Longitudinal narrative interviews were conducted at three-monthly intervals over an 18-month period in 2005-06. Interviews were analysed focusing on the form and content of the patients' narratives. FINDINGS: Four types of narrative, or storyline were identified. The sustaining storyline is about living life as well as possible through keeping active and engaged in life. In contrast, the enduring storyline concerns living in an insurmountable situation leaving the person feeling disempowered, unable to fight for life or against death. Survival is the essence of the preserving storyline, while the fracturing storyline concerns loss and fear of what is to come. CONCLUSION: Storylines help make sense of complex narratives by encouraging closer attention and active listening to the stories and serve as organizing threads to help patients, families and healthcare professionals better understand living with motor neurone disease.