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Palliative care in chronic progressive neurological disease
Changing perspectives
Seeber, A.A.
Publication date
2019
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Final published version
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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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Changing Perspectives
the studies presented in this thesis were funded by the netherlands organization for health research and Development (ZonMw), grant number sj.152.005.
Printing of this thesis was financially supported by the Department of general Practice of the academic Medical Center (aMC), university of amsterdam, and the st. antonius hospital nieuwegein/utrecht. all rights reserved. no part of this publication may be reproduced, stored in a retrieval system or transmitted, in any form or by any means, electronically, mechanically, photocopying, recording or otherwise, without the prior written permission of the author.
aCaDeMisCh ProeFsChriFt ter verkrijging van de graad van doctor
aan de universiteit van amsterdam op gezag van de rector Magnificus
prof. dr. ir. K.i.J. Maex
ten overstaan van een door het College voor Promoties ingestelde commissie, in het openbaar te verdedigen in de agnietenkapel
op vrijdag 7 juni 2019, te 10.00 uur door antje anna-luise seeber
geboren te göttingen
Promotores Prof. dr. D.l. Willems aMC-uva
Prof. dr. M. de visser aMC-uva
Co-promotor Prof. dr. a.J. Pols aMC-uva
Overige leden Prof. dr. M. vermeulen aMC-uva
Prof. dr. D. oliver university of Kent
Prof. dr. s. van de vathorst aMC-uva
Dr. J.C. reijneveld vrije universiteit amsterdam
Prof. dr. F. nollet aMC-uva
Dr. h.r.W. Pasman vrije universiteit amsterdam
härte schwand. auf einmal legt sich schonung an der Wiesen aufgedecktes grau.
Kleine Wasser ändern die Betonung. Zärtlichkeiten, ungenau,
greifen nach der erde aus dem raum. Wege gehen weit ins land und zeigens. unvermutet siehst du seines steigens ausdruck in dem leeren Baum.
Chapter One 11 general introDuCtion anD
outline oF the thesis
Chapter Two 29
DisCussions aBout treatMent restriCtions in ChroniC neurologiCal Diseases. a struCtureD revieW
Chapter Three 55
the role oF Palliative Care in ChroniC Progressive neurologiCal
Diseases – a surveY aMongst neurologists in the netherlanDs
Chapter Four 77
hoW DutCh neurologists involve FaMilies oF CritiCallY ill Patients in enD-oF-liFe Care anD DeCision MaKing
Chapter Five 93
eXPerienCes anD reFleCtions oF Patients With Motor neuron Disease on BreaKing the neWs in a tWo tiereD aPPointMent. a Qualitative stuDY
Chapter Six 117
aDvanCe Care Planning in ChroniC Progressive neurologiCal Disease. What We Can learn FroM als. a Qualitative stuDY
Chapter Seven 141
general DisCussion anD Future PersPeCtives Appendices 165 suMMarY saMenvatting list oF PuBliCations PhD PortFolio aBout the author DanKWoorD
general introDuCtion anD
outline oF the thesis
WorlDWiDe, ChroniC Progressive neurologiCal Diseases are
leaDing Causes oF Both MortalitY anD DisaBilitY.
1,2Patients
With aMYotroPhiC lateral sClerosis, high graDe glioMas,
MultiPle sClerosis, ParKinson’s Disease anD other MoveMent
DisorDers, Post-stroKe status With DisaBilitY anD DeMentia
are aMongst the Most hanDiCaPPeD Patients in MeDiCal
PraC-tiCe.
3While these Diseases DiFFer in sYMPtoMatologY anD
Disease traJeCtories, theY share a host oF PhYsiCal,
Cogni-tive, eMotional, anD eXistential ProBleMs. to aDDress these
ProBleMs aDeQuatelY is a Challenge For neurologists anD
other healthCare ProFessionals.
4the traDitional Care
aPProaCh oF MeDiCine eMPhasiZes on Cure, Preservation
oF FunCtion anD Prolongation oF liFe anD has the tenDenCY
to leaD to Both over- anD unDertreatMent oF Patients With
ChroniC Progressive Diseases. though there is groWing
eviDenCe that an earlY Palliative Care aPProaCh iMProves
the sYMPtoM ManageMent anD thus QualitY oF liFe oF these
Patients, suCh an aPProaCh is highlY unDerutiliZeD.
5-7in DailY
CliniCal PraCtiCe, QualitY oF liFe Questions usuallY BeCoMe a
MeDiCal PrioritY not earlier than in the last Phase oF liFe.
