Routine Sinus Infection Leads to Rare Disease: Mucormycosis

MARICOPA, Ariz., Aug. 26, 2009— -- Sue Carlisle has served nearly 2,000 people since she started her catering business about a year ago. She's passionate about her work, and bookings are up. There's just one issue: She can't taste her food.

"I joke that I'm going to write a book," Carlisle said. "It's going to be called, 'The Caterer That Can't Smell or Taste Her Food But People Love it Anyways.'"

It all began in October of 2006 with what she thought was a routine sinus infection. It wasn't her first one and, in the past, her doctors had prescribed powerful steroids to kill the infections.

According to Dr. Raquel Redtfeldt, Carlisle's ear, nose and throat doctor, her immune system was so weakened by repeated steroid use that an ordinary fungus, commonly found in everybody's sinuses, grew into something far from ordinary.

The 49-year-old from Maricopa, Ariz., was diagnosed with a rare and deadly infection in her face called mucormycosis, which led to the total loss of her palate. Now her taste buds are severely weakened and so is her sense of smell.

"This is a medication-caused horror," said Redtfeldt. "People who have this generally don't survive it."

An Unlikely Patient

Mucormycosis is usually only seen in people with severely weakened immune systems, such as AIDS patients, or those with uncontrolled diabetes.

Every year there about 1.7 incidents of invasive fungal infections per one million people, according to Redtfeldt. Ninety pecent of those are bone marrow transplant patients and 9.8 percent are poorly controlled diabetics.

Carlisle, Redtfeldt explained, was a very unlikely patient -- the probability of her both having the infection and surviving it was .085 in a million.

Redtfeldt said patients have two or three days, at most, before the infection gets into their blood stream and into the brain. But Carlisle's case was different.

"For whatever reason, [the infection] just stayed where it was," Carlisle said.

Carlisle's face soon became swollen and infected. Redtfeldt said she smelled of dead skin. The smile she had for more than 40 years was no longer the same to her young daughters, friends and even to herself.

"Some people didn't know how to react," she said.

"'My cheek kept expanding, and then my face got puffier, where all I could do was just put in a baby spoon," Carlisle said. "I couldn't open my teeth or open my mouth that much; and then one morning I woke up and my cheek had literally exploded." Her swollen cheek had burst, leaving a crater.

Mucormycosis Survivor: 'Half My Face Was Going to Be Gone'

At the time, the most recommended option to beat the infection was to completely remove Carlisle's right cheek all the way up to her eyelid. Hours before she went in for surgery, a doctor showed her a picture of what she might end up looking like.

"Half of my face was going to be gone," Carlisle said. "So two hours before the surgery, I'm going in thinking, OK, I'm coming out without a face."

Carlisle worried that she'd never taste her own food again. She knew that without a large portion of her face, her quality of life would change forever. So she refused to undergo the invasive surgery.

"I did not want to stare at my two young girls looking like that, and I would have no quality of life," Carlisle said.

So her doctors had to develop new techniques to treat her. Instead of removing what would amount to half of her face, Carlisle and her doctors chose to go with numerous, more conservative surgeries to remove small amounts of infected tissue at a time -- even when other physicians advocated for the complete removal of the infected area.

After nearly 30 surgeries in two months, and about 60 visits to a hyperbaric chamber to kill off the rest of the infection, Carlisle slowly began to grow healthy facial tissue. Doctors were confident that the mucormycosis was gone; the risk had paid off and her face was intact.

Lucky to Be Alive

"I had a lot of specialists tell me, whatever happens from here on out, you're lucky to be alive," Carlisle said.

But the infection had consumed most of her jawbone, and her palate. Unable to eat or drink anything solid, Carlisle was in danger of losing her entire upper jaw. Most doctors feared that drilling into her upper cheekbones to anchor a false palate would cause the fungus to return and spread.

Carlisle couldn't open her mouth to talk for more than 20 months because doctors gave her a temporary false palate that she held in place with her tongue. But Brian Williams, a dentist who specializes in reconstruction and prosthetics in Scottsdale, Ariz., was developing something more permanent for her.

A Magnetic Palate

"There was dead bone everywhere," Williams said. "Once that happens, it's not going to come back and become live again. So her jaw was literally floating in space."

Williams said he was apprehensive to create an impression of her mouth for an implant. But the mold worked on the first try, and Williams and his team of medical experts developed an entirely new implant model for Carlisle.

Williams attached magnets to pieces of metal called abutments in Carlisle's upper cheekbone. This allowed the palate implant to stay in place, and gave Carlisle a new level of functioning.

Even with her new magnetic palate, she has a very limited sense of taste.

Carlisle said she'll never be able to chew her food again; she must cut everything into small, soft pieces that she can swallow. It takes about seven minutes for her to eat a peanut butter cup, she said. But she can now move her mouth with ease, and she can tell her kids and her husband that she loves them.

"I didn't think it would hit me as hard as it did when I was able to open my mouth for the first time … and look in a mirror," Carlisle said. "It was pretty incredible."

She said the first thing she did was hug her husband. Everyone in the office knew something special had happened.

"There were tears and Kleenex all around," Williams said.

"Because of the positive person she is, and the fighter she is, she was able to adapt beautifully," he said. "The quality of her life improved tremendously."

Williams plans to tour and speak about the new magnetic palette, which has never been used before Carlisle. He hopes patients who lose their palates due to cancer and other illnesses will benefit from Carlisle's case.

"If we can get the news out that this can be done, people with these conditions have hope," Williams said.

A Fresh Start

It's been nearly three years since Carlisle first visited her doctor complaining of a sinus infection, and you would never know what she went through by looking at her.

"Now she's walking around with what just looks like a scar," Redtfeldt said of her patient. "She has her entire cheek, she can move her face, she can smile. None of that would've happened if we hadn't taken the time to sit with her and say, Look, these are your options."

Healthier Than Ever

Today, Carlisle is healthier than ever. For the first time in years, she has no scheduled doctor appointments. Although she almost lost her life, Carlisle said that fighting this battle taught her and her family that nothing is impossible.

"People repeatedly say, 'I feel sorry for you,' and I tell them, 'Don't feel sorry for me,'" Carlisle said. "I'm here, I get to spend every day with my family and there is nothing that can happen now that can top what we've been through."

Now the Carlisles have some rebuilding to do. Medical bills depleted their savings account because their insurance coverage would only cover around 80 percent of her expenses.

When their previous business went under about a year ago, Carlisle and her husband started Straight to the Plate Catering because she loves to cook. They are trying to book as many catering jobs as she can to help supplement her husband's income.

She called her taste loss "a minor inconvenience" compared with what she could have lost. Her doctors said her energy and determination kept her alive, even when some expected the worst.

"She has a lot to teach us, which is, just keep moving" Redtfeldt said. "Do not lie down and let it take you."

Redtfeldt thinks some patients may be afraid to do what Carlisle did: Ask questions and take their care into their own hands.

"If a patient does exert that control over their care, and they have a doctor who is receptive of that, then I think they are better off," Redtfeldt said. "You really can push your care to the next level."

Carlisle is more than happy that she was the model for the innovative practices like the magnetic palate and the slow, repeated tissue removal that was developed to save her life. She knows they can now be used to help other patients that suffer the loss of their palate.

"It's quite humbling to know that the techniques that were used on me worked and now they'll be able to help people all over the world," she said.

ABCNews.com contributors Toby Phillips and Lindsey Reiser are members of the Arizona State University ABC News On Campus bureau.