Tumor Devours Utah Man's Face, But Not Spirit

Oct. 11, 2010— -- When Maurice Simpson was only 6 weeks old, his mother noticed his eye was slightly puffy. By the time he was 5, fleshly lumps grew so large that they obscured his eye.

"Right away I got him to the doctor and they didn't know what it was," said his mother, Brenda Simpson of Flint, Mich. "He eyelid was totally closed and it started going into his jaw."

Over the years, the tumors -- elongated tubular lesions that hang from his eye socket and nose -- grew so large that they engulfed the right side of her son's face.

Maurice was diagnosed with neurofibromatosis, a genetic condition that has devoured his face, but hasn't stopped him from living life to the fullest.

"I am probably the best looking man in the world," said Maurice Simpson, now 34, and the subject of a documentary, "A Brand New Face," by Darlow Smithson Production Company, which aired Sunday night on TLC. "My parents taught me to be happy with who I am."

Simpson agreed to participate in the documentary to help others.

He let British filmmakers follow him through his daily life, at work as a manager at the fast food chain Arby's and at his home with his wife and five children in Ogden, Utah.

The crew also followed Simpson through life-threatening surgery to remove his eye and reconstruct his skull after the tumor -- untouched for 17 years -- had put pressure on his brain.

All Simpson wanted to do was to get the growth removed so he could fit a helmet on his head to ride his motorcycle.

An anonymous donor, a businessman whom Simpson had met while working a part-time job delivering pizza, offered to pay the $3,000 deductible to make surgery possible.

After a 13-hour operation in January and a 5-hour procedure in June, Simpson is exuberantly pursuing his life, coaching both his son's football team and his daughter's basketball team with a sense of humor that is contagious.

"Why can't you get that shot? You have two eyes," he tells his basketball players.

"Maurice walks up to people and is friendly and outgoing, once you get past the face," said his mother, who is 55 and still lives in Michigan. "You spend two minutes with him and you are laughing."

An estimated 100,000 Americans have neurofibromatosis, a disorder that occurs in both sexes and in all races and ethnic groups, according to the Children's Tumor Foundation (CTF), which supports much of the research.

There are three distinct forms of the disorder -- each causes tumors to grow on nerves throughout the body. Most tumors are benign, but some can become cancerous. It is more prevalent than cystic fibrosis, Duchenne muscular dystrophy and Huntington's disease combined.

Simpson has the most common form of NF type 1, also known as von Recklingshaus disease, which affects about 1 in 3,300 children, according to Dr. John G. Golfinos, associate professor of neurosurgery and otolaryngology and chair of the Department of Neurosurgery at New York University's Langone Medical Center.

"The disease shows up on the skin and causes tumor formation in the nerves," he said. "They are part and parcel of the nerve and end up taking them out when they get really big or really painful. They get hundreds and it's impossible to take them all out. Every one you take sacrifices a nerve."

A Century Ago, Those with NF Were Shunned

To diagnose NF, pediatricians look for characteristic skin lesions and big birth marks known as "cafe au lait" spots.

"The sad thing is that 100 years ago, people like [Simpson] showed up in the circus side shows like the Elephant Man," said Golfinos. "But it's extremely helpful to have a documentary like this to help others."

Scientists now know that John Merrick, the 19th Century Englishman who billed himself as the "Elephant Man," did not have NF1, but a similar disorder, Proteus syndrome.

Merrick suffered social ostracism and ridicule.

"Today the world is a more enlightened place and more welcoming of those with disabilities," said Golfinos.

Maurice Simpson, who not only has a facial tumor, but fatty tissue on his legs, arms and back, got plenty of support from a young age.

"His dad encouraged him," said Brenda Simpson. "This is life and the hand he was dealt and he was going to have to get through it and learn people were going to be cruel, but we love you. God made you this way for a reason."

"When he was young, we wouldn't allow him to lock himself up in the house or turn his face away," she said. "We let him know, he can't hide."

The boy's biggest mentor was his older brother by two years, Alan, who was murdered when Simpson was only 9.

