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MND

MND. Kate Thomas GPST2 Manorlands Hospice. Outline. MND overview Case study. MND. Selective degeneration of motor neurones Anterior horn cells Motor cranial nuclei Devastating illness Progressive paralysis and eventual death from respiratory failure

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MND

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  1. MND Kate Thomas GPST2 Manorlands Hospice

  2. Outline • MND overview • Case study

  3. MND • Selective degeneration of motor neurones • Anterior horn cells • Motor cranial nuclei • Devastating illness • Progressive paralysis and eventual death from respiratory failure • Palliative care involvement from diagnosis

  4. Epidemiology • Incidence 2 per 100,000 • Prevalence 5-7 per 100,000 • 6th and 7th decades • M>F • Sporadic (90%) • Familial MND (10%)

  5. Classifications • Classic MND (65-85%) • Amyotrophic lateral sclerosis (ALS) • Mixed UMN/LMN signs • More rarely • Purely UMN or LMN picture • Primary lateral sclerosis • Progressive muscular atrophy

  6. Signs & symptoms ?

  7. Presentation

  8. McDermott C J , Shaw P J BMJ 2008;336:658-662

  9. Bulbar onset • Slurred speech • Wasting and fasciculation tongue • Dysphagia • Association with emotional lability • Respiratory onset • Hypoventilation • Dyspnoea, orthopnoea

  10. Case study • 89 ♂ • AGH (July ‘11) • 3/12 gradual decline, transient left sided weakness, impaired coordination, dysphagia, dysarthria • △△ stroke disease, progressive neurological disorder • CT head: mild invol. change • Rapidly became bedbound, unsafe swallow, incontinent, fasciculations • Dx rapidly progressive MND

  11. At diagnosis • “I’m failing”, “just existing” • Anxiety, fatigue, disturbed sleep • Declined PEG and ventilatory support, accepted NGT • “I don’t want to exist like this” • O/A Manorlands • Apnoeic episodes • Respiratory secretions • Family uncertainty

  12. Communication • 75-80% experience difficulty • Direct effects on muscle control, weakness • Other barriers e.g. Eating, position, distractions

  13. Strategies to improve communication • Time • Place • Mood • Listen • Maintain individuality • Silences, verbal and non-verbal cues • Acknowledge distress • Forward planning

  14. Communication aids • Pen and paper • Pencil grips, book holders, typing sticks/pointers, page turners • MedicAlert bracelet • Signalling systems • Communication boards • Mobile phone • Buzzer • Emergency call alarm

  15. High tech devices • Digitised communication devices • Specific pre-programmed messages • Digitised speech • Specialised phone equipment • Breath-activated hands-free phone, “Type-talk” • Laptop with “text to speech” • Environmental controller • Dedicated speech synthesiser • Eye controlled systems

  16. Nutrition and feeding • Malnutrition and weight loss associated with shorter survival • Enteral feeding • When 10% weight loss or BMI <18.5 • Via PEG, RIG or NGT • RIG preferred

  17. Respiratory symptoms • Weakness of ventilatory muscles • Dyspnoea, orthopnoea, hypercapnia • Salivary drooling • Aspiration pneumonia/pneumonitis • Ineffective cough • Anti-cholinergics, postural drainage, chest physiotherapy, suction • Lorazepam, opiates

  18. Ventilation • Alleviate sx of chronic hypoventilation • Improved quality of life • Median survival benefit 7/12 NICE 2010

  19. Anxiety, agitation • Grief reaction to diagnosis • Why me? • How long have I got? • Fears of manner of death • Will it affect my mind? • Will my children be affected? • Often most disabling at night • Environmental factors, reassurance, reversible causes, complementary therapy

  20. Riluzole • Riluzole • 3-4/12m prolongation of life • £3,742 per year Brockington A, Shaw P ACNR 3(5)

  21. Anti-oxidants • Oxidative damage by free radicals implicated in pathogenic process • Cochrane – no evidence for anti-oxidant therapy

  22. Other considerations • Advance directive • Discussion of resuscitation status • Power of attorney • Advocates • PPD • Communication with relatives, carers • Palliative care register

  23. Useful resources • MND Association • MND Scotland

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