Family confronts tough choice to save baby

Melissa Haneline
The Post and Courier

Rebecca and Scott Butcher interact with their newborn for the first time Jan. 28. Annabelle was born a couple of hours earlier with a heart problem that will require three surgeries. The first operation was performed four days after her birth.

They knew there was trouble 14 weeks into the pregnancy. The baby's heart wasn't quite right.

In August, at 16 weeks, a preliminary diagnosis was made: a serious congenital heart defect.

The doctor presented three choices to Rebecca and Scott Butcher, three awful choices.

"Where are we going?" asks Rebecca. She is tense. The reality of the situation is sinking in. She is lying in the hospital room in the Labor and Delivery Unit at Medical University Hospital, surrounded by family.

It's nearly 8:30 a.m. on a Monday in January. Her father, Wayne Cole, tall, soft-spoken, a ready smile occasionally flaring on his face, is a reassuring presence. Her stepmother, Gail Cole, and sister Jennifer stand nearby.

"To the O.R.," answers nurse Adina Garner. It's time. Baby Annabelle is about to cross the threshold. She is about to confront a destiny she did not choose, a risk-filled infancy, an uncertain future.

"Can we pray first?" Rebecca says.

Attention shifts to Wayne Cole, executive pastor at Riverland Hills Baptist Church in Columbia, the family's church. Cole bows his head.

"Father, we thank you for this day ..." And then kisses, crying, joy and fear.

Rebecca is wheeled into the operating room down the hall. She sits on the edge of the table, her belly protruding, her back exposed to Dr. Gary Haynes, the anesthesiologist, who smears antiseptic, applies a little lidocaine to numb the skin, then gently wiggles a needle into the bottom of Rebecca's spine.

Dr. Eugene Chang scrubs in, then takes his position at the side of the table. A tray of instruments is wheeled over. A plastic sheet is stretched over Rebecca's round belly.

At 8:40 a.m., Scott Butcher joins his wife. He is sitting on a stool, leaning close to her left ear. The doctors and nurses are busy at work.

It doesn't take long, maybe 10 minutes. At 8:45, a robust cry. Annabelle is held high for Mom and Dad to see. The baby is plump, cute. She has a fine layer of dark hair on her head. Her vocal cords work magnificently. She looks completely normal.

A nurse brings the newborn to Rebecca for a brief encounter before whisking her away into the adjacent room. Chang is already attending to the necessary repairs.

Later, pediatric cardiologist Andy Atz explains that 20 years ago, babies with hypoplastic left heart syndrome were born looking utterly healthy, only to die mysteriously after a day or two. Doctors often attributed these terrible surprises to sudden infant death syndrome.

No one knew that they were born with half a heart.

The day before her C-section, Rebecca, 28, is in good humor as she settles into a comfortable couch in the Kiawah Island beach house on loan to the family by a church member.

Scott's mom is there, taking care of 3-year-old Wyatt. Though too young to comprehend all the facts and implications, Wyatt is aware of his soon-to-be-born sister. And he knows about the problem. He knows there's something wrong with her heart, something that the doctors are going to fix in the coming days.

Melissa Haneline
The Post and Courier

Hours-old Annabelle Lynn Butcher in the Pediatric Intensive-Care Unit at MUSC.

Sometimes Wyatt would lie next to his mother, resting his head on her tummy, listening to the vague gurgling within. Once he fell asleep like that, separated from his sister by an inch of their mother's tissue.

Wyatt has been a wonderful distraction for the Butchers. Thinking about Annabelle can be distressing. When Rebecca was 16 weeks pregnant, Dr. Osborne Shuler presented the options: terminate, heart transplant or three major surgeries.

The Butchers considered all three. They talked about their predicament. They remembered the suffering of Rebecca's mother, who struggled with cancer for 13 years. They thought about this precious daughter developing, the suffering she would endure. They learned about the complex surgery to repair the heart, called a staged palliation: the Norwood, bidirectional Glenn and Fontan operations. They thought about God's great gifts. They prayed and prayed.

"You don't want your child to go through this," Rebecca says.

The surgical solution was looking good. It promised not just improvement, it promised to be a solution. If the baby survived the surgeries, she probably would lead a normal life.

Scott, a 37-year-old safety director for a construction company, liked the idea of a permanent fix. But there was more to consider. Pregnancy came with risks. The chance of miscarriage increased with each passing day.

So Scott adhered to a simple philosophy: Doubt not what God plans. And he understood his role as the family protector.

This baby would be born.

Rebecca knew they had made the right decision. She launched a blog, posting photos and reflections, Scripture passages and detailed commentary. Family and friends were moved. Waves of love and support washed over the Butchers.

"Annabelle's already made a difference, and she's not even born yet," Rebecca says.

Scott puts it this way: "This is a series of short tunnels." At the end of each one is a light. Tunnel No. 1: birth. Tunnel No. 2: the first surgery. Tunnel No. 3: survival. Then other tunnels and more lights.

Step by step. Day by day.

Once Annabelle is pulled from her mother and lets out a vigorous cry, she is taken to an adjacent room, where doctors and nurses aspirate her throat, squeeze ointment into her eyes, apply heart monitors and insert intravenous lines into her tiny hands and the stump of her umbilical cord.

She weighs a healthy 7 pounds 4 ounces.

