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By JOSIE HUANG
Blethen Maine Newspapers 12/30/2007

Blethen Maine Newspapers

In a crowded doctor's office, children waiting for their appointments bury their faces in their parents' chests. Some stare at the television set on the wall, mouths open.

Still others are distracted by an animated voice coming from a corner of the room. A black-haired little girl in a purple wheelchair plastered with butterfly stickers is reading a Berenstain Bears book, loudly and fast like an auctioneer with no time for pauses.

"Brother Bear said Sister impatiently are you going to take all day to pick your books ..."

Shiloh Pepin, age 8, doesn't read to herself the way people have taught her, nor, for that matter, does she do exactly what people tell her. Ask her parents and doctor about her single-mindedness, and they'll say it's what has kept her alive all these years.

The Kennebunkport girl is one of three children in the world today known to have survived a condition in which their legs are fused together at birth.

Sirenomelia is known as mermaid syndrome because of how the single appendage, with feet protruding at the bottom, resembles the body of the mythical character. In Shiloh's case, the condition is so severe that she is the only one of the three children who has not had surgery to separate her legs.

She is missing a bladder, large intestine, uterus, colon, rectum and vagina. She was born with just one partial kidney, which functioned for only three months, and one ovary.

When Shiloh was born, Leslie and Elmer Pepin took her home with the intention of giving her the best life they could for the few months she was expected to live.

Eight years later, Shiloh is not only alive, but she is at her healthiest thanks to a kidney transplant in August -- her second -- which doctors hope will give her good health for the 10 to 20 years the kidney should last.

She is attending the third grade, where teachers chuckle at the wry sense of humor she developed from spending so much time in the hospital with adults. Her classmates, meanwhile, jockey to fetch books and supplies at her request.

"She will advocate for what she needs," said her case manager at Consolidated Elementary School, Michele Chadburn. "That has served her well. Her spirit is her greatest gift."

Now her parents, whose only focus had been on keeping her alive and comfortable, are confronting a different set of challenges.

What will puberty be like? What if Shiloh ever wants her legs separated?

"Shiloh Pepin," her mom called out from across the waiting room during the visit earlier this month, "It's time to go in to see the doctor."

"No-o-o-o-o-o," Shiloh said, knitting her brow. "I want to read my book."

With Shiloh clinging to her book until the very last second, Leslie and Elmer take her in for a checkup with Dr. Matthew Hand, director of pediatric nephrology at Barbara Bush Children's Hospital at Maine Medical Center in Portland.

Leslie preps Shiloh for the plan they hatched at home: Shiloh was going to surprise Dr. Hand by putting on a red plastic nose right as he walked in to see her.

"He'll go insane!" she told her mom earlier, the both of them rocking with laughter.

Leslie didn't think she was ever going to have children. It wasn't that she didn't want any, but her doctor had told her that health issues such as her weight and diabetes prevented it.

Single in her mid-30s, Leslie had a life that revolved around her job as a business analyst at the disability insurer Unum-Provident. She spent most of her free time with her large and rowdy family, the Kelletts, who ran the Lobster Pot in Kennebunkport, a favorite of the Bush family before it was sold two years ago.

Then, she found out that a friend of a friend, a quiet graphic artist obsessed with Spawn comic books and heavy metal music, was interested in her. She and Elmer Pepin began dating in 1997.

"He's the kindest man I've ever met," Leslie said. "He never says anything mean to anybody."

After four months together, Leslie was surprised to find out she was pregnant. Because of what her doctor had told her, she and Elmer hadn't used birth control.

But the couple couldn't have been happier with the idea of having a baby, and they got married within a couple months, and waited for the baby girl they had named Molly.

During an ice storm in January 1998, Molly arrived dead at birth. Her umbilical cord had wrapped around her tiny neck multiple times.

A devastated Leslie remembers reading about the havoc wreaked by the ice storm and feeling glumly satisfied that everybody else had a taste of what she was feeling.

But now that she and Elmer knew they could have a child, they were determined to try again.

"When we lost Molly, we didn't understand it," Leslie said. "Why, why, why? Then we got pregnant with Shiloh, and it all made sense. It gave us the will and desire to fight so hard for Shiloh. It was just so important for her to be here."

Before Dr. Hand walks into the room, Shiloh rummages through her mother's purse and puts on the plastic red nose.

"What happened to your nose? Are you going to lead a sleigh?" Hand asked, as Shiloh giggled uncontrollably.

The first time Hand saw Shiloh, she was 1 month old and sitting in a car seat, a strikingly beautiful baby with dark eyes and halo of black hair.

Her parents removed her clothes and showed Hand her single limb, with eight toes on two feet entwined so they looked like hands cupped in prayer.

He had never seen anything like this outside of a book. At the time, only one other girl, Tiffany Yorks, of Florida, was known to be living with sirenomelia, which kills most who have it in utero or within days of birth.

Hand quickly realized, though, that having fused legs was purely a cosmetic issue.

