Safe Harbor—
Rediscovering Life on a Vent
by Diane Huberty
This month marks my first anniversary of life on a vent —
a tracheostomy tube and mechanical ventilator. As I look back over
this year and try to sum up my feelings, the words that come to
my mind are “safe harbor.” I have found a shelter, a
place where I can literally and figuratively catch my breath, get
my bearings, and even make some repairs to my storm-battered body.
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A four-generation portrait that includes
Diane Huberty’s mother, Helen; Diane; Diane’s
daughter Kerry (left) and her daughter Taya; and Diane’s
daughter Kelly with her daughter Diana.
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The effects of the vent on my health — oxygen levels that
prevent headaches and give me energy, stamina, appetite, and pink
cheeks — are equaled by improvements in my quality of life.
My days are no longer dominated by shortness of breath, tiredness,
and fear of the next episode of lung congestion.
My world has re-expanded along with my lungs. BiPAP (bi-level positive
airway pressure, a noninvasive form of ventilation) was fine for
relieving nighttime breathing problems, but as my daytime use of
it increased, my world shrank.
Even if the BiPAP machine had fit on my wheelchair, the mask and
headgear were too bizarre-looking for me to want to go out. As a
result I seldom went outside, much less left home.
Simply talking left me breathless, dizzy and worn out, making socializing
too much work. I needed 10 or 12 hours of sleep a night and still
was exhausted by bedtime.
People talk about the horror of being “tied to a machine”
when they talk about vents, but they’ve got it all wrong.
I already was tied to another machine, my wheelchair, and like the
wheelchair, the vent has only increased my freedom, not tied me
down.
Now with the vent I’m out for hours shopping, sitting outside
chatting with neighbors, supervising outdoor projects, even accompanying
my husband to his part-time job and staying up too late! The vent
doesn’t draw stares like the BiPAP.
I actually feel rather proud to be seen out and about doing normal
things. I feel I’m representing the physically disabled and
showing able people that, vent and all, we’re still more like
them than different.
Feeling Secure
Of all of the improvements the vent has made, the greatest is the
feeling of safety it has given me. It sounds strange to say I feel
safer with my life dependent on a hunk of — egad! —
computer-run hardware and some batteries, but I do. It’s far
more dependable than my own body was and, unlike my body, comes
with a backup unit.
The vent alarms, something very few BiPAP machines have, also contribute
to safety. There are no more horrible episodes of not being able
to reach my call button when I can’t breathe because of congestion
or disconnected tubing. The vent alerts my family for me.
Life-threatening congestion is also a thing of the past. Relief
is just a suction tube away! That little rattle in my chest that
once heralded the onset of an exhausting and frightening hour of
trying to cough out congestion is now just a signal that I need
suctioning.
Suctioning seemed like a big deal at first but quickly became fast
and easy for my caregivers — a mere annoyance, not a medical
event! Suctioning makes me cough hard so it probably looks like
a miserable experience, but it isn’t at all. It’s no
more uncomfortable than any ordinary coughing spell and it leaves
me rattle free. It’s an incredible improvement over swigging
expectorant cough medicine and then trying to hack up congestion
using a cough-assist device or a series of Heimlichlike belly bruisers!
How does it feel to be on a vent? It doesn’t! I’m as
unaware of my breathing as anyone else. No feeling of air being
forced on me, no arguments with the vent over the rate and depth
of my breathing. I just breathe!
I expected a general ache or sore-throat feeling from the presence
of the trach tube, but I’m aware of it only if it gets tugged
sideways or needs repositioning. At times the skin around the opening
may get irritated, causing the only discomfort.
The Costs of a Vent
What does it cost to be on a vent? Thanks to Medicare Part B, the
cost is manageable. My vent costs include vent, nebulizer (to deliver
medicine) and suction machine rental, disposable supplies (hoses,
filters, suction catheters and gloves, trach dressings) and monthly
trach insert changes. The cost of those supplies has gone down as
we became more comfortable with less frequent hose, filter and trach
changes, and switched to plain old soap and water for trach care.
