Motor Neurone Disease

The four main types of motor neurone disease are as follows:

• Amyotrophic lateral sclerosis (ALS): the most common form, which leads to a variety of symptoms because both upper and lower motor neurones are affected, causing weakness and wastage of muscles, muscle twitching and spasm and difficulties speaking or swallowing

• Progressive bulbar palsy (PBP): a fairly rare form of the disease, PBP mainly affects the brain’s bulbar region, resulting in symptoms affecting the facial muscles, throat muscles and tongue, causing an inability to swallow, drooping face and speech difficulties

• Primary lateral sclerosis (PLS): very rare, this affects upper motor neurones, causing some level of weakness in the limbs, but not wasting of the muscles. This type often has a longer life expectancy than other types

• Progressive muscular atrophy (PMA): this quite rare type of motor neurone disease progresses a little more slowly than others and mainly affects the lower motor neurones, with symptoms including weight loss, wasting and weakness of the muscles and muscle twitching. Sometimes, people are diagnosed with PMA and then progress on to a different type of the disease, as their symptoms worsen

Here, we cover the symptoms of motor neurone disease, according to each stage of the disease.

In the ‘early stages’ of motor neurone disease, some people find they have one or more of these symptoms:

• Weakened arm or hand muscles, often noticed due to a lack of grip strength

• Weakened lower limbs, such as the ankle, which may result in tripping up

• Weight loss or visible loss of muscle mass

• Difficulties with speaking or swallowing e.g. slurring of words

• Shortness of breath (although this is rare)

• Pain e.g. caused by muscle cramps, joint inflammation, damaged nerves

• Fatigue

If you are concerned that you or someone you know may be exhibiting any of the symptoms above, it is important to see a GP as soon as possible. Motor neurone disease is quite rare, and there are many other conditions that can cause similar early symptoms, but these should be checked out to ensure a correct diagnosis.

As the disease progresses, which may take many years, the ‘advanced’ to ‘end stage’ symptoms may include:

• Muscles become very weak, stiff and may be difficult to move

• Difficulties speaking and swallowing become more pronounced

• Excess saliva and drooling

• Uncontrollable yawning

• Uncontrollable emotions e.g. laughing or crying

• Cognitive difficulties e.g. finding it hard to concentrate

• Increasing breathing difficulties

• Increasing physical disability and paralysis

• Increasing pain

MND Scotland provides a Managing MND Factsheet about the symptoms of MND.

If you or someone you know is concerned about any symptoms they may be experiencing, it is important to visit a GP. If you are presenting with symptoms that may be associated with motor neurone disease, your GP will examine you physically and may ask questions about your lifestyle and how long you have had these symptoms.

Motor neurone disease is quite rare, and many GPs may have never come across the condition before. The Motor Neurone Disease Association provides a helpful guide to support GPs in considering motor neurone disease early on, and when to refer to a neurologist, called ‘Could this be motor neurone disease?’

A neurologist will try to find out if you have motor neurone disease, or another neurological condition, by conducting blood tests, scans, and special tests that measure the electrical impulses transmitted by nerve cells. To find out more about specific tests, visit the NHS website.

A person receiving a diagnosis of motor neurone disease may find this very difficult. They may go through a range of emotions, such as grief, devastation, disbelief, panic, and sadness. It may take some time to come to terms with the diagnosis; some people may find they accept this over time, whereas others may find they need ongoing emotional support. It is important to find out as much as possible about the condition and the motor neurone disease support available to you and your family.

The Motor Neurone Disease Association provides information if you are newly diagnosed. If you are a parent recently diagnosed with motor neurone disease, MND Scotland provides a factsheet Telling Children about MND, which you may find useful.

Read on to find out more about treatments for motor neurone disease that can help relieve symptoms, as well as the challenges and possibilities if you are living with motor neurone disease.

Motor neurone disease is likely to have an impact on all areas of daily living at some stage.

Most people with the condition will find their mobility is impaired, which is likely to get worse over time. This may affect their ability to walk, get around, drive, exercise, and do all those daily tasks that we take for granted, such as using the toilet or having a shower.

