Girl, 3, full of life with half a heart

Oakland Tribune, Feb 14, 2007 by Rebecca Vesely

Upon first meeting Morgan Faith Keane, the first thing she will probably reveal -- in one breath, excitedly, jumping up and down -- is that her fourth birthday is two months away and she will definitely have a pony-themed party.

Then she might run into her family's living room, nearly on tip- toe, pink socks a blur, to show off her My Pretty Pony collection or her massive every-girl's-dream dollhouse.

If you're lucky, she will race to her room, silky black hair flying behind her, and bring back her favorite doll, Casey. She'll lift up Casey's nightgown and show off the doll's long abdominal scars, running vertically and horizontally, stitched with red thread -- scars mirroring Morgan's.

These are her "boo-boos." Improbably long and deep scars that serve as a reminder of the three open-heart surgeries and one abdominal surgery Morgan has endured, all before she was

6 months old. She since has had her tonsils and adenoids removed, three heart catheterizations, one seizure scare and hospitalizations for various infections. One more major surgery will happen soon.

Morgan only has half a heart. She was born with Heterotaxy syndrome, sometimes called Ivemark's syndrome, a constellation of heart defects and related abdominal defects. Morgan is missing her spleen and the left side of her heart. She was born with misplacement of the abdomen and intestines.

Her heart has only one ventricle. Just one heart valve connects the lower and upper chambers instead of two. Without a spleen, she is immune-compromised and must take penicillin daily to control infections.

This condition is exceedingly rare. About eight in 1,000 children are born with congenital heart defects, the most common birth defect. Of those, only three in 100 have Morgan's condition.

Until recently, her chances of survival would have been slim to none.

But advances in pediatric cardiac care in the past decade have offered new hope to families like the Keanes. Hope comes hand-in- hand with constant worry because there is no road map for longevity.

"We don't know what the long-term prognosis is," said Morgan's cardiologist, Dr. Kishor Avasarala. "Survivors are only now coming into their teen or young adult lives."

Morgan's mother, Angela, said she and her husband, Dan, are focused on making Morgan's life the best it can be today, which means allowing her to attend pre-school, despite very real concerns about contracting infections.

"We decided the way we wanted her to live was she would do what she wanted to do," Angela Keane said. "The advances in medicine have been so great that we don't know what is going to happen."

The Keanes were living on the Hawaiian island of Oahu four years ago, where Dan worked for the Coast Guard, when Angela got her routine 20-week pregnancy checkup.

"We went in to get the sex of our baby," Angela said. "We got a shock."

With no surgeon in Hawaii to perform the operations necessary to give the baby a fighting chance, the family obtained a list of top mainland pediatric hospitals.

Local doctors told the Keanes that their daughter likely would survive only a week even with surgery, and if she did survive longer she would have a poor quality of life.

"They told us about (pregnancy) termination over and over," Angela said.

Instead, the Keanes chose to leave Hawaii -- with their 3-year- old son, Braeden, in tow -- and move to Alameda where Rob continues to work in the Coast Guard as a lieutenant and naval engineer. Morgan was born at Alta Bates Summit Medical Center and immediately rushed to Children's Hospital Oakland for surgery.

"We put all our faith in it," Angela said.

Three days after birth, surgeons placed a shunt in her tiny newborn half-heart to improve blood flow. Soon after she had another surgery to rotate her intestines and stomach and required another open-heart surgery.

Morgan went home when she was 43 days old. Her weight was that of a newborn -- just 7pounds -- but better than her birth weight of 4pounds 14ounces.

"She slept all the time. She never woke up," her mother recalled. "She didn't play."

Angela was terrified she would walk into her daughter's room and find her dead in her crib.

She started a routine she continues to this day. She doesn't enter her daughter's room without first hearing Morgan make a sound - - either crying, turning over, sighing or some sort of movement. She listens for the bell on a favorite green worm plush toy.

Morgan improved, slowly. At 5 months, she had another open-heart surgery to organize blood flow in and out of her one ventricle.

Her parents created a photo album of Morgan's time in the hospital. It's an album that Morgan proudly shows visitors.

"They went to see me when I was purple," Morgan explains pointing to one photo of her in a hospital bed, tubes protruding from all over her body.

The next step, coming later this year, is what her physician describes as a "complex plumbing procedure." Called the Fontan, the procedure was developed nearly 40 years ago to reduce pressure on the heart by diverting venous blood directly to the lung arteries. Advances in the past 10 years have made it more successful and lowered the risk of complications, such as irregular heart beats.