【人性此时如何选择】朱伯特综合征病儿[:((][:((]（图） - 珍珠湾ART

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Two-month-old Kaylee has a fatal form of Joubert Syndrome and is unable to breathe without a machine. l两个月大的患有朱伯特综合征的小Kaylee 在没有设备帮助的情况下不能呼吸

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Jason Wallace, the father of Kaylee Wallace, speaks outside the Hospital for Sick Children in Toronto, Wednesday, April 8, 2009.

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Frank Markel of the Trillium Gift of Life Network appears on Canada AM on Wednesday, April 8, 2009.

Kaylee living 'on her own terms,' father says

Updated Wed. Apr. 8 2009 9:58 PM ET

CTV.ca News Staff

Kaylee Wallace, a two-month-old girl who has defied death even after being taken off life support, will choose her fate on her own terms, her father says.

"Kaylee is a complex little girl . . . she will make a decision on her terms," Jason Wallace said Wednesday evening.

Kaylee's parents, Wallace and Crystal Vitelli, thought they were saying goodbye to their little girl Tuesday as she was taken off life support with the intention of donating her healthy heart to another infant.

Wallace says he wants his daughter to live, but if she cannot, hopes that her heart can be used to save another infant.

"We obviously want our daughter to live, but we want to save a child if she can't live," Wallace told reporters Wednesday. "That's why we're going to meet with these doctors to decide whether there is a scintilla of hope that our daughter can, in fact, live life."

Kaylee is suffering from a severe case of a genetic brain condition called Joubert Syndrome, and is unable to breathe when she sleeps without a machine.

She also has kidney problems and her father says that doctors expect her to suffer renal failure within two years.

Kaylee was taken off life support Tuesday and brought to an operating room as transplant teams remained on standby.

However, she did not fall asleep, which would have eventually led to her death, so doctors called off the surgery.

Doctors at Toronto's Hospital for Sick Children say Kaylee is now breathing on her own, making her no longer a potential heart donor candidate.

However, her status could change at any time, doctors said.

"For her to still be here is surprising these doctors," her father said.

Dr. James Wright, the hospital's chief of surgery, said Wednesday morning that Kaylee is in the intensive care unit and is breathing without the help of a respirator.

"In view of the fact that she was stable overnight and not requiring breathing support, she is not a transplant candidate at this time," Wright told reporters on Wednesday.

However, Wright did not rule out another attempt, saying that "we will evaluate the clinical situation as it proceeds and clinical conditions can always change."

Initially, there were concerns Kaylee's heart could become damaged if she was taken off life support but Wright said that there are no current concerns about her heart's condition.

Patients who have the syndrome have a malformed or missing part of their brain that relays information from the brain to the body, Karen Tompkins, a representative of the Joubert Syndrome Foundation, told The Canadian Press.

"Anything that's (related to motor functions) is compromised: walking, talking, swallowing, grasping and breathing," according to Tompkins, who has a teenage daughter with the syndrome.

Medical, ethical problem

In Kaylee's case, the transplant is referred to as a cardiac death donation, which presents a unique set of problems for doctors.

"When someone is declared brain dead... we can recover the organs as soon as the ventilator is removed," Frank Markel, president of Trillium Gift of Life Network, told CTV's Canada AM on Wednesday.

"In the situation of donation after cardiac death, a decision is made to withdraw life support. At that point, the patient is still alive, so we have to wait and we have to be sure that the patient is dead."

Markel said doctors can only recover organs when they know someone is dead and doctors will never hasten death to recover organs.

"What that means is the speed at which a patient dies when life support is removed is crucial," he said. "In this case, the child survived for a considerable time still alive and that ruled out, for now, the possibility of donation."

Meanwhile, Kaylee's parents have already decided who they would like to receive their baby's heart. Her name is Lillian O'Connor and she is in the neonatal unit in the floor below Kaylee at SickKids.

Lillian is one month old and suffers from a condition called truncus arteriosus, which leaves her blood short of oxygen. Doctors say without a heart transplant, she can survive only a few more weeks.

Kaylee's parents got in touch with Lillian's parents after reading about their daughter in a local newspaper and offered them their daughter's heart.

However, Markel said his organization keeps a priority list of potential recipients and it's not a guarantee that Lillian will receive the heart.

"Of course, there are other families and other people waiting and their need is arguably more dramatic and that would be why they're potentially at the top of the list," he said.

"We have to work from an agreed upon priority setting and we don't, in this situation, allow the donor family to choose the recipient."

According to Wright, Lillian is at the top of the transplant list and a transplant will go forward as soon as a donor heart is available.

Here are some facts about Joubert Syndrome:

It's a genetic disorder.
It either affects the cerebellum -- the back of the brain -- or it affects the brain stem, which controls breathing. If the stem is malformed the child will have difficulty breathing.
Some of the characteristics of Joubert Syndrome include decreased muscle tone, abnormal eye development, physical deformities, cysts in the kidneys, seizures and delayed motor and intellectual development.
There is no cure but depending on the severity of the condition there are treatments, including speech therapy, physical therapy and special schooling.
According to the Joubert Syndrome Foundation, the disease affects about 500 families around the world.

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