Transcript Multiple Sclerosis

Unit 3
Management of Clients with
Degenerative Neurological Disorders
Gordons Functional Health Pattern
Activity and Exercise
Unit objectives :
1. Differentiate among the infectious disorders of the nervous system
according to causes, manifestations, medical care, and nursing management.
2. Describe the pathophysiology, clinical manifestations, and medical and
nursing management of Multiple Sclerosis, Parkinsons, Myasthenia Gravis,
and Guillain-Barré syndrome.
3. Use the nursing process as a framework for care of patients with Multiple
Sclerosis, Myasthenia Gravis, Parkinsons, and Guillain-Barré syndrome.
Unit 3
• Do Case Studies from Critical Thinking Book Before
Class!
1st CS on pg:363 Multiple Sclerosis
2nd CS on pg: 345 Parkinsons
3rd CS on pg:353 Amytrophic Lateral Sclerosis (ALS)
4th CS on pg:341 Guillain-Barre Syndrome
REQUIRED READING:
Smeltzer: Chapters 64 & 65
Multiple Sclerosis
• Chronic, progressive degenerative disease affecting
the myelin sheath of the white matter of the brain &
spinal cord
– Myelin sheath is highly conductive fatty material that
surrounds the axon and speeds the nerve impulses;
essential for normal conduction of nerve impulses
– Plaques form on myelin sheath  causing inflammation,
edema, & demyelination, eventually scarring  nerve
transmission becomes erratic, and slows down
– Age onset: 20 -40 yrs; affects women 2:1; whites are
affected more often than Hispanics, blacks or Asians.
– Ds more prevalent in colder climates: North America &
Northern Europe.
Multiple Sclerosis
Etiology:
– Exact cause unknown
– Theories: some evidence suggests that an
infective agent causes a predisposition to MS (an
immunogenetic-viral ds)
• Multiple genes are involved (one found so far – HLA
gene complex on chromosome 6)
– Precipitating factors: Infection, pregnancy,
physical activity, emotional stress.
Multiple Sclerosis
• Clinical Manifestations:
• Hx; vague and unrelated symptoms early period of MS, varies from
person-person
• Weakness or tingling sensation (parasthesias) of one or more
extremity - weakness & fatigue are the most common symptom.
• Difficulty with coordination and balance that is due to cerebellar
involvement – spastic weakness, ataxia, tremors, ↓ sensation to
temperature, foot dragging, staggering, or loss of balance
• Vision loss from optic neuritis - 70% experience involuntary,
rhythmic movements of eyes (nystagmus)
• Bowel & bladder dysfunction due to spinal cord involvement
(hesitancy, frequency, loss of sensation, incontinence & retention);
constipation.
• Symptoms worsening after tasking hot bath or shower (Uhthoff’s
sign)
Exacerbation and remissions.
Multiple Sclerosis
• Diagnostic tests: no single test reliable in
diagnosing MS.
– CSF eval: presence of oligoclonal banding –
presence of IgG antibody in CSF
– MRI of brain & spinal cord for presence of MS
plaques.
Multiple Sclerosis
•
Nursing Diagnosis:
–
Impaired physical mobility related to fatigue & weakness
–
Activity intolerance r/t weakness, dizziness, and unsteady gait
–
Self-esteem disturbance r/t loss of health & lifestyle changes
Nursing Management
1.
Promotes physical mobility – activity and rest
no vigorous physical exercise
frequent rest periods
walking and gait exercises
minimize spasticity and contractures – warm packs, daily muscle stretching
activities: swimming, stationary bike, progressive wt bearing
Minimize effects of immobility; skin integrity; cough and deep breathing exercises.
2.
Prevent injury – walk with feet wide apart, environment awareness and modification, gait training. Use
of assistive devices – walker, cane etc.
3.
Promote bladder & bowel control - Urinal/bedpan readily available, po fluids intake schedule/voiding
schedule, increase fiber in diet, intermittent self-catheterization
4.
Improve sensory and cognitive function:
Vision – eye patch for diplopia; prism glasses for reading; talking books
Speech – slurred, low volume, problems with phonation – speech therapist
cognitive & emotional responses – forgetfulness, easily distracted, emotionally labile, social activities;
hobbies.
5.
