On January 18th this year,
Shanice Clark 21 a college basketball star of California University of
Pennsylvania was found unresponsive in her dorm room. She was rushed to
Monongahela Valley Hospital but she could not be revived and was pronounced
dead. California borough police said that a preliminary report from medical
personnel indicated the death appeared to be accidental and there were
suggestions she had choked on gum while sleeping.
On Monday this week a Washington coroner Tim
Warco’s report blamed the death of the college star on sickle cell trait.
Sickle cell
trait means having one gene for a condition called sickle cell disease (SCD).
This in itself does not normally cause problems and sickle cell trait is not
considered as a disease. It is extremely rare for it to cause problems or
complications, which mainly occur under conditions of severe physical stress.
There has
been a long term discussion on whether sickle cell trait can cause pain and
other complications or not except under extreme conditions. Their debate has
existed for more than 50 years and some professors who have managed sickle cell
cases for long time have refused to accept that sickle cell trait can cause
complications despite the fact that they have come across many cases of trait
carriers who get complications like haematuria.
I remember a
review written by a professor in sickle cell about a research that had been
done and published in Journal of American Forensics by Charis Kepron M.D, Gino
R. Somers M.B.B.S, Ph.D and Michael S. Pollanen, Ph.D titled Sickle Cell Trait Mimicking Multiple Inflicted
Injuries in a 5-Year-Old Boy (September
2009), in his review he argued that a person with the trait cannot get
complications. In their research they had described a case of sudden unexpected
death in a 5-year-old child with undiagnosed Sickle Cell Trait due to the Acute
Chest Syndrome.
The boy’s
story according to the paper was “5-year-old boy of Ghanaian origin was
reportedly well except for a dry cough that was initially noted on the morning
of the day he died. He had been given a single dose of an over-the-counter
cough medication in the early evening, and on his way to bed began to vomit.
His parents described copious vomiting from the nose and mouth, and EMS was
called. Paramedics arrived to find the child with no vital signs and it
appeared to them at the time of initial assessment that he had aspirated.
Intubation attempts in the field were unsuccessful; therefore, the boy was
bag-ventilated until his arrival at the nearest Emergency Department (ED) 30
min after the onset of vomiting. He was successfully incubated in the ED, and
during the resuscitation efforts it was noted that his abdomen appeared
distended, a sign that appeared to worsen over the course of treatment. The anaesthetist
involved reported thick secretions in the airway and high airway resistance.
The resuscitation attempt was unsuccessful and the boy was declared dead 30 min
after arriving in the ED. There were no signs of injury, and the child appeared
well cared for.”
The issue of
sickle cell trait complications has remained controversial as the complications
experienced themselves. No body seems to explain why the complications exist.
In both cases
of Shanice and the 5 years old boy the preliminary investigations always focus
on the soft points.
With the
rampant increase in the number of people with the trait in the world there is a
need to educate everybody concerned.
There is a need to have comprehensive education and awareness programs
for everyone with a major emphasis on coaches, healthcare professionals,
parents, and student-athletes on sickle cell trait and the prevention of sudden
death. Sickle cell trait should not prevent anyone from participating in
athletic activity; however, recent deaths from exertional sickling events in
the athlete setting have demonstrated the need for more education. Education
and prevention programs are the key to preventing an exertional sickling event.
Sickle cell trait is a generally benign condition in red blood cells
sometimes causing them to carry oxygen differently. Athletes with sickle cell
trait run the risk of experiencing exertional sickling events that may have injurious
outcomes. Complications from sickle cell trait while exercising are mostly
preventable.
There is also a need to have population screening for the sickle cell
trait. A population unaware of the sickle cell trait (SCT) has resulted in a
large population of individuals entering childbearing age with no knowledge of
their risk.
Love life!
Lea.
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