Laughing Along With ALS
by Jeff Lester
Who knew that ALS could be such a funny disease? I don’t mean
that the everyday fight is funny, but the situations and perceptions
that ALS creates can be extremely humorous.
My first glimpse into the disease’s funny side came at Christmas
1995, when I made my first trip out in my power wheelchair with
my sister and she got the last accessible parking space at the mall.
As we were laughing about her struggle to release my chair from
the van locking mechanism, an elderly gentleman approached us in
a huff. He proceeded to scold and curse us out for taking the last
handicapped spot, saying he deserved it more since he was much older
than I. To which my sister yelled, “Oh yeah? Well, we’re
more handicapped than you are!” So much for a smooth first
outing.
It was at a St. Louis Rams football game that I realized just how
weird the general public can be about those of us with disabilities,
especially when they’ve consumed a certain amount of alcohol.
During halftime, my brother and I were waiting patiently in line
for the bathroom when a very inebriated gentleman approached. He
proceeded to fall all over me saying how great it was that I was brave enough to come to the game. I suppose that was nice,
except that he kept gushing and following us, to the point that
my brother had to ask him if he wanted to join us in the handicapped
stall!
Every night our 3-year-old treats
us to a little show using the Hoyer to spin like someone
in a circus act...
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It’s not just the general public. We’ve had more weird
and bizarre responses to my ALS from the medical establishment than
from anyone else. These incidents range from the nurses and aides
who literally yell at me because apparently they think ALS causes
deafness, to the occasional hospital staff who ask my wife if I’m
retarded — I assume because of my slurred speech (or at least
I hope so). My wife has started responding, “No, he’s
definitely not retarded, but sometimes he’s a little perverted.”
All You Had to Do Was Ask
Hands down the most hilarious incident occurred during one of my
ICU visits, when two aides were in my room gossiping. I guess they
thought I was comatose, because their gossip switched to a discussion
of a sexual nature. This became an embarrassing moment for them
when I began to laugh and they realized I’d heard everything
they’d said.
Speaking of sex, I noticed, when it was announced that Lisa was
pregnant with our second child, that certain family members seemed
befuddled, especially my older brother. I wrongly assumed he was
worrying about how we’d deal with our newest addition. A week
later, I found out what was really bothering him.
“Jeff, I don’t mean to be nosy but everyone’s
wondering — how is Lisa pregnant?” Dumbfounded, I sputtered
out, “What? Well, pretty much the normal way everyone gets
pregnant, but I thought you understood the birds and bees since
you have five kids.” To which he said, “Well everyone
thought you were like Christopher Reeve and couldn’t do that!”
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While we all
recognize the serious side of ALS, we also need to take
time to get a good laugh out of the humorous side.
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All I could do was laugh at the thought of my family having a discussion
about this topic — which to my shock and surprise also had
been the subject of discussion and assumptions by Lisa’s family.
After our third daughter was born in April, I’ve been told
that some family members now brag to their friends about my virility!
Go figure.
From the Mouths of Babes
Some of the funniest and sweetest moments of my journey with ALS
have been with children. Questions have ranged from, “Why
are you in a chair that moves? Can I have a ride?” to my favorite:
“Hey mister, are you a robot?” I love to hear my daughters
explain ALS to others because they view the disease as normal. When
asked what’s wrong with me, they matter-of-factly reply, “He just has ALS, so he can’t move his arms and legs.”
Recently my oldest daughter had a slumber party with three of her
friends. When they saw me being moved to bed in the Hoyer lift,
they thought that was so cool and proceeded to move each other around
in it, giggling and laughing as they did.
The eldest isn’t the only one to enjoy the Hoyer, though.
Every night our 3-year-old treats us to a little show using the
Hoyer to spin like someone in a circus act, sometimes spinning so
fast that she staggers when she’s done. Children just don’t
have the hang-ups that most of us adults do when it comes to things
like ALS. Wouldn’t it be nice if the adults around us had
the same attitudes about ALS as children?
While we all recognize the serious side of ALS, we also need to
take time to get a good laugh out of the humorous side. I know I
certainly have, which may be why I’m still going strong more
than 12 years after my diagnosis, although functionally I’m
a quadriplegic on a ventilator.
Well, perhaps I should qualify that, for any of my family reading
this: I’m a quadriplegic in my arms and legs only. I don’t
want to start that rumor again!
Jeff Lester (“ragingbear”), 39, founded and hosts
the weekly “Living with ALS” chat on the MDA Web site http://www.mda.org/chat/calendar.html.
He also co-edits the “Will Hubben ALS Research Digest”
(www.als.net/research/hubben/signup.asp).
Jeff and his wife Lisa recently opened an online gift store, Raging
Bear’s Closet (www.
ragingbearscloset.com). A portion of sales benefits
MDA and other ALS organizations.
Back to top
Nietos to Host Bash
Augie and Lynne Nieto, co-chairs of MDA’s ALS Division,
are set to host the Bash for Augie’s Quest to raise
funds for Augie’s Quest and MDA’s ALS Division.
To be held March 22 at the Las Vegas Hilton, the Bash is expected
to draw over 2,500 supporters to “the party that will make a difference.”
The Nietos are determined “to help put a face on this
disease and raise money for research in a fast-track format.”
Seven-time Tour de France champion Lance Armstrong is scheduled
to make a special appearance, and the Grammy-award-winning
Doobie Brothers will perform.
For more information or to purchase tickets, visit www.augiesquest.org,
or call MDA’s Orange County office at (714)
550-0161.
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Back to top
ALS Research Roundup
by Margaret Wahl
Stanley Appel Receives
2005 Forbes Norris Award
Neurologist Stanley H. Appel, who directs the MDA/ALS Center
at Methodist Neurological Institute in Houston, is the 2005
winner of the Forbes Norris Award from the International Alliance
of ALS/MND Associations.
