Recommendations

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 of our recommendations, and has information about safeguarding, consent and prescribing medicines (including 'off‑label' use).

1.1 Recognition and referral

1.1.1 Ensure that robust protocols and pathways are in place to:

  • inform healthcare professionals about motor neurone disease (MND) and how it may present

  • inform healthcare professionals in all settings about local referral arrangements

  • ensure continued and integrated care for people with MND across all care settings. [new 2016]

1.1.2 Be aware that MND causes progressive muscular weakness that may first present as isolated and unexplained symptoms. These symptoms may include:

  • functional effects of muscle weakness, such as loss of dexterity, falls or trips

  • speech or swallowing problems, or tongue fasciculations (this is known as bulbar presentation)

  • muscle problems, such as weakness, wasting, twitching, cramps and stiffness

  • breathing problems, such as shortness of breath on exertion or respiratory symptoms that are hard to explain

  • effects of reduced respiratory function, such as excessive daytime sleepiness, fatigue, early morning headache or shortness of breath when lying down. [new 2016]

1.1.3 Be aware that MND may first present with cognitive features, which may include:

  • behavioural changes

  • emotional lability (not related to dementia)

  • frontotemporal dementia. [new 2016]

1.1.4 If you suspect MND, refer the person without delay and specify the possible diagnosis in the referral letter. Contact the consultant neurologist directly if you think the person needs to be seen urgently. [new 2016]

1.1.5 Provide information and support for people and their family members and/or carers (as appropriate) throughout the diagnostic process, particularly during periods of diagnostic uncertainty or delay. [new 2016]

1.2 Information and support at diagnosis

Please also refer to the recommendations in NICE's guideline on patient experience in adult NHS services, which includes recommendations on communication, information and coordination of care.

1.2.1 Information about the diagnosis, prognosis and management of MND should be given by a consultant neurologist with up‑to‑date knowledge and experience of treating people with MND unless it is clinically necessary to give the diagnosis in an urgent situation. The neurologist should have knowledge and expertise in the following:

  • Symptoms of MND.

  • Types and possible causes of MND.

  • 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. [new 2016]

1.2.2 Ask people about how much information they wish to receive about MND, and about their preferences for involving their family members and/or carers (as appropriate). [new 2016]

1.2.3 Ensure people are provided with information about MND and support 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 (DVLA).

  • Opportunities for advance care planning. [new 2016]

1.2.4 When MND is diagnosed, provide people with a single point of contact for the specialist MND multidisciplinary team (see section 1.5). Provide information about what to do if there are any concerns between assessments or appointments, during 'out‑of‑hours' or in an emergency, or if there is a problem with equipment. [new 2016]

1.2.5 Offer the person with MND a face‑to‑face, follow‑up appointment with a healthcare professional from the multidisciplinary team, to take place within 4 weeks of diagnosis. [new 2016]

1.2.6 When MND is suspected or confirmed, inform the person's GP without delay and provide information about the likely prognosis. [new 2016]

1.2.7 Set aside enough time to discuss the person's concerns and questions, which may include the following:

  • What will happen to me?

  • Are there any treatments available?

  • Is there a cure?

  • How long will I live?

  • What will the impact on my day‑to‑day life be?

  • What will happen next with my healthcare?

  • Will my children get MND?

  • How do I tell my family and friends?

  • How will I die? [new 2016]

1.2.8 If the person has any social care needs, refer them to social services for an assessment. Be aware that some people with MND may not have informal care available, and may live alone or care for someone else. [new 2016]

1.2.9 Advise carers that they have a legal right to have a Carer's Assessment of their needs; support them with requesting this from their local authority. [new 2016]

1.3 Cognitive assessments

Please also refer to the recommendations in NICE's guideline on patient experience in adult NHS services.

1.3.1 Be aware that people with MND and frontotemporal dementia may lack mental capacity. Care should be provided in line with the Mental Capacity Act 2005. [new 2016]

1.3.2 At diagnosis, and if there is concern about cognition and behaviour, explore any cognitive or behavioural changes with the person and their family members and/or carers as appropriate. If needed, refer the person for a formal assessment in line with the NICE guideline on dementia. [new 2016]

1.3.3 Tailor all discussions to the person's needs, taking into account their communication ability, cognitive status and mental capacity. [new 2016]

1.4 Prognostic factors

1.4.1 When planning care take into account the following prognostic factors, which are associated with shorter survival if they are present at diagnosis:

  • Speech and swallowing problems (bulbar presentation).

  • Weight loss.

  • Poor respiratory function.

