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Palliative care in chronic progressive neurological disease
Changing perspectives
Seeber, A.A.
Publication date
2019
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Citation for published version (APA):
Seeber, A. A. (2019). Palliative care in chronic progressive neurological disease: Changing
perspectives.
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general DisCussion anD
Future PersPeCtives
introDuCtion
Patients with chronic progressive neurological disease share ongoing deterioration in functioning and are faced with a host of physical, cognitive, emotional, and existential problems. there is growing evidence that an early palliative care approach improves these patients’ well-being and thus quality of life. nevertheless, in daily neurological practice this approach is usually not implemented on time leading to both under- and overtreatment.
We aimed to investigate whether the discussion on treatment restrictions is an appropriate tool to timely start palliative care in patients with chronic progressive neurological disease. after all, in daily clinical practice this discussion appears to be the first moment that the neurologist or his patient explicitly considers that quality of life, not life expectancy, may become the most important treatment aim. initially, we thus focused on the actual timing and content of discussions on treatment restrictions between neurologists and their patients, and on the consequences of these discus-sions for the management of the individual patient’s further treatment.
our first research question was:
1. Does the discussion on treatment restrictions with patients suffering from chronic progressive neurological disease mark the start of palliative care?
We performed a review of the international literature on existing knowledge about treatment restrictions in chronic progressive neurological disease. subsequently we conducted in-depth interviews and a national online survey for neurologists evaluating timing and content of discussions on treatment restrictions. We found that discussions on treatment restrictions were not appropriate for marking a timely start of palliative care. therefore, we had to change our second research question ‘Which recommenda-tions can be given for appropriate timing of the discussion on treatment restricrecommenda-tions for the different diseases and illness trajectories?’ to:
2. Which recommendations can be formulated for timely integration of palliative care for the different chronic progressive neurological diseases and illness trajectories? We decided to evaluate an already existing policy of early integration of palliative care, i.e. the policy of the amsterdam tertiary als Center’s outpatient clinic. By means of a qualitative study including observations of the office hours and in-depth interviews with patients with amyotrophic lateral sclerosis (als) or progressive muscular atrophy (PMa), we aimed to evaluate this policy and formulate recommendations for timely integration of palliative care in the follow-up of patients with other chronic progressive neurological diseases.
topic. this was a deliberate choice, based on the following considerations. First, the project was inspired by the challenges which the principal investigator and other neurologists (in training) experienced in daily practice with timely discussions on treatment restrictions. second, we felt that the treating specialist, e.g. the neurologist, has the final responsibility in delivering patient-centered care. evaluating his role in discussing treatment restrictions and starting palliative care would be an important first step in the attempt to improve daily practice. third, a holistic evaluation of the topic, including experiences and opinions of paramedics and caregivers about actual practice and possible improvements, would simply have exceeded the time, manpower and resources which were available for this project.
in the next paragraphs, we present the answers to the key questions of this research project and our recommendations, with the input of the growing body of literature on the topic. We will then critically appraise our project and finally present ideas for further research.
1. Does the discussion on treatment restrictions with patients suffering
from chronic progressive neurological disease mark the start of
pallia-tive care?
1.1 Discussions on treatment restrictions in daily clinical practice
Before this project officially started we performed a pilot study consisting of several in-depth interviews with residents and consultants in neurology working at academic and non-academic hospitals in amsterdam, the netherlands. the results of these interviews were in line with our own experiences, i.e., discussions of treatment restrictions did not take place during follow-up at the outpatient clinic, but were mostly initiated during acute admission of severely ill patients who usually were incapable to participate at that moment.
