|Copyright Times Publishing Co. Sep 2,
She stared, in
shock, at the little blue line.
At 39, with a
daughter learning to drive, she thought it couldn't be. So she
drove to the store and bought another test. Then another. And
another. It took four trips and four blue lines to convince
her she was pregnant.
At first, joy
overshadowed worry. Then late one night as she lay in bed, she
felt a cold hand grab at her heart.
What if the baby's
She told herself
the odds were in her favor. Her genetic counselor didn't seem
concerned. There was a 75 percent chance the baby would be
Months later, her
doctor sat her down. The baby, a girl, wouldn't be fine. She
would probably live a short, sick life. If the baby grew up,
she would never run and play. Her muscles would slowly waste
away until she needed machines to eat and breathe.
She didn't think
she could put a baby through that. Not again. She had already
lived through it once before, 16 years ago with Eric.
But she was
already 4 1/2 months along. She could feel the baby kicking.
She already had a name: Jessica. That name had come to her out
of the blue, and when she looked it up, she saw its Hebrew
meaning as a sign: God sees.
Could she bring
Jessica into the world, knowing she was likely to suffer and
die? Or should she terminate the pregnancy?
Karen Slavik had
two weeks to decide.
Sept. 14, 1987.
mother at 23, Karen was on bed rest watching soaps. Somewhere
between General Hospital and All My Children her water broke.
It happened a
month early, not unusual for twins. Megan emerged first,
squalling and kicking, from a textbook Caesarean section.
Eric, her tiny fraternal twin, followed one minute later.
Megan was strong.
Eric struggled to breathe. Karen held him briefly before a
nurse rushed him off to another hospital with a neonatal
intensive care unit.
"Say goodbye now,"
the nurse said. "You might not see him again."
Low birth weight,
Karen was later told. Underdeveloped lungs. Everyone chalked
it up to complications from a premature birth. Karen didn't
worry too much. No one told her she should.
When Eric came
home two weeks later, everything seemed as normal as can be in
a household with newborn twins. Megan was the loud one. Eric
was quiet. He had chubby cheeks and an old man's smile. He was
so calm and sweet that his aunt nicknamed him "Buster" to
toughen him up.
No one was
concerned until Eric started missing milestones. Megan could
roll over, sit up, crawl. Eric couldn't hold his head up.
Karen took him to
a pediatrician, who held him up like an airplane. Instead of
spreading his wings, "he just flopped."
They were referred
to a pediatric neurologist. Cold and clinical, he delivered
the news in a monotone.
"He has SMA Type
I," the doctor said. "He's going to be dead before he's 2."
atrophy. It was the first time Karen had heard of the disease,
the leading genetic killer of children younger than 2. This
was how she learned that she and her husband were carriers of
the SMA gene.
She learned that
of the three types of SMA, Type I is the most severe. Science
could do little for babies like Eric. Doctors said there was
no cure. Their advice left a mother feeling powerless.
"Take him home and
love him until he dies."
Karen wanted to do
just that, but he ended up in the hospital. For 10 weeks they
kept him alive, eating and breathing through tiny tubes,
shrinking to half of what he had been.
Karen left his
side on May 8 to go home that night for a Mother's Day
barbecue. Her family met her in the driveway.
"We have to go
By the time they
got to the hospital, Eric's crib had been disassembled. His
belongings were stuffed in a garbage bag. He was 8 months old.
"I wasn't there
for him when he needed me," Karen says. "I have to live with
that for the rest of my life."
This time, torn
between two clashing instincts, Karen decided to terminate her
It seemed like the
most compassionate choice. For the baby. For her family. For
her. It would hurt less to mourn Jessica now.
She wasn't afraid
to face sickness and death. It's her job to deal with it every
day as a licensed practical nurse.
But to watch your
child suffer is another thing. She longed for this baby with
all her heart, but it seemed almost selfish to keep her.
Her mother and
sister agreed. They still weren't over Eric.
Eric's godmother, had loved him like a son. Hoping for a
miracle, she took him to see a healing nun in New York. On the
plane ride home, he went pale, stopped breathing and nearly
died in her lap.
