× NICE uses cookies to make the site better.  Learn more
A-Z
Topics
Latest


Motor neurone disease

About

What is covered

This interactive flowchart covers assessing and managing motor neurone disease in adults.

Updates

Updates to this interactive flowchart

26 September 2017 Intramuscular diaphragm stimulation for ventilator-dependent chronic respiratory failure caused by motor neurone disease (NICE interventional procedures guidance 593) added to starting and managing non-invasive ventilation.
26 July 2016 Motor neurone disease (NICE quality standard 126) added.
23 February 2016 Major update on publication of NICE guideline NG42 on motor neurone disease: assessment and management.

Person-centred care

People have the right to be involved in discussions and make informed decisions about their care, as described in your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

Your responsibility

Guidelines

The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals and practitioners are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or the people using their service. It is not mandatory to apply the recommendations, and the guideline does not override the responsibility to make decisions appropriate to the circumstances of the individual, in consultation with them and their families and carers or guardian.
Local commissioners and providers of healthcare have a responsibility to enable the guideline to be applied when individual professionals and people using services wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with complying with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Technology appraisals

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, health professionals are expected to take these recommendations fully into account, alongside the individual needs, preferences and values of their patients. The application of the recommendations in this interactive flowchart is at the discretion of health professionals and their individual patients and do not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or their carer or guardian.
Commissioners and/or providers have a responsibility to provide the funding required to enable the recommendations to be applied when individual health professionals and their patients wish to use it, in accordance with the NHS Constitution. They should do so in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Medical technologies guidance, diagnostics guidance and interventional procedures guidance

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, healthcare professionals are expected to take these recommendations fully into account. However, the interactive flowchart does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer.
Commissioners and/or providers have a responsibility to implement the recommendations, in their local context, in light of their duties to have due regard to the need to eliminate unlawful discrimination, advance equality of opportunity, and foster good relations. Nothing in this interactive flowchart should be interpreted in a way that would be inconsistent with compliance with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Short Text

Everything NICE has said on assessing and managing motor neurone disease in adults in an interactive flowchart

What is covered

This interactive flowchart covers assessing and managing motor neurone disease in adults.

Updates

Updates to this interactive flowchart

26 September 2017 Intramuscular diaphragm stimulation for ventilator-dependent chronic respiratory failure caused by motor neurone disease (NICE interventional procedures guidance 593) added to starting and managing non-invasive ventilation.
26 July 2016 Motor neurone disease (NICE quality standard 126) added.
23 February 2016 Major update on publication of NICE guideline NG42 on motor neurone disease: assessment and management.

Sources

NICE guidance and other sources used to create this interactive flowchart.
Motor neurone disease (2016) NICE quality standard 126

Quality standards

Motor neurone disease

These quality statements are taken from the motor neurone disease quality standard. The quality standard defines clinical best practice for motor neurone disease and should be read in full.

Quality statements

Information and support at diagnosis

This quality statement is taken from the motor neurone disease quality standard. The quality standard defines clinical best practice in motor neurone disease and should be read in full.

Quality statement

Adults diagnosed with motor neurone disease (MND) are given information about the diagnosis, prognosis and management of MND by a consultant neurologist with expertise in treating people with MND.

Rationale

Receiving a diagnosis of MND is distressing for a person, and they (and their family members or carers) are likely to have a variety of questions and concerns. MND is rare and most healthcare professionals will have little experience and knowledge of it. A consultant neurologist with expertise in treating people with MND can provide accurate and up-to-date information on the diagnosis, prognosis and management of MND with compassion and understanding.

Quality measures

Structure
Evidence of local arrangements to ensure that people diagnosed with MND are given information about the diagnosis, prognosis and management of MND by a consultant neurologist with expertise in MND.
Data source: Local data collection.
Process
Of adults with MND, the number who are given information at diagnosis about the diagnosis, prognosis and management of MND by a consultant neurologist with expertise in treating people with MND.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
Outcome
a) Adults diagnosed with MND understand how the condition is likely to affect them.
Data source: Local data collection.
b) Adults diagnosed with MND understand the type of care and support they can receive.
Data source: Local data collection.
c) Adults diagnosed with MND, and their families or carers, feel able to cope.
Data source: Local data collection. National data on the proportion of people who felt supported through the consultation when receiving a diagnosis are available from the Improving MND Care survey.

What the quality statement means for service providers, healthcare professionals and commissioners

Service providers (such as neurology services) ensure that adults who are diagnosed with MND are given information about the diagnosis, prognosis and management by a consultant neurologist with up-to-date knowledge and experience of treating people with MND.
Healthcare professionals (such as consultant neurologists with expertise in treating people with MND) ensure that they give adults who are diagnosed with MND information about the diagnosis, prognosis and management, and deliver the information with compassion and understanding. They give information according to the person’s needs.
Commissioners (such as clinical commissioning groups and NHS England) ensure that commissioning responsibilities for people with MND are clear, and that they commission services in which adults who are diagnosed with MND are given information about the diagnosis, prognosis and management by a neurologist with up-to-date knowledge and experience of treating people with MND.

What the quality statement means for patients, service users and carers

Adults who are diagnosed with MND receive information about the diagnosis, how MND is likely to progress and what will happen next, from a consultant neurologist with expertise in treating people with MND. A neurologist is a hospital doctor who specialises in disorders of the nervous system, including the brain, spinal cord and nerves.

Source guidance

Definitions of terms used in this quality statement

Consultant neurologist with expertise in treating people with MND
A consultant neurologist with up-to-date knowledge and experience of treating people with MND. They should have knowledge and expertise in:
  • symptoms
  • types and possible causes
  • treatment options
  • how MND may progress (including cognitive and behavioural changes) and how progression may affect the treatments offered
  • crisis prevention (for example, if there is an acute hospital admission or a breakdown in care arrangements)
  • opportunities for people with MND to be involved in research
  • likely needs and concerns of people with MND and their family members and/or carers (as appropriate)
  • advance care planning.
[Adapted from Motor neurone disease (NICE guideline NG42), recommendation 1.2.1]

Equality and diversity considerations

People with MND can experience cognitive problems. Cognitive change can occur early in the disease progression and may be present at diagnosis. All information should therefore be accessible, as much as possible, to people with cognitive problems. People receiving information about MND should have access to an interpreter or advocate if needed.
In some geographical areas, consultant neurologists without MND expertise may be involved in investigations and testing for suspected MND. Arrangements should be made for information on a diagnosis of MND, prognosis and management to be given by a consultant neurologist with expertise in treating people with MND.

Respiratory assessment and non-invasive ventilation

This quality statement is taken from the motor neurone disease quality standard. The quality standard defines clinical best practice in motor neurone disease and should be read in full.

Quality statement

Adults with motor neurone disease (MND) who have respiratory impairment are offered non-invasive ventilation (NIV) based on regular assessments of respiratory function and symptoms.

Rationale

Progressive respiratory muscle weakness resulting in respiratory impairment is a major feature of MND. The onset of respiratory failure (when a person is unable to breathe adequately to maintain normal oxygen levels and clear waste gases without support) can be sudden and unexpected, and may lead to emergency ventilation or death. Regular monitoring of respiratory function by MND multidisciplinary teams allows respiratory muscle weakness to be identified before symptoms of respiratory impairment appear. It also means that strategies such as NIV can be offered and started before an emergency situation occurs. NIV can improve symptoms and signs related to respiratory impairment, and improve quality of life and survival. Decisions to offer NIV are made between the MND multidisciplinary team, the respiratory ventilation service and the person with MND.

