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 Joshua's
Journey: Diagnosis and Decision (Part 1 of 3)
Joshua's
Journey: The surgery (Part 2 of 3)
Joshua's
Journey: A new beginning (Part 3 of 3)
Part 2 - Joshua's
Journey: The surgery
by Todd and Karen Schulz, Lansing State Journal,
August 19, 2002
The final seizure came at 4 a.m. The attack startled Joshua
from his sleep - the deep, serene kind only babies achieve -
as he laid between us in a queen-size bed in Detroit. Karen
cradled Joshua in the quiet darkness, watching what had become
a typical but terrible routine.
Todd Schulz shares a moment with his son before Joshua is
taken to surgery to have the right side of his brain removed.
Todd's wife, Karen, wipes his tears as both parents struggle
with the fear and anxiety of handing their child over to
doctors for an 11-hour operation. Joshua was born with a
severe seizure disorder.
Every 10 seconds or so, an electrical storm racked Joshua's
tiny 3-month-old brain, causing his eyes to bulge, his arms to
stiffen and straighten and his legs to curl toward his torso.
The short reprieves between strikes were filled with Joshua's
fussing and crying. As usual, the seizure raged for about five
minutes, leaving him limp and exhausted. It was one last
painful but powerful reminder of why we'd come to Children's
Hospital of Michigan and what was at stake this day.
We'd come seeking help and hope for Joshua, who was born with
hemimegalencephaly, an extremely rare seizure disorder
threatening to severely disable or kill him.
We'd come so a surgeon could remove the right half of his
brain. We'd come because we had no other choice.
At 7:20 a.m. on May 16, a dreary Detroit morning, a small
convoy left our hotel for the short walk to Children's
Hospital. Todd's mother, brother and sister-in-law were with
us as we trudged nervously through the drizzle.
Joshua was awake and hungry - but content - in his mother's
arms. He was blissfully unaware of his desperate circumstances
or the drastic procedure he was about to undergo. He did not
know where he was going or what fate held in store.
Neither did we.
What we did know was the gravity of our third child's
situation. Constant seizures - which started on the second day
of Joshua's life - were burning out the right side of his
brain, which was larger than the left and badly malformed. The
spasms were starting to affect the left side.
For Josh to have a chance at a normal life, or any life, the
seizures had to be stopped. Medication had failed. Removing
the flawed half of his brain - a procedure called a
hemispherectomy - was our last best hope.
Many major medical centers in the United States won't perform
the surgery on children under the age of 1. But Children's
Hospital, ranked among the nation's best, and its
world-renowned pediatric neurologist Harry Chugani take a more
aggressive stance.
Even so, Joshua was a young surgical candidate. Children's
Hospital performs on average only one hemispherectomy per year
on children his age.
Although the risk was tremendous, we couldn't wait. If surgery
was delayed until Joshua was 1 year old, Chugani told us, he
might never recover developmentally.
So on May 16, we reluctantly placed Joshua in the hands of
doctors - and a higher power.
7:30 a.m. Hemispherectomy primer
Preparation: The medical team prepares Joshua for a
hemispherectomy, a surgery reserved for people with severe
disease on just one side of the brain. Joshua's head is shaved
and anesthesia is administered. Joshua is placed on a
ventilator and hooked up to intravenous fluids and monitors
for his heart rate and other vital functions.
Surgery begins: An incision shaped like a backward question
mark is made. The scalp is peeled back and a piece of his
skull removed. In children as young as Joshua, the skull is
still soft.
Tracking the storms: More than 100 EEG (electroencephalogram)
wires are attached directly to the brain to monitor seizure
activity and help doctors determine which tissue to remove.
Surgeons then locate and secure three major blood vessels to
prevent blood loss. The surgery is essentially "bloodless."
Finding the trouble spots: Using EEG data and MRI and PET
scans performed earlier, Dr. Sandeep Sood tediously identifies
diseased tissue. Others, including Dr. Harry Chugani, assist.
