Blood disorder blamed in death:
An autopsy finds that he carried
two abnormal genes. Linked, they killed him after football
practice.
By KEVIN GRAHAM
Published August 23, 2006
TAMPA - When Bobby Stephens Jr. died after youth football practice,
many wondered whether the July heat caused the tragedy.
But a Hillsborough County medical
examiner's report said this week that the 12-year-old died from an
extremely rare genetic blood disorder that included the presence of
sickle cell trait.
The disorder usually has no symptoms,
but doctors say strenuous exercise can exacerbate it, causing blood
cells to sickle, or clot, and depriving the body of oxygen.
Dr. Leszek Chrostowski, the attending
physician who supervised Bobby's autopsy, said the boy carried genes
for hemoglobin S, which makes up sickle cell trait, a disorder
prevalent in people of African descent, and hemoglobin E, a benign
blood trait typically found in Southeast Asians. Before Bobby, there
were only 27 documented cases in the world of individuals who died
as a result of carrying both abnormal genes, Chrostowski said.
Bobby's mother is from Thailand and
his father is African-American, which explained why he carried both
genetic traits, doctors said.
"It's one answer, but it's given us a
lot more questions," said the boy's father, Bobby Stephens Sr.
"Right now, I'm trying to do some research on it since it is so
rare."
Carrying the hemoglobin E trait alone
is less deadly, Chrostowski said. Individuals with it may have
smaller-than-normal red blood cells and experience mild anemia,
according to medical research.
But Chrostowski said there are cases
of death in people caused by hemoglobin S, part of the genetic
makeup of sickle cell trait.
A recent and controversial death
initially linked to sickle cell trait was that of Martin Lee
Anderson, the 14-year-old who died after being roughed up by guards
at a North Florida boot camp. Bay County Medical Examiner Charles F.
Siebert Jr. ruled Martin died from internal bleeding caused by
sickle cell trait. He had been running laps when he stopped and
guards began to beat him.
After a second autopsy in
Hillsborough County as part of a state investigation into Martin's
death, pathologists concluded that he did not die from sickle cell
trait or any other natural causes, but from suffocation after being
forced to inhale ammonia fumes. The death investigation by State
Attorney Mark Ober is ongoing.
Because of the questions surrounding
sickle cell trait and strenuous exercise, members of the National
Athletic Trainers' Association are holding a summit in early 2007 to
discuss the disease. The death of a Missouri football player last
year was attributed to sickle cell trait. Most people who carry the
trait experience no symptoms or problems. But someone who has
inherited the trait from both parents has sickle cell anemia, a
serious disease.
Pinellas-Pasco Medical Examiner Jon
Thogmartin said screenings for sickle cell trait may be a good idea,
especially for athletes, so they know what to do if they experience
problems.
"If you feel faint, pain in your
muscles, you may want to stop exercising and for sure, drink, drink,
drink," Thogmartin said. "Heat and exercise are bad for anyone with
sickle cell trait."
Dr. Jerry Barbosa, medical director
of pediatric oncology at All Children's Hospital, said one of the
earliest cases of someone dying of complications from having both
hemoglobin S and hemoglobin E was an individual in Turkey in 1958.
"This is an extremely, extremely rare
combination," Barbosa said. "My guess is if you have a combination
of both, that may exacerbate the problem or put you at higher risk."
Bobby's father said the medical
examiner's report has caused him to educate his family about the
disease and get tested for the sickle cell trait.
"I'm going to get a physical done and
see if it's maybe dormant in me," Stephens said. "His mother
probably needs to do the same thing, and maybe we can figure out how
this all came together."
A member of the Progress Village
Panthers varsity team, Bobby complained during the first day of
practice July 17 that he was tired and not feeling well. He died on
his way to a hospital.
Just days later, 11-year-old Jamell
Johnson died while in a hospital, where he had been for nearly a
week after collapsing while practicing with the Nuccio Jaguars youth
football team. The medical examiner concluded that he died of
heatstroke.
Kevin Graham can be reached at (813)
226-3433 or
kgraham@sptimes.com.
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