As far as we can figure from the slow Medicare process, we now pay
about $300 to $400 a month.
What about the burden my care puts on my husband and friends? The
only daily additions to my care are trach dressing changes and suctioning.
Trach care takes about three minutes once a day. (I have a Bivona
brand trach with no inner cannula [a tube within the trach tube]
to change.) Suctioning takes about three minutes also, and the number
of times a day I need suctioning varies from a few to a dozen.
There are about two hours a week of equipment maintenance and an
occasional nebulizer treatment to be done.
All that doesn’t add up to a lot of extra time, but when
added to the hours of care I already require it does count. On the
positive side, it is easier, faster, and much less frightening and
frustrating to suction me than to assist me through a bad coughing
spell.
As any caregiver knows, the burden of care can’t simply be
measured in hours. The stress and emotional toll can be much worse.
For example, I can never be left home alone. Actually, it was probably
every bit as dangerous for me to be alone for the last several years,
but it’s “officially” dangerous now!
Thanks to having a live-in helper who, although she has a full-time
job, allows my husband to get away in the evenings and on weekends,
being available is now only annoyingly restrictive to him. And with
two wonderful friends and my daughters, who were also trained in
the basics of vent care, he gets time to run errands on weekdays
and even take a full day off once in a while.
Being on the vent has made many things easier, however. Even though
we have to pack a laundry basket of “just in case” equipment
to leave home, I’m a lot more portable than before simply
because I feel good again. For him, the good news is that I can
accompany him most places. The bad news is that I can accompany
him most places!
Clear Sailing
I’m no Pollyanna. I’m well aware that my safe harbor
is a temporary shelter. My ALS will continue to progress, taking
the wind out of my sails and warping my timbers. There will come
a day when I’ll need to decide if it’s time to abandon
ship, but that could be years from now.
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Diane Huberty with her husband,
John, and her friend and caregiver, Kathy Lee.
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In the meantime I can and do enjoy my life. It’s so much
easier to focus on the positives when breathing isn’t a moment-to-moment
battle!
Thanks to the vent, today I’m enjoying holding our newborn
granddaughter, scanning the pages of children’s books so I’ll
be able to read to our 14-month-old granddaughter, working on Web
sites. For years I was slowly disengaging from life, withdrawing,
preparing to leave. Now I’m re-engaging, rebuilding, and truly
enjoying life.
It’s a beautiful spring day and the breeze is gentle. I’m
going to weigh anchor, raise the mizzen mast and go for a little
sail around the yard to make plans for this summer’s gardens
... and I’ll be planting perennials, not annuals. Life is
very good here in Safe Harbor!
Diane Huberty, 56, of Fort Wayne, Ind., received a diagnosis
of ALS in 1985. She retired from her position as Neuroscience Clinical
Educator at Parkview Hospital in 1995 and has since become involved
in computer work including Web site design. She maintains two Web
sites devoted to ALS at http://home.att.net/~liveletdie5/ALS/home.html and http://living-with-als.org.
What Can (and Can’t)
We Learn From the SOD1 Mouse?
by Margaret Wahl
The first real break in solving the mystery of ALS causation came
in 1993, when MDA-supported investigators linked one form of familial
(genetic) ALS to a gene on chromosome 21 that gives rise to a protein
called superoxide dismutase 1 (SOD1), identifying 11 disease-causing
mutations in the SOD1 gene in 13 affected families. (Today, some
100 different SOD1 mutations are known to cause ALS in humans.)
Shortly after these findings, MDA grantee Mark Gurney, a biologist
at Northwestern University, in Evanston, Ill., led a team that developed
mice with an SOD1 mutation at position 93. The mutation, which changes
the amino acid glycine to one called alanine in the
SOD1 protein, caused a disease in the mice that closely resembled
ALS. The mice lost muscle-controlling nerve cells (motor neurons),
became paralyzed, and died by 5 to 6 months of age, far short of
the usual mouse lifeof two to three years.
These G93A mutant SOD1 mice quickly became the standard model in
which to test new ALS treatments and study ALS pathology, although
there are mice available with a few other SOD1 mutations.
