CHRIS MANN PASSED AWAY
d.01 September 2005
"YOU WILL BE REMEMBERED"
Chris we
will miss you, a fine man that had given so much.
May you rest in peace.
What is ALS?
Amyotrophic lateral sclerosis (ALS), also
known as Lou Gehrig's disease, was first described by Bell in 1830, and named by
Charcot in 1874. A translation of amyotrophic lateral sclerosis is:
| A |
absence of |
| myo |
muscle |
| trophic |
nourishment |
| lateral |
pertaining to side (of spinal cord) |
| sclerosis |
hardening of (lateral aspect of spinal cord) |
The term
amyotrophic refers to the loss of nerve cells called lower motor neurons (which
project from the spinal cord to the muscle), and the term lateral sclerosis
refers to the loss of nerve cells called upper motor neurons (which project
from the brain to the lateral part of the spinal cord). Upper and lower motor
neurons are necessary for muscle contraction and movement.
ALS is a progressive
disorder of the nervous system which causes degeneration in both upper and
lower motor neurons and results in muscle weakness. The loss of lower motor
neurons leads to weakness, twitching of muscles (fasciculations), and loss of
muscle bulk (atrophy). The loss of upper motor neurons causes stiffness,
cramping, and weakness.
ALS refers to involvement of
both lower and upper motor neurons and diagnostic efforts are
aimed at establishing the involvement of both motor neuron systems. While
motor function is profoundly affected in ALS, the disease leaves intellect and
sensation intact. At present, there is no known cause or cure.
The course of ALS is
extremely variable. In the majority of patients, the weakness progresses over
a three to five year period. A small number of patients progress more rapidly
over a one year period. In twenty percent of patients, the progression is slow
and the course is over five years or longer. At present, the reason for this
variation is unknown, and it is difficult to predict the rate of progression
in any single patient.
Not all muscles are involved. For example,
eye muscles and muscles involving bladder and bowel control are usually spared.
Sexual function also remains intact. Heart and intestines are not affected.
Other nerve cells are spared so that intellectual abilities, vision, hearing,
taste, smell, and skin sensations remain normal. * Material from Baylor
University
I was made a Customer
Service Manager on the ramp just after the New year 2001. As I did my job,
which required a lot of walking I began tripping. At first I considered that
I was doing a job that I was unfamiliar with (and did not really want to do)
and as such was not paying attention to where I was walking. I led a safety
audit in Halifax and St. John's. I tripped and fell a few times there however
blamed it on "uneven ground in St. John's" due airport construction (it was
either that or Denis was pushing me). At this time I began getting
uncontrollable twitching which is sort of like having an alien living inside
you. I said it was part of my new exercise program however I knew there was
something not right at that time. One of the problems here was my cholesterol
medication and it's possible side effects of sore muscles and cramps (bingo
there was an explanation). In June I fell down the last 10 steps of my
basement stairs putting my knee through the raised floor in the basement.
Eventually it was the twitching which I mentioned again to my local clinic
that got me set up to see a Neurologist. The Neurologist tested me, said
nothing about what he suspected and sent me to the Foothills hospital for
further tests. The further tests indicated I had ALS (Lou Gehrig's disease).
Well I do not really
know but it does damage certain muscle groups. In my case it began on the
right side. I always tripped with my right toe. I have lost almost all the
strength in my right arm with smaller loss of strength in the left arm now.
The ALS society has made splints for both my arms and these I use to extend
the amount of time I can use my arms. My index fingers began curling under
my hand around the beginning of November my hands now look like this.
October 2001 My legs
are presently okay however I notice when my wife and I go for our walks that I
am absolutely whacked at the end of a walk which would not have ever phased me
before.
Aug 2003 Legs =
Non-Ambulatory, Feet = paralyzed, Arms = unable to grasp pen or cut food.
"As the symptoms of ALS
become more apparent, patients may notice weakness of the arms and hands which
results in difficulty performing daily activities such as buttoning or zipping
clothing, handwriting, and opening jars or doors. ALS may also cause weakness
of the legs and feet which may first be noted when walking over uneven
surfaces or for long distances.
As other muscles become
involved, the patient will notice further compromise in overall endurance and
increasing difficulty in performing activities of daily living. Progressive
weakness of the arms and hands may result in difficulty in performing tasks
such as cutting meat and holding articles, and weakness of the shoulders may
make it difficult to raise the arms to brush the hair or remove articles from
a high shelf. Atrophy (a wasting of muscle mass) and/or increasing stiffness
of muscles may become apparent. Weakness of the legs and hips will make it
more difficult to climb up and down the stairs, rise from a chair, or walk
safely.
If the weakness continues to
progress, the loss of function in the arms and legs will have a major effect
on lifestyle. Falls may occur and lead to the need for a wheelchair, not only
to assure safety but also to prolong endurance and to maintain independence.
At this point, performance of daily tasks and self-care may require the help
of assistive devices and/or a family member."
The Dr. in charge of
pulmonary function told me they base the speed of the disease by the patients
loss in lung capacity or the ability to expel C02. She said
there is continuos loss
of muscle control until such time as you can no longer breathe on your own and
that is it.
Sorry but I did not want to see that so I made
it smaller. My progression chart with scale
click
here
ALS may also cause weakness
of the respiratory muscles, which in an early phase may go undetected. The
respiratory system can be considered to have a ventilatory phase (air sacs and
bronchial tubes), a circulatory phase (where the exchange of gases occurs),
and a mechanical phase (determined by the muscles of respiration and by the
compliance (stiffness) of the lungs. Different disease processes may involve
one or more phases of the respiratory system.
 |
 |
 |
|
Ventilatory Phase |
Circulatory Phase |
Mechanical Phase |
For example, bronchitis
causes significant disturbances of the ventilatory phase, whereas blood clots
to the lung (pulmonary emboli) primarily affect the circulatory phase.
Amyotrophic lateral sclerosis may directly or indirectly affect all phases of
the respiratory system.
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