Motor Neurone Disease Care Guide

A Care at Home Guide for people with MND

Introduction to MND

Motor Neurone Disease is a rare and devastating condition that affects the neurons in the brain and the spinal cord. As the name Motor Neurone suggests, the degeneration of the neurons over time will have an adverse effect on the muscles that govern the motor skills (this includes both gross and fine motor skills).

In order for muscles to work they need electrical signals from the brain. Signals from the brain are carried by neurons that pass messages through the nervous system via the spinal cord. These messages are usually a response to stimuli. As neurons degenerate, the muscles receive fewer signals, which in turn weakens the muscles and they deteriorate (muscular dystrophy).

The effect of muscle weakness can affect the way a person walks, moves, grips objects, talks, eats, drinks and breathes. It can also affect cognitive ability, thought processes and emotional wellbeing. However, not everyone suffers the same symptoms, and some symptoms develop at a later stage than others. Additionally, the symptoms do not appear in any specific order.

The disease usually occurs within the 40-70-year-old age group. The initial sign of symptoms may be as simple as feeling overwhelmingly fatigued to the point where it can adversely affect daily life. People may also experience muscle weakness in the face and neck which can cause speech to become slurred and difficulty in swallowing which can have an impact on health.

Motor Neurone Disease Statistics

Roughly two people in every 100,000 will develop MND each year. MND affects 5,000 people in the UK at any one time.

After being diagnosed with MND, life expectancy is greatly reduced. Approximately one in three will die within one year and a further half of those will die within two years.

In the UK, statistics show that six people are diagnosed with MND every day, which is an annual rate of 2,200 people

Just over one-third of the people with MND will also experience cognitive changes that will cause difficulty with executive functions. This may affect their planning abilities; decision making and difficulties with language.

Additionally, 5-10% of people diagnosed with MND will also display signs and symptoms of frontotemporal dementia, which will cause behaviour changes that are more pronounced and debilitating.

Types of Motor Neurone Disease?

There are four main forms of MND and several even rarer forms. Each of the forms has a slightly different way in which it affects the human body. Additionally, there is a possibility of crossovers between two or more of the differing MND forms. So in practical terms, although it is useful to categorise it is not always possible to do so.

There no particular set test to diagnose the disease nor is there a particular identified cause of MND. However, in 5% of cases, there is a family history of either MIND or a condition known as frontotemporal dementia. This type of dementia is known to contribute to the development of MND.

Frontotemporal dementia is caused by shrinkage and damage to the frontal and the temporal lobes of the brain. Any damage to this area of the brain can result in deterioration of the neurons which in turn will result in few or no messages being sent to the nervous system and muscles.

An experienced Neurologist can usually detect and accurately diagnose the presence of MND. However, tests are still needed to rule out the presence of other conditions that may have similar signs and symptoms and subsequently may require a different treatment plan. Rarer forms of MND affect only 5% of the people who have already been diagnosed with the disease.

Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is the commonest form of MND. ALS is also known by another name ‘Lou Gehrig’s disease (or classical motor neurone disease). Like all forms of MND, it is progressive and ultimately fatal. Over time it eventually stops all of the neuron signals to all of the body muscles.

The term ALS seems to be used interchangeably with MND, even by doctors. In the USA, the term ALS is used instead of term Motor Neurone Disease. ALS is the form of MND that affects both the neurons in the brain and neurons in the spine.

This form of MND involves what is termed as the ‘upper neurons’ and the ‘lower neurons,’ which in effect causes loss of strength and muscle wastage in both the legs and the arms. Initially, it can cause frequent unexplained trips and falls or a reduced ability to grip objects. Life expectancy is usually 2-5 years after the symptoms have started.

ALS gained publicity during 2014, with the ALS ice bucket challenge.

Progressive Bulbar Palsy

Progressive Bulbar Palsy (PBP) affects both the upper and lower neurons. Generally, 20% of people who have been diagnosed with MND, will be further diagnosed with this type of the disease. The average life expectancy is between six months to three years after the symptoms manifest themselves.

