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Entries in Epidermolysis Bullosa (2)

Sunday
Jan152012

Louisiana Toddler Who Battled Disease Dies in Mother's Arms

ABC News(PONCHATOULA, La.) -- The tiny toddler whose struggle against a devastating disease inspired thousands has died.

Tripp Roth, who was diagnosed with junctional epidermolysis bullosa at birth, died Saturday in the arms of his mother, Courtney Roth, who had chronicled her life with Tripp in the popular blog, "EBing a Mommy."

"He was exactly 2 years and 8 months old," Roth wrote. "It happened within minutes of me picking him up out of bed and rocking him. He took his last peaceful breaths in my arms, in his most favorite spot. My heart literally hurts more than I ever thought was possible."

Despite her grief, the Ponchatoula, La., mom asked readers to be grateful that her son is now at peace.

"I know he's flying high, pain free, and talking Jesus' ear off," she wrote.

In a recent interview, Roth said Tripp was "absolutely gorgeous" when he was born, with just a small blister on his head, a few on his back and deformed fingernails. Doctors diagnosed him immediately with epidermolysis bullosa, a genetic disorder that affects 1 in 50,000. He had the most severe form of EB, junctional epidermolysis bullosa, and was missing a protein the binds one layer of skin to another.

Though in early pictures Tripp appeared no different from any other newborn, eventually blisters would cover much of his body and his health deteriorated in other ways. He had to be fitted with a gastronomy tube and then a tracheotomy tube. He endured multiple eye surgeries but, despite the surgeries, Tripp lost his sight after multiple corneal abrasions and tissue growth that ultimately fused his eyelids shut.

There was no effective treatment for Tripp other than bandages and antibiotics to avoid trauma and infections. Roth, a 26-year old divorcee, gave up her nursing career to care for her son with the help of her mother, Anita Hotard.

"We were given a little angel and we have to take care of him," Hotard, 51, said last month. "If he can do it, I can do it ... I don't have near the agony and stress he has."

In a blog post last month, Roth wrote that his condition had grown so serious that his days consisted solely of being in bed, then being held in a rocking chair and then going back to bed.

It wasn't always that way.

"Before he was confined to the rocker, he would play every day, he could drum to the tune of a song like NO OTHER. He would smile, laugh, and melt your heart," Roth wrote.

Tripp, she said, astounded his doctors.

"He is just amazing. I have always said that from the beginning," Roth told ABCNews.com last month. "I have never been sad around him and I try not to cry around him. We've made it to where he is in the happiest environment possible. His spirit and personality are what keep me going."

Roth said she was inspired by her son's strength -- and so were many others.

As of last month, Roth's blog, which Roth began three months after Tripp was born, had nearly 2,000 subscribers and 3 million page views. This past summer, hundreds attended a prayer vigil for Tripp in Louisiana and last month, dozens wrote in to ABCNews.com asking that Roth and Tripp be named one of the site's "Heroes of 2011."

Copyright 2012 ABC News Radio

Monday
Dec192011

Epidermolysis Bullosa: 'Worst Disease You Never Heard Of'

Courtesy Christie Zink(PONCHATOULA, La.) -- The pain never ends for Tripp Roth, a 2-year-old boy who has outlived all medical predictions with a fatal disease called epidermolysis bullosa that has taken his sight and speech, and scarred his body from the inside out.

The tiny toddler, who has blisters on his face and hands, has not been outside for a full year.  He spends his life swaddled in bandages, being rocked in the arms of his mother, Courtney Roth, or his grandmothers.

Tripp breathes with the help of a trachea tube and requires sedatives so that his caregivers can bathe him, an excruciating process that takes three hours.

Infections and trips to the hospital are never-ending, and there is no cure for the disease.  The thousands of supporters who have rallied behind the family call it, "the worst disease you never heard of."

Born with epidermolysis bullosa, a genetic disorder that affects 1 in 50,000, Tripp is missing skin on much of his body, and what skin he does have is fragile and can easily erode.  He is missing a protein that binds one layer of skin to another.

The disease is a spectrum of inherited skin disorders, whose hallmark is a blistering response to minor injuries, heat or even rubbing or scratching.  It affects not only the skin, but anywhere in the body where tissues are held together more firmly: the eyelids, the upper esophagus, inside of the mouth and the genital-rectal area.

There is no effective treatment, other than bandages and antibiotics to avoid trauma and infections.

But Tripp's mother, who gave up a nursing career after Tripp's birth, said she derives inspiration from the remarkable little boy, who just won't stop fighting.  Initially, doctors said he wouldn't live beyond a year.

"He certainly astounded his doctors," said Roth, a 26-year old divorcee from Ponchatoula, La.

"He is just amazing.  I have always said that from the beginning," she said.  "I have never been sad around him and I try not to cry around him.  We've made it to where he is in the happiest environment possible.  His spirit and personality are what keep me going."

Copyright 2011 ABC News Radio







ABC News Radio