4,8oBviouslY, there are Certain MoMents anD CirCuMstanCes
During the Course oF a serious illness When the
aPProPri-ateness oF treatMent aiMing at Cure Can Be QuestioneD. in
DailY neurologiCal PraCtiCe, one oF these MoMents is When
treatMent restriCtions are DisCusseD, suCh as stoPPing a
Disease-MoDiFYing MeDiCation, a Do-not-resusCitate orDer
(Dnr) or a no-intensive-Care-treatMent orDer. in this
thesis, We investigate Whether the DisCussion on treatMent
restriCtions CoulD Be the oPPortune MoMent to eXPliCitlY
initiate Palliative Care, With iMProveMent oF the QualitY oF
liFe as the First anD Main oBJeCtive.
Illness trajectories in chronic progressive neurological disease
over the last century tremendous advances in medicine, improved hygiene and demo-graphic changes have contributed to nearly a doubling of the life expectancy in Western World countries and an – ongoing – increase in chronic diseases which interfere with daily activities and have a negative impact on the quality of life.9 Chronic progressive
neurological diseases are amongst the most common of these diseases.2 the vast
majority of them follow one of the three illness trajectories which have been described for chronic diseases in general.10
1. Steady progression with usually a clear terminal phase: e.g. high grade glioma (HGG) and motor neuron disease (MND)
Malignant primary brain tumors (high-grade gliomas, hgg) usually occur after the age of 40 years with a peak incidence between 65 and 75 years of age.11 the majority of
these rapidly growing tumors is associated with a life expectancy of several months to a few years only, despite sophisticated surgical techniques, chemo- and radiother-apy.12,13 Patients with hggs often have a relatively high performance status until the
last phase of life when deterioration may be rather rapid due to symptoms caused by accelerated tumor growth and side effects of anti-tumor treatment (figure 1a).14,15
importantly, the performance status is most often determined by the Karnofsky or Zubrod score which capture the patients’ general well-being and activities of daily life and have not been designed to reveal patients’ cognitive impairments.16 in contrast
to patients with other types of cancer, however, patients with hgg are threatened by significant cognitive deterioration throughout the whole illness trajectory. even up to 79% of patients with hgg have cognitive impairment before treatment, and more than 50% lack full decision-making capacity 4 months after diagnosis.17,18
Motor neuron diseases (MnD) such as amyotrophic lateral sclerosis (als) and progressive muscular atrophy (PMa) manifest at a median age of respectively 61 and 65 years. however, both conditions can already occur in patients in their twenties and as late as in their nineties.19 on average, patients with als die about three years after
symptom onset, due to respiratory failure or aspiration pneumonia as a consequence of dysphagia.20-22 Patients with PMa have a longer median survival of about four years.23
negative prognostic factors are older age, bulbar or respiratory onset, the presence of the behavioral variant of frontotemporal dementia, and recently there is some evidence that the presence of executive cognitive and/or behavioral impairment also has a negative impact on survival.24,25 thirty to fifty percent of patients with als have some
degree of cognitive and/or behavioral impairment which in most cases remains rela-tively mild. in 5-10% there is frank frontotemporal dementia.26,27 the life expectancy
of patients with als can be prolonged by weeks to a few months by administration of the drug riluzole.28 in general, for both patients with als and PMa the steepness of the
2. Gradual decline, punctuated by episodes of acute, life-threatening deterioration and partial recovery: e.g. multiple sclerosis (MS) and Parkinson’s disease (PD) the majority of patients with multiple sclerosis (Ms) develop their first symptoms in early and middle adulthood.29 in general, Ms is less rapidly progressive than hggs
or MnDs.30 a recent population based study showed that on average it takes more
than 29 years before patients need walking aids.31 on the other hand, it appears that
only one third of patients with Ms still works 15 years after symptom onset.32 this can
be explained by various motor and also non-motor symptoms such as fatigue and cognitive impairment which may already interfere with daily life activities in early stages of disease.33,34 For patients with relapsing-remitting Ms disease-modifying
therapy is available which has a beneficial effect on progression when used during the early course of disease.35,36
Parkinson’s disease (PD) is uncommon in people younger than 40 years. the incidence of the disease increases rapidly after the age of 60, with a mean age at diagnosis of 70 Figure 1. Illness trajectories in chronic progressive neurological diseases
years.37 next to the classical motor symptoms of PD, more than two thirds of patients
develop non-motor symptoms such as depression, sleep disturbances, incontinence and cognitive impairment.38 Motor symptoms often show a good to excellent response
to treatment with diseases-specific medication during on average 3 to 7 years, the so-called ‘honeymoon period’. however, non-motor symptoms are associated with a poor outcome at 10 years after diagnosis.39,40 other neurodegenerative movement
disorders such atypical parkinsonism multi-system atrophy (Msa) or progressive nuclear palsy (PsP) also occur after the age of 60. these disorders are less common than PD, show more rapid progression of motor and non-motor symptoms and often have a poor response to anti-Parkinson medication.41,42
in advanced stages, in both Ms and PD progression is increasingly associated with rather unpredictable deteriorations which often necessitate hospital admission and intensive treatment (figure 1C).43 reasons for such deteriorations may e.g. be
intercurrent, ‘trivial’ infections of bladder or respiratory tract or injuries due to falls. Patients may recover from such deteriorations, but do normally not regain their old functional status. as a consequence, the time of terminal phase and death remains somehow unpredictable.
