2. What is MND? What is MND? Colin Pearson Regional Care Development Advisor

3. Motor Neurone Disease 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

4. So what is a Motor Neurone?

6. Structure of a motor neurone

7. So what does a Motor Neurone do?

8. Function of Motor Neurones

9. Upper motor neurones originate in the base of the cortex of the brain Lower motor neurones originate in the spinal cord

12. Function of Motor Neurones

13. So what happens in Motor Neurone Disease?

14. The disease process

15. Types of Motor Neurone Disease

16. Amyotrophic Lateral Sclerosis – 65% Upper and lower motor neurones affected Spasticity (stiffness) Muscle weakness – often develops in hands and feet first Brisk reflexes

17. 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

18. Progressive Muscular Atrophy – 10% Lower motor neurones Starts in small muscles of the hand Muscle wasting Fasciculation

19. 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

20. Symptoms 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

24. Rarely affected Senses: touch, taste, sight, smell and hearing Bowel and bladder function Sexual function and sexuality Eye Muscles Heart muscles

25. Who gets MND?

26. Causes The cause of MND is thought to be a combination of lifestyle, environmental and genetic factors. These factors accumulate throughout a persons 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!

27. Key facts to underpin knowledge 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

28. How is MND diagnosed?

32. 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

33. The journey towards death The Caring Role

34. So what issues might you face?

35. Moving & Handling 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 cant do something?

36. Toileting / Bathing MND does not usually cause incontinence. What other problems though might you encounter around continence? What issues might you encounter helping someone who cant move on their own use the toilet?

37. Communication Where would you sit or stand? How much time would you need? Where would you look? What would you do if you didnt understand?

38. Eating & Swallowing How would you help a person who has difficulty chewing & swallowing? What things can be done to help control saliva and excessive secretions?

39. Clues to respiratory muscle involvement in MND Breathlessness on exertion or lying down Poor Sleep Excessive daytime sleepiness Headaches on awakening Excessive nocturnal sweating

40. Cognitive Change 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 fronto- temporal dementia (FTD)

41. 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

42. Psychological & Emotional Aspects

43. Issues to consider Fatigue Social Isolation Frustration / Anger Loss of identity Inability to communicate Emotional Lability Cognitive Change Carer issues

44. AVs MNDA Dietician MND Nurse Palliative Services Therapy Team RCA Resp Service

45. Mental Capacity Act 2007 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.

46. Mental Capacity Act 2007 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 youve 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

47. 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) 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.

48. 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 individuals 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.

49. 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 persons 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.

50. 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.

51. The Motor Neurone Disease Association

52. National Helpline

53. Information

54. 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.

55. 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)

56. Regional Support Regional Care Development Advisors Regional care development advisers: act as educators, facilitors and catalysts work closely with local statutory services and community care providers to ensure effective support for people affected by MND have detailed knowledge of the care and management of MND manage Association visitors (AVs) in their respective areas offer support and advice to people with MND and their families in complex situations 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.

57. Regional Support 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

58. Regional Support Association Visitors Local Volunteer visitors 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.