“Cut ’em off, doc,” Nicklas Nelson, 9, says as he assembles a Statue of Liberty puzzle. Nick was born with popliteal pterygium syndrome, a rare condition in which webbing runs from behind the knee down to the heel, making it impossible to straighten his right leg. So, at the age of 9, he decided to have his leg amputated.
“It was quite a surprise,” Nick’s mother Greta Nelson says of his syndrome. When he was born, the syndrome wasn’t well known, and doctors had to look into many other anomalies to diagnose him.
Nick had around 15 procedures done by the time he was 1, the first of which was to repair his cleft lip when he was 11 weeks old. “I’ve probably had like 30 surgeries,” Nick says. “A couple of times there wasn’t anesthesia. I’m deathly afraid of [anesthesia].”
A hallmark of popliteal pterygium syndrome is orthopedic issues, Nelson says. These include a cleft lip and lip pits, which are essentially holes in the mouth.
As a “bonus defect,” as Greta Nelson refers to it, doctors discovered Nick’s congenital brain defect, called chiari malformation, when he was 9 months old. This means Nick’s cerebellum is too large for his skull, which causes problems with swallowing, poor eyesight, impaired movement and speech, and headaches.
At one point, Nick had nearly 30 pins in his leg as the doctors attempted to straighten it out. The membrane behind his knee, which kept his leg bent at a near 90-degree angle, couldn’t be removed because it contains nerves.
This lack of control over his own body kept Nick from doing the things he wanted to do. When Nick was 5, he learned of Rudy Garcia Tolson, a boy with the same crippling syndrome. Rudy’s legs were amputated, and now with his prosthetic legs he is able to swim and run.
With that, Nick decided he, too, wanted to amputate his leg so that he could run and play with his friends, Nelson says. On Dec. 14, 2007, Nick underwent the amputation procedure. “Just because they’re taking my leg off doesn’t mean I’m not Nick anymore,” Greta Nelson quotes her son saying before the surgery.
“He couldn’t climb on the playground or run around [before the amputation],” says Jamie Murphy, Nick’s teacher at Rosemount Elementary School. “Now he can do all of that.”
Nick’s favorite thing about his new leg is running. “All I want to do is run,” he says with a smile on his face. “There’s no pain anymore.” To that his mother adds, “And this is coming from a kid whose legs have always hurt him.”
With his new leg, Nick says he is mostly excited to learn how to golf. He puts down a puzzle piece, climbs off of his chair and shows off his golf swing. As for the future, Nick says he wants to be a surgeon, a lawyer, or an astronaut.
Dealing with popliteal pterygium syndrome has caused Nick a lot pain, but with his prosthetic leg and confidence to go with it, Nick is a normal 9-year-old boy with a bright personality. The decision to amputate his leg has enabled him to lead a life just like his friends, and he’s still the same Nick he’s always been, his mother says.
“He’s very down-to-earth and funny,” Murphy says. “He is a good friend to others.”
POPLITEAL PYERYGIUM SYNDROME
What is it?
A rare genetic disorder wherein a triangular membrane runs from behind the knee to the heel, disabling the ability to straighten the leg. Other symptoms include cleft lip, lip pits, and webbing of toes and fingers. It is classified as a rare disease, meaning it affects fewer than 200,000 people in the U.S. population.
