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Caring for People Living With Motor Neurone Disease

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May be helped by physiotherapy and medication Pulling ... electrical wires of the human body Motor neurones control the muscles used in voluntary movement ... – PowerPoint PPT presentation

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Title: Caring for People Living With Motor Neurone Disease


1
Caring for People Living With Motor Neurone
Disease
  • Dr Monika Wilson
  • ReConnections Counselling Service
  • www.reconnectionscounselling.com
  • reconnections_at_monikawilson.com.au
  • 0428 777809 / 5457 3329

2
  • Umbrella term a group of diseases
  • First described by Jean-Martin Charcot in 1869
  • The cause of MND is still unknown and there is
    currently no cure

3
  • A progressive neurological disease
  • motor nerve cells (neurones) degenerate and die
  • muscles for voluntary movement, speech, breathing
    and swallowing gradually weaken and waste
  • no nerves to activate them
  • patterns of weakness vary from person to person

4
What are Motor Neurones?
  • Neurones are a network of nerve cells that are
    the electrical wires of the human body
  • Motor neurones control the muscles used in
    voluntary movement
  • Motor neurones -messages to muscles
  • Sensory neurones- messages to the brain

5
Types of Motor Neurones
  • Upper motor nerves (UMN)
  • from motor cortex
  • along spinal cord
  • connect with LMN
  • Lower motor nerves (LMN)
  • in spinal cord (anterior horn cells)
  • take message to muscles

6
  • Incidence Approx 1 in 37,500 people diagnosed
    each year
  • Prevalence Approx 1400 in Australia / 350-400 in
    Qld ?
  • Each day more than one person dies from MND and
    another is diagnosed
  • Duration Average 2 to 3 years, but 10 live gt 10
    years
  • Most common age of onset 50-60s
  • Gender Men affected slightly more frequently
  • (23 ratio)

7
  • 90 sporadic, 5-10 familial
  • amyotrophic lateral sclerosis (ALS)
  • 65 UMNs and LMNs
  • progressive bulbar palsy (PBP)
  • 25 LMNs
  • progressive muscular atrophy (PMA)
  • lt10 LMNs
  • primary lateral sclerosis (PLS)
  • rare UMNs

8
Not Affected
  • Muscles controlling bladder and bowels
  • not directly affected
  • Hearing, taste, smell and sensation
  • sensory nerves
  • Heart
  • autonomic nerves

9
Diagnosis
  • Difficult to diagnose
  • Mimics many other diseases
  • Tests to exclude other conditions
  • blood tests
  • electromyography (EMG)
  • nerve conduction tests
  • transcranial magnetic stimulation (TMS)
  • Xray
  • CAT scan / MRI
  • Muscle biopsy
  • Lumbar puncture

10
  • Rapidly changing physical abilities
  • Decreasing capacity of carer over time
  • Increasing levels of support and care required
  • Emotional and psychological demands of caring and
    being cared for
  • MND affects each person differently, the rate of
    progression varies and our caring strategies need
    to be flexible and creative

11
Multidisciplinary CareFact Sheets EB2/EB3
  • Multidisciplinary care
  • Health care professionals being knowledgeable
    about MND
  • Flexible, coordinated professional support
  • Referrals in a coordinated way
  • Regular review/assessment of symptoms
  • Opportunities to get specialist advice
  • Key worker role

12
RiluzoleFact Sheet EB4
  • Anti-glutamate medication (Rilutek)
  • Blocks the release of glutamate from nerve cells
  • May cause weariness, nausea, dizziness
  • Research prolongs median survival by 2-3 months
  • Those taking riluzole early are more likely to
    remain in the milder stages of the disease for
    longer
  • PBS

13
Non-Invasive VentilationFact Sheet EB7
  • Provides breathing support (positive pressure)
  • Relief of symptoms - fatigue, breathlessness and
    disturbed sleep
  • Does not prevent weakening of the muscles
  • Research prolongs median survival up to 7 12
    months
  • Suitability / availability

14
GastrostomyFact Sheet EB8
  • Permanent feeding tube into the stomach
  • Improved nutrition and QoL
  • Early decision required
  • http//www.mndaust.asn.au/
  • InformationgtNational Information

15
Goals of Care
  • relief of symptoms
  • preservation of independence
  • quality of life
  • support
  • choice and control
  • information and education
  • dignity and respect
  • quality relationships
  • peaceful dying process
  • listening, acceptance, acknowledgement
  • minimise suffering
  • comfort

