Transcript Document

Motor
Neurone
Disease
What is MND?
Regional Care Development Advisor
Colin Pearson
Motor Neurone Disease
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Not contagious
Not directly hereditary
Is a terminal disease
No cure
Path of disease is different in every person
Currently one medication that may improve quality /
prolonging of life – Riluzole (Rilutek)
Onset and progression is variable – can progress
swiftly with rapid deterioration
Mostly an adult Illness – most people affected are
over 50 years of age
So what
is a
Motor
Neurone?
Structure of a motor neurone
So what
does a Motor
Neurone do?
Function of Motor Neurones
Function of Motor Neurones
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Upper motor
neurones originate in
the base of the cortex
of the brain
Lower motor
neurones originate in
the spinal cord
Function of Motor Neurones
So what
happens in
Motor
Neurone
Disease?
The disease process
Types of
Motor
Neurone
Disease
Amyotrophic Lateral Sclerosis – 65%
• Upper and lower motor neurones affected
• Spasticity (stiffness)
• Muscle weakness – often develops in
hands and feet first
• Brisk reflexes
Progressive Bulbar Palsy – 25%
• Affects bulbar muscles which control
speech, swallowing and chewing
• Gradual loss of speech
• Swallowing problems – food and saliva
• Leads to muscle weakness – especially
upper limbs/neck/shoulder
Progressive Muscular Atrophy – 10%
• Lower motor neurones
• Starts in small muscles of the hand
• Muscle wasting
• Fasciculation
Primary Lateral Sclerosis - Rare
• Only upper motor neurone damage
• Characterised by muscle weakness,
stiffness of limbs, and increased reflex
response
• Affects men predominantly – twice as
often as women
• Onset usually after 50 years of age
• Survival similar to normal lifespan
Symptoms
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Muscle wasting and
weakness
Fasciculation (twitching)
Cramps
Spasticity (stiffness)
Respiratory problems
Speech and swallowing
problems
Saliva and mucus problems
Weight loss
Fatigue
Pain
Emotional Lability
Psycho-social aspects
Cognitive changes
Rarely affected
• Senses: touch, taste, sight, smell
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and hearing
Bowel and bladder function
Sexual function and sexuality
Eye Muscles
Heart muscles
Who gets
MND?
Causes
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The cause of MND is thought to be a
combination of lifestyle, environmental and
genetic factors. These factors accumulate
throughout a person’s life.
However, the evidence obtained in studies
about the above factors has often been
conflicting or circumstantial and there are no
clear conclusions (e.g. High levels of
exercise, mechanical trauma).
Lifestyle factors could have a cumulative
effect in weakening nerve.
Although some knowledge about different
factors is available the cause is basically yet
unknown!
Key facts to underpin knowledge
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MND is not contagious
90% - 95% of people have the sporadic (random)
form
There is however a 5 - 10% familial (genetic link)
pattern
Can affect adult at any age but most are over 40 with
highest numbers between 50 – 70
Men are affected twice as often than women
Precise figures of incidence and prevalence are still
uncertain.
Incidence (may develop) is approx 2 per 100,000
Prevalence (actually have) is between 5 – 10 in 100,
000
How is MND
diagnosed?
Diagnosis
• No specific test
• Elimination of other causes
• GP may see MND once in career
• Other conditions often diagnosed first
• Examination and tests required by a
Consultant Neurologist
• Often takes over a year to receive
diagnosis
The journey
towards death
The Caring Role
So what issues
might you face?
Moving & Handling
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How would you
change your caring
routine to respond to
the fatigue that a
person with MND
experiences?
Why is it important to
believe a person with
MND when they say
they can’t do
something?
Toileting / Bathing
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MND does not usually
cause incontinence.
What other problems
though might you
encounter around
continence?
What issues might
you encounter helping
someone who can’t
move on their own
use the toilet?
Communication
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Where would you sit or
stand?
How much time would
you need?
Where would you look?
What would you do if
you didn’t understand?
Eating & Swallowing
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How would you help a
person who has
difficulty chewing &
swallowing?
What things can be
done to help control
saliva and excessive
secretions?
Clues to respiratory muscle
involvement in MND
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Breathlessness on
exertion or lying down
Poor Sleep
Excessive daytime
sleepiness
Headaches on
awakening
Excessive nocturnal
sweating
Cognitive Change
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MND has been traditionally
viewed as a disease
affecting the motor system
with no compromise of
cognitive abilities.
Recent research shows
that 25% or more show
some cognitive changes in
the frontal lobe region
3-5% will have frontotemporal dementia (FTD)
Characteristics of FTD
Significant personality change
“they are not the same person”
Disinhibition – socially undesirable,
inappropriate behaviour
Impulsivity – acting without thinking.
