Motor Neurone Disease
• http://www.youtube.com/watch?v=R0cUwIvO
Lfo
• Words to describe motor neurone disease
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Progressive
Incurable
rare
Group of related diseases
Motor neurones are affected
Upper and lower limb weakness
Speech and swallowing difficulties
Breathing difficulties
How it presents
• Often insidious onset clumsiness mild
weakness slurred speech
• There is no definitive symptom which leads to
diagnosis
• Does not usually affect the senses or the
bowels or bladder
• Effects differ greatly between individuals
regarding symptoms, severity, rate and
progression and survival time.
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Over 40 usual age between 50 and 70
Men slightly more than women
2 per 100 000
Cause remains a mystery
Different Types
• Lots of overlap
• Classified
1) in terms of the motor neurones affected
2) Symptoms
Bulbar –refers to face/speech/swallowing
Amyotrophic lateral sclerosis (ALS
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Most common
Upper and Lower motor neurones affected
Weakness and wasting of the limbs
Average life expectancy 2-5 years from onset
of symptoms
Progressive bulbar palsy
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Quarter of people
Upper and Lower motor neurones involved
Slurring speech/difficulty swallowing
Life expectance between 6 months and 3
years from diagnosis
Progressive Muscular atrophy
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Small number of people 5%
Lower motor neurones
Sometimes slower progression
Early symptoms are sometimes weakness and
clumsiness in the hands
• Most people live for more than 5 years from
diagnosis
Primary Lateral Sclerosis
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Rare 0.5%
Affects only upper motor neurone
Mainly weakness in the lower limbs
Can get symptoms in the hands or slurred
speech
• Life span could be normal
• Can develop into ALS
Motor neurone disease: real story - YouTube
Tests
• Bloods see if raised CK – can be raised in MND but
not diagnostic
• EMG – Taken from each limb and the bulbar(throat)
muscles- abnormal in MND as the electrical activity
of the muscles is changed
• Nerve conduction tests
• Transcranial magnetic stimulation – tests upper
motor neurones
• MRI – eliminates other diseases
• May do Lumbar puncture or muscle biopsy
Treatment
• Riluzole
• Beneficial prolongs survival of people with
MND
• Slows down the progress by a few months
• Inhibits the amount of Glutamate released in
nerve impulses
• Glutamate is a neurotransmitter in excess
causes nerve damage
• Respiratory support
• NIV
• This provides the most pressure when breath
in when breath out still have positive pressure
to splint the airway open
• Extends survival increase QOL
• Medications for symptoms
• Muscle cramps – Carbamazepine and
Phenytoin
• Muscle Stiffness – Muscle relaxants
Botox and intrathecal baclofen
• Drooling – Hyoscine, Glycopyrrolate,
atropine
• Pain – usual analgesia/Gabapentin
Symptomatic treatment
• Physio
• SALT – communication aids, swallowing
difficulties
• OT
• Motor neurone disease - Treatment - NHS
Choices
What are the issues here ?
If you were a patient with MND what would be
of importance to you?
• Advance Decisions
• Carers issues
Advanced decisions
• Over 18 and have capacity can appoint lasting power of
attorney
• Person appointed can make decisions about medical
treatment when the individual lacks capacity
• If person being cared for wishes to refuse a treatment in the
future should make an advance decision
• Must have capacity when they make the advanced decision
• LPA only overides advanced decision if made after the
advanced decision and gives authority to do so
• Advanced decision can be made verbally or in writing
Details that should be provided on a
written statement
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Full details of the person you act for and who is making the statement, including
their date of birth, home address and any distinguishing features (so that an
unconscious person, for example, could be identified).
The name and address of their general practitioner (GP).
Whether the GP has a copy of the statement.
Something to say that the decision is intended to have effect if the person you're
looking after lacks the capacity to make treatment decisions.
A clear statement of the decision, specifying the treatment to be refused and the
circumstances in which the decision should be used or which will trigger a
particular course of action.
The date the document was written and, if appropriate, the date it was reviewed.
The person’s signature. If the person can't write, they must give authority to
somebody else to sign on their behalf in their presence.
A signature from a witness to the above.
Life saving treatment
• If the person you're looking after has made an advance decision to refuse
life-sustaining (or life-saving) treatment in the future, then the document
must be in writing and signed by:
• the person you're looking after (or in addition, someone they have
authorised to sign on their behalf and in their presence), and
• a witness to the signature of the above.
• The advance decision must then also include a specific statement by the
person you're looking after which:
• clearly states that it relates to the treatment they have specified “even if
life is at risk”, and
• is signed in the same way in the presence of a witness who must also sign
the statement.
• Whether treatment is life sustaining or not depends not only
on the type of treatment but also on the individual
circumstances in which it is prescribed.
• For example, in some cases, giving antibiotics to the person
you're looking after may help to keep them alive, whereas in
other situations the same medication might be prescribed to
treat conditions that aren't life-threatening.
• The person you're looking after cannot make an advance
decision refusing measures necessary to keep them
comfortable, such as warmth, shelter and hygiene.
• What you can’t use an advance decision for
• You can’t use an advance decision to:
• ask for specific medical treatment, or anything that is
against the law, like requesting help to commit
suicide
• say you want someone else to decide what
treatment you should have
• if you want to choose someone to make decisions
about your treatment you have to make what is
called a ‘Lasting Power of Attorney’
• Advanced statement general statement of
values and beliefs that may be helpful in the
future particularly with care
• Carers Assessment - Mutual Caring - YouTube
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A carer is someone of any age who provides unpaid support to family or friends who could not
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manage without this help. This could be caring for a relative, partner or friend who is ill,
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frail, disabled or has mental health or substance misuse problems.
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There are six million people in the UK looking after a loved-one who is sick, disabled,
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suffering from a mental health problem or an addiction - some as young as five years old.
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What carers have in common is the selflessness to put their family member needs before their
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own, but they face an on-going life of isolation, ill-health and poverty.
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General information:
Carersuk.org
http://www.carers.org
NHS choices
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Money issues
Carer's allowance - aged over 16y and care more than 35h a week. And not in full-time
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education >20h/week and not earning over £100/wk
Get supplement on top of your other benefits if entitled.
£55/week
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Assessment for care
Directly ask social services for assessment
Personalised budgets
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Breaks
Respite care
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Support
Barnsley CArers advice line Telephone: 01226 785 858
From GP
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What we should be doing
Supporting carers guide:
http://www.rcgp.org.uk/pdf/Carers%20Action%20Guide.pdf
eg depression screening, flu vaccinations, scheduling appointments