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DUPONT — Scott Decker has a tendency to get choked up when he speaks of his son, Zachary, and his daughter, Faith.
Nearly four years after Decker lost his second child to Cockayne syndrome (CS), a genetic disorder so rare that it afflicts only two in every 1 million children born in the U.S. and Europe each year, he sometimes needs to defer to his wife, Jeanie, when the subject comes up.
Although recalling might always prove difficult for the Pittston couple, they have found the courage to spread awareness of CS and celebrate the lives of their children.
In memory of Zach and Faith, a scratch out pool tournament was held on March 28 at the VFW in Dupont to raise money for the Share and Care Cockayne Syndrome (SCCS) Network.
Ten teams, totaling 20 participants, showed up to donate and compete, and at least 20 more who did not play were in attendance. Entry fees were collected and half of the money was saved for a prize pool while the other half will go to SCCS.
The foundation uses that money to bring families of children with CS to network events where they can get information and receive support. In addition, money goes to the continued genetic research of Dr. Edward Neilan of the Children’s Hospital Boston and the Pediatric Department of Harvard Medical School.
Dr. Neilan discovered the gene that carries the mutation responsible for CS, and he devised a way to diagnose patients and carriers by blood testing.
CS is a DNA repair disorder. When cells are damaged in a healthy body, they regenerate at a fast pace. In children with CS, cells regenerate slowly, if at all, resulting in the accelerated aging of the afflicted child. Other symptoms include extreme sensitivity to sunlight, abnormally small head size (microcephaly), failure to grow at the expected rate, and abnormal brain and nervous system development.
There are three types of CS, but the most common type, type II, has an average life expectancy of about 7 years.
There are so few cases of the disease worldwide, because each of a child’s parents must carry the recessive mutation of a specific gene for the child to have the disorder, and even then, a child might be born perfectly healthy.
When the Deckers had their first child, Zachary, very little was known about CS. In fact, Zach was misdiagnosed with cerebral palsy followed by a series of diagnoses that were symptoms of his disorder rather than the disease itself, including symptoms like microcephaly, failure to thrive, and others.
After getting good advice from a specialist that was visiting Geisinger, the couple began collecting information on CS. Jeanie Decker recalled how frustrating and unsettling it was to have a child suffering from a disorder that was widely unknown.
“We used to carry a brochure to give to the doctors so they would know what they were dealing with,” she said.
As disheartening as the medicine was at the time (Zach was born in 1994 and wasn’t diagnosed until 1997), the Deckers found support in SCCS. The president of the foundation called the parents in the hospital room the day that Zach was diagnosed.
Zach passed away in 1999.
When Faith was born in 2004 and then properly diagnosed, the family became more involved in the network. After living through trials and tribulations with Zach, the Deckers were more aware of CS via their personal experience and information and support they received from SCCS.
Faith passed away in 2011.
Jeanie credits that awareness as well as the advancements made by Dr. Neilan as the reasons that Faith had a good quality of life, and she and her husband intend to pay that support and awareness forward. She views every opportunity to spread information as another chance to ensure a proper diagnosis for a deserving child.
“They’ve been so great with us, Share and Care, and we just feel like we need to give back to them, and with our knowledge of having two children with this disorder, we’re able to help other families that are just learning that their children have it now,” she said.
Jeanie currently helps manage social media platforms for the network, using Facebook, Twitter and Instagram to spread information and awareness.
Scott, for his part, sees the faces of his own children on every child with CS that he meets through the foundation. He explained that all children with CS have similar facial features, but his explanation did not disguise the emotional connection of a loving and still grieving father.
“I get attached to the kids,” he said. “I think of them as my own kids.”
Jeff Burns and Christine Gagliardi were named champions of the tournament, and Jeanie pointed out that the players were devoted as the games went on from 1 to 8 p.m. The couple plans to host future tournaments at the Dupont VFW.
Jeanie Decker put years of struggle, experience and love into a few simple and courageous words as she reflected upon why she and her husband held this event, which by their standards was a success. “We feel it’s important to be there for everybody, because people were there for us … and honor Faith and Zach, because they meant so much to us.”