palliative care in neuromuscular disease
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Colleen O’Connell, MD FRCPC Assistant Professor, Dalhousie University Research Chief Stan Cassidy Centre for Rehabilitation. Palliative Care in Neuromuscular Disease. Objectives. Participants will: understand the principles of prospective care as they apply to neuromuscular diseases - PowerPoint PPT PresentationTRANSCRIPT
Palliative Care in Neuromuscular Disease
Colleen O’Connell, MD FRCPCAssistant Professor, Dalhousie University Research ChiefStan Cassidy Centre for Rehabilitation
Objectives
Participants will: understand the principles of prospective
care as they apply to neuromuscular diseases
identify important strategies for improving function and quality of life for patients with neuromuscular disease
apply a management approach to end of life care in persons with neuromuscular diseases
Mrs. A – referred for Hospice Care 58 year woman,
living with ALS for 3 years
Gastrostomy tube for most nutrition
Bilevel-PAP at night; intermittent daytime
Total assist for personal care and mobility
Communicates by letter board and eye gaze
Family struggling to meet needs at home
Understanding ALSPathophysiology, Progression and Prospective Care
What is Amyotrophic Lateral Sclerosis
ALS is a rapidly progressive neuromuscular disease; part of the family of motor neuron diseases.
Affects both upper and lower motor neurons = Motor Neuron Disease
Who has ALS?
One of the most common neuromuscular diseases
2000 new diagnoses each year in Canada 4-10/100,000 prevalence in Canada Average age 58 at diagnosis; most common
40-60, some reports in teens-30s 1.4:1.0 male:female Appears to be more in last 20 years
What happens to people with ALS Upper motor neuron:
Weakness Spasticity, brisk reflexes-
hyperreflexia Emotional lability Loss skill in fine
movements – dexterity Slowed movement
Lower motor neuron: Atrophy – muscle wasting Weakness Hyporeflexia Muscle cramps Fasciculations
ALS : a syndrome
Clinical evidence• Increasing age-related mortality rates• Variability in survival rates• Familial variants• Frontotemporal syndromes (Cognitive, behavioral, FTD)• Genetic risk factors (ie., VEGF, apo e4)
Biochemical evidence• Cytoskeletal derangement• Mitochondrial dysfunction• Impaired calcium homeostasis• Altered glutamate homeostasis/excitotoxicity• Microglial activation and proliferation
-Dr. M. Strong
ALS amyotrophic lateral sclerosis; FTD frontotemporal dementia; PSP progressivesupranuclear palsy; AOA ataxia-oculomotor apraxia syndrome; SMA spinal muscularatrophy; PLS primary lateral sclerosis; HSP hereditary spastic paraplegia; SOD1 superoxidedismutase1; SMN survival of motor neuron; VAPB vesicle-associated membraneprotein B; SETX senataxin; APTX aprataxin; MAPT microtubule-associatedprotein tau; VCP valosin-containing protein; PGRN progranulin.
intact
mild
intact
mild
moderatesevere
Age-matched control (n = 122)
Sporadic ALS (n = 136)
(Data kindly provided by Dr. Stan Appel)
Frequency of cognitive impairment in ALS
Progression
Progressive muscular weakness Usually begins in a single limb or with
bulbar involvement Speaking, swallowing, and breathing
difficulties Sensory, ocular, bowel and bladder,
autonomic and cognitive functions* typically remain intact
Prognosis
Diagnosed age < 40; median survival 8.7yrs
Diagnosed age > 60; median survival 2.4yrs
10-16% alive > 10 years
At 5 years, 28% alive
Palliative Care in ALS
Palliation is part of continuum of care from diagnosis forward
Symptom control and promoting quality of life is considered part of palliative care
Not limited to hospice or comfort care Unique to ALS: series of losses/grief
throughout the disease Goals of palliative care change through
course of disease, from maximizing function to dignified death according to patients beliefs and preferences
Living with ALS
“When I was first diagnosed with the disease I was told to get my affairs in order because I have 3-5 years to live by a doctor who had no right to tell me that unless he found an expiry date on me that I am unaware of.”
Palliative Care
Should be initiated early in disease course Quality of life/Symptom management Discussions include:
End of life decisions and directives – review q6months
Options for respiratory support Inform on legal situation for advanced
directives Formulation of advanced directives
Collaborate with health team and family Assist with grief and loss support
“I try to lead as normal a life as possible, and not think about my condition, or regret the things it prevents me from doing, which are not that many.”
“…when this all started, I asked myself. ‘Am I going to withdraw from the world, like most people do, or am I going to live?’ I decided I’m going to live – or at least try to live – the way I want, with dignity, with courage, with humor, with composure. There are some mornings when I cry and cry and mourn for myself. Some mornings, I’m so angry and bitter. But it doesn’t last too long. Then I get up and say, ‘I want to live…’”
- Morrie Schwartz1
“If I cannot give consent to my own death, whose body is this? Who owns my life?”
