Dominico "Nico" Bastolla initially seemed like such an unusually happy baby that his family nicknamed him "Capt. Giggles."
His laughs were not the sound of joy, however. They were the earliest symptoms of a rare brain tumor affecting only about one in a million people.
This month, Nico, nearly 8 months old, will become the youngest person in the United States to undergo a relatively new surgery to remove the noncancerous tumor, called hypothalamic hamartoma, in the base of his brain.
He and his parents, Rhonda Lunz-Ramsey, 41, and Michael Bastolla, 35, of West Homestead, are going to Barrow Neurologic Institute in Phoenix, the only place in the nation that performs the procedure.
Nico's giggles actually are gelastic or laugh-like seizures, commonly dismissed by parents and pediatricians as signs of a happy baby.
Nico's laughing jags became increasingly odd and prolonged. His fits of laughter -- progressing by the time he was 3 1/2 months old to belly laughter not normal in babies -- were mixed with crying and extreme fussiness that lasted up to 10 hours.
The laughing seemed happy at first, his father said. "About four hours later, he would have this pained look in his eyes like 'Please, help me.' He couldn't stop himself," Bastolla said.
His parents could not help him, either. They held him close, rocked him and did everything they could to comfort him, but nothing stopped the giggling or fussiness.
Nico initially was misdiagnosed with colic, then acid reflux. Lunz-Ramsey, a pediatric nurse, felt from the time Nico was born Dec. 12 that something was wrong with the couple's third child.
"I kept telling them, 'It has to be something more than this.' It was just a feeling," she said.
Discovering the tumor
Lunz-Ramsey took Nico to several doctors and pushed them to keep looking into what was wrong with her too-thin baby, who weighed 7 pounds, 11 ounces at birth.
He didn't stop eating, but had gained less than 2 pounds in three months.
"He was eating nonstop," said his mother. "I couldn't keep up (with breast-feeding), so I started to supplement, and still he wasn't gaining weight."
His thin body looked emaciated. His ribs protruded, his hair was sparse and patchy, his eyes bulged and his head looked too big for his body. Eczema covered his body, and he scratched at it incessantly.
His laughing and crying fits became so frequent that they totaled 18 hours on some days.
When the seizures stopped, he passed out from exhaustion, and their always-hungry baby would be too wiped out to eat, Lunz-Ramsey said.
Her persistence, coupled with Nico's failure to gain weight even though his mother carefully documented how much he was eating, led doctors in April to order an MRI.
"He was just belly-laughing. He was laughing so hard," recalled Dr. Sylvia Choi, a pediatrician at Children's Hospital of Pittsburgh Diagnostic Referral Service, of the first time she saw Nico, then 3 1/2 months old. "... At his age, it was so abnormal. It was disturbing to see."
A tumor slightly larger than 1 centimeter in diameter was discovered on his hypothalamus, a tiny endocrine organ at the base of the brain that controls body temperature and regulates hormones, some metabolic processes, nervous system functions and reflexes.
Doctors at Children's said they would not operate, Lunz-Ramsey said, but offered to treat him with medication.
"Usually, the younger a child is when they start to develop seizures, the even harder they are to control," Choi said.
Nico was unable to gain weight because his seizures were burning so many calories, Choi said.
The seizures would prevent Nico from developing normally both physically and mentally, she said.
A ray of hope
Young patients with hypothalamic hamartoma, commonly called HH, often go through puberty at an early age, suffer attacks of rage and can become mentally retarded.
Nico's prognosis appeared poor. Bastolla and Lunz-Ramsey were stunned. Lunz-Ramsey questioned doctors about their options, medications and prognosis. Bastolla, an insurance agent, researched the rare disease on the Internet and found hope.
"I'm just an overly optimistic person, and I thought, 'OK, there's something that can take care of this, so let's find it,'" he said.
"At first, it was dark and gloomy and depressing," Bastolla said. What he read led him to believe his son would be institutionalized by age 10.
Then doctors at Children's and support groups pointed them to the Barrow Neurologic Institute in Phoenix, "and the outlook just got 200 percent brighter," he said.
Dr. Jeffrey Rosenfeld, an Australian neurosurgeon, developed a surgical treatment for the disease in 2001 and trained surgeons at Barrow. Since then, 43 surgeries have been performed at Barrow on patients between 2 and 55 years old, said Dr. Yu-tze Ng, a pediatric neurologist specializing in epilepsy.
All the patients have survived and shown significant improvement, he said. The seizures stopped completely in more than 75 percent of the patients, he said.
Lunz-Ramsey said Barrow doctors initially told her that Nico would have to be 24 to 30 months old before they could perform the operation so that his skull would be larger. She dreaded the wait.
Dr. Harold Rekate, the Barrow surgeon who will operate on Nico, modified the surgical technique in recent surgeries, using an endoscope that enables surgeons to operate on smaller skulls, Ng said.
The right combination
Barrow doctors believe the earlier the surgery can be done, the better.
Surgeons go in the top of the skull, slightly separating the brain's two hemispheres to remove the tumor at the base of the brain, Ng said.
Lunz-Ramsey was told Nico could have the operation as soon as he gained weight.
At 6 months old, Nico weighed just 9.5 pounds -- the amount his older brother, Antonio "Tio," weighed at birth.
After feeding him hourly failed to do anything but keep the family awake endlessly, a feeding tube was put into Nico's stomach and intestines two months ago.
Doctors prescribed various anti-seizure medications for Nico, eventually finding a combination that has significantly reduced both the duration and frequency.
After hourly feedings of high-calorie food and fewer seizures burning up calories, Nico now weighs 15 pounds, 10 ounces -- a miracle to his mother. He now can sit up on his own and crawl.
Nico's surgery is scheduled for later this month.
"This still is very risky," Choi said. "... We all hope and pray that he is going to do very well, but, obviously, brain surgery can have complications."
