113 Care of the new-born with epidermolysis bullosa

Abstract

Background What is EB: Epidermolysis Bullosa (EB) is a rare group of genetic skin conditions which cause the skin to blister at the slightest touch. There are three main types of EB, and within each type are various subtypes ranging from mild to severe. The cause of each type lies in the absence of specific proteins which maintain skin integrity. These proteins can also present in the internal linings of the body which can lead to early infantile death in its severest form. Babies can also present with widespread skin loss at birth, leading to challenges in management in the neonatal unit. Previous experience of EB neonatal care is normally limited, therefore the GOSH EB nursing team offer an outreach service to deliver specialist care and support. Aims During the first visit, the GOSH EB nurses will perform a biopsy to determine the type of EB the baby has and complete the first dressing change. Specialist dressings are used that do not adhere to the skin and have anti-microbial properties. Blisters as EB are not self-limiting, therefore parents are taught how to manage this to decrease the risk of them spreading/enlarging. Web spaces must be protected to prevent fusion digits. Advice will also be given regarding handling, feeding, bathing and nappy cares. Results Different types of EB present very differently, therefore care must be modified to minimize skin damage. Delivery of an infant with a severe type of EB is a shock for parents therefore additional psychological support may be required. Infants with EB benefit from multidisciplinary input at a specialised centre. However, transportation of affected infants risks further skin damage. Pain reduction at dressing changes is complex and requires constant evaluation and adjustment of analgesia. Conclusion The main goals of EB care are protection of vulnerable skin/chronic wounds, prevention/management of infection, minimising the risk of accidental damage and supporting optimal nutrition. An interdisciplinary approach is imperative in ensuring that patients receive the best possible care. Many EB complications can be lessened with early intervention and care, however, it is not possible to completely stop the progression of the disease.