Transcript Update on ALS - Diagnosis and Treatment

Amyotrophic Lateral Sclerosis
(Lou Gehrig’s Disease)
Rod Sorensen, D.O.
Marshfield Neurosciences
April 26, 2007
Synonyms
Amyotrophic Lateral Sclerosis (ALS)
Motor Neuron Disease (MND)
Lou Gehrig’s Disease
Lou Gehrig
NY Yankees baseball star beginning in
1925
Once in the starting line-up he never
missed a game until 1939
Lifetime BA prior to 1938 was about
.350
Lou Gehrig
1938 season was disappointing, especially
late, not Mr. October
Ed Barrow (1938 counterpart of Brian
Cashman) took the opportunity to decrease
Gehrig’s pay from $39,000 TO $36,000
1939 - terrible spring training and early
season
Lou Gehrig
Lou was beginning to have falls and
significant balance problems
His baseball skills were visibly impaired
Lou’s first AB in 1939 resulted in a weak liner
to right field, caught by a 21year-old rookie
Red Sox outfielder, Ted Williams
Late in that same game, the pitcher walked
Joe DiMaggio, preferring to pitch to Gehrig
8 games into the 1939 season, Lou went to
manager Joe McCarthy and benched himself
Lou Gehrig
The numbers
14* years (retired at age 35)
2130 consecutive games
2721 hits
493 HR
Lifetime BA - .340 (.295 in 1938, .143 in
1939)
Lou Gehrig
Continued to travel with the team in
1939 and, as captain, gave the line-up
to the umpires at the beginning of the
game
Often had to be accompanied by
someone, to avoid the possibility of a
fall
Lou Gehrig - Diagnosis
ALS was diagnosed after Gehrig was
evaluated at the Mayo Clinic from June 1319, 1939
Wife Eleanor was phoned on June 19 (Lou’s
36th birthday) and was given the diagnosis
and told that Lou would be dead by 1942
Eleanor chose not to tell Lou that he had an
incurable, fatal disease
Lou Gehrig - Diagnosis
Lou Gehrig Day at Yankee stadium was
planned for July 4, 1939
While preparation for the celebration occurred
GM Barrow informed Eleanor Gehrig that Lou
would need to find a new job - both Lou and
Eleanor were surprised and infuriated by this
Lou Gehrig - Farewell Address
“Fans, for the past two weeks you have been
reading about a bad break I got. Yet today I
consider myself the luckiest man on the face
of the earth. I have been in ballparks for
seventeen years and have never received
anything but kindness and encouragement
from you fans. Look at these grand men.
Which of you wouldn’t consider it the highlight
of his career just to associate with them for
even one day?”
Lou Gehrig - Farewell Address
“Sure I’m lucky. Who wouldn’t consider it an
honor to have known Jacob Ruppert. Also,
the builder of baseball’s greatest empire, Ed
Barrow? To have spent six years with that
wonderful little fellow, Miller Huggins? Then
to have spent the next nine years with that
outstanding leader, that smart student of
psychology, the best manager in baseball
today, Joe McCarthy?”
Lou Gehrig - Farewell Address
“Sure I’m lucky. When the New York Giants,
a team you would give your right arm to beat,
and vice versa, sends you a gift - that’s
something. When everybody down to the
groundskeepers and those boys in white
coats remember you with trophies -- that’s
something. When you have a wonderful
mother-in-law who takes sides with you in
squabbles with her own daughter -- that’s
something.”
Lou Gehrig - Farewell Address
“When you have a father and a mother who
work all their lives so you can have an
education and build your body -- it’s a
blessing. When you have a wife who has
been a tower of strength and shown more
courage than you dreamed existed -- that’s
the finest I know. So, I close in saying that I
might have been given a bad break, but I've
got an awful lot to live for.”
Lou Gehrig
Despite his failing health, Lou continued to
travel with the Yankees throughout the 1939
season, including the World Series
NY mayor La Guardia asked Lou to become
a parole commissioner for the city of New
York - Lou finally accepted and was sworn in
on Jan. 2, 1940 for a ten-year term at
$5,700/yr.
