Bernie Corr ALS/MND Clinical Nurse Specialist Beaumont Hospital Dublin Ireland

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Bernie CorrALS/MNDClinical Nurse
SpecialistBeaumont HospitalDublin Ireland
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End-of-life decisions and Advance Care
Directives in ALS/MND
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Background
ALS/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-care professionals 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.
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Aims of the Study

• To identify the attitudes, understandings and
  experiences of patients with
• ALS/MND, their carers and their health-care
  professionals to end-of-life
• decisions and advance care directives
• To denote the differences and/or similarities
  between the experiences of
• the patients, their carers and their health-care
  professionals.
• This study was funded by the Health Research
  Board.

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Methodology

• A 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 professionals
• Purposive sampling was used. The participants
  selected had experience pertaining to the
  phenomena under investigation not just an opinion
• Participants included patients with ALS/MND their
  carers and their health-care professionals,
  including nurses, Palliative Care Consultants and
  Consultant Neurologists

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Inclusion and Exclusion Criteria

• Inclusion Criteria for patients
• Possible, Probable or Definite ALS/MND as defined
  by the El Escorial Criteria
• Patients gt18 years of age
• Patients diagnosed gt twelve months
• Patients had a nominated carer
• Patients without cognitive impairment
• Exclusion Criteria for patients
• Patients lt 18 years
• Patients without a nominated carer
• Patients diagnosed lt 12 months
• Patients with cognitive impairment
• Inclusion/Exclusion Criteria for carers
• Carers were identified as the person providing
  care for the patient

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Inclusion and Exclusion Criteria

• Inclusion criteria for health-care professionals
• All the health-care professionals had experience
  of the phenomena under
• investigation. In Hermeneutics it is the lived
  experience that is required
• and 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.

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Advance Care Directive

• An Advance Care Directive is a statement made by
  a
• competent adult relating to the type and extent
  of
• medical treatment he or she would or would not
  want to
• undergo in the future should he/she be unable to
• consent or dissent at that time.
• 
  (The Irish Council for Bioethics 2007)

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Sample 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 on
  --------------.
• The 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 ventilation
  
• Gastrostomy insertion Hydration - Nutrition
• Antibiotic treatment
• Palliative Care
• Cardio-pulmonary- resuscitation
• Invasive ventilation
• Locus of Care
• After 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 ------------------

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Advance Care Directives

• For the patients and carers that participated in
  this study, contemplating
• end-of-life decisions and having advance care
  directives were profound
• undertakings which were approached in a unique
  and individualized way.
• For some patients there are worse things than
  death for others long term
• invasive ventilation may be their preferred
  choice.
• The realistic medical options and interventions
  available will determine
• and influence these decisions. Johnston 2006
  Advance care planning
• should be firmly grounded in the values of the
  individual who, in turn,
• should understand the consequences of the
  decisions, both for
• themselves and their family.
• Advance care directives are increasingly
  recognised as important tools
• for safeguarding autonomy. However, not all
  patients wish to make
• advance care directives and some health-care
  professionals have
• difficulties implementing advance care directives.

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Autonomy

• 
  
  
  
  
  
  
  

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,
even demanded, 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 individuals doctor, family or friends

(Irish Council for
Bioethics 2007).




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Key findings

• An interpretative methodology was used and
  significant themes central to the
• participants emerged to illuminate the research.
• Patients/Carers
  Health-Care professionals
• Individualised Impact of Illness
  Difficulties Discussing E-of-L Issues
• Living for today
  Timing
• Loss
  Who Should Discuss E-of-L Issues
• Religiosity
  Family Inclusion
• Burden of Being a Carer
• Difficulties Discussing E-o-L issues
  Palliative Care
• Timing
• Consensus within families
  Education
• Legal Issues
  Legal Issues
• Sample Advance Care Directive Sample
  Advance Care Directive
• Advance Care Directive
  Advance Care Directive

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Individualised impact of the illness

• ALS/MND is a relentlessly progressive fatal
  illness resulting in a series of
• losses. It is characterized by progressive
  decline in motor function
• culminating in complete dependence on others for
  all activities of daily living.
• 
  
• The impact of ALS/MND is a unique and
  individualised experience influenced
• by how the patient and their carer perceive the
  changes caused by the illness,
• their previous coping mechanisms, the presence of
  support structures and the
• strength of their relationship prior to the
  diagnosis.
• For many patients with ALS/MND the shock of the
  diagnosis is compounded by
• the relentless progression of the illness. They
  continued to maintain hope
• and a positive outlook, considering themselves
  lucky and blessed and
• continued 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 as
• deeper, richer and more rewarding even while
  living with physical decline,
• realizing ones finitude, the present time
  becomes more precious
• 
  
  
  (Lambert 2006)

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Difficulties 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 something that is
abstract I live for now... I dont dwell on it
...I think its very good to plan your
dying...and Im saying youre dying as from the
time you start on, are on the last hill...Im on
one now but its only a slope, but on that last
hill, yes it is useful then Patients are
requesting that they remain in control and make
decisions about their death, just as they did
throughout their lives. For some their
end-of-life wish is not to die!
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Burden of being acarer

• Family caregivers are key figures in ALS/MND
  care. They usually provide
• the bulk of support to patients and play a
  significant and crucial role in
• clinical decision making process.
• The patients are so dependent on their primary
  caregivers that the couple
• represents a unique entity, a psychological dyad
  whose components cannot be
• considered separately (Chio
  et al 2004)
• There were concerns and anxieties expressed as
  advance care directives are not
• legal, would there be difficulties implementing
  them?
• Would there be a battle with some health-care
  professional at the end of a difficult
• and painful disease process to have the patients
  wishes respected? I hope the
• end of his life, will not result in an argument
  with an official

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Burden of responsibilityfor Carers

• Many of the participants identified the
  importance of family support throughout
• the disease trajectory but highlighted the
  importance of family consensus
• regarding end-of-life decisions and advance care
  directives. The carers
• reported that they had great difficulty
  discussing end-of-life issues and
• feared family recriminations if they supported or
  participated in the
• patients decision to request or refuse
  treatment.
• The carers reported their reluctance to revisit
  E-o-L discussions, once a decision
• was made, fearing that revisiting these issues
  would cause upset or that the
• decisions made would be revoked.
• Not all patients may wish to make ACD and this
  adds to the burden of
• responsibility for their carers.

