1. Motor Neurone Disease

2. http://www.youtube.com/watch?v=R0cUwIvO Lfo http://www.youtube.com/watch?v=R0cUwIvO Lfo

3. Words to describe motor neurone disease

4. Progressive Incurable rare Group of related diseases Motor neurones are affected Upper and lower limb weakness Speech and swallowing difficulties Breathing difficulties

5. How it presents Often insidious onset clumsiness mild weakness slurred speech There is no definitive symptom which leads to diagnosis Does not usually affect the senses or the bowels or bladder Effects differ greatly between individuals regarding symptoms, severity, rate and progression and survival time.

6. Over 40 usual age between 50 and 70 Men slightly more than women 2 per 100 000 Cause remains a mystery

7. Different Types Lots of overlap Classified 1) in terms of the motor neurones affected 2) Symptoms Bulbar –refers to face/speech/swallowing

8. Amyotrophic lateral sclerosis (ALS Most common Upper and Lower motor neurones affected Weakness and wasting of the limbs Average life expectancy 2-5 years from onset of symptoms

9. Progressive bulbar palsy Quarter of people Upper and Lower motor neurones involved Slurring speech/difficulty swallowing Life expectance between 6 months and 3 years from diagnosis

10. Progressive Muscular atrophy Small number of people 5% Lower motor neurones Sometimes slower progression Early symptoms are sometimes weakness and clumsiness in the hands Most people live for more than 5 years from diagnosis

11. Primary Lateral Sclerosis Rare 0.5% Affects only upper motor neurone Mainly weakness in the lower limbs Can get symptoms in the hands or slurred speech Life span could be normal Can develop into ALS

12. Motor neurone disease: real story - YouTube

13. Tests Bloods see if raised CK – can be raised in MND but not diagnostic EMG – Taken from each limb and the bulbar(throat) muscles- abnormal in MND as the electrical activity of the muscles is changed Nerve conduction tests Transcranial magnetic stimulation – tests upper motor neurones MRI – eliminates other diseases May do Lumbar puncture or muscle biopsy

14. Treatment Riluzole Beneficial prolongs survival of people with MND Slows down the progress by a few months Inhibits the amount of Glutamate released in nerve impulses Glutamate is a neurotransmitter in excess causes nerve damage

15. Respiratory support NIV This provides the most pressure when breath in when breath out still have positive pressure to splint the airway open Extends survival increase QOL

16. Medications for symptoms Muscle cramps – Carbamazepine and Phenytoin Muscle Stiffness – Muscle relaxants Botox and intrathecal baclofen Drooling – Hyoscine, Glycopyrrolate, atropine Pain – usual analgesia/Gabapentin

17. Symptomatic treatment Physio SALT – communication aids, swallowing difficulties OT Motor neurone disease - Treatment - NHS Choices Motor neurone disease - Treatment - NHS Choices

18. What are the issues here ? If you were a patient with MND what would be of importance to you?

19. Advance Decisions Carers issues

20. Advanced decisions Over 18 and have capacity can appoint lasting power of attorney Person appointed can make decisions about medical treatment when the individual lacks capacity If person being cared for wishes to refuse a treatment in the future should make an advance decision Must have capacity when they make the advanced decision LPA only overides advanced decision if made after the advanced decision and gives authority to do so Advanced decision can be made verbally or in writing

21. Details that should be provided on a written statement Full details of the person you act for and who is making the statement, including their date of birth, home address and any distinguishing features (so that an unconscious person, for example, could be identified). The name and address of their general practitioner (GP). Whether the GP has a copy of the statement. Something to say that the decision is intended to have effect if the person you're looking after lacks the capacity to make treatment decisions. A clear statement of the decision, specifying the treatment to be refused and the circumstances in which the decision should be used or which will trigger a particular course of action. The date the document was written and, if appropriate, the date it was reviewed. The person’s signature. If the person can't write, they must give authority to somebody else to sign on their behalf in their presence. A signature from a witness to the above.

22. Life saving treatment If the person you're looking after has made an advance decision to refuse life-sustaining (or life-saving) treatment in the future, then the document must be in writing and signed by: the person you're looking after (or in addition, someone they have authorised to sign on their behalf and in their presence), and a witness to the signature of the above. The advance decision must then also include a specific statement by the person you're looking after which: clearly states that it relates to the treatment they have specified “even if life is at risk”, and is signed in the same way in the presence of a witness who must also sign the statement.

23. Whether treatment is life sustaining or not depends not only on the type of treatment but also on the individual circumstances in which it is prescribed. For example, in some cases, giving antibiotics to the person you're looking after may help to keep them alive, whereas in other situations the same medication might be prescribed to treat conditions that aren't life-threatening. The person you're looking after cannot make an advance decision refusing measures necessary to keep them comfortable, such as warmth, shelter and hygiene.

24. What you can’t use an advance decision for You can’t use an advance decision to: ask for specific medical treatment, or anything that is against the law, like requesting help to commit suicide say you want someone else to decide what treatment you should have if you want to choose someone to make decisions about your treatment you have to make what is called a ‘Lasting Power of Attorney’

25. Advanced statement general statement of values and beliefs that may be helpful in the future particularly with care

26. Carers Assessment - Mutual Caring - YouTube

27. A carer is someone of any age who provides unpaid support to family or friends who could not manage without this help. This could be caring for a relative, partner or friend who is ill, frail, disabled or has mental health or substance misuse problems. There are six million people in the UK looking after a loved-one who is sick, disabled, suffering from a mental health problem or an addiction - some as young as five years old. What carers have in common is the selflessness to put their family member needs before their own, but they face an on-going life of isolation, ill-health and poverty. General information: Carersuk.org http://www.carers.org NHS choices

28. Money issues Carer's allowance - aged over 16y and care more than 35h a week. And not in full-time education >20h/week and not earning over £100/wk Get supplement on top of your other benefits if entitled. £55/week Assessment for care Directly ask social services for assessment Personalised budgets Breaks Respite care Support Barnsley CArers advice line Telephone: 01226 785 858 From GP What we should be doing Supporting carers guide: http://www.rcgp.org.uk/pdf/Carers%20Action%20Guide.pdf eg depression screening, flu vaccinations, scheduling appointments