Soul Cycle, an indoor bicycling track in Culver City is gearing up for a fundraiser to aid in the development of treatments for Nemaline Myopathy (NM) on March 13 at 2:30 p.m.
At the event, riders, who were asked to raise a minimum of $500 will ride bikes around the track for 45 minutes. This effort is meant to not only raise money for treatments, but to spread awareness of a disease which has stricken Liv, the 4-year-old daughter of the event’s founder, Sharon Shimanovsky.
Shimanovsky said that last year, the event raised about $80,000. She hopes that this year’s fundraiser will do at least the same. So far, as of this writing, about $53,000 has been raised.
With the help of a scientific advisory board, Shimanovsky, through her non-profit organization, A Foundation Building Strength (AFBS), channels all money raised into the most promising medical research projects at various universities designed to help Liv and other victims of NM.
Research laboratories at Harvard, the University of Arizona, and the Medical college of Wisconsin, all receive funding through AFBS.
Since its inception in 2009, AFBS has funded 22 studies and awarded $1.5 million to scientists. AFBS funded the first Standard of Care paper for NM patients and currently is raising money to fund groundbreaking gene therapy and gene editing studies.
In order to ensure that every dollar raised go directly to research funding, the members of the AFBS board take no salary and cover most administrative costs.
Two of the researchers, Henk Granzier of the University of Arizona and Dr. Jeffrey Chamberlain of the University of Washington’s School of Medicine, are working on a treatment for Duchenne muscular dystrophy and were able to successfully treat a mouse by taking the gene that causes muscular dystrophy and making it smaller, creating a less severe form of the disease. Shimanovsky is hoping he can do the same thing with her daughter’s NM.
Another use of the money would be to fund a natural history study which would create a timeline of the progression of NM.
Nemaline Myopathy (NM) is a disorder often caused by a genetic mutation that primarily affects skeletal muscles, which the body uses for movement. People with NM have muscle weakness throughout the body, but it is typically most severe in the muscles of the face, neck, and limbs. It is a disease that can worsen over time.
Liv’s development of NM began shortly after birth when Shimanovsky noticed her daughter’s problems with eating as Liv would choke and gag every time she was fed. As Liv’s eating problems did not improve, she was hospitalized and given a battery of tests, all of which came back normal, but Shimanovsky was not convinced as Liv was unable to perform gross motor skills such as lifting her head and rolling over.
Matters only worsened when Liv would contract common colds. She often found herself so congested that she couldn’t breathe. The problem was that the NM weakened Liv’s muscles so much, that they weren’t strong enough to force the mucus out of her lungs.
More tests followed but the results were still normal. It wasn’t until after a feeding tube was placed in Liv’s stomach at seven months old that Shimanovsky and her husband received the news that Liv had been diagnosed with NM.
Currently, Liv is doing well with daily speech and physical therapy sessions. She has a constant aide with her at school and while she is completely dependent on adults as she cannot crawl, stand, walk or move on her own, she is described by Shimanovsky as a happy little girl who is extremely bright, very social, and wants to do what her classmates all take for granted.
“She had a rough first half of the year because she started school they are very cautious about germs because her respiratory muscles are compromised so when she gets sick its very dangerous,” Shimanovsky said. “She had three pneumonias and hospitalizations within the first three months of school but she is feeling much better.”
Although there is no treatment or cure yet, Shimanovsky is confident that her efforts with AFBS and her annual fundraising events will bring greater awareness to NM and more financial support to medical researchers.
Shimanovsky believes that when more people are made aware of the disease, it will be that much easier to find treatments and eventually a cure.
“For me, being able to think that there might be treatments that will make her life better is what gives us hope and keeps us going,” Shimanovsky said.
Finding a treatment and eventally a cure for Liv is the driving force for everything that Shimanovsky has done and continues to do so that Liv can continue being the happy little girl who loves playing with Legos, singing with grandpa, dancing, anything purple, and ice cream.
“Watching your child suffer is torture and I spend nearly every second of every minute of every hour of every day worrying about Liv’s future and how I can better it,” Shimanovsky said. “It is my mission to better Liv’s life, and those living with NM through meaningful research that leads to treatment.”
Anyone who would like to donate to the Soul Cycle ride on March 13, can do so at www.loveliv.org/donate.
