There are so many different situations which can make
someone a caregiver, like a
loved one getting into a horrible accident and being
rendered immobilized. The years can
begin to take a toll on the mind and body, and help is
needed with the simplest of things.
Then there are the ravages of a horrific disease, ALS
(Amyotrophic Lateral Sclerosis),
also known as “Lou Gehrig’s Disease” in the United
States (after the baseball great),
MND (Motor Neurone Disease) in England, and Maladie de
Charcot in France, which
robs mobility and is eventually fatal for those who are
in the prime of their lives. ALS is a
neuromuscular disease which begins as muscle weakness,
and then slowly progresses into
total and complete paralysis throughout the entire body.
ALS becomes a bit easier to
understand when it is broken down by its Greek meaning:
- A—without
- Myo—muscle
- Trophic—nourishment
- Lateral—side (of the spinal cord)
- Sclerosis—hardening or scarring
Although ALS was first described by French neurologist
Jean-Martin Charcot as long
ago as 1869, there is still no cure for this devastating
disease. About 50 percent of ALS patients
die within the first 18 months of diagnosis, with 20
percent of ALS patients surviving five years;
however, people who go on a ventilator to help them
breathe may be able to live for many
years. The average age for onset seems to be around 55,
but people as young as 12 and as
old as 98 have been diagnosed. Around 80 percent of all
ALS cases usually begin between the
ages of 40 to 70. In recent years, more and more people
in their 20s and 30s are being
diagnosed with ALS. This terrible disease does not
usually discriminate between sex or
race; however, men are more likely to be diagnosed than
women. The cause of ALS is still
unknown, but environmental factors are a strong,
suspicious factor as a possible cause,
with a higher incidence among people who have been
exposed to agricultural chemicals
and solvents. Whatever the cause, medical researchers
have discovered that an excess
amount of a neurotransmitter called glutamate is
produced, clogging the synapse nerve
cells, preventing transmission of neural impulses. This
clogging of the synapses causes
eventual death of these nerve cells, resulting in motor
neuron damage and muscle atrophy
(shrinking), specifically to nerve cells in the brain
and spinal cord that control voluntary
movement. The motor nerves that are attached to muscles
gradually degenerate and die
from lack of receiving nerve impulses, and as a result
of this nerve death, the muscles
atrophy and waste away. By the time someone with ALS
first notices neurological
symptoms, more than half of the motor neurons may
already be dead. Muscle paralysis is
the end result of this type of nerve death.
Symptoms of ALS may include tripping, stumbling and
falling, loss of muscle control
and strength in hands and arms, difficulty speaking,
swallowing and/or breathing, chronic
fatigue, and muscle twitching and/or cramping, and
drooling. Both upper and lower motor
neuron damage is experienced, with the symptoms of upper
motor neuron damage
resulting in stiffness, muscle twitching, and muscle
shaking, and the symptoms of lower
motor neuron damage including muscle weakness and muscle
shrinking (atrophy). The
most common path for ALS to take first is to weaken
either the arms or legs, making this
known as “limb-onset” ALS. While the symptoms of ALS are
not generally painful, some of
the effects may be, such as pressure sores, muscle
cramps, joint contractions, constipation,
burning eyes, swelling feet, and muscle aches.
Surprisingly, there are a few muscles that
remain untouched by ALS, enabling people to retain their
bowel, bladder, and sexual
functions. In a cruel twist of fate, the mind also
remains unaffected and remains quite
sharp, even when there is total paralysis. Eating can
also become more difficult as muscles
begin to weaken, with a loved one’s diet including
softer foods and the eventuality for a
feeding tube, but this can greatly extend the quality
and quantity of life.
Three types of ALS exist, with the most common type
called sporadic ALS. This type
is not hereditary, but does account for about 90 percent
of all ALS cases. Only about 10 percent of
ALS is recognized as hereditary (familial ALS), which is
passed on by a dominant gene.
The third type of ALS is called “Guamian,” named for the
unusually high incidence of
ALS on the island nation of Guam. Diagnosis is made
usually through a process of
elimination and can take several weeks or even months to
detect and confirm existence,
since early symptoms can mimic many other neuromuscular
diseases. The usual diagnostic
tests include MRI (magnetic resonance imaging), EMG
(electromyogram), muscle biopsy,
and blood tests. A definitive diagnosis of ALS can only
be made when the existence of
damage is evident in both upper and lower motor neurons,
and at least three limbs are
sufficiently affected.
When it comes to treatment, the FDA approved a drug
called Rilutek, which appears
to slow the progression of the disease. There are also
several other medications that can
help with the relief of symptoms, along with different
therapies, vitamins, antioxidants, and
proper nutrition, which are included as part of a
treatment plan. In recent years, the
over-the-counter supplement creatine seems to be
effective in preventing ALS in studies
using mice.
The unfortunate truth at this point and time regarding
ALS is that it is almost always
fatal, with the majority of patients dying painlessly
and peacefully in their sleep, because of
the build-up of carbon dioxide in the blood, caused by
insufficient amounts of oxygen due
to the shrinking of muscles which help with breathing.
But there is also some good news,
with the past five years seeing more research conducted
than in the last 150 years since its
discovery. Studies and research for other neurological
diseases such as Parkinson’s are
shedding new light on how to possibly treat ALS. Also,
many loved ones do not have to
remain in bed even though they may be totally paralyzed.
Thanks to the improvements of
modern technology, specially designed wheelchairs are
lightweight can accommodate
portable ventilators, allowing for loved ones to enjoy a
lot more freedom and movement
than in prior years. The most important thing that loved
ones and their caregivers must
remember is that they need to surround themselves by a
strong network of support
through family, friends, and the medical team. Look to
the future, because there are
hundreds of strides being made every day in research for
finding better treatment, as well
as to attaining the ultimate goal ... a cure.
The ALS Association
www.
alsa.org
Les Turner Amyotrophic Lateral Sclerosis Foundation
www.lesturnerals.org
Muscular Dystrophy Association (MDA)
ALS Division
www.als-mda.org
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