our systematic review on actual timing and content of discussions on treatment restrictions showed that conclusive empirical data about this topic are missing. the sparse information we could evaluate suggested that specialists in various Western World countries discuss treatment restrictions mainly in (acute) life-threatening situations, caused by either the disease itself or treatment-related complications. By then, patients suffering from stroke or dementia appeared to be incapacitated so that they could not take part in the discussions. even in patients with amyotrophic lateral sclerosis (als), which is generally considered a ‘model disease’ concerning timely discussions on treatment restrictions, decisions about treatments such as assisted ventilation and feeding were quite often forced by sudden deterioration. nevertheless, some als studies indicated that timely discussions about possible treatment options rather than treatment restrictions improved decision-making on end-of-life issues for
patients and their families. another important finding of our review was the lack of empirical data concerning discussions on treatment restrictions regarding patients suffering from high-grade gliomas (hgg), multiple sclerosis (Ms) and Parkinson’s disease (PD). (Chapter 2)
in our national survey, the latter aspect was the reason why we focused on timing and content of discussions on treatment restrictions in patients suffering from one of these three conditions. given the relatively low response rate (15%) of the approached neuro-logists working as residents or consultants across the whole country, the results of this study have to be interpreted with caution. however, we assume that the number of participants represents a fair proportion of the neurological practices since in most Dutch hospitals subspecialized neurologists care for patients with hgg, Ms or PD, respectively. the results seemed to indicate that, in general, discussions on treatment restrictions take place rather late in the course of the diseases, except for discussions about do-not-resuscitate (Dnr)-orders in patients with hgg. again, discussions appeared to be mainly triggered by distinct deterioration of patients which often marked – according to the participants – the start of the palliative phase. For more than half of them this phase was the terminal, i.e. dying phase. the participants reported that significant cognitive decline was one of the most important reasons to initiate discus-sions on treatment restrictions, and therefore one might conclude that at the moment discussions took place decision-making capacity was impaired in a fair proportion of patients. (Chapter 3)
the in-depth interviews with neurologists about timing and content of discussions on treatment restrictions done in the exploring phase of the whole project had yielded the same results. When asked to report about two recent examples of these discussions, more than 90% of the interviewees had selected cases admitted to hospital after se-vere stroke who, at that moment, were too ill to take part in discussions and treatment decisions. in general, discussions on treatment restrictions were discussions on end-of-life care and done on behalf of the patients with the patients’ cargivers. (Chapter 4 and unpublished data)
1.2 Misconceptions about discussions on treatment restrictions
in the general introduction section we already mentioned that the term ‘treatment restriction’ could erroneously give the impression that treatment codes are exclusively about a reduction of treatment options, whereas the codes were developed to identify which treatment options are appropriate and proportionate for the specific patient. interestingly, synchronously with this research project, the term ‘treatment restriction’ was replaced by the term ‘treatment agreement’ in many Dutch hospitals.
discussions on treatment restrictions to the last phase(s) of life.1 Both general
practitioners and medical specialists (studies have mainly been done in various fields of internal medicine) gave the same reasons for postponing discussions on treatment restrictions or not discussing them at all. in summary, they experience difficulties in prognostication and do not want to diminish patients’ hope ‘earlier than necessary’ and, in line with that, ‘they do not want to harm the patient or jeopardize the doctor-patient relationship’.2-6 the in-depth interviews with residents and consultants in neurology in
the exploring phase of this project revealed the same arguments and the specialists’ struggle with the right timing and (amount of) information when patients do not show distinct physical or cognitive deterioration. (Chapters 3, 4 and unpublished data) For many health care professionals, including neurologists, the term ‘treatment restriction’ seems to be related to the end of life of patients in life-threatening situations, due to either the disease itself or complications. thus, as far as we know discussions on treatment restrictions are mainly done not earlier than in the last phase(s) of life, Consequently, a considerable number of patients cannot be fully involved in both discussions and decisions about the appropriate treatment plan. as the literature shows, treatment decisions of surrogate caregivers often do not reflect patients’ choices properly.7
1.3 Misconceptions about palliative care
Both the in-depth interviews and the national survey amongst neurologists showed a lack of knowledge of palliative care. this is not exclusively a Dutch problem - or a problem of neurology alone.1 as discussed above, recent research done in various
(Western world) countries indicates that the majority of health care professionals erroneously perceive palliative care as synonymous with care of the imminently dying patient.8 this leads to delayed introduction of palliative care in the vast majority of
