HM Medical Clinic

Long-term neurological conditions

CONCISE GUIDANCE TO GOOD PRACTICE A series of evidence-based guidelines for clinical management Long-term neurological conditions:
management at the interface between
neurology, rehabilitation and palliative care
NATIONAL GUIDELINES British Society of Rehabilitation Medicine Clinical Standards Department Guideline Development Group The purpose of the Clinical Standards Lynne Turner-Stokes DM FRCP, Nigel Sykes FRCP FRCGP, Eli Silber Department of the Royal College of Physicians FCP(Neuro)SA MD and Lucy Sutton MSc prepared this guidance on is to improve patient care and healthcare behalf of the multidisciplinary Guideline Development Group provision by setting clinical standards and convened by the National Council for Palliative Care (NCPC) and the monitoring their use. We have expertise in the British Society of Rehabilitation Medicine (BSRM) in association with development of evidence-based guidelines and the Clinical Standards Department of the Royal College of the organisation and reporting of multicentre comparative performance data. The Professor Lynne Turner-Stokes (BSRM)
department has three core strategic objectives: Consultant in Rehabilitation Medicine, Herbert Dunhill Chair of to define standards around the clinical work
Rehabilitation, King's College London of physicians, to measure and evaluate the
Dr Nigel Sykes (Association of Palliative Medicine)
implementation of standards and its impact Consultant in Palliative Medicine, St Christopher's Hospice, on patient care; and to effectively implement
these standards. Dr Eli Silber (Association of British Neurologists)
Our programme involves collaboration with Consultant Neurologist, King's College Hospital Trust specialist societies, patient groups and Lucy Sutton (NCPC)
national bodies including the National National Policy Lead, National Council for Palliative Care, London Institute for Health and Clinical Excellence Vicki Morrey (NCPC)
(NICE), the Healthcare Commission and the National Council for Palliative Care Long-term Neurological Health Foundation.
Conditions Group Chair; Chief Executive, Prospect Hospice, Swindon Tricia Holmes (Motor Neurone Disease Association (MNDA))
Concise Guidance to Director of Care Development, Motor Neurone Disease Association, Good Practice series The concise guidelines in this series are Sue Thomas (Royal College of Nursing)
intended to inform those aspects of physicians' Nursing Policy and Practice Advisor, Royal College of Nursing, clinical practice which may be outside their own specialist area. In many instances the Sue Smith (MNDA)
guidance will also be useful for other clinicians Regional Care Advisor, Motor Neurone Disease Association, including GPs and other healthcare Dr Mark Lee
The guidelines are designed to allow clinicians Consultant in Palliative Medicine, St Benedict's Hospice, Sunderland to make rapid, informed decisions based Judi Byrne (Sue Ryder Care)
wherever possible on synthesis of the best Palliative Initiative in Neurological Care (PINC) Co-ordinator, available evidence and expert consensus Sue Ryder Care, London gathered from practising clinicians and service Janice Brown
users. A key feature of the series is to provide Senior Lecturer, University of Southampton both recommendations for best practice, and Gill Ayling (Department of Health)
where possible practical tools with which to Lead, National Service Framework for Long-term (Neurological) implement it.
Conditions, Department of Health Series Editors: Lynne Turner-Stokes FRCP and
Dr Christopher Roy (BSRM)
Bernard Higgins FRCP
Consultant in Rehabilitation Medicine, Southern General Hospital, Glasgow The British Society of Rehabilitation Medicine (BSRM), the Guideline Development Group i
Royal College of Physicians and the National Council for Palliative Care all contributed funding or resources towards the guidelines' development. Support for the Background 2
initial literature search was provided by a grant from the The challenge of lifelong care for people with LTNCs 3
Department of Health, and funding for its update and preparation of the manuscript was provided by the Aims and methodology 3
Dunhill Medical Trust and the Luff Foundation. The Brief summary of the evidence 3
authors are grateful to Tariq Saleem, Barbara Sullivan The respective roles of neurology, rehabilitation and
and Richard Harding (King's Centre for Palliative Care in palliative care 4
Neurology, King's College London) and to Judith Sargeant (Department of Health) for their help with the initial THE GUIDELINES 9
literature gathering and grading.
Implications for implementation 10
Training 10
Tools for implementation 10
Citation: Royal College of Physicians, National Council for
Palliative Care, British Society of Rehabilitation Medicine.
Long-term neurological conditions: management at the interface between neurology, rehabilitation and palliative 1 Guideline development process 12
care. Concise Guidance to Good Practice series, No 10.
2 Methods used to evaluate the evidence 13
London: RCP, 2008.
3 Tools for implementation 15
A. Checklist to use on admission of patients with LTNCs 15
All rights reserved. No part of this publication may be B. Key areas of symptom management with care reproduced in any form (including photocopying or storing it in any medium by electronic means and Bowel management 17
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use of this publication) without the written permission of the copyright owner. Applications for the copyright Nausea and vomiting 20
owner's permission to reproduce any part of this Shortness of breath 21
publication should be addressed to the publisher.
