Aileen Crowley

The sound echoes louder each day in the sprawling Princeton, N.J., home of John and Aileen Crowley.
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It's just so unbelievable, all for the love of his children," Aileen Crowley said.

But timing is everything.

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John and Aileen Crowley brought their eldest son, John Jr., now 8, who doesn't have the disease.
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Later that day, when Crowley accepted his diploma, he held Megan, only 6 months at the time, in his arms.The dean gave her a teddy bear, and Megan almost flopped over on the stage.

It proved to be a sign.

At 9 months, Megan was intellectually sharp but couldn't pull herself up or crawl, and she had trouble swallowing.The pediatrician ordered tests, and on March 13, 1998, when Patrick was just 7 days old, Megan was diagnosed with Pompe, a disease identified by a Dutch doctor in 1932 and later often called floppy baby syndrome.

While adults can get Pompe, children are hit the hardest.

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Aileen Crowley, 34, will never forget the doctor's words."He said he was sorry but she's going to die.He didn't know of any child who lived past 2, and there's nothing doctors could do."

That night, she cried herself to sleep.Aileen Crowley woke before dawn to hear the tip-tap of her husband on the Internet."I knew exactly what he was doing," she said.He was searching for any information about the killer disease that was stealing their little girl.Five months later, the cruel disease began to steal Patrick, too.

When Megan was 20 months old, she battled pneumonia and was reliant on a ventilator as she lay paralyzed in a hospital bed. Within 24 hours, her heart stopped three times.Doctors couldn't stabilize her and told her parents that she probably wouldn't make it.

"At that point we just wanted nature to take its course.We didn't want her to suffer," Crowley said.

The Crowleys walked into the room and looked at their daughter who could move only her eyes."She looked around, then locked on us," Crowley said.Suddenly, tears streamed down her cheeks.

"That's when I knew she didn't want to quit.I said, `OK, kid.If you want to fight, I'll fight, too.' "

"In my opinion, God puts some special people on Earth to do great things.
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"John and Aileen are our heroes," said Tom Westdyk, a father of four who's known the Crowleys since high school.
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It wasn't that Crowley had the expertise of a drug developer.With a background as a health-care litigation attorney and business executive, Crowley didn't know much more about science than what he retained from his high school biology class.

He talked to scientists and researchers across the country who thought they could cure his children.He chose to believe in Dr. William Canfield, a quiet, unassuming University of Oklahoma scientist.By March 2000, he partnered with Canfield and became CEO of Novazyme.

He borrowed $100,000 against his house at the time.He raised more than $1 million from friends and relatives.Within a year, he raised $26 million more, mostly from venture capitalists.
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Crowley "is not only out to save his own children," Slonim said.
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In January, Crowley quit his executive position, but remains a consultant for the company's Pompe program."It got to the point where it's set up for success.I want to spend more time at home.I missed a lot of them growing up," he said.

Although Patrick was a stronger baby, Megan has fared better against the ravages of Pompe.Patrick can't hold his arms up and his finger movement is so limited that communication is difficult.He can't sit upright by himself.

The disease has weakened Megan's mouth muscles, but she knows hundreds of words in sign language.She works on a computer and goes to kindergarten with a nurse.

"Her nickname at school," Crowley said, "is the mayor."

He wishes, of course, he could give them the enzyme."As a dad, you think of it every day.But if it's not done right, you can kill them.We're so close now.We have to do it right."

The Crowleys rarely videotape Megan and Patrick anymore.
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"Without this, they'd be institutionalized," Crowley said.

On a recent morning, Megan in sign language, told her dad that she wanted to go outside.The Crowleys bundled both kids up and pushed their wheelchairs across ice and snow to a circle at the end of the drive.

Megan sped around the circle, her brown pigtails blowing in the wind, as she pulled a sled, with a doll propped on top, behind the wheelchair.With her eyes, she smiled.Then she glared at her parents, clearly telling them to watch.

After lunch, it was time for a nap.Aileen Crowley carried Patrick to his hospital bed inside his room decorated with Buzz and Woody of "Toy Story" fame.

She attached the feeding tube in his stomach to a machine.She suctioned his mouth, changed his diapers and clothes and stroked his sandy blond hair.

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"I couldn't get through each day if I thought they may not be here this time next year," Aileen Crowley said."I just try to give them as good a life as I can."

"You can decide life isn't fair, but we try to be as happy as possible," she said."Sometimes you just want to do something normal, like put them in a car and go to Burger King.But we're blessed in other ways."