Pathway of Care for PSP - A guide for Health and Social Care Professionals - PSPAP

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Pathway of Care
for PSP
A guide for Health and
Social Care Professionals

Working for a World Free of PSP
Introduction

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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 Association
Standards of Care

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About Progressive
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Supranuclear Palsy

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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.
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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 wishes
Best 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

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Best 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.

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Best 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.

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Best Practice in PSP

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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.

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Best Practice in PSP

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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’advancecareplanning
Symptom Snapshots

                                                                                                                            PSPA
                                                                                                                          r Palsy

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.

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Symptom 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-
                                                                    1565
Symptom Snapshots

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                                                                                                                              r Palsy

Problems with vision
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- including diplopia, photophobia, apraxia, vertical gaze palsy

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                      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.

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Symptom Snapshots

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                                                                                                                                    r Palsy

Problems with vision
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- including diplopia, photophobia, apraxia, vertical gaze palsy

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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 20.10.11
Symptom Snapshots

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                                                                                                                           r Palsy

Problems with swallowing and respiration
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                  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

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Symptom Snapshots

                                                                                                                                 PSPA
                                                                                                                               r Palsy

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

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                                                                                                                             r Palsy

Problems with cognition and mood
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- including neuropsychiatric features: cognitive change (memory, language, disorientation, executive

                                                                                                                 ve
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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