Karen's mother had
watched Eric waste away in the hospital. Today, she can't hear
his name without breaking down.
handle that again.
that the law should give women a right to choose. But she
believes that choice should not be taken lightly. Her family
more or less agrees. This was one of those times when it
husband, Eric, was strongly opposed.
"What if the test
was wrong?" he asked.
Karen was told
that the test was 99 percent accurate. But that 1 percent was
enough to open the door to doubt. What if? What if? What if?
refused to perform the procedure, so she called a clinic. Her
mother said she wanted to accompany her, to hold the baby.
"Mom," Karen said
gently, "you might not be able to."
That thought, and
the thought that the test might be wrong, sent the family into
a tailspin. Suddenly what had seemed right now seemed wrong.
approaching the third trimester of pregnancy, during which
Florida law would not allow her to abort unless her health
depended on it. She could feel Jessica moving inside her. She
had already fallen in love with her. She knew what she had to
"I knew in my mind
that to abort the pregnancy would be the right thing to do,"
she says. "It would have been right for anyone but Jessica."
In a way, it would
be like a second chance. Eric was gone before she understood.
This time, she would be ready.
Karen vowed to
make every moment count. She studied up on SMA. She promised
Jessica the best toys, the best medicine, the closest thing to
a normal life.
"I bought her a
walker," Karen says. "I knew she probably wouldn't be able to
use it. But damn it, she was going to have one anyway."
She found an even
better gift: a clinical trial. She didn't know how far
medicine had come in the past 16 years, but she prayed it
would be enough.
She was floored
when Dr. Kathryn Swoboda called her. An associate professor of
pediatrics and neurology at the University of Utah, Swoboda
was about to launch a study of a drug that might slow the
progression of SMA.
"All of a sudden
we had hope," Karen says. "Not only did we have medicine, we
had the leading researchers."
Swoboda is a
petite, bespectacled physician who is as compassionate as she
is passionate. Moved by the desperation in Karen's voice, she
rushed her study through the bureaucratic protocol to start in
time for Jessica. She was the first baby enrolled.
To Karen, sodium
phenylbutyrate was a clunky word for hope. With the drug still
in the earliest stages of clinical trials, its effects are
largely unknown. One study showed that it extended the life
span of mice genetically engineered to have SMA.
It might give
Jessica a chance.
"One minute I was
ready just to take her home and love her," Karen says. "The
next I had hope for a possible cure. I thought my prayers were
Sept. 30, 2003.
Jessica came out
all lungs and legs. Swoboda was struck by the strength of her
cry and her kick. She had flown to Florida from Salt Lake City
the day before Karen's C-section so she could examine the baby
beautiful," Swoboda says. "She looked absolutely 100 percent
Karen and Eric
held her up for the camera, as giddy as kids on Christmas. She
was pink, with a full head of hair and a tiny furrow on her
brow. Jessica didn't look sick. She looked perfect.
"It's a miracle,"
Karen's sister said.
Megan said, "She
doesn't have this disease."
nothing, unwilling to dash their hopes. She was already
The first weeks
were a blur of bottles and baths and baby's firsts. But the
medicine provided an ominous reminder that Jessica was sick.
Karen squirted it down her throat with a syringe and an
apology. Then she learned to mix it in a bottle so it wouldn't
taste so bad. At $2,300 a refill, paid for by the study, it
was liquid gold.
Eric, a strong and silent type, serenaded her during her
baths. He didn't like to talk about her sickness, so he fell
back on his faith. Karen would find an open Bible lying on the
table when he left for work at the auto body shop.
Karen and Eric
took Jessica to Salt Lake City for checkups with Swoboda. One
time, it snowed. Eric made a snowball, put it in a cup and
pressed his daughter's hand into the cold.
At 7 weeks,
Jessica still ate well and cried strong, but she was
weakening. At 7 months, unable to cough and swallow, she was
at constant risk of drowning in fluids that her body could not
It was happening.
Karen swore she
wouldn't put Jessica through what baby Eric had had to endure.