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Difficulties for H-C-P discussingEnd-of-Life
issues

• The difficulty faced by most health-care
  professionals in initiating end-of-life
• discussions with patients with ALS/MND is that
  there needs to be an
• acknowledgement by the patient at some level that
  death, while not imminent, will
• be the likely outcome of the disease progression.
• One of the difficulties for H-C-P is to identify
  the most appropriate time to
• initiate end-of-life discussions and the
  importance of having these discussions
• before the patient loses the ability to
  communicate verbally.
• The H-C-P highlighted the importance of knowing
  the patient, of having a good
• rapport, of having a trusting relationship and
  also the importance of having the
• skills 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.

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Burden of Responsibilityfor Health-Care
Professionals

• The responsibility of ensuring that their
  patients are fully informed
• about the likely disease outcome.
• That the patients understand the implications of
  their decisions. That
• they understand that their decisions may
  influence the treatments they
• receive, the services that are available to them
  and may determine their
• place of death.
• Many reported the difficulties they encountered
  in dealing with different
• multidisciplinary teams and how the lack of
  communication and personal
• beliefs resulted in patients wishes not being
  respected.
• All the health-care professionals agreed that
  attendance at the ALS/MND clinic
• ensured that E-o-L issues and advance care
  planning would be discussed in a
• timely and appropriate manner. However, their
  concerns emanated from their
• experience that although patients had ACD these
  were not respected in local
• hospitals.

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Dilemmas

• Many health-care professionals believe it
  appropriate to respect and implement
• ACD. 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 stating
• that we dont 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 expected
• date 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-care
• professionals 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 for
• individuals without abandoning their own
  integrity. Many may not know the
• patient and they may have concerns that the
  directives may not actually reflect
• the current situation.

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Palliative Care

• All the participants identified the importance of
  including Palliative Care
• services and how vital their skills are in
  dealing with the complexities of
• end-of-life issues.
• There continues to be a stigma attached to
  including Palliative Care.
• Patients believe that their days are numbered
  if referred. Health-care
• professionals tend to back off, withdrawing their
  service, believing that if
• Palliative Care services are involved, there is
  nothing left to be done.
• Many believed that the palliative care team
  should be introduced earlier
• in the disease trajectory and that their services
  should be available for
• symptom management throughout the illness, not
  just in the
• terminal phase.

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Legal Issues

• Some of the participants expressed concern that
  ACD are not legal they
• hoped that legislation would provide clear
  guidelines and therefore
• ensure that they would be respected.
• Others believed that even if they are not legal,
  by presenting an ACD you
• are alerting a health-care professional to the
  expressed wishes of the
• patient.
• Amongst the health-care professionals that
  participated, there was
• ambivalence regarding the legislation of ACD and
  a view that a
• combination of having a verbal directive and
  having a nominated proxy
• would work very well.
• All the participants agreed that their preference
  was for a disease specific
• advance care directive.

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Findings of the study

• There needs to be improved clinical supervision
  and ongoing educational
• programmes for health-care professionals who are
  dealing with the very
• challenging, emotional and ethical difficulties
  identified in caring for these
• vulnerable patients.
• There needs to be improved communication between
  the tertiary hospital
• multidisciplinary teams and the community teams.
• An individualised approach is required and
  end-of-life issues should be discussed
• earlier 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 appropriate
• end-of-life and advance care discussions taking
  place.
• A sample advance care directive document or a
  booklet may facilitate discussing
• end-of-life issues.

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Conclusions

• A disease specific advance care directive was
  deemed more appropriate than the
• generic advance care directive proposed by the
  Law Reform Commission.
• There is a need for increased public information
  and awareness regarding end-of
• life decisions and advance care directives
  whereby it does not have to be a
• sentinel event before these issues are discussed.
• End-of-life decisions and advance care directives
  are an extremely important
• process that emerges in the context of the
  patient-healthcare professional
• relationship. It involves much more than
  completion of a formal advance care
• directive form or ticking the box
• The discussion of end-of-life may appear to be
  fraught with difficult decisions and
• ethical challenges. The potential for conflict
  may lead health-care professionals
• to avoid open discussion and advance care
  planning.
• Johnston (2006) believes that informing and
  guiding patients and their families
• through the decision making process to a peaceful
  death should be integral to

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Conclusions

• This study has illuminated the difficulties and
  the burden of responsibility faced
• by health-care professionals and the carers
  providing end-of-life care for
• patients with ALS/MND.
• Patients need to be aware that their autonomy is
  not absolute, that they cannot
• insist on receiving specific, unrealistic or
  illegal treatment nor can they compel
• their attending health-care professionals to act
  against their conscience.
• The difficulty for patients who wish to invoke an
  advance care directive and have
• their end-of-life wishes respected is that they
  are completely dependent on the
• attending Physician or health-care professional
  to respect and/or implement their
• wishes.
• Despite a hope that the use of ACDs would ensure
  that patients preferences for
• their end-of-life wishes would be respected this
  study has illuminated that
• the use of ACDs cannot promise or guarantee the
  patients a say in their future
• care.

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Thank You

• Many thanks to the patients, their carers and the
• health-care professionals who kindly participated
  in
• this research study.