‘Spina bifida is a diagnosis, not a destiny’

(Photo courtesy of Amanda Kern Photography)

In February, when New Englanders have long since wearied of the icy, mud-caked snow piles, an 11-year-old from Florida is seeing it — in all its splendor — for the first time.

And it’s magical.

“I can’t believe it! I never thought I’d get to see anything like this.”

There is a lot Adam Paul, now 14, wasn’t certain he would see or do.

Adam was born with spina bifida.

“I had to grow up a lot faster,” says Adam. “I had to think — What is going to happen to me? What am I going to have to go through today?

Spina bifida – a term meaning “split spine” – happens when the brain, spinal cord and/or meninges (protective covering around the brain and spinal cord) do not completely develop. It is the most common neural tube defect (NTD) in the U.S.

In Adam’s case, he has a subtype called lipomyelomeningocele, a closed form of spina bifida in which the spinal cord is tied down and stretched — tethered — to a fatty mass.

His first surgery was at two months old.

“He did super until he was 9 years old, and then he retethered,” says Adam’s mom Debbie Paul, who also happens to be a nurse. “So he had to have surgery again, but this time things didn’t go well.”

Adam spent five weeks in a hospital near their home in Bradenton, Florida. He was leaking spinal fluid and ended up with a methicillin-resistant staphylococcus aureus (MRSA) infection, a type of staph bacteria that’s difficult to treat, because it is resistant to most antibiotics.

The basic idea behind untethering the spinal cord is to prevent loss of function and further deterioration. Adam’s spinal cord was tethered in two places — posteriorly (toward his back and up from the end) and terminally (at the end). So, before the surgery, he was losing function in his gait, bowel and bladder and having excruciating back pain.

Adam underwent four surgeries within three weeks. After each of the first three surgeries, he developed a spinal fluid leak, so he had to go back to the OR for surgeons to seal the leak, clean out the tissue and close the incision.

“It was terrifying,” says Debbie. “At one point we weren’t sure if he was going to have encephalitis or brain damage. We began doing some research online for a second opinion, and in doing so we found Dr. Warf at Boston Children’s.”

Adam Paul, spina bifida

From Bradenton to Boston

Within 24 hours after Debbie phoned the Center for Spina Bifida and Spinal Cord Conditions at Boston Children’s Hospital, Rebecca Sherlock, a pediatric nurse practitioner returned her call.

“We were lost and trying to figure out what to do,” says Debbie. “When you are looking at taking your child back into a surgery, it’s very scary, and she was so reassuring. She was the first voice we heard from Boston Children’s, and she has been a lifeline ever since.”

Eighteen months after his previous surgery in Florida, the Pauls were headed to Boston for Adam to undergo yet another surgery to untether his spinal cord.

It meant finding a place the family could stay while Adam recovered. It meant conquering Adam’s growing fears of hospitals, doctors and nurses. And it meant learning to trust in the team that would become a familiar circle in Adam’s recovery and ongoing care — neurosurgeon Dr. Benjamin Warf, orthopedic surgeon Dr. Lawrence Karlin and urologist and director of the Spina Bifida Center Dr. Carlos Estrada.

“When we went to the Spina Bifida Center for the first time they all looked at his testing and everything we’d had done in the past few days,” says Debbie. “And they all came together and said, ‘OK, yes, we do think he’s tethered and this is why.’ And each one of them chimed in on it. It was a whole team working together, and that was something we hadn’t seen before.”

Dr. Warf with Adam Paul who has spina bifida

When Warf, director of the Boston Children’s Neonatal and Congenital Anomaly Neurosurgery Program, performed Adam’s surgery, he said it was like moving a millimeter a minute. The procedure lasted eight hours.

Warf was able to free up the cord from the fatty tumor and the dura — the outermost membrane enveloping the spinal cord. He also was able to remove some of the tumor from between the nerve roots at the base of the spinal cord. And, to his family’s relief, the surgery was successful, and Adam never developed a spinal fluid leak.

“It was a whole team working together, and that was something we hadn’t seen before.”

Things were looking up for Adam.

“Dr. Warf took one very scared young boy — and his equally scared family — and taught us to trust once again,” says Debbie. “This man is amazing. He has a bedside manner that is truly a gift, and his talent in the OR is unsurpassed.”

Adam spent a month in Boston recovering and learning to walk again with the help of his favorite ‘physical therapist’ — his big sister Jacqueline. “I can’t say enough positive things about her,” says Adam. “She has been a shoulder for me to lean on.”

Learning to live with spina bifida

Adam’s spina bifida journey has been a lonely one at times. He misses his friends, his family and his pets whenever he is in the hospital for extended stays. But he’s able to make up the schoolwork and still manage to get straight As — despite everything.

He returns to Boston Children’s for annual visits and also has returned for several surgeries — a second spinal surgery and two orthopedic surgeries to help him walk again.

He wears a brace on his left leg to keep his tendons stretched and his ankle supported. “I have had to relearn to walk multiple times. I can’t run nearly as fast as the other kids. I don’t have the stamina. At times it limits me a lot.”

But despite the physical limitations, each year Adam participates in the Walk ‘N Roll for Spina Bifida with the new friends he’s made by joining the Spina Bifida Association of Central Florida. Last year, he was in a wheelchair.

This year, he is walking.

“Spina bifida doesn’t hold you back as much as people think,” says Adam. “Hard work and persistence pay off. “Spina bifida is a diagnosis, not a destiny.”

The Paul family
The Paul family: From left, Dad, Steve; Mom, Debbie; sister, Jacqueline; and Adam

Learn more about the Boston Children’s Center for Spina Bifida and Spinal Cord Conditions. Request an appointment or an online second opinion.