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Children's Health Q3 2022

Difficulties children are facing to manage a rare, debilitating skin disease

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Paid for by Amryt Pharma

Dr Joe Wiley

Founder and CEO, Amryt Pharma

Jimmy Fearon

CEO, Debra Ireland

Epidermolysis Bullosa (EB) is one of about 7,000 rare diseases. EB is a rare and distressing genetic skin disorder affecting young children and adults, and there is no cure.

Children born with EB are often called ‘Butterfly Children’ because their skin seems as fragile as a butterfly wing. In Ireland, there are about 300 patients, of whom about 90 have the two more severe forms of the disease.

Unseen effects

The condition causes the skin to become fragile and tear or blister at the slightest touch. Patients with severe forms of EB suffer from chronic blistering, scarring of the skin, mutilating of the hands and feet, joint contractures, strictures of the oesophagus and mucous membranes and are at high risk of developing infections and aggressive squamous cell carcinomas, which can result in a risk of premature death. 

Patient Care

Children often go through painful bandage changes which can take up to three hours every other day. Jimmy Fearon, the CEO of DEBRA Ireland, the charity that supports children and families in Ireland affected by EB, says: “The impact of EB on a family is devastating. The trauma is both physical and psychological.”

Founder and Chief Executive of Irish biotech firm Amryt Pharma, Dr Joe Wiley says: “EB patients can require opiates to get through bandage changes, so what we want to do is to reduce wound burden as much as possible.”

DEBRA Ireland is a family and patient-led organisation. They provide practical support and grants, advocate on behalf of families and invest in research.

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