caring for patients with end stage neurological …...1 caring for patients with end stage...

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Caring for Patients with

End Stage Neurological

DiseasesSamuel Frank, MD

Karen Baker, LICSW MSW

Course Materials & Disclosure

�Course materials including handout(s) and

conflict of interest disclosure statement are

available to download with this course.

�This presentation is for educational and

informational purposes only. It is not

intended to provide legal, technical or

other professional services or advice.

Objectives

� Review the differential diagnosis and clinical features of

parkinsonism

� Idiopathic Parkinson’s disease (PD)

� Other parkinsonian syndromes

� Review the clinical features of ALS

� Review the clinical features of Huntington disease

Hospice Education Network - Caring for Patients with End Stage Neurological Diseases

Hospice Education Network (c) 2013

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Who Gets PD?

Epidemiology of PD

� 2:1 male:female prevalence

� Less common in Black and Asian populations

� Average onset age = 63

� 5-10% of people have symptoms < 45

� 1.6% of U.S. population over 65 has a dx of PD

� ~1 million people in North America

� Up to 130,000 people are newly diagnosed each year in

the US

Willis AW et al, 2010

Parkinsonism

� Clinical syndrome characterized by a variable

combination of:

� Tremor

� Bradykinesia

� Rigidity

� Postural instability



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Classification of Parkinson

Syndromes in a Community

Syndrome Prevalence

Idiopathic PD 85%

Medication-induced parkinsonism 7-9%

Multiple system atrophy 2-3%

Progressive supranuclear palsy 1-2%

Vascular parkinsonism 3%

MPTP, CO, Mn, recurrent head trauma rare

Postencephalitic parkinsonism None since 1960’s

Note: does not include essential tremor or dementia with motor

signs or other forms of parkinsonism

Non-Motor Symptoms of PD

� Depression or anxiety (50% of patients)

� Sleep disturbance

� Insomnia

� REM sleep beh d/o

� Poor handwriting

� Loss of smell

� Drooling

� Constipation

� Seborrheic dermatitis

� Dizziness

� Orthostatic BP

� Bladder dysfunction

� Sexual dysfunction

� Weight loss

� Dysphagia

� Dementia

Associated Problems - Late

� Dementia

� Dysphagia

� Weight loss

� Autonomic dysfunction

� Dizziness, typically from orthostasis

� Sweating

� Sexual dysfunction

� Dystonia

� Drooling



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Red Flags It May Not Be PD

� No tremor

� Symmetric findings

� Poor response to

medications

� Early falls or postural

instability

� Prominent dystonia or

ataxia

� Early dementia or

hallucinations

� Apraxia

� Early severe autonomic

dysfunction

� Gaze difficulty (especially

looking down)

� Upper motor neuron signs

Some Causes of “Parkinsonism”

� Drugs

� Anti-psychotics

� Anti-nausea

� Illicit drugs

� Vascular

� Progressive Supranuclear Palsy

� Multi-System Atrophy

� Shy-Drager

� Striato-nigral degen.

� OPCA

� CBGD

� Dementia with Lewy Bodies

� Normal Pressure Hydrocephalus

� Wilson’s disease

� Huntington’s disease

� Alzheimer’s Disease

� Dystonia

� Structural lesions

Drug Induced Parkinsonism

� Neuroleptics

� Any, except Clozapine

� Includes Zypreza, Risperdal

� Quetiepine is generally acceptable

� Keep in mind antidopaminergics for other uses

� Thorazine for hiccups

� Metoclopramide (Reglan)

� Reserpine for hypertension

� Tetrabenazine

� Illicit drugs



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Vascular Parkinsonism

� Criteria:

� Stepwise progression, risk factors for vascular disease, subacute onset, at least 2 infarcts in basal ganglia or widespread white matter abnormality.

� Typically worse in legs

� “lower body” parkinsonism

� Gait disturbance, freezing, wide base, gait apraxia

� Similar to Binswanger or NPH

� Typically no rest tremor or signs in arms

� May partially respond to levodopa

Progressive Supranuclear Palsy

� Onset after age 45

� Postural instability and falls

� Dysarthria

� Supranuclear gaze palsy

� Bilateral parkinsonism

� Predominantly akinetic-rigid

� Absence of benefit with levodopa

� Typically progresses faster that PD

� Dx to death in 9 years

� Avg time to dx is 5 years!

