A Pennsylvania teenager with a rare rapid-aging disease died on Christmas Eve at age 14.
Josiah Viera had Hutchinson-Gilford Progeria Syndrome, also known as rapid-aging disease. It affects just one in four million newborns worldwide.
“They develop a characteristic facial appearance including prominent eyes, a thin nose with a beaked tip, thin lips, a small chin, and protruding ears. Hutchinson-Gilford progeria syndrome also causes hair loss (alopecia), aged-looking skin, joint abnormalities, and a loss of fat under the skin (subcutaneous fat).”
The condition does not affect intellectual development or the development of motor skills such as sitting, standing, and walking.
The syndrome is caused by mutations in the LMNA gene and almost always occurs in people with no history of the disorder in the family.
An avid baseball fan, Viera was the honorary bench coach for the State College Spikes, a Class A short-season affiliate for the St. Louis Cardinals.
A book about Viera’s life was written by former St. Louis Cardinals player Jake Gronsky and Viera’s grandfather Dave Bohner.
The book, “A Short Season,” is sold at the Threading Love store in Lewisburg, where employees were mourning the loss of Viera.
“Christmas morning I woke up and I realized he got the best Christmas gift of ever. He’s home with Jesus where he belongs,” store manager Jody Gates told WNEP. “You would meet him and you'd know his story of how much pain and struggle he’s gone through but all you would see is this smile.”
She said the book sold out shortly before Viera passed away and that the book is always popular.
“We‘ll get an order in and it’ll be off the shelf very, very quickly. It’s just great to see how much support he has not even in a town where he lived,” sales associate Abigail Carney said.
It said that besides baseball, he enjoyed cooking, dancing, and spending time with friends.