cancer and non-cancer patients. Consequently, medical specialists, including neuro-logists, frequently have difficulties with timely and adequate assess- and treatment of various symptoms, including depression, fatigue, pain, loss of weight and sleeping problems which have a negative impact on their chronically ill patients’ condition, and thus quality of life.9-13 Furthermore, patients with incurable non-cancer conditions have
less access to palliative care specialty teams including hospices.14 except for patients
with als and gliomas, hospice deaths are still uncommon for patients with other neurological diseases such as PD and Ms.15-17 For patients with dementia a recent
study found that although thirty percent of patients with advanced dementia went to a hospice, 25% of hospice stays were 3 days or less.18
in addition, both recent literature and our project show that the average health care professional - and in our study the neurologist (in training) - has difficulties discussing a treatment restriction or addressing end-of-life issues including anxiety and despair.2-6
in the in-depth interviews, neurologists provided several reasons for their restraint to initiate and continue discussions on these topics, next to the ones mentioned above: insufficient time, mainly in the outpatient clinic’s setting; probably not being the responsible physician anymore when end-of-life wishes have to be translated into practice; and lack of explicit or documented wishes of patients to be engaged in end-of-life decision-making. (Chapter 3, 4 and unpublished data) We are of the opinion that every physician should (learn to) be familiar and comfortable with communicating these topics, which can be regarded as a fundamental palliative care skill.1
the recent literature indicates too that the prevailing idea of palliative care amongst medical specialists is that it is about care in the last phase(s) of life. it is very likely that the link to the concept of treatment restrictions, equally related to the end of life, would sustain this misconception. Currently, the optimal palliative care approach as described in the general introduction is not to take treatment restrictions as a starting point, but rather to discuss treatment options in the broadest sense and as soon as needed in chronic progressive (neurological) disease.
in sum, our answer to the first research question is that the link between discussions on treatment restrictions and the initiation of palliative care for patients with chronic progressive neurological disease does not render justice to what we currently consider to be state-of-the-art palliative care. We argue that these two concepts should be uncoupled to help establishing the palliative care approach which is urgently needed in neurological practice.
2. Which recommendations can be formulated for timely integration of
palliative care for the different chronic progressive neurological
dis-eases and illness trajectories?
Following the conclusion of the first research question, we decided to evaluate an already existing policy on early integration of palliative care in a chronic neurological disorder, i.e. als, by observing patients’ visits in the amsterdam als Center outpatient clinic and by interviewing these patients.
2.1 Early integration of palliative care in MND
We started our evaluation with non-participating observations of the regular follow-up of patients suffering from amyotrophic lateral sclerosis (als) or progressive muscular atrophy (PMa) with their own motor neuron diseases (MnD) specialist (a dedicated rehabilitation physician) and his specialist nurse and noticed the following: (1) there was not a single particular moment to discuss and decide about supportive treatment options and/or treatment restrictions. rather, discussions on the appropriate treat-ment plan was part of an ongoing communication process. (2) in this context Dnr-orders and other advance directives (aDs) became a tool to pursue discussions, rather
than the ultimate goal of consultations. (3) the ongoing communication process was facilitated by both clearly structured follow-up appointments and concrete, routinely used communication strategies of the MnD specialist and his assistant. ’setting the agenda’ and a custom-made approach helped preparing patients for the biannual update on earlier discussed aDs during the next appointment. repetition and titration of information in light of the patients’ (physical) decline and symptom burden allowed the specialist to stay close to how the individual patient perceived his own situation. Furthermore, it made exchange of medical information meaningful and facilitated tailor-made decisions. (Chapter 6)
since the palliative care approach starts at the very moment that the diagnosis als or PMa is given, we expanded our study by observing two-tiered bad news appointments of the amsterdam als Centre. here, the fact that the patient suffered from a rapidly progressive and fatal disease was reason to discuss the diagnosis and its consequences twice within two weeks and to refer the patients immediately to a multidisciplinary care team for further follow-up. Main findings of both obser-vations and in-depth interviews with patients with als or PMa included: (1) Patients considered the straightforward and full disclosure of the devastating news unavoidable — and manageable as short-term follow-up was offered. (2) the time between appoint-ments provided the opportunity for a first reorientation. (3) in line with that, the second appointment could be used for detailed discussions of various aspects of the diagnosis and its consequences and to develop a plan for the palliative treatment trajectory. (Chapter 5)
With this part of the research project, observations of bad news conversations and follow-up of patients with als or PMa, followed by in-depth interviews, we were able to study a feasible practice of early integrated palliative care.
