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Caring for People Living With Motor Neurone Disease Dr Monika Wilson ReConnections Counselling Service www.reconnectionscounselling.com [email protected] 0428 777809 / 5457 3329 Umbrella term – a group of diseases First described by Jean-Martin Charcot in 1869 The cause of MND is still unknown and there is currently no cure A progressive neurological disease: motor nerve cells (neurones) degenerate and die muscles for voluntary movement, speech, breathing and swallowing gradually weaken and waste no nerves to activate them patterns of weakness vary from person to person What are Motor Neurones? • Neurones are a network of nerve cells that are the electrical wires of the human body • Motor neurones control the muscles used in voluntary movement • Motor neurones -messages to muscles • Sensory neurones- messages to the brain Types of Motor Neurones Upper motor nerves (UMN) from motor cortex along spinal cord connect with LMN Lower motor nerves (LMN) in spinal cord (anterior horn cells) take message to muscles Incidence: Approx 1 in 37,500 people diagnosed each year Prevalence: Approx 1400 in Australia / 350-400 in Qld ? Each day more than one person dies from MND and another is diagnosed Duration: Average 2 to 3 years, but 10% live > 10 years Most common age of onset: 50-60s Gender: Men affected slightly more frequently (2:3 ratio) 90% sporadic, 5%-10% familial amyotrophic lateral sclerosis (ALS) 65% UMNs and LMNs progressive bulbar palsy (PBP) 25% LMNs progressive muscular atrophy (PMA) <10% LMNs primary lateral sclerosis (PLS) rare UMNs Not Affected Muscles controlling bladder and bowels not directly affected Hearing, taste, smell and sensation sensory nerves Heart autonomic nerves Diagnosis Difficult to diagnose Mimics many other diseases Tests to exclude other conditions: blood tests electromyography (EMG) nerve conduction tests transcranial magnetic stimulation (TMS) Xray CAT scan / MRI Muscle biopsy Lumbar puncture Rapidly changing physical abilities Decreasing capacity of carer over time Increasing levels of support and care required Emotional and psychological demands of caring and being cared for MND affects each person differently, the rate of progression varies and our caring strategies need to be flexible and creative Multidisciplinary Care Fact Sheets EB2/EB3 Multidisciplinary care: Health care professionals being knowledgeable about MND Flexible, coordinated professional support Referrals in a coordinated way Regular review/assessment of symptoms Opportunities to get specialist advice Key worker role Riluzole Fact Sheet EB4 Anti-glutamate medication (Rilutek) Blocks the release of glutamate from nerve cells May cause weariness, nausea, dizziness Research: prolongs median survival by 2-3 months Those taking riluzole early are more likely to remain in the milder stages of the disease for longer PBS Non-Invasive Ventilation Fact Sheet EB7 Provides breathing support (positive pressure) Relief of symptoms - fatigue, breathlessness and disturbed sleep Does not prevent weakening of the muscles Research: prolongs median survival up to 7 – 12 months Suitability / availability Gastrostomy Fact Sheet EB8 Permanent feeding tube into the stomach Improved nutrition and QoL Early decision required http://www.mndaust.asn.au/ Information>National Information Goals of Care relief of symptoms preservation of independence quality of life support choice and control information and education dignity and respect quality relationships peaceful dying process listening, acceptance, acknowledgement minimise suffering comfort Common Symptoms Symptoms experienced: weakness/ fatigue dysphagia dyspnoea pain weight loss speech problems constipation poor sleep emotional lability drooling 94% 90% 85% 73% 71% 71% 54% 29% 27% 25% Oliver, 2008 Muscles: Lower Limb Weakness •Often begins with foot drop •Difficulty climbing stairs •Difficulty arising from chairs •Possibility of falls •Eventually leading to hoist transfer •Fasciculation and cramps Care Strategies Ongoing assessment for equipment needs Home modifications Grab rails, chairs and beds on blocks, toilet raiser, shower chair, hoist Ongoing assessment for Movement and mobility Transfer belt, walker, wheelchairs Ankle / foot orthosis Manage swollen limbs Elevation, pressure stockings, recline chair, passive exercise, keep cool Muscles: Upper Limb Weakness Hand weakness difficulty with fine motor tasks using hands Shoulder girdle weakness difficulty using arms Neck weakness Care Strategies Ongoing assessment for equipment needs hand and body functional aids alternative clothing home modifications Ongoing assessment for Splints / orthotic devices / neck collars Movement / light exercise Care when transferring, esp shoulder joint Massage, pressure garments, elevation Maintaining Comfort Repositioning Subtle adjustments (small moves) Satin sheets, kylies, bed stick Support – cushions Care for weakened limbs Discomfort & Pain 1. 