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@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 > 10 years Most common age of onset: 50-60s Gender: Men affected slightly more frequently (2:3 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) • <10% 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/ Information>National 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 • Musculoskeletal pain • Cramp/spasm pain • Skin pressure pain • Physiotherapy and passive movement • Massage • Hydrotherapy • Use of splints and cushions • Medications (initially non-narcotic analgesics, anti-inflammatory and anti-spasticity agents) • Opioids

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. 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) Placement of PEG * 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