Brain tissue is slowly removed: For Joshua, the entire right
side is removed, including the four lobes. Deeper structures
called basal ganglia and thalamus — responsible for balance
and reflexes — remain. he damaged
right side of the brain is removed, leaving the left side.
Right-side functions include visual spatial skills, movement
on the left side and memory for pictures and faces.
Waiting and hoping: About 11 hours after entering the
operating room, Joshua's surgery is done. The void once
occupied by the right side of his brain is now filled with
blood and cerebrospinal fluid. The brain does not regenerate.
The remaining tissue does not "float" in his head. The piece
of skull removed for surgery is reattached with dissolvable
screws. The scalp is stitched to close the incision.
As we arrive at the hospital with Joshua, we're greeted by
more family. We take Joshua into the admitting department and,
although it sounds like something from a bad science fiction
movie, sign paperwork granting the hospital permission to
remove the right side of Joshua's brain. We are overwhelmed
but determined and, in a strange way, relieved. Finally, we
are taking action for Joshua. We're quickly ushered to the
bustling pre-operative area on the second floor, where a nurse
weighs Joshua and checks his vital signs. Joshua weighs 14
pounds 2 ounces, with clothes on. A few weeks ago, Chugani had
said we'd wait until Joshua weighed 15 pounds to do the
surgery. But we cannot wait.
8:55 a.m.
We've met and/or talked with about a half-dozen doctors and
nurses, including neurosurgeon Dr. Sandeep Sood, and we say
goodbye. We shower Joshua with our kisses, run our fingers
through his thick, black hair - which will be shaved - for the
last time and watch helplessly as he's carried away. We're
frightened beyond words, yet reconciled to the necessity of
the surgery. More importantly, we trust in God's will for our
child and that brings peace.
What will the next hours hold? Joshua's surgery is scheduled
for 9 a.m. The operating room has been reserved for six hours.
The first hour will be spent preparing Joshua for surgery.
Sood asks if we want just half of Joshua's head shaved and
then jokes he doesn't charge more to shave the entire head.
Hospital chaplain Anna Souto joins us prior to Joshua's
surgery. We pray on the Bible verse Joshua 1:9, suggested by a
friend.
9:12 a.m.
Joshua enters the operating room.
10:02 a.m.
We wait in a parent waiting room with about a dozen family
members and friends, including our pastor Jeff Nunham. We
pray, make idle chitchat, and wait to hear that Joshua's
surgery is under way.
10:58 a.m.
The first incision is made.
11:06 a.m.
The nurse attending to Joshua calls on a waiting room
telephone to tell us surgery is under way. "Joshua is doing
well," she says. The news is encouraging but tempered by
reality as we dig in for a long day.
11:45 a.m.
Chugani comes to the waiting room and his presence is
assuring. Everything is going well, he says. The surgeons have
"opened" the skull that, in the case of children Joshua's age,
is still soft and are hooking EEG wires directly to the brain.
The idea is to specifically identify the trouble areas that
must be removed. Chugani says the plan is to take the whole
right half but, if the EEG tests confirm the back portion
appears to be normal, perhaps they will only remove a portion
of that side after all. We've been praying, both with Souto
and Nunham, and that helps. Chugani tells us to eat now, as we
may not have another chance. Most of our mob walks to get
take-out from a Wendy's restaurant in the basement of Harper
Hospital located next to Children's Hospital.
1:28 p.m.
Another update from Chugani. The right side of Joshua's brain
is "very deformed, but we knew that," he said. The back of the
right side was spiking dramatically, so they will remove the
entire right side. The tissue is not ridged, like normal brain
tissue. Parts of it appear flat, Chugani said. It's like the
right side is dying, he explains. After Chugani leaves, we
take a quick walk to the cafeteria for a snack. Karen says,
"Even though tests indicated Joshua's brain was deformed, part
of me was hoping that when they opened his head and looked at
the brain, that it would appear normal. That it would at least
look OK." Surgery was not a mistake. This is the right
decision. It will be several more hours.