She didn't want Jessica hooked to machines she would never
again live without.
imagine Jessica growing up in a wheelchair, sprouting tubes,
watching other kids run and play. She knew that SMA kids tend
to be bright, and she could think of nothing more awful than
an agile mind trapped in a body that doesn't work.
She told the
doctors that she didn't want heroic measures. She understood
why other parents couldn't do the same. But she thought it
best to let nature run its course.
"The older she
gets, the harder it will be," Karen says. "She'll know things.
She'll realize things. She'll know to be afraid of death."
On that Friday,
Karen was speeding home to get Jessica out of the car seat.
There was a bird in the road, but Karen didn't brake. The dove
flew off too late. As it bounced off the front of the minivan,
Karen knew the time was coming.
A month later, she
was in the hospital, faced with dreadful decisions. Jessica
was on the brink, and the doctors were in her face. What
measures did Karen wish to take? A tracheostomy? A ventilator?
Would she sign a "do not resuscitate" form?
"Everything but a
ventilator," Karen said.
Once they're on
the breathing machine, it's hard to wean them off. And then
the decisions get tougher. Karen knew it might prolong
Jessica's life, but what kind of life would that be? It seemed
selfish at this point.
The doctors tried
Karen just reached
for her baby.
"I need to hold
her," she said. "I need to hold her if she's going to die."
away peacefully in Karen's arms, surrounded by her family. She
had lived eight months and 12 days.
She has been gone
for almost three months, but Jessica's crib still stands in a
corner of the master bedroom, with 26 stuffed animals poised
around a vacant spot, like an audience watching an empty
As her family
dances around a subject too painful to discuss, Karen is
delving inside and reaching out. She wants to share her story.
She wants to educate the public, support other SMA parents,
raise money for research for a cure.
She frequents the
online SMA community that has ushered her through the journey.
She visits the message boards and swaps sympathetic e-mails
with Laurie from Massachusetts, Gladys from Malaysia, Normizah
from Singapore and Laura from Indiana.
These women have
never met, but Karen persuaded them to hold a candlelight
vigil on a recent Saturday. Karen and her family nestled their
candles in the sand at St. Pete Beach, where she and Eric had
dangled Jessica's feet in the waves just days before she died.
She still agonizes
over her decisions about Jessica's care. They're mostly
technicalities, but Karen wonders whether Jessica would still
be here if she had done things differently. Dr. Swoboda, who
cried with Karen when Jessica died, tells her gently and
repeatedly not to beat herself up.
"You have to be
confident that you made the right decision," Swoboda wrote
Karen in an e-mail. "Please know that it had to be the best
decision for Jessica, and don't second-guess yourself."
Perhaps the one
thing Karen does not second-guess is her decision to have
wait your whole life for something special," Karen says. "I
was ready for her. I just loved being her mom."
remember anything about her twin. The only memories she has
are pieced together from photographs.
But her memories
of her tiny sister are vivid and real. She smiles when she
thinks of babysitting, how she would play the Beatles and
dance to make Jessica stop crying.
"Her smile was the
best, and the brightest ever," Megan wrote in an e-mail. She
isn't ready to talk about Jessica aloud, but sometimes writing
helps. "Nothing could ruin your day once she looked at you and
gave you that little grin."
Megan is by nature
shy and sweet, a thinker but not a talker. At 16 she carries
herself with quiet dignity and an inner resolve, because she
feels she needs to live for three.
"I don't live my
life just for me, I live it for Eric and Jessica also," she
writes. "Any time that I think of something I could only dream
of doing, I tell myself to get out there and do it for them,
because they didn't have the chance."
Kim Cross can be
reached at firstname.lastname@example.org.
atrophy is a degenerative neuromuscular disease similar to Lou
SMA is the No. 1
genetic killer of children younger than 2.
SMA occurs in one
in 6,000-10,000 live births.
One in 40-50
people carry the SMA gene.
If two genetic
carriers conceive, the child has a 25 percent chance of having
SMA Type I, also
called Werdnig-Hoffmann disease, is the most severe form and
occurs with the highest incidence.
percent of babies with SMA Type I do not live past age 2
without extensive respiratory support.