Thursday, December 15, 2011

Cockayne Syndrome: Rare Genetic Disorder Affects Local Area Youth

On the Process of Writing this Story: I wrote this piece as my feature print story for my Multi-Platform storytelling class. This piece challenged me as a reporter to take great care in asking appropriate questions and being sensitive to the parents who were kind enough to sit down with me and share this information about their children. It was a tough topic but I feel by writing this story I helped the Deckers bring about more awareness on this rare disease that affected their children. Furthermore, as a feature story, I learned how to craft the piece to create drama and generate reader interest.

Since he was born, Zach Decker suffered from various health problems. His parents Jeanie and Scott Decker of Pittston struggled with doctors for two and a half years to find an accurate diagnosis for him.

“He had a lot of problems and we didn’t understand why,” Jeanie said. “And none of the doctors could tell us why. He had this big, long diagnosis: failure to thrive, microcephaly (small head), poor growth, he had contractures in his muscles and legs. They couldn’t fit him in a category. They couldn’t find out what was wrong.”

After suffering from severe malnourishment, Zach was hospitalized at Janet Weis’ Children’s Hospital in Danville. While there, different specialists examined him. Dr. Carpenter was visiting from Philadelphia and, after looking at Zach, knew what was wrong with him.

“It only took him like two minutes and he said, ‘I know what your son has, I’ll be right back.’ And he comes back with a brochure about cockayne syndrome,” Jeanie said. “In it, we open it and my husband says, ‘how did you get a picture of my son.’ And he said, ‘that’s not your son, Mr. Decker. That little boy is 13-years-old.’ It was another child that looked just like Zach.”

Cockayne syndrome or CS is a form of premature aging dwarfism that ages the organs of its victims. Physically, CS children look smaller and baby-like. Children with CS share similar facial features such as deep set eyes and small peak noses. They look a lot like each other.

Dr. Carpenter also explained to the Deckers how children suffering from the disease pass away young.

“That was hard to deal with. But it was kind of a relief to finally know what it was,” Jeanie said. “In the back of my mind, I had always been thinking they had said something must have happened while I was pregnant. There must have been some kind of trauma. And I’m thinking, what did I do when I was pregnant? I always felt guilty about it.”

Suffering Loss and Learning More

In 1999, Zach passed away from the disease. Then eleven years later, this past may, at 7-years-old, his sister Faith Decker would also die of cockayne syndrome. After suffering through the death of their children, the Deckers have been working hard to use what they’ve learned about the disease to raise awareness.

“For every year a CS child lives, they kind of age eight years in a year,” Jeanie said.

Children suffering from cockayne syndrome have poor immune systems. They don’t follow the regular developmental growth chart, and therefore most CS children don’t walk or talk.

Because of their poor growth, many CS children are on feeding tubes. In fact, most of them are on medications to prevent them from vomiting a lot.

Another unique quality about CS kids is they are sun sensitive. Most CS children are unable to be out in the sun. They don’t sweat so sun block can do them more harm than good.

“You would think he had sunburn with the sun block on, but it wasn’t actually the sunburn, it was just from the sun block,” Scott said. “He would get really, really red and hot. And there are times, he even looked like a lobster.”

CS Types

There are three types of cockayne syndrome: CS1, CS2 and CS3. Children with CS1 have some medical problems but can walk and have some speech skills. Their average life span can be between 10 and 20 years.

Children with CS2 have more medical problems and don’t have the ability to walk or talk. Their average life span is between four and eight years. Both Zach and Faith were considered CS2.

Children with CS3 are those kids that live longer and healthier lives. They are the rare exception of children who live to be forty or even fifty years old. Doctors can’t tell if a child has CS3 right away, but rather it is a diagnosis made as the child lives out his or her life.

Cockayne syndrome is a rare disease because both parents have to carry the mutated gene. The mutation stems from a missing protein in the carrier’s DNA. When two carriers reproduce, there is a chance their child will have both mutated genes and be born with CS.

Making Changes

When Zach was born, Jeanie was only 19-years-old and attending college. Scott and she were not married.

“You went from being a regular college student doing stuff to this mom,” Jeanie said. “Then, you think it’s going to be ok and you think ‘I can handle it. I can do this.’ And then you end up having a child with special needs. And it just compounds it.”

Not only did she adjust to having a child with special needs but to the fact that she would lose her child in a short span of time.

“You have to decide at that point what’s more important in life,” Jeanie said. “I ended up quitting school at the time to stay home to be with him because I knew I wouldn’t have him forever.”