16
Common Symptoms
  • Symptoms experienced
  • weakness/ fatigue 94
  • dysphagia 90
  • dyspnoea 85
  • pain 73
  • weight loss 71
  • speech problems 71
  • constipation 54
  • poor sleep 29
  • emotional lability 27
  • drooling 25
  • Oliver, 2008

17
Muscles Lower Limb Weakness
  • Often begins with foot drop
  • Difficulty climbing stairs
  • Difficulty arising from chairs
  • Possibility of falls
  • Eventually leading to hoist
  • transfer
  • Fasciculation and cramps

18
Care Strategies
  • Ongoing assessment for equipment needs
  • Home modifications
  • Grab rails, chairs and beds on blocks, toilet
    raiser, shower chair, hoist
  • Ongoing assessment for
  • Movement and mobility
  • Transfer belt, walker, wheelchairs
  • Ankle / foot orthosis
  • Manage swollen limbs
  • Elevation, pressure stockings, recline chair,
    passive exercise, keep cool

19
Muscles Upper Limb Weakness
  • Hand weakness
  • difficulty with fine motor tasks using hands
  • Shoulder girdle weakness
  • difficulty using arms
  • Neck weakness

20
Care Strategies
  • Ongoing assessment for equipment needs
  • hand and body functional aids
  • alternative clothing
  • home modifications
  • Ongoing assessment for
  • Splints / orthotic devices / neck collars
  • Movement / light exercise
  • Care when transferring, esp shoulder joint
  • Massage, pressure garments, elevation

21
Maintaining Comfort
  • Repositioning
  • Subtle adjustments (small moves)
  • Satin sheets, kylies, bed stick
  • Support cushions
  • Care for weakened limbs

22
Discomfort Pain
  • Physiotherapy and passive movement
  • Massage
  • Hydrotherapy
  • Use of splints and cushions
  • Medications (initially non-narcotic analgesics,
    anti-inflammatory and anti-spasticity agents)
  • Opioids
  1. Musculoskeletal pain
  2. Cramp/spasm pain
  3. Skin pressure pain

23
Pain Management
  • Similar to other advanced diseases
  • Careful assessment of pain
  • Differing types of pain (cramps, spasticity,
    musculoskeletal discomfort)
  • Severity
  • Time course
  • WHO guidelines
  • Unique issues
  • Pain assessment with non-vocal plwMND
  • Impaired swallowing and PEGs

24
Creative Thinking
  • Need an effective way of calling for assistance
  • Door chimes
  • Jelly bean switches
  • Baby monitors
  • Intercom systems
  • Location of best position
  • Minimise anxiety

25
  • Physical body weakness, deterioration and
    immobility
  • Dysphagia (difficulty swallowing eating,
    drinking, saliva, choking, aspiration pneumonia)
  • Dysarthria (changes in speech volume, slurred,
    weakness, no communication)
  • Respiratory weakness (dyspnoea, orthopnoea,
    respiratory failure)

26
Muscles Bulbar Weakness
  • Drooling
  • Choking on thin liquids
  • Slurring of speech
  • Quiet voice
  • Loss of speech
  • Difficulty chewing and swallowing
  • Weight loss

27
Signs and symptoms of weakness in the muscles
involved in chewing and swallowing
  • making an extra effort to chew
  • coughing whilst eating or drinking or soon
    afterwards
  • needing several swallows for each mouthful
  • muffled or wet sounding voice after eating
  • eating or drinking appears tiring - the person
    may be breathless after a meal
  • meal times take longer
  • frequent chest infections - caused by food and
    liquid residue in the lungs
  • difficulty clearing saliva
  • Swallowing difficulties can lead to dehydration,
    malnutrition and constipation.