Copyright 2008 Royal College of Physicians
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Review date: 2011 Designed and typeset by the Publications Unit of the Royal College of Physicians Printed in Great Britain by The Lavenham Group Ltd, Suffolk Long-term neurological conditions 1
The National Service Framework (NSF) for Long-term Conditions* advocates lifelong care for people Long-term neurological conditions (LTNCs)
with LTNCs.2 It highlights the need for integrated comprise a diverse set of conditions resulting
care and joined-up services. Within its 11 Quality from injury or disease of the nervous system
Requirements (QRs), it makes recommendations for that will affect an individual for life. Some 10
the provision of specialist neurology (QRs 2/3), million people in the UK are living with a
rehabilitation (QRs 4–6) and palliative care (QR 9) neurological condition which has a significant
services to support people throughout and to the end impact on their lives, and they make up 19%
of their lives. In addition, QR 11 makes of hospital admissions. These guidelines build
recommendations about the management of people on the Quality Requirements in the National
with LTNCs when receiving care for other Service Framework for Long-term
conditions, in any health or social care setting.
(Neurological) Conditions to explore the
When someone with an LTNC is admitted to a interaction between specialist neurology,
general hospital setting for a procedure or because of rehabilitation and palliative care services, and
acute illness, hospital staff have to manage both the how they may best work together to provide
illness and the LTNC. Many patients are maintained long-term support for people with LTNCs and
on finely tuned management routines (eg 24-hour the family members who care for them. The
spasticity management programmes, treatment for guidelines also provide some practical advice
PD symptoms, or bladder/bowel regimens), which, if for other clinicians when caring for someone
disturbed, may lead to increased morbidity and with an LTNC, and outline indications for
distress, and can take weeks to re-establish. In specialist referral.
addition, patients require an accessible environmentand access to their usual equipment, eg wheelchair,communication aid. The NSF emphasises theimportance of recognising the expertise of a personand their family in managing the condition, of maintaining close contact with the individual's Long-term neurological conditions (LTNCs) form a regular team, and of calling for specialist help, if diverse set of conditions resulting from injury or required. However, as many generalists have received disease of the nervous system that will affect an little training in these areas of clinical practice, they individual for the rest of their lives. They include: are sometimes uncertain about the type of help thatthe different services can offer for people with ■ sudden onset conditions (eg acquired brain injury of any cause (including stroke), spinal cord injury) ■ intermittent conditions (eg epilepsy) ■ progressive conditions (eg multiple sclerosis (MS), motor neurone disease (MND), Parkinson'sdisease (PD) and other neurodegenerative *National Service Framework for Long-term (Neurological)
Conditions: The Department of Health's National Service
■ stable conditions with/without age-related Framework (NSF) for Long-term Conditions was publishedin March 2005. Although much of the guidance applied to degeneration (eg polio or cerebral palsy). anyone living with a long-term condition, the main focus of Taken together, LTNCs are more common than most the document was on neurological conditions. To avoidconfusion with other policy documents contained within the clinicians realise. Some 10 million people in the UK Long-term Conditions Strategy (which includes the are living with a neurological condition which has a frameworks for renal services and for diabetes) the NSF has significant impact on their lives, and they make up subsequently been re-badged as the NSF for Long-term 19% of hospital admissions.1 Long-term neurological conditions The challenge of lifelong care for between specialist neurology, rehabilitation andpalliative care services, and how they may best work people with LTNCs together to provide long-term support for peoplewith LTNCs and the family members who care for The place for palliative care in non-cancer patients is them. They also provide practical advice for other increasingly recognised,3–6 especially in rapidly fatal clinicians who may find themselves caring for neurological conditions such as MND,7–8 and many someone with an LTNC, as well as outlining guidelines now recommend early referral to palliative indications for specialist referral. care services.9–10 However, there are some significantdifferences in the palliative care needs of people with ■ They were drawn up in accordance with the LTNC, compared with those with cancer.4,11,12 Appraisal of Guidelines Research and Evaluation(AGREE) system for guideline development. ■ In general, neurological conditions have a longer and more variable time course: it is often hard to ■ They build on work carried out by the determine exactly when a patient is entering the neurological conditions policy group of the terminal stages of life. National Council for Palliative Care (NCPC).
Further information, including good practice ■ Symptoms are diverse, and many patients have examples and details of a pathway to assist joint complex disabilities which include cognitive, working, can be found in Focus on Neurology behavioural and communication problems as well available at the NCPC website: as physical deficits. Palliative care teams used tocaring for people who can talk to them may find it The Guidelines themselves are on page 9, and the challenging to manage someone with profound methods of guideline development are shown in dysphasia or cognitive dysfunction. Teams may also need specific training in posturalmanagement and physical handling, eg for Brief summary of the evidence
someone with severe spasticity.
