Eight-year-old Shiloh Pepin is a force of nature. With a husky voice she can barely contain, and a level of enthusiasm that could derail a freight train, she has a way of expressing herself that is half grade-schooler and the rest someone who became too wise, too soon, about the prospect of spending her life under the care of doctors.
Shiloh is one of only three people in the world known to have a condition called sirenomelia, also known as "Mermaid Syndrome." She was born with her legs fused together, and her parents, Leslie and Elmer Pepin, knew it was possible that their child would be affected by the syndrome before Leslie gave birth.
Shiloh describes one part of her life as "dreaming good dreams." But she is also keenly aware of the complications of her medical history. "When I was about 2," she said matter-of-factly, "I had my first kidney transplant."
Doctors stressed that the condition is almost always fatal for a newborn.
"So if she had it," said Leslie, "we were worried we wouldn't be able to love her …[We] went through all these emotions, like you couldn't even believe. But she came out, and she had the biggest, blackest eyes. I remember [Elmer] saying, "She's beautiful."
When she did not die at home, as some had expected, Shiloh's parents brought her to the Barbara Bush Children's Hospital at the Maine Medical Center, where Shiloh saw Dr. Matthew Hand, a kidney specialist.
"Matt was the first physician who looked at Shiloh and saw a little girl who needed help, instead of a medical case that was uncertain," said Elmer.
Hand deferred to the parents in settling on a course of treatment. "The first thing I said to them was, 'What is it you want out of this?' They said three things. The first was that they wanted her to be home as much as she could, to be surrounded by her family. The second was for her to live as long as she could. And the third is that when it came time for her to pass away that she would be surrounded by people who loved her, and that she wouldn't be alone. And I said, 'Okay, we'll work with that, and we'll do the best that we can.'"
The cause is still a mystery, but it is not believed to be genetic. Doctors think that because the blood circulation system doesn't develop normally in the womb, kidneys and other organs don't form as they should.
Shiloh's fused legs were the visible evidence of her condition, but they were not the reason it was so dangerous, and so often fatal.
"She was missing a whole series of organs including her uterus and her bladder, and her large intestine," said Dr. Hand. "She had no vaginal vault or rectum, and no way for urine to get out of her body. Most of these babies die because of poor renal tissue makeup in their body."
But Shiloh did have a small section of a kidney in her back that ultimately had allowed her to survive until doctors could arrange a transplant when she was two years old. She had a second transplant in August of last year, the day after her eighth birthday.
In all, she has undergone more than 150 surgeries, many to reconstruct or compensate for the missing internal organs.