Multiple Systems Atrophy (MSA)

� Parkinsonism with:

� Autonomic dysfunction

� Shy-Drager Syndrome

� Cerebellar dysfunction

� Olivopontocerebellar atrophy (OPCA)

� Striatonigral degeneration (SND)

� Can be most difficult to distinguish from IPD

� Upper motor neuron dysfunction



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Cortical Basilar Ganglionic

Degeneration (CBGD)

� Onset after 45

� Asymmetric movement disorder

� Parkinsonism

� Dystonia

� Myoclonus

� Cognitive Symptoms

� Aphasia

� Ideomotor apraxia

� Alien limb

� Cortical Sensory Deficits

� No delusion or hallucination unrelated to levodopa

� Many features overlap with PSP

Alzheimer’s Disease with

Parkinsonism

Scarmeas, Neurology 9/2004

Alzheimer’s Disease with

Parkinsonism

Scarmeas, Neurology 9/2004



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Dementia with Lewy Bodies (DLB)

� Parkinsonism

� Hallucinations and dementia within one year of motor

findings

� Fluctuating cognition, attention and alertness

� Poor response to pro-dopaminergic medications

� Poor response to anti-psychotics, especially neuroleptics

Clinical Staging:

Modified Hoehn and Yahr

Stage Definition

0 No signs of disease

1 Unilateral disease (minimal functional impairment)

1.5 Unilateral plus axial involvement

2 Bilateral disease, without impairment of balance

2.5 Mild bilateral disease with recovery on pull test

3 Mild to moderate bilateral disease; some postural instability;

physically independent

4 Severe disability; still able to walk or stand unassisted

5 Wheelchair bound or bedridden unless aided

Hoehn & Yahr. Neurology. 1967;17:427-442.

Mortality in Parkinsonism

� Reduced life expectancy

� 1.5-3 times mortality rate compared to age-matched

controls

� Longer in non-demented PD cases

� Longer with L-dopa use

� PD survival > MSA, PSP

� The most common causes of death:

� pulmonary infection/aspiration, urinary tract infection,

pulmonary embolism and complications of falls, fractures

and immobility



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Treatments

� Prevention of progression

� Symptomatic - medical

� Symptomatic - surgical

� Non-pharmacologic

� Restorative

� Complementary or alternative

ALS

What is ALS ?

� A Motor Neuron Disease which affects the nerve path to

voluntary muscles resulting in progressive muscle

weakness

� Amyotrophic Lateral Sclerosis

� A myotrophic (literally without - muscle - nourishment)

� L ateral (refers to position of damage in the spinal cord)

� S clerosis (scarring or hardening)

� Identified over one hundred years ago. Called “Lou

Gehrig’s Disease” in the US

ALSA-MA Chapter © 2009



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ALS

� Progressive weakness, ultimately involving all limbs,

trunk and respiratory muscles

� Eye and sphincter control involved much later in disease

course

� Other symptoms include sialorrhea, muscle spasms,

spasticity, pain & fatigue

� Incidence 1-2 cases per 100,000

� Prevalence 6-8 per 100,000


What Causes ALS ?

No Known Cause: may be multi-factorial

� Environmental (viral or toxin)

� Genetic pre disposition

No known Cure

� One approved drug: Rilutek may slow down symptoms

Types of ALS

� Approximately 10% are “familial” or genetic.

� These families may face fear of “who will be next” or over-

burdened with care-giving.

� 90% are “sporadic” or cause unknown.

� These families are often blindsided, may not even have

heard of ALS. Unknown cause may lead to self-blame.



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Diagnosis and Progression of ALS

� No “test” for sporadic ALS, other diseases must be ruled out for 6 – 12 months.

� EMG has specific criteria, but early in disease course, some patients fit it only vaguely

� Misdiagnosis is common (Lyme disease, minor stroke, MS)

� ALS may progress very rapidly.

� 50% of people die by 3 years into illness

� Loss of breathing ability means a significant choice may need to be made about electing a tracheotomy and ventilator.

� Swallowing lost necessitating decision about PEG

Other Risks in ALS

� Cognitive loss or frank dementia in 30%.

� Higher risk of developing disease amongst veterans.