For evaluation of the evidence, we used the typology Many physicians think of rehabilitation as a short- and grading system developed for the NSF for Long- term intervention following a single incident illness term Conditions.15 The typology is designed to place or injury. However, long-term disability management value on the experience of users and professionals is also a core element of many rehabilitation services, (Expert evidence E1 and E2 respectively) as well as
which often work in the community to support research, and also to value high-quality research people to the end of their lives. In addition, many regardless of the design. Full details of this system are neurology departments now have specialist nurses (for example in MS, MND or PD), who also provide There is strong evidence (Research grade A (RA))
long-term support for patients and their from Cochrane and other systematic reviews that families.12,13 Given the current financial pressures on multidisciplinary rehabilitation can improve the the NHS, an understanding of the interface between experience of living with a long-term neurological neurology, rehabilitation and palliative care is critical condition, both at the level of functional activity and to ensure that services work together to provide coordinated care for people with LTNC, rather thanduplicating care provision and then competing for There is strong indirect (Research grade B (RB))
the scarce resources.2 evidence from the cancer literature that palliativecare improves quality of life19 and is cost effective.20Community-based palliative care teams can reduce Aims and methodology time in hospital, allowing people to spend more timeat home and increasing patient satisfaction in the These guidelines build on the NSF Quality terminal stages of their lives.21,22 Requirements to explore further the interaction Long-term neurological conditions There is also evidence (RB) that these principles
for example a network of MND care centres, and translate into other non-cancer conditions. 5,23,24 regional networks with user-centred Specifically within long-term neurological multidisciplinary teams who have specialist conditions, reviews by Voltz 1997,11 O'Brien 1998,25 knowledge of the needs of patients with neurological Mitchell 2007,26 and Travers27 demonstrate that patients in the final stages of amyotrophic lateralsclerosis (ALS), multiple sclerosis (MS), Huntington'sdisease and dementia experience very similar The respective roles of neurology, symptoms to those observed in cancer patients (iepain, dyspnoea, death rattle, restlessness, delirium, rehabilitation and palliative care in drowsiness, nausea and vomiting, depression), and these experts suggest (E2) that the approach to
controlling symptoms in progressive non-malignant
As part of the preparation for these guidelines, a conditions can be adopted from the strategy for cross-sectional postal survey using parallel managing cancer-related symptoms.
questionnaires was sent out to consultants in There is strong evidence (RA) for the effectiveness of
neurology, rehabilitation and palliative medicine by pain relief – in particular in MND,7,12,28 but also in the National Council for Palliative Care through MS and PD.29 A number of authors (E2) have
their specialist societies.37 The full findings from this emphasised the need for well coordinated palliative can be found in Neurological conditions: from and neurological care,8,30 to reduce the call for active diagnosis to death available at euthanasia and physician-assisted suicide, and to The aim of this study was to explore the interaction reduce the rate of suicides associated with many between the three specialties and the perceptions of the respective consultants regarding their relativeroles in caring for people with LTNCs. However, the literature also highlights the longer-term and more variable course of conditions such as The survey showed general agreement about the core MS, and the variety of symptoms which may be less contributions offered by each specialty. common in cancer patients – fatigue, spasticity, ■ Neurologists were seen by most as being the weakness, visual loss, sexual dysfunction, swallowing primary providers of assessment, diagnosis and and speech problems, epileptic seizures, management of the disease. myoclonus.26,31 There is a need for palliative care ■ Rehabilitation physicians were primary providers teams to learn additional skills – in particular of therapy, equipment, social/psychological postural handling and support, management of support and service coordination during the phase percutaneous endoscopic gastrostomy (PEG) between diagnosis and death. feeding8,11 and ventilation32,33 (RA/E2), as well as
■ Palliative physicians were primary providers of skills in management of cognitive and behavioural terminal care, and the management of death and problems27 (Research grade C (RC)). Therefore the
need has been stressed for coordination betweenpalliative care and rehabilitation teams, which also The respective roles are described in more detail in have a track record in providing long-term care and Table 1 and illustrated in Fig 1. These are a guide only, as the roles described will vary betweendifferent specialists and different clinical services.
Despite the strength of this evidence, the research has However, non-specialist clinicians may find this a demonstrated that a relatively small proportion of useful resource when considering referral for patients with palliative care needs not related to specialist help with a given problem.
cancer can access the services,3 and that many have
unmet palliative care needs (RA).28,38 Nevertheless,
There were also some important areas of overlap there are some pockets of good practice in the UK, which highlight the need for collaborative working Long-term neurological conditions Table 1. Key roles of neurology, rehabilitation and palliative care services in supporting people with long-
term neurological conditions.
