Survival's not the only worry for people dealing with
By Devin Anderson
December 11, 2006 | Utah resident and hemophiliac Scott
Anderson, 15, knows the burden of hemophilia all too
well. Scott has dealt with his hemophilia all his life
and endured all the necessary factor infusions that
go with it. Scott isn't even able to drive yet, but
has been told by his parents that he's already beginning
to get close to his lifetime insurance cap. Scott is
afraid he'll find himself in a position where no insurance
company will want to take him as a liability because
of his condition.
"I have to eventually find a job somewhere that will
cover my insurance or I'll be screwed," he said.
Scott's parents, USU alumni Rod and Terri Anderson,
both felt that Scott needed to know what he would be
facing the rest of his life. The hemophilia gene is
only carried by females and only affects males. Rod
and Terri also have an 8-year-old named Derek who has
severe hemophilia, as well as an 18-year-old daughter
named Niki who is a genetic carrier of the disorder.
Rod and Terri's oldest daughter Kasi is a junior at
USU. Kasi managed to avoid inheriting the hemophilia
carrier gene. Terri said she wasn't aware she was a
carrier of the hemophilia gene until Scott was born.
She said Scott nearly died when he was born because
the doctors didn't know what was wrong with him.
"We were told there was basically no chance of survival,"
Terri said. "He's our little miracle."
For people with bleeding disorders, especially those
with hemophilia, the burden of chronic pain and expensive
long-term treatment is a way of life. According to the
Foundation, current factor concentrates cost an
average of $60,000 to $150,000 per patient annually,
depending on the brand and type of factor. The injections
must be administered regularly to prevent internal joint
bleeding from normal everyday movement. Other bleeding
disorder-related complications, such as the need for
surgery, can also significantly increase treatment costs.
This means that even at $60,000 per year, a person with
hemophilia could reach a lifetime insurance coverage
cap of $1 million before they reach adulthood.
Hemophilia is the result of an inherited genetic mutation
that inhibits the body's ability to create certain blood
clotting factors. A deficiency of clotting factor VII
is known as hemophilia A, and a deficiency of clotting
factor IX is known as hemophilia B. The severity of
the disorder varies, depending on which clotting factor
is deficient and the factor level. Hemophilia A is far
more common and about seven out of 10 people with hemophilia
A have the severe form, meaning that there is less than
1 percent of the normal level of factor XIII. The National
Heart Lung and Blood Institute estimates that there
are about 18,000 hemophiliacs in the United States and
about 400 people are born with the disorder each year.
Rod Anderson, a physician himself, said he was aware
of how debilitating the disorder could be but never
thought that it could become a part of his life. Rod
said his medical knowledge was almost a curse when his
son was fighting for his life because he could see just
how grim his son's condition was by looking at the various
machines Scott was hooked up to.
Rod expressed hope that a cure could be on the horizon
but also expressed frustration that his son's joints
could be forever affected by the chronic bleeding. Research
is being done to develop a method to insert better functioning
factor VIII or factor IX genes into the cells of people
with hemophilia so that their blood will clot more effectively.
Scientists have effectively been able to do this gene
therapy procedure in animals. Rod said he hopes
this type of gene therapy will eventually be able to
safely increase the levels of the missing clotting factor
in humans as well.
"The ability to increase Scott's factor level at all
would be a blessing," Rod said. "All we can do for now
is pray and hope for the best."