2End-of-life decisions and Advance Care Directives in ALS/MND:
3BackgroundALS/MND is a relentlessly progressive neurological disorder culminating in complete dependence on others for all activities of daily living. In Ireland one person dies every five days.ALS/MND has been identified as an illness that is particularly prone to engendering a sense of helplessness and failure which leads to feelings of frustration and de-skilling that can result in health-careprofessionals avoiding patients. (Skyes 2006 )The clinical management of ALS/MND is palliative from the time of diagnosis and is focused on symptom control and adjustment to the progressive loss of neurological function with the certainty of early death.As treatments are limited, inevitable decisions regarding accepting or forgoing life-sustaining therapies should be made.The failure to address advance care planning leads to unplanned interventions, particularly mechanical ventilation. Decisions to withhold or withdraw life-prolonging treatments are among the most difficult for patients, carers and health-care professionals.
4Aims of the StudyTo identify the attitudes, understandings and experiences of patients withALS/MND, their carers and their health-care professionals to end-of-lifedecisions and advance care directivesTo denote the differences and/or similarities between the experiences ofthe patients, their carers and their health-care professionals.This study was funded by the Health Research Board.
5MethodologyA hermeneutic approach, guided by the philosophy of Hans Georg Gadamer, was the chosen methodology for this study as it illuminates the meaning and understanding of the lived experience of contemplating end-of-life decisions and advance care directives for patients with ALS/MND, their carers and their health-care professionalsPurposive sampling was used. The participants selected had experience pertaining to the phenomena under investigation not just an opinionParticipants included patients with ALS/MND their carers and their health-care professionals, including nurses, Palliative Care Consultants and Consultant Neurologistskkj
6Inclusion and Exclusion Criteria Inclusion Criteria for patients:Possible, Probable or Definite ALS/MND as defined by the El Escorial CriteriaPatients >18 years of agePatients diagnosed > twelve monthsPatients had a nominated carerPatients without cognitive impairmentExclusion Criteria for patients:Patients < 18 yearsPatients without a nominated carerPatients diagnosed < 12 monthsPatients with cognitive impairmentInclusion/Exclusion Criteria for carers:Carers were identified as the person providing care for the patientkkj
7Inclusion and Exclusion Criteria Inclusion criteria for health-care professionals:All the health-care professionals had experience of the phenomena underinvestigation. In Hermeneutics it is the lived experience that is requiredand not just an opinion.Exclusion criteria for health-care professionals:Health-care professionals who had no experience of discussing end-of-life issues and advance care directives with ALS/MND patients.kkj
8Advance Care Directive An Advance Care Directive is a statement made by acompetent adult relating to the type and extent ofmedical treatment he or she would or would not want toundergo in the future should he/she be unable toconsent or dissent at that time.(The Irish Council for Bioethics 2007)
9Sample Advance Care Directive To my Family, Doctors, Health Care Team and other persons concerned!Name:Address:Date of Birth:I received a diagnosis of MND/ALS onThe objective of this Advance Directive is to spare my family, carers and medical advisers the burden of making difficult decisions on my behalf. I am fully informed regarding diagnosis and prognosis on my MND/ALS. I am also aware of the treatment options available to me.Medical Therapy Decisions:Non-invasive ventilationGastrostomy insertion Hydration - NutritionAntibiotic treatmentPalliative CareCardio-pulmonary- resuscitationInvasive ventilationLocus of CareAfter careful consideration and discussion with my medical advisors and my family I have freely and in sound mind decided with the support of my family that:As my illness progresses I wish my future nursing care and symptom management to be Palliative.I do not wish to have the following treatment interventions:I wish to have to have the following treatment interventions:I am aware that I cannot insist on receiving any particular treatment:I have discussed this document with myGeneral Practitioner: G.P Signature:Address: Date:Phone number:I reserve the right to revoke this directive at any time.Signature: _________ Witness: _________Date: _________ Date: _________Copies of this document are with my GP, solicitor, next of kin and hospital records.
10Advance Care Directives For the patients and carers that participated in this study, contemplatingend-of-life decisions and having advance care directives were profoundundertakings which were approached in a unique and individualized way.For some patients there are worse things than death: for others long terminvasive ventilation may be their preferred choice.The realistic medical options and interventions available will determineand influence these decisions. Johnston 2006 “Advance care planningshould be firmly grounded in the values of the individual who, in turn,should understand the consequences of the decisions, both forthemselves and their family”.Advance care directives are increasingly recognised as important toolsfor safeguarding autonomy. However, not all patients wish to makeadvance care directives and some health-care professionals havedifficulties implementing advance care directives.
