Kate's Story - Surgery to Treat Severe Scoliosis is First in State
Born with a 40-degree curvature in her spine, Kate Rueckert had a muscle condition and associated scoliosis that presented a huge challenge for parents Jack and Lynn Rueckert.
As the curvature continued to worsen through Kate's infancy, Lynn Rueckert remembers having to "fight for every calorie" that went into her little girl's body.
Battling with both Kate's contorted posture and problems with her sucking reflex, the Rueckerts resorted to tube feedings to make sure Kate was being properly nourished and gaining weight.
"We had to fight to get her to finish anything by mouth," says Lynn Rueckert. As an infant, Kate couldn't handle anything thicker than yogurt - even small chunks of fruit mixed in the yogurt would make her gag and vomit.
"If she got upset, she'd throw up very easily," Lynn says. "Every day was a horrible stress, and every day she threw up felt like a failure because all those calories I fought to get into her were gone."
As Kate turned one, the curvature in her back had reached 90 degrees. The Rueckerts traveled frequently from their home in Kewaunee, Wis., to Green Bay, where their local doctor referred the family to UW Children's Hospital (now known as the American Family Children's Hospital) to treat Kate's back problems.
Room to Grow
At UW Children's Hospital, pediatric orthopedist Ken Noonan, MD, suggested body casting to help straighten Kate's bones. But when casting and a back brace failed to yield sufficient results, Dr. Noonan suggested trying a surgical procedure that had been showing promise in treating congenital scoliosis at a hospital in San Antonio, Texas. Only a handful of medical centers in the country implant the device, known as VEPTR (Vertical Expandable Prosthetic Titanium Rib).
The device is used to treat pediatric patients with thoracic insufficiency syndrome (TIS), a congenital condition in which severe deformities of the chest, spine and ribs prevent normal lung development. In the VEPTR procedure, surgeons implant an expandable metal rod that's curved to fit the back of the chest and spine, using hooks located at both ends of the device.
Unlike more standard surgeries to treat congenital scoliosis, the VEPTR device is designed to give the rib cage room to grow in children who suffer from chest wall and/or spine defects. While helping to straighten the spine, the VEPTR device separates the ribs to allow lungs to develop and fill with enough air so the child is better able to breathe.
The length of the device can be adjusted as the patient grows.
"With more traditional spinal fusion surgery, the non-expanding metal rods and screws stop the spine from getting worse - but they also essentially stop the spine from growing," says Dr. Noonan. "That's not a huge deal when you're 14 and done growing, but it is when you're two and need a lot of remaining spine growth."
And without some sort of corrective surgery, Kate could have developed serious and potentially fatal breathing problems due to inhibited lung growth.
First in the State
With Kate's surgery on Aug. 25, 2005, UW Children's Hospital was the first center in Wisconsin to offer the VEPTR procedure. Dr. Noonan and pediatric general surgeon Aimen Shaaban, MD, performed the surgery, assisted by visiting orthopedic surgeon David Skaggs, MD, from Children's Hospital Los Angeles.
"Hopefully, now she'll be able to maintain her lung function so she can have a normal lifespan," says Dr. Shaaban, who co-directs the UW VEPTR program with Dr. Noonan. "It's only a couple of weeks out, but she seems to be doing very well."
"We are so happy with the results of the surgery," said Lynn Rueckert. She's not sure yet exactly how much the curvature in Kate's back has improved, but Lynn jokes that her two-year-old is no longer able to stick her toes in her mouth like she did in her hunched, pre-surgery position.
"My family couldn't believe the difference, looking at her," Lynn says. "She looks two inches taller! You can see her whole neck now - she used to be squished to one side."
And even Kate's eating habits have improved as the pressure in her abdomen has been relieved. When her father eats his regular evening bowl of ice cream, Kate asks for some of her own.
"She tells me she's hungry, and that's just amazing," says Lynn Rueckert. "Just the simple joy of having her want to eat - so many people take that for granted in their kids."
Kate will eventually need spinal fusion surgery as a more definitive correction of her scoliosis, but for now, the VEPTR device will allow for sufficient growth of her spine and lungs.
"I'd love to see her get to age 10 before we do the spinal fusion," says Dr. Noonan. "For now, we certainly expect her development to improve."
VEPTR was developed in 1987 by Drs. Robert Campbell and Melvin Smith of CHRISTUS Santa Rosa Children's Hospital in San Antonio, Texas, when they were approached by a family with a child missing ribs on one side of the thorax. Drs. Campbell and Smith implanted the first VEPTR-like device to stabilize and enlarge the thorax of this patient. Following the surgery, the patient thrived.
Prior to the development of the UW VEPTR team, only seven centers in the United States were performing this procedure. In addition to the United States, the VEPTR device is also being used clinically in Europe, Canada, South America, Australia and New Zealand.