� Gulf War vets

� Disability

� May be increased in patients with TBI

Medication for ALS

� Riluzole

� May extend life up to 6 months by improving swallowing,

especially earlier in disease course



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Treatment Goals: No Cure

� Symptom Management

� Goal: Maintain Quality of Life

� Multi- disciplinary Approach

� Respiration

� Speech and Swallow

� Nutrition

� Secretions

� Augmentative Communication

� Mobility

� Assistive Technology

� Mental Health – pseudobulbar affect

� Pain Management

� Fatigue


Huntington Disease

Epidemiology

� 30,000 people in U.S. estimated to have disease

� ~300 new cases per year

� 150,000-200,000 at risk individuals

� Men and women equally affected

� Average age of onset is 39

� Range 2-90 years

� Majority have documented (or suggestive) family history

� 5-10% have juvenile HD (onset <20)



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Genetic Disease

� Each child of effected parent has 50/50 chance of inheriting disease

� Does not skip generations

� If you do not inherit the gene, you cannot pass it to the next generation

� Symptoms progress over years and begin insidiously

� The disease progresses and inevitably leads to death.

Trio of Disorders

� Movement Disorder

� Cognitive Disorder

� Psychiatric Disorder

Movement Disorder

� Involuntary movements� Chorea

� Dystonia

� Akathisia (inner feeling of restlessness)

� Inability to control voluntary movements� Balance

� Coordination

� Motor impersistance

� Grasp

� Modulation of the force of movement

� Slurred or loss of regularity of speech

� Difficulty swallowing



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Speech/Communication

� Muscles weaken

� Speech becomes difficult to understand

� Will become non-verbal but need to communicate remains

Swallowing

� Swallowing will deteriorate

� Higher risk for aspiration

� Look at diet, positioning, exercise, drinking behavior

� SLOW DOWN

� Minimize distractions

� Early discussion of G-tube preference

Cognitive & Behavioral Disorder

� Most challenging for caregivers

� Memory/Learning

� Organization

� Lack of initiation/apathy

� Delay in processing information (bradyphrenia)

� Perseveration

� Impaired judgment

� Impulse control



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Psychiatric Manifestations

� Depression & Anxiety (>50%)

� Apathy (48%)� May be secondary to depression or neuroleptic use

� Psychosis (12%)� Delusions > hallucinations

� Obsessive Compulsive Disorder

� Mania (10%)� Irritable or elevated mood

� Some meet criteria for bipolar disorder

� Sleeping Disorders

� Delirium

Advanced Disease

� Stiffness and slowness of movement

� More prominent than chorea

� Dystonia (Abnormal fixed postures)

� Weight loss

� Dementia

� Rate of progression varies, but disease range is reported to be

10-25 years

� Mean duration 19 years

Causes of Death

� Causes of death related to loss of functions:

� Pneumonia

� Aspiration

� nutritional deficiencies

� chronic skin ulcers

� Suicide risk

� ~ 25% attempt at least once

� 8-9% of all deaths due to completed suicide

� Risk factors include childlessness, depression, single, living alone, other suicides in family



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Treatments - Symptomatic

� Movement disorders

� Psychiatric treatments

� Non-pharmacologic interventions

� Always consider removing exacerbating factors

� Medications

� Environment

� Emotional triggers

Treatments – Non-pharmacologic

� Caregiver/nursing ‘routine’ care

� Social work

� Nutrition

� Social support

� Occupational therapy

� Physical therapy

� Speech therapy

Issues in Advanced Disease

� Defining “advanced”

� Tracking progression of disease

� Issues in management

� Dignity in late stage



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Rate of Progression

� Early in disease, loss of TFC is 0.5-1 point per year until

total is 5-6.

� Average TFC of a patient admitted to an extended care

facility is about 4.

� Later in HD – not well studied

� Patients may spend 5-10 years or longer under near total

to total care

Average Characteristics of

Patients in Extended Care

� Nance, 1996� 45 years old

� Disease duration = 10.0 years

� 47% women

� Previously employed

� High school graduate

� Unmarried (44% never, 33% divorced)

�Wheelock, 2003� 52 years old

� Disease duration = 8.6 years

� 63% women

Nance, 1996

Medical Issues in Advanced HD

� Behavioral disturbance

� Movements

� Including falls

� Dementia

� Delusions & other forms of psychosis

� Swallowing & Nutrition

� Weight loss/gain

� Mood & anxiety



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Everyday Issues in Advanced HD

� Medication use (and when to stop)

� How to assess pain and anxiety in the non-verbal patient

� Preventing purposeful physical violence

� Falls, falls, falls, falls, falls, . . .

� Weight in the patients who no longer walk

� How to keep patients entertained and out of trouble

Everyday Issues in Advanced HD

� Posture

� Communication with patients

� Communication devices

� Non-verbal behavior

� Screaming

� Eating/feeding with swallowing issues and time

� End of life management

� Hyperthermia and other signs of imminent death

� Pain

� Brain donation

Small Interventions Can Make Large

Differences

� Prepare for advancing disease

� CPR, feeding tubes, hospitalization, etc.