3. Prolonged gradual decline: e.g. post-stroke status with disability and dementia ischemic and hemorrhagic stroke are very common diseases in the elderly, its overall incidence increases dramatically after the age of 55.44 recent estimates rank stroke
as the second most common cause of death and the third most common cause of disability-adjusted life-years worldwide.45 Five-year survival is similar to that of all
cancers combined and heart failure.46 the various types of dementia also occur most
often in the elderly. alzheimer disease (aD) is the most common cause of dementia and one of the leading causes of morbidity and mortality in the aging population.47 globally,
an estimated 47 million people are affected by dementia, and this number is predicted to double every 20 years until 2040.48
the course of decline is well described by the third illness trajectory: the dwindling patient suffering from frailty due to the combination of old age and chronic conditions such as post-stroke status with disability, mild cognitive impairment (MCi) and de-mentia.49,50 over the years, such patients are liable to acquire intercurrent infections
or injuries, due to their overall low level of functioning (figure 1D). in line with that, the individual’s slow deterioration may easily be accelerated by an acute event such as a hip fracture, pneumonia or urinary tract infection.
the outlined illness trajectories do present some challenges. More and more advanced life-supporting and -sustaining treatment options are available, however, such treatments may raise ethical questions, especially toward the end of life. the
unpredictability of the individual patient’s illness trajectory, often combined with impaired communication and cognition, requires timely agreements between physi-cians, i.e. neurologists, and their patients on the extent of curative treatment and the upscaling of symptomatic treatment, to prevent both over- and undertreatment.
A code for treatment restrictions
the idea that doctors and patients should have an agreement on the appropriate level of curative treatment is widely shared in the Western World countries. soon after cardiopulmonary resuscitation (CPr) had been developed in the early 1960s, it became clear that routine application of CPr on a patient with cardiopulmonary insufficiency often prolonged suffering and the process of dying instead of preserving the quality of life.51 in the 1970s the first guidelines about do-not-resuscitate (Dnr) orders were
established in the united states, and during the following two decades most of the other Western World countries followed.52,53 in the netherlands, awareness of the need
for such agreements in frail patients often suffering from more than one disease, led to the development of the so-called ‘treatment restrictions code’ during the 1990s.54
to enhance the general awareness concerning end-of-life treatment decisions it was even recommended to discuss potential treatment restrictions with all adult patients. Currently, in most Dutch hospitals three different codes are used:
Code A: no treatment restrictions.
Code B: treatment restrictions: for specific treatments it is determined whether or not they will be applied if necessary during admission. the most common treatments discussed are resuscitation, ventilatory support, treatment demanding admission to an intensive care unit, surgery, treatment with antibiotics, inotropics to stabilize circulation, medication to prevent thrombosis, and nutritional support including fluid intake. Particular details can be added.
Code C: only treatment that aims at comfort, such as pain control, prevention of thirst, anxiety and shortness of breath.
the term treatment restriction could erroneously give the impression that treatment codes are exclusively about a reduction of treatment options. however, the aim of the code discussions is to identify which treatment options are appropriate and proportion-ate for the specific patient. this may be one reason why the term ‘treatment restriction’ has been replaced by ‘treatment agreement’ in many hospitals at the same time as our research project took place. the overall idea remains the same: one of the three codes, a, B or C, is supposed to be assigned to every patient, and to be prominently placed on the front page of every patient’s file, such as is the case for allergies, etc.