  • Older age.

  • Lower Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS or ALSFRS‑R) score.

  • Shorter time from first developing symptoms to time of diagnosis. [new 2016]

1.5 Organisation of care

1.5.1 Provide coordinated care for people with MND, using a clinic‑based, specialist MND multidisciplinary team approach. The clinic may be community or hospital based. [new 2016]

1.5.2 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 coordination 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. [new 2016]

1.5.3 The multidisciplinary team should assess, manage and review the following areas, including the person's response to treatment:

  • Weight, diet, nutritional intake and fluid intake, feeding and swallowing (see section 1.10).

  • Muscle problems, such as weakness, stiffness and cramps (see recommendations 1.8.1–1.8.9 in section 1.8).

  • Physical function, including mobility and activities of daily living (see section 1.9).

  • Saliva problems, such as drooling of saliva (sialorrhoea) and thick, tenacious saliva (see recommendations 1.8.10–1.8.15 in section 1.8).

  • Speech and communication (see section 1.11).

  • Cough effectiveness (see section 1.13).

  • Respiratory function, respiratory symptoms and non‑invasive ventilation (see sections 1.12 and 1.14).

  • Pain and other symptoms, such as constipation.

  • Cognition and behaviour (see section 1.3).

  • Psychological support needs (see recommendations 1.6.1–1.6.4 in section 1.6).

  • Social care needs (see recommendations 1.6.5–1.6.6 in section 1.6).

  • End of life care needs (see section 1.7).

  • Information and support needs for the person and their family members and/or carers (as appropriate) (see section 1.2). [new 2016]

1.5.4 The core multidisciplinary team should consist of healthcare professionals and other professionals with expertise in MND, and should include the following:

  • 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). [new 2016]

1.5.5 The multidisciplinary team should have established relationships with, and prompt access to, the following:

  • 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. [new 2016]

1.5.6 Tailor the frequency of the multidisciplinary team assessments to the person's symptoms and needs, with more or less frequent assessments as needed. [new 2016]

1.5.7 Ensure arrangements are in place to trigger an earlier multidisciplinary team assessment if there is a significant change in symptoms identified by the person, family members and/or carers (as appropriate), or healthcare professionals. [new 2016]

1.5.8 Tailor the multidisciplinary team assessment to the person's needs, for example, adjust the format if the person has cognitive or behaviour changes or difficulties with communication. [new 2016]

1.5.9 Inform all healthcare professionals and social care practitioners involved in the person's care about key decisions reached with the person and their family members and/or carers (as appropriate). [new 2016]

1.5.10 Ensure that all healthcare professionals and social care practitioners involved in the person's care are aware that MND symptoms may get worse quickly, and that people with MND will need repeated, ongoing assessments. Priority should be given to ensuring continuity of care and avoiding untimely case closure. [new 2016]

1.5.11 Consider referral to a specialist palliative care team for people with current or anticipated significant or complex needs, for example, psychological or social distress, troublesome or rapidly progressing symptoms and complex future care planning needs. [new 2016]

1.5.12 For guidance on the use of riluzole for people with MND, see the NICE technology appraisal guidance on the use of riluzole (Rilutek) for the treatment of motor neurone disease. [new 2016]

1.6 Psychological and social care support

1.6.1 During multidisciplinary team assessments and other appointments, discuss the psychological and emotional impact of MND with the person and ask whether they have any psychological or support care needs. Topics to discuss may include the following:

  • Their understanding of MND and how it affects daily living.

  • Accepting and coping with the diagnosis and prognosis, including concerns and fears about dying.

  • Their ability to continue with current work and usual activities.

  • Adjusting to changes in their life and their perception of self.

  • Changes in relationships, familial roles and family dynamics.

  • Sexuality and intimacy.

  • Concerns about their family members and/or carers.

  • Decision-making. [new 2016]

1.6.2 Offer the person information about sources of emotional and psychological support, including support groups and online forums. If needed, refer the person to counselling or psychology services for a specialist assessment and support. [new 2016]

1.6.3 During multidisciplinary team assessments and other appointments, discuss the psychological and emotional impact of MND with family members and/or carers (as appropriate), and ask whether they have any psychological or social care support needs. Topics to discuss may include the following:

  • Their understanding of MND and how it affects daily living.

  • Accepting and coping with the diagnosis and prognosis, including concerns and fears about the person with MND dying.

  • Adjusting to changes in their life.

  • Changes in relationships, familial roles and family dynamics, including their change to a carer role (if appropriate).

  • Sexuality and intimacy.

