Noah's Hope

PHOTOGRAPH BY TANGIE SHEETS

Noah’s Hope

Despite losing two of their own children, Downers Grove parents Jennifer and Tracy VanHoutan have saved the lives of countless others

BY KERRIE KENNEDY

Downers Grove residents Jennifer and Tracy VanHoutan have experienced the unimaginable: in the past three years, they have lost two of their four children.

Their son Noah died in March 2016 at the age of 11, and their daughter Laine passed away two years later in March 2018 at the age of 12. Their children succumbed to a rare but devastating disease called CLN2 Batten disease.

PHOTO BY KAREN KARDATZKE

“Jen and I were both unknowingly carriers of this condition, and each of our children had a 1 in 4 chance of being affected,” Tracy VanHoutan said. “The symptoms don’t present until the age of 3 or 4, when [the affected children] start having seizures and language delays. Eventually, they lose their vision, their speech and their ability to walk and eat by mouth. An easy way to explain it is, combine ALS with Alzheimer’s, and put that in a three-and-a-half-year-old.—Not that any life-threatening disease is worse than another, but this is just cruel.”

2019 A FIFTH SEASON: (L-R) JENNIFER VANHOUTAN, TRACY VANHOUTAN, SARA KENNICOTT, DR. JON COOPER, DR. JILL WEIMER, DR. ELIZABETH BERRY-KRAVIS, DAVE KENNICOTT, DR. MICHELLE HASTINGS

Within months of their son Noah being diagnosed, the couple set up a research fund at the DuPage Foundation for this devastating and little-understood disease, and named it Noah’s Hope. Eventually, they merged with a local foundation (Hope 4 Bridget Foundation) that was founded by the Kennicott family— another Chicago-area family whose daughter was diagnosed with Batten disease—and formed a 501(c)(3) in 2013.

“We couldn’t believe that a horrible disease like this did not have any treatments,” VanHoutan said.

There are more than 7,000 rare diseases, and one in ten Americans is affected by one or more of them; although not all of these diseases are deadly. But unfortunately, Batten disease is another story.

“Some people walk around with a rare disease and don’t even know it,” Jennifer VanHoutan said. “No one on either side of our families [has] been afflicted. It’s just a genetics lottery.”

In short order, Jennifer and Tracy became leaders in the rare disease community. They met with members of Congress to increase research funds and understanding of rare diseases, in the hopes of finding a treatment for the disease that would eventually take the lives of two of their children.

“Early on, we figured there were four different therapeutic strategies: small molecules, stem-cell transplants, gene therapy and enzyme-replacement therapy,” Tracy VanHoutan said. “The last one—enzyme replacement therapy— seemed to be the most promising. Batten disease is caused by a genetic mutation that disrupts the cells' ability to dispose of wastes. Cells are thrown out of balance with the build-up of proteins and lipids.”

Jen and I were both unknowingly carriers of this condition, and each of our children had a 1 in 4 chance of being affected

—TRACY VANHOUTAN

The VanHoutans co-funded ongoing research with a group at Rutgers that figured out a way to synthetically make the missing enzyme in the lysosome that leads to CLN2 Batten disease. Agonizingly, by the time it got through the animal-testing phase, as well as pre-clinical studies in order to begin a clinical trial, both Noah and Laine were too far progressed in the disease to participate.

“The results were pretty incredible, though,” Tracy VanHoutan said. “The kids [who received the treatment] showed significant stabilization in some symptoms of the disease. If you started treating them at 1-and-a-half or 2, they would have better outcomes than if they started treatment at age 6 or 7. The data was so incredible, the life-saving drug named Brineura was approved both in Europe and in the United States as a therapy for kids with CLN2 Batten, and was written up in The New England Journal of Medicine.”

Today, the VanHoutans, who have two healthy children, an eighth-grader who was Laine’s fraternal twin and does not have Batten disease, as well as a 4-year-old who is also unaffected, focus their efforts on getting Batten testing into the newborn screening panel.

“Noah was misdiagnosed initially,” Jennifer VanHoutan said. “Part of our mission is awareness building—for parents and doctors. You don’t want to scare new moms, but you want to catch it early.”

Because the treatment can’t undo past damage—only stop it from progressing any further—early diagnosis is crucial.

It took nine long years to bring Brineura to market, and the process for including Batten in the newborn screening panel is equally arduous.

“Once you get an FDA-approved treatment, people have to advocate state by state to get it into state legislation,” Jennifer VanHoutan said. “A CLN2 Batten newborn screening panel pilot program began this past July, which is the first step in adding CLN2 Batten disease to a newborn screening panel. Even if we had done genetic testing [with Noah and Laine, Batten] wouldn’t have been detected; so a newborn screening panel is critical.”

And so, despite the heartbreak, the VanHoutans continue their crusade to help children living with CLN2 Batten, and fundraising is a big part of that. On Nov. 9, the VanHoutan and Kennicott families will host A Fifth Season: Hope Shines on Batten, at the Marriott Oak Brook, featuring dinner, open bar, music, live and silent auctions, and a lot of twinkling lights, which Jennifer VanHoutan said “represent our little angels shining down on us.” ■

To purchase tickets to Hope Shines on Batten or to make a donation, go to noahshope.com.