Quality measures

Structure
a) Evidence that MND multidisciplinary teams include respiratory physiologists or healthcare professionals who can assess respiratory function.
Data source: Local data collection.
b) Evidence of local arrangements and written protocols to ensure that adults with MND have their respiratory function and symptoms assessed at diagnosis and then every 2–3 months.
Data source: Local data collection.
c) Evidence of established relationships and decision making arrangements between MND multidisciplinary teams and respiratory ventilation services.
Data source: Local data collection.
d) Evidence of local arrangements to provide NIV for adults with MND who have respiratory impairment.
Data source: Local data collection.
Process
a) Of adults diagnosed with MND, the number of adults who had their respiratory function and symptoms assessed as part of the initial assessment to diagnose MND.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
b) Of adults with MND, the number who have had their respiratory function and symptoms assessed within the past 3 months.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers. At national level, self-reported information on the frequency of breathing monitoring and assessments is presented in the Improving MND Care survey.
c) Of adults with MND who have respiratory impairment, the number who are established on NIV.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
Outcome
a) Survival of adults with MND from diagnosis.
Data source: Local data collection.
b) Adults with MND able to maintain activities of daily living.
Data source: Local data collection.

What the quality statement means for service providers, healthcare professionals and commissioners

Service providers (such as MND multidisciplinary teams and respiratory ventilation services) ensure that they assess people’s respiratory function and symptoms as part of the initial assessment to diagnose MND, or soon after diagnosis, and then every 2–3 months. Providers ensure that there are local arrangements for timely provision of NIV and agreed local protocols for referral to this service.
Healthcare professionals (members of the MND multidisciplinary team, such as respiratory physiologists or healthcare professionals who can assess respiratory function) ensure that they assess respiratory function and symptoms as part of the initial assessment to diagnose MND, or soon after diagnosis, and then every 2–3 months. Members of the MND multidisciplinary team should make decisions to offer NIV in conjunction with the respiratory ventilation service and the person with MND.
Commissioners (such as clinical commissioning groups and NHS England) ensure that the services they commission have MND multidisciplinary teams that include healthcare professionals with appropriate competencies to perform respiratory function tests and monitor signs and symptoms of potential respiratory impairment. Decisions to offer NIV should be taken by MND multidisciplinary teams in conjunction with respiratory ventilation services, and based on the respiratory assessments.

What the quality statement means for patients, service users and carers

Adults with MND are regularly checked to see if they are having any problems with their breathing. These checks should happen when they are diagnosed with MND (or shortly afterwards) and then in regular appointments with a team of specialists called an MND multidisciplinary team (usually every 2–3 months). Adults with MND who are having difficulty breathing are offered non-invasive ventilation using a portable ventilator – a machine that supports the person’s breathing. Decisions about non-invasive ventilation are shared between the multidisciplinary team, the respiratory ventilation service and the person with MND.

Source guidance

  • Motor neurone disease (2016) NICE guideline NG42, recommendations 1.5.3, 1.12.1, 1.12.2, 1.14.7, and 1.14.8–1.14.15

Definitions of terms used in this quality statement

Non-invasive ventilation
Non-invasive ventilation refers to methods of providing ventilatory support to a patient without placing an artificial airway in the main windpipe (trachea). This is usually achieved by fitting a mask covering the nose, or mouth and nose, or using nasal tubes or a mouthpiece, which is connected to a ventilator by tubing. The ventilator detects when the patient tries to take a breath in and delivers an extra flow of air to increase the volume of air inhaled.
[Motor neurone disease (NICE guideline NG42), full guideline glossary]
Regular assessments of respiratory function and symptoms
Respiratory function tests performed as part of the initial assessment to diagnose MND, or soon after diagnosis, by MND multidisciplinary team members such as respiratory physiologists or healthcare professionals who can assess respiratory function. Thereafter respiratory function tests are performed every 2–3 months, although occasionally tests may be performed more or less often depending on:
  • whether there are any symptoms and signs of respiratory impairment
  • the rate of progression of MND
  • the person’s preference and circumstances.
[Adapted from Motor neurone disease (NICE guideline NG42), recommendations 1.14.7, 1.14.8 and 1.14.10, and expert opinion]
Respiratory function tests to be undertaken at diagnosis and ongoing assessment are those detailed in motor neurone disease (NICE guideline NG42), section 1.14.
Symptoms and signs monitored in an assessment to detect potential respiratory impairment are set out in box 1 motor neurone disease (NICE guideline NG42), recommendation 1.14.7.
Assessment in the context of this statement also includes assessment of bulbar function, because this affects the type tests performed and the response to NIV.
[Motor neurone disease (NICE guideline NG42), recommendations 1.14.9, 1.14.18; expert opinion]
Assessment for potential respiratory impairment includes monitoring cough effectiveness (weak cough being a sign of potential respiratory impairment) potentially by measuring cough peak flow.
[Motor neurone disease (NICE guideline NG42), recommendation 1.14.7; expert opinion]

