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Zach Butler, at the
steering wheel of the golf cart provided by Pat Argo and
other caring citizens of the Town of Trezevant, gets
ready for a day of fun with best friend Jonathan
Triplett.
Trezevant's Zachary Butler is an amazing young man with
boundless energy and an urgent smile that tolerates only
briefly notions that, sometimes, he might need to slow
down.
His zest for living could be due to the fact that he
learned at a tender age hard truths regarding life and
death that many people don't encounter for decades. He
was just 12 years old when he lost his father
unexpectedly, then was forced to come to terms with his
own mortality after learning he was victim of the same
disorder that likely cut short the life of his father.
At first glance, no one is the wiser that Zach has a
disabling disease that limits his ability to walk
without stumbling, or would guess that, at 18 years old
as of August 5 last year, he has already endured two
heart attacks. His mom, Cynthia Mayberry, has traveled
the uncertain road alongside him, bearing the bumps and
wildly winding curves with the help of family and a
caring community.
Most recently, Pat Argo, owner of Pat's Restaurant in
Trezevant where Cindy works on Saturdays after working
Monday through Friday at the Dura Plant in Paris,
spearheaded an effort to provide a golf cart for Zach.
Forced to rely upon walking when his state-provided,
motorized wheelchair was picked up for repairs several
months ago with no date certain for its return, people
about town became concerned for his safety.
Cindy explains, "People here in town kept saying,
'Cindy, he's going to get hurt.' He was falling a lot,
because he's an active kid; he just gets out and goes,
nothing stops him."
Finally, according to Cindy, Pat and her daughter Stacey
Hames "just couldn't stand it" any longer. Pat decided
to buy the golf cart and ask for donations; if she
didn't get enough to cover the costs she would pay for
it on her own.
But help came through as local farmers, merchants, and
other customers of the restaurant pitched in. "Just
people who know us gave money," says Cindy. She plans to
pass on the kindness by giving back the golf cart once
Zach can no longer use it, for use by someone else in
need. However, with the rapid advances being made in the
disease that afflicts him, Zach may well wear it out by
then.
In the meantime, Cindy says she's glad she doesn't have
to worry about him falling out of a tree while partaking
in his favorite sport - hunting. The cart is now rigged
to Zach's specifications with camouflage material
covering the canopy and a wooden bar attached to the
canopy's front supports as an aid in keeping his aim
steady while hunting.
Growing up the son of sportsman Bubba (Henry Harold)
Butler, Zach took to the woods early in life, learning
to love hunting and the outdoors.
"I've been interested in hunting my whole life," he
says, "My dad was a big time sportsman - hunting,
fishing, sports, everything."
Zach took his first deer, a one-pronged buck, when he
was in the eighth grade on a hunting trip with his
grandfather, Willie Rogers of McKenzie.
"He was so excited, so excited," Cindy smiles.
This past Christmas, he couldn't wait for his gift, a
silver and black muzzleloader that he convinced his
mother he had to have early for deer muzzleloader
season.
He got the gun the week of Thanksgiving, just after
receiving the golf cart that symbolizes the commitment
of the community to ensuring Zach has the independence
and mobility so important to a young man his age.
"I've never seen a kid so happy in my whole life," Cindy
says regarding her son's reaction at receiving the cart.
"I love having it," smiles Zach, appreciative of his
increased freedom. The wide seat allows him to share the
ride with friends, especially best friend and hunting
buddy Jonathan Triplett.
"We've grown up together," grins Zach.
Another major influence in Zach's life is his brother,
27-year-old Bobby Birdwell, who is also a hunting
enthusiast. "I did go hunting with him all the time,
until he got a good job at Nashville," Zach says with
just a tiny lump in his throat, happy for his brother's
good fortune.
In January 2002, Zach and Bobby were invited on an
all-expense paid weekend hunting trip to Rich Wingo's
Willow Oaks Farm just outside Tuscaloosa, Alabama. The
trip transpired after Zach sent a letter to Buckmaster's
Whitetail Magazine T.V. series. The letter was read by
Brother Thomas Dunlap who shared the letter with Wingo,
formerly a linebacker for the Alabama Crimson Tide from
1976-1978.
"He told me he was touched by my letter," relates Zach,
recalling Dunlap's brother had the same disease.
"It was always his dream to go on a guided hunt and kill
a big deer to put on the wall," says Cindy, and that is
just what happened when Zach brought down a big eight
pointer that Wingo had mounted and delivered to his
door.
Zach was also treated to a turkey hunt last spring in
Lavinia, courtesy of the Lavinia Gun Shop.
"Mike Smith took me hunting," smiles Zach, describing
the hunt that took place in a tent.
And during the fall of 2001, the Make-A-Wish Foundation
sent Zach on a six-day, seven-night trip to Hawaii where
he went deep sea fishing, visited the lush valley and
mountains where Jurassic park was filmed, went
parasailing and viewed by helicopter the volcanoes that
formed the islands, including the earth's most massive
volcano, Mauna Loa, whose summit rises 13,677 feet, and
Kilauea, the world's most active volcano.