Development of coping strengths – education about diseases process; stress relief; network of
services – social, speech, PT, psychological, homemaker/meal on wheels
6.
Improve self care – assistive devices, raised toilet seat, shower bench, reached tongs, decrease
physical and emotional stress, decrease exposure of extreme temperatures
7.
Adapting to sexual dysfunction – counseling, plan sexual activity, willingness to experiment.
Multiple Sclerosis
• Tx: for acute relapses
– IV or oral corticosteroids
• Methylprednisolone, followed by oral Prednisone
taper
• Azathioprine (Imuran)
• Cyclophosphamide (Cytoxan)
• Tx: treating exacerbations
– Interferon B (Betaseron) used for ambulatory
pts. [genetically engineered complex protein w
both antiviral and immunoregulatory properties
which reduce the # of exacerbations
Multiple Sclerosis
• Symptomatic treatment: pharmacologic tx of
the symptoms– Bladder dysfunction: oxybutynin (ditropan),
propantheline, urecholine,
– Constipation: psyllium hydromucilloid, bisacodyl
– Fatigue: amantadine, modafinil
– Muscle spascity: baclofen diazepam, dantrolene
– Tremors: propranolol, thenobarbial, clonazepam
– Trigeminal neuralgia: carbamazepine, phenytoin,
amitriptyline
– Dysesthesia: TENS transcutaneous electrical
nerve stimulation
Multiple Sclerosis
• Collaborative Tx:
– PT if pt needs instruction on assistive devices, or learn
muscle tone to remain active; conduct ROM exercise
– For vision impairment – teach to scan the environment,
remove sources of injury.
– For urological control – drink 1500 cc q day of fluids and
void q 3 hrs. if urine is retained teach intermittent
catherization & clean technique
• Neurogenic bladder (most common in MS)
– Refers to several bladder dysfunctions caused by lesions of central &
peripheral nervous system: 5 types
» Uninhibited
» Sensory paralytic (detrusor muscle hyperreflexia)
» Motor paralytic (detrusor muscle areflexia)
» Autonomous
» reflex
Multiple Sclerosis
• Tx for constipation
– High-fiber diet, bulk laxatives, stool softners,  fluids > 2000
cc/ day
• Laxatives & enemas should be avoided  lead to dependency
• Bowel program should be performed every other day
– 45 min’s after largest meal (gastrocolic reflex) give suppository
• Keep environment cool – no hot baths, plan activity at
peak energy level
– Drugs: amantadine (symmetrel)
–
modafinil (Provigil) may alleviate fatigue
• Require assistance w ADL’s (w/c or canes)
– ADL nursing aides, assistive devices – raised toilet seat,
reacher tongs
• Fear of loss of independence & fear of disability
– Depressive episodes (short anti-depressent drug therapy)
Multiple Sclerosis
• Knowledge Deficit
– About MS, its unpredictable course, (remissions &
exacerbations), the role of stress, National Multiple Sclerosis
Society – excellent resource for education & support
• Discharge & Home Health Care
– Medications: be sure pt understands meds – dosage, route,
action, & SE –write them out
• Bowel & bladder elimination – routine
• Worsening of conditions – telephone access & support
network
• Prevention – avoid stress, fatigue and being over
headed stimulate exacerbations
Multiple Sclerosis
• Evaluation: the pt with MS will:
– Develop a realistic daily schedule that allows for
adequate rest
– Achieve an activity level appropriate for the extent
of disability
– Achieve bowel & bladder function
– Demonstrate self-catheterization if prescribed
– Verbalize and understanding of required lifestyle
changes, eg: ways to manage emotional stress,
maintain a nutritious diet, avoid infection, etc
Parkinson’s Disease
• A progressively degenerative neurological disorder
affecting the brain centers (substantia nigra and basal
ganglia) responsible for control & regulation of
movement
• Occurs in 1% of pop. Over 50, affects men > women
• Pathophysiology: depletion of dopamine levels in the
basal ganglia of the mid brain, which is where
dopamine is produced & stored.
