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Holden's Hope
Four-year-old Holden Guilfoyle, who suffers from a rare genetic disease, has been doing well lately.
"He's fantastic," Holden's mother, Jenny Guilfoyle, said during a Thursday interview with the Tribune. "He's playing tee ball now. He had surgery just a month ago, and now he's out there swinging a baseball bat. The doctor said he has full range of his hands now. He just needs to take time to build his strength and grip back up."
At the age of 2, Holden was diagnosed with type-six mucopolysaccharidosis (MPS), also known as Maroteaux-Lamy Syndrome, a metabolic disorder caused by the absence of enzymes that break down complex sugars called glycosaminoglycan (GAG). Because of the disorder, Holden's body has not been able to properly break down cells, causing problems with his spine and growth.
There is currently no known cure for the disease, which affects only 50 people in the United States and 1,100 people worldwide. Type-six MPS is the only type of the disease that is not fatal or affects the child's intellect. It is also only one of three types of the disease that is treatable by enzyme infusions. Without the enzyme infusions, the GAG would build up in Holden's system eventually causing organ failure and death.
Holden started preschool last fall, and last month underwent bilateral carpal tunnel surgery on both of his hands to regenerate nerves that had been damaged due to MPS.
His hands have since healed, and Holden is doing his best to live the life of a normal four-year-old boy, although many aspects of his life are not normal for a youngster his age. Since late 2006, Holden has received weekly, four-hour enzyme infusions to help control and treat the illness -- a procedure he will most likely endure for the rest of his life.
"He's had infusions for three years and he's never missed a week," Jenny said.
Jenny added that Holden is slowly beginning to understand how he is different from most other children his age.
"He doesn't understand the term 'disease,'" she said. "He understands he needs his medicine. He knows about his Hope Day because he wants people to come and see him. He does know that he's different. He knows that he goes to the doctor a lot."
Jenny and her husband, Tim, have created a support network for families affected by MPS, and continue to raise money for MPS research and to educate people about the disease. They also organize the annual Holden's Hope Day fundraiser, an event in which friends, family members and other local residents turn out to enjoy food, games and other activities, to pay tribute to Holden, and to also raise awareness about MPS. Some of the funds are also used to help with Holden's medical expenses.
"Our goal is to eventually send money to the MPS Society for research and to hopefully find a cure," Jenny said.
The Guilfoyles pay for Holden's expensive infusions -- a month's worth of treatments cost more than $30,000 -- through Tim's insurance, so the couple only has to pay a $40 co-pay each month to have the procedure done, but they also pay small co-pays for numerous doctors that Holden has to see each year for check-ups on various parts of his body. Without Tim's insurance, the Guilfoyles would be faced with paying between $800,000 and $1 million each year for Holden to get the infusions, Jenny said.
Holden had various ailments before being diagnosed with MPS, which led the Guilfoyles to believe he might have the disease. His adenoids were removed twice by the time he was 2 years old, and there is a slight curvature at the base of his spine.
Holden has responded well to treatments and has shown improvements since being diagnosed with MPS, Jenny said.
"His disease does not affect his brain, so it should never affect his cognitive abilities," she said. "His spine has stayed the same and his heart has improved since he started (infusions)... Even though our son has a disease, he can live with it and it's manageable."
For more information about Holden or Holden's Hope Day, which is scheduled to take place from 11 a.m. to 5 p.m. Saturday, June 20, at the Bourbon County Fairgrounds, visit www.holdenshope.com. Wristbands for the event cost $5 each. Between $1,700 and $1,800 was raised during last year's event.
Flyers promoting Holden's Hope Day were sent home with children in Fort Scott elementary schools and Holden's preschool on Thursday, Friday is National MPS Awareness Day.