• Investigation and diagnosis • Ongoing medical management • Multi-professional management of • Information about condition and distressing symptoms, usually in – diagnosis/investigation based patients with limited life on longer-term observation expectancy for rapidly progressive • Ongoing specialist advice – interventions for long-term • Treatments to modify the disease symptoms, eg spasticity, – pain, nausea and vomiting, nutrition, pain, depression, – interferon, steroids etc bladder and bowel programmes – anxiety/depression, insomnia • Interventions for neurological • Practical holistic support and – management of confusion, disability management agitation in conjunction with – restoring independence where psychiatric and psychology – tremors, and other abnormal – supported care on long-term • Support for end-of-life decisions complex disability and advance care planning, eg: • Practical advice from specialist – advance statements and • Coordinated multidisciplinary team – incontinence, pain interventions including physio, O/T, – choice over place of care – sources of additional help and SLT, psychology, SW, orthotists – assessment of capacity in relation to these decisions • Aids and equipment: – links with the voluntary sector – eg wheelchairs, environmental • Support of the dying person and their family, eg:– psychosocial • Planning and support: – integrated care planning – between health, social services, voluntary services etc • Bereavement counselling – support for benefits, housing, • Advisory/liaison service adapted accommodation etc – links to local palliative care – vocational rehabilitation, resources and teams education, leisure – professional education in the – driving/community mobility provision of generic palliative • Communication and psychosocial care and support – provision and coordination of – adjustment for long-term community support services disability for patients and their families/carers – supported communication for – management of confusion/ unwanted behaviours (including verbal and physical aggression) in conjunction with neuro-psychiatric services • Medico-legal issues – assessment of mental capacity– Power of Attorney, Court of O/T = occupational therapy; SLT = speech and language therapy; SW = social worker.
Long-term neurological conditions Physical management of long-term
Disease modification communication deficits profound brain injury Dealing with loss Spiritual support Fig. 1. The interaction between specialist neurology, rehabilitation and palliative care services in the
management of patients with long-term neurological conditions.
practice, and for clinicians to respect the expertise helpful to share these skills with palliative physicians of others in related areas. For example, in addition during the later stages of care in dealing with to diagnosis and prognostication, neurologists saw symptom management and end-of-life decisions. themselves as key providers of therapy, spasticity A further interesting point to emerge was a management etc, and rehabilitation physicians need difference in the frame of reference. Rehabilitation to respect that. Conversely, neurologists need to teams tend to be good at defining and working recognise the diagnostic skills of rehabilitation towards goals, but sometimes struggle where a physicians, who sometimes have the opportunity to patient's deteriorating health changes the goalposts observe patients at close quarters for longer periods rapidly and repeatedly. By contrast, palliative care to reach diagnoses that may have evaded earlier teams, who are used to this progression, sometimes have difficulty with patients at stages where the It is also important for neurologists and disease is not advancing. Therefore rehabilitation rehabilitation physicians to embrace the involvement teams may be slower to move to a ‘symptom- of palliative physicians at earlier stages, and take management' mode, whilst palliative care teams advantage of their experience in managing symptoms may be slow to move to adaptive strategies – such as nausea, vomiting and breathlessness which eg wheelchair training, seating programmes, use of can occur in relative early phases of the disease. orthoses, and environmental controls etc. Again this In return, rehabilitation physicians may have useful may be addressed by closer collaboration and cross- experience in managing people with profound referral between the different services.
cognitive and communication deficits, and it may be The survey highlighted a general shortfall in service Long-term neurological conditions Fig. 2. ‘Life circles': proposed model for the relationship between neurology, rehabilitation and palliative care
in caring for people with long-term neurological conditions. In this model, the shading illustrates intensity of
involvement of neurologists and palliative care physicians in acute and terminal care respectively. There is very close interaction between neurology and palliative care throughout the duration of rapidly progressive conditions, with a relatively smaller role for rehabilitation medicine physicians. However, rehabilitation plays a major role in providing long-term care and support, often over many years, in the more slowly progressive or stable conditions.
As the patient's condition becomes more advanced, rehabilitation and palliative care approaches often overlap – we have called this ‘neuropalliative rehabilitation'. (Reproduced from Ref 37.) provision for both palliative care and rehabilitation their independence and autonomy, with input from services for people with LTNCs, particular in the neurology as required. Towards the later stages, the community. There was also a marked lack of service roles of rehabilitation and palliative care become coordination. Perhaps not surprisingly, each specialty more closely intertwined in an approach which we reported greater ease of access to services within their have termed ‘neuropalliative rehabilitation'.
own field, which further emphasises the need towork closely together.
Figure 2 illustrates a proposed model for serviceinteraction. People with rapidly progressiveneurological conditions require closely coordinatedneurology and palliative care services throughout therelatively short span of their condition. However, forpeople with more slowly changing conditions, themajor role for neurology is in the early stages ofdiagnosis and treatment, and for palliative care in thelate stages. For many years in between, rehabilitationservices provide the mainstay of support tocoordinate services which help them to maximise Long-term neurological conditions Table 2. Key skills in neurological palliative care and rehabilitation.