- Sue Rodriguez
“Even if your illness f**ks up your plans, there is still cool stuff to do. I would rather be well but maybe helping others better understand and care for this illness is better.”
- Peter LeBlanc, living with ALS
Prospective Care
Function
Modifications
Quality of Life
Treatment
“care provided to improve a situation”
Treatment Issues
Incurable IS NOT Untreatable
Principles of Care in Progressive Diseases
Prospective Care Coordinated Care – Interdisciplinary ideal
Maximize Quality of Life Minimize Stress and Burden Maintain Function Plan in advance
GOAL: Improve ability to live with the disease
Practice Parameters - Guiding Principles
High priority on self-determination
Timely information for decision making, well in advance of management crossroads; decisions are dynamic and can change
Health care professionals should address full continuum of care – diagnosis to death
Interdisciplinary Team
Patient and Family
Doctors, Nurses
Social Workers,
Psychologists, Spiritual Counselors
Clinical Dietician, Speech
Pathologists,
Respiratory Therapists
Occupational and
Physical therapists
Home Care workers, Hospice
Plan for Coordinated Care
Diagnosed with Disease
Interdisciplinary Team
Primary Care Provider
Palliative CareServices
Family and Caregivers
Community Services and
SupportsSpecialty Services
Case ManagerClinic or
Community
Impact of Impairments
Participation in: Self-care Activities of Daily Living Communication Mobility Recreation Vocation Social Activities Sexual Activities
Emotional and Socio-economic impact on patient and family
Comprehensive Functional Assessment
Get baseline on all areas in initial assessment
Monitor progression via email, phone calls, video conference, visits
Predict changes in function as report of abilities change
Intervene early as changes occur to maximize function
Patient Perspectives
All people with this illness have value and their lives depend on acquiring the necessary resources to make sure they are able to use their value.
the necessity of a dedicated caregiver to prolong ones life and to greatly enhance ones quality of life
Symptom ManagementOptimizing Function and Quality of Life
Symptom Management
Optimizing Function and Quality of Life
RespiratoryNutritionSwallow and Secretions
Communication WeaknessPsychosocial
Respiratory Function
Respiratory impairment due to diaphragm, chest wall, and abdominal muscle weakness
Risks: atelectasis, aspiration, pneumonia, respiratory failure
Respiratory complications main cause death in MD and ALS (>80%)
Prevent ER/crisis intubation
Respiratory Function
Airway Clearance (Cough)
Ventilation (Breathe)
Symptoms
Signs of nocturnal desaturations/ hypoventilation: Am headache, restlessness, nightmares, daytime
somnolence Early signs of respiratory impairment:
Weak cough, low voice volume, supine dyspnea (hallmark of diaphragm weakness), accessory muscle use, paradoxical respiration, fatigue
Late signs of respiratory impairment: Severe orthopnea, upright dyspnea, daytime
drowsiness
Airway Clearance
Poor secretions (mucus, debris) clearance causes:Reduced ventilationLow perfusion statesLeads to atelectasisIncreased risk pneumoniaIncreased risk respiratory failure
Be Proactive – educate and prevent
Lung Volume Recruitment – Airway Clearance
Assisted Cough
When assisted PCFs have decreased to less than 270 L/min with LVR:
Teach manually assisted coughing (abdominal thrusts timed to glottic opening following maximal lung insufflation).
Mechanical Insufflator-Exsufflator
Tracheostomy in Palliative Care Is NOT synonomous
with invasive ventilation
Aid in airway clearance
Only option for some with advanced bulbar involvement
Can improve symptoms and quality of life
Can use MI-E via trache
Non Invasive Ventilation - ALS Patients using NIV in RC study showed median
survival benefit of 205 days (no benefit in poor bulbar function)
>4 hours/day did better than < 4 hours/day (7 months longer survival)
Slowed rate of FVC decline Improved quality of life with NIV No standard on when to start – likely at earliest
sign of nocturnal hypoventilation or respiratory insufficiency
Amyotrophic lateral sclerosis: monitoring
Review every 2-6 months Symptom review FVC sitting ± supine MIP, SNP if possible PCF ABG or ETCO2 when hypercapnea suspected Nocturnal oxymetry ± tCO2 when symptomatic
nocturnal hypoventilation suspected
Non Invasive Ventilation
Invasive Ventilatory Support Planned and
prepared for Equipment, training,
and human resources Advance of crisis improves quality of
life for patient, but increase caregiver burden
ALS Functional Rating Scale can predict outcomes of tracheostomy
Nutrition
Insufficient caloric intake can be related to: fatigue while eating, fear of choking difficulty manipulating food in the mouth difficulty transferring food to the mouth due to
arm weakness.