28
Muscles Swallowing
  • Dysphagia requires
  • Thorough and regular mouth care / hygiene
  • Regular assessment by speech pathologist and
    dietician
  • Maximise hydration and nutrition
  • Modify diet and consistency
  • Time over meals no distractions
  • Correct posture upright, chin tuck
  • Conscious swallowing, food positioning

29
Sialorrhoea Saliva beyond the margin of the lip
(drooling)
  • We produce approx 600 ml each day
  • Handling of saliva is affected due to
  • Weakness of the tongue
  • Weakness of throat muscles
  • Anatomical structure (poor lip seal)
  • Poor head control

30
The Impact of Drooling
  • Social participation
  • Withdrawal, embarrassment
  • Emotional wellbeing
  • Loss of independence and self esteem
  • Physical function
  • Speech
  • Swallowing
  • Oral health ie infection, odour
  • Dehydration

31
Saliva Care Thin
  • Strategies
  • Upright position
  • More conscious swallow
  • Wipes and clothing protection
  • Assisted cough technique
  • Natural remedies
  • Golden rod drops
  • Sage and hibiscus tea
  • Horseradish tablets
  • Medications
  • Glycopyrrolate
  • Amitriptylilne
  • Benztropine
  • Suction
  • Collar
  • Botox injections

32
Saliva Care Thick
  • Natural remedies
  • Dark grape, pineapple, apple or lemon juices
  • Papaya extract
  • Suck sugar-free citrus lozenges
  • Hydration
  • Reduce / eliminate alcohol, caffeine, dairy
    products
  • Nebulizer (with saline solution)
  • Steam inhalation
  • Mouth care products i.e. Biotene
  • Assisted cough technique

33
Complications
  • Aspiration pneumonia
  • Defined as the inhalation of either oropharyngeal
    or gastric contents into the lower airways
  • Due to poor swallow weakness or gag reflex
  • Reducing the risk
  • Elevate the bed
  • Peg tube
  • Avoid eating 1 to 2 hours before bedtime
  • Saliva control
  • Oral hygiene

34
Complications
  • Choking due to
  • Impaired respiration
  • Muscle spasm (laryngospasm)
  • Care strategies
  • stay calm
  • reassure person
  • wait for attack to pass
  • Seek advice from physiotherapist for assisted
    cough technique
  • Medications i.e. Morphine, Benzodiazepines
    Clonazepam (drops), Lorazepam (Ativan)

35
Nutrition Eating well
  • Speech pathologist / dietician to assess
  • Increasing dysphagia
  • Modified diet pureed food, thickened fluids,
    nutritional supplements, gravies
  • Positioning, use of equipment

36
Why Consider PEG?
  • Stabilise weight loss
  • Maximise nutrition and hydration
  • Maximum energy
  • Improve quality of life
  • Prevent choking on thin fluids (safety)
  • Prevent prolonged mealtimes (distress)
  • Reduce risk of aspiration

37
Placement of PEG
  • Early placement recommended
  • can be left un-used
  • use as a top up
  • some risks involved
  • Respiratory assessment
  • Should be inserted before vital capacity falls
    below 50 of predicted (for safe anaesthetic)
  • Latest research evidence suggests an
  • improvement in nutrition and QOL only

38
Muscles Speech
  • Dysarthria (motor speech disorder)
  • Slurred speech, quiet voice
  • Changes in vocal quality
  • Requires coordinated movement of several muscle
    groups
  • Speech pathologist to review and advise
  • Affects
  • Vulnerability
  • Isolation
  • Inability to express needs
  • Exclusion from decision making
  • Loss of independence and social role
  • Loss of self identity
  • Challenges relationships

39
Care Strategies
  • Key word of sentence first
  • First letter of word
  • Eye contact and signals
  • Gestures
  • Translation by carer
  • Letter / phrase chart
  • Yes/no questions
  • Be patient slow down

40
Communication Aids
  • Low tech aids
  • Writing
  • Magna doddle / white boards
  • Laser pointer and chart
  • Etran boards
  • High tech aids
  • Lightwriter / Polyanna / Alora
  • VMax
  • Essence Vantage Light

41
Fatigue
  • Most common symptom
  • Everything is exhausting
  • Rest following activities (smaller rest periods)
  • Small aids and equipment can help
  • Conserve energy
  • Be aware of insomnia
  • Visit in the pre-lunch hours
  • Bigger meals earlier in the day

42
Swelling
  • Due to lack of movement
  • Legs elevated with cushion support
  • Use of massage
  • Elastic stockings
  • Be aware of deep vein thrombosis

43
Bladder Bowels
  • Fasciculation may irritate the bladder
  • Hand weakness or mobility limitations
  • Use of pads
  • Uridomes
  • Catheter
  • Weak abdominal and chest muscles
  • Diet / hydration
  • Privacy
  • Require adequate fibre, fluid
  • Routine, comfort, aids
  • Laxatives

