NICE has published its guideline on the assessment and management of motor neurone disease (MND). The guideline calls for a range of health and social care professionals to be involved at every stage in the care of people with the condition so that decisions about the person's care can be informed by and tailored to their individual needs and circumstances.

MND is a group of rare (about 1100 people will develop MND in the UK each year and around 5000 adults are currently living with the disease), incurable, progressive, fatal neurodegenerative diseases that attack the motor neurones in the brain and spinal cord.

Motor neurones are the nerve cells along which the brain sends instructions to the muscles. Deterioration of these cells leads to weakness and wasting of muscles. This causes increasing loss of mobility and stiffness or cramps in the limbs, and difficulties with speech, chewing, swallowing and breathing. Some people may experience changes in thinking and behaviour (cognitive impairment), but only a few (10-15%) will experience severe cognitive change.

The effects of MND can vary from person to person, including the initial symptoms and how quickly the disease progresses, to the length of time people live after being diagnosed.

The aim of treatment is to manage symptoms so that people with the condition can maintain functional ability and enable them and their family members to live as full a life as possible.

Mark Baker, Director for the Centre of Clinical Practice at NICE, said: "MND is a devastating, fatal disease with no cure and limited treatment options. Perhaps because of this, and the fact that every person with MND has a unique, individual experience of the disease, care of people with MND varies across the country. In some areas people receive co-ordinated multidisciplinary care, however, some people with MND are left isolated and their care is less than ideal.

"This guideline, by making evidence-based recommendations covering the care of people with MND from the time of diagnosis until the preparation for end of life care, will go some way towards helping to mitigate the effects of this devastating disease and enable people with MND and their family members to live as full a life as possible."

Commenting on the guidance, Dr David Oliver, Consultant in Palliative Care and Chair of the group which developed the guideline, said: "MND is a complex and difficult illness and treating it can be complicated because it requires the management of a variety of medical problems. There are only about 5000 people with MND in the UK but the impact on them, their families and the professionals involved in their care can be great.

"This guideline covers the care that people with MND and their families and carers should receive from the time of diagnosis. This includes communicating the diagnosis, monitoring disease progression, managing symptoms such as swallowing, breathing and muscle weakness, stiffness or cramps, and preparing for end of life care.

"There is particular emphasis on the importance of a multidisciplinary team approach, and a clinic based multidisciplinary team has been shown to be cost effective. It also recognises the need to ensure support from health and social care professionals is consistent and people with MND and their families receive support from professionals and carers that they know.

"Providing people with MND the opportunity to talk about their symptoms and progression of the disease at an early stage, before communication abilities decline and prevent them from being involved in decisions about their care, is also important.

"The guideline will enable all people with MND across the country, whether in hospital, at home, in a care home or hospice, to receive care that is co-ordinated, consistent, comprehensive and responsive to their needs, that will improve their quality of life and support them, and their families. The challenge is now to see the recommendations put into practice in services across the country."

Sally Light, Chief Executive of the Motor Neurone Disease Association, said: "The MND Association strongly welcomes the publication of the NICE guideline on MND. This long awaited guideline has come about after more than four years of campaigning by the Association and our supporters. It is a hugely significant document that sets out in detail what good care looks like and how it should be delivered. It will shape future care and have a huge influence over the quality of life for people living with MND and their families and carers. We look forward to working with our members and supporters to ensure the NICE guideline on MND has a positive impact on the care and support available to people with MND, their families and carers in England, Wales and Northern Ireland."

Rachael Marsden, Motor Neurone Disease Advanced Nurse Practitioner and Care Centre Coordinator and member of the MND guideline group, said: Having an insight in to how a guideline is developed has been a great privilege. To have been part of something that will have such a great influence on how care for people living with MND is provided across the county has been a unique experience. This guideline has been written with the person living with MND and their family firmly placed at the heart of all our discussions."