A palliative care approach for chronic progressive neurological disease
after the turn of the century, the growing awareness of unmet care needs of patients with chronic progressive diseases has also led to a redefinition of palliative care. Developed in the 1960s in the context of terminal, i.e. hospice care for patients with cancer, the contemporary World health organization’s (Who) definition of palliative care reads as follows:
Palliative care
- provides relief from pain and other distressing symptoms; - affirms life and regards dying as a normal process; - intends neither to hasten nor postpone death;
- integrates the psychological and spiritual aspects of patient care;
- offers a support system to help patients live as actively as possible until death; - offers a support system to help the family cope during the patient’s illness and in
their own bereavement;
- uses a team approach to address the needs of patients and their families, including bereavement counseling, if indicated;
- will enhance quality of life, and may also positively influence the course of illness; - is applicable early in the course of illness, in conjunction with other therapies
that are intended to prolong life, such as chemotherapy or radiation therapy, and includes those investigations needed to better understand and manage distressing clinical complications.55
the goals of this care approach fit well with the complex needs of patients with chronic progressive neurological diseases following any of the outlined illness trajectories, and with the needs of their caregivers. this becomes even more compelling with the increasing awareness of the considerable impact of non-motor and non-disease specific symptoms on the quality of life of patients with chronic progressive neuro-logical diseases. not seldom these symptoms occur relatively early in the course of disease.40,56-58
the broadening of the palliative care approach is in keeping with growing evidence that timely input of palliative care improves symptom management and quality of life of patients with various chronic progressive diseases, and of their caregivers, and may even extend survival. 7,59-64 in line with that, the first consensus review on palliative care
in chronic progressive neurological diseases, published in 2016, recommends that the palliative care approach (1) should be integrated early in the disease trajectory; (2) should include communication with patients and families on advance care planning and; (3) should be multidisciplinary to comply with the complex care needs of patients and their families.6 a care model fitting such an approach could look like figure 2.
in this care model, palliative care is offered increasingly alongside treatment to modify disease symptoms and cure complications. the focus lies, from the beginning or at least from an early stage of chronic progressive neurological disease, on enhancement of quality of life and, later on, on quality of dying. this approach requires professional, open communication, including the setting of goals and therapy options throughout the disease trajectory.
The problem: when and how to start palliative care in chronic
progres-sive neurological disease?
Despite the above-described developments, most doctors, including neurologists, still associate ‘palliative care’ with ‘terminal or hospice care’. Medical school, specialist training and daily practice mainly focus on cure and subsequently curative, i.e. dis-ease-directed treatment, even in chronic progressive disease.5,59,65 Consequently,
there is an ongoing unawareness of physicians, including neurologists, of the current palliative care approach. there is widespread unfamiliarity with the need for developing professional skills to communicate bad news and possible treatment restrictions, to timely assess and manage non-motor and non-disease-specific symptoms and to timely refer to specialist palliative care services including hospice admissions.
in-depth interviews in the exploring phase of this research project showed that both medical specialists and patients have difficulties with considering palliative care as a treatment option during the illness trajectory. there are many misconceptions, such as ‘i do not want to diminish hope and harm the relationship with my patient’ to ‘i won’t be in charge of the patient at the time that end-of-life decisions have to be discussed’ and ‘My patient does not want to consider a Dnr order’ or, from the patient perspective, ‘i do not want to disappoint my doctor’.66-69 By now those misconceptions can be refuted
by quantitative and qualitative research data which shows that patients with chronic Figure 2. The pro-active care model for chronic disease
progressive neurological diseases – and their caregivers – suffer from complex problems and unmet care needs, and want to be involved in communication on medical treatment considerations throughout the course of disease.39,56,70-75
looking at the three illness trajectories outlined above, the precise course of chronic progressive disease in the individual patient remains hard to predict. even for doctors with knowledge of the palliative care approach, it is hard to estimate the right moment to discuss withholding a certain medical treatment or shifting the focus of care towards primarily the enhancement of quality of life.7,76 in expert tertiary
cen-tres there is increasing recognition of the palliative care needs of patients with rapidly progressive MnD, from the moment that the diagnosis is given, including timely discussions on treatment restrictions.77 however, palliative care in other progressive
chronic neurological diseases is lagging behind despite the earlier mentioned imminent cognitive impairment of many patients with these diseases, behavioral issues, communications problems and other unmet non-motor and non-disease specific problems.5,78
Research questions and outline of this thesis
our project aimed to investigate whether the discussion on treatment restrictions could be an appropriate and thus helpful tool for neurologists to timely start palliative care for their patients with chronic progressive neurological diseases. after all, in daily clinical practice the discussion on treatment restrictions appears to be the first moment that the neurologist or his patient considers a treatment not opportune anymore. initially, we focused our research on the actual timing and content of discussions on treatment restrictions between neurologists and their patients, and about the consequences of these discussions for the management of the individual patient’s further care.
our first main research questions was:
1. Does the discussion on treatment restrictions with patients suffering from chronic progressive neurological disease mark the start of palliative care?
