OBJECTIVES: To consider the impact of juvenile Huntington disease (JHD) from a biomedical, symptom burden, and total pain palliative care perspective.
METHODS: This case report was informed by a narrative review of the literature with inclusion of expert opinion from pediatric palliative care, an adult and pediatric neurologist, and a child psychiatrist. Audio-recorded qualitative interview and coauthorship with the pediatric patient's primary caregiver (his mother).
RESULTS: The JHD impacts all domains of child and family function.
SIGNIFICANCE OF RESULTS: Application of the concept of total pain to JHD informs and guides care for this complex, challenging condition.
OBJECTIVES: Mechanical ventilation (MV) has been shown to improve survival and quality of life in motor neuron disease (MND). However, during the progression of MND, there may come a point when MV is no longer felt appropriate. Association of Palliative Medicine Guidelines have been recently published to help clinicians withdraw MV at the request of patients with MND in a safe and compassionate manner to ensure that symptoms of distress and dyspnoea are minimised.
METHODS: In this report, we discuss the palliative and ventilatory management of six ventilator-dependent patients with MND who had requested the withdrawal of MV as part of their end-of-life care.
RESULTS: We have withdrawn MV from six patients with MND at their request and our practice has been influenced by the Association of Palliative Medicine Guidelines.
CONCLUSION: Withdrawal of MV in MND at a patient's request is challenging but is also a fundamental responsibility of healthcare teams. We discuss the lessons we have learnt which will influence our practice and help other teams in the future.
OBJECTIVES: To compare characteristics and needs of inpatients with neurologic disease to those with cancer referred for palliative care (PC) consultation.
METHODS: This prospective cohort study used data collected by the Palliative Care Quality Network from January 2013 until December 2016. We compared demographics, reasons for consultation, discharge disposition, Palliative Performance Scale (PPS) score, and outcomes of care among patients with a primary diagnosis of neurologic disease vs cancer.
RESULTS: The most common reason for PC consultation in all patients was assistance with goals of care and advanced care planning. PC consultation was less often requested for pain and symptom management in patients with neurologic disease compared to patients with cancer (13.7% vs 43%, odds ratio 0.3) and more often for assistance with transition to comfort measures only and withdrawal of life-sustaining treatment (19.1% vs 7.1%, odds ratio 1.3). Patients with cancer had higher PPS scores (42.1% vs 23.4%) and were more likely to be discharged home from the hospital, while patients with neurologic disease were more likely to die in hospital.
CONCLUSIONS: Patients with neurologic disease as a reason for PC consultation are more in need of end-of-life care planning and more likely to die in the hospital than those with cancer, suggesting that targeted approaches may best address the needs of each patient population. Our results can direct further research and education in neuropalliative care.
Sam, 9 ans, fait sa rentrée dans une nouvelle école et doit affronter de nouveaux copains avec la tristesse d'un petit garçon qui vient de perdre sa petite soeur d'une maladie neurodégénérative.
Nous le suivons dans sa nouvelle vie avec toutes ses questions, ses doutes, ses chagrins, les rencontres qui le consolent, les premières fois sans... Et les sentiments qui l'assaillent à la naissance d'une autre petite soeur.
Palliative care has a very important role in the care of patients with motor neurone disease and their families. There is increasing emphasis on the multidisciplinary assessment and support of patients within guidelines, supported by research. This includes the telling of the diagnosis, the assessment and management of symptoms, consideration of interventions, such as gastrostomy and ventilatory support, and care at the end of life. The aim of palliative care is to enable patients, and their families, to maintain as good a quality of life as possible and helping to ensure a peaceful death.
A terminally ill man with motor neurone disease has urged MPs to change the law to allow assisted dying in the UK in an open letter sent on the day of his assisted death in Switzerland, in which he recounted the distress caused to him and his family during a police investigation.
Geoffrey Whaley, 80, was given a diagnosis of motor neurone disease in 2016. With no legal option of assisted dying in the UK, he decided to travel to Dignitas in Switzerland to end his life.
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Patients with neurologic illnesses are commonly encountered by palliative care (PC) clinicians though many clinicians feel uncomfortable caring for these patients. Understanding how to diagnose, treat, communicate with, and prognosticate for neurology patients will improve the confidence and competence of PC providers in the neurology setting. This article offers PC providers 10 useful tips that neurologists with PC training think all PC providers should know to improve care for patients with neurologic illness.