Every physician should have an understanding of the general principles of management, and should also be
aware of when and where to refer if more specialist advice is needed in the areas shown below
Exposure to people with long- • Understanding disease progression and prognosis term neurological conditions • Ability to control key symptoms including: – pain in neurological conditions– breathlessness– nausea/vomiting– anxiety/depression– spasticity management– 24-hour postural support – bladder and bowels– seizure control • Basic understanding of common communication problems including dysphasia, dysarthria, cognitive speech disorders and the different approaches to their management.
• Ability to communicate with people who have cognitive/communication impairments – using assistive communication devices • Communicating with patient and family – breaking bad news– addressing end-of-life decisions and advance care planning which will include choice over place of care.
• Managing expectations • Ability to assess for mental capacity, and to assist people to make advance decisions and statements • Understanding of the Mental Capacity Act 2005 and ability to work alongside lasting power of attorney/court appointed deputy or independent mental capacity advocates* Additional skills for physicians specialising in neurological palliative care and rehabilitation
Specialist interventions • Local and intrathecal interventions for spasticity (eg injection of botulinum toxin/phenol and use of baclofen pumps) • Specialist procedures for pain control • Management of confusion/unwanted behaviours – management under sections of the Mental Health Act 1983 Specialist equipment • Wheelchair seating systems• Environmental control systems• Specialist communication aids Counselling and psychological • Dealing with loss and fear of loss • Spiritual support• Bereavement – past and future • Understanding the social care system and benefits• Vocational support Additional sources of help and • Understanding the interaction between health, social services and voluntary • Negotiating skills in obtaining services * Terms such as ‘living will' or ‘advance directives' are often used to describe documents in which people write down their wishes andchoices about their future care. However, these terms do not have any legal standing. Under the Mental Capacity Act 2005 (MCA) it is nowpossible for people to make legally binding advance decisions to refuse treatment as well as non-binding advance statements about theirwishes and priorities for care, to take effect should they lose the capacity to make decisions for themselves. For clarity, it is important to usethis language wherever possible. Any statements about future care and treatment must be considered in the light of the MCA. For further Long-term neurological conditions Recommendation General service coordination
Neurology, rehabilitation and palliative care services should develop closely coordinated working links to support people with long-term neurological conditions (LTNCs) from diagnosis to death, including: • proper flow of communication and information for patients and their families • a designated point of contact for each stage in the pathway • a needs assessment identifying the patient's individual problems.
1 A person who is suspected of having an LTNC should be referred promptly to a specialist neurological service for investigation and diagnosis.
2 A person who is confirmed to have an LTNC should have: • ongoing access to specialist neurological services for disease-modifying treatment, if appropriate • ongoing support and advice with regard to management of their condition and its sequelae• support from specialist neurological nurses for practical advice on living with their condition.
1 A person with an LTNC should be referred to a specialist neurological rehabilitation service if: • they develop significant disability or symptoms such as incontinence/spasticity management/ nutrition/pain/depression which fall within the remit of the rehabilitation physician and may require an interdisciplinary approach, and/or • their circumstances change in a way that affects their independence or participation in their current 2 A person with significant ongoing disability due to an LTNC should have timely and ongoing access to specialist neurological rehabilitation and support services which include:• initial needs assessment and provision of support according to the list in Table 1• ongoing integrated care planning – including an annual multi-agency needs assessment including health, social services, and voluntary sector input • coordinated service provision in accordance with changing need, including equipment, environmental adaptation, rehabilitation for vocation/leisure, psychosocial support.
Palliative care services
1 A person with an LTNC should be referred to specialist palliative care services if they have: • a limited lifespan – usually 6–12 months, and/or• distressing symptoms – especially pain, nausea and vomiting, breathlessness, which fall within the remit of the palliative physician, and/or • a need or desire for end-of-life planning, with or without competence issues.
2 A person who is dying from an LTNC should have timely and ongoing access to specialist palliative care services which include:• symptom control• planning and support to the end of their life• aftercare and bereavement support for their families.
* For explanation of grades of e Long-term neurological conditions Implications for implementation The literature review and survey undertaken to For key to bold type below underpin these guidelines highlighted gaps anddeficiencies in the services at every level.
Neuro numbers: a brief review of Rehabilitation and palliative care have long been the numbers of people in the UK with a neurological condition.
London: The Neurological Alliance, 2003. R2 Medium
‘Cinderella specialties' within the NHS and to a certain extent the need for investment is inevitable.
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R2 High Direct/E2
35 Ward CD, Phillips M, Smith A, Moran M. Multidisciplinary Long-term neurological conditions Appendix 1. Guideline development process Scope and purpose
Overall objective of To improve the quality of care offered to people in the later stages of a long-term neurological condition (LTNC) through better collaboration between neurology, rehabilitation and palliative care teams.
The patient group covered Adults with long-term neurological conditions including: • sudden onset conditions (eg acquired brain injury of any cause (including stroke), spinal cord injury) • intermittent conditions (eg epilepsy) • progressive conditions (eg multiple sclerosis, motor neurone disease, Parkinson's disease and other neurodegenerative disorders) • stable conditions (eg polio or cerebral palsy).
