Rare childhood cancer hits 3 area families

<http://www.fdlreporter.com/article/20081214/FON0101/812140570/1985>http://www.fdlreporter.com/article/20081214/FON0101/812140570/1985
 

By Dorothy Bliskey • Special to The Reporter • December 14, 2008

Childhood cancer researchers and Wisconsin 
Department of Health officials consider it 
unusual that three Fond du Lac area children who 
live within 12 miles of each other were diagnosed 
with a rare form of childhood cancer nearly at the same time.

The disease ­ neuroblastoma ­ is a rare and 
deadly form of cancer that strikes about 650 children nationwide each year.

Three area families, each with a child suffering 
with high-risk stage four neuroblastoma, have hopes for a better year ahead.

The youngsters have tolerated a regimen of 
treatment since being diagnosed in April and May of 2008.

For the past seven months, Lieym Bovee, 
1-year-old son of Tom and Angie Bovee; Lydia 
Ford, 4-year-old daughter of John and Bernadette 
Ford; and Lilyana (Lily) Barthuly, 4-year-old 
daughter of Josh and Penny Barthuly, have endured 
extensive surgeries and aggressive chemotherapy 
in preparation for stem cell transplants that will hopefully save their lives.


Stem cells

In the process, their own stem cells are removed, 
stored and returned to replenish their bone 
marrow with good cells once chemo and surgery 
have chased the cancer cells from their bodies.

"On the surface, three cases in Fond du Lac looks 
excessive," said Dr. Leslie Robison, a researcher 
who is chairman of the department of epidemiology 
and cancer control at St Jude Children's Research 
Hospital in Memphis, Tenn. "But to evaluate this 
cluster, you really need to look back at the Fond 
du Lac area and determine the occurrence of neuroblastoma over time."

Robison noted that that neuroblastoma represents 
only 8 percent of all cancers among children and 
strikes 1 in 100,000 children annually.

"It could be a random event, but state statistics 
are required to determine the degree of excess 
these three cases may represent," Robison said. "It's very rare."

Laura Stephenson, who gathers and tracks cancer 
statistics for the Wisconsin Department of Health 
in Madison, reports that 53 children statewide 
were diagnosed with neuroblastoma during the 
five-year period from 2000 to 2004. Two of those 
53 children were from Fond du Lac County.

According to American Cancer Society statistics, 
childhood cancers in Wisconsin during a five-year 
period from 2001 to 2005 claimed the lives of 26 children statewide.

"We don't have statistics that show Fond du Lac 
County specifically because there were three or 
fewer deaths from childhood cancer in that time 
frame," said Jen Thompson, health promotions 
coordinator at the American Cancer Society branch 
in De Pere. "Milwaukee County, for example, had five deaths."

Lydia, Lieym and Lily are all being treated at 
Children's Hospital in Milwaukee. Lily Barthuly 
received the first of two stem cell transplants 
there in late October. Lieym Bovee received his 
stem cell transplant around Thanksgiving.

Lydia Ford will receive her transplant soon. 
"She's about a month behind the others," said Bernadette Ford, her mother.


Research study

The three children are enrolled in a research 
study in which a specific drug is being tested, 
and the effectiveness of having two stem cell 
transplants versus one is being analyzed. 
Children in the study are picked at random as to 
who receives more than one stem cell transplant.

Of the three children, so far Lily is the only 
one slated to receive two transplants.

The parents of Lydia, Lieym and Lily think it is 
odd all three children were struck with such a 
rare cancer at nearly the same time and locale.

"Almost anyone I talk to thinks it's really 
strange to have three cases in the area," said 
Penny Barthuly, Lily's mother. "I do, too. Some 
nurses I talked to think it's strange and wonder 
if there isn't some cause in our area. Doctors, 
however, just think it is coincidental."

Bernadette Ford said, "We were told that there 
are always lots of funds available for healing 
and cures, but that there's not usually as much 
funding for researching the whys."

The three cases of neuroblastoma are also on the 
"radar screen" on a state level.

