The Woman Behind the "New" Face

This is where you can truly say, WOW!



Nation's first face transplant patient shows face
By MARILYNN MARCHIONE, AP Medical Write




Five years ago, a shotgun blast left a ghastly hole where the middle of her face had been. Five months ago, she received a new face from a dead woman.

Connie Culp stepped forward Tuesday to show off the results of the nation's first face transplant, and her new look was a far cry from the puckered, noseless sight that made children run away in horror.

Culp's expressions are still a bit wooden, but she can talk, smile, smell and taste her food again. Her speech is at times a little tough to understand. Her face is bloated and squarish, and her skin droops in big folds that doctors plan to pare away as her circulation improves and her nerves grow, animating her new muscles.

But Culp had nothing but praise for those who made her new face possible. "I guess I'm the one you came to see today," the 46-year-old Ohio woman said at a news conference at the Cleveland Clinic, where the groundbreaking operation was performed.

But "I think it's more important that you focus on the donor family that made it so I could have this person's face." Up until Tuesday, Culp's identity and how she came to be disfigured were a secret.

Culp's husband, Thomas, shot her in 2004, then turned the gun on himself. He went to prison for seven years. His wife was left clinging to life. The blast shattered her nose, cheeks, the roof of her mouth and an eye. Hundreds of fragments of shotgun pellet and bone splinters were embedded in her face. She needed a tube into her windpipe to breathe. Only her upper eyelids, forehead, lower lip and chin were left.

A plastic surgeon at the Cleveland Clinic, Dr. Risal Djohan, got a look at her injuries two months later. "He told me he didn't think, he wasn't sure, if he could fix me, but he'd try," Culp recalled. She endured 30 operations to try to fix her face. Doctors took parts of her ribs to make cheekbones and fashioned an upper jaw from one of her leg bones. She had countless skin grafts from her thighs. Still, she was left unable to eat solid food, breathe on her own, or smell.

Then, on Dec. 10, in a 22-hour operation, Dr. Maria Siemionow led a team of doctors who replaced 80 percent of Culp's face with bone, muscles, nerves, skin and blood vessels from another woman who had just died.

It was the fourth face transplant in the world, though the others were not as extensive. "Here I am, five years later. He did what he said — I got me my nose," Culp said of Djohan, laughing.

In January, she was able to eat pizza, chicken and hamburgers for the first time in years. She loves to have cookies with a cup of coffee, Siemionow said.

No information has been released about the donor or how she died, but her family members were moved when they saw before-and-after pictures of Culp, Siemionow said.

Culp said she wants to help foster acceptance of those who have suffered burns and other disfiguring injuries.

"When somebody has a disfigurement and don't look as pretty as you do, don't judge them, because you never know what happened to them," she said.

"Don't judge people who don't look the same as you do. Because you never know. One day it might be all taken away." It's a role she has already practiced, said clinic psychiatrist Dr. Kathy Coffman.

Once while shopping, "she heard a little kid say, `You said there were no real monsters mommy, and there's one right there,'" Coffman said. Culp stopped and said, "I'm not a monster. I'm a person who was shot," and pulled out her driver's license to show the child what she used to look like, the psychiatrist said.



Culp, who is from the small town of Unionport, near the Pennsylvania line, told her doctors she just wants to blend back into society. She has a son and a daughter who live near her, and two preschooler grandsons.

Before she was shot, she and her husband ran a painting and contracting business, and she did everything from hanging drywall to a little plumbing, Coffman said. Culp left the hospital Feb. 5 and has returned for periodic follow-up care. She has suffered only one mild rejection episode that was controlled with a single dose of steroid medicines, her doctors said.

She must take immune-suppressing drugs for the rest of her life, but her dosage has been greatly reduced and she needs only a few pills a day.

Also at the Cleveland Clinic is Charla Nash of Stamford, Conn., who was attacked by a friend's chimpanzee in February. She lost her hands, nose, lips and eyelids, and will be blind, doctors said.

Clinic officials said it is premature to discuss the possibility of a face transplant for her. In April, doctors at Harvard-affiliated Brigham and Women's Hospital in Boston performed the nation's second face transplant, on a man disfigured in a freak accident. It was the world's seventh such operation.

