Community goes to bat for Holden

Saturday, June 21, 2008
Three-year-old Holden Guilfoyle, Fort Scott, takes a swing at a foam baseball in the backyard of his parents' Fort Scott home. Holden suffers from type-six mucopolysaccharidosis (MPS), a rare disease that requires him to undergo four-hour enzyme infusions each week. The disease has not stopped the youngster from enjoying some of his favorite activities, including baseball, swimming and playing with toys. --Jason E. Silvers/Herald-Tribune

Fort Scott, Kan. -- Holden Guilfoyle has not let a very rare disease keep him from living and enjoying his young life.

"He loves baseball, and he's basically into anything related to sports right now," Holden's mother, Jenny Guilfoyle, said. "He's really obsessed with T-ball right now because his big sister is playing."

Holden, who will turn 4 years old later this year, is not like most children his age. Two years ago, he was diagnosed with type-six mucopolysaccharidosis (MPS), also known as Maroteaux-Lamy Syndrome, a disease that affects only 50 people in the United States and 1,100 people worldwide. There is no known cure for the disease, which requires the patient to receive weekly enzyme infusions, the first of which Holden underwent in late 2006.

Despite the effects that the rare metabolic disorder has had on the youngster's body since birth, Holden has managed to enjoy an active life thus far, and has since shown those closest to him the little boy behind the disease, Jenny said.

"He likes to swim, and drive his toy tractor," she said. "He's a normal kid, he just has to sit still for four hours once a week. He's accepted his treatments although he doesn't understand what it means yet."

Holden receives a weekly four-hour enzyme infusion that over the course of a month costs more than $30,000. The Guilfoyle's insurance has helped them deal somewhat with the cost of this weekly procedure and other medical costs associated with the treatment of Holden's condition.

Some of the symptoms that Holden showed before being diagnosed with MPS, which led his parents to believe he might have the disease, include a double ear infection, respiratory infections, colds, strep throat, runny noses, pneumonia, and other ailments.

Holden's body has not been able to properly break down cells, causing problems with his spine and his growth. Holden's adenoids were removed twice by the time he was 2 years old, and there is a slight curvature at the base of his spine, Jenny said.

But doctors have told Jenny and her husband, Tim, that Holden is making progress because the disease was caught early and Holden continues to grow gradually and learn, and is responding well to treatments.

Holden also receives a physical each year so doctors can monitor his progress.

"He now has perfect hearing, and he's had some vision problems and corneal clotting, but he's doing better," Jenny said. "His heart is strong, his airway is narrower, and his spine has stayed about the same."

Type-six MPS is the only type of the disease that does not affect the child's intellect or lead to death, and is one of only three types of the disease that is treatable by enzyme infusions.

"Because it was caught in time, Holden has a great outcome, and it (the disease) is not a death sentence," Jenny said.

In 2006, after several other specialists and physicians were unable to determine what was wrong with Holden's health, Nigel Price, a pediatrician in Kansas City, evaluated Holden and eventually came to the conclusion that Holden had some type of MPS.

Holden lacked an enzyme that breaks down cells, which then begin to store in organs, skin and tissue.

A urine test in the fall of 2006 confirmed that Holden had MPS, and a blood test at the time showed that Holden's liver and spleen had become enlarged.

"He's (Price) a hero in our eyes," Jenny said.

The Guilfoyles, who did not know MPS existed before their son began developing symptoms of the disease, were not shocked by the news because they had researched many of Holden's symptoms before his diagnosis and tried to prepare themselves for the reality that their son may have the disease, Jenny said.

But they did not know which type of MPS that Holden had until Price told them following the results of a blood test, she said.

"After I looked at the Web site, I knew he had MPS, because right there was a list of 10 things, and my son had nine of them," she said. "There was no denial phase for us."

The Guilfoyles have created a Website for Holden, www.holdenshope.com, and an annual Holden's Hope Day fundraiser to spread awareness about MPS.

They have also created a support network for families affected by the disease, and continue to raise money for MPS research and to educate people about the disease.

This year, Holden's Hope Day will take place from 11 a.m to 4 p.m. Saturday, June 28, at Lincoln Park in Pittsburg, Kan.

The event will include a variety of games, activities and food for families to enjoy. Wristbands for the event cost $4 each.

Last year's event drew between almost 200 people and raised about $2,700.