Download Mental Health

Amyotropic
Lateral
Sclerosis
(ALS)
Jerry Carley RN, MSN, MA, CNE
AH II Summer 2010
Concept Map: Selected Topics in Neurological Nursing
ASSESSMENT
Physical Assessment
Inspection
Palpation
Percussion
Auscultation
ICP Monitoring
“Neuro Checks”
Lab Monitoring
PATHOPHYSIOLOGY
PHARMACOLOGY
Traumatic Brain Injury
Spinal Cord Injury
Specific Disease Entities:
Amyotropic Lateral Sclerosis
Multiple Sclerosis
Huntington’s Disease
Alzheimer’s Disease
Huntington’s Disease
Myasthenia Gravis
Guillian-Barre’ Syndrome
Meningitis
Parkinson’s Disease
Care Planning
Plan for client adl’s,
Monitoring, med admin.,
Patient education, more…based
On Nursing Process:
A_D_P_I_E
--Decrease ICP
--Disease /
Condition
Specific Meds
Nursing Interventions & Evaluation
Execute the care plan, evaluate for
Efficacy, revise as necessary
ALS is also known as Lou Gehrig's Disease, after the famous
baseball player who died of the disease in 1941
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Characteristics of ALS
 Disease of the motor system
 Progressive muscle atrophy
 Fatal (2 – 5 years) d/t respiratory
failure
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Characteristics
 Most commonly diagnosed in 40
- 70s
 Affects men more often than
women
 The disease has no racial,
socioeconomic, or ethnic
boundaries
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What’s in a Name ?
 Amyotrophy refers to the atrophy (progressive muscle
wasting)
 Lateral sclerosis refers to demyelination followed by
hardening of the spinal column from buildup of scar
tissue (sclerosis = scar)
 As the disease progresses, it will move up the affected leg
or arm until eventually all muscle groups become involved.
This spread into all muscle groups is the defining
characteristic of ALS
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Why ?
... Etiology…..
 Mitochondrial dysfunction?
Genetic defect (chromosome 21) suggests the disease is
inherited and accounts for 5 to 10%
 Environmental factors, since the disease tends to cluster in
geographical pockets?
(Extremely high incidence of ALS has been observed in
Guam and the Trust Territories of the Pacific )
 Free radical damage?
 Glutamate excitotoxicity? (Apoptosis / Programmed Cell
Death)
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Signs and Symptoms
 Difficulty swallowing (dyphagia)
 Slurred speech (dysarthria)
 Fatigue
 Fasciculations of tongue (twitching) while at rest
 Usually the first muscles affected are those in the arms and legs
(Walking or climbing stairs may be difficult, may drop things, fall, experience muscle
cramps. The arms and legs may feel especially tired. If the hands are affected, may have
difficulty picking up small objects or turning keys)
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Diagnostics
 EMG / NCV studies
- NCV is administered before EMG and measures the
speed
at which nerves transmit electrical
signals
- EMG measures nerve impulses within the muscles
 Muscle Biopsy
 Tests to rule out other neurological disorders
- MRI may be used to rule out spinal cord diseases
- Blood tests may be done to detect the presence of heavy
metals such as lead, abnormal proteins or hormone
levels associated with other neurological diseases
- Lumbar puncture to analyze the cerebrospinal fluid for
genetic abnormalities (e.g., viral, autoimmune,
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Renowned scientist Stephen Hawking
suffers from amyotrophic lateral sclerosis
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Late Stage…
 As disease progresses and more muscle groups are affected, the
person becomes progressively incapacitated
 When respiratory muscles weaken, the client will require a
ventilator
 Percutaneous Endoscopic Gastrostomy (PEG) or feeding tube
 ALS patients often experience fear, anxiety, & depression
** Ability to think or reason remain intact !
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Collaborative Goals
Focus on maintaining quality of life
Control symptoms
Prevent complications
Provide adaptive devices to increase mobility
and self-care
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Collaborative Team
 Physical therapy helps to relieve cramping and
muscular pain. Passive stretching helps to avoid
permanent contraction of muscles (contractures)
that may cause joint problems
 Dietician ensures diet of high-energy foods that are
easy to swallow
 Splints, braces, and wheelchairs are used to help
with mobility
 Occupational and Speech therapy as their motor
control gradually deteriorates
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Medication
Riluzole (Rilutek®) is one of the few
drugs effective against ALS and may
prevent progression and prolong life for a
few months or so…
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Medication
Antispasmodics:
- Baclofen (Lioresal)
- Damtrolene sodium (Dantrium)
- Diazepam (Valium)
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Meds…
 Nonsteroidal anti-inflammatory drugs
(NSAIDs) such as ibuprofen or naproxen may
relieve general discomfort
 Tramadol (Ultram®) is often prescribed for
pain relief
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Depression
• Very common
• Antidepressant medication and
counseling can help patients
and their families cope
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Affairs in Order…
 While it may be emotionally difficult, it is important for clients
and caregivers to make informed, carefully considered
decisions regarding the future while the patient is capable of
making his or her contribution to a planned course of action
 Patients and their family members should discuss and consider
issues such as legal concerns, home care, assisted care, and
institutionalization
 Draw up wills and other important documents as early as
possible to avoid legal problems later on, when the patient
may be unable to represent his or her own interests
 Legal assistance may be necessary if the patient encounters
discrimination over insurance or employment.
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Prognosis
• Fifty percent of patients die within 3 years of diagnosis
 20% live 5 years
 10% live 10 years
 Hospice care can provide comfort and dignity to
patients and their loved ones
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