Zach enjoys fun in the
surf during a Hawaiian vacation made possible by the
Make-A-Wish Foundation.
Accompanying Zach was cousin Jason Mayberry of Trezevant,
his mom's sister, Rina Rogers and her daughter Taylor,
who live in Florida.
The Make-A-Wish Foundation's mission is to "grant the
wishes of children with life-threatening medical
conditions to enrich the human experience with hope,
strength and joy."
That's a goal both Zach and Cindy can understand, given
the uncertainty of Zach's future. Yet, notes Cindy,
"Young or old, you never know when you're not going to
be able to get around."
Zach's rationale for the present is simple. "I try not
to think about it," he says.
"I just thank God he's alive," Cindy continues, emotion
tugging at the corners of her smile. "He doesn't like
anybody to feel sorry for him. He lives every day to the
fullest. Everything he can do, he finds a way, and if he
can't he finds someone to help him."
Cindy and Zach track the years since discovering his
illness, a time line that begins in 1997 with his
father's death of a heart attack in his early 40's.
Two months later, Zach was outside dragging old
furniture into a nearby wooded area to make a fort.
"He got real quiet," recalls Cindy, who walked into the
yard calling after him only to discover him lying face
down in the dirt. Panicked at the blue hue of his skin,
she shook him into consciousness.
"I just remember waking up crying," says Zach, who
recalls complaining previously about his chest hurting
simply from walking.
An immediate trip to the doctor revealed Zach had
experienced a heart attack, the result of a form of
cardiomyopathy (disease of the heart muscle). His
particular form of cardiomyopathy is characterized by a
thickening of cardiac muscle that reduces the size of
the heart's chambers, thereby decreasing its pumping
efficiency. The enlarged heart does not adjust properly
to the demands of the body, resulting in arrhythmias -
heartbeats that are too fast or too slow.
Later tests at a Nashville neurologist's office revealed
Zach was the victim of Friedreich's Ataxia, a rare
genetic disorder (affecting one in 50,000 persons) that
affects the nervous system and the heart, causing muscle
weakness and "ataxia", a loss of balance and
coordination.
As a rule, the ataxia first affects the legs and torso,
causing an increasingly unsteady gait, a tendency to
stumble or reduced athletic ability. Over time, walking
becomes more difficult as muscles in the legs become
weak and tire easily. Persons afflicted with the disease
generally begin using a wheelchair between five and 15
years after the disease process begins. Even then,
however, many people who have lost the ability to walk
maintain their upper-body strength and coordination for
years to come. Eventually, they may have difficulty
speaking clearly due to a loss of coordination and
weakness of the tongue and other facial muscles, and may
have problems swallowing.
Problems like these make it increasingly difficult for
school age victims to cope with peers whose
misunderstanding of the disease leads to overly harsh
judgments regarding intellect and aptitude, however,
Friedreich's Ataxia in no way affects those portions of
the brain involved in mental functions and does not
affect thinking or intelligence.
Nevertheless, unwilling to endure the taunts of other
teens at school, Zach decided two years ago to
discontinue his formal schooling, opting to study at
home. He plans to obtain his G.E.D. and to enter
vocational school in a plan of study having "something
to do with computers."
He has little concern of his ability to excel in his
choices. "I was always real smart in school," he says.
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More About Friedreich's Ataxia
Although no cure exists for Friedreich's Ataxia,
there are treatments for the heart symptoms and
"ways to manage ataxia and muscle weakness."
Because of available treatments and the fact that
mental ability is unchallenged by the disease,
many people with Friedreich's Ataxia complete
college, hold rewarding careers, and lead
satisfying lives that often include marriage.
HOW IS FRIEDREICH'S ATAXIA ACQUIRED?
Friedreich's Ataxia is only acquired when both
parents are carriers of a mutated gene, located on
chromosome number 9, that is passed on to their
offspring. Amazingly, one in every 100 persons in
the United States is a carrier of the bad gene.
That goes up to one in 70 for some individuals,
such as those in the Cajun population.
Generally, in people with Friedreich's Ataxia, the
two defective genes (one from each parent) contain
a section of genetic code that is repeated many
times - up to hundreds or thousands of times above
the normal range of five to 30 repeats. As a rule,
the longer the repeat, the earlier the onset of
the disease and the faster its progression
The gene, when normal, is believed to control the
production of a protein called frataxin that
regulates levels of iron inside the mitochondria
(tiny cellular powerhouses that produce energy
from oxygen.) Research indicates frataxin may act
as a "storage depot" for iron, releasing it only
when it's needed. While iron is essential for the
growth and reproduction of cells, excess levels
are highly toxic, allowing the build up of
destructive oxygen-based free radicals which
damage the mitochondria.
For the most part, Friedreich's Ataxia affects the
spinal cord and the peripheral nerves, which
includes those connected to the muscles and
sensory organs. It also affects the cerebellum,
that portion of the brain situated beneath and to
the back of the cerebrum, which controls muscular
action, helping to provide "smooth, coordinated
body movement."
