Facioscapulohumeral dystrophy (FSHD) is one of the most common forms of inherited muscular dystrophy that produces muscle wasting and leads to profound disability. Age of onset is variable, but the most severe forms manifest as infantile and childhood conditions with accompanying hearing loss and vision problems. With limited treatment options there is a need for novel therapies that can potentially halt the progression of this debilitating genetic disease. We recently discovered that a specific signaling response in the muscle cells of FSHD patients can contribute to their deaths, which likely underlies the systemic progression of muscle wasting in FSHD.