Knowing of Dustin’s disease and
shortened life expectancy since the age of three, I grew up well informed about
myotonic dystrophy. Or rather, I thought
I did. My father told me soon after Dustin was born that my brother was likely
to die before I did. As a three year
old, I doubt I understood much of what that really meant, but Dustin’s
condition and outlook were never a secret from me. I knew my brother needed open heart surgery
soon after birth, required help breathing in the first three months of his
life, had a feeding tube for the first six years and wore oxygen in his nose to
bed until he was 11 or so. I knew
myotonic dystrophy gave my brother weaker muscles, that he had inherited the
gene from my mother and that I was likely unaffected based on my good health
and academic success. It seemed I knew
quite a lot about muscles and genetics for being so young.
Growing up I grappled with the
proposition that my brother was likely never to walk. I went along to multiple doctor visits to
evaluate Dustin’s ‘club foot’, a condition I didn’t know the technical term
for, ‘talipes’, until 15 years later. I
knew my brother’s feet curved in a way that caused his toes to curl in and
under towards the arch of his foot. I
remember reading in the hospital waiting room as Dustin underwent surgery for a
third time to tighten his Achilles tendon to correct the bend of his feet and
enable him to walk. The doctor let me
feel the mold for Dustin’s leg braces before it were to be cast. In caring for
a severely handicapped child, it is important all capable willing members of
the family are knowledgeable in how to assist in the daily exercises to build
strength and gain skill, and that the family members feel excited and important
in assisting the handicapped child. In
subsequent visits, I was taught how to put the braces on Dustin, tied his shoes
over them, lead Dustin to the rail, and helped him take steps.
In one visit where the doctor wanted to
train the family to teach Dustin to walk, I wondered if my brother would let me
help, or if I would be any good in helping my brother to do something he had
never before been able to do. I
approached the room my brother was in tentatively, wanting so much to be a part
of helping my brother to walk, to be more like me, to overcome the odds and
nervous that Dustin might not let me help.
Luckily, he was in a good mood and receptive; when I walked into the
room and said his name with open arms to go hug him, his lips raised in a
smile, he brought his hand to his mouth, shot his legs out in excitement, and gurgled
in delight, welcoming me in the activity as if what the doctor had done was a
game.
I smiled at my brother, and playfully
called him by his nickname: “Hey udey-dude.”
Dustin seemed relaxed, and apparently the doctor approved as he placed
the brace in my hand. In my left palm I
held the smooth back of the brace which was molded to my brother’s leg, curved
and smooth. In my right hand I held the
flat based that would go under his foot.
The inside was molded to his foot, but the outside was formed to create
a solid base and felt more like an odd type of dress shoe. I rubbed my thumb on the inside where the
ball of my brother’s foot would sit. It
had a crisscrossed pattern across it to create friction and prevent
slippage. The brace was impressively
light and durable. I raised the brace to
my brother’s foot, slid his heel deep into the corner of the brace, moved my
left hand to cover the front of his leg and hold the grace, then took the
bottom Velcro in my right hand, pulled it over his foot, and secured the Velcro
tightly onto the other side of the brace.
I pushed his calf against the back of the brace securely and began to
take the bottom strap diagonally to the top Velcro catch, then crisscrossed the
top strap to the bottom Velcro. Dustin’s
foot was secure and he didn’t seem to mind the brace. I eagerly took his
already loosened shoes, maneuvered them over the brace and his foot, and
carefully tied his shoe over the brace. I
had gotten to practice the steps of strapping my brother into the brace and
tying his shoes over them before; however, today was going to add another step
I had not yet been allowed to do.
I
felt my chest leap with excitement as we lead Dustin to the rails. I held my brother’s hand, smiled at him and
placed his hand on the rail. He let out
a breath which hissed in a tone between excitement and caution. His hand said he trusted me, but his eyes
widened as he looked down at his feet and realized how high up his head was
compared to when he sat on the floor.
