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Motor neurone disease Disability Snapshot
This Disability Snapshot provides general information about motor neurone disease to assist you in communicating effectively and supporting the participant in developing their goals in a planning meeting. Each person is an individual and will have their own needs, preferences and experiences that will impact on the planning process. This information has been prepared for NDIA staff and partners and is not intended for external distribution.
Peak body consulted
In developing this resource we consulted with the peak body, Motor Neurone Disease Australia.
What is motor neurone disease?
Motor neurone disease (MND) is the name given to a group of diseases in which the muscles that enable us to move, speak, breathe and swallow fail to work normally. These muscles and the nerves that supply them are known, collectively, as the motor system - the system that is concerned with action and movement (as opposed to sensation).
The system fails because the nerve cells that control movement (the motor neurones) degenerate and die. This loss of motor neurones causes an increasing loss of function over time. The patterns of weakness in MND vary from person to person and currently there is no effective treatment or cure.
There are two different types of motor neurones, and both may be affected:
Upper motor neurones in the brain - damage to these neurones causes spastic or
stiff paralysis of the muscles that they serve. Lower motor neurones in the spinal cord - damage to them causes flaccid or floppy
paralysis.
Without nerve impulses (messages from the brain and spinal cord) the muscles have nothing to activate them and they gradually waste away.
The average life expectancy for a person with MND is two to three years from diagnosis or three to five years from the onset of early symptoms. However, about five to ten per cent of people with MND will have a slowly progressive form of the disease and may live with the disease for up to ten years or more.
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Common characteristics and impacts of motor neurone disease
The pattern of symptoms of MND varies from person to person. Usually the condition begins by affecting only one part of the body then gradually spreads. In some people the symptoms are widespread from the start. Often only upper or lower motor neurones are affected in the early stages, but both are usually involved as the condition progresses.
Early symptoms are usually mild. They may include problems with walking or holding objects in the hand, slurred speech or difficulty swallowing. Some people find that emotional responses such as laughing or crying are more easily triggered.
Cramps are common as is muscle twitching (fasciculation), which is a sign of damage to lower motor neurones (those in the spinal cord). Damage to upper motor neurones (those in the brain) results in stiffness of the muscles (spasticity) and slowed movements. Sometimes the muscles will spasm or jerk spontaneously.
Overtime, the loss of mobility and independence means that many people with MND will need help with personal care, including washing, dressing and going to the toilet. While most people maintain their ability to control their bowel and bladder, their reduced capacity to move about can mean it is harder to get to the toilet. This can be very distressing. Constipation can also occur because they are moving less and have made diet changes to adapt to their swallowing difficulties.
In the past, it was thought that MND only affected the neurones controlling the muscles that enable us to move, speak, breathe and swallow. It is now known that up to 50 percent of people with MND can experience changes in cognition, language, behaviour and personality. Most people experience relatively mild changes. However, a small proportion (5 -15 percent) will show more significant changes and will receive a diagnosis of motor neurone disease with frontotemporal dementia (MND/FTD). Often the symptoms of dementia come before the motor symptoms, sometimes by a number of years.
MND does not affect memory or the senses (sight, hearing, taste, smell and touch).
What are the different types of motor neurone disease?
There are four main forms of MND:
Amyotrophic lateral sclerosis (ALS) is the most common form, characterised by
muscle weakness and stiffness, over-active reflexes and rapidly changing emotions.
Upper and lower motor neurones are both affected and the limbs cease to work
properly. Progressive muscular atrophy (PMA) is characterised by muscle wasting and
weakness, loss of weight and muscle twitching. Only the lower motor neurones are
affected.
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Progressive bulbar palsy (PBP) mixed bulbar palsy and pseudo-bulbar palsy all
involve the muscles of speech and swallowing. The nerves that control these
muscles are in the lower part of the brain (the bulb), hence the term bulbar palsy
(palsy means paralysis). Primary lateral sclerosis (PLS) is very rare with upper motor neurone damage
causing stiffness and paralysis of the limbs. PLS may begin in the arms (flail arm
type) or the legs (flail leg type).