3:05 p.m.
Nursing shift change. The nurse who has been at Joshua's side
since the morning reports Joshua is doing well. The doctors
are close to removing the right side - perhaps 2 1/2 more
hours. Joshua has had one blood transfusion, using one of two
units of donor blood supplied by family friends.
4:57 p.m.
Chugani updates us again. About 30 more minutes until the
right side of Joshua's brain will be entirely removed. Pastor
Nunham leads the entire group in prayer to celebrate this good
news. Chugani estimates it will take an hour to close Joshua's
head and then one more hour of monitored recovery before we
can see him. The mood in the waiting room visibly lightens. We
acknowledge that the end is near; we sense that Joshua,
although still in surgery, has made it without complications.
The awful waiting - for a diagnosis, seizures, tests, a
surgery date, the operation - is behind us. Even most of this
day, when we handed our baby boy over to a doctor we barely
knew to remove half of his brain, is nearly over. Relief
washes over us. We can, and do, relax. We start talking about
more than seizures and therapies and other medical stuff.
5:30 p.m.
The waiting is brutal, but you can only wring your hands and
pray so much. Eventually, the guys migrate to one side of the
waiting room, breaking out a deck of cards and dragging over a
small table to play euchre. We talk about guy stuff: Golf,
hunting and other sports. The women are sewing and chatting.
Laughter eases the tension. At one point someone describes a
decision as a "no-brainer." Todd shoots back, "as long as it
wasn't a half-brainer." At least we can make jokes, albeit bad
ones, about it. We all start to play word games, calling out
answers like guests at a dinner party. For an instant, the
room takes on the tone of a family gathering - until we
remember the reason we're gathered. Then the angst and urgency
come rushing back.
7:30 p.m.
It's been 2 1/2 hours since our last update. Did something go
wrong? Why haven't we heard? We were told the surgery would
last six to eight hours. It's already been more than 10 hours.
We are worried, exhausted and in need of answers. Our group is
the only one left in the waiting room. Even the volunteer who
tends to the telephone is gone. We are at our breaking point.
Karen begins pacing and searching the halls for anyone who
could tell us anything about Joshua's status.
7:40 p.m.
Karen tracks down a nurse, who tells us Joshua has about 20
more minutes in the operating room. We were told the surgery
would last six to eight hours. He's been gone for 10 hours, 40
minutes. We are tired, haggard. We cannot wait to see Joshua.
8 p.m.
Finally, Dr. Sood emerges alone, still wearing surgical
scrubs, to report that Joshua's surgery is complete. He was
transfused with blood twice and did "remarkably well," Sood
says. After 11 hours of drama, Sood's three-minute speech
seems anti-climactic. The surgeon doesn't seem to grasp what
he's done for our family. But the few words he offers are
encouraging and he is obviously exhausted as well. We just
want to see Joshua.
As Sood leaves, we embrace. Most of the friends and family
who've gutted out the day with us head for home. Todd's
mother, Marti Kenel, and Karen's sister Ruth Mayercak and her
husband, Phil - Josh's godparents - are staying overnight. As
they go in search of much-needed food, we head to another
waiting room near the pediatric intensive care unit, where
we'll finally be able to see our son.
8:15 p.m.
Joshua leaves the operating room.
9:02 p.m.
Joshua is in his room, where the staff is getting him
stabilized. Soon we will see him.
9:15 p.m.
We get our first glimpse at Joshua. His face is barely
swollen, although that will change dramatically in the coming
days. His head is wrapped in a thick white bandage. He has a
ventilator tube up his nose and three intravenous lines plus
various other monitors. Todd says he looks good, considering
all he's endured. Karen agrees but is fixated on the fact that
her son now has just half of a brain. His feet are cool to the
touch. His right foot moved when Karen touched it; the left
foot did not move.
