One family’s Christmas endures as a promise of remembrance
Joshua Frase was 15 years old when he passed away on Christmas Eve in 2010. On that Christmas Eve eleven years ago, as families the world over celebrated the birth of the Christ child, Joshua’s 9-year-old sister, Isabella, told her mother Alison as she wiped the tears from her eyes with her pajama sleeve, “This is the best Christmas ever for Joshie, because he can see Jesus.”
The son of Paul and Alison Rockett-Frase, Joshua was born on February 2, 1995 with a rare genetic neuromuscular disease called Myotubular Myopathy (MTM). He was not expected to live past the age of two, but despite difficulty in breathing, inability to walk or ingest food orally, Joshua earned a cumulative 4.3 GPA at Ponte Vedra High School, attending by being wheeled from class to class, his frail body stretched out on a gurney.
“Josh had the highest average in class,” recalled his 10th Grade Geometry Teacher Beth McBride. “His work ethic was something I wish I could bottle and pass out to all the students. Information just jumped into his brain … he and I talked about homework once. I told him he did not need to do all the problems because he had shown me that he knew the material and, guess what, he still did all of it by hand!”
Popular with his classmates as well as his teachers, Joshua Frase never complained and was known for his dry sense of humor, bright mind, deep faith, and positive attitude. His stated goal was to attend Wake Forest University and become a research scientist specializing in muscle gene therapy in hopes of finding a cure for his peers. The fact that Joshua could verbally communicate so well with others was due to the determination of his parents to avoid traching him (A tracheostomy -a tube placed in the windpipe to help one breath). Instead, they supported his every possible effort to breath on his own.
“Alison was determined that Josh would live as full a life as possible,” said Paul Frase. “She researched everything, became an expert at caregiving, and soon had the doctors working with her instead of trying to tell her what to do.” When they first brought Joshua home, Alison and her mother, Elsie, were the main caregivers, as Paul was away from home most of the first four years, as a professional NFL football player – being a lineman with the New York Jets, Jacksonville Jaguars, Green Bay Packers and Ravens. In 1996, Paul and Alison began using his National Football League and Wall Street connections to raise awareness and millions of dollars for cutting edge regenerative research through the Joshua Frase Foundation (www.joshuafrase.org).
Every waking hour was a challenge for the Frase family, but their deep faith in God and the constant love and support of family and friends brought cherished moments, too. Laughter abounded in the Frase household, especially after Joshua’s sister Isabella, was born. One day, Paul heard Joshua screaming ‘Help, Dad! Help!’” He ran to Joshua’s room only to find that 6-year-old Isabella had taken her mother’s make-up case and made up her brother’s face with eye-shadow and lipstick! Laughter reigned, also, as Joshua, the teenager, watched the television series, “The Office” regularly with his Dad. Josh would slap Paul’s leg and laugh hilariously. “Dad, Dad, Dwight is such an idiot!” he’d gasp, trying to catch his breath. “It’s painful!”
Struggling long hours daily just to keep her son alive, Alison Rockett-Frase also took on management of the foundation’s efforts and its website, which developed an international registry of hundreds of families. “When Joshua was born, there were only 50 known cases of MTM in the world,” Alison said, adding that she now collaborates with families and institutions world-wide, providing lifesaving care-guidelines, and encouragement.
By 2008, the Joshua Frase Foundation had raised more than $7 million toward MTM research, but Joshua’s health was declining rapidly. While there had been remarkable advances in gene transfer therapy in mice, the Federal Drug Administration (FDA) required that pre-clinical trials of genetic neuromuscular therapies had to be run on large animals before human trials could begin. A miracle had to happen soon if Joshua’s life was to be spared.
That miracle occurred in 2008 when Alison’s network of doctors led her to Dr. Elizabeth Snead, a Canadian Veterinarian. Together, Alison and Dr. Snead tracked down Nibs, a female Labrador Retriever who possibly carried the MTM gene. Nibs had two puppies that potentially displayed symptoms of MTM. It was a hunch and a ”knowing” Alison needed to pursue.
Alison immediately called Nibs’ owners, Vic and Karen Wagman of Saskatchewan Province in Canada. The Wagman’s responded to the desperate plea of a mother, and within days, met Alison at the Saskatchewan Airport where she picked up Nibs and delivered the beautiful chocolate lab to Dr. Casey Childers and his researchers at Wake Forest University.
On April 15, 2009, Nibs gave birth to twelve puppies, eight females and four males. Five of the females carried the MTM gene and one of the males was affected with the disorder like Joshua. Nibs’ progeny lives on, contributing to research and wagging their way into the hearts and homes of affected families. Dr. Childers credits Alison with launching the large animal studies. “Alison flew from Florida to Saskatchewan with an empty dog crate and came home with the founder of what is now a colony of dogs which has been strategic in solving the mysteries of MTM.”
While “the miracle of Nibs” and her contribution is now rescuing affected dozens of MTM patients in clinical trials, young Joshua Frase was not destined to be one of them. His memorial service was attended by nearly all of Ponte Vedra High School’s faculty and student-body.
Fast forward to September 19, 2017 when Audentes Therapeutics announced dosing the first patient in ASPIRO, a Phase ½ clinical trial of AT132 for treatment of X-Linked Myotubular Myopathy.
“After four years, that patient has shown marked improvement, as have several other patients,” said Alison, while she and Paul viewed video after video of MTM children standing and walking. Joshua’s legacy is about saving lives “If my life’s work was just for one … it’s been worth it all,” says Alison. “We fought for his life every day and prayed to God that a cure would be found to save our son. Now our Foundation is working to perfect the cure for all the Joshua’s of the world.”
You can invest in the lives of children by supporting their efforts by visiting www.pleasedonate.org.
By Susan Brandenberg
Resident Community News
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