The muscles that are first affected are usually the facial and neck muscles, the ones that are used for talking, chewing and swallowing. Symptoms include slurred speech, difficulty in swallowing and sometimes choking, either for no reason or on small pieces of food. Over time without adequate support and care, life can be very challenging.

Progressive Muscular Atrophy

Progressive Muscular Atrophy (PMA) is an uncommon but not rare type of MND. This type of MND only usually affects a few MND sufferers. Most people who have been diagnosed with this type of MND can usually live for up to five years after the symptoms first appear. It seems to affect mainly the lower motor neurones.

The first part of the body that is usually affected is the small muscles that are found in the hands and feet. One of the main symptoms is experiencing a loss of strength or manual dexterity with their hands. The muscles do not seem to be tensed or rigid (spasticity), but will have difficulty in gripping objects; opening jars or writing.

Primary Lateral Sclerosis

Primary Lateral Sclerosis (PLS) is also an uncommon form of MND. This type of MND affects the neurons that send messages to the lower limbs. As this type of MND affects mainly the legs, the muscles that help to breathe are not affected so life expectancy can be within normal limits, but if the disease progresses to ALS, then life expectancy is shortened.

Although PLS causes weakness mainly in the leg muscles, some people may also develop problems with the small muscles in their hands causing problems with manual dexterity. Additionally, their facial muscles may be affected which will cause them to slur their speech which will cause communication problems and frustration.

MND Diagnosis

It is important to get a diagnosis as soon as possible to help relieve the symptoms, but also to plan ahead for specific support and care that will be needed. Especially if other diseases such as dementia or COPD are present.

Signs & Symptoms

Motor Neurone Disease can affect different people in different ways. Motor Neurones control muscle activity that we take for granted. Simple activities that may previously have required little or no effort will become difficult to the point where they are impossible to maintain without help and support.

The first symptoms are that are experienced may be in the hands and arms or the feet and legs. However, in other cases, it may be the muscles around the face and throat (bulbar muscles) are initially affected.

Hand and Arm symptoms. This can affect the ability to grip objects as reduced muscle control will affect the ability to hold onto things, open jars, turn keys and sew. This may appear to be similar to dyspraxia, but these difficulties would be new symptoms.

Feet and Leg symptoms. One of the first symptoms may be experiencing a problem with tripping, which may be caused unintentionally by dragging a leg. Problems with the muscles in the lower leg can cause problems getting out of low chairs and climbing the stairs.

Bulbar muscle symptoms. As these muscles affect the face and throat changes may include losing the ability to shout or sing. Changes may occur in voice quality and speech may become slurred. Difficulties with swallowing will make eating difficult.

Other symptoms may include: experiencing muscle cramps; feeling overly tired or finding simple activities may be exhausting; muscles that have already become weakened may twitch due to the interrupted neuron messages received; jerking of an arm or leg while at rest.

Progression of the disease

Initial symptoms may be experienced in just one leg or one arm, but as the disease progresses, it spreads to the other limbs and other muscle groups. Some muscle groups can be more affected than others. Observable signs may include:

  • Walking becomes increasingly difficult to the point where it can be dangerous due to unexplained frequent trips or falls.
  • Tasks that involve the use of hands and arms become more and more difficult. This can be life changing for someone who has worked with their hands all of their life.
  • As the muscles around the mouth, tongue, and throat become weaker, it can cause difficulty in swallowing, which will affect the ability to communicate and to eat.
  • Sneezing and coughing become weaker. This can cause problems as it can be difficult to clear the lungs due to mucus or irritation.
  • As the chest muscles are also affected, any exertion may result in breathlessness and a feeling of total exhaustion. Breathing may become more and more difficult.

However, there are things that MND does not affect

Brain – Very rarely does MND impact on intellect. However, there are always exceptions to the rule, and in the very rare cases where there is a history of frontotemporal dementia or early dementia the brain may be affected.

Sensory System – Although the sensory system is fed by a system of nerves and nerve endings, it is not affected by MND. The senses of smell, sight, taste, and hearing are unaffected and likewise, the sense of movement and body awareness should remain unchanged.