Background: Chronic progressive neurological diseases like high grade glioma (HGG), Parkinson's disease (PD), and multiple sclerosis (MS) are incurable, and associated with increasing disability including cognitive impairment, and reduced life expectancy. Patients with these diseases have complex care needs. Therefore, timely advance care planning (ACP) is required. Our aim was to investigate timing and content of discussions on treatment restrictions, i.e., to initiate, withhold, or withdraw treatment in patients with HGG, PD, and MS, from the neurologists' perspective. Methods: We performed a national online survey amongst consultants in neurology and residents in The Netherlands. The questionnaire focused on their daily practice concerning timing and content of discussions on treatment restrictions with patients suffering from HGG, PD or MS. We also inquired about education and training in discussing these issues. Results: A total of 125 respondents [89 neurologists (71%), 62% male, with a median age of 44 years, and 36 residents (29%), 31% male with a median age of 29 years] responded. Initial discussions on treatment restrictions were said to take place during the first year after diagnosis in 28% of patients with HGG, and commonly no earlier than in the terminal phase in patients with PD and MS. In all conditions, significant cognitive decline was the most important trigger to advance discussions, followed by physical decline, and initiation of the terminal phase. Most discussed issues included ventilation, resuscitation, and admission to the intensive care unit. More than half of the consultants in neurology and residents felt that they needed (more) education and training in having discussions on treatment restrictions.
Conclusion: In patients with HGG discussions on treatment restrictions are initiated earlier than in patients with PD or MS. However, in all three diseases these discussions usually take place when significant physical and cognitive decline has become apparent and commonly mark the initiation of end-of-life care. More than half of the responding consultants in neurology and residents feel the need for improvement of their skills in performing these discussions.
OBJECTIVES: An increasing number of patients with motor neuron disease (MND) in the UK and Ireland use assisted ventilation, and a small proportion of these use long-term tracheostomy ventilation (TV).1 2 NICE guidelines recommend that patients with MND should routinely receive specialist palliative care input. The aim was to establish the extent to which hospices and specialist palliative care units (SPCUs) in the UK and Ireland currently manage patients with MND using assisted ventilation especially TV and to identify any associated barriers.
METHODS: A 25-item questionnaire was developed in Survey Monkey. A link to the questionnaire was emailed to every medical director (n=185) of inpatient hospices/SPCUs in the UK and Ireland.
RESULTS: The response rate was 42.4% (n=78). 97.4% of units admit patients with MND on non-invasive ventilation (NIV), but only 28.2% admit those using TV. 80.8% of units have adequate expertise in the management of NIV, compared with 7.7% for managing TV. 35.9% and 2.6% of units have a policy for managing patients using NIV and TV, respectively. 14.1% respondents had been involved in the care of patients with MND using TV, in the specialist palliative care setting in the last 5 years.
CONCLUSIONS: A minority of UK and Irish hospices/SPUs provide support to TV MND patients and few units currently have management or admission policies for this cohort of patients. Respondents indicated a lack of appropriate expertise and experience. Further exploration of these barriers is required to establish how to optimise care for TV MND patients in this setting.
Dans le cadre de la fin de vie d'une maladie neurodégénérative, les praticiens n'ont pas engagé leur responsabilité en refusant de mettre en oeuvre une réanimation active, qui aurait été scientifiquement inadaptée. La règle du consentement aux soins ne dérive pas vers un libre choix du traitement (CAA de Lyon, 14 mai 2018, n° 16LY02121).
Origine : BDSP. Notice produite par APHPDOC R0xFIH77. Diffusion soumise à autorisation
Background: The use of specific triggers has been suggested to help identify patients with progressive neurological disease who would benefit from palliative care.
Aim: This study aimed to improve the evidence base for the use of triggers for patients with progressive neurological disease.
Design: An evaluation of palliative care services was undertaken using a retrospective case note review of the timing and presence of triggers in the last 2 years of life.
Setting/Participants: A total of 12 specialist palliative care units across the United Kingdom provided data from 300 patients: mean patient age 70 years, 50% male, diagnoses included motor neurone disease 58%, Parkinson's disease 17% and Parkinson's Plus syndromes 12%.
