Beating All Odds

From Children's Magazine, Spring 2007
Story by Melissa Howell
Photography by Tia Brayman

Dylan Rivera

Dylan Rivera is a three-year-old boy who knows what he likes: playing outside, playing with cars, and watching the movie, Cars. He also loves the staff in the Orthopedic Clinic at The Children's Hospital. He has spent a lot of time here in his young life and is well known by many of the caregivers in the clinic. Because of his congenital birth defect, Dylan has had a number of surgeries and faces additional surgeries in the coming years, including a relatively new treatment performed at a small number of pediatric hospitals, including Children's.

An unexpected diagnosis

When Dylan's mom, Melissa, was pregnant with Dylan, a routine ultrasound showed that Dylan's left arm was two-thirds shorter than his right arm. He had a radial club hand, the result of a missing radius bone in his forearm; he had only one bone in his forearm instead of two. The ultrasound showed no other deformities.

Then, when Dylan was just three-weeks-old, he developed respiratory syncytial virus (RSV), a very serious illness that can infect people of all ages, though infants are at the greatest risk for severe illness. In children younger than three, symptoms include high fever, wheezing, rapid or labored breathing and severe cough.

Dylan spent nearly a month at Children's while he was treated for RSV. It was during this time that physicians at Children's discovered Dylan's club hand was part of a much larger congenital birth defect: Dylan has VACTERL syndrome.

VACTERL syndrome is an acronym for abnormalities of the: vertebrae (V), anus (A), cardiovascular tree (C), trachea (T), esophagus (E), renal system (R), and limb buds (L). In order to be diagnosed with VACTERL syndrome, a patient must have at least three of these abnormalities. Dylan had five - abnormalities of the vertebrae, cardiovascular tree, trachea, esophagus and limb buds.

"I was worried, wondering if he was going to be OK, what was wrong, what caused it, what were they going to do," Melissa said. "All these things were going through my head."

"We had all these questions about what caused it," said Dylan's grandmother, Mary. "Nobody in the family has anything like this."

According to Mark Erickson, MD, chairman of the department of orthopedics at Children's, there isn't a specific gene that causes VACTERL, but rather something that happens in a two-month-old embryo as all of these systems develop in a similar time frame.

"There's a cascade effect that causes all these systems to be affected," Dr. Erickson said.

Repairing Dylan's heart and hand

Because of the wide range of abnormalities associated with his VACTERL syndrome, Dylan has been cared for by staff in a number of departments at Children's. When his RSV had been resolved, the first step was to repair Dylan's cardiovascular abnormality, a ventricular septal defect (VSD); Dylan had two holes in the septum wall of his heart. When he was just four-months-old, surgeons in The Children's Hospital Heart Institute performed open-heart surgery to repair the VSD.

When he recovered from his heart surgery, the next step was to operate on his club hand, which according to physician assistant Manuel Dennis Gurule, PA-C, was "very severe."

"His hand looked like it was at a 90-degree angle," Gurule said.

For the first several months, Gurule casted Dylan's arm to prevent the hand from drifting in the direction of the missing bone. After months of casting, Dylan was ready for surgery. This involved removing the first row of bones from his wrist and moving his hand over the existing arm bone and then fusing the hand to the bone. His existing bone had a large arc on it, so surgeons made a cut in the bone, straightened it, and then fused it.

"We didn't look to give him full function, because that's not possible," Gurule said. "But in the absence of that surgery, the amount of assistance that hand can give the hand that works in a more normal fashion is significantly less. So basically we give him a position so it can assist his good hand. Right now he's using it in that fashion well, so he's had an excellent response."

Dylan's hand required two additional surgeries, one to reposition a pin that had started to work its way out and a second to remove the pin altogether.

"Dylan has become somebody who's extremely well known in our office in a relatively short amount of time," Gurule said. "All the staff know him. He really has a lot of personality. He's a very interesting little guy. He interacts with everybody, his esteem is excellent. What's most striking about him is how he's able to persevere and endure. He's been through a lot, he's a tough little guy. That's part of what adds to his personality."

By 18 months of age, Dylan had already had four surgeries, but he and his family still faced the biggest surgical commitment, which would correct his severely deformed spine and ribs.

Buying time

"Dylan has a peculiar form of spine deformity," Dr. Erickson said. "In addition to the curved spine, he also has a rib cage that's not normal. He has some ribs that are fused. It's called thoracic dysplagia. Normally we have 12 sets of matched ribs and matching 12 vertebrae. He has less than the right number of ribs, plus some are fused together congenitally. They were never separated embryologically. He also had a substantial spinal deformity where he has eight to 10 abnormal vertebrae that, rather than block-shaped, are wedge-shaped or fused together. That's what's created his rather severe deformity that involves not only his spine but his chest wall."

For many patients who have scoliosis, Dr. Erickson and his colleagues often treat the curved spine with a Risser casting technique. The Risser cast is made with a combination of plaster and fiberglass casting materials, and is put on a patient in the operating room when the patient is under anesthesia. The cast encompasses the patient's trunk, and somewhat resembles a tank top, with an area around the stomach cut out to allow for growth. The cast is changed every six to eight weeks, and the patients are seen in the Orthopedic Clinic in between each casting for X-rays and to monitor the progress.