2. 3. Musculoskeletal pain Cramp/spasm pain Skin pressure pain Physiotherapy and passive movement Massage Hydrotherapy Use of splints and cushions Medications (initially nonnarcotic analgesics, antiinflammatory and antispasticity agents) Opioids Pain Management Similar to other advanced diseases: Careful assessment of pain Differing types of pain (cramps, spasticity, musculoskeletal discomfort) Severity Time course WHO guidelines Unique issues: Pain assessment with non-vocal plwMND Impaired swallowing and PEGs Creative Thinking Need an effective way of calling for assistance Door chimes Jelly bean switches Baby monitors Intercom systems Location of best position Minimise anxiety Physical body weakness, deterioration and immobility Dysphagia (difficulty swallowing: eating, drinking, saliva, choking, aspiration pneumonia) Dysarthria (changes in speech: volume, slurred, weakness, no communication) Respiratory weakness (dyspnoea, orthopnoea, respiratory failure) Muscles: Bulbar Weakness •Drooling •Choking on thin liquids •Slurring of speech •Quiet voice •Loss of speech •Difficulty chewing and swallowing •Weight loss Signs and symptoms of weakness in the muscles involved in chewing and swallowing making an extra effort to chew coughing whilst eating or drinking or soon afterwards needing several swallows for each mouthful muffled or ‘wet’ sounding voice after eating eating or drinking appears tiring - the person may be breathless after a meal meal times take longer frequent chest infections - caused by food and liquid residue in the lungs difficulty clearing saliva Swallowing difficulties can lead to dehydration, malnutrition and constipation. Muscles: Swallowing Dysphagia requires: Thorough and regular mouth care / hygiene Regular assessment by speech pathologist and dietician Maximise hydration and nutrition Modify diet and consistency Time over meals – no distractions Correct posture – upright, chin tuck Conscious swallowing, food positioning Sialorrhoea: Saliva beyond the margin of the lip (drooling) We produce approx 600 ml each day Handling of saliva is affected due to: Weakness of the tongue Weakness of throat muscles Anatomical structure (poor lip seal) Poor head control The Impact of Drooling Social participation Withdrawal, embarrassment Emotional wellbeing Loss of independence and self esteem Physical function Speech Swallowing Oral health ie infection, odour Dehydration Saliva Care: Thin Strategies: Upright position More conscious swallow Wipes and clothing protection Assisted cough technique Natural remedies: Golden rod drops Sage and hibiscus tea Horseradish tablets Medications: Glycopyrrolate Amitriptylilne Benztropine Suction Collar Botox injections Saliva Care: Thick Natural remedies: Dark grape, pineapple, apple or lemon juices Papaya extract Suck sugar-free citrus lozenges Hydration ++++ Reduce / eliminate alcohol, caffeine, dairy products Nebulizer (with saline solution) Steam inhalation Mouth care products i.e. Biotene Assisted cough technique Complications Aspiration pneumonia Defined as the inhalation of either oropharyngeal or gastric contents into the lower airways Due to poor swallow weakness or gag reflex Reducing the risk Elevate the bed Peg tube Avoid eating 1 to 2 hours before bedtime Saliva control Oral hygiene Complications Choking – due to: Impaired respiration Muscle spasm (laryngospasm) Care strategies stay calm reassure person wait for attack to pass Seek advice from physiotherapist for assisted cough technique Medications i.e. Morphine, Benzodiazepines: Clonazepam (drops), Lorazepam (Ativan) Nutrition: Eating well • Speech pathologist / dietician to assess • Increasing dysphagia • Modified diet – pureed food, thickened fluids, nutritional supplements, gravies • Positioning, use of equipment Why Consider PEG? • Stabilise weight loss • Maximise nutrition and hydration • Maximum energy • Improve quality of life • Prevent choking on thin fluids (safety) • Prevent prolonged mealtimes (distress) • Reduce risk of aspiration Placement of PEG Early placement recommended can be left un-used use as a ‘top up’ some risks involved Respiratory assessment Should be inserted before vital capacity falls below 50% of predicted (for safe anaesthetic) Muscles: Speech Dysarthria (motor speech disorder) Slurred speech, quiet voice Changes in vocal quality