Pathway of Care for PSP - A guide for Health and Social Care Professionals - PSPAP
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PSPA
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Pathway of Care
for PSP
A guide for Health and
Social Care Professionals
Working for a World Free of PSPIntroduction
PSPA
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Care Pathway for PSP – a guide to the standards of
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care and best practice for all those working with,
providing services for and supporting people living
with PSP and CBD.
Introduction
In 2011, the PSP Association commissioned Neurological In so doing, it will enhance the capabilities of those
Commissioning Support to develop a Care Pathway for individual health and social care professionals who are
PSP. This guide arose from that work and now describes dealing with PSP and CBD in the present, rather than
acceptable and deliverable standards of care and best attempting to educate a large population of those who
practice guidelines for the management of PSP by all might be required to do so at some time in the future.
disciplines. Contributions to the guide were made by
People living with PSP experience symptoms at different
people living with PSP, consultants and clinicians with
points, in different ways and to varying severities from
expertise in PSP and CBD.
person to person, and this can make acceptance and
adjustment very difficult for the individual and their family.
The guide comprises the following sections:
It can also make planning and providing services across
• A set of Standards of Care: before and at diagnosis, both health and social care hugely challenging.
during disease progression, and at end of life
CBD is a related progressive neurological disorder
• Health and social care guidelines for best practice at characterised by nerve cell loss or deterioration and
four stages of disease progression atrophy of multiple areas of the brain. Although some
characteristics differ from PSP, and overall progression is
• Symptom ‘snapshots’ of different symptom aspects,
generally slower, symptom management has much in
depicting likely prevalence and suggested interventions
common with that recommended for PSP, particularly the
• A visual care pathway depicting the range of services need for coordinated care, access to information and early
and support required by a person with PSP. palliative support.
In response to this work, in April 2012 the PSPA launched We hope this guide will help those supporting people
its new strategy “PSPA: SUPPORT FOR FAMILIES”. with PSP and CBD to plan coordinated and timely care
management, and enable greater access to services for
Our vision is to make life better for people with PSP
people affected by the conditions. The PSP Association
and CBD. In the next three years we will create closer
supports people living with both conditions and
relationships with people with PSP and CBD and we will
recommends that until a specific pathway for CBD is
deliver realistic, practical help and advice to ensure that
developed, this care pathway should inform best practice
they receive all the services and benefits they should. The
for CBD also.
PSPA will now focus on acting as advocates for individual
member families. The PSP AssociationStandards of Care
PSPA
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About Progressive
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Progressive Supranuclear Palsy (PSP) is a progressive Standards of Care
neurological disease caused by the death of nerve cells in
The PSP Association has set out the following Standards of
the brain. It causes severe and unpredictable impairments
Care as a guide, to ensure people with PSP receive the best
which have an enormous impact on the individual and
possible care, support and advice from the outset.
their family. Average life expectancy is six to seven years
from symptom onset for the majority of people.
As PSP progresses, it causes difficulty with balance, Before diagnosis
movement, vision, speech, swallowing and cognition. • People with suspected PSP should be referred quickly
The individual’s ability to walk, talk, feed themselves or to a movement disorder specialist or consultant
communicate effectively with the world around them specialising in Parkinson’s
decreases, thought processes often slow down, yet people
• PSP is frequently misdiagnosed, often as Parkinsonism.
with PSP usually remain mentally aware.
Suspicion of a misdiagnosis due to presentation of
PSP is a very individual disease, with symptoms being PSP symptoms requires prompt referral for further
experienced at different points, in different ways and to assessment by a consultant neurologist
varying severities from person to person. This can make
• Initial symptoms which may indicate PSP include:
acceptance and adjustment very difficult for the individual
and their family, whilst requiring increasing levels of care - Problems with balance, unsteadiness, and frequent
which often falls predominantly to family members, falls
particularly spouses. - Visual disturbances, such as blurring or double
People with PSP often find that their communication vision
and cognitive difficulties mean that social inclusion is - Cognitive difficulties, such as memory problems or
a challenge. General lack of understanding of both the changes in mood or behaviour.
condition and the needs of those living with it, means that
access to appropriate advice, support and information is a
constant struggle. The planning and provision of services is At diagnosis
further complicated by the variation of symptoms and the • Sensitive communication of the diagnosis to the
severity of the condition from person to person. individual and a supporting family member or friend is
essential.
• Offer of appropriate information should be made
including:
- A named professional or first point of contact for
information, advice and questions following the
diagnosis
- Local health and social care services and support
- About PSP and how it might affect the individual
and their family
- The PSP Association and any local support groups.Standards of Care
If the information isn’t accepted at diagnosis, the individual During the course of the condition
should be made aware of how they can access it at a later
date. No two people will experience PSP in the same way,
and care and support must be tailored to the individual.