Doctors and health professionals involved in the long-term support, rehabilitation and palliative care of people in the later stages of LTNC. Providers and purchaser of neurology, rehabilitation and palliative care services.
Clinical areas covered • How should specialist neurology, rehabilitation and palliative care teams integrate in the management of this group of patients? • How should non-specialist clinicians care for people with LTNCs when admitted to hospital for other conditions, and when should they call for specialist help? • What training should be provided within each field to ensure that future generations have the necessary understanding and competencies to provide best quality of care? A multidisciplinary working party convened through the National Council for Palliative Care Development Group • physicians, nurses and other allied health and social services professionals practising in neurology, rehabilitation and palliative care • representatives of patients and user groups including Sue Ryder Care, and the Motor Neurone The British Society of Rehabilitation Medicine (BSRM), the Royal College of Physicians and the National Council for Palliative Care all contributed funding or resources towards the guideline development. Conflicts of interest All GDG members were asked to declare any personal or financial conflicts of interest, but none were identified.
Rigour of development
Evidence gathering Evidence for these guidelines was based on systematic reviews of the literature undertaken in preparation for the National Sevice Framework (NSF) for Long-Term Conditions15 in 2005 and updated in 2006/7. Reviews covered the major databases (Medline, Embase, Cochrane etc). A survey of UK physicians in the three fields was undertaken37 to ascertain current working patterns and perspectives, and two open conferences have been held with a wide selection of stakeholders, both professional and users, to explore the proposed pathways for more integrated care. Full details of this process can be found in Focus on Neurology available a The evidence was evaluated by members of the GDG. Links between evidence The system used to grade the evidence and guidance recommendations is that published for and recommendations Piloting and peer review Not yet piloted. Tools for application Tools for implementation ar The guideline will be reviewed in 3 years (2011).
Long-term neurological conditions Appendix 2. Methods used to evaluate the evidence The typology and grading system developed for the National Service Framework for Long-term (Neurological) Conditions were used to evaluate the evidence.15 The typology is designed to place value on the experience of users and professionals as well as research, and also to value high-quality research regardless of the design.
Each piece of evidence is reviewed and given an ‘E' and/or an ‘R' rating: E = Expert evidence. This is evidence expressed through consultation or consensus processes rather than formal research
designs. It could be professional opinion, or that of users and/or carers or other stakeholders.
R = Research evidence. This is evidence gathered through formal research processes. Each piece of research-based evidence is
awarded a rating based on three categorisations:
research design – category of research design
research quality – rated high, medium or low
applicability of research – whether the study population is within the context of long-term
neurological conditions (‘direct') or in other populations (‘indirect').
Research design is classified according to the following categories:
Primary research-based evidence
P1 Primary research using quantitative approaches
P2 Primary research using qualitative approaches
P3 Primary research using mixed methods (qualitative and quantitative)
Secondary research-based evidence
Meta-analysis of existing data analysis Secondary analysis of existing data Review-based evidence
R1 Systematic reviews of existing research
R2 Descriptive or summary reviews of existing research
Research quality is assessed using five questions with a possible score on each question
of 0, 1 or 2, giving a maximum score of 10: Each quality item is scored as follows: Yes = 2; In part = 1; No = 0 Are the research question/aims and design clearly stated? Is the research design appropriate for the aims and objectives of the research? Are the methods clearly described? Is the data adequate to support the authors' interpretations/ conclusions? Are the results generalisable? Long-term neurological conditions High-quality research studies are those that score at least 7/10.
Medium-quality studies score 4–6/10.
Poor-quality studies score 3/10 or less.
Applicability of research is classified as shown below:
Studies that focus on people with long-term neurological conditions Indirect
Extrapolated evidence from populations with other conditions A well-conducted qualitative study, scoring 8/10 and demonstrating the benefits of a given
intervention in people with multiple sclerosis would be classified as: P2 High Direct.
A post-hoc analysis scoring 5/10 on quality assessment, demonstrating the benefits of
palliative care in cancer would be classified as: R2 Medium Indirect.
Some sources, such as a pre-existing set of guidelines/standards, reviews or book chapters, may
include both research evidence and expert opinion and so might be graded as: R1 High Direct/E2.
Grade of research evidence Each individual recommendation or statement is then given an overall evidence rating of A, B or C
based on the quality of all the research evidence supporting it and how much of it was directly
relevant. The overall grade of evidence is rated as shown below.
Grade of evidence Research Grade A (RA)
* More than one study of high-quality score (7/10) and* At least one of these has direct applicability Research Grade B (RB)
* One high-quality study or* More than one medium-quality study (4–6/10) and* At least one of these has direct applicability * More than one study of high-quality score (7/10) of indirect applicability Research Grade C (RC)
* One medium-quality study (4–6/10) or* Lower-quality (2–3/10) studies or* Indirect studies only Long-term neurological conditions Appendix 3. Tools for implementation A. Checklist to use on admission of patients with a long-term neurological condition Patients with LTNCs may be admitted to hospital for a variety of reasons including: exacerbation or progression of disease complication of disease (eg infection/pressure sores) an unrelated problem.