Results: There was a high burden of triggers - 17 in the last 2 years of life and 10 in the last 6 months of life. The most frequent triggers were deteriorating physical function, complex symptoms and dysphagia. Four factors were found to explain 64% of the total variance: Factor 1 - Deterioration in physical function, dysphagia, significant complex symptoms and pain; Factor 2 - Weight loss and respiratory symptoms; Factor 3 - Recurrent infections and cognitive decline; Factor 4 - Aspiration pneumonia. Cox regression analyses found different triggers were associated with survival from diagnosis versus survival from referral to palliative care. Different triggers were also associated with survival for different neurological conditions.
Conclusion: This study demonstrates that there is a high burden of triggers in the last months and years of life and that these could potentially be reduced to fewer components. Prospective studies assessing which triggers are useful for different conditions are now required.
BACKGROUND/OBJECTIVES: Although Alzheimer disease and other dementias are life limiting, only a minority of these patients or their proxy decision makers participate in advance care planning. We describe end-of-life care preferences and acute care and hospice use in the last 6 months of life for persons enrolled in a comprehensive dementia care management program.
DESIGN: Observational, retrospective cohort.
SETTING: Urban, academic medical center.
PARTICIPANTS: A total of 322 persons enrolled in dementia care management after July 1, 2012, who died before July 1, 2016.
INTERVENTION: Dementia care comanagement model using nurse practitioners partnered with primary care providers and community organizations to provide comprehensive dementia care, including advance care planning.
MEASUREMENTS: Advance care preferences, use of Physician Orders for Life Sustaining Treatment (POLST), hospice enrollment, and hospitalizations and emergency department (ED) visits in the last 6 months of life obtained from electronic health record data.
RESULTS: Nearly all decedents (99.7%, N = 321) had a goals-of-care conversation documented (median = 3 conversations; interquartile range = 2-4 conversations), and 64% had advance care preferences recorded. Among those with recorded preferences, 88% indicated do not resuscitate, 48% limited medical interventions, and 35% chose comfort-focused care. Most patients (89%) specified limited artificial nutrition, including withholding feeding tubes. Over half (54%) had no hospitalizations or ED visits in the last 6 months of life, and intensive care unit stays were rare (5% of decedents). Overall, 69% died on hospice. Decedents who had completed a POLST were more likely to die in hospice care (74% vs 62%; P = .03) and die at home (70% vs 59%; P = .04).
CONCLUSIONS: Enrollees in a comprehensive dementia care comanagement program had high engagement in advance care planning, high rates of hospice use, and low acute care utilization near the end of life. Wider implementation of such programs may improve end-of-life care for persons with dementia.
RESEARCH AIMS: The motivations that lead to wishes to die (WTD) in palliative care patients with cancer are relatively well studied. But little is known about WTD in other pathologies and the relation between subjective understandings of dying trajectories and a WTD. We investigated the WTD of palliative patients in four different dying trajectories: neurological diseases, organ failure, frailty due to age, and cancer.
STUDY POPULATION: 62 palliative cancer (n = 30) and non-cancer (n = 32) patients (10 neurological disease; 11 organ failure; 11 frailty), their families and health professionals in different palliative care settings (248 interviews).
STUDY DESIGN AND METHODS: Qualitative semi-structured interviews. Data analysis through Interpretive Phenomenological Analysis and Grounded Theory.
RESULTS: In addition to personal motivations, we found that people dealing with similar trajectories were often confronted with similar questions and concerns due to similar challenges. For four trajectories we show typical patterns, similarities and differences that should be considered when talking with patients about their WTD. These illness-related considerations do not explain the WTD completely, but give important information on the challenges for particular patient groups that might experience a WTD. In all patient groups, there were clear moments that triggered a WTD: for neurological patients it was experiencing breathlessness, high-dependency care, or when considering tube feeding or respiratory support; for persons with organ failure it was an acute burdensome crisis; for patients with cancer after the initial diagnosis, it was the first relapse or the move into advanced palliative care; for elderly frail persons it was the move into care facilities, or the loss of important relationships or capabilities. The feeling of being a burden to others was reported in all patient groups.
INTERPRETATION: WTD can be triggered within disease trajectories by specific conditions and transitional points that affect agency and self-understanding. A better understanding of the concerns and challenges of a particular dying trajectory as well as its characteristic trigger points can facilitate early and comprehensive communication about patients' WTD, and the underlying motivations and protective factors.