We started off with a review on existing knowledge about the timing and content of discussions on treatment restrictions in patients with one of the following six chronic progressive neurological conditions: ischemic or hemorrhagic stroke, amyotrophic lateral sclerosis, primary brain tumors, Parkinson’s disease, multiple sclerosis and dementia syndromes. (Chapter 2)
subsequently, we investigated when and how Dutch neurologists discuss treatment restrictions with their patients in daily clinical practice by performing in-depth inter-views and subsequently by obtaining data on the topic via an online survey. (Chapter 3)
one important finding of both the review and the interviews was that many neurological patients are incompetent at the moment that treatment restrictions and codes are dis-cussed. therefore, we also looked at the way in which caregivers, i.e. family members, of incompetent patients were said to be involved in discussions on treatment restric-tions by the neurologists which we had interviewed in depth. (Chapter 4)
We concluded that discussions on treatment restrictions are not appropriate for marking a timely start of palliative care, and therefore we changed the second research question ‘Which recommendations can be given for appropriate timing of discussions on treatment restrictions 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 searched for a care approach which indeed would enable timely integration of palliative care and found such an approach in practice at the tertiary als outpatient clinic where, in general, palliative care starts as soon as the diagnosis is given. By means of non-participating observations of the office hours of the clinic and subsequent in-depth interviews with the patients, we tried to investigate how palliative care is integrated in the medical care of patients with als, how patients experience this policy, and what we can learn from it in order to integrate palliative care on time in the follow-up of patients with other chronic progressive neurological diseases. (Chapters 5 and 6)
a general discussion, including general recommendations for improvement of clinical practice concerning early integration of palliative care by neurologists for their patients with chronic progressive neurological diseases, and suggestions for further research are provided in Chapter 7.
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DisCussions aBout treatMent
restriCtions in ChroniC
neurologiCal Diseases.
a struCtureD revieW
antje a. seeber, albert hijdra, Marinus vermeulen, Dick l. Willems neurology 2012;78(8):590-7
aBstraCt
Objective
Many incurable neurologic diseases have predictable complications during their course or at their end stage. timely discussions of potential treatment restrictions may improve the quality of treatment decisions toward the end of life. What is known about the actual practice of these discussions?
Methods
We performed a literature search in MeDline, eMBase and Cinahl for empirical studies about discussions and decisions to restrict treatment in the course of 6 conditions: motor neuron disease (amyotrophic lateral sclerosis [als]), primary malignant brain tumors, multiple sclerosis, stroke, Parkinson disease’s, and dementia (alzheimer’s disease).
Results
in 10 of 43 studies, the actual practice of decision-making was studied; in the remaining 33, caregivers were interviewed about this practice. three scenarios were described: 1) acute devastating disease (severe stroke); 2) stable severe neurologic deficit with complications (post-stroke brain damage); and 3) chronic progressive disease with complications (dementia and als). We found no studies concerning the other condi-tions. in all 3 scenarios, discussions and decisions seemed to be mostly triggered by the occurrence of life-threatening situations, either caused by the disease itself (1), or complications (2 and 3, including many patients with als). some als studies showed that timely discussion of treatment options improved end-of-life decisions-making.
Conclusion
the actual practice of discussions about treatment restrictions in chronic neurologic disease has hardly been studied. the currently available empirical data suggest that discussions are mainly triggered by life-threatening situations, whereas anticipation of such situations may be beneficial for patients and their families.
Introduction
the prevalence of chronic neurologic diseases has increased considerably throughout the last century. this can be attributed to the rise in life expectancy and the develop-ment of new diagnostic tools as well as sophisticated treatdevelop-ments. Consequently, the time course of many neurologic conditions has been prolonged significantly and many acute life-threatening conditions have been transformed to chronic ones.
the focus on cure in the initial stages of chronic neurologic diseases may gradually shift to the emphasis on symptomatic and palliative care in more advanced stages. as patients become older and more impaired, neurologists are confronted with the question of whether the benefits of medical treatment still outweigh the burdens. Patients and their families will have to be informed of shifts in medical treatment goals in order to participate in treatment and non-treatment decisions. in this respect, an important question for both neurologists and patients is at which moment the advantages and disadvantages of conceivable treatment options should be considered. there is a wealth of literature in which these problems are discussed, but most papers focus either on ethical issues regarding end-of-life decision-making or analyze procedures in specific cases (see references 1-10 for an overview). some empirical
data can be found in studies in patients with als, with its relatively short time course and impending respiratory failure.11-13 however, it is unclear when and to what extent
the restriction of medical treatment is discussed with patients with more common diseases, such as stroke, Parkinson’s disease (PD), alzheimer’s disease (aD), multiple sclerosis (Ms) and primary malignant brain tumors.
as part of a larger project to study the cure-care shift in patients with chronic neuro-logic diseases, we performed a literature search to find studies that provide empirical data concerning the restriction of medical treatment in the course of these diseases. our research questions were at what moment in the course of incurable neurologic disease are treatment restrictions discussed; which factors determine the timing of these discussions; and who initiates and participates in the discussions?