His scrawny legs were weak and wobbly, awkwardly small amid his large
knobby knees and bulky braces. The physical
therapist in the room reached down to move Dustin’s leg, one at a time, and my
brother stepped about three feet with the therapist moving his legs, me holding
just above his hips, and the doctor watching.
It felt like a success and for a while my young heart thought anything was
possible.
As a child, I was told about myotonic dystrophy
in cautionary, optimistic tones. The
adults around Dustin wanted me to care about my brother and be hopeful, but
they didn’t want me to think the answer to Dustin’s troubles were like
something that Santa was going to deliver after flying to my roof and climbing
through my chimney. When the doctor
wanted to train me to help Dustin walk, I was partially seen as a resource who
could assist in getting Dustin daily practice and repetitions. I was an eager young girl who believed in her
brother getting stronger without the limits of logic or the experience of
seeing someone get weaker instead of stronger.
I was youthfully and eternally convinced that my brother could learn to
walk. I think the doctor was partially
willing to involve me in the process so that I didn’t hurt my brother by trying
on my own without training.
As Dustin passed infancy and grew from a
toddler to a young child, his body seemed to become healthier and more
durable. His vitality and capabilities
increased with time. By the time of the
attempts to get Dustin to walk, he no longer needed his feeding tube, but could
feed himself with a spoon or fork. He no
longer needed oxygen and could communicate with varying pitches and tones in
most vowel sounds and a large range of consonants. Dustin was able to wheel himself to the balloons
at Wal-Mart, interact during recess at school, and scoot anywhere in the house
using his arms to push his bottom forward. While his obstacles at birth were
severe and life threatening, his prognosis seemed to be ever improving as he
outlasted life expectancy predictions and progressed in ways doctors said he
never would. Learning to walk seemed
like the next logical step. All the news
that reached my ears seemed to be good news and besides when he was sick, I had
only seen my brother get stronger over the years; even when Dustin was sick, I’d
be told with the right medical attention, he would be ok. I began to think that
with enough prayer and willpower, anything could happen if God were
willing.
I wished fervently for my brother to be
able to walk. I prayed for him nightly,
threw quarters in wishing wells, and was diligent in helping him wear his
braces and insisted on constant walking practices with him after school. I would stretch
my brother’s legs, rotate his ankles, do resistance exercises and help him
practice standing. However, it didn’t
take long before Dustin didn’t enjoy wearing the braces much, and
sometimes I would want to practice longer than he was comfortable. My 10 year old brain was convinced my brother
was gaining strength and would one day be able to walk because he had broken so
many limiting expectations in the past.
At times I would hold Dustin’s hand next
to a rail, at others I would have him hold onto my waist and we would walk
together. After a while, our public
school built an apparatus which was basically a long flat board with side
supports and hand rail on either side so Dustin had a platform to walk
across. He had a stander at school, and
various walking assistance equipment. At
home, when Dustin would seem as if he were done walking, often I would have him
stand leaning on the couch. My fifth
grade logic thought this good practice, that standing after he had worked the
muscles would build good endurance and lead to muscle strength. I wanted my
will power and effort to be able to change the predictions on the limits of my
brother’s potential.
Dustin would lean with his forarms
resting on the edge of the couch. He was
tall enough that he would bend at the hip, his waist jutting out awkwardly from
side to side as he rotated his balance.
His knobby knees would dwarf the size of his calf muscles, which looked
like flabby flat skin hanging loosely from his bone. The braces seemed to double the width of his
ankles and extended almost half way up his leg.
He had to wear high top sneakers with the braces, which were filled to
capacity and were laced tight to hold the shoe in place. Occasionally, even with the braces, his
ankles would give as he adjust the weight around his hips and he would stand
with one foot flatly on the ground and the other would have rolled so that most
the side of his shoe touched the carpet.
After the third surgery, my brother again
seemed to be defying odds and was able to stand longer and even began to show
some definition in his calf muscles.
However, with his muscle condition, my brother’s feet did not quite look
like mine, even after a third surgery.
