Extraordinary Measures to Save Your Life

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Extraordinary Measaures
John and Aileen Crowley were typical parents, married with three children, when their lives changed forever. In 1998, their two youngest children, Megan, then 15 months old, and Patrick, then 4 months old, were diagnosed with Pompe disease, a rare and often fatal inherited neuromuscular disorder that disables the heart and muscles.
The disease is linked to a deficiency of the enzyme responsible for the breakdown of glycogen to glucose. At the time, the disease was incurable and fatal.  E.R. physician Dr. Travis Stork explains how Pompe disease affects the body.

Instead of accepting the inevitable death of Megan and Patrick, John and Aileen fought to find a treatment to save their children's lives. John quit his job to take over as CEO of a start-up biotech company focused on developing a treatment for Pompe disease, while Aileen took care of their three children.

"We made the decision together," John says. "It was a time when Megan had gotten really sick, and it was, in part, the natural progression of the disease. The doctors told us that morning that she wasn't going to survive and live through the morning, but he came back into the room and said, 'You have some little fighter. Her eyes [are] so defiant.'

"I remember looking in her eyes, and Aileen looking in her eyes, and we knew she didn't want to quit," John continues. "And that's when we decided we weren't going to quit, either. We never knew if we could succeed, but we at least wanted to try."

Five years after their diagnoses, Megan and Patrick received the enzyme-replacement therapy that saved their lives, which was developed by the biotech company John was involved with. Within months, their hearts, which had swollen to nearly twice the normal size, returned to normal.

John wrote Chasing Miracles: The Crowley Family Journey of Strength, Hope, and Joy, a personal memoir about the family's battle. The family's story inspired the new film Extraordinary Measures (
CBS Films), which is in theatres now starring actors Harrison Ford and Brendan Fraser. Brendan joins The Doctors onstage.

"I don't see material such as this come across my radar," Brendan says. "I'm portraying John Crowley, who is a living, breathing individual. He's a remarkable person. He's one of the most principled people I've ever met in my life. I wanted the opportunity to take on the challenge to encapsulate everything that he is about, in a character, in a film, and at the same time, still service the story and journey he went on for the well-being of his family and his children."

During the making of the movie, Brendan became close with the Crowleys and now considers himself an honorary member of the Crowley family. Megan and Patrick also left a lasting impression on him.

"They're kids; they're funny," Brendan says. "While their bodies are atrophied, while they're confined to wheelchairs, that certainly doesn't affect their mentality whatsoever. Megan is one smart kid and Internet savvy.

"I could not get the release date of this movie out of the studio, anybody who worked for me, for anything," he continues. "I get an e-mail, and there it is, January 22. [Megan wrote], Dear movie-dad Brendan, the movie's coming out. Go to this Web site. She found a spoiler, geek, nerd site, and she was right! She's probably going to be running the studio any day."

Dr. Travis sits down with Brendan's co-star, Harrison Ford, who is also the executive producer of Extraordinary Measures, to discuss his role in the film and why the Crowleys' story means so much to him.

Watch a clip from Extraordinary Measures!

Having a Child to Save a Child
Lisa and Jack's first child, Molly, was born with Fanconi's anemia, a rare and fatal blood disorder that decreases the body's ability to produce healthy blood cells and can lead to leukemia. By age 6, Molly, who was born without thumbs and with deformed arms, a small head and a hole in her heart, was in desperate need of a stem-cell transplant. Without it, she had only a 20 percent chance of survival.

"When Molly was little, we were given a death sentence that Molly wasn't going to live beyond 7," Lisa says. "And any other kids that we would have would most likely have this disease."

A stem-cell transplant of umbilical cord blood from a sibling would increase Molly's chance for survival by more than 60 percent. However, the likelihood that another child Lisa gave birth to would suffer from Fanconi's anemia made the decision difficult. "In my heart, I wasn't going to let her die without a fight," Lisa says. "I was going to do everything in my power to try to give her a chance and to have a family."

To ensure another child was not born with the disorder, Lisa and Jack used preimplantation genetic diagnosis (PGD), a process similar to in vitro fertilization.

"Once the embryos grow for three days, we take one cell from an eight-cell embryo," says founder and medical director of the Colorado Center for Reproductive Medicine Dr. William B. Schoolcraft. "We can remove one cell without harming the embryo. That single cell is used to analyze the DNA of the entire embryo — in this case to check for Fanconi's anemia and also to see if it's an HLA [human leukocyte antigen] match for its sibling."

HLA is a major factor in determining donor compatibility, and Dr. Schoolcraft found one of the couple's embryos to implant that was both an HLA match and free of Fanconi's anemia. Lisa became pregnant and had a son, Adam, whose cord-blood was transplanted into Molly.

Lisa reports that Molly is cured and she and Adam are doing great. "Molly is 15 and she is a 15-year-old. She's sweet and darling one minute, and the next second, she's possessed," Lisa jokes. "Adam is 9 and he is all boy. He plays every sport known to man. They fight like siblings and they love like siblings, and they have a little sister [Delaney] who was also conceived through [PGD]. And we have a family."

A New Face
Brittany, 21, was born with Crouzon's Syndrome, a rare form of craniosynostosis that caused her skull to stop expanding, which put her at risk of having her brain crushed by the pressure. The disorder caused Brittany's face to become distorted.

Crouzon's Syndrome Symptoms

• Bulging eyes/shallow eye cavities
Outward deviation of one eye
Widely spaced eyes
Underdeveloped upper jaw
Protruding upper jaw

Brittany has had three surgeries to relieve pressure in her head and brain, and to correct her skull, forehead and jaw deformities.

Plastic surgeon Dr. Steven Cohen joins The Doctors to detail Brittany's condition and procedures.

"Now I'm going in for my fourth surgery, and that's going to focus on eye alignment," Brittany says. "I told them that when I look in the mirror, I'd really like both of my eyes to look exactly the same."

Watch Brittany's surgery and see how she looks now!


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