The word “believe” is prominently displayed throughout seventeen-year-old Emily Koesters’s home. And for good reason: at age four she was diagnosed with Schimke Immunoosseous Dysplasia (SIOD), which is a rare genetic, life-threatening dwarfism disease with an average life expectancy of nine years old. The disease is so uncommon, Emily is only one of about nine children in the country and about 50 worldwide who are currently known to have it. But despite surviving a kidney transplant, two occurrences of lymphoma, and undergoing 55 surgeries, Emily soldiers on with unwavering faith, a positive attitude, and the desire to constantly help others.
That passion to believe is evident with Emily’s family too. Her parents, Erin and Joe, and her younger sister, Taylor Jo, all fight for Emily’s continued survival as well as the survival of other SIOD patients. It’s been a journey that started from the day Emily was born. She experienced numerous developmental difficulties as a newborn and toddler that were all thought to be a result of her having been born eight weeks prematurely. It wasn’t until her kidneys started failing and Joe donated one of his kidneys for a transplant that the Koesters realized there was more to it than just having been a preemie.
Even though the transplant was considered a success, Emily spent the next two and a half years at the University of Nebraska Medical Center fighting one battle after another, including lymphoma, twice. After conducting genetic testing, the SIOD diagnosis was finally discovered in 2009. “It wasn’t the answer we were looking for, but at least it was an answer,” Erin said. Symptoms of the disease include kidney failure, stroke, infection, all of which Emily has had.
Classified under the Spondyloepiphyseal dysplasia-SED type of dwarfism, yet Emily’s rare genetic dwarfism disease suggests it is a type of SED all its own. The Koesters connected with a family in Tulsa, OK, whose five-year-old son also had SIOD. That family had founded a non-profit called Little Giants, and the Koesters quickly became involved with fundraising efforts. Shortly after, the family’s son passed away and it was difficult for them to keep running the Foundation. So, in 2014 the Koesters took over Little Giants.
“We didn’t want to see it go away,” Erin said. “We wanted to carry on the mission, raise awareness, and have Emily become the new face and voice of SIOD.” With so few cases and many doctors not even aware of the disease, it seemed like a huge undertaking. But the Koesters found a key advocate who had been following Emily for years via another geneticist—a researcher at Stanford University, Dr. David Lewis, dedicated to understanding SIOD. Erin said his work is not only leading to treatments for SIOD, but it’s also helping people with other immune-suppressive diseases. Nearly every dollar raised by Little Giants goes toward that ground-breaking research. “Dr. Lewis and his team have a photo of Emily in their lab so they know who they are helping, and they truly care,” Erin added.
When a child is diagnosed with SIOD, they often find their way to Erin and Little Giants through an online search. She directs them to Dr. Lewis, acting as a liaison between the family and the research lab at Stanford. She also serves as a personal connection and open line of communication to support the family. “I’m always trying to grow and build and help more families,” she said.
Little Giants holds several fundraisers throughout the year. This fall was the 7th Annual Little Giants Farm Run. Even though Covid-19 forced the run to be virtual, it still raised almost $5,000. Every July Little Giants holds a blood drive, which honors Emily as the Gretna Hometown Hero. For every pint of blood that is donated, the Nebraska Community Blood Bank gives money back to Little Giants. Former Governor Heineman declared February 10th as Little Giants Day, and Erin hopes to hold a “Share Your Heart” event in honor of that as well. Although Covid has put a damper on many events, Erin said it’s also had its blessings. “It’s really opened up opportunities for what we can do. Every year we try to do something new anyway, so this is no different.”
Paying it forward is a big part of the Foundation’s mission. Emily’s “comfort pillows” are a passion project for the family. Her grandmother from Iowa sews pillows and their covers that they donate to Children’s Hospital and Medical Center, UNMC, and Madonna Rehabilitation Hospital so patients can use them for physical and emotional support. They send them to other SIOD patients and hope to offer them to senior living centers for Alzheimer’s patients as well. “It’s our way to share love,” Erin said.
Emily has touched the lives of so many with her story, including some celebrities. Nebraska comedian Larry the Cable Guy sponsored the run one year and visited Emily at her house. Country band Sugarland also fixed and autographed Emily’s guitar. “It stinks [having this disease] but I got to meet Rodney Atkins last year,” Emily said. She also got to pet a cheetah, which was a thrill for someone who is a large cat lover.
Erin admitted that sometimes she forgets how foreign the disease is to everyone else because they live it every single day. But she never tires of sharing Emily’s story because it may inspire someone. “Her just being here is amazing,” she said. “She’s so positive, and her resiliency is amazing. Her experiences teach others, not just about the disease but about being a warrior. She’s very humble as well. Emily is doing great today, but it’s been a rough journey.”
Emily has already defied all odds with her incredible survival, and the Koesters stress living your life to the fullest capacity each and every moment. “Even though only nine children have SIOD in the country, that’s still one too many,” Erin added. Every dollar that Little Giants raises is one step closer to prolonging Emily’s life. And that alone is worth believing in.