  • Involvement in decision‑making.

  • Impact on other family members and/or carers.

  • Their ability and willingness to provide personal care and operate equipment. [new 2016]

1.6.4 Offer family members and/or carers (as appropriate) information about respite care and sources of emotional and psychological support, including support groups, online forums and counselling or psychology services. [new 2016]

1.6.5 A social care practitioner with knowledge of MND or rapidly progressive complex disabilities should discuss the person's needs and preferences for social care, and provide information and support for them to access the following:

  • Personal care, ensuring there is continuity of care with familiar workers, so that wherever possible, personal care and support is carried out by workers known to the person and their family members and/or carers (as appropriate).

  • Equipment and practical support (see section 1.9).

  • Financial support and advice (for example, money management, how to access carers' and disability benefits and grants, continuing healthcare funding and funeral expenses).

  • Support to engage in work, social activities and hobbies, such as access to social media and physical access to activities outside their home.

  • Respite care. [new 2016]

1.6.6 Be aware that as MND progresses, people may develop communication problems and have difficulty accessing support or services. For example, they may be unable to access a call centre. Ensure people are given different ways of getting in touch with support or services, and a designated contact if possible. [new 2016]

1.7 Planning for end of life

1.7.1 Offer the person with MND the opportunity to discuss their preferences and concerns about care at the end of life at trigger points such as: at diagnosis, if there is a significant change in respiratory function, or if interventions such as gastrostomy or non‑invasive ventilation are needed. Be sensitive about the timing of discussions and take into account the person's current communication ability, cognitive status and mental capacity. [new 2016]

1.7.2 Be prepared to discuss end of life issues whenever people wish to do so. [new 2016]

1.7.3 Provide support and advice on advance care planning for end of life. Topics to discuss may include:

  • What could happen at the end of life, for example, how death may occur.

  • Providing anticipatory medicines in the home.

  • Advance care planning, including Advance Decisions to Refuse Treatment (ADRT) and Do Not Attempt Resuscitation (DNACPR) orders, and Lasting Power of Attorney.

  • How to ensure advance care plans will be available when needed, for example, including the information on the person's Summary Care Record.

  • When to involve specialist palliative care.

  • Areas that people might wish to plan for, such as:

    • what they want to happen (for example, their 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. [new 2016]

1.7.4 Think about discussing advance care planning with people at an earlier opportunity if you expect their communication ability, cognitive status or mental capacity to get worse. [new 2016]

1.7.5 Offer people the opportunity to talk about, and review any existing, ADRT, DNACPR orders and Lasting Power of Attorney when interventions such as gastrostomy and non‑invasive ventilation are planned. [new 2016]

1.7.6 Provide additional support as the end of life approaches, for example, additional social or nursing care to enable informal carers and family to reduce their carer responsibilities and spend time with the person with MND. [new 2016]

1.7.7 Towards the end of life, ensure there is prompt access to the following, if not already provided:

  • A method of communication that meets the person's needs, such as an AAC system.

  • Specialist palliative care.

  • Equipment, if needed, such as syringe drivers, suction machines, riser–recliner chair, hospital bed, commode and hoist.

  • Anticipatory medicines, including opioids and benzodiazepines to treat breathlessness, and antimuscarinic medicines to treat problematic saliva and respiratory secretions. [new 2016]

1.7.8 Offer bereavement support to family members and/or carers (as appropriate). [new 2016]

1.8 Managing symptoms

Pharmacological treatments for muscle problems

1.8.1 Discuss the available treatment options for muscle problems. Take into account the person's needs and preferences, and whether they have any difficulties taking medicine (for example, if they have problems swallowing). [new 2016]

1.8.2 Consider quinine[1] as first‑line treatment for muscle cramps in people with MND. If quinine is not effective, not tolerated or contraindicated, consider baclofen[1] instead as second‑line treatment. If baclofen is not effective, not tolerated or contraindicated, consider tizanidine[1], dantrolene[1] or gabapentin[1]. [new 2016]

1.8.3 Consider baclofen, tizanidine, dantrolene[1] or gabapentin[1] to treat muscle stiffness, spasticity or increased tone in people with MND. If these treatments are not effective, not tolerated or contraindicated, consider referral to a specialist service for the treatment of severe spasticity. [new 2016]

1.8.4 Review the treatments for muscle problems during multidisciplinary team assessments, ask about how the person is finding the treatment, whether it is working and whether they have any adverse side effects. [new 2016]