Earlier, Sood warned us that Joshua may have no movement at
all on his left side for several weeks. The right side of the
brain is usually responsible for movement on the left side of
the body. Joshua's abnormal right side is gone now - donated
to an epilepsy study at Wayne State University - and,
hopefully, the left side of his brain will reorganize and
assume responsibility for this function. Doctors believe
Joshua's left side of the brain had begun to reorganize even
before surgery because the right side of brain was constantly
seizing, allowing little opportunity for it to function
properly.
As we sit with Joshua, he occasionally
makes a sucking motion with his mouth. Maybe he's
dreaming of a bottle. The nurse just took his temperature
under his right arm. Joshua cried, but there was no sound. The
ventilator tube prevents him from making any noise with his
vocal cords. His entire face cried, but he was silent. It
broke our hearts. We are surrounded by the hums and beeps of
the ventilator and monitors, and a Scooby Doo rerun airing on
the television above a small girl in the next bed.
10:43 p.m.
Todd's entry in our personal journal: "Thank you, God! Joshua
is alive - and will have a life. We praise you."
11:35 p.m.
We return to the hotel next to the hospital, exhausted but
exhilarated. Our two-block walk through the dark isn't nearly
as long as it was this morning. Just moments ago, as we were
leaving Joshua's bedside, Karen nudged her finger into
Joshua's left hand. He squeezed Mommy's hand.
Part 3 - Joshua's
Journey: A new beginning
by Todd Schulz, Lansing State Journal, August
20, 2002
The startling question came from the pew in front of us. "Did
Joshua die?" a friend's daughter asked, craning her neck to
look at our family.
"Pardon?" Karen asked, as the Sunday church service began.
"Did Joshua die?" the little girl repeated.
"No, honey," Karen replied, and pointed to Joshua, our
6-month-old son seated next to his mom. "He's right here."
"Oh, he looks different."
"Yes, he does," Karen answered, smiling.
The little girl's confusion was understandable. We, too, have
marveled at the differences in our son since doctors removed
the right half of his brain to control crippling seizures.
A baby once too exhausted to keep his eyes open is now alert
and attentive. A baby whose body was once racked by
convulsions is now content and responsive. A baby who showed
no emotion now squeals with delight at Daddy's kisses, cries
for his mother's attention and interacts with his older
siblings.
No, Joshua did not die.
Our son was reborn on May 16 - the day doctors stopped the
seizures and gave him a new start. It's a day we will
celebrate in years to come as a second birthday of sorts for
Joshua.
Doctors, therapists, friends and family members are impressed
with Joshua's progress in the three months since his
hemispherectomy. After months of seizures, Joshua hasn't had a
single one since the surgery.
But, as expected, the surgery has brought new challenges.
Joshua has limited use of the left side of his body. The
remaining half of his brain must work extra hard to learn the
skills the right side of the brain usually controls, including
movement. He's lost nearly half of his vision. He still takes
anti-seizure drugs and receives extensive physical and
occupational therapy.
Our lives are hectic. But we are fulfilled, too.
The six months since Joshua was born have helped us realize
the importance of relationships - with each other, and with
extended family and friends. It also has deepened our
Christian faith.
Joshua is very much alive.
Rays of hope
Joshua was born with hemimegalencephaly (HME), a seizure
disorder that strikes about one in 200,000 children. The right
side of his brain was enlarged and underdeveloped. About the
only thing the right side of Joshua's brain did for him was
cause seizures.
As doctors informed us of the risks of the surgery, they told
us that Joshua might not move the left side of his body until
several weeks after the operation. Within hours, however,
Joshua was moving his left leg. Doctors predict Joshua will
walk, possibly with an ankle brace.
Joshua's left arm and hand move less than his leg, but the
movement itself is encouraging. The neurologists remain
cautious. The movements in his arm and hand are random. Last
month, Joshua received a splint for his left hand to wear
during active times, including play and therapy sessions. The
splint helps extend his left thumb. Without it, Joshua's hand
is curled tightly much of the time.