À l'âge de treize mois, Gaspard est diagnostiqué de la maladie de Sandhoff, maladie neuro-dégénérative rare, incurable.
Dans un témoignage aussi émouvant que salutaire, écrit à quatre mains, ils racontent leur cheminement et la façon dont la maladie a radicalement changé leur regard sur la vie.
Se sachant condamnée, l'auteure, souffrant d'une maladie neuro-dégénérative, découvre le poème d'E. Dickinson qui donne son titre à cet essai. Créatrice de son jardin en Toscane, elle trouve son havre de paix dans ces vers sur le jardinier et la mort. Avant de décéder dans sa propriété, elle se rapproche de plus en plus de son jardin et s'y réfugie, profitant jusqu'au bout de la beauté de la nature.
Management of patients with terminal brain disorders can be medically, socially, and ethically complex. Although a growing number of feasible treatment options may exist, there are times when further treatment can no longer meaningfully improve either quality or length of life. Clinicians and patients should discuss goals of care while patients are capable of making their own decisions. However, because such discussions can be challenging, they are often postponed. These discussions are then conducted with patients' health care proxies after patients lose the capacity to make their own decisions. Disagreements may arise when a patient's surrogate desires continued aggressive interventions that are either biologically futile (incapable of producing the intended physiologic result) or potentially inappropriate (potentially capable of producing the patient's intended effect but in conflict with the medical team's ethical principles). This article explores best practices in addressing these types of conflicts in the critical care unit, but these concepts also broadly apply to other sites of care.
Introduction: Very little has been written on seizure management in palliative care (PC). Given this situation, and considering the forthcoming setting up of the Palliative Care Unit at our neurorehabilitation centre, the Clínica San Vicente, we decided to establish a series of guidelines on the use of antiepileptic drugs (AEDs) for handling seizures in PC.
Methods: We conducted a literature search in PubMed to identify articles, recent manuals, and clinical practice guidelines on seizure management in PC published by the most relevant scientific societies.
Results: Clinical practice guidelines are essential to identify patients eligible for PC, manage seizures adequately, and avoid unnecessary distress to these patients and their families. Given the profile of these patients, we recommend choosing AEDs with a low interaction potential and which can be administered by the parenteral route, preferably intravenously. Diazepam and midazolam appear to be the most suitable AEDs during the acute phase whereas levetiracetam, valproic acid, and lacosamide are recommended for refractory cases and long-term treatment.
Conclusions: These guidelines provide general recommendations that must be adapted to each particular clinical case. Nevertheless, we will require further well-designed randomised controlled clinical trials including large samples of patients eligible for PC to draft a consensus document recommending adequate, rational, and effective use of AEDs, based on a high level of evidence, in this highly complex area of medical care.
BACKGROUND: Most of the neuromuscular disorders (NMDs) have poor prognosis and lead to various symptoms amendable to palliative care. However, the suitable time of referral is uncertain.
METHODS: A retrospective study was conducted to describe the trajectories of NMDs. Early death within one year after NMD diagnosis was set as the outcome. Total of 86 adult NMD patients were recruited in a university hospital. Demographic variable including gender, age at diagnosis and early-onset symptoms including dyspnea, dysphagia, loss of mobility, constipation, mood and sleep disorders, and pain were correlated with the outcome. Prediction models for early death were tested.
RESULTS: Age at NMD diagnosis, early-onsets dyspnea, dysphagia, constipation and impaired mobility were found to have statistically significant correlation with early death. A prediction model consisted of these four factors had area under receiver operating characteristic (ROC) curve of 0.919.
CONCLUSIONS: Elder age at NMD diagnosis, early-onset dyspnea, dysphagia, constipation and impaired mobility within the first year after NMD diagnosis may predict mortality within the first year after diagnosis. It may provide guidance to clinicians for early palliative care referral in this patient group.
In neurology, many diseases are still incurable and have a fatal outcome. Palliative care (PC) definitely has a role in neurology. We discuss the screening criteria for PC referral, known as 'triggers' in different neurological diseases. Different clinical settings including in-patient and out-patient are covered. We hope this review can remind clinicians to involve PC in the appropriate patient group. Further clinical studies are expected to validate the triggers and trajectories of various neurological diseases.