2.2 Early integration of palliative care in all chronic progressive neurological diseases: a prerequisite for good patient care
Both our results of the observations of the clinical practice in the amsterdam als Centre and recently published papers on palliative care in neurology indicate that palliative care must be considered early in the trajectory of all progressive chronic neurological diseases.
First, research has shown that patients in general face a crisis when they receive the diagnosis of an incurable disease, no matter the precise diagnosis or the estimated speed of decline.5,19-21 second, though there is still a lack of empirical data on
neuro-logical patients’ preferences regarding exact timing and detailedness of prognosis and treatment information, there is some first evidence of the need of pro-active dissemination of information, education and (psychosocial) support for patients with
e.g. hgg, Ms, PD and disability after stroke.22-27 in addition, many of these patients expect
their health care professionals to bring up these issues.12,23,28-30 our project underlines
that clear communication of the diagnosis should also include information about the (para-)medical follow-up, and that this follow-up should be offered at short notice. (Chapter 5) as has been shown before, easy access to the medical team in the period after diagnosis helps patients to understand better what the diagnosis ‘incurable disease’ means in the individual context and where to find professional support.31,32
Follow-up at short notice also contributes to the professional patient-doctor relation-ship as it minimizes patients’ feeling of abandonment.33,34
third, just like patients with MnD, patients with other chronic progressive neurological diseases need regular follow-up, also in early stages of diseases. there is growing evidence that symptoms like pain, fatigue, cognitive and behavioral problems can occur quite early in the course of both rapidly progressive and slowly progressive chronic neurological diseases. as these symptoms may (severely) interfere with patients’ functioning in daily life, they need to be addressed in a timely manner (see also paragraph 3). Furthermore, research indicates that patients with e.g. gliomas, Ms and PD want ongoing monitoring of motor and non-motor symptoms, education about their disease and involvement in decision-making.12,20,28 our evaluation of the
amsterdam als Centre’s palliative care approach also suggests that an ongoing process of (re)evaluation of patients’ status quo may reinforce itself. the predictabil-ity of what will happen during the next appointment(s), including regular discussions of supportive treatment options and advance directives (aDs), seemed to make communication less delicate for both the health care professional(s) and the patients. (Chapter 6)
in conclusion, we are of the opinion that palliative care should be integrated in the follow-up of patients with any chronic progressive neurological disease, from diagnosis onwards. Furthermore, we argue that the iterative way in which supportive treatment options were discussed at the studied tertiary als center may be a useful way to realize this integration. We will elaborate on this in the following paragraph.
2.3 Advance care planning: facilitating early integration of palliative care in chronic progressive neurological diseases
the systematically organized and ongoing ‘process of communication that supports people at any age or stage of the disease in understanding and sharing their per-sonal values, life goals, and preferences regarding future medical care’ is called ad-vance care planning (aCP).35 the aim of aCP is to obtain well-considered treatment
preferen-ces on time, that is to say before patients become incapable to take part in decision-making. aCP may result in a written aD. lately, experts in the field warn against a document-driven or ‘tick box’ approach to aCP in which the focus lies on the
use of specific aCP tools to reach legally valid documents about patients’ preferences. increasing the completion rate of written aDs does not necessarily improve end-of-life care.36,37 the experts rather advise to focus on the dynamic communication process
itself throughout the illness trajectory.38,39 importantly, in daily practice the concept of
aCP is mainly used in the context of progressive illness and anticipated deterioration, and in the context of the end of life.12,39-41
looking at our evaluation of the amsterdam als Centre’s aCP approach, one might conclude that we investigated a rather well-implemented and complete communication practice. We observed an ongoing assessment of patients’ status quo, including actual expectations and wishes, leading to patient-centered care trajectories. the iteration was the mainstay of the process, and checking the validity of (earlier discussed) aDs only part of it. in line with that, we argue that aCP as systematically organized and ongoing communication process is indeed more than just a tool to discuss treatment wishes and preferences for (future) treatment options . as outlined above it may enable the development of a care plan that is mutually acceptable for patients, caregivers and healthcare professionals.42 likewise, establishing this communication process may
help integrating questions about advance directives more easily, even in early stages of chronic progressive neurological disease.