Caroline Brown, Principal Physiotherapist in Emergency, Cardiothoracic and Specialised Medicine and member of the MND guideline group, said: "As a committee we were aware that individuals with MND experience difficulties in the way they receive their diagnosis and variation in the delivery of the care they receive. This guideline recommends that individuals receive their diagnosis from a neurologist, with knowledge and expertise in MND so that they can provide support and information about the condition. The guideline advises that any support people subsequently receive is coordinated through a clinic based, specialist MND multidisciplinary team. Regular MND multidisciplinary team clinic reviews will ensure people's symptoms and needs are assessed as their condition changes, whilst any care provided is consistent and responsive. Prompt access to professionals who understand the condition will ensure that individuals have the opportunity to discuss and access appropriate care at the appropriate time which can help improve quality of life and survival."

Jean Waters, patient/care member on the MND guideline group, said: "Being involved in the development of this guideline has been a huge privilege. As someone with MND, the key message has to be that integrated and coordinated multi-disciplinary care from professionals with knowledge and experience of the condition provides the best means of achieving cost effective, timely and appropriate provision of services in health and social care. This can improve quality of life and survival for those diagnosed with MND, the carer journey alongside them, the knowledge and skills of the health and social care professionals looking after them and significant cost savings when the right care is given at the right time.

"This guideline shows what good care should look like and will help both those affected by the disease and the many professionals involved in their care achieve timely, cost effective and quality care irrespective of where they live."

Recommendations in the guideline include:

  • Ensure people are provided with information and support about MND at diagnosis or when they ask for it. If the person agrees, share the information with their family members and/or carers (as appropriate). Information should be oral and written, and may include the following:
    • What MND is.
    • Types and possible causes.
    • Likely symptoms and how they can be managed.
    • How MND may progress.
    • Treatment options.
    • Where the person's appointments will take place.
    • Which healthcare professionals and social care practitioners will undertake the person's care.
    • Expected waiting times for consultations, investigations and treatments.
    • Local services (including social care and specialist palliative care services) and how to get in touch with them.
    • Local support groups, online forums and national charities, and how to get in touch with them.
    • Legal rights, including social care support, employment rights and benefits.
    • Requirements for disclosure, such as notifying the Driver and Vehicle Licensing Agency.
    • Opportunities for advance care planning.
  • Provide coordinated care for people with MND, using a clinic-based, specialist MND multidisciplinary team approach. The clinic may be community or hospital based.
  • The multidisciplinary team should:
    • include healthcare professionals and social care practitioners with expertise in MND, and staff who see people in their home
    • ensure effective communication and co-ordination between all healthcare professionals and social care practitioners involved in the person's care and their family members and/or carers (as appropriate)
    • carry out regular, coordinated assessments at the multidisciplinary team clinic (usually every 2-3 months) to assess people's symptoms and needs.
    • provide coordinated care for people who cannot attend the clinic according to the person's needs

    The guideline covers what to do if people with MND find it difficult to eat. It recommends that the use of a thin feeding tube that allows food to pass directly into the stomach through the skin (gastrostomy) should be discussed with people at an early stage, and at regular intervals as their MND progresses.

    Managing other symptoms of MND including problems with coughing, breathlessness, the use of drugs to treat muscle cramps and stiffness, and exercise programmes to help maintain joint movement and prevent contractures, is also addressed in the guideline. It also highlights that appropriate equipment and adaptations to aid activities of daily living and mobility should be provided without delay to maximise people's participation in activities of daily living and maintain their quality of life.

    For people with MND who have difficulty speaking clearly as a result of the muscles in their mouth, throat and chest being affected, the guideline recommends equipment called Augmentative and Alternative Communication (AAC) should be provided. The use of both low level technologies, for example alphabet, word or picture boards, and high level technologies, such as PC or tablet-based voice output communication aids, may be helpful.

    Progressive weakness of the muscles that control breathing is a major feature of MND. The existing NICE clinical guideline on the use of non-invasive ventilation in the management of MND has been incorporated into this guideline. It includes new recommendations for healthcare professionals responsible for starting non-invasive ventilation treatment in people with MND. These state that such professionals should ensure that support is available for other healthcare professionals who may be involved if there is a plan to stop non-invasive ventilation, including the legal and ethical implications.