Provision of equipment and adaptions based on multidisciplinary team assessment

This quality statement is taken from the motor neurone disease quality standard. The quality standard defines clinical best practice in motor neurone disease and should be read in full.

Quality statement

Adults with motor neurone disease (MND) receive tailored equipment and adaptions without delay, based on regular multidisciplinary team assessments.

Rationale

People with MND can have multiple functional problems and may therefore have complex equipment needs that will change as MND progresses. Regular assessment by the MND multidisciplinary team can ensure the provision of equipment and adaptations is responsive to a person’s changing needs. Providing equipment and adaptions without delay maximises the impact on the person’s quality of life, allowing them to continue with usual activities and reduce the likelihood of harm from adverse events such as falls.

Quality measures

Structure
a) Evidence of local arrangements to ensure that adults with MND have regular assessments from MND multidisciplinary teams.
Data source: Local data collection.
b) Evidence of local arrangements to ensure that equipment is provided, or adapted, in a timely way to meet the individual needs identified for adults with MND.
Data source: Local data collection.
Process
a) Of adults with MND, the number who have had a comprehensive assessment by the MND multidisciplinary team in the past 3 months.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
b) The length of time in days between an equipment need or an adaption need being identified in a comprehensive multidisciplinary team assessment, and the equipment being provided or adaption made.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
Outcome
a) Adults with MND able to maintain activities of daily living.
Data source: Local data collection. National data on whether people with MND feel that the equipment or adaption they are using meets their needs is available from the Improving MND Care survey.
b) Adults with MND feel they are able to maintain their quality of life.
Data source: Local data collection.
c) Number of injuries to adults in the home associated with functions impaired by MND.
Data source: Local data collection.

What the quality statement means for service providers, healthcare professionals and social care practitioners, and commissioners

Service providers (such as MND multidisciplinary teams) ensure that they assess people’s needs, including mobility and daily living needs and abilities every 2–3 months, and that services are integrated between providers so that equipment and adaptions can be provided to meet people’s changing needs without delay.
Healthcare professionals and social care practitioners (such as members of MND multidisciplinary teams) ensure that they assess and anticipate changes in the person’s needs, including mobility and daily living needs and abilities, every 2–3 months, and provide, or refer to services that can provide, equipment and adaptions to meet the person’s individual needs without delay.
Commissioners (such as clinical commissioning groups and NHS England) ensure that they commission MND services in which MND multidisciplinary teams assess people’s needs, encompassing mobility and daily living needs and abilities, every 2–3 months, and provide tailored equipment and adaptions without delay.

What the quality statement means for patients, service users and carers

Adults with MND have regular checks to make sure that any equipment they have been given to help them manage their condition, such as a wheelchair, is still meeting their needs. These checks also help to anticipate any future changes in their needs, including whether any changes to existing equipment or new equipment will be needed. The checks should happen in appointments with a team of specialists called an MND multidisciplinary team (usually every 2–3 months).

