Annual Poker Run to Benefit Infant With Rare Spinal Disease
Three-month-old Stephen Pierce, who has Spinal Muscular Atrophy, will be supported by the 10th running of the Friends of Christian Poker Run and Pig Roast.
(Pictured above: 2022 Friends of Christian recipient three-month-old Stephen Pierce with father Sean Pearce, mother Amanda Wentling, and siblings Jaxson and Hunter.)
When Amanda Wentling and Sean Pearce welcomed Stevie into the world, they had no idea that they were about to learn about a genetic disease that few people had even heard of. Stevie has Spinal Muscular Atrophy (SMA), which comes into play when both parents have the gene for it. His older half-brothers Hunter and Jaxson, do not have the disease. For Stevie’s mother, it was a shock.
“I didn’t even know what SMA was until the doctor said that he had it,” Wentling said. “My first thought as a mom was, ‘Is he going to live?’ The more I looked into it – I’m a Google fanatic – it didn’t look good. The neurologist said that the treatment should help, though.”
The treatment is a gene replacement therapy, which will happen before the end of July.
According to Children’s Hospital of Philadelphia, SMA is a genetic disease that affects the spinal cord and nerves. This causes muscles to deteriorate and become weak. If left untreated, it continues to worsen and may result in death. Many of the treatments for SMA put in a copy of a gene to replace the missing or nonworking genes that direct motor neurons’ activities. In this case, the new gene tells the neurons to produce a protein that is necessary for the cells to survive but would not be produced due to SMA.
“It’s just a one-time gene replacement therapy,” Wentling said. “They described it as a blood transfusion. They take out what they need from one IV and put in what they need through another. He’ll be on steroids for a few months and head down to Pittsburgh for a few months for monitoring.”
The cost for the treatment is $2.1 million. Most insurances cover the initial treatment, but other steps in his treatment are not covered.
“We’ve been fighting with insurance, finding out that they cover the treatments but not any of the steroids and other appointments,” Wentling said. “Our good friends, Cayla Silvis and Garry Myers, said that their friend puts on a benefit every year, and asked if Stevie could be nominated. I didn’t expect anything from it. I was surprised.”
Because insurance was covering the $2.1 million, Wentling tried to get Friends of Christian to consider someone else, but she said that Stevie had won their hearts.
“They said they couldn’t stop talking about Stevie,” Wentling said. “I’m just thankful for all the support everyone’s giving us, and that everything keeps going well for Stevie.”
Wentling has nothing but positive things to say for Children’s Hospital in Pittsburgh.
“They have helped us out a lot,” she said. “The nurse from neurology is amazing. He keeps me from stressing out.”
Friends of Christian’s Rodney Beck said that the process of choosing a recipient this year was difficult, but Stevie stood out to the committee.
“His diagnosis and prognosis moving forward set him apart,” Beck said. “With the type of disease he has, there are many, many different costly medications, hospital and doctor visits, and general expenses that will be a daily fact of life for Stevie and his family.”
As Friends of Christian moves forward after a decade of helping others, Beck said that there are some similarities among the recipients. Many of those were present with the current family.
“There are lots of similarities,” Beck said. “First, the love and caring they have for Stevie – their love is unconditional and without any limits. They would do anything they can to help him and care for him. Secondly, the frustration and realities of their situation. They must humble themselves daily to allow a myriad of professionals into their lives who are capable of diagnosing and caring for Stevie’s needs. This is incredibly invasive and goes against what most parents want for their child.
“Most parents want to be the sole provider for their child. Children with these types of diseases make that impossible. Lastly, the willingness to be there for our organization. Being in the situation that has been thrust upon them makes them very empathetic to other families with similar issues. When we interviewed the family, they were already talking about helping our organization in the future, regardless of whether we chose them.”
However, Beck added that each family’s experience is unique, so that is why when making these decisions, Friends of Christian keeps an open mind.
“I do not believe we have had any families who set of circumstances were exactly the same,” he said. “We start out each search with no limitations or expectations. We are looking for the child and family who need our help and have the greatest need within FOC’s service area. This has enabled FOC to help a diverse group of families, and that diversity is expanding. I do feel that is one of FOC’s greatest strengths, and it enables us to look for the family who needs the most help.”
The Friends of Christian organization keeps in touch with previous recipients. Beck said that they have been able to help them keep up with bills and give support and rest when needed.
“A lot of families have given feedback to indicate they were near the edge of what they could handle financially and mentally before getting our help,” he said. “These families had the chance to recover enough to be there for their children and have been successful in supporting their children until this day. They also had their faith in humanity restored and learned there are people who care for more than just their own needs.”
The Friends of Christian ride will be on Sunday, July 24, at the Toby Hill Bar and Grill at 11:00 a.m. The event at the Limestone Fire Hall at 4:00 p.m. will be family-friendly. More information can be found at the Friends of Christian Facebook page.
This is Part Two of the tenth running of the Friends of Christian Poker Run and Pig Roast. Part One can be found here, including information on previous events and about Christian Burgdofer, for whom the group is named.
Copyright © 2024 EYT Media Group, Inc. All rights reserved. Any copying, redistribution or retransmission of the contents of this service without the express written consent of EYT Media Group, Inc. is expressly prohibited.