1. MOTOR NEURONE DISEASE AND PALLIATIVE CARE- AN EXAMPLE OF GOOD PRACTICE
Intro. Personal
How MND sits with Palliative care

2. WHAT IS A MOTOR NEURONE?
Motor neurones are the neural link, which innervate the muscles that enable us to move, speak, breathe and swallow. With no nerves to enervate them they will weaken and waste.
In MND effects only skeletal muscles, smooth muscles stay intact, bladder and bowel function remain intact

3. MND – A PROGRESSIVE NEUROMUSCULAR DISORDER
Progressive degeneration of motor neurons in the brain, brainstem and spinal cord
Three main types of MND
ALS (Amyotrophic Lateral Sclerosis) 50%: weakness and stiffness
PMA (Progressive Muscular Atrophy) 25%: weakness, wasting and twitching,
PBP (Progressive Bulbar Palsy) 25%: initially swallowing and speech impairment

4. MND
PLS(Primary Lateral Sclerosis), very rare, affecting speech, lower limbs with normal life expectancy
Often a combination of upper and lower motor neurones
Different presentations
Most common: mixed, with slow onset, one sided weakness
‘Man in a barrel’
Different presentations
‘Man in a barrel’ (prob.with swallowing, mobility, vision, poor core stability)
Most common: mixed, with slow onset, one sided weakness
Hence spasticity if upper Motor Neurones involved

5. MND Age related disease 7:100,000 2:1 male/female
Average life expectancy appr.18 month to 5 years from diagnosis
Poor prognosis if older at diagnosis or bulbar symptoms from onset of disease
Often lengthy process and numerous investigations before diagnosis, due to variety of symptoms
On the increase due to increasingly aging population
Oldest person 88, died within 3 month of diagn.

6. EARLY SIGNS Often missed Patient reports being ’clumsy’
‘Tripping over’
Occ. Falling
Increasingly struggling to get up stairs, but nothing very specific
Appears ‘normal’ in elderly population
Mistaken for CVA, OA

7. COMMON PRESENTING PROBLEMS
Muscle wasting, weakness
Muscle fasciculation
Bulbar involvent - Saliva management,
dysphagia, dysphasia- Risk of aspiration
Pain
Tonal changes: rigidity, spasticity
2. Tongue,
leading to further complications due to poor nutrition
Pain – loss of muscle power and musculo-skeletal’scaffolding’
Body sinking into itself causing muscular pain or neurogenic pain

8. COMMON SYMPTOMS Loss of mobility - movement Poor postural support
Complex seating and manual handling problems
Excessive secretions
Respiratory failure, mechanical, due to diaphragmatic involvement
Neurological deficit
Oral hygiene problems
Eventually totally unable to move ( brain stuck on a ’useless’ body )

9. MND Patient and Family Primary Care Intermediate Care Secondary Care
Social Services
Partnership working

10. PROFESSIONALS INVOLVED?
Primary care: GP, X-Ray, Physiotherapy,D/N, Dentist
Consultants:3-4,eventually Neurologist
Intermediate Care Teams :Rehab-OT,PT, Soc.Worker etc, Support Workers
Hospital:OT,PT,SALT,Dietician,Surgical Appliances, Respiratory Nurse, Hospital Soc.Worker
Social Services:Soc.Worker, Carers
3, OT, Pt, Soc Workers, Support workers

11. WHAT DOES THE PATIENT FEAR MOST?
Will I choke to death?
How will I die?
Loss of role
Loss of independence
Loss of communication
3, family and work

12. HOW IT ALL BEGAN
Working in MDT with patients with progressive neurological conditions:
Progressive Supranuclear Palsy, MS
Multiple Systems Atrophy
Motor Neurone Disease
Cortico-Basilar Degeneration

13. WHY SET UP LOCAL SUPPORT GROUP?
More common neurological conditions have ‘specialist clinics’, i.e.MS, PD
Late stage MND can’t travel easily
On average 6-7 patients with MND in locality
Taken ‘clinic’ into community setting

14. BENEFITS OF LOCAL GROUP
MND is a ‘rare’ condition
Forward planning is essential component
Primary care service lacks expertise and confidence to deal with complexity of symptoms
Historically PwMND seen by Physiotherapists in acute or rehab setting
Emphasis on ‘rehab’ sending wrong message
YOU CANNOT STRENGTHEN AND REGENERATE LOST MOTORNEURONES,
REHAB NEEDS TO FOCUS ON MEANUNGFUL ACTIVITY TO MAXIMISE FUNCTION AND PRESERVE MUSCLE ACTIVITY FOR AS LONG AS POSSIBLE