According to Dr. Erickson, the Risser cast works better in children who have idiopathic scoliosis, an unknown cause in an otherwise healthy child, with normal vertebrae and normal ribs. In the case of patients like Dylan, the casting can buy critical growth time in young patients who will eventually need surgery.

Dylan was about a year-and-a-half when he was casted, and he had several casts.

"Dylan got used to the cast after a while," Melissa said. "But I was anxious to get the cast off and hold him."

"When they took it off he was sort of off balance again," Mary said.

"The casting did buy time, but we knew he would eventually need the VEPTR surgery," Dr. Erickson said.

'A really big commitment'

The Children's Hospital is one of 10 pediatric hospitals that offer the VEPTR, or Vertical Expandable Prosthetic Titanium Rib, treatment for severe scoliosis. The VEPTR is a titanium device that is curved to fit the back of the chest and is placed in an up-and-down position. It is made longer as a child grows. The device helps the spine become straighter and allows the lungs to grow and fill with enough air to breathe. The attachments can be secured to the ribs or the spine.

The decision to commit to the VEPTR treatment is a big one for patients and families. The treatment involves an initial four-hour surgery to implant the device, and then surgeries approximately every six months for a number of years to lengthen the device.

"We try to do the lengthenings in the spring and fall if possible to avoid flu and cold season. We're actively researching the optimal age to find out the best age to stop the lengthenings. That's probably going to boil down to when the spine growth and lung growth have reached a capacity that allows for as normal an adult life as possible. We think that will be between ages eight and 10. It's a really big commitment."

Staff in the Orthopedic Clinic are working to establish a "high-risk pathway" for patients undergoing the VEPTR treatment. This involves establishing a multi-disciplinary care team to address the patients' various medical needs before and after surgery. The care team includes specialists from respiratory therapy, social work, nutrition and pulmonary medicine. In addition, a specialized team of physicians, including a pediatric surgeon, anesthesiologist, pulmonologist, and a gastroenterologist are included in the care team.

Elise Benefield, RN, clinical coordinator of the Spine Center, and Jennifer Hawley, RN, are nurses in the Spine Center who work with families to help them prepare for surgery. They help families schedule all necessary preoperative appointments with various specialties, and work with the patients and families to help them understand what to expect in the hospital.

"We put together a presentation to help prepare them for what they'll experience during surgery and recovery and also when they go home," Hawley said.

Dylan had his VEPTR device implanted in November 2006.

"Initial implantation involved a thoracostomy where we actually separate a segment of the ribcage that allows it to grow and then we ply the rib cradles and spread them apart. This creates a bigger chest capacity and the lungs can expand into it," Dr. Erickson said.

"Dylan did amazing (during the surgery)," Benefield said. "He was a little trooper. He tolerated his pain pretty well."
"He's a happy, outgoing boy," Mary said. "His pain toleration is very good - he tolerates pain even better than I can. He bounces back from his surgeries really well."

Dylan's first lengthening is scheduled for this spring, Dr. Erickson will extend the VEPTR device by one-and-a-half to two centimeters.

"Dylan is a character," Dr. Erickson said. "I love taking care of these kids in general, but especially kids like Dylan who have so much to overcome because it can give you a perspective on your own life as well as what the kid deals with.

"He's very enthusiastic, nothing slows him down," he continued. "Whenever he's ready to go, he's ready to go. He's so enthusiastic with his activity. He's an amazing guy and always will be. From my standpoint, getting to see and know somebody for essentially a 6-year commitment process through good times and bad times and in-between times, I probably learn as much from Dylan as he learns from me."

Dylan also has some trachea and esophagus abnormalities, in addition to a paralyzed vocal chord from a breathing tube. As a result, his speech and fine motor skills have been delayed. Dylan currently is working with a speech therapist to help him overcome his speech delay. An occupational therapist is working on his fine motor and feeding and swallowing abilities. Dylan has a difficult time swallowing thin liquids, as he doesn't have the muscle tone to properly swallow.

For now, his family is taking each surgery as it comes and looks forward to his future.

"I see Dylan going to school and having fun with the children there," Melissa said. "I really don't think too much about the surgeries."

"His perseverance and his ability to come back with all these odds, I think he's going to do pretty well," Gurule said. "I think he'll have that tenacious personality. There will be things he won't be able to do at the pace of other kids his age; however, I think thus far we've given him some potential to succeed at the highest level that he can. We are taking care of his spine and that upper extremity, and if we can keep him healthy nutritionally and otherwise I think he'll do well."

"It's hard to predict the prognosis," Dr. Erickson said. "We know a fair amount about how normal vertebrae grow, but when you throw in the mix abnormal vertebrae and kids with VACTERL syndrome and fused ribs, it's really off the charts in trying to figure out how it will grow. What we can say is if we don't do anything, his curve will continue to get bigger; it's already shown us that by how it's behaved. With the VEPTR device, although it's a huge commitment with lots of surgeries coming up, our goal is to get his thorax to grow enough to allow his lungs to grow so hopefully he can have a more normal life without oxygen needs and things like that, or less of them if he does need it."

"I think Children's is a really great hospital," Melissa said. "They've done a really good job taking care of Dylan. I'm happy all his surgeries have been great."