Exercise programmes

1.8.5 Consider an exercise programme for people with MND to:

  • maintain joint range of movement

  • prevent contractures

  • reduce stiffness and discomfort

  • optimise function and quality of life. [new 2016]

1.8.6 Choose a programme that is appropriate to the person's level of function and tailored to their needs, abilities and preferences. Take into account factors such as postural needs and fatigue. The programme might be a resistance programme, an active‑assisted programme or a passive programme. [new 2016]

1.8.7 Check that family members and/or carers (as appropriate) are willing and able to help with exercise programmes. [new 2016]

1.8.8 Give advice to the person and their family members and/or carers (as appropriate) about safe manual handling. [new 2016]

1.8.9 If a person needs orthoses to help with muscle problems, they should be referred to orthotics services without delay, and the orthoses should be provided without delay. [new 2016]

Saliva problems

1.8.10 If a person with MND has problems with saliva, assess the volume and viscosity of the saliva and the person's respiratory function, swallowing, diet, posture and oral care. [new 2016]

1.8.11 If a person with MND has problems with drooling of saliva (sialorrhoea), provide advice on swallowing, diet, posture, positioning, oral care and suctioning. [new 2016]

1.8.12 Consider a trial of antimuscarinic medicine[1] as the first‑line treatment for sialorrhoea in people with MND. [new 2016]

1.8.13 Consider glycopyrrolate[1] as the first‑line treatment for sialorrhoea in people with MND who have cognitive impairment, because it has fewer central nervous system side effects. [new 2016]

1.8.14 If first-line treatment for sialorrhoea is not effective, not tolerated or contraindicated, consider referral to a specialist service for Botulinum toxin A[1]. [new 2016]

1.8.15 If a person with MND has thick, tenacious saliva:

  • review all current medicines, especially any treatments for sialorrhoea

  • provide advice on swallowing, diet, posture, positioning, oral care, suctioning and hydration

  • consider treatment with humidification, nebulisers and carbocisteine. [new 2016]

1.9 Equipment and adaptations to aid activities of daily living and mobility

1.9.1 Healthcare professionals and social care practitioners, which will include physiotherapists and occupational therapists, should assess and anticipate changes in the person's daily living needs, taking into account the following:

  • 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. [new 2016]

1.9.2 Provide equipment and adaptations that meet the person's needs without delay, so that people can participate in activities of daily living and maintain their quality of life as much as possible. [new 2016]

1.9.3 Refer people to specialist services without delay if assistive technology such as environmental control systems is needed. People should be assessed and assistive technology provided without delay. [new 2016]

1.9.4 Refer people to wheelchair services without delay if needed. Wheelchair needs should be assessed and a manual and/or powered wheelchair that meets the person's needs should be provided without delay. [new 2016]

1.9.5 Ensure that equipment, adaptations, daily living aids, assistive technology and wheelchairs meet the changing needs of the person and their family and/or carers (as appropriate) to maximise mobility and participation in activities of daily living. [new 2016]

1.9.6 Ensure regular, ongoing monitoring of the person's mobility and daily life needs and abilities as MND progresses. Regularly review their ability to use equipment and to adapt equipment as necessary. [new 2016]

1.9.7 Healthcare professionals, social care practitioners and other services providing equipment should liaise to ensure that all equipment provided can be integrated, for example, integrating AAC aids and devices and environmental control systems with wheelchairs. [new 2016]

1.9.8 Enable prompt access and assessment for funding for home adaptation. If the person is not eligible for funding, continue to offer information and support in arranging home environment adaptations. [new 2016]

1.10 Nutrition and gastrostomy

Please also refer to the recommendations in NICE's guideline on nutrition support for adults.

1.10.1 At diagnosis and at multidisciplinary team assessments, or if there are any concerns about weight, nutrition or swallowing, assess the person's weight, diet, nutritional intake, fluid intake, hydration, oral health, feeding, drinking and swallowing, and offer support, advice and interventions as needed. [new 2016]

1.10.2 Assess the person's diet, hydration, nutritional intake and fluid intake by taking into account:

  • fluids and food intake versus nutritional and hydration needs

  • nutritional supplements, if needed

  • appetite and thirst

  • gastrointestinal symptoms, such as nausea or constipation

  • causes of reduced oral intake (for example, swallowing difficulties, limb weakness or the possibility of low mood or depression causing loss of appetite). [new 2016]

1.10.3 Assess the person's ability to eat and drink by taking into account:

  • the need for eating and drinking aids and altered utensils to help them take food from the plate to their mouth