Rocco Barbella, aka Rocky Graziano case
Lou Gehrig
Died in his sleep June 2, 1941at the
age of 37
Amyotrophic Lateral Sclerosis
Epidemiology
Features
Diagnosis
Natural History
Treatment and symptom management
Epidemiology
Annual incidence of about 1-2 per
100,000
More common with increased age, peak
incidence at 65-70 years
More common in white persons
Male/female ratio about 2
Mean survival is 3 years
NOT an inherited disease
Typical Features
Typical initial symptom is weakness
Hands are most common initial location,
but can start anywhere
Often asymmetric
As weakness progresses muscle
atrophy occurs
Typical Features
Fasciculations and cramps
Slurred speech and trouble swallowing not usually at onset
Difficulty breathing - usually a later
symptom
Drooling
Typical Features
Loss of dexterity
Clumsiness
Stiffness and spasticity
Exaggerated emotionality
Diagnosis
Exam
Definite - UMN & LMN signs in three
regions
El Escorial WFN criteria – definite,
probable, possible…
Electrodiagnostic studies
Imaging studies and lab testing when
appropriate
Natural History
Up to 40% of motor nerve cells may already
be lost by the time a patient presents with
weakness
Fairly consistent pace of worsening, spread is
typically to contiguous anatomic areas
Bulbar and respiratory involvement is usually
later in course
Death in 3 years is typical
Treatment
No effective treatment for cure or
improvement of disease
Riluzole
MAY slightly slow speed of progression
Reasonably safe
Expensive
Symptom Management
Drooling, depression, emotionality
Drying agents for drooling
Anticholinergic medications
Can result in thick secretions
Botox in parotid glands
Antidepressants for depression and
emotionality
Tricyclic antidepressants often help with all 3
symptoms
Symptom management
Spasticity and cramps
Antispasticity medications
Can increase weakness
Muscle relaxants
Best medication for cramps is quinine - now
unavailable due to FDA concerns
Symptom Management
Pain
Usually not a problem until later in disease
when immobility occurs
Physical therapy stretching, heat therapy
can be very helpful
Medications
Anti-inflammatories
Other meds including narcotics if needed
Symptom Management
Mobility
Home assessment by RN, OT, PT with
appropriate devices recommended
Braces, collars and orthotics
Home ROM, stretching program
Wheelchairs, scooters
Symptom Management
Speech and swallowing issues
Communication devices
Nutrition consult
Optimize food consistency to assist swallowing ease
Supplements
Forward head position, chin-tuck
PEG tube when swallowing no longer safe and
when patient decides they want it
Symptom Management
Respiratory problems
Secretion control
BiPAP
Non-invasive, intermittent
Improves survival
Tracheostomy and mechanical ventilation
Prolongs survival, but increases and prolongs
dependency
Decision to do this requires careful consideration and
family involvement
Palliative Care
In late stages of ALS, a number of different
factors can produce physical and medical
discomfort not unlike many other terminal
illnesses
Palliative care physicians/nurses/other health
providers are particularly skilled at
recognizing and treating these problems
Contrary to what patients and families initially
expect, death is usually not painful/stressful
Dependency
Lack of independence or selfsufficiency
That which is attached to something
else as its consequence, subordinate,
satellite, and the like.
Dependency
In ALS, the hardest part of the disease is
probably the loss of physical capacity
(despite normal cognitive abilities) and the
associated loss of independence
Loss of limb use
Mobility issues
Eating, speech
Ultimately, often entirely dependent on someone
else for virtually everything
Dependency
What happens with others around you family, friends
Our reaction to people with physical
disabilities - particularly those who were
previously “whole”
Less interaction
Change in the interaction
Dependency
ALS patient’s responses
Withdrawal & silence
Depression
Anger & frustration
Dependency
How we can help
Don’t forget them
Be natural - maybe act like it’s a briefer, less
serious illness (sure they are sick, but they are still
the person we know)
Little things done a lot are better than big things
done rarely
Get in their shoes
Remember their spiritual being