11Autonomy“Autonomy is not just a status, but a skill, one that must be developed.Health-care interactions rely upon assent, rather than upon genuinely autonomous consent. Throughout most of their medical lives, patients are socialised to be heteronomous, rather than autonomous.At the worst possible time – when life and death consequences are attached to the choices, the paradigm shifts and real consent is sought, evendemanded, making an often traumatic situation even harder.” ( C. Myers 2004).“While an individual might want to express the right to self-determination, any decision they make regarding medical treatment will be influenced by the views of third parties, the individual’s doctor, family or friends”(Irish Council for Bioethics 2007).
12Key findingsAn interpretative methodology was used and significant themes central to theparticipants emerged to illuminate the research.Patients/Carers: Health-Care professionals:Individualised Impact of Illness Difficulties Discussing E-of-L IssuesLiving for today TimingLoss Who Should Discuss E-of-L IssuesReligiosity Family InclusionBurden of Being a CarerDifficulties Discussing E-o-L issues Palliative CareTimingConsensus within families EducationLegal Issues Legal IssuesSample Advance Care Directive Sample Advance Care DirectiveAdvance Care Directive Advance Care DirectiveBurden of Responsibility for Burden of Responsibility forImplementing ACD Implementing ACDParticipation in Research
13Individualised impact of the illness ALS/MND is a relentlessly progressive fatal illness resulting in a series oflosses. It is characterized by progressive decline in motor functionculminating in complete dependence on others for all activities of daily living.The impact of ALS/MND is a unique and individualised experience influencedby how the patient and their carer perceive the changes caused by the illness,their previous coping mechanisms, the presence of support structures and thestrength of their relationship prior to the diagnosis.For many patients with ALS/MND the shock of the diagnosis is compounded bythe relentless progression of the illness. They continued to maintain hopeand a positive outlook, considering themselves lucky and blessed andcontinued to enjoy and reported having a good quality of life, living for today.“As one finds meaning in the present, it is possible for life to be experienced asdeeper, richer and more rewarding even while living with physical decline,realizing one’s finitude, the present time becomes more precious”(Lambert 2006)13
14Difficulties discussing end-of-life issues for patients and their carers There needs to be an acknowledgement that death, while not imminent, will be the likely outcome of the illness.One of the major problems is trying to plan for somethingthat is abstract: “I live for now... I don’t dwell on it ...I think it’s very goodto plan your dying...and I’m saying you’re dying as from the time you start on,are on the last hill...I’m on one now but it’s only a slope, but on that last hill,yes it is useful then”Patients are requesting that they remain in control andmake decisions about their death, just as they didthroughout their lives.For some their end-of-life wish is not to die!
15Burden of being a carerFamily caregivers are key figures in ALS/MND care. They usually providethe bulk of support to patients and play a significant and crucial role inclinical decision making process.“The patients are so dependent on their primary caregivers that the couplerepresents a unique entity, a psychological dyad whose components cannot beconsidered separately” (Chio et al 2004)There were concerns and anxieties expressed as advance care directives are notlegal, would there be difficulties implementing them?Would there be a battle with some health-care professional at the end of a difficultand painful disease process to have the patient’s wishes respected? “I hope theend of his life, will not result in an argument with an official”
16Burden of responsibility for Carers Many of the participants identified the importance of family support throughoutthe disease trajectory but highlighted the importance of family consensusregarding end-of-life decisions and advance care directives. The carersreported that they had great difficulty discussing end-of-life issues andfeared family recriminations if they supported or participated in thepatients’ decision to request or refuse treatment.The carers reported their reluctance to revisit E-o-L discussions, once a decisionwas made, fearing that revisiting these issues would cause upset or that thedecisions made would be revoked.Not all patient’s may wish to make ACD and this adds to the burden ofresponsibility for their carers.
17Difficulties for H-C-P discussing End-of-Life issues The difficulty faced by most health-care professionals in initiating end-of-lifediscussions with patients with ALS/MND is that there needs to be anacknowledgement by the patient at some level that death, while not imminent, willbe the likely outcome of the disease progression.One of the difficulties for H-C-P is to identify the most appropriate time toinitiate end-of-life discussions and the importance of having these discussionsbefore the patient loses the ability to communicate verbally.The H-C-P highlighted the importance of knowing the patient, of having a goodrapport, of having a trusting relationship and also the importance of having theskills to discuss theses issues sensitively and competently .The H-C-P who participated believed that initiating the discussion, while difficult,was welcomed by most patients and their families.
