(1)
Wisdom Hospice, Consultant in Palliative Medicine, High Bank, Rochester, UK
(2)
University of Kent, Kent, UK
(3)
Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Dublin, Ireland
Keywords
Quality of lifePalliative careSymptom controlAdvance care planningEnd-of-life care17.1 Introduction
End of life care relies heavily on a dynamic partnership between the patient, carers and the multidisciplinary palliative care team. Palliative care provides symptomatic management of late stage conditions focussing on relief of suffering, dignity and respect for patient autonomy, regardless of the underlying diagnosis.
In addition to medical management, palliative care seeks to address individual psychological, social and spiritual needs. While specialist palliative care has been traditionally associated with the symptomatic management of advanced neoplastic disorders, in recent years palliative care has gradually expanded into the management of progressive non-malignant conditions such as neurodegenerative conditions, chronic obstructive pulmonary disease, HIV, renal and cardiac failure.
In the absence of effective disease modifying therapies in most neurodegenerative conditions, specialist palliative care has a major role in improving the quality of life of patients. The effectiveness of palliative care intervention has been demonstrated by multiple studies in progressive neurological conditions.
17.2 The Definition of Palliative Care
Palliative care is defined by the WHO as “an approach that improves the quality of life of patients and their families facing problems associated with life-threatening illness, through the prevention and relief of suffering, early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual”.
This definition has been expanded to include, that palliative care:
Provides relief from pain and other distressing symptoms
Affirms life and regards dying as a normal process
Intends neither to hasten or 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 bereavement
Uses a team approach to address the needs of patients and their families, including bereavement counselling
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 to prolong life, such as chemotherapy or radiation therapy, and includes those investigations to better understand and manage distressing clinical complications.
17.3 The Definition of End of Life Care
End of life care is more complex to define and there is often uncertainty. This period is often considered to be the last 6–12 months of life, when there is increasing deterioration and the prospect of recovery has been abandoned. Within neurology there is increasing awareness of the need to consider both palliative and end of life care in patients with progressive disuse. This has been emphasised in the various guidelines on the care of people with neurological disease – including the Practice Parameters from the American Association for Neurology, the European Federation of Neurological Societies Guidelines on ALS (Andersen et al. 2012).
17.4 Palliative Care in Neurology
There is considerable evidence that palliative care intervention is beneficial in neurological conditions. A randomised controlled trial in multiple sclerosis, using a waiting list delayed service protocol, demonstrated superior symptom management, in particular nausea, as well as improved patient and caregiver satisfaction. Using a similar randomised waiting list for patients with ALS/MND, MS, progressive supranuclear palsy (PSP) and Parkinson’s disease (PD), improvement in a variety of symptoms – pain, breathlessness, sleep disturbance and bowel symptoms- and overall quality of life was observed.
Thus there is robust evidence that palliative care may help people with progressive neurological conditions and that this will extend to end of life care. It is also recognised that multidisciplinary care for people with progressive neurodegenerative conditions enhances quality of life (Van den Berg et al. 2005; Rooney et al. 2014) (Fig. 17.1).
Fig. 17.1
The multidisciplinary team in neurodegeneration. Evidence suggest improved quality of life as well as survival benefit if patients are cared for by an integrated multidisciplinary team (Rooney et al. 2014)
Given the complex nature of the illness, with inexorable decline and death as the inevitable outcome, a significant proportion of evidence of palliative care intervention in neurology comes from ALS/MND. It has been elegantly demonstrated by several authors that overall quality of life is not directly dependent on physical function (Neudert et al. 2004; Simmons et al. 2000). In fact, health related quality of life is only a minor contributor to overall quality of life. This provides an important rationale for specialist palliative care intervention in neurological conditions. Indeed, with the expansion of specialist palliative care provision to non-malignant conditions, a number of national frameworks have recently been developed to enable the optimal timing of palliative intervention and coordination of care between primary care physicians, neurologists, the multidisciplinary team, and palliative care specialists (Table 17.1).
