Longview Boy Copes With Rare Combination of Medical Conditions

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Wednesday, November 4, 2009

By Marney Simon

Conner Reed Jones has defied the odds by having one disease that’s life-threatening and another condition that few people have ever heard of.

Conner, 6, has cystic fibrosis, a genetic disease that causes mucus to build up and clog his lungs.

He also has prune belly syndrome, which means he’s lacking abdominal walls and has urinary problems.

How rare is the combination? “I’ve never heard of it before,” said Dr. Michael Wall, director of the cystic fibrosis center and a professor of pediatrics at Oregon Health Sciences University in Portland.

Conner’s mother, Sarah Jones, said every other doctor who has treated Conner has agreed with Wall. Her son is “the only one to have both of these ever,” Jones said.

Conner also has been diagnosed with scoliosis, a curvature of the spine.

Conner, the recipient of funds raised at this year’s Toy Soldier Bazaar, has been in Doernbecher Children’s Hospital in Portland seven times in the past year, for one or two weeks at a time. When he’s home in Longview, he spends at least an hour a day in a vibrating vest that loosens mucus in his lungs. He gets his nutrition through a tube in his stomach, antibiotics through a port in his chest and other medications with an inhaler.

Despite all his medical problems, Conner is active much of the time. During a reporter’s visit, he and his little brother, Hunter, kept busy playing together.

Sarah and her husband, Brad, have become experts in both of their son’s medical conditions.

With cystic fibrosis, mucus clogs the lungs, making breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation or swelling and infections that lead to lung damage.

About one in every 4,000 babies born has cystic fibrosis, said Wall, who is Conner’s doctor.

Much rarer is prune belly syndrome. According to the Prune Belly Syndrome Network, it occurs in only one in 40,000 births. The name refers to the wrinkled skin that sometimes accompanies the condition.

“Most doctors don’t know anything about it, or will never see a kid with it in their lives,” Jones said.

Conner has a relatively mild version of prune belly syndrome, but it still complicates the treatment for his severe cystic fibrosis.

With cystic fibrosis, “among other things you’d like him to have an effective cough,” Wall said. But Conner can’t cough because he lacks abdominal muscles.

And through lung transplants are sometimes an option for cystic fibrosis patients, Conner’s health problems rule that out, Wall said.

Some prune belly patients have reconstructive surgery to take out extra skin, and muscle tissue from the upper thigh is moved to the stomach, Jones said. But Conner can’t have surgery because of his weakened lungs.

Jones found out about Conner’s prune belly syndrome when she was pregnant with him and had her first ultrasound. “His stomach was the size of a full-term infant,” she said. Doctors drained about three coffee mug-sized containers of fluid out of his stomach with a needle. Doctors delivered Conner by C-section two weeks before his due date. “They didn’t want him to go through labor,” Jones said.

He was diagnosed with CF when he was one. Thus began the regular trips to OSHU in Portland for treatment. Two-and-a-half years ago, the Joneses moved from Long Beach to Longview to be closer to the hospital.

Sarah stays in his room at the hospital. “Doernbecher is very family oriented,” she said. “They have an extra bed in the room.”

Brad continues his work as a field technician for Cascade Networks. They cobble together a care network for their other two boys between their mothers and friends.

Conner’s treatment is intense at home, too.

He gets oxygen every night, so he can sleep better, and when he’s sick.

The boy receives three different antibiotics through a port in his chest and six kinds of medications inhaled with nebulizers.

At least one hour a day, Conner wears a vest connected to a pump that emits pulses of air. The vest’s vibration loosens the mucus in his lungs.

Though his stomach can digest food, his CF breathing problems make it difficult for him to eat, Jones said. So Conner gets most of his nutrition through a stomach tube.

“He’s not even on the growth charts,” Jones said. Conner is smaller than his brother, Hunter, who’s two years younger.

A home health care nurse comes to change Conner’s tubes but his parents handle the rest of the care. When her son’s bladder stopped working for several weeks earlier this year, Sarah Jones inserted a catheter.

She takes in stride what might seem overwhelming.

“It’s a lot, but I get used to it,” Jones aid. “I’m really good at organizing.”

She and Brad spell each other for exercise — walking around Lake Sacajawea for her, basketball for him.

Sarah Jones quit her job as a bank teller when Conner was 6 months old. Not that she would have time for a job with all her care for Conner, but Jones said she if she worked, the family’s income would be too high for Conner to qualify for Medicaid. His CF medications alone cost $8,000 per month, she said.

Though Conner attended kindergarten at nearby Kessler School, she doesn’t want to risk sending him to first grade this quarter. “If he ever got a cold it could send him back to the hospital.” She’s working with the school to have a tutor come by the house.

She and her husband plan to sign up Conner for soccer, though he can’t run much.

“We want him to live as full a life as he can but we aren’t willing to jeopardize his health,” Jones said.

For a child to have cystic fibrosis, both parents must be carriers of the CF gene. There’s a 25 percent chance the children of two such parents will have the disease.

Brad and Sarah didn’t let the odds stop them from having other children.

“We really didn’t want to let CF dictate our lives,” she said. Their son Hunter is now 4 and Braden is 1. Neither has CF.

Conner is “on every therapy and every medication they have available for a kid with CF,” Sarah Jones said.

But there’s no denying that cystic fibrosis is a progressive disease. “He’s going to get worse no matter what we do,” said Wall, the pediatrician.

“Our goal is just to keep him stable,” Jones said. “We don’t tell him there’s no cure.”

The Joneses have met other families with CF. Their church, Longview Community, bought them tickets to fly to the annual prune belly syndrome conference in Texas so Conner could meet other children with the condition. “He still talks to them by Skype,” she said

The family has started meeting with the Make-a-Wish Foundation about a trip planned for January. Conner decided he wants to go to the Big Island of Hawaii for whale watching and to take a helicopter tour to see lava.

Through all the treatment and turmoil in their lives, Jones puts a positive spin on Conner’s health.

“When he’s sick, he’s laid up. But when he’s well he looks like any little boy — you don’t even know.”

Toy Soldier Bazaar

The 32nd annual Toy Soldier Bazaar will be held from 9 a.m. to 4 p.m. Saturday at the Monticello Hotel, 1405 17th Ave., Longview.

The event, sponsored by Xi Alpha Beta Sorority, benefits a child with special health needs; this year’s recipient is Conner Reed Jones of Longview. If Conner feels well enough, his family plans to attend in the morning.

An entrance donation of at least $1 is suggested, and all proceeds from table rentals will go to Conner’s family.

The hotel will be open for lunch. For more information or to give a private donation, call Lori at (360) 751-5106.

Copyright © 2007, The Daily News, Longview, WA

Source: The Daily News Online

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