A life abridged Series: SPRING HILL; [SOUTH PINELLAS Edition]
KIM CROSSSt. Petersburg TimesSt. Petersburg, Fla.: Sep 2, 2004. pg. 1.E
Copyright Times Publishing Co. Sep 2, 2004

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 like Eric?

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 fine.

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.

A first-time 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."

Spinal muscular 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 back."

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 pregnancy.

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.

Karen's sister, 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.

They couldn't handle that again.

Karen believes 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 seemed humane.

Only Karen's 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?

The hospital 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.

Karen was 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 do.

"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 answered."

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 at birth.

"She looked beautiful," Swoboda says. "She looked absolutely 100 percent normal."

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."

Karen said nothing, unwilling to dash their hopes. She was already bracing herself.

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.

Jessica's father, 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 clear.

It was happening. Again.

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.

She couldn't 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 everything else.

Karen just reached for her baby.

"I need to hold her," she said. "I need to hold her if she's going to die."

Jessica passed 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 stage.

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 Jessica.

"Sometimes you wait your whole life for something special," Karen says. "I was ready for her. I just loved being her mom."

Megan doesn't 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 features@sptimes.com.


Spinal muscular atrophy is a degenerative neuromuscular disease similar to Lou Gehrig's disease.

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.

SMA Type I, also called Werdnig-Hoffmann disease, is the most severe form and occurs with the highest incidence.

Ninety-five percent of babies with SMA Type I do not live past age 2 without extensive respiratory support.


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