New Year, new heart

November 03, 2010

One of two sisters awaiting heart transplant receives gift of life

At home in Newark, Texas, giggling, blonde sisters run in and out of the house with their friends, as if they don't have a care in the world. While playing with their miniature beagle, the girls talk about their favorite activities at school. For Shayde it is writing fairytales; Emily loves art. But rows of medication lined up on Emily's dresser in her pink and purple princess room serve as a reminder of all they have been through and what is yet to come.

Rare diagnosis

Less than one in one million children are diagnosed with restrictive cardiomyopathy. For two in the same family to be struck with it is rare; for sisters to have it is almost unheard of. When Emily and Shayde Smith's parents were told in March 2008 that their daughters share the disease, shock doesn't accurately describe the feelings that followed.

"It felt like getting hit with a baseball bat on one side and a cricket bat on the other," said Matt Smith, the girls' dad. "But, once we got home and held the girls, their bravery became a source of inspiration for us."

With restrictive cardiomyopathy, the heart is stiff between each heartbeat, so it doesn't fill properly with blood. The Smith sisters have been at risk for blood clots or sudden cardiac arrest.

The girls' parents were told that a heart transplant would be the only option. Without a transplant, 75 percent of people with the condition do not survive more than five years.

A mother's instinct

When Shayde, 9, was diagnosed with a heart murmur in August 2007 and Emily, 7, couldn't get over the flu and a fever in February 2008, Natalie Van Noy, the girls' mom, suspected a deeper problem. She took Emily to a local Emergency Department where tests revealed an enlarged heart, and Van Noy put it all together. Soon, the girls' diagnosis was confirmed and, on April 3, both were listed for a heart transplant at Children's Medical Center.

"We nicknamed Emily ‘pokey' because she was always tagging behind, but now it all makes sense," Smith said.

Replacing Emily's broken heart

As time went on, both parents anxiously waited and prayed. The girls continued a normal routine at home, with the exception of soccer and cheerleading for fear of overworking their already strained hearts.

On Jan. 19, more than nine months after being listed, Van Noy's pager signaled a heart was available for Emily. At 5 a.m., she rushed to "Grandy's" house where the girls were having a sleepover.

Three hours later, Emily was prepped and wheeled from the cardiovascular intensive care unit to the Operating Room. Emily clung tightly to Van Noy's hand as Shayde and Grandy rounded the corner, just in time for the sendoff. In true sisterly fashion Shayde ran to Emily's bed and hugged her tightly, letting her know she would feel all better afterward.

"I'm pretty excited for my little sister," Shayde said. "I wanted her to get the transplant first because she's worse than me. Her getting better makes me feel better."

Dr. Kristine Guleserian, surgical director of Pediatric Cardiac Transplantation at Children's, came down the hall with a silver heart dangling from her neck. Van Noy and her daughters also wear matching guardian angel heart necklaces, but Emily's recently broke.

"We're fixing Emily's broken heart with a real one today and we'll work on the necklace afterward," she said. Dr. Guleserian, who also is assistant professor of Cardiothoracic Surgery at UT Southwestern, has performed 38 heart transplants since joining the team at Children's in 2005.

As their daughter was placed in the hands of the heart team, family and friends surrounded Smith and Van Noy.

Emily gets a new lease on life

Soon after Dr. Guleserian began the life-saving operation, Dr. Damon Kennedy, a fellow in Cardiothoracic Surgery at Children's, and Dr. Steven Leonard, a cardiothoracic surgeon at Children's and professor of Cardiothoracic Surgery at UT Southwestern, arrived with the special delivery.

Potential risks precede every surgery, but Emily's presented an added concern. Pressure had built up in the arteries that supply her lung leading to a severe case of pulmonary hypertension. When Dr. Guleserian took Emily's heart out, it measured double the size of a normal heart for a girl her age.

Nevertheless, the transplant continued without a hitch, and after five hours, a new heart beat perfectly in Emily's chest. Nine days later, Emily packed up and headed home.

"I feel like it's one of the girl's birthdays," said their grandfather, Mark Smith. "Like the day they were born." 

In a sense, transplant day was Emily's rebirth. Her new heart brought many changes, among them marked improvements in her physical appearance. Emily's previously ashen cheeks turned rosy, and, instead of blue, her lips are a vibrant pink. But the most important change to this 7-year-old is leaving the sidelines in a couple of months to play soccer with her team, the Tsunamis.

Worth the wait

Smith said they are left with overwhelming feelings of gratitude for the donor's family that gave their youngest daughter life. But while a new life has begun for Emily, the waiting continues for Shayde.

"Waiting is the hardest part — [you feel] helpless," Smith said. "But now we have one new heart and instructions on how to care for Emily.

"We feel empowered — but we have to be ready to wait again."