• The offer of emotional/psychological support should However, everyone will require the following core
be offered elements:
• Follow up information and appointments should be
made Coordination of Care
• Comprehensive information should be sent to the GP • Access to a key worker or named point of contact
and the person’s key worker to coordinate the individual’s care and provide
• Referral for a clinical appointment with the person’s support. This might be a specialist nurse, a member
key worker or member of the multi-disciplinary team of the multi-disciplinary team or other professional
should be set up within two weeks of diagnosis. with an understanding of PSP, and may change as the
condition progresses
• Liaison and coordination between professionals,
sharing information to avoid duplication and ensure
effectiveness of treatments and management of
the disease. Multi-disciplinary working is the ideal in
effective management of PSP
• A holistic approach to management from diagnosis
onwards, so that the individual’s impairments are seen
in relation to each other and care which is provided
by one professional, considers or complements that
provided by another
• Regular reviews as necessary, following a referral,
without discharge, to ensure continuity of care
• Liaison across health and social care, and supported
care planning, reviewed and referred to by all
disciplines
• Referral to the specialist palliative care team
from an early stage for support and advice on
approaching the end of life, and early consideration
of advanced decisions, particularly if cognitive and /
or communication difficulties present early on in the
condition
• Access to appropriate medication with regular
reviews.PSPA
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Access to information Support for Carers
• Early access to information, in line with the • Information and support in using a Carer’s Plan
individual’s wishes, should be made from diagnosis. from social care should be offered, and ongoing referral
If the individual is not ready to receive certain made to it by health and social care professionals
information, their family, friends and/or carer may be
• Respite from the caring role and domiciliary care
more appropriate to share this with
to support the care of the individual at home should
• A single/first point of contact for advice, support be offered, with respite opportunity on a regular short-
and signposting should be offered, ideally through a term basis, or for longer periods
keyworker
• Advice and support on the condition and how to
• A contact number for initial out of hours support provide care to the individual is essential
should be given
• Access to services in the carer’s own right, to
• Regular signposting to appropriate information manage their own physical and mental health,
should be made, as impairments and disabilities alter, including bereavement counselling and support
and as new symptoms present following the end of life.
• Early information and discussions around
advanced decision-making should be made once Access to Equipment
the person has come to terms with their diagnosis, to • Consideration of appropriate equipment for specific
ensure the individual feels in control of their care and needs should be considered in line with other needs
wishes, at a time when they are able to make those and/or disabilities
wishes understood clearly.
• Speed of access to appropriate equipment to ensure
the individual can remain independent for as long as
Education to professionals
possible is essential. Consider sourcing core equipment
• Education and information for paid carers prior to immediate need.
including domiciliary and residential care staff, may
be required to address appropriate needs, especially
around swallowing, cognition and communication
impairments
• Clear understanding is required by the social
services’ representative for the individual, around
the rapidity of progression and complex needs of that
person, to ensure access to appropriate, timely support
and equipment, access to respite care and other
supportive services
• Education and support should be given to the
GP from clinicians, neurologists or specialist nurses
to ensure appropriate support and understanding,
prompt referrals, and effective palliative and specialist
palliative care is accessed.Standards of Care
Approaching an advanced stage and at
the end of life
• Careful monitoring in advanced stages of the • Access is available to appropriate support and staff
condition is important, to ensure coordination of who have been trained in end of life care, whether
care and access to the right support services. End of in the person’s home, in a residential home, hospital or
life is difficult to identify in PSP, and triggers to consider other care setting
include:
• Access to emotional and practical support should
- Inability to eat and drink/refusal of PEG be available for carers and family, including
- Infection which might require hospitalisation but bereavement counselling and support, if required.
which is refused
- A fall or a major fracture
- Rapid and significant weight loss
- Reduced consciousness level without reversible
cause and distinct from cognitive impairment,
which may be longstanding
• Access to specialist palliative care as appropriate and
with early referral to ensure prompt access as required
• GP palliative care registration should occur promptly,
to ensure continuity of care and access, and in
conjunction with the Gold Standards Framework
• Advanced decisions, which should have been
discussed in earlier stages of the disease, should be
consulted on again to ensure the individual’s wishes are
adhered to, including:
- Reviewing the individual’s preferred place of death
- Reviewing the individual’s wishes regarding hospital
admission for infections, Peg decision, resuscitation
and organ donation
- Ensuring that all aspects of care, i.e. physical,
psychological, spiritual and social, are considered in
line with the individual’s wishesBest Practice in PSP
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Early Stage (including diagnosis)
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IN BRIEF... Aims
Able to walk but falls occasionally; difficulty To ensure that people with PSP and their families are:
reading due to gaze; mild vocal changes such
as quietening; some changes in mood and • Given a prompt and accurate diagnosis (including ‘possible’ and ‘probable’)
reduced levels of social interaction. • Well supported at, during and after diagnosis, including in coming to terms with the
(see reverse for symptoms) condition
• Clearly directed to information and support
• Given details of an identified key worker to support ongoing information and access to
services
Key Considerations • Assisted to develop awareness, and supported to adapt to the changes of PSP
PSP is often misdiagnosed. Prompt referral • Helped to manage and reduce any symptoms
for assessment by a movement disorder • Assisted in maximising independence and participation in everyday life
specialist at first recognition of possible
symptoms is essential. Initial symptoms Assessment
which may indicate PSP include: • Establishment of accurate diagnosis by a movement disorders specialist, ideally with a
• Problems with unsteadiness, balance and special interest in PSP (could include consultant neurologist or geriatrician)
frequent falls (often backwards) • Assess understanding of person with PSP and their family and provide relevant
• Visual disturbances, such as difficulty with education
gaze, blurring or double vision • Evaluate symptoms, impairments and patient’s concerns
• Cognitive difficulties, such as changes in • Carry out Care Needs Assessment
mood or behaviour, including apathy and • Assess social and financial circumstances and support available (including work and
anxiety. driving).
PSP may often present through a fracture
clinic, eye specialist, falls clinic or speech and
Management
language therapist, and greater awareness of • Offer information and support delivered at individual’s pace:
the condition and diagnostic markers within – Signpost to the PSP Association
these areas could prompt a more timely – Provide contact details for the individual to initiate contact if they wish
diagnosis. – Offer contact directly with the individual two to four weeks following diagnosis to
provide post-diagnostic support (may be via telephone)
Access to a keyworker is likely to ensure
• Outline and discuss support available, including drugs treatment
coordination of care and prompt access to
appropriate services as they are needed. This • Discuss development of a care plan
role may be fulfilled by a specialist nurse • Identify, refer to, and ensure ongoing support from a coordinator or carer (e.g. a
(Parkinson’s or neurology), member of the keyworker)
MDT, consultant, community matron, or GP, • Ensure early access to:
and may change as the condition progresses. – Core multi-disciplinary team (MDT)*
– Counselling and / or psychological support
The individual may be at an early stage, but
– Local voluntary and support organisations
different symptoms present and progress at
• Ensure regular access to therapy
different times and rates.