Table 17.1
International frameworks for palliative care in neurology
Palliative care initiatives for non malignant conditions | |||
|---|---|---|---|
Name of the program | Country | Year | Focus |
Gold Standards Framework | UK | 2000 | Community based service, Carer Support |
PEACE – Palliative Excellence in Alzheimer’s Care Efforts | US | 2003 | Patients with dementia, Advance planning, Death at desired location |
Liverpool Care Pathway (Version 12) | UK | 2009 | Holistic approach to physical, psychological, social and spiritual care |
Preferred Priorities for Care | UK | 2004 | Discussion and respect of patient preferences |
Neurological Care Pathway | UK | 2007 | Management algorithm with indicators to advance care planning and specialist palliative care referral |
Neurology Taskforce of the EAPC | EU | 2009 | Palliative Guidelines for ALS, HD, AD, PD |
Guidelines for a Palliative approach in Residential Aged Care | Australia | 2006 | Evidence based palliative guidelines for elderly patients in residential units |
Respecting Patient Choices Program | Australia | 2005 | A comprehensive advance care planning program |
My Home Life | UK | 2007 | Support for staff working in care homes |
17.5 Aims of Palliative Care
Palliative care aims to carefully address to physical, psychological, social, and spiritual needs of patients in an integrative approach.
17.5.1 Physical
These aspects of care involve consideration of
Symptoms – such as pain, dyspnoea, dysphagia, communication issues
Mobility and comfort issues
Positioning and provision of help to aid daily living – small aids to help with limb/arm weakness, mobility aids such as frames and wheelchairs, and adaptions to housing and work environment
17.5.2 Psychological
This aspect of palliative care monitors reactions to the diagnosis, disability, dependency and dying and attempts to foresee and address psychological distress, such as anxiety, depression.
17.5.3 Social
Most patients will be part of local communities or wider social circles – whether family, friends or carers (both informal and professional). It is important to recognize and evaluate the personal reaction to the disease process.
17.5.4 Spiritual
Existential questions and spirituality are unique aspects of palliative care which require attentive consideration of individual cultural and religious needs. In a multi-cultural society an open-minded, empathetic, and sensitive approach is needed to address individual existential concerns about the deeper issues of life. There may be concerns about the meaning of life, about facing the changes that may occur with progressive disease, and about specific religious issues. Pastoral care or the involvement of the patients own support group, religious or otherwise, can be highly beneficial in reducing anxiety and improve quality of life.
17.5.4.1 Assessment Scales
Underreporting of pain and discomfort and inability to recall symptoms is a well-known problem of treating patients with long term conditions and in particular for those with impaired cognition. A comprehensive assessment and review of symptoms is the initial step following referral to specialist palliative services. It is clear the generic quality of life (QoL) instruments such as the Sickness Impact Profile(SIP), SF-36 Health Survey, or EuroQol EQ-5D are not directly applicable to neurological conditions. Disease specific instruments are superior in neurodegeneration, such as individualized QoL scales based on patients own QoL priorities, instruments optimised for patients with cognitive impairment or dementia, visual analogue scales or observer rated instruments taking the caregivers perspective into account. A number of validated tools are available for QoL evaluation in neurology. Some of them had been optimised for elderly people and have a role in neuropalliative assessments. Validated assessment scales also enable the measurement of the efficacy of palliative interventions.
The Memorial Symptom Assessment Scale (MSAS) is a scale used to assess 32 physical and psychological symptoms in three different dimensions: intensity, frequency, and distress.
The Rotterdam Symptom Checklist (RSCL) is another tool that measures both psychological and physical aspects of quality of life. These instruments, despite being recognised as highly sensitive, are too lengthy to be administered routinely in progressive neurological conditions.
The Edmonton Symptom Assessment Scale (ESAS) is patient-rated symptom visual analogue scale that may be particularly useful in patients with neurodegeneration. It is based on the assessment of 15 domains; activity, fatigue, physical discomfort, dyspnoea, pain, lack of well-being, appetite, depression, anxiety, nausea, difficulty sleeping, weakness, dizziness, cognition, and constipation.
