In the simpler forms, the blisters healed without permanent damage. In more severe forms, there is a strong tendency to scarring, similar to a burn. These scars, which can also appear on the internal organs such as the esophagus, and the constant formation of blisters and open wounds lead to significant functional disorders and severe disability in some of those affected, for example due to the fingers and toes growing together.
Skin cancer is also a possible impending secondary disease that can ultimately be life-shortening.
EB is not contagious. Mental performance is not affected. Nevertheless, those affected often have to endure a lot of misunderstanding and ridicule from the ignorant environment.
EB is currently not considered curable. According to media reports, doctors succeeded years ago in transplanting new skin that had been genetically modified to make it healthy for a seven-year-old boy whose skin was 80 percent affected. According to the Neue Zürcher Zeitung, he can lead a normal life today. According to Debra Germany, skin cultivation is currently not permitted in Germany, and this external treatment does not yet help children like Mathilda, because, as her father says, “I can’t just transplant a new stomach lining.”
The need for research is huge, but because of the small number of cases, which does not result in a lucrative market for any drugs – there are currently up to 4,700 people affected in Germany and around 30,000 in Europe – the pharmaceutical industry has so far shown very little interest.
Because the disease is so rare, only a few doctors, nurses, emergency doctors and paramedics know about it. But for the person affected by EB, every band-aid, every toothbrush can mean terrible pain. That’s why Daniel Kenst is also concerned with making the disease better known. “Further training for medical staff would be very important for those affected,” he says.
So far there are only a few clinics that have specialized in EB patients. One of the leading international clinics is the EB Clinic in Salzburg. That was also the reason why Daniel Kenst and his family moved from Landshut to Freilassing, where they feel very well cared for and lovingly accepted, just like in Salzburg.
Interested doctors, nurses or paramedics can find guidelines on what to do and what not to do with an EB patient in an emergency at the EB house in Salzburg. There is an EB manual and an emergency card for the patients there, so that they are not in pain, for example by sticking on electrodes for an ECG, if the skin peels off with them.
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