“We were lucky that the SOD1 mutant mouse disease looked
a lot like human ALS,” says Denise Figlewicz, a neuroscientist
and MDA research grantee at the University of Michigan in Ann Arbor.
“We decided to make the most of it.” Figlewicz and many
other ALS experts believe that, after the initial disease-causing
event, whether a genetic mutation or something else, a similar cascade
of biochemical changes leads to the death of motor neurons.
But some researchers think studying the G93A mouse as a model for
sporadic ALS is like looking for your keys where the light is better
instead of where you dropped them.
'Discordant' Results
Since the mid-1990s, several drugs that helped the G93A mice by
delaying disease onset or prolonging life failed to help patients
in trials. Among the drugs that showed these “discordant”
results are celecoxib (Celebrex), creatine and gabapentin (Neurontin).
Even Gregory Cox, an associate staff scientist at the Jackson Laboratory
in Bar Harbor, Maine, a nonprofit facility that conducts research
and supplies the G93A mouse to dozens of labs, has reservations
about the scope of its applicability. The mouse, he says, develops
an “incredibly reproducible motor neuron disease. It’s
a great model for human SOD1 mutations, but it’s not clear
how applicable what we learn is to all sporadic cases.”
Tighter Testing
One approach to increasing the usefulness of the SOD1 mutant mouse
model is to apply more rigorous testing procedures.
Gwendolyn Wong, director of in vivo pharmacology at the Research
Center of the ALS Therapy Development Foundation (TDF) in Cambridge,
Mass., says that tighter controls would have predicted the failed
clinical trial of Celebrex.
Wong thinks the SOD1 mouse is “a fabulous model of the clinical
disease.” But she also believes that you have to minimize
every possible cause of variability in the mouse experiments other
than the test drug’s effects if you’re going to get
results worth your attention.
“I firmly believe that of all the animal models out there,
this is the best one to use for drug discovery in ALS,” she
says.
The TDF carries out experiments using SOD1 mutant mice only after
matching the treatment and nontreatment groups for litter of origin,
body weight, gender, and number of copies of the mutated SOD1 gene.
When all these sources of variation were minimized, Wong says,
a test of Celebrex in SOD1 mutant mice didn’t show any benefit
— just as it didn’t in the patients. In Wong’s
experience, “failed mouse experiments lead to failed clinical
trials.” And, one hopes, with tighter controls, successes
in mouse trials will predict successes in humans.
Mimicking Human Variation
Denise Figlewicz has a somewhat different take on why the SOD1
mouse results have often not predicted human results. She’s
suggested that if the G93A mutation in SOD1 were bred into several
different strains of mice, each with its own genetic background
in other respects, the resulting mix of mice would be closer to
mimicking human variation. Experimental drugs that show benefit
in more than one strain would be promising. She’s now breeding
such mice, with MDA support.
“The problem,” she says, “is that most of us
are using the same G93A mice, which are genetically identical. It’s
as if somebody cloned you and tested a drug on you and the clones
and said, ‘It works!’ But there would be people unrelated
to you for whom it wouldn’t work.” In a genetically
diverse population, she says, any possible benefits of an experimental
ALS drug to a small percentage of people with a certain genetic
background are likely to be masked by the drug’s lack of effect
on others with different backgrounds.
The ultimate answer probably lies in a combination of both approaches:
Compounds that look very good in a rigorously controlled set of
experiments would be even stronger candidates if they subsequently
proved beneficial in SOD1 mutant mice from several different genetic
backgrounds.
It’s unclear how applicable any results will be to non-SOD1
ALS. But Cox, for one, is encouraged by the fact that you can’t
tell the difference between patients who have SOD1-caused ALS and
other ALS patients without doing genetic testing, which suggests
there’s considerable overlap. “We’ve got that
going for us,” he says.
HELPFUL
RESOURCES
MDA offers four publications for people with ALS, as well
as family members and caregivers. Contact your local office
for information.
