Life with Epidermolysis Bullosa

Epidermolysis Bullosa is a debilitating and devastating genetic disorder that affects a child from birth. EB is extremely rare - a child has 1 in 50,000 chance of being born with EB - and EB is not specific to any ethnicity or gender. EB is technically defined as a group of diseases characterized by blister formation after minor trauma to the skin. Practically speaking, a child who suffers from EB lacks a critical protein that binds his or her layers of skin together. This protein acts as the "velcro" that attaches one layer of his or her skin to the other. Without this "velcro," when this child moves, his or her skin slides apart, blisters and shears off leading to severe pain, disfigurement, and in too many cases, a premature death. A child with a severe form of EB can have a 60% cumulative risk of dying by age 15 and nearly a 100% chance of developing an aggressive, painful and fatal form of skin cancer over the course of his or her shortened life. There are three major EB subtypes - Simplex, Junctional and Dystrophic and within each type there are multiple subsets. The difference among them is the level at which a blister forms within the skin and which particular protein is missing or impaired. Life with EBrn rnTo say that EB impacts every aspect of a child's life is a gross rnunderstatement. Skin is the body's largest organ. Among its most important functions, skin is the first line of defense to protect the body from trauma and infection. Everything we do in life impacts our skin - walking, eating, playing, sitting, writing, hugging, sleeping - the list goes on. For children with EB, every aspect of their lives at every moment is overshadowed by this terrible disorder. These children are often born missing large areas of skin leaving gaping wounds that never heal; walking and standing are impaired over time because their toes become fused as the result of continuous injury; the simple joy of holding a crayon to draw becomes impossible because their fingers fuse and contract, turning their young hands into mittens. During a typical day, a child with EB undergoes a special bath and bandage change. Given the large areas of skin that can be missing from such a child's body, bathing is an extraordinarily painful experience. Bandage changes can last anywhere from 30 minutes to several hours and bandages can cost a family as much as an astounding $14,000 per month. Treatment and Cures for EBrnThe Jackson Gabriel Silver Foundation was founded with the mission to find treatments and cures for Epidermolysis Bullosa ("EB"), a group of incurable and devastating blistering disorders that affect children from birth. The Silver family started JGSF in 2010 to find a cure for their son, Jackson, and all other children who suffer from this terrible disease. By funding scientific research, JGSF can help children with EB who lack the critical proteins that act like velcro, binding their layers of skin together. When these children move, their skin slides apart, blisters and shears off, leading to severe pain, disfigurement and, in too many cases, a premature death. rnFor More Details and the latest update on Jackson, Visit http://www.jgsf.org