  • the need for help with food and drink preparation

  • advice and aids for positioning, seating and posture while eating and drinking

  • dealing with social situations (for example, eating out). [new 2016]

1.10.4 Arrange for a clinical swallowing assessment if swallowing problems are suspected. [new 2016]

1.10.5 Assess and manage factors that may contribute to problems with swallowing, such as:

  • positioning

  • seating

  • the need to modify food and drink consistency and palatability

  • respiratory symptoms and risk of aspiration and/or choking

  • fear of choking and psychological considerations (for example, wanting to eat and drink without assistance in social situations). [new 2016]

1.10.6 Discuss gastrostomy at an early stage, and at regular intervals as MND progresses, taking into account the person's preferences and issues, such as ability to swallow, weight loss, respiratory function, effort of feeding and drinking and risk of choking. Be aware that some people will not want to have a gastrostomy. [new 2016]

1.10.7 Explain the benefits of early placement of a gastrostomy, and the possible risks of a late gastrostomy (for example, low critical body mass, respiratory complications, risk of dehydration, different methods of insertion, and a higher risk of mortality and procedural complications). [new 2016]

1.10.8 If a person is referred for a gastrostomy, it should take place without unnecessary delay. [new 2016]

1.10.9 Pay particular attention to the nutritional and hydration needs of people with MND who have frontotemporal dementia and who lack mental capacity. The multidisciplinary team assessment should include the support they need from carers, and their ability to understand the risks of swallowing difficulties. [new 2016]

1.10.10 Before a decision is made on the use of gastrostomy for a person with MND who has frontotemporal dementia, the neurologist from the multidisciplinary team should assess the following:

  • The person's ability to make decisions and to give consent[2].

  • The severity of frontotemporal dementia and cognitive problems.

  • Whether the person is likely to accept and cope with treatment.

    Discuss with the person's family members and/or carers (as appropriate; with the person's consent if they have the ability to give it). [new 2016]

1.11 Communication

1.11.1 When assessing speech and communication needs during multidisciplinary team assessments and other appointments, discuss face‑to‑face and remote communication, for example, using the telephone, email, the Internet and social media. Ensure that the assessment and review is carried out by a speech and language therapist without delay. [new 2016]

1.11.2 Provide AAC equipment that meets the needs of the person without delay to maximise participation in activities of daily living and maintain quality of life. The use of both low‑level technologies, for example, alphabet, word or picture boards and high‑level technologies, for example, PC or tablet‑based voice output communication aids may be helpful. Review the person's communication needs during multidisciplinary team assessments. [new 2016]

1.11.3 Liaise with, or refer the person with MND to, a specialised NHS AAC hub if complex high technology AAC equipment (for example, eye gaze access) is needed or is likely to be needed. [new 2016]

1.11.4 Involve other healthcare professionals, such as occupational therapists, to ensure that AAC equipment is integrated with other assistive technologies, such as environmental control systems and personal computers or tablets. [new 2016]

1.11.5 Ensure regular, ongoing monitoring of the person's communication needs and abilities as MND progresses, and review their ability to use AAC equipment. Reassess and liaise with a specialised NHS AAC hub if needed. [new 2016]

1.11.6 Provide ongoing support and training for the person with MND, and their family members and/or carers (as appropriate), in using AAC equipment and other communication strategies. [new 2016]

1.12 Respiratory function and respiratory symptoms

1.12.1 Assess and monitor the person's respiratory function and symptoms. Treat people with MND and worsening respiratory impairment for reversible causes (for example, respiratory tract infections or secretion problems) before considering other treatments. [new 2016]

1.12.2 Offer non-invasive ventilation as treatment for people with respiratory impairment (see section 1.14). Decisions to offer non‑invasive ventilation should be made by the multidisciplinary team in conjunction with the respiratory ventilation service, and the person (see recommendations 1.5.1–1.5.5). [new 2016]

1.12.3 Consider urgent introduction of non‑invasive ventilation for people with MND who develop worsening respiratory impairment and are not already using non‑invasive ventilation. [new 2016]

1.12.4 Consider opioids[1] as an option to relieve symptoms of breathlessness. Take into account the route of administration and acquisition cost of medicines. [new 2016]

1.12.5 Consider benzodiazepines[1] to manage breathlessness that is exacerbated by anxiety. Take into account the route of administration and acquisition cost of medicines. [new 2016]

1.13 Cough effectiveness

1.13.1 Offer cough augmentation techniques such as manual assisted cough to people with MND who cannot cough effectively. [new 2016]

1.13.2 Consider unassisted breath stacking and/or manual assisted cough as the first‑line treatment for people with MND who have an ineffective cough. [new 2016]