Everyday Life With ALS: A Practical Guide,
released in May, is a revision and expansion of MDA’s ALS: Maintaining Mobility. The updated
guide is designed to help readers manage their daily experiences
with ALS. A free copy of the book will be provided to any
person with ALS who’s registered with MDA. The book
is available on CD for a fee.
Facts About ALS discusses the history, description and causes of ALS. 
When a Loved One Has ALS:
A Caregiver's Guide is a comprehensive manual
of practical advice for meeting the medical, emotional, financial
and everyday challenges faced by primary caregivers of people
with ALS. The primary caregiver of anyone with a diagnosis
of ALS who is registered with MDA can receive a free copy.
Meals
for Easy Swallowing
is no longer in print but is available
on the MDA Web site at www.als-mda.org/publications/meals.
The online version, which contains
all of the original text, comprises
a collection of recipes for people
who have swallowing difficulties because
of neuromuscular diseases such as
ALS.
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ALS RESEARCH ROUNDUP
Long-Ago Residence on Guam Is Likely
ALS Risk Factor
In a study that included 140 people with ALS and another 140 without
the disease, those with ALS were found to be eight times more likely
to have lived on Guam, even for a few months, than were those who
didn’t have ALS.
Only one person from the non-ALS (control) group had ever lived on
Guam, while nine of the ALS-affected subjects had lived on this island
in the Pacific for between one and 15 months. The nine hadn’t
lived there for an average of 43 years when they learned they had
ALS. (The time between living on Guam and an ALS diagnosis ranged
from 27 to 57 years.)
The findings, published in the May 24 issue of Neurology, and reported
by Lewis Rowland of the MDA ALS Center at Columbia University in New
York; Ruth Ottman, also at Columbia; and investigators at Erasmus
Medical Center in Rotterdam, the Netherlands, shed new light on the
ALS-Guam connection, a subject that has been under scrutiny for decades.
In 2002, researchers proposed that the unusually high incidence of
ALS on Guam seen between 1940 and 1965 probably had to do with the
islanders’ tradition of eating fruit bats (see “Bats
and Nuts,” May 2002). These bats eat cycad nuts that contain
a known toxin and then concentrate the toxin in their tissues.
The investigators on the new study say their findings “could
indicate that one of the key characteristics of the exposure occurring
during the period of endemic ALS on Guam is the long delay in clinical
expression [outward signs of disease].” They say their data
“support the vision that exposure to slow-acting toxic agents
is important in the pathogenesis of ALS, most likely in combination
with a genetic predisposition.”
Studies Examine Genetic and
Environmental Factors
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Genetic background and exposures to
toxins in the natural and industrial worlds probably combine
to produce ALS.
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Several studies are now probing genetic and environmental factors
that contribute to ALS.
One, open to people who either have ALS, are a sibling or parent
of someone with ALS, or have neither ALS nor a family history of the
disease, is being conducted at Northwestern University in Chicago
and Massachusetts General Hospital in Boston. No travel is necessary.
You can contact Nailah Siddique in Chicago at (312) 503-2712 or nsiddique@northwestern.edu.
Or contact Diana McKenna-Yasek in Boston at (617) 726-5750 or dmckennayasek@partners.org.
The Massachusetts General Hospital group is also conducting a study
to identify more genes that directly cause ALS. The investigators
are seeking participants whose family contains at least two people,
living or deceased, who have or had ALS.
In New York, an MDA-funded study of environmental and genetic factors
in ALS is under way at Columbia University Medical Center. This study,
which prefers to accept participants from the New York area, seeks
people whose ALS has been diagnosed within the past year, parents
and siblings of those with ALS, and married-in family members.
Contact Ani Sara Thankachan in New York at (212) 305-4746.
Four with ALS
Use Thought to Control Computer Cursor
Using brain waves, signals from brain cells that resemble electrical
discharges, to control a computer cursor or other electronic device
has long been a dream of people who can’t use their voluntary
muscles. But until now, the relevance to ALS of this approach has
been far from certain, since the motor cortex, the part of
the brain from which movement signals originate, is damaged in this
disease. (The cortex is usually intact in people paralyzed by spinal
cord injuries.)