The muscular dystrophy association website (www.mdausa.org,
which addresses a wide variety of neuromuscular
diseases) explains that when one decides to move
his or her arm, nerve cells in the brain send an
electrical signal to the spinal cord which is
passed on to the arm muscles through the
peripheral nerves. When the arm moves, the
movement is felt due to nerve cells in the arm
muscles that return a signal back through the
peripheral nerves to the brain. In persons with
Friedrich's Ataxia, however, the flow of
information is hampered, complicated by similar
impairment of muscle-controlling signals between
the cerebellum and spinal cord. These are the
causes for the "progressive loss of balance,
coordination and muscle strength" in Friedrich's
Ataxia. |
Zach experienced his second heart attack while
attending a high school football game in 2001, a couple
of months after coming home from Hawaii.
"It started at the ballgame," says Cindy, "When he came
in the door I knew something was wrong. I went to the
neighbors and called 911 and within three minutes they
were there."
After the second attack, Cindy stepped up efforts at
making Zach's environment more friendly to his needs.
She expresses again and again her thanks for everyone
who has made a difference in his life.
"There are so many that have helped; most people around
Trezevant have helped in one way or another," she says
sincerely. "I wanted to say thank you to the people in
Trezevant for all they've done. Trezevant is a small
community where everybody knows everybody, and they are
really good people."
She extols again Pat Argo, who, she says, donates time
as well as money in philanthropic endeavors. "She raises
money for kids - it's just something all the time -
she's behind a whole lot of help that goes into this
town."
Zach agrees, nodding, "She's got a good heart."
"A heart of gold," Cindy says quietly.
Help from state-run family support agencies has been
less forthcoming, with officials stating, according to
Cindy, problems in permanently altering for handicapped
persons a home that is rented rather than owned. Cindy
relates how Pat arrived at her home with a ruler and
planks to try and figure out herself how to build a
wheelchair ramp, which is one of the improvements
requested by Cindy. She says she has received increased
communication since involving State Representative Chris
Crider in her efforts and hopes to learn in February
what steps can be taken to provide Zach with the ramp
and other needs, such as handrails along their home's
entryway.
"All I want to do is make his life easier," she sighs.
As for the immediate future, Zach's plans are to go
hunting and have fun.
"It's been a good winter for Zach," his mom smiles.
Echocardiograms (ultrasound scans of the heart) once
scheduled every two months are now six months apart, a
trend Cindy hopes will continue though she worries his
recent bout of walking after months without the
wheelchair may have taken a toll.
Still, she acknowledges, "Miracles happen every day."
Miracles do seem to be taking place lately on the
research front in Friedreich's Ataxia. According to the
Muscular Dystrophy Association, the average lifespan of
people with the disease was gauged some ten to 20 years
ago to be between 30 and 40 years after the initial
diagnosis, with heart disease and diabetes causing the
greatest risk of fatality.
But heart disease, Friedreich's Ataxia's most
life-threatening symptom, can be treated with the same
medications and devices, such as pacemakers, used by
anyone with heart disease, easing the workload of the
heart and stabilizing arrhythmic heartbeats. Another
frequent complication in Friedrich's Ataxia victims -
diabetes - can be managed with diet and insulin as in
the general diabetic population.
Physical therapists can help maintain flexibility in
tight muscles, making it easier to cope with progressive
problems involving balance and muscle weakness, and
speech therapists are actually able to help retrain
tongue and facial muscles if speech and swallowing are
affected.
More exciting are the remarkable advances made by
researchers who, having gained a greater understanding
of the disease processes, are on the threshold of
delivering treatments that are expected not only to slow
the effects of the disease but to actually reverse
abnormalities of the heart and potentially deter the
disease's attack on the nervous system.
Clinical trials in which victims of Friedreich's Ataxia
are treated with antioxidants such as Coenzyme Q10,
vitamin E and idebenone have yielded promising results
in eliminating the free radicals that accumulate in
their cells. Other antioxidants being tested in the
laboratory have yet to be tested in clinical trials.
The antioxidant coenzyme Q10 (coQ10) - also known as
ubiquinone - is available over the counter as a dietary
supplement. It works inside the mitochondria of the cell
to help combine oxygen with "fuel" from carbohydrates
and fat to produce energy. Clinical trials show that
coQ10 combined with vitamin E (also available over the
counter) can increase energy production in both the
heart and voluntary muscles of individuals with
Friedreich's Ataxia. As research continues, it is
important to note these antioxidants under study remain
unregulated by the U.S. Food and Drug Administration;
therefore there is no guarantee of quality or safety of
the products, which are not covered by insurance.
Because coQ10 is available without a prescription,
researchers unable to interest pharmaceutical companies
in the product created idebenone, a synthetic variety of
coQ10. Remarkably, the synthetic version has proven even
more promising, actually acting to shrink the enlarged
hearts of people with FA.
Despite the promise of new advances, life in the real
world for Zach and Cindy has its ups and downs.
"It's really hard; I don't want him to miss nothing in
life," Cindy says, her strong façade breaking into the
heart of every-mother. "You can't stop an 18-year-old;
He is so energetic, he goes and goes and goes. I can't
stop him but I don't want to, I want him to do what he
wants to. I just make sure he has a cell phone in his
pocket." |
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