Answers for Devin
Surgery corrects rare connective tissue disorder
At a visit to the Children’s Mercy Kansas City Orthopaedic Clinic, 12-year-old Devin Corwin-Herrick was the picture of confidence—smiling and tanned as he waited for his doctor, Richard Schwend, MD, FAAP, Director, Orthopaedic Research Program.
Clearly, Devin doesn’t let the fact that he has a rare connective tissue disorder get the best of him. He has better things to do, like play X-box, or hunt, or hang out with his best friend, Brayden. But that wasn’t always the case.
At birth, Devin’s mom, Stephanie Lewis, said it was clear something was wrong with her baby. “A nurse at the hospital where Devin was born shoved a flier on Marfan’s Syndrome in front of me and said, ‘This is what your baby has.’”
That was the beginning of a diagnostic and treatment odyssey that’s taken Devin and his family from his small Kansas hometown of Alton to Kansas City, Iowa City, Iowa, Baltimore, and back to Kansas City again.
At just 2 years old, Devin needed surgery to stop the vertebrae in his neck from compressing his spinal cord, preventing severe deformity and possible paralysis. That’s when Stephanie began to question whether Devin actually has Marfan’s Syndrome, a genetic disorder that affects the body’s connective tissues.
“Devin does have some of the characteristics of Marfan’s, but not all,” she said. Because Devin has two extra vertebrae in his neck, the physician who performed his first neck surgery suggested he might have an even rarer disorder called Loeys-Dietz Syndrome (LDS) instead.
“At that point, I didn’t want to put Devin through anything else, so I decided against genetic testing,” Stephanie said.
As Devin grew, so did the physical complications he was facing. That included complex surgery to reduce the size of his aorta when he was 7 years old. That operation was performed at Johns Hopkins in Baltimore, the hospital where LDS was first identified as a disorder in 2005.
With the outstanding cardiac program at Children’s Mercy, the specialist in Baltimore referred Devin back to Children’s Mercy for his ongoing cardiac care, saving the family from traveling across the country.
By now, Stephanie had researched Marfan’s and LDS, comparing the characteristics of both, concluding for herself that Devin might have LDS. Still, she wasn’t certain.
At Children’s Mercy, pediatric cardiologist, Robert Ardinger Jr., MD, also suggested testing to validate that Devin has Loeys-Dietz Syndrome. This time Stephanie agreed.
“It was just a blood test and the results only took a couple of weeks, but they confirmed what we had suspected, that Devin has LDS,” she said.
LDS is a rare genetic disorder marked by characteristics not usually seen all together in other connective tissue disorders. These include aneurysms, twisting or spiraled arteries, widely spaced eyes, skull deformities and a split or broad uvula.
Devin also has many of the skeletal characteristics of LDS, including long fingers and toes, clubfoot, joint laxity, scoliosis and cervical-spine instability. Surgery at Children’s Mercy in 2008 with Brad Olney, MD, Division Chief, Orthopaedic Surgery, tightened the ligaments in his left knee and ankle.
'...In really bad shape'
In the meantime, the degenerative nature of the disorder caused Devin’s spine to become more and more unstable. “Devin was in really bad shape when he came to see us,” Dr. Schwend said. “His head was literally sitting on his shoulder. He had lost most of the function in his left side. The extra vertebrae in Devin’s neck were compressing his spine. Our goal was to stabilize Devin’s spine, remove the two areas that were compressing the spinal cord in his neck, and improve function.”
To do that, Dr. Schwend started by placing Devin in halo traction for a month, straightening his head and neck as much as possible prior to operating, and making surgery safer.
A multidisciplinary team including Dr. Schwend, John Clough, MD, neurosurgeon, and Jill Arganbright, MD, pediatric otolaryngologist, stabilized Devin’s spine in two separate six-hour operations. The first was performed in February 2015 on the back of his neck, and the second in July 2015 on the front.
“This was a very high-risk surgery,” Dr. Schwend said. Dr. Clough made sure the vertebral arteries weren’t compromised and performed the decompressions in the back, as well as the front of Devin’s spine. During the July surgery, Dr. Arganbright helped navigate Devin’s complex anatomy so the team could reach the problem vertebrae safely. “Their collaboration is what made Devin’s surgery possible.”
“Going in to the operation, we knew Devin’s odds weren’t good, but Dr. Schwend was always very honest with us,” Stephanie said. “He told us what they could do and what to expect.”
Initially, Devin’s recovery was difficult, but one year later his function and quality of life have improved greatly. In fact, when Dr. Schwend asked if he’d had a good summer, he grinned and said, “Yes! I’ve been fishing and camping. It’s been great!” A seventh grader at Osborne Middle School, Devin’s favorite subject is science. He and his best friend are helped with the football team, and he is active in Special Olympics.
CM: Fighting for Devin
With 16 surgeries in 12 years under his belt, Devin is a veteran patient who knows more physician visits and operations are likely in his future, including possible dental surgery and knee surgery. “I probably hold some kind of record,” Devin said.
Through it all, Stephanie said it’s the doctors and nurses at Children’s Mercy who have helped their family find the answers to the complex questions surrounding Devin’s diagnosis and treatment.
“I’ve taken Devin to hospitals all across the country to try to get him help,” Stephanie said. “When others were reluctant to do anything because it was too risky, the doctors and nurses at Children’s Mercy were willing to try … to fight as hard for Devin as I do. That’s what makes this place so special.”
Learn more about pediatric orthopedics at Children's Mercy.
Learn more about The Children's Mercy Division of Clinical Genetics.