Source guidance

Definitions of terms used in this quality statement

Tailored equipment and adaptions
Daily living aids, assistive technology and adaptions that can be integrated and adapted to meet the changing needs of the person with MND and their family or carers. Examples include wheelchairs, hoists, head supports, arm supports, riser–recliner chairs, drinking and eating aids, environmental controls, and home adaptions.
[Motor neurone disease (NICE guideline NG42), recommendations 1.9.5, 1.9.6 and 1.9.7]
Regular assessments
Assessments carried out by the multidisciplinary team every 2–3 months unless the person’s symptoms and needs require more or less frequent assessment. Assessments cover the areas set out in recommendation 1.5.3 in NICE’s guideline on motor neurone disease that include mobility and activities of daily living, communication, nutritional intake, swallowing, muscle problems, physical function, pain, respiratory function, cognition and behaviour, and social care needs.
Assessments include assessing and anticipating changes in the person’s daily living needs, taking into account:
  • activities of daily living, including personal care, dressing and bathing, housework, shopping, food preparation, eating and drinking, and ability to continue with current work and usual activities
  • mobility and avoiding falls and problems from loss of dexterity
  • the home environment and the need for adaptations
  • the need for assistive technology, such as environmental control systems
  • a person’s ability to use equipment.
[Adapted from Motor neurone disease (NICE guideline NG42), recommendations 1.5.2, 1.5.6, 1.9.1 and 1.9.6]
Multidisciplinary team
An MND multidisciplinary team that:
  • includes healthcare professionals and social care practitioners with expertise in MND, including staff who see people in their home
  • ensures effective communication and coordination between all healthcare professionals and social care practitioners involved in the person’s care (and their family members and/or carers, as appropriate)
  • provides coordinated care for people who cannot attend a clinic, according to the person’s needs.
The core multidisciplinary team should consist of healthcare professionals and other professionals with expertise in MND, and should include:
  • neurologist
  • specialist nurse
  • dietitian
  • physiotherapist
  • occupational therapist
  • respiratory physiologist or a healthcare professional who can assess respiratory function
  • speech and language therapist
  • a healthcare professional with expertise in palliative care (MND palliative care expertise may be provided by the neurologist or nurse in the multidisciplinary team, or by a specialist palliative care professional).
The multidisciplinary team should have established relationships with, and prompt access to:
  • clinical psychology and neuropsychology
  • social care
  • counselling
  • respiratory ventilation services
  • specialist palliative care
  • gastroenterology
  • orthotics
  • wheelchair services
  • assistive technology services
  • alternative and augmentative communication (AAC) services
  • community neurological care teams.
[Motor neurone disease (NICE guideline NG42), recommendations 1.5.2, 1.5.4 and 1.5.5]

Continuity of care

This quality statement is taken from the motor neurone disease quality standard. The quality standard defines clinical best practice in motor neurone disease and should be read in full.

Quality statement

Adults with motor neurone disease (MND) receive personal care and support from a consistent team of workers who are familiar with their needs.

Rationale

A consistent team of personal care workers can ensure familiarity with the person with MND and their specific needs. This avoids care needs having to be repeatedly explained to new workers. Such explanations can be difficult for people with MND, who may have reduced communication abilities and need a family member or carer to be present to help them. Ensuring continuity of care can avoid this additional burden or source of stress on the person with MND, and their family or carers.

Quality measures

Structure
Evidence of local arrangements to ensure that adults with MND receive personal care and support from a consistent team of care workers who are familiar with their needs.
Data source: Local data collection.
Process
a) Number of workers providing personal care and support for an adult with MND.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or local authority group level, data collection will involve small numbers.
b) Number of first visits of workers providing personal care and support for an adult with MND.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or local authority group level, data collection will involve small numbers.
Outcome
a) Adults with MND feel they receive the personal care and support they need.
Data source: Local data collection. National data on the proportion of people with MND who feel they get the social care services they need, are available from the Improving MND Care survey.
b) Adults with MND feel they are treated with dignity and respect.
Data source: Local data collection. National data on the proportion of people with MND who feel their independence/autonomy is respected when receiving social care services are available from the Improving MND Care survey.