1.13.3 For people with bulbar dysfunction, or whose cough is ineffective with unassisted breath stacking, consider assisted breath stacking (for example, using a lung volume recruitment bag). [new 2016]

1.13.4 Consider a mechanical cough assist device if assisted breath stacking is not effective, and/or during a respiratory tract infection. [new 2016]

1.14 Non-invasive ventilation

Information and support about non‑invasive ventilation

1.14.1 Offer to discuss the possible use of non‑invasive ventilation with the person and (if the person agrees) their family and carers, at an appropriate time and in a sensitive manner. This may be at one or more of the following times:

  • soon after MND is first diagnosed

  • when monitoring respiratory function

  • when respiratory function deteriorates

  • if the person asks for information. [2010]

1.14.2 Discussions about non‑invasive ventilation should be appropriate to the stage of the person's illness, carried out in a sensitive manner and include information on:

  • the possible symptoms and signs of respiratory impairment (see box 1)

  • the purpose, nature and timing of respiratory function tests, and explanations of the test results

  • how non‑invasive ventilation (as a treatment option) can improve symptoms associated with respiratory impairment and can be life prolonging, but does not stop progression of the underlying disease. [2010, amended 2016]

1.14.3 When discussing non‑invasive ventilation, explain the different ways that people can manage their breathlessness symptoms. This should include:

  • non-invasive ventilation, and its advantages and disadvantages

  • using non-invasive ventilation at different points in the course of the person's lifetime

  • the possibility of the person becoming dependent on non‑invasive ventilation

  • options for treating any infections

  • support and information on how to recognise and cope with a distressing situation

  • the role of medication for breathing problems

  • psychological techniques and support. [new 2016]

1.14.4 Check that the person thinking about non‑invasive ventilation:

  • understands what non‑invasive ventilation is and what it can achieve

  • recognises the need for regular review

  • has enough information about non‑invasive ventilation and other options for breathing problems to make decisions about how and when to use it.

  • understands possible problems with compatibility with other equipment, for example, eye gaze access systems. [new 2016]

1.14.5 Explain that non-invasive ventilation can be stopped at any time. Reassure people that they can ask for help and advice if they need it, especially if they are dependent on non‑invasive ventilation for 24 hours a day, or become distressed when attempting to stop it. Inform people that medicines can be used to alleviate symptoms (see recommendation 1.14.29). [new 2016]

1.14.6 Ensure that families and carers:

  • have an initial assessment if the person they care for decides to use non‑invasive ventilation, which should include:

    • their ability and willingness to assist in providing non‑invasive ventilation

    • their training needs

  • have the opportunity to discuss any concerns they may have with members of the multidisciplinary team, the respiratory ventilation service and/or other healthcare professionals. [2010]

Identification and assessment of respiratory impairment

Symptoms and signs

1.14.7 Monitor the symptoms and signs listed in box 1 to detect potential respiratory impairment. [2010, amended 2016]

Box 1 Symptoms and signs of potential respiratory impairment

Symptoms

Signs

Breathlessness

Increased respiratory rate

Orthopnoea

Shallow breathing

Recurrent chest infections

Weak cough1

Disturbed sleep

Weak sniff

Non-refreshing sleep

Abdominal paradox (inward movement of the abdomen during inspiration)

Nightmares

Use of accessory muscles of respiration

Daytime sleepiness

Reduced chest expansion on maximal inspiration

Poor concentration and/or memory

Confusion

Hallucinations

Morning headaches

Fatigue

Poor appetite

1 Weak cough could be assessed by measuring peak cough flow.

Respiratory function tests

1.14.8 As part of the initial assessment to diagnose MND, or soon after diagnosis, a healthcare professional from the multidisciplinary team who has appropriate competencies should perform the following tests (or arrange for them to be performed) to establish the person's baseline respiratory function:

  • oxygen saturation measured by pulse oximetry (SpO2):

    • this should be a single measurement of SpO2 with the person at rest and breathing room air

    • if it is not possible to perform pulse oximetry locally, refer the person to a respiratory ventilation service.