Continence – It is very rare for incontinence to be experienced as part of MND, but due to progressive mobility difficulties, this could be a problem during the later stages of the disease.

Emotion – Although feelings and desires (including sexual desire) may not be directly affected by MND, depression and anxiety are widespread. It has been known that people with MND may laugh or cry involuntarily at inappropriate times, this can be embarrassing for the patient.

The test for MND – what to expect

Unlike many other diseases, no single test will indicate the presence of MND. Many of the early stage symptoms can be explained away by ageing, or they may be initially attributed to other diseases. The usual route for diagnosis is by referral to a Neurologist for assessment. Sometimes a diagnosis can only be made when the symptoms become more apparent.

However, by gathering a medical history and using some or most of the following tests, a diagnosis can be made or at least can help to rule out other causes of the symptoms.

Magnetic Resonance Imaging (MRI) scans – As these are very expensive and time consuming these are usually performed if there is a concern that the experienced symptoms may be due to another cause or to rule out any other disease.

Electromyography (EMG) – This test involves the use of small fine needles that are placed under the skin. These small needles help to record the nerve impulses that are taking place in the muscles particularly the ones in the arms, legs and throat.

Nerve Conduction Studies In this test small pads are applied strategically on the body. A small electrical impulse is applied. Measurements are taken to determine the speed at which the electrical signal affects particular muscles (involuntary movements).

Transcranial Magnetic Stimulation (TMS) – This test involves the measurement of the activity of the nerves that send messages from the brain and the spinal cord. This test can be beneficial in the diagnosis of the particular type of MND and its prognosis.

Palliative care focuses not just on the improvement of the physical quality of life but also on the enhancement of mind and spirit. It supports not just the person receiving the care but also helps enable loved ones to cope with the situation.

Who delivers Motor Neurone Disease Care?

A good care agency whose care philosophy is based on a person-centred approach can understand that every client is unique. A good care agency also promotes client independence, encourages a positive attitude by supporting them to achieve a high quality of life, by making choices rather than have choices made for them.

Care Agencies

Not every care agency can provide such a range of support to meet the needs of clients who have severe conditions such as MND.

Live-in care has become increasingly popular as an alternative to moving into a care home, especially if being at home means that there are better resources that can accommodate changing health needs.

Assessment for MND Care

As the effects of progress, you may find that you will need help and support for everyday living. The assessment is informal and it involves a senior care manager meeting you in your own home. During this meeting, the care manager will get to know you as a person, how your illness affects you and the level of care that you will need initially.

Questions about your care

  • What level of care do you think that I need at the moment?
  • If my disease gets worse how quickly can you put in additional support?
  • How often do you review my package of care?
  • When my MND gets worse, will I be able to continue to live in my own home?
  • Can you help me to look at the advantages and disadvantages of having a live-in carer?
  • What expectations should I have about my quality of life if I have a live-in carer?
  • How do I organise the funding for my care package?
  • Would my carer be able to organise my medications and communicate with my GP for me?
  • Can my carer help me attend hospital appointments?
  • Will I get the chance to meet my carer before we make a decision?

What sort of care to expect?

The type of care that you can expect will depend on your need. Your care package may include:

  • Help with personal care.
  • Support with taking medication.
  • Assistance with household task including gardening and looking after pets.
  • Organising meals and doing shopping.
  • Accompanying you when you attend medical appointments.
  • Arranging for you to take part in social activities.
  • Being an advocate. Being a companion.
  • Look after you at night.
  • If you live alone, by having a live-in carer will make sure that you have support at night when MND symptoms can be more troublesome.

You also need to know what plans are in place should your carer become unwell. You need reassurance about continuity of care.

Treatments & Prognosis

Although there is no cure for MND, there are treatments available to help cope with the symptoms that are experienced and will contribute to slow down the progression of the disease.