During the evaluation of the amsterdam als Centre’s actual practice of aCP we observed that the agendas of the medical team and patients regularly differed: while the MnD specialist was for example very concerned about the timely discussion on ventilatory support options for his patient with decreasing respiratory function, the patient could struggle much more with the question whether to finally surrender to a wheelchair. By means of structured evaluation of the patient’s condition and preferences both issues could be addressed in a mutually satisfactory way. in our view, this finding is another important argument to regard aCP as distinctive feature of good (long-term) medical care and not as a distinct activity to make well-informed decisions on future treatment options and end-of-life issues.42
We argue that such a broadened understanding of aCP could well be applied to patients with any other chronic progressive (neurological) disease, from diagnosis onwards. translating the findings from the amsterdam als Centre’s approach, we suggest that after diagnosis this communication process should focus on general information about the disease and where to find professional support, and on discussions about end-of-life perspectives and preferences whenever needed. During follow-up, aCP may be facilitated by regular appointments and thus regular discussions on the actual physical, cognitive, social and existential needs of the patient and his caregivers, and regular reflections on the appropriate (amount of) treatment, at short notice and on the long run in light of imminent cognitive deterioration.
We conclude that the answer to the second research question indicates that palliative care should be integrated in patient care as soon as the diagnosis of any chronic progressive neurological disease is given. after all, palliative care can be used along-side curative treatments throughout the whole course of chronic progressive neuro-logical diseases. Furthermore, we suggest that aCP may facilitate this integration when it is implemented as an ongoing professional communication process during structured follow-up.
in the next paragraph, we will discuss several challenges regarding our general recommendations.
3. Challenges to integrate palliative care
early integration of palliative care was investigated in a specific setting, i.e. a single tertiary referral centre for als. as outlined in the general introduction, patients with als and PMa have a unique set of features which allow no delay in anticipating to the future with rapidly progressive symptom burden. Prognosis of physical and cognitive decline, and of (late-stage) complications may be less predictable in other chronic neurological diseases which are more slowly progressive and for which initially disease-modifying treatment is available. indeed, ‘unpredictability of the individual patient’s illness trajectory’ is an often mentioned argument given by professional caregivers, including the interviewed neurologists of this research project, to postpone discussions about the appropriateness of (future) treatment.1 We argue, however,
that chronic progressive neurological diseases share many characteristics which justify early integration of palliative care, irrespective of diverging (individual) illness trajectories.
First, in many chronic progressive neurological diseases early cognitive impairment may develop. in addition, dys- or anarthria may also hamper communication. up to 79% of patients with hgg were found to have cognitive impairment before treatment, and more than 50% lack full decision-making capacity four months after diagnosis.43,44
in patients with Ms and PD behavioral and cognitive problems can also occur rather early in the course of disease and may affect many aspects of daily life.45-48 Cognitive
impairment is common in the acute stage after stroke. new-onset cognitive decline or worsening of cognitive impairment has been reported in up to 50% of those who have survived a stroke.49 Patients with dementia present with cognitive and/or behavioral
problems. We argue that imminent cognitive and communication impairment, includ-ing dysphasia and dysarthria, behavioral disturbances and impaired consciousness are strong arguments to integrate palliative care early, irrespective of all differences concerning the nature or progression of chronic neurological diseases. Patients have to be assisted to cope with the diagnosis ‘incurable and progressive disease’, to reflect
on values, beliefs and (treatment) goals, to consider future medical treatment options and to choose a surrogate.41
second, both disease-specific and non-disease specific non-motor symptoms may affect patients early in the course of various chronic progressive neurological disease. in Ms fatigue, cognitive impairment and incontinence are such disabling symptoms, in PD depression, sleep disturbances, cognitive impairment and incontinence, in hgg headache, nausea, epilepsy and mood disorders, and in stroke depression, anxiety and grief.11,12,50,51 there is increasing evidence that non-motor symptoms have a negative
impact on the quality of life of patients and affect caregiver burden. therefore, they must be timely addressed, assessed and treated.
