Rare condition might have triggered cheerleader's death

By SUSAN R. MILLER

Palm Beach Post Staff Writer

Tuesday, March 25, 2008

Stephanie Kuleba's motto on her MySpace page was "live your life."

But her's was cut tragically short after something went terribly wrong in a doctor's office Friday where she was undergoing breast surgery. The West Boca High School cheerleader died Saturday of complications during surgery to correct asymmetrical breasts and inverted nipples after being rushed to Delray Medical Center Friday morning.

Memorial information: Visitations for Stephanie Kuleba will be held from 2 to 4 p.m. and 6 to 8 p.m. today at Babione Funeral Home, 10060 Calle Comercio, in suburban Boca Raton. A funeral Mass will be held at 10 a.m. Wednesday at St. Jude Catholic Church, 21689 Toledo Road, in suburban Boca Raton.


A rare and silent but deadly condition, called Malignant Hyperthermia is believed to be what killed the popular teen who was looking forward to her senior prom and headed to the University of Florida.

The condition is triggered by anesthesia. Symptoms include rapid heart rate, muscles that become rigid, and a fever of 110 or higher. The condition is reversible if recognized and acted upon - usually within 30 minutes of onset- with Dantrolene, the only know antidote, said Dr. Henry Rosenberg, president of the Malignant Hyperthermia Association, whose organization fielded a call to its hot line about Kuleba's case.

Although he could not talk specifically about Kuleba, Rosenberg said they generally help callers assess the situation, see what's been done, and answer any questions.

"This isn't a common event so when you are dealing with this unusual problem, it's nice to have someone who has expertise," he said.

Malignant Hyperthermia is an inherited disorder and generally those who have it don't know they do until they are exposed to certain anesthesia. Additionally, there is no simple, straightforward test to diagnose the condition, said Rosenberg.


Prior to the discovery of the antidote, about 80 percent of those who suffered from Malignant Hyperthermia died. But today it's more like 5 percent.

"There have been patients who have survived because people were well prepared," said Rosenberg.

The surgery was performed by Dr. Steven Schuster, a board certified plastic surgeon with offices in Fort Lauderdale, Boca Raton and West Palm Beach.

"I am devastated by the loss and I feel for the family," he said Tuesday in a statement.

It's unknown whether Shuster's practice had Dantrolene on hand. The drug, which has a shelf life of about three years, isn't cheap. It costs about $2,200 for 36 vials, the dosage needed for a single treatment, said Rosenberg.

"It's like an insurance policy. You hope you never need it, but when you do, you do," he said. He said about 80 percent of hospitals have it.

Although incidents of Malignant Hyperthermia are extremely rare, it's not an uncommon legal defense in medical malpractice cases, said attorney Stuart Grossman of Boca Raton who has handled more than 100 cases involving anesthesia and surgical complications.

"Assuming that the diagnosis is accurate, people don't simply die of anesthesia, they die because they are not monitored while their vital signs are drifting away," said Grossman. "The basic premise of anesthesia is that the patient is rendered unconscious and when that happens the patient's life is in the hands of others."

Doctors won't know for sure the exact cause of death until the results of an autopsy are in, but the Kulebas' attorney, Roberto Stanziale, told reporters Tuesday that he believed the fatal complication could have been prevented. According to Stanziale, Kuleba's surgery began at 8:05 a.m., and paramedics were not called until 9:45 a.m.

"If in fact the medical examiner does come back and indicates to us that the cause of death was Malignant Hypothermia the questions are now going to be: 'Why wasn't she diagnosed quickly? Why wasn't she administered Dantrolene? And if she was administered Dantrolene, was it done at the appropriate times and in the appropriate dosages?' " Stanziale asked.

The American Society of Plastic Surgeons has a database of 1.4 million plastic surgery procedures performed in accredited outpatient surgery centers and they have never seen a case of Malignant Hyperthermia, said Dr. Michael McGuire, a spokesperson for the society and an associate professor at UCLA.

Rosenberg, of the Malignant Hyperthermia Association, said statistics are hard to come by and range from as low as one in 5,000 to one in 60,000.

"We just don't have good data on this," he said.


Records show Schuster had two medical malpractice claims filed against him. He has never been disciplined by the Florida Board of Medicine. A formal complaint would have to be filed against either him or the anesthesiologist, who is said to have been present during surgery, before the board would initiate an investigation in this case, said Eulinda Jackson, deputy press secretary for the Florida Board of Health.

Experts note that Kuleba's death also serves as a reminder that any surgery, no matter how commonplace, has its risks.

"We have become sanguine about this, it's just a lift, a tuck, an implant. But at the end of the day you are still cutting someone open and doing something that's ordinary only after it's a success," said Kenneth Goodman, director of the University of Miami's Bioethics program.

Despite the risks, the number of cosmetic procedures has skyrocketed in recent years, particularly among those 18 and younger.

More than 333,000 adolescents 18 years or younger underwent plastic surgery and cosmetic procedures in 2005, according to the American Society of Plastic Surgeons. Breast augmentation was one of the most popular.

In 2007, there were 10,505 breast augmentation procedures performed on 18- and 19-year-olds; up from, 9,104 the year before. Another 1,700 teens between 13 and 19 underwent breast lift surgery.

And it's not just teenage girls undergoing breast surgery. Close to 14,000 males between the ages of 13 and 19 underwent gynecomastia, or breast reduction surgery, in 2006.

Asymmetrical breasts is a not uncommon problem for many women, said McGuire. But it ranges from mild to severe.

"For the individual, it can be a real social issue, especially in the teen years," said McGuire, who has performed plastic surgery on teenagers.

"There are procedures that are very appropriate in the teenage range, but it depends on the individual. The vast majority of these people are suffering and want to be able to wear normal clothing, to wear a bathing suit and interact with their peers."

It's a shame when a society would push this kind of stuff on a child. And it's even worse, when a parent give it the stamp of approval. I am sad that this beautiful young girl is dead. But happy that others may learn from her parents horrible mistake.