Boy, 4, heading for eighth surgery
By Robyn Lydick
Highlands Ranch Herald
HIGHLANDS RANCH, CO - Sawyer Benson, 4, is excited about going to Legoland in Carlsbad, Calif. A few days after that trip he is off to San Antonio, Texas, not for Sea World or Fiesta Texas, but a surgery that will allow him to keep breathing.
Sawyer was born with Jeune's Syndrome, a bell-shaped dwarfing of the ribcage. Children with the disease often died from suffocation until titanium rib "extenders" were developed by Drs. Robert Campbell and Melvin Smith in 1989. The bars pry the ribs apart in two directions, allowing the lungs more room as the child grows.
Sawyer was recipient number 141. He is part of the Food and Drug Administration trials, and his progress will be followed for years. Last fall, the FDA approved the surgery.
Committing to the extenders, used in several conditions, is a several-year task. Currently, Sawyer has the extenders adjusted each six months. This adjustment in June will be the last on his current set. The following surgery will be for new extenders. The titanium ribs open space vertically and around the circumference of the rib cage. In Jeune's syndrome, the ribs do not adequately grow. At birth, and for a year or so after, Sawyer was tethered to an oxygen tank. This spring two bouts with pneumonia created a need for additional oxygen, and he again measures his world in feet of clear tubing. It doesn't slow him down. At all.
During an hour-long conversation, Sawyer bounced from the family's player piano to living room furniture, raced upstairs to his room to bring favorite toys down to show off, toys documented surgery by surgery in scrapbooks his mother Melissa makes. A favorite toy, a stuffed terrapin named Turtie, spent most of the time airborne as Sawyer sent it twirling toward the ceiling again and again.
Only a map of the dreamed-about Legoland stilled his energy momentarily. Sawyer loves Legos, a nd the trip was financed by the Make-a-Wish Foundation. He stopped by the Lego display at The Wildlife Experience recently to whet his appetite for the California trip. "I saw a submarine and a steering wheel made of Legos," he said. "Part of the window, the outside, was also Legos. And a fish."
Sawyer knows why he goes to Texas. "When I go to Texas, I get surgery," he said. "The doctor is nice, I see the nurses. The nurses are nice." Sawyer will continue with the surgeries twice a year until he is full grown. He is a small boy, weighing 25 pounds at age 4. He is short, and Melissa Benson says he will always be small. His shoulder blades are prominent in his back, so prominent that his grandmother, Darlene Erickson, calls them his angel wings. "It's where his angel wings will grow, she says," Melissa explained.
Sawyer was in San Antonio's Christus Santa Rosa Children's Hospital on Sept. 11, 2001. Melissa made the best of the situation. "The only good thing for us was his dad was able to stay with us longer," she said. The war that followed called up one of the hospital's surgeons, delaying several patients' surgeries. "One surgeon was called to active duty from September through April, so the hospital was behind," Melissa said. "We stretched the six months out a bit for the wish trip because of it."
Jeune's syndrome is a genetic disease that must be present in both parents for a child to have the syndrome. Sawyer's older sister Jessica does not have any medical problems. If a couple has a Jeune's child, the chance of the next having the syndrome is 25 percent. Melissa and William did not know anything was wrong until an ultrasound at 22 weeks showed Sawyer's ribs were abnormally small. The radiologist checked for leg bone length. "Out of the blue the obstetrician called and said he saw something," Melissa said. "Then, I was fine. I thought the woman who did the ultrasound didn't get a good picture. She had some trouble when I was in. I had another ultrasound a month later, and they told me something was wrong."
Melissa was scheduled for a cesarean section at 36 weeks. Sawyer didn't wait that long. "My mother was taking me to my appointment at 34 weeks when my water broke," she said. Sawyer spent two months in the neonatal intensive care unit at Children's Hospital. Melissa learned there was little information about Jeune's syndrome, also called asphyxiating thoracic dystrophy, or many of the related diseases. All are forms of dwarfism. Today she runs a foundation, the Jeune's Family Foundation, that helps families defray the costs of travel to the two hospitals that perform rib expansion surgeries. Which form of the surgery needed decides to which hospital a child will go.
In addition to Campbell's titanium ribs at Santa Rosa, Dr. Terry Davis at Columbus Children's Hospital uses titanium plates to expand the chest cavity. Initial stays for the implants can stretch for weeks. Follow-up surgeries take several days in hospital. "it isn't a case of if a family takes the step," Melissa said. "It is how, and we intend to help with the how."
Using the Colorado Nonprofit Development Center, Melissa is able to get more exposure to hew Web site, www.jeunesfamily.org. The foundation has money for families, it has assisted four families with surgery and travel costs, but getting the word out is difficult. "We have the money to help people," Melissa said. "But with HIPAA (Health Insurance Portability and Accountability Act, which creates a wall of privacy around patient information) the doctors can't tell us who their patients are with Jeune's. we have fliers, but the disease is so rare and patients are few and far between."
For more information about Jeune's Syndrome or the foundation, visit www.jeunesfamily.org.
To contact Robyn Lydick, e-mail firstname.lastname@example.org or call 303-794-1606 ext. 1007.
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Sawyer enjoys showing visitors his collection of toys.
The dog, Josh, and the fish, Nemo, are both from
organizations that have helped Sawyer.
Sawyer likes to play on the family's player piano.
Turtie is a favorite toy.
The turtle spends a lot of time airborne.