The Dystrophic Epidermolysis Bullosa Research Association of America (Debra) describes EB as “The Worst Disease You’ve Never Heard Of”. It’s a painful disease without a cure and it’s rarity makes it unknown to many.
EB is a connective tissue disease in which the layers of the skin are not held together and are often separating. There is no cure. People with EB often wear bandages to keep the wounds on their skin protected and clean and avoid causing any further trauma. The wounds and blisters are very painful, especially when their dressings need to be changed despite the avoidance of adhesives. Kids with EB are often called “Butterfly Children” because their skin is as fragile as a butterfly’s wing.
Far more is involved with EB than one might expect. The sores aren’t limited to the outside of the body. People with EB can develop sores on the inside of their mouths and in the lining of their throat among other places. This can make eating and simply getting enough nutrients a difficult and painful experience. Malnutrition is a fairly common complication of EB and many people get feeding tubes to supplement their diet.
Another complication is the formation of contractures in the hands. Careful wrapping of bandages between fingers could help prevent this, but for some, the use of their hands becomes limited and hands can regress post correctional surgery.
There are different types and sub-types of EB and they range in severity. Sometimes a lot of bandaging isn’t necessary, and sometimes a wheelchair is. Take a look at the video by Debra below to better understand the courage necessary for facing the day for EB kids.