Review articlePalliative care and its emerging role in Multiple System Atrophy and Progressive Supranuclear Palsy
Section snippets
Phases of MSA and PSP compared with Parkinson's disease
Multiple System Atrophy (MSA) is a sporadic condition producing early disability. It includes features of Parkinsonism, autonomic failure and cerebellar dysfunction [4]. Progressive Supranuclear Palsy (PSP) is characterized by early falling, supranuclear gaze palsy (with vertical gaze typically affected first) and cognitive problems [5]. MSA and PSP, like Parkinson's disease (PD) are incurable and progressive. Unlike PD, they have poorer response to dopaminergic therapy and a faster course,
What is palliative care?
The 2002 WHO definition of palliative care (PC) is: Palliative Care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual [8].
This definition of PC also takes into account psychological and spiritual aspects of living with
How PC relates to neurodegenerative disease
Palliative care can be challenging in the context of neurodegenerative disorders. The progression of disease can be fluctuant with unforeseen rapid deterioration on a background of inexorable decline. Considerable loss of function and symptom burden can occur relatively early in the disease process resulting in significant period of time with marked morbidity [16].
Evidence for the use of PC exists for several neurodegenerative conditions such as PD, MND and dementia.
PD is known to produce
Trajectories of disease in context of MSA and PSP
Trajectories of decline have been described in an effort to allow identification of patients who are approaching the end of their lives, to permit preparation, planning and provision of services such as PC and hospice services. Glaser and Strauss [43] described four distinct trajectories;
- 1.
Sudden death, such as after an acute illness or accident.
- 2.
Terminal decline, whereby morbidity is fairly low until a rapid decline is experienced. This is suggested as the usual course in cancer.
- 3.
Frailty,
Palliative care need in Parkinsonian disorders
Studies have appraised the palliative care requirement in patients with MSA and PSP, together with advanced PD. In a study over one year, overall two-thirds were found to be living with profound symptoms and functional impairment. The majority of PSP patients had an H&Y score of 5, and nearly half (47%) had this score in MSA. Furthermore, half of the patients deteriorated in terms of their symptom burden or level of distress during the course of the 12-month study; survival in MSA and PSP being
Quality of life in MSA and PSP
Quality of life is often described as being of great importance to patients and families with progressive conditions and clinicians should have an appreciation of the effect of any intervention to improve patient well-being. Factors which have an influence, both negative and positive on QoL in particular conditions should be considered in an effort to improve patient experience wherever possible.
Depression has been studied in terms of its effect on life satisfaction in MSA. In one group 36% had
Caregiver quality of life in MSA and PSP
There is a paucity of evidence for caregiver experiences specifically in the context of PSP and MSA. Uttl et al. [59] investigated caregiver strain in 180 PSP caregivers and found that strain increased with disease severity then plateaued after 18 months. Patient aggression and low mood together with caregiver gender were reported to account for 37% of variance in caregiver strain (with female carers reporting more strain). A qualitative study using focus groups with PSP patients and their
Barriers to palliative care
In practice, many practitioners find introducing PC as a concept to patients and relatives to be difficult. Barriers to accessing palliative care have been explored in the context of cancer treatment in the USA. Analysis of surveys from practitioners identified inadequate training in the palliative approach and financial constraints to its use, preventing more extensive provision of palliative care to oncology patients in these centers [68], [69]. A Japanese perspective showed a wide range of
Advance planning decisions
Advance decision making is one of the fundamental tenets of palliative care. However, it is important to individualize approaches and not to see completed documentation as the ultimate goal.
Preferences around care can vary and shift with time or stage of illness, so it should be made clear to patients and families that any discussions had or forms completed are not ‘set in stone’ but are the summary of patient preferences with physician guidance at that point of time and can evolve [72].
It may
Provision of palliative care
There is growing recognition of the benefit of the palliative approach in neurodegenerative conditions. How this will be integrated and who will deliver palliative care is still uncertain. Specialist palliative care physicians will likely be required for the especially difficult cases but are unlikely to be able to provide a service for whole cohorts. This suggests that neurologists and movement disorder specialists, who diagnose and provide the main contact for MSA and PSP patients, will
Summary
The recognition that patients with atypical Parkinsonism might benefit from early general palliative care has been described [53]. The complex combination of symptoms and their severity displayed by this patient group are comparable to those of patients with advanced cancer [32].
The current practice of PC arose from the care of patients with malignant disease. It is becoming apparent that patients with non-malignant, life-limiting conditions can benefit from the application of PC using a
Acknowledgement
This work did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors are employed by Newcastle University and have clinical honorary contracts with Newcastle Hospitals NHS Trust. Dr Lee has a contract with South Tyneside, NHS Foundation Trust.
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