The first, in 2005, was performed in France on Isabelle Dinoire, a woman who had been mauled by her dog.

The Heart of the Matter

Teen lives 4 months with no heart, leaves hospital

By RASHA MADKOUR, Associated Press Writer

MIAMI – D'Zhana Simmons says she felt like a "fake person" for 118 days when she had no heart beating in her chest. "But I know that I really was here," the 14-year-old said, "and I did live without a heart."

As she was being released Wednesday from a Miami hospital, the shy teen seemed in awe of what she's endured. Since July, she's had two heart transplants and survived with artificial heart pumps — but no heart — for four months between the transplants.

Last spring D'Zhana and her parents learned she had an enlarged heart that was too weak to sufficiently pump blood. They traveled from their home in Clinton, S.C. to Holtz Children's Hospital in Miami for a heart transplant.

But her new heart didn't work properly and could have ruptured so surgeons removed it two days later.

And they did something unusual, especially for a young patient: They replaced the heart with a pair of artificial pumping devices that kept blood flowing through her body until she could have a second transplant.

Dr. Peter Wearden, a cardiothoracic surgeon at Children's Hospital of Pittsburgh who works with the kind of pumps used in this case, said what the Miami medical team managed to do "is a big deal."

"For (more than) 100 days, there was no heart in this girl's body? That is pretty amazing," Wearden said.

The pumps, ventricular assist devices, are typically used with a heart still in place to help the chambers circulate blood. With D'Zhana's heart removed, doctors at Holtz Children's Hospital crafted substitute heart chambers using a fabric and connected these to the two pumps.

Although artificial hearts have been approved for adults, none has been federally approved for use in children. In general, there are fewer options for pediatric patients. That's because it's rarer for them to have these life-threatening conditions, so companies don't invest as much into technology that could help them, said Dr. Marco Ricci, director of pediatric cardiac surgery at the University of Miami.

He said this case demonstrates that doctors now have one more option.

"In the past, this situation could have been lethal," Ricci said.

And it nearly was. During the almost four months between her two transplants, D'Zhana wasn't able to breathe on her own half the time. She also had kidney and liver failure and gastrointestinal bleeding.

Taking a short stroll — when she felt up for it — required the help of four people, at least one of whom would steer the photocopier-sized machine that was the external part of the pumping devices.

When D'Zhana was stable enough for another operation, doctors did the second transplant on Oct. 29.

"I truly believe it's a miracle," said her mother, Twolla Anderson.

D'Zhana said now she's grateful for small things: She'll see her five siblings soon, and she can spend time outdoors.

"I'm glad I can walk without the machine," she said, her turquoise princess top covering most of the scars on her chest. After thanking the surgeons for helping her, D'Zhana began weeping.

Doctors say she'll be able to do most things that teens do, like attending school and going out with friends. She will be on lifelong medication to keep her body from rejecting the donated heart, and there's a 50-50 chance she'll need another transplant before she turns 30.

For now, though, D'Zhana is looking forward to celebrating another milestone. On Saturday, she turns 15 and plans to spend the day riding in a boat off Miami's coast.

Face Value

Face transplant patient can smile, blink again



By MARIA CHENG, AP Medical Writer
Fri Aug 22, 7:37 AM ET

LONDON - Transplanting faces may seem like science fiction, but doctors say the experimental surgeries could one day become routine. Two of the world's three teams that have done partial face transplants reported Friday that their techniques were surprisingly effective, though complications exist and more work is still needed.

"There is no reason to think these face transplants would not be as common as kidney or liver transplants one day," said Dr. Laurent Lantieri, one of the French doctors who operated on a man severely disfigured by a genetic disease.

In Friday's issue of the British medical journal Lancet, Lantieri and colleagues reported on their patient's status one year after the transplant. Chinese doctors also reported on their patient, two years after his surgery.

Last year, the French team operated on a 29-year-old man with tumors that blurred his features in a face that looked almost monstrous. They transplanted a new lower face from a donor, giving the patient new cheeks, a nose and mouth. Six months later, he could smile and blink.