Malnutrition can lead to further muscle weakness and can cause immunodeficiency.
Swallowing
PEG or equivalent (radiologically inserted feeding device) probably prolongs survival
Better to do PEG prior to decline in respiratory function; increased risk in FVC<50%
You can still eat with a PEG! PEG is probably effective in stabilizing
weight Clinical Dietician to follow with modified
intake recommendations: positioning, textures, supplements
Swallowing - PEG Strategy:
Discuss with patient and family early: Pros and Cons
Opportunity to meet with surgeon Determine access to resources ahead of
procedure Determine options for when to
discontinue
Swallowing
Interventions Texture modification Supplementation Positioning Education Appetite stimulants (stout, maltevol) Oral hygiene
Secretions and Saliva
Saliva: atropine, glycopyrrolate, scopolamine, amitriptyline, botox, radiation
Thick secretions: guaifenesin, B-blocker Thin secretions: anticholinergic
bronchodilator Suction Machine, Airway clearance
techniques Oral Care: “magic mouthwash”
Communication
Not being able to speak, does not mean having nothing to say
Teach non-verbal strategies early Record voice Evaluate skills, resources, motor abilities;
referral to OT, Aug Comm, Environmental Control
High tech and low tech Scanning techniques, family and caregiver
education, signals There are no studies to support any particular
treatment
Augmentative and Alternative Communications
Weakness Skeletal muscle weakness ultimate cause of
most problems Moderate resistance exercise (submaximal):
strength gains 4-20% without decline High risk for overwork weakness – caution and
common-sense No exercise to exhaustion Weaker within 30 min of exercises Excessive muscle soreness 24-48 hours
postexercise Severe cramping, heaviness, prolonged SOB
Mobility Aids - Orthoses
Safety Muscle Efficiency Comfort Positioning Contracture
prevention
Shoulder Inferior Subluxation
One of most common UE issues for ALS pts
Catch it early in the ambulators and treat with support. Support:
▪ Taping (kinesiotape, etc)▪ Walker handles (added bonus with balance)▪ Slings▪ Creative options (fanny pack, hoody sweater,etc)
Neck Support
When neck weakens true weight of head felt
Swallowing can be easier
Open
HandsResting hand splints for nighttime to
avoid flexor shortening. Typical thermoplastic mitten style
resting hand splint. Best success has been with the
restorative hand
Active finger/wrist extension Kinesiotaping to
help with finger and wrist extension assist
Lightweight and with proper skin prep lasting 3 days.
Eating
• Modify food texture• Universal cuff• Plate guards• Foam • Dicem
Toileting
Issue for hand/arm weakness
Thank the universe for bidets!
Excellent model with remote control that can be mounted anywhere to operate with hand, elbow, feet has increased independence for many!
Warm water and blow dry features are lovely!
Beds and Mattresses
Beds: Up/down Head and foot Controls…switch adapted, remotes May be critical for breathing
Mattresses Soft: lose functional movement such as
rolling. Match the client carefully to the product Skin issues/turning surfaces, Braden
score, functional movement will guide choice wisely
Mobility Aids: Manual and Power Wheelchairs
Start early to supplement mobility so they learn while they have the energy to learn.
Predict for electronics for IR access
Power seating; what will be necessary
Tilt for trunk and head control and pressure reduction
Rehabilitation Issues-Depression, anxiety, grief
Reactive depression expected
Counseling, religious or spiritual support
Allow time for grieving, anger
Don’t forget family!
Patient Perspectives
There are a lot of possibilities for patients to accomplish unfortunately I feel that I am here to bide my time until I pass on. But that could be 20 years or more as proved by Steven Hawkins or Jason Becker. More and more people are living longer yet the perception remains the same about the lifespan of someone with this disease.
How much am I not worth?
Emotional and Social
Advanced Directives
Indications of criteria for discontinuation of nutrition and respiratory support
Planning for tissue donation Establishing powers of attorney in
advance Revisit recommended every 6 months
Patients can live many years with the disease!!
End of Life Care
Discuss wishes and options well in advance
DocumentEnsure family aware of choicesEstablish non-verbal communications
strategiesReview regularly; OK to change mind
▪ Feeding (disposition of G-tube)▪ Respiratory (disposition of ventilation)
Comfort measures – oxygen, morphineLocation for care– Home, hospital, other
Medications
Incremental opioids for dyspnea and pain, reduce cough reflex
Sedatives (diazepam, midazolam, chlorpromazine) reduce anxiety
Oxygen for comfortPalliative sedation if appropriate
Summary
Palliative care occurs from diagnosis to death Quality of life objectives vary patient to
patient Symptomatic treatments (respiratory,
nutrition) do prolong survival while maintaining good quality of life
Patients goals change from maximizing function to dignified death
Proactive care can reduce complications, maintain function, ease stress, an aid planning