MND is a group of 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, in the form of electrical impulses, to the muscles telling them what to do.

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 the speed of the disease as it progresses, to the length of time people with MND live after diagnosis.

There is currently no cure for MND and the aim of management of symptoms is to help people with the condition maintain functional ability and enable them and their family members to live as full a life as possible.

About 1100 people will develop MND in the UK each year and around 5000 adults are currently living with the disease in the UK.

Commenting on the draft guidance, Dr David Oliver, Consultant in Palliative Care and Chair of the group developing the guideline, said: “MND is a complex and difficult illness and treating it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. Perhaps because of this care of people with MND varies across the country; in some areas MND care centres provide co-ordinated multidisciplinary care, however, some people with MND are left isolated and their care is less than ideal.

“This draft guideline recognises the importance of providing people with MND the opportunity to talk about their symptoms and progression of the disease at an early stage, before communication and cognitive abilities get worse or make them less able to be involved in decisions about their care.

“It 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. The draft guideline places particular emphasis on the importance of a multidisciplinary team approach, including support from social care services, to the care and management of people with MND. 

“In this way 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 go some way to mitigate the effects of this devastating disease.”

Draft recommendations include:

• Offer information and explanations about MND at diagnosis or when people 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 should 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 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, multidisciplinary team approach.
  
  
• 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 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.

The draft 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 draft 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 draft 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 is being amalgamated with 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.

Ends

For more information call the NICE press office on 0300 323 0142, out of hours on 07775 583 813 or email pressoffice@nice.org.uk

Notes to editors

About the draft guidance

1. The draft guidance is available from the NICE website at http://www.nice.org.uk/guidance/indevelopment/GID-CGWAVE0680 
2.  This draft guidance amalgamates new guidance on assessment and management of motor neurone disease with a partial update of NICE guideline 105: The use of non-invasive ventilation in the management of motor neurone disease (published July 2010) and will replace it

About motor neurone disease

1. MND can affect any adult at any age but the onset of disease occurs predominantly between the ages of 55 and 65 years, affecting slightly more men than women. MND is severely life-shortening, with 50% of people dying from respiratory failure within 3 years of developing their first symptoms. However, some people have a slower disease course and may survive for 10 years or more.
2. MND presents in 4 main forms:

• Amyotrophic lateral sclerosis (ALS) which results in upper and lower motor neurone damage with symptoms of wasting and weakness. This occurs in approximately two-thirds of people diagnosed with MND.
• Progressive bulbar palsy, which is caused by lower motor neurone damage in the head and neck, leading to difficulties with swallowing and speech. Progressive bulbar palsy occurs in a quarter of people with MND.
• Progressive muscular atrophy, which is due to lower motor nerve loss, resulting in weakness and wasting of muscles, especially in the arms. It affects 1 in 10 people with MND.
• Primary lateral sclerosis, in which upper motor neurone nerve loss causes increasing stiffness. With this slowly progressive form the prognosis is usually longer – an average of 10–15 years in total

3. Although MND may initially present in one of the above forms, as the disease progresses the effects on each person vary as both lower and upper motor neurones can become affected in any part of the body. Most people lose the ability to walk, move their arms, swallow, speak and have difficulty breathing, eventually leading to death.
4. Approximately 50% of people with MND show cognitive change, varying from mild frontal lobe changes, which may affect decision making, to frontal temporal dementia.
5. MND is thought to develop as a result of a complex interplay between genetic, environmental and lifestyle factors in the ageing brain.
6. The majority of people with MND do not have a family history of the disease (known as ‘sporadic’ MND), but approximately 5–10% have a close family relative who has the disease (‘familial’ MND). Of those who have familial MND, a large proportion have a mutation in the C9orf72 gene and the other genes and proteins that are linked to familial MND, including SOD1, TARDBP-43 and FUS.

Although MND may initially present in one of the above forms, as the disease progresses the effects on each person vary as both lower and upper motor neurones can become affected in any part of the body. Most people lose the ability to walk, move their arms, swallow, speak and have difficulty breathing, eventually leading to death.

1. Approximately 50% of people with MND show cognitive change, varying from mild frontal lobe changes, which may affect decision making, to frontal temporal dementia.
2. MND is thought to develop as a result of a complex interplay between genetic, environmental and lifestyle factors in the ageing brain.
3. The majority of people with MND do not have a family history of the disease (known as ‘sporadic’ MND), but approximately 5–10% have a close family relative who has the disease (‘familial’ MND). Of those who have familial MND, a large proportion have a mutation in the C9orf72 gene and the other genes and proteins that are linked to familial MND, including SOD1, TARDBP-43 and FUS.