Download Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS)
Also known as Lou Gehrig's Disease
• Named after the New York
Yankees baseball star who
played first base and was
diagnosed with the disease in
1939 and died in 1941 but,
contrary to popular belief, this
disease had been discovered
almost 100 years earlier.
History
• ALS discovered in 1850 by
English scientist Augustus
Waller who didn’t know he had
discovered ALS but saw nerve
fibers that had extreme
atrophy
• 19 years later a French
neurologist Jean-Martin
Charcot took credit for it in
publishing the disease in a
scientific journal
Objectives
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What is this disease?
Signs and Symptoms
Probable causes of ALS
Forms of ALS
Diagnosis of ALS
Medications you will see for ALS patients
Prognosis (outcome)
Stats for ALS
What is this disease?
• a progressive
neurodegenerative disease
• “ALS is a condition in which
cells of the brain and spinal
cord are lost.”
• This affects mostly motor
neurons the cells that control
needed voluntary muscle
activity such as speaking,
skeletal muscle movement,
breathing, and swallowing.”
What is ALS cont…
• When the motor neurons eventually die, the
ability of the brain to control muscle
movement is lost. Causing paralysis of
essential body systems.
• When muscles no longer receive the
messages from the motor neurons that they
require to function, the muscles begin to
atrophy (become smaller).
Signs and Symptoms
• Early symptoms
• Increasing muscle weakness
“especially in the arms and legs”
• Difficulty speaking
• Trouble swallowing
• Problems with breathing
• Twitching
• Cramping of muscles (mostly
hands and feet)
Signs and symptoms cont…
• Late Symptoms
• Extreme muscle atrophy
• Reflexes that are slow to nonexistent
• Excessive drooling
• Babinski's sign: “the big toe
dorsiflexes and the other toes fan
out “
• Increased spasticity (muscle
rigidity)
• Weight loss
• Choking
• Cardiac arrest due to respiratory
arrest usually resulting in death
Probable Causes of ALS
• Free radicals. The inherited form of ALS
often involves a mutation in a gene
responsible for producing a strong antioxidant
enzyme that protects your cells from damage
caused by free radicals — the byproducts of
oxygen metabolism.
• Glutamate. People who have ALS typically
have higher than normal levels of glutamate,
a chemical messenger in the brain, in their
spinal fluid. Too much glutamate is known to
be toxic to some nerve cells.
Probable Causes of ALS cont…
• Autoimmune responses.
Sometimes, a person's immune
system begins attacking some
of his or her body's own normal
cells, and scientists have
speculated that such antibodies
may trigger the process that
results in ALS.
• ALS is not contagious.
Forms of ALS
• Sporadic - the most common form in the
United States - 90 to 95% of all cases.
• Familial - genetic “accounts for a small
amount of cases in the United States” - 5 to
10% of all cases.
• Guamanian - an extremely high incidence of
ALS was observed in Guam in the 1950's.
Due to toxins in the water and food .
How to diagnose
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x-rays
MRI
Blood and urine studies
muscle and nerve biopsy
electomyography (EMG)
nerve conduction velocity (NCV)
spinal tap
Even with all this technology ALS is
extremely difficult to diagnose. This
is because many diseases mimic
signs of ALS
Prognosis
• Usually end up needing to go to a nursing
home or have hospice
• Only half of all people affected with ALS live
three or more years after diagnosis.
STATS
• Based on U.S. population studies, a little over
5,600 people in the U.S. are diagnosed with
ALS each year (approximately 15 new cases
a day.)
• Most people who develop ALS are between
the ages of 40 and 70
• ALS is 20% more common in men than in
women.
• 93% of patients are Caucasian.
Stages of ALS
• The natural course of ALS consists of 6 stages.
• Stages are based on the progressive loss of function in
trunk and extremity muscles.
• Identifying the stage can assist physical therapists in
designing intervention throughout the disease process.
Stage 1
• Patient is independent in mobility and ADLs.
• Muscles may just feel mildly weak.
• Physical therapy consists of:
• Patient and caregiver education
• Energy conservation
• Psychological support
• AROM and stretching of affected joints and resistive
strengthening exercises of unaffected muscles using
moderate weights and aerobic activities
(eg, swimming, walking, bicycling)
Stage 2
• The patient has moderate weakness (eg, foot drop, fine
motor skills).
• Assess for appropriate assistive devices to support
weak muscles.
• Physical therapy:
• Patients are encouraged to continue stretching and
AROM exercises, strengthening of unaffected muscles,
and aerobic activities as able.
• In addition, the patient and caregiver are instructed in
performing AAROM and PROM of affected joints to
prevent contractures.
Stage 3
• Patient still ambulatory but severe weakness of certain
muscle groups.
• Patient may be unable to stand without help
• Mild to moderate limitation of function.
• Physical Therapy:
• Assistive devices (AFO, neck collar, splints, electric WC)
• Continue exercises to weak muscles
• Decrease energy expenditure
• Improve patient’s safety and mobility
Stage 4
• Severe weakness of the legs and mild involvement of
the arms.
• Patient uses a WC but may still be able to perform
most ADLs.
• Physical Therapy:
• PROM and AAROM to prevent contractures
• Strengthening of any uninvolved muscles.
• As mobility decreases the need to inspect skin for
pressure areas increases.
• Need for sleeping and sitting systems that allow
position changes and pressure relief.
Stage 5
• This stage is characterized by progressive weakness
and deterioration of mobility and endurance.
• WC bound when out of bed.
• Arms now exhibit moderate to severe weakness.
• Transfers may require lift
• Patient unable to move themselves in bed and chairs
• Pain is a major problem ( immobilized joints, etc.)
• May have breathing problems
• Physical Therapy:
• Stretching, ROM and massage
• Modalities for pain
• Assistive devices
• Caregiver instruction
Stage 6
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Bed ridden and requires max assistance with ADL
Positioning
Pain continues to be a major problem
Breathing a problem
Physical Therapy:
• Goals at this stage are similar to those of hospice care:
• Address patient’s and caregivers needs
• Maximize the quality of each day
• Caregiver instruction to continue PROM, stretching,
transfers, massage, airway clearance techniques.
Common MEDS
• Most commonly seen is riluzole Rilutek© is aimed
at symptomatic relief basically maintaining
optimal quality of life.
• Side note : there is no drug interactions to any of
the drugs we use as paramedics.
• Medical marijuana
• Baclofen or diazepam may be used to control
spasticity
• Trihexyphenidyl or amitriptyline may be
prescribed for people with problems swallowing
their own saliva.
Meds Cont….
• O2 and gastric tubes
maybe prescribed as
well due to trouble
breathing and choking
• Coming Soon due to
Stem cell research
maybe a cure ?