A LIFE OF MEANING

After a Lake Highlands child dies of a little-known genetic disorder, the neighborhood pushes for ALD screening in newborns

By CHRISTINA HUGHES BABB

What happened to Julian Kampfschulte is a tragedy that defies comprehension.

By the time he died on Oct. 6, the 8-year-old was well known among residents of Lake Highlands and beyond.

Until Julian, most of us — including Julian’s parents, Jenna and Kurt, and sisters Quin and Casey — had never heard of adrenoleukodystrophy (ALD).

Now many are uniting to get the word out about the genetic disorder so other families might be saved from its ravages.

JULIAN’S FIRST-GRADE teacher at White Rock Elementary, Toyia Crisp, says he was one of her brightest students, when he began experiencing “a little bit of trouble” in the classroom.

He seemed to be having difficulty with both his hearing and sight, his mom says.

But they soon learned the problem was his brain, Jenna says, which was failing to transmit messages from his ears and eyes.

When MRI results indicated adrenoleukodystrophy, Julian’s pediatrician advised they stay calm — they needed to be sure. But Jenna immediately began researching the prognosis and, she says, things fell chillingly into place.

ALD, an X chromosome-linked disorder that occurs primarily in boys, progressively damages myelin, which protects the nerves of the brain and spinal cord. Victims gradually lose the capacity to see, hear, eat and walk. Death soon follows.

“There was a lot of denial in there. We knew more or less what was going to happen, but we didn’t know exactly how it would unfold,” Kurt says. “Every case is a little different.”

For three months or so after the diagnosis, though Julian’s hearing was deteriorating, nothing else happened. Julian, his family, classmates and teachers learned sign language, awaited the inevitable, prayed for a miracle.

Toward the end of February, Julian’s vision started to go, Kurt says, “and it went pretty fast, over the course of like three weeks.”

Blinking back tears, Jenna recalls the first time Julian seemed truly anguished. “When he realized he would no longer be able to see to read his books, he cried.”

Crestfallen Classmates

, friends, neighbors and strangers conceived creative attempts to console and support the Kampfschultes. Many helped organize a holiday party complete with bounce slides, Santa and gingerbread houses; volunteer photographers from local nonprofit Fotolanthropy documented the day. Brazos Films produced a short film, “Snuggle Julian,” about Julian’s plight. Julian’s aunt, Katy Dilland, launched an organization by the same name. The images of Julian — his laughter, his tears, his tiny schoolmates gathered around him, guiding him to class, communicating with him by sign language — spread like wildfire and drew TV news reporters to his door.

At the September meeting of the 100 Women of Lake Highlands (200-plus women who pool $100 each quarter to donate to one local cause), several charities in the running for the $22,000 pool ceremoniously, one by one, withdrew their ballots from consideration, instead requesting that all funds go to Julian.

“This was no small gesture as the amount undoubtedly would make a huge difference for any organization,” 100 Women president Crispin Deneault says.

Those who gave did not care how the family used the money, which they say they divided between vacations, gatherings with loved ones, time off for Julian’s caretakers, hospital and funeral expenses, grief counseling, helping other ALD families and lobbying for ALD prevention.

People paying attention to the Kampfschultes’ journey felt powerless. Because nothing else could be done, we pulled out our wallets.

“We can’t possibly know the depths of their struggle,” Deneault says, “but hope in some small way the gesture gave a wonderful family a moment of peace.”

Two nights before he died, children gathered outside Julian’s window and sang “Amazing Grace,” a deed that Julian could not see or hear, but maybe felt.

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A MONTH AFTER his memorial service, rainbow-colored bows hug tree trunks and street signs throughout the Kampfschultes’ White Rock Valley neighborhood.

When we speak, Jenna says she’s ready for the ribbons’ removal.

“They are sad. But I’m not sure Kurt wants them to come down yet. We have our own ways of dealing.”

On the important things, though, they agreed — like when they both realized and accepted that extreme medical treatment was not an option for their son.

After meeting with specialists in Houston who suggested a bone marrow transplant, they weighed the option and decided on a different course of action.

Both by now were well-read on therapeutic prospects, and they sought the opinion of Westin Miller, a foremost New York doctor specializing in bone marrow transplants and treating ALD. More straightforward about their options, he made clear that a bone marrow transplant would do little to improve Julian’s condition, it couldn’t save him, and it would cost millions.

Jenna and Kurt opted instead to dedicate all the time they had left to enjoying their sensitive, funny, creative and humble son — embracing his too-small yet beautiful life, they say, and showing him how much they love him.

As the old saying goes: Instead of counting the days, they would make the days count.

Accepting others’ generosity on behalf of Julian, the family swam with dolphins in the Hawaiian ocean, courtesy of the Make A Wish Foundation; played with penguins and polar bears, thanks to the Dallas Zoo; caught a show at Medieval Times; traveled to Michigan; snow skied and stuck together.

JENNA AND KURT met fellow parents of terminally ill children. They learned and advocated. Talking to family, Jenna discovered two distant relatives had died from symptoms that resembled ALD, though no one knew what to call it back then.

“It came from my mom’s side of the family ... from me. Women who have the defective gene are carriers,” Jenna explains.

That means Julian’s sisters could carry it too and will be tested before they ever consider having children.

There is no cure for ALD. It can be treated, but only if detected before symptoms appear.

Mandatory testing at birth is the most sensible way to prevent its fatal progression, most experts agree.

The Secretary of the Department of Health and Human Services in February 2016 approved the addition of ALD to the list of genetic diseases suggested for newborn screening. In June, The Texas Newborn Screening Advisory Committee — after Jenna and a friend traveled to Austin to share Julian’s experience — unanimously recommended to the Texas Department of State Health Services that ALD testing be added to the state panel.

But Texas still has not secured funding to screen newborns for adrenoleukodystrophy. Lawmakers will have an opportunity to nail down the needed capital in January.

Thousands of people from our area have sent letters to Gov. Greg Abbott, asking him to make sure that, during