Methods
We performed a systematic search of the literature using MeDline ovid (1950 – november 2010), eMBase (1980 – november 2010) and Cinahl (1981 – november 2010). all terms were searched in MeDline as words in text, title and abstract and as Mesh terms; in eMBase and Cinahl as subject headings, terms and key words. We used the following search terms: palliative care, incurable disease, decision making, treatment restriction, motor neuron disease (als), stroke (cerebral infarction, intracerebral hemorrhage), parkinsonism (PD), Ms, primary malignant brain tumor, dementia syndrome (aD). the complete strategy, derived from MeDline ovid, can be found in the supplementary area (table s-1).
We performed no hand search of rele-vant journals and did not pursue un-published grey literature. however, we reviewed reference lists from retrieved articles and tracked down and included potentially relevant articles throughout the review.
We included articles in the review if they dealt with 1) adults with the above-mentioned neurologic conditions; 2) studies about the discussion of treat-ment restrictions within the doctor-patient relationship; and 3) if they were published in a peer-reviewed journal, with available full text in english, Dutch, german or French. We excluded case reports, reviews, and studies primarily focusing on advance directives, eutha-nasia, physician-assisted suicide, or terminal sedation.
given the paucity of articles presenting empirical data regarding treatment restrictions, all the various types of studies which remained were used. however, as most of these publications would not meet the stringent criteria necessary for a systematic review, our research aim shifted towards provision of a general overview.14,15
We classified studies according to the type of empirical data presented: 1) studies of actual practice, providing data regarding the actual process of decision-making (chart reviews and prospective cohort-studies), and 2) interview studies, providing data regarding practice and opinions of care-givers (including in-depth interviews and structured interviews, and vignette studies). From all studies, we extracted the following data: country of study population; factors studied (e.g. characteristics of patients, attitudes of caregivers); type of treatment restrictions considered; initiation of discussion of treatment options; final decision makers.
Figure 1. Flowchart of search strategy
* the complete strategy, derived from MeDline, can be found in table s-1.
Results
a total of 43 articles met our selection criteria (Figure 1). all of them were published within the past 21 years, 13 before and 30 after 2000. they described studies conducted in north america (20), the netherlands (10) and other european countries (18), Japan (5), Brazil (1) and australia (1). in 10 studies the actual practice of decision-making was investigated; the remaining 33 articles discussed interview-studies. though we included six neurologic conditions in the search strategy, only three of them were represented in the 43 articles: stroke, dementia and als. For the extracted data and conclusions of individual studies, see supplementary tables s-2 to s-7. the main characteristics of all studies are summarized in table 1. the nature of treatment restrictions and the context in which these were discussed are presented in table 2. all findings are described according to their disease category.
