Caring for someone with motor neurone disease at the end of life
Please be aware - this information is for healthcare professionals
Motor neurone disease (MND) is a fatal, rapidly progressing disease that affects the brain and spinal cord. MND attacks the nerves that control movement so muscles no longer work. It does not usually affect senses, for example sight, sound and touch.
There is no cure for MND, but there are interventions such as non-invasive ventilation and gastrostomy that can help manage symptoms. A third of people with MND die within a year of diagnosis, and more than half die within two years.
Up to half of all people with MND will be affected by some degree of cognitive or behavioural change. A proportion will be diagnosed with frontotemporal dementia (FTD). Some people will experience cognitive changes, or be diagnosed with FTD, before they are even diagnosed with MND. These changes can affect behaviour, decision-making and the way they use language.
How can MND affect people at the palliative and end of life stage?
MND affects each person differently, so symptoms, progression and what to expect at the palliative and end of life stage can vary from person to person. Some of the symptoms that may affect a person in the later stages of MND are listed below, but it’s important to note that these symptoms could occur throughout the course of the disease.
- Muscle weakness: Widespread muscle wasting will affect a person’s mobility and their ability to undertake daily tasks. Most people with MND eventually need to use a wheelchair and will require support from carers.
- Respiratory problems: Most people with MND will experience breathing difficulties late in the course of their disease, because their diaphragm and accessory muscles will be affected. Some people may choose to have assisted ventilation. More information can be found on the Motor Neurone Disease Association's website .
- Dysphagia: Weakness of the bulbar muscles (tongue, mouth, and throat muscles) can lead to swallowing problems. Eating and drinking can become more difficult, which may lead to episodes of coughing and sometimes to choking and aspiration. Some people may choose to have a gastrostomy. More information can be found on the Motor Neurone Disease Association's website .
- Saliva problems: Bulbar weakness can also lead to the pooling and drooling of thin saliva, or difficulty clearing thick, tenacious saliva. More information can be found on the Motor Neurone Disease Association's website .
- Dysarthria: Bulbar weakness may lead to slurred or quiet speech. In rare cases, the person may lose their speech completely. People with MND may use a range of alternative and augmentative communication strategies and devices. More information can be found on the Motor Neurone Disease Association's website .
- Pain: People with MND may experience pain, usually as a result of muscle cramps or spasticity.
What should I be aware of when treating a patient with MND at the palliative and end of life stage?
Cause of death: It is important to provide reassurance to your patient and their loved ones that the cause of death in people with MND is very rarely choking. The usual cause is respiratory insufficiency. If symptoms are well managed, in the majority of cases death can be peaceful.
Posture and positioning: If a person has respiratory problems, they may feel breathless when lying flat. This may feel uncomfortable, scary and, in some cases, may be dangerous. It is vital to find positions that make it easier for the person with MND to breathe. The ideal position is usually upright or slightly reclined, with the arms, back, head and neck supported. See our page on breathlessness for more information.
Oxygen: Although there are some exceptions, oxygen therapy can have a harmful effect in people with MND, reducing respiratory drive and worsening their condition. For more guidance on this, speak to the specialist palliative care team or a respiratory consultant with links to the neurology team.
Emotional and psychological support: People with MND and those close to them often experience considerable psychological and emotional distress. Caring for someone with MND can also be physically and emotionally challenging. Support should be available to help people cope with the impact of the condition.
Cognitive change: Up to half of all people with MND will experience some degree of change in thinking and behaviour. So you need to take care to ensure they are able to give informed consent during decision making. Everyone diagnosed with MND should be screened for cognitive change using appropriate assessment tools. People with MND and frontotemporal dementia may lack mental capacity and care should be adapted accordingly. More information can be found on the Motor Neurone Disease Association's website.
What special considerations should I make in my approach to providing care for someone with MND?
Multidisciplinary team working: The National Institute for Health and Care Excellence (NICE) guideline on MND advise thatcare for people with the condition should be coordinated, using a specialist multidisciplinary team approach. See the Motor Neurone Disease Association website or the NICE website.
Early referral to specialist palliative care: Because MND can progress rapidly, support from specialist palliative care services should be available as soon as your patient feels it is appropriate for them. Specialist palliative care can provide a crucial source of support as part of the multidisciplinary team and can make a huge difference to quality of life.
Timely access to equipment and support: Equipment, support and symptom management should be planned ahead and put in place early, where possible. However, this should be approached with care and sensitivity and discussed with your patient and their family first. Also, every effort should be made to prevent untimely case closures, as needs can change quickly.
Advance care planning: It’s crucial to have discussions about end of life choices with your patient and their family before the need is urgent, the capacity to communicate is limited or tiring, or in case a person experiences cognitive change.
Discussions should include whether or not the person would wish to have particular interventions if needed in the future, including assisted ventilation and gastrostomy. It is important to discuss whether the person may wish to record an Advance Decision to Refuse Treatment (ADRT) or a Do Not Attempt Cardiopulmonary Resuscitation order (DNACPR).
Palliative or end of life care registers: The person should be included on any local palliative or end of life care register, to ensure appropriate support in case of an emergency.
The Motor Neurone Disease Association offers a wide range of information, resources, and support to people affected by MND, family, carers and health and social care professionals.
Contact their free helpline MND Connect on 0808 802 6262 or email@example.com
This content has been provided by the Motor Neurone Disease Association.
- MND can be rapidly progressive and has no cure, so all care and support for people with the condition must be timely and is essentially palliative.
- Early support from specialist palliative care services can make a huge difference to quality of life.
- People should be given opportunities to discuss and record preferences for the end of their life, before the need is urgent, their ability to communicate is limited or they find it too tiring.
- Symptoms should be managed in line with the wishes of the person with MND to ensure they have the best possible quality of life.
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