Long Island Roller Rebels, the region’s longest-running flat-track roller derby team, hosted its annual charity game on Saturday, Nov. 4, to raise money for the Wiedemann-Steiner Syndrome (WSS) Foundation.
(Photo credit: Seth Berman from Rapid Shutter Photography)
According to the foundation, Wiedemann-Steiner Syndrome, or WSS, is a rare genetic disorder resulting from mutations in the MLL (also known as the KMT2A) gene on the long arm of chromosome 11. The syndrome was clinically described in 1989, but was not genetically identified until 2012 by a group of researchers in England led by Dr. Wendy Jones. The gene encodes a histone-modification enzyme — that is, it helps modify the expression of other genes. The condition is autosomal dominant, meaning that only one abnormal copy of the gene is needed for a person to have the syndrome. In a majority of cases to date, the mutation occurred de novo — meaning that neither parent was affected and the mutation is sporadic. Offspring of those with WSS have a 50 percent chance of having WSS.
Weidemann-Steiner Syndrome may be related to global developmental delays, sleeping difficulties, feeding and digestion complexities, unusual facial features, short / petite stature, hypotonia, dental issues, excessive body hair, long eyelashes, and more. Those affected with Weidemann-Steiner Syndrome often receive physical, occupational, speech, feeding, and / or behavioral therapies. Hippotherapy and music therapy have also been helpful to those affected by WSS. School-aged children affected with WSS may benefit from one-on-one aides, modified instruction, and / or special day environments.
Medical researchers actively involved with WSS have projected that only a few hundred patients have been documented worldwide. Because this is such a new syndrome and because there have been so few diagnoses, anecdotal evidence suggests that awareness of WSS is generally quite low, even amongst medical professionals.
There is limited diagnostic testing in this area. The standard screening tests that take place during pregnancy that can diagnose syndromes such as Down Syndrome, don’t diagnose WSS. In addition, baseline genetics diagnostic tests conducted after birth don’t include testing for WSS. Frequently, patients are given other incorrect medical explanations or a less specific and broader diagnosis, like autism and Rubenstein-Taybi Syndrome.
The fundraiser took place at Skate Safe America in Old Bethpage, where the Roller Rebels were cheered on by family, friends and community members. The Rebels were divided up into a White Team and a Black Team to face off. “Mixers are a lot of fun because we all get to intermingle… It’s not necessarily an ‘us’ versus ‘them’ mentality,” explained teammate Indy “Persephindy” Johnson. “It’s a very relaxed game.” Also present were several local small businesses showing their support for the cause. The WSS Foundation was present to give out fliers and informational packets to curious audience members. The halftime show was performed by Sharp Violet, a Long Island Rock band, and also featured a wheel toss and 50/50 raffle.
The charity chosen varies from year to year, and is chosen by the skaters and sometimes their fans. “There’s a week where everyone can nominate different charities and then we choose one,” said Johnson. But this year’s choice was especially special because it hit so close to home. “This year the charity means a lot to us. One of our skaters, her son was recently diagnosed. So doing this for him is very special.”
In the end, the Black team dominated with a 20-point lead. However, fun was had by all, and the Roller Rebels were thrilled to raise so much for a great cause.
About Long Island Roller Rebels
Long Island Roller Rebels is Long Island’s first flat-track roller derby league and a member of the Women’s Flat Track Roller Derby Association (WFTDA), the sport’s foremost governing body with more than 360 member leagues. The league is run by the skaters and other non-skating members. Day-to-day operations are handled by elected officers, long-term operations are handled by a board of directors, and specialized committee leaders are all chosen by a formal vote. Funding comes from skater dues, fundraising events, ticket sales, and a wide array of sponsorships. The league was founded in 2005 and received non-profit 501(c)(3) status on March 7, 2016. Learn more at longislandrollerrebels.org, and follow us on Facebook and Instagram.
About the WSS Foundation
The WSS Foundation is a non-profit organization that provides education, fosters community, and stimulates research to improve the lives of everyone impacted by WSS. Wiedemann-Steiner Syndrome is a rare genetic disorder related to developmental delays, sleeping difficulties, feeding and digestion complexities, unusual facial features, short/petite stature, hypotonia, dental issues, hairy elbows, long eyelashes, etc. A lack of public information means that there are many people who have WSS and are undiagnosed. Learn more at wssfoundation.org.
—Additional information provided by WSS Foundation