These admissions are often poorly managed in general wards where the teams do not always have the experience and resourcesto manage people with complex neurological problems. Table A1. Checklist for the management of patients with an LTNC when admitted to a general hospital ward.
Patients with LTNCs and their families or carers are often expert at managing the disease and medications.
They live with the consequences of the management decisions that are made on their behalf, so always consider
and respect their advice and wishes.
Prior to admission consider the following:
Is the admission necessary? – Is it appropriate, given the level of disability/prognosis?– Can the patient be managed as a day case or in the community? Plan the admission/coordinate with the team caring for the patient. Inform the neurological/rehabilitation/palliative care team caring for the patient.
– Obtain old notes.
Check medication and continue unless contraindicated (especially anti-epileptics and anti-Parkinsonian medication).
Check that the patient has been admitted with their equipment (hearing aids, communication aids, adaptedwheelchair) and that staff are capable of using it.
Check the patient's competence to make decisions regarding their care. Is there an advance directive (AD)? Review in hospital:
Posture and spasticity management, especially with pain, infection, fractures. Neuro-physiotherapy review is oftenhelpful. Pressure sores and management strategy to prevent these.
Anticoagulation prophylaxis to prevent deep vein thrombosis.
Bladder: is the patient continent? In retention? Bowels: is the patient incontinent/constipated?– especially with altered diet/opiates.
Swallow: is this safe? Is the patient aspirating? Table A1 continued overleaf Long-term neurological conditions Table A1. Checklist for the management of patients with an LTNC when admitted to a general hospital ward
Nutrition: is this adequate? Is the patient able to feed themselves? Respiratory capacity: – check and monitor vital capacity if compromised.
Cognition:– beware of an acute deterioration with illness, medication.
Depression:– triggered by hospitalisation/change in condition/pain. Pain: is important. In addition to the acute problem, pain may be due to a combination of:– neuropathic pain (which may respond to anti-epileptics and tricyclics)– spasticity– musculoskeletal pain – pay careful attention to positioning. If considering a procedure, consider once again:
Is this appropriate given the patient's underlying neurological condition and prognosis? Does the patient have the capacity to consent – is there an AD? Respiratory function – is there need for anaesthetic advice? Prior to discharge:
Consider whether the arrangements at home are appropriate – did they trigger the admission? Assess how the patient's discharge will affect the family and their ability to cope.
Review and re-start the care package – revise if necessary. Inform the team that usually cares for the patient in hospital, in the community, or at home. Arrange follow-up if necessary – try to coordinate this if the patient has difficulty accessing hospital. Long-term neurological conditions B. Key areas of symptom management Take a bowel history.
Is there a complaint of constipation or the absence of any bowel action for over 3 days?
Ensure adequate fluid and nutritional Examine per rectum – exclude local pain/fissure etc.
intake, with sufficient, but not excess fibre Examine whether the colon is loaded with faeces• plain abdominal X-ray if necessary Find out what time patient normally opens their bowels (eg after breakfast, taking account of the gastro-colic reflex). If colon is loaded: Toilet at that time: • phosphate enemas • preferably on toilet, rather than bed-pan or commode • manual evacuation if necessary • ensure comfort and privacy • Movicol (up to 8 sachets) (may need Continue their normal bowel regimen, if they have one addition of softening laxative to avoid colic) • persist until clear – may take 2–3 weeks If nothing happens for 3 days running and rectum is empty: If no response, refer to gastroenterology to • give senna or other stimulant laxative on third night exclude other pathology • add softening laxative if colic occurs If nothing happens, make it happen: If not loaded, or once cleared: • digital stimulation for insensate bowel • Movicol (4 sachets/day) or • glycerine suppository or microlax enema • stimulant/softening laxative combination • ensure stimulant/softening laxative combination and titrate • titrate dose against response to ensure a bowel action dose against response to ensure a bowel action every 3 days at least every 3 days Fig. A1. Bowel management in patients with long-term neurological conditions.
Movicol = polyethlene glycol 3350.
Long-term neurological conditions Intermittent catheterisation
At least once daily
Spontaneous voiding but incontinent:
Exclude obstruction, Establish no remediable cause before (>50–100 ml) constipation, drugs etc.
settling for catheterisation (If not possible, may requirelong-term catheterisation) Increased: >2000 ml
Consider: diabetes, diabetes
insipidus, chronic renal
failure, diuretics, obsessive
drinking
Total volume
charts x 3
Decreased: <2000 ml
• incomplete chart
• insufficient fluid intake
Encourage more frequent emptying,
if necessary with intermittent catheterisation as required to keep voided volumes <500 ml If no dyssynergia but detrusor over- Consider sphincter dyssynergia or other causes of outflow obstruction,
activity consider: eg stones, urethral stricture, or prostatic hypertrophy • anticholinergics (eg oxybutinin,
• U/S upper tracts to exclude dilatation • urodynamics to assess pressures • botulinum toxin to detrusor
• urological advice Failure to establish urinary continence
Consider botulinum toxin
eg upper limb dysfunction as required to keep volumes <500 ml To urethral sphincter With anticholinergics, if associated detrusor overactivity • supra-pubic preferable Fig. A2. Managing urinary incontinence in patients with long-term neurological conditions.
