Nicholas stands tall with Prader-Willi syndrome

Nicholas, who had spinal fusion surgery, shakes hands with Dr. Glotzbecker while his parents watch.
Nicholas presents an award to his surgeon, Dr. Michael Glotzbecker. Nicholas wears headphones in public to protect himself from sensory overload. (Michael Goderre/Boston Children’s)

When he rose from his chair to shake his surgeon’s hand, 17-year-old Nicholas Peters stood 4 inches taller than he had just a few months before. “Thank you for making me feel better,” Nicholas said to Dr. Michael Glotzbecker, the surgeon from the Boston Children’s Hospital Spinal Program who had operated on his spine. With a little prompting from his parents, Nicholas added, “I can bend over to play with my jeep.”

Nicholas and his parents, Lisa and Jeff, made a special trip to Boston Children’s to thank the clinicians who worked together to stabilize his spine after years of progressive scoliosis and kyphosis. “Everyone worked so cooperatively,” says Lisa describing the team that also included specialists from anesthesia, intensive care, complex care, neurology, nephrology, pulmonology, gastroenterology and physical therapy. “They put their egos aside, they listened and they learned so they could give Nick the best possible care.”

Nicholas, who has Prader-Willi syndrome, plays outside with his brother and a child-size Jeep.
Nicholas (left) and his brother Weston play with their beloved jeep in 2010.

A rare and complicated condition

Born with Prader-Willi syndrome, Nicholas has been a medical puzzle since he was a baby. Prader-Willi syndrome is a rare genetic condition that affects the hypothalamus, a small organ at the base of the brain. In addition to neurological, endocrinological, behavioral and intellectual issues, people with the condition constantly feel hungry. Parents have to lock refrigerators and kitchen cabinets to prevent their children from uncontrollable binge eating.

Spinal problems are also common in children with Prader-Willi syndrome. Due to low muscle tone and low bone density, two common hallmarks of the condition, children’s spines lack strength to support their growing bodies.

Repairing Nicholas’ spine

Nicholas’ physical development was a series of fits and starts. When he was young, orthopedic surgeon Dr. Seymour Zimbler told Lisa and Jeff he would need spinal fusion surgery at some point, probably in his teens. Nicholas wore a brace for many years but as he matured, his spine became increasingly disfigured.

Nicholas, who had spinal problems as a result of Prader-Willi syndrome, stands in one photo with one shoulder higher than the other. In another, he sits on a stool with a visible hunchback.
As Nicholas’ scoliosis grew more pronounced, one of his shoulders inched up higher than the other. As his kyphosis progressed, he developed a hunchback.

By the time he was 16, Nicholas was noticeably bent over, especially when he sat down. When he stood up, one of his shoulders was higher than the other. “It was clear he was in pain,” recalls Lisa. Eventually his curvature became so significant his lungs didn’t have room to take full breaths. His doctors and parents agreed, the time for surgery had come.

The surgery would involve fusing 15 of the vertebrae in Nicholas’ spine. Dr. Glotzbecker would realign the vertebrae by attaching two rods with screws to either side of the spine. Over time, the vertebrae would grow into a solid bone, providing the stability Nicholas needed to sit and stand upright.

Preparing for the OMG factor

Over the years, Lisa and Jeff have relied on humor to get them through the ups and downs of their son’s care. “The OMG factor” has become shorthand for the unpredictability of his health. “With Nick, there’s always that one thing from left field that no one expects,” explains Lisa. He might develop a mysterious symptom overnight or an offhand comment might trigger an emotional outburst. “You just have to be ready to deal with it.”

With the OMG factor in mind, as well as the complexity of the surgery, Dr. Glotzbecker assembled a diverse team to plan ahead for every possible aspect of Nicholas’ hospital experience. A behaviorist compiled a list of things that could cause him unnecessary stress. Well before the surgery, all of his caregivers received a copy, which included warnings like not telling Nicholas he had to wear a johnnie, not using the word “IV,” and making sure to have popsicles and jello ready when he came out of surgery.

A series of three photos of Nicholas, who had spinal fusion surgery, with his clinicians at Boston Children's Hospital.
Nicholas with his caregivers. From left Michael Felber, Dr. Glotzbecker and Dr. Mohammad.