• Ensure regular review according to the individual’s need
• Provide information about opportunity to be involved in research
*Core MDT would ideally include physiotherapist, occupational therapist, speech & language therapist,
This stage typically might span years 0-1. dietitian, specialist nurse, social worker and GP.
This document is intended to be used by
professionals supporting people with PSP. It is Outcomes
only a guide, and every individual should be • Reduction in distress and acceptance of diagnosis
treated as such, considered holistically, and
• Support for person with PSP and their family carer(s) ensuring maximised understanding
supported as they prefer. This guide will not
describe every individual’s journey, and everyone • Establishment of care networks - medical, nursing, therapy, social, voluntary
will progress at different rates: some slower, some • Coordination and continuity of care and timely referrals due to rapid progression of
faster than this describes. Above all, an individual, condition
personally tailored approach to care, coordinated • Prevention of complications including reduced hospital admissions
and timely, is the ultimate goal. • Maximised quality of life
Continued overBest Practice in PSP
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Early Stage (Symptoms/Information & Resources)
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Typical Symptoms
Ensure equal consideration is given to both motor and non-motor symptoms. A range of symptoms
may present to various degrees of severity at any stage, although typical at this stage are:
• Unsteadiness, balance problems and falls
• General slowing down
• Quietening of voice
• Emotional / behavioural changes such as apathy
• Depression
• Anxiety
• Problems with vision
• Fatigue.
Information & Resources for people affected by PSP
Consider providing information on the following:
• PSP Association
• Guide to benefits
• Employment support
• Local exercise opportunities
• Family support
• Regional Driving Assessment Centre, DVLA, Blue Badge Scheme and local transport
• Local equipment suppliers
• Disabled Living Foundation
• Access to Work / Job Centre Plus
• Local voluntary organisations including support groups, and organisations such as
Age UK and Carers UK
• Information on counselling, including family and individual counselling
• Brain Bank and opportunities for involvement in research.
Information and Resources for Professionals
• Diagnostic criteria: Litvan, I. et al. Movement Disorders Society Scientific Issues Committee report:
‘SIC Task Force appraisal of clinical diagnostic criteria for Parkinsonian disorders’, Mov Disord 18, 467-
86 (2003)
• PSP Association, including PSP Specialist Nurses for advice and support, PSP ‘Standards of Care’ and
‘Symptom Snapshots’ for more in-depth information
• Early recognition that equipment is likely to be needed, and prompt procurement of this will
maximise its use for the individual before they progress further.Best Practice in PSP
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Mid Stage
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IN BRIEF... Aims
Individual walks with aids and limited eye
• Symptomatic management and prevention of complications
movement makes eating and walking more
difficult. There is high risk of falls. The individual • Maintenance of function, self-care and quality of life despite advancing condition
will not often speak unless directly spoken to, • Access to therapeutic intervention from multi-disciplinary team
and speech is only understood by those listening • Agreed individual / family / professional goals
carefully. Behaviour is more impulsive with • Coordination and communication between all professionals
marked apathy. There is a risk of choking when
eating and a high level of supervision is required.
• Open communication about the individual’s wishes in more advanced stages
(see reverse for symptoms) • Carer support.
Key Considerations Re-Assessment
Many people may have already reached • Assess symptoms, impairments and disabilities including nutritional status
this stage before they are diagnosed, and • Risk assessment regards impulsive or risky behaviour
so practice set out in ‘Early’ stage should be • Ensure appropriate treatment, medication, services and support are available
considered in addition to ‘Mid’, specifically around
• Review Care Needs Assessment, and review and update care plan
information, coordination, and psychological
support. • Support carer - review and update carer’s plan and consider respite options
Discussion about advance care planning**, • Assessment against prognostic indicators (GSF) to consider access to specialist
preferred priorities of care and advance decisions palliative care.
to refuse treatment should be addressed early on,
as deterioration can be very rapid or sudden and
changes in communication or cognition could Management
make these discussions difficult. • Ensure access to core multi-disciplinary team (MDT)*
Early assessment for inclusion on the • Ensure access to social services
Palliative Care Register, and access to specialist • Ensure timely referrals for prompt access to and provision of appropriate equipment
palliative care with regular reviews for inclusion and adaptations due to extremely rapid progression of condition
ought to occur from here onwards due to the
rapidly degenerative nature of PSP. • Referral to specialist palliative care team if appropriate following assessment
Risky or impulsive actions and behaviour • Early opportunity to discuss Advance Care Planning for end of life
may cause a significant increase in pressure on • Facilitate symptom control
carers, and an increase in hospital admissions. The • Ensure optimum medication is prescribed and regularly reviewed
individual may require a high level of supervision
• Discuss development of carer’s plan.
at all times and in all care settings.
Education to paid carers and paid support is *Core MDT would ideally include physiotherapist, occupational therapist, speech & language therapist,
incredibly important as the individual’s needs dietitian, specialist nurse, social worker and GP.
become more specific and complex.
Particularly consider if appropriate: Outcomes
• Domiciliary care • Effective coordination of all services and professionals and across health and social
• Day care care
• Residential or respite care • Prompt access to equipment enabling optimum management and independence
• Hospice at Home.
• Optimum symptom control
• Clear goals of therapy and care that are both set and adhered to across disciplines,
This stage typically might span years 2-3. This
maximising independence, control and quality of life.
document is intended to be used by professionals
supporting people with PSP. It is only a guide,
and every individual should be treated as
such, considered holistically, and supported as
they prefer. This guide will not describe every
individual’s journey, and everyone will progress
at different rates, some slower: some faster than
this describes. Above all, an individual, personally
tailored approach to care, coordinated and timely, **Care plans and carer’s plans are only useful if referred to by multiple disciplines and across both
is the ultimate goal. health and social care. They should be referred to and updated regularly.