U/S = ultrasound; UTI = urinary tract infection.
Long-term neurological conditions Non-neuropathic pain – musculoskeletal
Assess to identify:
• positioning and support • cause of pain • non-steroidal anti-inflammatory drugs • amitriptyline – 10 mg nocte (NSAIDs) unless contraindicated • pain severity increasing to tds or 50–75 mg nocte (eg GI history, renal failure) • aggravating factors – eg slow-release diclofenac/ibuprofen • mood disturbance • anti-epileptic • simple analgesia if NSAIDs not suitable eg carbamazepine, gabapentin or pregabalin – paracetamol plus preparations, – build up dose gradually – watch for side effects Pain due to spasticity
Opioids – only to be used if:
Spasticity management programme: • clear diagnosis • positioning, stretching, splinting • good understanding of prognosis • if movement related: • exclude aggravating factors, • no underlying psychological or addictive – assess need for antispastics eg infection, tight clothing etc – NSAID or paracetamol • avoid sudden movements • closely monitored – fentanyl lozenge/spray – watch for pain response and dose • if neuropathic • generalised spasticity – oral agents: – baclofen or tizanidine • seek advice from palliative medicine • if end of opioid dose failure • regional or focal spasticity – consider: physician or pain specialist – titrate regular opioid – botulinum toxin injection or nerve • in association with other medication, as a – intrathecal baclofen or phenol • seek advice from rehabilitation • tramadol• morphine• fentanyl patch (in some form) Pain should be re-assessed regularly using suitable self-report tools – if necessary those adapted forpeople with communication and cognitive difficulties (eg the Scale of Pain Intensity)1 or evaluationsof pain-related behaviour (eg the PAINAD tool).2 Fig. A3. Managing pain in patients with long-term neurological conditions.
1Jackson D, Horn S, Kersten P, Turner-Stokes L. Development of a pictorial scale of pain intensity for patients with communicationimpairments: initial validation in a general population. Clin Med 2006;6(6):580–5.
2Ward V, Hurley AD, Volicer L. Development and psychometric evaluation of the pain assessment in advanced dementia (PAINAD) scale.
J Am Med Dir Assoc 2003;4:9–15.
Long-term neurological conditions Assess to identify:
• cause of nausea – abdominal X-ray if necessary • aggravating factors • raised intracranial pressure
Delayed gastric emptying:
Large volume vomiting with undigested • haloperidol – 1.5 mg nocte • adjust meal pattern – increase to bd – eat little and often – stop if reach 5 mg bd and nausea • metoclopramide start 10 mg tds – titrate up to 80 mg per day (Avoid in Parkinson's due to • domperidone 10–20 mg dopaminergic effect) 3–4 times daily • cyclizine 50 mg up to tds (generally less effective, but less dopaminergic effect) If first-line agents fail, consider:• levomepromazine 6–25 mg daily If PEG-fed
• ondansetron (short term) Slow feeds – night-time continuous feeding – orally 8 mg bd or If vomiting persists: – per rectum 16 mg daily • check endoscopy to exclude pyloric obstruction by balloon/toggle • consider per jejunostomy feeding tube Fig. A4. Managing nausea and vomiting in patients with long-term neurological conditions.
CT = computed tomography; PEG = percutaneous endoscopic gastronomy.
Long-term neurological conditions Assess to identify:
Acute SOB with potential for reversal eg:
• cause of breathlessness • lung pathology – pulmonary embolus, pneumonia • acute respiratory muscular paralysis • exclude anxiety responding to simple reassurance Manage aggressively
• with ventilation if necessary
Irreversible breathlessness in known diagnosis eg:
• pneumonia, eg in chronic aspiration
• respiratory muscle failure, eg in motor neurone disease,
Benzodiazepine – especially if anxious
or
Low-dose opioid
• start oromorph 2.5 mg 4-hourly and work up
Relief of respiratory distress (sustained release preparations less effective in this context) • breathing exercises• relaxation• consider assessment for non-invasive ventilation Anticholinergic – if ruled out infection or cardiac
failure
• hyoscine patch or sublingual
Manage excess secretions • glycopyyrolate sc or oral• amitriptyline elixir – 5–10 mg tds• atropine – oral (use eye-drop solution) Terminal phase palliation
• combination: opioid + midazolam + glycopyrrolate
Fig. A5. Managing shortness of breath (SOB) in patients with long-term neurological conditions.
Long-term neurological conditions

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