Zanelle Chambers is a happy, bubbly 3-year-old who loves to share kisses with her parents and play with her older siblings. The toddler, diagnosed with spina bifida, has come a long way since overcoming two major surgeries within 48 hours of birth.
“She is so sweet and loving but also headstrong and feisty,” said Katie Stephens, Zanelle’s mother. “She is the baby of four children, and she runs the house.”
Spina bifida/myelomeningocele
Zanelle’s spina bifida was discovered during a routine pregnancy ultrasound.
“Once I saw it on the ultrasound, it was obvious,” said Katie. “It looked like she had a hump on her lower back.”
A congenital anomaly, spina bifida is typically diagnosed before birth with imaging. It occurs when the spine doesn’t close all the way. The opening in the spine allows the spinal cord and spinal nerves to develop in a sac outside the body.
Complications from spina bifida can range from very minimal to severe, depending on how much damage there is to the spinal nerves.
Zanelle has myelomeningocele, the most severe form of spina bifida, which is prone to urinary tract infections, paralysis, and urinary incontinence and bowel issues.
Specialized spina bifida care and treatment
Katie was referred to the UTHealth Houston Fetal Center for a specialized delivery plan and the UT Physicians Spina Bifida Comprehensive Program for her daughter’s long-term spina bifida care and treatment.
Baby Zanelle was born via cesarean section in 2022.
“I got to see her for just a moment before they had to take her away,” said Katie.
The newborn underwent her first surgery the day she was born to repair her back and a second surgery the next day to place a shunt in her skull for hydrocephalus, an abnormal buildup of fluid within the brain.
She spent a total of 12 days in the neonatal intensive care unit, was discharged, and began treatment with the UT Physicians Spina Bifida Comprehensive Program just weeks later.
“Spina bifida can be a multiorgan problem,” explained Jason K. Au, MD, a pediatric urologist with UT Physicians and medical director of the UT Physicians Spina Bifida Comprehensive Program. “Even after the spinal cord is repaired by a neurosurgeon, there are other impacts to the lower extremities and multiple organs, and this makes our multidisciplinary approach necessary for the best outcome possible.”
Complications from spina bifida
Zanelle’s condition resulted in a series of complications. She had no feeling below her hips and could not move her legs, ankles, or feet. Both her hips were out of their sockets, she could not bend her knees, her left foot was clubfoot (turned inward), and the right was rocker bottom foot (an abnormal curvature of the sole of the foot).
Treatment
At 3-weeks old, Zanelle began treatment for spina bifida. Lindsay M. Crawford, MD, a pediatric orthopedic surgeon with the UT Physicians Spina Bifida Comprehensive Program, focused on the long-term function of Zanelle’s knees, ankles, and feet.
“The nerves in our spinal cord drive all the muscles in our lower extremities,” explained Crawford, an associate professor in the Department of Orthopedic Surgery with McGovern Medical School at UTHealth Houston. “When these nerves are involved in spina bifida, this can lead to effects downstream and impact movement, feeling, and ability.”
Leg and foot casting
Zanelle went through a series of leg castings from her thighs to her toes to slowly and gently increase mobility in her knees and correct the positioning of each foot.
“We had several goals. Her knees were stuck in a fully extended position, so with each cast, we were trying to bend the knee more and more,” explained Crawford. “We were also working to correct her feet and did a different cast on each foot.”
Doctors changed her casts every week for six weeks.
Achilles surgery
After her final casting, Crawford performed a procedure on the clubfoot to lengthen Zanelle’s Achilles tendon.
“A clubfoot looks like it’s pointing down like a ballerina because the Achilles tendon is too short. So, we cut the tendon and bring the foot into a flat position, separating the two parts of the tendon, and the missing piece will then grow in between, becoming the correct length,” said Crawford. “Babies can completely redevelop the Achilles tendon.”
Bracing
The UT Physicians team outfitted Zanelle with a series of leg braces to continue improving muscle strength and alignment. She wears an ankle-foot orthosis (AFO) brace during the day to support and straighten her feet into the correct position. Zanelle also wears a knee-ankle-foot orthosis (KAFO) at night to maintain the stretching on her knees. A third brace called a hip-knee-ankle-foot orthosis (HKAFO) attaches at her waist. She wears the HKAFO during therapy and when practicing standing.
“We have to keep working on stretching and range of motion,” said Crawford. “There is a lifelong component to this treatment because her joints and tendons want to revert to their position from birth.”
Urodynamics
A common complication with spina bifida is the lack of bladder and bowel control, leading to spontaneous emptying or retention.
Without an urge to release the bladder, there is a risk of bladder infections that can turn into kidney infections. In Zanelle’s case, ultrasounds and testing show her bladder and kidneys are safe.
“Our patient care includes a lot of testing early on to get a clear understanding of what’s going on with the kidneys and the bladder,” said Au, an assistant professor with McGovern Medical School. “This way, we can tailor our treatment, and we know if we need to be aggressive with intervention.”
Zanelle takes a low-dose daily laxative to help with her bowels and does not need any intervention for her bladder. But for some patients, catheterization may be necessary to avoid urinary tract infections.
Hydrocephalus
Hydrocephalus occurs in spina bifida patients when excess cerebrospinal fluid builds up around the brain before birth. This is a common complication for patients like Zanelle with spina bifida myelomeningocele.
Zanelle underwent surgery when she was just 2 days old to have a shunt placed in her skull. The shunt works to drain fluid and relieve pressure on her brain.
In Zanelle’s case, hydrocephalus has not impacted her neurodevelopment.
“She is smart as a whip,” said Katie.
Mobility
Each day, Zanelle is stronger and more mobile as feeling improves in her knees, ankles, and feet.
While she can’t walk, she can stand with assistance wearing braces, and she has found independence with a wheelchair.
Zanelle received her first wheelchair at 18 months old and learned to use it within a week.
“She hasn’t missed a beat since getting her wheelchair,” said Katie. “She is a pro.”
Katie is hopeful Zanelle will one day be able to stand and walk independently.
“It all depends on Zanelle and how much she wants to work for it,” said Katie. “She can’t bend her knees right now, but she has movement at the hip, which is what she needs in order to walk.”
As a curious and growing toddler, she has progressed from sitting to standing with help and weight-bearing, marking significant milestones in her progress.
“All the treatments that we do are to set her up with the potential to walk,” said Crawford. “I truly believe Zanelle is going to do what Zanelle is supposed to do, and I would never put limitations on her.”
A care team that feels like home
Finding a dynamic and multidisciplinary spina bifida care team for long-term treatment is not usually part of preparing for a new baby. Still, when faced with a spina bifida diagnosis, Katie says she is thankful she was referred to the UT Physicians Spina Bifida Comprehensive Program.
“I could not have handpicked a better care team,” said Katie. “Zanelle loves them. When she sees them, she lights up. The way my daughter reacts to her doctors tells me we are in the right place.”