third, progressive chronic neurological diseases have a huge impact on psychosocial and mental well-being. For example, patients with MnD have been found to experience their care as a burden with adverse effects on their family caregivers.52 the
standard-ized mortality ratio for suicide in Ms appears to be approximately twice that of the general population. Young(er) males in the first years after diagnosis seem to be most at risk.53 Patients with high grade gliomas have more cognitive and communication
deficits than patients living with other types of cancer. increasing loss of function contributes to feelings of being ‘useless’ or ‘a burden’ too.54 Furthermore, cognitive
and behavioral change and functional deterioration of patients have a negative impact on caregivers during the disease process and during bereavement.55-57
3.1 Realizing awareness and knowledge of palliative care
During this research project we observed that much more attention for palliative care is needed, both in research and during medical education, training period and working life of health care professionals, including neurologists, in order to provide ill patients and their caregivers with the care they need.
For adequate management of symptoms like pain and depression early recognition by the neurologist or another health care professional who does the follow-up of patients with a progressive chronic neurological disease is essential.50,58 there are
early and – especially in later stages – complex palliative care needs of patients with chronic progressive neurological diseases. scientific research is needed to further explore disease-specific and non-disease-specific palliative care needs, and how to best assess and manage them. even in als, in which the need of palliative care is well established, there may be new insights concerning burdensome, undertreated symptoms. Moderate or severe pain in the last months of life was found in 52% of patients, although traditionally pain was not believed to affect patients with als. nine-teen percent of these patients received no painkillers at all in the last month of life.59
specialists (in training) and should be integrated into treatment protocols.
there are several fundamental palliative care skills all physicians, including neuro-logists, should be familiar and comfortable with, such as communicating bad news, discussing future care options, preferences and wishes, and caregiver assessment.1
Currently, these skills are not sufficiently trained.60 our national survey showed that
the majority of neurologists (in training) has had no or only basic training in discussing treatment restrictions. (Chapter 3) ‘Delivering bad news’ is currently a standard part of the curriculum of medical students in the netherlands, but this is not the case for ‘advance care planning’ or ‘discussions about end-of-life wishes and options’. Furthermore, ‘delivering bad news’ is not trained systematically during specialist training and as part of continuous medical education. as discussed before, there appears to be a misconception concerning information needs. neurologists and other health care professionals are hesitant to initiate discussions about disease progression and its implications while (a fair proportion of) their patients and caregivers prefer to be informed. 23,61,62 Furthermore, even breaking bad news, which
is part of daily clinical practice for most doctors, is often experienced as being stressful and difficult by all parties involved.63-65 Communication training does improve
discussions about diagnosis, treatment options and preferences including end-of-life care as experienced by both health care professionals, patients and their next of kin.66-71
3.2 Realizing systematic long-term follow-up
in many Western World countries, including the netherlands, the aim is to provide care for patients with MnD by multidisciplinary teams with palliative care expertise. it has been shown that this care approach, involving careful symptom assessment and management, and psychosocial support of both patients and caregivers, improves quality of care and life, survival and eventually quality of dying. 72,73 our study at the
tertiary als Centre amsterdam underlines the positive impact of well-structured follow-up regarding the understanding of medical information, taking well-considered treatment decisions, and patients’ feeling of being supported. For patients with various types of cancer there is also increasing evidence that early palliative care provided in addition to standard oncology care improves quality of life, mood and survival.74-77
in neurology, over the past decade some papers have been published which indicate that involvement of a multidisciplinary care team leads to better symptom control and improvement of quality of life of patients with late-stage Ms and PD.78-80 in line with
this evidence, the authors of the first consensus review on palliative care in chronic progressive neurological diseases, published in 2016, recommend that palliative care should be provided by a multidisciplinary team and considered early in de disease trajectory.31 again, research and education in palliative care are needed for all parties
patients with chronic progressive neurological diseases, as regards their symptom burden, quality of life and possibly survival.81
3.3 Developing the neurologist’s role in palliative care
