Transcript Amyotropic Lateral Sclerosis Classroom

Amyotropic
Lateral
Sclerosis
(ALS)
Jerry Carley RN, MSN, MA, CNE
Spring 2009
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
3
Characteristics
 Disease of the motor system
 Progressive muscle atrophy
 Fatal (2 – 5 years) d/t respiratory failure
4
Characteristics
 Most commonly diagnosed in 40 - 70s
 Affects men more often than women
 The disease has no racial, socioeconomic, or ethnic
boundaries
5
6
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
7
8
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)
9
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)
10
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,
neurotoxic)
11
Renowned scientist Stephen Hawking suffers from amyotrophic lateral sclerosis
12
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 !
13
Collaborative Goals
 Focus on maintaining quality of life
 Control symptoms
 Prevent complications
 Provide adaptive devices to increase mobility and selfcare
14
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
15
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…
16
Medication
Antispasmodics:
- Baclofen (Lioresal)
- Damtrolene sodium (Dantrium)
- Diazepam (Valium)
17
Meds…
 Nonsteroidal anti-inflammatory drugs (NSAIDs) such
as ibuprofen or naproxen may relieve general
discomfort
 Tramadol (Ultram®) is often prescribed for pain relief
18
Depression
• Very common
• Antidepressant medication and
counseling can help patients and their
families cope
19
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.
20
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
21