Heart Found for 1 of 2 Sisters Awaiting Transplant

Jan. 19, 2009— -- DALLAS (AP) - A heart has been found for the younger of two North Texas sisters placed on the transplant list.

A spokeswoman at Children's Medical Center Dallas says that the transplant surgery for 7-year-old Emily Smith began Monday morning and was expected to last at least till mid-afternoon.

Both Emily and her sister 9-year-old Shayde were diagnosed with restrictive cardiomyopathy, which means their hearts don't relax like they should between pumps, so they don't properly fill with blood.

The sisters were both put on the transplant list on April 3, 2008.

Dr. Aliessa Barnes is one of the girls' pediatric cardiologists at Children's Medical Center Dallas, where eventually they will both hopefully undergo transplants. Last June, Barnes said that without a transplant, the girls' chances of surviving the next two years were 50-50. She said that the condition could cause a blood clot or sudden cardiac death.

While it isn't rare for siblings to need transplants, it is uncommon that they would need them at the same time, said Pam Silvestri, a spokeswoman for Southwest Transplant Alliance, one of 59 organ donation agencies across the country that provide organs to transplant hospitals.

"It's usually years in between," she said, noting that genetic illnesses can often mean siblings would both need some kind of transplant.

While the girls' condition seems to run in families, it hasn't yet been proven to be genetic, Barnes said. And, she said, the condition itself is rare, striking less than one person in a million.

Barnes said that they don't know what caused the girls' restrictive cardiomyopathy. Their mother said there is no history of the condition in her family or the family of the girls' father. The girls' 2-year-old half brother has a normal heart.

The first inkling that something might be wrong came in August 2007, said Natalie Van Noy, the girls' mother. It was at this time that a doctor discovered that Shayde had a heart murmur. She was referred to a cardiologist, who Van Noy said mentioned restrictive cardiomyopathy as a possibility late last year and suggested Shayde return in several months to be re-examined.

Then in February 2008, Van Noy took Emily, who had been suffering from the flu and a persistent fever, to a local emergency room. The doctor said Emily's heart was enlarged, and said she needed to be checked out by her pediatrician, who told Van Noy that Emily also needed to see a cardiologist.

But on the ride home from the pediatrician's office in February, Emily's lips and fingers turned blue. Van Noy headed directly to Cook Children's Medical Center in Fort Worth, where Emily was diagnosed with restrictive cardiomyopathy.

Van Noy immediately mentioned that her older daughter had been seeing a cardiologist who had mentioned the same condition. Van Noy said that after looking at Shayde's files, doctors confirmed she, too, had the condition.

So both girls were sent to Children's Medical Center Dallas for evaluation and in April, both were put on the transplant list.

Emily is a higher priority for transplant than Shayde since she's had more symptoms. Because of their sizes, they are eligible for different sized hearts. Emily's heart will be from a toddler to a small girl and Shayde could take a heart from a small girl to a thin teen or small woman.

Shayde said a procedure in which she was put under anesthesia to check the blood pressure in different chambers of her heart made her a bit less scared of the upcoming surgery.

"I'm not as nervous as I was after the catheter," said the third-grader.

Emily, who has wheezing spells and whose lips, toes and fingertips turn blue when she gets too cold, says she's looking forward to being able to be active in sports again. Barnes said that Emily has lost weight as the calories she consumes go to make her heart work.

"I want to play soccer again," said the kindergartner.

After surgery, the girls are expected to be in the hospital for a week or two and then spend three months confined to their home.

"I'd prefer to get one back recuperated before I get the other one started, but we don't have a choice," said Van Noy, a hair stylist currently on medical leave.

She said that she wants to wait a week after the first girl is transplanted before letting her sister see her so she won't be scared by the tubes.

Van Noy said that that week will be the longest the two -- who share a toy-filled bedroom with alternating pink and purple walls -- have been separated. "I think it'll be hard on both of them," she said.

Van Noy said that they've talked about the surgeries, and how the hearts will come from people who died. The girls have also talked to a psychologist and Van Noy said she suspects the sisters also have talked to each other about what they are going through.

Silvestri said that 25,000 to 30,000 people a year get the transplants they need. Another 6,000 people each year die waiting for transplants.

"I think that we're all really hopeful that we can get a heart in the next few months," Barnes said.

There are over two thousand people in North Texas waiting for organs.

If you want to donate your organs, the best thing you can do is make your family aware of your intentions.

Compiled from AP reports.