Now, researchers at the State University of New York at Albany, the
New York State Department of Health, and centers in Italy and Germany,
describe four people severely disabled by ALS who learned to direct
a cursor to a target with greater than 75 percent accuracy, using
a brain-computer interface (BCI) method. With training, they
learned to use thought alone to move the cursor, and electrodes placed
over the cortex showed that thinking changed their brain waves.
The findings of this pilot study, published in the May 24 issue of
Neurology, show that people with ALS can use thoughts to control electronic
devices, although the researchers say increasing users’ speed
and accuracy is necessary for the application to be practical. They
also caution that control may wane as ALS progresses.
ADDRESS
CHANGE?
We need your help to improve our mailing
list for the MDA/ALS Newsmagazine.
If you’re a person with ALS who’s registered with
MDA, and you aren’t receiving the newsmagazine by mail, please contact your local MDA office and be
sure its list has your correct, current address.
If your newsmagazine is being sent to the wrong address, please
notify your local MDA office.
If you receive more than one copy of the newsmagazine, please
contact publications@mdausa.org,
or mail both address labels to MDA.
To find out which MDA office serves you, go to www.mda.org and enter your zip code under “Find Your Local MDA.”
Or call (800) 572-1717 and your call will be
forwarded to your local office.
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Back to top
ALS Caregiver
Suggestions: My Perspective
by Alison Teichgraeber
We’re not alone on this journey through ALS. My husband, Patrick,
had ALS and I cared for him throughout his illness.
As caregivers, we have the potential to burn out both emotionally and
physically. My biggest piece of advice is don’t! I wore
myself out, and that caused my husband more anguish.
Take Care of Yourself
- Get enough sleep. Figure ways to help the person
you care for sleep through the night so you can too. Loss of sleep
can affect both your physical stamina and your cognitive abilities.
I became so tired I had trouble with third-grade math!
- Exercise. Doing something for yourself feels decadent,
but a 20-minute walk around the block can release stress and refresh
your soul.
- Talk. Talk therapy saved my life. Talking with
someone like a counselor or a minister will help you process all the
changes that affect you. Denying stress only exacerbates it and causes
more problems.
Educate Yourself
It’s important to be familiar with all the changes happening
to you and the person you care for. By educating yourself about ALS,
medical supplies, insurance coverage and finances, for example, you’ll
become an informed consumer. This will help you prepare for necessary
adjustments and unexpected events. By becoming an educated consumer,
I was able to be a levelheaded advocate for my husband.
Ask for Help
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Patrick and Alison Teichgraeber with
their sons, Matthew, now 10, and Justin, now 7.
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Get help early. As a caregiver, you can overextend yourself.
There are ways to avoid this. Many people offered me help, and I was
too proud to accept it. Finally, a friend told me, “Take the food!”
I did, and twice a week neighbors and church members brought a special
dinner for my kids and me. These meals would often be enough to feed
a small army; the leftovers lasted all week. So say yes!
For example:
- Ask four people to mow the grass: Your friends mow only once a month
and your lawn looks manicured year-round.
- Ask friends to drop off and pick up your dry cleaning when they
go to the dry cleaners; that way, they aren‘t even making extra
trips.
- Ask friends to pick up a few items at the grocery store when they
go; you save a trip and they have the satisfaction of helping.
- Ask friends to bring photos or other “props” over to
share with the person you care for. He or she can have company, and
you can have a moment to yourself. I loved props; they made everyone
feel more comfortable.
Stay Ahead of ALS
ALS is relentless. By staying ahead of it, you have a greater chance
of being prepared for the unexpected twists and turns of the disease.
- Have the person you care for use a walker before one is needed.
There’s nothing worse for him or her than falling and getting
stuck. My husband was once stuck on the floor for 30 minutes before
I came home from work. My kids found him on the floor and they all
had a good (albeit embarrassing) laugh.
- Order the wheelchair before your person with ALS needs it. It can
take months to receive the chair and only one fall or the flu to suddenly
need one.
- Allow time for the person you care for to adjust to the BiPAP. My
husband hated it. By “practicing,” he slowly got
used to his “space mask.”