Requires coordinated movement of several muscle groups Speech pathologist to review and advise Affects: Vulnerability Isolation Inability to express needs Exclusion from decision making Loss of independence and social role Loss of self identity Challenges relationships Care Strategies Key word of sentence first First letter of word Eye contact and signals Gestures Translation by carer Letter / phrase chart Yes/no questions Be patient – slow down Communication Aids Low tech aids: Writing Magna doddle / white boards Laser pointer and chart Etran boards High tech aids: Lightwriter / Polyanna / Alora VMax Essence Vantage Light Fatigue Most common symptom Everything is exhausting Rest following activities (smaller rest periods) Small aids and equipment can help Conserve energy Be aware of insomnia Visit in the pre-lunch hours Bigger meals earlier in the day Swelling Due to lack of movement Legs elevated with cushion support Use of massage Elastic stockings Be aware of deep vein thrombosis Bladder & Bowels Fasciculation may irritate the bladder Hand weakness or mobility limitations Use of pads Uridomes Catheter Weak abdominal and chest muscles Diet / hydration Privacy Require adequate fibre, fluid Routine, comfort, aids Laxatives Emotional Lability – pseudo bulbar effect Unpredictable episodes of crying and laughing Disease damages the area of the brain that controls normal expression of emotion Anxiety and embarrassment, particularly in public Explanation (part of the disease), reassurance (not going mad) Medication in more severe situations Cognitive Changes previously thought cognition was not affected research indicates up to 75% may have some frontal lobe dysfunction 15% to 41% meet criteria for frontotemporal dementia (FTD) Miller & others, 2009 Cognitive Changes Cognitive Impairment (CI): deficits in attention, word generation, cognitive flexibility Behavioural Impairment (BI): changes in social interaction Fronto-temporal Dementia (FTD): altered social conduct, emotional blunting, loss of insight, language change, poor self care, emotional recognition, lack of empathy • Changes in decision-making • Reduced awareness of risk, concerns about risk taking • Frustration; forgetfulness • Communication • Obsessional behaviour; impulsiveness • Lack of self care Issues for Professionals Decision making • Assessment earlier to make decision –but person may not want to discuss the issues Communication • Unsure if discussion retained and able to be involved in the discussion Assessing symptoms • Pain / depression / swallowing problems Coping with memory loss / confusion Care Strategies Education for caregivers Give simple directions Establish a regular routine Possible medical management Cognitive and behavioural challenges in caring for patients with frontotemporal dementia and ALS (2010). Amyotrophic Lateral Sclerosis, 11: 298-302. Muscles: Respiratory Disturbed sleep Daytime sleepiness Increased fatigue Morning headaches Quieter voice Fewer words per breath Shallow, faster breathing Reduced movement of the rib cage or abdominal muscles Excessive use of the muscles in the upper chest and neck Weakened cough and sneeze Respiratory muscle weakness can cause Breathlessness (dyspnoea) even at rest Breathlessness lying flat (orthopnoea) Impaired concentration or confusion Irritability and anxiety Decreased appetite Care Strategies Be vigilant for symptoms Refer to a specialist respiratory service for regular assessment Avoid infections (people with coughs/cold) Treat reversible causes of dyspnoea Discuss NIPPV support Avoid crisis situations Improve ventilation – fans, air flow, humidifier Adjust room temperature Reclined or fully upright position Respiratory / breathing / relaxations exercises Medications: lorazepam, midazolam, morphine Non-Invasive Positive Pressure Ventilation • The use of positive pressure to do some of the work of breathing • BIPAP (bi-level) or VPAP (variable) • Used overnight to improve symptoms • Does not prevent weakening of muscles Benefits of Assisted Ventilation Decreased daytime sleepiness Better appetite Rests fatigued respiratory muscles Improved sleep Quality of life More energy Improved defence against infections Implications to Consider Significant improvement in survival Mask issues, intolerance Costs, availability, accessibility, back up Increasing dependency Carer burden Advance care planning (AHD/POA) See NICE Clinical Guidelines for the use of noninvasive ventilation: http://guidance.nice.org.uk/CG105 Advance Care Planning 75% preferred early discussion of Advanced Directives Oliver, International Symposium, 2007 plwMND preferred that doctor initiates discussion Communication issues Ventilation withdrawal issues Shown to change their preference for lifesustaining measures (e.g. ventilators) over a six month period Silverstein et al., 2006 = periodically reevaluate AHD Cultural differences Six Triggers for Initiating Discussion About End of Life Issues 1. 