What the quality statement means for service providers, social care practitioners and commissioners

Service providers (such as independent home care agencies, voluntary sector organisations and local authorities) ensure that personal care and support for adults with MND is provided by a consistent team of workers who are familiar with the person’s needs.
Social care practitioners (such as care workers and personal assistants) should ensure that they get to know the person they care for, what their needs are and provide care in the way that person wants.
Commissioners (such as local authorities) ensure that services they commission provide personal care and support for adults with MND from a consistent team of workers who are familiar with the person’s needs.

What the quality statement means for patients, service users and carers

Adults with MND have their personal care and support provided by the same workers, who are familiar with their needs.

Source guidance

Planning for end of life care

This quality statement is taken from the motor neurone disease quality standard. The quality standard defines clinical best practice in motor neurone disease and should be read in full.

Quality statement

Adults with motor neurone disease (MND) are given opportunities to discuss their preferences and concerns about end of life care at diagnosis and key stages of disease progression.

Rationale

MND can present in different ways and the prognosis may be variable, but the majority of people with MND die within 2–3 years of diagnosis. Discussions about end of life are difficult, but sensitive discussions can address concerns and result in increased support, control and choice for the person and their families and carers. Such discussions can take place at any time, but there are particular times when people should be given the chance to discuss the topics. These times include at diagnosis, if there is a significant change in respiratory function, or if interventions such as gastrostomy or non-invasive ventilation are needed. Early planning for care at the end of life also ensures that families and professionals can be made aware of a person’s wishes for end of life care before communication or cognitive changes make this difficult or impossible.

Quality measures

Structure
Evidence of local arrangements to ensure that adults with MND are given opportunities to discuss their preferences and concerns about end of life care at diagnosis and key stages of disease progression.
Data source: Local data collection.
Process
a) Of adults with MND, the number given opportunities to discuss their preferences and concerns about end of life care at diagnosis.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
b) Of adults with MND, the number given opportunities to discuss their preferences and concerns about end of life care at key stages of disease progression.
Data source: Local data collection based on extracts from or reviews of individual care records. At provider or clinical commissioning group level, data collection will involve small numbers.
Outcome
a) Adults with MND feel they have choice and control over their end of life care.
Data source: Local data collection.
b) Adults with MND receive end of life care informed by their wishes and preferences if they lose the ability to communicate.
Data source: Local data collection.

What the quality statement means for service providers, healthcare professionals and commissioners

Service providers (such as MND multidisciplinary teams) ensure that healthcare professionals are able to provide support and advice on advance care planning for end of life to people with MND and their family members and carers, and that they give people with MND the opportunity to discuss their preferences and concerns about end of life care at diagnosis and key stages of the disease progression.
Healthcare professionals (such as members of the MND multidisciplinary team) ensure that they give people with MND the opportunity to discuss their preferences and concerns about end of life care at diagnosis and key stages of the disease progression. Healthcare professionals should ensure that they are sensitive about the timing of discussions and take into account the person’s current communication ability, cognitive status and mental capacity.
Commissioners (such as clinical commissioning groups and NHS England) ensure that they commission services that have staff that are able to provide support and advice on advance care planning for end of life to people with MND and their family members and carers.

What the quality statement means for patients, service users and carers

Adults with MND are asked if they would like to talk about any concerns they have about dying and what they would prefer to happen (or not happen) at the end of their life.

