Caring for someone with motor neurone disease towards the end of life
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Key points
- Motor neurone disease (MND) can progress rapidly and has no cure.
- Early support from specialist palliative care services can make a difference to quality of life and symptom control.
- Give opportunities to discuss and record preferences for end of life on a regular basis. Do this before the need is urgent, their ability to communicate is limited or they find it too tiring.
- Manage symptoms of motor neurone disease to ensure the person with MND has the best possible quality of life.
What is motor neurone disease?
Motor neurone disease (MND) is a life-shortening, progressive disease that affects the brain and spinal cord. MND is classed as a terminal illness.
MND attacks cells in the nerves called motor neurones. These cells control movement. Motor neurone disease weakens the muscles until they no longer function, and causes other symptoms listed below. It does not usually affect the sensory nerves, like sight, hearing and the sense of touch.
While it does not usually affect the bladder and bowels, going to the toilet might be more difficult if the person has mobility problems.
People living with MND might be referred to palliative care services at the beginning of their diagnosis to support their condition. Some people access palliative care throughout the course of the disease. They will also need this care towards the end of life.
Motor neurone disease (MND) is the name for a group of diseases. In some instances you may hear it called Amyotrophic Lateral Sclerosis (ALS).
Signs and symptoms of motor neurone disease
Signs and symptoms of MND include:
- muscle weakness, affecting mobility of arms, legs, hands and the neck
- weakness in breathing muscles
- slurred speech
- difficulty swallowing
- muscle cramps and twitches
- weight loss
- changes in cognition (see below)
- being unable to regulate or display emotions appropriately, such as laughing or crying.
Up to 50 out of 100 people (50%) with MND experience changes which affect their behaviour or cognition. This rises to 80 out of 100 people (80%) in the final stage of the disease. Cognition refers to the mental processes you use to make decisions, understand things and form language.
Some people with MND might also have frontotemporal dementia (FTD). Sometimes a person can experience cognitive symptoms, or be diagnosed with FTD, before they are diagnosed with MND.
Living with and managing motor neurone disease
There is no cure for MND. But there are treatments to help reduce the impact is has on someone's life. So, the management of MND is to control its symptoms. Some people are appropriate for medication that can slow down the progression of the disease. Others might not be able to have this medication.
There are some interventions that can help manage symptoms:
- a face mask to help the person breathe (non-invasive ventilation)
- a feeding tube, such as a gastrostomy.
These interventions may not be appropriate or wanted by some people living with MND. MND affects everyone differently and decisions about care can vary.
What is the life expectancy of someone with motor neurone disease?
A person with MND will usually die between two to three years after diagnosis, but this can vary from person to person. Some people live many years after their diagnosis.
What are the symptoms of motor neurone disease towards the end of life?
People with MND may have the symptoms listed below at any stage of the disease. They become more likely and more severe in the final year of life.
MND affects each person differently. So, symptoms of MND, progression and what to expect towards the end of life can vary.
Muscle weakness
Widespread muscle wasting will affect a person’s ability to move around and do daily tasks.
MND Association estimate that 80 out of 100 (80%) of people with MND will need to use a wheelchair. Some people with MND might spend most of their time in bed or in a chair. This might mean that they need more support from carers with moving around, going to the toilet or eating. It is important to get input from an occupational therapist to find out what assistive equipment would be helpful to the person.
Read more abut managing muscle weakness on the MND Association website.
Breathing problems
Most people with MND will experience breathing problems. This is because MND affects the diaphragm and chest wall muscles which help them breathe. Breathing problems can lead to fatigue, trouble sleeping or shortness of breath, particularly when lying flat.
Some people choose to use a non-invasive breathing mask to help with breathing problems. This is sometimes called assisted ventilation. This requires specialist input as assisted ventilation is not right for everyone.
Use of a breathing mask also needs specialist monitoring. This is because some people with MND may be unable to get rid of the carbon dioxide in their blood. A build-up of carbon dioxide in the bloodstream is called hypercapnia. This can make someone drowsy and unwell.
The person may be prescribed opiates such as morphine and lorazepam which can help the feeling of breathlessness.
For more guidance on breathing problems speak to the specialist palliative care team, a respiratory consultant, or the person's neurologist.
Read more about breathing and ventilation on the MND Association website.