  • Medications
    Several useful medications are used, but the one that has proven to be the most successful is Riluzole. This medication slows down the chemical that causes the degeneration of the neuron messages that travel between the brain and the spinal cord. This medication slows down the progression of the disease by a few months.
  • Support with breathing difficulties
    Continuous positive airway pressure (CPAP) machines can be used overnight. Over the past few years, the modification of these machines for use at home has been tremendous. The machines can be portable enough to be used outside of the home. In the past, they would have only been available for use in a hospital or a nursing home. This machine not only helps the MND patient to breathe it also improves their quality of life.
  • Eating. Help may also be needed for eating
    Coping with swallowing saliva can be difficult enough but to swallow food can be even more difficult. By sitting very upright while eating may help, but if solid foods are too difficult, then liquids can be thickened to aid swallowing. Additionally, support may be required if there is significant weakness in the hands and arms. Weight loss can occur, but the use of a gastrostomy tube (PEG) may be very beneficial.
  • The Team
    Usually, there is a whole team of professionals that are involved in the care of a patient with MND. The team may comprise of neurologists, specialist nurses, occupational therapists, support workers, dieticians and speech & language therapists as well as the GP. Additionally, the team will include a counsellor as MND can cause several mental health difficulties, most notably depression.

As the disease takes its course, there will be the need for most if not all of the team.


The speed at which the disease progresses varies from person to person. It will also be determined by the type of MND that they have. Damage to the muscles is irreversible, unlike nerve damage through trauma or other diseases which can often be reversed through stimulation and exercise that help build new neural networks and muscle memory in order to accomplish tasks such as walking or writing.

The rate at which MND progresses is not linear. Sometimes there will be little or no change for months but eventually, the disease will become severe and the patient will be unable to walk, talk, eat or talk. People with MND will die within 3-5 years of the onset of the symptoms: two out of ten will survive for five years and 1 in 10 will survive beyond ten years. Research is ongoing.

Caring for someone with Motor Neurone Disease

When caring for someone who has MND, it is important to realise that each person is an individual, and has their own preferences and interests. Likewise, MND will affect each individual differently and the symptoms of the disease will progress at different rates. Additionally, they will have good days and days that are not so good. Routines are good, but they need to be adaptable.

How to help someone with MND

10 ways you can help

  1. Movement. As the disease progresses, they may need help with moving around. Sitting in a chair, especially if it is for any length of time can become uncomfortable. If they are not moved correctly, it can be discomforting to the person with MND and to the carer trying to move them. Get advice from a physiotherapist about how to lift them safely.
  2. Fatigue. Fatigue is common in most illnesses and it can aggravate symptoms that are already experienced. It is important to conserve energy, by not overextending activities and to use labour saving gadgets where possible. Plan activities in advance, establish a good sleep pattern and respect the limitations that the disease has caused. Overexertion will cause tiredness and disinterest.
  3. Sleep. Encourage a good sleep time routine. Use pillows to spread body pressure and ideally use silk or satin sheets as they will make it easier to move if they feel uncomfortable. Insomnia can be caused by being uncomfortable, difficulty breathing or a dry mouth. Bedtime medications may help but only with the advice from the GP: massage may help to promote relaxation and a C-Pap machine will aid breathing.
  4. Breathlessness. This can occur during the day or the night. Ask them to sit with the back straight as this will help the lungs to fill properly and also help to avoid problems with mucus due to any difficulty of being able to cough. Breathing exercises may help but only follow the ones that have been recommended by a physiotherapist or GP. Avoid contact with people who have a cold or flu.
  5. People with MND feel the cold. Like many other diseases, people with MND will feel cold even on a warm sunny day. Make sure that they are physically warm by using blankets or clothes that are made of natural fibres such as wool or cotton. If they are wheelchair bound or sit on a chair for extended periods of time, make sure that they are sitting on a seat pad and are wearing leg warmers. A gentle massage may help.
  6. Swelling. Due to lack of movement, swelling in the legs or feet can be a problem. If possible, elevate the legs to help disperse the buildup of fluid. Ask the GP if the use of support stockings may help. Also, ask the physiotherapist for some exercises that will help to counteract this problem and try some gentle massage to encourage natural circulation.
  7. Cramp. Due to inactivity, joints may become stiff, and muscles may tense and not easily relax which can result in cramp. The risk of this happening can be alleviated by using cushions, tables or by sitting in an armchair as they will help relieve some of the physical stress that the body is enduring. Cramp can be alleviated by gentle massage and applied warmth (not heat).
  8. Neck weakness. The weakness of muscles in the neck can cause the head to droop. The head can be supported either by using reclining chairs, headrests and supports or by using a velcroed headband that is attached to the head rest. Liaise with the physiotherapist or occupational therapist for more suggestions and appropriate ideas to help.
  9. Arm Function. Lack of arm function may be a problem, but there are many ideas for aids that can help people to be independent for as long as possible. Ideas include specially designed cutlery with handles, mugs with two handles and straw, plates with deep rims to prevent food disappearing off the plate before it has had a chance to be eaten. Everyday cutlery may be too heavy for weak muscles.
  10. Identifying when to get more help. It is important to make sure that the right aids are available because as time passes and the disease progresses help will be needed to cope with daily tasks in order for continued independence and quality of life. Talk to the GP, physiotherapists and support workers as they will be able to help identify and locate additional resources.