Continued overBest Practice in PSP
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Mid Stage (Symptoms/Information & Resources)
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Typical Symptoms
Ensure equal consideration is given to both motor and non-motor symptoms. Any variety and severity
of symptoms may present at any stage, although typical at this stage are:
• Frequent falls and increased muscular rigidity
• Slowness of thought and increasing difficulty with recall
• Emotional / behavioural changes such as apathy, depression and / or anxiety
• Problems with vision including blethrospasm
• Swallowing problems
• Communication difficulties, e.g. reduced speech and echolalia
• Constipation
• Sleep disorders
• Fatigue
• Pain.
Information & Resources for people affected by PSP
Consider providing information on the following:
• PSP Association
• Guide to benefits
• Employment support for carer
• Family support
• Local equipment suppliers
• Disabled Living Foundation
• Blue Badge Scheme and local transport (likely carer driving)
• Respite, breaks, and other means of alleviating demands on carer
• Information on advanced care directives and discussion about end of life
• Information on counselling, for both family and individual.
Information and Resources for Professionals
• Continuing Healthcare Decision Support Tool
• Gold Standard Framework Prognostic Indicators
• Information on advanced decisions and advance care planning
– Preferred place of care and preferred place of death
– Preferences for care, e.g. PEG feeding, catheterisation
– Advanced decisions to refuse treatment
– Enduring power of attorney.Best Practice in PSP
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Advanced Stage
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IN BRIEF... Aims
Highly reduced mobility and severe muscle
stiffness, requiring a wheelchair or confined to • Relieve symptoms and distress in person with PSP and family
bed; severe communication difficulties including • Prevent (and where necessary alleviate problems arising from) complications
lack of expression but fully comprehensive; • Access to respite and carer support
high risk of aspiration and pneumonia; likely
pain, and periods of sleepiness; functional • Maintenance of dignity and remaining function despite advancing condition
incontinence and severe social withdrawal. • Supported social interaction and communication as far as possible.
(see reverse for symptoms)
Re-Assessment
Key Considerations • Assess symptoms, impairments and disabilities including pain and nutritional status
The individual should already have been placed • Assess if treatment, medication, services & support are appropriate
onto the GP Palliative Care Register and should – Critical review of risk to benefit ratio, of all medication
be considered for access to specialist palliative • Assess method of communication with individual as appropriate, increasing liaison
care. This might include: with carer
• Clinical nurse specialists (Macmillan Nurses) • Review and update care plan and consider transfer to Continuing Healthcare
• Hospice day care • Review and update carer’s plan
• Hospice residential / in-patient care. • Review respite care options.
The individual may be in pain, and
communication difficulties may prevent this
from being understood or managed well. Management
This should be taken into account regarding • Manage symptoms, impairments, disabilities and pain
optimum care.
• Ensure access to core multi-disciplinary team (MDT)* and social services and if
Education to paid carers and paid support is necessary transfer across to Continuing Healthcare
incredibly important as the individual’s needs – Offer telephone or home visits if preferred by the individual
become more specific and complex. • Ensure access to community matron or district nurse for regular monitoring
Particularly consider if appropriate: • Ensure timely referrals for prompt access to and provision of appropriate equipment
• Domiciliary care and adaptations due to extremely rapid progression of condition
• Day care • Ensure access to regular assistance and support for carer through domiciliary care
• Residential or respite care • Ensure appropriate out of hours support to minimise unnecessary hospital
• Hospice at Home. admissions
Considering the individual’s needs: • Ensure access to specialist palliative care as appropriate
communication difficulties may mean that • Facilitate symptom control
preparing questions prior to appointments • Optimise medication and mode of administration according to the individual’s
with professionals should be considered, and needs
difficulty travelling to appointments at this stage • Support of carer e.g. through respite, domiciliary care, day care
may make home visits necessary.
• Management of feeding issues, whether with or without use of PEG
Increased support for the individual and their
carer may be required regarding emotional *Core MDT would ideally include physiotherapist, occupational therapist, speech & language therapist,
responses and fears approaching the end of life. dietitian, specialist nurse, social worker and GP.
Outcomes
This stage typically might span years 3-6. This
• Maintenance of dignity and support in line with individual needs and preferences
document is intended to be used by professionals
supporting people with PSP. It is only a guide, • Maintenance of autonomy as far as possible
and every individual should be treated as • Optimum symptom control, managing risk and disability (e.g. aspiration whilst
such, considered holistically, and supported as feeding)
they prefer. This guide will not describe every • Support to the patient, carer and family
individual’s journey, and everyone will progress • Maximised quality of life.
at different rates: some slower, some faster than
this describes. Above all, an individual, personally
tailored approach to care, coordinated and
timely, is the ultimate goal.
Continued overBest Practice in PSP
PSPA
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Advanced Stage (Symptoms/Information & Resources)
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Typical Symptoms
Ensure equal consideration is given to both motor and non-motor symptoms. Any variety and severity
of symptoms may present at any stage, although typical at this stage are:
• Immobility
• Severe muscle stiffness (particularly neck and back)
• Weight loss
• Dementia, severe slowness of thought and response, and difficulty with recall
• Functional incontinence
• Emotional / behavioural changes, e.g. apathy, depression and / or anxiety
• Severe problems with vision and eye movement
• Swallowing problems
• Severe communication difficulties
• Constipation and incontinence
• Sleep disorders
• Pain.
Information & Resources for people affected by PSP
Consider providing information on the following:
• PSP Association
• Guide to benefits
• Family support
• Blue Badge Scheme and local transport (carer driving)
• Local equipment suppliers
• Disabled Living Foundation
• Respite, breaks, and other means of alleviating demands on the carer
• Information on counselling, including family counselling, individual counselling.
