“Epidermolysis bullosa (EB) is a rare genetic skin fragility disorder and the majority of patients live with life-long wounding. EB is not specific to any racial group and those affected by the disorder can be found all over the world. EB describes a group of rare, genetically inherited skin fragility disorders. Inheritance is either autosomal recessive or dominant, with the more severe forms being recessively inherited. The common presentation in all types of EB is the tendency for skin and mucous membranes to blister or shear away in response to minimal everyday friction and trauma. In severe forms, there is a high risk of squamous cell carcinoma (SCC). Regular monitoring is essential with a low threshold for biopsy of suspect areas.
There are four categories of Epidermolysis; EB simplex (EBS), junctional EB (JEB), DEB and Kindler syndrome. The severity of EB varies from simple blistering affecting the hands and feet,particularly in warm weather, to death in early infancy from the devastating combination of laryngeal disease and failure to thrive.”
Wound Management is an integral element of the care of those who have Epidermolysis Bullosa. Nurses must ensure they are adept in the handling of these patients and in particular the specific wound management practices that support wound healing in these patients. Recently Wounds International has published the Development of best clinical practice guidelines for epidermolysis bullosa which in part includes the following information of interest to Nurses.
Blister Management for patients with Epidermolysiss Bullossa
“‘Blisters are not self-limiting and will fill with serous fluid and rapidly expand if left intact. In contrast to recommendations for other dermatological conditions or wound management, intact blisters should be lanced at their lowest point to limit tissue damage (Denyer, 2010). A sterile hypodermic needle should be used and, should be passed through the blister roof, parallel to the skin, to create an entry and exit hole through which fluid can be expelled. A soft gauze, can be used to gently compress the blister to encourage complete emptying.
Some patients advocate using sterilised scissors to create a larger hole to prevent the blister from refilling. The roof should be left on the blister unless patient preference is to de-roof it to prevent
refilling, but de-roofing can lead to additional pain,due to exposed nerve endings and should be discouraged if possible.
Examples of Key Recommendations
- People with EB and their carers are experts in the management of their condition and their involvement is paramount
- In severe EB the individual’s ability to heal can be compromised by malnutrition, anaemia, pruritus and pain, and should be /treated appropriately
- Atraumatic dressings should be used to prevent further blistering, skin and wound bed damage
- Silicone medical adhesive removers (SMARS) should be used when removing adherent dressings or clothing
- Care must be taken to ensure dressings do not slip and adhere to the clothing or bedding leading additional damage to the fragile skin or open wounds
- The retaining bandage or tape can also lead to additional blistering from movement or contact with the surrounding skin.
- Staff caring for EB patients must be trained in specific handling techniques to avoid further harm
- Management of EB wounds must address issues such as critical colonisation, infection, and protection from trauma
- Every effort should be made to treat the intense pruritus seen in EB and thereby minimise scratching that leads to further skin damage
- Optimal pain management is vital for patients with all forms of EB and include pharmacological and non-pharmacological interventions
Wounds are often large and dressings are often extensive and maximum sizes must be sought in order to avoid blistering where two smaller dressings join. The chosen dressing should be appropriate to the level of exudate and should provide protection by absorbing exudate and holding the moisture within the dressing to avoid maceration and leakage. The most commonly used dressing in EB are those designed to be removed easily, such as those coated with soft silicone, foam or mesh, lipido-colloid and polymeric membrane. Many people with EB will change their dressings either daily or alternate days with frequent ‘patch ups’ and frequency of dressing changes will dictated by the patient. Changing of the dressings is part of their personal hygiene regime (akin to a daily shower), rather than being based on the recommended wear time of the dressings.”
Often wounds are chronic and due consideration must be given to the management of odour, biofilm and care and preservation of the periwound area.
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