� Revisit use of medications

� Positioning

� Routine/predictability

� Adjunctive therapies such as speech & language,

music, recreational, and art therapies may improve

quality of life



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Medications Can Be Used in

Advanced HD for:

� Chorea

� Neuroleptics, tetrabenazine, amantadine, clonazepam

� Mood

� Anti-depressants, mood stabilizing agents

� Weight loss

� Megace, cannabinoids, olanzapine, low dose

mirtazipine

� Behavioral dyscontrol

� Anything that can get into the patient and sedates

Long on Information, Short on Time



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Family and caregiver

support Karen Baker, LICSW MSW

Stressors for the family with Huntington’s

Disease

� HD is a genetic disease

� Several family members can be disabled or dependent

within one family, leaving the caregivers outnumbered

by the “patients”

� Family may still be actively grieving deaths of other

relatives

� Family may be angry at previous generation for not

disclosing genetic risk

Stressors for the family with HD

� HD’s cognitive deterioration makes it very challenging

for one to succeed in school, finish college or training

programs, receive promotions. Many people with HD

are out of work, applying for disability benefits.

Caregivers face a loss of income while taking time from

work to provide care. Imagine the financial toll to a

family over several generations.



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� A person with HD has passed on a 50/50 chance that

biological offspring will inherit the gene. Families live

in great fear of children inheriting the disease.

� Genetic testing has been available since 1994 but raises

several ethical issues. Much consideration should be

taken prior to testing, ideally with the support of a

genetic counselor. Families may strongly disagree with

pre-symptomatic testing.


� Risk of suicide needs to be addressed for those testing

positive. Counseling and evaluation for anti-depressant

medication is strongly advised.

� Financial planning should be considered. Once a person

tests positive, there is a risk of not being able to

purchase disability, long term care, or life insurance.

� Relatives may argue over whether to test or not,

causing additional family conflict. An individual may

test in secret, not feel comfortable sharing results with

family, especially if the parent does not wish to test or

admit the diagnosis.

� People at risk feel “under a microscope” as relatives are

looking for symptomatic behavior




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� Opportunity to finalize advanced directives can be lost

by cognitive impairment

� Fear of discrimination leaves at-risk individuals private,

secretive, afraid to even attend an HD support group

� Families live in great fear of who may show signs of HD

next

Stressors for the family with ALS

� Two types of ALS:

� Approximately 10% are “familial” or genetic. These

families may face fear of “who will be next” or over-

burdened with care-giving.

� 90% are “sporadic” or cause unknown. These families

are often blindsided, may not even have heard of ALS.

Unknown cause may lead to self-blame.

Stressors to the family with ALS

� No “test” for sporadic ALS, other diseases must be ruled

out for 6 – 12 months.

� Misdiagnosis is common (Lyme disease, minor stroke),

can lead to anger or mistrust towards medical

community.

� ALS may progress very rapidly.

� Loss of breathing ability means a significant choice may

need to be made about electing a tracheotomy and

ventilator.



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Stressors to the family with ALS

� Cognitive loss is a risk (10 – 25%).

� Studies have shown a higher risk amongst Veterans.

Stressors to the family with PD

� Long course of PD can lead to care-giver exhaustion.

Common stressors to all families

� “There is no cure” may not be heard by the individual

or family due to lack of diagnosis, denial, or cognitive

impairment.

� Participation in clinical research trials to find a cure is

encouraged, but can lead to ethical issues. Participants

may believe they are receiving a “cure”, but can be

disappointed when study ends. Participants may use

research to avoid making decisions.



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Stressors to all families

� “Envy” towards other diseases with a cure or more

support or media coverage

� Cognitive and speech loss limits the window of

opportunity to address advanced directives.

Suggestions to utilize disease-specific

non-profit agencies

� Several non-profit agencies can offer support to families

and professional caregivers:

� Educational materials (printed books, conferences,

webinars)

� Training to professional caregivers

� Support Groups

� Assistance with referrals to specialists

� Fund-raising and advocacy events

Suggested non-profits

� Huntington’s Disease Society of America

� www.hdsa.org

� ALS Association

� www.als.org

� American Parkinson Disease Association

� www.apdaparkinson.org



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Questions and further information

� Karen Baker LICSW MSW

[email protected]

� Samuel Frank, MD

[email protected]

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