Lancaster Physician Fall 2021 - 7

FALL 2021
for during childhood as the risk primarily
doesn't present until young adulthood.
With most of these cancer genetic syndromes,
children carry a 50 percent risk of
inheriting the gene mutation from a parent.
THE QUARTERBACK
Parents come to the clinic, where they meet
with a genetic counselor and Dr. McKeone
to talk about very specific plans for how their
child's cancer risk will be managed.
The clinic and supporting staff guide the
Families across Pennsylvania come to Penn State Cancer Institute for its leading-edge cancer care for children and adults.
For providers, talking with parents about
pediatric cancer is often more about putting
them at ease than the diagnosis, said Dr.
Andrew Freiberg, a pediatric oncologist
at Penn State Cancer Institute and Penn
State Health Medical Group - Lime Spring.
" Unlike many other specialties, we see
new referrals quickly, because patients and
families are always very anxious about the
possibility of cancer in their child, " he said.
" We are very sensitive to that anxiety, as
well as the obvious fact that early detection
and treatment of cancer can improve the
cure rate dramatically. However, most of
the children who are referred to us do not
have cancer, and we are able to provide
reassurance to the family. Even when we do
diagnose cancer, explaining the high cure
rate to families offers more than just hope,
but a real chance at a healthy, long life. "
GENETIC FACTORS MATTER
The most common defense against the
disease for adults - regular screening - has
little application for most childhood cancer.
For the vast majority of children, there are no
recommended screening tests for cancer, and
studies have found that catching the cancer
earlier doesn't affect the course of the disease
for the most common cancer, leukemia.
A small percentage of children who
develop cancer, about 10 percent, have a
genetic predisposition that their parents
may or may not know about.
These predispositions come to light
only when the first case of cancer in the
family arises, signaling the opportunity for
other family members to be tested for the
predisposition, said Dr. Daniel McKeone,
director of the Pediatric Cancer Predisposition
Clinic at Penn State Health Children's
Hospital - one of only 50 such dedicated
centers nationwide.
Familial adenomatous polyposis carries
a risk for several types of cancer including
colon and liver. Because the risk for liver
tumors is highest up to age 7, children with
this predisposition will be screened with
ultrasound and blood tests every six months.
When risk for colon cancer increases at age
10, they start having yearly endoscopies.
The clinic also sees children with Li-Fraumeni
syndrome, caused by a mutation, or
change, in the TP53 gene, which normally
acts as a tumor suppressor gene. These children
are at much higher risk of developing
any kind of cancer, and often early in life.
They need whole-body surveillance with
MRIs on an ongoing basis.
Some genetic predispositions, such as
the BRCA gene mutation that can cause
breast and ovarian cancers, are not screened
LANCASTER 7 PHYSICIAN
family through the complex journey of coping
with a child's predisposition. That includes
coordinating care from multiple specialists,
dealing with the stress and often guilt at
having passed on a genetic predisposition and
connecting parents to resources that can help
them talk about the risks with other family
members and with the children themselves
in a way they can understand.
" I call our clinic the quarterback. We
really have to be that resource that coordinates
the team and makes sure we address
any barriers to care, " McKeone said. " Some
parents have firsthand experience with
these cancers or have seen it in other family
members, and others have not. Either way,
it can be a significant cause of stress. "
Penn State Health provides comprehensive
hematology and oncology care to
infants, children and adolescents at Lime
Spring Outpatient Center in Lancaster and
Penn State Health Children's Hospital in
Hershey. Physicians and researchers at Penn
State College of Medicine work to advance
pediatric cancer treatment with funds raised
by Four Diamonds, including those raised
by Penn State THON.

Lancaster Physician Fall 2021

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