Stroke – acute phase
in patients with acute stroke, severity of function loss, presence of significant co-morbidity and advanced age are considered to be the most important factors to motivate the treatment restriction order ‘do-not-resuscitate’ (Dnr).16 impaired mental
capacity may be another factor, since until the 1990s mentally impaired patients were 9 times more likely to receive Dnr orders than patients with intact cognition.17 (Data
on this issue are lacking for the contemporary situation in stroke units.) Dnr orders are most often implemented in patients with devastating stroke, such as intracerebral hemorrhage unsuitable for surgery, directly on admission, or when further clinical deterioration occurs later in the course of the disease.16 Dnr-decisions are solely
based on the actual medical condition of the patients, and are made by the treating neurologist. Patients are not involved due to mental impairment shortly after stroke.16,17
evidence from interview-studies regarding the influence of families on treatment decisions reveals conflicting results, but the patients’ formerly expressed preferences often seem to be ignored.18-20
Stroke – chronic phase
For patients who fail to recover from severe neurologic impairment, decisions have to be made whether to withhold or withdraw life-sustaining measures such as artificial nutrition and hydration (anh) and antibiotic treatment. specifically, at the very moment patients are threatened by malnourishment and aspiration pneumonia, most are unable to express their treatment preferences.18,20-25 studies of actual practice concerning
discussions about treatment restrictions for such patients are not available. evidence from interview-studies suggests that physicians generally feel they should comply with the earlier expressed wishes of patients and the preferences of patients’ families. one study indicated that the decision to start or withdraw artificial nutrition and hydration
abbreviations: aB = antibiotic treatment; anh = artificial nutrition and hydration; Dnh = do-not- hospitalize order; Dni = do-not-intubate order; Dnr = do-not-resuscitate order; n/iMv = non/invasive mechanical ventilation; PC = palliative care as symptomatic care in terminal phase of life; PCh = palliative care as symptomatic care in terminal phase of life, if necessary in hospice; sPt = spectrum of treatment options (from maximal curative efforts to symptomatic terminal care)
was more often related to the wishes of families than to wishes formerly expressed by patients.23 another study showed that most physicians endorsed family preferences for
tube feeding even when patients had specifically opposed to this intervention in their living will.18
in three Japanese studies, a reserved attitude towards limitations of medical treatment is described. With life preservation as prevailing value, treatment such as anh is considered part of natural care to be provided until death. once started anh is rarely withdrawn,22,24 and if so, only ‘in a secret manner’.21 (supplementary tables s-2 and s-3)
Dementia
treatment restrictions are generally discussed and applied during the very last period of life in nursing homes.26 the older and more severely demented patients are, the
more likely medical treatment will be restricted.27,28 however, postponement of
deci-sions about treatment restrictions until life-threatening complications occur is seen in this patient group as well.27 a recent Canadian study showed that more than 40% of all do-not-hospitalize (Dnh) orders for seriously demented nursing home patients were written during the last 30 days of life, ‘when death [was] imminent’.29
these findings are corroborated by data from interview-studies.29-34 advance directives
may reduce the difficulties which physicians experience when they have to make treatment decisions.35 the more physicians know about the patients’ treatment
preferences and regard them as helpful, the less aggressive treatment they will choose, in line with these preferences, when further deterioration occurs.36,37 however,
end-of-life decisions may be more influenced by the severity of dementia, presumed quality of life and family preferences than by advance directives.38-40 in order to
create the broadest possible basis for medical decision making, physicians involve the patients’ families more actively.33,41,42 Frank communication about end-of-life issues
not only seems to support patients’ families acceptance of the dying process,38,43 but
also to help physicians to formulate treatment goals more explicitly.44-46
according to one cross-cultural study, Dutch physicians assume active and primary responsibility for treatment restrictions, whereas in the usa physicians seem to be more passive and deferential to family preferences even if they consider these to be inappropriate.47,48 in Japan physicians are compelled to provide anh due to legal and
ethical aspects of the medical system, with the preservation of life as prevailing value.49
(supplementary tables s-4 and s-5) Amyotrophic lateral sclerosis
als-patients are encouraged to decide for themselves about noninvasive and invasive mechanical ventilation, anh and other palliative care measures, and to be able to
make well-considered decisions they have to be informed about medical treatment options in time.50 Both the relatively rapid and predictable disease progression and
the limited treatment options at the end-of-life may force als-specialists to discuss supportive care options in a more timely and frank manner than oncologists. as a recent study showed, cancer patients are less adequately prepared for end-of-life decision-making.51 advance care planning seems to improve communication of patients’
prefer-ences with both professionals and families.52 in addition, well-considered preferences
for life-sustaining treatment such as tracheostomy and percutaneous endoscopic gastrostomy are apparently consistent with patients’ eventual treatment choices.53 on
the other hand, a recent study from a specialized multidisciplinary als-team showed that, although the great majority of patients wanted to have detailed knowledge about their present state of health, 48% refused to consider end-of-life treatment options and only 20% explicitly expressed advance directives.54
Most of these aspects are stressed in interview-studies too.11-13,52 the results of
two of them suggest that als-patients who receive sufficient information from the medical team about invasive mechanical ventilation before treatment is started and have sufficient time for reflection and family consultation, may not regret their choice afterwards whereas patients who cannot make well-considered choices may feel remorse.12,52 Furthermore, fear of the process of dying and respiratory distress seems
to influence decisions about mechanical ventilation at the end-of-life more than fear of death itself.13,55
some patients may not be able to make decisions about life-extending technologies until they have experienced the consequences of their disease as for example shown in 1996 when 42% of a studied als-group decided that they wanted to undergo long-term Mv at the very moment of respiratory failure.11,52 however, in daily life, many physicians
seem to follow a wait-and-see-policy concerning discussions about end-of-life treatment options too.12,50 (supplementary tables s-6 and s-7)
Discussion
the studies we found describe 3 scenarios: 1) acute devastating disease (severe stroke); 2) stable severe neurologic deficit with complications (poststroke brain damage); and 3) chronic progressive disease with complications (dementia and als). in all 3 scenarios, discussions about treatment restrictions are often triggered by the occurrence of life-threatening situations, either the disease itself (acute stroke, in which this is inevitable), or complications (particularly in patients with dementia). even in patients with als, which is generally considered a model disease as far as advance care planning is concerned, end-of-life decisions quite often seem to be forced by sudden deteriorations caused by predictable complications.