      Then one or both of the following:

  • forced vital capacity (FVC) or vital capacity (VC)[3]

  • sniff nasal inspiratory pressure (SNIP) and/or maximal inspiratory pressure (MIP). [2010]

1.14.9 If the person has severe bulbar impairment or severe cognitive problems that may be related to respiratory impairment:

  • ensure that SpO2 is measured (at rest and breathing room air)

  • do not perform the other respiratory function tests (FVC, VC, SNIP and MIP) if interfaces are not suitable for the person. [2010]

1.14.10 A healthcare professional with appropriate competencies should perform the respiratory function tests every 2–3 months, although tests may be performed more or less often depending on:

  • whether there are any symptoms and signs of respiratory impairment (see box 1)

  • the rate of progression of MND

  • the person's preference and circumstances. [2010, amended 2016]

1.14.11 Perform arterial or capillary blood gas analysis if the person's SpO2 (measured at rest and breathing room air):

  • is less than or equal to 92% if they have known lung disease

  • is less than or equal to 94% if they do not have lung disease.

    If it is not possible to perform arterial or capillary blood gas analysis locally, refer the person to a respiratory ventilation service. [2010]

1.14.12 If the person's SpO2 (measured at rest and breathing room air) is greater than 94%, or 92% for those with lung disease, but they have sleep‑related respiratory symptoms:

  • consider referring them to a respiratory ventilation service for continuous nocturnal (overnight) oximetry and/or a limited sleep study and

  • discuss both the impact of respiratory impairment and treatment options with the patient and (if the person agrees) their family and carers. [2010]

1.14.13 If the person's arterial partial pressure of carbon dioxide (PaCO2) is greater than 6 kPa:

  • refer them urgently to a respiratory ventilation service (to be seen within 1 week) and

  • explain the reasons for and implications of the urgent referral to the person and (if the person agrees) their family and carers. [2010]

1.14.14 If the person's PaCO2 is less than or equal to 6 kPa but they have any symptoms or signs of respiratory impairment, particularly orthopnoea (see recommendation 1.14.15):

  • refer them to a respiratory ventilation service for nocturnal (overnight) oximetry and/or a limited sleep study and

  • discuss both the impact of respiratory impairment and treatment options with the person and (if the person agrees) their family and/or carers (as appropriate). [2010]

1.14.15 If any of the results listed in box 2 is obtained, discuss with the person and (if appropriate) their family and carers:

  • their respiratory impairment

  • their treatment options

  • possible referral to a respiratory ventilation service for further assessment based on discussion with the person, and their wishes. [2010, amended 2016]

Box 2 Results of respiratory function tests

Forced vital capacity (FVC) or vital capacity (VC)

Sniff nasal inspiratory pressure (SNIP) and/or maximal inspiratory pressure (MIP)

(if both tests are performed, base the assessment on the better respiratory function reading)

FVC or VC less than 50% of predicted value

FVC or VC less than 80% of predicted value plus any symptoms or signs of respiratory impairment (see recommendation 1.14.7), particularly orthopnoea

SNIP or MIP less than 40 cmH2O

SNIP or MIP less than 65 cmH2O for men or 55 cmH2O for women plus any symptoms or signs of respiratory impairment (see recommendation 1.14.7), particularly orthopnoea

Repeated regular tests show a rate of decrease of SNIP or MIP of more than 10 cm H2O per 3 months

People with a diagnosis of frontotemporal dementia

1.14.16 Base decisions on respiratory function tests for a person with a diagnosis of frontotemporal dementia on considerations specific to their needs and circumstances, such as:

  • their ability to give consent[2]

  • their understanding of the tests

  • their tolerance of the tests and willingness to undertake them

  • the impact on their family and carers

  • whether they are capable of receiving non‑invasive ventilation. [2010, amended 2016]

Non-invasive ventilation for treatment of respiratory impairment in people with MND

1.14.17 Offer a trial of non-invasive ventilation if the person's symptoms and signs and the results of the respiratory function tests indicate that the person is likely to benefit from the treatment. [2010, amended 2016]

1.14.18 Consider a trial of non‑invasive ventilation for a person who has severe bulbar impairment or severe cognitive problems that may be related to respiratory impairment only if they may benefit from an improvement in sleep‑related symptoms or correction of hypoventilation. [2010, amended 2016]

1.14.19 Before starting non-invasive ventilation, the multidisciplinary team together with the respiratory ventilation service should carry out and coordinate a patient‑centred risk assessment, after discussion with the person and their family and carers. This should consider:

  • the most appropriate type of non‑invasive ventilator and interfaces, based on the person's needs and lifestyle factors and safety

  • the person's tolerance of the treatment

  • the risk, and possible consequences, of ventilator failure

  • the power supply required, including battery back‑up

  • how easily the person can get to hospital

  • risks associated with travelling away from home (especially abroad)

  • whether a humidifier is required

  • issues relating to secretion management

  • the availability of carers. [2010]

