Meet Zach, The 8-Year-Old Conquering Spina Bifida with a Smile

At 28 years old, Megan Wilson’s pregnancy was considered high risk, meaning the joy and expectation of impending motherhood were marked with more intensive screenings, office visits and prenatal care. During her 20-week ultrasound, the atmosphere in the room shifted from anticipation and excitement to concern.

The doctor delivered the news: her baby had the most severe form of spina bifida.

Spina bifida is a birth disorder where the spine doesn’t fully develop, leaving some bones open at the back and potentially damaging the spinal cord and nerves. This occurs when the neural tube, which forms the brain and spinal cord, fails to close properly during the first month of pregnancy. Neural tube defects, like spina bifida, can also affect brain development, leading to nerve damage and possible learning or intellectual disabilities.

Megan’s journey from a high-risk pregnancy to receiving the highest level of specialty care and undergoing rare surgeries for her son, Zachariah, has been both challenging and transformative. From the initial diagnosis of severe spina bifida to intricate intrauterine and postnatal surgeries, Megan and Zachariah, from Checotah, Oklahoma navigated a path filled with uncertainty and hope. Her unwavering determination and expert care have given Zachariah a chance at a better quality of life.

Intrauterine Surgery

After finding out that Zachariah had myelomeningocele spina bifida, Megan was told she was a candidate for intrauterine surgery. Also known as fetal surgery, the procedure is performed while the baby is still in the womb to repair the spinal defect caused by spina bifida.

With myelomeningocele — the most severe form of spina bifida — a portion of the spinal cord or nerves are exposed in a sac through an opening in the spine. The spinal cord may or may not be covered by the meninges, the three layers of protective membranes that cover and safeguard the brain and spinal cord. This sac contains parts of the spinal cord, nerves, and cerebrospinal fluid and can cause moderate to severe disabilities such as muscle weakness, loss of bladder and bowel control, and paralysis.

Intrauterine surgery to close the spinal cord reduces the risk of further damage. During pregnancy, the spinal cord can come into contact with the amniotic fluid causing more mobility and bowel problems and increasing the risk of hydrocephalus.

Megan had intrauterine surgery when she was 24 weeks and five days pregnant to close Zachariah’s spine.

Living With Spina Bifida

Zachariah was born prematurely at just under 34 weeks, and he spent seven weeks in the neonatal intensive care unit (NICU) so he could be monitored for hydrocephalus. He was born with bladder, bowel, and movement issues – his bladder is unable to contract on its own, and drainage is difficult. When a bladder is unable to contract on its own, it means that the muscles of the bladder are not able to squeeze or tighten to push urine out. This condition is often referred to as bladder atony or neurogenic bladder. It can result from various causes, including neurological conditions like spina bifida, which affect the nerves that control bladder function.

Without the ability to contract, the bladder cannot empty itself properly, leading to urine retention. This can increase the risk of urinary tract infections, kidney damage, and other complications. In such cases, medical interventions like catheterization or surgical procedures are often necessary to help manage bladder function and ensure safe and effective urine drainage.

Zachariah’s bladder was able to store urine at safe pressures but unable to drain on its own, so he required intermittent catheterizations through his urethra. Intermittent catheterization involves inserting a soft, flexible tube called a catheter, into the bladder through the penis to drain urine. This procedure is typically done several times a day to ensure the bladder is emptied completely. Pain is common during the procedure, especially for males, and for some it is intolerable. Some children born with spina bifida lack sensation in the area so do not feel the pain, but Zachariah experienced significant pain and would scream every time Megan took him to the restroom.

“I felt like I was torturing him every time,” she said. “He would start screaming as soon as I said it was time to go to the restroom.”

Once Zachariah started school, Megan knew they needed to find a better solution.

Surgical Intervention

In August 2022, when Zachariah was six, pediatric urologist Dr. Adam Rensing, M.D., associate professor in the Department of Urology at the University of Oklahoma College of Medicine, assessed Zachariah’s bladder function and its ability to store and empty urine. His focus was on enhancing Zachariah’s independence, simplifying his care, and removing the need for painful catheterization a few times each day.

The family chose a catheterizable channel, a technique developed in 1976 by French pediatric consultant urologist, Paul Mitrofanoff. The Mitrofanoff procedure helps individuals with incontinence and incomplete bladder emptying by creating a channel from the bladder to the skin’s surface using tissue from the appendix or bowel. A small opening, or stoma, is made on the skin, typically near the navel, allowing a catheter to be inserted into the channel to drain urine. Additionally, a valve is created where the channel meets the bladder to prevent urine leakage.

As Zachariah also had bowel evacuation issues, Dr. Rensing and his team performed a Malone Antegrade Continence Enema (MACE) procedure. This surgical technique helps manage bowel movements more effectively, especially for those with conditions like spina bifida.

During the MACE procedure, a small channel is created from the beginning of the large intestine to the skin’s surface, often using the appendix. A stoma is made on the abdomen, allowing a catheter to administer an enema and flush out the colon.

The MACE procedure provides a controlled and convenient way to manage bowel movements, reducing accidents and improving quality of life. The stoma remains continent, ensuring cleanliness and comfort.

The surgeries took about four to six hours, and Zachariah’s recovery involved a hospital stay of several days. About a month after surgery the catheters were removed, and the family was trained in how to use the new channels. Initially, Zachariah faced some challenges with catheterization, but with patience and perseverance, he’s gained some independence and can now empty his bladder on his own every three to four hours.

“Now it’s just so easy,” said Megan. “We're like, OK, it's time to use the restroom. There's no more screaming. There's no more feeling like you're torturing your child because the urology team was able to fix the problem. It has made our whole lives better.”

A Bright Future

Zachariah is thriving at school, and he has control over his bladder emptying during the day.

“Zachariah is in a modified class right now, in the special education room. They’re working with him so he can do more and more. He plays with the other kids and participates in all the activities. It’s wonderful to see him doing pretty much everything the other kids do, and he loves it,” said Megan. “He has braces on his legs and limited mobility, but if he’s holding someone’s hand, he can walk and even go short distances by himself. It’s heartwarming because some of the kids in his class will hold his hand and help him walk to wherever they’re going.”

The procedures Zachariah had at Oklahoma Children’s Hospital to help him empty his bowel and bladder are permanent solutions.

“We trust the team at Oklahoma Children’s Hospital — they go above and beyond, and I tell everybody to go to Oklahoma Children’s Hospital,” said Megan.

OU Health Pediatric Urology

Dr. Rensing explains that the bladder and bowel surgeries are common for children with spina bifida, and Oklahoma Children’s Hospital OU Health performs them regularly.

“Oklahoma Children's Hospital is unmatched in the state and has all the specialties for conditions such as spina bifida,” said Dr. Rensing. “We have the specialists associated with neurosurgery, urology, nephrology, and orthopedics in one place, collaborating to provide the best care for each patient. With spina bifida, every patient is different, and each case has its own complexity. We approach every child in a personalized manner to provide the best possible outcome for their situation."

Learn more about care at the state’s only comprehensive pediatric hospital, Oklahoma Children’s Hospital OU Health. Your family has access to multidisciplinary experts, including specialists in pediatric urology or urogenital care. Request an appointment or get a second opinion by calling (405) 271-3800.