our conclusions are tentative, since they are based on only a small number of empirical studies of the actual practice of decision-making. Most data we analyzed were retrieved from interview-studies, using interviews or vignettes to investigate opinions and attitudes of decision-makers, and responses to hypothetical scenarios do not necessarily reflect choices made in real life situations. it should also be noted that 10 of the 24 studies about end-stage dementia patients were generated by one Dutch research center and that 5 studies about treatment restrictions for a demented patient presenting with an acute life-threatening event were based on the same vignette. additionally, according to the criteria of the graDe Working group, the validity of the results of many of the included studies and the overall quality of evidence are poor.14,15 * Conditions not discussed in article are between parentheses; abbreviations: aB = antibiotic treat-ment; anh = artificial nutrition and hydration; Dnh = do-not-hospitalize order; Dni = do-not-intubate order; Dnr = do-not-resuscitate order; n/iMv = non/invasive mechanical ventilation; PC = palliative care as symptomatic care in terminal phase of life; PCh = palliative care as symptomatic care in terminal phase of life, if necessary in hospice; sPt = spectrum of treatment options (from maximal curative efforts to symptomatic terminal care)
Most studies were performed in north america, several Western european countries and australia, countries with reasonable consensus about the ethical acceptability of withholding and withdrawing life-prolonging treatment. as indicated by 4 of the 5 articles from Japan, discussions about treatment restrictions at the end-of-life take place there as well.12,22,24,49 naturally, our conclusion can only apply to these countries.
an important negative finding of our analysis is the absence of studies concerning dis-cussions about treatment restrictions in the highly prevalent conditions Parkinson’s disease, multiple sclerosis, and primary malignant brain tumors. these conditions would probably follow scenario 3, with the opportunity for the neurologist to initiate discussions about potential treatment restrictions in the course of these diseases and thereby to promote advance care planning. an important consideration is that, in contrast to als, all the above-mentioned conditions will – to a variable extent – lead to cognitive decline.
Despite the restrictions of our study, our analysis uncovers at least some common difficulties with discussions about treatment restrictions. When decisions have to be taken in life-threatening situations, inclusion of patients’ views is likely to be limited. this may be unavoidable for patients in scenario 1, but applies to the other scenarios as well if, as suggested by the existing empirical data, advance care planning rarely takes place. Considering the threat of cognitive decline in many neurologic conditions, assessment of medical treatment options, patients’ expectations and treatment preferences should not be postponed. as recently discussed in this journal, more than 50% of patients suffering from malignant brain tumors have an impaired capacity to make treatment decisions already shortly after diagnosis is made.56 therefore,
anticipation of physicians is needed to allow for patients’ well-considered treatment decisions and advance treatment wishes.
engaging patients in treatment considerations from an early stage of chronic disease onwards should not only be regarded as a valuable goal in itself. it may also improve the quality of the end of life for both patients and their families, as shown by several als studies.12,13,52,57 the sooner discussions are initiated, the stronger the patients’
acceptance of the medical prospects and the more stable their eventual treatment choices seem to be.12,53 involving the patients’ families in the discussions appears to
improve acceptance of decisions to withhold or withdraw medical treatment at the end of life for both the patients and their caregivers as well.7,58,59
in our view, neurologists, with their detailed knowledge of neurologic diseases and possible complications, are primarily responsible for the timely initiation of discussions about potential treatment restrictions, not only to anticipate difficult decisions during serious complications and at the end of life, but also to enable adequate
symptomatic treatment throughout the course of chronic neurologic diseases.
the practice and outcomes of discussions about potential treatment restrictions should be prospectively evaluated in clinical studies. there is no lack of opinion about how these discussions should be done properly; there is a serious lack of empirical data.
\\
Acknowledgment
the authors thank rené spijker, Clinical librarian of the Medical library of the university of amsterdam, for the assistance in performing an elaborative search of the literature.
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