– Dopamine promotes smooth, purposeful movements of
motor function
– Dopamine depletion  impairment of the extrapyramidal
tracts w loss of movement coordination
Parkinson’s Disease
• Cause: unknown
– Some heredity causes
– Secondary iatrogenic PD – is drug or chemical related
• Dopamine depleting drugs: reserpine. Phenothiazine,
metoclopromide, butyrophenones (droperidol & haloperidol)
• Clinical manifestations: 3 cardinal signs
– Bradykinesia / akinesia – slowness of movement or
complete or partial loss of movement; difficulty w balance
– involuntary tremors– course, rest tremor of the fingers &
thumb ( pill-rolling movement) of one hand, occurs during
rest, & intensifies w stress, fatigue, cold, disappears during
sleep, the tremor can occur in tongue, lip, jaw, chin;
eventually spreads to the foot on the same side
Parkinson’s Disease
– Progressive muscle rigidity – to antagonistic muscle groups,
causing resistance to both extension & flexion
• Flexion contractures develop in the neck, trunk, elbows, knees & hip
• Face – expressionless, mask-like appearance, drooling & ↓ tearing
ability
• Propulsive gait
• Speech: high-pitched monotone voice & parroting the
speech of others
• Hypothalmic dysfunction: ↓ or  perspiration, heat
intolerance, seborrhea, &  oil production
• Psychosocial : PD does not affect intellectual ability
– 20% of pts will end up having dementia (Alzheimer’s ds)
Parkinson’s Disease
• Diagnostic Evaluation: no specific dx tests
• History: progresses thru stages
– Mild unilateral dysfunction
– Mild bilateral dysfunction, expressionless face &
gait changes
–  dysfunction w walking, initiating movements, and
maintaining equilibrium
– Severe disability- difficulty in walking, & maintaining
balance & steady propulsion, rigidity
– Invalid  bed rest
Question
Which of the following is not a cardinal sign
of Parkinson’s disease?
a. Tremor
b. Dementia
c. Bradykinesia
d. Rigidity
Parkinson’s Disease
Drug therapy
• Goal is to enhance dopamine transmission
• Dopaminergics
– Levodopa  is to provide dopamine to the basal ganglia,
is a synthetic metabolic precursor to dopamine. Dopamine
itself can not be used because it can not cross the blood
brain barrier. Given in combination w Sinemet (carbidopa)
to allow more levodopa to reach the brain and it prevents
peripheral metabolism of levodopa; beneficial first few
years; on & off reactions;
• Sinemet
– most common drug (carbidopa-levodopa)
– SE = Orthostatic hypotension, nausea, hallucinations, dyskinesia
– Nsg considerations: monitor B/P, use TED hose to  venous return,
monitor for urinary retention.
PD – drug therapy
• Dopaminergics
– Symmetrel (amantadine)
•
•
•
•
Action – causes release of dopamine in CNS
Indications – rigidity, bradykinesia
SE – dizziness, ataxia, insomnia, leg edema
Nsg considerations – monitor for postural hypotension,
do not administer at bed time
PD - drug therapy
• Anticholinergic  to block the release of
acetylcholine (balance between dopamine &
acetylcholine) and to block the excitatory effects
of the cholinergic system
• Artane (trihexphenidyl)
• Cogentin (benztropine)
• Parsidol (ethopropazine)
– Indications: tremor, rigidity, drooling
– SE: dry mouth, constipation, blurred vision, confusion,
hallucinations
– Nursing Indications: usually contraindicated in acute-angle
glaucoma, & tachycardia, monitor pulse & B/P during dosage
adjustments, administer w meals, do not withdraw meds sudden
PD – drug therapy
• Antihistamines:
• Benadryl
– Indications: tremor, rigidity, insomnia
– SE: dry mouth, lethargy, confusion
– Nsg considerations: use w caution in pts w seizures,
hypertension, hyperthyroidism, renal ds, diabetes, administer w
meals or antacids.