The weak muscles would not hold the tendon tight, and his feet again
began to curve in. His braces became a
discomfort, and weren’t fixing the problem or giving my brother a stable
surface to stand on. So, we took on more stretches and foot
rotations, trying to build strength in and around Dustin’s ankles so the
tendons would straighten. Sometimes, I
would add extra stretches in before bed or before school when Dustin woke up. I
persisted in practicing standing and walking with my brother. Three surgeries would be a waste without
appropriate practice.
I was watching my brother stand at the
couch a few months after his third foot surgery when my father came home from
working at the water treatment plant on Hill Air Force Base, in Utah . My father was dressed in his fatigues and had
the usual sweat and dirt on his uniform from a hard day’s work. Dustin was
standing, bent over on the hip with most the weight resting on his arms against
the back of the couch, grunting in a whining tone and shifting his weight
uncomfortably. My father saw my brother,
recognized his discomfort, and asked in a startled voice, “How long has he been
standing there?”
I had wanted my brother to practice, and
as a young child, even as an adult, I am not gifted in understanding other’s
limits. I’m not sure how long I had my
brother standing there, but I knew he wanted down, and I know I didn’t want to
let him, because in my mind practice, determination, and positive thinking lead
to physical growth and strength. My brother
had no graceful harm-free way to let himself down from standing next to the
couch without simply crumpling to the floor.
My father walked at a quickened pace over to where my brother stood,
bent over so his knee was on the ground and wrapped one arm under my brother’s
legs and one arm around his back. My
brother immediately embraced his father, reaching desperately around Randy’s
neck and letting his frail body fall into my father’s strong embrace. Randy
carried Dustin over to his favorite toys in the living room, placed him gently
on the floor, and began to play puppets with my brother, having Mickey ask
Dustin how his legs felt as if it were part of the game. I watched as my father’s tired and tight
muscles relaxed on the floor next to my brother’s tired and flaccid muscles,
watched their joy in playing and enjoying their moments together. I knew something in what I did was wrong.
After my brother went to sleep, my
father explained that Dustin’s body was different, that the surgeons were
trying to help Dustin walk by enabling his feet to look and stand the way mine
did, but even after that, Dustin’s muscles would have to grow strong enough to
hold his body weight and he would have to learn the skill of walking. It was going to be a long process. I had to be gentle with my brother. Tough practice wasn’t going to make his body
like mine; Dustin was just built different from me and for that I was very
fortunate. Dustin was never going to run
as fast as I did or try to practice pushups with his feet propped up on the
coffee table in the living room to try to win a grade school Presidential
Fitness award. The way I thought wasn’t
going to change my brother’s muscles and I could really hurt him in trying.
At that age, I was bigger than all the
boys in my class, finished fourth in the mile out of all the top runners, and
was routinely picked first or second when all the boys got together to play
recess football. If I tried harder, I
did better; I was strong and I was big.
For a month before the fifth “President’s Physical Fitness Challenge” I
‘trained’ at the house doing push-ups using the coffee table as an incline,
pull ups in dad’s closet, trampoline jumping acrobatics, and gave targeted
attention to the V-Stretch, my usual lowest score. Limits, in my young mind, were obstacles set
to be mentally broken by those tough enough to push through pain. I thought because my brother had defied so
many odds and expectations with good health in the past that with the right
mind set and unfaltering effort he could be pushed into walking. However, pushing my brother in the way I
pushed myself didn’t bring him to walk, and my blind optimism and stubborn
determination brought him pain.
As the months passed after the surgery,
it became obvious that the progress was temporary and that my brother’s feet
would continue to curl back to where they were or worse. The doctors thought about taking muscle from
his knee or shoulder into his ankle, but in the end the process and training
was ruled too painful or futile to be worth the higher risks. Each year of my
brother’s life, my father’s military insurance deductible had to be paid in
full. Perhaps there were surgery types
available that could have helped Dustin walk, but with the financial bills we already
had and the rates the insurance company was paying the hospital, our money was
not going to buy Dustin a cutting edge experimental surgery. There would be no more corrective surgeries
or medical attempts to help Dustin walk on his own.