If this is the case, there are specialists called Occupational Therapists (OT) who can assess a person’s needs and recommend different ways to approach tasks, or equipment that may help make tasks easier. Speak to your local social services department regarding an OT assessment. There is lots of adaptive equipment available that can help you to live well with a disability.

Many people are able to overcome some of the potential limitations of living with motor neurone disease, and find ways to live well and do the things they love to do. You may find it helps to hear stories of other people who are living with motor neurone disease, such as Lucy Lintott, or personal stories provided by supporters of the Motor Neurone Disease Association.

Marie Curie provides a wealth of information about living with a terminal illness, and has a telephone support helpline that you may wish to contact.

MND Scotland provides a variety of factsheets on lots of topics related to daily life with motor neurone disease, such as travel, finance, receiving care at home, how to manage physical disability, and preparing for end of life.

Caring for someone with motor neurone disease

If you are caring for someone with motor neurone disease, you are likely to feel an impact on your own wellbeing and life as well. For advice and support on being a carer, visit the Carers UK website.

You may also wish to find out what benefits you are entitled to, and the NHS provides Your Guide to Care and Support which may be helpful.

Motor neurone disease treatment focusses on managing symptoms. There is currently no cure for the disease, but research continues in this area.

There is one key drug treatment available called Riluzole, which slows down the progression of the disease and can increase life expectancy by a couple of months. It does not reverse the damage already caused by the disease, it isn’t suitable for everybody and some people choose not to take it.

There are many general medications available that may provide some relief from symptoms such as pain or inflammation, and your neurologist or GP is able to prescribe these to you.

Some people require surgical procedures or special medical equipment to help them eat or breathe independently during the later stages of their disease, such as:

• Percutaneous endoscopic gastrostomy (PEG) tube – this procedure inserts a feeding tube into a person’s stomach when they are unable to eat orally, and allows special, nutrient full liquid food to be syringed into the tube

• Non-invasive mechanical ventilation (NIV) – this is a ventilator machine, used when a person is struggling to breathe, either for short periods during the day or night, or all the time

A motor neurone disease diagnosis usually results in you having a team of health and social care professionals to support you:

• A physiotherapist may assist with muscle, joint and walking difficulties

• A speech and language therapist may assist with swallowing, eating, drinking and speaking difficulties

• An Occupational Therapist may assist with adapting your daily tasks and environment to enable you to carry out activities

• A neurologist will monitor the progression of your disease

• A GP and sometimes a specialist nurse may provide other healthcare support and monitoring

Some people also find that counselling can help them and their family cope with the challenges of having motor neurone disease. For more information on counselling and how to find a counsellor, visit the NHS website.

Some people choose to explore complementary therapies as motor neurone disease treatments, such as acupuncture, reflexology, massage, reiki, etc. Many people report that these treatments provide relaxation, comfort and reduce negative emotions such as stress and depression, but they will not reverse or slow down the progression of the disease.

At NRS Healthcare, we are experts in daily living aids – these are products and devices that are designed for people living with health conditions or disabilities, to assist them in carrying out daily tasks. This also includes equipment for people who are providing care for someone else.

Here, we suggest some products that may be useful if you or someone you know has motor neurone disease, depending on the symptoms being experienced and how a product can help. This is by no means an exhaustive list, and our website features our full range of disability products and daily living aids.

Difficulty gripping objects

• Cutlery

• Tap turners

• Key turner

Difficulty swallowing

• Pureed food bottle

• Non-spill, easy grip mug

Mobility aids when walking is difficult

• Walking stick

• Rollator

• Manual wheelchair

• Ramp

Using the toilet independently or with assistance

• Toilet frame and seat

• Wheeled commode

Bathing independently or with assistance

• Bath lift

Showering independently or with assistance

• Shower commode chair

Getting in and out of a chair

• Furniture raisers

• Rise and recline chair

Getting in and out of bed

• Bed rail

Moving someone you care for

• Hoist

• Sling

• Transfer aid

• Slide sheet

High dependency chairs and beds

• Electrically operated bed

• Pressure relief adjustable chair

If you are unsure what products may help you or the person you provide care for, contact our Occupational Therapist product advisors who can advise you on which NRS Healthcare daily living aids for motor neurone disease might help you. Contact them on 0345 121 8111 or email [email protected] You may also wish to contact the Disabled Living Foundation.