A relaxing start

Because they knew Nicholas would be nervous going into surgery, the pre-operative team led him and his parents to a quiet room away from the other patients in the surgical waiting area. Soon, anesthesiologist, Dr. Shoaib Mohammad, entered the room and crouched down next to Nicholas. As Lisa and Jeff watched in silence, Dr. Mohammad held Nicholas’ hand and spoke softly to him about what was going to happen next. Nicholas drifted off. “Dr. Mohammad lulled him to sleep before giving him any medication — we couldn’t believe it,” says Lisa.

The surgery seemed to go well and Nicholas was in a room on the orthopedic unit by the next day. Here, the family met nurse Michael Felber who quickly became one of Nicholas’ favorite people at the hospital. “He knew exactly how to talk to Nick to keep him calm and relaxed,” says Lisa. The family nicknamed Felber “Mike the nurse.”

Nicholas, who had spinal fusion surgery, takes a tentative step while his father steadies him from behind and a physical therapist supports his hands.
Nicholas with his father and physical therapist Maggie Anzalone after his second surgery.

The Peters were looking forward to going home, but a few days after the surgery, Nicholas gained an alarming amount of water weight. “His urine was the color of Coca-Cola,” says Lisa. Dr. Jane O’Brien from the Complex Care Service got involved, overseeing Nicholas’ treatment for this unexpected complication and ensuring his kidneys remained healthy. In total, Nicholas remained in the hospital for nine days but he came out walking tall. “He looked good, he felt good and he was not in pain.”

An unexpected setback

A month later, Nicholas’ posture had improved remarkably, but the pain had returned. At a follow-up appointment, Dr. Glotzbecker showed Lisa and Jeff an x-ray in which it was clear that two of the screws in Nicholas’ spine were coming loose. He would need another surgery.

For the second surgery, Dr. Glotzbecker would fuse two more vertebrae to further stabilize his spine. But first Nicholas needed time to recover, his parents needed time to break the news to him, and the clinical team needed to figure out what went wrong. “That’s when they started asking us, ‘how can we improve?’” says Lisa. She and Jeff suggested transferring Nicholas to the general floor earlier in the day and giving him more time before removing his breathing tube.

In addition, the team made sure Nicholas would be surrounded by caregivers he knew and liked when he returned to the hospital. This included Felber, Dr. Glotzbecker and Dr. Mohammad.

Nicholas, who had spinal fusion surgery, waits with his father while his mother looks at radiological images of his spine.
Lisa and Dr. Craig Birch review an image of Nicholas’ spine. (Michael Goderre/Boston Children’s)

A second surgery and a superman scar

Nicholas had his second surgery on January 28. That morning, Dr. Mohammad once again crouched down next to him. This time he put his hand on Nicholas’ solar plexus, a gesture that helped the whole family relax. “Then Dr. Glotzbecker came in with his game face on,” says Lisa. “Seeing him, we felt confident he knew what he had to do and how to do it.”

Nicholas grew four inches after his first surgery. His pain improved after the second surgery. “The pain is gone,” says Lisa. “We are seeing the old Nick, the sweet, loving young man from years ago.” With trademark humor, the Peters have named Nick’s scars. “The first scar was his ‘Man Scar.’ The second one is his ‘Superman Scar.’”

Heroes helping heroes

As they watched their son’s mobility improve and his sweet personality reemerge, Lisa and Jeff decided they wanted to recognize the team that cared for him. To this end, they created their own award, named the Nicholas Peters Guardian Angel Award, for Felber and Drs. Glotzbecker, Mohammad and O’Brien. Each plaque is engraved “Heroes Helping Heroes,” and features a photo of Nicholas and the caregiver.

Nicholas, who had spinal fusion surgery, stands posing with his surgical team.
From left, Dr. Glotzbecker, Nicholas, Jeff and Lisa Peters, physician assistant Joe Tremmel and Dr. Birch (Michael Goderre/Boston Children’s)

“We cannot thank you enough,” said Lisa as the family presented Dr. Glotzbecker his award. Nicholas held the plaque and his father kissed his head as his mother continued, “For us, as parents, it was awful to have our child go through surgery. But you didn’t just use your heads, you used your hearts. Seeing all of you working together cooperatively as a team was so amazing, it was almost a spiritual experience.”

Learn more about the Spinal Program.