Difficulty swallowing (dysphagia)
Weakness of the tongue, mouth, and throat muscles can lead to speech and swallowing problems. Eating and drinking can become more difficult, which may lead to coughing. Sometimes, this can mean small amounts of food and liquid get into someone's lungs. And some people may experience choking, or choking episodes.
It might help to talk to the person and those close to them about any fears they might have to help reduce anxiety.
Some people choose to have a feeding tube so they do not have to swallow. This tube allows them to get nutrition and take medicines. The procedure of fitting the tube is called a gastrostomy. The tube allows specially prepared liquid to go straight to the stomach, through a tube in the abdomen.
View a tool that helps people make a decision about using a gastronomy tube.
Some people may experience painful spasms of the vocal cords, which can temporarily make it difficult to speak or breath. This is known as laryngospasm.
Read more about difficulty swallowing on the MND Association website.
Saliva problems
Weakness of the tongue, mouth and throat muscles can also lead to the pooling and drooling of thin saliva, or difficulty clearing thick saliva. Sometimes it requires input from the person's consultant or specialist MND practitioner to control these symptoms.
Read more about saliva problems on the MND Association website.
Difficulty speaking (dysarthria)
Weakness of the tongue, mouth and throat muscles may lead to slurred or quiet speech. In some cases, the person may lose their speech completely. People with MND may use a range of alternative and augmentative communication strategies and devices to allow them to continue to communicate.
Ask the person how they would prefer to communicate. This could be using strategies such as gestures, notepads and alphabet boards. There might be the opportunity to use voice and message banking. This can help preserve the sound of the person's voice, for use with computer-based communication aids.
Read more about difficulty speaking on the MND Association website.
Pain
People with MND may experience pain, usually because of muscle cramps or tightness in their muscles (spasticity). Pain can usually be managed with painkillers.
Physiotherapists and occupational therapists might be able to help with pain management by helping with posture management, exercise and accessing equipment.
Read more abut muscle weakness and pain on the MND Association website.
How can I support someone with motor neurone disease?
As a healthcare professional, you are well-placed to support someone with MND in many ways. If this is not within your role, speak to your line manager, or one of the person's specialists helping to manage this condition. They should be able to offer more support.
Multi-disciplinary team working
Care for people with MND should be co-ordinated using a specialist multi-disciplinary team approach.
Some areas of the UK may have MND care co-ordinators, MND specialist nurses, neurology specialist nurses or palliative care specialists. These professionals may have specialist knowledge or know the person well. Co-ordinating care with these professionals will help to ensure the person and those around them have the support they need.
You might also think about working with the following people:
- neurologists or consultants
- physiotherapists
- occupational therapists
- community or district nurses
- speech and language therapists
- dieticians
- social workers
- counsellors or spiritual advisers
- complementary therapists.
Early referral to specialist palliative care
MND can progress rapidly, so support from specialist palliative care services should be available as soon as the person feels it is appropriate and is able to accept the referral. Palliative care might help if they:
- have complex symptoms
- need home adaptations or equipment
- experience a change in physical or cognitive ability
- are going through emotional or spiritual distress.
Even if the person does not need palliative care yet, this specialist team can provide support and information to help you care for someone with MND. This team might also be able to signpost to support groups and information resources for the person, and those important to them.
Timely access to equipment and support
Plan ahead for equipment, support and symptom management. Put these plans into place early, where possible. A person's needs can change quickly. So, delays can mean the equipment or support is no longer useful by the time it is in place. Consider referring the person to an occupational therapist. They might be able to help with accessing equipment.
Think about how you can support the person emotionally, as well as physically. Circumstances, cultures and beliefs may change the way a person responds to changes in their condition. Timely access to appropriate support might make the person more comfortable. This could be talking to a counsellor or spiritual adviser, seeing a physiotherapist, or trying complementary therapies.
Read information on supporting someone emotionally on the MND Association website.
Talk to the person about what equipment they might need. This might include:
- communication aids, such as an image board or computer software
- suction machines
- mobility aids
- syringe drivers.
Planning their future care
Talk to the person with MND, and those close to them about the care they'd like in the future. This is sometimes called advance care planning (ACP). It is best to talk about a person's future care before:
- the decision must be made urgently
- their ability to communicate is limited
- communication becomes too tiring.