ALS Isn't Your Whole Life
An ALS caregiver experiences the same challenges as the person with
ALS. Each step along the ALS path can be debilitating, exhausting and
overwhelming. But, it’s only part of the picture.
There’s much more to life than ALS. It’s important to pay
attention to the other aspects of your life such as your children, education,
and friends.
We all know life sometimes gives us lemons. By enlisting friends and
family, you and the person you care for can make lemonade. One of the
most challenging tasks of a caregiver is making life feel “normal.”
People who aren’t used to the changes ALS produces feel uncomfortable
about them, and it’s up to the caregiver to help ease this discomfort.
For visitors who feel squirmy, I suggest keeping a “chat list”
list like this one:
Ways to Feel Less Uncomfortable Visiting Someone With ALS
- Talk about the weather
- Talk about your kids
- Talk about sports!
- Talk about your work
- Talk about the person with ALS
- Don’t talk, just enjoy the game/movie/etc.
- Bring a friend/spouse/child
Sometimes, a visit with few words spoken is the best visit of all.
ALS can be isolating. It’s up to you to reach out to others,
not only for the person you care for, but also for yourself. Although
spending a third of your time on yourself, a third of your time on your
family, and a third of your time on your career is probably impossible,
it should be a goal. Balance is optimal.
By taking care of yourself, educating yourself, asking for help, staying
ahead of ALS and not allowing ALS to be your whole life, you can be
the best caregiver possible.
Alison Teichgraeber, of Houston, is a writer, advocate for the
severely disabled and mother of Matthew, 10, and Justin, 7. Her husband,
Patrick Teichgraeber, was a major in the Marine Corps when he received
a diagnosis of ALS in 2001. He died in September 2004, at age 40.
Back to top
Best-Case Scenario
by Alyssa Quintero
In January 2003, John Baird received a diagnosis of ALS. An avid golfer,
Baird wasn’t prepared when he found himself in this “sand
trap.”
Today, when he’s asked what it’s like to have ALS, Baird
responds, “It’s not really too bad,” but “I
really miss Mexican food.”
Baird, 60, no longer can speak, and he has a feeding tube and breathes
through a hole in his throat. But, he can still get around on his own
and plays golf whenever possible.
Baird is gradually losing the use of his fingers on his left hand —
a problem especially because he is left-handed.
But, he says, it could be worse. Regardless of what ALS has done to
his body, Baird is the self-proclaimed “best-case scenario of
ALS.”
“I would much rather be able to get around, take care of myself,
and use a computer and type than be able to talk,” Baird said.
Baird, who took up golf four years ago, remains competitive with other
60-year-olds.
“I can no longer hit the ball very far, and I am losing some
accuracy, so I have implemented some new rules of golf. I play the par
threes, but on the par fours and fives, I take a drop about 100 yards
out and assume I got there in one or two strokes.
“The other thing I have learned is to quit betting,” he
said.
The Communicator
Baird uses a speech communication device and explains, “It has
been a real life-saver. It slows conversations considerably, but it
permits me to stay a part of the world.”
Until recently, with his wife Annabel’s help, Baird owned, published
and edited a community newspaper in Highland Village, Texas.
Although he’d worked in software development and had owned a
number of other businesses, Baird opened The News Connection in 2002.
The biweekly’s original circulation of all 4,000 homes in Highland
Village expanded in the fall of 2003 to 35,000 homes and more than 100,000
people.
Despite the ALS diagnosis, Baird never failed to meet his publishing
responsibilities.
“Perhaps the biggest pleasure came from the feeling that I was
part of the process of defining what was important. I enjoyed bringing
information to other residents and having a voice to express my views
and opinions.”
In the summer of 2004, Annabel’s stress-related health problems
forced the couple to sell the paper.
“I hated to see it go,” he said. “It began to seriously
affect my wife’s health, and it is not her time to develop a serious
illness.”
Today, Baird works mostly from his home as a software development consultant.
The Bairds have two children, Brian, 32, and Brittany, 29, and a granddaughter,
Nikki, 8.
“I have had a very good life,” he said.
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