the role of neurologists should be made more explicit regarding the integration of palliative care in their practices, requiring training in all fundamental palliative care skills. there are several reasons for that. First, the care of patients with chronic progressive neurological diseases is initially within neurological services – when the diagnosis is made and treatment is initiated.31 as discussed above, expertise in (non-)
motor symptom evaluation and management and in communication about prognosis and treatment options, offers a good start for long-term follow-up, even if the latter is provided by other health care professionals and services.10,82 second, in the
netherlands neurologists usually do the – long-term – follow-up of many patients with chronic progressive neurological diseases, in particular for patients with Ms, PD and gliomas. third, patients with chronic progressive diseases are admitted to hospital and thus often to neurological wards in various stages of disease including the end of life.83 Forth, neurological expertise is also needed when patients with chronic
progressive neurological diseases are followed by other health care professionals. in the netherlands, one might think of neurological consultation of patients with als and after stroke, but also of patients in advanced stages of e.g. Ms and PD patients who have become too ill to be seen in the outpatient clinics. it is very likely that expertise in palliative care will increase neurologists’ ‘satisfaction, by enabling deeper, more meaningful relationships with their patients across the continuum of care’.84
equipped with basic palliative care skills neurologists should also take responsibility for the (re)organization of care for their patients with palliative care needs. two simple steps to improve daily practice in this regard could be the following: neurologists could stimulate the education of their nurse practitioners in palliative care. in the netherlands, the latter ones are usually involved in long-term follow-up of patients with Ms and PD. however, their current tasks are mainly the assessment of disease-specific symptoms, treatment outcome and treatment-related complications. interest-ingly, as far as it has been studied the same focus on optimization of life-prolonging treatment regimens seems to apply to the (para)medical neuro-oncology team.12,27
neurologists should also improve the collaboration with gPs which becomes more and more important when patients reach advanced stages of disease with (more) complex care needs. the tertiary als center amsterdam provides short updates of the follow-up appointments in writing including the patients’ status quo, actual agreements on supportive treatment (options) and aDs which serve to keep the gPs informed. to date, there are considerable differences regarding the organization of palliative care in various countries and health care systems.85 in addition, palliative care for patients
with non-cancer conditions is generally relatively underdeveloped. therefore, it has not been settled which care model should be used in the netherlands to provide optimal palliative care both for patients and caregivers and for the neurologists and their (multidisciplinary) team. in Belgium a similar situation seems to arise, i.e., neurolo-gists seem to be increasingly aware of the important role of palliative care for patients with chronic progressive neurological diseases, but suitable models for the provision of this care still have to be developed.86
neurologists need to actively develop and improve strategies to help their patients cope with chronic progressive diseases. recently, a ‘dynamic care model’ has been promoted as a possible succeeding model of early integration of palliative care expertise in chronic progressive neurological diseases.87 according to this care model
basic palliative care is provided by neurologists and their teams, whereas specialist palliative care teams are available for consultation when needed (figure 1). this model is not (yet) applicable in the netherlands – or elsewhere in the world. however, it paves the way for the strategy to obtain palliative care expertise by neurologists as basis of long-term follow-up of patients with chronic progressive neurological diseases.
4. Critical appraisal
We consider the fact that we modified the second research question on the basis of the outcome of our first substudies as a strength of this project. a second strength is the wealth of qualitative data, especially concerning the evaluation of aCP in real-life practice of the tertiary als Centre amsterdam. Furthermore, we used both qualitative and quantitative research methods to evaluate the views and experiences of both neurologists and neurological patients, i.e. patients with als/PMa, concerning pallia-tive care in practice.
there are also weaknesses. a limitation of our project is the focus on one single Dutch practice. hence it may be questioned whether our findings can be extrapolated, also
Figure 1. The dynamic care model for chronic progressive neurological disease
in light of the fact that in the netherlands questions about end-of life wishes and practices are more openly discussed than in most other countries. on the other hand, our findings are largely supported by recent international literature. the focus on the neurologists’ perspective (more than on the perspective of patients’ with als/PMa) might also be seen as a limitation. however, in-depth analysis of the perspectives of caregivers and other involved health care professionals was beyond the scope of this project.