1.14.20 Before starting non-invasive ventilation, the multidisciplinary team together with the respiratory ventilation service should prepare a comprehensive care plan, after discussion with the person and their family and carers (who should be offered a copy of the plan). This should cover:

  • long-term support provided by the multidisciplinary team

  • the initial frequency of respiratory function tests and monitoring of respiratory impairment

  • the frequency of clinical reviews of symptomatic and physiological changes

  • the provision of carers

  • arrangements for device maintenance and 24‑hour emergency clinical and technical support

  • secretion management and respiratory physiotherapy assessment, including cough augmentation (if required)

  • training in and support for the use of non-invasive ventilation for the person and their family and carers

  • regular opportunities to discuss the person's wishes in relation to continuing or withdrawing non‑invasive ventilation. [2010, amended 2016]

1.14.21 When starting non‑invasive ventilation:

  • perform initial acclimatisation during the day when the person is awake

  • usually start regular treatment at night, before and during sleep

  • gradually build up the person's hours of use as necessary. [2010]

1.14.22 Continue non-invasive ventilation if the clinical reviews show:

  • symptomatic and/or physiological improvements for a person without severe bulbar impairment and without severe cognitive problems

  • an improvement in sleep‑related symptoms for a person with severe bulbar impairment or with severe cognitive problems that may be related to respiratory impairment. [2010]

1.14.23 Provide the person and their family and/or carers (as appropriate) with support and assistance to manage non‑invasive ventilation. This should include:

  • training on using non‑invasive ventilation and ventilator interfaces, for example:

    • emergency procedures

    • night-time assistance if the person is unable to use the equipment independently (for example, emergency removal or replacement of interfaces)

    • how to use the equipment with a wheelchair or other mobility aids if required

    • what to do if the equipment fails

  • assistance with secretion management

  • information on general palliative strategies

  • an offer of ongoing emotional and psychological support for the person and their family and carers. [2010, amended 2016]

1.14.24 Discuss all decisions to continue or withdraw non‑invasive ventilation with the person and (if the person agrees) their family and carers. [2010]

1.14.25 Before a decision is made on the use of non‑invasive ventilation for a person with a diagnosis of frontotemporal dementia, the multidisciplinary team together with the respiratory ventilation service should carry out an assessment that includes:

  • the person's capacity to make decisions and to give consent[2]

  • the severity of dementia and cognitive problems

  • whether the person is likely to accept treatment

  • whether the person is likely to achieve improvements in sleep‑related symptoms and/or behavioural improvements

  • a discussion with the person's family and/or carers (with the person's consent if they have the capacity to give it). [2010, amended 2016]

1.14.26 Consider prescribing medicines to help ease breathlessness that people using non‑invasive ventilation can take on an 'as‑needed' basis at home, for example, opioids[1] or benzodiazepines[1]. [new 2016]

1.14.27 Inform services that may see the person in crisis situations, such as their GP and services that provide emergency or urgent care, that the person is using non‑invasive ventilation. [new 2016]

Stopping non-invasive ventilation

1.14.28 The healthcare professionals responsible for starting non‑invasive ventilation treatment in people with MND 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. [new 2016]

1.14.29 If a person on continuous non‑invasive ventilation wishes to stop treatment, ensure that they have support from healthcare professionals with knowledge and expertise of:

  • stopping non‑invasive ventilation

  • the ventilator machine

  • palliative medicines (see the NICE guideline on care of dying adults in the last days of life)

  • supporting the person, family members and/or carers (as appropriate)

  • supporting other healthcare professionals involved with the person's care

  • legal and ethical frameworks and responsibilities. [new 2016]

1.14.30 If a person on continuous non‑invasive ventilation wishes to stop treatment, seek advice from healthcare professionals who have knowledge and experience of stopping non‑invasive ventilation. [new 2016]

1.14.31 Healthcare professionals involved in stopping non‑invasive ventilation should have up‑to‑date knowledge of the law regarding the Mental Capacity Act, DNACPR, ADRT orders, and Lasting Power of Attorney. [new 2016]

To find out what NICE has said on topics related to this guideline, see our web pages on motor neurone disease, dementia, nutrition and end of life care.



[1] At the time of publication (February 2016), these medicines did not have a UK marketing authorisation for this indication. The prescriber should follow relevant professional guidance, taking full responsibility for the decision. Informed consent should be obtained and documented. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.

[3] The difference between the measurement of vital capacity and forced vital capacity is very subtle and so either can be used.

  • National Institute for Health and Care Excellence (NICE)