"It shook Maurice's world," said his mother. "It was rough, but he continued to work harder because he didn't want Alan to be disappointed in him."

Every other year until he was 15, Simpson had painful surgery on his face -- seven operations in all. "He was so disappointed, he threw the mirror down and said, no more," said Brenda Simpson.

Her son said he wanted to live his life, and he did, first marrying Tara Williams and having two children, Shayla, 13, and Christopher Rashawn, 11, then, after a divorce, finding the love of his life, Charity, a supervisor at a food shop.

They married in 2002 and had Maurice Jr., 6, and Jaylen, 3. Andre, 5 months, was born right after Simpson's last successful surgery.

"The first time I saw him I was a little shocked by his face," Charity, 30, told British film makers. "But Maurice was so confident and flirtatious, he really shone. I thought he was very attractive. Maurice is so funny and charming, everybody wants to be near him."

But after ignoring the tumors for 17 years, they eventually grew back. The right side of Simpson's face was as large as a basketball, dislocating his ears and invading his eye socket.

When a local businessman offered his help, Simpson underwent surgery in January at Utah's Huntsman Cancer Center. What surgeons found was life-threatening: Colossal tumors were breaking apart his skull.

In June, he had a second operation to remove a half-pound of sagging skin on his face and reposition his drooping mouth.

Now television reality shows are courting Maurice, who hopes the publicity surrounding his condition will help others with their self-esteem.

"Hats off to him," said John Risner, president of the Children's Tumor Foundation (CTF), whose 21-year-old son has NF1. "We need some awareness. We are only starting to gather attention."

NF is primarily a genetic disorder that can be passed on by either parent. But in about half the cases, there is no family history, but a genetic mutation.

It is the largest genetic disorder that stems from a single gene -- chromosome 17.

The disorder is also highly variable, according to Risner. Unlike Simpson, some show no outward physical signs. Others, like his son, have only small skin lesions. About 55 to 60 percent will have accompanying learning disabilities.

NF Is Not Always Visible, But It Can Kill

"NF can be disfiguring, but there are a lot of invisible cases that can have a high mortality," said Risner.

Though most tumors are benign, an estimated 15 percent will go on to develop malignancies. Secondary problems including blindness and deafness can result when tumors grow on the optic and hearing nerves.

"There are a whole variety of complications," said Risner. "It's not one simple thing. But it comes from tumor activity."

"Doctors describe it as a bag of worms so intertwined, they never get it all," he said. "You have to manage NF throughout your life."

Advocates are hopeful, with about seven clinical trials underway, for new treatments. One day, researchers may find a cure through gene therapy. "I think we will see that in my lifetime," said Risner.

The social costs are just as challenging as the physical ones.

"Teasing and bullying are a component," he said. "But so is the burden the bear because of the uncertainty of the disorder."

But today, with more open attitudes, social networking groups and online chat groups, those with NF bond and have full lives.

Such is the case with Bara Colodne of New York City, who has had more than 30 operations to remove tumors from her airway, heart, chest, and neck since she was diagnosed with NF at 10 days old.

Today, at 30, she has some difficulty with her voice because of a tracheotomy, but her face is only slightly deformed.

"No matter what I have to undergo, I have not let NF stop me from living the life that I have imagined," said Colodne, who graduated cum laude from the University of Hartford with a degree in human services then went on to get a master's degree in social work from New York University and a certificate in psychodynamic psychotherapy.

"I love life," she said. "NF is only one part of me, but I feel that it has given me a reason to see things different and to be so compassionate about life."

Colodne volunteers with CTF to help others. "The biggest challenge is fear of the disease and being accepted. "I try to have them think of living in the moment and loving themselves for who they are."

As for Simpson, he can now easily wear his motorcycle helmet, and feels lucky to have survived life-threatening surgery.

"I feel great," he told film makers. "My face is never going to be like everyone else's but that's what makes me unique and I'm happy for that."

Simpson says he owes his brand new face to the anonymous donor.

"None of this would have been possible without my guardian angel," he said. "I wish he would let me say his name."