Joshua's neurologist, Dr. Harry Chugani, warned us his left
hand will be a "helper hand."
"Will he throw a ball?" Todd asked.
"He will be right-handed," Chugani told us in March. "He will
be strongly right-handed."
As for throwing a ball, the other day Joshua used his right
hand to launch his rattle across the room.
Watch out, Roger Clemens.
As we wrote this article, Joshua was laying on the floor. He
was on his belly, grunting and straining to lift his head up,
to roll over. He seems to want to do things typical of
children his age, but his weaker left side doesn't always
cooperate.
Our older children - Samuel, 5, and Madeline, 3 - notice the
differences in Joshua, too. One day recently, Sam and a friend
were playing with action figures. They wanted to include Josh,
who was staring wide-eyed at the "big boys."
"That's his hand that doesn't move," Sam told his buddy. "You
have to put toys in the other one."
Karen pried open Joshua's tightly fisted left hand and placed
one of the action figures inside.
"Joshua likes your toys," she said.
"Cool," Sam answered. "Josh can play with us."
The road we assume children will follow, one we took for
granted with our first two babies, is shrouded in fog for
Joshua. We can only pray, watch and wait to see how he keeps
pace with milestones to come.
The prognosis for children with HME varies wildly. Some
parents we've befriended via the Internet tell us their
children are still struggling to walk at age 3. On the other
hand, we recently read a story about a girl who underwent a
hemispherectomy - for a different seizure disorder - and is
now a teenage honor student and a member of her school's
bowling team.
The doctors can tell us little about Joshua's potential. Or,
perhaps they won't, for fear we'll set the bar too low or baby
him.
Before the surgery, Chugani would say only that he's seen
children with HME do "very well."
"Define very well," Todd said.
Pressed, Chugani said he's seen patients develop with low
normal IQ ratings and attend school. But those patients, he
said, underwent the surgery at a later age than Joshua.
At Joshua's age, the brain is better able to make new
connections, Chugani told us. The left side of Josh's brain
may learn how to do functions usually performed by the right
side.
Doctors have told us to treat Josh like our other children. To
hold the same high expectations in our hearts. To push him,
and to see where he leads us.
Joshua already has come an amazing distance. There are even
rare times - beautiful slivers between the constant therapy
sessions and medicine mixing - when we forget all he's
endured.
Earlier this month, Todd and the kids accompanied Karen to a
work-related conference in northern Michigan. It was the first
week in six months that we didn't visit a doctor's office, an
emergency room or a therapy session. It felt great as we rode
rented bikes - the breeze and lake spray blowing in Joshua's
face as he held his head up in a baby seat - and braved the
crowds on Mackinac Island. Life is good, we were reminded.
Baby steps
Like all parents of newborns, we measure Josh's progress in,
well, baby steps. But for him, even the tiniest improvement is
cause for celebration.
Will he follow the shiny beads just a fraction farther to his
left? Can he reach for the rattle with his left hand? Will he
hold his head up just a second longer?
Our living room erupted in cheers earlier this month, when
Joshua twice rolled from his belly to his back. Exhausted
after the feat, he devoured his right thumb, a reliable
pacifier.
Joshua delivers new victories every week, thanks to frequent
therapy. We drive to Sparrow Hospital four times a week -
twice each for physical and occupational therapy - and a
school district therapist visits weekly.
Therapy is expensive - more than $4,000 a month - but a
necessary component of Joshua's recovery. Joshua's medical
expenses total more than $170,000. We are grateful for our
health insurance, which has covered the cost.
The therapists play with Joshua. They give us tips about how
to position him, and show us how to exercise his body and
encourage emerging skills.
They share our excitement at the progress Joshua makes.
