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For hemophiliacs who received factor before 1990, there
was a high risk from exposure to tainted blood products.
The majority of O’Leary’s patients—57
percent—are hepatitis C-positive, and another
21 percent are HIV-positive.
Horyza, who is hepatitis C-positive, believes that
limiting his factor infusions to times when he was at
risk of a bleed from increased activity, instead of
preventively every two days as some patients choose,
helped keep him HIV-free. Since 1990, when the AIDS
virus was identified, factor has become far safer.
Cost of care
Most people with hemophilia rely on clinics like O’Leary’s
as their primary care provider. About 150 federally
funded centers are in operation nationwide, with 13
in California. One of O’Leary’s administrative
tasks is to help patients pay for treatment. Clotting
factor products—derived from human plasma or recombinant
genes—are among the most costly treatments in
the world, often exceeding $100,000 a year, according
to the National Hemophilia Foundation. Most patients
and their families rely on a combination of private
health insurance and state subsidies to cover the cost
of treatment.
Patients visit anywhere from once a month to once a
year for annual checkups. For patients who live far
from a designated clinic, outreach programs are available.
O’Leary travels to Fresno every month and is frequently
on the phone coordinating care long-distance.
“I’ve got one guy who’s in the ER
all the time in Fresno. He’s got factor with him,
and the ER staff calls here asking what to do,”
O’Leary said. “A lot of times, my patients
know more about what’s happening to them than
the practitioners.”
Horyza concurred, saying that he’s “very
particular when [practitioners] draw blood. I bring
my own tourniquet and my own butterfly needle. If someone
blows my vein or bruises it, I can’t access that
vein, in some cases, for a year,” he added, pointing
to several veins in his right arm that he no longer
can stick.
Horyza is one of an increasing number of hemophilia
patients who enjoy a full life, in relatively good health.
Hemophilia treatment has come a long way since the days
when such patients were called “sufferers,”
and their life expectancy was less than 30 years, according
to the National Hemophilia Foundation.
“There’s a lot more we can do now,”
said Jim Riddel, MS, CPNP, RN, a pediatric nurse practitioner
in the hematology department at Children’s Hospital
and Research Center at Oakland (Calif.). “We start
earlier, preventing more common disabilities like joint
disease. With the availability of factor products that
are free of HIV and hepatitis viruses, it’s much
safer now to do that.”
Nevertheless, a hemophilia diagnosis—often the
first indicator is prolonged bleeding during and after
circumcision—can be devastating to parents, particularly
if there has been no family history of the disease.
Although hemophilia is considered a hereditary disorder,
one-third of all cases occur when there is no family
history of the disease, resulting instead from a spontaneous
mutation. The majority of hemophiliacs are men. Hemophilia
A—a deficiency in clotting factor VIII—accounts
for about 80 percent of all cases, and is caused by
an inherited sex-linked recessive trait with the defective
gene on the X chromosome. Women are carriers of the
gene, but rarely exhibit symptoms.
Riddel, who has worked with hemophilia patients for
about six years, told of one mother who was shocked
when she learned her son had the disease.
“There was no family history,” he recalled.
“She didn’t even know what it meant. I’m
impressed at how far she has come in the last two to
three years. Now, she works with teachers so they are
relaxed having her son in school. She and her husband
both infuse him at home every other day. It’s
part of their life now.”
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