Texas girl gets heart transplant, sister waits
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One of two sisters awaiting heart transplant receives gift of life
Less than one-in-a-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 last March 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 condition doesn't have a known cause. It seems to run in families, but it hasn't yet been proven to be genetic.
A heart transplant is the only option. Without a transplant, 75 percent of people with restrictive cardiomyopathy do not survive more than five years.
A mother's instinct
When Shayde, 9, was diagnosed with a heart murmur last August and Emily, 7, couldn't get over the flu and a fever last February, 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 us, but now it all makes sense," Smith said.
Replacing a broken heart
Both parents anxiously waited and prayed. On Jan. 19, more than nine months later, 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 rounded the corner, just in time for the sendoff.
"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," said Dr. Guleserian, who also is assistant professor of Cardiothoracic Surgery at UT Southwestern.
A new lease on life
After all preparations had been made and the donor team alerted the Operating Room that the heart was en route, Dr. Guleserian began the life-saving operation. Soon after, 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 new heart.
"Emily has pulmonary hypertension which adds risk to a transplant," Dr. Guleserian said.
But the risk didn't present any issues. After five hours, Dr. Guleserian emerged victorious, a new heart beating perfectly in Emily's chest.
When Shayde sees Emily for the first time, she plans on telling her sister "I told you so."
"I'm going to tell her I told you it would all be better."
Emily's previously ashen cheeks have changed to a rosy color, and her lips are pink.
Worth the wait
"Waiting is the hardest part — it brings on feelings of helplessness," Smith said. "Transplant day was filled with anger, fear, relief; name an emotion and I'll tell you what order it came in. But now we have a new heart and instructions on how to care for Emily. We feel empowered. But, we have to be ready to wait again."
Smith said they are left with overwhelming feelings of gratitude for the donor's family that gave their youngest daughter life.
And Dr. Guleserian hopes the family soon will experience those feelings again.
Pointing to her necklace, she said, "Shayde, this is for good luck, which means I'll be wearing it for your transplant, too."
View photo gallery of Emily's journey.
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Natalie Van Noy reassures her 7-year-old daughter, Emily Smith, before she is wheeled to the Operating Room for a heart transplant. A couple of hours before, Van Noy received a page that a heart was available for Emily.