Information and Resources for Professionals
• Continuing Healthcare Decision Support Tool
• Risk assessment tools for pressure ulcers
• Universal or locally adapted malnutrition screening tool
• Pain assessment tool i.e. PACSLAC Checklist.Best Practice in PSP
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End of Life Stage
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IN BRIEF... Aims
Severe impairments and disabilities and a
• Relieve distress in person with PSP and family
rapid and marked deterioration in condition;
this stage is usually triggered by a decision • Prevent (and where necessary alleviate) complications
not to treat, in accordance with the • Ensure carer support
individual’s previous expressed wishes.
• Enable maintenance of dignity
(see reverse for symptoms)
• Comply with patient wishes at the end of life as far as possible
• Comply with Advance Directives including decisions to refuse treatment.
Key Considerations
Before this stage has been reached, preparation Re-Assessment
should have been made for the individual’s
• Reassessment of capacity to make decisions (if making amends to advanced decisions)
wishes around death, and any Advance
Directives, including decisions to refuse • Review Advanced Directives and Advance Care Plans including decisions to refuse
treatment, should have been made. treatments preferences for care and place of death, and organ donation
• Assess support networks for carer.
The individual should already have been
placed onto the GP Palliative Care Register and
should be considered for access to specialist
palliative care. This might include:
Management
• Ensure access, and clear communication of the individual’s wishes, to all relevant
• Clinical nurse specialists (Macmillan Nurses) professionals
• Hospice at Home • Ensure access to community matron or district nurse for regular monitoring
• Hospice residential / in-patient care. • Ensure regular contact with GP
• Ensure appropriate out of hours support to enable care in preferred setting
The previous Advanced Stage lasts for an
uncertain period of time but there will come a • Ensure access to specialist palliative care team and specialist palliative nurse
point where the carer and family, and possibly • Facilitate symptom control and optimum medication
professionals also, will notice a difference in the • Maximise comfort and pain relief
individual.
• Support of carer e.g. through respite, domiciliary care, Hospice at Home
End of life is very difficult to detect in PSP as • Referral of carer to bereavement support and services.
many of the ‘triggers’ highlighted for other
conditions are already being experienced.
However, triggers to consider for PSP might be: Outcomes
• Reduced consciousness • Support during time of distress for individual and family
• Inability to eat and drink in absence / • Maintenance of dignity and adherence to patient wishes, including regards preferred
refusal of PEG place of care
• Maintenance of autonomy as far as possible
• Infection which might require
hospitalisation but which is refused • Optimum symptom control
• Support to the patient, carer and family
• A fall or a major fracture
• Individual dies in preferred place.
• Rapid and significant weight loss.
This stage typically refers to the last 6-8 weeks
of a person’s life. This document is intended to
be used by professionals supporting people
with PSP. It is only a guide, and every individual
should be treated as such, considered holistically,
and supported as they prefer. This guide will not
describe every individual’s journey, and everyone
will progress at different rates: some slower, some
faster than this describes. Above all, an individual,
personally tailored approach to care, coordinated
and timely, is the ultimate goal.
Continued overBest Practice in PSP
PSPA
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End of Life Stage (Symptoms/Information & Resources)
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Typical Symptoms
Any variety and severity of symptoms may present at any stage, and not everyone will experience all
symptoms. At this point the individual may also be experiencing comorbidities, and are likely to be
experiencing significant pain.
Information & Resources for people affected by PSP
Consider providing information on the following:
• PSP Association
• Family support
• PALs
• Spiritual support and services
• The Queen Square Brain Bank for Neurological Disorders (QSBB) and other donor options
• Bereavement support and services such as Cruise
• Information on bereavement counselling for the carer and wider family.
Information and Resources for Professionals
• Legal guidance regarding advanced directives e.g. ‘The Code of Practice,’ British Medical Association
1995
• Capacity, care planning and advance care planning in life limiting illness - A Guide for Health and
Social Care Staff 17 May 2011
• National Council for Palliative Care
• www.endoflifecareforadults.nhs.uk
• Advance Care Plans are not legally binding; Advanced Directives are legally binding.
www.goldstandardframework.org.uk’advancecareplanningSymptom Snapshots
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Problems with fatigue and sleeping
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Minimal Impairment Moderate Impairment Severe Impairment
Impairment - The individual may experience The individual may experience The individual may experience
considerations some low-level problems. some low-level problems. more severe sleep problems.
Considerations include: Considerations include: Considerations include:
• Occasional or low-level fatigue • Occasional and low level fatigue • Consistent fatigue which causes
which requires minimal • Some difficulty in both falling distress to the individual
intervention asleep and remaining asleep, • Some difficulty in falling asleep
• Some difficulty in either falling and averages 5 hours per night and/or remaining asleep, and
asleep or remaining asleep and or more. averages less than 5 hours of
averages 5 hours per night or sleep per night.
more.
Likelihood of At early stages, the individual may As the condition progresses, sleep patterns often become disturbed, there
symptom at still be at work, or living relatively can be difficulty falling or remaining asleep, or alterations in body clock
each stage independently, but general (awake at night, asleep during the day).
activities of daily living are likely
Pain, bladder and bowel problems, difficulties with movement (both
to require more effort, causing
reduced movement and spasms such as ‘restless leg’) and communication,
possible fatigue.
anxiety and medication side-effects can all impact an individual’s sleep.