Shiloh's health problems were being driven by the fact that she had been born with just a partial kidney. This affected her ability to filter out waste, which in turn raised her blood pressure and slowed her growth.

"I compare it to a car accident, where you get distracted by the broken leg when you should make sure someone is breathing OK," Hand said.

Shiloh reached the top of the wait list for a kidney transplant at age 2. Hand arranged for Shiloh to go to the Children's Hospital of Pittsburgh since no one in Maine was performing kidney transplants on children at the time.

SHILOH'S KIDNEY was expected to last six months, but it worked for four years. Afterward, Shiloh went back on dialysis, going to Portland three times a week to be hooked up to a machine that cleaned her blood for four hours at a time.

After four years, though, dialysis was starting to take a toll on Shiloh, suppressing her appetite and making her weak. Scooting around in her bedroom, where she had a television set and vanity on the floor, became much more tiring.

And she was running out of veins in her body to hook up to the dialysis machine: Almost all had developed scar tissue from the insertion of catheters since she was a baby. The remaining veins were too small.

"We'd have to find another way to put an access in her," Hand said. "One of the ways was to crack open her chest and sew a catheter directly into her heart."

Good news came in June, when the Pittsburgh hospital said it had a kidney for Shiloh. But after Shiloh was on the operating table, the doctors discovered that the kidney was not a good fit for her.

Hand decided to get Shiloh on the transplant list at Maine Med, which had started to perform transplants on children. A kidney became available Aug. 5, just a day after Shiloh turned 8.

Several weeks after the transplant, Leslie's friends and co-workers held a benefit for Shiloh in Windham to help with medical costs -- which include co-payments on medication that cost up to $300 a month and a chair lift that insurance won't cover but the Pepins hope to install one day.

Fundraisers at Shiloh's school and a saloon in Arundel also followed the surgery.

The Pepins are floored by the generosity of their supporters. At the same time, they hope people do not feel sorry for Shiloh or uncomfortable around her.

Leslie got a sense of how some people might view their daughter after she read comments posted on the Internet in reaction to a story about the birth of a Peruvian girl, Milagros Cerron, in 2004. Milagros is known as the youngest living survivor of sirenomelia.

"I have never been so horrified," Leslie said. "Someone wrote that South America must be backwards, and that's why they have kids like this. And that the woman must have made it with a fish. Just horrible, horrible cruel things."

Leslie and Elmer decided it would be a good thing for people to know Shiloh is just a regular 8-year-old and have allowed reporters to chronicle her story.

"The more people saw her as normal despite her remarkable situation, the more she would be treated that way and the less people would stare and gawk," Leslie said.

The television news magazine "20/20" has come up to Maine to shoot footage of Shiloh, and the Pepins say producers hope to fly Tiffany Yorks, now a teenager, to Maine to meet Shiloh.

The week after Shiloh's visit to Dr. Hand was a bumpy one for the family.

First off, the nurse had been unable to draw blood from Shiloh for testing because there was a blood clot in her port. To avoid having her finger pricked, Shiloh shouted "abracadabra" and had her mother pick her up, and jiggle her around, while singing "shake, shake, shake."

Still, after two hours, nothing.

"I'm sorry, Daddy," she said, gazing sadly up at Elmer.

He looked at her tenderly: It's not your fault.

Shiloh had to go back the next day, where the same problem occurred, giving the nurse no choice but to take blood from Shiloh's finger.

Should Shiloh decide to go through with separating her legs, she would endure multiple surgeries spanning years that would involve straightening out her feet and repositioning her pelvis.

At the end of it all, the legs might not work normally. The vascular system in Shiloh's single limb is more complicated than the systems of the other two girls with sirenomelia.

"Her blood vessels cross over from one side to the other side," Hand said. "If you cut off the blood supply to the limb, the limb can't develop. There's a reasonable chance that part of the limb would have to be amputated."

Elmer and Leslie wonder whether Shiloh will ask for surgery when puberty hits. It is the only way Shiloh can have any female genitalia.

This year, for the first time ever, Shiloh gave some indication that she was at least considering the operations.

She told her mother that she could wear yellow dress shoes like Minnie Mouse if she had two legs.

And there is the question of what happens once Shiloh's second kidney fails.

Ten to 20 years from now, the kidney will be battered by the body's constant attempts to reject the foreign organ and from the medication she takes to keep the body from rejecting it.

She will need to get another transplant, but after having had two, her body will have developed antibodies that will make it harder to find matching donors, Hand said.

But all that comes later. For now, Shiloh has more pressing concerns.

Playing "Over the Hedge" on her PlayStation, Shiloh taps rapidly on her control pad to swing objects at animated rodents on the screen.

In her enthusiasm, she has corralled her mother into playing a game with her.

"OK, move over, sister!" Leslie said, bouncing onto the bed beside her.

Leslie hunched over the control pad and squinted at the screen.

"I don't know what I'm doing," Leslie said.

"You're trying," Shiloh said. "You're doing pretty good."

Staff Writer Josie Huang can be contacted at 791-6364 or at:

jhuang@pressherald.com

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