Source guidance

Definitions of terms used in this quality statement

Discuss preferences and concerns about end of life care
Discussions should involve providing support and advice on advance care planning for end of life to the person with MND and their family members and carers (as appropriate). The discussion should include:
  • what could happen at end of life, for example how death may occur
  • providing anticipatory medicines in the home
  • advance care planning, including Advance Decisions to Refuse Treatment and Do Not Attempt Cardiopulmonary Resuscitation orders, and Lasting Power of Attorney
  • areas that people might wish to plan for, such as:
    • what they want to happen (for example preferred place of death)
    • what they do not want to happen (for example being admitted to hospital)
    • who will represent their decisions, if necessary
    • what should happen if they develop an intercurrent illness.
Sensitivity is needed regarding to the timing of discussions, and the person’s current communication ability, cognitive status and mental capacity should be taken into account.
[Adapted from Motor neurone disease (NICE guideline NG42), recommendations 1.7.1 and 1.7.3]
Key stages of the disease progression
Key stages of the disease progression include significant changes in the condition of the person with MND, and the trigger points set out in the guideline recommendation:
  • when there is a significant change in respiratory function
  • when interventions such as gastrostomy or non-invasive ventilation are needed.
[Adapted from Motor neurone disease (NICE guideline NG42), recommendation 1.7.1 and expert opinion]

Equality and diversity considerations

People with MND can experience cognitive problems. About 10% of people with MND show signs of frontotemporal dementia and a further 35% show signs of mild cognitive change. Cognitive or behavioural change does not mean that a person with MND will no longer be able to make decisions but rather that care should be provided in a way that accommodates the cognitive or behavioural changes. This could include allowing more time for decision making and an understanding of behaviour change. All information provided should be accessible, as far as possible, to people with cognitive problems. People receiving information about MND should have access to an interpreter or advocate if needed.

Effective interventions library

Effective interventions library

Successful effective interventions library details

Implementation

Information for the public

NICE has written information for the public on each of the following topics.

Pathway information

Person-centred care

People have the right to be involved in discussions and make informed decisions about their care, as described in your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

Your responsibility

Guidelines

The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals and practitioners are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or the people using their service. It is not mandatory to apply the recommendations, and the guideline does not override the responsibility to make decisions appropriate to the circumstances of the individual, in consultation with them and their families and carers or guardian.
Local commissioners and providers of healthcare have a responsibility to enable the guideline to be applied when individual professionals and people using services wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with complying with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Technology appraisals

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, health professionals are expected to take these recommendations fully into account, alongside the individual needs, preferences and values of their patients. The application of the recommendations in this interactive flowchart is at the discretion of health professionals and their individual patients and do not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or their carer or guardian.
Commissioners and/or providers have a responsibility to provide the funding required to enable the recommendations to be applied when individual health professionals and their patients wish to use it, in accordance with the NHS Constitution. They should do so in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Medical technologies guidance, diagnostics guidance and interventional procedures guidance

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, healthcare professionals are expected to take these recommendations fully into account. However, the interactive flowchart does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer.
Commissioners and/or providers have a responsibility to implement the recommendations, in their local context, in light of their duties to have due regard to the need to eliminate unlawful discrimination, advance equality of opportunity, and foster good relations. Nothing in this interactive flowchart should be interpreted in a way that would be inconsistent with compliance with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Supporting information

Symptoms
Signs
  • Breathlessness
  • Orthopnoea
  • Recurrent chest infections
  • Disturbed sleep
  • Non-refreshing sleep
  • Nightmares
  • Daytime sleepiness
  • Poor concentration and/or memory
  • Confusion
  • Hallucinations
  • Morning headaches
  • Fatigue
  • Poor appetite
  • Increased respiratory rate
  • Shallow breathing
  • Weak cough (could be assessed by measuring peak cough flow)
  • Weak sniff
  • Abdominal paradox (inward movement of the abdomen during inspiration)
  • Use of accessory muscles of respiration
  • Reduced chest expansion on maximal inspiration

Glossary

augmentative and alternative communication
advance decisions to refuse treatment
do not attempt resuscitation

Paths in this pathway

Pathway created: November 2013 Last updated: September 2017

© NICE 2017. All rights reserved. Subject to Notice of rights.

Recently viewed