This award, named for neurologist Forbes Norris, who died
in 1993, goes to professionals who have made major contributions
to ALS patient care and research.
Appel graduated from Harvard College in 1954 and from Columbia
College of Physicians and Surgeons in 1960. He has authored
some 175 articles in medical journals and is on the editorial
board of the journal ALS and Other Motor Neuron Disorders.
He says the award, announced in December at the 16th International
Symposium on ALS/MND, was “totally unexpected. I wasn’t
even wearing a tie.”
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How Do SOD1 Mutations
Kill Nerve Cells?
Since 1993, ALS researchers have known that about 2 percent of
ALS cases result from a mutation of the gene for superoxide dismutase
1 (SOD1), but the mechanism by which the mutated gene and the
resulting abnormal SOD1 protein molecules kill motor neurons (nerve
cells that control muscles) in this disease has so far eluded them.
Now, scientists at Laval University in Quebec and Riken Brain Science
Institute in Wako City, Japan, may be closing in on an answer.
Makoto Urushitani and Jean-Pierre Julien at Laval University, and
colleagues, who published their results in the January issue of
Nature Neuroscience, say that, in the SOD1 form of ALS, motor neurons
and probably other cells in the vicinity secrete abnormal SOD1 (from
mutated SOD1 genes) in the company of proteins known as chromogranins.
Normal SOD1 protein molecules are also secreted by nerve cells,
the investigators say, but without chromogranins. In fact, the secretion
of normal SOD1 from cells may protect the area from potentially
hostile conditions. But the combination of abnormal (improperly
folded) mutant SOD1 proteins and chromogranins apparently leads
to inflammation and cell death by activating the local immune system.
“Chromogranins may act like chaperones that accompany misfolded
proteins when they’re secreted,” says MDA-funded neurologist
Stanley Appel at the Methodist Neurological Institute in Houston.
Appel calls the new study significant and says it suggests that
the abnormal SOD1 may activate microglia, the immunoactive
scavengers of the central nervous system, causing them to attack
and kill motor neurons.
He believes there may be similar immunologic triggers present in
non-SOD1 forms of ALS. “The process of interacting with microglia
could be comparable,” Appel says. “You just have to
change the name of the player. We know the name of the player in
one form of ALS; it’s SOD1. But any protein can look unusually
unique to the immune system, because of a genetic mutation or a
viral disease or for other reasons. There may be one altered protein
in one patient and another in another patient.”
MDA Launches Program of ALS Translational
Research
The Muscular Dystrophy Association has launched a new translational
research program specifically for ALS. The term translational describes moving strategies that are ready to leave the laboratory
through the appropriate administrative and regulatory pathways into
clinical trials.
Translating research from “bench to bedside” can be
difficult, because scientists aren’t always fully aware of
how to move their cell and animal studies into the clinical arena,
and physicians aren’t always fully aware of what’s going
on in academic laboratories.
MDA’s general translational research program, which eases
the way for such transitions, has been highly successful in moving
gene re-reading strategies for Duchenne muscular dystrophy and myostatin
protein-blocking strategies for adult muscular dystrophies out of
the lab and into testing in patients.
Minocylcine
Trial Closes
The phase 3 trial of minocycline, which has neuroprotective
and anti-inflammatory properties, is now closed. |
Pentoxifylline
Not Helpful in ALS
The drug pentoxifylline, which has some anti-inflammatory
and possibly some anti-cell death actions, has been found ineffective
and possibly harmful in ALS, in a 400-person, multicenter, European
study. There were 120 survivors in the placebo (inert substance)
group and only 103 in the pentoxifylline group.
Vincent Meininger at the Salpetriere Hospital in Paris, and colleagues,
who published the results in the Jan. 10 issue of Neurology, say
the drug may have interacted negatively with riluzole, which all
the patients also took.
Who Tolerates Noninvasive
Ventilation?
Neither age nor gender nor duration of disease are good predictors
of whether a person with ALS will tolerate noninvasive ventilation
(NIV) — air delivered under pressure through the nose or mouth
— says a new study from the University of Michigan.
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Nasal-Aire Interface. Photo
courtesy of InnoMed Technologies
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Investigators say, however, that when ALS-related weakness starts
in an arm or leg (limb-onset), rather than the mouth or throat (bulbar-onset),
NIV tolerance is more likely.
The investigators, who published their findings in the December
issue of Muscle & Nerve, analyzed data from 50 people with ALS
between 2000 and 2003. They found that 36 (72 percent) were able
to use NIV for at least four hours a night, while 14 (28 percent)
weren’t.
They controlled patients’ excess saliva with medication and
nasal congestion with steroid sprays; prescribed heated, humidified
air, delivered through small, nasal interfaces (Nasal-Aire brand);
and made small, weekly air pressure increases as needed.
Arming You With Tips for Living
With Arm Weakness
by Kathy Wechsler
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A splint can provide helpful support
for
your hand.
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Everyone’s ALS is different. There’s no set process
in which the disease occurs. Weakness can start in the legs, arms
or the muscles that control speech and swallowing.
Weakness may start in your hands, or it may start in your shoulders,
creating problems as functional abilities change.
“Arm weakness affects everything from getting out of bed
to getting dressed, bathed, and doing the things that you want to
do, like turning pages in a book or working on a computer,”
says Gail Miller, an occupational therapist (OT) at the MDA/ALS
Center at Johns Hopkins University in Baltimore.
“What we are looking to do is find out where the problem
is and then facilitate that action so that the bottom line is that
the job gets done.”
To “get the job done,” Miller uses the “KISS”
System: Keep It Safe and Simple.
Here are some simple suggestions for living a safe life with arm weakness:
