Friday, May 25, 2018
Health

Toddler returns to Florida hospital after eight-month stay in Philadelphia hospital

This was her third boy, and Mandy Sheridan knew what needed to be in order before the baby was born.

Call the hospital, call the baby photographer, call the priest, call the funeral home.

Doctors diagnosed two of her sons with short-rib polydactyly syndrome, a rare disease that affects the rate of growth in a baby's ribs.

Jake lived only 20 minutes.

Ethan, who had a different problem, was stillborn.

When she was pregnant with Madden, diagnosed with short-rib at 26 weeks, she braced for the worst. When she went into labor, she didn't want to push, because she knew what would happen next.

"We wanted to meet him," she said. "Those 20 minutes we had with Jake were worth it."

But Madden made it through the first few hours. His oxygen-absorption rate was 96 percent.

"He was pink," Sheridan said. "I don't think I've breathed again since that day."

Their geneticist suggested it was asphyxiating thoracic dystrophy — also called Jeune syndrome — a similar disease that has a longer life expectancy.

Sheridan then called the funeral home to cancel the plans.

"It was great to call and say, 'Well, it's, um, definitely not what we expected,' " Sheridan said. "We didn't expect it, that's for sure."

That was May 25, 2012.

On Wednesday, the Sheridan family returned to Florida after an eight-and-a-half-month stay at Children's Hospital of Philadelphia, or CHOP, where Madden underwent surgery to place a metal implant that will help separate his ribs over time and give him a better shot at living to adulthood.

"Madden is getting stronger every day," Sheridan said. "He still has a long journey left."

• • •

Jeune syndrome, a genetic disorder of bone growth inherited from both parents, affects one in every 130,000 people, according to the National Institutes of Health. Common symptoms of the disease include small chest and ribs, which ultimately restrict the ability of the lungs to expand and grow. Those who live past infancy face such challenges as the possibility of kidney problems, heart defects and a narrowing of the airway, according to the NIH.

Children diagnosed with the disease usually die in infancy or early childhood, but a treatment developed in 1989 by Dr. Robert Campbell — Madden's doctor at CHOP — helps improve life expectancy with generally good long-term outcomes.

It's called the vertical expandable prosthetic titanium rib. The VEPTR is a curved metal rod that pulls on the ribs, forcing them to grow and expand as the child gets bigger.

Campbell said that the survival rate for Jeune syndrome with the VEPTR is about 80 percent. Without it, the fatality rate is 70 percent.

"We sort of flipped the survival rate," he said. "I'm encouraged by the children who have survived this."

Implanting the device took an intense, two-part surgery that left Madden at the hospital in Philadelphia for months. Doctors had to break eight ribs in the back and eight ribs in the front of Madden's body, Sheridan said.

"The first two (surgeries) are horrific," she said.

Now that the device is implanted, Madden will just have to go to Philadelphia every four to six months for a less-invasive expansion surgery. The next trip will be in mid August, Sheridan said.

"This little guy has had a lot," Campbell said. "I'm proud of how well he's done."

• • •

On their way home, Madden had to stop for a short stay-over at St. Joseph's Children's Hospital in Tampa. CHOP transferred him there so local doctors could get to know his case and the family could ease into at-home care. The family expects Madden to head "home, home" to St. Petersburg some time this week.

Madden sits upright in a big yellow and white crib in the hospital, with a beeping heart monitor nearby. He sometimes has to wear a mask that forces air into his lungs. It sounds like a miniature Darth Vader.

Mandy stands by while her husband, Chris, plays with Madden's plush blocks and medical gloves blown up into purple balloons. Their 3-year-old daughter, Bailey, who is happy and healthy, plays with an iPhone at their feet.

Madden has two Philadelphia Phillies baseball caps, from the holidays spent near the CHOP gift shop. Chris is a big Tampa Bay Rays fan, and said the Phillies red and blue will soon be swapped for navy blue and white.

"We definitely got to switch all our Phillies gear for Rays gear," Chris said. "We need a Rays hat, Madden."

Chris looked away briefly to see what Bailey was up to. A soft wooshing sound came from the crib.

"Chris," Mandy said, "his mask is off."

He turned around, and adjusted the strap, making sure it covered Madden's nose and fit snugly on his face. It's minor compared to what they call "Madden Moments," when nurses and doctors come rushing in. Chris and Mandy have been left standing by as Madden has "coded" or stopped breathing — almost losing him altogether — three times.

"That's the kind of thing," Chris said. "You look away for a second."

Once home, Madden will be on oxygen during the day and on a ventilator at night. There's a feeding tube too, and all the equipment he'll need will be moved into his room.

"It's like a minihospital in his room," Mandy said. "The Madden ICU. The MICU."

Leaving the safety of the hospital will be tough, Mandy said, but she's looking forward to it. Madden went outside only three times during his long stay at CHOP, and Mandy is looking forward to playing with him in the grass.

"Leaving the safety of the NICU is a shock," she said. "I'm so happy to leave. It's just going to take a while."

Charles Scudder can be reached at [email protected] or (813) 225-3111.

 
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