44
Emotional Lability pseudo bulbar effect
  • Unpredictable episodes of crying and laughing
  • Disease damages the area of the brain that
    controls normal expression of emotion
  • Anxiety and embarrassment, particularly in public
  • Explanation (part of the disease), reassurance
    (not going mad)
  • Medication in more severe situations

45
Cognitive Changes
  • previously thought cognition was not affected
  • research indicates up to 75 may have some
    frontal lobe dysfunction
  • 15 to 41 meet criteria for fronto-temporal
    dementia (FTD)
  • Miller others, 2009

46
Cognitive Changes
  • Cognitive Impairment (CI) deficits in attention,
    word generation, cognitive flexibility
  • Behavioural Impairment (BI) changes in social
    interaction
  • Fronto-temporal Dementia (FTD) altered social
    conduct, emotional blunting, loss of insight,
    language change, poor self care, emotional
    recognition, lack of empathy

47
  • Changes in decision-making
  • Reduced awareness of risk, concerns about risk
    taking
  • Frustration forgetfulness
  • Communication
  • Obsessional behaviour impulsiveness
  • Lack of self care

48
Issues for Professionals
  • Decision making
  • Assessment earlier to make decision but person
    may not want to discuss the issues
  • Communication
  • Unsure if discussion retained and able to be
    involved in the discussion
  • Assessing symptoms
  • Pain / depression / swallowing problems
  • Coping with memory loss / confusion

49
Care Strategies
  • Education for caregivers
  • Give simple directions
  • Establish a regular routine
  • Possible medical management
  • Cognitive and behavioural challenges in caring
    for patients with frontotemporal
  • dementia and ALS (2010). Amyotrophic Lateral
    Sclerosis, 11 298-302.

50
Muscles Respiratory
  • Disturbed sleep
  • Daytime sleepiness
  • Increased fatigue
  • Morning headaches
  • Quieter voice
  • Fewer words per breath
  • Shallow, faster breathing
  • Reduced movement of the rib cage or abdominal
    muscles
  • Excessive use of the muscles in the upper chest
    and neck
  • Weakened cough and sneeze

51
Respiratory muscle weakness can cause
  • Breathlessness (dyspnoea) even at rest
  • Breathlessness lying flat (orthopnoea)
  • Impaired concentration or confusion
  • Irritability and anxiety
  • Decreased appetite

52
Care Strategies
  • Be vigilant for symptoms
  • Refer to a specialist respiratory service for
    regular assessment
  • Avoid infections (people with coughs/cold)
  • Treat reversible causes of dyspnoea
  • Discuss NIPPV support
  • Avoid crisis situations
  • Improve ventilation fans, air flow, humidifier
  • Adjust room temperature
  • Reclined or fully upright position
  • Respiratory / breathing / relaxations exercises
  • Medications lorazepam, midazolam, morphine

53
Non-Invasive Positive Pressure Ventilation
  • The use of positive pressure to do some of the
    work of breathing
  • BIPAP (bi-level) or VPAP (variable)
  • Used overnight to improve symptoms
  • Does not prevent weakening of muscles

54
Benefits of Assisted Ventilation
  • Decreased daytime sleepiness
  • Better appetite
  • Rests fatigued respiratory muscles
  • Improved sleep
  • Quality of life
  • More energy
  • Improved defence against infections

55
Implications to Consider
  • Significant improvement in survival
  • Mask issues, intolerance
  • Costs, availability, accessibility, back up
  • Increasing dependency
  • Carer burden
  • Advance care planning (AHD/POA)
  • See NICE Clinical Guidelines for the use of
    non-invasive ventilation http//guidance.nice.org
    .uk/CG105

56
Advance Care Planning
  • 75 preferred early discussion of Advanced
    Directives Oliver, International Symposium, 2007
  • plwMND preferred that doctor initiates discussion
  • Communication issues
  • Ventilation withdrawal issues
  • Shown to change their preference for
    life-sustaining measures (e.g. ventilators) over
    a six month period Silverstein et al., 2006
    periodically re-evaluate AHD
  • Cultural differences

57
Six Triggers for Initiating Discussion About End
of Life Issues
  • The plwMND or the family asks - or opens the
    door for end of life information and/or
    interventions
  • Severe psychological and/or social or spiritual
    distress or suffering
  • Pain requiring high dosages of analgesic
    medications
  • Dysphagia requiring a feeding tube
  • Dyspnoea or symptoms of hypoventilation, a
    forced vital capacity of 50 or less is present
  • Loss of function in two body regions (bulbar,
    arms or legs)
  • Promoting excellence in end of life ALS care,
    2004