Interventions • Advice on fatigue management • Advice on fatigue management • Advice on fatigue management
and Guidance and possible support and advice as appropriate as appropriate
to consider in altering or staggering activities • Support from domiciliary care as • Support from domiciliary care as
through MDT needed needed
• Support from carer or paid carers • Oral fluid and nutritional intake • Oral fluid and nutritional intake
to manage some day to day tasks can be affected. Small but can be affected. Small but
• Liaison with social care regards frequent meals should be taken frequent meals should be taken
domiciliary support may be • Assessment of sleep using sleep • Assessment of sleep using sleep
appropriate history leading to support and history leading to support and
• Assessment of sleep using sleep advice advice
history leading to support and • A sleep history should be taken • A sleep history should be taken
advice by the neurologist/geriatrician if by the neurologist / geriatrician
• A sleep history should be taken the individual reports difficulty if the individual reports difficulty
by the neurologist/geriatrician if sleeping sleeping
the individual reports difficulty • Support from MDT to advise on • Support from specialist nurse or
sleeping sleep hygiene and possible aids to referral to occupational therapist
• Support from MDT to advise on support more restive sleep to advise on sleep hygiene and
sleep hygiene and possible aids • Referral to a sleep clinic may possibly provide aids to support
to support more restive sleep be necessary, if the individual more restive sleep
• Possible medication to support consents and is able to attend • Consider support to carer should
sleep • Consider contributing symptoms individual’s sleep patterns cause
• Regular visits outdoors to access to sleep disturbance such the carer to become sleep
fresh air can improve sleep as breathing or swallowing deprived
cycles. difficulties • Liaison with social care for
• Consider support to carer should support, including nightsitters
individual’s sleep patterns cause • Regular visits outdoors to access
the carer to become sleep fresh air can improve sleep
deprived cycles.
• Liaison with social care for
support including nightsitter
• Regular visits outdoors to access
fresh air can improve sleep cycles.
Continued overSymptom Snapshots
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Problems with fatigue and sleeping
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• Where sleep disturbances are experienced, • Problems with communication can mean that
medication type and time of administration should conveying the reason for awaking or for not being
be reviewed to ensure it is not a contributing able to sleep can protract the length of time
factor before being able to fall asleep. For example, if the
individual wakes with a pain, explaining to their
• Pain, bladder and bowel problems, difficulties
carer that they are in pain and where it is may take
with movement and communication, anxiety
some time where communication is impaired
and medication side-effects can all impact an
individual’s sleep: • Appropriate products and equipment to support
sleep are important, for example products to
• Pain such as in the neck and back, referred pain
support bladder and bowel problems to ensure
such as headache, musculoskeletal pain, or pain
this does not contribute to sleeplessness or sleep
caused by comorbidities may all affect sleep, and
disturbance, and supportive mattresses for comfort
understanding and treatment of pain experienced
in reclining positions might be considered
may reduce difficulties with sleep
• Support and education to the individual, their carer
• Difficulties with movement including rigidity
and any nightsitters regarding ideal postures in
can mean that waking in the night to use the
bed can support better sleep
bathroom or to change position in bed require
carer support and take longer, delaying and • Anxiety regarding end of life can impact sleep.
increasing the difficulty of falling asleep again Clear discussions around end of life preferences
and opportunity for counselling, emotional and/or
• Other movement difficulties such as spasms or
spiritual support may help alleviate some concerns.
‘restless leg’ syndrome can impact an individual’s
ability to fall asleep and to remain asleep.
Medication may be able to minimise this in some
people
References
Golbe PSP Rating Scale (number 7) used to create sleep
assumptions. Golbe, L and Ohman-Strickland, P. ‘A clinical rating
scale for progressive supranuclear palsy’, Brain (2007), 130, 1552-
1565Symptom Snapshots
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Problems with vision
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- including diplopia, photophobia, apraxia, vertical gaze palsy
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Progressi
Minimal Impairment Moderate Impairment Severe Impairment
Impairment - The person may have The person may have more The person may have severe
considerations slight problems with vision. significant difficulties with vision. difficulties with vision, possibly
Considerations include: Considerations include: experiencing multiple vision
• Slight decrease in blink rate • Difficulty in opening and closing impairments. Considerations
possibly causing dry eyes eyes and/or involuntary eye include:
• Slower or hypometric eye closure possibly causing dry • Difficulty in opening and closing
movement (hypometric refers to eyes eyes
inaccuracy in looking at chosen • Excessive watering of eyes • Significantly slower or
target) (notably slower or hypometric hypometric eye movement, less
• Slight double or blurred vision. eye) than half the speed or accuracy
• Difficulty with gaze both of average expectation
upwards and downwards • Difficulty with gaze
• Double or blurred vision and/or • Double or blurred vision and/or
photophobia (aversion to bright photophobia (aversion to bright
light). light).
Likelihood of Double vision or vertical gaze Vertical gaze palsy is likely from Vertical gaze palsy is likely from
symptom at palsy occasionally present early mid-stage onwards, and double mid-stage onwards, and double
each stage on and often people experience vision is reasonably common. vision is reasonably common.
visual disturbance of some kind Photophobia and eyelid apraxia Photophobia and eyelid apraxia are
early on, even pre-diagnosis. are found in up to half of cases. found in up to half of cases.
Problems with gaze can cause trips
and falls.
Interventions • Early referral to orthoptist/eye • Regular review by orthoptist/eye • Regular review by orthoptist/eye
and Guidance specialist specialist specialist
to consider • Communication with key worker • Occupational therapist to • Eyelid apraxia will require
or MDT regarding possible aids consider and/or educate on eyedrops. Botulinum toxin may
or solutions to make activities of interventions. e.g. environmental be used to treat bletharospasm
daily living easier aids to bring objects into person’s and apraxia of eyelid opening. If
• For dry eyes, artificial tears or visual field botox is unsuccessful, surgery to
eye sprays should be prescribed • For dry eyes, artificial tears or eye the eyelid may be considered
by GP. Ocular lubricants may be sprays should be prescribed by • Infections need to be checked
used before the individual sleeps GP. Ocular lubricants may be used for regularly. GP can provide this
• Medications may affect tearing before the individual sleeps support and prescriptions
and should be carefully • Eyelid apraxia may be treated with • Liaison with community
considered during regular botulinum toxin matron or district nurse may be
medicine reviews by neurologist, • Education to family and carer appropriate
geriatrician, community regards possibility of tripping • Occupational therapist to
pharmacist or prescribing due to gaze, and alteration of consider interventions. e.g.
specialist nurse. environment. i.e. de-cluttering the environmental aids to bring
floor to help prevent falls. objects into person’s visual field,
de-cluttering of floor to help
prevent falls.