The special education department at school
still had time for Dustin in the stander, but he practiced taking steps for
shorter periods and eventually it was generally accepted that Dustin was not
ready to walk and would probably never walk unassisted. When the military changed my father’s
assignment and moved us to a new school district, Dustin did not have a walking
training program that focused on steps, but instead focused on him standing
longer periods. My brother’s progress
was no longer going forward with walking and instead the focus was shifted on
making his arms strong enough to maneuver himself in his wheelchair.
Most who are born with congenital
myotonic dystrophy and survive to age 10 can walk. However, Dustin’s mere survival was laughing
in the face of known medical odds.
Dustin’s myotonic dystrophy carried so many repeats of the wrong protein
triplet on chromosome 19 that he didn’t really fit medical textbook cases, or
there were enough additional birth complications that there was no simple
comparison. When he was born in 1989,
there weren’t many children who survived birth with myotonic dystrophy as
severe as his, and I have never met another as severe as my brother. The muscle disease often creates weak enough
hearts that infants don’t survive outside of the womb. My brother had successful heart surgery right
after birth. My brother turned blue from
lack of oxygen almost immediately after birth, and infants with myotonic
dystrophy often cannot breathe on their own.
However, Dustin had grown strong enough to only need oxygen at night
when he was sick. Many children born
with severe congenital myotonic dystrophy simply don’t survive except by stroke
of miracle or luck, or the improving medical services available and good
staff. Perhaps now, over twenty years of
medical advances later, I could find more children like my brother, or perhaps
with early detection that type of child is still rare because more are aborted
in utero than born in the modern era.
My brother had constant medical attention
from an experienced doctor at Wolford Hall Military Hospital. The military moved our family strategically
close to the best Air Force Hospital available.
In Salt Lake City, Dustin had a team of doctors, and a university
research hospital paid us to bring Dustin in for additional studies involving
graduate students and sleep studies.
Dustin was treated by well informed, well paid, highly intelligent,
caring individuals and he thrived in those circumstances to become a healthy
and happy young man who did not need oxygen, a feeding tube, or overnight
medical stays for years at time. His
severe case helped rewrite the medical research as a living example of a
genetic mutation that cause most to die in infancy and was a brilliant success
story of modern medicine and prayer.
My mother, however, fought the creeping
slowness of adult onset myotonic dystrophy in oblivion, away from all the
geneticists, large military hospitals, or graduate work research. She was diagnosed with myotonic dystrophy
soon after Dustin was born by a simple handshake. Myotonia is a condition where the muscles
have trouble relaxing after contracting.
If my mother shook someone’s hand, she would have to physically push her
palm back into place or wait a long time for it to return there itself. Myotonia is not present in congenital
myotonic dystrophy, but if a child survives into adulthood, the hand release
difficulty of myotonia appears. My
mother had it.
My mother was diagnosed in her medical
records as a carrier since 1989; she would be affected by the disease more and
more as time went on. While my father
was still in the military, when we were around the best medical care, my
mother’s disease did not have overt affects.
My father retired from the military in 1999, three years before Dustin
passed away, and thirteen before my mother died. Dustin’s medical records were extensive,
multiple huge folders worth. When we
lived in Utah, the last place my father was stationed, we had most of his
records, but some of the earliest were lost or discarded. My brother had a large enough file that no
doctor could go through all of it, nor would they need to. His disease was obvious. When my father retired from the military, the
family medical records were sent to the National Archives. If we knew more, we could have kept them, or
requested all of our records. We
didn’t. Dustin regularly saw a doctor in
Kansas, and the records from the last hospital were requested and sent. He lived in one city for the rest of his
life. His medical care was good enough.
My mother, however, did not have her
full medical records when she was taken to the hospital in small town Larned,
Kansas. From Larned, she had to wait
over two hours for an ambulance to take her to Hays Medical Center. Hays
Medical Center did get the medical records from Larned, but that apparently did
not include the diagnosis that my mother had myotonic dystrophy.
I've been told this was the first, or one of the first times I got to meet my brother - in a mask in a hospital.
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