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Doctors debate autopsy report

One doctor called the report "totally preposterous," while another declared in absolutely convincing. What is sickle cell trait and can it kill?

Published February 17, 2006

Most people with sickle cell trait never have health problems because of it.

Often, they don't even know they have it.

But the autopsy report on a teenager who died at a North Florida boot camp sparked unusually fierce debate among prominent doctors Friday as to whether the genetic defect can kill in rare cases.

One doctor called the report "totally preposterous." Another declared it absolutely convincing.

The report, released Thursday, said 14-year-old Martin Lee Anderson didn't die because he was beaten by guards. Instead, strenuous exercise triggered an unusual blood reaction because of his sickle cell trait, said medical examiner Charles F. Siebert Jr.

Several doctors have written about such deaths, and sickle cell trait was cited as a likely factor in the sudden death of a Florida State University football player five years ago. The American College of Sports Medicine has issued a statement on sickle cell trait deaths, though the college says there is debate over the possibility.

Siebert's report created a storm of controversy. Some of Florida's best-known sickle-cell specialists said it couldn't happen. Unlike sickle cell anemia, which causes crippling pain and other health problems, sickle cell trait is silent, they said.

"In 30 years of taking care of children with sickle cell disease, I never, ever heard of anybody dying from sickle cell trait," said Dr. Jerry Barbosa, medical director of pediatric hematology oncology at All Children's Hospital in St. Petersburg.

Barbosa called the report "totally preposterous, out of context and very unscientific."

Barbosa wasn't the only one. Dr. Judith Ranells, an associate professor of pediatrics at the University of South Florida, called the report "ridiculous." And Dr. Stuart Toledano, pediatrics professor and director of pediatric hematology oncology at the University of Miami, said the report "doesn't quite jibe."

"You can't explain the findings on the autopsy and the death as it relates to sickle cell trait," said Toledano. He and Barbosa reviewed the autopsy report.

But other doctors said there are documented cases of people with sickle cell trait dying suddenly during exercise.

Anderson's autopsy was one of the most clear-cut cases of death due to sickle cell trait he has seen, said Dr. Jon Thogmartin, Pinellas-Pasco medical examiner. Thogmartin said he has handled five or six such cases.

"It's the most remarkable case I've ever seen," said Thogmartin, who has seen some of the autopsy slides. "It's just unbelievable."

Doctors who treat sickle cell anemia are less familiar with such deaths because these people aren't their patients, Thogmartin said. They generally have no symptoms until they drop dead.

People with sickle cell anemia have a genetic abnormality that causes their red blood cells, which carry oxygen throughout the body, to "sickle," stiffening into crescent-shapes and clumping together, leading to pain and various health problems.

The gene is most common among African-Americans, and Hispanic-Americans also are at higher risk. About 1 in 600 African-Americans have sickle cell anemia, which means they inherited abnormal genes for the disease from both parents.

About 1 in 12 have the trait, which means they carry one abnormal gene and one normal gene. Generally, they have no symptoms, but can pass the trait along to their children.

In rare cases, said the medical examiners and other doctors, people with the trait can have problems, especially in heat or at high altitudes, because some of their red blood cells, usually about a third, are abnormal. During intense exercise, the muscles use oxygen faster than the lungs can take it in. Oxygen levels in the blood drop.

If they drop low enough, the abnormal cells change shape - they begin to sickle, or stiffen and turn crescent-shaped.

"They may cling together and cause a blockage to blood vessels," said Dr. Amos Kadar, director of the pediatric sickle cell program at the University of Florida.

As tiny blood vessels become blocked, muscles and organs start losing oxygen. As oxygen dips lower, more abnormal cells begin to sickle, leading to more blockages.

"It starts a clotting cascade," said Dr. William O. Roberts, immediate past president of the American College of Sports Medicine and associate professor of family and sports medicine at the University of Minnesota Medical School. "It's kind of a vicious spiral down."

As the entire body becomes starved of oxygen, organs begin to fail.

Death follows.

"It is unusual, but it is not impossible," Kadar said.

Somebody in such a crisis likely would complain of being unable to breathe, and could become panicked, confused or delirious, Thogmartin said. In Anderson's case, he might not have understood what guards were saying to him.

The sports medicine college is cautious in its advice. In its statement, it says that when exercising, people with sickle cell trait should be careful to avoid dehydration, take time to get used to heat and altitude, and condition gradually before starting intense exercise.

But, the group says, all exercisers should do the same thing.

Times staff writer Jamie Thompson contributed to this report.

[Last modified February 17, 2006, 21:03:02]

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