18Burden of Responsibility for Health-Care Professionals The responsibility of ensuring that their patients are fully informedabout the likely disease outcome.That the patients understand the implications of their decisions. Thatthey understand that their decisions may influence the treatments theyreceive, the services that are available to them and may determine theirplace of death.Many reported the difficulties they encountered in dealing with differentmultidisciplinary teams and how the lack of communication and personalbeliefs resulted in patients’ wishes not being respected.All the health-care professionals agreed that attendance at the ALS/MND clinicensured that E-o-L issues and advance care planning would be discussed in atimely and appropriate manner. However, their concerns emanated from theirexperience that although patients had ACD these were not respected in localhospitals.18
19DilemmasMany health-care professionals believe it appropriate to respect and implementACD. However, there are some who feel that life should be preserved at all cost.An anaesthetist refused to implement the ACD of a patient with ALS/MND statingthat “we don’t polish off patients in the I.T.U. setting”The I.T.U. nurses who said that “if having a baby you would have an expecteddate of delivery, are we now being asked to have an expected date of death”“When working in an acute setting the philosophy is often guided by technical,scientific and curative treatments, resulting in difficulties for health-careprofessionals switching to the holistic philosophy of palliative care”Roche-Fahey and Dowling (2008)Health-care professionals have a right to their autonomy and the right to care forindividuals without abandoning their own integrity. Many may not know thepatient and they may have concerns that the directives may not actually reflectthe current situation.
20Palliative CareAll the participants identified the importance of including Palliative Careservices and how vital their skills are in dealing with the complexities ofend-of-life issues.There continues to be a stigma attached to including Palliative Care.Patients believe that their “days are numbered” if referred. Health-careprofessionals tend to back off, withdrawing their service, believing that ifPalliative Care services are involved, there is nothing left to be done.Many believed that the palliative care team should be introduced earlierin the disease trajectory and that their services should be available forsymptom management throughout the illness, not just in theterminal phase.
21Legal IssuesSome of the participants expressed concern that ACD are not legal: theyhoped that legislation would provide clear guidelines and thereforeensure that they would be respected.Others believed that even if they are not legal, by presenting an ACD youare alerting a health-care professional to the expressed wishes of thepatient.Amongst the health-care professionals that participated, there wasambivalence regarding the legislation of ACD and a view that acombination of having a verbal directive and having a nominated proxywould work very well.All the participants agreed that their preference was for a disease specificadvance care directive.
22Findings of the studyThere needs to be improved clinical supervision and ongoing educationalprogrammes for health-care professionals who are dealing with the verychallenging, emotional and ethical difficulties identified in caring for thesevulnerable patients.There needs to be improved communication between the tertiary hospitalmultidisciplinary teams and the community teams.An individualised approach is required and end-of-life issues should be discussedearlier in the disease process and these issues should be initiated by the health-care professionals.Referral to the ALS/MND centre of excellence may result in timely and appropriateend-of-life and advance care discussions taking place.A sample advance care directive document or a booklet may facilitate discussingend-of-life issues.
23ConclusionsA disease specific advance care directive was deemed more appropriate than thegeneric advance care directive proposed by the Law Reform Commission.There is a need for increased public information and awareness regarding end-oflife decisions and advance care directives whereby it does not have to be asentinel event before these issues are discussed.End-of-life decisions and advance care directives are an extremely importantprocess that emerges in the context of the patient-healthcare professionalrelationship. It involves much more than completion of a formal advance caredirective form or “ticking the box”The discussion of end-of-life may appear to be fraught with difficult decisions andethical challenges. The potential for conflict may lead health-care professionalsto avoid open discussion and advance care planning.Johnston (2006) “believes that informing and guiding patients and their familiesthrough the decision making process to a peaceful death should be integral tomedical practice”.
24ConclusionsThis study has illuminated the difficulties and the burden of responsibility facedby health-care professionals and the carers providing end-of-life care forpatients with ALS/MND.Patients need to be aware that their autonomy is not absolute, that they cannotinsist on receiving specific, unrealistic or illegal treatment nor can they compeltheir attending health-care professionals to act against their conscience.The difficulty for patients who wish to invoke an advance care directive and havetheir end-of-life wishes respected is that they are completely dependent on theattending Physician or health-care professional to respect and/or implement theirwishes.Despite a hope that the use of ACD’s would ensure that patients’ preferences fortheir end-of-life wishes would be respected this study has illuminated thatthe use of ACD’s cannot promise or guarantee the patients a say in their futurecare.24
25Thank You Many thanks to the patients, their carers and the health-care professionals who kindly participated inthis research study.