15. CARMARTHENSHIRE MND SUPPORT GROUP
MISSION STATEMENT:
To provide easily accessible and equitable services across Carmarthenshire for people with MND
Prior to that a group of HCP used to meet to discuss and review patients informally
Patients are given contact numbers and advised who to contact,
Database is kept at Ty Bryngwyn
Avoid ‘fragmented’ service

16. UNDERPINNING VALUES MNDA Standards of care
NSF for longstanding neurological conditions
Gold Standard Framework in Palliative care

17. KEY PRINCIPLES
Management of disease determined by needs and wishes of the person
Flexibility and speed in response to referral
Timely access to clinical intervention
Continuity of care throughout the process of the disease
Co-ordination and co-operation between service providers (partnership working)

18. AIMS AND OBJECTIVES OF GROUP
To establish a care pathway in order to map out current service with a view to achieving
Management of the disease determined by the needs and wishes of the person with MND, their families and carers
Flexibility and ‘fast-tracking’ in response to referral
Continuity of care throughout disease process
Co-ordination and co-operation between service providers (partnership working)
Regular monitoring and review

19. ‘AIM AND OBJECTIVES’ -CONTINUED
To have a forum to discuss issues/ or case review to ensure to keep up with current trends, update on information and drug trials
The membership of this group is open to interested professionals who have an involvement with MND patients and who agree to work within the statement set out above
To ensure ‘best practice’ for the benefit of the patient

20. WHY BECOME INVOLVED EARLY?
Forward planning, ‘staying one step ahead’, to facilitate effective ‘timely intervention’
Encourage patients to accept carers at a time when communication is not impaired in order to establish a good rapport
1, recognise when NIV must be considered, PEG, Powered wheelchair
2, which deodorant you like, very personal and intimate procedures

21. STAY ONE STEP AHEAD! To minimize unnecessary complications
Monitor retention of CO2,
NIV, and respiratory support
Communication aids
Monitor nutrition. Weight loss
PEG
Complex postural management – pressure management
If breathless, O2 not the answer as prob is mechanical,
diaphragmatic involvement

22. STAY ONE STEP AHEAD! Complex manual handling TSE – pain management
Liaise with ALAC for mobile arm support
Liaise with ALAC for provision of powered w/chair
Provision of collars and orthotics, or custom made support to prevent painful contractures
TSE - Trans Spinal Electro Analgesia

23. RESPIRATORY FAILURE Mechanical failure and not due to lung disease
Excessive use of auxilliary muscles
Dyspnoea on exertion
Dyspnoea at rest
Sleeping with several pillows
Ineffective inspiratory/ expiratory effort
2, O2 not the answer

24. RESPIRATORY FAILURE Paradoxical movement of abdominals
Headaches during the day
‘Catnapping’
Disturbed sleep
Reduced appetite
Poor concentration and cognitive changes

25. ‘EIGHT OUT OF TEN AIN’T BAD’
Occupational Therapist
Speech and Language Therapist
Physiotherapist
Palliative Care Service (Consultant and Macmillan CNS)
Respiratory Service
Dietician
RCDA
Neurological link
No care manager
No Soc Worker

26. WHERE ARE WE NOW?-VISION FOR THE FUTURE
Meet every six weeks
Monitor and up-date on patients
Plans to publish leaflet
Plans to develop ICP
Ongoing training of care staff in Hospice
‘Learning from each other’

27. CASE STUDY – D J 61 y/o male Farmer, living with wife
On diagnosis: speech problems, reduced mobility, rigidity,
Reluctant to accept intervention and
Refused hospital bed or carer support
Eventually allowed OT and Physio in
One son in university, living private isolated life, difficulty accepting changes, living in very old farmhouse

28. Cont. ‘CASE STUDY’ Within four month deteriorated significantly
Accepted carers, equipment
Communication now only with alphabet board
Accepted medication for tonal changes to maintain transfers with ‘Rotunda’

29. Cont. ‘CASE STUDY’ Increasing swallowing problems
Increasing problems to protect airways and at risk of aspiration
Poor respiratory effort
Discussion around PEG, NIV
Refused all, but agreed to be treated with antibiotics if indicated

30. Cont. ’CASE STUDY’ Expressed wish to die at home
Liaised with GP and Acute Response Team regarding his request to avoid ‘emergency admission’ and unwanted intervention
Died at home, 8 month after diagnosis

31. TO SUMMARISE:
Early referral to specialist team essential – central database
Patient knows who to contact
Patient is seen by HCP with experience in MND
MDT approach to ensure ‘best practice’

32. THANK YOU FOR LISTENING
ANY QUESTIONS?