2. 3. The plwMND or the family asks - or ‘opens the door’ – for end of life information and/or interventions Severe psychological and/or social or spiritual distress or suffering Pain requiring high dosages of analgesic medications 4. Dysphagia requiring a feeding tube 5. Dyspnoea or symptoms of hypoventilation, a forced vital capacity of 50% or less is present 6. Loss of function in two body regions (bulbar, arms or legs) Promoting excellence in end of life ALS care, 2004 Use of Oxygen Breathlessness is due to muscle weakness not low oxygen If oxygen is given inappropriately it can: Increase carbon dioxide retention Reduce the body’s spontaneous signals to breath Put increased pressure on weakened muscles Common Cause of Death Most people die of respiratory failure Without NIPPV Choose not to Intolerance With NIPPV Eventual failure or voluntary withdrawal The duration between an acute deterioration and death is less then 24 hours Choking rarely occurs Neurvert, C, Oliver D Journal of Neurology 2001: 248 Other Causes of Death • Malnutrition and dehydration • Without peg •Refusal •Anatomical considerations •Respiratory status • With peg •Voluntary stopping •Intolerance or other complications • Aspiration pneumonia • Sepsis • Pulmonary embolus • Head injury/falls • Suicide • Co-morbidity Terminal Phase is recognised by Increased, progressive weakness Deterioration over a few days Often proceeded by Reduction in chest expansion Quietening of the breath sounds Accessory muscles for breathing Morning headaches Medical Management during Terminal Phase Use range of routes: oral, peg, patch or continuous subcutaneous infusion (syringe driver) Morphine or diamorphine to reduce pain/breathlessness Lorazepam, Diazepam (Valium) or Midazolam, a sedative, to reduce agitation/restlessness Glycopyrronium bromide to reduce the chest secretions and saliva (or hyoscine hydrobromide) Ethically appropriate to sedate; no muscle-paralyzing agents should be used Used appropriately (start small & increase) these medications will not hasten death Withdrawal of Ventilatory Support Major decision making as to when to cease ventilation Education of what to expect Comfort maintained Physician should be present (established relationship) Planned event; no haste Cultural or religious rituals discussed and planned Location prepared Medications ready Subcutaneous route is preferred Family and friends present End of Life Care Fear of choking (rarely occurs) and breathlessness Increasing immobility Discussions and anticipation of the final time Advance care planning, directives and EPOA done ahead of time regular review Build up of carbondioxide will anesthetise Step-process of withdrawal of NIPPV Use of adequate medications Support for the family and friends (bereavement) Features of Optimal End of Life Care care plans and information are shared adequate nursing cover comprehensive symptom control Psychological, social & spiritual support family and friends are providing practical support for the primary carer the opportunity to find completion Psychological, Emotional & Social Issues o A spiralling series of progressive losses (grief) o Changes in ability to influence their external and internal environment (control) o Changed relationship with body/self/identity o Aware of what is happening, what will come and increasing dependency o Carer burnout, relationship issues o Many psychological, emotional, sexual, financial, spiritual adjustments to be made Family & Friends Create relationships: open and honest, ongoing communication, inclusion, nonabandonment Family also have needs Respite Involvement in care planning Discuss fears and concerns Health Care Professionals Stretches the physical, emotional and spiritual ‘resources’ of staff Acknowledgement and support Awareness of self reactions (buttons) Flexible approach to care (share the care) Remember self care and understanding of own loss, grief and death fatigue Conclusion Be aware of the unique challenges of caring for a person living with MND Understand the disease and rapidly changing need The disease is the problem, not the person Early contact, relationship building Ongoing, preemptive assessment and referral Well coordinated teamwork •www.mndcare.net.au for information on MND care, symptom management and support for health professional •MND Aware: online training modules