The Chinese patient had part of his face ripped off by a bear. Surgeons in Xian gave him a new nose, upper lip and cheek from a donor. After a few months, he could eat, drink and talk normally, and returned home to Yunnan province in southwest China.

The patients were not identified although photos were included in the reports.

As is the case with all transplants, doctors use immune-suppressing drugs to prevent the recipient's body from attacking the donated tissue. In both face transplants, the patients started rejecting the transplanted tissue more than once. Their doctors solved the problem by juggling their medications.

The French patient now takes three pills a day to prevent rejection.

"That's less than most people with diabetes," said Lantieri, a plastic surgeon at the Henri Mondor-Albert Chenevier Hospital in suburban Paris.

Other doctors were reassured by the results.

"To be able to wean down the dosage of the medication in small amounts and relatively quickly, that is encouraging," said Dr. Bohdan Pomahac, a plastic surgeon at the Brigham and Women's Hospital in Boston.

Pomahac has permission to do a face transplant in the U.S., as do doctors at the Cleveland Clinic.

Experts have worried that if patients take lifelong anti-rejection drugs after a transplant, their cancer risk will jump. Some also predicted that rejection would destroy the face within a few years. Those fears seem to have been allayed, Pomahac said.

With three successful partial face transplants so far — including the world's first on a woman whose face was bitten off by a dog in France — doctors say that some of the surgery's initial uncertainties, like how functional the new face would be, are being answered.

For example, Lantieri's patient's face was paralyzed by tumors for more than a decade. The French team wasn't sure if nerves could grow after the transplant. But they discovered later their patient could blink, proving the brain was able to restore long-forgotten facial nerve connections.

Not everyone is convinced that face transplants are so revolutionary.

Dr. Patrick Warnke, a plastic surgeon at the University of Kiel in Germany, calls them a "dead-end road," because he doesn't think the rejection problem can be solved. Instead, he hopes to re-grow tissue from patients' own stem cells.

Still, the biggest obstacle to more face transplants may not be scientific, but social.

"When kidney transplants first began, people were reluctant to donate because there were a lot of cultural, social and religious issues," Pomahac said. "This is exactly the same scenario now."

Doctors plan to do more face transplants, but are having a hard time finding donors.

"Everyone says they would accept a face transplant if they were disfigured," Lantieri said. "The real question is, would you be a donor, or would you allow your family member to donate their face? That is the answer we need to change."

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On the Net:

http://www.lancet.com

On Organ Transplants and Medical Ethics

The issue of when to declare a person dead in order to harvest his/ her vital organs so that another person may live is a very delicate one no matter from which angle you look at it. An issue that's hard to ignore and poses a lot of questions and quite a dilemma to all the parties involved.

There’s an on- going debate as to when to declare potential organ donors dead. The old criterion of brain death as the sole determining factor is now being challenged by a lot of people in the medical field. Many of them are now advocating on a new protocol called Cardiocirculatory death as the new basis to declare a person dead.

The New England Journal of Medicine tackle this issue in its August edition and the Associated Press pick up the story. I copied the said article as the link to the NEJM website and posted them here.

So, read on…



Doctors debate when to declare organ donors dead

By STEPHANIE NANO, Associated Press Writer Thu Aug 14, 3:56 PM ET

NEW YORK - A report on three heart transplants involving babies is focusing attention on a touchy issue in the organ donation field: When and how can someone be declared dead?

For decades, organs have typically been removed only after doctors determine that a donor's brain has completely stopped working. In the case of the infants, all three were on life support and showed little brain function, but they didn't meet the criteria for brain death.

With their families' consent, the newborns were taken off ventilators and surgeons in Denver removed their hearts minutes after they stopped beating. The hearts were successfully transplanted, and the babies who got the hearts survived.

"It seemed like there was an unmet need in two situations," said Dr. Mark Boucek, who led the study at Children's Hospital in Denver. "Recipients were dying while awaiting donor organs. And we had children dying whose family wanted to donate, and we weren't able to do it."

The procedure — called donation after cardiac death — is being encouraged by the federal government, organ banks and others as a way to make more organs available and give more families the option to donate.