How is motor neurone disease diagnosed?
It can be difficult to diagnose MND as there are several other conditions resembling it. Doctors may need to keep the person under review for weeks, months or years to formally diagnose. The person will probably be referred to a neurologist - a doctor who specialises in conditions affecting the brain and nervous system.
The doctor may order various tests, including some to eliminate other conditions. These include nerve conduction studies (NCS) and electromyography (EMG) which look at how nerves are functioning by stimulating a nerve electrically and recording the muscle activity that results. The EMG will involve putting a fine needle into muscles which can be uncomfortable.
Currently there is no cure for MND, although worldwide research is showing encouraging progress.
Enabling social and economic participation
A person’s support needs for social and economic participation will change as the disease progresses. Maintaining maximum independence in work, personal relationships and in the community will be an important focus.
To maintain work, ongoing customisation of work tasks to match the level of function
will be important, along with consideration of personal care in the work place or
assistance with travel. This might require NDIS funding for specialist disability
assessment services as well as access to external employment retention and
support initiatives such as the Work Assist program provided by Disability
Employment Services.
Common barriers to social and economic participation include:
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Lived challenges: mobility, communication, decision making, social connection,
respiration, emotional lability, swallowing and secretion control, workplace and
housing modifications, transport, obtaining appropriate assistive technology (AT). Concerns and sensitivities: the future, maintaining relationships, living
arrangements such as being able to remain in their own home, embarrassment,
activities of daily living, personal care, family and carers, finances, death.
How can I tailor a meeting to suit a participant with motor neurone disease?
It is common for people in the early stages of MND to present with reasonable functional capacity and a person therefore may decline formal personal supports. While a person may be currently able to continue with their current duties at work, it will be important to discuss and plan for functional changes. Participants and employers should be encouraged to work together as early as possible to discuss customisation of work tasks. This conversation would include planning for the supports that may be introduced to maximise their independence and longevity at work.
It is important to be aware that support needs will change over time and sometimes rapidly. Sensitivity is needed when discussing the wishes of the person with MND and their family. Planning for formal support needs may have to be encouraged initially to enable a smoother transition when there is a need for more intensive supports.
As MND causes progressive deterioration and loss of function it is more appropriate to ask the person about their priorities and what actions to take to address these in their plan. This is preferable to using the term, goal setting.
Planning meetings should provide the person with MND and their family with the opportunity to think about, discuss and set some immediate and longer term priorities. These may include:
maintaining employment and independence in the community deciding how they would like to be supported health and lifestyle decisions maintaining relationships carer needs equipment and assistive technology including rental or purchase, maintenance and
replacement (to maintain independence, social inclusion, safety and communication) assistance with planning and managing changing supports.
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Supports for a person with MND are often complex and cross many sectors including health, disability, community and housing. Individuals often have an ongoing need for assistance with planning and using their informal, mainstream and funded supports. Given the rapidly degenerative nature of MND and complexity of supports required, it is highly recommended that support coordination is included in the plan.
Accessibility or support
The participant may prefer the planning meeting takes place in their home due to mobility issues, assistive technology in use, toileting concerns, transport issues and fatigue. Accessibility or support requirements for the meeting should be discussed with the participant prior to the meeting taking place.
Ensure the participant is aware they can have one or more support people present at the meeting. Ask about communication and AT needs when making arrangements for the meeting. Remember the needs of people living with MND can be complex and will vary from person to person.
Helpful links
MND Australia MNDcare
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Version control
Version Amended Brief Description of Change Status Date by
2.0 KHM678 Editing, correction of spelling error. Class APPROVED 2019-09-23 one approval. CRG656
3.0 KHM678 Class one approved. APPROVED 2020-02-14
CRG656 Annual review of resource. Additional information provided on supporting employment goals for MND.
4.0 KHM678 Class one approved APPROVED 2020-04-03
NAN927 Update to clarify people with MND may need help with personal care, including washing, dressing and going to the toilet.
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