In the IV is prostaglandin, a hormone that will keep her heart valve open. Normally, this valve closes in the first few hours after birth, diverting blood from the left ventricle to the lungs, where it receives oxygen. But Annabelle has no left ventricle.

The dose is carefully administered, kept low lest it provoke apnea, a condition in which a sleeping infant stops breathing.

Annabelle will be examined well during these first few days to ensure nothing but her heart ails her, to allow her to get used to this new environment in which air must be breathed, in which lights shine and sounds are acutely heard.

On her fifth day of life outside the womb, Annabelle will reveal her troubled heart to Dr. Scott Bradley. She will undergo the Norwood procedure.

It takes a while, but eventually the baby is secured in an incubator and wheeled into her mother's recovery room.

"There are her little feet that kicked me!" Rebecca says, delighted to see her daughter, to reach a hand into the compartment and touch her. "She looks just like her brother."

"Like a small version," adds Scott.

"She's very alert," Rebecca observes.

Then the nurse approaches the bed.

"I have a present for you," she tells Rebecca.

It's a sheet of paper with the inky imprint of Annabelle's tiny feet.

All newborns with congenital heart problems come to Bradley and the other pediatric cardiologists at the Medical University Children's Hospital. He operates on babies three or four times a week. The hospital treats perhaps 15 cases of hypoplastic left heart syndrome a year. On any given day, there is an infant with the condition in the Pediatric Cardiology Intensive-Care Unit. That's because three surgeries are required, and each hospitalization lasts for weeks.

Bradley says about 1 percent of all newborns have some kind of congenital heart defect, and less than half of them will require medical intervention.

Because the health system funnels such cases to the Medical University, the staff gets lots of practice.

"We do enough volume that everyone stays competent," Bradley says in a typical understatement. Infant heart surgery is complicated and difficult. It requires the use of special eyeglasses that magnify the field of vision 2 1/2 times so doctors can see the walnut-size heart and intricate blood vessels.

The first surgery, called the Norwood procedure, is among the most complicated of all heart operations, Bradley says.

Less than a week after Annabelle is born into the bright world, her tiny body is transported into the operating room.

The chest is opened. Bradley is manipulating the tiny organ through a 2-by-1 1/2-inch space. Blood flow is diverted. The baby's body temperature is deliberately reduced about 10 degrees to stop the heart.

Bradley sets to work on enlarging the aorta, using donor human tissue. A heart with this type of defect is an unfinished work: The aorta is too small to manage normal blood flow, the left ventricle is missing altogether and blood cannot move to the lungs to get oxygenated.

Bradley installs a shunt, a quarter-inch-round prosthetic tube that will take returning blue blood to the lungs.

He is meticulous. The tiniest leak could mean disaster. The heart surgery itself should take about 2 1/2 hours of a six-hour procedure, Bradley says, but this time, he is left unsatisfied. A patch on the aorta is a little too tight, and he is afraid that, as the baby grows, pressure on the heart would increase, posing an avoidable risk.

So he takes extra time to enlarge the patch. "It's important to be a perfectionist," Bradley says.

Outside, the Butchers are receiving hourly pages all morning: Baby's doing fine; so far so good; Annabelle is stable. But at noon, there is no page. At 12:25 p.m. Scott and Rebecca look at each other with concern.

Eventually, a nurse emerges from the operating room and approaches the Butchers.

"My heart just dropped," Scott says.

The nurse explains the reason for the delay.

Rebecca is a little relieved. She understands her daughter is in good hands. She thanks God that Dr. Bradley is a perfectionist. But Scott remains tense.

"I was not at peace until I saw Dr. Bradley come through the door," he says. "I have never been so happy to see a man in my life!"

After surgery, they have to wait 45 minutes to see her. She is stabilizing after the physical trauma, her chest left open to accommodate the inevitable swelling.

"I thought she looked good," Rebecca says.

"I did, too," Scott says.

"I expected her to look a lot worse."

"I thought she looked beautiful."

The end of a tunnel has been reached. A light shines clear and bright.

The Butchers are praying for little things. To be able to hold their baby. To feed her. To bond.

Annabelle has a paralyzed vocal cord and a swollen throat. Her breathing is a bit labored, but she is showing signs of improvement, and the Butchers are not too worried.

Rebecca pumps breast milk. "I've been able to feel like I'm doing something for her," she says.

When the baby poops, her parents are thrilled.

"We know things are working," Rebecca says.

The day after the surgery, doctors close Annabelle's chest. Often it takes a few days for the swelling to subside, but this little girl is doing well.

Wyatt has been great, offering his parents a necessary distraction, reminding them that someone else needs them, too. The first night after surgery, Scott and Wyatt go to the hotel and order pizza. Father and son. Bonding. Loving one another.

The Butchers visit the ICU all the time. Rebecca carries in all the pink cloths, blankets, stuffed animals and hair ribbons she has collected in recent weeks. She dresses up her daughter. She takes 82 photographs. She wants Annabelle to look like a normal little girl, despite the wires and tubes.

A few days later, the baby is experiencing some typical problems: a little reflux, some jaundice. Nothing serious. And her condition improves with treatment and time.

Rebecca and Scott have entered the next tunnel, a passage that leads to the second surgery in about five months.

In a couple of weeks, Annabelle probably will go home. The Butchers have a simple goal: to keep the baby healthy.

"We're very blessed, very fortunate right now," Scott says.

Reach Adam Parker at 937-5902 or aparker@postandcourier.com.