Severe Spine Curves and Related Complications: Vaia’s Story
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When Brittanie and Tom welcomed 18-month-old Vaia into their home as a foster child in December 2018, doctors expected her to only live another six months. Diagnosed with the most severe type of spinal muscular atrophy (SMA), a progressive, neurodegenerative disease, Vaia had no muscle tone, limited mobility and only spoke one word: “hey.”
Initially, the couple wanted to give Vaia a few precious memories in whatever time she had left – to experience Christmas with a loving family, to celebrate her birthday – but as they got to know her, they saw she was capable of so much more. An incident at a Labor Day party in 2019 sparked a change that would alter the little girl’s future.
“Vaia had just learned she could roll on the floor – and move where she wanted to go,” Brittanie explains. “That day, she just kept rolling and rolling – all the way across the room and she was laughing the whole time. From that tiny moment of joy, we knew she wanted to live, and that we had to do everything possible to help her.”
While Vaia’s feisty personality began to shine through, her health continued to deteriorate. Along with dealing with the worsening effects of SMA, Vaia was diagnosed with neuromuscular scoliosis (a C-shaped curve in spine) and kyphosis (a forward “hunchback” curve). As her misshapen spine grew, she developed thoracic insufficiency syndrome (TIS), affecting her breathing and lung growth.
Vaia’s orthopaedic doctor near the family’s home in Milford, DE, suggested casting the then 2-year-old’s spine from her arm pits to her hips to prevent her scoliosis, kyphosis and TIS from getting worse. Brittanie was devastated by the recommendation: “Anything we just got her to do, she wouldn’t be able to do with this type of massive cast on.”
Brittanie fought for a second opinion – complicated by the fact that, at the time, Vaia was still a foster child and her care overseen by the state. Eventually, the state allowed the family to get a second opinion at Children’s Hospital of Philadelphia (CHOP).
In October 2019, Vaia’s family brought her to CHOP’s Wyss/Campbell Center for Thoracic Insufficiency Syndrome, where a multidisciplinary team of experts from orthopaedics, pulmonology, neurology, radiology and genetics could evaluate Vaia and develop a customized treatment plan. They met with Patrick J. Cahill, MD, Director of the Center, who evaluated Vaia and reviewed images and diagnostic tests to determine the best course of treatment.
Dr. Cahill told Brittanie and Tom that Vaia was a candidate for a treatment for young children with severe spinal deformities: MAGEC growing rods. Like traditional growing rods, the MAGEC rods are used to straighten the spine; but have the benefit of requiring fewer surgeries than growing rods or vertical expandable prosthetic titanium ribs (VEPTR) – a definite plus for a medically complex child like Vaia. MAGEC growing rods use an external remote control to lengthen the rods as the child grows.
Dr. Cahill initially recommended delaying surgery to determine how fast Vaia’s spinal curves were worsening. By June 2020, it was obvious her condition would need surgical intervention and he recommended surgery as soon as possible. But first, Dr. Cahill wanted Pulmonologist Oscar H. Mayer, MD, to evaluate Vaia and help her family prepare her for surgery.
Dr. Mayer met with Vaia’s family and advised them to wean Vaia off certain medications that could complicate her recovery after surgery. Brittanie and Tom – who’d adopted Vaia in May – worked with doctors to carefully decrease medications Vaia no longer needed.
In mid-August 2020, Vaia was cleared for spine surgery. On Aug. 13, 2020, Dr. Cahill performed the delicate procedure to straighten Vaia’s spine, implanted two MAGEC rods – one on each side of her spine to guide future growth – and used VEPTR hardware to secure the expandable devices in place.
“She looked so big after surgery – like she grew up overnight,” Brittanie says of Vaia. “One of our biggest concerns after surgery was about her being extubated – because that’s often a challenge for kids with SMA. But the team at CHOP had her extubated before we even got into her room after surgery.”
Vaia received and responded well to aggressive respiratory therapy at the hospital and was able to go home four days post-op. For a short time, she received respiratory care at home and has continued to improve.
The past two years have brought a multitude of changes to Vaia’s life. Now 5 years old, Vaia is a sassy, firecracker of a kid who loves to read, play with her dolls and play tag with her brothers in her motorized wheelchair. Currently homeschooled, Vaia reads between a kindergarten and first-grade level, loves to swing and spend time with the babies her family fosters. For Vaia’s fifth birthday, the family decorated the house in unicorns – her favorite mythical creature.
Vaia returns to CHOP every three months to have her MAGEC rods expanded, a simple outpatient procedure. In March 2022, she had surgery to replace the MAGEC rods with longer devices that will grow with her for the next few years. The family is planning a move to a southern state next year but wants to continue Vaia’s care at CHOP.
“CHOP saved her life,” Brittanie says. “She’s outlived the life expectancy for SMA type 1 that doctors in Delaware gave us when we first began fostering her.
“We were so lucky to find world-renowned doctors, like Dr. Cahill and Dr. Mayer, and a leading hospital like CHOP that’s ready to fight for kids like Vaia and give them a chance at happily ever after … for however long they have. ”