PLEASE NOTE: our Product Advice Team can only give advice about equipment and products which may help you to live more independently. They cannot give any advice on medications or treatments for symptoms of this condition.

Diet for motor neurone disease

Healthy eating is important for everybody, especially those with long term health conditions, but the actual process of eating independently with motor neurone disease may become difficult.

Motor neurone disease symptoms include muscle weakness. This often occurs around the face and throat, which may cause difficulties chewing and swallowing. A speech and language therapist and dietician can help advise on a motor neurone disease diet.

Weakness may also occur in the hands, which may make eating with standard cutlery, plates and so on more difficult. There are daily living aids available to assist with this, such as large handled cutlery, which is easier to grip.

The Motor Neurone Disease Association provides a leaflet about eating and drinking with MND, including information, tips and easy-swallow recipes.

Exercise for motor neurone disease

Taking regular exercise is still possible for someone with motor neurone disease, especially in the earlier stages of the disease. There are many forms of exercise that a person can still enjoy, within their physical limits. Exercise will help you to maintain mobility for as long as possible. A physiotherapist will be able to advise on an exercise regime suitable for you. There are many types of adapted exercise equipment now, such as bicycles that are operated with arms rather than pedalled with legs. The NHS provides fitness advice for wheelchair users, and there are many exercise classes available for people locally who have healthcare conditions.

Motor neurone disease and employment

Many people with motor neurone disease are able to work in the early stages of the condition – some choose to continue working and some choose to change jobs or leave work if they are financially able to. Some people feel they have to leave employment, and some people find their symptoms are too intense for them to continue to work. You may need to explore financial help if you’re disabled. The charity Scope also provides information about many types of financial benefit available to you and your family if you have motor neurone disease.

The Motor Neurone Disease association provides a leaflet for more information on MND and employment.

Motor Neurone Disease Association Forum – provided by the UK’s leading MND charity, this forum connects people affected by the disease, to discuss all aspects of life and health

ALS forums – a US based site open to people across the world to discuss their experiences of motor neurone disease

Brain & Spine Foundation – a source of information about a variety of neurological conditions, including MND, as well as a nurse-led advice helpline

Marie Curie – a UK charity that provides support for people living with terminal illness and their families, including nursing support with information on care and end of life care, plus practical matters such as benefits, finances etc.

Motor Neurone Disease Association (MNDA) – the leading UK charity for educating and researching MND, which provides information for people with the condition as well as healthcare professionals, local support services and includes a helpline

MND Scotland – a Scottish charity that provides information about living with MND as well as funding for research projects

NHS Choices – a source of official medical information about causes, symptoms and treatments for MND, as well as a range of other conditions

Scope – a UK charity providing information related to disability, as well as an online community and helpline

Brain stem

– part of the brain that connects to the spinal cord and controls the messages which are essential for basic actions of the body e.g. breathing, heart rate, consciousness

Bulbar region

– part of the brain that includes the brain stem, cerebellum and pons, which are all responsible for bodily functions that are crucial to keeping us alive

Central nervous system

– the brain and spinal cord which work together to transmit messages around the body to control all the body’s actions

Frontotemporal dementia

– a medical condition which causes dementia symptoms due to damage in the frontal and temporal lobes of the brain, which are responsible for behaviour, language, memory and judgement

Motor neurones

– nerve cells in the body through which the brain and spinal cord transmit messages to muscles

Neurologist

– a doctor who specialises in disorders affecting the nerves, brain and spinal cord i.e. the nervous system

Neurones

– these are nerve cells that transmit messages or ‘impulses’ to and from the brain

Spinal cord

– part of the body that consists of nerves that transfer messages to and from the brain, all around the body