Introduction

This quality standard covers the assessment and management of motor neurone disease. For more information see the motor neurone disease topic overview.

Why this quality standard is needed

Motor neurone disease (MND) is a neurodegenerative condition affecting the brain and spinal cord. MND is characterised by the degeneration of primarily motor neurones, leading to muscle weakness.

Presentation of the condition varies. It can be as muscle weakness, wasting, cramps and stiffness of arms or legs; problems with speech or swallowing or, more rarely, with breathing. As the disease progresses, the pattern of symptoms and signs becomes similar, with increasing muscle weakness in the person's arms and legs, problems swallowing and communicating, and weakness of the muscles used for breathing, which ultimately leads to death. Most people die within 2–3 years of developing symptoms, but 25% are alive at 5 years and 5–10% at 10 years. The most common type of MND is amyotrophic lateral sclerosis (ALS). There are rarer forms, such as progressive muscular atrophy or primary lateral sclerosis, which may have a slower rate of progression.

Every person with MND has an individual progression of the disease. About 10–15% of people with MND will show signs of frontotemporal dementia, which causes cognitive dysfunction and issues in decision‑making. A further 35% of people with MND show signs of mild cognitive change, which may affect their ability to make decisions and plan ahead.

MND can affect adults of any age, but mainly affects people aged 55 to 79. There are approximately 4,000 people with MND in England and Wales at any one time. The cause is unknown. About 5–10% of people with MND have a family history of the disease, and several abnormal genes have been identified.

There is no cure for MND. Therefore, care focuses on maintaining functional ability and enabling people with MND and their family members to live as full a life as possible.

The quality standard is expected to contribute to improvements in the following outcomes:

  • quality of life

  • functional ability

  • patient-reported outcome: symptoms

  • patient- and carer-reported outcome: satisfaction with care and support provided

  • survival from onset of symptoms.

How this quality standard supports delivery of outcome frameworks

NICE quality standards are a concise set of prioritised statements designed to drive measurable improvements in the 3 dimensions of quality – safety, experience and effectiveness of care – for a particular area of health or care. They are derived from high‑quality guidance, such as that from NICE or other sources accredited by NICE. This quality standard, in conjunction with the guidance on which it is based, should contribute to the improvements outlined in the following 3 outcomes frameworks published by the Department of Health:

Tables 1–3 show the outcomes, overarching indicators and improvement areas from the frameworks that the quality standard could contribute to achieving.

Table 1 The Adult Social Care Outcomes Framework 2015–16

Domain

Overarching and outcome measures

1 Enhancing quality of life for people with care and support needs

Overarching measure

1A Social care‑related quality of life**

Outcome measures

People manage their own support as much as they wish, so they are in control of what, how and when support is delivered to match their needs

1B Proportion of people who use services who have control over their daily life

Carers can balance their caring roles and maintain their desired quality of life

1D Carer‑reported quality of life**

3 Ensuring that people have a positive experience of care and support

Overarching measure

People who use social care and their carers are satisfied with their experience of care and support services

3A Overall satisfaction of people who use services with their care and support

3B Overall satisfaction of carers with social services

Placeholder 3E The effectiveness of integrated care

Outcome measures

Carers feel that they are respected as equal partners throughout the care process

3C The proportion of carers who report that they have been included or consulted in discussions about the person they care for

People know what choices are available to them locally, what they are entitled to, and who to contact when they need help

3D The proportion of people who use services and carers who find it easy to find information about support

People, including those involved in making decisions on social care, respect the dignity of the individual and ensure support is sensitive to the circumstances of each individual

This information can be taken from the Adult Social Care Survey and used for analysis at the local level

Alignment with NHS Outcomes Framework and/or Public Health Outcomes Framework

* Indicator is shared

** Indicator is complementary

Indicators in italics in development

Table 2 NHS Outcomes Framework 2016–17

Domain

Overarching indicators and improvement areas

2 Enhancing quality of life for people with long‑term conditions

Overarching indicator

2 Health‑related quality of life for people with long‑term conditions**

Improvement areas

Ensuring people feel supported to manage their condition

2.1 Proportion of people feeling supported to manage their condition

Improving functional ability in people with longterm conditions

Enhancing quality of life for carers

2.4 Health‑related quality of life for carers**

Enhancing quality of life for people with dementia

2.6 i Estimated diagnosis rate for people with dementia*

ii A measure of the effectiveness of postdiagnosis care in sustaining independence and improving quality of life*,**

4 Ensuring that people have a positive experience of care

Overarching indicators

4b Patient experience of hospital care

4c Friends and family test

4d Patient experience characterised as poor or worse

i Primary care

ii Hospital care

Improvement areas

Improving people's experience of outpatient care

4.1 Patient experience of outpatient services

Improving the experience of care for people at the end of their lives

4.6 Bereaved carers' views on the quality of care in the last 3 months of life

Improving people's experience of integrated care

4.9 People's experience of integrated care**

Alignment with Adult Social Care Outcomes Framework and/or Public Health Outcomes Framework

* Indicator is shared

** Indicator is complementary

Indicators in italics in development

Table 3 Public Health Outcomes Framework 2016–19

Domain

Objectives and indicators

4 Healthcare public health and preventing premature mortality

Objective

Reduced numbers of people living with preventable ill health and people dying prematurely, whilst reducing the gap between communities

Indicators

4.13 Health‑related quality of life for older people

Alignment with Adult Social Care Outcomes Framework and/or NHS Outcomes Framework

* Indicator is shared

** Indicator is complementary

Indicators in italics in development

Safety and people's experience of care

Ensuring that care is safe and that people have a positive experience of care is vital in a high‑quality service. It is important to consider these factors when planning and delivering services relevant to MND.

NICE has developed guidance and an associated quality standard on patient experience in adult NHS services (see the NICE pathway on patient experience in adult NHS services), which should be considered alongside this quality standard. They specify that people receiving care should be treated with dignity, have opportunities to discuss their preferences, and be supported to understand their options and make fully informed decisions. They also cover the provision of information to people using services. Quality statements on these aspects of patient experience are not usually included in topic‑specific quality standards. However, recommendations in the development sources for quality standards that affect people's experience of using services and are specific to the topic are considered during quality statement development.

Coordinated services

A person‑centred, integrated approach to providing services is fundamental to delivering high‑quality care to people with MND. Services should be commissioned from, and coordinated across, all relevant agencies encompassing the whole MND care pathway.

The Health and Social Care Act 2012 sets out a clear expectation that the care system should consider NICE quality standards in planning and delivering services, as part of a general duty to secure continuous improvement in quality. Commissioners and providers of health and social care should refer to the library of NICE quality standards when designing high‑quality services. Other quality standards that should also be considered when choosing, commissioning or providing a high‑quality MND service are listed in related NICE quality standards.

Training and competencies

The quality standard should be read in the context of national and local guidelines on training and competencies. All health, public health and social care practitioners involved in assessing, caring for and treating people with MND should have sufficient and appropriate training and competencies to deliver the actions and interventions described in the quality standard. Quality statements on staff training and competency are not usually included in quality standards. However, recommendations in the development source on specific types of training for the topic that exceed standard professional training are considered during quality statement development.

Role of families and carers

Quality standards recognise the important role families and carers have in supporting people with MND. If appropriate, health and social care practitioners should ensure that family members and carers are involved in the decision‑making process about investigations, treatment and care.