1. Motor Neurone Disease Louise Rickenbach Regional Care Development Adviser Andrew Lane Group Leader and Association Visitor Portsmouth and SE Hants Group MND Association

2. MND is a progressive neurological disease that attacks motor nerve cells in the brain and spinal cord Rare, terminal and difficult to diagnose

3. Upper motor nerves originate in the motor cortex of the brain and brain stem. Carry information to Lower motor nerves originate in the spinal cord and brainstem nuclei Sensory and autonomic nerves are unaffected

4. Motor Neurone Disease Incidence: 2-3 per 100,000 Prevalence: 7 per 100,000 people in UK MS incidence : 3-4 per 100,000 MS prevalence: 80-120 per 100,000

5. Wembley Stadium holds just under 100,000

6. Hampshire population 1.7 million You would expect there to be 80-110 people living with MND in Hampshire. Perhaps 40 new cases per year About 50 on the Portsmouth list!

7. Prognosis

9. Average survival is about 2-5 years 30% die within one year of diagnosis, 50% within two years

10. 90% of people have the sporadic form (just occurs, no family history) Mainly an adult illness Age of onset 50-70 commonest 50% are under 65 i.e. of working age

11. Diagnosis No specific diagnostic test Clinical picture & elimination of other causes Mean delay in diagnosis of 1 year This has significant psychological consequences for the person with MND and can delay access to support.

12. Motor Neurone Disease types Amyotrophic lateral Sclerosis (ALS) Most common UMN & LMN Progressive Bulbar Palsy (PBP) UMN &UMN Progressive Muscular Atrophy (PMA) Mainly LMN Primary lateral sclerosis (PLS) Mainly UMN About 90%

13. Motor Neurone Disease types Amyotrophic lateral Sclerosis (ALS) Most common Progressive Bulbar Palsy (PBP) Progressive Muscular Atrophy (PMA) Primary Lateral Sclerosis (PLS) 2-5 yrs <2 yrs 5-10yrs >10 yrs Prognosis

14. 10% have Familial MND A genetic disease Gene X (2014)

15. Environmental Age Gender Diet? Toxins Electric Shock? Athleticism? Military Service? Infection? Genetic Susceptibility genes NF-H VEGF PER ELP3 UNC13A PON ATXN2 ZNF512B SigmaR1 C9orf72 Sporadic Causes?

16. What does Motor Neurone Disease affect ? You tell me some things....

17. Some of the Signs & 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 Fronto- temporal dementia (5%) Cognitive changes (30-40%)

18. Characteristics of Fronto- temporal Dementia in MND Significant personality change Apathy Loss of inhibition Impulsivity Perseveration Impaired often severe verbal expression and initiation Difficulty with learning new tasks Withdrawn

19. C9orf72 Discovered in two breakthrough studies in 2011 and now thought to be the most common genetic cause of fronto-temporal dementia. It accounts for 25% of the Familial MND cases and 4% of the sporadic cases.

20. Improving life expectancy? Respiratory Support Non invasive ventilation has shown to improve quality of life. Cough assist Improved nutrition PEG’s RIG’s PIG’s Multi-disciplinary Team Approach to care Riluzole

21. People with MND experience a series of relentless losses Sex Personal relationships Hobbies Job Role in the family Sport Socialising Ironing! Recently, I was told... Mealtimes Independence to choose activities Money

22. A little Game.....

23. How do you tell this to someone if you cant talk? My arm hurts What about this? What do you think about the Chinese investing in UK Nuclear Power Stations? But what if you can’t move either?

24. I am very scared of dying. How will you make sure that I am not in pain. I want to discuss my hopes for the future for with the children I don’t want tube feeding, do want antibiotics, not resuscitation but want to be ventilated!

25. Find out how the person communicates They and their families are usually the experts!

27. The majority of people with MND develop respiratory symptoms in the terminal phase

28. Respiratory changes SymptomsSigns Breathlessness Orthopnoea Recurrent chest infections Disturbed sleep Non-refreshing sleep Nightmares Daytime sleepiness Poor concentration and/or memory Confusion Hallucinations Morning headaches Fatigue Poor appetite Increased respiratory rate Shallow breathing Weak cough Weak sniff Abdominal paradox (inward movement of the abdomen during inspiration) Use of accessory muscles of respiration Reduced chest expansion on maximal inspiration (NICE)

29. Just in Case Box

30. Some useful medications Pain due to muscle cramps / spasticity Baclofen Tizanidine Benzodiazapines ( clonazepam) Care should be taken as these may alter mobility and ability to stand and transfer. Quinine sulphate Joint pain Simple analgesia, NSAIDs Neuropathic pain Tricyclic antidepressants, gabapentin., pregabalin

31. Some useful medications Nausea Antiemetics,levomepromazine haloperidol, cyclizine Excessive saliva Glycopyrronium bromide, hyoscine hrdrobromindide Anxiety Diazepam,midazolam,lorazepam Breathlessness Opiod analgesics morphine sulphate Oxygen should only be used with extreme caution as it can depress the respiratory centre

32. End of Life A guide for people with MND

33.  The Guideline covers MND care from referral and diagnosis, through to organisation of care, information and support, managing symptoms and planning for end of life.  It urges a joined-up approach where different specialists work together to improve overall quality of life.

34. Motor Neurone Disease: assessment and management Nice.org.uk/guidance/ng42

35. It is written for  Health and social care professionals.  Commissioners and providers of Health and social care services.  People with MND, their families and carers. Have a look

36. Portsmouth & SE Hants Group MND Association Local Care & support

37. Portsmouth & SE Hants Group MND Association Support people with MND, their families and carers Trained Association Visitors Coffee Mornings ( 1 st Monday of the month, 10.30-12.30) Quality of Life Grants

38. Portsmouth & SE Hants Group MND Association Andrew Lane (Group Leader & AV) Sue Tate (AV Coordinator) Carrie Swinburne (AV) Lorraine Jarvis (AV)

39. National Helpline Over 600 calls a month from people affected by MND and health and social care Professionals mndconnect 0808 8026262 mndconnect@mndassociation.org portsmouth@mndassociation.org www.mndportsmouth.org @MNDportsmouth and Facebook

40. MND Support Grants MND Care Grant Application to Support Service Team required Funding the provision of equipment or service MND Quality of Life Grant (up to £500) Funding to maintain/increase Quality of Life Apply to local Branch Carers Grant (up to £500) Young Persons Grant (up to £250) To support young carers/people affected by MND

41. Support Grants and equipment loan support.services@mndassociation.org Online application by HSCP’s 01604 611802 www.mndassociation.org/financialsupport

42. Join our Professionals’ Forum

43. Lend your expertise Share your knowledge Be part of the conversation proforum.mndassociation.org

44. Louise Rickenbach louise.rickenbach@mndassociation.org 0777 4268931 03453 751831 Thank you!