But the approach raises legal and ethical issues because it involves children and because, according to critics, it violates laws governing when organs may be removed.

As the method has gained acceptance, the number of cardiac-death donations has steadily increased. Last year, there were 793 cardiac-death donors, about 10 percent of all deceased donors, according to United Network for Organ Sharing. Most of those were adults donating kidneys or livers.

"It is a much more common scenario today that it would have been even five years ago," said Joel Newman, a spokesman for the network.

The heart is rarely removed after cardiac death because of worries it could be damaged from lack of oxygen. In brain-death donations, the donor is kept on a ventilator to keep oxygen-rich blood flowing to the organs until they are removed.

The Denver cases are detailed in Thursday's New England Journal of Medicine. The editors, noting the report is likely to be controversial, said they published it to promote discussion of cardiac-death donation, especially for infant heart transplants.

They also included three commentaries and assembled a panel discussion with doctors and ethicists. Many of the remarks related to the widely accepted "dead donor rule" and the waiting time between when the heart stops and when it is removed to make sure that it doesn't start again on its own.

In two of the Denver cases, doctors waited only 75 seconds; the Institute of Medicine has suggested five minutes, and other surgeons use two minutes.

State laws stipulate that donors must be declared dead before donation, based on either total loss of brain function or heart function that is irreversible. Some commentators contended that the Denver cases didn't meet the rule since it was possible to restart the transplanted hearts in the recipients.

"In my opinion, it's an open-and-shut case. They don't have irreversibility, and they don't have death," said Robert Veatch, a professor of medical ethics at Georgetown University.

But others argue the definition of death is flawed, and that more emphasis should be on informed consent and the chances of survival in cases of severe brain damage.

The Denver transplants were done over three years; one in 2004 and two last year. The three donor infants had all suffered brain damage from lack of oxygen when they were born. On average, they were about four days old when life support was ended.

In the first case, doctors waited for three minutes after the heart stopped before death was declared. Then the waiting time was reduced to 75 seconds on the recommendation of the ethics committee to reduce the chances of damage to the heart.

The authors said 75 seconds was chosen because there had been no known cases of hearts restarting after 60 seconds.

The hearts were given to three babies born with heart defects or heart disease. All three survived, and their outcomes were compared to 17 heart transplants done at the hospital during the same time but from pediatric donors declared brain dead.

"We couldn't tell the difference," said Boucek, who's now at Joe DiMaggio Children's Hospital in Hollywood, Fla.

There were nine other potential cardiac-death donors at the hospital during the same period, but there wasn't a suitable recipient in the area for their hearts, the report said.

The parents of one of the infants in the study, David Grooms and Jill Airington-Grooms, faced the devastating news on New Year's Day 2007 that their first child, Addison, had been born with little brain function and wouldn't survive.

After they decided to remove life support, they were asked about organ donation, and quickly agreed.

"The reality was Addison was not going to live," said Jill Airington-Grooms. "As difficult as that was to hear, this opportunity provided us with a ray of hope."

Three days later, Addison was taken off a ventilator and died. Her heart was given to another Denver-area baby, 2-month-old Zachary Apmann, who was born five weeks premature with an underdeveloped heart.

His parents, Rob and Mary Ann Apmann, said they were given several options and decided to wait for a transplant. They agreed they would accept a cardiac-death donation to increase Zachary's chances.

Mary Ann Apmann said she wasn't worried that the first available heart came from a cardiac-death donor.

"At that point, Zachary was so sick. We did have him at home. But we knew it wasn't much longer," she said.

After the transplant on Jan. 4, his condition quickly improved, and his blue lips disappeared.

Now, at 21 months: "He's just a crazy little kid who loves to play and swim and throw rocks," his mother said.

The two families haven't met yet but have been in touch through letters and calls. Coincidentally, David Grooms said he had an older brother who died three days after he was born in the 1970s with the same heart condition as Zachary's. The Grooms now have an 8-month-old daughter, Harper.

"Addison did only live three days in this world, but because of this, she lives on," her mother said.


Here's the link to--
The New England Journal of Medicine