Continued overSymptom Snapshots
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Problems with vision
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• Difficulty making eye contact may impact • For double vision (diplopia) it is worth considering
communication. Liaison with a speech & language a temporary eye patch/shield to be worn when
therapist may be appropriate, as may tailoring watching television or eating meals
communication methods accordingly
• Prismatic spectacles can be used short term to
• Watering eyes may be problematic as eyes enable more normal vision, for example watching
overcompensate for reduced blink rate. Eye drops television with extreme axial rigidity
are still necessary, however, as cornea is still at risk
• Wrap around sunglasses can ease photophobia.
of ulceration. Watery discharge from eyes only
bathes the periphery of the sclera and without the
blink mechanism the cornea remains vulnerable to
becoming dry
• If certified vision impaired by the ophthalmologist/
orthoptist/eye specialist, information should be
provided on talking books and newspapers, and
signposting to RNIB
• Simple alterations to the individual’s environment
to avoid trip hazards, movement of certain objects
within common view, and other such interventions
ought to be suggested if difficulties with vision
arise
References
Golbe PSP Rating (numbers 13-17) used to consider severity
ratings. Golbe, L and Ohman-Strickland, P. ‘A clinical rating scale
for progressive supranuclear palsy’, Brain (2007), 130, 1552-1565
Article: Timothy Hain MD, 080211: ‘Progressive Supranuclear
Palsy’ http://www.dizziness-and-balance.com/disorders/central/
movement/psp.htm accessed 20.10.11Symptom Snapshots
PSPA
r Palsy
Problems with swallowing and respiration
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Progressi
Minimal Impairment Moderate Impairment Severe Impairment
Impairment - The person may have The person finds swallowing The person finds swallowing
considerations slight difficulty swallowing. difficult. Considerations include: increasingly difficult.
Considerations include: • Occasional coughing when Considerations include:
• Difficulties eating, including drinking fluids • Aspiration and respiratory
occasional choking or aspiration, • Drooling of saliva problems such as pneumonia
potentially caused by cramming • Difficulties eating, including • Choking and asphyxiation
mouth occasional choking or aspiration, • Consider PEG feeding
• May require small sips with potentially caused by cramming • Excess or sticky saliva may
liquids and small mouthfuls mouth increase swallowing and / or
• Occasional coughs to clear fluid • Excess or sticky saliva may breathing difficulties.
• May experience excess or sticky increase swallowing and / or
saliva compounding difficulties. breathing difficulties.
Likelihood of Initial bulbar dysfunction including Over half of people with PSP will experience difficulty swallowing, often
symptom at swallowing problems may present from mid/advanced stage onwards.
each stage an early stage for around 14% of
people, although these problems
are more likely in mid-stage.
Interventions • Prompt referral to speech • Regular reviews and management • Regular reviews and
and Guidance & language therapist and by speech & language therapist management with speech &
to consider coordination with MDT and key and wider MDT, to support safety language therapist and wider
worker where appropriate and efficiency of swallowing and MDT, to support safety and
• Possible referral to dietician to to minimise the risk of aspiration. efficiency of swallowing and to
ensure nutrition is appropriate • Referral to dietician as appropriate minimise the risk of aspiration
and to suggest certain dietary to tailor nutrition and food types • Liaison between speech
changes to guard against weight loss & language therapist and
• Weight should be recorded • Fluid intake may need to be dietician to optimise intake and
initially and at regular intervals to monitored if dehydration is nutrition through diet texture
ensure any weight loss is noted suspected modification, and advice on
• Likely to require supervision • Discussion of future treatment suitable food choices
from carer during meals, snacks options such as PEG should • Regular visit from community
or when drinking, particularly if be held early on, in a sensitive matron/ district nurse due
tending to cram manner to likelihood of pneumonia
• May require additional paid • Likely to require supervision/ resulting from aspiration
carers during meal times to support from carer during • Likely to require support with
support carer and guard against meals, snacks or when drinking, feeding from carer, possibly
choking/ aspiration. Liaison with particularly if tending to cram requiring additional support from
social care needed • May require additional paid carers paid carers at meal times. Liaison
• May experience minimal during meal times to support with social care needed
breathing difficulties, possibly carer and guard against choking/ • Consider PEG feeding if indicated
requiring an inhaler or nebuliser, aspiration. Liaison with social care as acceptable to the individual
but unlikely to affect activities of needed • May experience difficulty
daily living. • Equipment provision through breathing. MDT to advise on
• Medication may be used to dry liaison with occupational therapist positioning to ease breathing,
up saliva may be required, such as adapted relaxation techniques and how to
• (use hyoscine with caution as can cutlery and plate guards maximise breathing efficiency
cause hallucinations). • May experience difficulty • Education on optimal breathing
breathing positions and possible positional
relief through equipment may be
useful
Continued overSymptom Snapshots
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Problems with swallowing and respiration
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Minimal Impairment Moderate Impairment Severe Impairment
Interventions • Consider supporting • If necessary, referral to a
and Guidance individual and carer in ‘assisted respiratory specialist may be
to consider cough’ technique through useful
physiotherapist • Provision of equipment and
• Education on optimal breathing education to carers to manage
positions and possible positional aspiration at home may be
relief through equipment may be required.
useful • Medication may be used to dry
• If necessary, referral to a up saliva (use hyoscine with
respiratory specialist may be caution as can use hallucinations)
useful. mucolytics may also be helpful.