"Yes, it's unusual," said Dr. Henry Anderson, 
chief medical officer for the Wisconsin 
Department of Health, Division of Occupational 
and Environmental Health. "For a disease that 
rare, even one occurring is unusual. But with so 
few numbers statewide, analysis on a national 
rather than state level would make more of an 
impact. Trying to find commonality in so few can 
be futile. But there could be an interest from 
researchers at the university here. What you can 
do with a rare disease is to find out who is studying it."

He noted that so far, there are no proven 
chemical or occupational causes to neuroblastoma.


The cause

How important is learning the cause?

"It's extremely important," said Robison, from 
St. Jude Children's Research Hospital. "Only by 
understanding the cause do we have potential to 
look toward prevention. However, we need to 
recognize that even by understanding the cause, 
we still might not be able to prevent it."

Closer to home, medical experts agree that 
finding a cause is the only way to prevent a disease.

"As long as we don't know causes, we're not going 
to have prevention," said Dr. Meghen Browning, 
pediatric oncologist at Children's Hospital of 
Wisconsin and assistant professor at the Medical 
College of Wisconsin. "But if we could find and 
reduce contributing factors, which is very 
different from causes, we might reduce pediatric cancers."

Browning says the timing of the diagnosis of the 
Fond du Lac area children seems unusual.

"But chances are high that it is a statistical 
blip," Browning said. "No pediatric cancer 
cluster has ever been identified. It seems, 
unscientifically, that we see things run in 
streaks. We'll have a series of patients with a 
specific tumor, then not see that tumor again at all for a few months."

Most cancer researchers, Browning says, believe 
that a combination of multiple events such as 
toxic exposures, along with susceptible genetics, 
might be a cause of cancer. However, that theory, she says, is unproven.

"Pediatric tumors are, in general, not inherited. 
But we don't know if a slight lack of resistance 
to cancer might be. Some of those events might be 
toxic exposures, but we don't know that, and it 
would almost certainly have to be a combination, rather than any one exposure."


Statistical rarity

Bryon D. Johnson, Ph.D., who is also on staff at 
the Medical College of Wisconsin and works as an 
investigator and researcher at Children's 
Research Institute, indicated that the three Fond 
du Lac area cases of neuroblastoma all at one time are a statistical rarity.

"Based on current knowledge, the three cases 
would be considered as coincidental," Johnson 
said. "It doesn't appear that the environment 
plays a major role. Since the cause of 
neuroblastoma is unknown, there is nothing on the 
horizon for preventing the disease."

Johnson says research in his laboratory is 
centering on novel ideas for treating high-risk 
neuroblastoma, with the focus on immune-based therapy.

"This involves learning how to make a person's 
own immune system see their cancer as foreign and 
destroy it," he said. "In my opinion, it is 
likely that new and effective treatments will 
probably consist of several different strategies 
that work together to be effective."

Help for digging deeper into potential causes and 
cures for neuroblastoma arrives in the form of 
research. The American Cancer Society provides 
funding for it. Currently, the ACS has six 
researchers around the nation studying neuroblastoma at a cost of $4.5 million.

"One of those researchers is housed at the 
University of Wisconsin in Madison," said Jen 
Thompson. "And 20 percent of her research grant is on neuroblastoma.

"The American Cancer Society's Web site 
(<http://www.cancer.org>www.cancer.org) has 
clinical trials that may potentially be open to 
the (Fond du Lac area) children diagnosed."

In a positive light, the parents of Lily, Lieym 
and Lydia are facing the New Year with hope for 
better health for their children. So far, the 
youngsters are marching through the aggressive 
treatments with innocence and resiliency only a 
child can exhibit, as illustrated recently on the 
caringbridge Web site where Lieym's mom, Angie, frequently writes.

Hospitalized and suffering with a sore mouth and 
digestive tract and vomiting blood, Lieym paused for politeness.

"He is still not eating anything," Angie wrote. 
"But, if this says anything about Lieym, he feels 
so crappy but still said, 'Bless you' when his dad Tim sneezed."


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