Case study & care plan for Karen

Karen was a nurse and until the age of 58. She was still working full-time, attended keep-fit classes, enjoyed craft work in particular pottery. On the rare occasions when she was not working, she looked after her three grandchildren. She lost her husband six years ago when he died of cancer.

Two years ago, Karen found was finding keep fit classes exhausting. On top of that, she had several falls, which she put down to age and difficulties with balance. Additionally, she realised that she was experiencing problems talking and swallowing. She would often end up choking on simple foods. Before seeking help from her GP, she had lost 15 kilograms of weight.

She was given the diagnosis of PBP (Progressive Bulbar Palsy). One of her daughters moved in with her as she felt that it was important that her mother continued to live at home, where she could be near to her family and friends. Soon after the diagnosis, Karen’s health deteriorated even further to the point where she needed a PEG to receive any nutrition.

Two weeks later, due to breathing difficulties, she had a tracheostomy and intermittent BIPAP ventilation was initiated. Her daughter still felt that her Mother should be nursed at home. Karen appeared to be very low and depressed before the arrangements were made for her to stay at home.

Initially, her daughter coped well, but as Karen’s disease progressed it was decided that they would get a specialist carer to help them both cope with the situation. Both Karen and her daughter still have the support of the Neurologist, GP, Physiotherapist and the rest of the team. Just recently the Counsellor has been a great support to both of them.

In agreement with each other and the supporting medical team, GP and the care agency, they are now looking for a live-in carer so that Karen and her daughter can still experience independence. Additionally, the daughter can work outside of the home, which turns out well for both of them.

The financial support for Karen’s live-in carer was the result of a Care Needs Assessment by a Nurse Consultant. This means that her care plan covers a rota that is focussed on continuity of care with a live-in carer and the additional support of a specialist nurse, who will supervise the carer on the use of oxygen; feeding via the PEG and administering any medications.

Care for Karen can be challenging due to her lack of speech, but by using a lightwriter, she is able to give instructions to her nurse and carer as well as her daughter. Karen is less depressed now she has more control and more independence. Additionally, her daughter can leave the house knowing that her mother is being well cared for.

Karen is looking towards being supported through palliative care and has with the help of her Counsellor and a Palliative Nurse drawn up an advanced directive which expresses her wishes to be nursed at home even in the final stages.

Live in Care

People with MND need a care package that is sensitive to their needs, their situation and also has the capacity to allow independence for as long as possible. Independence includes living in their own home, surrounded by their family, friends and neighbours.

Having a live-in carer can relieve much stress especially at night when symptoms can seem worse, especially if it involves breathing difficulties. Live-in carers are specially trained to provide you with a seamless support that focuses on individual support.

Palliative Care

As a broad definition, palliative care is the management of both physical and mental problems, including stress that is associated with patients that are suffering from a terminal or chronic illness including MND.

Palliative care cannot cure or treat the illness, but it does help to relieve the symptoms that are linked with the illness or disease in order to help promote an active and fulfilling lifestyle in so much as the illness allows.