By Mike Donahey 

TIMES-REPUBLICAN

Tracy VanHoutan and wife Jennifer of Downers Grove, IL., long for the days when son Noah, six, rode his bike, played catch, camped and went to baseball games. 

“We have memories,” Tracy VanHoutan said, “but they are not enough and it is not fair.”

Noah has Batten disease. It is a rare, genetic disorder that afflicts only about 450 children in the United States. 

It has been traced to the lack of an enzyme in the brain which removes toxic fats and proteins.

The disease is fatal and the strain afflicting Noah usually claims its victims between the ages of 8 and 12. 

The disease has robbed home-bound Noah of 90 percent of his sight. He receives nourishment through a tube. He can not talk. 

There is no cure. 

There are four main types, but the symptoms become evident at different ages and progress at different levels.

Noah, who was developing as a normal child and was even ahead in some areas of development, according to Jennifer VanHoutan, developed slurred speech and tremors. 

The symptoms continued and puzzled the couple.

It was later diagnosed as epilepsy. However, the couple’s  concerns elevated as the conditions persisted and they called on more health care professionalsbefore receiving the correct diagnosis almost three years ago.

Then another setback.

It was discovered sister Laine, four, also has the disease but fraternal twin Emily does not. 

“It was devastating,” Tracy VanHoutan, a Marshalltown native and 1990 MHS graduate said. 

The VanHoutans said Laine is not displaying the Noah’s symptoms.

“We have Laine on a variety of anti-oxidants and other medications, diet and exercise which appear to be helping,” Tracy VanHoutan said. 

The one-two punch of news dreaded by every parent inspired the VanHoutans to fight and fight hard.

They have worked tirelessly to raise awareness and funds to fight the disease.

They set up a website noahshope.com to make others aware of the disease, raise funds and inspire action.

“Every childhood disease deserves a cure,” is their message.

Other work include attending conferences, visiting researchers, testifying before the Food and Drug Administration, appearing at and organizing fundraising events.

Mandy Abernathy, Tracy VanHoutan’s mother has organized garage sales on behalf of the cause. She has been helped by Tracy VanHoutan’s sister, Brandi Staker. 

“We are planning one for October 1 and 2 here at my home,” Abernathy said. “There will be another one in the spring. We believe this is going to be a ten year effort (of fundraising).”

The fundraising, ranging from garage sales to foundation request, takes up much of the VanHoutans time but it is critical. 

Because the disease was one of approximately 7,000 identified as rare and neglected, awareness and resources were low.

But that is changing.

The VanHoutans have focused their energies this past year on applying to foundations, recruiting researchers and promoting studies on the disease.

The studies are taking place in the United States and England. 

Additionally, they have worked to coordinate with other Batten related organizations to present information to the National Institute of Health. 

“The purpose of this conference will be to identify gaps in knowledge about Batten disease and to develop collaborative efforts among the attending scientists to develop a concrete plan to transition work from the lab into the clinic, Tracy VanHoutan said. “We also expect the NIH will issue a funding request as a result of this conference that could lead to significant funding for  disease research at the federal level.”

They have made personal visits to the office of Dr. Beverly Davidson, a University of Iowa researcher and professor of medicine, neurology, molecular physiology and biophysics who manages an important Batten research group.

According to an article in the Chicago Tribune, Davidson said, “The parents’ visits are extremely motivating for us. Some of the most meaningful experiences are when the children and families visit the lab.” 

The VanHoutans are also excited about research conducted by the Sanford Children’s Health Research Center in Sioux Falls, S.D. 

They are especially pleased with the Dr. David Pearce, the facility's director.

According to promotional material from facility, Pearce’s “research focuses on juvenile Batten disease...his efforts have helped establish a basis to beginclinical trials for treatment.”

It is from the lab that the VanHoutans and others hope a cure will break through.