• Dopamine agonists
– Parlodel (bromocriptine)
• Action: activates dopamine receptor in the CNS, helpful
for tx of on-off reactions
• Indications: fluctuation of manifestations, dyskinesia,
dystonia
• SE: Hallucinations, mental fogginess, orthostatic
hypotension, confusion
• Nsg considerations: monitor B/P & mental status
PD – drug therapy
• COMT inhibitors [catechol-O-methyltransferase}
– Tolcapone
•
•
•
•
Action: enhance effect of dopamine
Indications: adjuvant treatment
SE: diarrhea,  liver enzymes
Nsg: monitor LE
• MAO inhibators
– Deprenyl (selegiline)
• Action: inhibitmonoamine oxidase B, an enzyme that converts
chemical byproducts in the brain into neurotoxins that prevent
substantia nigra cell death
• Indications: adjuvant tx
• SE: nausea, dizziness, confusion, hallucinations, dry mouth
• Nsg: monitor for levodopa SE, Selegiline may  effects of levodopa
• Antidepressants
Parkinsonian Crisis
• Sudden or inadvertent withdrawal of anti-PK
drugs or emotional trauma, pts experiencing
severe exacerbation of tremor, rigidity and
bradykinesia, along w acute anxiety,
sweating, tachycardia.
– Interventions: respiratory & cardiac support,
subdued lighting, mild barbiturates, anti PK
drugs
PD – nursing management
Improve mobility
• Exercise & stretch regularly (first thing in morning)
• Encourage daily ROM to avoid rigidity & contractures
• Enhance walking – walk erect, watch horizon, wide-based
gait, heel-toe gait, long strides.
• Use cane or walker  prevent falls
Improve hydration & nutrition
• Maintain fluid intake 2 L/24 hrs
• Monitor weight & ability to chew & swallow
• Upright position to chew & swallow, offer small freq. meals, soft foods
& thick cold foods  supplemental puddings
• Prevent aspiration  think thru the steps of swallowing, keep lips
closed, keep teeth together, chew, finish one bite before another
PD – nursing management
• Improve bowel elimination
– Stool softners, mild laxatives, regular routine,  fiber,
raised toilet seat
• Enhance self-care
– Extra time needed to perform ADL’s, use of side
rails, overhead trapeze.
– Reinforce occupational & physical therapy
– Sleep on firm mattress, prevent neck contractures
• Improve communication
– Speech therapy– speak slowly, use board,
mechanical voice synthesizer
PD – nursing management
• Support coping abilities
– Feel embarrassed, depressed, lonely, bored
• Pd progresses, more rigidity & unresponsive to verbal
stimuli, treat w dignity, do not ignore clients
– Client to be active participant in EO
– Explore feelings
– Education
– Services: American Parkinson’s Disease
Foundation
Myasthenia Gravis
• Autoimmune disorder affecting the myoneural
junction
• Antibodies directed at acetylcholine at the
myoneural junction impair transmission of
impulses
• Manifestations
– Myasthenia gravis is a motor disorder
– Initially, symptoms involve ocular muscles, causing
conditions such as diplopia and ptosis
– Weakness of facial muscles, swallowing and voice
impairment (dysphonia), generalized weakness
Myasthenia Gravis (cont.)
Normal ACh
receptor site
ACh receptor site in myasthenia
gravis
Medical Management
Pharmacologic therapy
– Cholinesterase inhibitor: pyrostigmine
bromide (Mestinon)
 See Chart 64-4
– Immunomodulating therapy
• Plasmapheresis
• Thymectomy
Myasthenic
Crisis
• Result of disease
exacerbation or a
precipitating event,
most commonly a
respiratory infection
• Severe generalized
muscle weakness with
respiratory and bulbar
weakness
• Patient may develop
respiratory compromise
failure
Cholinergic
Crisis
• Caused by
overmedication with
cholinesterase
inhibitors
• Severe muscle
weakness with
respiratory and bulbar
weakness
• Patent may develop
respiratory compromise
and failure
Management of Myasthenic
Crisis
• Patient instruction in signs and symptoms of
myasthenic crisis and cholinergic crisis
• Ensuring adequate ventilation; intubation and
mechanical ventilation may be needed
• Assessment and supportive measures include:
– Ensure airway and respiratory support
– Take ABGs, serum electrolytes, I&O, and daily weight
– If patient cannot swallow, nasogastric feeding may be
required
– Avoid sedatives and tranquilizers
MG - management
• No cure
• Drug therapy: two groups of medications
– Short-acting anticholinesterase compounds
• Mestinon (pyridostigmine)
• Prostigmin (neostigmine)
– Corticosteriods
• Prednisone = assists in reducing the levels of serum
Ach receptor antibodies, steroids may temporary
worsen the symptoms followed by improvement
• Immunosuppressive therapy: ↓ the level of
circulating Ach receptor antibodies
– Imuran (azaathioprine)
– Sandimmune (cycloporine)
Question
A patient is undergoing a Tensilon test to
diagnose myasthenia gravis. Which of the
following medications should be available
to control the side effects of Tensilon?
