OT6 - Amyotrophic Lateral Sclerosis

7/30/2019 OT6 - Amyotrophic Lateral Sclerosis

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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=Fu86wIffib4


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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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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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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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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.

OT6 - Amyotrophic Lateral Sclerosis

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