While Jeanie stayed home with Zach, Scott worked to provide for the family. Although times were not easy, they wouldn’t change a thing.

“It made me a stronger person, made me a good mom, a good caring person,” Jeanie said. “I don’t put up with a lot of stuff anymore because at first, when I use to tell doctors something was wrong with Zach, it was like you’re a first time mom and a kid. You learn how to fight for what’s important.”

Family and Friends

Taking care of a child with special needs is challenging in itself but it can become more stressful without the support of those who care.

“I think we lost some friends who couldn’t handle it, who didn’t want to deal with people that were too busy to go out and have fun because they were home taking care of their child all the time,” Jeanie said.

The absence of certain friends will remain a constant void even today.

“I still don’t talk to the people that basically wouldn’t support us,” Scott said.

While some friends abandoned Scott and Jeanie, others including family kept them strong.

“We had very supportive families and friends who wanted to know everything they could know and what they could do to help and I don’t think we would’ve gotten through taking care of Faith, or Zach, without our families,” Jeanie said.

Zach, the Hugger

Despite having the same syndrome, both children had unique personalities. Zach was more shy and conservative. He enjoyed being held and protected.

He also didn’t sleep very well at night. Scott and Jeanie would take two-hour shifts throughout the evening and early morning to take care of him. However, sometimes he would break from his cycle and sleep longer.

“It kind of freaks you out when you get use to that cycle and all of a sudden, he just wants to sleep,” Scott said. “You just wake up and you’re like I don’t know what to do. ‘Do I wake him up?’ You end up giving him little nudges to make sure he’s ok.”

Faith, the Daredevil

Faith was different in that she was adventurous and active.

“As small as she was and as much as she couldn’t walk, she was still an active, hyper child who wanted to go,” Jeanie said. “She didn’t like to sit still for very long. She would roll all over the floor and attack us.”

She enjoyed rough play with her dad.

“She would roll over top my head. I’d be lying there like ‘you planning on getting off daddy’s head. And she start laughing at me,” Scott recalled.

Faith was healthier than Zach; therefore, she was able to have a more normal childhood.

“She went to Wyoming Valley Children’s in Kingston,” Jeanie said. “It was nice because it’s a preschool setting where there are kids with special needs mixed with typical children. She really thrived in that environment.”


After discovering Zach had CS in 1996, Jeanie and Scott did their best to handle the news.

“You can’t prepare, you just have to deal with what’s given to you,” Jeanie said. “And you deal with it the best way you can. And for us, at first, it was educating ourselves about it which was through Share and Care basically.”

Share and Care: Cockayne Syndrome Network is a global network dedicated to providing resources to families with children suffering from CS. The network sponsors annual retreats where families from all over the world come together.

During this past July, Scott and Jeanie drove to this year’s retreat in Orlando, Fla.

“It’s like when you leave those retreats, you have this withdrawal because you’re in this environment where you’re with everybody who knows exactly how you’re feeling, what you’ve been through, what you’re going through,” Jeanie says.

After losing Faith in May, they found this year’s retreat to help in the healing process.

“I told all the families at the first night, the dinner where you introduce yourself, I’m like thank you for letting me hug your child because it just helped me. It was nice,” Jeanie said.

Butterfly Walk

During the fall, families across the country sponsor walks for the Share and Care network to raise awareness and funds for the cause.

On Saturday, Sept. 24, the Deckers sponsored a 2011 Butterfly Walk in loving memory of their children at Community Park in Hazelton. There was a food vendor who donated 35 percent of proceeds to the cause. Other events included face painting, a duck pond and baskets being raffled off.

At the walk, there was a butterfly release.

“The butterflies relate to the children that have passed,” Jeanie said. “It represents their free spirit once they passed, that they can fly away and be a butterfly and fly away from all the pain and suffering that they had and just be happy and free.”

There is a 2-year-old little girl named Julianne who was recently diagnosed with CS1 in June. She is from Nanticoke.

“They had thought she had it in May which is right around when Faith passed away,” Jeanie said. “So I had just talked to her mom on the phone and the next week, Faith went in the hospital and never came back home. It’s odd we’re in the same area but its good, because we’re closer.”

The Deckers are hoping to raise awareness so that children can be diagnosed sooner. Many CS children are diagnosed incorrectly as having cerebral palsy.

“Children that are diagnosed sooner, I think, have a better quality life,” Jeanie said.

The Deckers also believe each CS child is sent to earth with a special purpose.

“I always say they’re like angels sent from heaven with the purpose to spread love,” Jeanie said. “They just love everyone.”

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