a. Lidocaine
b. Atropine
c. Mestinon
d. Copaxone
MG – Nursing Management
•
•
•
•
•
Improve resp function
Increase physical mobility
Improve communication
Provide eye care
Prevent aspiration
Nursing Process—The Care of the
Patient With Myasthenia Gravis
•
•
•
•
Focus on patient and family teaching
Provide medication teaching and management
Implement energy conservation measures
Implement strategies to help with ocular
manifestations
• Prevent and/or manage complications and avoid
crisis
• Implement measures to reduce risk of aspiration
• Avoid stress, infections, vigorous physical
activity, some medications, and high
environmental temperatures
Amyotrophic Lateral Sclerosis
(ALS)
• “Lou Gehrig’s disease”
• Loss of motor neurons in the anterior horn of
the spinal cord and loss of motor nuclei in the
brain stem cause progressive weakness and
atrophy of the muscles of the extremities and
trunk; weakness of the bulbar muscles impairs
swallowing and talking; and respiratory
function is also impaired
Amyotrophic Lateral Sclerosis
(ALS)
• Course of ds- death from pneumonia in 3-5 yrs.
• Diagnosis: clinical s&s; EMG changes, muscle
biopsies, CSF analysis, CT, & MRI
• Nursing Management – is focused on the tx of
symptoms & rehabilitation measures
– Nutrition:
– Airway management:
Guillain-Barré Syndrome
• Autoimmune disorder with acute attack of
peripheral nerve myelin
• Rapid demyelination may produce respiratory
failure and autonomic nervous system
dysfunction with CV instability
• Most often follows a viral infection
• Manifestations are variable and include
weakness, paralysis, paresthesias, pain,
diminished or absent reflexes starting with the
lower extremities and progressing upward, bulbar
weakness, cranial nerve symptoms, tachycardia,
bradycardia, hypertension, or hypotension
Guillain –Barre’ Syndrome
• Also known as acute demyelinating
polyneuropathy or acute idiopathic polyneuritis,
results in a temporary, flaccid paralysis lasting
4-8 weeks
• Medical emergency, 80% will recover
• The syndrome is preceded by a resp. or GI,
viral infection 1 to 3 weeks prior to the onset of
paralysis.
– Autoimmune hypothesis
– Campylobacter jejuni is the organism most assoc.
• Affects both men & women equally & of all
ages.
Guillain-Barré Syndrome (cont.)
• Medical management
– Requires intensive care management with
continuous monitoring and respiratory support
– Plasmapheresis and IVIG are used to reduce
circulating antibodies
• Recovery rates vary but most patients
recover completely
Nursing Process—Assessment of the
Patient With Guillain-Barré Syndrome
• Conduct ongoing assessment with
emphasis on early detection of lifethreatening complications of respiratory
failure, cardiac dysrhythmias, and deep
vein thrombosis
• Monitor for changes in vital capacity and
negative inspiratory force
• Assess VS frequently/continuously including
continuous monitoring of ECG
• Encourage patient and family coping
Interventions
• Enhance physical mobility and prevent DVT
– Support limbs in a functional position
– Perform passive ROM at least twice daily
– Initiate position changes at least every 2 hours
– Provide elastic compression hose and/or sequential
compression boots
– Provide adequate hydration
• Administer IV and parenteral nutrition as prescribed
• Carefully assess swallowing and gag reflex and take measures to
prevent aspiration
• Develop a plan for communication individualized to patient needs
• Decrease fear and anxiety
– Provide information and support
– Provide referral to support group
– Implement relaxation measures
– Maintain positive attitude and atmosphere to promote a sense
of well-being
– Implement diversional activities
Interventions (cont.)
• Develop a plan for communication
individualized to patient needs
• Decrease fear and anxiety
–
–
–
–
Provide information and support
Provide referral to support group
Implement relaxation measures
Maintain positive attitude and atmosphere to
promote a sense of well-being
– Implement diversional activities