• Medication may be used to dry
up saliva
• (use hyoscine with caution as can
hallucinations).
• The individual should not be discharged from • Frequent infection, aspiration pneumonia and/or
their speech & language therapist following initial breathlessness may all be indicative of a move into
assessment the end of life stage. Referral to specialist palliative
care should be considered.
• The speech & language therapist should liaise with
the MDT* to coordinate care. If there is no MDT,
liaison across occupational therapist, dietician and
NB. PEG feeding may be necessary, although should
key worker, as well and social services (particularly
be avoided if the individual is cognitively impaired
if agency or domiciliary carers are involved) should
or likely to be distressed by it. If considered, the
occur from the individual’s initial assessment
patient should be fully consensual, with involvement
onwards
and coordination across the MDT ,and possibly
• Education and information sharing with the specialist palliative care team and community
professional carers, as well as family carers, is matron as applicable. If PEG feeding is an option it
extremely important. If the individual attends should be instigated before weight loss is too severe.
a day centre, has domiciliary care in the home,
or is in residential care, it is essential the speech
& language therapist provides information and *Core MDT would ideally include physiotherapist,
education to those professional carers around occupational therapist, speech & language therapist,
swallowing dietitian, specialist nurse, social worker and GP.
• Early, sensitive discussion around later treatments,
such as PEG feeding - with provision of appropriate
information - should be held with the individual
and their family carers References
Golbe PSP Rating (number 32) used to consider severity ratings
• Swallowing should be carefully monitored Golbe, L and Ohman-Strickland, P. ‘A clinical rating scale for
throughout the course of the condition as progressive supranuclear palsy’, Brain (2007), 130, 1552-1565
recurrent respiratory infections are frequent in Continuing Healthcare Decision Support Tool #6 ‘Nutrition –
individuals with PSP and are commonly associated Food and Drink’ and #9 ‘Breathing’ Care Domain considered:
•For #6: ‘No needs’ and ‘Low’ for ‘Minimal Impairment’; ‘Moderate’
with respiratory-related deaths for ‘Moderate Impairment’ and ‘High’ and ‘Severe’ for ‘Severe
Impairment’
•For #9: ‘No needs’ and ‘Low’ for ‘Minimal Impairment’; ‘Moderate
and ‘High’’ for ‘Moderate Impairment’ and ‘Severe’ and ‘Priority’ for
‘Severe Impairment’Symptom Snapshots
PSPA
r Palsy
Problems with cognition and mood
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- including neuropsychiatric features: cognitive change (memory, language, disorientation, executive
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function, flexibility), behavioural disturbance (challenging behaviours, impulsivity, apathy) and mood Progressi
disorders (depression, anxiety)
Minimal Impairment Moderate Impairment Severe Impairment
Impairment - The person may show some The person may show some The person may show cognitive
considerations indication of cognitive difficulty, cognitive difficulties, behavioural difficulties, behavioural disturbances
behavioural disturbance and/ disturbance and/or mood and/or mood disorders that require
or mood disorder which, with disorders that require consistent constant care and supervision,
support and reassurance and / or support and/or supervision to and possible need for access to
other interventions, is manageable. ensure their safe environment immediate and skilled response.
Considerations include: and quality of life. Considerations Considerations include:
• Occasional difficulty with recall include: • Slowness of thought, difficulty
and/ or some slowing of thought • Slowness of thought, difficulty with recall, mental capacity
processes with recall or reduced mental and/or disorientation, making it
• Altered behaviour such as capacity requiring support in impossible for the individual to
impulsivity and/or evidence of making decisions about daily life make appropriate choices and
apathy (often associated with • Altered behaviour, impulsive decisions about their daily life
executive dysfunction) and/or apathetic, affecting • Altered behaviour, impulsive and/
• Mood changes (including the individual and their carers’ or apathetic, significantly affecting
anxiety) having some impact on quality of life the individual and their carer’s
the individual • Mood disturbance (including quality of life
• Some withdrawal from attempts anxiety or distress) and/or • Mood changes including
to engage in planning, support hallucinations that have an depression and frustration,
or activities of daily life. increasing impact on quality of possibly exhibiting as aggression,
• Coming to terms with the life which has an impact on the
diagnosis itself can often cause • Withdrawal from most attempts individual and their carer’s life
anger, aggression and frustration to engage in planning, support • Withdrawal from any attempts to
as well as anxiety and depression. or activities of daily life. engage in planning, support or
activities of daily life.
Likelihood of Problems with cognition can occur Cognitive difficulties can arise as a side effect of some medication, as
symptom at early on. The recklessness tends to symptomatic of the condition and/or as a reaction to aspects of the
each stage becomes less of a problem once condition, and are likely to increase as the condition deteriorates.
mobility deteriorates. Apathy and
Apathy, slowness of thought and recall, mood alteration and withdrawal
behavioural disturbances usually
are likely to increase as the condition progresses.
develop later in a high percentage
of people. Slowness of thought may be compounded by increasing difficult with
communication.
Some emotional changes at
diagnosis, such as depression
or anxiety and difficulty with
acceptance, is to be expected.
Interventions • Counselling or psychological • Medication management should • Medication management should
and Guidance support offered early on to be tailored to the individual be tailored to the individual
to consider support coming to terms with • If cognitive impairment could • If cognitive impairment could
diagnosis be due to medication, the be due to medication, the
• Testing of memory function and neurologist/ geriatrician/ neurologist/ geriatrician /
possible referral to memory clinic PNS should consider altering PNS should consider altering
• Counselling for family members medication. A medicines review medication. A medicines review
(individual or group counselling) by community pharmacist may by community pharmacist may
also be considered also be considered
• Review by clinical psychologist • Review by clinical psychologist
and/ or community psychiatric and/ or community psychiatric
nurse and referral to psychiatrist nurse and referral to psychiatrist
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