58
Use of Oxygen
  • Breathlessness is due to muscle weakness not low
    oxygen
  • If oxygen is given inappropriately it can
  • Increase carbon dioxide retention
  • Reduce the bodys spontaneous signals to breath
  • Put increased pressure on weakened muscles

59
Common Cause of Death
  • Most people die of respiratory failure
  • Without NIPPV
  • Choose not to
  • Intolerance
  • With NIPPV
  • Eventual failure or voluntary withdrawal
  • The duration between an acute deterioration and
    death is less then 24 hours
  • Choking rarely occurs
  • Neurvert, C, Oliver D Journal of Neurology 2001
    248

60
Other Causes of Death
  • Malnutrition and dehydration
  • Without peg
  • Refusal
  • Anatomical considerations
  • Respiratory status
  • With peg
  • Voluntary stopping
  • Intolerance or other complications
  • Aspiration pneumonia
  • Sepsis
  • Pulmonary embolus
  • Head injury/falls
  • Suicide
  • Co-morbidity

61
Terminal Phase is recognised by
  • Increased, progressive weakness
  • Deterioration over a few days
  • Often proceeded by
  • Reduction in chest expansion
  • Quietening of the breath sounds
  • Accessory muscles for breathing
  • Morning headaches

62
Medical Management during Terminal Phase
  • Use range of routes oral, peg, patch or
    continuous subcutaneous infusion (syringe driver)
  • Morphine or diamorphine to reduce
    pain/breathlessness
  • Lorazepam, Diazepam (Valium) or Midazolam, a
    sedative, to reduce agitation/restlessness
  • Glycopyrronium bromide to reduce the chest
    secretions and saliva (or hyoscine hydrobromide)
  • Ethically appropriate to sedate no
    muscle-paralyzing agents should be used
  • Used appropriately (start small increase) these
    medications will not hasten death

63
Withdrawal of Ventilatory Support
  • Major decision making as to when to cease
    ventilation
  • Education of what to expect
  • Comfort maintained
  • Physician should be present (established
    relationship)
  • Planned event no haste
  • Cultural or religious rituals discussed and
    planned
  • Location prepared
  • Medications ready
  • Subcutaneous route is preferred
  • Family and friends present

64
End of Life Care
  • Build up of carbon-dioxide will anesthetise
  • Step-process of withdrawal of NIPPV
  • Use of adequate medications
  • Support for the family and friends (bereavement)
  • Fear of choking (rarely occurs) and
    breathlessness
  • Increasing immobility
  • Discussions and anticipation of the final time
  • Advance care planning, directives and EPOA
  • done ahead of time
  • regular review

65
Features of Optimal End of Life Care
  • care plans and information are shared
  • adequate nursing cover
  • comprehensive symptom control
  • Psychological, social spiritual support
  • family and friends are providing practical
    support for the primary carer
  • the opportunity to find completion

66
Psychological, Emotional Social Issues
  • A spiralling series of progressive losses (grief)
  • Changes in ability to influence their external
    and internal environment (control)
  • Changed relationship with body/self/identity
  • Aware of what is happening, what will come and
    increasing dependency
  • Carer burnout, relationship issues
  • Many psychological, emotional, sexual, financial,
    spiritual adjustments to be made

67
Family Friends
  • Create relationships open and honest, ongoing
    communication, inclusion, non-abandonment
  • Family also have needs
  • Respite
  • Involvement in care
  • planning
  • Discuss fears and concerns

68
Health Care Professionals
  • Stretches the physical, emotional and spiritual
    resources of staff
  • Acknowledgement and support
  • Awareness of self reactions (buttons)
  • Flexible approach to care (share the care)
  • Remember self care and understanding of own loss,
    grief and death fatigue

69
Conclusion
  • Be aware of the unique challenges of caring for a
    person living with MND
  • Understand the disease and rapidly changing need
  • The disease is the problem, not the person
  • Early contact, relationship building
  • Ongoing, preemptive assessment and referral
  • Well coordinated teamwork

70
  • www.mndcare.net.au for information on MND care,
    symptom management and support for health
    professional
  • MND Aware online training modules
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