1 of 2 sisters awaiting transplant gets new heart

By Jamie Stengle, Associated Press Writer

January 19, 2009

DALLAS -- A 7-year-old North Texas girl who was placed on a transplant list along with her sister last spring got a new heart on Monday.

The sisters, both placed on the list on April 3, had been diagnosed with restrictive cardiomyopathy, which means their hearts don't relax like they should between pumps, so they don't properly fill with blood.

Emily's 9-year-old sister, Shayde Smith, is still waiting for a new heart.

Following the five-hour surgery Monday, Emily was "doing wonderfully," said Dr. Kristine Guleserian, the pediatric cardiothoracic surgeon who led the team during the operation at Children's Medical Center Dallas.

"She looks awesome," said the girls' mother, Natalie Van Noy.

One difference she's already noticed is that Emily's hands and feet are now warm. They used to be "icy cold," she said.

Van Noy said Shayde has also gone to see her sister in intensive care and held her hand.

Guleserian, surgical director of the cardiac transplantation program at Children's and the University of Texas Southwestern Medical Center, said that the condition the sisters shared is rare, with less than one in a million children diagnosed.

One to two years after diagnosis, the chance of survival is only 40 to 50 percent, she said. The condition could cause a blood clot or sudden cardiac death.

While it isn't rare for siblings to need transplants, it is rare that they would need them at the same time, said Pam Silvestri, a spokeswoman for Southwest Transplant Alliance, one of the organ donation agencies across the country that provides organs to transplant hospitals.

"Two at the same time is fairly unheard of," she said.

Emily was a higher priority for transplant than Shayde because she had more symptoms, including wheezing spells and her lips, toes and fingertips turning blue when she got too cold.

Guleserian said that as they were preparing for the surgery, she realized that she, Shayde and the girls' mother were all three wearing heart necklaces. When Guleserian noticed that Emily wasn't wearing a heart necklace, they told her that Emily's had broken. Guleserian replied: "Today we're giving her a new one."

Van Noy said that a friend had given her a necklace with a guardian angel in a heart over the summer. Then the girls got the same necklace for Christmas. She said that Emily's chain broke a few days ago.

Van Noy said she's now on a mission to get Emily a new chain for the necklace.

Guleserian said that she thinks that the successful surgery has provided a sense of relief for Shayde on what she'll be undergoing.

Van Noy agreed. "She's still nervous about it, but she said that now that Emily's made it through, she knows she will."

It's not known what caused the girls' conditions. One of the girls' pediatric cardiologists has said that while the girls' condition seems to run in families, it hasn't yet been proven to be genetic. Their mother said there is no history of the condition in her family or the family of the girls' father. The younger half brother has a normal heart.

The girls are from the Boyd area, about 30 miles northwest of Fort Worth.

Children's Medical Center Dallas: http://www.childrens.com

Donate Life America: http://www.donatelife.net

Copyright © 2009 The Houston Chronicle.

Copyright © 2009 The Associate Press

This article posted February 22, 2009.