Sean Kisielnicki’s life is ruled by Hunter syndrome. This rare condition not only affects him, but takes a toll on the entire family.
The morning routine is always the same. The kids wake up early to get started on the farm’s chores. Their youngest of the pack Sean, stays behind most of the time. His mother Trish, goes to check on him. She finds him very still and fears that he’s not breathing, that today is the day.
When she feels his chest, she is given a wave of relief and is assured that today is not that day. It’s a routine that she dreads.
“Some of the boys just die in their sleep. They can be just like he is now, and you go up in their bedroom and they’re gone,” she said. “There’s been several this year. Mom goes up in the morning, and they’re dead. … And these are kids who are functioning like him.
Sean’s case of Hunter syndrome is a genetic disorder that affects several areas of the body and primarily occurs in males. People who suffer from severe cases can deteriorate very quickly. It affects only 400 to 500 people in the United States. To learn more, click here.
According to York Daily Record, Sean has the rarest and most severe form of the disease that affects both the body and mind. His enzyme deficiency knocks down the body’s ability to break down sugar chains. This leads to clogging, stunted growth and organ damage.
The Hunter syndrome has relentlessly thrown challenges in Sean’s way, including autism, attention deficit hyperactivity disorder and sensory processing disorder. In some cases he can’t feel a thing, like the time he touched a gas burner flame and fell down the stairs without any pain.
Thanks to enzyme replacement therapies, Sean’s condition is stable and he’s even seeing some progress. Most days, Sean is a bundle of energy on his eight acre farm in Hopewell Township, New Jersey.
In total, it’s six home schooled kids living in their secluded ecosystem in the country. There is never a dull moment and rarely any silence, especially with their clan of cousins that come to visit often.
Sean’s features have been softened because of IV therapies and he’s gradually broken out of what used to be a shell. Now he’s excited to play with his siblings and interact with everyone he comes into contact with.
He’s taken on ice hockey with the beginner’s youth disabled team, the New York Polar Bears. With them, he’s perfected his balance and coordination. He also goes to Leg Up Farm, where he undergoes physical and occupational therapy.
“He’s our ticket to heaven,” said Karen Shriner, Brian’s mother. “If we can be patient with him, if we can love him …”