Although he is delayed, he isn't drastically behind others his
age. He isn't sitting up yet, but he recently started to roll
over with minimal assistance and lays on his tummy while
holding up his head for long stretches. He holds his hands
together in the middle of his body and sometimes uses both to
play with toys.
Before surgery, Joshua was nearly always asleep and rarely
followed objects with his eyes. In hindsight, he looked as
drugged as he was. Family photographs show glassy eyes and a
stress-filled face.
Now, although he's taking the same doses of the same
medicines, Joshua's eyes are bright and open. He is awake. He
is attentive. We're working to improve his ability to follow
objects - toys, people, his hands - with his eyes.
Because the part of the brain that controls his left
peripheral vision was removed, Joshua can't see toward the
left. He has lost nearly half his vision with no hope of
regaining it. Therapy - and time - will teach Joshua to
compensate, to turn his head and look where he's going.
Joshua's sense of touch on his left side is probably different
now, too. Touching his left side stimulates a pins-and-needles
sensation like when your foot falls asleep.
The mission is to boost Josh's use and awareness of his left
side. Other parents of children who have undergone a
hemispherectomy for HME have told us their kids ignore the
world on their weaker side. They say it's easy to forget the
disconnected side of your body if you do not see it and cannot
use it.
Prayers answered
Our Christian faith has never been tested as it has been this
year. Belief is an easy concept when your life seems to be
sailing a charmed course. In the first six years of marriage,
we bore two healthy babies, traded up for more satisfying and
better-paying jobs, and bought a bigger house.
There were plenty of blessings for which to thank God - and we
did. At the same time, we assumed the good times would
continue to roll, including the birth of our third child.
Surely, we thought, he'd arrive just like the first two.
We weren't angry with God because of Joshua's circumstances.
In fact, we decided pretty quickly to praise him for the
beautiful gift - one we refused to view as flawed or damaged.
But we were frustrated. We wanted answers. Mainly, we wanted
to know why. And God wasn't giving up the game plan.
We're beginning to get glimpses of Joshua's purpose. Perhaps
it is to show what's possible when a spiritual community makes
a collective show of strength for a single purpose.
God still hasn't revealed the total picture and, make no
mistake, it is difficult.
We've never been forced to rely solely on God for comfort,
encouragement and life itself. It isn't easy or natural.
We've leaned heavily on prayers and our church community to
help guide us. We prayed for health. For an end to the
seizures. For guidance to choose the right option for our son.
For the medical team's wisdom and skill to improve Joshua's
quality of life.
We were not the only ones to pray. Hundreds of people, perhaps
thousands, including many we don't know, prayed for Joshua
after hearing about our need. The collective power of our
pleas has been a major factor in Joshua's progress.
No, we weren't given a perfectly healthy baby boy. Joshua's
seizures didn't miraculously disappear, nor did his brain
suddenly develop the way it should have in the beginning.
But we were blessed with the strength and encouragement to
succeed as parents of three special children. God granted us
the patience to tell and retell Joshua's story to
pediatricians, neurologists, geneticists and anyone else who
wanted to know about him. We learned to navigate the medical
community to search out the best services for our son.
We found our way to doctors who are sought out by parents from
Hong Kong and Italy and other far-flung locations. It's no
coincidence they live and work just 90 minutes away.
Did we ever doubt Joshua would survive the seizures or
surgery? Yes. Were we scared? Yes. Did we cry a lot? You bet.
But, in the end, we've gotten more from this experience than
we gave. We've learned more, laughed (and cried) more and
loved more in the past six months than any other time in our
lives.
The journey with Joshua during the past six months has been
long. It's taken us to unexpected places. And we have no way
to know where it will lead us next.
Our son will walk an interesting road in the coming years. The
surgery might have taken away half his brain, but it's given
him what he did not have: A chance.
Although we do not have divine answers for every question
we've posed, we have enough to sustain us as the journey
continues.
We consider our prayers answered, many times over.
Contact Todd Schulz at 377-1051 or
tschulz@lsj.com.
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