by Cally Johnson, Evey’s Mom
Draper, UT
At three months old, our daughter, Evey, was diagnosed with a rare genetic disorder called 17q21.31 microdeletion syndrome (or Koolen De Vries syndrome).
As part of her disorder, Evey has many physical anomalies. Throughout the first year of her life, these created many challenges and she had several surgeries to help give her a better quality of life. As time went on, we noticed how severe the curve in her spine was, and that it was affecting her breathing. It became more obvious through different x-rays that we needed to see an orthopedic doctor.
When looking for a doctor, we started to hear great things about Dr. John Smith. We heard from several other doctors around the nation that he was one of the top doctors in scoliosis treatment, and that he had actually developed one of the main surgeries used to treat severe scoliosis. We couldn’t believe how lucky and blessed we were that one of the top orthopedic doctors in the nation worked at a hospital 30 minutes away from our house.
After meeting with Dr. Smith, we knew we were in the best hands. He advised us that he should meet regularly with our daughter to monitor how her scoliosis progressed over the next couple of months – and then, we would make a decision about treatment. We were so grateful that he didn’t jump to any treatment decisions without getting a very clear idea of how her scoliosis would progress.
X-ray showing Evey’s VEPTR device.
When Evey was 14 months old, her Scoliosis had progressed to a 69 degree curvature in only a few months. It became evident to Dr. Smith that she would need surgical treatment to prevent her spine from compressing her lungs and heart, a procedure called the Vertical Expandable Prosthetic Titanium Rib (VEPTR). We were ecstatic to hear that Dr. Smith would do the VEPTR procedure himself, and that he personally had been one of the developers of that procedure. Evey was one of the youngest patients on whom Dr. Smith had done the procedure, and we knew that she was in the absolute best hands.
With the VEPTR procedure, Evey had two titanium rods placed on both sides of her spine, connecting to her ribs and pelvic bone. She will have them surgically expanded every 6 months for the next 12+ years. Her life and health has dramatically improved with the VEPTR procedure, and the surgery decreased the curve in her spine by 35 degrees.
Even though we know we have dozens of spinal surgeries to be performed over the next dozen years – and sometimes that can seem daunting – we know we are not only in the hands of a great doctor, but also someone we would call a friend. Looking back, we are grateful that Evey’s medical team caught her scoliosis quickly, and that we were referred to a doctor that helped guide us to make the right decision at the right time for our daughter.
We are forever indebted to the Orthopedic team and Dr. Smith at Primary Children’s Hospital, and we truly look forward to seeing them regularly.
Story Lines is a feature on Play Ground telling the personal stories and experiences of people cared for at the hospital. If you would like to share your experience on the blog, please contact us.
Play Ground | Primary Children's Hospital Blog 