We are open to any of the four therapeutic studies going on now,” Tracy VanHoutan said. “Stem cell research, gene therapy, pharmaceuticals and direct enzyme replacement. We are open to other ideas as too. It’s about our kids and other children.”

The VanHoutans are eager for one of the therapies “to get to the finish line first” as Tracy VanHoutan said. 

He was candid about the controversy surrounding stem cell research.

“I can understand the ethical considerations about stem cell research,” he said. “We learned two years ago that researchers at Massachusetts General Hospitalwere working on a technique whereby they took skin cells and through a process, revert them back to stem cells. That gets rid of the ethical questions regarding stem cell research but it will take the researchers another 10 years to move forward with the process.”

Despite the tremendous commitment the VanHoutans devote to fighting the disease, both work. Tracy VanHoutan is a trader at the Chicago Board Options Exchange and employed by the Ronin Company. 

“They have been great about this situation,” he said of his employer. 

Jennifer VanHoutan works from their home and is employed by General Growth Development of Chicago. She manages a large number of advertising accounts for the company’s many properties.

“The company has been very supportive and helpful to us,” she said.

Motivating the couple to get up every day and fight hard comes down to kids. 

“It’s our kids,” Tracy VanHoutan said. “We are fighting for our kids and others.”

@Reporter Contact:Contact Mike Donahey at 641-753-6611 or mdonahey@timesrepublican.com

 

Column

 

“Congress plods back to work” was the headline recently in an Iowa newspaper.

It referred, despairingly, to members of Congress returning to Washington after a recess.

Economic measures, the disposition of the Bush tax cuts and numerous other issues are to be worked on before it adjourns again so members may return to their districts and campaign for the November election.

Little, if anything will be accomplished during this session, is the opinion of some observers and commentators.

However, the VanHoutan family of Downers Grove, IL. Is hoping two members of Congress will ignore the skeptics and continue their efforts to promote the Rare and Neglected Disease Caucus.

Tracy VanHoutan is a Marshalltown native and 1990 Marshalltown High School graduate. His mother and sister live here.

What Congress does, or does not do with this initiative impacts the family greatly. 

Two of their three children are afflicted with Batten disease, a rare genetic disorder which gradually destroys the brain. No child has ever survived the disease. Death typically comes between the ages of 8 and 12.

There is no cure.

While work is being done to find a cure, Dr. David Pearce, a prominent researcher, put it succinctly in an interview with the Chicago Tribune last year: “there are not enough of us, not enough labs, not enough funding to get clinical trials.”

As described in this month’s cover story on page four, the VanHoutan’s son Noah, six, and daughter, Laine, 4, are battling the disease now. 

Emily, a fraternal twin of Laine, is not afflicted.

The VanHoutans are working to make people aware of the disease and raising funds for research. 

They and others have accomplished much in the past 12 months. 

Joseph Crowley, D-NY and Fred Upton, R-MI, are the two congressmen the VanHoutans are watching.

In July they formed the bipartisan effort to make their colleagues and others aware of the issues surrounding rare and neglected diseases.

It is important work.

According to a press release the “caucus will focus on bringing greater congressional attention to the 6,800 known rare or orphan diseases that currently have no approved therapy and by working to ensure funding for research into these diseases…”

Other statistics are attention-getting.

The diseases afflict nearly 30 million people, or one out of every 10 Americans.

Heart-wrenching is this: children make up 50 percent of those fighting the diseases and they, like Noah and Laine, are waiting for treatments, which as the press release said, may or may never come. 

Unfortunately, Noah, Laine and other children don’t have much time.

While other issues generate bluster, puffery, foot dragging and finger-pointing, credit Crowley and Upton for joining forces and recruiting others to a worthy cause that will provide hope to families like the VanHoutans.

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Contact Mike Donahey at 641-753-6611 or mdonahey@timesrepublican.com

Reporter Mike Donahey is a columnist for the Times-Republican. The views expressed in this column are personal views of the writer and don’t necessarily reflect the views of the T-R.