Nov. 2, 1995 - Aug. 4, 2013

Almost a decade after becoming the first patient with Batten disease to undergo gene replacement therapy, Zachary Balog died at home Sunday in Cranberry. He was 17.

"We're very proud of Zach, of the fight that he fought," said his father, Steve Balog, who called his son a "pioneer" for the experimental treatment he underwent at Weill Cornell Medical Center in New York City in June 2004.

Batten disease, also referred to as Spielmeyer-Vogt-Sjogren-Batten disease, is a rare neurodegenerative disorder that affects roughly 2 to 4 births out of every 100,000 in the United States.

Like other victims of the disease, for the first few years of his life Zach seemed like a normal, healthy child.

"As a child, he loved 'Barney' and 'Blue's Clues.' He loved construction vehicles," his mother, Susan Balog, said, adding, "and his favorite foods were pizza and chicken nuggets."

When he was 4, he began experiencing seizures, soon accompanied by other common symptoms of Batten's, like poor balance and deteriorating eyesight and speech abilities.

Batten's is one of 40 or 50 known types of lysosomal disorders. In such diseases, the enzymes that typically break down or eliminate materials like fats and proteins are missing, so these substances build up in brain, eye, skin, and muscle cells. The buildup is what causes the gradual loss of eyesight, along with motor and cognitive skills.

The disorder is autosomal recessive, so both parents must be carriers of the recessive gene. Their offspring must inherit the gene from both parents, meaning he or she has a one in four chance of developing the disease.

The Balogs have a younger son, Joshua, 15, who is a carrier but does not have the disease.

See the article here:
Obituary: Zachary Ordy Balog / Cranberry youth battled rare disease

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