Inability to delay gratification
Perseveration – continuing to conduct
activity inappropriate to present situation
Impaired verbal expression and initiation
Withdrawn – failure to initiate activities apathy
Psychological
& Emotional
Aspects
Issues to consider
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Fatigue
Social Isolation
Frustration / Anger
Loss of identity
Inability to communicate
Emotional Lability
Cognitive Change
Carer issues
Palliative
Services
MND
Nurse
Resp
Service
Dietician
MNDA
Therapy
Team
RCA
AVs
Mental Capacity Act 2007
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The Mental Capacity Act 2005 came into force during 2007.
A number of government departments sponsored its
implementation: Ministry of Justice, the Department of
Health, the Office of the Public Guardian and the Welsh
Assembly Government.
The MCA says that a person is unable to make a particular
decision if they cannot do one or more of the following
four things:
– understand information given to them
– retain that information long enough to be able to make
the decision
– weigh up the information available to make the decision
– communicate their decision.
Mental Capacity Act 2007
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At the heart of the MCA in terms of concepts and values
are the five ‘statutory principles’.
Consider the five principles as the benchmark – use them to underpin all
acts done and decisions taken in relation to those who lack capacity. In
doing so, you will better empower and protect individuals who lack
capacity. It is useful to consider the principles chronologically: principles
1 to 3 will support the process before or at the point of determining
whether someone lacks capacity. Once you’ve decided that capacity is
lacking, use principles 4 and 5 to support the decision-making process.
Principle 1: A presumption of capacity
Principle 2: Individuals being supported to make their own
decisions
Principle 3: Unwise decisions
Principle 4: Best interests
Principle 5: Less restrictive option
Safeguarding &
The Vulnerable Adult
‘Social Care has an important role in the protection of members
of the public before harm has happened and after it has
happened’ (No Secrets, 2009)
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Those who need safeguarding help are often elderly and frail,
living on their own in the community, or without much family
support in care homes.
Safeguarding encompasses six key concepts: empowerment,
protection, prevention, proportionate responses, partnership
and accountability. Social care organisations play an
important role in the protection of members of the public from
harm and are responsible for ensuring that services and
support are delivered in ways that are high quality and safe.
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Safeguarding &
Self Neglect
Capacity is a highly significant factor in both understanding
and intervening in situations of self-neglect
There are a wide range of perspectives that inform
professionals’ understanding of self-neglect.
There are tensions between respect for autonomy and a
perceived duty to preserve health and wellbeing. The former
principle may extend as far as recognising that an individual
who chooses to die through self-neglect should not be
prevented from doing so; the latter may engage the view that
action should be taken, even if resisted, to preserve an
individual’s safety and dignity.
The autonomy of an adult with capacity is likely to be
respected, and efforts directed to building and maintaining
supportive relationships through which services can in time be
negotiated.
DOLS –
Deprivation of Liberty Safeguard
• The Deprivation of Liberty Safeguards are an
amendment to the Mental Capacity Act 2005.
They apply in England and Wales only.
• The Mental Capacity Act allows restraint and
restrictions to be used – but only if they are in a
person’s best interests.
• Extra safeguards are needed if the restrictions
and restraint used will deprive a person of their
liberty. These are called the Deprivation of
Liberty Safeguards.
DOLS –
Deprivation of Liberty Safeguard
• The Deprivation of Liberty Safeguards can only
be used if the person will be deprived of their
liberty in a care home or hospital. In other
settings the Court of Protection can be asked if a
person can be deprived of their liberty.
• Care homes or hospitals must ask either a local
authority/Northern Ireland health and social care
trust or health body if they can deprive a person
of their liberty. This is called requesting a
standard authorisation.
The
Motor Neurone
Disease
Association
National Helpline
Information
Financial Support
The MNDA will consider
applications from
professionals (following
assessment) if • Equipment or service has
not been provided by
statutory service, or does
not fall within their
criteria or responsibility.
• Unacceptable delay in
statutory provision. We
may bridge the gap.
• Provision is a ‘Quality of
life intervention’.
Equipment Loan
The MNDA will consider
loaning the following
pieces of equipment
through an application to
the Financial Support
Team from a professional
(following assessment)
• Lite-writers
• Riser Recliner Chairs
• Suction Units (£50
Charge)
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Support & advice to
people with MND, carers
& their families.
Manage local Association
Visitors (Volunteers).
Act as facilitators and
educators around MND.
Work with local statutory
services to influence and
improve care provision.
Regional Care Development Advisors
Regional Support
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Monthly Support / Garden Centre
Meetings
Offer Financial Support to those
affected by MND.
Finance local Association Visitors
• ‘Carers / Families & Friends’
Support Group
• Fund Educational Events
Regional Support
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Support & advice to
people with MND, carers
& their families.
Manage local Association
Visitors (Volunteers).
Act as facilitators and
educators around MND.
Work with local statutory
services to influence and
improve care provision.
‘Association Visitors’
Local Volunteer visitors
Regional Support
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