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.
“Hutchinson-Gilford progeria syndrome is a genetic condition characterized by the dramatic, rapid appearance of aging beginning in childhood. Affected children typically look normal at birth and in early infancy, but then grow more slowly than other children and do not gain weight at the expected rate (failure to thrive),” according to the National Library of Medicine.
“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.
“He was our friend, our coach, our teammate, and our inspiration. We were truly blessed to have him in our lives, and his spirit will live on with us always,” the Spikes said in a statement.
He was our friend, our coach, our teammate, and our inspiration. We were truly blessed to have him in our lives, and his spirit will live on with us always.https://t.co/SeqKqCzns8
— State College Spikes (@SCSpikes) December 25, 2018
Going to miss my friend. Thankful for the time I was able to spend with him. Our thoughts are with his grandfather, family, friends & our hearts are with Josiah!” added Penn State football coach James Franklin.
A book about Viera’s life was written by former St. Louis Cardinals player Jake Gronsky and Viera’s grandfather Dave Bohner.
“Everything I went through—good, bad, otherwise—it was all worth it,” Gronsky told State College earlier this year about spending time with Viera during rehab from an injury. “Meeting Josiah and telling his story was way better than ever making the big leagues.”
You gave me perspective I wouldn’t of found otherwise. You impacted more lives than you could ever imagine. Careful giving God suggestions on his line up…Love you bud! @josiahdviera pic.twitter.com/fUglwLIoVD
— Oliver Marmol (@OliMarmol) December 24, 2018
“He always has a different outlook on life, sometimes more than what we do,” Bohner told WNEP about his grandson.
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.
According to his obituary, Viera was an eighth-grade student at Tri-Valley High School and a member of St. Andrew’s United Methodist church in Valley View.
It said that besides baseball, he enjoyed cooking, dancing, and spending time with friends.
From NTD News