5. Future research and development
First, the actual meaning of palliative care should be communicated. our project and the recent literature show that the majority of physicians still have difficulties to distinguish palliative care from hospice care.88,89 lately, the term ‘supportive care’ is
increasingly used in oncology to define palliative care which is not equal to terminal care. Perhaps, this or another less delicate term than ‘palliative’ could help both physicians and patients to more easily talk about and start treatment for various symptoms, physical, mental and existential, which are not directly associated with the treatment of the disease itself. on the other hand, one may argue that physicians’ change in attitude and knowledge of palliative care would also change patients’ and their caregivers’ acceptance.90 of course, the change of the physicians’ attitude towards
and knowledge of palliative care is the real challenge as it implies a ‘paradigm shift’.
1,42,91 still, in our opinion this shift is mandatory to improve the care of the ever growing
number of patients with (progressive) chronic diseases.
second, non-motor symptoms and their management must be mapped for the most frequent progressive chronic neurological diseases. in line with that, screening tools for both disease-specific and non-disease specific symptoms should be developed and if they already exist they should be evaluated for their usefulness in daily clinical practice. For example, there is no evidence whether symptom- and diseases-specific outcome measures such as the Depression and anxiety stress scale (Dass), Multi-ple sclerosis impact scale (Misi-29), and Parkinson’s Disease non-Motor symptoms Questionnaire (nMsQuest) might be useful for early and ongoing monitoring of patients’ functional status, or whether they could be sufficient for mapping the complexity of care needs in late stages of disease.20,92-94 as another example, the
prevalence of anxiety and depression seems to be significantly higher in patients with PD when screened with the Beck anxiety inventory (Bai) than when evaluated by the neurologist during office hours.95
third, the existing knowledge of palliative care including communication ranging from ‘giving bad news’ to ‘evaluation of wishes for and at the end of life’ for patients with neurological diseases should be integrated in the curriculum of medical students, in the training of residents and in the continuing medical education of neurologists.
Disease-specific guidelines should be established and implemented. a good example is the niCe guideline on als which is developed in the uK.96
Fourth, there are interesting questions about the role of so called ‘trigger points’ which define evident deterioration of patients, such as swallowing problems, loss of weight, (recurrent) infection or delirium, marked decline in functional status, but also cogni-tive decline. We argue that palliacogni-tive care should be an integrated part of care as soon as the diagnosis chronic progressive neurological disease is given. however, trigger points could help intensifying or diminishing life-prolonging treatment and optimizing care plans.11,70 More research is needed to learn about the prognostic value of such
trigger points in various chronic progressive neurological diseases. an equally important question concerns how to deal with patients who possibly or probably have a chronic progressive neurological disease, but do not yet fulfill all diagnostic criteria during the first consultations. Furthermore, neurologists need to be educated how to discuss evaluation and presence of trigger points, including the start of the terminal phase, with patients and their caregivers.
Fifth, and in line with the last point, studies are needed to explore patients’ and caregivers’ ideas and perspectives about (actual) integration of palliative care in short- and long-term follow-up. one research question may be ‘which amount of information about the disease and its prognosis do patients with high grade gliomas, Ms and PD wish to obtain shortly after diagnosis? and how should discussions and agreements be documented?’ other research questions may be the following: ‘(how) do patients with incurable progressive disease want to talk about advance directives?’ and ‘is there a role for caregivers in advance care planning when patients are actively or passively avoiding discussions about future care?’
6. Conclusion
this project describes our own paradigm shift concerning palliative care. We started off with the question whether the discussion about potential treatment restrictions could be the appropriate moment to initiate palliative care for patients with progressive chronic neurological diseases. gradually, we realized that palliative care is needed alongside curative treatments during the whole course of progressive disease, irrespective of the diagnosis or illness trajectory.
Care for patients with progressive chronic neurological diseases is not about restrictions, but about the appropriate use of treatment options for both disease- specific and non-disease-specific symptoms. these symptoms include psychological, social and existential questions of both patients and caregivers. neurologists, like other physicians, need to be aware of the fact that palliative care know-how is essen-tial for adequate care of their patients with chronic progressive neurological diseases.
in addition, it is crucial that neurologists, like other physicians, realize that palliative care communication skills such as giving bad news and discussing end-of-life wishes are important for any patient-doctor relationship, and that these are professional skills which need to be learned and further improved as part of continuing medical education.
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