The Disability Distress Assessment Tool (DisDAT, Regnard et al.) was developed in 2001 to identify distress in people with severe communication difficulties. The assessment tool uses a so-called “Distress Passport” a summary of signs and behaviours when patient is content and when distressed. Assessment takes Appearance, Vocal signs, Habits, Mannerisms and Posture into consideration.
Additionally, a number disease-specific of Quality of life assessment tools have been developed and used both in clinical practice and in the assessment of palliative interventions (Table 17.2).
Table 17.2
A selection of Quality of Life assessment instruments in neurodegenerative conditions
Alzheimer’s Disease-Related Quality of Life – ADRQL | Optimised for AD, 47 items in 5 domains: Social Interaction, Awareness of Self, Feelings and Mood, Enjoyment of Activities, Response to Surroundings |
The Cornell-Brown Scale for Quality of Life in Dementia – CBS | Incorporation of caregivers perspective |
Dementia Care Mapping -DCM | Observational assessment tool developed to be used in residential care for patients with moderate to severe disability |
Parkinson’s Disease Quality of Life – PDQL questionnaire | 37 item questionnaire; four subscales: parkinsonian symptoms, systemic symptoms, emotional functioning, and social functioning |
Quality of Life in Late-Stage Dementia -QUALID Scale | Observational scale for late stage dementia, brief, used in residential care |
Psychological Well-Being in Cognitively Impaired Persons PWB-CIP | 11 item, observer rated assessment instrument |
Dementia Quality of Life questionnaire D-QOL | 29 item scale in 5 domains: Positive Affect, Negative Affect, Feelings of Belonging, Self-esteem, Sense of Aesthetics |
Quality of Life-Alzheimer’s Disease (QOL-AD) | Composite scores from patient and caregiver responses on a 13 item scale |
Schedule for the Evaluation of Individualized Quality of Life-Direct Weighting SEIQoL-DW | A brief instrument used extensively in ALS research. Respondents identify the areas of life which are most important to their QoL, then rate their level of functioning or satisfaction with each. |
McGill QoL Questionnaire – MQOL | A 20 item scale frequently used in ALS, 5 domains: physical well-being, physical symptoms, existential well-being, psychological symptoms and support |
17.6 The Timing and Triggers of Palliative Care Interventions
More recently a Consensus document produced by the European Association for Palliative Care (EAPC) and the European Federation of Neurological Societies (EFNS)/European Academy of Neurology (EAN) has recommended that: “Palliative care should be considered early in the disease trajectory, depending on the underlying diagnosis” (Oliver 2014).
This is based on the evidence that for cancer care the earlier involvement of palliative care can increase survival of patients with lung cancer, reduces the cost of hospital care (Bakitas et al. 2009) and may improve symptom management and family satisfaction.
The initiation of specialist palliative care involvement will depend on the specific disease process – with involvement for patient with ALS/MND suggested from the time of diagnosis in many cases (Bede et al. 2011; Oliver 2014) whereas in MS involvement may only towards the end of life. In traditional care models, palliative input is sought at the relatively later stages of a progressive condition, resulting in patients potentially missing out on the expertise of palliative physicians in controlling distressing symptoms. More flexible models advocate early, and episodic involvement based on specific triggers – for instance for a person with ALS/MND this may be at diagnosis, consideration of gastrostomy, consideration and commencement of ventilatory support and in the final stages of life. The model of intermittent, symptom-based involvement has been proposed in neurodegenerative conditions where palliative involvement may overlap with active neurological care (Fig. 17.2).
Fig. 17.2
Models of palliative care intervenetion in neurology
17.7 Specific Symptoms Requiring Specialist Palliative Input
While the underlying pathophysiology may be different in various neurodegenerative conditions, there are a number of symptoms that may be common to most progressive neurological diseases.
17.7.1 Pain
Many patients will experience pain during the course of the disease progression – varying from 76 % for ALS/MND, 86 % in MS, and 85 % in PD. The aetiology of pain may be different in these conditions and should be managed accordingly:
Musculoskeletal pain – due to abnormal tone around joints which may respond to physiotherapy, positioning and non-steroidal anti-inflammatory medication.
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