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Amyotrophic Lateral Sclerosis
Lou Gehrigs Disease
http://www.youtube.com/watch?v=Fu86wIffib4
http://www.youtube.com/watch?v=Fu86wIffib4http://www.youtube.com/watch?v=Fu86wIffib47/30/2019 OT6 - Amyotrophic Lateral Sclerosis
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Causes:
A Progressively neurodegenerative disease, which causesdeath to upper motor neurons located in the cerebral cortex,
as well as peripheral lower motor neurons, originating in the
spinal cord.
Death of motor neurons causes the brain to lose the ability to
produce and control motor muscle movements. As ALSprogresses the patient becomes paralyzed and eventually
dies.
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Causes continue
The initial cause of ALS is unknown. The onlyidentified cause, which only accounts for 10% of allcases of ALS is hereditary/ familial ALS (FALS).The remaining 90% causes of ALS are unknown.
Familial (FALS) is autosomal dominate withmutation on chromosome 21, which is calledsuperoxide dismutase (SOD1). Free Radicals may be the cause of this mutation
The 90% unknown causes of ALS may be due to: High Glutamate levels: Glutamate a chemical messenger
found in the brain. High levels of glutamate can be found inALS patients spinal cord.
Autoimmune Response: Self attack self.
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Incidence & Prognosis:
ALS occurs in 1 to 3 people per 100,000.
The onset is usually between 40-70 years of age
It is more common in men than women by 2:1
Most people with ALS die from respiratory failure,
usually within 2 to 6 years after diagnosis.
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Sporadic Amyotrophic Lateral Sclerosis
Sporadic forms (unknown cause) account for about 90-
95 percent of ALS cases.
Slight male predominance for sporadic ALS.
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Assessment findings:
Initial symptoms are often very vague and can varyfrom person to person, they may even be so slight
in the beginning that they are overlooked.
Symptoms can begin in the muscles that controlthe hands, arms, feet, legs, or speech. Although
not all people with ALS experience symptoms in
the same order or have the same pattern of
progression. But progressive muscle weakness is auniversal experience.
Muscle weakness is a hallmark sign.
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Symptoms include:
Difficulty lifting thefront part of the footand toes (foot drop)
Weakness in theankles and feet
Tripping over smallthings objects.
Hand weakness andclumsiness
Dropping things often
Trouble using thehands for simplethings such aswashing, dressing,and buttoning clothes
Abnormal fatigue ofthe legs and/or arms
Muscle cramps andtwitching(fasciculation)
Thick or slurredspeech and difficultyprojecting the voice
Uncontrollable periodsof laughing or crying
Dysarthia Dysphagia
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Assessment finding
continue As the disease progresses muscles become weaker and weakeruntil they are paralyzed.
Ventilatory support will eventually be needed as breathingmuscles are affected and paralyzed
As ALS progresses, muscles that control swallowing areaffected, dehydration and malnutrition can become problems,along with aspirating secretions, food, or liquid into the lungs cancause a PNA.
The most common cause of death for people with ALS isrespiratory failure.
ALS only affects the motor neurons so there are no problemswith sight, touch, hearing, taste, or smell.
Bowel and bladder control are not directly affected. Constipationand skin breakdown may occur due to immobility.
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Diagnostic Evaluation:
ALS is often difficult to diagnose early because it is verysimilar to other neurological diseases. There is no one testor procedure that can be done to establish the diagnosis of
ALS.
It is often through a series of diagnostic tests to rule out
other diseases that a diagnosis of ALS can be established.
The initial appointment may include testing of: Coordination
Balance
Muscle strength Muscle tone
Senses of touch and sight
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Diagnostic Evaluation continued
Workup to rule out other neurological diseases includes:
A thorough neurological examination
Electrodiagnostic tests including, electromyography
(EMG) and nerve conduction velocity (NCV)
X-Rays including MRI Myelogram of the cervical spine
Blood and urine studies including, thyroid and parathyroidhormone levels, 24 hour urine for heavy metals, and highresolution serum protein electrophoresis
Spinal tap (lumbar puncture)
Muscle and/or nerve biopsy
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Medication:
Riluzole (Rilutek) extends survival and/or time toinvasive breathing assistance. Rilutek is not curative.
Rilutek works by decreasing your body's levels of
glutamate, an amino acid that affects nerves that sendmessages from your brain to your muscles. People with
amyotrophic lateral sclerosis may have very high levels
of glutamate, which can damage these nerve cells.
Side Effects include: nausea, lung function decrease,
headache, dizziness, nasal symptoms, muscle
tightness, abdominal pain, high blood pressure, and
liver dysfunction.
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Medication continuedSpecial considerations:
Liver function will need to be checked with blood tests
every month during the first 3 months of treatment.
