For 15 years, Josh Bigham lived without the typical teenage luxuries – pizza, burgers and the like – because his body could not properly process proteins found in meats, dairy products, aspartame and some vegetables. Not any more.
Josh Bigham was saved, not by fire and brimstone, but by flamebroiled cheeseburgers and Coldstone Creamery.
He’s found heaven in the sugary goodness of rocky road ice cream, the hickory-smoked flavor of a perfectly grilled steak and the well-balanced blend of a sweet and spicy barbecue sauce.
For 15 years, Josh, a Canal Fulton, Ohio, resident, lived without the typical teenage luxuries – pizza, burgers and the like – because his body could not properly process proteins found in meats, dairy products, aspartame and some vegetables.
Not any more.
Through Akron Children’s Hospital two years ago, Josh Bigham, now 17, enrolled in a clinical study for Kuvan, a drug developed by BioMarin Pharmaceuticals Inc. that helps him properly digest the proteins he eats.
“Now,” he said with a broad grin, “I can eat anything I want.”
“The medication,” Josh’s mother, Marsha Bigham, added, “has transformed his life.”
As an infant, Josh Bigham was diagnosed with PKU – phenylketonuria – a metabolic disorder. Simply, Josh’s body doesn’t produce enough of a specific enzyme needed to break down protein into substances his body can use. One component of protein – phenylalanine – could build up in his blood stream as a result.
Build-up of phenylalanine could cause brain damage or developmental retardation in children as young as three months.
“It’s only one of 20 components of the protein,” Marsha Bigham said, “but it’s the most-potent and, to Josh, it’s the most dangerous.”
Approximately one in every 14,000 babies are diagnosed each year with PKU – and testing for the disorder is done as part of routine newborn screenings. The condition can be monitored with a strict diet and now, with medication.
But it comes at a cost.
“That, there,” Josh Bigham says pointing to the cluster of 12 pills in the palm of his hand, “that’s two grand right there.”
OK, he admits with a mischievous grin, it doesn’t cost that much for the medication. But it is expensive. The 12 pills he takes every day cost $29 each.
“Which would you rather have, $360 or this, right here?” Josh Bigham asks before cupping his hand, leaning his head back and tossing the handful of medication into his mouth.
“It’s like that commercial,” Marsha Bigham said. “To you, those pills are priceless.”
Marsha Bigham shakes her head and says she can’t imagine not having access to the medication her son needs. Her insurance plan – one she says they couldn’t live without – helps to pay for the medication now that the clinical study is complete.
“Three hundred and sixty dollars a day?” Marsha Bigham said. “I don’t care who you are – no one can afford that.”
Before the freedom to snack as he pleased, Josh Bigham said he had to stick to a strict diet – one that would be hard for anyone to follow. It’s surprising where phenylalanine is found. It’s in things like milk, eggs, cheeses, meats and fish, but it’s also found in aspartame – artificial sweeteners used in a variety of foods including diet sodas, teas and even sugar-free chewing gums.
“Josh used to chew Juicy Fruit all the time, but Wrigley changed things,” Marsha Bigham said. “They added phenylalanine so the flavor could last longer. He can’t chew it now. Think about it. Something as simple as opening a pack of gum, we take that for granted, but he can’t chew gum.”
Josh Bigham’s diet prevented him from spending time with his friends because he was embarrassed to tell them he couldn’t share their snacks or stay for dinner.
“It was hard,” he said. “I’d go over to a friend’s house and they would order pizza – cheese pizza – and I couldn’t eat it.”
Instead, he would decline the treats they offered, saying he wasn’t hungry. Occasionally, he might eat a small slice of pizza and say he was full.
“I had to eat only something my mom made for me,” Josh Bigham said. “(The medication) let me eat anything I want and that has helped me socially.”
Diet aside, nothing about Josh Bigham would indicate that he isn’t a healthy, normal teenager. He loves mechanics and hopes to make a career out of repairing cars. He spends as much time as he can tinkering with cars and helping at his father’s auto shop – Paul’s Car Care in Manchester, Ohio.
He lives for off-roading and playing basketball with his friends and “up until they lost” Josh was a big a Cavs fan, following each game closely.
Now, Akron Children’s Hospital wants Josh Bigham on their team.
Spreading the word
Josh Bigham and his mother will take their story all the way to Capitol Hill, giving voice to children across the nation who live with PKU, but also those who live medical disorders that often fly under the radar.
Their story, they hope, will help to shape health care reform for children.
“When we go as lobbyists we are telling someone else’s story but when families go they are relating their experience first-hand,” said Linda Emore, Akron Children’s Director of Child Advocacy and Government Relations. “Josh is really a perfect example because he can speak for himself and he can speak for children.
“Kids can’t speak up for themselves so they need adults to speak for them,” she added. “I think they get lost in the health care information that is out there because of that.”
Emore believes that issues such as Medicare – though critical – get so much attention because so many voices clamor for the cause. When it comes to kids, the voices get lost in the noise.
“Parents can only do so much or may not have the wherewithal to know where to go or what to do, so children don’t have a unified voice,” Emore said. “Kids need access to health care and they need to be involved in clinical trials and they need the right amount of education about their conditions.”
While in Washington, Josh Bigham, his mother and Emore will have the chance to meet with 10 legislators from Ohio or their representatives. He has scheduled meetings with Sens. Sherrod Brown and George Voinovich. He also plans to meet with Reps. John Boccieri, Betty Sutton, Zack Space, Bob Latta, Jim Jordan, Steven LaTourette, Charlie Wilson and Tim Ryan.
“If we can help to educate them about this rare disease and the importance of health care for kids,” Marsha Bigham said, “that’s all we want. I just want to get that across.”