They may want to record their wishes in an advance care plan. This can include how they want to be cared for, and who they want to be involved in decisions about their care. This can be a formal document, but does not need to be. Revisit advance care plans regularly as the person's condition changes.
Topics to discuss include:
- Which interventions, if any, they would like to have (such as assisted ventilation or gastrostomy).
- Whether they would like to record an Advance Decision to Refuse Treatment (ADRT) or a Do Not Attempt Cardiopulmonary Resuscitation order (DNACPR).
- Whether they will need 'just in case' or anticipatory medicines at home to quickly treat symptoms like breathlessness and chest secretions.
Read more about antipatory medicines here.
Explain that the person cannot insist on having a specific treatment in their advance care plan.
Some people choose to legally nominate someone to make decisions about their health and care or their property and finances. This is called appointing a Lasting Power of Attorney.
Palliative or end of life care registers
The person should be included on any local palliative or end of life care register. This helps ensure they get the right help in case of an emergency. Some areas have electronic palliative care coordination systems (EPaCCS) or electronic patient records.
What to expect when caring for someone with motor neurone disease towards the end of life
MND Association have a guide for professionals on caring for someone with MND towards the end of life. The guide includes practical information about care needs towards the end of life. It also includes tip on how best to support the person with MND and those close to them.
Read MND Association's A professionals' guide to end of life care in motor neurone disease.
Below, we've outlined some things you might need to be aware of when caring for someone with MND towards the end of life.
Posture and positioning
If a person has problems breathing, they may feel breathless when they lie flat. This can be uncomfortable and scary. Ask them which positions they find most comfortable and make it easier to breathe. The ideal position is usually upright or slightly reclined, with the arms, back, head and neck supported.
Read more about breathlessness.
Emotional and psychological support
People with MND, and those close to them, often experience psychological and emotional distress. Caring for someone with MND can be phsyically and emotionally challenging. So, people living with MND, and those important to them, often need practical and emotional support.
Information about emotions, thinking and behaviour is available from MND Association. You may want to signpost the person with MND to their webpage for more support.
Read more about MND and emotions on the MND Association website.
You can call Marie Curie's Support Line for free at 0800 090 2309 or online chat.
Withdrawal of assisted ventilation
People with MND who use assisted ventilation may decide that they no longer wish to receive this intervention. This means the person will have to rely solely on their own breathing muscles. If their muscles are not working well enough, withdrawing this intervention may result in their death. This is not the same as assisted dying, and the person with MND has the right to refuse ventilation.
Palliative care specialists can help support the withdrawal process. This is to ensure that any symptoms of breathlessness and distress can be controlled, and the person is comfortable. It must be a planned procedure following palliative care and organisational guidelines.
Read the guidelines from the Association of Palliative Medicine.
How can I support those important to someone with motor neurone disease?
MND is unpredictable, and a person's condition can deteriorate quickly. People close to the person may need practical and emotional support. Here are some ways you can help:
- Take time to discuss what end of life care might look like as early as possible, with the person's permission. This can help them to feel prepared and be involved in discussions around care.
- Provide contact details of people who can support out of hours or in an emergency.
- Read MND Association's information for professionals about caring for someone with MND towards the end of life.
- Share information for friends, family and carers about supporting someone with MND.
- Share information about Marie Curie's Support Line.
- Share information about Marie Curie's Bereavement Support Service.
Who to ask for help
If you're worried about medication or symptoms, speak to:
- your line manager
- the person's palliative care team
- the person's neurologist
- another member of the person's healthcare team.
Useful resources
The MND Asssociation offers a wide range of information, resources and support to people affected by MND. They also have information for health and social care professionals.
Contact their free helpline MND Connect on 0808 802 6262 or email mndconnect@mndassociation.org
Visit MND Association's website for further information and education opportunities.
NICE Guideline [HG42]: Motor neurone disease: assessment and management
NICE: Motor neurone disease quality standard. Quality statement 5: Planning for end of life care
View a decision-making tool for patients about gastronomy tubes. The tool was created by the DIAMOND research project at the University of Suffolk, jointly funded by Marie Curie and Motor Neurone Disease Association.
This content has been provided with support from the Motor Neurone Disease Association .
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Disclaimer
This information is not intended to replace any training, national or local guidelines, or advice from other health or social care professionals.
The Palliative Care Knowledge Zone is not intended for use by people living with a terminal illness or their family and friends, who should access our information for the public.