Do not smoke while taking medication as smoking
decreases the effectiveness of Rilutek.
When taking Rilutek, avoid drinking excessive amounts
of alcohol; alcohol may contribute to compromising liver
function and may be associated with an increased riskof liver problems.
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Medication continuedSpecial considerations:
Avoid coffee, tea, cola, or other products that contain
caffeine. Caffeine may cause too much Rilutek to build
up in your body.
Take this medicine with a full glass of water. Rilutek hasreduced absorption when taken with meals that are high
in fat. It is therefore recommended that the drug is
taken at least one hour before a meal or two hours after
to avoid reducing the absorption of the drug.
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Treatments:
Treatments are based on symptomatic control. Thisincludes:
Physical and Occupational Therapy to preserve mobility
and promote muscle strength.
Usage of cane, supportive brace, walker, andwheelchair.
Speech and language therapy to help control the loss of
speech and ability to swallow.
Speech-generating device to maintain communication.
NG tube, PEG
Respiratory therapist, noninvasive ventilation (BiPAP),
trach tube.
Support groups: local MDA
Medication: riluzole (Rilutek)
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Nursing Considerations:
Psych/Social issues play a HUGE role in caring for ALS patients.1. Assess patients level of acceptance and understanding of the ALS diagnosis.
2. Assess patients level of coping for loss of independence.
3. Evaluate level of support system the patient has.
Management of psych/social issues.1. Provide a therapeutic environment for the patient and family to express their
concerns and fears with each other and members of the health care staff.2. Allow patient to participate in care to their maximum potential to support a
sense of independence.
3. Provide patient and family information for ALS support groups in theircommunity.
4. Possibly refer patient and family to a counselor, psychiatrist, or psychologist forfurther supportive needs.
5. Provide encouragement and anticipate unasked questions.
6. Discuss changes in body image and how they affect the patients self-esteem.7. Encourage patient to focus on the positive aspects of life.
8. Allow time for the patient/family to grieve over the diagnosis.
9. Discuss advanced directives and DNR code status with patient/family.
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Nursing Considerations continued
Muscle Weakness and Skin Integrity1. Assess motor strength and measure level of spasticity/flaccidity.
2. Assess for muscle contractures.
3. Assess skin integrity daily for breakdown.
Management of Muscle status/Skin Integrity.1. Encourage patient to participate in daily activities as tolerated.
2. Patient should follow an exercise routine to preserve muscletone.
3. Range-of Motion exercises to prevent contractures, may beactive or passive with Physical Therapy involved in care.
4. Reposition patient every 2 hours, while assessing skin with each
turn.5. Wheel chair repositioning is key. Wheelchair should have acushion to prevent breakdown.
6. Educate caregiver on how often the patient should be turnedand how to assess skin for changes.
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Nursing Considerations continued
Nutrition/Elimination Needs1. Assess ability to swallow and presence of gag reflex.
2. Assess diet/fluid intake.
3. Assess elimination patterns.
4. Assess for incontinence and ability to transfer to acommode/toilet.
5. Assess for s/s of UTI.
Management of Nutritional and Elimination needs.1. Elevate HOB to prevent aspiration for patients with dysphagia.
2. Have a Dietician evaluate the patients caloric needs.
3. Encourage fluid intake of 2500 mL/day unless contraindicated
by difficulty swallowing.4. Educate patient about need for increased dietary fiber intake to
prevent constipation.
5. Encourage patient/care givers to document bowel movements.
6. Daily stool softeners and Metamucil are recommended toprevent constipation.
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Nursing Considerations continued
Respiratory status.1. Obtain history from patient/caregivers that indicates SOB or difficulty
breathing.
2. Assess patients respiratory status including: respiratory rate, effort ofbreathing, use of accessory muscles, nasal flaring, skin color,gag/swallow reflex, and coughing.
3. Auscultate lungs to assess for adventitious sounds
Management of Respiratory status.1. Suction oral secretions to prevent aspiration.
2. Administer medications that reduce secretions as ordered by the MD.
3. Encourage TCDB and use of Incentive spirometer.
4. Administer Oxygen PRN.
5. Elevate HOB to allow for easier breathing and decrease the risk of
aspiration.6. Educate caregiver on how to prevent aspiration.
7. Monitor/Assess need for mechanical ventilation. This may occur ascondition worsens. Life support measures should be discussed prior tothe need of treatment.
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Nursing Considerations continued
Mobility/Safety1. Assess current level of mobility (i.e.: use assistive devices
and ability to perform own ADLs).
2. Teach patient/family to remove all slippery rugs from home
and replace with slip-resistant rugs.
3. Teach patient/family to remove all cords and wires from walkways.
4. Ongoing Physical Therapy is needed to maintain mobility and
muscle function.
5. Educate caregiver/family on how to safely transfer patient.